MODERN MEDICIN E 
ITS THEORY AND PRACTICE 
IN ORIGINAL CONTRIBUTIONS BY AMERICAN AND 
FOREIGN AUTHORS 
EDITED BY 
SIR WILLIAM OSLER, Bart., M.D., F.R.S. 
REGIUS PROFESSOR OF MEDICINE IN OXFORD UNIVERSITY, ENGLAND; HONORARY PROFESSOR OF MEDICINE IN 
THE JOHNS HOPKINS UNIVERSITY, BALTIMORE; FORMERLY PROFESSOR OF CLINICAL MEDICINE IN 
THE UNIVERSITY OF PENNSYLVANIA, PHILADELPHIA, AND OF THE INSTITUTES OF MEDICINE 
IN MC GILL UNIVERSITY, MONTREAL, CANADA 
AND 
THOMAS McCRAE, M.D. 
PROFESSOR OF MEDICINE IN THE JEFFERSON MEDICAL COLLEGE, PHILADELPHIA; FELLOW OF THE ROYAL COLLEGE OF 
PHYSICIANS, LONDON; FORMERLY ASSOCIATE PROFESSOR OF MEDICINE, THE JOHNS HOPKINS UNIVERSITY 
VOLUME V 
DISEASES OF THE NERVOUS SYSTEM-DISEASES 
OF THE LOCOMOTOR SYSTEM 
SECOND EDITION, THOROUGHLY REVISED 
ILLUSTRATED 
LEA & FEBIGER 
PHILADELPHIA AND NEW YORK 
1915 Entered according to the Act of Congress, in the year 1915, by 
LEA & FEBIGER, 
in the Office of the Librarian of Congress. All rights reserved. CONTRIBUTORS. 
LEWELLYS F. BARKER, M.D., LL.D., 
Professor of Clinical Medicine in the Johns Hopkins University; Visiting 
Physician to the Johns Hopkins Hospital, Baltimore, Md. 
EDWIN BRAMWELL, M.B., F.R.C.P. (Edin, and Lond.), F.R.S. (Edin.), 
Assistant Physician to the Royal Infirmary and Lecturer on Medicine in the 
School of the Royal College, Edinburgh, Scotland. 
CHARLES W. BURR, M.D., 
Professor of Mental Diseases in the Medical Department of the University of 
Pennsylvania, Philadelphia. 
E. FARQUHAR BUZZARD, M.D., F.R.C.P. (Lond.), 
Out-Patient Physician to the National Hospital for the Paralyzed and Epileptic, 
and to St. Thomas' Hospital, London; Consulting Neurologist to the Royal 
Free Hospital, and to the Hospital for Diseases of the Throat, London, England. 
L. PIERCE CLARK, M.D., 
Formerly First Assistant Physician to the Craig Colony for Epileptics; Con- 
sulting Neurologist to the Manhattan State Hospital; Neurologist to 
Randall's Island Hospital; Chief Consultant of Letchworth Village for Mental 
Defectives. 
JOSEPH COLLINS, M.D., 
Physician to the Neurological Institute of New York, New York City. 
HARVEY CUSHING, M.D., 
Professor of Surgery in the Harvard University Medical School, Boston, Mass.; 
Formerly Associate Professor of Surgery in the Johns Hopkins University. 
GEORGE DOCK, M.D., 
Professor of Medicine in the Washington University Medical School, St. Louis, 
Missouri. 
* 
CHARLES P. EMERSON, M.D., 
Professor of Medicine in the Indiana University, School of Medicine, Indian- 
apolis, Ind.; Late Associate in Medicine and Resident Physician in the Johns 
Hopkins University and Hospital; Late Superintendent of the Clifton Springs 
Sanatorium. 
GORDON M. HOLMES, M.D.. M.R.C.P., 
Assistant Physician to the National Hospital for the Paralyzed and Epileptic, 
London; Physician to the Out-Patients, Seamen's Hospital, London, England. 
SMITH ELY JELLIFFE, A.M., M.D., Ph.D., 
Adjunct Professor of Diseases of the Mind and Nervous Svstem in the Post- 
Graduate Medical School and Hospital; Visiting Neurologist to the City 
Hospital; Consulting Neurologist to the Manhattan State Hospital, New York. 
daniel j. McCarthy, m.d.. 
Professor of Medical Jurisprudence in the Medical Department of the University 
of Pennsylvania; Neurologist to the Henry Phipps Institute and to the Phila- 
delphia and St. Agnes' Hospitals, Philadelphia. 
V VI 
CONTRIBUTORS 
THOMAS McCRAE, M.D., F.R.C.P. (Lond.), 
Professor of Medicine in the Jefferson Medical College, Philadelphia. 
COLIN K. RUSSEL, M.D., 
Lecturer in Clinical Neurology in McGill University, Montreal, Canada 
BERNARD SACHS, M.D., 
Alienist and Neurologist to Bellevue Hospital; Neurologist to the Mt. Sinai 
Hospital; Consulting Physician to the Manhattan State Hospital, New York. 
E. E. SOUTHARD, A.M., M.D., 
Bullard Professor of Neuropathology in the Medical School of Harvard Univer- 
sity, Boston, Mass.; Director of the Psychopathic Department of the Boston 
State Hospital; Pathologist to the State Board of Insanity, Massachusetts. 
WILLIAM G. SPILLER, M.D., 
Professor of Neuropathology and Associate Professor of Neurology in the Medical 
Department of the University of Pennsylvania, Philadelphia. 
WALTER R. STEINER, M.D., 
Visiting Physician to the Hartford Hospital; Consulting Physician to the Hart- 
ford Orphan Asylum; Consulting Physician to the Middlesex Hospital of 
Middletown, Conn. 
EDWARD W. TAYLOR, M.D., 
Assistant Professor of Neurology in the Medical School of Harvard University, 
Boston, Mass.; Chief of the Neurological Service in the Massachusetts Gen- 
eral Hospital, Boston, Mass. 
HENRY M. THOMAS, A.M., M.D., 
Clinical Professor of Neurology in the Johns Hopkins University; Neurologist 
to the Johns Hopkins Hospital and Dispensary, Baltimore, Md. CONTENTS. 
PART I. 
DISEASES OF THE NERVOUS SYSTEM. 
CHAPTER I. 
INTRODUCTION TO DISEASES OF THE NERVOUS SYSTEM . . 17 
By Lewellys F. Barker, M.D. 
CHAPTER II. 
DISEASES OF THE MOTOR TRACTS 80 
By William G. Spiller, M.D. 
CHAPTER III. 
THE COMBINED SYSTEM DISEASES OF THE SPINAL CORD . . 125 
By Colin K. Russel, M.D. 
CHAPTER IV. 
SCLEROSIS OF THE BRAIN 140 
By Edwin Bramwell, M.B., F.R.C.P. (Edin, and Lond.) 
CHAPTER V. 
THE DISEASES OF THE MENINGES 162 
By Edwin Bramwell, M.B., F.R.C.P. (Lond. and Edin.) 
CHAPTER VI. 
DIFFUSE AND FOCAL DISEASES OF THE SPINAL CORD . . 178 
By E. Farquhar Buzzard, M.D., F.R.C.P. (Lond.) 
CHAPTER VIL 
TOPICAL DIAGNOSIS OF DISEASE OF THE BRAIN 265 
By Joseph Collins, M.D. 
CHAPTER VIII. 
APHASIA 292 
By Joseph Collins, M.D. 
CHAPTER IX. 
TUMORS OF THE BRAIN AND MENINGES 308 
By Harvey Cushing, M.D. 
CHAPTER X. 
HYDROCEPHALUS 351 
By Harvey Cushing, M.D. 
VII VIII 
CONTENTS 
CHAPTER XI. 
ACUTE ENCEPHALITIS AND BRAIN ABSCESS . . . .359 
By E. E. Southard, M.D. 
CHAPTER Xil. 
DISEASES OF THE CEREBRAL BLOODVESSELS .387 
By Henry M. Thomas, A.M., M.D. 
CHAPTER XIII. 
DISEASES OF THE CEREBRAL NERVES 471 
By E. W. Taylor, M.D. 
CHAPTER XIV. 
DISEASES OF THE PERIPHERAL NERVES 533 
By Gordon M. Holmes, M.D., M.R.C.P. 
CHAPTER XV. 
EPILEPSY 592 
By L. Pierce Clark, M.D. 
CHAPTER XVI. 
NEURASTHENIA. THE TRAUMATIC NEUROSES AND PSYCHOSES 624 
By Charles W. Burr, M.D. 
CHAPTER XVII. 
HYSTERIA 654 
By Smith Ely Jelliffe, A.M., M.D. 
CHAPTER XVIII. 
MIGRAINES. NEURALGIA. PROFESSIONAL SPASMS. OCCUPA- 
TION NEUROSES. TETANY 709 
By Smith Ely Jelliffe, A.M., M.D. 
CHAPTER XIX. 
PARALYSIS AGITANS. CHOREA. CHOREIFORM AFFECTIONS. 
INFANTILE CONVULSIONS 764 
By Daniel J. McCarthy, M.D. 
CHAPTER XX. 
MYASTHENIA GRAVIS. PARAMYOCLONUS MULTIPLEX. PERI- 
ODIC PARALYSIS 798 
By Daniel J. McCarthy, M.D. 
CHAPTER XXL 
ASTASIA-AB ASI A. ADIPOSIS DOLOROSA 815 
By Daniel J. McCarthy, M.D. 
CHAPTER XXII. 
SYPHILITIC DISEASES OF THE CENTRAL NERVOUS SYSTEM . . 822 
By Bernard Sachs, M.D. 
CHAPTER XXIII. 
AMAUROTIC FAMILY IDIOCY (TAY-SACHS DISEASE) ... 863 
By Bernard Sachs. M.D. CONTENTS 
IX 
PART II. 
DISEASES OE THE LOCOMOTOR SYSTEM. 
CHAPTER XXIV. 
MYOSITIS-MYOTONIA CONGENITA: THOMSEN'S DISEASE . . .871 
By Walter R. Steiner, M.D. 
CHAPTER XXV. 
ARTHRITIS DEFORMANS 895 
By Thomas McCrae, M.D., F.R.C.P. (Lond.) 
CHAPTER XXVI. 
OSTEOMALACIA 947 
By George Dock, M.D. 
CHAPTER XXVII. 
ACHONDROPLASIA. HYPERTROPHIC PULMONARY OSTEO- 
ARTHROPATHY. PAGET'S DISEASE. OSTEOGENESIS IM- 
PERFECTA. OSTEOPSATHYROSIS. LEONTIASIS OSSEA. 
MICROCEPHALUS. FACIAL HEMIATROPHY 955 
By Charles P. Emerson, M.D. 
Index 993 
General Index 1029 DISEASES OF THE NERVOUS SYSTEM. 
CHAPTER I. 
INTRODUCTION TO DISEASES OF THE NERVOUS SYSTEM. 
By LEWELLYS F. BARKER, M.D., LL.D. 
THE SENSES AND THEIR SYMPTOMATOLOGY. 
It is convenient to divide the senses into two groups, interior and 
exterior. The interior senses are those of which we project the sensations 
into some part of our own bodies. They include pain, movement sense, 
position sense, resistance sense, the vestibular sense, hunger, thirst, the 
sexual sense, and the sense of fatigue. The exterior senses are those of 
which we project the sensations into the world outside ourselves. They 
include sight, hearing, taste, smell, touch and pressure, and the two 
temperature senses, heat and cold. 
The Interior Senses.-Pain.-Physiologists now regard this as a 
special sense, and not simply as a negative feeling tone associated with 
overstimulation of other sense organs (von Frey). This pain sense has 
a punctiform distribution in the skin. As tested by very fine needles, 
the points are more numerous even than the touch points. The strength 
of the stimulus required to call forth a sense of pain is called the thresh- 
old stimulus, and this varies for different pain points. The localization 
of pain sensations is due less to the pain nerves than to the simultaneous 
stimulation of the sense of touch. 
Visceral pain is often not localized in the viscera themselves, but is 
referred to certain areas in the skin (Head). The cutaneous hyper- 
esthesias in cardiac, gastric, intestinal, and prostatic diseases are examples 
of such mis-reference. The pain sense of the skin and viscera has been 
designated by Head and Rivers as protopathic sensibility, in contrast 
with touch and temperature senses, which they call epicritic sensibility. 
Sometimes the peripheral nerves are oversensitive to pressure, par- 
ticularly at places where they emerge from bony openings (Valleix's 
points). 
Deep or Mesoblastic Sensibility. Sensations of Movement of Parts of 
the Body; of Their Position, and of Resistance. Bathyesthesia.-Under the 
term "muscle sense," a whole series of different sensations have been 
roughly lumped together. This is due largely to the lack of sharpness 
of these sensations in consciousness. We must distinguish at least the 
following: (1) The sense of position of the individual parts of the body; 
17 18 
DISEASES OF THE NERVOUS SYSTEM 
(2) the sense of movements of these parts; (3) sensations of resistance 
and weight; (4) the stereognostic sense; (5) the so-called vibration sense. 
1. Sense of Position of Individual Parts of the Body.-The normal 
person is able actively to imitate with one-half of his body positions in 
which portions of the other half are passively placed. 
2. Sense of Movement of Individual Parts of the Body.-When the eyes 
are closed and active or passive movements of a part of the body are 
made, a normal person is able to state (a) that a movement has occurred, 
(6) the degree of this movement, and (c) its direction. This sense of 
movement differs from the sense of position. In normal individuals any 
movement that the examiner himself can see should be recognized. 
When a joint has to be flexed or extended through several degrees before 
a sensation is detected, the movement sense is subacute. It should be 
remembered that smaller movements can be recognized in the shoulder 
and wrist than in the knee, hip, or elbow. The ankle-joint and the joints 
of the fingers and toes are still less sensitive. In right-handed individuals 
the movement sense is more delicate on the right side than on the left. 
3. Sensations of Resistance and of Weight.-The sense of weight is not 
identical with that of resistance and is more delicate in the upper than in 
the lower extremities. 
4. Stereognostic Sense or Perception.-The capacity to recognize the 
form and consistence of objects by touching and feeling them is known 
as stereognosis. This capacity involves the combined activities of the 
movement sense, resistance sense, position sense, sense of touch, and 
sense of temperature. Even more, certain higher cortical faculties are 
involved, including the spatial and temporal arrangement of the sense 
impressions and the memory pictures of previous sensations. Obviously, 
we have to do here not simply with sensation, but with a combination 
of sensory, associative, and reproductive processes. 
5. Vibration Sense.-Rapidly vibrating objects when placed upon 
the skin over a bone yield a peculiar vibrating sensation known as pal- 
lesthesia. Clinically, one examines the sensation best by placing the 
handle of a vibrating tuning-fork of 64 or 128 oscillations per second 
firmly upon the surface of the skin over one of the bones. 
Vestibular and Allied Senses. Sense of Static Equilibrium. Sensations of 
Position of the Body as a Whole. Sensations of Movement of the Body as a 
Whole. Sensations of Orientation in Terrestrial Space. Sensibility to 
Accelerations in a Straight Line and to Angular Accelerations.-Here we 
have to deal with a great group of centripetal impulses which are of 
high importance for the functions of equilibrium, locomotion, and orien- 
tation. It seems likely that most of the centripetal impulses concerned 
are infraconscious, or that their psychic correlate is relatively insignificant. 
The directions up and down are recognized by everybody, though 
how the force of gravity is made known to us is not clear. Even under 
water the capacity for orientation as to the vertical direction is unim- 
paired. The mechanism, whatever it is, is sensitive to accelerations in a 
straight line, and gives rise not only to our knowledge of the vertical 
direction, but also to sensations of progressive or non-rotary movements. 
In addition to this mechanism, there is another apparatus in the head INTRODUCTION TO DISEASES OF THE NERVOUS SYSTEM 
19 
which is sensitive to angular accelerations and gives ns sensations of 
change of velocity of rotary movements. These sensations, which follow 
accelerations in a straight line and angular accelerations, are associated 
with characteristic eye-muscle reflexes. An alteration of the position 
of the head relative to the vertical causes a compensatory change in the 
setting of the eyeballs, and if the body be rotated around its vertical 
axis the eyes show movements of nystagmus as long as the sensations 
of rotation are felt. 
These sensations are connected with the functions of the membranous 
labyrinth of the internal ear. It seems probable that the information 
which the brain receives regarding the position of the head when it is at 
rest and regarding the acceleration or retardation of progressive (non- 
rotary) movements depends upon the nerve endings in the utricle and 
saccule, upon the maculae of which the little ear-stones or otoconia rest 
(Breuer), while the sense organ underlying the sensations dependent upon 
angular accelerations appears to be situated in the ampullary crests of 
the membranous ampullae of the semicircular ducts. While both the 
utricle and saccule, on the one hand, and the semicircular ducts, on the 
other, appear to have to do with static as well as dynamic sensations, 
it seems probable that the static are mediated rather more by the former, 
the dynamic rather more by the latter. The loss of orientation as to the 
vertical when under water and the absence of vertigo on rotation so often 
seen in deaf mutes are thought to be due either to imperfection of the 
otoconic apparatus or of the semicircular ducts. 
Other Interior Senses.-Hunger.-In its mild form this sensation is 
familiar to everyone as the appetite for food localized in the gastric region. 
The hunger sense is to some extent dependent upon metabolism. The 
well-known increase of appetite after muscular exertion, after exposure 
to cold, and in diabetes mellitus makes this clear. On fasting, the sense 
of hunger soon disappears, in marked contrast with the sense of thirst. 
Thirst.-The sense of thirst is referred to the back of the throat, and if 
no water be taken the sensations become painful. 
Sense of Fatigue.-If the muscles are made to contract beyond a 
certain point, a feeling of fatigue is encountered. Whether this is due to 
centripetal impulses arising in the muscles themselves or to a change 
in the nerve centres in the cerebral cortex is not certain. 
Sexual Sense.-The impulse to sexual gratification is technically 
known as libido. The sexual impulse of adults appears to be a unifica- 
tion of various childhood impulses; these culminate normally in an 
effort toward a single specific goal. Originally auto-erotic, the impulses 
become later hetero-erotic. 
The Exterior Senses.-Touch or Pressure.-The skin contains sensory 
mechanisms that permit us to recognize very slight contact and very 
light degrees of pressure. The sense organs are closely related to the 
hairs in parts of the body which possess these and to Meissner's corpuscles 
in other parts. When delicate stimuli are employed it is found that 
sensations are set up only when definite points are pressed upon. These 
are the so-called touch points of von Frey. They are much more numer- 
ous than cold points and warm points, but are less numerous than pain 20 
DISEASES OF THE NERVOUS SYSTEM 
points. They are very numerous on the lips, tongue, and finger tips, and 
are relatively much scantier on the leg and upper arm. Touch sensations 
following brief stimuli last only a short time, and if stimuli be repeated 
rapidly one receives a corresponding number of sensations without fusion 
in consciousness. The threshold stimulus for individual touch points 
varies. One can determine the threshold most easily by the use of von 
Frey's test hairs. 
No marked feeling tone accompanies touch sensations, a fact which 
distinguishes them sharply from sensations of pain. The time elapsing 
from the moment of application of the stimulus to a touch organ to the 
moment of appearance of sensation in consciousness is shorter for touch 
than for temperature or pain (reaction time). Touch sensations can be very 
accurately localized upon the surface of the body; indeed, the sense of 
touch, together with sight and movement sense, has done most to give 
to man his conceptions of spatial relations. Each touch point on the 
surface of the body seems to have its own "local sign," that is, the sensa- 
tion from it can be distinguished from that from every other. If two 
touch points are close together, however, they must be stimulated suc- 
cessively and not simultaneously if the two separate sensations are to 
be recognized (successive threshold of von Frey). When touch points are 
a certain distance apart, stimulation of them simultaneously gives rise 
to two separate sensations (simultaneous duplicity threshold of von Frey; 
space threshold of Weber). 
Tickling and Itching.-These are as yet not well understood. Tickling 
is probably to be separated physiologically from itching. However, 
both are closely related to touch sense and pain sense. It is possible 
that they should be regarded as secondary sensations rather than primary 
(II. Quincke), and that they are associated with vasomotor reflexes. It 
is interesting that the sensations are aroused by slight stimuli and can 
often be inhibited by more powerfid stimulation in the same areas. 
The Temperature Sense.-In the skin and mucous membranes there are 
at least three varieties of sensory nerve beginnings which respond to 
thermal stimuli; they are connected with the so-called cold points, warm 
points, and pain points. The adequate stimulus for a cold point is cold, 
that for a warm point is warmth, but both sets of points react to various 
disparate stimuli also; thus one gets a sensation of cold when he stimu- 
lates a cold point by a sharpened stick, by the application of menthol, 
or by an electrical current, and a warm point can be stimulated not 
only by heat, but by mechanical irritation with fur, by exposure to 
carbon dioxide, or by electricity. Pain points are stimulated by cold 
below a certain temperature or by heat above a certain temperature. 
Touch points appear to be uninfluenced by thermal stimuli. The cold 
points are far more numerous than the warm points, but are less evenly 
distributed than the touch points and the pain points. 
Natural stimuli, as a rule, affect areas of the skin which contain a 
number of sensory points, so that our ordinary cutaneous sensations are 
complexes made up of a number and variety of elementary sensations. 
When the thermal stimulus corresponds in temperature to that of the 
skin no sensation at all is felt; if it correspond to a temperature slightly INTRODUCTION TO DISEASES OF THE NERVOUS SYSTEM 
21 
lower than that of the skin, the cold points alone respond and a sensation 
of coolness results; if it corresponds to a temperature a little above that 
of the skin, a sensation of warmth is felt. Very low temperatures affect 
both the pain points and the cold points; very high temperatures may 
affect the warm points, the cold points, and the pain points simultan- 
eously, and yield a mixed sensation compounded of warmth, cold, and 
pain, usually designated " burning hot." 
Taste.-There are four simple elementary taste qualities: sour, sweet, 
salty, and bitter. All other so-called taste sensations are either com- 
binations of these or are mixtures of taste sensations with sensations of 
smell, touch, temperature, or pain. Some taste sensations are associated 
with pleasurable feeling tone, others with negative feeling tone. Sub- 
stances which can be tasted are known as "sapid substances," and are 
soluble in water or in saliva. They are crystalloid in nature, colloids 
being devoid of taste. 
Apparently there are entirely different sense organs for sour, sweet, 
salty, and bitter sensations. The adequate stimulus for arousing sour 
sensations appears to be the hydrogen ion of acids. Xo common chemical 
character has as yet been ascribed to the sapid substances which arouse 
sweet sensations; salty sensations are best aroused by a 2 per cent, solu- 
tion of sodium chloride, though various other salts can stimulate the sense 
organ concerned. Bitter sensations are aroused by very different chemi- 
cal substances. Many of the alkaloids (strychnine) are bitter, but num- 
erous sapid substances are bitter which are chemically unrelated to these 
(e. g., magnesium sulphate). The taste organs thus acted upon by chemi- 
cal stimuli do not respond to mechanical or thermal stimuli. They do, 
however, sometimes respond to electric currents (so-called electric taste}. 
Single taste modalities are variably represented in various parts of the 
tongue; thus, sweet taste is best represented at the tip, salty at the tip 
and margins, sour at the margins, and bitter at the posterior part of the 
dorsum linguae. The centripetal nerves of taste leave the tongue by 
way of the lingual branch of the trigeminal nerve and by way of the 
glossopharyngeal nerve. The few taste buds of the larynx belong to the 
pneumogastric nerve. 
Smell.-This is closely related to taste sense, and naive observers 
often confound sensations of smell with those of taste. The nose possesses 
touch sense, temperature sense, and pain sense; when we inhale forma- 
lin, it is the pain sense which is chiefly aroused, and when we inhale 
menthol the temperature sense is also affected. Aside from distinguishing 
sensations of smell proper from other modalities of sensation, we have 
the still greater difficulty of analyzing olfactory sensations among them- 
selves and resolving them into their simpler psychological constituents. 
The olfactory sensations are mediated by the neuro-epithelial cells in 
the olfactory region high up in the nose. 
Smell is to be regarded as a chemical sense, and the adequate stimuli 
are chemical substances which act directly upon the peripheral olfactory 
neurones. Very minute amounts of certain chemical substances are 
sufficient to stimulate the sense organ, and they must be gaseous or 
volatile in order that they may mix with the air that enters the cavity 22 
DISEASES OF THE NERVOUS SYSTEM 
of the nose. For human beings the smell sensations aroused by the en- 
trance of chemical substances through the nasopharynx are more 
important than those dependent upon substances entering through the 
front of the nose. 
While there are only four fundamental varieties of taste sensations, it 
seems likely that there are, at least, twenty or thirty elementary olfactory 
sensations; the difficulties surrounding experimental investigation pre- 
vent any dogmatic statement concerning this number. 
Attempts at quantitative methods of examination of the sense of smell 
have been made, especially by Zwaardemaker. The term olfactometry 
has been applied to the measurement of the sensibility of the olfactory 
organ for adequate stimuli, and the term odorimetry is used to indicate 
the making of comparative measurements of the stimulating action of 
different substances. 
We know nothing of exact localization of olfactory sensations. The 
olfactory sense is not easily fatigued, although it varies in this regard 
in its different modalities. The close relation of the olfactory sense to 
various reflex, instinctive, and affective phenomena need only be men- 
tioned to be appreciated. The odor of food excites the impulse to eat 
and starts the secretion of gastric juice. The relation of the sense of smell 
to the excitation of the sexual impulses is well known, and the relation of 
odors to various affective states is close. The mood may be changed in 
a moment by a scent or a stink. 
The clinical examination of the sense of smell is usually carried out by 
holding odoriferous substances before one nostril at a time; the patient 
inhales and tries to name the substance. Well-known substances, chiefly, 
are used. As the majority of people are surprisingly inaccurate in the 
discrimination of smell sensations, the following series can be recom- 
mended: Rubber, gutta-percha, ether, yellow beeswax, camphor, oil of 
anise, vanilla, musk, asafetida, cacodyl or ammonium sulphide, roasted 
coffee, pyridin, tar or carbolic acid, cheese or mutton tallow, tincture of 
valerian, skatol. 
Where smell is defective a careful examination of the nose should be 
made to make sure that the defect is not due to obstruction of the air 
currents in the nose rather than to any disturbance of the nervous mech- 
anism itself. In addition to trying the olfactory sense, one may, if he 
likes, test the functions of the trigeminus in the nasal mucosa with the 
fumes of acetic acid, ammonia, formalin, and menthol. 
Hearing.-The organ of hearing mediates the sensations of sound. 
Sounds are divided into two great groups known as musical sounds and 
noises. The vibrations reach the organ of Corti either by air conduction 
through the external and middle ear or by osteotympanal conduction, 
the transmission occurring then through the bones of the skull. The 
organ of hearing is attuned to certain periodic movements only; thus, 
oscillations less frequent than 16 per second or more frequent than 50,000 
per second fail to stimulate the ear. 
Sounds are ordinarily heard with both ears (diotic hearing), though 
sometimes with one alone (monotic hearing). If a tuning-fork (c-c2) be 
placed on the mastoid, the sound is heard by osteotympanal conduction. 
If at the moment when the sound ceases the fork be removed from the INTRODUCTION TO DISEASES OF THE NERVOUS SYSTEM 
23 
mastoid and placed before the ear, the tone can again be heard through 
air conduction (Rinne's experiment). If a vibrating fork be placed in the 
median line of the head, exciting the two ears equally, and then one ear 
be loosely closed with the palm of the hand, the sound is markedly inten- 
sified in the closed ear-so-called lateralization of the sound (Weber's 
experiment). In otiatric diagnosis it is often important to determine 
the upper and leaver limits of audible tones. The best apparatus for this 
is the "continuous tone series" of Bezold-Edelmann, which permits of 
stimulating with vibrations numbering from 16 to 48,000 per second. 
Clinical Examination of the Sense of Hearing.-Ordinarily clinicians 
are satisfied with (1) determining the so-called auditory acuity; (2) test- 
ing the upper and lower tone limits; (3) Rinne's test; (4) Schwabach's 
test; (5) Weber's test; (6) Gelle's test. 
To determine the auditory acuity, various rough-and-ready tests, serv- 
ing for general orientation as to hearing power, have been introduced. 
One tests the ear first for sounds of different kinds. Thus the power to 
hear spoken and especially whispered words is examined, preferably in a 
still, closed room. The average distance at which whispered sounds can 
be heard (the whisper being produced with the aid of the residual air 
left in the thorax after deep expiration) is about 25 meters in a very 
quiet room, about 20 meters in ordinary rooms. A patient whose hearing 
permits these words to be heard as far away as 6 meters suffers no practi- 
cal inconvenience. Each ear is tested separately, the patient standing 
at the end of the room, the other ear being tightly closed with the finger 
and the face and eyes looking straight forward so as to render lip reading 
impossible. Beginning at a distance of 6 meters a word is whispered, and 
the distance gradually decreased until the word is recognized and cor- 
rectly repeated. 
If whispered words are not heard at all, one uses "conversational tones" 
in the same way. Spoken words can, however, be heard, when one ear is 
deaf, at a distance of 6 meters, even when the healthy ear is closed. To 
be sure about this, if the patient hears conversational tones at a distance 
of 3 meters when the good ear is closed and the bad ear open, one tries 
again when he closes also his bad ear (Lucae-Dennert test); if he can no 
longer repeat the test words, this proves that he heard with the bad 
ear, but if he repeats them one can be sure that the bad ear did not hear. 
We can now exclude hearing in one ear entirely by the use of Barany's 
" noise-apparatus." 
More accurate are measurements w'ith Politzer's acumeter, an instru- 
ment which yields sounds of equal intensity; the drawback to it is that 
the noise it produces consists exclusively of a few high tones. For the 
same purpose the tick of a watch may be used. But the distance at which 
sound can be heard is not a good measure of auditory acuity. 
The poorer the hearing in one ear, the more quickly a waning tone 
ceases to be perceived, and the measurement of the time a tone is heard is, 
therefore, an important aid in otiatric diagnosis. The numerical expres- 
tion of defective auditory acuity is not simple. If a patient, for example, 
hears the fork c for 92 seconds, while a normal ear hears it 183 seconds, the 
auditory acuity under test (H P) is not one-half the normal acuity (II N) 
but a much smaller fraction, about (Ostmann). This depends on 24 
DISEASES OF THE NE EV OUS SYSTEM 
the fact that during the waning of vibrations of a fork the intensity 
does not diminish by equal amounts in successive seconds; at first the 
intensity decreases rapidly, then ever more slowly. 
But for any measurement of auditory acuity which pretends to accu- 
racy one must be able easily (1) to use tones of any desired pitch, (2) to 
use pure tones, (3) to measure their intensity exactly, and (4) to offer 
them to the ear in even intensity for any desired period. The instrumen- 
tarium which best meets these requirements is that of Wien, in which 
a telephone is used as a source for the tones, the sinusoidal alternating 
currents being yielded by a sinus inductor (for low tones) and by an 
alternating current siren (for higher tones). 
Having determined the degree of auditory defect, one has next to 
try to ascertain its cause; one must decide whether the defect is due to the 
sound-conducting apparatus (auditory meatus, tympanic membrane, 
middle ear) or to the sound-perceiving apparatus in the internal ear. 
The data to be gathered consist in the following: 
1. Determination of upper and lower tone limits by means of the Bez- 
old-Edelmann continuous tone series or more simply by tuning-forks 
cb c, ce, the upper limit may be normal and the lower limit changed in 
middle-ear disease, while the opposite holds for labyrinthine deafness. 
2. Rinne's Test.-The difference between the time of air conduction 
and of bone conduction should be noted in seconds for a fork having 
a pitch somewhere between c and c2. 
3. Schwabach's Test.-Comparison of the time a fork is perceived when 
held on the vertex with the time in a person of normal hearing; the time 
is shortened in labyrinthine disease. 
4. Weber's Test.-The sound may be "lateralized" in one ear without 
artificial closure-toward the deaf ear in middle-ear disease, toward the 
healthy ear in labyrinthine deafness. 
5. Gelle's Test.-If one compresses the air in one meatus by means of a 
rubber bulb, the tone of a fork held on the same half of the skull will be 
less loud at the moment of compression if the base of the stirrup be 
movable, while it will remain unchanged if it be fixed (ankylosis). 
Sight.-When the eyes are open the visual sensations are spatially 
extended and exteriorized as the so-called binocular visual field. If one 
eye is closed we project a monocular field. When both eyes are closed, 
or if they be open in the dark, there is a sense of blackness, something 
quite different from the absence of sensation corresponding to the region 
peripheral to the visual fields. 
There are two series of visual sensations: (1) An achromatic series- 
black, gray, white-which can be represented geometrically in their 
transitions by a straight line; and (2) a chromatic series-red, orange, 
yellow, green, blue, violet, purple (in all at least 160 tones at full satura- 
tion)-which can be represented geometrically in their transitions by a 
line which returns to the starting point, e. g., by a circle. This series of 
sensation is related to the achromatic series in such a way that the greatest 
saturation of each color occurs normally at a certain luminosity, e. g., 
yellow is brighter than orange when each is fully saturated. This rela- 
tion of the chromatic to the achromatic series gives rise to the so-called INTRODUCTION TO DISEASES OF THE NERVOUS SYSTEM 
25 
variables in sensations of color. There are three conditions of variability 
-the first dependent on quality, the second on luminosity, the third 
on saturation. 
The organ of visual sense includes the eye and a series of neurone 
chains in the central nervous system. The dioptric apparatus of the eye 
determines the mode of application of the adequate stimuli (light rays) 
to the sense organ proper, the retina. The impulses arise in the rods 
and cones and pass through the bipolar cells and the ganglion cells of the 
retina into the optic nerve and thence to the primary and secondary optic 
centres of the brain. The whole path from the retina to the cortex, com- 
posed of several sets of superimposed centripetal neurones, is known as the 
visual conduction path. 
It is interesting that the different portions of the retina behave very 
differently in regard to color sensations; thus, at the fovea (central 
vision) and the area immediately adjacent (paracentral vision) the color 
sense is quite different from that of the more peripheral portions of the 
retina (excentric vision). Objects which seem colored in central vision 
may have different colors in paracentral vision, and may be colorless 
when excentrically viewed. In other words, the periphery of the retina 
is totally color blind and its vision is monochromatic; the central portion 
of the retina has polychromatic vision, while there is an intermediate 
zone which is partially color blind, having dichromatic vision. This 
explains why it is that the visual fields for colors are of different sizes 
even in the normal eye. 
The ether vibrations of the light rays lead to photochemical, histo- 
logical, and electrical changes in the retina and give rise to the nervous 
impulses. The luminosity of a visual sensation is a function of the 
actual energy of the ether vibrations and of the parts of the retina stimu- 
lated. The eye cannot analyze white into its constituent components, and 
differs from the ear, which can recognize the partial tones in a clang. 
Color blindness is a deviation from the normal relations in a system 
of color sensations. It may be most properly defined simply as abnormal 
color sense. In its least abnormal form it manifests itself in a general 
weakening of the color sense, while in its most abnormal form no color 
can be seen at all but only differences in luminosity are distinguished. 
Between these extremes various deviations from the normal occur. 
The following may serve as a classification of such abnormal cases.1 
1. Abnormal polychromates, who see many colors but who differ from 
normal individuals (a) in that one or more color qualities are absent or 
(b) in outstanding color relations (e. g., in the similarity of certain colors 
to one another). These are the most common cases, and in the majority 
it is the colors red and green which are not distinguished. 
2. Dichromates, who see only one pair of colors, these being com- 
plementary, though sometimes different from the normal complementary 
colors. 
3. Achromates, who see no colors at all, all lights giving sensations of 
gray in varying intensity. These are extremely rare. 
1 A. Kirschmann, Arch. f. d. gesamte Psychol., 1907 26 
Diseases of the nervous system 
Visual sensations depend not only upon the stimuli applied to the eye, 
but also upon the state of the eye at the time of application of the stimulus. 
The change in the eye in the different phases of activity is known as its 
power of adaptation. Among the phenomena which pertain to this 
function of adaptation are: (1) Positive and negative images; (2) light 
induction and color contrasts; and (3) the adaptations undergone by 
the eye in the light and in the dark. Studies of the dark-adapted eye make 
it probable that the rods of the retina are especially concerned with vision 
in dim light (night), while the cones of the retina have to do rather with 
vision in strong light (day). 
Localization of Visual Stimuli.-Two retinal stimuli which have the 
same value as regards quality and luminosity may differ in that they 
are projected into different parts of the visual field. All the retinal 
excitations taken together give nise in consciousness to the continuous 
extended field. We concentrate our attention normally upon the point 
upon which our eyes focus, while the more peripheral portions of our 
visual fields are less focal in consciousness. The arrangement in space 
of visual perceptions, therefore, depends upon the local sign which 
adheres to the sensation aroused by stimulation of each retinal point. 
The localization depends, however, not only upon this local sign, but 
especially as concerns measurement, also upon eye-muscle sensations. 
In examining a patient clinically regarding his capacity for visual 
sensations we are provided with very exact methods and delicate appara- 
tus. Here we can call attention only to some of the methods most 
essential for neurological diagnosis, especially to (1) the determination 
of visual acuity, (2) the determination of the type of central vision, (3) 
the determination of the capacity for vision in dim lights, (4) the deter- 
mination of the extent of the visual fields, and (5) the ophthalmoscopic 
examination. 
In determining the visual acuity, one uses letters or similar signs, made 
so as to be as long as they are broad; the strokes of the letters should be 
of even thickness and one-fifth of the thickness of the whole letter (e. g., 
Snellen's "Optotypi"). The visual acuity V (visits') is expressed ^y a 
fraction whose numerator gives the distance d in which the letter is 
recognized, and whose denominator gives the distance 1) which corre- 
sponds to a visual angle of five minutes for the letter: V = £ The diop- 
tric apparatus must be borne in mind (anomalies of refraction, accommo- 
dation), and if faulty, the visual acuity determined before and after 
correction. 
In determining the types of central vision we decide whether the 
patient has polychromatic (normal), dichromatic, or achromatic vision. 
For this Holmgren's colored wools are very satisfactory. From a large 
collection of wools of different color one chooses one of rose-color (pale 
bluish red) and asks the patient to put beside it from the collection all 
the wools of the same color, telling him that he may make mistakes either 
by choosing wools of a wrong color or leaving behind wools of the same 
color. If his vision be dichromatic, the patient will pick out bluish green 
and gray wools and put them with the rose-colored wools. If his vision 
be achromatic, he will be wholly unable to discriminate among the wools INTRODUCTION TO DISEASES OF THE NERVOUS SYSTEM 
27 
except by differences in brightness. One may use Adler's colored crayons 
or Stilling's pseud o-isochromatic charts, Nagel's lantern with colored 
lights, or color equations with rotating disks. 
In determining the capacity for vision in dim lights, one darkens the 
room and finds out whether the patient sees as long as the examiner and 
whether his eye becomes dark-adapted as quickly. For quantitative 
tests, Foerster's photometer or Masson's disk may be employed. 
The determination of the extent of the visual field for each eye (perimetry) 
is of very great importance in neurological diagnosis. A rough test may be 
made by seating the patient with his back near a window before the 
examiner; a bandage is placed over one eye and he is told to look with 
the other into the eye of the examiner opposite (distance, 40 cm.). Clos- 
ing one eye, one holds the white or colored object (1 cm.) midway between 
the patient and the examiner and controls by the extent of his own visual 
field the examination of that of the patient. With a little practice one 
can detect existing hemianopsias, scotomata, or concentric contractions 
easily; the method is important for quick preliminary orientation. 
For exact registration of the extent of the visual fields, Foerster's 
perimeter or some similar apparatus (e. g., Sydow's) should be used; 
self-registering perimeters are obtainable, but are not necessary. Care 
must be taken to avoid fatigue; one should not work too long at one 
visual field, but determine limits quickly (though accurately), encourag- 
ing the patient frequently to give the matter his closest attention. The 
patient is told that while he may wink as often as he likes, he is to look 
straight forward at a fixed point and on no account to follow the object 
with his eye. The objects are brought in from the periphery and the 
patient announces the time of first appearance and the color; white 
objects are brought in vibrating, colored objects quietly, from the per- 
iphery. No one color field should be determined all at once, but one tries 
different colors, so that the patient never knows which color is entering 
the visual field. If a defect (scotoma) be found, its limits may be very 
accurately localized by using small test objects (2 mm. broad). For test- 
ing color fields, one usually uses green, red, and blue, the field for the 
former being smallest, that for the latter largest, under normal conditions. 
There are great individual differences, however, in normal color fields, 
and much depends upon the exact colors used. If a green which is com- 
plementary for a red be employed, the fields for red and green are 
approximately co-extensive. 
The technique of ophthalmoscopic examination should be learned and 
regularly practised by every active physician. Through it we are able 
to look directly at the papilla N. optici, a part of the central nervous 
system, and, from it, to draw conclusions regarding a whole series of 
nervous diseases; it is one of the chief aids to neurological diagnosis. 
The examination in the inverted image is more desirable than in the 
upright image, since the technique is easier and a wider view of the retina 
is afforded; the upright image is, however, very valuable for the study 
of finer details. As to the papilla N. optici itself, one notes any changes 
(a) in the normally distinct boundaries, and (6) in its normally delicate 
rose color. It is to be borne in mind that the temporal half of the papilla 28 
DISEASES OF THE NERVOUS SYSTEM 
is normally of a somewhat lighter rose color than the nasal, but it is 
never really white. The centre of the papilla often looks white normally, 
owing to the "physiological" excavatio papilla*; the periphery is, how- 
ever, always rose color. Acquaintance with the normal appearances of 
the arterioles and venules of the retina, the color of the eye-ground, 
and the macula lutea should be cultivated. 
DISTURBANCES OF SENSATION. 
General Remarks.-It is customary to divide sensory disturbances 
into subjective and objective. By subjective disturbances one means 
those which are announced spontaneously, in the consciousness of the 
patient; by objective disturbances, those abnormalities of sensation recog- 
nized by the patient when he himself or his physician applies adequate 
stimuli to his organs of sense. 
Subjective disturbances are nearly always pathological sensations 
which arise, owing to (1) abnormal internal stimuli (acting on the sense 
organ, conduction path, or cortex cerebri), or (2) abnormal increase in 
excitability of these parts. The pathological sensations may not differ 
in quality from the sensations producible by adequate stimuli; when 
they do differ (e. g., formication) they are spoken of as dysesthesias or 
paresthesias} 
Objective disturbances are usually classed under the headings: (1) 
Anesthesia, (2) hyperesthesia, and (3) paresthesia, corresponding to (1) 
decrease or loss, (2) increase, and (3) perversion of sensibility. This 
is a very crude classification, and must sooner or later be modified and 
extended. A more rational classification is needed, and we shall doubtless 
from now on begin to classify anomalies of sensation according to changes 
in (1) quality, (2) intensity, (3) reaction time, (4) duration, (5) feeling tone, 
(6) localization or exteriorization, (7) fusion, (8) adaptation, (9) fatigue, and 
(10) relation to reflex, affective, and associative processes. 
By anesthesia is meant a condition in which an adequate stimulus 
no longer arouses a sensation. It may be temporary (e. g., hysteria, 
cocainization) or permanent (e. g., optic atrophy). It may be per- 
ipheral in its origin (sense organ, centripetal nerve) or central (conduc- 
tion path, cortical sense area). Aside from the complete loss of sensation 
which anesthesia denotes, conditions of hypesthesia are met with in which 
the sensations aroused by adequate stimuli are less intense than normal, 
or the physical stimulus necessary to elicit a just perceptible sensation 
must be stronger than normal (elevation of intensity threshold). 
In hyperesthesia stimuli when applied call forth sensations of greater 
intensity than normal or a stimulus normally subliminal possesses a 
threshold value (depression of intensity threshold). Much of what is 
designated hyperesthesia clinically, is really an affective state due to 
abnormal negative feeling tone (e. g., the "acoustic hyperesthesia" of 
psychoneurotics). The "positive or pleasurable feeling tone" of other 
1 The term paresthesia is used very loosely; some mean by it abnormal sensations 
like formication; others use it for anomalies in reaction time, fusion, localization, etc. INTRODUCTION TO DISEASES OF THE NERVOUS SYSTEM 
29 
sensations may be lost (anhedonia), diminished (hyphedonia), or increased 
(hyperhedonia); changes here are met with especially in mental diseases. 
The area of sensory surface in which the anomaly of sensation exists, 
or so-called distribution or extent of the anesthesia or hyperesthesia, should 
be accurately determined and its limits exactly recorded, since these 
are of much importance in differential diagnosis, permitting one fre- 
quently to locate the lesion in periphery, spinal cord, or brain. 
When an organ of sense is diseased it is important also to make sure 
whether the anomaly concerns all the sense modalities or sense qualities 
mediated by it (total anomaly') or only certain of them (elective anomaly). 
Thus', as an example of an elective anomaly of cutaneous sense, the 
condition in syringomyelia may be mentioned in which touch sense may 
be intact when the sense of temperature and pain is abolished. 
The apperception time for sensation may be pathologically delayed 
(e. g., delayed pain and touch sensations in hypesthetic, or more often 
hyperesthetic, areas in neuritis and tabes). 
The localization and exteriorization of sensations are sometimes abnor- 
mal. Irritation of a nerve trunk is, according to the "law of excentric 
localization," always referred to the region whence the nerve receives 
normally its centripetal impulses; thus, when one "hits his funny-bone," 
the little finger tingles, and in amputation neuromata the disagreeable 
sensations are referred to the lost member. Again, a stimulus applied 
to one part of a sensory surface may be localized in another; in tabes, 
hysteria, and multiple sclerosis a skin stimulus to one leg may be local- 
ized in the other (allocheiria or aHesthesia). When a stimulus applied 
to one point is properly localized there but gives rise also to a subjective 
sensation (perhaps of wholly different modality) localized elsewhere 
we speak of synesthesia.1 There may be anomalies of fusion of sensa- 
tions; stimuli which should be felt as continuous and simultaneous may 
be experienced as successive, and one touch to the skin felt as two or 
more, or two compass-points as three or four (polyesthesia). 
Disturbances in Individual Sense Domains. -Anomalies of Pain Sense. 
-Subjective anomalies are very common. Spontaneous pains occur in 
various organic diseases (e. g., neuritis, tabes, visceral diseases), as well 
as in so-called functional diseases (psychoneuroses). Neuralgias, cephal- 
algias, hemicranias, visceralgias, and topoalgias or psychalgias are 
symptoms met with daily. In studying abnormal pain sensations, the 
elements of the pain should be carefully analyzed, its relations to space 
and to time, the exact quality of the pain, its topography, and any 
accompanying phenomena should be attended to. Violent pains on the 
side of the body which is the site of a hemianesthesia point to a lesion 
of the optic thalamus ("thalamic syndrome" of Roussy). The modifying 
influence of position, of motion, of pressure, of food, of drugs and chem- 
icals, and of the organic functions of the body should be looked into. 
The excellent treatise of Schmidt2 can be heartily recommended as a val- 
uable guide to the interpretation of pains complained of by patients. 
1 Smith, H. L., Johns Hopkins Hosp. Bull., 1905, xvi, 256. Here belongs the so- 
called sound feeling, color hearing, or sound seeing, and number form. 
2 Pain; Its Causation and Diagnostic Significance in Internal Disease. English 
translation by Vogel and Zinsser, 1908. 30 
DISEASES OF THE NERVOUS SYSTEM 
Anesthesia in the domain of the sense of pain is called analgesia, while 
hyperesthesia here is called hyperalgesia. When spontaneous pains appear 
in an anesthetic area, we speak of analgesia dolorosa. 
Anomalies of the Deep or Mesoblastic Sensibility.-Anesthesia in this 
domain has been designated bathyanesthesia (Oppenheim). (1) Anomalies 
of the sense of position. Hypesthesia and anesthesia are common in 
neuritis and tabes. If a limb be placed in a certain position, this can be 
only imperfectly or not at all reproduced by the opposite limb. (2) 
Anomalies of the sense of movement. If anesthesia exists, passive move- 
ments are not felt; if hypesthesia, greater excursions than normal have 
to be made to arouse the sensation of movement (elevation of threshold). 
Not only the extent, but the direction of the movement may be mistaken 
in these kinanesthesias. As a result, there is disturbance of the coordina- 
tion of movements, or ataxia. Localized loss is often cortical in origin 
and helps in topical diagnosis (cerebral tumor, etc.). (3) Anomalies 
of the sense of resistance and weight. This sense is prone to suffer also when 
the sense of movement is disturbed. Weights are improperly judged and 
the difference sensibility is altered. (4) Anomalies of stereognostic per- 
ception. In disease the stereognostic perception may be more or less 
impaired, even to complete loss (astereognosis of C. K. Mills). It may 
be lost even when the sense of touch, temperature, and pain are normal. 
Astereognosis is common in hemianesthesia of cerebral origin, but it is 
difficult to be sure about it unless the accompanying hemiplegia is so 
slight as to permit the patient to make the movements necessary in 
palpation. It is more likely to be present if the hemianesthesia be cortical 
(Regio centroparietalis) in origin rather than capsular, pontine, or bulbar. 
In the cerebral palsies of children the power of stereognostic perception 
may never develop. Astereognosis will appear in tabes and peripheral 
neuritis if the sensory conduction paths from the upper extremities be 
involved. Astereogenosis is sometimes a symptom in hysteria. (5) 
Anomalies of the vibration sense (osseous sensibility). Anesthesia of the 
bones to the vibrating tuning-fork (pallanesthesia) is common in neuritis, 
tabes, syringomyelia, hematomyelia, Brown-Sequard paralysis, myelitis, 
cerebral hemianesthesias, and hysteria (Dejerine). 
Anomalies of the Vestibular and Allied Senses.-Vertiginous Disorientation. 
-Disturbances of the vestibular or labyrinthine sense in its two compo- 
nents: (a) Sensibility to acceleration in a straight line (sense of the 
vertical direction and of progressive, non-rotary movements), and (b) 
sensibility to angular accelerations (sense of change of velocity of rotary 
movements)-have only recently had attention more carefully directed 
to them. 
A remarkable phenomenon associated with disturbance (even tempo- 
rary) of the vestibular sense is an illusion regarding the state of movement 
of the body, a deception as to its momentary stability, in which one has 
the mistaken sensation either (1) that objects in the environment are 
revolving around one's body, or (2) that the body is rotating around 
one of its axes. This sensation, often accompanied by a decided feeling 
of discomfort, occasionally, even with the nausea or vomiting, con- 
stitutes the form of dizziness or vertigo known as sham-movement vertigo INTRODUCTION TO DISEASES OF THE NERVOUS SYSTEM 
31 
(to distinguish it from height vertigo, felt on looking downward from a 
great height or upward on a steep wall). This sham-movement vertigo 
appears to be the result of a "contradiction" of one set of sensations by 
another. If one of our sense organs signals "the body is at rest," and 
another signals "the body is in motion," or if the signals conflict regard- 
ing movement in the environment, we feel confused, disoriented, dizzy. 
This vertiginous disorientation is most often due to a discrepancy between 
the reports given us by our vestibular sense and those emanating from 
other sense organs; it is usually associated with a tendency to move the 
body in a direction opposite to a movement really occurring or to a sham 
movement, and, indeed, the body is often so moved. 
This feeling of vertigo can be aroused by stimulating the N. vestibuli 
in various ways: (1) By rotating the body around any one of its axes; 
(2) by moving the body progressively in a curved path (swinging; pitch- 
ing of a ship); (3) by passing a galvanic current through the head from 
one ear to the other (galvanic vertigo). Here the abnormal vestibular 
sensations contradict visual, muscle-joint, and tactile sensibility. But 
the same sensation can be aroused when vestibular sense is normal through 
contradictory abnormal sensations of sight or of eye-muscle movement, 
e. g., whenever binocular vision is suddenly disturbed (eye-muscle par- 
alysis), or whenever the image in one eye is sharply focussed and the other 
is blurred. Further, in cerebral lesions (local irritation of abscess), cere- 
bral anemia or cerebral intoxications (acute alcoholism), in all probability, 
the central stimuli affect sensory neurones of certain modalities more than 
others, so as to produce contradictory impressions with disorientation. 
Recently, attempts have been made to analyze the different forms of 
vertigo and other disturbances in vestibular domains by means of Barany's 
tests,1 especially by the so-called "caloric nystagmus tests," made by 
injecting cold water and warm water into the external auditory canal. 
Cold water normally produces a nystagmus directed toward the opposite 
side, warm water a nystagmus toward the same side. When the vesti- 
bular paths are interrupted these normal reactions do not occur, or are 
altered. 
Obviously, then, any factors which disturb the functions of the vestib- 
ular nerve or its central connections or the eye-muscle apparatus (per- 
ipherally or centrally) may cause vertigo, especially if the disturbance 
take place quickly. Many deaf mutes experience no vertigo on rotation, 
probably owing to loss of function of the labyrinth. 
Anomalies of Other Interior Sensations.- Hunger.-Hypesthesia and 
anesthesia in this domain are met with in the various grades of anorexia; 
hyperesthesia, expressed as exceedingly strong feeling of hunger, is called 
bulimia, or "wolf hunger," when this is accompanied by powerfully 
unpleasant feeling tone, the patient describing it as painful, it is called 
gastralgokenosis (Boas). 
The sense of satiety may be diminished or abolished (akoria, aplesia); 
patients (tumor cerebri, dementia) may then ingest extraordinary 
amounts of food and yet remain entirely unsatisfied; they exhibit 
1 Lewandowsky, Handb. d. Neurol., Berlin, 1910. 32 
DISEASES OE THE NERVOUS SYSTEM 
polyphagia. The feeling of nausea is probably an affective state rather 
than a sensory disturbance in the strict sense. Gastralgia (including 
cardialgia and pyrosis) is a disturbance of visceral pain sense. 
Thirst -Abnormal thirst occurs in diabetes and often also in the 
psychoneuroses; it may lead to excessive drinking (polydipsia), and is 
probably central in origin. 
Fatigue.-Under abnormal feelings of fatigue are often grouped three 
distinct varieties of sensations: (1) The painful weariness or feeling of 
being completely "knocked out," which is associated even with slight 
exercise in many nervous patients; (2) the weakness which is experienced 
by patients who have paralysis or paresis of muscles; and (3) the general 
lassitude felt on slight exercise in states of exhaustion, in which slight move- 
ments caust' palpitation, dyspnoea, perspiration, tremor, and faintness. 
Sexual Sense.-Anesthesia of the sexual sense (abnormal frigidity) 
may be present owing either to loss of libido or to dyspareunia; the libido 
may be pathologically increased, as in nymphomania and satyriasis; for 
various perversions of the libidos, special texts may be consulted. The 
hyperesthesia of the vagina (with painful contraction of the constrictor 
cunni), known as vaginismus, is an anomaly of visceral pain sense and 
nearly always associated with abnormal affective states. 
Anomalies of the Sense of Touch or Pressure.-Anesthesia and hypesthesia 
of the touch sense very frequently occur; it is doubtful whether true 
hyperesthesia of this sense has been observed. The distribution of the 
anesthesia or hypesthesia is important for diagnosis, as is association 
with (or dissociation from) anomalies of pain and temperature sense. 
Spontaneous itching (pruritus) is regarded as a dysesthesia. Special 
forms of formication are met with in acroparesthesia (tingling and pain 
at ends of extremities), in meralgia paresthetica (dysesthesia in the 
domain of N. femoris lateralis), and in erythromelalgia. 
Anomalies of the Temperature Senses.-Subjective feelings of chilliness 
or cold or heat are common in infections (e. g., initial chill), and in the 
psychoneuroses ("hot flushes''). The feeling of heat (especially in the 
back) which occurs in paralysis agitans is well known, and patients 
suffering from exophthalmic goitre frequently complain of abnormal 
sensations of heat. In cutis anserina the "chilliness" contains besides 
its cold component also a factor due to the stimulation of the touch points 
by contraction of the musculi pilorum. In objective disturbances the 
thermanesthesias usually involve cold sense and warmth sense simul- 
taneously, but this is not necessarily the case; there may be loss of cold 
sense (kryanesthesia or alg anesthesia) without change in warmth sense, 
or there may be loss of warmth sense (ardanesthesia) without involvement 
of cold sense, the latter dissociation being more frequent than the former. 
Thermhypesthesias are common; thermhyperesthesia is rare, much that is 
described as such being in reality hyperalgesia. 
Topographical Distribution of Disturbances of Sensation.-Disturbances 
of cutaneous and deep sensibility, especially studied accurately as to 
their topographical distribution on the surface of the body, throw much 
light upon the site of the lesions which give rise to them. In the per- 
ipheral nerve trunks and in the roots of the spinal and cerebral nerves INTRODUCTION TO DISEASES OF THE NERVOUS SYSTEM 
33 
the fibres which carry centripetal impulses are more intimately mixed 
with one another than are their intracentral communications, and this 
explains why it is that dissociations, although they occur in peripheral 
and radicular lesions, are more common when the pathological process 
is located in the spinal cord or cerebrum. 
Anomalies of the Sense of Taste.-Anesthesia in this domain is known as 
ageusia, hypesthesia as hypogeusia, and hyperesthesia as hypergeusia. If 
one or more of the four fundamental qualities of taste sensations remain 
intact when others are abolished, wTe speak of an elective ageusia. Various 
perversions have been described under the designation parageusia. 
Anomalies of the Sense of Smell.-Here, similarly, we have to deal with 
anosmia, hyposmia, and hyperosmia, which may be total or elective. 
When an anosmia is due to interference with the passage of odoriferous 
air currents toward the regio olfactoria it is called an anosmia respiratoria, 
a subdivision of which is the anosmia gustatoria when the odoriferous 
currents from food and drink by way of the choanae are obstructed. 
Anosmia may be due also to disease of the regio olfactoria itself (tobacco 
poisoning; syphilis) or to intracranial lesion of the olfactory nerves, 
bulbs, tracts, or centres. The left-sided anosmia which sometimes 
accompanies right-sided hemiplegia and aphasia is said to be due to 
lesion of the lateral root of the olfactory tract. In hysteria a unilateral 
"functional" anosmia frequently accompanies, or rather forms a part of, 
the hysterical hemianesthesia. Anosmia may be a congenital anomaly, 
and hyposmia is common in imbeciles, although not constant, some im- 
beciles appearing to be hyperosmic. 
Anomalies of the Sense of Hearing.-Total deafness is known as ana- 
cusis, partial as hypacusis, while abnormally sharp auditory acuity, rarely 
met with, is hyperacusis or true hyperesthesia acustica.1 The painful 
sensations in the ear felt when noises or tones are heard not only in 
diseases of the middle and internal ear, in otitic paralysis of the N. 
facialis, in trigeminal neuralgia, in migraine, and in many psychoneurotic 
states has been called dysacusis (false hyperesthesia acustica); in facial 
paralysis it takes especially the form of increased sensitiveness to noises, 
and has been called oxyakoia. 
But little is known as yet regarding auditory disturbances due to 
lesions of the central auditory paths; the latter, owing to the semidecussa- 
tions at the corpus trapezoideum, are laid down bilaterally, the lemniscus 
lateralis, etc., on each side containing conduction paths from both ears. 
Focal lesions in the pons and midbrain (involving the lemniscus lateralis 
or the collicula inferior) or higher up (involving the medial geniculate 
bodies or the auditory radiations) cause disturbances of hearing on both 
sides (not complete deafness on either side). Total unilateral deafness 
is sometimes caused by neurofibromata of the N. acusticus, but usually 
only after the tumor has attained to a considerable size; the deafness 
is here due chiefly to the lesion of the peripheral (not the central) audi- 
tory neurones. Cortical lesions involving the auditory sense areas in 
both temporal lobes must be widespread to cause total "cortical deafness." 
1 The latter term has unfortunately been applied to the pain, or negative feeling- 
tone, experienced by psychoneurotics when noises or any sounds are heard. 34 
DISEASES OF THE NERVOUS SYSTEM 
Anomalies of the Sense of Sight.-Anesthesia in this domain is known 
as anopsia, hypesthesia as hypopsia, and hyperesthesia as hyperopsia. 
Anopsia (amaurosis or blindness) or hypopsia (amblyopia) may involve 
the whole of one eye or of both eyes. Or one-half of each retina or its 
conduction paths may be blind, leading to loss of half of the visual 
fields (hemianopsia); if it be the two right halves or the two left halves of 
the visual field which are lost, we speak of hemianopsia homonyma (due 
to lesions of visual sense area in the cortex, of occipitothalamic radiation, 
or of one tractus opticus), while if it be the two medial (nasal) halves 
or the two lateral (temporal) halves (the latter due to chiasm lesion) of 
the field, we call it hemianopsia heteronyma. If a hemianopsia involve 
the color sense only or chiefly, it is called a hemichromatopsia; if vision 
be present in half the field but dimmed, the condition is designated 
hemiamblyopia. A hemianopsia is said to be total when the line separating 
the blind area from the area in which the field is still visible passes per- 
pendicularly through the fixation point; if the visual field is not so 
limited, but encroaches upon the blind area, the hemianopsia is not total 
and the area of encroachment is called the surplus field (Wilbrand). 
Sometimes this surplus field is so large as to occupy a whole quadrant 
of the visual field and then a quadrantic hemianopsia is spoken of. There 
are even references in the bibliography to sextantic and octantic hemian- 
opsis; when the loss in the visual field is as small as this, however, it is 
probably better to call it a scotoma, as this name is applied to small 
areas of loss or defect in the visual field, such as the blind spot, which 
exists normally. It is customary, however, to reserve the term scotoma 
for a defect in the visual field which does not reach the periphery. A 
central scotoma is one situated at or near the fixation point; if it involves 
only sensations of red and green, it is called a relative scotoma (e. g., 
in chronic form of retrobulbar neuritis, limited to the papillomacular 
bundle, most often seen in alcoholism or tobacco poisoning), while if 
no light be sensed in the defect it is said to be an absolute scotoma (met 
with in diabetes, multiple sclerosis, and occasionally "idiopathically"). 
When the visual field is restricted regularly at the periphery we speak 
of concentric restriction or contraction of the visual fields; when this is due 
to progressive diseases of the Nn. optici (primary and secondary optic 
atrophy) the contraction proceeds from the periphery centralward, the 
central acuity for white and colors (especially green and red) also suffer- 
ing early; the vision is "fogged;" then marked disturbances of orienta- 
tion supervene. The beginning and progress may be very insidious in 
simple (so-called primary) progressive atrophy (tabes; dementia para- 
lytica), while the fogginess and darkness may come suddenly in neuritis 
(meningitis; lues). In choked disk (tumor, lues, brain abscess, hydro- 
cephalus) vision may remain good for some time; if the cause persists, 
there will be gradual loss, due to atrophy; the color sense may go first, 
and, finally, there may be complete loss of the visual field. 
It is interesting that the contraction of a visual field due to glaucoma 
is especially marked in the medial field, the last of the field to go being an 
excentric temporal area (glaucomatous visual-field slit); in contrast to 
what happens in simple optic nerve atrophy the color sense remains INTRODUCTION TO DISEASES OF THE NERVOUS SYSTEM 
35 
long intact in the retained field and the central visual acuity is longer 
maintained (Axenfeld). 
In detached retina there is contraction of the field corresponding to 
the degree of detachment; this may in daylight affect, at first, colors 
only, especially blue, although in dim light the contraction is absolute. 
Recent retinitis due to nephritis or diabetes frequently injures central 
vision and may give rise to central scotomata of different kinds cor- 
responding to the various patches and hemorrhages; the periphery is 
less often injured. 
In retinitis pigmentosa and in the retinal atrophy following syphilitic 
chorioretinitis there may be either marked concentric contraction of the 
visual field, or, in cases in which the degeneration attacks an interme- 
diary zone of the retina, a so-called ring scotoma which later extends both 
peripheral ward and central ward. Central vision may, despite this, be 
long retained as an " island" of good visual acuity and good color vision, 
the patient seeing as though through a tube of small caliber. Similar 
limitations of the visual field are met with in hysteria and in traumatic 
neurosis; here, however, by testing first at a distance of 10 cm. (and mark- 
ing limits with chalk) and then at a distance of a meter, the visual field 
does not correspondingly enlarge, the psychoneurotic state here behav- 
ing like that of a stimulant. 
In contrast with the immobile positive scotomata (due to diseases of the 
retina or visual path) above described, the mobile positive scotomata (mov- 
able shadows) due to turbidity of the corpus vitreus should be borne in 
mind. In neurasthenics and myopics these muscoe volitantes may be 
present when the vitreus is normal {myodesopsia); the threads, flocculi, 
veils, chains, etc., seen in certain cases may, however, be due to patho- 
logically formed elements in the vitreous visible with the ophthalmo- 
scope. Important for neurologists, too, is the so-called fortification 
scotoma of migraine; a positive and sharply localized sensation of glim- 
mering followed by darkening due to irritation of the retina or visual 
sense area. 
Sudden blindness of one eye may be due to embolism or thrombosis of 
the arteria centralis retinae. Other sudden disturbances of vision may 
occur in (1) cerebral hemorrhage involving the central visual paths 
(hemianopsia associated with other symptoms of cerebral apoplexy); 
(2) retinal detachment (disturbance beginning at periphery of visual 
field, preceded by characteristic irritative phenomena); (3) choked 
disk (obscurations temporary). Less sudden but rapid blindness may 
occur in (1) very acute inflammatory glaucoma (excruciating unilateral 
headache); (2) large hemorrhages into the corpus vitreum (red or green 
stripes before the eye precede the blindness); and (3) acute retrobulbar 
neuritis. 
The terms nyctalopia and hemeralopia need explanation. When a 
patient can see better in dim light (or when the eye is in shadow) than in 
bright light he has nyctalopia (e. g., in beginning cataract, the widening 
of the pupil in dim light leaving niore transparent lens uncovered). 
When, on the other hand, a patient does not see at all in dim light, or 
only after long adaptation, hemeralopia or night blindness exists (retinitis 36 
DISEASES OF THE NERVOUS SYSTEM 
pigmentosa; chorioiditis diffusa; glaucoma; retinal detachment; general 
disturbances of nutrition). 
By photopsia is meant an abnormal sensation of color or light due to 
retinal lesions, or to central irritation, by metamorphopsia, the distorted 
vision, occurring in lesions of the retina and chorioid, in which straight 
lines look curved or jagged, or letters seem out of place. The term 
macropsia is used to designate the symptoms "of seeing things too large" 
(e. g., strained accommodation), while micropsia is the term for "seeing 
things too small" (e. g., paresis of accommodation). 
In many functional nervous cases a study of anomalies of refraction 
has an important bearing upon etiology and treatment. We distinguish 
the normally refracting eye (emmetropia) from the short-sighted eye (myo- 
pia) and the far-sighted eye (hyperopia, hypermetropia). When in order 
to secure normal visual acuity it is necessary to use not simple spherical 
lenses, but lenses which correct certain meridians only, the condition of 
astigmatism exists; if the curvature in each meridian is equal throughout 
its course, but the curvatures of different meridians vary so that the 
meridians of greatest and least curvature are approximately at right 
angles to each other the astigmatism is regular; when the vertical merid- 
ian has the greatest curvature it is astigmatism with the rule, but when 
the horizontal meridian has the greater it is astigmatism against the rule. 
The astigmatism is irregular either when the meridians of greatest or 
least curvature are not approximately at right angles or when the curva- 
ture in a single meridian is irregular. 
Anomalies of vision due to disturbances of accommodation and conver- 
gence may also be mentioned here, although they belong more properly 
to disturbances of motility. If near vision is faulty, or cannot be main- 
tained long, asthenopia exists. If this is due to faulty power of accom- 
modation, it is known as accommodative asthenopia. The most frequent 
form of this is due to the progressive sclerosis of the crystalline lens, 
which normally diminishes the power of accommodation as life advances, 
and soon after the fortieth year the near-point of a normal (emmetropic) 
eye comes to lie more than 25 cm. distant (presbyopia). Accommodative 
asthenopia due to paralysis of the M. ciliaris often follows diphtheria. 
Another form of defective near vision is muscular asthenopia, especially 
that due to faulty convergence (most often seen in incorrected myopia) 
with consequent faulty binocular near vision (convergence asthenopia). 
The lesions of the eye, most important for the neurologist, discoverable 
by the ophthalmoscope, may be briefly referred to here. In optic neuritis 
the papilla looks turbid, grayish-red in color, its margins are indistinct, 
and there are often minute hemorrhages in or near it and the veins 
may be tortuous and dilated. It is most often due to cerebral disease, 
especially when bilateral. If the papilla be swollen (2 diopters or more) 
and enlarged, we speak of choked disk; it is most often due to increase of 
intracranial pressure (brain abscess, hydrocephalus, syphilis, tumor).1 
Unilateral optic neuritis occurs in diseases of the orbit, in various infec- 
tions and intoxications, and in beginning tumor, etc. Optic neuritis 
1 See Cushing and Bordley, Johns Hopkins Hosp. Bull., 1909, xx, 95. INTRODUCTION TO DISEASES OF THE NERVOUS SYSTEM 
37 
may be followed by optic atrophy; the papilla has indistinct margins, 
but becomes whiter as the swelling goes down; the arteries gradually 
narrow and have white sheaths (neuritic optic atrophy'). 
Retrobulbar neuritis occurs in two forms-acute and chronic. The 
acute form is seen in multiple sclerosis, the chronic in diabetes, in various 
intoxications (tobacco, alcohol), and sometimes when its cause is not 
known. Here the ophthalmoscopic picture is very different from that 
described above for "optic neuritis;" no distinct alteration can be seen 
in the papilla N. optici for some time, although after a while a retrograde 
atrophy leads to pallor, especially on the temporal side of the disk. 
When the papilla N. optici looks white, grayish-white, or greenish- 
white instead of its normal rose color there is atrophy of the optic 
nerve (atrophia N. optici); ophthalmologists distinguish several types: 
(a) Simple, (b) retinal, (c) glaucomatous, and (d) neuritic atrophy. 
Of simple optic atrophy in which there is simply pallor of the disk with 
retention of its well-outlined margins and but little change in the vessels, 
three main types are described: (1) That occurring in tabes and dementia 
paralytica, the changes in the disk being visible from the beginning of 
the visual disturbance; (2) retrograde atrophy, following upon diseases 
at base of brain, e. g., tumors, chiasm lesions, sphenoidal-sinus disease, 
etc., the changes in the papilla becoming visible through the ophthalmo- 
scope only after the visual disturbance has lasted some time; and (3) 
atrophy after occlusion of the A. centralis retinae, in which the simple 
atrophy differs from (1) and (2) by the existence of a high grade of 
vascular constriction. 
The so-called retinal atrophy of the optic nerve is characterized by a 
yellowish-white turbid papilla, constricted bloodvessels, and degenera- 
tion of the peripheral retina; it occurs in diffuse degenerations of the 
retina (chorioido-retinitis; retinitis pigmentosa). 
The form of atrophy accompanying chronic glaucoma is very char- 
acteristic. The increased intra-ocular pressure leads to "glaucomatous 
excavation," and this after a time is followed by atrophy with abrupt 
kinking of the bloodvessels at the margin of the papilla. 
Anomalies of Sense Perception Known as Illusions, Hallucinations, 
and Pseudo-hallucinations.-Normal sense perception is more complex 
than mere sense impression; it involves association and assimilation. An 
"object" is composed only in small part of sense impressions directly 
received by way of the peripheral sense organ, but very largely of revivals 
of impressions earlier experienced (reproduced images; representations). 
A percept arises largely through the arousal of a host of old experiences 
by a few newly entering sensations; perception is not, as it may seem, 
the mere entrance of a group of new sensations. The percept as thus 
constituted depends, especially in its meaning or the value which it has 
in consciousness, upon the general direction of thought or upon the most 
outstanding idea in the immediately preceding moment; this is often, 
though inadequately, expressed by the term attention. 
We can profitably pass on to the consideration of the anomalies of sense 
perception known as sense deceptions, including illusions, hallucinations, 
and pseudo-hallucinations. In all these sense deceptions there is an 38 
DISEASES OF THE NERVOUS SYSTEM 
"abnormal relation between the objective and the subjective setting, and 
the factors that control the course of the associations are not suited to the 
circumstances of the physical world." Events occur in accord with the 
laws governing normal perception, but the conditions are unusual and 
individual, not those which obtain with the mass of healthy people. 
By an illusion we mean a sense perception in which the subjective 
factor takes too large a part in the process of assimilation, so that the 
mental supplementation of the sense impressions entering into it leads 
to a result contradictory to the reality as determined in more accurate 
examination with the same sense organ or on correction by control 
through other senses. 
By an hallucination we mean a sense perception in which the abnormal 
relation between subjective and objective conditions is very pronounced; 
objective sense impressions may be entirely absent or present in unusually 
small amount and dissimilar to the content of the hallucination, and yet 
the sense perception is vivid, possessing for the patient the ear-marks 
of objective reality, being definitely localized in perceived space; the 
annihilation of the perception normally localized in this space is unex- 
plained. 
By a pseudo-hallucination we mean a psychic experience which resembles 
a sense perception in intensity and in its involuntary appearance (thus, 
different from a mere image, idea, or representation called up by asso- 
ciation), but differing from a normal sense perception, from an illusion, 
and from an hallucination in that the patient notices the absence of the 
character of objectivity. 
We have then a series of transitionally related psychic phenomena 
beginning with sense impressions at one end and pure reproductions at 
the other, the series consisting of (1) sense impressions, (2) normal sense 
perceptions, (3) illusions, (4) hallucinations, (5) pseudo-hallucinations, 
and (6) pure reproductions (ideas, images, representations). In normal 
people in ordinary circumstances, the sense deceptions (illusions, hallu- 
cinations, and pseudo-hallucinations) do not occur; they are experienced 
occasionally by normal people in extraordinary circumstances and fre- 
quently in mentally abnormal people in ordinary circumstances.1 
Illusions.-The psychic experiences known as illusions are best studied 
in connection with the sense of sight and hearing, since with these senses 
one can be sure as to the character of the external stimuli acting. Sounds 
heard and sights seen are misinterpreted owing to the incorrect sup- 
plementation (by reproductions-images; ideas) of the sense impressions 
received. A melancholic patient, hearing the whistle of a locomotive 
engine, says that she hears the cry of her child being murdered, or, seeing 
a relative, fails to identify him as such, but takes him to be a stranger 
and impostor. Even normal people are often the subjects of illusion 
when the sense impressions are feeble and the mind is in a state of expec- 
1 Some of the more important contributions to the general bibliography of sense 
deceptions are the following: Kahlbaum, Die Sinnesdelirien Allg. Ztschr. f. Psychiat., 
xxiii, 1-86; Parish, E., Hallucinations and Illusions, 1897; Sully, J., Illusions, 1883; 
Text-books on Psychiatry by Krafft-Ebing, Kraepelin, and Paton have good chapters 
on the subject. The discussion in Storring's volume is excellent. INTRODUCTION TO DISEASES OF THE NERVOUS SYSTEM 
39 
tation; for example, an anxious mother may take the mewing of a cat 
for the cry of her sick child in a distant room. But the stronger and 
more distinct the sense impressions when illusions occur, other things 
being equal, the greater the degree of abnormality indicated. Deficient 
attention to sense impressions (as in alcoholic delirium) and short dura- 
tion of the sense impressions favor the origin of illusions. Tense expecta- 
tion, emotional states or moods, a lively imagination, or pathologically 
increased facility of reproducing ideas will also favor illusion, for in these 
circumstances certain ideas are prone to be held focal in consciousness and 
they will obviously have the best opportunity for cooperating with any 
sense impression which may enter. In insanity, illusions are probably just 
as important as hallucinations, the sense deceptions being due to the 
delusional ideas which are in consciousness at the time of entrance of 
the sense impressions. 
Hallucinations.-These may be elementary or complex. An elementary 
hallucination is one in which the subjective experience approaches that 
of a simple sense impression (e. g., a flash of light, a simple sound), while a 
complex hallucination is one in which the subjective experience approaches 
that of a compound sense perception (e. g., vision, voice). Hallucinations 
and illusions conform to the law of excentric projection or exteriorization 
of sense perception; the patient "projects" what he sees, hears, or feels 
into the external visual, auditory, or tactile fields, or localizes the sensa- 
tion either in space outside or in various parts of his body. 
Hallucinations seem even more real to those who experience them 
than do normal sense perceptions; their power is irresistible; the patient 
is compelled to believe in them, the judgment being quickly overcome 
by them. This peculiar feature is due in part to the sensory distinctness, 
the seeming objective reality of the experience, but much more to the 
fact that hallucinations most often correspond to the pathological ideas of 
the patient, to his abnormal thoughts, fears, or wishes. Thus, although 
sense deceptions often play a part in the origin of delusions, some per- 
sistent direction of thought (Aufgabe) is still more often provocative of 
sense deceptions. Further, the strong feelings and emotions co-existent 
with the hallucinations go far toward giving the latter their power in the 
mental life. 
Among visual hallucinations the elementary types are represented by 
flashes of light or color, stars, flames, and the like, the complex types by 
visions in which, especially at night, illuminated animal and human 
forms appear, or God, angels, the Devil, or various horrible shapes may 
be seen; if the hallucination occur in the daytime, which is less common, 
the patients complain of seeing a black dog, the face of a corpse looking in 
at the window, and the like. Sometimes in hemianopsia the visions are 
projected to the blind halves of the visual fields; occasionally, as in 
convalescence from alcohol delirium, visions which have begun to dis- 
appear may be recalled by pressure on the closed eyes (Liepmann). 
Even more influential in mental life than visual are auditory hallucina- 
tions, the elementary forms of which are called akoasmata, the complex 
forms being known as phonisms or voices. The akoasmata consist of indefi- 
nite noises of different sorts-rustling, murmuring, thundering, and the 40 
DISEASES OF THE NERVOUS SYSTEM 
like; the phonisms or voices are often whispered words, more rarely 
words or brief sentences spoken aloud. The voices make unpleasant 
references to the patient, they insult or threaten him ("rascal," "thief"). 
They may emanate from the patient's children, who are being murdered 
by an enemy, from an annoying neighbor, or it may be the Deity or Satan 
who addresses him. Occasionally a voice may bring happiness and inspi- 
ration, but this is unusual; nearly always the voices irritate and pain, 
arouse suspicion and defence, cause anxiety or confusion; they may 
command, and the patient may be led to the most unnatural acts in the 
belief that he is obeying the order of a supernatural power. The patients 
can tell the distance and direction from which the voices come-they 
localize them in space-a fact closely related to the objectivity (per- 
ception value) which attaches to the voices. If the voices are heard in 
one ear only, there is usually abnormal stimulation of the auditory 
path on that side, and the hallucination (or illusion) can often be called 
forth by electric stimulation. 
A word must be said about audible thinking (Gedankenlautwerden of 
the Germans), not of the delusional form of it in which the patient is 
convinced that his thoughts are audible to other people, although he 
does not hear them himself, but of the hallucinatory form in which a 
man hears his own thoughts, perhaps in his feet or in the ticking of a 
clock or the ringing of the doorbell. The patient hears the voices only 
when he pays attention to them. Sometimes a patient's thoughts seem 
doubly existent to him, first, in the normal way and then also as audible 
voices (double thinking); sometimes the voice dictates what the patient 
writes or it may repeat what the patient reads; occasionally the voice 
precedes the reading. Many of these "inner voices" are examples of 
pseudo-hallucination rather than of true hallucination. 
It is extremely difficult to distinguish olfactory and gustatory hallucina- 
tions from illusions of these senses, and the same is true of hallucinations 
of visceral sense. They are, however, of great practical importance, for 
they often account for the refusal of food. If the food smells of feces 
or fold sweat, one can scarcely blame a patient for not eating it. In the 
domain of the cutaneous senses, tactile or hepatic hallucinations and 
thermal hallucinations are not infrequently met with, the former especially 
in toxic psychoses; some of the subjective dysesthesias may be hallucina- 
tory in nature, as may also certain so-called psychalgias or topoalgias 
(hallucinations of pain sense). Much study has been devoted also to 
hallucinations oj the movement and position senses, sometimes referred to 
as kinesthetic hallucinations. They lead to the maintenance of peculiar 
attitudes and also affect the voluntary movements, being sometimes the 
cause of imperative positions or monotonous defensive movements. 
The hallucinations may concern the eye-muscle movements, the loco- 
motory movements, or the speech-muscle movements. Sometimes there 
are hallucinations of vestibular sense, the patient having the sensation 
of progressive or rotary movement. Doubtless many impulsive acts 
and various psychomotor inhibitions are traceable to hallucinations as 
a cause; in epilepsy and alcoholism there may be an irregular hallucina- 
tory agitation, sometimes combined with paroxysms of rage. INTRODUCTION TO DISEASES OF THE NERVOUS SYSTEM 
41 
While the hallucinations above described are subjectively equivalent to 
perceptions, the patients believing that they are dealing with something 
objective, this is not true of what Hagen has called pseudo-hallucinations 
(psychic hallucinations of Baillarger; apperception hallucinations of 
Kahlbaum; imagination deceptions of Kraepelin). Faces printed pages, 
groups of people appear for a moment and vanish, to be followed by 
others not logically connected with them. They seem to stand before 
the eyes, but are not at all related to the black field of vision of the closed 
eyes. Despite their sharp contours and lively colors, the images do not 
possess the character of objectivity; the patient says he sees them 
with "inner eyes," not with his "outer eyes." Although the objects 
seen "stand before the eyes," they are not related to any real objects 
about him. In pseudo-hallucinations of hearing the patients complain 
that they are "forced to hear internally" (not with their ears) things 
which they least wish to hear. Obviously, although these psychic states 
resemble perceptions on the one hand, and lively reproductions of 
mere ideas on the other, they differ from both. They differ from per- 
cepts in that the patients recognize the lack of objective reality (physical) 
and their independence of the movements of the peripheral sense organ 
concerned; they differ from ordinary reproductions (images, ideas) in 
that they have greater intensity and are independent of the will. 
DISTURBANCES OF MORE COMPLEX PSYCHIC PROCESSES. 
Anomalies of Consciousness as a Whole.-Consciousness is the fact 
that we have experiences-sensations, ideas, feelings, etc. To understand 
consciousness, therefore, we must understand experience fully. In spite 
of ages of speculation we are certainly, as yet, only making a beginning 
in the understanding of experience, in that we are gradually determining 
some of the conditions under which it occurs. These conditions are (1) 
partly of the order of physical facts (e. g., light-waves, sound waves); 
(2) partly of the order of physiological facts (e. g., processes in sense 
organs, nerves, and central nervous structures); and (3) partly of the 
mind itself (e. g., imagination, feeling, volition). 
As ideas come and go in consciousness we are aware that some of them 
are more prominent or outstanding than others. We commonly speak 
of paying attention to these more prominent ideas. Subjectively this 
prominence of certain ideas is, at times, definitely accompanied by a 
peculiar feeling of " activity," a sense of strain or effort, which is in con- 
trast to the feeling of "submission" (passivity) which accompanies a 
distracting sensation or memory. When ideas have the prominence 
just referred to we feel that they are ours in a peculiar sense-that we 
are doing something with them. In analogy with the visual field, we often 
refer to these prominent ideas as being in the focal part of consciousness 
(Apperception of Wundt), while the other ideas are marginal (Perception 
of Wundt). 
By the mind we usually mean not simply the abstract state of conscious- 
ness or the total content or field of consciousness at any given moment; 
we include in it also that unity with past experience through which the 42 
DISEASES OF THE NERVOUS SYSTEM 
present moment has a meaning and value, dependent on the whole 
education of the individual which makes it truly personal. When we 
distinguish between the activity of the mind (Bewusstseinstatigkeit) and 
the content of the mind (Bewusstseinsinhalt) we should recognize that 
an abstraction is being made between two aspects of every idea which 
have no significance apart from one another. 
In the central neurones changes take place which permit the calling 
up of memory pictures in simple and complex groups. The totality of 
memory pictures possibly revivable is what is designated as the contents 
of the mind. This totality of possible memories is divisible into three 
parts: (1) That which refers to the external world; (2) that which refers 
to the body or soma; and (3) that which refers to the personality of the 
individual. Wernicke has given to these three parts of the mind's con- 
tents the names allopsyche, somatopsyche, and autopsyche. 
The allopsyche, or portion of the mind's contents which concerns the 
external world, is made up of memories of concrete things, the result 
of sense impressions, their combination into perceptions, and the short- 
hand epitomes of these which we call concepts and notions. The allo- 
psyche of an individual will depend then largely upon his sense organs; that 
of the congenitally blind will differ greatly from that of the congenitally 
deaf, and that of the anosmic will be wholly other than that of the ageusic. 
Given brains of equal birth, the allopsychic contents will vary quantita- 
tively according as the bearer is a resident of a city or a backwoodsman, 
or may differ qualitatively with the environment (e. g., tropical or arctic). 
The somatopsyche or portion of the mind's contents which concerns 
the body consists of all the memories which represent residues of previous 
visceral, muscular, fascial, and cutaneous sensations. In all probability 
the so-called "local sign" which accompanies the exterior sensations 
is to be counted as a part of the body consciousness, the local sign of 
a visual sensation representing, for example, an "organ sensation" of 
the retina (Wernicke). Here the allopsyche and the somatopsyche 
overlap. The latter depends largely upon the condition of the viscera, 
and the tendency is to relate the hypochondriacal symptoms of mental 
disease to anomalies of visceral sensations. 
It is probable that in every allopsychic sense perception the soma- 
topsyche plays a part; indeed, this somatopsychic participation may be 
the main difference between a perception and a revived image of memory, 
for in the perception an organ sensation (local sign, feeling tone) is present 
while it is absent in the memory pictures (Wernicke). 
The various projection fields of the somatopsyche must be intimately 
connected with one another by associative neurone systems. The 
connections of the memory pictures concerning the body are much more 
intimate than those uniting the memories of concrete things of the 
external world on account of the unalterable relations of the different 
parts of the body as contrasted with the continuous changes in the world 
outside the body. In sleep the impressions from the external world 
cease to affect the brain, although bodily impressions are still entering 
it. The external world is at times cut off; the body is always there. 
When the body is undergoing marked changes, as at puberty, the climac- INTRODUCTION TO DISEASES OF THE NERVOUS SYSTEM 
43 
teric period, there must be a marked alteration in the character of the 
afferent impulses from the viscera. Is it any wonder, then, that dis- 
turbances of somatopsychic consciousness tend to prevail at such 
periods? 
The relative constancy of bodily sensations, the continuity of the 
somatopsyche, as compared with the variety of sensations from the 
external world and its discontinuity, give rise in each individual to a 
conception of unity, of individuality, of the body consciousness {primary 
ego of Meynert). 
The autopsyche or portion of the mind's contents which concerns the 
personality or individuality is something built upon the allopsyche and 
the somatopsyche which serve as its foundations. The autopsyche or 
consciousness of personality includes all the mental possessions and 
acquisitions resulting from instruction, culture, and education, the 
influence of the family and of the social milieu. Speech and written 
language play an important part in its development. School knowledge 
is among the early acquisitions of the autopsyche; later on come all the 
experiences peculiar to the individual. The interest in work, in family, 
in friends, and in other people belongs here. The development of the 
autopsyche depends largely upon the kinds of allopsyche and somato- 
psyche which form its foundations; as Wernicke puts it, "consciousness 
of personality is a function of the consciousness of the external world 
and of that of the body. . . . The man with sharp senses and 
strong body must develop a mental personality different from that of 
a man with dull senses and a weak body." An individual's own estimate 
of his place in human society depends upon the state of his autopsyche. 
Among the striking anomalies in this domain may be mentioned ideas 
of grandeur or of self-depreciation, ideas of reference and delusions of 
persecution. 
There seems to be no doubt that the somatopsyche, the allopsyche, 
and the autopsyche are, to a certain degree at any rate, localizable; 
or, more exactly, their physiological correlates appear to be to some 
degree localizable in the cerebrum. Clinical and pathological studies 
of dementia paralytica, of cerebral arteriosclerosis, etc., show us how the 
autopsyche, the allopsyche, and the somatopsyche may separately or 
successively become involved. 
Circumspection; Confusion; Unconsciousness.-In the normal awake state 
there exists a certain equilibrium in perceptions, ideas, and feelings, the 
external manifestation of which is known as circumspection or presence 
of mind (Besonnenheit of German writers); here the mind is calm and 
collected with its faculties ready at command. It is the opposite of 
mental confusion (Venvirrtheit of German writers) or incoherence, in which 
ideas are jumbled and chaotic, the mind perplexed or bewildered, the 
speech aimless or unintelligible, the acts disconcerted. By unconscious- 
ness is meant a state in which the psychical powers are wholly in abey- 
ance (dreamless sleep, profound narcosis). Attempts have been made to 
distinguish various grades of pronounced lowering of consciousness, and 
references will be found to a series of states designated in order as 
somnolence, sopor, coma, and earns. 44 
DISEASES OF THE NERVOUS SYSTEM 
States of Psychical Cloudiness or of Clouded Consciousness; Mental Fog; 
Twilight States; Automatism.-By mental fog, psychical cloudiness, or a 
twilight state (Dammerzustande of German writers) is meant a condition 
in which there is diminution of clearness of consciousness or a loss of 
memory for a part of past experiences or both together. Such mental 
fog is often met with in epilepsy and in hysteria. In epilejjtic mental 
fog there are often fluctuations of consciousness intensity, partial loss 
of power of recalling the past, alterations of mood and false identification. 
In hysterical mental fog we meet the so-called monoideic and polyideic 
somnambulisms and fugues. In somnambulism an individual thinks and 
acts in an odd way, different from that of other people, while he is in some 
way like a person asleep. During a monoideic somnambulism the patient 
acts as though in a dream, but the phenomena of the dream are most 
perfectly and intensely developed. 
In fugues (flights or ambulatory automatisms') we have to deal with the 
hysterical mania for running away. During the abnormal state a certain 
idea or trains of ideas develop to an exaggerated degree (attempt to 
elude capture; desire for travel, etc.), and lead to the flight. At the 
same time other thoughts which would normally counteract this idea or 
train of ideas appear to be suppressed; the patient may forget his name, 
his family, his social position, or, in other words, his ordinary personality. 
After the fugue is over and circumspection returns the patient has for- 
gotten many or even all of the events of the flight, and also the ideas 
which dominated him during it. Through hypnosis it is sometimes 
possible to reproduce the mental state of the fugue artificially. 
Intermediate between fugues and monoideic somnambulisms are the 
so-called polyideic somnambulisms. These are characterized by the multi- 
plicity of the ideas that fill them. Instead of the one idea or event of 
the monoideic type, we see evidence of the existence of a series of ideas 
succeeding one another in the somnambulic state, ideas which pertain 
to some strong feeling which develops independently of the rest of con- 
sciousness. Where still larger systems of ideas and feelings develop 
independently we get the manifestation of what are known as double 
personalities or multiple personalities. 
Twilight states are usually temporary, but they may last for varying 
lengths of time; they may last for weeks or months. The cessation of the 
state is, as a rule, sudden, the patient waking as though from sleep. 
Indeed, sleep seems to be the physiological paradigm of a twilight state, 
dreams being the analogue of the psychical activity, but differing from 
the consciousness of the twilight state in that usually they are unac- 
companied by motor reactions. 
Disorientation.-Many patients are not well oriented as to their own 
persons and their spatial and temporal relations. For differential diag- 
nostic purposes it is very important to determine the presence or absence 
of certain other symptoms at the same time orientation is tested, especially 
anomalies of mood, ideas of persecution, and hallucinations. A few simple 
questions along with those concerning orientation may quickly give the 
clue to the existence of a hallucinatory confusion, of a paranoid state, 
or of some other well characterized type of mental disorder. Especially INTRODUCTION TO DISEASES OF THE NERVOUS SYSTEM 
45 
important for the general practitioner is the valuation of the syndrome 
in which disorientation as regards time and space is combined with so- 
called pseudo-reminiscences, in which the patient narrates long tales 
of recent events as though perfectly true, which on inquiry are found to 
have no basis in fact (Korsakow's syndrome, polyneuritic psychosis). 
Anomalies of Attention.-The importance of disorders of attention in 
psychiatry is coming to be very generally recognized. The power of 
directing thought oward a definite task (Aufgabe) (vigility), and of 
maintaining this task despite intercurrent stimuli (tenacity) are essential 
in all intellectual operations. We meet with most variable ability to 
direct and maintain the task, some being able to do this easily, while others 
are easily distracted. The greater the distractibility the more dirigible 
from without, the less thorough is the intellectual work. The term 
hyperprosexia is by some reserved for the pathological persistence of a 
task in which the mind is concentrated upon certain abnormal ideas or 
sensations (hypertenacity), while aprosexia is defined as a state in which 
there is inability to set or to maintain a task (e. g., in imbecility). There 
is both hypovigility and hypotenacity. Some writers use the word 
hyperprosexia to indicate a state in which there is increased tendency 
to change the task set (hypervigility) with diminished tenacity. 
Examination of the Recording Faculty.-For testing the recording faculty, 
which depends in large part upon attention, much simpler methods must 
be used in medical practice than in experimental psychological examina- 
tions, and we have to thank Ranschburg1 and Boldt2 for supplying us 
with such simple methods. It is necessary to test the recording faculty 
in several different domains, and most experiments consist of acoustic 
and optic tests. Thus, in one group of experiments the acoustic word- 
memory, using the principle of idea associations, may be tested. The 
patient memorizes fifteen pairs of words, of which five represent custo- 
mary word compounds, like dog-kennel chair-leg; five according to the 
laws of causality (spatial and temporal coincidence), like fish, pond; 
day, night, etc.; . and five according to the principle of similarity of 
sound, like wound, wonder; die, dynasty. In another group of tests the 
memory of persons is tried by using twenty-five portraits, from which 
the patient is to recognize five. In a third group the memory for colors 
may be tested. Many colored skeins of red, blue, green, and yellow 
wool are shown and a definite example of each is to be recognized. In 
a fourth group the orientation memory is tested. One shows the patient 
a chart containing six simple geometrical figures, exposes it for twenty 
seconds to permit of certain perception, and after a time the patient is 
required to pick out these six figures from a chart containing twenty 
or twenty-two figures. In a fifth group the memory for nonsense words 
is tested, five pairs of words being chosen, e. g., lum, rar; grat, smor. 
In a sixth group of tests the memory for names is tried. The patient is 
shown five photographs and the name and surname given. Later on the 
patient picks out these five photographs from a group of twenty-five and 
tries to give correct name and surname. Lastly, in a seventh group the 
1 Monatsschr. f. Psychiat. u. Neurol., Berlin, 1901, ix, 241 to 259. 
2 Ibid., 1905, xvii, 97 to 115, 46 
DISEASES OF THE NERVOUS SYSTEM 
memory for numbers is tested. Some number is given to each one of the 
five fingers, and later on, naming each finger, he is asked to give the cor- 
responding number. The reproduction is best tried shortly after the 
perception (five or fifteen minutes) and again after twenty-four hours. 
The recording faculty consists of two factors, namely, perception and 
reproduction. Of course, where perception is deficient there can be no 
reproduction later. One makes sure, first, that the task is correctly 
understood. The patient is warned not to try to reproduce repeatedly 
in his mind the things perceived, for even in normal persons the test turns 
out less favorably later when such efforts are made. The first reproduc- 
tion is permitted after five minutes have elapsed, the second after fifteen 
minutes, and twenty-four hours later the third test is made. The repro- 
duction at the end of twenty-four hours may in normal persons be 
much better than that at the end of five minutes, but in patients with 
injured recording faculty this is not the case. The recording faculty 
appears to be the one of the mental powers first and most seriously to 
suffer in dementing processes. Injury to the recording faculty as mani- 
fested in loss of memory for most recent events never exists long alone; 
there are added to it very quickly other characteristic symptoms of 
mental deterioration. The recording faculty is most efficient in childhood 
especially between the twelfth and the fourteenth years of life. 
When the recording faculty begins to be impaired it seems to be the 
memory for numbers which goes first. As Kraepelin has emphasized, 
studies of this recording faculty have drawn the attention of clinicians 
to a group of anomalies previously entirely unknown to us, viz., (1) 
the quick fading of perceptions, and (2) the slow development of percep- 
tions, both of which appear especially to favor the origin of memory 
falsifications. 
Anomalies of Ideation.--Great importance attaches to the examination 
of the processes of ideation; that is, to the formation and association 
of ideas. Ideation may be disturbed on the formal side, or there may be 
falsification of the content of ideas. Of the formal disturbances of 
association may be mentioned: (1) Anomalies in velocity; (2) anomalies 
in the order of association or mode of reaction; (3) anomalies in the inten- 
sity and duration of ideas; (4) anomalies in comprehension or apper- 
ception; (5) anomalies in so-called identical reproduction (memory); (6) 
anomalies in so-called original production (phantasy). The falsification 
of content of ideas is manifest in the production of the so-called insane 
ideas or delusions. 
Anomalies in the Velocity of Association; Flight of Ideas; Thought 
Inhibitions; Stupors.-The association of ideas may be pathologically 
accelerated or pathologically retarded. Even in health there seems to be 
an acceleration of the velocity of association during excitement, especially 
during joyful excitement. In maniacal states we meet with a peculiar 
form of association known as flight of ideas (Jdeenflucht of the Germans), 
which is often associated with the form of motor agitation known as 
pressure of activity and accompanied by marked gesticulation and rapid 
speech fogorrhoeaf In a careful study of the flight of ideas, however, it 
is found that we have to deal less with acceleration of association than INTRODUCTION TO DISEASES OF THE NERVOUS SYSTEM 
47 
with an alteration in the mode of reaction. The quicker thinking of the 
maniacal patient seems rather due to the fact that in the same time 
unit more different objects pass through consciousness than in normal 
states (Liepmann). In the healthy person ideation is dominated by some 
principal idea, while in mania the process of association is not controlled 
by such a dominant idea. One of the most striking characters of the 
associations in the so-called flight of ideas is the frequent change of 
direction of ideation. 
In normal man the kind of reproduction of ideas and the duration 
depend not only upon the reproduction tendency present in the individual, 
but also upon the effect of concentration upon the task set. Consciousness 
is, in a way, set, determined, or prepared in the sense of a specific task. 
In mania the task set has but little effect upon the process of association 
as compared with healthy states. The healthy man goes only very 
gradually from one ideational direction to another and tends to remain 
for a longer time within the confines of the idea made dominant by the 
task set. The healthy man, it is true, may for a short time experience 
rapid associations frequently changing in direction, the quality of the 
association then being superficial, like those seen in flight of ideas; but 
he is incapable of so continuing to associate for any length of time, 
whereas the characteristic of flight of ideas is the continuance of such 
associations until the physical strength is exhausted. 
At bottom the disturbance known as flight of ideas must be largely 
a disturbance of attention. The main change in the attention seems 
to be an increase in the capacity for directing the thought quickly and 
energetically to single ideas (hypervigility), and also away from them 
again (hypotenacity). To explain the abundance of ideas reproduced, 
however, it is necessary to assume also an alteration of the power of 
reproduction, permitting the appearance in consciousness of a wealth of 
lively ideas capable of being easily replaced one by another (Isserlin). 
Psychiatrists distinguish a primary flight of ideas (e. g., reminiscence 
flight in neurasthenic states and in the exaltation of mania) from a 
secondary flight of ideas due to quickly changing hallucinations and 
delusions; in the latter case the idea flight is not a new disease symptom, 
but is secondary to the affective excitation called forth by the hallucina- 
tions or delusions. When the associations run unbridled by a dominant 
idea, as they do in the marked forms of idea flight, there is an inability to 
follow anything like a normal train of thought {secondary incoherence). 
Pathological retardation of association (thought inhibition) may also 
appear, either as a primary phenomena or as a secondary symptom. 
When pronounced it leads to the condition known as stupor. Such 
slowed association is nearly always associated also with motor inhibition 
in which the so-called voluntary movements are slowed or absent alto- 
gether. The patient is unable to complete the series of association 
between the initial idea and the terminal motor idea. Speech becomes 
more and more slowed until there may be complete mutism. The various 
voluntary muscles of the body may be held rigidly (as in catatonic rigidity) 
As examples of primary stupor may be mentioned: (1) the so-called 
excitation stupor which appears in the slowly developing conditions of 48 
DISEASES OF THE NERVOUS SYSTEM 
cerebral excitation, and (2) the stupors which appear in the course of 
various intoxications. Unless the history is known in such cases it is 
sometimes difficult to tell whether one is dealing merely with an inhibitory 
process or with actual defect (dementia). As secondary stupor may be 
designated the form which accompanies or follows various states of 
psychic depression. An emotional shock or a paroxysmal anxiety condi- 
tion may call forth a severe secondary thought inhibition. A special form 
of secondary stupor due to delusions and hallucinations accompanied 
by strong affective states is met with in certain conditions of catatonic 
rigidity (pseudo-stupor of Westphal). 
The so-called waxy flexibility (flexibilitas cerea) may be regarded as 
a part of the general inhibition of association. 
Anomalies in the Order of Association or Mode of Reaction.-Perhaps 
most progress in recent years has been made in the study of the mode 
of reaction in association tests. A study of diseased conditions reveals 
marked deviations from the normal modes of reaction. It is customary 
to classify the kinds of association as follows: 
A. Sense of test words correctly understood. 
(a) Internal associations. (1) Associations of coordination and 
subordination. (2) Predicative associations. (3) Causality 
associations. 
(6) External associations. (1) Associations of spatial and 
temporal co-existence. (2) Identities. (3) Speech rem- 
iniscences. 
B. Sense of test words not understood. 
(c) Test words acting only through their sound. (1) Word 
supplements. (2) Sound associations and rhyme associa- 
tions. (A) Intelligible. (I?) Nonsensical. 
(d) Test words acting only by setting free reactions. (1) 
Repetition of test word. (2) Repetition of earlier 
reactions without sense. (3) Associations for words 
used earlier. (4) Reactions without recognizable con- 
nection. 
It is also important to note how many of the reactions are more objec- 
tive in nature and how many of them are more subjective (egocentric). 
A word of explanation as to the meaning of some of the terms used 
above seems desirable, By an internal association is meant one directed 
toward the sense of the test word, while an external association is one 
dependent upon custom, habit of speech, and sound relation. In the 
predicative associations there is some affirmation or assertion regarding 
the object of the test word. In the causality associations there is a rela- 
tion of cause and effect. By word supplementation one means only such 
reaction words as, taken with the test word, make together an indivisible 
word. Such reactions belong to clang reactions in the wider sense. 
A distinction is made between mediate association and immediate 
(Aschaffenburg). In the former the only way one can understand a 
connection between the test word and the reaction is by assuming some 
intermediate member of an association series. As a rule, such inter- 
mediate member turns out to be a clang association for the test word, INTRODUCTION TO 'DISEASES OE THE NERVOUS SYSTEM 
49 
while the relations of the reaction word to the intermediate member may 
be any one of the forms of association mentioned. 
It is customary to designate instances in which more than sixty seconds 
elapse before an answer is given to the test word as "faults." Reactions 
in which there is simply a repetition of the test word are in a class by 
themselves. It is well also to count up the number of perseverations and 
repetitions which occur. By perseveration is meant a reaction in which 
there has been evidently a connection on an earlier test or reaction word 
instead of to the actual test word given; by repetition is meant a reaction 
in which the test or reaction word immediately preceding recurs. 
Discursive associations should also be especially noted (Weitersch- 
weifen of Isserlin). By this is meant a spontaneous continuation of the 
association. Such further association may be intelligible or nonsensical. 
Special attention should be paid to the instances in which the test 
word is repeated by the patient. When this is a marked feature in a case 
it should be determined whether it is due to anomalies of comprehension 
from marked distractibility, in which the attempt to concentrate leads 
to repetitions, or whether it is dependent upon inability to draw upon the 
reproductive elements sufficiently, that is to say, is due to a lack of 
associated ideas (as in the thought inhibition of depressive states). A 
patient will often give the clue himself, since he may mention, on the 
one hand, that nothing occurs to him, or, on the other, that so many 
things pass through his mind that he repeated the test word in order to 
answer correctly. The two types are further distinguishable by the 
fact that, in the one, reproductions often in large number appear imme- 
diately after the repeated test word, while in the instances due to thought 
inhibition associations occur only after quite a pause, and often only 
after several slow repetitions of the test word. 
Experience with these association tests soon permits one by their aid 
to differentiate quickly among the great groups of psychotic symptoms. 
Another advantage of systematic monthly association tests consists in 
the ability to follow the exacerbation or retrogression of symptoms. 
In valuing the results of association tests one should familiarize himself, 
first, with a large material obtained from healthy people. Educated 
people show, on the average, a more "superficial" type of reaction than 
uneducated people, the latter reacting more to the sense of the test 
word than the former. Differences in individuality determine also certain 
important variations in association. Jung and Riklin distinguish two 
main types: (1) Objective types, and (2) subjective types with egocentric 
adjustment. To the objective types belong those persons by whom 
the test word is understood objectively, either according to its sense 
or as a speech stimulus, while to the subjective- types belong (a) the 
so-called "constellation" types, in which personal experiences (idea com- 
plexes) with strong feeling tone appear in the associations, and (6) the 
"predicate" types, which present lively, subjectively valued ideas. 
The reaction time of normal persons varies greatly according to the 
age, education, and individuality. The more phlegmatic and the intel- 
lectually feebler individuals show, as a rule, onger reaction times. The 
quality of the association is an important factor in its duration; thus the 
vol. v-4 50 
DISEASES OF THE NERVOUS SYSTEM 
more complicated an association is psychologically the longer the reac- 
tion time. The associations which are most used in daily life are those 
with the shorter reaction times, a fact which explains the velocity of 
"external" associations as contrasted with "internal" associations. One 
of the most important results of association tests is the bringing of the 
proof of the delay in reaction time in associations to which idea com- 
plexes with strong feeling tone are attached. 
Association tests in the melancholic states of manic-depressive insanity 
reveal a marked prolongation of the reaction time and apparently a 
characteristic change in the content of the associations. There appears 
to be a limitation to idea change. The reaction type is usually pre- 
dominantly objective. Even in the very delayed associations when the 
reaction is recorded, it, as a rule, corresponds in sense to the test word. 
In the maniacal states of manic-depressive insanity the striking features 
are a "flattening" of the associations and the replacement of associations 
due to the sense of the test words by associations which depend upon 
custom, speech, and mere sound relations (clang associations). The 
frequency of discursive associations (Weiterschweifen) in mania has 
been pointed out by Isserlin. As in flight of ideas in general the reaction 
times are not shorter than normal; indeed, they are sometimes really 
longer, although in maniacal states one does meet perhaps with a greater 
number of short reaction times than in health. The most characteristic 
phenomenon in mania is the greater number of single ideas produced 
during a given time-many more than one ever meets with in health. 
It is the extraordinarily frequent change of direction of the ideas which 
is so striking. Healthy persons are incapable of associating so quickly 
with such frequent change of direction and in such a "superficial" man- 
ner as is characteristic of the idea flight of maniacal states. 
Progress has been made in the study of associations in the so-called 
mixed states of exaltation and depression, in which certain of the signs 
of mania are associated with certain of the signs of depression. One 
tries to find out whether the reaction time is prompt or delayed, whether 
the type of reaction is according to the sense of the test words or super- 
ficial, whether the direction of the ideas is changed frequently or rarely, 
whether there are many or few perseverations and repetitions, whether 
the form of the answers indicates a precise reaction or a vague "talking 
around the subject," whether the feeling tone is positive or negative, 
and whether or not egocentric relations, faults, and test-word repetitions 
are prominent (Isserlin). In the "mixed states," egocentric relations 
may be extremely frequent. Test-word repetitions tend also to be 
especially common in these mixed states. 
In view of the help which comes from a study of the result of asso- 
ciation tests, some physicians seem inclined to overestimate their value. 
It should be borne in mind that they reflect only one side of mental life, 
and that one must not neglect other psychological methods of examina- 
tion. The general practitioner has not yet formed the habit, however, 
of resorting to association tests, and to him the method can be heartily 
recommended as a means of extending the anamnesis. Especially in the 
differential diagnosis of manic-depressive insanity from dementia precox 51 
INTRODUCTION TO DISEASES OF THE NERVOUS SYSTEM 
the method will be found helpful. If one bears in mind the characteristic 
signs of inhibition and excitation revealed in the patient's entrance upon 
the task of association in manic-depressive insanity, and compares there- 
with the refractory, perverse behavior with regard to the task char- 
acteristic of the patient suffering from dementia precox, and observes, 
further, the stereotyped replies and repetitions or perseverations which 
may seem to disregard the test words, the paralogia, the mannerisms 
which come out during the association tests, one will find the method 
one of the best available in distinguishing these two types of psychosis 
from one another. Even in cases where strong inhibition simulates 
apathy and dementia the association experiment helps to differentiate. 
If, in spite of long reaction times, we see an effort to understand the 
sense of the test word, we can, as Isserlin emphasizes, make the diag- 
nosis of manic-depressive insanity even when the change of direction 
of the ideas is only moderate and there seems to be relatively little affec- 
tivity. In dementia precox the patient reveals himself by the reaction 
time (ordinarily not so evenly prolonged as in the depression of manic- 
depressive and, when not normal, more lawlessly desultory) and by the 
apathetic or perverse behavior toward the task set. 
The association tests also help in differentiating the depressive states 
of manic-depressive insanity from some forms of hysteria. In the latter 
disease it is especially the emotional phenomena, the signs of the idea, 
complexes emphasized by feeling (gefuhlsbetonte Komplexe), which are 
characteristic. In manic-depressive insanity the inhibition is demon- 
strable aside from the slowing effect upon the reaction time of such 
emotive complexes, and, further, the associations in general in this psy- 
chosis present a much less capricious and variable appearance. It must 
not be forgotten, however, that hysteria and manic-depressive insanity 
may be combined in the same patient. 
Anomalies in the Associative Connection of Ideas (Incoherence or Disso- 
ciation).-Normally, the course followed in associations from the initial 
idea to the terminal idea is a well-regulated one, depending upon some 
dominant or superior idea. In many psychoses this normal connection 
of ideas in the associative processes is disturbed. The initial idea may 
be followed by a second idea which stands in no recognizable relation to 
it, an anomaly designated by psychiatrists as dissociation or incoherence 
of association. If a patient be asked what time it is, and she answer 
"fox," a severe disturbance of this sort is manifest. In milder cases 
the disturbance is manifested by the fact that the patient continuously 
loses the thread of thought. When a patient is disoriented as to time, 
place, and personal relations, along with general incoherence of asso- 
ciation, the movements and speech of the patient being correspondingly 
disturbed (motor incoherence}, the condition is technically known as 
confusion (V erwirrtheit of the Germans). Very often incoherence and 
confusion are used as synonymous terms. 
It is customary to distinguish a primary from a secondary incoherence, 
the former appearing autochthonously, independent of any other psycho- 
pathic symptom (e. g., in the so-called acute hallucinatory confusion or 
amentia), the latter as a sequel to other psychopathic phenomena (e. g., 52 
DISEASES OF THE NERVOUS SYSTEM 
flight of ideas, hallucinations, strong emotions, dementias, etc.). The 
secondary dissociation or incoherence is, therefore, common to many 
severe mental disturbances. The process of loosening or dissociation 
has been designated sejunction by Wernicke. When a dissociation occurs 
suddenly and lasts only a short time, there result lacunae in consciousness 
(the so-called psychic eclipses or deliquia of L. Meyer), which may form 
the starting point of manifold disturbances of the content of the 
ideas. 
In mild instances of general incoherence a peculiar symptom is some- 
times met with, known as pathological notions (Einfalle of the Germans). 
In the midst of a well-ordered and often extensive thought series, there 
comes suddenly and apparently without reason an idea or complex of 
ideas which stands in no connection whatever, by external or internal 
association as far as can be seen, with the former. 
Anomalies in the Intensity and Duration of Ideas.-Instead of the ordi- 
nary course of associations, single ideas or idea complexes may attain 
to abnormal strength and be very frequently repeated in consciousness. 
Here belong the exaggerated or hyperdynamic ideas (iibenoerthige Ideen of 
Wernicke) and the imperative ideas (Zwangvorstellungen of Krafft-Ebing), 
sometimes known as obsessions. In individuals who are constitutionally 
psychopathic such ideas arise especially at times when they are mentally 
or physically exhausted. They are especially characteristic in the so- 
called psychasthenic states (Janet). 
Wernicke distinguishes between hyperdynamic ideas, imperative ideas, 
and autochthonous ideas. The exaggerated or hyperdynamic ideas are 
characterized by the fact that the patients do not recognize them as 
intruders in consciousness; on the contrary, the patient regards them 
as the expression of his innermost nature, and in battling for them he is 
struggling for the maintenance of his own personality. They are, never- 
theless, often felt as troublesome, and the patients frequently complain 
that they can think of nothing else. Imperative ideas or obsessions, 
however, are recognized as unjustifiable ideas, and are often spoken of 
as absurd. The autochthonous ideas have the attention forcibly directed 
toward them and are felt as troublesome intruders. Wernicke separates 
them from imperative ideas, since the latter are not regarded as foreign 
to the personality of the patient, and hence are not serious for the mental 
life of the patient as are autochthonous thoughts. The closest relations 
between hallucinations and autochthonous ideas have been pointed out 
by various psychiatric writers; indeed, transitions between the two 
sometimes may occur. 
Many of the imperative ideas or obsessions are combined with marked 
affective disturbances, giving rise to the so-called phobias; thus, a 
patient may fear to cross an open place (agoraphobia), may fear shut-in 
places (claustrophobia), may fear contamination (mysophobia), may fear 
disease (nosophobia), or may be afraid of everything (panphobia). 
When the same idea comes up over and over again the patients may 
be led to stereotyped methods of expression. The patient may answer 
a first question correctly, but may tend to give the same answer to 
various other questions subsequently put (persevtion). INTRODUCTION TO DISEASES OF THE NERVOUS SYSTEM 
53 
In contrast with hyperdynamic ideas arc the hypodynamic ideas. 
Certain experiences which in normal life may be well remembered and 
attended to are lost sight of or do not seem to have their normal value 
in the patient's mental life. 
Anomalies of Reproduction or Memory (Hypermnesia; Hypomnesia; 
Amnesia; Paramnesia).-The power of reproduction may be pathologic- 
ally facilitated {hypermnesia), diminished or retarded {hypomnesia), 
abolished {amnesia), or perverted {paramnesia). 
Hypermnesia is often met with in exalted or maniacal states and is 
usually associated with abnormal affective phenomena. Past acts, feel- 
ings, or ideas appear vividly in the mind, which in its natural state may 
have wholly lost the remembrance of them. Ordinary individuals some- 
times experience hypermnesia in sleep or in certain unnatural conditions, 
e. g., under the influence of drugs (alcohol, morphine). 
Weakened memory or hypomnesia may betray itself in the increased 
length of time it takes to recall an image or in the vagueness of the 
reproduction. 
In outspoken pathological diminution or destruction of the power of 
memory {amnesia) there 4s complete inability for reproduction of a part 
or the whole of the past experiences. The term amnesia is by some 
reserved for complete loss of memory for a certain period; for example, 
that of dreamless sleep, of epileptic coma, of hysterical somnambulism, 
and the like. When the amnesia concerns not only the period of the 
pathological condition itself, but extends to a longer or shorter time 
preceding the attack, it is called retrospective or retrograde amnesia. The 
term anterograde amnesia has been used to designate the defect of mem- 
ory which extends to occurrences of the period subsequent to the termina- 
tion of the clouding of consciousness, a condition doubtless due to the 
depression of the recording faculty and the injury to the associative 
processes following the period of mental fog. When in the period sub- 
sequent to the termination of the mental fog the patient still remembers 
certain experiences, but soon after forgets them entirely, the condition 
is spoken of as retarded amnesia. Among the most interesting amnesias 
are those of the alternating personalities of hysterical patients, in which 
the first and third and second and fourth may stand in close associative 
connection with one another, while in the second and fourth states 
complete amnesia for the experiences of the first and third exists. 
The amnesia may be partial, involving only the events of the recent 
past and not those of the distant past. Especially in progressive failure 
of memory, such as that met with in dementia, there is a gradual loss 
of memories, beginning with the most recent ones and involving grad- 
ually those of the past {law of regression of Ribot). 
Circumscribed amnesias in special domains are met with very fre- 
quently in focal diseases of the brain. Thus, when optic memory images 
can no longer be called up, the condition is known as mind blindness 
{Seelenblindheit of the Germans). The mind-blind person may still have 
intact visual sensations, but he is unable to recognize what he sees because 
his visual memories are lost or, at any rate, cannot be awakened in the 
ordinary way by retinal stimuli, since the association between the primary 54 
DISEASES OF THE NERVOUS SYSTEM 
optic centres in the cortex and the areas concerned with visual memory 
are interfered with. Two great groups of cases can be distinguished from 
one another. In the first group we have to deal rather with a disturb- 
ance in optic perception than in optic memory. In this group belongs 
the so-called apperceptive form of mind blindness of Lissauer, which 
corresponds to the so-called cortical blindness (Rindenblindheit of Munk). 
In this group the principal disturbances are hemianopsia, diminution of 
visual acuity, and disturbance of color sense. Stereoscopic vision is also 
sometimes involved. On the psychic side there is usually an incapacity 
for orientation in space. There need not, however, be any marked dis- 
turbance of the visual memory or of the capacity for recognizing ordinary 
objects. Reading and writing may be nearly intact. 
The second group of cases includes mind blindness proper, which is 
less a disturbance of perception than a loss of capacity for the intellectual 
valuation of retinal images. The condition is spoken of by Lissauer as 
the associative form of mind blindness. The patient is unable to recognize 
objects shown him because the visual images do not call up by association 
the memories which permit their identification, and so they appear to 
him as strange confusing figures (optic asymbolia}. A patient looking 
at his wife, may know that she is a woman and see the details of her form 
and clothing exactly, but yet not recognize that she is his wife. 
If very circumscribed areas in which optic memories are localized or 
the pathways to them be destroyed, the phenomena of word blindness 
or of optic aphasia may be met with. In word, blindness or alexia there is 
an incapacity to read, although speech and the understanding of speech 
may be retained. The power to write may or may not be present. In 
the former case one speaks of subcortical or pure alexia (probably due to 
lesion of the the associative .paths between the visual sense area in the 
cortex and the sensory speech areas). In the optic aphasia of Freund, a 
rare condition, objects held before the patient are seen and recognized 
but cannot be designated, although the patients can otherwise speak 
well and can find the particular word concerned when the stimulus is 
awakened through some other sense organ. It appears to follow most 
frequently upon lesions at the junction of the left occipital and left 
temporal lobes interrupting the association paths which extend from the 
two occipital lobes to the centre for word-clang memories. 
A similar isolated loss of acoustic memorial images is known as mind 
deafness. The patient may hear everything, but cannot recognize the 
sounds. The most frequent form met with is that known as word deaf- 
ness in the sensory aphasia of Wernicke. The patient hears the word, 
but no longer recognizes it and cannot understand it (lesion of the first 
temporal gyrus on the left side). Here, too, we probably have to dis- 
tinguish a group of cases in which the disturbance is perceptive and a 
second group in which it is associative, the latter being sometimes referred 
to as amnestic aphasia. 
When tactile memorial images can no longer be awakened we have 
mind anesthesia for touch. The patients feel contact, but cannot recog- 
nize objects through it. Similarly, there may be a loss of the memorial 
images for kinesthetic sensations. INTRODUCTION TO DISEASES OF THE NERVOUS SYSTEM 
55 
Under the term paramnesia come the falsifications of memory, includ- 
ing what Sully has described as memory phantasms and memory illusions. 
In the memory phantasms the patient seems to remember things which 
he has never experienced. The combinations of pure phantasy are 
regarded as actual experiences. They seem to be due to a loss of critical 
power, and depend upon enfeebled judgment. Here must be placed the 
tendency to confabulation and pseudo-reminiscences, so common in 
Korsakow's psychosis. 
By memory illusions the memorial images themselves are falsified, the 
disturbance being due to faulty reproduction and lively fancy or the 
falsifying influence of a temporary affective state. As Sully puts it, the 
past now appears in the colors of the present. Such patients may appear 
to be liars, since they unconsciously distort their experiences in repro- 
duction. A very good example of such illusions of memory is often seen 
in connection with the uncinate gyrus fits of H. Jackson. 
A special form of memory deception is seen in the identification of 
a present situation with one presumably previously experienced. This 
sentiment du deja vu is one of the most interesting paramnesias met with 
among psychasthenics. It seems to be as much a disturbance of per- 
ception as of memory. The patient, feeling that impressions escape him, 
comes to the belief that he is experiencing memories. 
Methods of Testing Memory.-The tendency among neurologists at 
present is to treat all memory loss not as a disturbance of a unitary 
capacity of the brain, but rather as disturbances of a large group of 
single capacities. It is true that the memory as a whole, that is, the 
conglomeration of all these partial faculties, may be impaired particularly 
in some of the psychoses, but in neurological conditions in which cir- 
cumscribed lesions of the cerebrum are met with we see more often injur- 
ies of the partial memory faculties. On testing the memory clinically, 
therefore, one must use methods which permit us to judge not only of 
the memory power in general but of the capacity for and fidelity of 
reproduction in each individual domain. 
It is best to begin by testing the memory for fresh impressions or for 
recent events. One determines a defective power for the immediate 
acquisition and retention of ideas (recording faculty), and also defective 
capacity for the acquisition of general and relative ideas (power of 
abstraction). In addition, it is necessary to test the memory for older 
impressions or for events which have occurred earlier in the life of the 
individual, and here one does best to examine the patient's memory 
for what he has learned at school and to try his power of calculation. 
In testing school memories, one should have some knowledge of the 
extent to which the patient has been educated, as obviously the testing 
of illiterate persons for school memories would be useless. It is advan- 
tageous to use a uniform set of questions in order that comparisons of 
value may be made among the replies received from different patients. 
It is customary, therefore, to ask the patient to repeat a number of 
series which in school are learned by heart; for example, the alphabet, 
the numerals, the names of the months, and the names of the days of 
the week. Certain national and religious facts which everyone may 56 
DISEASES OF THE NERVOUS SYSTEM 
reasonably be expected to know may also be inquired into. To these 
questions may be added others regarding well-known facts of geography 
and history. Normal answers do not necessarily indicate mental nor- 
mality since in non-paralytic dementias, for example, school memories 
are often very well retained. 
The solution of the problems themselves is not the only important 
point. The time taken to solve them and concomitant phenomena of 
physiognomy and speech should be taken into account. The use of these 
methods has demonstrated that certain phenomena recur typically in 
different groups of clinical cases, and Sommer especially recommends 
the methods for the differential diagnosis of different forms of dementia. 
Through their application one obtains not only a more exact insight into 
the partial functions of the power of calculation, but also can give mathe- 
matical expression (where tests are repeated at intervals in the same 
cases) to the symptoms of periodic variation, of advancing intellectual 
disintegration, of sterotypism, etc. 
Falsification of the Content of Ideas (Delusions).-The power of forming 
judgments and conclusions by means of the association of ideas depends 
in health upon the existence of certain regular relations between the var- 
ious memories derived from preceding sensations regarding the body 
and the external world. Judgments depend, however, not entirely upon 
one's own personal experience, but upon tradition and upon the ideas 
dominant in the circle in which one lives at the time. One's own personal 
knowledge is supplemented by certain prevalent beliefs (religious, scien- 
tific, political, etc.). On account of the stronger feeling tone which 
accompanies beliefs their influence upon the power of judgment can 
scarcely be overestimated. While it is true that men of rich individual 
experience with strong powers of observation and analysis may attain 
to a high power of independent judgment; still no one, even the intel- 
lectually greatest, remains uninfluenced by belief, for individual expe- 
rience, no matter how extensive, must be supplemented by beliefs which 
bridge over the gaps of experience. Everyone is, therefore, liable to 
make mistakes due to prejudice and superstition, and there is every 
transition from the mistaken judgments of human beings whom we call 
normal to the delusions or false judgments of the insane. In both instances 
the false judgment arises as a result of combinations of ideas which are 
out of accord with the facts of the so-called external world. The mistakes 
of the normal man can, however, be corrected by subsequent judgments 
depending upon new perceptions and more correct inferences, but the 
insane delusion is not amenable to such correction. Even in people 
who are not insane there may be certain false judgments which cannot 
be corrected. People brought up in an atmosphere of strong prejudice 
and superstition may be incorrigible in their beliefs and yet not be insane 
in the strict sense. Believers in Christian Science, in spiritualism, and 
the like, may form false judgments, but we would hestitate to call them 
insane. The false judgments are to be regarded as insane delusions when 
associated with other undoubted phenomena of alienation. 
Insane delusions are divided into primary delusions and secondary 
delusions. The former usually arise from abnormal states of feeling in INTRODUCTION TO DISEASES OF THE NERVOUS SYSTEM 
57 
connection with a pathological exaggeration of the consciousness of 
personality. The latter arc, as a rule, explanation delusions or attempts 
on the part of the individual who has passed through a psychosis with 
injury to his mental processes to explain the changes in his consciousness. 
In the primary delusions egocentric ideas dominate the consciousness 
and lead to the formation of false ideas of reference-that is to say, to 
false ideas regarding the individual and the processes of the external 
world. Such false ideas may, if there be a decided alteration in the 
associative mechanism; form the starting point for a permanently develop- 
ing chronic delusional system (paranoid states). Clinically such individ- 
uals may appear to have normal recording faculty, recognition, and 
memory, but despite thereof manifest fundamental disturbance in the 
formation of judgments. On superficial examination such patients may 
seem very intelligent, and one would not suspect them of being insane 
until one learned from prolonged conversation the falsification of their 
judgments regarding their own personality and their entire lack of disease 
insight as regards such falsification of the content of their ideas. Tem- 
porary and curable delusions may occur in acute psychoses as a symptom 
of inhibition of normal associations, but the chronic, incurable delusional 
insanities depend rather upon permanent loss of certain possibilities of 
association, the patient being unable critically to sift and arrange his 
judgments as a normal individual can do. 
Many of the chronic systematized and progressive delusional states 
begin with a period of psychic eclipse. The patients complain that for 
some days or weeks their minds have been empty and that they have 
felt restless. Some go so far as to assert that they must have been anes- 
thetized or poisoned at the time. In the acute psychoses, especially 
those accompanying infections and intoxications, delusions are prone to 
arise as a result of the primary incoherence. The mixed-up ideas of 
the patient lead to peculiar and nonsensical judgment associations. In 
some cases delusions arise in which a primary associative disturbance 
cannot be demonstrated, the falsification of content of the ideas here 
depending upon pathological affective processes. 
There can be no doubt that illusions and hallucinations are of great 
importance also for the origin of delusions. This is well illustrated in 
the so-called acute hallucinatory confusion or amentia; in curable cases 
the delusions disappear, but where a dementing process succeeds, the 
hallucinatory delusions may continue unaltered or develop farther. 
In chronic paranoid states the delusions may precede the hallucination 
and illusions, the latter being the outcome of the former. In the so-called 
acute hallucinatory paranoia the delusions arise primarily (in contrast 
with amentia, in which they are more secondary); in this disease the 
primary suspiciousness of the patient may be followed by hallucinations 
which convert the suspicions into certainty. 
Explanation delusions (secondary delusions in the strict sense) occur 
where pathological states of feeling are protracted (prolonged exaltation 
or prolonged depression) and form the basis of the false judgments. 
The hyperdynamic ideas corresponding to an exalted state may easily 
lead to delusions of grandeur. Similarly, the hyperdynamic ideas cor- 58 
DISEASES OF THE NERVOUS SYSTEM 
responding to the depressed mood of the melancholic and the hypochon- 
driac may become fixed as depressive delusions, which persist even after 
the pathological mood has disappeared. 
The expansive (megalomanic) delusions and depressive (micromanic) 
delusions together make up the primordial deliria of Griesinger. Depres- 
sive delusions include the delusions of sin, the hypochondriacal delusions, 
the poverty delusions, and the delusions of persecution. 
Delusions of persecution differ from the other forms of micromanic 
delusions in that other people (enemies, persecutors) are held responsible 
therefor. Sometimes they arise primarily and are connected with abnor- 
mal sensations. The feeling of fatigue which sometimes follows the 
drinking of a glass of beer may excite the persecutory idea that the 
beer has been poisoned or that someone intended to drug the patient. 
Still more frequently persecutory ideas arise as a result of hallucinations 
or illusions. Complementary or contrasting persecutory delusions are 
often associated with delusions of grandeur, the patient assuming'that 
he is being attacked on account of the greatness of his position. 
Ideas of reference (Beziehungswahri) may be the forerunners of ideas 
of persecution. The patient refers looks, acts, remarks of other people 
to himself without sufficient reason. He thinks he is being influenced 
or watched or injured by someone else. He may become abnormally 
jealous {jealousy delusion) or he may get the idea that he is suspected of 
a crime and that he is to be brought into court (imputation delusion). 
The patient may attribute the persecution sometimes to definite persons 
about him, sometimes to invisible and unknown enemies. Sooner or 
later such a patient is likely to become convinced that a great conspiracy 
has been formed against him. 
A word must be said about the so-called general delusion of denial 
(delire de negation generalise of the French). It usually follows delusions 
of sin, the patient becoming so bad in his own estimation that he identifies 
himself with Satan. He can atone for this only by eternal punishment, 
and so comes to regard himself as immortal. Such a patient may think 
that his body is infinite in size as well as in duration (delire d'enormite). 
When delusions first appear they are usually accompanied by affective 
states which correspond to the content of the delusion, but after long 
standing this feeling tone of the delusions may disappear. 
This mode of onset of a delusion may be sudden or it may develop 
gradually. Primary delusions tend to have the latter form of develop- 
ment, and are more apt to become fixed; hence the unfavorable prognosis 
in such cases, as a rule. 
Fixed delusions are prone to be followed later by a so-called systematiza- 
tion, the patient adding complementary delusions to the fixed delusion, 
the new delusion being connected logically with the original false judg- 
ment. Such systematized delusional states may last throughout life. 
To be distinguished from the delusions above described are the defects 
in judgment met with in imbeciles and patients, who for some reason 
or another have had some arrest of development. In such cases the 
memory pictures are defective and the associative relations among 
them are fewer than normal; as a result, incorrect judgments are very INTRODUCTION TO DISEASES OF THE NERVOUS SYSTEM 
59 
likely to be formed. As Ziehen puts it, poverty in ideas and in associative 
connections among them, together with feebleness of judgment, are the 
essential features of the intellectual defect, both in acquired and congenital 
feeble-mindedness. 
All grades of such feeble judgment are met with, from the mildest 
lack of critical power to the most complete lack of judgment. The former 
is due to absence of a few complicated ideas and associations, while 
the latter depends upon absence of the simplest everyday ideas and 
associations. Imperative ideas and delusions often disappear, but the 
feeble judgment once established nearly always remains. 
Anomalies of Affective and Emotional States.-We have referred 
to the feeling tone accompanying sensations and ideas. Whereas sensa- 
tions and their memory pictures (the so-called ideas or images) repre- 
sent the elementary phenomena of consciousness which refer to objects 
external to a given perceiving subject or personality, the feelings, on the 
other hand, and the affective or emotional states in which they play the 
essential role, represent the more intimately subjective side of experience 
in that this portion of the content of consciousness expresses the attitude 
of the individual to objects, etc., in its most fundamental form; that 
is, in particular, as it bears upon his conduct. 
The elements of our experience of external objects about which no 
difference of opinion exists are sensations. In the same sense the ele- 
ments of the affective side of consciousness include agreeableness and dis- 
agreeableness (pleasure and pain of the older psychologists, and positive 
and negative feeling tones used by physiologists generally); these two 
at least, are elementary feelings. Whether Wundt's assumption that 
there are six directions in which elementary feelings may vary-the 
agreeable and disagreeable, the exciting and depressing, and the straining 
and relaxing-be correct or not need not here detain us. 
Emotions are more complex than simple feelings, in that they contain 
not merely affective, but, quite as essentially, sensational (perceptional) 
elements also. The analysis of an emotion, for example, fear, reveals not 
only a feeling of disagreeableness, but also the sensational factors which 
represent the object which is feared, with complexes of kinesthetic and 
visceral sensations, the exact nature of which has not yet been discovered. 
The close relation between emotions and bodily movements is evident. 
Moods are of the nature of emotions having the same general consti- 
tution, but, as a rule, they lack definiteness in that the sensational or 
objective side of a mood is not so frequently one particular object. 
Moods are, in general, of longer duration than emotions, and do not so 
readily express themselves in some definite act which tends to bring 
the state, as in emotion, to an end. On the contrary, they persist for 
longer periods as a kind of general affective tone of experience (e. g., 
depression, exaltation). Manifestly, in both emotions and moods, mem- 
ory and imagination are of, at least, quite as much importance as the 
sensations of the present moment, and in the majority of cases of much 
greater significance, since they permit of the constant development of 
the objective content of both emotions and moods entirely freed from 
objective control. 60 
DISEASES OF THE NERVOUS SYSTEM 
Feelings and emotions become associated with objects other than 
those originally experienced with them, and this association occurs at 
times in most fantastic ways. This fact of the transfer of feelings helps us 
to understand the origin and permanence of moods in both those of 
normal and abnormal mentality. 
It has long been held that the feelings have an important relation to the 
state of the body; as far back as the seventeenth century one meets the 
view (in Hobbes) that pleasurable feelings are associated with bodily 
well-being, while disagreeable feelings are both expressive of and condu- 
cive to the ill-being of the body. 
The effects of strong feeling and emotion upon the intellect are often 
marked, depending, of course, upon the intensity as well as upon the 
quality of the affective state. If not too strong, the association of ideas 
is favored and accelerated, but when violent there may be inhibition or 
even complete arrest. The positive affective states tend to increase the 
rapidity of associations, while those negative tend to retard them. 
1. Pathological Exaltation or Hyperthymia.-This is best seen as a 
primary phenomenon in maniacal states. All the intellectual processes 
are accompanied by positive feeling tone. Even some of those which 
normally are associated with slight negative feeling tone appear in many 
to be accompanied by joyful feelings. It is not known whether the 
hyperthymia is really primary or is secondary to the psychic overexcita- 
tion. The experimental production of pleasurable feelings by the use 
of alcohol, opium, etc., supports the view that .the affective state may be 
primary (Binswanger). Hyperthymic states may appear in the course of 
many psychoses, both acute and chronic. The hyperthymia is usually 
an episodic phenomenon. A form of secondary exaltation has been 
described in acute and chronic paranoid states. Here hallucinations and 
delusions of joyful content determine the affective anomaly (Ziehen). 
2. Pathological Sadness (Depression) or Dysthymia.-This pathological 
predominance of negative feeling tones may be primary or secondary. In 
the primary depression the sadness may be out of all proportion to that 
which would normally accompany any sensations and ideas present. 
The depression may, indeed, be present without any apparent reason 
whatever. In addition to his general sadness the patient may present 
symptoms of anxiety. Usually this anxiety is accompanied by abnormal 
somatic sensations (precordial anxiety, epigastric pulsation, general 
unrest). Many have looked upon these somatic sensations as primary, 
but it seems more likely that they are cortical in origin. 
The motor phenomena accompanying anxious states are characteristic. 
The rubbing of the hands, the restless movements of the legs, the rapid 
respiration, the palpitation of the heart, the constriction of the peripheral 
vessels are some of the motor and vasomotor phenomena accompany- 
ing anxious states. Such primary depressions with anxiety are usually 
followed by symptoms of mental retardation (thought inhibition) and 
the patient answers questions and performs the simplest calculations 
only very slowly. The general musculature of the body may be abnor- 
mally lax or abnormally rigid, as in the catatonic states. Where the 
anxiety movements of the body are pronounced, one speaks of anxious INTRODUCTION TO DISEASES OF THE NERVOUS SYSTEM 
61 
agitation. Sometimes movement inhibition alternates with agitation of 
movements. 
The patients are inclined to seek an explanation for their anxiety, and 
then arise the so-called explanation delusions. These may take the form 
of delusion of sin, of poverty, or of incurable disease. Primary depressions 
are met with as episodic states in various psychoses; in a mild form they 
are not infrequent in neurasthenia. The severest forms are met with in 
true melancholia. In the so-called secondary depressions the affective 
states seems to be directly dependent upon sensations and ideas with 
strong feeling tone. 
3. Pathological Irritability.-The pathologically irritable person shows 
an abnormal tendency to anger and vexation. These affects appear too 
easily; exciting causes which in a normal person would not cause vexation 
call forth an affective reaction of abnormal intensity and long duration. 
Anger is an affect which acompanies the tendency to attack persons 
or objects in the surroundings. Although it is a negative affect, it differs 
from sadness or depression in that the feeling of self is usually elevated. 
The association of ideas may at first be slowed, although as the anger 
grows there may be a sudden and explosive acceleration of the course of 
ideas. At the beginning there may be a tendency to motor inhibition, 
but after a time, as though through summation, actions are accelerated 
and are often explosively violent (raving mad). 
During anger there is but little time for motives to come into play. 
The patient seems to fail to consider things at all; inhibitory ideas seem 
to have lost their power, or do not often come into his consciousness. 
IIis violence pays no regard to the welfare of his own person or of his 
surroundings. The energy of the movements may be greatly increased. 
The movements of anger are, however, so irregular as to be almost inco- 
herent. This reveals itself in the speech of the angry man. He stammers 
and his sentences lack grammatical sequence or coherence (anacoluthia'). 
It is not surprising, therefore, that after an attack of anger has passed 
individuals frequently show a partial amnesia of the motives leading to 
the passion, and even of their acts during the outbreak. 
4. Pathological Apathy, General and Circumscribed (Hypothymia; Athy- 
mia).-In certain mental states feeling tones appear to be depressed or 
abolished. The condition is most pronounced in cases of melancholia. 
In many instances of neurasthenia the patients complain of loss of interest 
in things which normally should arouse them. Their feelings for their 
ideals have left them and they complain that to those dearest to them 
they have become indifferent. Some patients seem much exercised over 
this coldness of feeling; others show no depression on this account. 
An appearance of general apathy may be simulated when it does not 
exist. The non-participation of the patient in her usual interests may 
be due to a conscious suppression of emotional expressions, depending 
upon delusions, or it may be the result of a motor inhibition. 
In contrast with this general apathy or depression of emotional excit- 
ability, as a whole, are the circumscribed defects in intellectual and 
reflected feeling tone met with especially in imbecility and dementia. 
In imbecility there is a faulty development of the emotional nature which 62 
DISEASES OE THE NERVOUS SYSTEM 
may keep paee with the defect in intellectual development or may exceed 
it, as in moral insanity. In imbecility of milder grade, anger, envy, 
hate, love, gratitude, hope, and fear may be well developed, but the 
individual does not attain to any intellectual interests. In spite of care- 
ful education it is impossible to arouse interest in art or science. In 
still milder cases the ethical or altruistic feelings alone may be deficient, 
the egoistic feelings being abnormally strong. 
In acquired dementia, such as one sees in dementia paralytica, the 
loss of the higher feelings usually goes parallel with the loss of intel- 
lectual power. The changes in this disease show us very clearly the 
relation of conduct and behavior to our emotional life (altruistic, social, 
religious feelings). In alcoholic dementia, epileptic dementia, and in 
senility one meets with symptoms similar to those presented by the 
paralytic dement. In the later stages of these acquired dementias the 
whole emotional life may be undermined and a general apathy result. 
5. Pathological Instability of Feelings (Moods).-In normal individuals, 
there is a certain persistence or inertia to emotional states. In mental dis- 
ease the moods are often much more variable. Two forms of pathologi- 
cal instability of moods may be distinguished, a primary and a secondary. 
The primary form occurs most often in imbecility and dementia, where 
it is associated with intellectual defects. The loosening of the associative 
connection characteristic of the intellectual defect also accounts for the 
instability of the irradiated feeling tones. A new sensation or idea will 
then sometimes in a moment lead to new irradiations of feeling tones 
and cause a bouleversement of mood. This is best illustrated perhaps in 
dementia paralytica, in which a patient in tears or anger is by a suitably 
chosen word converted into a state of joyful exaltation. 
In the secondary form of instability of moods the changeability of 
feeling is not a primary affective disturbance, but is secondary to a 
pathological inconstancy and incoherence of the contents of the ideas or 
sensations of the patient. Where the ideas are constantly changing 
the moods also change. This is well illustrated in hysteria and in some 
forms of paranoid states. A pathological capriciousness is very char- 
acteristic of many hysterical patients. 
6. Pathological General Increase of Affective Excitability.-In contrast 
with general apathy, a general increase of affective excitability seems 
sometimes to be met with. The individual is abnormally susceptible 
for all emotional impressions. This is the case in some instances of 
neurasthenia, but it is also occasionally met with at the beginning of 
severe organic psychoses. When the increase in excitability is limited 
to the so-called higher feelings-ethical, esthetic, and religious-it is 
designated as pathological transport or ecstasy (krankhafte Er griffenheit'). 
Such patients become over-enthusiastic for political, religious, or humane 
movements. When delusions develop upon the basis of this pathological 
ecstasy an eknoic state is said to exist. 
The so-called imperative affects are those pathological feelings which 
arise without cause, and which the patients themselves designate as 
strange or compulsory and independent of imperative ideas, delusions, 
or hallucinations. A patient in good humor may suddenly and without INTRODUCTION TO DISEASES OF THE NERVOUS SYSTEM 
63 
reason have a strong sympathy or a strong antipathy for some person 
near him, combined with a feeling that some force has artificially brought 
about such a tendency in him. 
Anomalies of Conduct (So-called Will or Volition).-Psychologists 
no longer recognize will as a special "faculty" of the mind. Conduct 
is, for them, the direct result of the total content of consciousness (sen- 
sations, percepts, memories, imaginations, feelings, emotions) of the 
moment. In states in which there is a general increase of motor action 
in mental disease we speak of motor agitation; where motor action is 
diminished or abolished, as a whole, we speak of about ia or motor stupor. 
In observing anomalies of volition one should notice not only the ordi- 
nary voluntary acts, but also alterations in speech, in expression, and in 
gesticulation. The so-called voluntary movements depend directly upon 
an associative process leading to some goal idea, while the expressive 
movements (mimic and pantomimic) appear to be determined chiefly 
by the intensity and quality of the feelings. Disturbances of sensation, 
of memorial reproduction, affective disturbances, as well as of the asso- 
ciation of ideas proper, can all influence conduct. Of the sensory dis- 
turbances which influence volition, hallucinations and illusions are most 
important. A sudden hallucination may lead to a wholly unexpected 
act of violence. 
Wernicke has classified movements (outside of the reflex movements) 
into expressive, reactive, and initiative movements. By expressive 
movements are meant all those by which the affective and emotional 
states of a person are manifested. The reactive movements are those which 
follow direct external stimulation (answer to questions by speech or 
other movement; behavior on physical examination). The initiative 
movements are those which arise apparently spontaneously and not as a 
result of an immediate external stimulus, the whole conduct, behavior, 
action of a person in a certain situation. All movements not reflex, 
expressive, or reactive belong to the initiative group. Wernicke divides 
all psychomotor disturbances into (1) those in which there is lowered 
excitability or conductivity of nerve paths (akinesis); (2) those in which 
there is increased excitability or power for conduction (hyperkinesis)-, 
and (3) those in which there is a perverse excitability (parakinesis). 
Action may be pathological on account of the absence of ideas which 
are present in normal states; this absence of ideas may be due to defective 
development (imbecility) or to loss of memory images, following upon 
disease (acquired dementias). Pathological actions of this sort are 
designated defective acts. They often resemble the normal acts of lower 
animals. They may be sly and skilful, but are pathological in human 
beings on account of the fact that they are uninfluenced by higher and 
more abstract ideas. 
In the motor agitation of mania the pleasurable feelings lead to remark- 
able mimic and pantomimic motor discharges and to a logorrhoea char- 
acterized by rhymes and alliterations. 
In the milder forms of maniacal exaltation in which the associative 
connection of the ideas is still fairly well retained, initiative movements 
occur more rapidly and in greater numbers than normally, and lead to 64 
DISEASES OE THE NERVOUS SYSTEM 
conduct which, although apparently consistent, is absurd in its motives 
and aims. In the severer outbreaks, stormy, impulsive acts result from 
the hodge-podge of ideas which swim through the consciousness. 
The inhibiting and slowing of motor action which accompanies negative 
feeling tones is characteristic of melancholic states. In simple depression 
pantomimic movements (gesticulation) are reduced to a minimum. The 
voluntary muscles are generally relaxed, the arms hanging loose by the 
sides or the hands folded on the lap. The head sinks upon the breast, 
the eyes are lowered, and there is a tendency to convergence. The 
mandible falls and the angles of the mouth are drawn downward. The 
lid slits are narrowed and the eyebrows lowered except at their medial 
extremities. Usually the patients do not weep; indeed, they sometimes 
complain of an inability to weep. Often the secretion of the lacrimal 
glands is actually diminished, accounting for the lack of lustre of the 
eyes. Depressed patients have little to say, and speak in low tones. 
The influence of depression upon motor activity becomes changed 
as soon as a state of anxiety is added to the depression. At first anxiety 
increases the inhibition of cortical associations and tends also to slow' 
action, but as the anxiety increases there arises the idea of flight, and 
the patient seeks in every wTay deliverance from his anxiety. He cannot 
rest and is impelled to walk about day and night, lamenting his state 
(motor agitation of anxiety). It is here that the patient so often seeks 
relief in suicide. Perfectly quiet for a time as a result of motor inhibi- 
tion, the anxious patient may suddenly make a suicidal attempt, or 
become violent. Other patients try to still their anxiety by alcoholic 
excess or onanism. These attacks due to anxiety occur in paroxysms. 
Between the paroxysms the motor inhibition of depression is resumed. 
The behavior of apathetic patients varies according to the extent of 
the apathy (general or partial). In general apathy motor activity is 
reduced to a minimum. Since, normally, acts occur only when move- 
ment ideas with positive feeling tones arise in consciousness, acts cease 
in general apathy because most of the movement ideas which arise in 
consciousness are devoid of feeling tone (apathetic motor stupor). In 
partial apathy, w'here certain only of the feeling tones are lacking, it is 
the conduct which depends upon the presence of more complex and 
especially the ethical ideas which is most prone to be defective. The 
patient acts wholly according to his low'er egoistic interests, not dis- 
tinguishing between right and wrong. 
In general apathy all the muscles of the body are lax; even the cheeks 
hang down. The head and limbs assume positions which depend upon 
gravity. The upper lids fall so as to simulate ptosis. The patients incline 
to lie down much of the time; in severe forms the apathy may resemble 
sleep. The condition of the pupils distinguishes the pseudo-sleep of 
apathy from true sleep; in the latter, when the eyelid is opened there 
is at first dilatation and subsequent contraction, while in apathy the 
pupils are usually midway between dilatation and contraction and there 
is a distinct contraction when light enters the eye on opening the lid. 
Acceleration of Motor Actions -When motor discharges occur more 
rapidly than normal we speak of motor agitation, or sometimes of patho- INTRODUCTION TO DISEASES OE THE NERVOUS SYSTEM 
65 
logical pressure of activity. One meets with every degree of it from 
abnormal talkativeness with overactive facial expression to the enormous 
acceleration of speech known as logorrhoea. The mimic and pantomimic 
movements may be so much exaggerated that we have excessive grimac- 
ing and violent gesticulation. The patients are restless, they assert 
that they cannot sit still, and they spend their time pacing up and down 
the room or wandering about. Such patients may be pathologically 
busy, beginning a dozen and one tasks hastily and enthusiastically but 
quickly turning from each. 
In the severest forms of motor agitation when the patient becomes 
"raving mad," his cries may become inarticulate and his movements 
violent and purposeless. Such a patient may tear up his clothing or his 
bedding, break the furniture of his room, and attack people near him. 
Psychiatrists distinguish a primary from a secondary pressure of 
activity, the former corresponding to primary flight of ideas and occurring 
with it, the latter depending upon hallucinations or violent affects 
{hallucinatory agitation and affective agitation). 
Retardation of Motor Actions.-Slowing of motor acts accompanies 
depressed states, which are accompanied by thought inhibition. When 
there is motor inhibition, together with thought inhibition and aprosexia, 
the condition of stupor is said to exist. One meets with mild and severe 
grades of motor stupor. In the milder forms movements seem difficult 
and are carried out more slowly than normal. The patient understands 
questions or commands less promptly than when he is in health. He 
pronounces the words of the answer more slowly, and any movements 
he makes are more deliberate than they should be. Mimic and pan- 
tomimic movements may be suppressed entirely. In his every-day 
life the patient manifests feeble will (aboulia), and is the victim of 
indecision. 
When the motor stupor reaches a high grade it may assume any one 
of three different forms: 
1. There may be complete relaxation {resolution) of the body muscu- 
lature, the patient lying for weeks or months without stirring a limb, 
with complete absence of resistance to passive movements, the eyes 
kept closed. 
2. There may be a general rigidity or tension of the muscles of the 
body {catatonic rigidity or attonnity), the body being held stiffly in a 
general position of flexion (flexion type) or of extension (extension type), 
or in various bizarre attitudes, the eyes held tight shut, the teeth clinched. 
Passive movements are strongly resisted, and, as a rule, attempts at 
passive movement increase the general rigidity. The patients are mute 
and in general negativistic. Sometimes, how'ever, there is a peculiar 
susceptibility to motor suggestion known as command automatism. On 
passive movement the limbs move like wax, retaining the position in 
which they are put often for three or four hours {flexibilitas cerea); 
sometimes they repeat movements made before them {echopraxid), or 
repeat words pronounced in their hearing {echolalid). 
3. There may be a limitation of initiative movements to a few acts 
repeated continuously for hours, days, or months at a time {stereotyped 66 
DISEASES OF THE NERVOUS SYSTEM 
movements'), the patient rocking to and fro or from side to side, or con- 
tinuously rotating the head or the trunk. 
One tries to make out in each case whether the motor inhibition is 
primary (true stupor) or secondary (pseudo-stupor). Primary motor 
stupor corresponds to primary depression of thought activity, being 
part of the general slowing of associative processes (e. g., in melancholia). 
Usually it takes the type of relaxation of the muscles or of simple cata- 
tonic rigidity. In secondary motor inhibition the pseudo-stupor may 
depend upon definite hallucinations, delusions, or affects.1 
Incoherence of Motor Actions.-When there is incoherence of the asso- 
ciation of ideas the motor acts also become incoherent (motor incoherence); 
the patient is in a state of confusion. In its mild form it betrays itself 
in a peculiar lack of plan in the more complex acts of the patient. On 
going for a walk he may wander hither and thither without aim and out 
of accord with the purpose which he at first had in mind. Such a patient 
will busy himself first with one thing and then with another in a most 
unsystematic way. 
In the worst forms of confusion the acts become nonsensical and stand 
in no correct relation to the ideas preceding them (parapraxia and 
apraxia).2 The patient does not know how to use simple objects. He 
cannot light a match or open a knife, may try to eat milk with a fork, 
and may bite his own finger instead of a morsel of food held in his hand. 
The confusion may extend to speech and writing, the patient designating 
objects falsely (paraphasia, paragraphia). The movements'of the body 
may become incoordinated, resembling ataxia or chorea. Even the mimic 
movements may no longer correspond to the underlying affect. The 
patient's grimaces are out of accord with his feelings; his laughing and 
crying stand in no ascertainable relation to the content and feeling tone 
of his ideas (paramimia). 
Motor incoherence is sometimes combined with motor agitation, the 
mixed state being designated incoherent agitation and the excessive 
incoherent movements described as jactitations (chorea magna of the 
older writers). When this is accompanied by fever the condition has 
been called delirium acutum. 
The motor incoherence is called primary when it cannot be attributed 
to other psychopathic states (e. g., in the incoherent form of paranoia). 
It is called secondary when it is due to hallucinations, delusions, flight of 
ideas, affective disturbances, or imbecility. 
Anomalies in the Intensity and Duration of Acts (Change of Acts).-In 
health initiative movements undergo changes corresponding to the 
changes which take place in the sensations and ideas in consciousness. 
When special motives are present certain movements may become 
dominant. In mental disease, however, dominant movements or acts 
may appear without adequate motivation (hyperdynamic acts). These 
hyperdynamic acts may be primary (not due to other psychopathic 
symptoms). A good example is seen in the so-called tics. Most hyper- 
1 For the differential diagnosis of the different forms of stupor the reader is 
referred to Ziehen's Psychiatric, second edition, Leipzig, 1902, p. 156. 
2 Wilson (S. A. K.), Studies in Apraxia, Brain, 1908. INTRODUCTION TO DISEASES OF THE NERVOUS SYSTEM 
67 
dynamic acts are, however, secondary to delusions, hallucinations, or 
anxious states. The monotonous recurrence of a certain act or attitude 
{motor stereotypy) is probably due to a more or less permanent psycho- 
motor hyperdynamy. 
Among the interesting stereotyped movements frequently met with are 
the snout-like movements of the lips {snout cramp) and constant ble- 
pharospasm. Sometimes the stereotypy manifests itself in certain 
bizarre movements, the so-called mannerisms. Many patients have a 
stereotyped scanning speech or vary the pitch of their voice in monoto- 
nous repetition. Sometimes single syllables or sounds are intercalated 
in the speech, a habit usually regarded as affectation, but spoken of by 
psychiatrists as stereotyped embolophrasia. 
When a motor innervation has been completed and there is a tendency 
on the part of a patient to repeat it afterward, even when other move- 
ments are required of him, the condition is designated motor persevera- 
tion. A common example is the repeated showing of the tongue, after 
one has asked to see it, even when the patient is subsequently requested 
to show his teeth, to beckon with his finger, or to close his eyes. This 
is to be distinguished from the stereotypy which has its origin within. 
Temporary motor hyperdynamy reveals itself in the so-called im- 
pulsive acts, due to a sudden affect, delusion, imperative idea, or 
hallucination. 
Anomalies of Conduct Due to Delusions and Imperative Ideas or to Im- 
becility and Dementia.-A patient who has delusions or imperative ideas 
will often reveal the character of the delusions or the ideas in his conduct. 
The whole facies and the attitude of the patient with delusions of grandeur 
are characteristic and are in marked contrast with the appearance and 
behavior of the patient suffering from delusions of self-depreciation. 
When delusions of persecution exist, the patient is ever on the defence 
(bolting of doors, sudden flights to escape enemies). Sometimes the 
persecute becomes the persecuteur; then, instead of defensive movements, 
he may assume the attitude of attack. Such patients are usually danger- 
ous in the community (paranoia). 
The so-called imperative acts are the result of the imperative ideas 
also previously described. Here the patient recognizes the absurdity of 
his act, but performs it all the same. One should distinguish carefully 
the imperative idea which is accompanied by the impulse to motor action 
and that not associated with such motor impulse. In the former case 
the imperative act frequently results, in the latter it does not. In myso- 
phobia the continuous washing of the hands for hours at a time is an 
illustration of an imperative act. A child's imitation of its parents may 
be regarded at a certain stage of development as a normal echokinesis, 
but later on direct imitation of movements observed ceases to be desir- 
able, and the tendency is normally suppressed. 
The influence of defective judgment upon the conduct becomes obvious 
in the study of imbecility and the various forms of dementia. The acts 
of defectives reveal the absence of those complex ideas which normally 
determine the play of our motives. There is an absence of due considera- 
tion before action. The so-called criminal acts doubtless belong here. 68 
DISEASES OF THE NERVOUS SYSTEM 
Disturbances of Motility Proper.-In the preceding sections motor dis- 
turbances of psychic origin have been referred to. We have now to con- 
sider the disturbances of motility less directly psychic or entirely infra- 
psychic in their origin, disturbances due to alterations in function of the 
motor pathways from the cerebral cortex to the muscles, or to alterations 
in the cortex itself or in the muscles themselves. 
Atrophy and Hypertrophy of Muscles.-The .state of nutrition of the 
muscles yields important clues to the nature of many nervous diseases. 
Muscular atrophy may or may not be associated with paralysis of muscles. 
Simple atrophy of the muscles should be distinguished from so-called 
degenerative atrophy. In the former there is only a quantitative diminu- 
tion in size; in the latter there is in addition an actual degeneration of 
the muscle substance. Degenerative atrophy occurs in lesions of the 
lower motor neurones (anterior horn cells, motor nuclei, or cerebral 
nerves, peripheral motor nerves). Simple atrophy may be due to disease 
of the muscles themselves or to lesions of the motor conduction paths 
situated above the lower motor neurones (e. g., pyramidal tract lesions). 
It may be due simply to disuse. In true degenerative atrophy the so- 
called reaction of degeneration is found. Where the degenerative atrophy 
is due to lesion of the anterior horns or of the motor nuclei of the cerebral 
nerves, fibrillary twitching is commonly present. It is rare to find it in the 
lesions of the peripheral motor nerves. 
The muscles may be the seat of a true hypertrophy in athletes. There 
is localized hypertrophy of muscles which are subject to cramp-like 
processes which continue a long time. A condition resembling hyper- 
trophy of the muscles, but which in reality is due to increase of connective 
tissue and fat, is seen in certain types of muscular dystrophy (so-called 
pseudo-hypertrophy}. 
Musculature Tone, Atony, Hypotony, Hypertony, Contractures.-The con- 
dition of tone (tonus} presented by the voluntary muscles is of importance. 
When the resistance to passive movement is abnormally small, we speak 
of hypotony or atony or resolution of the voluntary muscles. When there 
is no abnormally strong resistance to passive movement, we speak of 
rigidity or hypertony of the muscles. It is conspicuously present in the 
various spastic paralyses, in many motor spasms, in catatonia, etc. When 
along with hypertony a muscle remains permanently contracted, we 
speak of spastic contracture. When certain muscles are paralyzed and on 
subsequent voluntary innervation only the antagonists contract, there 
comes a time when relaxation of these antagonists no longer results in 
a normal position of the limbs. Such a permanent shortening of the 
antagonists is sometimes spoken of as paralytic contracture. In contrast 
with these neuropathic contractures (spastic and paralytic) there occur also 
myopathic contractures due to inflammation of the muscles and tendons, 
contractures from shortening of fascia as a result of scars, contractures of 
reflex origin, and hysterical contractures. 
Paralyses.-Inability to bring the muscles to contraction is known as 
paralysis. Paralyses may be classified in different ways: (a} According 
to the state of nutrition of the muscles; (b} according to the state of 
tonus of the paralyzed muscles; (c) according to the distribution of 
the paralysis. INTRODUCTION TO DISEASES OF THE NERVOUS SYSTEM 
69 
As regards the classification according to the state of nutrition of the 
muscles, paralyses may be divided into (1) atrophic, and (2) non-atrophic 
paralyses. By atrophic paralysis is meant a special form not due simply 
to disuse and accompanied by rapid reaction of degeneration. It indi- 
cates a lesion of the lower motor neurones, either in their nuclei of origin 
or in the peripheral nerves. By non-atrophic paralysis is meant the 
form in which the paralyzed muscles undergo but little shrinking in 
volume; no reaction of degeneration can be made out. 
According to the state of tonus of the paralyzed muscles, paralysis may 
be divided into (1) flaccid paralyses, and (2) spastic paralyses. Flaccid 
paralyses are usually associated with loss of the deep reflexes, while 
spastic paralyses are usually associated with exaggeration of the deep 
reflexes. The flaccid paralyses are due, as a rule (although not always), 
to lesions of the lower motor neurones. The spastic paralyses are due 
nearly always to lesions of the upper motor neurones. As regards the 
distribution of the muscles paralyzed, paralyses are divisible into: (1) 
Neural paralyses; (2) plexus paralyses; (3) radicular paralyses; (4) 
paralyses of one extremity or of the face (monoplegias); (5) paralyses of 
half the body (hemiplegias); (6) paralyses of both lower or both upper 
extremities (paraplegias) 
In the neural paralyses a single muscle or the muscle innervated by a 
single nerve may be involved (e. g., Bell's palsy). In the plexus paralyses 
the whole or a part of the muscles supplied by the brachial or lumbosacral 
plexuses may be involved. The paralyzed muscles in radicular paralyses 
correspond to the motor innervation from single nerve roots. The 
monoplegias may be due to lesions of peripheral nerves or of the central 
nervous system. The face may be alone affected (monoplegia facialis}; 
if it be the arm alone we speak of monoplegia brachialis', if the leg alone, 
of monoplegia cruralis. When the musculature of one-half of the body 
is paralyzed the condition is known as hemiplegia. If the muscles are 
only weakened, the term hemiparesis is employed. A hemiplegia may be 
organic or functional; it may be flaccid at first but is usually spastic 
later; it may or may not be accompanied by sensory disturbances 
(hemianesthesia; hemianopsia). It may be cortical, capsular, peduncular, 
pontile, or medullary in origin. A hemiplegia may be associated with 
paralysis of the muscles supplied by the opposite oculomotor nerves 
(hemiplegia alternans superior, or Weber-Gubler type}', or the face may 
be paralyzed on one side and the arm and leg on the other (hemiplegia 
alternans inferior, or Millard-Gubler type}. 
The term paraplegia is usually restricted to paralysis of both lower 
limbs, with more or less involvement of the trunk muscles. The para- 
plegia may or may not be associated with sensory changes; it may be 
organic or functional, flaccid or spastic. The flaccid paraplegias may be 
due to disease of the spinal cord or the peripheral nerves, while the 
spastic paraplegia is due to lesions of the upper motor neurones, either 
in the spinal cord or at a higher level. The Brown-Sequard syndrome 
is sometimes spoken of as a hemiparaplegia. When all four extremities 
are paralyzed, owing to a lesion in the cervical cord, the paralysis is 
spoken of as a cervical paraplegia. 70 
DISEASES OF THE NERVOUS SYSTEM 
Motor Irritation.-The various forms met with include: (1) Tremor. 
(2) Fibrillary twitchings. (3) Athetoid and choreiform movements. (4) 
Clonic and tonic spasms. 
By tremor is meant rhythmical, involuntary, frequent oscillatory 
movements of slight extent. The tremor may be fine or coarse; thus, 
that of Graves' disease is very fine and the rate varies from 8 to 10 oscil- 
lations per second; in paralysis agitans, on the other hand, the tremor 
is coarser and the oscillations less frequent (2 to 4 per second). A tremor 
which appears only when voluntary movements are undertaken is known 
as an intention tremor. Closely related to it is nystagmus. 
By fibrillary twitchings are meant the contractions of single fibre 
bundles of any given muscle, the contractions being insufficient to lead 
to any locomotor result. Fibrillary twitching is a totally different thing 
from tremor. It is met with especially in disease processes in which the 
muscle is degenerating, owing to lesion of the cell bodies of the lower 
motor neurones. 
By athetoid movements are meant certain slow, involuntary movements 
of extension, flexion, adduction, and abduction, each individual part 
moving by itself independent of the others, so that the various parts 
may at any given moment occupy very different relative positions in 
space. The movements may be unilateral (hemiathetosis) or bilateral. 
Such movements are prone to occur in lesions of the corpus striatum. 
They do not seem to be prominent, however, in the progressive degener- 
ation of the lenticular nucleus described by S. A. K. Wilson. 
Choreiform movements include the involuntary, quickly changing 
movements, made without plan or purpose, met with in various diseased 
conditions. They may involve any of the voluntary muscles of the body. 
This fact, together with the greater quickness of the movements, dis- 
tinguishes them from athetoid movements. The movements are best 
studied in chorea minor (St. Vitus' dance; Sydenham's chorea). A 
unilateral chorea is not infrequent after hemiplegia (post-hemiplegic 
hemichorea). In hysteria a very coarse form of chorea known as chorea 
major is sometimes met with. It is frequently associated with clownism; 
in the same disease a form of chorea occurs in which there are quick, 
lightning-like contractions in single muscles similar to those which 
follow upon electrical stimulation (chorea electrica). 
Involuntary movements of certain muscles which accompany voluntary 
movements of other muscles are known as associated movements (muscle 
synergies). One sees such movements frequently in the paralyzed limb 
of hemiplegics on strong voluntary innervation of the healthy limb. 
The tibial phenomenon of Strumpell may be regarded as an instance of a 
spinal associated movement due to injury of the pyramidal tracts. 
When it is present flexion of the lower extremity at the hip-joint and 
knee-joint calls forth a dorsal flexion of the foot and elevation of the 
medial margin of the foot, despite the patient's effort to suppress these 
movements. 
Spasms and convidsive movements are among the commonest instances 
of motor irritation. By clonic spasms are meant involuntary quick jerk- 
ings of the muscle which follow one another rapidly without interruption. INTRODUCTION TO DISEASES OF THE NERVOUS SYSTEM 
71 
If the distribution includes a large number of muscles at any one time, 
we speak of clonic convulsions. In tonic spasm the individual muscular 
contractions last a long time. When a single muscle is involved with 
severe pain the condition is known as cramp. When muscle groups are 
affected together, or the whole body is involved, we speak of tetanus 
and tetanic contractions. 
Convulsive seizures or convulsions occur in epilepsy, hysteria, tetanus, 
and eclampsia. In the ordinary epileptic attack the convulsion consists 
at first of a tonic spasm of the whole body musculature, followed after a 
short time by clonic spasms. In cortical irritation a peculiar type of 
epileptiform convulsions known as the Jacksonian attack occurs. The 
muscular contractions begin in single muscle groups and radiate into 
other motor domains (so-called "march" of the convulsion). In hysteria 
the convulsive movements may resemble those of epilepsy, although they 
are usually much more violent and the movements more varied. 
In tetany we meet with intermittent tonic spasms of bilaterally sym- 
metrical groups of muscles, associated with painful sensations and 
paresthesias. The attitude of the hands and forearms is especially char- 
acteristic ("obstetrical hand"), but muscles in various parts of the body 
may be attacked. Even when the spasm has passed off in the arms it may 
be produced by applying pressure to the arm above the elbow with a blood 
pressure apparatus (Trousseaus phenomenon). The mechanical excita- 
bility of the muscles of the face is greatly increased. Mere stroking of 
the cheek or tapping upon the branches of the facial nerves (pes anserina) 
with a percussion hammer calls forth quick muscular contractions in the 
face (Chvostek's phenomenon). The sensory nerves may also be hyper- 
sensitive, and tapping at Valleix's points then calls forth abnormally 
intense sensations (Hoffmann's symptom). On electrical stimulation the 
motor nerves are found to be hyperexcitable in tetany (Erb's jjhenomenon). 
In Thomsen's disease (myotonia congenita) there exists a so-called 
intention rigidity of the muscles. On voluntary contraction of any 
group of muscles there results an abnormally strong contraction of 
long duration which leads to motor inhibition. Gradually the muscles 
relax again. 
In psychasthenia and in certain other psychoneurotic states sudden 
contractions of the muscles known as tics are met with. In the so-called 
maladie des tics of Gilles de la Tourette the musculature of the whole body 
may be involved, and the condition is associated with mental deterioration. 
Disturbances of Coordination (Ataxia).-The mechanism by which 
muscles act together for purposeful effects is known as coordination. 
Almost every voluntary movement requires the simultaneous and suc- 
cessive activity of several muscles (synergists); some of the muscles are 
contracting (agonists); others are relaxing (antagonists). The grouping 
of contractions, their succession, and the force of each have to be care- 
fully regulated in order that the movements shall be harmonious and 
purposeful. Disturbance of this mechanism leads to the anomaly of 
movement known as ataxia. 
The presence of ataxia is not necessarily combined with weakness of the 
muscles. It is, however, frequently associated with loss of deep sensi- 72 
DISEASES OF THE NERVOUS SYSTEM 
bility and with hypotony of the muscles. It seems certain that ataxias 
are due more to interference with the centripetal or sensory paths than to 
interference with motor conduction paths. 
In the so-called cerebellar ataxia there is a disturbance of equilibrium, 
manifested especially on standing and walking. The patient stands with 
his feet wide apart and sways on walking from side to side like a drunken 
man. His tendency on walking is to let his legs run ahead of his body 
{asynergic cerebelleuse of Babinski). In trying to rise from the recumbent 
position, the patient, instead of lifting his trunk, is likely to lift his legs in 
the air. 
In cerebellar disease, the patient lying on his back with the lower 
extremities in the air, the thighs flexed and the legs abducted, can main- 
tain sometimes a fixation of position beyond that possible for a normal 
man; the lower extremities behave as though cataleptic (Babinski). On 
the other hand, the capacity {diadokokinesis) to carry on quickly a series 
of antagonistic movements, such as rapidly alternating pronation and 
supination, may be lessened (so-called adiadokokinesis). 
Electrical Condition of the Muscles and Nerves.-Much information 
of value concerning the state of the muscles and motor nerves can be 
arrived at by careful electrical examination. One determines the excita- 
bility and conductibility of the motor nenes and the direct excitability of 
the muscles. 
Increased excitability to electrical stimulation is not very common. It 
is occasionally met with in a beginning neuritis, in tetany, in beginning 
dementia paralytica, etc. A lessened excitability is met with in all old 
paralyses due to lesions of the upper motor neurones and also in the 
myopathic form of muscular atrophy. 
The most important electrical reaction for diagnostic purposes is the 
so-called reaction of degeneration {De R). When it is complete, changes 
in the electrical excitability, both in the nerves and muscles, are demon- 
strable. The excitability of the nerves for both faradic and galvanic 
current grows less and less until finally it disappears. The excitability of 
the muscle decreases for the faradic current pari passu with that of the 
nerves until it also is finally abolished, but the excitability to the gal- 
vanic current undergoes a remarkable change. Instead of a quick, 
lightning-like contraction the reaction to the galvanic current becomes 
slow, worm-like, and long drawn out, and the current, when applied 
directly to the muscle, shows an increased excitability of the latter, very 
feeble currents calling forth contractions. In addition there is a reversal 
of the normal law of contraction; normally, the cathodal closure contrac- 
tion can be produced with the feebler current, whereas in reaction of 
degeneration anodal closure contraction is more easily produced than 
cathodal closure contraction, and the cathodal opening contraction 
approaches in case of producibility that of anodal opening contraction. 
When the reaction of degeneration is present it is proof positive of 
a lesion of the lower motor neurones, although it may affect either the 
cell bodies of the neurones, the peripheral motor nerves, or the nerve 
endings in the muscles; thus, a reaction of degeneration is common in 
anterior poliomyelitis, in progressive muscular atrophy, in syringo- INTRODUCTION TO DISEASES OF THE NERVOUS SYSTEM 
73 
myelia involving the anterior horns, in the various forms of neuritis, 
and in diseases affecting the bulbar motor nuclei. There is no reaction of 
degeneration in lesions of the brain and cord which do not involve the 
lower motor neurones, nor is it present in the so-called pure primary 
muscular atrophies (dystrophies). 
In myotonia congenita the so-called myotonic reaction of Erb is obtained 
which is peculiar in this, that the muscular contraction lasts for a long 
time on direct faradic and galvanic stimulation after the current is again 
opened, and, besides, peculiar wave-like contractions can be produced if 
one stimulates the muscle near its insertion, placing the other electrode 
near the origin of the muscle. 
In myasthenia gravis the so-called myasthenic reaction of Jolly can 
usually be obtained. A tetanizing faradic current when first applied 
shows the presence of normal excitability, but gradually the response 
diminishes and in a short time no response can be elicited. 
Anomalies of Gait.-The limits of normal variation in gait are wide 
and several tolerably characteristic gaits are to be distinguished. Among 
the more important are the wobbly gait, the paretic gait, the spastic- 
paretic gait, the spinal ataxic gait, the cerebellar ataxic gait, the gait of 
tremor, the hemiplegic gait, and the gait of intermittent claudication. 
The ivobbly gait is often due to paralysis or atrophy of the M. gluteus 
medius et minimus. It is also seen in congenital dislocation of the hip-joint. 
The paretic gait assumes two types, the simple paretic gait and the 
partial paretic gait. In the simple paretic gait, due to muscular weak- 
ness only, the movements of walking are slowed and the steps are short- 
ened. Frequently there is an exaggerated flexion at the knee-joints. In 
severer cases the patient has to walk with crutches. In the partial paretic 
gait certain only of the muscles are weak. 'The most common form is that 
due to paralysis of the peroneal muscles on both sides. There is toe-drop, 
leading to lengthening of the leg; in order to compensate for this the 
lower extremity must be overflexed at the hip and knee. This gait 
was described by Charcot under the name of steppage. 
In the spastic-paretic gait the weak muscles are hypertonic and the 
stiffness slows the movements and diminishes the excursions. The lower 
extremity moves, more or less, as a whole. The toes cling to the ground; 
the difficulty in flexing the knee and hip is partly overcome by elevation 
of the pelvis on the side of the swinging leg. Often there is adductor 
spasm in the thighs, so that the knees rub against one another, and there 
is a tendency of the legs to cross on walking. In hemiplegia with spastic- 
ity the rigid limb swings lateral ward, making a movement of circumduc- 
tion, distinguishing it from the gait of hysterical hemiplegia, where the 
paralyzed leg is "dragged" forward. In cerebral softening and espe- 
cially in pseudo-bulbar paralysis the steps are often very short, the foot 
being lifted from the ground only with difficulty. This gait is spoken 
of as demarche a petits pas. 
The spinal ataxic gait is very characteristic and is easily recognized. 
The excursions of the movements are all exaggerated, the hip is over- 
flexed, and rotated lateralward, the toes are lifted and the whole leg 
suddenly thrown forward, the foot being brought to the ground with a 74 
DISEASES OF THE NERVOUS SYSTEM 
stamping sound. The feet are kept wide apart and the patient watches his 
movements closely, being almost sure to fall if he looks away. 
Two kinds of cerebellar ataxic gait are described, one due to disturbance 
of equilibrium and often associated with vertigo, the other depending 
upon a movement ataxia. In the former, the gait resembles that of a 
drunken man, the patient swaying in a very irregular manner. In the 
latter, the patient walks with his feet far apart, stamping on the ground, 
but without the wide excursions of the spinal ataxic gait. 
The character of the gait in tremor depends upon the cause of the tremor. 
Gaits more or less characteristic are met with in multiple sclerosis, in 
hysteria, and in paralysis agitans. In the latter, in addition to the 
stooped attitude of the patient, we meet with the phenomena of propul- 
sion and retropulsion; the patient walking forward or backward has 
difficulty in stopping himself. In hysteria inability to stand and walk 
is sometimes a prominent symptom (astasia-abasia'). 
Anomalies of Speech and Writing.1-Disturbance in the articulation 
of the speech sounds is known as dysarthria. In its highest grade speech 
sounds can no longer be emitted (anarthrid). Dysarthria may depend 
upon weakness of the muscles of the lips, of the tongue, of the velum 
palatinum, or of the larynx. The speech has a nasal twang in cleft palate 
and in paralysis of the velum palatinum. It is often heard in patients 
with adenoids. In bulbar paralysis it is the lingual letters (s, 1, d, t, n) 
which are difficult at first; later the labials (p, b, m, f, w, o, u) are affected, 
and finally the gutterals (g, k, ch, r) become indistinct. 
When the speech is especially slowed (convalescence from acute 
disease, psycho-motor retardation) the condition is known as bradylalia. 
When the individual syllables are separated by abnormally long pauses, 
the condition known as scanning speech is said to exist. It is especially 
characteristic of multiple sclerosis. 
In stuttering there are spasmodic contractions of some of the speech 
muscles, interfering with production of the speech sounds. It is to be 
distinguished from stammering (dysarthria literalis) by the fact that in 
the latter spastic muscle contractions are absent. In stuttering the 
trouble with speech often disappears when the words are sung. Under 
observation stuttering is exaggerated, while stammering is usually 
somewhat lessened. There is a form of syllable stumbling which is met 
with especially in dementia paralytica. 
In studying aphasic disturbances it must be kept in mind that speech 
consists of two great groups of functions, the perceptive (understanding of 
speech) and the expressive (act of speech). The anomalies on the per- 
ceptive side are included under the general term of amnesic or sensory 
aphasia (Wernicke). The memory for words can be disturbed in various 
ways; thus, the memory of names may be wholly or partially lost. The 
disturbance in which a patient hears spoken words but does not recognize 
their meaning is known as word deafness. 
1 For the literature of the subject and for excellent accounts the reader is referred 
to von Monakow (C.), Gehirnpathologie, second edition; Dejerine (J.), Semiologie du 
systeme nerveux in Bouchard's Trade de pathologic generale, Paris, 1901, vol. v; and 
Moutier (F.), L'Aphasie de Broca, Paris, 1908. INTRODUCTION TO DISEASES OF THE NERVOUS SYSTEM 
75 
When the patient whose speech muscles are not paralyzed has certain 
words and syllables in his consciousness but is unable to give expression 
to them in speech, he is said to suffer from motor aphasia. Such patients 
also are unable to speak words pronounced before them. They are never 
entirely dumb, being always able to give expression to a few words or 
parts of words; indeed, there is every gradation from syllable stumbling 
to almost complete dumbness. 
Similar to aphasia are the disturbances known as agraphia and apraxia. 
In agraphia, although there is no actual paralysis of the muscles of the 
upper extremity, the patient is unable to write down words wThich he 
hears or sees or remembers. Usually agraphia is combined with aphasia, 
but in pure agraphia it exists by itself without aphasia. In apraxia 
(or dyspraxia) the patient is unable to carry out from memory certain 
complicated movements of his limb muscles. Apraxia seems to be 
due most often to lesions of the left hemisphere or of the corpus 
callosum. 
The incapacity to understand written or printed matter is known as 
alexia. 
When all the components of speech (perceptive and expressive) are 
interfered with, the condition is known as total aphasia. When the 
speech anomaly is chiefly on the expressive side, we designate it a motor 
aphasia. In the most common form of motor aphasia, that known as 
Broca's type, speech is almost abolished and the patient is unable to 
pronounce words spoken before him. Ue cannot read aloud, he has 
difficulty in spontaneous writing, in writing to dictation, and in copying. 
He may, however, understand words which he hears and sees. In cases 
of this type, in which the agraphia is much more pronounced than the 
speech disturbance, the special term of cheirokinesthetic agraphia has 
been applied. 
In so-called pure motor aphasia {subcortical motor aphasia or aphemia; 
pure word dumbness) there is almost complete inability for spontaneous 
speech, for pronouncing words spoken before the patient, and for reading 
aloud, but there is no disturbance of writing and the understanding of 
speech and of writing is normal. In sensory aphasia, it is the perceptive 
side of speech which is interfered with. Here, again, we distinguish 
ordinary sensory aphasia (Wernicke's type) from pure word deafness. 
In ordinary sensory aphasia of Wernicke's type (cortical sensory aphasia) 
the patient is unable to understand what he hears and reads. His store- 
house of spoken words is somewhat diminished and his speech is para- 
phasic in that instead of using the words which he intends to employ he 
gives expression to others which have a similar sound. He is unable to 
pronounce words spoken before him, or if able to do so does not under- 
stand their meaning. The emphasis he puts upon spoken words is faulty. 
On spontaneous writing and on writing to dictation he manifests a verbal 
agraphia or mixes up the letters in words (paragraphia). He copies only 
with difficulty. 
In so-called pure word deafness (subcortical sensory aphasia) internal 
speech may be normal, there is some deafness, and the patient is totally 
unable to understand words spoken to him, nor can he speak words pro- 76 
DISEASES OE THE NERVOUS SYSTEM 
nounced before him. He may, however, be able to express himself quite 
well in writing and to understand what he reads. 
At the beginning of the sensory aphasias, especially in tumors or after 
brain injuries, patients sometimes pass through a transitory state in 
which motor speech is undisturbed, but there is difficulty in finding the 
names for objects and persons (concrete substantives). Such patients 
understand spoken and written words perfectly well, but on spontaneous 
writing they have the same difficulty as on speaking, although writing 
to dictation and copying may be quite normal. This particular condition 
is sometimes spoken of as word amnesia or anomia. 
Two kinds of pure word blindness (subcortical alexia of Wernicke) have 
been described, one without agraphia, the other with agraphia. In pure 
word blindness without agraphia spontaneous speech and the understand- 
ing for spoken words are normal, but single letters and syllables cannot 
be read. The patients may write, but fail to understand the words that 
they have written themselves. They can write to dictation, but cannot 
copy. The condition is usually associated with hemianopsia dextra. 
In pure word blindness with agraphia spontaneous speech and the under- 
standing of spoken speech are normal, but there is complete alexia com- 
bined with agraphia and paragraphia, these being optic in their origin. 
The patient cannot write to dictation nor copy the writing of others. 
Anomalies of the Reflexes.-A study of the disturbances of reflex action 
is of great importance in neurological and psychiatric diagnosis. Three 
main groups of reflexes should be examined: (1) The pupillary reflexes. 
(2) The deep reflexes (tendon and periosteal reflexes). (3) The superficial 
reflexes (cutaneous and mucosal reflexes). 
Pupillary Reflexes.-These include (1) the light reflex, and (2) the 
accommodation and convergence reaction. 
In testing the light reflex one must avoid calling forth movements due to 
accommodation or convergence. Each eye should be tested for itself, and 
one should also see whether or not the consensual reflex is present, re- 
membering that on unilateral illumination there should be a pupillary 
contraction in both eyes. 
In testing the reaction on convergence one asks the patient to look first 
at the ceiling and then quickly at the end of the nose. When the eyes 
converge for near vision there is also a contraction of the pupil due to 
accommodation. But even when the medial recti are paralyzed and 
convergence is no longer possible, there may be an accommodative pupil- 
lary contraction. 
If the light reflex is abolished or diminished the condition is known as 
reflex pupillary rigidity. This may be due to partial or complete blindness 
(amaurosis or amblyopia) or to paralysis of the M. sphincter iridis; in 
other words, it may depend upon the centripetal fibres of the reflex arc 
(nervus opticus) or upon the centrifugal fibres (nervus oculomotorius). 
When the pupils do not react to light but do react well on accommoda- 
tion and convergence, we speak -of the Argyll-Robertson pupil. Its 
presence is of very great significance in the diagnosis, especially of tabes 
and of dementia paralytica. The condition is usually bilateral, but may 
in early stages be unilateral. When the pupils react neither to light INTRODUCTION TO DISEASES OF THE NERVOUS SYSTEM 
77 
nor accommodation (absolute pupillary rigidity'), there is complete ophthal- 
moplegia interna. This condition is not uncommon in cerebral syphilis, 
and may be met with also in brain tumor, tabes, or dementia paralytica. 
The size of the pupils varies a good deal in health with moderate 
illumination. Abnormal contraction of the pupils (myosis) occurs in 
opium poisoning and in various conditions which irritate the oculomotor 
nerve or paralyze the sympathetic nerve. It should be borne in mind 
that myosis occurs physiologically in old people and in sleep. Abnor- 
mally large pupils (mydriasis) are seen in atropine and cocaine poisoning. 
The condition may also be due to oculomotor paralysis, to atrophy of the 
optic nerve, or to irritation of the cervical sympathetic. 
Inequality in the size of the pupils (anisocoria) may be due to unequal 
illumination or to differences in the refractive media of the two eyes, but 
most often it indicates unilateral nervous disease of the optic, oculomotor, 
or sympathetic nerves. 
Deep Reflexes.-Of these, by far the most important are the knee-jerk, 
the heel-jerk, and the periosteal reflex. 
The knee-jerk (patellar tendon reflex, knee-kick) is the contraction of the 
M. quadriceps femoris which follows tapping upon the ligamentuni 
patellae. Sometimes the so-called reinforcement of Jendrassik is necessary; 
the patient clasps his hands and is told to pull at the moment one taps 
the patellar tendon. As a rule, it is more satisfactory to make the exami- 
nation in the recumbent position. 
Achilles-jerk or Foot-jerk.-The patient is placed on his knees in a 
chair, the feet hanging loosely over the end of the chair. One then taps 
upon the Achilles tendon with a percussion hammer. The reflex is not 
always present, even in healthy people, although generally so. If the 
Achilles jerk be exaggerated the percussion stroke may give rise to clonic 
contractions or an actual foot clonus instead of to a single contraction. 
Periosteal Hadial Reflex.-On tapping the lower end of the radius one 
can see a contraction of the M. brachioradialis at the bend of the elbow, 
leading to flexion and slight pronation of the forearm and hand. 
The other deep reflexes which may be tested are: (1) Tibial reflex 
(striking anterior surface of tibia to get contraction of M. quadriceps 
femoris); (2) the biceps reflex (tapping the biceps tendon at the bend 
of the elbow); (3) the triceps reflex (holding arm at a right angle and tap- 
ping on the triceps tendon); (4) the jaw-jerk (laying finger above chin with 
the mouth closed and giving slight tap with percussion hammer to get 
contraction of the masseters). 
The deep reflexes may be increased or diminished. An increase may 
be due to irritation of the sensory limb of the arc (neuritis, meningitis), 
to stimulation of the anterior horn cells (strychnine poisoning) or to 
diminution of inhibitory influences acting from above upon the reflex 
arc (neurasthenic states, lesions of the pyramidal tract). The deep 
reflexes may be diminished or abolished through injury to the sensory 
or motor nerves or injury to the portion of the arc within the central 
nervous system. 
Cutaneous Reflexes.-The three principal ones to be considered are: 
(1) The plantar; (2) the cremaster; (3) the abdominal reflex. 78 
DISEASES OE THE NERVOUS SYSTEM 
The plantar reflex is elicited by applying a stimulus to the sole of the 
foot. Under normal conditions it leads to an involuntary contraction of 
certain muscles of the lower extremity, the so-called "tickle response" 
being a kind of movement away from the irritating object. Most impor- 
tant, however, is the behavior of the toes, and especially of the great toe. 
Under normal conditions plantar stimulation is followed by plantar 
flexion of the toes. On the contrary, when the pyramidal tract is injured, 
instead of plantar flexion there is dorsal flexion, especially of the great 
toe, and the movement of the toe occurs less rapidly than under normal 
conditions (Babinski's phenomenon'). In children during the first few 
months of life, plantar stimulation causes dorsal flexion of the great toe, 
but after the first few months of life the normal reflex is one of plantar 
flexion. When the pyramidal tract is diseased the dorsal flexion of the 
great toe is often accompanied by spreading of the other toes, and espe- 
cially by abduction of the little toe. This has been called the fan sign 
(signe de I'eventail}. Similar in its meaning is the response obtained 
by rubbing the medial surface of the tibia downward. Normally as the 
malleolus is approached, this causes plantar flexion of the toes, but in 
spastic states there results, as a rule, dorsal flexion of the foot and 
especially of the great toe (Oppenheim's sign). 
Still another sign of similar import may be mentioned. If one taps the 
lateral part of the proximal half of the back of the foot, corresponding 
to the base or middle of the third and fourth metatarsal bones, the 
cuboid bone, and the second cuneiform bone, dorsal flexion of the toes 
occurs under normal conditions, while in spastic states we meet with a 
plantar flexion, sometimes with spreading of the toes (Mendel's sign). 
Cremaster Reflex.-This is elicited by stroking the medial surface of 
the thigh in the adductor region or by pinching the skin in this location. 
The normal response consists in a contraction of the cremaster muscle, 
with elevation of the testicle. This reflex is not to be confused with 
the scrotal reflex, which consists of a contraction of the tunica dartos 
with wrinkling of the skin of the scrotum on stimulation of the skin in 
this neighborhood. 
Abdominal Reflexes.-One strokes the skin of the abdomen with the 
end of the finger or with the blunt point of some instrument, and notices 
the contraction of the abdominal muscles which follows. It may be 
elicited either in the supra-umbilical or in the infra-umbilical region of 
each side (epigastric and hypogastric reflexes). A unilateral absence of 
the reflex is of most importance. 
Other Cutaneous and Mucosal Reflexes.-Among the other reflexes may 
be mentioned the palmar reflex, scapnlar reflex, and uvular reflex. Espe- 
cial mention perhaps should be made of the lid reflex (closure of the lids 
when an object is brought suddenly near one eye) and the conjunctival 
and corneal reflexes. 
The reflexes set free by stimulation of the skin and mucous membranes 
have an entirely different significance from the deep reflexes. The 
cutaneous reflexes may be absent when the peripheral nerves are diseased; 
indeed, whenever the reflex arc is interrupted. 
One of the most interesting facts connected with the cutaneous reflexes INTRODUCTION TO DISEASES OF THE NERVOUS SYSTEM 
79 
is that they are especially disturbed in unilateral cerebral disease. Aside 
from the Babinski phenomenon, a hemiplegia usually leads to loss of 
the abdominal and cremaster reflexes on the paralyzed side, perhaps 
owing to an increase of the influences which inhibit the activity of the 
reflex arc, although this explanation is not universally accepted. 
Anomalies of the Vasomotor, Secretory, and Trophic Functions.- 
The vasomotor functions of the nervous system are very complex, and 
disturbances in different parts can lead to vasoconstriction on the one 
hand, or to vasodilatation on the other. For a full discussion of these 
vasomotor phenomena the reader is referred to the lecture by Porter,1 
the article by Eulenburg-Landois, and to the treatise of Cassirer.2 Here 
belong the angioneuroses, acroparesthesias, angioneurotic oedemas, sym- 
metrical gangrenes, erythromelalgias, acrocyanoses, etc. 
Trophic disturbances may concern the muscles, the bones and joints, 
the skin, and its appendages. The trophic changes in the bones and 
joints have been best studied in tabes, in syringomyelia, and in acrome- 
galy. In tabes spontaneous fracture of the bones frequently occurs, 
and still more frequently certain arthropathies, which occur suddenly, 
usually without pain, and lead quickly to disintegration of the joints. 
Trophic changes which take place in the skin include bed-sores (decubitus), 
falling of the hair, nails, and teeth in tabes, perforating ulcer of the foot 
in tabes and syringomyelia, and the panaritium of the finger tips in 
syringomyelia. Scleroderma and progressive facial hemiatrophy also 
belong here. Disturbances of the secretion of the sweat glands and of 
the sebaceous glands of the skin are sometimes met with in nervous 
disorders. The anomalies of the secretion of saliva, of the stomach 
juice, of the urinary secretion, etc., should also be mentioned. 
1 Vasomotor Relations, The Harvey Lectures, 1906-1907. 
2 Die vasomotorisch-tropischen Neurosen, 2nd ed., Berlin, 1912. CHAPTER II. 
DISEASES OE THE MOTOR TRACTS. 
WILLIAM G. SPILLER, M.D. 
PROGRESSIVE SPINAL MUSCULAR ATROPHY. 
Synonyms.-Muscular atrophy, type Duchenne-Aran; chronic anterior 
poliomyelitis. । 
Definition.-By the Duchenne-Aran type of muscular atrophy is meant 
a progressive muscular wasting related to degeneration only of the cells of 
the anterior horns of the spinal cord and their peripheral processes. While 
it usually begins in the small muscles of the hand, it may begin in the 
muscles of the shoulder. It is usually bilateral, but it may be unilateral 
for a time. 
The name of Aran is sometimes placed before that of Duchenne and 
sometimes follows it. A paper was published by Duchenne in 1849, and it 
was not until a year later that Aran's work appeared. A further study by 
Duchenne was made in 1853. Both Duchenne and Aran looked upon the 
atrophy as muscular in origin. The anatomical investigations of Cru- 
veilhier led to the disease being regarded as atrophy of the anterior roots. 
Luys demonstrated degenerative changes in the 'cells of the anterior 
horns. These and the investigations of Charcot, Joffroy, and others 
led to the conclusion that all progressive muscular atrophy was of spinal 
origin from disease of the cells of the anterior horns, a view that later 
was shared by Duchenne, although it was contrary to that he had pre- 
viously held. The confusion at that period was very great, nor can it 
be said at the present day that complete order has been brought out of 
the chaos. Duchenne included all progressive muscular atrophy, whether 
occurring in families, in young or old persons, or as an acquired process, 
under one head. Gradually different types of muscular wasting were 
separated from the Duchenne-Aran form. Progressive muscular dys- 
trophy in the pseudo-hypertrophic form had been observed by Duchenne 
in 1853. He at first erred in believing the pseudo-hypertrophic muscular 
paralysis was of cerebral origin, as his earlier cases manifested feeble 
intelligence, but later he recognized his mistake. He called the disease 
paralysie musculaire pseudo-hypertrophique, and this name has clung to it 
to this day. Erb established the unity of the various types of myopathy, 
but at first only the pseudo-hypertrophic and the Leyden-Mobius types 
were recognized. Charcot separated the amyotrophic lateral sclerosis 
from the general group of atrophy. Charcot and Joffroy distinguished 
the cervical hypertrophic pachymeningitis in 1871 to 1873, although it 
cannot be said that the symptoms of this disease are so sharply defined 
as are those of amyotrophic lateral sclerosis. Schultze arid Kahler, 
80 DISEASES OF THE MOTOR TRACTS 
81 
in 1888, weakened the Duchenne-Aran type of muscular atrophy by 
separating the great group of syringomyelia. It began to look as though 
nothing would be left in the Duchenne-Aran type, and indeed it is stated 
that Charcot himself felt that it stood on an insecure foundation. 
In 1897, Marie came forth in defiance of the accepted teaching. He 
pointed out that Duchenne at first believed that progressive muscular 
atrophy was of peripheral origin; later, he believed it to be of spinal 
origin. The disease at first, as understood by Duchenne, included all 
the forms of muscular atrophy not resulting from fracture. Marie then 
described how amyotrophic lateral sclerosis, progressive muscular dys- 
trophy, multiple neuritis, and syringomyelia were separated from the 
great group of atrophies. Fully one-third of Duchenne's cases, from his 
own description, Marie thinks, belonged to syringomyelia, although this 
disease at that time was not recognized. Thus, Marie says that the 
progressive muscular atrophy of Duchenne-Aran, which earlier authors 
regarded as the most solid foundation of neuropathology, has ceased to 
exist. Even the hand which is commonly regarded as of the Duchenne- 
Aran type is, according to Marie, not the deformity Duchenne described, 
and is what Duchenne believed to be characteristic of leprosy. The 
hand that he described as typical of his progressive muscular atrophy 
Marie believes belongs to syringomyelia. The atrophy is not so intense, 
and the fingers are not so much flexed; and yet in comparing the two 
hands, as pictured by Marie, the differences do not appear very great. 
Marie believes that Duchenne merely described a symptom complex, 
and that nothing now remains of his progressive muscular atrophy. 
This view is not generally accepted. 
Etiology.-Pregnancy may have some influence on the development of 
spinal muscular atrophy, though it would seem questionable whether 
it be able to originate it when no tendency to the disease exists. In 
Oppenheim's second case of chronic poliomyelitis, a complicated case, 
weakness of the muscles of the right shoulder and of the right upper limb 
developed during the fourth pregnancy; the following year the left upper 
limb became affected during pregnancy. Nonne attributed the polio- 
myelitis in his second case to diabetes. 
Heredity does not play a role, and yet Bruining has observed chronic 
anterior poliomyelitis in father and son, and in one of the cases obtained 
a necropsy; this family occurrence was probably merely a coincidence. 
Age seems to be the most important factor, the symptoms usually 
appearing in middle life: The infantile form of progressive spinal mus- 
cular atrophy is considered separately. Myelopathy is probably an 
abiotrophy, and is the result of an imperfectly developed motor system 
unable to withstand the stress of advancing years. 
It is exceedingly questionable whether infections or intoxications cause 
the symptoms, although the development of progressive muscular atrophy 
many years after an arrested acute poliomyelitis is regarded by Leri as 
evidence of this origin. C. S. Potts has paid particular attention to 
muscular atrophy occurring in this way, and collected the records of 
36 cases. In 28 the condition was probably progressive muscular atrophy; 
in 2, amyotrophic lateral sclerosis; in 2, myelitis; and in 4, another 82 
DISEASES OF THE NERVOUS SYSTEM 
attack of acute poliomyelitis. The interval elapsing between the primary 
and secondary attacks ranged from seven years to about fifty-five years, 
the average being about twenty-three years. In 18 cases out of 33, in 
which the part secondarily affected could be determined, the late atrophy 
began in a limb which had previously been affected by the primary disease; 
in 7 it began in the other limb on the same side that had been primarily 
affected; in 5 in the corresponding limb of the side opposite to the part 
first affected; in 2 in a limb of the opposite side not corresponding to 
the limb first affected; and in one the involvement was general, this 
being a case in which acute poliomyelitis occurred a second time. The 
nerve cells left weakened by acute poliomyelitis may degenerate later, 
but this is not a proof that the chronic form is so caused. Some toxic 
substance acting on the cells weakened by acute poliomyelitis may cause 
progressive muscular atrophy. It seems probable that lead may be one 
of these agents. The acute and chronic forms probably have a very 
different pathology. The male sex is regarded by certain authors as 
much more frequently affected than the female. 
Some of the cases supposed to represent progressive spinal muscular 
atrophy are caused by myelitis, especially the syphilitic form. The 
inflammation may be almost confined to the gray matter, and such being 
the case, sensory disturbance may be absent. The symptoms then 
would be those of progressive spinal muscular atrophy, although the 
lesion would be a diffuse process of inflammatory character. 
Pathology.-The characteristic changes are atrophy and degeneration 
of the nerve cells of the anterior horns of the spinal cord, and also in 
some cases of the motor nuclei of the medulla oblongata. Sometimes 
many of the nerve cells entirely disappear. Numerous hemorrhages 
have been observed in the gray matter in some instances (Bielschowsky, 
Spiller), extending even throughout the cord. The motor roots are 
atrophied, and contain fewer fibres than normal. The white matter of 
the spinal cord remains unaltered'in a typical case, or at least the sclerosis 
is not in the area occupied by the crossed pyramidal tracts. It is question- 
able whether those cases in which a very slight degeneration of these 
tracts occurs, detectable only by the Marchi stain, should be classed as 
progressive spinal muscular atrophy or amyotrophic lateral sclerosis. 
These symptoms and the findings in the anterior horns may indicate 
that the lesions of the anterior horns have been of long duration, while 
the degeneration of the lateral columns evidently is very recent, and no 
clinical evidence of the slight degeneration of the pyramidal tracts may 
have been detected during life. It seems best to regard such cases as 
belonging to progressive spinal muscular atrophy or chronic poliomyelitis, 
but as marking a transitional stage to amyotrophic lateral sclerosis. 
Beevor, in disputing the correctness of Gowers' view that amyotrophic 
lateral sclerosis and progressive spinal muscular atrophy are the same 
disease, stated that he (Beevor) had a case in which the symptoms began 
with atrophy of the small hand muscles, and later the shoulder muscles 
became affected, the lower limbs were only slightly implicated. Rigidity 
or increase of the deep reflexes was not present. Atrophy of the cells of 
the anterior horns was found, but the lateral columns were intact. DISEASES OF THE MOTOR TRACTS 
83 
Degenerative changes in the anterolateral columns may be recognized 
as belonging to anterior poliomyelitis and progressive spinal muscular 
atrophy, provided the pyramidal tracts are not affected. It must be 
accepted that certain of the column cells may degenerate as well as the 
cells of the anterior roots. When the pyramidal tracts become implicated 
the cases are on the border line of amyotrophic lateral sclerosis. Peri- 
vascular round-cell infiltration has been seen (Bielschowsky). 
It is uncertain whether the cellular changes are primary or secondary. 
In some instances they may be primary, in others they may be the 
result of inflammation in the surrounding tissues. Where the process 
has advanced so far that most of the cells have disappeared, the neuroglia 
of the anterior horns appears denser and the few nerve cells that remain 
show much pigmentation. The cellular destruction is usually greater 
in the cervical swelling; and the anterior roots usually are much atro- 
phied. The peripheral nerves and muscles show more or less alteration. 
Syphilitic myelitis probably is the lesion underlying a certain number 
of the cases of progressive muscular atrophy. Progressive ophthalmo- 
plegia may become associated with degeneration of the motor cells of 
the medulla oblongata and spinal cord, as in two cases observed by 
Dana. 
It seems to Dana an unnecessary confusion of symptomatology to 
transfer the diagnosis of progressive muscular atrophy of the Duchenne- 
Aran, or other type, to that of amyotrophic lateral sclerosis so soon as 
a little spasticity begins, especially as there may be nothing peculiar in 
age, course, or duration. The atrophy may start in the hands and ascend 
to the shoulders before there is any evidence of lateral sclerosis. The 
disease may then become arrested, the spastic symptoms disappear, and 
the patient may again present the type of Duchenne-Aran atrophy with- 
out any spastic symptoms. All that we can do, in the writer's opinion, 
is to acknowledge that the lateral columns may be slightly affected without 
producing clinical manifestations, but because we cannot always recognize 
this slight degeneration clinically is not sufficient reason to decline to 
make distinctions in the pathological forms of spinal muscular atrophy. 
It is a question whether we are solving the problem by following Dana in 
limiting the use of the term amyotrophic lateral sclerosis to those cases 
which show only from the beginning and dominantly the spastic and 
contracturing type of progressive muscular atrophy. Why must degen- 
eration of the lateral columns develop previously to or simultaneously 
with disease of the cells of the anterior horns, and not later? 
Symptoms.-The symptoms of chronic anterior poliomyelitis and pro- 
gressive spinal muscular atrophy vary in typical cases. In the former the 
paralysis is supposed to develop within a few days or a few weeks, the 
atrophy appears later, and entire muscles or groups of muscles are par- 
alyzed, the course of the disease is more rapid, and the paralysis develops 
first in the muscles of the lower limbs or shoulders. In progressive spinal 
muscular atrophy, the paralysis is proportional to the atrophy, one 
muscle fibre after another is affected, and the course is longer than in 
poliomyelitis. These distinctions are not always regarded, and some of 
the reported cases of so-called poliomyelitis might with greater right be 84 
DISEASES OF THE NERVOUS SYSTEM 
regarded as examples of progressive spinal muscular atrophy, or even as 
transitional forms of amyotrophic lateral sclerosis. 
Dejerine and Thomas, who describe the disorder under the name of 
chronic anterior poliomyelitis, say that atrophy is the primary and 
essential symptom, and paralysis is secondary and proportional to the 
atrophy. Thus, according to these authors, one of the most important 
diagnostic points between chronic poliomyelitis and progressive spinal 
muscular atrophy disappears. Beevor made no sharp distinction between 
the two processes, and remarked that wasting may begin in the lower 
limbs. 
The first indication of disturbance may be awkwardness in the use of 
one or both hands. Usually one is affected before the other. Abduction 
and apposition of the thumb with the fingers become affected, the inter- 
ossei and lumbricals waste, and the hand becomes much atrophied, includ- 
ing the thenar and hypothenar eminences. When the thenar eminence is 
wasted the hand is of the simian type. Later, when the interossei and 
lumbricals are attacked the flexion of the phalanges upon the metacarpal 
bones and the extension of the phalangeal articulations become impaired, 
so that the first phalanges are extended and the second and third flexed, 
the palmar tendons are prominent (main en griffe). The atrophy grad- 
ually extends to the flexors on the forearm, then to the extensors. Accord- 
ing to Beevor, the flexors of the fingers and thumb are affected before 
the flexors of the wrist. The atrophy reaches the deltoid and muscles of 
the upper part of the limb and shoulder girdle. Beevor states that the 
triceps, latissimus dorsi, and the lower half of the pectoralis major usually 
escape. The trunk and neck muscles waste and the ribs become prom- 
inent; the lower limbs likewise become greatly atrophied. Dejerine and 
Thomas dispute the extension of the process to muscles innervated by 
bulbar nerves, as no necropsy has demonstrated such an extension, and 
they assert that the face is always intact, and Dejerine believes that 
bulbar palsy belongs to amyotrophic lateral sclerosis. Beevor, however, 
speaks of bulbar paralysis as a part of chronic anterior poliomyelitis, and 
Leri also mentions atrophy of the muscles of the face, although he adds 
that the condition is very different from that of bulbar palsy. 
There are a number of cases recorded in which the atrophy began in 
the lower limbs, especially in the peroneal distribution, and in some 
instances (Moleen and Spiller) the weakness and atrophy developed 
rapidly. The upper limbs became implicated later. In other cases the 
muscles of the shoulder girdle are first affected (Vulpian, Dejerine) or 
the extensors of the fingers and wrists, as in a case reported by C. S. 
Potts. The muscles of the trunk may be first affected. 
The weakness and atrophy probably begin more commonly in the hands, 
because the movements of these parts are highly differentiated; it is 
not probable that the weakness is first noticed in muscles employed 
frequently in specialized movements simply because the interference with 
these movements is more striking to the patient, and that in reality other 
muscles are affected at the same time. Edinger's exhaustion hypothesis, 
that those muscles most used are most likely to be the first to show 
alteration, is a reasonable explanation for the com... .cement of the DISEASES OF THE MOTOR TRACTS 
85 
symptoms in the hands in most cases. The paralysis is flaccid and the 
joints relaxed, producing the condition known as flail joint, especially 
noticeable at the wrists. Grunow and Loevegren go so far as to make 
the atrophy of the lower limbs the first sign diagnostic for poliomyelitis 
in distinction to progressive spinal muscular atrophy. 
Fibrillary tremors are very common in the atrophying muscles; some- 
times they implicate small bundles of muscle fibres, and then they may 
more properly be described as fasciculary. These quick, wave-like move- 
ments are not present in the atrophying muscles at all times, but in most 
cases may be detected at some period or other. They may be so pro- 
nounced as to correspond to the condition known as "muscular madness" 
(folie musculaire), but they do not cause movement of a segment of the 
limb, unless possibly of a finger. They are an early sign, and disappear 
as the atrophy of the muscles becomes intense. They may be present 
at one period in spinal atrophy, but later disappear, so that the absence 
of fibrillary tremors by no means excludes a spinal origin. 
The tendon reflexes of the affected limbs become diminished or lost, and 
may be lost before atrophy appears, the cutaneous reflexes become less 
active where the muscles are wasted. Exaggerated reflexes should make 
the diagnosis of progressive spinal muscular atrophy or chronic anterior 
poliomyelitis doubtful, but, according to certain authors, do not exclude 
this diagnosis. Thus Oppenheim does not regard exaggeration of the 
tendon reflexes as sufficient to make a case of muscular atrophy one of 
amyotrophic lateral sclerosis. 
The electrical reactions are important in the diagnosis between the 
myelopathic and the myopathic forms of muscular atrophy. In the latter 
the irritability is diminished, but the formula is not altered; in the 
former the nerves may respond to the faradic and galvanic currents, 
although not in the same degree; later the response to either current 
may be lost. The muscles at first require a stronger current, then their 
faradic irritability may be lost; when the galvanic irritability is increased, 
the formula may be reversed, so that the anodal closing contraction is 
equal to or greater than the cathodal closing contraction (reaction of 
degeneration); still later all electrical irritability of the affected muscles 
may be lost. Especially valuable as a sign of myelopathy is the modal 
change, so that the muscles contract very slowly to the galvanic current. 
The entire muscle does not always show electrical alteration, and only a 
few fibres may present this change, while adjoining fibres in the same 
muscle are normal; this is because fibres much atrophied may be in 
juxtaposition to normal fibres. 
Sensory changes are not a part of myelopathy. Objective disturbance 
of sensation does not occur in an uncomplicated case, and while pain may 
occasionally be complained of, it is of a peculiar kind; it is the pain from 
overuse of wasting muscles, and not that from irritation of sensory 
fibres. It is more a dull aching like that in fatigued muscles, and not 
sharp, such as is felt in toothache. Some authors speak of pain occurring 
in the beginning of myelopathy, but skepticism as regards the correctness 
of the diagnosis is justifiable when pain is intense. 
The sphincters of the bladder and rectum escape, but theoretically one 86 
DISEASES OE THE NERVOUS SYSTEM 
may understand how they could become affected if the nerve cells of 
the conus were degenerated. Atrophy of bone has been described by 
some authors. Cutaneous lesions are probably epiphenomena. Vaso- 
motor symptoms may be present, and the atrophied limbs may be cold. 
Chronic external ophthalmoplegia is probably a form of progressive 
muscular atrophy of the central type. It is a progressive paralysis of 
the external ocular muscles. Numerous cases are recorded in the litera- 
ture. It is usually a disease of infancy, but may develop later in life. 
Diagnosis.-Remembering the pathology we understand why the symp- 
toms are muscular atrophy, weakness, loss of reflexes, and changes in 
electrical reactions. The process is slow (progressive muscular atrophy) 
or in periods of exacerbations (subacute or chronic poliomyelitis). 
Progressive muscular dystrophy is to be distinguished by the age at 
the onset, the involvement first of the proximal parts of the limbs, the 
hereditary or family tendency, the absence of fibrillary tremors and of 
reaction of degeneration, the presence of pseudo-hypertrophy, etc. 
Amyotrophic lateral sclerosis is clinically progressive spinal muscular 
atrophy or chronic poliomyelitis with exaggeration of tendon reflexes, 
and pathologically with degeneration of the cells of the anterior horns 
and peripheral motor fibres and the pyramidal tracts; the latter causes 
exaggeration of the tendon reflexes. Multiple neuritis is usually asso- 
ciated with spontaneous pain, objective sensory disturbances, and tender- 
ness of nerve trunks. The onset is usually more rapid, and the symptoms 
may be confined to the distribution of certain nerves. Acute poliomyelitis 
is to be recognized by signs of infection and rapid development of symp- 
toms. The paralysis at first may be extensive, but gradually certain 
of the affected muscles recover, leaving others permanently paralyzed. 
Syringomyelia has the dissociation of sensation, impairment or loss of 
temperature, and pain sensations, with much better preservation of 
tactile sensation; also trophic disturbances, and it may be more nearly 
unilateral. The neurotic muscular atrophy of Charcot, Marie, and Tooth 
may closely resemble chronic poliomyelitis, but where sensory disturb- 
ances are present the distinction is clear. It is likely to be hereditary, 
and is more common in males. The atrophy is confined to the peripheral 
parts of the limbs, even after many years. Leprosy may have some 
resemblance in that it may begin in the hands and extend toward the 
trunk in the upper limbs, implicate the lower limbs in a similar manner, 
and be associated with reaction of degeneration. The lepra bacillus in 
the tissues, the dissociation of sensation like that of syringomyelia, the 
local swellings in the nerves, and the history of contagion make the diag- 
nosis possible. Cervical hypertrophic pachymeningitis may cause an 
atrophy of the hands, but there is a period of pain in the upper limbs 
with objective sensory disturbances. 
Prognosis.-Arrest is possible but improbable. The weakness and 
atrophy are likely to extend until a large portion of the body is affected, 
or nerve cells concerned with respiration or controlling the heart, are 
attacked, or death occurs from some intercurrent disease, but usually 
the process is very slow, and in one of Dejerine's cases the symptoms 
lasted about sixteen years. DISEASES OF THE MOTOR TRACTS 
87 
Treatment.-This is of little value. Electricity, especially in the form 
of the constant current, moderate exercise, and passive movements are 
recommended and may be useful. Tonics and exercise in moderation 
help to keep up the general strength, but nothing will restore the muscles 
atrophied from degeneration of their nerve cells, or probably have much 
effect in arresting the course. 
PROGRESSIVE SPINAL MUSCULAR ATROPHY OF CHILDHOOD, 
OF FAMILIAL OR HEREDITARY CHARACTER (TYPE 
WERDNIG-HOFFMANN). 
History.-The first cases were described by Werdnig, who reported 2; 
Hoffmann followed, with a report of 20 cases; Thomson and Bruce, with 
1 case; Bruns gave, in 1901, a description founded on the cases in the 
literature to that date. Hoffmann's cases occurred in three families; 
he examined 6 clinically, 2 anatomically. Both of Werdnig's cases were 
with necropsy, and occurred in the same family, the patients had the 
same mother but different fathers. In Hoffmann's third family the 
disease was evidently transmitted through the mother. Bruns' 3 cases 
came from three different families. The family character was evident 
in the first case, as two of the other offspring of the parents had died from 
the same disease. Nothing concerning family tendency could be deter- 
mined in the second case. The third patient was the only one affected 
of four children. Batten has reviewed the literature of this disease. The 
cases of Werdnig and Hoffmann began between the sixth and ninth 
months; only a few of the children could stand before the disease began, 
and none could walk. Death occurred in the most rapid case in the 
eleventh month; in the slowest in the fifth year. Bruns' first patient 
could walk when about a year and three quarters old, and died in the 
fifteenth year. His third patient also could walk when about one year 
and one-quarter old, and was still alive in the twelfth year. 
Hoffmann attempted to classify the cases of hereditary progressive 
spinal and bulbar muscular atrophy, and makes the following types: 
1. Occurrence in early childhood. Implication first of the pelvic girdle 
and atrophy extending from there toward the ends of the limbs (Werdnig, 
Hoffmann). 
2. The bulbar paralytic facial type of childhood (Fazio, Londe). 
3. A Duchenne-Aran type (Strumpell, Gowers). 
4. An intermediate form (Bernhardt), which Bernhardt regards as of 
spinal and bulbar origin. 
It seems to the writer that the atrophy of Werdnig-Hoffmann may be 
regarded clinically as progressive spinal muscular atrophy, differing from 
the usual type seen in adults in the commencement within the first years 
of life, in the implication first of the pelvic girdle and portions of the 
limbs near the trunk, and in the strong hereditary tendency, a tendency 
very feebly manifested in the progressive spinal muscular atrophy of 
adults; but pathologically the disease is amyotrophic lateral sclerosis of 
childhood, differing from the type in adults By the absence of exaggera- 
tion of tendon reflexes, by which we may infer that the peripheral motor 88 
DISEASES OF THE NERVOUS SYSTEM 
segments are always involved first. The case of Thomson and Bruce 
shows that they alone may be affected. 
Pathology.-The findings described by Hoffmann are: Symmetrical 
intense degeneration of all the peripheral motor neurones below the 
hypoglossus, including the accessorius, i. e., degeneration or disappear- 
ance of the multipolar cells of the anterior horns, so that few are 
left in a transverse section; intense degeneration of the anterior roots 
of the spinal cord, less intense changes in the peripheral nerves and the 
intramuscular nerve endings; degeneration of the crossed and direct 
pyramidal tracts, and of a portion of the lateral fundamental columns 
of the cord, more intense in the upper thoracic and cervical regions, but 
not traceable above the pyramidal decussation. The muscles show simply 
atrophy in all stages without complete disappearance of fibres, without 
multiplication of the sarcolemma nuclei, and with very little lipomatosis. 
The alteration of the spinal cord is more intense than of the muscles. 
Symptoms.-The description given by Bruns is as follows: The dis- 
ease begins in early childhood, progresses slowly, without fever or con- 
vulsions. The children never learn to walk, or gradually loge the ability 
to walk if they learned before the symptoms began. They cease to 
move the lower limbs in bed and to hold the trunk erect. Standing be- 
comes difficult, and is possible only in the1 beginning of the disease, and 
then only with support. Paresis and atrophy of muscles occur nearly 
simultaneously in the muscles of the pelvic girdle and trunk, implicating 
symmetrically the iliopsoas and quadriceps femoris, and later the upper 
limbs and neck muscles. The proximal portions of the limbs, shoulder 
girdle, and pelvic girdle are affected, and the atrophy and weakness 
diminish toward the hands and feet, although these parts do not entirely 
escape in the late stages, but their movements are better than would be 
expected from the atrophy. The erect position of the head and trunk 
is difficult to maintain; the child cannot lie down slowly, and raises 
itself with difficulty. The vertebral column is distorted because of 
muscular weakness. The feet are in the position of talipes equinus. 
The atrophy is en masse, and may be partly concealed by fatty tissue. 
Diminished electrical response or reaction of degeneration is observed 
in the paralyzed muscles. Fibrillary tremors are present in some cases, 
absent in others. The bulbar muscles are occasionally affected. Hyper- 
trophy or pseudo-hypertrophy does not develop. The paralysis is flaccid, 
the tendon reflexes are absent, and contractures occur. There are no 
sensory or mental disturbances, or impairment of the sphincters of 
bladder and rectum. The course is rather rapid, and death results from 
paralysis of the muscles of respiration. The disease is progressive, the 
progress of the atrophy is from the parts near the trunk to the peripheral 
portions of the limbs, and the atrophy is entirely symmetrical. Hoffmann 
believes that fibrillary tremors do not occur, mental development is not 
arrested, and the muscles of face, tongue, and throat are not paralyzed. 
Diagnosis.-The greatest difficulty is in regard to muscular dystrophy, 
and so true is this that Senator says of his two cases that when the 
children were seen dressed and their mode of sitting, lying down, and 
standing up (climbing upon the lower limbs as in muscular dystrophy) DISEASES OF THE MOTOR TRACTS 
89 
was observed, the diagnosis of pseudo-hypertrophy seemed correct, but 
when the clothes were removed no hypertrophy was visible. Most 
important in the diagnosis were the presence of fibrillary tremors and 
diminution in the electrical response, and possibly a commencing reac- 
tion of degeneration in one case. The cases were without necropsy, and 
it seems questionable to which type they belonged. The type of Werdnig- 
Hoffmann shows a more rapid extension of the atrophy than occurs in 
progressive muscular dystrophy. 
Congenital myatonia (congenital hypotonicity of the muscles) differs 
in being a congenital disorder, in the absence of heredity and family 
tendency, and in the much greater involvement of the trunk and portions 
of the limbs near the trunk at a very early age. The differential diagnosis 
may be difficult. 
AMYOTROPHIC LATERAL SCLEROSIS. 
In this chapter amyotrophic lateral sclerosis is considered as distinct 
from progressive spinal muscular atrophy and from chronic or subacute 
poliomyelitis. / 
Etiology.-Amyotrophic lateral sclerosis is probably an abiotrophy. 
The individual is born with a certain potentiality of his motor system, 
but at middle age the strain becomes too great and degeneration begins. 
There is very little to warrant the opinion that the disease is of toxic 
origin. It is possible that lead may be a cause, or at least it may cause a 
symptom complex very closely resembling that of this disease, as shown 
by S. A. K. Wilson. This author has studied four cases in which chronic 
lead poisoning began with double wrist drop, and in three of these the 
small muscles of the hand were not affected Until late in the process, and 
in the other they escaped so long as the patient was under observation. 
In all four cases the atrophy was progressive, and was associated with 
some exaggeration of the tendon reflexes. Babinski's reflex may be 
found in this lead palsy associated with a certain degree of spasticity and 
with ankle clonus. The electrical reactions may be altered quantitatively 
but not qualitatively, and sensation may be intact, although in one of 
Wilson's cases lancinating pains were present, and this occurrence in 
association with the Argyll-Robertson pupil led to a diagnosis at first 
of tabes. No necropsies have been obtained, and, therefore, it is not 
known whether lead may produce exactly the changes of amyotrophic 
lateral sclerosis. 
It is not unreasonable to assume that lead poisoning may appear as a 
systemic disease of the nervous system, and there is even a possibility 
that it may assume the type of tabes dorsalis, as shown by a case reported 
by Redlich. The commencement of the paralysis in the extensors of 
the hands and fingers is unusual in amyotrophic lateral sclerosis, but 
does occur. Muscular pains are not uncommon in lead poisoning, and 
cramps sometimes are felt in amyotrophic lateral sclerosis. An Argyll- 
Robertson pupil is rare in the latter disease, but Wilson seems to have 
observed it three times. 
The attempt to give causes for which there is no evidence is hardly a 90 
DISEASES OE THE NERVOUS SYSTEM 
justifiable procedure. We have no reason to believe that traumatism, 
exposure to cold and wet, or sexual excesses play a role. 
Syphilis may cause the symptoms of amyotrophic lateral sclerosis 
although it is not probable that all cases are caused by syphilis. 
Syphilitic disease of the cord may produce spasticity, weakness of the 
limbs, atrophy of the small muscles of the hands, exaggerated tendon 
reflexes and fibrillary tremors. Careful examination, however, will 
usually reveal other symptoms; thus there may be severe pain in different 
parts, or disease of the optic nerves; or the history may mention a 
hemiplegia, perhaps transitory. The writer has studied such cases, in 
which many of the symptoms suggested amyotrophic lateral sclerosis. 
Frequency and Sex.-Raymond and Cestan reported, in 1905, 18 cases 
with necropsy in the service of the former at the Salpetriere; 13 were 
in men and 5 in women. In Collins' cases, 55 were males and 49 females. 
Probst, in 1898, found that of 53 cases the number was evenly distributed 
between males and females. Heredity and occupation do not seem to 
exert any influence. 
The writer has reported 8 cases of primary degeneration of the central 
motor tracts with necropsy; 2 of these belong to the type of primary 
lateral sclerosis, as the nerve cells of the anterior horns of the spinal cord 
escaped, and in neither of these cases was muscular atrophy present. 
Amyotrophic lateral sclerosis seems to be a rare disease, about as rare or 
possibly more so than syringomyelia. 
Pathology.-There is a degeneration of the pyramidal tracts, of the 
nerve cells of the anterior horns of the spinal cord, of the anterior roots, 
intramedullary and extramedullary portions; of the peripheral nerves, 
of the muscles, and sometimes of the nerve cells and fibres in the motor 
cortex. The corpus callosum and the posterior longitudinal bundles may 
be affected. It is essentially a death of the motor system and muscles. 
The fibres that stain by hematoxylin in the pyramidal tracts of the 
spinal cord in this disease were noticed by Charcot and Marie to be very 
fine. Anton also observed an unusual number of fine fibres in the pyra- 
midal tracts, and a similar observation was made by the writer, although 
fibres of larger size were mingled with these small fibres. The latter in 
the sclerotic tracts suggest that atrophy as well as degeneration of nerve 
fibres plays a role, but the Marchi method indicates very clearly that 
degeneration of the myelin sheaths is very important. The central motor 
tracts develop from above downward, and the terminal portions being 
the last formed are the least resistant, and, therefore, degenerate first. 
The degeneration of the pyramidal tracts is not so intense in amyo- 
trophic lateral sclerosis as in the sclerosis resulting from a cerebral lesion. 
This is probably because one fibre after another is attacked in the process 
and all do not begin to degenerate at the same time. There is some 
doubt whether the process in most cases is ascending in each nerve fibre 
attacked, and the mere cessation of the degeneration at a certain level 
is no proof of an ascending process. It is reasonable to believe that there 
is a gradual death of the portions of the central motor neurones most 
remote from the cells of origin, and that the extent of the degeneration 
varies in different cases, but that the whole portion that is affected in DISEASES OF THE MOTOR TRACTS 
91 
each fibre is diseased usually almost simultaneously, although the indi- 
vidual nerve fibres are attacked at different periods. 
Kojewnikoff was the first (1883) to trace degeneration of the pyramidal 
fibres from the lower end of the cord to the cerebral hemisphere. In 
the two hemispheres the "corps granuleux" (fatty granular cells) were 
in symmetrical areas, and in almost equal quantity on the two sides. 
Charcot and Marie (1885) traced the degeneration to the motor cortex 
in two cases. Kojewnikoff (Koschewnikoff) in a second case found de- 
generated fibres throughout the central motor tracts, and did not depend 
merely on the presence of fatty granular cells. In Lennmalm's case the 
pyramidal tracts were said to be degenerated from the cerebral cortex 
throughout their extent. Lumbroso traced the degeneration to the 
cortex. Mott traced the degeneration through the inner capsule and 
into the motor cortex. Rossi and Roussy, in 1907, found 18 cases on 
record in which the degeneration had been traced to the cortex. 
As regards the degeneration of the pyramidal tracts the writer has been 
able to trace degeneration of the tracts as high as the motor cortex in 
1 case, as high as the internal capsule in 2 cases, as high as the cerebral 
peduncle in 2 cases, as high as the pons in 3 cases. The degeneration does 
not usually extend above the pons, and gradually disappears. The 
higher regions of the pyramidal tracts show a more normal manner of 
staining. In one case degeneration of the pyramidal tract was traced 
on one side as high as the pons, and on the other as high as the lower 
part of the internal capsule. The degeneration therefore is not always 
symmetrical on the two sides, although it usually is. The writer has 
observed great spasticity of the limbs when the degeneration of the pyram- 
idal tracts was barely detectable. The weakness may be due entirely 
to implication of the central motor tract alone, but the writer has observed 
paralysis on one side greater when the degeneration of the pyramidal 
tract on the same side was less intense than that of the other side. A 
study of the nerve cells showed that the motor cells of the spinal cord 
on the side of the less affected pyramidal tract were the more diseased. 
Marie has stated that the degeneration of the lateral columns involves 
a greater area than that occupied by the pyramidal tracts, as shown by 
secondary degeneration from cerebral lesions or by myelination of the 
cord. It is unquestionable that the degeneration does extend beyond the 
pyramidal tracts, and this alteration of the supplementary zone has been 
described in progressive spinal muscular atrophy by J. B. Charcot. 
Pilcz found degeneration by the Marchi method in the anterolateral 
columns much greater than in the pyramidal tracts. This would indicate 
that the fibres in the former were more recently affected, and the condition 
in this respect resembles the degenerative changes occurring in the spinal 
cord in cases of grave anemia. There is, however, another explanation. 
The fibres of the pyramidal tracts extend a long distance, and when they 
are degenerated the change is seen through a great part of these tracts, 
and has the appearance of long-standing alteration; whereas in the antero- 
lateral columns the fibres are much shorter, and many more fibres must 
degenerate to cause a long-standing alteration as intense as that of the 
pyramidal tracts. 92 
DISEASES OF THE NERVOUS SYSTEM 
The anterior horns may contain fewer fibres than normal; the cells of 
the anterior horns may be much affected, in some the nucleus is dis- 
placed to the periphery, and the chromophilic elements entirely destroyed, 
except where they surround the nucleus, and the remainder of the cell 
may have a pale green appearance by the thionin stain. The pigment 
is sometimes much increased. In some cells the nucleus may disappear, 
in many the chromophilic elements are changed to fine granules. Many 
cells have no dendritic processes, or only imperfect ones; some of the 
cells are tumefied and rounded, some have altered cell bodies and normal 
dendritic processes. 
The bulbar changes are often pronounced. The nuclei of the hypoglossal 
nerves may be much degenerated or even disappear, so that the cells may 
be few in number, with chromatolysis and much pigmentation, and loss 
of dendritic processes; or they may entirely disappear. The intramedul- 
lary fibres of the hypoglossus may be intensely degenerated. The tongue 
muscles may be greatly wasted, in association with the alteration of the 
hypoglossal nerve, and show excess of interstitial fatty tissue. The nucleus 
ambiguus, as well as the posterior nucleus of the vagus nerve, may be 
much degenerated, but as the cells of the former do not form a very 
sharply defined group, it is often difficult to determine a partial destruc- 
tion of this nucleus. The soft palate may be wasted. 
The motor nucleus of the trigeminal nerve has been found affected 
(Rossi and Roussy, and others), but may escape when other bulbar nuclei 
are diseased. The facial, the glossopharyngeal, and spinal accessory 
nerves, and their nuclei, may share in the alteration. The nuclei of the 
ocular muscles, according to Marie, are never affected in this disease, but 
Hoche has found degeneration of the oculomotor, trochlear, and abducent 
nerves, and this indicates that ophthalmoplegia might occur. 
Bulbar symptoms may exist without distinct alteration of the bulbar 
nuclei. The microscopic examination of a case reported by Dercum and 
the writer showed that the bulbar nuclei were for the most part intact, 
but the degeneration of the pyramidal tracts extended above these 
nuclei. It is possible that nerve fibres controlling these bulbar nuclei 
were affected, and this would seem to support Charcot's view of the pro- 
cess as primarily a degeneration of the pyramidal tracts. Oppenheim 
observed symptoms of disease of the ninth, tenth, and eleventh nerves in 
amyotrophic lateral sclerosis without changes in the nuclei of these nerves. 
The bulbar nuclei, on the other hand, may be affected without implica- 
tion of the pyramidal tracts. Duval and Raymond have reported a case 
in which the bulbar nuclei were affected and no trace of sclerosis of the 
motor tracts was seen. Raymond refers to a number of other cases in 
which the nuclei were affected but the motor tracts escaped. Miura 
asserts, however, that no case of progressive bulbar palsy without degen- 
eration of the pyramidal tracts is to be found in the literature. 
It occurred to the writer in 1899 that inasmuch as amyotrophic lateral 
sclerosis is a disease of the motor system, a case in which the cortex was 
degenerated could be employed in delineating the extent-of the motor 
area in man. The process seems almost like an experiment. This use 
of amyotrophic lateral sclerosis was original with the writer, and later DISEASES OF THE MOTOR TRACTS 
93 
was adopted by others. Intense degeneration of the precentral convolu- 
tion was found and considerably less degeneration of the postcentral con- 
volution, and therefore it was concluded that the latter is also a part of 
the motor cortical area. The postcentral convolution may escape. It is 
proper to state, however, that the tendency is to exclude the postcentral 
convolution from the motor region. 
Degeneration of the corpus callosum has been found by Probst, Spiller, 
Rossi and Roussy, and Naka in the middle portion of this structure. In 
the writer's case it extended in less intensity to the knee. 
The large cells of the motor cortex have been found diseased. Mott 
found that many of them had disappeared. Marinesco has found the 
giant cells of the motor cortex greatly diseased. Most of these cells 
had disappeared, and the few remaining were atrophied, and had under- 
gone chromatolysis and displacement of the nucleus. 
Some degeneration of the cdlumns of Goll in the cervical and upper 
thoracic regions has been observed in a case of primary lateral sclerosis 
reported by Dejerine and Sottas. Sensation had been normal. Moeli 
observed degeneration of these columns in these regions in a case of 
amyotrophic lateral sclerosis. 
The columns of Goll are very liable to undergo a slight sclerosis in the 
lower cervical and upper thoracic regions in cases where no symptoms 
of this sclerosis have been present, and in some way it seems to be related 
to disease of the central motor tracts. Oppenheim, and Charcot and 
Marie have noticed sclerosis of these columns in amyotrophic lateral 
sclerosis. These cases must be distinguished from those in which the 
symptoms of posterior sclerosis were added to those of sclerosis of the 
lateral columns, a posterolateral sclerosis. Nonne observed a distinct 
though not excessive rarefaction of fibres in the median portion of the 
posterior columns in chronic anterior poliomyelitis; it was most evident 
in the cervical region. The degeneration of the posterior columns in these 
cases of primary degeneration of the motor system is not tabetic in char- 
acter, it is not systemic. The posterior roots are not degenerated, and 
the region affected, lower cervical and upper thoracic, is not that usually 
diseased in tabes. 
The muscles may be intensely atrophied,- but even in many of these 
atrophied fibres the transverse striation may be distinct; in others the 
longitudinal striation may be more visible. The muscle fibres may or 
may not show fatty degeneration. In some of the fibres striation may 
not be clearly seen. The vessels may be much altered. Much fatty tissue 
may be found in some parts of the field. The nerve fibres between the 
muscular fibres may appear normal. The ulnar nerve has been found 
degenerated in one case (Spiller). 
Symptoms.-This is a disease of middle life. Marie regards those 
cases (Erb, Seeligmuller) in which it is said to have begun in childhood 
as very doubtful, and believes that some of them belong to the family 
spastic paralysis. Marie gives the commencement between the ages of 
thirty-five and fifty years; Dejerine, the second half of adult life; Oppen- 
heim, middle life; Eichorst, between thirty-five and fifty years; Gowers, 
between twenty-five^and forty-five years; Strumpell, between twenty- 94 
DISEASES OF THE NERVOUS SYSTEM 
five and forty years. In Probst's statistics of 47 eases the disease began 
most frequently between thirty and fifty years, in 13 cases between fifty 
and sixty years, in 5 cases between sixty and seventy years, and in one 
case after seventy. Rossi and Roussi conclude that the beginning is 
usually between thirty and fifty, although frequently between fifty and 
sixty years; it is rare between sixty and seventy, and exceptional after 
seventy years. They have not been able to find any case recorded with 
a commencement after seventy 
years, except one case of Probst, 
but they report 2 cases from 
the service of Pierre Marie in 
which the commencement was 
at the seventy-first and sev- 
enty-third year. 
Marie fixes the duration at 
eighteen months to two years, 
but death may occur in three 
to six months. Dejerine has 
observed a duration of nine 
years, and Florand of ten 
years. Marie questions whether 
these were truly cases of amyo- 
trophic lateral sclerosis. Col- 
lins selected the records of 94 
typical cases, and added to 
these 10 of his own but not 
all were with necropsy. He 
found that in 100 selected 
cases, 30 patients were between 
thirty and forty, 29 between 
forty-five and fifty, 28 between 
fifty and sixty, and 2 under 
thirty. In 26 cases the average 
duration was a little more than 
two years. In 2 cases the dis- 
ease seems to have terminated 
in six months. Records of 4 
cases were found in which the 
disease lasted only nine months. 
A few cases lasted only about 
five years; the longest on record 
was ten years. 
Amyotrophic lateral sclerosis may begin occasionally at an early age. 
The writer saw with Dr. Gittings, a boy, aged nineteen years, who had 
marked bulbar symptoms with signs of degeneration of the nerve cells 
of the anterior horns in the cervical and thoracic regions, and possibly 
slight degeneration of the pyramidal tracts. The course was rapidly 
fatal, the symptoms being present only about one year. 
The symptoms were first manifested in two of the writer's cases in one 
Fig. 1 
Amyotrophic lateral sclerosis. With bulbar symp- 
toms and greater implication of the proximal part of 
the upper limbs, in a boy, aged nineteen years. (Spiller 
and Gittings.) DISEASES OF THE MOTOR TRACTS 
95 
lower extremity, although in one of these the upper limb of the same side 
may have been implicated at nearly the same time. The paralysis in 
these two cases became a hemiplegia and later a triplegia. In another 
case the paralysis seemed to begin as a hemiplegia. The statements of 
the patient or of the relatives must be taken in determining the part first 
affected, and there is, therefore, an element of doubt. The weakness and 
atrophy usually begin in the muscles of the hands, but sufficient cases are 
on record to show that they may commence in the feet. Ina case reported 
by Dercum and the writer the weakness of the lower limbs preceded the 
bulbar symptoms about three years. In 81 cases collected by Collins, 
the upper extremities were first involved in 39 cases, the lower in 14 cases, 
the limbs of one side in the hemiplegic type, or all four limbs in 1.1 cases. 
The writer collected reports of 11 cases (Dejerine, Pick, Vierordt, 
Leyden, Lennmalm, IXIott, Senator, Probst (4)) in which the symptoms 
were confined to one side of the body, although both pyramidal tracts 
were affected in some of these cases. A case reported by C. S. Potts 
should be included in the list. Where the disease begins as a unilateral 
affection it does not remain as such, but sooner or later, and usually soon, 
the other side of the body becomes implicated. The shoulder muscles 
in rare instances may be affected first, and Dejerine and Thomas report 
two such cases, or the extensor muscles of the hand may be first affected. 
Raymond and Cestan1 from a study of their cases make four types of 
amyotrophic lateral sclerosis: (1) Ordinary spinal type; (2) labio-glosso- 
laryngeal paralysis; (3) amyotrophic type; (4) spastic type. 
1. Ordinary Type.-This corresponds to the classical descriptions of 
Charcot with spastic paraplegia or tetraplegia, ankle clonus, later amyo- 
trophy, fibrillary tremors, and disturbances in the electrical reactions. 
Babinski's reflex was not observed, which seems to be remarkable. Only 
three of their cases corresponded to this type, and in two of them the 
amyotrophy began in the lower limbs and later implicated the upper 
limbs and the muscles innervated from the medulla oblongata. In one 
case the amyotrophy began in the hands. These cases lasted twenty-four 
twenty-six, and twenty-six months respectively. 
2. Labio-glosso-laryngeal Type.-This was observed five times, four 
times in women, and Raymond and Cestan are inclined to think it is 
more common in this sex. The affection began in the muscles of the lips, 
tongue, and larynx, with exaggeration of the masseter reflex. Soon the 
limbs became implicated with exaggeration of the reflexes and progressive 
amyotrophy of the upper limbs. One patient had in the beginning attacks 
of choking, probably from spasm of the glottis; another had spastic 
laughing and weeping, without impairment of intellect. The cord showed 
degeneration in the anterolateral columns of the cervical and thoracic 
regions by the Marchi method. The duration of these five cases was from 
fourteen to twenty-four months. 
3. Amyotrophic Type.-The disease appears as progressive muscular 
atrophy. The spasticity is slight, and ankle clonus and Babinski's sign 
are not obtained, but the bone and tendon reflexes are exaggerated, some- 
1 Raymond and Cestan, Revue Neurologique, 1905, p. 504. 96 
DISEASES OF THE NERVOUS SYSTEM 
times only slightly. The slight spasticity makes the diagnosis somewhat 
doubtful. The duration ranged from eight months to five years. The 
diagnosis must be made from the Duchenne-Aran type of muscular 
atrophy and subacute anterior poliomyelitis. The necropsies showed 
degeneration of the anterolateral columns. Nine of their cases were of this 
type. The disease of the peripheral motor segments is prominent in 
this type, but the implication of the central motor segments is indicated 
Fig. 2 
Amyotrophic lateral sclerosis. Intense atrophy of the hands. 
by some exaggeration of the tendon reflexes, although spasticity is slight 
or even absent. It resembles subacute anterior poliomyelitis very closely, 
and, indeed, several of the cases of this latter disease with necropsy that 
have been reported belong to the non-spastic type of amyotrophic 
lateral sclerosis, as pictured by Raymond and Cestan. 
4. Spastic Type.-Only one of Raymond and Cestan's cases belonged to 
this type. Spastic paraplegia was intense during one year, and was DISEASES OF THE MOTOR TRACTS 
97 
associated with ankle clonus and Babinski's sign, presenting the clinical 
picture of what is described by French writers as spastic dorsal tabes. 
Disseminated sclerosis, Erb's form of spinal syphilitic myelitis, and com- 
bined sclerosis in an early stage may present the same clinical picture. 
After a year progressive amyotrophy of the hands was observed, then 
the legs became affected, and still later the bulbar muscles. 
Mills excludes Gowers' atonic 
variety in which both upper and 
lower limbs are the seat of 
wasting without spasm, except 
in cases in which the lower 
motor segments degenerate first, 
especially if the degeneration be 
complete, as any degeneration 
of the upper motor segments 
may then fail to produce the 
usual symptoms. It may be 
said, however, that all such cases 
clinically belong to progressive 
spinal muscular atrophy or 
chronic poliomyelitis, and their 
true relation cannot be recog- 
nized without necropsy. Xot 
only may the peripheral motor 
segments be alone affected, but 
degeneration may be confined 
to the central motor segments. 
The most important symp- 
toms are weakness, spasticity, 
fibrillary tremors, exaggerated 
tendon reflexes, contractures, 
muscular atrophy, and bulbar 
disturbances. The weakness is 
an early manifestation, and is 
caused by tht1 degeneration oc- 
curring in the central and per- 
ipheral motor tracts as well as by 
the atrophy of the muscles. It 
is usually first noticed in the 
hands; the patient loses the 
finer movements of the fingers 
and can no longer approximate 
the thumb to the little finger 
with the same force, because of weakness in the muscles of the distribution 
of the median nerve. Sewing and writing become difficult. If he has learned 
any form of skilled movements, like playing on a musical instrument or 
typewriting, he has difficulty in performing these. Soon the wasting in 
the thenar and hypothenar eminences and in the interosseous spaces 
becomes pronounced, the weakness of the hand muscles progresses, and 
Fig. 3 
Amyotrophic lateral sclerosis beginning in the 
shoulder girdle and proximal parts of upper limbs. 
The utmost effort was made to elevate and flex the 
upper limbs, with little result. Flexion of the fingers 
was feeble, but far greater than movement in the rest 
of the upper limbs. 98 
DISEASES OF THE NERVOUS SYSTEM 
causes the simian hand, so called because of the loss of the thenar emin- 
ence. The extension to the forearm is gradual, and yet likely to occur 
early, while the arm and shoulder muscles usually are not implicated 
until considerably later. The weakness and atrophy increase pari passu, 
but the former is not dependent alone on the latter, as degeneration of 
the pyramidal tracts must cause weakness independent of. any atrophy 
of the muscles. 
The lower limbs are more or less spastic from the beginning, and 
weakness of them gradually develops. They do not, as a rule,'present 
much atrophy until the disease has attained a considerable development; 
this is because the pyramidal tracts are early affected, while the cells of 
the anterior horns in the lumbar region may escape until late. Cases in 
which the symptoms begin in the lower extremities, and the upper 
extremities are not implicated until later, occur much more frecfuently 
than those in which the shoulder muscles are first affected. The muscles 
of the neck and trunk, as well as those of the face, may become greatly 
wasted, and the patient may be unable to hold the head erect. 
The tendon reflexes, the biceps and triceps, von Bechterew's scapular 
reflex, the patellar and Achilles tendon reflexes are early exaggerated, and 
those of the lower limbs usually as much as those of the upper limbs. 
Clonus is common, both ankle and patellar clonus. It may even be 
seen in the upper limbs, as wrist, biceps or triceps clonus; but it is much 
less likely to occur in the upper than in the lower limbs. In 10 cases 
reported by Raymond and Cestan, in which the Babinski reflex was 
searched for, it was found only once, and that in an extremely spastic 
case. It is hard to understand why this sign should have been absent 
in the other cases, as extreme spasticity is not necessary for its produc- 
tion when the central motor tract is affected. The masseter reflex 
often remains normal when the tendon flexes of the limbs are exaggerated, 
but if the disease be intense, or extend far upward in the pyramidal 
tracts, the chin-jerk may be intensified as well as the other reflexes. 
Beevor was the first to observe, in 1881, spontaneous clonus of the lower 
jaw. The sign, according to Beevor, shows that the sclerosis affects the 
pyramidal tract as high as the level of the fibres going to the motor nucleus 
of the fifth cranial nerve. He never observed clonus of the lower jaw in 
any other disease, except once in a case of hemiplegia. There may be 
some rigidity of the lower jaw with jaw clonus preceding the wasting of 
the bulbar muscles in the bulbar form of amyotrophic lateral sclerosis, 
and this may be absent in the true bulbar palsy. 
In the case reported by the writer in which degeneration was traced 
to the cortex, there was some loss of power in the orbicularis palpebrarum 
of the left side, preventing the patient from closing the left eye tightly. 
The case is like one reported by Marinesco in the implication of the 
upper part of the face; and this is remarkable, as in bulbar palsy the 
upper part of the face usually escapes. The patient had a low-grade 
neuroretinitis and pronounced perivasculitis. The presence of this ocular 
condition does not exclude amyotrophic lateral sclerosis. 
Fibrillary tremors occur usually in the atrophying muscles and may be 
intense, but may not be always present when the patient is under observa- DISEASES OF THE MOTOR TRACTS 
99 
tion, and may disappear when the atrophy has attained an intense 
degree. They usually persist, however, a long time, and may precede 
the atrophy. They are indicative of degenerative changes going on in 
the cells of the anterior horns of the spinal cord, and have been described 
by the not very appropriate name of "fibrillary chorea." 
The upper and lower limbs are usually spastic, but if the atrophy 
attains a considerable development the spasticity gradually diminishes 
and may disappear. It is not unusual to observe the spasticity greater 
in the lower than in the upper limbs, as atrophy is less likely to develop 
in the former. Contractures of the intensity seen in hemiplegia are rare, 
but there may be more or less fixity of the limbs from the increased 
tonicity of the muscles, and if this continue sufficiently long, contraction 
will occur and cause permanent fixity even after the spasticity has 
disappeared. Contractures are not very pronounced, but when present 
are usually of the flexor muscles. They may be so intense that the feet 
may be in the position of talipes equino-varus. In the upper limbs the 
arm may be held closely to the chest, the forearm flexed on the arm 
almost at a right angle, the hand flexed at a right angle to the forearm, 
the thumb turned inward and the fingers flexed. Involuntary jerking of 
the fingers is common, and may be related to fibrillary contractions. The 
wasting of the muscles and the gradual diminution in their tonicity 
diminish the contractures as the case progresses. 
Marie has observed intention tremor of less intensity than in dis- 
seminated sclerosis. If such occur it probably is caused by muscular 
weakness and inability to coordinate properly the movements. 
The reaction of degeneration is not usually very pronounced, as normal 
muscle fibres are mingled intimately with degenerated fibres, and the 
former mask the reaction presented by the latter. A change in the 
formula as well as a modal change may occur, but much more common 
is diminished response. 
Retention of urine and loss of control over the bowels have been ob- 
served, and are probably the result of lesion of the pyramidal tracts. 
A certain degree of mental failure may be observed occasionally, and 
the patients may take little notice of what is going on about them. 
Some writers have recognized mental deterioration as a part of the 
symptom complex. Marie says that mentality is always more or less 
affected, intelligence is enfeebled, emotionality is increased, and ten- 
dency to laugh or weep is pronounced. Sometimes a demented condition 
exists. The patient is moody, and intellectually and morally approaches 
the attitude of a child; at times the condition is like that of neurasthenia. 
These conditions are in association with bulbar symptoms. Suicidal 
impulses are not necessarily a sign of mental failure. One cannot wonder 
that the hopelessness of the disease should so impress the patient as to 
make death preferable to a miserable and prolonged existence. 
Pain is sometimes observed. In one of the writer's cases the man 
complained of pain in both lower limbs below the knees five or six months 
previously. These were probably the pains of fatigue, as the patient 
was weak in the lower limbs and became very tired by night. Muscles 
undergoing atrophy and partially paralyzed may readily cause pain if 100 
DISEASES OF THE NERVOUS SYSTEM 
exercised beyond the limited power remaining in them. Naka observed 
pain in the beginning of the disease in his case, and no cause for it was 
found. He could not attribute it to the spasms, as Oppenheim has done, 
as his patient did not have spasms. Sensory disturbances of the objective 
form (anesthesia, hypoesthesia, analgesia, hypoalgesia) do not belong to 
the ordinary clinical picture, but pain on pressure has been observed. 
It seems reasonable to attribute these painful phenomena to an associated 
neuritis. Much caution is needed in making a diagnosis of amyotrophic 
lateral sclerosis when sensory symptoms are pronounced, as in most 
such cases some complication is probable or the diagnosis is incor- 
rect. 
The disease may begin as bulbar palsy, and when this type occurs 
death is usually too rapid to permit of much implication of the limbs. 
Or the bulbar implication may develop within a few months or a year or 
two after the limbs are affected, as in one of the writer's cases in which 
dysphagia and dribbling of saliva developed almost at the same time 
as the loss of power in the upper and lower limbs. The symptoms in 
this case lasted about one year when death occurred. 
The muscles about the mouth become affected, the patient can no 
longer properly pucker up his lips as in whistling or in blowing out a 
candle, the lips sounds-1, b, t, r, s, k, g, ch, i, d, n-become imperfect, 
and fibrillary tremors become very distinct in the face and tongue. 
The upper part of the face, as in progressive bulbar palsy, usually is 
not affected. The tongue becomes deeply furrowed, and the atrophy 
and weakness may be so great that the patient may be unable to protrude 
it beyond the lips. The soft palate may be paralyzed and atrophied, 
so that fluids regurgitate through the nose when the attempt is made 
to swallow; the voice is nasal and very indistinct, and the soft palate no 
longer rises, or rises imperfectly when the attempt is made to say "a." 
The pharyngeal muscles also may be affected and swallowing becomes 
very difficult, and the pharyngeal reflex may disappear. The face may 
be much atrophied, death not occurring rapidly enough to prevent the 
atrophy, even when the bulbar nuclei are involved. Even when the 
lower part of the face is intensely implicated the eyelids usually can be 
held firmly closed. The vocal cords may be paralyzed. 
Bulbar symptoms do not necessarily indicate a speedy fatal termina- 
tion. In Raymond and Cestan's cases, in which the bulbar disturbances 
were the first symptoms, the duration of the disease was not shorter than 
in those in which the first symptoms were spinal, and bulbar disturbances 
developed later. 
The muscles of the face, outside of those of the lips, usually escape. 
The muscles of mastication are not usually among the first affected in 
bulbar palsy, but they may be to such an extent that the patient is 
unable to chew or to move the jaw from side to side. Food may enter 
the larynx and cause attacks of coughing or aspiration pneumonia. 
Bulbar symptoms occurred first in 21 of the cases collected by Collins. 
In upward of 50 per cent, of all the cases, bulbar symptoms appeared 
within the first year. Marie is inclined to believe that labio-glosso- 
laryngeal paralysis is always a part of amyotrophic lateral sclerosis. DISEASES OF THE MOTOR TRACTS 
101 
Diagnosis.-What has been said in the differential diagnosis of pro- 
gressive spinal muscular atrophy applies equally well to amyotrophic 
lateral sclerosis, when it is remembered that in the latter disease we 
have the symptoms of progressive spinal muscular atrophy with the 
symptoms of spasticity, exaggerated reflexes, and Babinski's sign. 
Prognosis.-This is exceedingly grave. The disease is fatal, and may 
terminate within one year, and usually does so within three or four 
years. We have no means to arrest its course, and the end usually comes 
through the implication of vital centres in the bulbar palsy. 
Treatment.-The section on treatment in the primary degenerations of 
the motor system need not be very extensive, as the results are not 
brilliant. The administration of strychnine may be of service. Over- 
exertion is to be avoided, as in this way too rapid exhaustion of the 
nervous system may be prevented. Massage, passive movements, and 
electricity are of doubtful value when the atrophy and weakness are 
progressive, although when the course is very slow or has been arrested 
they may be of great service. 
LATERAL SCLEROSIS (SPASTIC TABES, SPASTIC SPINAL PARALYSIS, 
SPASTIC DORSAL TABES). 
In a paper written in 1903, Erb remarks that he did not suppose when 
he first described spastic spinal paralysis in 1875 he would be compelled 
twenty-eight years later to fight for its recognition. His views expressed 
in 1875 received the support of Charcot and others, but met with the 
skepticism of prominent neurologists, especially of the Berlin school. 
The condition was regarded merely as a symptom complex caused by 
various disorders of the spinal cord and brain; as a condition without a 
definite pathology. Necropsies did not confirm the views of Erb and 
Charcot that primary degeneration of the pyramidal tracts was the 
underlying lesion, and while degeneration of these tracts was found, it 
was usually secondary to hydrocephalus, multiple sclerosis, tumor, 
syringomyelia, or symmetrical cerebral lesions; or was part of postero- 
lateral sclerosis or of amyotrophic lateral sclerosis. 
Leyden and Goldscheider acknowledge the possibility of primary 
degeneration confined to the pyramidal tracts, but the clinical diagnosis 
of this condition they believe is impossible, and therein is the important 
factor. It is impossible, they think, to speak of an etiology, pathogenesis, 
and therapy of spastic spinal paralysis. Strumpell does not accept this 
view, but regards as the important factors the clinical and anatomical 
findings; when these are established the diagnosis will be easy. As 
justifying the recognition of spastic spinal paralysis, Erb accepts 11 cases; 
they are those of Morgan and Dreschfeld, Minkowski, Strumpell (2 
cases), Dejerine and Sottas, Donaggio, Friedmann, Bischoff (2 cases), 
Demock, and Kuhn. 
Pathology.-This, if it is to be accepted as an entity, consists of primary 
degeneration of the pyramidal tracts and nothing else, and this degenera- 
tion may stop at any level or extend to the cortex. If we accept all 102 
DISEASES OE THE NERVOUS SYSTEM 
the J1 cases that Erb includes, we must be a little more liberal in our 
interpretation. Thus, in Morgan and Dreschfeld's case, atrophy of 
some cells of the anterior horns in the thoracic and lumbar regions was 
found, but Erb accepts it because this degeneration seemed unimportant 
and amyotrophy was not present during life. Grainger Stewart stated 
that he examined the sections of this case and found the changes in the 
gray matter distinct. At the time this case was reported Nissl's method 
was not in vogue, and could it have been employed it is not improbable 
that more evidence might have been obtained justifying the classification 
of the case as one of amyotrophic lateral sclerosis. 
Implication of the direct cerebellar tracts, as in Minkowski's case, 
may be accepted as possibly causing no clinical signs, and therefore need 
not exclude a case from the designation of spastic spinal paralysis. 
Whether the same may be said of a slight degeneration of Goll's columns 
in the upper part of the cervical region, as in one of Strumpell's cases and 
in others, is open to question; but it occurs in certain cases of amyotrophic 
lateral sclerosis, in which no clinical evidence of its presence is observed. 
Strumpell, however, made the diagnosis in his case as combined system 
disease. Where syphilitic meningomyelitis exists the degeneration of 
the pyramidal tracts cannot be regarded as primary, and if a nuclear 
stain be not employed the cellular infiltration of the pia and cord may be 
overlooked. 
Symptoms.-These, as originally described by Erb, are motor weakness, 
spasticity, and exaggerated reflexes; to these should be added the Babinski 
reflex. No other symptoms should be present, there should not be 
sensory disturbance, atrophy, sphincteric disorder, tumor, ataxia, or 
implication of the cranial nerves. The symptoms may persist many 
years, and Erb quoted cases in which they have lasted nineteen to twenty- 
seven years. They may develop very slowly, during twenty-three years 
in the case of Dejerine and Sottas. 
The weakness usually affects one lower limb some time before the other 
lower limb becomes implicated, but it is often much less intense than 
the spasticity. It is at first a sense of fatigue after slight exertion, and 
spasticity is associated with the paresis. The patient stubs his toe in 
walking because of the spastic condition of the muscles of the lower limbs, 
and the manner in which the soles of the shoes are worn is often very 
diagnostic, the sole at the point of the shoe may be completely worn 
away. The Achilles tendon and patellar tendon reflexes are much 
exaggerated, so that ankle clonus and patellar clonus may be persistent, 
or the whole lowrer limb may be thrown into clonic movement, a condition 
inappropriately called spinal epilepsy by French writers. The lower 
limbs are rigid, the thighs adducted, so that walking is interfered with, and 
the scissors gait, so called because of the tendency for one limb to be 
drawn in front of the other, is produced. The spasticity may be so 
pronounced that marked spasm is caused by passive movement. If 
the leg be extended upon the thigh, passively, a sudden spasm may 
jerk the leg to full extension, the "clasp-knife" reaction. 
The upper limbs may escape entirely, but if they be affected the 
symptoms may be less marked, though of the same type as those in DISEASES OF THE MOTOR TRACTS 
103 
the lower limbs. In later stages of the disease the patient becomes 
bedridden and unable to stand. Death results from some intercurrent 
disease. 
Slight flexion at the knees Striimpell believes will be found in all cases 
of spastic spinal paralysis beginning in early youth. This is usually 
attributed to spastic contracture in the affected muscles, but Strumpell 
believes it is caused by differences in growth of certain muscle groups. 
The flexors are more affected than the extensors, therefore their growth 
is arrested when the lesion develops early in life. 
Strumpell distinguishes the following forms of spastic spinal paralysis: 
1. A form certainly endogenous, characterized by family and hereditary 
appearance, males especially affected, commencing between the twentieth 
and thirtieth years, progressing very slowly, lasting a long time (twenty to 
thirty years or longer). The symptoms are gradually increasing rigidity 
and hypertonia of the muscles of the lower limbs, exaggeration of tendon 
reflexes, etc. Gait is spastic and for a long time without marked paresis, 
but paresis occurs later, especially in the flexors and muscles of the lower 
part of the lower limbs. The upper limbs remain intact until the end, 
likewise the bulbar and cerebral functions. 
2. Infantile spastic spinal paralysis. This commences in childhood, 
at times as early as the third to sixth year, although it may begin later. 
This type is related to the cases of congenital abnormal development. 
A careful history must be obtained in order to decide whether the con- 
dition be congenital agenesia, or progressive degeneration beginning 
later. Inquiry must be made regarding injuries at birth, healed enceph- 
alitis, etc. The symptoms are those of spastic paralysis of adults, but 
modified by certain conditions of the developing organism. The upper 
limbs are often unimplicated, or at least only slightly so, but they may 
be distinctly affected. Arrest in cerebral development is often present, 
shown by mental deficiency. Such cases are not very rare. 
3. A third type is also endogenous in origin. It begins in late life, 
progresses comparatively rapidly, implicates the entire pyramidal tracts, 
so that the upper limbs and bulbar muscles are affected. The symptoms 
are general spastic rigidity, peculiar spasticity in bulbo-cerebral territories 
(glottis spasm, forced phonation, forced laughter, forced crying, invol- 
untary movements of the facial muscles, etc.). The tendency to implica- 
tion of the peripheral motor neurones is strong, shown by fibrillary 
twitchings, muscular atrophy, etc. These cases are closely related to 
amyotrophic lateral sclerosis. They usually are isolated, not occurring 
in families. 
4. An exogenous type. The syphilitic spinal paralysis, with the 
exception of lathyrismus, is the only important form. The lesions are 
seldom confined to the pyramidal tracts, and the columns of Goll are 
usually partially affected. The clinical picture of spastic spinal paralysis 
is seldom uncomplicated. 
5. A fifth form may occur in women after confinement and the puer- 
perium. Strumpell has observed a number of cases in which spastic 
spinal paralysis developed following these conditions. No necropsies 
of cases of this form have been obtained. 104 
DISEASES OF THE NERVOUS SYSTEM 
Diagnosis.-This is often difficult so far as the exclusion of other pro- 
cesses is concerned, and among these may be mentioned spinal tumor 
without pain, multiple sclerosis, hysteria, latent caries of the spine, 
myelitis, secondary degeneration from cerebral lesions, and combined 
sclerosis in which the symptoms of posterior column disease are indistinct. 
Indeed, it is questionable whether the diagnosis of primary lateral sclerosis 
can be made with absolute certainty during the life of the patient. 
Tumor of the spinal cord is occasionally difficult to distinguish. In it 
the symptoms are usually unilateral at first, bilateral later, and associated 
with severe shooting pains, but cases of spinal tumor occur in which 
the symptoms are bilateral from the beginning and pain is absent. In 
a number of cases the diagnosis between spinal tumor and primary lateral 
sclerosis seems impossible. 
Multiple sclerosis usually presents characteristic symptoms, but 
occasionally it may cause exactly the symptom complex of lateral sclerosis, 
when the lesions are almost confined to the spinal cord. In such cases 
the differential diagnosis may be impossible. 
Hysteria may cause a spastic paraplegia, but careful study will usually 
reveal some evidence of this neurosis. There may be some disturbance 
of sensation in segmental forms, some ocular signs; or the symptom 
complex may not be typically that of lateral sclerosis. An ankle clonus, 
if it occur, is seldom exactly the same as that of organic disease, although 
it may be found in grave hysteria. This statement is disputed by certain 
writers. If hysterical the gait will usually afford some clue to the neuro- 
sis, and the symptoms may disappear under suggestive treatment. 
Caries of the spine may occur without kyphosis and without other 
symptoms than those of implication of the lateral columns by compression. 
The diagnosis, while not usually difficult, may be impossible. 
Myelitis may be very hard to distinguish and the clinical picture may 
be exactly that of lateral sclerosis. It is often syphilitic in character, 
and examination of the spinal fluid is important. 
Secondary degeneration from cerebral lesions is usually distinguishable 
by the presence of cerebral symptoms and the much greater implication 
of one side of the body. The onset is likely to be acute, with first one 
side affected in one attack and later the other side in a second attack. 
The paralysis is more intense, as in lateral sclerosis the spasticity is usually 
more pronounced than the weakness. 
Combined sclerosis usually will cause at least slight indication of involve- 
ment of the posterior columns; there may be some indication of ataxia. 
Spastic spinal paralysis must also be distinguished from congenital 
arrest in the development of the pyramidal tracts or degeneration of 
these tracts from injury of the brain, with epilepsy, mental defect, etc. 
Similar cases may be caused by premature birth, intra-uterine cerebral 
disease, hydrocephalus, etc. The upper limbs may be more affected 
than they are in the typical spastic spinal paralysis, or cranial nerve 
implication may be detected. 
Treatment.-Division of a certain number of the posterior lumbar 
roots may prove to be of benefit in lessening the spasticity, as may also 
injection of the nerves supplying the more spastic muscles with alcohol DISEASES OF THE MOTOR TRACTS 
105 
(Schwab and Allison). These forms of treatment are too recent to 
permit a judgment as to their value. It is possible that massage and 
electricity directed only to the less spastic muscles may bring about a 
more even degree of tonicity in all the muscles. Special forms of move- 
ments may be of service. 
HEREDITARY SPASTIC SPINAL PARALYSIS. 
Etiology.-Syphilis, trauma, etc., seem to have no strictly causal rela- 
tion, although trauma may act as an exciting cause in persons predisposed. 
The second of Newmark's cases with necropsy seems to show that agenesia 
of the pyramidal tracts may exist, so that these tracts from the beginning 
are imperfectly developed, and if slight degeneration occur it would be 
likely to produce pronounced symptoms. This hypothesis would explain 
those mild cases where the only sign of the disease is exaggeration of the 
tendon reflexes, as in two members of Newmark's O'Connor family. 
There is much to support the view that the spastic spinal paralysis of 
family type is an abiotrophy, especially the commencement in several 
members of the same family at nearly the same age. The motor fibres 
have limited potentiality, and sooner or later degenerate. 
Strumpell thinks the male sex is more predisposed, and that when 
the males are affected in a family the females always escape, but this 
latter statement may be challenged. The difference between the sexes 
as regards frequency is not very striking. The disease is both familial 
and hereditary. Several observations show, especially Bernhardt's, 
that in the same family other nervous diseases occur in addition to spastic 
paralysis. The heredity is not always in exactly the same type, some 
neurosis or some organic nervous disease of different character may 
have been present in an ancestor, and yet the cases in which exactly 
the same form of paralysis has been observed in parent and child are 
sufficiently numerous to show that the heredity may be direct. 
Pathology.-The findings are degeneration of the pyramidal tracts 
extending throughout the cord, most intense in the lumbar and lower 
thoracic regions, and becoming indistinct in the upper thoracic region 
(Newmark), or extending in lessened intensity to the pyramidal decussa- 
tion (Strumpell). The degeneration of the crossed pyramidal tracts in a 
mild case may be found only in the lower lumbar region, and may be 
very slight even there, with a little greater involvement of one side of 
the cord than of the other (Newmark). The direct pyramidal tracts 
were probably degenerated in one of Strumpell's cases (Gaum). 
The direct cerebellar tracts may be degenerated, but not intensely 
(Strumpell), or may be intact (Newmark). This degeneration may 
be followed above the pyramidal decussation into the medulla oblongata. 
The columns of Goll are degenerated, and more in the upper regions of the 
spinal cord than in the lower. Whereas, Striimpell has found the degen- 
eration of the columns of Goll less intense than that of the crossed pyra- 
midal tracts, Newmark has observed the reverse. The degeneration 
has been tracted to the nuclei of these columns in the medulla oblongata. 
Newmark found in one of his two cases the net-like condition in the 106 
DISEASES OF THE NERVOUS SYSTEM 
posterior columns, due to degeneration and dropping out of fibres, such 
as is seen in cases of severe anemia, and he suggests the possibility of 
an exogenous factor in addition to the endogenous factor. 
Strumpell has maintained that the degeneration of Goll's columns 
is secondary. If these columns degenerate first and the pyramidal 
tracts much later, real spasticity could not occur, as shown in Friedreich's 
ataxia, but Newmark believes, and apparently with reason, that his own 
second case with necropsy proves that a previous degeneration of Goll's 
columns does not prevent the spasticity resulting from the degeneration 
of the pyramidal tracts, as the degeneration of these tracts was only 
in the lower lumbar region and very slight, while that of Goll's columns 
was pronounced. Although the degeneration of the last-mentioned 
columns seems to have occurred in every case with necropsy, in none 
has it produced recognizable symptoms. The lesions are those of a 
combined sclerosis. The anterior and posterior roots, and the nerve 
cells of the anterior horns have not been found affected. 
Symptoms.-Spastic paraplegia occurs in children or adults of the same 
family and may be observed in several generations of the same family, 
sometimes commencing at an earlier age in the later generations. The 
symptoms, at first with little or no weakness, are spasticity, exaggerated 
reflexes, usually only in the lower limbs, Babinski's reflex, and paralysis 
as a late manifestation. Contractures of the feet may be very pro- 
nounced, but sensory symptoms are not present; although in Strumpell's 
case Polster tactile sensation was a trifle less acute in the legs than in the 
thighs, and temperature sensation was not entirely normal in the legs, 
although it was so in the thighs. This disturbance could not be attributed 
to vasomotor changes, as these as well as trophic symptoms were absent. 
Newmark would not exclude cases in which optic atrophy, feeble men- 
tality, muscular atrophy, bulbar symptoms, bone lesions, and cataract 
occur. Such cases may not be pure examples of spastic paraplegia, 
but they are manifestations of abiotrophy. The symptoms usually 
developed nearly at the same period in the same family, but not always, 
and the periods vary in different families. In some families the disease 
first shows itself in the first years of life; in others, in early adult age; 
in others, still later. In one family Newmark found the ages at which 
the symptoms began fourteen, seven, nine, sixteen, eight, and eight 
years respectively. Krafft-Ebing observed the commencement at about 
twenty-seven, twenty-four, and twenty-two years respectively in mem- 
bers of the same family. 
The symptoms may reach their height within a short time, especially 
after fever or injury, and remain at a certain stage a long time or per- 
manently. In mild cases some improvement is possible. In some 
cases slow progression begins again after a period of arrest; in others the 
symptoms progress slowly from the time they first become manifest. 
Cases with mental symptoms, implication of cranial nerves, unless 
possibly strabismus, may be regarded as unimportant, history of trau- 
matism at birth, symptoms existing from birth, epileptic attacks, dis- 
turbances of sensation or muscular atrophy, can hardly be regarded as 
typical of the spinal form of spastic family paralysis. The family form DISEASES OF THE MOTOR TRACTS 
107 
of spastic paraplegia, probably being the result of degeneration of the 
distal portions of the pyramidal tracts from some imperfection of develop- 
ment, differs from the cases of Little's disease resulting from premature 
birth, in that in the latter the pyramidal tracts are arrested in their 
development, but are capable of further development even many years 
after birth, and probably do not degenerate. In the form occurring 
in childhood, the pyramidal tracts probably degenerate at their distal 
ends early, while in Strumpell's type the resistance of these tracts seems 
to be greater, and disturbance of function does not occur until adult life. 
In some families the cerebral type seems to occur in certain members, 
and the spinal type in others (Bernhardt, Pribram, Melotti, and Cantal- 
emessa). This would seem to indicate that there may be a close relation 
between these two types. In a family studied by the writer, a father 
and his second son showed clearly the typical symptoms of the disease, 
and a younger son showed indications of it. Only the lower limbs were 
affected. The disease seemed in the latest generation to be commencing 
at an earlier period. It has been almost as common in this family in 
the female sex as in the male. In late years the disease is said to have 
commenced before or about the fifth year of life, and it has always pre- 
sented the same symptoms. A young member of the family manifested 
the first symptoms about the age of eighteen months. The disease 
in this family was traced back five generations. This family is doubtless 
the same as that reported at an earlier date by Bayley, although the 
writer was not aware of this fact at the time of his report. 
Dejerine and Thomas say the affection extends later to the upper 
limbs and eyes and to the muscles of speech, and yet in most cases these 
parts, except for exaggeration of the tendon reflexes, escape. 
In late stages the lower limbs may be almost completely paralyzed; 
Strumpell says it is characteristic of the hereditary spastic paralysis to 
have great exaggeration of tendon reflexes precede many years true 
paralysis. It is uncertain whether reflex exaggeration and weakness 
result from disease of different fibres or from different degrees of degenera- 
tion of the same fibres. Strumpell has not seen involvement of the upper 
limbs beyond exaggerated reflexes. The condition must be distinguished 
from the spastic cerebral paralysis of childhood, not always with defect 
of mentality. This is an agenesia and may be familial in type and has 
an etiological relation to the spinal form. 
Diagnosis.-This must be made from cerebral paralysis, Little's dis- 
ease, myelitis, Pott's disease, lateral sclerosis, family form of disseminated 
sclerosis, Friedreich's ataxia, and cerebellar hereditary ataxia. The 
difficulties are those mentioned for primary lateral sclerosis. Strumpell 
acknowledges that he, like Charcot, diagnosed primary sclerosis in a case 
in which the necropsy showed multiple sclerosis. 
In some cases bulbar and cerebral disturbances existed in addition to 
the spastic paraplegia (Bernhardt), suggesting multiple sclerosis; in 
others premature birth, strabismus, slow acquirement of speech, feeble 
intelligence suggested cerebral origin. Family spastic paraplegia may 
be cerebral when associated with such symptoms, and Dejerine and 
Thomas would regard it merely as a symptom complex common to family 108 
DISEASES OF THE NERVOUS SYSTEM 
disease of different character and situation. It seems that cases in which 
the upper limbs are contractured, intention tremor, scanning speech, 
nystagmus, atrophy of the optic disks, strabismus, etc., are present, 
should be accepted with reservation. They do not belong to the typical 
form and may be examples of multiple sclerosis. 
UNILATERAL ASCENDING AND DESCENDING PARALYSIS. 
Attention was first called to these forms of paralysis by Charles K. 
Mills. In many cases it is a symptom complex instead of a well-defined 
disease, just as bilateral spastic paraplegia is often a symptom complex, 
but may be the clinical expression of primary degeneration of the 
pyramidal tracts. The question that now interests us is, Does unilateral 
primary degeneration of the pyramidal tract occur? Some years ago 
the writer searched for all the cases of the unilateral form of amyotrophic 
lateral sclerosis in the literature, and found ten; to these should be added 
a case reported by Potts, one reported by the writer, and one by Mills. 
The unilateral type of amyotrophic lateral sclerosis is only of short 
duration, and bilateral implication of the spinal cord occurs within a short 
time. The 13 cases are those of Dejerine, Pick, Vierordt, Leyden, 
Lennmalm, Mott, Senator, Probst (3, possibly 4), Potts, Spiller, and 
Mills. Of these, only 5 were of the ascending type, the weakness com- 
mencing in one lower limb and later extending to the upper limb of the 
same side. These were the cases of Vierordt, Mott, Probst, Potts, and 
Spiller. The symptoms may begin with bulbar palsy (Dejerine, Leyden, 
Lennmalm, Probst (2 cases) ). The upper limb of one side may be 
affected before the lower (Pick, Leyden, Probst). In some of the cases 
the limbs of one side seem to have been affected simultaneously or nearly 
so (Dejerine, Lennmalm, Senator, Probst (2 cases, Mills). In those cases 
in which a necropsy was obtained the pyramidal tract of each side was 
degenerated. It is yet to be shown whether amyotrophic lateral sclerosis 
may ever remain confined to one side; at present it seems improbable. 
A case of primary lateral sclerosis, reported by Mills and Spiller, would 
seem to indicate that the unilateral form of the disease without implication 
of the nerve cells of the anterior horns is possible. The patient, a male, 
aged sixty years, developed gradually hemiplegia on the right side, 
the lower extremity being more markedly, and for this reason probably 
earlier, affected than the upper; the case, therefore, at first belonged to 
the clinical type of unilateral progressive ascending paralysis. After 
several years the left lower extremity also became paralyzed, but not to 
the same extent as the right. The reflexes were all markedly exaggerated, 
the Babinski response being present. Sensory symptoms were absent. 
The lesions were primary degeneration of the motor tracts, much greater 
and of longer duration in the right crossed and left direct pyramidal 
tracts, with integrity of the nerve cells of the anterior horns. 
It seems positive that unilateral ascending or descending paralysis 
may be caused by different conditions. It may, in the opinion of Mills, 
be the expression of a gradually developing cerebral lesion such as soften- 
ing or tumor, of disseminated sclerosis, of a developing posterolateral PLATE I 
Progressive Neural Muscular Atrophy. 
Atrophy of the hands and forearms, with escape of the upper arms in 
the writer's case, lasting about forty-five years. PLATE II 
Atrophy and Contracture of the Feet and Legs. 
With escape of the thighs, in the case represented in Plate I. DISEASES OE THE MOTOR TRACTS 
109 
sclerosis, of spinal syphilis, of tumor of the cord or of other form of 
compression of the cord, of paralysis agitans, of hysteria. The type 
has varied in different cases even when the degeneration has been believed 
to be confined to the pyramidal tract. In some spasticity has been 
present from an early period, in others it has been absent or slightly 
marked. The tendon reflexes have usually been exaggerated, but in a 
few instances have been diminished or slight. The wasting of the 
muscles has varied in degree. Optic atrophy and vesical disturbance 
have been observed. The explanation of these differences is to be 
sought chiefly in the greater or less implication of the cells of the anterior 
horns of the spinal cord. 
Pathological evidence as yet does not warrant the assumption that 
degeneration of the crossed pyramidal tract may begin in the lower part 
of this tract, or in the upper part, gradually extend, so as to implicate 
the motor fibres for the other limb of the same side, and remain confined 
to one pyramidal tract. 
PROGRESSIVE NEURAL (NEUROTIC) MUSCULAR ATROPHY (PRO- 
GRESSIVE MUSCULAR ATROPHY OF THE CHARCOT- 
MARIE TOOTH TYPE). 
Charcot and Marie, and Tooth described this form of atrophy indepen- 
dently in the same year (1886). The subject soon received attention 
from Joffroy, Hoffmann, B. Sachs in this country, and from many others, 
but evidently much has been included that does not properly belong here. 
Etiology.-No satisfactory cause has been found, but the fact that it is 
likely to occur both as an hereditary and as a family disease shows that 
some defect in development is probably responsible for it. It is more 
likely to occur in males (five times in males to once in females, according 
to Sainton), but no explanation for this can be found. The disease may 
show a similar heredity, i. e., father and son may both be afflicted in 
the same way, and this is true also of the spastic family paralysis. It 
has usually begun before the age of twenty-two years. 
Pathology.-The lesions are sclerosis of the posterior columns of the 
cord, slight degeneration of both pyramidal tracts (Sainton), with integ- 
rity of the anterolateral columns in some instances (Marinesco), alteration 
of the columns of Clarke, atrophy of the cells of the anterior horns of the 
cord, alteration of the peripheral nerves, which may be slight, and of the 
intramuscular branches, atrophy of muscle fibres, and chronic meningitis 
(Dejerine and Armand-Delille). The nerve trunks, the cutaneous 
sensory nerves, and the anterior and posterior nerve roots, with slight 
exception, may be normal. 
Symptoms.-The characteristic features of this form of atrophy as given 
in the first description by Charcot and Marie are: Progressive muscular 
atrophy implicating first the feet and legs, and not appearing in the 
upper limbs (hands and, later, forearms) until several years later, the 
progression of the atrophy being slow. Relative integrity of the muscles 
of the limbs near the trunk, or at least much longer preservation of these 
than of the muscles of the distal ends of the limbs. Integrity of the 110 
DISEASES OF THE NERVOUS SYSTEM 
muscles of the trunk, shoulders, and face. Fibrillary contractions in the 
atrophying muscles. Vasomotor disturbances in the portions of the 
limbs atrophied. No pronounced contractions of tendons in the atrophied 
limbs. Sensation usually intact, but sometimes affected. Cramps 
frequent. Reaction of degeneration in the atrophying muscles. Com- 
mencement of the affection usually in childhood, the disease often found 
in several brothers and sisters, and sometimes in the previous generations. 
Charcot and Marie, in their first publication, acknowledged the possi- 
bility of implication of the muscles of the limbs near the trunk, as they 
speak of relative integrity of these muscles. They also stated that the 
muscles of the thigh seem to preserve their power and volume during a 
certain period, but that this integrity often is not real. The vastus 
internus is the first of the thigh muscles involved. Undoubtedly they 
emphasized the earlier and greater implication of the muscles at the 
distal ends of the limbs as the most characteristic feature of the disease. 
Cases absolutely typical in other respects have existed with grave con- 
tractures of the tendons of the feet. 
The implication of the distal portions of the limbs, with complete or 
nearly complete integrity of the proximal parts, is the essential feature 
of the disease. So soon as we permit cases with intense atrophy of the 
proximal parts of the limbs, and especially with atrophy of the muscles 
of the trunk, to be classified under this designation, the diagnosis is prob- 
ably incorrect. Typical cases with necropsy reported in the literature 
are those of Marinesco, Sainton, and Dejerine and Armand-Delille. 
The cases of Dubreuilh and Siemerling are questionable. 
The tendon reflexes are usually lost, but at least one case, although 
without necropsy, is reported in which they were exaggerated (Dercum). 
This is so extraordinary, and so in contradiction to what we know of the 
pathology of the affection, that when it occurs we must think of a com- 
plicating lesion, such as pronounced degeneration of the lateral columns. 
Although the atrophy begins so frequently in the distal parts of the 
lower limbs that the type has been designated by Tooth as the peroneal, 
it may begin in the upper limbs, but its commencement is always in the 
peripheral parts of the limbs, and the progress of the disease is slow. A 
few cases have been observed in which the atrophy began in the distal 
parts of the upper and lower limbs nearly at the same time. 
Contractures (talipes equino-varus) are not uncommon in the muscles 
controlling the movements of the feet, and often they impede the gait 
very greatly. The atrophy of the legs below the knees may be excessive, 
and when this stage has been reached the lower parts of the thighs will 
usually be found comparatively less well developed than the upper parts. 
All movements of the toes or of the foot at the ankle may be impossible, 
both because of the atrophy and because of the contractures. The hands 
may present the condition seen in progressive spinal muscular atrophy, 
with wasting of the interosseous spaces and contracture, the so-called 
griffon's claw. The lower part of the forearms may be intensely atro- 
phied, but the upper part is usually in a more nearly normal condition. 
Fibrillary contractions occur in the wasting muscles, just as they occur 
in all muscular atrophy when the cells of the anterior horns are diseased. DISEASES OF THE MOTOR TRACTS 
111 
Vasomotor disturbances are common. The wasted limbs feel colder to 
the touch and appear more or less cyanotic. Sensation may be disturbed 
in the peripheral parts of the limbs, or pain may be felt, but, as a rule, 
the sensory symptoms are not nearly so pronounced as the motor. 
The electrical reactions are often altered, and this alteration may vary 
from diminution in the intensity of the electrical response to reaction of 
degeneration or even complete loss of the electrical response. Diminution 
in the faradic response of nerves in regions where muscular atrophy has 
not been as yet detected has been emphasized especially by Hoffmann. 
Mental symptoms, implication of the sphincters, pupillary phenomena, 
are not part of the symptom complex. 
The disease is of slow development, and after reaching its height the 
patient may live many years without additional symptoms. Thus, in a 
typical case reported by the writer, the symptoms are said to have been 
present about forty-five years, and the patient's condition has not changed 
during the many years he has been under observation. There is not 
likely to be any tendency toward improvement. 
The ocular manifestations have been studied by W. Krauss. They 
are not frequent, or at least little has been described concerning them. 
Krauss has found pupillary changes mentioned four times, which, how- 
ever, are inaccurate, and in 3 or 5 cases atrophy of the optic nerve. He 
concludes that the only reliable ocular manifestation is optic atrophy. 
Diagnosis.-When the symptom complex is typical the diagnosis is 
easy. The hereditary or family tendency, the youthful age at which the 
symptoms commence, the slow progress, the limitation of the atrophy 
to the peripheral parts of the limbs, the loss of tendon reflexes, the motor 
disturbances much greater than the sensory-all these make a definite 
clinical picture. The diagnosis will not be difficult if we refrain from 
including cases with atypical features. 
Progressive muscular dystrophy is most liable to cause mistakes, and a 
case with necropsy reported by the writer shows the difficulty in diag- 
nosing between these two diseases. The atrophy began in the lower 
parts of the lower limbs, and was associated with slight reaction of 
degeneration in these parts and talipes equino-varus on each side. These 
and the symmetry of the atrophy suggested the neurotic form, but the 
extension of the atrophy to the proximal parts of the limbs and the 
muscles of the trunk was unlike the typical form of the neurotic muscular 
atrophy. The microscopic examination showed that the nervous system 
was normal, but the muscles were intensely degenerated. 
Anterior poliomyelitis with paralysis confined to the distal parts of 
the lower limbs may occur and cause some confusion, but when a history 
of the onset is obtainable the diagnosis is easy. 
Progressive spinal muscular atrophy or chronic poliomyelitis may 
resemble the Charcot-Marie-Tooth form, but these diseases are neither 
hereditary nor familial, do not occur at so early an age, nor does the 
atrophy remain confined to the distal ends of the limbs. 
Multiple neuritis usually does not implicate the peripheral portions 
of the limbs, and the sensory disturbances are pronounced. Motor 
neuritis, with little or no sensory involvement, does occur, and the 112 
DISEASES DE THE NERVOUS SYSTEM 
Charcot-Marie type is regarded by some as a motor neuritis in which 
the peripheral ends of the longest nerves are affected; there is much to 
be said in favor of this view, although the spinal cord is also implicated. 
INTERSTITIAL HYPERTROPHIC PROGRESSIVE NEURITIS OF 
CHILDHOOD. 
This disease was first observed by I )ejerine and Sottas, and was reported 
by them in 1893. They had two cases occurring in brother and sister, 
and necropsy was obtained in both. They referred to a case of Gombault 
and Mallet as belonging to this type, and this case was the first published, 
although it was considered by the authors as a form of tabes. Syphilis 
may have some causal relation; at least, in the second case of Dejerine's 
a history of syphilic infection at the age of twenty-four years was obtained. 
Alcoholism and poor mental development were present in this second case. 
Marinesco regarded the disease as a variety of the neurotic muscular 
atrophy, and this view Brasch takes in the report of two clinical cases, 
although it has been most vigorously combated by Dejerine, who mentions 
that in the Charcot-Marie-Tooth form incoordination of movements, 
marked disturbance of sensation, Argyll-Robertson pupils, kyphoscoliosis, 
and hypertrophy of nerves do not occur, no matter how long the affection 
may have lasted. The duration of the affection has nothing to do with 
the hypertrophy of the nerves, according to Dejerine. 
Marie presented at the Neurological Society of Paris, in 1906, two cases 
occurring in a family of seven children, all similarly affected. He regarded 
the disease as a special form of the interstitial neuritis, although his 
cases differed in certain respects from the cases of Dejerine and Sottas. 
Dejerine accepts Marie's cases. They presented the same deformity 
of the feet, kyphoscoliosis, loss of reflexes, diminution of sensation, 
disturbance of gait and station, muscular atrophy, and considerable 
hypertrophy of the nerve trunks. Marie believed the muscular atrophy 
was different in his cases, inasmuch as it affected only the muscles of the 
feet and legs, while in the cases of Dejerine the atrophy was general. 
Marie did not find the Argyll-Robertson sign, but the light reflex was 
diminished; he also noted absence 6f disturbance of the sphincters and 
of the sexual functions. 
Pathology.-The lesions of the spinal cord are only such as result from 
degeneration of the posterior roots, and are like those of tabes. The 
lesions of the nerves are very characteristic, and are parenchymatous and 
interstitial. The nerve fibres may be altered to such a degree as to leave 
only empty neurilemma sheaths, but only a certain number are so 
intensely degenerated. The connective tissue is greatly thickened, and 
the alteration begins in the periphery and extends to the nerve trunks 
and nerve roots. The lesions are more pronounced in the nerves and 
roots of the lower limbs than in those of the trunk and upper limbs, and 
still more so than in the bulbar nerves; but only the optic and olfactory 
nerves escape. Such hypertrophy occurs in no other form of neuritis. 
Symptoms.-These may be said to be ataxia of the four limbs with 
muscular atrophy, marked disturbance of sensation with retardation DISEASES OF THE MOTOR TRACTS 
113 
of sensation, fulgurant pains, nystagmus, myosis, inequality of the 
pupils, Argyll-Robertson's sign, kyphoscoliosis, marked hypertrophy and 
hardness of all nerve trunks accessible to palpation, i. e., the symptoms 
of tabes associated with general muscular atrophy, kyphoscoliosis, and 
hypertrophy of nerves. 
Muscular atrophy may vary in different cases; in some it may be very 
intense, and predominate in the distal parts of the limbs, diminishing 
toward the trunk. The muscles of the face may be paralyzed and 
atrophied, and the facies may resemble that of myopathy, with the 
prominent lips and protrusion of the upper lip, the lips of the ant-eater 
(tapir), with the transverse laugh (rire en travers), so that the corners of 
the mouth are not drawn upward, and with difficulty to pout the lips or 
whistle. The distribution of the upper branch of the facial so far has 
escaped. The feet are always in the varus position, the nails are 
deformed, the first phalanx of the toes is dorsally flexed, and the second 
and third are plantary flexed upon the first, forming a right angle with it. 
The disturbance of function in the lower limbs is the result of atrophy 
and ataxia. The gait is steppage, but not like that resulting from 
paralysis of the extensors of the toes and of the anterior tibial muscle. 
The steppage is abrupt, the legs are raised and thrown outward, the feet 
falling quickly upon the ground. A cane is necessary in walking, and the 
head is held forward, and the steps are carefully watched. Turning 
around is done slowly, and with caution. Ataxia is pronounced when 
the feet are together and is pronounced in the upper limbs. The muscles 
of the larynx may be paralyzed, as in tabes, but, unlike tabes, the 
sphincters and genital powers are always intact. 
The nerves may be double the normal size, and the hypertrophy is 
uniform without nodes of swelling. Pressure on these enlarged nerve 
trunks does not cause pain; on the contrary, the nerves are analgesic 
to pressure and electrical irritation. 
There can be no doubt that the interstitial hypertrophic neuritis of 
childhood resembles the neurotic muscular atrophy in certain of its 
symptoms, but in others it resembles tabes dorsalis; and yet it is impossible 
to regard it as an association of the two diseases in the same person. 
There is evidently a strong family tendency to the affection. 
The treatment is unsatisfactory. The pains may be relieved by anal- 
gesics, and the ataxia lessened by coordinated movements. The treat- 
ment is much like that of tabes. 
PROGRESSIVE BULBAR PALSY (GLOSSO LABIO LARYNGEAL 
PARALYSIS). 
Etiology.-No cause is known, but it is probably an abiotrophy. The 
motor cells of the medulla oblongata and pons have a vitality sufficient 
to enable the functions of life to be properly performed until about 
middle age, or later, and then they begin to degenerate and finally dis- 
appear. No reason can be given for the escape of the sensory nuclei 
in the same regions. Syphilis does not seem to play a role, although 
it is possible that lead may do so. Infectious diseases may cause acute 
vol. v-8 114 
DISEASES OF THE NERVOUS SYSTEM 
bulbar palsy by producing a bulbar encephalitis, but it is questionable 
whether they ever give origin to chronic bulbar palsy. Bulbar palsy 
may develop as a result of electricity. It is exceedingly rare in early 
life, but the occasional occurrence at that period and in several members 
of the same family can be explained only by a congenital tendency. 
Most authors state that males are more frequently affected. In the 
writer's experience females have been as often subjects of the disease as 
males. The patient is usually in or past middle life before the onset. 
Pathology.-The nuclei of the hypoglossal nerves are usually intensely 
altered, so much so that nerve cells in these nuclei may have disappeared; 
in other cases they are few in numbers and much atrophied, with loss 
of dendritic processes. The intramedullary fibres of the hypoglossal 
nerves become atrophied and may also disappear. The alteration of the 
nucleus ambiguus may be very pronounced, but great care is requisite 
in forming an opinion concerning its condition. The posterior nuclei 
of the pneumogastric nerves in a case examined by the writer were so 
intensely pigmented that they appeared degenerated. The nuclei of 
the facial nerves and more rarely the motor nuclei of the trigeminal 
nerves are degenerated. The nerves whose function is interfered with 
are the hypoglossal, facial, pneumogastric, glossopharyngeal, vago- 
accessorial, and motor trigeminal. Hemorrhages or round-cell infiltra- 
tion are not found in true progressive bulbar palsy. The process is 
gradual, and atrophy and degeneration of the motor nuclei and nerves 
of the medulla oblongata and pons occur. 
Raymond believed that progressive bulbar palsy is almost always a 
part of amyotrophic lateral sclerosis, although it may occasionally exist 
alone. Leyden did not admit the latter as a possibility, and Dejerine 
mentions that in 1883 he demonstrated that the lesion is not confined to 
the bulbar nuclei, and that the pyramids are distinctly affected. The 
bulbar paralysis of Duchenne is a pyramidal amyotrophic sclerosis de- 
scending in type. With the exception of Reinhold's case (1890), which is 
not entirely satisfactory, no necropsy of a case of labio-glosso-laryngeal 
paralysis exists with integrity of the pyramidal tract. Remak's case 
(1892), quoted by some writers as one with integrity of these tracts, is 
not one of glosso-labio-laryngeal paralysis of Duchenne, as the superior 
facial branch was as much implicated as the lower, and the elevators of 
the eyelids were paralyzed. These symptoms belong to polioencephalitis, 
but not to Duchenne's bulbar palsy. The latter may occur in two 
forms, alone or in association with the symptoms of amyotrophic lateral 
sclerosis. The lesions of the bulbar nuclei are the same in either case, 
and the pyramidal tract is involved, as demonstrated by exaggeration 
of the tendon reflexes when the picture is typically that of Duchenne's 
bulbar palsy. 
Chronic poliomyelitis, it is said, never terminates by bulbar palsy. 
Notwithstanding this statement, reasoning by analogy would lead us 
to believe that uncomplicated bulbar palsy might occur. Dejerine 
himself has reported two cases in which the nerve cells of the anterior 
horns of the spinal cord were degenerated without implication of the 
motor tracts, and if this may occur in the spinal cord it is hard to under- DISEASES OF THE MOTOR TRACTS 
115 
stand why it may not occur in the medulla oblongata and pons. In 
several instances the writer has found bulbar palsy in association with 
amyotrophic lateral sclerosis, but never with integrity of the motor tracts. 
The muscles of the tongue are intensely atrophied, and if examined 
by the method of Marchi may show the presence of fat drops within the 
muscle fibres. 1 he muscles of the lips and larynx also may be atrophied. 
Symptoms-The clinical picture is most distressing. The patient has 
difficulty in speaking, progressing to loss of speech; dribbling of saliva, 
disturbance in swallowing, so that fluids return through the nose, and 
with much coughing, from the entrance of some of the fluid into the 
larynx. Ihe ability to pucker the lips, to whistle, or blow out a lighted 
match is lost, and with all this, intelligence is preserved, and the unfortu- 
nate individual has no dulling of 
mentality. The disease is re- 
markably symmetrical in its de- 
velopment. 
Speech. - Difficulty in pro- 
nouncing certain letters-d, g, k, 
1, r, s, t-is at first noticed; the 
words are slurred, and gradually 
speech becomes so indistinct 
that those who have known the 
patient many years and are 
familiar with his voice fail to 
understand what he says. Beevor 
observed one case in which the 
disturbance was observed in sing- 
ing before it was perceptible in 
conversation. The defect at 
first may be noticed only after 
the patient is a little fatigued 
in speaking. The voice becomes 
nasal because of paralysis of the 
soft palate, and when the larynx 
is paralyzed the defect of speech 
has reached its height. The 
tongue is atrophic, and may be so intensely affected that it cannot be 
protruded beyond the lips, is full of wrinkles, and shows an intense degree 
of fibrillary tremor. The reaction of degeneration may be obtained. 
The weakness of the tongue interferes with mastication of food, as the 
food cannot be controlled by the action of this organ, and pushed into 
the pharynx. The atrophy of the lips may be masked by the deposit of 
fat in the tissue. 
The muscles of mastication are less often affected, but may be weak, 
so that mastication of food becomes difficult. The atrophy and weakness 
in the distribution of the facial nerve is limited almost to the muscles of 
the mouth and chin, so that the upper part of the face is not involved, 
except in the infantile form of bulbar palsy. 
Paralysis of the- pharynx makes deglutition impossible when the 
Bulbar palsy. 116 
DISEASES OF THE NERVOUS SYSTEM 
paralysis is complete, but the weakness develops gradually. Liquids then 
not only may ascend into the nasal passages, but solids may pass into the 
larynx and thus cause bronchitis or aspiration pneumonia. The reflexes 
of the soft palate and pharynx are much impaired. The dribbling of 
saliva may be so great that patients are obliged to wear bibs. 
The upper part of the facial distribution escapes in progressive bulbar 
palsy, and this peculiar limitation of the paralysis has led to the conclusion 
by some neurologists that the centre for the upper distribution probably 
has a situation remote from that of the facial nucleus, and possibly is in 
the oculomotor nucleus. 
The tendon reflexes of the upper and lower limbs are usually exag- 
gerated, and there may be a pronounced jaw-jerk. Beevor described 
a lower jaw clonus. 
The disease is essentially motor in its manifestations, and sensory 
disorder or enfeeblement of the intellect does not occur. Intelligence 
is preserved until near death, but the patient is often emotional, and 
for a long time maintains a hopeful attitude. 
The heart may become affected, the pulse rapid, weak, and irregular, 
and the muscles of respiration impaired. When these signs develop, 
death cannot be delayed long. The end may be from inanition. • 
The gait is often peculiar. The steps may be short and slow, but 
this form of progression is more common in pseudo-bulbar palsy. 
In some instances ocular palsies have been associated with bulbar 
palsy, but they are to be regarded more as epiphenomena, and not as an 
essential part of the disease. 
Diagnosis.-The greatest difficulty may be in regard to pseudo-bulbar 
palsy, and yet in typical cases this is not great. The writer has seen 
pseudo-bulbar palsy, however, in which the only distinguishing features 
were the absence of atrophy, of fibrillary tremors, and of reaction of 
degeneration in the tongue. Pseudo-bulbar palsy is caused by multiple 
lesions in the cerebrum, implicating the nerve fibres innervating the 
motor nuclei of the medulla oblongata and pons. As the lesions are 
supranuclear, atrophy, fibrillary tremors and reaction of degeneration 
do not occur. 
Multiple neuritis confined to the cranial nerves may occur, but it is 
seldom that the typical picture of bulbar palsy will be presented; often 
nerves other than the cranial are affected, and sometimes sensory as well 
as motor nerves are implicated, and the development is usually more 
rapid in multiple neuritis. Following diphtheria, the soft palate may be 
paralyzed as well as the sphincters of the eyes and mouth,the frontalis may 
be weak, and the muscles of expression about the mouth may escape, 
although the tongue may be affected, and there may be difficulty of 
deglutition. 
Myasthenia Gravis Pseudo-paralytica.-The resemblance of this 
disease to progressive bulbar palsy may be very striking, but the rapid 
exhaustion after slight exertion, the myasthenic reaction to electricity, 
the implication of ocular nerves and of the limbs, the absence of atrophy, 
at least in most cases, make the diagnosis possible. DISEASES OF THE MOTOR TRACTS 
117 
Tumor, hemorrhage, softening, or encephalitis of the pons and medulla 
oblongata does not cause symmetrical lesions, does not develop in the 
same way, and implicates sensory fibres as well as motor. 
The bulbar type of muscular dystrophy, as described by Hoffmann, 
is so rare that it may almost be ignored. The presence of other signs 
of muscular dystrophy should make the diagnosis easy. 
Bulbar palsy may be a part of tabes dorsalis, but in all the cases except 
those of Charcot and Pfeifer the tabetic symptoms occurred first. The 
symptom complex differs from that of progressive bulbar palsy in the 
implication of sensory cranial nerves, so that the diagnosis of tabes 
associated with progressive bulbar palsy could hardly be maintained. 
Optic atrophy, disturbance of deglutition, reflex rigidity of the iris, 
ocular muscle palsies, and sensory disturbances in the distribution of the 
trigeminal nerve were present in all the cases; and weakness of the muscles 
of mastication in all the cases except one. Increased flow of saliva and 
facial paresis were observed in most of the cases, and only the distribution 
of the lowest branch of the facial was affected. Disturbances of coordi- 
nation in the muscles of the face, associated movements, and involuntary 
movements were present in some of the cases. Atrophy of the tongue 
was seen in only about half the cases, and was not complete in any case, 
this being unlike the condition in progressive bulbar palsy. 
A syphilitic infiltration of the medulla may give symptoms like those 
of true bulbar palsy. 
The bulbar palsy of childhood has been observed only a few times. In 
the two young brothers studied by Londe the symptoms began by bilateral 
paralysis of the upper branch of the facial nerve, with greater intensity 
of one side. Winking was infrequent, and the eyes could not be opened 
quickly. In one case slight ptosis was present. There was at first no 
paralysis of the muscles of the eyeballs. In the beginning of the disorder 
the facies resembled that of myopathy of the Landouzy-Dejerine type. 
It differed from the facies of labio-glosso-laryngeal paralysis, as in the 
latter the lower part of the face is affected, and in Londe's cases the 
upper part of the face at first alone was affected. Gradually in Londe's 
cases the double superior facial palsy extended, and the entire facial 
distribution became implicated as well as the oculomotor, the hypo- 
glossal, the spinal accessory, the trigeminal, and the pneumogastric 
nerves. The lips became inactive and flaccid, without being protruded. 
The face became an immobile mask, and the smile was impossible. 
Fibrillary tremors occurred in the chin. The tongue was paretic and 
atrophied. The muscles of mastication were affected in one case. Paraly- 
sis of the abductors of the larynx occurred in one of the brothers. 
Treatment.-Little can be done to arrest the development of progressive 
bulbar palsy, and nothing to stop it. The feeding with liquid or semi- 
liquid food is important, and tonics, especially strychnine, may be used. 
PROGRESSIVE MUSCULAR DYSTROPHY (PRIMARY MYOPATHY). 
Etiology.-Like in the other affections considered in this chapter, 
abiotrophy is the chief cause of this disease. Here, however, the muscu- 118 
DISEASES OF THE NERVOUS SYSTEM 
lar system instead of the central nervous system is affected, and its vitality 
may be so slight that wasting begins in some cases soon after birth. It 
is not improbable that some acute infectious disease may be the exciting 
cause, but the predisposition already exists. The resistance of the 
muscles varies in different cases, and, therefore, the time of commence- 
ment of the disease varies. Heredity and familial occurrence demonstrate 
clearly the congenital weakness of the tissues, and the mother seems 
to be more liable to transmit the disease than the father. There are many 
cases in which no heredity or familial tendency can be traced. A few 
cases have been observed in which the disease seemed to develop after 
typhoid fever (Friedlander, Josserand, Guillain). 
The disease first makes its appearance in early childhood, but cases 
are on record in which it began in middle life or even later. Erb believed 
that functional alteration of the nerve cells, undetectable by any means 
at our command, may cause the alteration of the muscle fibres, so that 
in this way muscular dystrophy may be of nervous origin. 
Convulsions, as well as diabetes insipidus, nystagmus, hemicrania, 
hysteria, chorea, and psychoses have been observed with muscular 
dystrophy. Such combinations, in Erb's opinion, are too frequent to 
be without influence on our judgment regarding the central origin of 
the dystrophy. Hypertrophied fibres also have been seen in poliomyelitis 
(Muller, Dejerine), and Hitzig and Kawka have found the same changes 
in the muscles in this disease as in muscular dystrophy. 
Direct heredity in muscular atrophy can no longer, according to 
Hoffmann, possess the diagnostic value which it was once supposed to 
have, although it must be conceded that it is more important in the 
myopathic and neurotic forms (Charcot-Marie-Tooth type) than in the 
myelopathic variety. At first the Leyden-Miibius type was regarded 
as the only form of muscular dystrophy hereditary in origin. Onuf 
has found six cases of epilepsy in which symptoms of muscular dystrophy 
were present. The congenital tendency was well shown in a family 
observed by Bunting, in which three male members were affected at 
the same age (five years). The boys of this family were susceptible, 
the girls escaped. It seems extraordinary that sex should influence the 
onset of this disease, especially at so early an age. 
Pathology.-The lesions are in most cases purely muscular, the atrophy 
may be so intense that no muscle fibres remain, in other cases the 
fibres are smaller in size, and yet the muscle spindles are intact, even in 
the most extreme atrophy of the surrounding muscle. It is probable 
that these spindles are organs of sensation and possibly are concerned 
with the sense of position. 
Landouzy and Dejerine believed muscular dystrophy to be a progressive 
atrophic myopathy resulting from a primary parenchymatous myositis 
with hypertrophy of certain muscle fibres, terminating in "simple" 
atrophy. The interstitial myositis is slight, and fatty infiltration may 
be found in some muscles. The vessels and intramuscular nerves, they 
stated, had never been found diseased. Some investigators have believed 
that the disease begins in the interstitial tissue of the muscles, others in 
the vessels, and still others in the muscle fibres. DISEASES OF THE MOTOR TRACTS 
119 
The findings are hypertrophy and atrophy of muscle fibres, proliferation 
of the nuclei, vacuolation, splitting of muscle fibres, hyperplasia of 
connective tissue and proliferation of its nuclei, thickening of the walls 
of the vessels and proliferation of their nuclei, accumulation of fat cells. 
Erb mentions that more or less important changes have been found 
in the anterior horns of the spinal cord, but that negative findings are 
the ride, the positive the exception. These 
cellular changes are important in the 
understanding of the pathology, as they 
demonstrate that the most typical clinical 
manifestations of progressive muscular 
dystrophy may be with important alter- 
ation of the nervous system, although we 
do not know why this should be; whether 
it depends on the type, duration, inten- 
sity, external causes or complications. 
The changes in the muscles are primary, 
those in the connective tissue secondary. 
The question as to whether the atrophy 
of muscular dystrophy differs from that 
of other processes has interested many, 
and the question arises whether the his- 
tological findings are sufficient to permit 
a diagnosis of the disease. Erb acknowl- 
edged that muscular findings similar to 
those of dystrophy might occur in other 
diseases, as they had been seen in spinal 
disorders, in myositis, near tumors 
situated in muscles, and in muscles in 
regeneration after injury. 
Symptoms.-The characteristic features 
are: The early age of commencement. 
The hereditary or familial appearance. 
The portions of the body affected, the 
atrophy beginning in the proximal portion 
of the limbs and trunk. Pseudo-hyper- 
trophy of certain parts of the body, 
especially of the calves. Quantitative 
diminution of electrical responses, de- 
pending on the degree of atrophy, but 
reaction of degeneration absent in most 
cases. Diminution or loss of tendon 
reflexes. Absence of fibrillary tremors. 
The atrophy almost invariably begins in the proximal parts of the body 
and the muscles of the trunk; in some instances the muscles of the shoulder 
girdle are affected first, in others those of the lower part of the trunk. 
The atrophy, however, is not always the first sign, for the muscles of 
one calf may enlarge in association with a little weakness of the leg, 
before any atrophy can be detected. In rare instances the atrophy 
begins in the distal portions of the limbs. 
Fig. 5 
Progressive muscular dystrophy. In- 
tense and general muscular wasting. 120 
DISEASES OF THE NERVOUS SYSTEM 
11 'cakness usua 1 ly precetles 
atrophy, and at first is con- 
fined to certain portions of the 
body; later the muscles of all 
the limbs as well as those of 
the trunk may be greatly 
wasted. Where two or mon* 
members of a family are af- 
fected the atrophy may show 
different types, not commenc- 
ing in corresponding parts in 
members of the same family, 
but occasionally the same type 
is preserved in a family. Dys- 
trophy of the muscles of the 
shoulder girdle in father and 
son was observed by Ogilvie 
and Easton. The shoulder 
girdle is frequently implicated, 
and the muscles affected are 
the pectorals, supraspinatus, 
infraspinatus, trapezius, rhom- 
boid, subscapularis, biceps, 
triceps, brachialis anticus, and 
supinator longus; and it is not 
until late that the muscles of 
the forearms and hands are likely to show wasting. In the lower limbs 
the muscles commonly affected are the gluteal, and the muscles of the 
anterior and posterior parts of the 
thighs. The muscles of the face and 
neck are only occasionally wasted, 
and when this occurs a distinct type 
is produced. Usually, in such cases, 
only the muscles of expression are in- 
volved, but the masseters, tongue, 
and ocular muscles have been found 
implicated a few times. 
In a patient observed by Marie 
closure of the eyelids was impaired, 
as in the infantile type, and marked 
double ptosis existed. The muscles 
of mastication were affected in the 
same case. Marie has found a 
few cases reported in which either 
ptosis or implication of the mus- 
cles of mastication occurred alone 
in muscular dystrophy, but no 
case in which both occurred to- 
gether. 
Fig. 6 
The method of rising from the floor as shown in pro- 
gressive muscular dystrophy. This is seldom seen so 
late in life as in the patient represented, inasmuch as the 
disease begins usually in childhood, and by middle life 
the disability has become too great. 
Fig. 7 
The transverse laugh (rire en travers) seen 
in facial type of progressive muscular dystrophy. DISEASES OF THE MOTOR TRACTS 
121 
The pseudo-hypertrophy attacks most frequently the muscles of the 
calves. This is caused by overgrowth of fibrous and fatty connective 
tissue, although here and there true hypertrophy of certain muscle 
fibres may be found in the midst of the atrophied muscles, but they do 
not cause any increase in the bulk of the muscle. In some cases the 
pseudo-hypertrophy may occur in the upper limbs in the triceps or 
deltoid muscles. The atrophy is usually seen in groups of muscles 
functionally associated. 
Reactions of degeneration should arouse suspicion that the atrophy 
may be both myopathic and myelopathic. Certain cases have been 
reported of the myopathic type in which it has occurred. 
Fibrillary tremors do not belong to progressive muscular dystrophy, 
but they have been observed in some cases. They are of much value in 
diagnosis when the nerve cells of the spinal cord and bulb are believed 
to be affected. When they occur in muscular dystrophy they are usually 
confined to one or a few muscles, and are usually of transitory duration. 
The condition of the tendon reflexes depends, in large measure, upon 
the degree of atrophy. Where the quadriceps femoris is much wasted 
the patellar reflex must be diminished or lost, but in some cases this 
reflex may disappear before any diminution in the size of the muscles 
can be detected. It is presumable that some change has begun in the 
muscle fibres, making them less capable of responding to stimulation. 
Peculiar attitudes may be caused by the wasting of certain groups of 
muscles. Where the shoulder girdle is attacked the condition of " winged 
scapula" and "loose shoulders" may be produced. If the patient be 
lifted by the axillae the shoulders are carried so far upward as the tissues 
permit. When the muscles of the back are wasted lordosis may be very 
pronounced, in rare cases it may be so extreme that the abdomen rests 
upon the thighs. More rarely lordosis is caused by weakness of the 
abdominal muscles. A condition occurring in muscular dystrophy, 
in which the muscles of the neck are affected, has been called by Ballet 
and Delherm "facies of the sphinx." It consists of an enlargement 
transversely of the base of the neck and flattening in the anteroposterior 
diameter. It is associated with atrophy of other muscles, and is said 
be common in muscular dystrophy. 
Contraction of the biceps tendon so that the forearm cannot be extended 
has been noted especially by Landouzy and Dejerine, but it is not of 
common occurrence, and probably depends on pronounced muscular 
atrophy. The flexor muscles on the back of the thighs are sometimes 
much contracted, and the legs are greatly flexed upon the thighs. Retrac- 
tion of muscles occurs early or late in the disease, especially in the biceps 
of the arm, flexors of the knee, and muscles of the calf. The cause is 
not very evident. Where contracture is the first sign of dystrophy it is 
probably not produced by retraction of fibrous tissue. 
The wasting of the gluteal muscles causes a peculiar elevation of first 
one hip then of the other in walking, the duck walk (marche de canard), 
as it has been called by the French writers. It is extremely characteristic 
of progressive muscular dystrophy. 
The weakness of the muscles of the back causes a peculiar disturbance 122 
DISEASES DE THE NERVOUS SYSTEM 
in rising from a sitting position on the floor. The patient turns so as to 
support himself upon his feet and hands; he then places one hand after 
the other upon his lower limbs, gradually by raising his hands pushing 
his trunk upward, and in this way climbs up on his lower limbs to the 
erect position. While not pathognomonic of progressive muscular 
dystrophy, it is seldom seen in any other affection. 
Landouzy and Dejerine believed that the muscles of respiration escape, 
and in their first case with necropsy the diaphragm was normal, but in a 
case studied by the writer it was intensely atrophied. I)uchenne observed 
clinically atrophy of the muscles of respiration, and it seems probable, 
from the study of Bunting, that the heart muscle may be affected. 
The belief in the escape of the muscles innervated from the bulb, 
except those affected in the infantile form, has been shattered by Hoff- 
mann. This investigator has shown that the disease may begin as 
myopathic bulbar paralysis, and remain as such for some time, and that 
external ophthalmoplegia may be a part of the symptom complex. 
Marie observed one case of progressive muscular dystrophy in which 
the orbicularis palpebrarum muscles were feeble, but the lids could be 
closed. Double ptosis was pronounced and the muscles of mastication 
were very weak. It is questionable if any group of muscles possesses 
more than a relative immunity. 
Mental symptoms are not pronounced, but the tendency to degen- 
erative change in the muscles seems to be associated with a form of 
abiotrophy of the brain; the child, while not imbecile, usually lacks the 
capacity for high mental development. 
Atrophy of bone has been observed in a few cases (Lloyd, Marie, 
Cruzon, Spiller, Schlippe). In a case reported by the writer the bones 
of the face as well as those of the limbs were atrophied. An x-ray exami- 
nation showed that the humerus and scapula on the left side were smaller 
than the corresponding bones on the right side. The left acromion 
process and glenoid cavity were unusually small, and the head of the 
humerus appeared as though it were dislocated forward. The humerus 
seemed to be deficient in lime salts. The ribs on the left side were larger 
than those on the right side. 
Scleroderma occurs with muscular dystrophy, and is regarded by 
Meige as not infrequently associated with it. 
Various types of progressive muscular dystrophy have been recognized: 
(1) The pseudo-hypertrophic; (2) the infantile (Duchenne); (3) the 
scapular, or juvenile (Erb); (4) the facio-scapulo-humeral (Landouzy- 
Dejerine); (5) the hereditary (Leyden-Mobius). 
The pseudo-hypertrophic is the form best known, as it was the earliest 
recognized (Duchenne), although the juvenile is of frequent occurrence. 
The implication of the face is extremely uncommon in America, and, 
indeed is seldom seen in other countries than France. This form affects 
especially the muscles of the calves, and is more common in males. 
One or both calves may be affected, and where only one is involved 
the implication of the other is usually only a question of time. Later, 
the enlargement may disappear when the atrophy has become intense. 
The facio-scapulo-humeral type includes the infantile type of Duchenne DISEASES OF THE MOTOR TRACTS 
123 
and the juvenile type of Erb. The muscles of the shoulder girdle are 
wasted, also those of the upper part of the upper limbs (deltoid, biceps, 
triceps, brachialis anticus), as well as the muscles of the neck and face. 
The eyelids cannot be closed, so that the appearance of bilateral facial 
paralysis is presented on superficial examination, the lips cannot be 
puckered as in whistling, and they are usually prominent because of 
pseudo-hypertrophy, the corners of the mouth are drawn outward in 
laughing instead of upward, and the muscles of the cheeks and those of 
mastication may be much wasted. The peculiar appearance of the 
face has received the name of facies myopathique. 
Infantile form is an unfortunate designation, as the atrophy may 
commence in adult life, or even at an advanced age. It is often associated 
with atrophy of the rest of the trunk and of the lower limbs, and some- 
times may follow the latter. This form has interested the French 
especially. Duchenne was the first to clearly separate this type clinically; 
he called it progressive muscular atrophy of childhood, and believed 
that the commencement in the facial muscles never occurred in the adult, 
lie regarded it as the same disease as the progressive spinal muscular 
atrophy of adults, and called it "atrophie musculaire progressive de 
1'enfance." 
The hereditary type (Leyden-Mobius) hardly deserves recognition as 
distinct from other forms. It begins in the lower limbs, as does the 
pseudo-hypertrophic, ascends from the leg and thigh muscles to those 
of the pelvic girdle and trunk, but is not associated with pseudo-hyper- 
trophy. It shows very pronouncedly the hereditary tendency. It is 
dystrophy on an hereditary basis, as are all the other forms. Only 
a few distinct cases of this type are on record, and Leyden himself 
reported only one; this, according to Erb, was merely the juvenile 
form, with predominance of the involvement of the lower limbs. The 
pseudo-hypertrophic form may, by disappearance of the fatty tissue, 
change into the Leyden-Mobius type. The latter has, therefore, merely 
historical interest, and can no longer be recognized. 
The type of Zimmerlin consists of atrophy of the proximal parts of the 
limbs and upper part of the trunk but may begin in the lower limbs and 
be associated with atrophy of the face. It begins usually about puberty 
or a little later, but the type is not sharply defined and is closely allied 
to Erb's juvenile form. 
Diagnosis.-The clinical appearance usually is so well defined that the 
diagnosis is not difficult. Babinski observed hypertrophy of a limb 
developing after typhoid fever, and this condition may resemble muscular 
dystrophy. Hypertrophy may occur also in syringomyelia. Lipomato- 
sis may be found in the limbs paralyzed in acute anterior poliomyelitis, 
and may then present the appearance of pseudo-hypertrophic muscular 
paralysis. Multiple neuritis may be confined to the thighs, as observed 
by Patrick. The subjective and objective sensory disturbances usually 
make the diagnosis easy, but in a later stage where the symptoms are 
chiefly or entirely motor, difficulty in diagnosis might arise. 
The resemblance to the Charcot-Marie-Tooth type has already been 
mentioned, and so soon as this type is enlarged to include cases in which 124 
DISEASES OF THE NERVOUS SYSTEM 
the atrophy is not confined to the distal portions of the limbs, or atypical 
signs such as implication of facial muscles occur, the diagnosis becomes 
questionable. 
Anterior i)oliomyelitis may resemble in its late stages progressive 
muscular dystrophy, especially if the abdominal muscles be paralyzed, 
but when a history of the onset can be obtained the diagnosis is easy. 
In rare instances myasthenia gravis may have some resemblance to 
progressive muscular dystrophy, but the diagnosis should be easy. 
The greatest difficulty may occur in distinguishing between the myelo- 
pathic and myopathic forms, and in certain cases the decision must be 
left in doubt, at least for a time. 
Disease of the spinal column or of the lumbar muscles may cause the 
patient to rise from the sitting posture on the floor much as does one 
afflicted with progressive muscular dystrophy. Congenital defect of 
muscle may cause difficulty but the defect is not progressive 
Prognosis.-Muscular dystrophy is very chronic in its development, 
and not likely to cause death unless vital muscles become implicated. 
The afflicted person may have diminished resistance to other diseases. 
Treatment.-Overexertion is to be avoided. A moderate degree of 
massage and electricity may be useful in the early stage when only a few 
muscles are wasted. Exercise should be recommended, as it is probable 
that muscle will not waste so rapidly if it be employed in such a manner 
that use does not become abuse. It seems doubtful whether we have 
any means of influencing the progress, but in certain cases massage, 
passive movements, and electricity seem to be of decided benefit. CHAPTER III. 
THE COMBINED SYSTEM DISEASES OF THE SPINAL CORD. 
COLIN K. RUSSEL, M.D. 
Combined disease of both afferent and efferent systems of fibres in the 
spinal cord is found in many conditions, although not all of these can be 
regarded as true system diseases. In meningomyelitis, myelitis, and mul- 
tiple sclerosis, the involvement of several of the spinal tracts may, with 
the subsequent ascending and descending degeneration, give the clinical 
and anatomical picture of a combined sclerosis. Such a condition has 
been described also in cases of arteriosclerosis and following injury with 
contusion of the spinal cord. In these cases we have rather a pseudo- 
systemic disease of the cord. Clinically, the fundamental character of 
this form is the spastic weakness of the lower extremities, associated in 
many cases with ataxia, some loss of sense of position in the extremities or 
other form of sensory loss. These conditions will be taken up more fully 
under their respective chapters. It will suffice to state here that ataxic 
paraplegia of Gowers and spastic paraplegia of Striimpell should be 
regarded as symptoms only, and not as definite clinical entities. In the 
majority of cases showing these symptoms in young or middle-aged indi- 
viduals, with the exception of the subacute conditions to be taken up later, 
they are merely the early evidence of disseminated sclerosis. 
In tabes dorsalis, while the disease is usually confined to the posterior 
columns, occasionally the direct cerebellar tract also shows degenerative 
changes following on the destruction of the cells in Clark's column (Katt- 
winkel, Oppenheim) without causing recognizable symptoms. Not infre- 
quently also, as was first observed by Erb in advanced cases of tabes, 
degeneration of the upper motor neurone may occur, giving rise to asso- 
ciated sclerosis of the crossed and direct pyramidal tracts-a true systemic 
combined sclerosis in some cases-while in others, and according to 
Crouzon1 more frequently, it is a pseudo-systemic sclerosis, due to involve- 
ment of the lateral columns by disease spreading in from the periphery of 
the cord from lesions of the lymphatic and circulatory systems. Clini- 
cally we have then the symptoms of a spastic paraplegia superimposed on 
the ordinary tabetic features. If, on the contrary, these myelitic lesions 
involve the lumbar enlargement and destroy the root zone of the posterior 
columns, we will have a flaccid paraplegia, and the only direct evidence of 
involvement of the pyramidal tract may be the presence of Babinski's 
extensor plantar response. 
In general paresis combined degeneration of the posterior lateral 
columns was first observed by Westphal, and has since been confirmed 
1 Paris Thesis, 1904. 
125 126 
DISEASES OF THE NERVOUS SYSTEM 
by many other observers. In the combined statistics of Tuczek, Furstner, 
Sibelius, and Crouzon, comprising 200 cases, combined sclerosis was 
present in 96 cases, that is, in 48 per cent. 
SUBACUTE COMBINED SCLEROSIS OF THE SPINAL CORD. 
Etiology.--Subacute combined sclerosis occurs usually in individuals 
in the fourth or fifth decade, although some cases have been reported as 
occurring in the third and a few in the sixth. Males are just as frequently 
affected as females, and there is some neuropathic hereditary tendency 
present in many cases. The writer has recently had under his observa- 
tion two cases of this condition associated with severe anemia in patients 
whose mothers were sisters and whose fathers were brothers. Other 
similar cases of the disease occurring in families have been reported. 
More important and more constant than this is the almost invariable 
evidence of some toxic condition, some wasting disease, prolonged sup- 
purative process, chronic digestive disturbances with diarrhoea, or acute 
or chronic infection. Syphilis apparently plays no important part as an 
etiological factor. The blood changes so commonly found in these cases 
are more often of the nature of a secondary anemia than of the pernicious 
variety, and are probably associated conditions rather than etiological 
factors, certainly in the majority of cases. 
Pathology.-The cerebrum, cerebellum, and pons in the great majority 
of cases show no lesions. Externally the spinal cord and membranes are 
normal except for an anemic appearance in some cases. Macroscopically, 
on section the grayish discoloration of sclerosis may be evident in the pos- 
terior columns, and sometimes in the region of the crossed pyramidal 
tract. In many cases this becomes evident only after hardening in 
Muller's fluid. Microscopically, with the Pal-Weigert method, the most 
extensive lesions are found in the mid-dorsal region of the cord. At this 
level almost the whole of the white matter, especially the peripheral parts, 
may be involved, leaving only the gray matter and a small area of white 
matter immediately surrounding it perfectly normal. Endogenous and 
exogeous fibres are alike affected. 
The posterior columns, which are usually most affected, may be com- 
pletely sclerosed except for a small area immediately on the inner border of 
the gray matter of the posterior horns. At higher levels of the cord the 
lesions diminish progressively and tend to be limited to the posterior 
columns, the direct cerebellar tracts, and the pyramidal tracts, both the 
direct and crossed. Especially in the upper dorsal and lower cervical seg- 
ments is the degeneration in the region of the direct pyramidal tracts 
marked. The degeneration in the pyramidal tracts gradually diminishes, 
and usually has disappeared at the medullary level, although occasionally 
it can be followed up into the pons by the Marchi method. The degener- 
ation of the direct cerebellar tract is followed by the same method into the 
inferior cerebellar peduncle, and that of the upper portion of the ventral 
cerebellar tract (Gowers) has been traced into the velum medullare 
anterius. 
Below the mid-dorsal region the diffuse destructive lesion diminishes PLATE III 
Subacute Combined Sclerosis. COMBINED SYSTEM DISEASES OF THE SPINAL CORD 
127 
progressively until in the lumbar region the degeneration is confined to the 
crossed pyramidal and posterior columns. Marchi's method reveals 
lesions similar to those already described, but somewhat more extensive. 
Lissauer's tract is not affected, even in the mid-dorsal region, where the 
periphery of the cord shows marked destructive changes. The cells of the 
gray matter may show degenerative changes (Putnam, Dana), but, as a 
rule, these are not at all marked. The nerve roots, especially the anterior 
ones, were more or less affected in Putnam's cases, but in only one of 
the cases, reported by Russell, Batten, and Collier,1 could degenerative 
changes be demonstrated in the peripheral nerves. 
The vessels in the cord, in the affected areas at least, may be engorged 
and their walls in some cases thickened, but no evidence of thrombosis has 
been observed. In other parts of the cord they may be quite normal. 
Minute hemorrhages may be present, associated with the anemia, but 
having no etiological relationship to the degenerative changes. Russell 
and his fellow observers are of the opinion that there are two distinct pro- 
cesses at wrork, first, a system degeneration, and secondly, a focal destruc- 
tive lesion. With regard to the latter, the first step is a swelling of the 
medullated sheaths, going on to fatty degeneration, with disappearance of 
the axis cylinders, absorption of the degenerative products, and later, 
overgrowth of fibrous tissue. There was in Dana's cases no evidence of 
any true inflammatory process. As to the former-the system degener- 
ation-the long tracts of the cord exhibit well-marked degeneration and 
sclerosis. Generally there is clinical evidence to show that the combined 
sclerosis exists for some time before the onset of the diffuse lesion. This 
latter always affects the lower cervical or dorsal part, and is usually a 
warning of an early exitus. 
Symptoms.-While Risien Russell has divided the course of the disease 
into three stages, further observations show that these are not always so 
evident. For the purpose of description, however, they will be retained. 
1. The period of mild spastic or ataxic paraplegia. The onset of the 
disease is in most cases slow and insidious, although in some instances 
quite acute, being ushered in by headache, vomiting, pyrexia, and malaise. 
In others it is the symptoms of anemia, palpitation, fainting attacks, and 
so on, that bring the patient under observation. The first symptoms com- 
plained of, attributable to the involvement of the nervous system, are 
subjective feelings of numbness and tingling in the lower extremities with 
perhaps some stiffness. These are never severe at first, but soon signs of 
slight spasticity and ataxia develop. The lower extremities are clumsy, 
feel stiff and cold, and the toes stick to the floor a little in walking. At 
this period there is no loss of control of the sphincters and no evidence 
of involvement of any other part of the nervous system. One finds on 
examination some impairment of the sense of position in the lower 
extremities when they are moved passively, with increased reflexes, and 
the extensor plantar response (Babinski). As a rule, later, though some- 
times coincidently, the same symptoms appear in the upper extremities. 
This period of ataxic paraplegia occupies half or three-quarters of the 
whole duration of the disease. 
1 Brain, 1900, xxiii, 39. 128 
DISEASES OF THE NERVOUS SYSTEM 
2. This period is one of complete spastic paraplegia, with considerable 
loss of sensibility in all its forms in the lower extremities and lower part of 
the trunk. The transition from the first to the second stage is, as a rule, 
abrupt; often in the course of twenty-four hours the patient loses the power 
of standing or walking, owing to the absolute loss of sense of position in the 
limbs, and not from any loss of motor power, which is usually not much 
impaired. Throughout this second stage the motor power diminishes 
gradually, as a rule, but is not entirely lost until the third stage is reached. 
The upper extremities are affected also with the loss of sense of position, 
and often show tabetic athetosis. The loss of cutaneous sensibility is at 
first peripheral in type, but as it extends upward becomes segmental in dis- 
tribution, and its upper limit on the trunk is usually well defined, it may 
extend as high as the area of distribution of the sixth cervical segment. 
Girdle sensation and lightning pains are not uncommon, and frequently 
the patient complains of a severe constant dragging pain beneath the lower 
costal margin. Herpes may occur, and Russell reports one case with 
cutaneous hemorrhages similar to those observed by Strauss in tabes. 
The mental condition remains unimpaired; and only occasionally is there 
any implication of the cranial nerves. There may be some loss of sphincter 
control; trophic disturbances are not present. The deep reflexes are all 
exaggerated, and there is extensor plantar response (Babinski). 
In some, though by no means in all cases, the symptoms of involvement 
of the nervous system are associated with the evidences of anemia. There 
is constantly some irregular pyrexia. The average duration of this stage 
of the disease is about five weeks, then in the course of a few days the third 
period follows. 
3. The third stage, that of flaccid paraplegia, is characterized by an 
absolute flaccid motor paralysis of the lower extremities. The trunk 
muscles also, with the onset of the third stage, show complete paralysis 
below a segmental level, and Beevor's sign (i. e., the riding up of the um- 
bilicus on the attempt to raise the head from the bed, due to the paralysis 
of the lower segments of the recti abdominis) can, as a rule, be observed in 
this stage. Subsequently the upper abdominal and spinal muscles and the 
lower intercostals are progressively attacked. There is absolute loss of 
sensibility, loss of the tendon-jerks, rapid muscular atrophy, and rapid loss 
of excitability of the muscles to faradic stimulation; oedema of the lower 
extremities and the lower part of the trunk with loss of sphincter control 
follows. 
With the onset of these signs there is an elevation of temperature, with 
general malaise, drowsiness, anorexia, and general asthenia. If anemia be 
already present, it is much increased. In the lower limbs and trunk, then, 
the wasting is general, but in the upper extremities it is of a segmental 
character, the intrinsic muscles of the hand being first affected, then the 
ulnar flexors, radial flexors, extensors of the wrist, extensors of the elbow, 
supinators, and fifth cervical group in that order. In some cases the dia- 
phragm may become paralyzed. Involvement of the cranial nerves is 
very exceptional. Mental disturbance is usually seen in this stage, and 
there may be convulsions. Trophic changes and bedsores are apt to occur 
in spite of the most careful treatment. The deep reflexes are lost with the COMBINED SYSTEM DISEASES OF THE SPINAL CORD 
129 
onset of flaccidity, while the extensor plantar response (Babinski) remains 
present. The duration of this period is about six weeks, though excep- 
tionally it may last several months, death being due either to sudden 
syncope or respiratory failure. 
One of the characteristic features of this disease is its progressiveness; 
neither remissions nor amelioration of the nervous symptoms ever occur, 
even though the general condition may improve somewhat. There is 
always some irregular fever not to be accounted for by associated condi- 
tions, such as cystitis, bedsores, etc. The duration of the disease varies 
from a few months to two or three years. The relation of the anemia to 
the cord lesion is not invariable; in some cases it precedes the appearances 
of the nervous symptoms, in others it may appear coincidently with the 
nervous symptoms and increase progressively with them; or it may be 
present only at the end of a prolonged third stage when emaciation 
becomes extreme, or there may be no anemia during the whole course. 
The essential nature of this process is evidently a primary nerve degener- 
ation. The same toxin that brings this about may cause more or less 
severe disturbance of the blood-forming elements. Its action on the 
nervous system affects first, as one would expect, those long tracts whose 
mere length must necessitate a more elaborate nutrition for their main- 
tenance. The circulatory conditions probably affect the preterminal 
diffuse lesion in the dorsal region in the same manner that acute trans- 
verse myelitis usually affects the dorsal region of the cord. 
Diagnosis.-In the first stage this disease must be differentiated from 
the early condition of disseminated sclerosis, that is, the ataxic paralytic 
stage. Of course, when the classical advanced picture is present with 
nystagmus, intention tremor, scanning speech, etc., no special art is 
required. In the early stages, however, the similarity may be striking. 
Disseminated sclerosis usually comes on at an earlier age than does sub- 
acute combined sclerosis. If anemia be present, it should be suggestive; 
and the subjective and objective sensory disturbances are much more 
marked and persistent in subacute combined than in disseminated sclerosis. 
The subacute progressive character of the one disease with its preterminal 
emaciation is of course quite different from the slow and prolonged course 
of the other. 
In the later stages, with the flaccid paraplegia, the condition may simu- 
late peripheral neuritis, myelitis, tumor of the cord, or tabes. The history 
of spastic gait, the involvement of the sphincters, the paralysis and loss 
of sensibility extending to the trunk and its nerve-root distribution, but 
especially the extensor plantar response, should be sufficient to make a 
diagnosis from multiple neuritis. Acute myelitis is of course excluded by 
the prolonged history. The absence of severe root pains and the distribu- 
tion of the physical signs in unequal degree in the lower and upper limbs 
should exclude a tumor in the spinal canal. 
The condition of flaccidity, anesthesia, loss of deep reflexes, and com- 
plete incontinence often may suggest tabes. The history of lightning pains 
in the second stage may make it more suggestive. The previous history 
of spasticity, changing rapidly to flaccidity, the absence of the Argyll- 
Robertson pupil, the great loss of motor power, the rapid muscular wast- 
vol. v-9 130 
DISEASES OF THE NERVOUS SYSTEM 
ing and the loss of faradic excitability and the extensor plantar response, 
should prevent error. 
Enough has been said in describing the symptoms and course of the dis- 
ease to make it evident that the prognosis is extremely bad. Any treat- 
ment, to be at all effectual, must be prophylactic. Attention to the 
general nutrition in all such debilitated and cachectic conditions is, of 
course, necessary. The patient should be at rest in bed in the early 
stages, to prevent as far as possible any exhaustion of neurones whose 
vitality and recuperative power are impaired by the presence of the hypo- 
thetical toxic matter in the circulation. Iron and arsenic are indicated 
to combat the anemia, and the usual dietary and hygienic measures. 
FRIEDREICH'S ATAXIA. MARIE'S HEREDITARY CEREBELLAR 
ATAXIA. 
Friedreich's ataxia is a disease, probably of abiotropic nature, charac- 
terized by a slow and progressive incoordination of the four limbs. The 
disturbance begins in the lower limbs, extends to the trunk and upper 
limbs, and finally involves the tongue, larynx, and eyes. The disease 
generally affects several members of the same family. It is named after 
the famous Heidelberg professor, who first described the disease.1 He 
looked upon it as an hereditary form of tabes dorsalis. One must remem- 
ber that Duchenne, of Boulogne, had recently described his progressive 
locomotor ataxia, so that sclerosis of the posterior columns of the cord and 
tabes were synonymous in the minds of most neuropathologists of that 
time. Schultz, of Bonn, first drew attention to the associated sclerosis of 
the lateral columns and posterior horns. In Rutimeyer's publication,2 
in 1883, the view was advanced that we have in this disease a primary 
systemic combined sclerosis developing on an hereditary foundation. 
In 1893 Pierre Marie3 gave the name of hereditary cerebellar ataxia to a 
syndrome characterized by ataxic gait and cerebellar incoordination. He 
took this stand on the study of sixteen cases of familial disease, published 
by Fraser, Nonne, Sanger Brown, Klippel, and Durante. The main differ- 
ences between this type and the type of Friedreich are, in the first place, 
a more definite hereditary influence in the former cases, although this is 
accounted for by the fact that the onset being much later, in fact, about 
the third decade, allows an opportunity for procreation, which is not pos- 
sible in the patient suffering from the usual Friedreich type, in which the 
onset is before or about puberty; and secondly, the occurrence of ocular 
disturbances, the retention and even exaggeration of the knee- and ankle- 
jerks, and the absence of those trophic disturbances, the pes cavus and 
curvature of the spine, so characteristic of the Friedreich type. Marie 
considered that the syndrome bearing his name and Friedreich's disease 
were possibly different types of one and the same affection, due to an 
initial heredo-dengeneracy of the nervous system. He was of the opinion 
that the lesion in the cases of his type was localized more definitely in the 
cerebellum itself. 
1 Virchows Archiv, 1863, xxvi, 391, 433; 1864, xxvii, 1; 1876, Ixviii, 145; 1878, Ixx. 
2 Ibid., 1883, xci, 106. 3 Semaine Medicate, 1893, xiii, 444. COMBINED SYSTEM DISEASES OF THE SPINAL CORD 
131 
The appearance of cases which clinically are intermediate between the 
ordinary Friedreich type and Marie's hereditary cerebellar ataxia, and 
moreover, the microscopic examination in Sanger Brown's and similar 
cases, which reveal no particular affection of the cerebellum, but a condi- 
tion very similar to that found in the ordinary spinal type of the disease, 
justify us in including Marie's type as a form of the same disease. In 
fact, no dividing line can be drawn between the two types either clinically 
or pathologically. 
Etiology.-The disease is evidently what Sir William Gowers describes 
as an abiotrophic condition, a congenital inherited and inherent lack of 
vitality in certain parts of the nervous system. Other stigmata of degener- 
ation are, as a rule, not lacking, and cases have been reported associated 
with infantilism, feminism, maldevelopment of the testicles, and so on. 
The congenital tendency is shown by the usual occurrence of the disease 
in several members of the same family, its onset at approximately the same 
age, and the similarity in the type of the developed disease in the dif- 
ferent members affected. A neuropathic diathesis is present in most cases, 
as is frequently shown by the occurrence of hysteria, migraine, epilepsy, or 
insanity in collaterals or ancestors. Alcoholism in the parents was first 
insisted upon as an etiological factor by Friedreich, his first six cases 
being members of two families the fathers of which were drunkards and 
the children conceived in drunkenness. Quincke, Rutimeyer, Althaus, 
Everett Smith, Destree, and others have related analogous examples. 
Hereditary lues and tuberculosis may be predisposing causes. Con- 
sanguinity in the parents has been reported in many of the cases. Any 
fall, injury, or infectious disease may appear to determine the onset, and 
any cause acting as a depressant to the vital forces produce a rapid 
advancement of the symptoms. The influence of sex is not noticeable, 
females being as frequently affected as males. 
Pathology.-In many cases of Friedreich's ataxia the cord has been 
found to be markedly smaller than normal. This atrophy affects chiefly 
the posterior columns. Anomalies of structure may be present; thus 
Friedreich found a supplementary central canal in three cases. The cere- 
bellum has been reported as smaller than normal in some cases. The 
cerebrum is not affected. There is occasionally some thickening of the 
membranes, particularly over the posterior aspect of the cord, sometimes, 
as in Mingazzini's case, more accentuated in the lumbar region. 
On section of the cord, microscopically there is found a posterolateral 
sclerosis affecting the columns of Goll and Burdach, the posterolateral 
or direct cerebellar tract, and in many cases the anterolateral or Gowers' 
tract. The crossed pyramidal tract is constantly affected in some degree 
at least, and in a few cases the direct pyramidal tract has also shown 
degenerative changes. In the gray matter, cellular lesions in the column 
of Clarke have been invariably found in association with the degeneration 
of the direct cerebellar tract. Associated with atrophy of the muscles of 
the extremities, atrophy of the anterior horn cells and thinning of the 
reticulum has been described by some observers. 
Lissauer's tract shows degeneration, which may be chiefly in the lumbar 
region. The posterior roots in most of the cases examined recently show a 132 
DISEASES OF THE NERVOUS SYSTEM 
Fig. 8 
variable amount of degenera- 
tion. The spinal ganglia are, 
as a rule, practically normal, 
although atrophic degeneration 
of the nervous elements with 
connective-tissue hyperplasia 
has been found by some ob- 
servers. The peripheral nerves 
show no constant change. The 
sclerosis of Goll's column, is, as 
a rule, complete, and can be 
followed up to its nucleus in the 
medulla, while that in Burdach's 
column is not so complete and 
varies in extent at different 
levels. In Mingazzini's case, 
and in those reported by^Du- 
Friedreich's ataxia. Lower medulla. 
Fig. 9 
Friedreich's ataxia. Cervical region. 
mon, Philippe, and Oberthiir, the 
lesions of the posterior roots seem 
to correspond more or less closely 
with the lesions in the posterior 
columns. 
There are always some intact 
fibres left along the inner border 
of the posterior horns and imme- 
diately posterior to the gray com- 
missure. Dejerine first called 
attention to the formation of what 
he termed "tour-billions," i. e., 
small twirls of neuroglial tissue, 
in sclerosed areas of the posterior 
columns. These probably have 
no particular significance, and 
Fig. 10 
Friedreich's ataxia. Lumbar region. COMBINED SYSTEM DISEASES OF THE SPINAL CORD 
133 
may be found in other long-standing sclerotic conditions in the cord. The 
sclerosis of the direct cerebellar tract follows its usual course, beginning 
in the lower dorsal region and gradually becoming attenuated toward the 
medulla. Gowers' tract shows an almost constant sclerosis, which accord- 
ing to Marie is invariably present if the disease be sufficiently advanced. 
The sclerosis of the crossed pyramidal tract is not as dense as in the pos- 
terior columns, and may be present only in the lumbar and lower dorsal 
regions, though in more advanced cases it may be visible even at the upper 
cervical level. 
Marie, in his type, termed hereditary cerebellar ataxia, basing his 
views on the findings in Fraser's and Nonne's cases, was of the opinion 
that the underlying pathological lesion here was an atrophic condition of 
the cerebellum. In Fraser's1 cases the cerebellum was reduced to less 
than half its normal weight, its cortex was little more than half the normal 
thickness, very few Purkinje cells remained in it, and these were atrophied 
and shrunken. There was no macroscopic degeneration in the spinal cord. 
In Nonne's2 case the cerebellum was abnormally small, but no further 
lesion was demonstrable. Later autopsies, however, performed by Meyer 
and by Barker on three cases of Sanger Brown's remarkable series, have 
not altogether confirmed these observations. Adolph Meyer3 found no 
circumscribed cerebellar lesion; Barker4 found no marked diminution in 
the size of the cerebellum, although it, with the cord, medulla, and pons 
looked small in proportion to the cerebrum, which itself was small. The 
microscopic study in both cases revealed a marked degeneration in the 
gray and white matter of the spinal cord, medulla oblongata, and cere- 
bellum. The degeneration involved chiefly the nerve cells and fibres of the 
centripetal paths, including the posterior columns, the dorsal nucleus of 
Clarke, the direct cerebellar tract with its continuation in the restiform 
body. There was practically no change in the cerebellum beyond a 
possible diminution in the number of cells in the nucleus dentatus and 
nucleus fastigii. 
Symptoms.-Following the order taken up in the preceding section, that 
type of the disease described by Friedreich will be first discusssed, then the 
so-called hereditary cerebellar ataxia of Marie, and finally, attention will be 
directed to the numerous intermediate forms between these clinical types. 
In the type of Friedreich, the disease commences at an early age, usually 
between four and ten, or at puberty; in exceptional cases the onset may be 
later. It is probable that in many cases the first symptoms pass unob- 
served until after some infectious fever or injury of some kind, when a 
slowly developing ataxia of the lower extremities is noticed. The child 
walks somewhat hesitatingly, with legs rather far apart, and attempts to 
balance himself with the arms; the feet are brought down in rather a 
stamping fashion, the paces are irregular, with some deviation in the line of 
march, the picture resembling somewhat a partially intoxicated person. 
The child is not steady on his feet, but falls more readily than other chil- 
1 Glasgow Medical Journal, 1880, vol. i. 
2 Arch. f. Psych., 1891, xxii, 283. 
3 Brain, 1897, xx, 276. 
4 University of Chicago, Decennial Publication, 1903, vol. x. 134 
DISEASES OF THE NERVOUS SYSTEM 
dren. In standing alone there is usually some unsteadiness, and this, as a 
rule, is not increased by closing of the eyes; so that, although Romberg's 
sign has been reported in some cases, it is not typical of the disease. 
Fig. 11 
Friedreich's ataxia, showing the spinal curvature. 
On further examination in this early stage the cranial nerves are not 
found affected; speech may in exceptional cases be somewhat slow and 
drawling quite early, but, as a rule, the defects in speech and nystagmus 
are observed later. There is no atrophy or loss of power to be made out 
Fig. 12 
Friedreich's ataxia, showing the typical deformity of the feet. 
in the muscles. Their tone is usually about normal; there is no impair- 
ment of the sense of position of the muscles, nor can any loss of any other 
form of sensibility be demonstrated. The knee-jerks in this early stage COMBINED SYSTEM DISEASES OF THE SPINAL CORD 
135 
are most commonly lost, but are often retained and may even be active. 
The extensor plantar response (Babinski) may or may not be present. 
There is very often, even at this early stage, some scoliosis of the spinal 
column, and there may be evidence of the beginning of the club foot so 
typical of the fully developed disease. The bladder functions are not 
disturbed, nor do they usually suffer. 
Gradually the disease progresses, and in the course of a few years the 
typical picture is developed. The ataxia affects the muscles of the upper 
extremities, trunk, and neck. At first it may be observed associated with 
voluntary movements only. If the child has already learned to write, it is 
noticed that his penmanship, instead of improving becomes awkward and 
clumsy. Articles are knocked over in the attempt to grasp them. The 
movements are associated with a swaying, sometimes almost choreiform, 
jerkiness, until finally the patient may not be able to convey a glass of 
water to his mouth. The ataxia is of the nature of a motor incoordination, 
due to the imperfect fusion of simple contractions, which compose volun- 
tary movements. The disturbance of speech and the nystagmus are of a 
similar nature. A condition of static ataxia also develops, caused by this 
same defect in the permanent, regular, harmonious innervation of those 
muscles and their antagonists, which are necessary for the maintenance of 
equilibrium and the upright position of the body. This may be first 
noticed in the muscles of the neck, causing a jerky, irregular, almost 
tremor-like movement of the head. Later, as the disease progresses and 
the trunk muscles become affected, the patient has difficulty in maintain- 
ing the sitting position without support, on account of the irregular move- 
ments of the trunk on the pelvis. Associated with the ataxia there may be 
a certain amount of muscular weakness, but this is not usual except in 
advanced cases, when the patient becomes confined to a chair or bed- 
ridden. In this stage and sometimes even earlier, atrophy of the muscles 
of the extremities may be present, associated with contractures and mal- 
position. Faradic excitability of the muscles may be lost and the reaction 
to galvanism diminished. The writer has observed this also in the mus- 
cles of the lower extremities where there was no apparent atrophy. 
The cerebral functions are commonly not impaired, although the general 
facial expression may suggest extreme stupidity; this is more often due to 
simple apathy and indolence. Impulsive laughter has been described in 
some cases. Excessive salivation has been known to occur. Speech 
becomes slower, hesitating, and jerky; articulation is imperfect, some 
syllables are prolonged, others forced out quickly in an explosive manner, 
as if the ataxia had affected the muscles of articulation. In a typical 
case the optic nerves are not affected, nor is the Argyll-Robertson pupil 
a symptom. Nystagmus, associated with lateral movements of the eyes, 
is not infrequent. Disturbances of respiration may be caused by the 
involvement of the abdominal muscles in the ataxia, and if the medullary 
centres be affected a definite dyspnoea results. 
The sensory system is, as a rule, unaffected. Lancinating pains were 
present in some few cases reported, but are not common. Cramp-like 
pains in the limbs are perhaps rather more frequently complained of. 
Only in exceptional cases is there any impairment of sensibility, and then 136 
DISEASES OF THE NERVOUS SYSTEM 
usually late on in the disease and of an indefinite incomplete type, being 
confined to the more distal parts of the extremities. There is rarely any 
loss of the sense of position of the muscles, although it has been observed in 
exceptional cases (Oppenheim). Visceral crises never occur. The tendon- 
jerks gradually diminish, disappearing first in the lower extremities. The 
presence of the extensor plantar response (Babinski), with the absence of 
the knee- and ankle-jerks, is typical. 
Vasomotor paresis occurs, so that in many cases the feet and ankles are 
congested, reddish blue in color, and clammy. Lateral curvature of the 
spine is almost always present, commencing early in the disease and often 
reaching an extreme grade. A peculiar deformity of the feet is commonly 
seen, namely, a condition of pes equinovarus, with hyperextension of the 
proximal phalanx and flexion of the distal phalanx of the great toe. In 
families affected with the disease this sign has in some cases been what they 
themselves looked for, knowing from hard experience what the ultimate 
outcome would be when it once made its appearance. 
In hereditary cerebellar ataxia the same familial character is present, 
but, as has been remarked above, the hereditary tendency is more evident 
on account of the disease commencing only in the third or fourth decade, 
and thus permitting the victims to marry and produce offspring. In the 
celebrated series reported by Sanger Brown,1 twenty-three members of one 
family, spread over four generations, were affected. Females are perhaps 
slightly more frequently affected than males; and Sanger Brown remarks 
that the disease was inherited more frequently through the girls than 
through the sons of the family. Occasionally the disease may skip one or 
even two generations, appearing perhaps in several members of the third. 
The disease commences with a slowly progressive ataxia, more or less 
marked in walking and standing; lancinating pains in the legs may be 
present. After some time the ataxia affects the upper extremities, and 
soon disturbances of speech are noticed, and the patient complains of diffi- 
culty of vision. Tendon-jerks are increased, instead of being diminished 
or lost, as we find in the Friedreich type, and occasionally other evidences 
of spasticity in the lower extremities are seen. The extensor plantar 
response is present. Only in a few cases has there been any disturbance 
of cutaneous sensibility. Frequently mental weakness develops in the 
course of the disease. Disturbance of deglutition and impairment of 
the control of the sphincters occur only very rarely. The disease is 
essentially progressive, but may present remissions. Some intercurrent 
disease, probably years after the onset, is the usual cause of death. 
The disturbance of function of the lower extremities is absolutely anal- 
ogous to what is observed in Friedreich's disease. The gait and station are 
similar to that already described, starting gradually, progressing steadily, 
increasing with fatigue. Frequently in the early stages the unsteady stag- 
gering gait leads to the accusation of inebriety. Closing of the eyes does 
not increase the unsteadiness in standing to any extent. The upper 
extremities are affected to a much less degree and later in the course of the 
disease. As in Friedreich's type, it is the more delicate movements that 
1 Brain, 1892, xv, 250. COMBINED SYSTEM DISEASES OF THE SPINAL CORD 
137 
are most interfered with in the early stages; later even the gross move- 
ments may become equally affected. Static ataxia also develops; there 
is no muscular weakness. 
The signs of spasticity of the lower extremities, on which the differen- 
tiation of this type from Friedreich's is based, to a great extent consist in 
increased knee-jerks and in some cases ankle clonus, contrasting with the 
absence of knee-jerks in the typical picture of Friedreich. The extensor 
plantar response (Babinski) is present. The tone of the muscles is 
increased, in some cases to such a degree that it can be overcome only 
with difficulty. 
Apart from the lancinating pains occasionally present in the earlier 
stages, there is seldom any disturbance of sensibility. The sense of posi- 
tion in the muscles was disturbed in Klippel and Durante's cases, but, as 
a general rule, it is unaffected. 
In the domain of the cranial nerves ptosis is sometimes present, nystag- 
mus is a common symptom, and frequently one finds paralysis of one or 
other of the external ocular muscles. The pupils often show a diminished 
or even an abolished reaction to light and occasionally disturbance of the 
reaction to accommodation. The visual field often shows some diminution 
in size, the color vision may be impaired. Primary optic atrophy is not 
uncommon. The disturbance of speech is similar to that described in 
Friedreich's type. A peculiar overaction of the muscles of the face, espe- 
cially marked on the attempt to speak, is frequently observed. Spinal 
curvature and club foot are not, as a rule, included among the symptoms 
of cerebellar ataxia; they do sometimes occur (Londe, Muira, Rossolimo). 
Numerous forms intermediate between these two types have been 
observed. Thus, for instance, Bonnus reported a case, otherwise conform- 
ing to the classical description of Friedreich's disease, in which the onset of 
symptoms began after the twenty-fifth year. In a brother and sister, 
reported by Baumlin, typical cases of the cerebellar ataxia type, with the 
ataxia, pains, convergent strabismus, optic atrophy, and increase of the 
patellar and other tendon-jerks, the onset in the boy during childhood, in 
a girl at the age of fifteen years. Similar cases to these have been reported 
by Rossolimo and Lennmalin. 
In typical cases of Friedreich's disease the knee-jerks are abolished. 
Many cases have been observed, however, in which in the early stages they 
have been preserved, and disappeared only later in the course presumably 
when the degeneration in the posterior fibres affected the part of the cord 
interested. 
The two types cannot be differentiated clinically by the presence or 
absence of signs of spasticity in the lower extremities. Erb has reported 
two sisters whom he regards as examples of Friedreich's disease in spite of 
normal knee-jerks. Hodge Jias described three cases of the same type, all 
presenting marked increase in the knee-jerks. Allen Starr found in one 
family two of the affected members with loss of knee-jerks, while in the 
youngest, with quite similar symptoms, the knee-jerks were rather in- 
creased. Brock and Tressider report similar instances in which the knee- 
jerks were present in some and absent in others of the affected members of 
a family, and it is by no means infrequent. Paralysis of the external 138 
DISEASES OF THE NERVOUS SYSTEM 
muscles of the eye in otherwise typical cases of Friedreich's disease has 
been observed by Gowers, Mendel, Omerod, Jaffroy, Anderson, Starr, and 
others. Optic atrophy was present in the case of two brothers reported by 
Cohn. 
It is evident that no fast dividing line can be drawn between the various 
forms of this disease. We have here an abiotrophic condition in which the 
localization of the degenerative process determines the clinical signs. 
Diagnosis.-In the early stages it is possible that this disease may be 
mistaken for chorea, especially if there happens to be only one member of 
the family affected. The family history, curvature of the spine, absent 
knee-jerks, with the extensor plantar response, should leave no doubt, and 
of course the progress of the disease renders such an error impossible for 
any length of time. 
Tabes dorsalis may be suggested by the ataxia and the loss of knee-jerks, 
but as a matter of fact there is little danger of such a mistake. The ataxia 
is quite different. In tabes it is due to a loss of the sense of position of the 
muscles; in Friedreich's it is entirely a motor incoordination, not increased 
to any extent in the dark or when the eyes are closed. The gait is quite 
distinctive; there is not the same overaction of all the movements in Fried- 
reich's ataxia. The difficulty is more in maintaining the equilibrium than 
in governing the action of the limbs, and the patient does not watch his 
feet so intently. The age of onset of the disease, the history of antecedent 
lues, the disturbance of the bladder functions, the lightning pains, girdle 
sensations, and paresthesias in tabes, with objective sensory disturbances 
and the Argyll-Robertson pupil, leave no doubt about the diagnosis. 
Scoliosis and the characteristic club foot form no part of the picture of 
tabes. The mental condition and absence of Argyll-Robertson pupil 
exclude juvenile general paresis. 
The differential diagnosis between disseminated sclerosis and those 
sporadic cases of Friedreich's ataxia is sometimes difficult, especially when 
the knee-jerks are not lost in the very early stages. The incidence of the 
disease in several members of a family gives the clue immediately, and 
spinal curvature, club foot, and cerebellar gait settle the question. Espe- 
cially in sporadic cases of the cerebellar ataxic type will the differential 
diagnosis be difficult. The disturbance of volitional movement, the nys- 
tagmus, the speech defect, the increased knee-jerk, and extensor plantar 
response will suggest disseminated sclerosis most strongly. The difficulty 
will be much increased if the patient be already in a bedridden condition 
when he first comes under observation. The muscular incoordination of 
the upper extremities with no sensory disturbance is similar in the two 
diseases; increased reflexes and extensor plantar response are common to 
both. In each there is disturbance of speech, nystagmus, ocular paralyses, 
and not infrequently optic atrophy. In the cerebellar ataxia, scoliosis 
and club foot are only exceptionally seen, and never in disseminated 
sclerosis. The gait, however, is distinctive. In sclerosis it is spastic, 
progression is direct enough, the paces regular but short, the legs stiff, 
and the toes, clinging as it were to the floor, are apt to catch on any little 
obstacle or unevenness. 
The steady progression in hereditary ataxia is quite different from the COMBINED SYSTEM DISEASES OF THE SPINAL CORD 
139 
intervals of remissions even with some improvement followed by exacer- 
bations, which one sees in the disseminated form. In the latter, dis- 
turbances of bladder functions are more common. Absence or slowness of 
the reaction of the pupil to light is more common in hereditary ataxia. 
The appearance of the movements, and the family character of the dis- 
ease in Huntington's hereditary chorea, may also give rise to some uncer- 
tainty. The presence of nystagmus, ocular palsies, optic atrophy, and 
especially the state of the reflexes, should remove all doubt. In hereditary 
chorea mental aberration is the rule sooner or later. 
Prognosis.-In either type the prognosis is bad. Its steadily progres- 
sive character has been sufficiently emphasized. Sooner or later the 
patient becomes bedridden, but he may live for twenty or thirty years, 
eventually being carried off by some intercurrent disease. 
Treatment.-Drugs have no effect on the disease process. Reeduca- 
tive exercises, as in tabes, may be of some benefit. More might be looked 
for from the prophylactic treatment of the younger and unaffected mem- 
bers of the family; proper hygienic measures and every care should be 
taken to improve and maintain their general health. 
COMBINED SCLEROSIS OF THE CORD CAUSED BY VEGETABLE 
POISONS. 
Lathyrism.-Lathyrism, according to Sheube, is a disease with a spastic 
spinal paralytic course, of the nature of an intoxication which is attribu- 
table to poisoning by various kinds of grass pea or Papilionaceae lathyrus. 
Etiology.-The disease is endemic in certain parts of France, British 
India, and Algiers. Only the poorer farm laborers are affected, as a rule, 
and the incidence is much increased in famine years, the poorer classes 
being forced to live on the cheapest sort of food, such as Lathyrus sativus, 
L. cicera, or L. clymenum. Men are more frequently affected than 
women. 
Symptoms.-The disease commences with general weakness and trem- 
bling of the legs, and, according to Brunelli,1 if the food be not changed, a 
condition of spasmodic tabes develops in the course of a month or two; the 
onset may in some cases be even more abrupt. It is characterized by 
spasticity of the lower extremities, bladder disturbance, paresthesia, and 
blunting of cutaneous sensibility. There is no muscular atrophy, and no 
involvement of the cranial nerves. The patient walks with the aid of a 
long two-handed staff in a typically spastic manner. 
Pigs, dogs, and rabbits are also occasionally affected, the disease show- 
ing itself by paralysis of the posterior extremities. Birds appear to be 
immune. 
The underlying pathological lesion is a posterolateral sclerosis, but the 
pathology is still obscure. 
Pellagra.-It is only necessary to mention here the combined sclerosis 
of the posterior columns and the crossed pyramidal tracts that one finds 
in advanced cases of Pellagra and the reader is referred to the proper 
chapter for the further consideration of this disease.2 
1 Trans. Seventh International Congress, London, vol. ii. 2 Volume II, p. 472. CHAPTER IV. 
SCLEROSES OF THE BRAIN 
EDWIN BRAMWELL, M.B., F.R.C.P. (Lond. and Edin.). 
Introduction.-An increase of the neuroglia and mesodermic connective 
tissue of the central nervous system occurs in a variety of pathological 
conditions. Proliferation of the neuroglia is met with, for example, in the 
systemic disorders, whether these be of abiotrophic, ischemic, or toxic 
origin. A similar neuroglial increase accompanies the systemic degener- 
ations consequent upon interruption of the nerve fibres in some part of 
their course. A local proliferation of the interstitial tissue may be 
observed in the neighborhood of inflammatory lesions and old foci of 
hemorrhage, while a cortical sclerosis is found postmortem in some cases of 
infantile hemiplegia as a sequel, in the opinion of Strumpell, to a primary 
acute encephalitis. The neuroglial proliferation in the instances men- 
tioned is either of compensatory origin, the tissue multiplying in order to 
occupy the place left vacant by the disappearance of the nerve elements, 
or secondary, in the sense that the change constitutes a later stage in the 
course of an inflammatory process. 
A group of affections remains for consideration-the scleroses proper of 
the nervous system-in which the neuroglial proliferation, if not a definite 
primary process, is at least the dominant feature. I )isseminated sclerosis, 
one of the commonest diseases of the nervous system, is by far the most 
important of these. Diffuse sclerosis, a very rare affection, belongs 
naturally to this group. Pseudo-sclerosis, another disease of great rarity, 
may also be included, since it closely resembles clinically the two last 
mentioned affections, while pathologically it is regarded by some authori- 
ties as an early stage of diffuse sclerosis. Miliary and tuberose scleroses 
are pathological entities of little clinical interest which call for brief notice. 
DISSEMINATED SCLEROSIS. 
Synonyms.-Multiple sclerosis; insular sclerosis; sclerose en plaques. 
Frequency.-Disseminated sclerosis is one of the commonest organic 
affections of the nervous system. Its frequency appears to vary in differ- 
ent countries. In Scotland it would appear to be commoner than in the 
United States (Byrom Bramwell, Jelliffe). E. W. Taylor (1906), who 
has collected 13 cases with autopsies from the American literature, is of 
opinion that even in the States it is a common disease, though far less fre- 
quent than tabes, and is probably often overlooked. Among 1000 con- 
secutive cases of nervous disease seen by Byrom BramWell in his private 
practice, there were 32 cases of disseminated sclerosis and 34 of tabes. 
Strumpell observes that in a mixed population, to judge from his Erlangen 
140 SCLEROSES OF THE BRAIN 
141 
and Breslau material, disseminated sclerosis is distinctly commoner than 
tabes. E. Muller, who analyzed 80 cases of disseminated sclerosis from 
Strumpell's Erlangen clinic, found that during the same period syringo- 
myelia had been diagnosed on 29 occasions. 
Etiology.-The causes are most obscure, and no factor common to a 
large proportion of cases has hitherto been demonstrated, although 
in individual instances indications of an etiological relationship either 
predisposing, exciting, or augmentative are discernible. 
Sex.-There is a surprising difference of opinion as to the incidence of 
sex, in part, at least, to be explained by the hospital accommodation at 
the disposal of various observers. Thus, among 34 cases observed by 
Charcot there were only 9 males. In Byrom Bramwell's series of 110 
cases, 67 were females. Bruns and Berlin record a preponderance in the 
female sex. On the other hand, Uhtoff, among 100 cases, met with 67 
males, while in Frankl-Hoch wart's series of 206 cases there were 140 males. 
Tredgold, in 1904, collected 373 recent cases from the literature; 200 of 
these were males as against 173 females. Thus it would appear that the 
sexes are almost equally liable. 
Age.-The first symptoms usually develop during early adult life. E. 
Muller found that in 44 per cent, of his cases the earliest signs appeared 
during the third decade. Byrom Bramwell met with 55 per cent, com- 
mencing during this period, while in 85 per cent, of his cases the disease 
first manifested itself between the fifteenth and thirty-fifth years. Oppen- 
heim has seen 3 cases confirmed by autopsy in which symptoms were first 
noticed between the ages of thirteen and fifteen. It is very unusual for 
the disease to begin after forty years of age. 
Instances have been reported in which some of the symptoms appeared 
to date back to infancy. Schupfer,1 who in 1902 collected 59 cases 
reported as commencing in childhood, considers that the diagnosis was 
often unwarranted. Muller, after discussing the data, came to the con- 
clusion that a true multiple sclerosis in childhood identical with that 
occurring in the adult has yet to be demonstrated. 
Infections.-Kahler and Pick indicated in 1879 that insular sclerosis 
often develops after an acute infective disease. Pierre Marie in his Dis- 
eases of the Spinal Cord (1892) strongly advocates this view. Of 25 cases 
which appeared to follow upon an infective malady, typhoid fever was in 
11 instances the preceding disease. The apparent onset of the first symp- 
toms after influenza, measles, scarlet fever, diphtheria, smallpox, cholera, 
etc., has been observed. To account for this, Marie supposes that its 
cause is " the result of one of those combined infections which are so fre- 
quent during the course of the different infective diseases." It cannot be 
denied that an infective malady sometimes appears to act as an augmen- 
tatory cause, yet it is extremely doubtful whether it alone is ever the essen- 
tial factor. The writer's experience has convinced him that in a consider- 
able proportion of these cases, careful inquiry demonstrates that un- 
doubted symptoms of the affection have been previously in existence. 
The prevalence of influenza during the last two decades has been regarded 
1 Monatschr. f. Psych, u. Neurol., Band xii, Heft 1 und 2. 142 
DISEASES OF THE NERVOUS SYSTEM 
as possibly accounting for the increase in the number of cases of dis- 
seminated sclerosis met with during the same period. Advance in diag- 
nosis offers an alternative explanation. Finally, although it is improb- 
able that the infective diseases actually determine the incidence of dis- 
seminated sclerosis, for, in the great majority of cases, there is no history 
of a preceding illness, it must be admitted that a very pronounced increase 
in the severity of the symptoms is a common sequel to these disorders. 
Syphilis plays no causative role in relation to disseminated sclerosis. 
Proof of this assertion is found in the rarity with which a history of syphilis 
or evidence of that disease is forthcoming (there was a history of syphilis 
in only 3 of the 206 cases analyzed by Frankl-Hoch wart), the fact that the 
sexes suffer almost equally, and the circumstance that among a popula- 
tion in which both syphilis and the parenchymatous syphilic affections 
are common there is no corresponding increase in the cases of dissemin- 
ated sclerosis. The writer's experience in this connection is of some 
interest. In the Out-patient Department of Leith Hospital, which 
draws its material from a seaport town of 80,000 inhabitants, during a 
period of four years he met with 62 cases of tabes as compared with 2 
cases of disseminated sclerosis. 
Trauma.-In 1901 Gumbrecht1 collected 54 cases in which it was claimed 
that trauma was of etiological importance. Grossmann2 has reviewed 
the literature. Von Leyden, Hoffmann, and other observers recorded 
cases in which they regarded injury as a factor of importance. E. Mendel 
held, and with this opinion the writer is inclined to agree, that 
exceptionally injury may act as an exciting cause in an individual who is 
predisposed. 
Metallic Poisons.-Oppenheim published observations which suggest 
that poisoning by lead and other metals may play a role. Among 28 cases 
in which the diagnosis was certain, and in which special inquiry was made 
into this point, in 11 a history of exposure to possible metallic poisoning 
by lead, copper, zinc, and other metals was obtained. The rarity of such 
a possible etiological relationship is generally admitted. 
Hereditary Predisposition.--Eichorst and Lenot have recorded cases of 
disseminated sclerosis occurring at an early age, in each instance the 
mother of the patient having suffered from that disease. In Eichorst's 
case, which was examined postmortem, the pathological findings are not 
altogether conclusive. In very rare instances, as in cases recorded by 
E. S. Reynolds, more than one member of a family has been affected. 
There is no undoubted instance on record, so far as the writer knows, 
in which a diagnosis of family disseminated sclerosis has been verified by 
autopsy. Some of the cases referred to have undoubtedly belonged to the 
category of the familial spastic paraplegias. 
Social Conditions.-The disease is as common, possibly more so, among 
the fairly well-to-do upper and middle classes as among the lower grades 
of society. City life does not seem to predispose to it; indeed, the fre- 
quency with which the symptoms occur in otherwise healthy young 
adults who have lived a country life is rather striking. 
1 Inaug. Diss., Leipzig. 
2 Deutsche med. Wochenschr., 1903, No. 41. SCLEROSES OF THE BRAIN 
143 
Emotion.-Mental shock, fright, worry, or grief is sometimes blamed by 
the patient as the cause of the symptoms, which certainly appear to be 
aggravated by psychical disturbances. 
The symptoms sometimes appear after fatigue or exposure to cold. 
Pathology.-Disseminated sclerosis is characterized anatomically by 
plaques or islets of disease scattered haphazard throughout the central 
nervous system. The cranial nerves are often involved, while degenera- 
tion of the spinal nerve roots has been observed. A classification into 
cerebral, spinal, and cerebrospinal types, although of clinical value, 
cannot in the pathological sense be strictly adhered to, since it seldom if 
ever happens that the patches are confined to the brain or spinal cord. 
The pia mater has been sometimes described as unduly adherent. A 
slight degree of atrophy of the cerebral convolutions may be occasionally 
observed. In one or two exceptional instances, as in a case examined 
by the writer, the unusually small size of the spinal cord attracted atten- 
tion. Glistening patches of variable size and shape and of grayish color 
are in some cases to be seen shining through the pia mater which covers the 
surface of the cord, and on section these are seen to correspond with 
diseased plaques situated in its substance. 
Upon section, islets of a gray or grayish-pink color are, as a rule, readily 
recognizable. It may be, however, that the diseased patches cannot be 
detected until the tissue has been placed in Muller's fluid or chrome alum. 
A slight degree of shrinking of the cord is sometimes observed in the 
neighborhood of patches of old standing, yet the contour of the transverse 
section is seldom modified in a striking manner thereby. The islets of 
disease are often firmer than the surrounding tissue; indeed, they may 
have almost the consistence of india-rubber. Sometimes the plaques 
are distinctly softer than the normal cord. 
The patches vary greatly in size and shape. In the brain they may be 
as large as a walnut; the majority, however, are very much smaller, and 
many are only recognizable on microscopic examination. The greater 
part of the transverse section of the spinal cord may be involved at one 
level, while a section a centimeter above or below may present an almost 
healthy appearance. A patch involving a large area may sometimes be 
traced through several segments. The patches may be wedge-shaped, 
rounded, oval, or quite irregular in contour; indeed, there is no character- 
istic shape. It is most unusual to find a patch of any size which does not 
extend to the periphery of the cord. The one situation in which the 
writer has found a notable exception to this rule is in the posterior 
columns. In two cases a large oval patch extended through several 
segments in this region. 
It has been affirmed that certain parts of the transverse section are more 
liable to suffer than others. Thus most observers hold, and with this con- 
clusion the writer is inclined to agree, that the white matter is especially 
implicated. E. W. Taylor,1 from a study of eight cases, concludes that 
both gray and white matter are irrespectively involved. Hibbert asserts 
that the gray matter often offers a barrier to the spread of the diseased 
1 Journal of Nervous and Mental Disease, June, 1906. 144 
DISEASES OF THE NERVOUS SYSTEM 
area. The writer's personal observations are in accord with those of 
Dejerine and E. W. Taylor, who failed to find evidence in support of 
this conclusion. An examination of seven cases lends no support to 
Obersteiner's statement that the lumbar region of the spinal cord is less 
commonly involved than the parts above this level. 
Bourneville and Guerard regarded the long tracts, Ziegler and E. Muller 
the posterior columns, as the regions of predilection. Obersteiner and 
others have noted a striking tendency to symmetrical affection of the 
spinal cord, medulla, and pons. This is, however, far from a constant 
feature. Williamson and Rossolimo have reported cases in which the dis- 
tribution of the patches corresponded to that of the bloodvessels. In not 
one of the seven cases examined by the writer was it possible to assert 
that this was so. 
In the brain the basal ganglia and the neighborhood of the ventricles, 
the corpus callosum, the pons (Strumpell), centrum ovale (Gowers), and 
the neighborhood of the olives are favored sites. For long it was sup- 
posed that the cerebral cortex was always spared. Oppenheim, E. W. 
Taylor, and Sander have shown that patches in the cortex are by no means 
uncommon. Islets of disease are comparatively rarely met with in the 
cerebellum. Weigert, however, has drawn attention to diffuse changes 
consisting in an increase of the tangential and radial fibres in this situation. 
For long it has been known that patches of sclerosis may occur in all the 
cranial nerves in some part of their course. The optic nerve and tract 
appear to be favorite situations. Changes in the anterior and posterior 
spinal nerve roots have been noted. On the other hand, it is doubtful 
whether the peripheral spinal nerves are ever implicated, although it 
must be admitted they appear to have been seldom systematically 
examined. 
Histology.-The two most striking features of the diseased areas are the 
disappearance of the myelin sheaths and the increase in the neuroglial 
tissue. The consistence of the patches varies with the degree of neuroglial 
proliferation. Tredgold1 divides the islets of disease into three varieties, 
hard, soft, and intermediate. Hard islets occur chiefly in the cord, and 
consist of a dense interlacement of thickened neuroglial fibres with very 
few glia cells. Nerve fibres are practically absent, although occasionally a 
naked axis cylinder is met with. There are no products of degeneration 
and but few bloodvessels. Soft islets were confined to the brain in Tred- 
gold's three cases, usually have a well-defined margin, often surrounded 
by a zone of leukocytes, and consist of a loose reticulum containing a semi- 
fluid material. They are generally devoid of nerve cells or fibres, while 
there is no neuroglial proliferation, and usually no degeneration products. 
The vessels are often much engorged. Intermediate islets contain nerve 
fibres in every stage of degeneration. Products of degeneration are 
abundant, while the neuroglia is somewhat thickened and has a finely 
granular structure. Glia cells are often plentiful, the vessels are dis- 
tended, and their lymphatics filled with leukocytes and fat-containing 
cells. Although the borders of the patches usually appear well defined to 
1 Rev. of Neural, and Psychiat., 1904. SCLEROSES OF THE BRAIN 
145 
the naked eye, under the microscope a gradual transition from diseased to 
healthy tissue is generally recognizable. 
The changes in the myelin sheaths are well seen in the intermediate 
islets, particularly toward their periphery. The first alteration appears to 
consist in swelling of the myelin substance. Later the myelin sheaths 
begin to disintegrate, undergoing a process of lecitholysis or fatty degen- 
eration. Finally, the myelin sheath disappears. 
The axis cylinders persist after their medullary sheaths have disap- 
peared. The unclothed axis cylinders do not, however, always present a 
healthy appearance. Some of them are swollen, many, on the other hand, 
are considerably reduced in diameter, and on longitudinal section are 
found to be fusiform in shape. Popoff, Erben, and Strahuber assert that 
these fine axis cylinders are newly formed, while Weigert and others 
believe that they are atrophic fibres. Bielschowsky traced the axis cyl- 
inder through a diseased patch to its continuation once more as a myelin- 
ated fibre. Kaplan demonstrated what he regards as a cement substance 
(axostrome) in the axis cylinder. Some naked axis cylinders retain their 
axostrome, others lose it. 
Secondary degenerations, although described (Babinski, Jolly, Ros- 
solimo), are undoubtedly uncommon, as the comparative integrity of the 
axis cylinders within the plaques might lead one to expect. Even when 
present they rarely extend more than a short distance from the plaques 
which produce them. The nerve cells of the spinal cord and posterior 
ganglia often show a degree of chromatolysis and an increase of the 
intracellular pigment, but these alterations are usually surprisingly slight. 
The neuroglia in the firmer patches consists of a dense felt-work of fibres 
with few nuclei. Recent patches show a more open network. 
Changes in the bloodvessels were first described by Rindfleisch. Rib- 
bert found a large vessel situated more or less in the centre of most of the 
patches in those cases he examined, and in two instances he found appear- 
ances suggesting with great probability an arterial thrombosis. Dejerine 
and Thomas point out that the vessels may be more numerous than normal 
in the sclerosed areas. Thickening of the vessel walls has beeen described 
by several observers, and narrowing of the lumen with, indeed, actual 
occlusion has been recorded. Hyaline degeneration of the wall has also 
been noted. In a number of cases which have been examined no patho- 
logical changes in the bloodvessels have been met with (Taylor, Schuler, 
Buchwald, Jolly, the writer), while the circumstance that these changes 
when present are limited to the patches, and may well be secondary to the 
neuroglial increase, must be kept in view. 
Rossolimo indicates that slight inflammatory changes are to be found 
in some cases in the neighborhood of the vessels. Thus the perivascular 
spaces are dilated and contain both mononuclear and polynuclear leuko- 
cytes in various stages of fatty degeneration. Borst, who also emphasized 
these changes in the perivascular lymph, holds that a lymph stasis may be 
a factor of importance. 
Pathogenesis.-This is most obscure. The problem involves the nature 
and sequence of the histological changes and their cause. In the first 
place, the author sides with E. Muller, who, following Ziegler and Schmaus, 146 
DISEASES OF THE NERVOUS SYSTEM 
speaks of a primary as opposed to a secondary disseminated sclerosis, 
thereby excluding such, cases as those described by Williamson, Marburg, 
and others, in which prominent changes in the bloodvessels and the dis- 
tribution of the diseased areas suggest their dependence upon interference 
with the vascular supply. It is obvious that the diseased process may 
begin either in the neuroglia or in the parenchyma, or that it may affect 
both tissues simultaneously to a greater or less degree. E. Muller is a 
strong supporter of the view that the initial change consists in a neuroglial 
proliferation, as a result of which the nerve elements are strangled. In 
favor of his opinion he states that the patches are especially prone to occur 
in regions where the neuroglia is normally most dense, and, further, that 
the density of the neuroglia in disseminated sclerosis is much in excess of 
that met with in any other affection of the nervous system. Again, he 
points out that toward the edge of a plaque increase in the neuroglia may 
sometimes be seen surrounding perfectly healthy nerve fibres. Even if it be 
granted that the neuroglial increase is greatest in the regions in which the 
neuroglia is normally most dense, this is not a weighty argument in favor 
of the primary affection of the neuroglia. In cases of sharply-defined 
secondary degeneration in the spinal cord, a neuroglial proliferation 
which must be secondary to the atrophy of the nerve fibres is sometimes 
observed beyond the limits of the degenerated tract (Mott and others). 
Very strong evidence against the primary neuroglial origin of the disease 
is afforded by the facts that in some patches very little neuroglial increase 
is to be observed, and that such islets may co-exist in the same case with 
others in which the neuroglial proliferation is excessive. Further, the 
rapidity with which symptoms develop and the manner in which they 
vary in intensity are difficult to explain on this hypothesis. 
The view, which is supported among others by Risien Russell,1 and Tred- 
gold, that the primary change is in the parenchyma, the neuroglial increase 
being a compensatory process, has much more to commend it in the 
opinion of the writer. The strongest argument in support of this view is 
the circumstance that very profound changes are sometimes found in the 
myelin sheaths, with little or no increase of the neuroglia in the same 
region. Toward the periphery of a patch, for instance, degenerative 
changes are sometimes found in nerve fibres which are lying in the midst of 
perfectly healthy neuroglia. 
A third body of observers takes up the position that both the myelin 
sheaths and the neuroglia suffer primarily as a result of a common cause. 
Erb, Gowers, Rossolimo, and others regard the process as a form of 
chronic myelitis. E. Muller and E. W. Taylor stoutly deny any evidence 
of inflammatory changes in the very considerable material at their dis- 
posal. Strumpell, who holds very similar views to Muller, regards the 
condition as a multiple gliosis predisposed to by a congenital factor, 
although determined, it may be, by a variety of exogenous causes. 
Ribbert, E. W. Taylor, Strahuber, T. Buzzard, and others believe that 
some toxin of undetermined nature circulating in the blood is the agent 
responsible for the histological changes. Alexander Bruce believed that 
1 Allbutt's System of Medicine, 1899, vii, 83. SCLEROSES OF THE BRAIN 
147 
the plaques were caused by a gradual infiltration of the tissues by the 
toxic lymph spreading from a central focus until it exhausts itself.1 
W. E. Bulloch2 has recently succeeded in producing paralysis of the 
limbs in rabbits injected subcutaneously with the cerebrospinal fluid 
from a case of disseminated sclerosis. On histological examination vas- 
cular engorgement and fragmentation of the myelin sheaths were observed 
and in the late stages degeneration throughout the cord. 
Symptoms.-As the multiplicity of the lesions and their widespread 
distribution would lead one to expect, the symptoms are numerous and 
varied. A classification into three clinical types-spinal, cerebral, and 
cerebrospinal-as suggested by Charcot, can only be accepted in a relative 
sense, for cases are seldom met with in which the symptoms are exclusively 
spinal, while still more rarely is a diagnosis made in the presence of purely 
cerebral manifestations. The circumstances, first, that the patches are 
scattered indiscriminately throughout the nervous system, and secondly, 
that in the spinal cord the motor fibres are gathered into small compass, 
readily explain the frequency with which paresis or paralysis of the lower 
limbs is observed in the early stages. Moreover, small patches scattered 
through the brain substance, unless they implicate special situations, may 
fail to afford indications of their presence. 
The characteristic features of the morbid process, viz., the preservation 
of the axis cylinders and ganglion cells, and the destruction of their 
medullary sheaths, may account for the special characters of certain 
symptoms, such as the peculiar form of optic atrophy, the absence of 
muscular wasting and the frequency of fleeting symptoms. 
Motor Symptoms.-Weakness in the lower extremities is almost always 
complained of, even in the earliest stages. The paresis may be so slight 
that its presence is only perceptible after prolonged exercise. Thus, undue 
fatigue in one or both lower extremities, after a long walk, is one of the 
commonest early symptoms. The loss of power in the limbs, which is 
often asymmetrical, may be so pronounced that it is only with difficulty 
that the patient can walk up stairs or across the room. In advanced cases 
the legs may be completely paralyzed. Temporary paralysis of one arm is 
by no means uncommon as an early symptom. A slight degree of weakness 
in the hands and arms is often present, yet persistent paralysis in the 
upper extremities is exceptional unless in the terminal, stages. Pro- 
nounced muscular atrophy is very rare. When this symptom is present 
the case may simulate amyotrophic lateral sclerosis. 
Muscular hypertonicity is usually associated with the paraplegic state. 
The spasticity is slight in some cases; occasionally it is so pronounced that 
this rather than the motor weakness is the chief cause of difficulty in loco- 
motion. Sudden flexor spasms often constitute a troublesome symptom, 
since they interfere with sleep. In the later stages of the disease flexor 
contractures tend to develop. 
A slight degree of ataxia is a common early symptom in the upper limbs. 
The uncertainty of the required movement is clearly to be distinguished 
from the intention tremor presently to be referred to. Ataxia in the lower 
1 Rev. of Neurol, and Psychiat., 1911, ix. 
2 Lancet, Oct. 25, 1913. 148 
DISEASES OF THE NERVOUS SYSTEM 
extremities may usually be demonstrated; indeed, in some cases this 
symptom constitutes the most striking feature of the picture, even in the 
earliest stages. The ataxia is, as a rule, of the cerebellar type, and hence 
is little increased when the eyes are closed. 
The gait is usually altered at a very early date, yet it is sometimes sur- 
prising to find no appreciable alteration even in the presence of certain 
evidence of pyramidal disease. A common type of progression is the 
spastic paretic gait, in which there is a combination of spasticity and 
weakness, one leg being often more affected than its fellow. The patient 
shuffles along, dragging his feet as if the toes were stuck to the ground. 
In addition to the spasticity there is often some unsteadiness in walking, 
a condition, in short, of ataxic paraplegia. Sometimes, indeed, inco- 
ordination is the conspicuous feature; the ataxia, however, differs notably 
from that of tabes, for it is rarely increased when the eyes are closed. The 
stiff way in which the patient holds his head and the rhythmic tremor in 
head and trunk met with in the classical type are almost pathognomonic. 
The gait is sometimes staggering and reeling, as in cerebellar disease. 
Another symptom, and one which is so characteristic that Charcot 
included it with nystagmus and speech defect as one of the cardinal signs 
of the disease, is the so-called intention tremor. So long as the patient is 
in a posture of complete muscular relaxation no tremor is to be observed; 
if he raises his hand with the object of carrying out some definite move- 
ment, the tremor commences. It is well brought out on attempts to write. 
Even when the patient is able to touch the point of the nose with the finger 
tip without any tremor, if the finger is kept in contact with the nose for a 
few seconds a rhythmical tremor of small amplitude may manifest itself. 
A test often employed in eliciting this symptom is to ask the patient 
to lift a glass of water to his mouth when the muscular effort required, 
together with the associated mental pertubation, commonly intensifies 
tremors. The rapidity of the tremor varies from four to six per second. 
The tremor may spread to other groups of muscles not directly called into 
action by the required movement. The intention tremor is usually most 
marked in the hands, but it is also seen in the lower extremities. It may 
be seen also in the head and neck during the act of walking. This consti- 
tutes no. exception to the statement that the tremor only occurs during the 
performance of a purposive act, for while the patient is sitting or walking 
the muscles of the neck require to be constantly in a state of contraction to 
maintain the poise of the head. Tremor of the face and jaw has occa- 
sionally been observed. Intention tremor, when present, is one of the 
most characteristic symptoms, yet it is often absent. 
Much discussion has taken place as to the origin of the tremor. Charcot 
was of opinion that it was due to an irregularity of conduction of the motor 
impulses through the sclerosed areas, a result of defective insulation of the 
axis cylinders or changes in them consequent upon disease of the medullary 
sheaths. Others hold that it is due to disease located in a particular situ- 
ation, and in support of their view, point out that intention tremor is 
absent in cases in which the disease is practically limited to the spinal cord. 
Sensory Symptoms.-The older writers emphasized the absence of sensory 
disturbances. This experience is quite contrary . that of more recent SCLEROSES OF THE BRAIN 
149 
observers, by whom subjective sensory symptoms, often of a fleeting 
character, it is true, are regarded as among the most characteristic of the 
early signs. Feelings of numbness, formication, deadness, tingling and 
pins and needles, particularly in one or both hands or feet, are very com- 
mon. A girdle sensation is of comparatively frequent occurrence, while 
a sensation of constriction around a limb is occasionally experienced. 
Shooting pains, especially in the lower limbs, are sometimes complained of. 
Giddiness, a common symptom, may occur early, and often it occurs in 
paroxysms. Headache, although seldom severe, is often present. Vomit- 
ing is an occasional symptom, and may occur in association with par- 
oxysms of headache or dizziness, or independently. A fixed pain in the 
back situated over the lower part of the sacrum is of frequent occurrence. 
Objective sensory defects of slight degree may be detected in most cases 
at one time or another if carefully looked for. Thus, Freund, who made a 
special study of 33 cases, found some definite alteration in no less than 29. 
All forms of sensation may be implicated. The hands and feet are most 
often involved, but hemianesthesia is not uncommon. The very slight 
degree of the objective sensory disturbances and their fleeting nature are 
very characteristic. 
Tendon-jerks and Skin Reflexes.-The knee-jerks are almost always ex- 
aggerated and the increase is often more marked on one side than the 
other. In some cases a patella clonus can be elicited. Absence of the 
knee-jerks has been recorded, but is undoubtedly very rare. The writer 
cannot recall ever having seen such a case. The activity of the tendo- 
Achilles jerk is almost always increased. Ankle clonus, usually bilateral, 
but sometimes confined to one side, is demonstrable in a considerable pro- 
portion of cases. The tendon reflexes in the upper extremities are, as a 
rule, abnormally active. 
The great value of Babinski's sign or the extensor plantar reflex as an 
indication of disease of the corticospinal descending tract has beeen amply 
confirmed. The collected experience of recent observers shows that in 
disseminated sclerosis, even at the earliest date at which a diagnosis is 
possible, the plantar reflex is almost always of the extensor type. More- 
over in the writer's experience Babinski's sign is usually present in its 
most typical form, the slow dorsiflexion of the great toe being easily 
elicited from the whole plantar surface. A crossed plantar reflex may 
sometimes be demonstrated (Byrom Bramwell). 
The abdominal reflexes have attracted considerable attention in this 
malady. Observations indicate (1) that their absence is specially char- 
acteristic of disseminated sclerosis, and (2) that they disappear early in the 
disease. Thus, Strumpell, who examined 185 persons free from nervous 
disease, noted their absence in 13.5 per cent, of these; while among 24 
cases of disseminated sclerosis they were absent in 67 per cent. E. 
Muller found that the reflex was only absent in 5 per cent, of individuals 
free from nervous disease, as compared with 62.5 per cent, in 47 cases of 
disseminated sclerosis. The latter writer remarks that although the 
abdominal reflexes fail in many organic affections of the nervous system, 
they never fail with so much regularity as in disseminated sclerosis. 
There is nothing of special importance to note with regard to the cremas- 
teric and other superficial reflexes in this disease. 150 
DISEASES OF THE NERVOUS SYSTEM 
Micturition and Defecation.-Disorders of micturition are ultimately 
present in all cases. Notwithstanding contrary statements, evidence of 
disturbance of the function of the bladder, often so slight as to cause little 
or no inconvenience to the patient, is usually forthcoming if carefully 
inquired for even before the more prominent symptoms are fully estab- 
lished. Thus in 80 per cent, of Oppenheim's cases there was some disorder 
of micturition. The writer obtained a history of some disturbance in 
30 out of 34 consecutive cases in which a special inquiry was made. "Pre- 
cipitate micturition," or a feeling of inability to retain the urine immedi- 
ately the desire to empty the bladder is felt, is the abnormality usually 
first experienced. Another indication of defective function which usually 
appears at a later date is the delay which precedes the commencement of 
the act. The patient may require to exert considerable pressure before the 
urine begins to flow. When the urine commences to flow it comes in a 
stream of full volume, although it may be defective in force. Retention 
of urine, a not uncommon symptom later, is a complication which must 
always be regarded with anxiety on account of the liability to cystitis. 
Incontinence is common in advanced cases, and is usually of the remittent 
type, but rarely depending upon complete paralysis of the bladder wall. 
Like so many of the symptoms of this disease, the bladder symptoms often 
vary from time to time. 
Constipation is the rule. Some loss of control is also often seen when 
any tendency to looseness of the bowels exists. True incontinence of feces 
is exceptional unless in the later stages. 
The sexual functions are at times interfered with. Impairment both of 
sexual desire and power has been described, and an exaggerated sexual 
desire has beeen noted in a few cases. 
Defects of Vision and Optic Atrophy.-Visual defects, which are frequent 
and characteristic, often appear at an early date. A temporary unilateral 
amblyopia of sudden onset and often regarded as hysterical in origin, since 
it is unaccompanied by changes in the fundus oculi, may indeed be the first 
indication of disease. Diplopia with no visible impairment of the ocular 
movements, another early symptom for which the patient seeks advice, 
was present in 13 of 31 cases recently examined by the writer. Although 
the visual defects are often fleeting, a permanent diminution in central 
vision, associated with atrophic changes in the optic disks, is often met 
with. Unlike the amblyopia of tabes, this defect of sight very rarely 
progresses to complete blindness. A central scotoma, usually bilateral, it 
may be for color alone, is often to be found if carefully looked for, and 
when present is a valuable aid in diagnosis. Concentric contraction of the 
field of vision is found in some cases. Uhtoff,1 who has studied the alter- 
ations of the visual fields in 150 cases, describes six groups of cases: (1) 
In one-half of all cases with anomalies of the visual fields, central scoto- 
mata, usually bilateral, often relative, with no alteration of the periphery 
of the field, are present. (2) In rare cases peripheral contraction of the 
field occurs together with scotomata. (3) In a considerable percentage of 
cases there is an irregular peripheral contraction of the field. (4) Isolated 
1 The Ophthalmoscope, 1905, p. 429. SCLEROSES OF THE BRAIN 
151 
cases present regular concentric contraction. This condition occurs in 
pure multiple sclerosis as well as in those cases which are complicated by 
hysteria. (5) In one case there was a ring scotoma, and in another (6) 
a central scotoma was present, but cleared up, and then a persistent 
contraction of the field appeared. 
Atrophic changes in the optic disks are of very great importance. 
There may be some pallor of the temporal side of the disk, or an incomplete 
pallor of the whole papilla, so that the inner part still retains a pinkish tint, 
or, when the process is more advanced, the whole papilla may be uniformly 
pale. As T. Buzzard1 pointed out, slight differences in the color of the 
papillae on opposite sides is often of the greatest value in determining 
whether the appearance is to be regarded as pathological. The frequency 
of recognizable pallor of the disk has been variously estimated. Uhtoff 
found that the papillae presented a pathological degree of pallor in 37 per 
cent, of all cases, and other observers have found an even higher per- 
centage. The dependence of the changes in the optic disks upon an islet 
of disease situated in the optic disks has been repeatedly demonstrated. 
A small number of cases have been observed in which there was a slight 
degree of optic neuritis, never amounting to a true papillitis. The appear- 
ances, which are those of a retrobulbar neuritis, have been proved to be due 
to the presence of a plaque in the immediate vicinity of the papilla. 
Ocular Muscles, Nystagmus, Pupils, etc.-The ocular muscles are particu- 
larly liable to be affected (Uhtoff, 20 per cent.). Diplopia has been men- 
tioned. Usually when this symptom is present the paresis is so slight in 
degree as to escape detection unless specially examined for by the appro- 
priate visual tests. The sixth nerve is most commonly affected. Paresis 
of the third is usually partial. These muscular defects are almost always 
of slight degree, but complete ophthalmoplegia has been recorded. 
Ptosis either confined to one side or bilateral is not infrequent. Wild- 
brand and Sanger lay stress on this symptom, believing that in certain 
cases it may be of even greater diagnostic importance than nystagmoid 
movements. Muller, who met with ptosis in 8 per cent, of his cases, also 
observed paresis of lateral conjugate movement in three and of upward 
movement in two cases. 
Nystagmus belongs to the symptomatic triad formulated by Charcot. 
Three degrees may be conveniently recognized: (1) Nystagmus which 
occurs while the eyes are in a position of rest; (2) nystagmus which is only 
present in association with a fixed purposive movement, such as lateral or 
vertical deviation or upon fixing a near object; and (3) movements which 
occur during fixation, but which rapidly cease and are at the same time 
frequently irregular both in time and amplitude. To this last group we 
would reserve the term nystagmoid movements. In few cases of dissemi- 
nated sclerosis do we find the type which we have classed in the first group. 
On the other hand, the second and third types are very common. The 
movements are usually fairly rapid and regular, but vary considerably in 
amplitude in different cases. Coarse nystagmus, for example, may be 
elicited when the patient looks to one side, while when he looks in the oppo- 
1 British Medical Journal, 1893. ii, 779. 152 
DISEASES OF THE NERVOUS SYSTEM 
site direction the movements may be much finer in quality. The nys- 
tagmus is usually most obvious when the patient looks to the extreme left 
or right and at the same time directs the eyes slightly upward; sometimes, 
however, when he looks directly upward or downward the movements are 
also visible. Occasionally the movements are only to be seen when the 
eyes are fixed at the extreme of the vertical plane. The direction of the 
oscillation is commonly from side to side, the two eyes acting in unison. 
A certain amount of rotation of the eyeball is by no means uncommon, 
and in rare cases the oscillations take place in a vertical direction. The 
writer has seen one case in which the movements were confined to one eye. 
Kunn has noted a "Zitterbewegung" in the ciliary muscle. The writer 
has also observed evidence of rhythmic contractions in this muscle on 
ophthalmoscopic examination. Alterations in the size and activity of the 
pupil are not uncommon. There was a difference in the size of the pupils 
in 24 per cent, of Uhtoff's cases. Parinaud remarks that inequality of the 
pupils occurs early in the disease, and that miosis when present is spastic 
as opposed to the paralytic miosis of tabes. 
The pupils react well on convergence provided that the movement of 
convergence is not itself defective. The light reflex, although sometimes 
sluggish, is very rarely lost. Muller notes that among 364 cases observed 
by Frankl-Hochwart, Probst and Uhtoff, the Argyll-Robertson pupil was 
only present in 4. Frankl-Hochwart met with hippus in 12 cases. 
Speech and Articulation.-In the older description of this disease speech 
affections occupy a prominent position. They are, however, by no means 
of constant occurrence. Byrom Bramwell found some defect of speech in 
62 per cent, of his series, while some alteration was present in two-fifths 
of Miiller's cases. Among 33 consecutive cases recently examined by the 
writer, in 15 (45 per cent.) speech was altered. 
It may be that the patient notices some slight difficulty in speech, espe- 
cially toward the end of a sentence, even before any change can be detected 
by the observer. The alteration in speech in the early stages of the disease 
may be so slight that it is difficult to describe, and yet it may be suffi- 
ciently characteristic to the experienced observer to help him greatly in 
making his diagnosis. The slow and measured way in which the patient 
enunciates and a singular monotony of voice are usually the salient feat- 
ures. When he reads aloud it is interesting to note the way in which he 
tries to vary the pitch of his voice, of the monotony of which he is ob- 
viously conscious. At a later date he chops his words, each syllable is 
pronounced slowly, hesitatingly, distinctly, and precisely. This is the 
syllabic, scanning, or staccato speech which has been compared by Charcot 
to that of incipient intoxication, by Oppenheim to that of a child learning 
to spell, and by Marie to that of an individual who is steadily but surely 
ascending a hill. Each syllable is produced with apparent effort, and 
this is accentuated by the associated tremor often present. Muller 
found a true staccato speech in only 15 per cent, of his cases. Certain 
letters, notably 1, p, g, b, and d, are especially apt to give rise to difficulty in 
pronunciation. Later in the course of the disease, defective articulation 
may render speech quite unintelligible. As Oppenheim observes, when an 
attempt is made to intone the letter e, a distinct tremor in the voice is often 153 
SCLEROSES OF THE BRAIN 
noticeable. This observer has drawn attention to the scanning character 
of the respiration, to which he is inclined to attribute the corresponding 
peculiarity in speech. Goldscheider believes that the monotonous char- 
acter of the speech may be due to defective control over the act of expira- 
tion, for he has shown that the augmentations and interruptions in the 
expiratory blast, which he has demonstrated take place during the act of 
speaking, are in this disease abnormally slow and defective. Aphonia is 
occasionally present. Rethi has given a detailed description of the' 
appearances in 44 collected cases in which the larynx was examined. In 
63 per cent, some form of laryngeal paralysis was present, while in half 
the cases tremor of the vocal cords was observed. 
Mental Symptoms.-It is somewhat surprising to find that profound 
psychic alterations are exceptional. Even in fully developed cases there 
may be no obvious mental disturbance. As a rule, however, after the 
disease has been in progress for any length of time, a certain degree of 
mental impairment is noticeable. Thus in Byrom Bramwell's series of 
cases defective memory was present in 50 per cent., some emotional alter- 
ation in 40 per cent., and further evidence of intellectual impairment in 27 
per cent., while E. Muller found a slight enfeeblement of the psychical 
faculties in about 25 per cent. The psychic alteration shows itself espe- 
cially in enfeeblement of memory, in a certain slowness of thought, in the 
apathy and indifference with which the patient regards his surroundings, 
and in a degree of stupidity which the facial expression would almost lead 
one to expect. A sense of well-being, or euphoria akin to the spes phthisica, 
is often observed, less commonly a depression of spirits which has been 
known to pass into actual melancholia. Emotional control is often 
impaired. The occurrence of outbreaks of emotion does not necessarily 
imply a corresponding defect in the intellectual capacity. It is interest- 
ing to note that several cases have been reported in which a pathological 
examination established the co-existence of general paralysis of the insane.1 
Trophic and Vasomotor Changes.-These are extremely rare and of corre- 
sponding minor significance. James Collier has reported five cases in 
which he noted the presence of erythromelalgia. There are a number 
of cases on record in which muscular atrophy was present, although a 
pronounced degree of wasting is altogether exceptional. Bedsores may 
develop in the terminal phases. 
Congestive and Epileptic Attacks.-Attacks similar in character to the 
congestive attacks of general paralysis of the insane have been recorded. 
Their rarity may be gathered from the fact that the writer, who has 
examined over 150 cases of this disease, can only recall one instance. 
Frankl-Hochwart met with apoplectic attacks in 10 of his series. The 
attacks have been sometimes preceded by giddiness, the patient falls to 
the ground, is sometimes convulsed, and may remain comatose for several 
hours. The temperature is raised. Hemiplegia is an occasional sequel. 
Sudden attacks of giddiness and faintness without loss of consciousness are 
less uncommon. Muller has met with two cases in which Jacksonian 
attacks occurred. Epileptic attacks were noted in 3 of Frankl-Hoch wart's 
206 cases. 
1 Hunt, Am. Jour. Med. Sci., 1903, cxxvi, 126. 154 
DISEASES OF THE NERVOUS SYSTEM 
Earliest Symptoms.-Motor weakness in the limbs, and especially in 
both lower extremities, paresthesias in the limbs, giddiness, tremor or 
ataxia, and amblyopia are, in the order stated, the most common early 
symptoms. The development of the initial symptoms as complained of 
by the patient has been studied by Byrom Bramwell (110 cases), who 
states: " In 41 cases the initial symptoms occurred in the extremities, and 
were purely motor or ataxic; in 3 cases the symptoms were purely sensory; 
in 22 cases the symptoms were both sensory and motor; in 24 cases the 
symptoms were purely cerebral or ocular; in 17 cases there were different 
combinations of motor and sensory symptoms with cerebral or ocular 
symptoms." Ashley W. Mackintosh, who has tabulated 80 cases, for the 
most part drawn from Ferrier's material, remarks on the extraordinary 
variability of the modes of onset, the comparatively large number of cases 
with acute or sudden onset (10 cases), the frequent occurrence of purely 
unilateral symptoms at the onset (15 cases), and the comparative fre- 
quency of the occurrence of sensory symptoms at the onset, either alone 
(10 cases) or combined with other symptoms (19 cases). 
Types.-A number of clinical types are recognizable according to the 
preponderance of certain symptoms or different modes of onset. 
1. The classical type, characterized by intention tremor, nystagmus, 
speech affection, and it may be other cerebral manifestations in association 
with a spastic or ataxic paraplegia. 
2. The cerebrospinal type, in which even in the absence of the symp- 
tomatic triad of Charcot, symptoms alike cerebral and spinal point to 
widespread disease of the central nervous system. 
3. The hysterical or intermittent type, in which fleeting symptoms sug- 
gestive of hysteria constitute the most striking feature of the case. 
4. The spinal type, characterized by prominent spinal symptoms re- 
sembling a variety of spinal diseases. Of these, a progressive paraplegia 
unaccompanied by sensory disturbance and indistinguishable at first, it 
may be, from primary lateral sclerosis is very common. Again, a spastic 
paraplegia may develop with the rapidity of an acute myelitis. The 
co-existence of ataxia and paraplegia may constitute a symptom complex 
which closely resembles the combined systemic disease. 
5. The cerebral type is much less common. Occasionally vomiting, 
giddiness, and headache are the most striking symptoms. In rare cases, a 
cerebral hemiplegia is met with as the leading feature of the case. Very 
exceptionally, bulbar manifestations or mental symptoms are the most 
conspicuous alterations. 
6. The optic or ocular type in which some disturbance of vision or 
ocular palsy constitutes the prominent symptom. 
7. The sacral type, recently described by Oppenheim and Kurt Mendel, 
in which pains in the legs, disturbance of the sphincters and sexual func- 
tions, point to a lesion in the lower part of the spinal cord. 
Diagnosis.-This is not as a rule so difficult as the irregular distribution 
of the pathological lesions and the multiplicity of types might lead one 
to expect. At the same time there are few diseases which present such 
puzzling diagnostic problems. The diagnosis is based upon (1) the detec- 
tion of certain characteristic symptoms or association of symptoms, upon 155 
SCLEROSES OF THE BRAIN 
which alone a positive opinion may sometimes be advanced; (2) a history 
of variation in the severity of the symptoms, which, when present, is in 
itself so suggestive as to be almost pathognomonic; and (3) the exclusion of 
a variety of diseases, which may closely simulate disseminated sclerosis. 
The classical type presents features which are distinctive, for intention 
tremor, scanning speech, and nystagmus in association with a spastic or 
ataxic paraplegia and an extensor response place the problem beyond all 
doubt. These cases, however, form only a small percentage. 
It is especially in the early stages that errors are made, notably when 
temporary manifestations disappear, leaving the patient in perfect health, 
or when the symptoms are such that the supposition of a single focus of 
disease appears to satisfactorily explain them. Weakness in the lower 
extremities, it matters not how slight, in a young adult, if accompanied 
by exaggerated tendon-jerks and Babinski's sign, should suggest to the 
physician the possibility of this disease, particularly when on examination 
no evidence of vertebral disease, no muscular wasting, and no objective 
sensory disturbances are detected. If in addition a degree of ataxia is 
present and the abdominal reflexes are absent, the suspicion is considerably 
strengthened. Further, if there is a history of previous temporary paresis 
or paresthesia in a limb or of a fleeting amblyopia or diplopia, the suspicion 
amounts almost to a certainty. A slight degree of ataxia in the upper 
extremities, the occurrence of vertigo, and, above all, pathological pallor 
of one or both optic disks in conjunction with a spastic or ataxic paraplegia 
render the diagnosis almost certain, even in the absence of a history of 
variations in the clinical picture or of nystagmus, intention tremor, or 
characteristic alterations in speech. 
Hysteria.-Of the many diseases for which disseminated sclerosis may 
be mistaken, this takes the first place. Disseminated sclerosis often 
occurs in young women, its early symptoms are often transient and com- 
pletely recovered from, while hysterical stigmata are often met with in 
association with the organic affection. To Thomas Buzzard especially is 
due the credit of showing the frequency with which disseminated sclerosis 
is mistaken for hysteria. Transitory paresis or paresthesia in a limb, even 
though completely recovered from, symptoms which some years ago would 
have been diagnosed as certainly functional, are to be regarded with the 
gravest suspicion, since they are so often the precusors of the affection 
under consideration. Even although a hemianesthesia with involvement 
of the special senses is detected, the observer must remember that the 
recognition of hysteria does not exclude associated organic disease. Nys- 
tagmus, pallor of the optic disks, and Babinski's sign justify a positive 
diagnosis of organic disease, while incontinence of urine, a symptom 
which it is true does not usually occur until the later stages of the disease, 
carries with it the same significance. 
Acute disseminated myelitis or encephalomyelitis presents a very similar 
picture; some believe that it is a more intense degree of the same process. 
The symptoms develop acutely, it may be with fever, and reach their 
height in a few days. There is muscular wasting. Variations in the 
intensity of the symptoms do not occur. Progress is in the direction of 
improvement, although this is very often limited. 156 
DISEASES OF THE NERVOUS SYSTEM 
Subacute combined degeneration of the spinal cord may simulate dissemi- 
nated sclerosis very closely. The diagnosis is very important, for these 
cases often run a very rapid course. In both diseases ataxic paraplegia is 
often the most striking feature. Subacute combined degeneration usually 
develops rather later in life, and its symptoms once present show no ten- 
dency to disappear. Pallor of the optic disks is not met with, while pro- 
nounced anesthesia, beginning in the feet and progressing steadily upward, 
is the rule. The symptom complex of the later stages, viz., the flaccid 
palsy, often suddenly developed, with abolition of the tendon jerks and 
complete loss of sphincter control, places the diagnosis beyond all doubt. 
Cerebrospinal syphilis occasionally closely resembles disseminated 
sclerosis. The peculiar pallor of the optic disks, the presence of a color 
scotoma, and absence of the abdominal reflexes speak strongly for dis- 
seminated sclerosis. Scanning speech and intention tremor do not occur 
in cerebrospinal syphilis. The Argyll-Robertson pupil is almost con- 
clusive evidence of syphilis of the nervous system, corroborative evidence 
of which is afforded by the detection of a cerebrospinal lymphocytosis 
or a positive Wassermann reaction. Improvement under antisyphilitic 
remedies may help to confirm the diagnosis. 
General paralysis of the insane has been known to cause trouble in diag- 
nosis. The writer has met with one instance in which this was so. The 
more profound mental alterations, a history of syphilis, and the existence 
of the Argyll-Robertson pupil, a cerebrospinal lymphocytosis or positive 
Wassermann reaction are data of importance. 
Intracranial tumor is a diagnosis sometimes made in the early stages of 
disseminated sclerosis. The writer has seen this error made on three occa- 
sions by eminent authorities, the subsequent progress of the case demon- 
strating its true nature. Giddiness with vomiting, unsteady gait, nystag- 
mus, and tremor may all result from a patch of sclerosis situated in the 
cerebellar peduncles. Headache may be present, though rarely severe, 
while in very exceptional cases there may be a slight degree of optic 
neuritis. The intensity of the symptoms due to increased intracranial 
pressure in most cases of new growth and the absence of remissions are 
points of importance in relation to the diagnosis. 
All forms of spastic paraplegia in which the paralysis of the lower limbs 
is the dominant feature may cause difficulty. Many of the cases formerly 
classed as primary lateral sclerosis, an affection which we now know to be 
one of the rarest of organic nervous diseases, were without doubt examples 
of disseminated sclerosis. It may be, indeed, that there1 is at the present 
time a tendency for the pendulum to swing too far in the opposite direc- 
tion. The possibility of amyotrophic lateral sclerosis must always be 
kept in view. 
The differential diagnosis from pseudo-sclerosis and diffuse sclerosis will 
be considered under these diseases. 
Prognosis.-It is very doubtful whether permanent recovery ever 
takes place although cases have been observed in which the patient has 
remained free from symptoms for several years. Thus, Oppenheim men- 
tions three examples in which the patients had recovered and been in per- 
fect health for from five to ten years, and similar instances have been met SCLEROSES OF THE BRAIN 
157 
with by others. No less uncommon, however, are cases such as those 
recently reported by T. Buzzard in which after intervals of several years 
the symptoms of the disease have reappeared. Time may show that very 
exceptionally permanent recovery may take place. For the present this 
question cannot be answered in the affirmative. 
The disease tends in the majority of cases to run a chronic course, often 
interrupted, it is true, by remissions and relapses. There are instances in 
which death has occurred within a year, but these are exceptional. 
Byrom Bramwell has ascertained the duration of life from the appearance 
of the first symptom in the 35 fatal cases of his series. He found that in 
10 cases (28.5 per cent.) death occurred in less than five years, in 23 
(65.7 per cent.) in less than ten years, and in 31 (88.5 per cent.) in 
less than fifteen years; one patient died within a year. In no instance 
was the duration more than twenty-one years. On the other hand, 
among 61 non-fatal cases, 5 had lived at the time of writing for 
periods varying from twenty-two to thirty-three years. The average 
duration of life in 96 fatal and non-fatal cases was found to be ten and 
a half years. 
There are practically no available data which permit of an opinion as 
to the probable course or duration of a particular type of case when seen 
in its early stages, although if bulbar symptoms or pronounced bladder 
trouble appear early the expectation of life will probably be less than the 
average. 
Treatment.-Conclusions as to the beneficial effect of this or that line of 
treatment are naturally extremely difficult to arrive at in the case of an 
affection in which there is a tendency for intermissions and remissions to 
occur independently of all therapeutic intervention. No known remedy 
has been proved beyond doubt to exert a favorable effect, hence treatment 
is limited (1) to certain general directions indicated by common-sense; (2) 
the avoidance of circumstances which may be expected or have actually 
been proved to have a deleterious influence; and (3) to attempts to alle- 
viate individual symptoms as they arise. 
The spirit of optimism which these patients not uncommonly present 
should, so far as possible, be left undisturbed for even although it is per- 
haps unreasonable to suppose that such a mental state can favorably 
influence the disease, yet it is certain that prospects of improvement do 
much to make the patient's outlook on life more bearable. 
Strict injunctions are to be laid down as to the .avoidance of muscular 
exhaustion and in particular the patient should be cautioned when walking 
always to stop short of fatigue. Confinement to bed is, on the other hand, 
a mistake; indeed, the patient should rather be encouraged to regularly 
exercise his muscles. A warm climate may be recommended to those who 
can afford it. Plenty of fresh air and a nutritious diet are advisable. 
Worries of all kinds are to be, as far as possible, avoided. Hot baths are 
contra-indicated, experience having shown that sometimes harm is done 
thereby. Marriage should be forbidden. The prejudicial effect of preg- 
nancy is undoubted and should be explained to female patients. The 
induction of abortion may be even justifiable, when it is ascertained that 
during a previous pregnancy the symptoms were intensified. 158 
DISEASES OF THE NERVOUS SYSTEM 
Ataxia when pronounced is at times benefited by a course of coordinated 
exercises, although the brilliant results sometimes obtained in the treat- 
ment of tabes are not to be expected. Hydrobromate of hyoscine in doses 
of to gr. may influence the tremor. Flexor spasms, often a 
troublesome symptom, since they interfere with sleep, are occasionally 
alleviated by five to ten grains of veronal given at night. When rigidity 
of the limbs is extreme the cautery applied to the back on either side of the 
spine may prove of service. Incontinence or urine, if due to overflow 
from a distended bladder, is to be treated by the passage of the catheter at 
regular intervals, while if it is of the intermittent variety,benefit may result 
from tincture of belladonna in 10 minim doses. Obstinate constipation 
is best relieved by a daily soap and water enema. 
Associated hysterical symptoms should be treated by a confident pre- 
diction that they will disappear and by the methods of suggestion and 
persuasion commonly employed in the treatment of such manifestations. 
Although no drug has been definitely proved to have any effect upon the 
course of the disease, the writer is inclined to believe that arsenic in 
moderate doses, 2 to 6 minims of the liquor three times a day, is some- 
times of service. Strychnine is to be avoided, since it tends to increase 
the spastic state. Silver nitrate and potassium iodide have, it is 
affirmed by some writers, a universal beneficial effect. Fibrolysin has 
been employed but apparently without definite benefit. 
Pseudo-sclerosis.-Westphal described under this name two cases 
diagnosed during life as disseminated sclerosis, in one of which the autopsy 
was entirely negative, while in the other the change found was a general 
increase in the consistency of the brain. Strumpell gave the first dis- 
tinctive description of the symptom complex. Frankl-Hoch wart1 has 
studied the cases reported up to 1903, 13 in number. Two important 
contributions have since appeared, one by Fickler2 who reported two 
cases and reviewed the literature. The other is by Rebizzi,3 who regards 
pseudo-sclerosis and diffuse sclerosis as the early and late stage of the same 
affection, for which he proposes the title of Westphal-Striimpell's disease. 
Etiology.-Baumlin's four patients were sisters, and Rebizzi has de- 
scribed cases occurring in father and daughter. A strong neuropathic 
heredity has been a striking feature in several instances. In no case has 
there been any definite relationship to trauma. Males and females are 
equally affected. The symptoms developed in 5 cases during the first 
decade, in 3 during the second, in 1 in the third, and in 4 in the fourth. 
Pathology.-No changes were found postmortem in the majority of the 
cases examined. It should be mentioned, however, that in 6 cases a micro- 
scopic examination of the brain was not made, and that the majority of the 
observations predated the introduction of elective staining methods. The 
consistence of two of the brains examined is described as abnormally dense. 
A spinal leptomeningitis, slight in degree, was found by Baumlin in one 
case. Strumpell met with a very slight degeneration in the cervical por- 
tion of the pyramidal tracts in both his cases, in one of which similar 
1 Arb. aus dem Neurol. Institut an der Wiener Universitat, 1903, Heft 10, p. 1. 
2 Deutsche med. Wochenschr., 1904, No. 51. 
3 Riv. di patol. nerv. a ment., vol. x. SCLEROSES OF THE BRAIN 
159 
changes were also observed in the cervical portion of Gowers' tracts. 
Rebizzi and Frankl-Hoch wart remark that the morbid anatomy of pseudo- 
sclerosis cannot be strictly separated from that of diffuse sclerosis, for 
cases are on record which might be included in either group. 
Symptoms.-Mental changes are the rule. A degree of apathy, pecu- 
liarities of disposition, irritability, and attacks of temper are among the 
alterations noted, while maniacal outbursts with hallucinations have been 
reported. Epileptic attacks were present in 6 of 13 cases collected by 
Frankl-Hochwart. Some disorder of speech was observed in 9 cases, in 5 
of which speech was described as scanning, while in 2 it was unintelligible. 
Facial paralysis has not been noted, although in 3 cases a defect in facial 
mimicry was remarked on. The optic disks were normal in every case, 
with the exception of one instance reported by Fickler. The special senses 
have always been normal, as were the pupils. Nystagmus was present in 
one case only, and once paralysis of the oculomotor nerve was observed. 
Dysphagia was present in one case. Giddiness was complained of by 
three patients, while headache, faints, apoplectiform attacks, and uncon- 
trollable outbursts of laughter were each noted on two occasions. 
Weakness of the extremities is described in two-thirds of the reported 
cases. Three times the paresis was hemiplegic in distribution; in one case 
all four extremities were involved. The gait is referred to as "clumsy, 
ataxic, or unsteady" in 5 cases, twice it is noted as spastic, and in two 
instances the patient was unable to walk. Tremor in the upper extremi- 
ties was a constant symptom. In four cases it occurred during repose. 
It was of greater amplitude and less regular than in disseminated sclerosis. 
Three times the lower limbs were affected, the head was implicated in 3 
cases, while twice the face and tongue were also involved. Contractures, 
sometimes temporary, sometimes permanent, were present in 9 cases. 
Pains sometimes rather intense, were occasionally complained of. 
Three patients suffered from paresthesias of various kinds. In one of 
Westphal's cases hyperesthesia in different situations was noted at times 
together with some loss of sense of position. 
The tendon reflexes, examined in 10 cases, were once found to be normal, 
while on nine occasions they were exaggerated, clonus being present in 
five. In Fickler's case the superficial reflexes were absent, otherwise no 
anomalies of the skin reflexes have been met with. In 2 cases there was 
incontinence of urine, while in 1 there was incontinence of feces. Transi- 
tory vasomotor disturbances were occasionally noted. 
In 3 cases death occurred in from nine to ten years, in 3 from four to 
six years, and in 4 from one to two years. Frankl-Hochwart's patient 
lived for fifty-seven years after the onset of symptoms. Pronounced inter- 
missions were present in three cases, while in the records of several others 
there are indications of the same. 
Diagnosis.-Pseudo-sclerosis bears a very close resemblance clinically to 
disseminated sclerosis, from which it has rarely been distinguished during 
life. The chief points of importance which distinguish pseudo-sclerosis are 
the frequency of mental changes, notably attacks of excitement and pro- 
nounced dementia; the wild character of the tremor, which sometimes 
occurs during rest, a very rare occurrence in disseminated sclerosis; the oc- 160 
DISEASES OF THE NERVOUS SYSTEM 
currence of epileptic attacks, which were present in half the recorded cases 
of pseudo-sclerosis, but very rare in disseminated sclerosis; the absence of 
changes in the optic disks: the comparative insignificance of the paretic 
symptoms in the lower limbs until the later stages, and the relative rarity 
of bladder symptoms. The development of the earliest symptoms during 
the first decade would be a strong point in favor of pseudo-sclerosis, as 
would a history of a similar malady affecting a relative. 
Diffuse Sclerosis.-This rare affection, like pseudosclerosis, bears a 
close resemblance clinically to disseminated sclerosis. 
Etiology.-It commences not uncommonly in childhood, sometimes not 
until the third or fourth decade. Heredity does not appear to be of etio- 
logical importance. There is a history of associated trauma in about a 
third of the reported cases. A relationship with alcohol, meningitis, and 
syphilis has been claimed in individual cases. 
Pathology.-The most striking feature is the dense consistence of the 
brain, which has been described by individual writers as leathery, like 
India rubber, elastic, and cutting like cartilage. The cut section looks 
almost like ivory. Weiss points out that where there is much shrinking, 
hydrocephalus with ventricular dilatation may result. 
Symptoms.-These resemble pseudo-sclerosis so closely that it will be 
unnecessary to do more than indicate the main resemblances and differ- 
ences. In both the mental alterations occupy a prominent place, though 
in diffuse sclerosis dementia is more common and more profound. Speech 
disturbances occur in a large proportion of cases of diffuse sclerosis, and are 
also more marked than in pseudo-sclerosis. Tremor occurs in about two- 
thirds of the cases while paresis in the lower limbs with an unsteady gait 
are about as common as in pseudo-sclerosis, although, as a rule, more pro- 
nounced in degree. Aphasia, present in a third of the reported cases, is 
unknown in pseudo-sclerosis. Facial paresis has been described in a 
number of instances, and dysphagia, a symptom only once observed in 
pseudo-sclerosis, was present in half the reported cases. Giddiness and 
epileptic attacks occur about as frequently in the two conditions. Muscu- 
lar atrophy has been described in a few cases of diffuse sclerosis, never in 
pseudo-sclerosis. Objective disturbances of sensation are much more 
frequent in diffuse sclerosis, occurring in almost half the cases; inconti- 
nence of urine and of feces is frequent. The average duration of life is 
from one to three or four years, and the course is steadily progressive. 
Diagnosis.-The distinctive features from disseminated sclerosis are 
almost identical with those laid down in considering the differential diag- 
nosis of pseudo-sclerosis from that disease. 
Tuberous or Tuberose Sclerosis. - Bourneville1 described in 1880, 
under the title "Sclerose tubereuse des circonvolutions cerebrales," a 
condition characterized by overgrowth of the neuroglia in circumscribed 
islets which project from the surface of the cortex cerebri and from the 
walls of the lateral ventricles. Vogt has in several communications 
drawn attention to the clinical features. A. W. Campbell,2 in 1905, ana- 
lyzed 20 cases. J. S. Fowler and W. E. Carnegie Dickson, who in 1910 
1 Archiv. de Neurol., 1880, i, 91. 
2 Brain, 1905, p. 382. SCLEROSES OE THE BRAIN 
161 
collected 29 cases, give an admirable summary of the literature.1 Of 
these cases 20 were males and 9 females. There was a well marked neu- 
ropathic heredity in 12 out of 20 cases. Syphilis is not a cause. Epilepsy 
and mental defect are invariable. In only two cases was the mental defect 
described as moderate. The convulsions began in just one-half the cases 
during the first year. Some patients appear to have been normal up to 
the time of the first convulsion. Contractures and spastic paralysis were 
noted in six cases only, tremor in none. Congenital dislocation tumors 
are met with especially in the brain, kidney, heart, and skin. The diag- 
nosis may be made " by the recognition of some of the associated tumors 
in a mentally defective person who is, or has been subject to epileptiform 
convulsions." Eight of the patients died by the fifth year and 20 by the 
sixteenth, while one patient lived to 35. 
Miliary Sclerosis.-Under this name Gowers2 described a case in 
which "throughout both hemipheres of the brain, and in all parts of them, 
the cortex contained minute reddish-gray spots at the junction of the gray 
and white substance." There was wasting of the nerve elements and an 
increase in the connective tissue in the affected areas. The patient was a 
man, aged fifty years; the chief symptom was general weakness of the 
limbs, with some rigidity and a few unilateral convulsive attacks. The 
speech became mumbling and unintelligible, and the patient became 
comatose and died. There was history of syphilis. The total duration 
of the symptoms was about ten weeks. Greiff has described similar 
appearances in general paralysis of the insane. 
1 Quart. Jour, of Med., 1910-11, p. 43. 
2 Lancet, 1886, i, 145. CHAPTER V. 
THE DISEASES OF THE MENINGES 
EDWIN BRAMWELL, M.D., F.R.C.P. (Lond. and Edin.) 
Anatomical and Physiological Considerations.-The central nervous 
system is invested by three membranes: the dura mater, the arachnoid 
mater, and the pia mater. The dura mater, a dense fibrous tissue mem- 
brane, divisible into two layers and lined on its inner surface by endothe- 
lium, lies in close contact with the inner table of the skull and is continued 
as a fibrous sheath on to the cranial nerves. Three prolongations of 
the dura mater, the falx cerebri, tentorium cerebelli, and falx terebelli, 
extend into the cranial cavity and play an important role as supporting 
structures. The dura mater is for the most part irrigated by the middle 
meningeal artery, while the trigeminus is its source of nerve supply. 
The arachnoid mater and pia mater, the leptomeninges, are commonly 
described as the "pia arachnoid," since disease processes which affect 
the one always involve the other. The pia mater is a thin vascular 
layer of fibrous tissue, which lies in the closest contact with the outer 
surface of the central nervous system. A prolongation of the pia mater, 
the velum interpositum, passes into the transverse fissure of the brain, 
carrying with it the choroid plexuses. The arachnoid, on the other 
hand, is a non-vascular fibrous tissue membrane. It lies external to 
the pia mater, with which it is connected by a delicate meshwork of 
fibrous tissue strands, and it is covered on its outer surface by a layer 
of endothelium. The arachnoid does not dip into the sulci as does the 
pia, but bridges them over. The inner surface of the ventricles is lined 
by a layer of endothelium known as the ependyma. 
The subarachnoid space which lies between the pia and arachnoid 
contains the cerebrospinal fluid. Its meshes communicate freely with 
one another. The two membranes are widely separated where they lie 
between the cerebellum and medulla, and the space so formed is some- 
times known as the posterior subarachnoid space or reservoir. The 
foramen of Magendie and the two adjacent foramina of Luschka offer 
the only means of communication between the subarachnoid space and 
the intraventicular system. 
The cerebrospinal fluid is a perfectly clear fluid with a slight alkaline 
reaction and a specific gravity of 1006. Its principal constituent is 
sodium chloride. Traces of carbonates and phosphates are also present. 
It contains traces of globulin and of urea, also a reducing substance 
which is probably glucose. With the exception of a few lymphocytes 
it is devoid of cellular elements. Although it is held by some that the 
cerebrospinal fluid is a transudation, the researches of Halliburton as 
to its chemical composition afford strong evidence for believing that it 
162 DISEASES OF THE MENINGES 
163 
is not an exudation, but a true secretion. The fluid is in all probability 
secreted by the ependymal cells, which cover the choroid plexuses, and 
the secretion is probably continuous. The amount produced in the 
twenty-four hours has been variously estimated, some observers placing 
the quantity as high as one or two liters. Observations upon this point 
are possible when after certain cerebral operations the cerebrospinal 
fluid which escapes from the wound can be collected. It is, however, 
somewhat doubtful whether these results can be regarded as a true 
estimate of the normal secretion, since the operative procedure and the 
pathological condition for which it has been undertaken may alter its 
amount (Cushing). The fluid fills the whole intraventricular system, 
and through the foramina of Magendie and Luschka has free access 
to the cerebrospinal subarachnoid space. Since the secretion of fluid 
is in all probability continuous, it follows that in order to regulate the 
intracranial pressure there must be a constant return of the fluid into 
the blood stream. From the therapeutic standpoint it is of importance 
to remember that the nerve cells are surrounded by pericellular lymph 
spaces, that these are irrigated by cerebrospinal fluid, that medicinal 
substances before they can enter the cerebrospinal fluid have to be 
secreted by the secreting cells which cover the choroid plexuses of the 
brain, and that these cells have a selective action. 
PACHYMENINGITIS. 
The diseases of the dura mater are naturally divisible into two groups, 
according as the pathological process is situated on the outer or inner 
surface of this membrane. 
Pachymeningitis Externa.-Inflammation of the outer surface of the 
dura mater is uncommon. It occurs almost always as a secondary 
consequence of neighboring disease. A cerebral and a spinal form are 
described. 
Cerebral.-The outer surface of the cerebral dura mater lies in close 
contact with the inner table of the skull, hence external pachymeningitis 
in this situation is always localized. It may be a sequel to fracture of 
the skull with extravasation of blood on the outer surface of the dura, 
erysipelas of the scalp, syphilitic caries, or tumors of the cranium. The 
extension of an inflammatory process arising in the labyrinth or mastoid 
cells may result in a localized external pachymeningitis or an extradural 
abscess. The clinical features may be overshadowed by those of the 
primary affection. 
Spinal.-The dura mater which surrounds the spinal cord does not 
lie in contact with the bony canal, but is separated from it by a space, 
which is occupied by loose areolar tissue. Hence pachymeningitis 
externa spinalis may be either widespread or localized. In the great 
majority of cases the process is secondary to disease of adjacent structures. 
A bedsore perforating the sacrum may set up an acute and extensive 
perimeningitis; the infection may even penetrate the dura and lead to a 
leptomeningitis. Very rarely does a leptomeningitis extend through the 
dura and cause inflammation on its outer surface. Primary acute osteo- 164 
DISEASES OF THE NERVOUS SYSTEM 
myelitis or periostitis, actinomycosis, syphilitic disease of the vertebrae, 
or an aneurism which has eroded the vertebral bodies, may determine a 
localized external pachymeningitis. Tuberculous disease of the spine is 
a much more common cause. The dura which is often thickened is sur- 
rounded by a mass of tuberculous granulation tissue, in which caseous 
foci and abscesses of variable size may be contained. The tuberculous 
tissue is firmly adherent to the dura, the inner surface of which is usually 
smooth. The dura, however, may be infiltrated, and the morbid process 
may even extend through to its inner surface. In very rare cases 
tuberculous pachymeningitis is a primary affection (Schlesinger). 
Symptoms.-These vary according to the nature and extent of the 
lesion upon which they depend. Thus when the process is acute and 
diffuse, pain in the back, root pains, muscular spasms, and hyperesthesia, 
with elevation of temperature, are met with. Again, when the pachy- 
meningitis is localized and chronic, symptoms indicative of increasing 
pressure on the spinal cord, similar to those which occur in the case of a 
tumor, are produced. Indeed, in cases of tuberculous pachymeningitis 
in which there is no spinal curvature or evidence of disease of the vertebrae 
the diagnosis from tumor may be difficult if not impossible. 
Treatment.-The treatment is that of the primary disease. 
Pachymeningitis Interna.-A purulent internal pachymeningitis occurs 
as a very rare result of an extension from inflammation of the lepto- 
meninges. Osler has met with a pseudo-membranous inflammation 
of the lining membrane of the dura as a secondary process in pneumonia. 
Pachymeningitis Interna Hemorrhagica.-This condition is sometimes 
known as hematoma of the dura mater. It is customary to describe 
it with inflammatory diseases of the meninges, although it is not unlikely 
that the initial change is a hemorrhage from the inner surface of the dura. 
Pathology.-This has evoked considerable discussion. A clinical 
diagnosis is rarely made, and even when recognized during life it is 
doubtful whether therapeutic measures have any distinct influence 
in arresting its progress. Huguenin's article in von Ziemssen's Cyclopedia 
still remains the classical monograph upon the subject, and during the 
past thirty years little of importance has been added. 
The condition is rarely seen in the postmortem room of a general 
hospital, but is not uncommon in asylum practice. Thus in 1185 post- 
mortems at the Government Hospital for the Insane, Washington, 
there were 197 cases with "a true neomembrane of internal pachymen- 
ingitis" (Blackburn). The disease is an affection of later life. Of the 
cases collected by Huguenin, 41 per cent, were between sixty and eighty, 
and 31 per cent, between forty and sixty years of age. During the first 
year of life 2.7 per cent, occurred; injury to the cranium during delivery 
no doubt accounts for this. Males suffer more frequently than females 
in the proportion of three to one (Durand-Fardel). 
A great variety of associated morbid conditions have been met with. 
Among Blackburn's 197 cases in 45 there was chronic dementia, in 37 
general paralysis, in 30 senile dementia, in 28 chronic mania, in 28 chronic 
melancholia, in 22 chronic epileptic insanity, in 6 acute mania, and in 
1 case imbecility. Kronig, who analyzed 135 cases examined post- DISEASES OF THE MENINGES 
165 
mortem at the Berlin Pathological Institute, found phthisis (23 per cent.), 
general paralysis of the insane (19 per cent.), cardiac valvular disease 
(18 per cent.), syphilis (11 per cent.), puerperal sepsis (9 per cent.), 
and chronic alcoholism, carcinoma, and infectious diseases (about 6 per 
cent. each). Hemophilia and scurvy are also recognized causes, as 
are diseases of the blood, especially pernicious anemia. Traumatism 
is sometimes a determining factor (Schneider, 17 out of 74 cases). 
According to Huguenin, the great majority of cases occur with changes 
which cause a reduction in the volume of the brain, especially general 
paralysis of the insane. It is reasonable to suppose that the loss of 
support to the dural vessels which must accompany wasting of the brain 
predisposes to hemorrhage. Further, disease of the vessels is usually 
demonstrable in association with the pathological states which give rise 
to shrinkage of the cerebrum. 
On postmortem examination there may be only a thin red or grayish- 
red film on the inner surface of the dura mater. The convexity of the 
cerebrum is its most common situation, although it may extend over a 
considerable extent of one or both hemispheres. Among 54 cases per- 
sonally examined by Wigglesworth, 20 were strictly unilateral and 34 
were bilateral, while 56 per cent, of the cases observed by Huguenin 
had a bilateral distribution. The membrane may extend to the base 
of the brain and is not uncommonly met with on the upper surface of 
the tentorium, although very rarely beneath it (Wigglesworth). In 
more advanced cases it may be of considerable thickness, consisting of a 
series of laminae which have been successively deposited, the most recent 
layers being next the cerebrum. There may be as many as twenty of 
these laminae, of which those of later origin are of a reddish color, the 
older layers being paler. Between the individual layers collections of 
fluid blood may be found. In some cases the false membrane may 
actually measure 2 or 3 cm. in thickness. 
Symptoms.-These are various, and do not form a precise clinical 
picture. In many cases no symptoms are produced, and it may be 
that those which are present are obscured by those dependent on the 
Original disease. A cerebral complication may be diagnosed, but its 
exact nature often remains a matter of uncertainty, The symptoms 
depend on the situation and extent of the morbid process. A degree of 
excitability with general restlessness, elevation of temperature, headache, 
giddiness, and vomiting may signalize the onset or extension of the lesion. 
The headache is sometimes very severe, and the patient may become 
delirious. Localized and recurring convulsive attacks, monoplegic 
or hemiplegic in distribution with, it may be, conjugate deviation of 
the eyes, paralysis or paresis of the hemiplegic or monoplegic type, 
slowing and irregularity of the pulse, and the gradual development of 
stupor and coma is a common history. The cranial nerves are rarely 
involved. Aphasia is occasionally present. There may be optic neuritis. 
Recovery may take place, to be followed by another attack. 
Diagnosis.-The diagnosis may present the greatest difficulty. The 
condition may be suspected when in an individual who is suffering from 
one of the predisposing diseases, cerebral irritative phenomena develop 166 
DISEASES OF THE NERVOUS SYSTEM 
with associated paralysis, these symptoms varying from time to time 
and being accompanied by more or less stupefaction and, it may be, 
coma. Hemorrhagic fluid may be obtained on lumbar puncture in 
traumatic cases. 
Treatment.-The head may be elevated and ice applied, but it is very 
doubtful whether any known treatment tends to arrest the bleeding. 
Pachymeningitis Cervicalis Hypertrophica (Hypertrophic Internal 
Pachymeningitis).-This is characterized anatomically by great thicken- 
ing of the spinal membranes, more especially in the cervical region, 
with consequent pressure upon the spinal cord and its nerve roots. In 
a typical case the lower cervical portion of the cord is found to be sur- 
rounded by a dense fibrous covering, which may be five or ten times 
(Oppenheim) as thick as the normal dura. The cord and its nerve roots 
are in fact embedded in an annular fusiform tumor of dense fibrous 
tissue of, it may be, the consistence of cartilage, usually thickest poste- 
riorly and consisting of concentric layers inseparable from one another. 
The anatomical features are similar to those in chronic syphilitic meningo- 
myelitis. According to Wieter, the process actually originates in the 
leptomeninges, and should be named meningomyelitis cervicalis chronica 
rather than pachymeningitis, since the dura is only secondarily involved. 
Pressure on the nerve roots induces necrotic changes in them with increase 
of interstitial tissue. The cord is flattened anteroposteriorly and ulti- 
mately ascending and descending degenerations are produced. Cavity 
formation, a consequence of intramedullary softening, is sometimes 
observed. It must not be supposed, however, that the process is limited 
to the cervical region. True, it is usually most pronounced here, but 
it often extends through a considerable extent and occasionally implicates 
the medulla and pons 
There is little known with regard to etiology. Cold, injury, and over- 
exertion are all cited as possible causes. Syphilis appears to be an 
undoubted factor in some cases. 
Neuralgic pains, often very severe, and referred to the distribution 
of the posterior roots entering the cord at the level of the lesion, are 
usually the earliest symptoms. This is the neuralgic stage of Charcot. 
At a later date, local and increasing muscular weakness, with wasting 
and impairment or loss of sensation, a consequence of interference with 
conduction in the spinal nerve roots, result. The small muscles of the 
hand and the flexors of the fingers and wrist are usually, because of the 
situation of the lesion, first affected, hence the deformity known as the 
"preacher's hand," which is not uncommon. Later still a third stage is 
reached, which is characterized by interference with conduction in the 
spinal cord, spastic paraplegia with increased reflexes, contractures, 
anesthesia of the lower extremities, loss of power over the bladder and 
rectum, and bedsores. When the process involves the medulla and 
pons, various cranial nerves may be implicated. 
The very gradual development of severe bilateral pains in the neck 
and arms, succeeded by weakness, muscular wasting, and, it may be, 
objective sensory disturbance having a root distribution, and at a later 
date by symptoms of progressive pressure on the spinal cord, is a very DISEASES OF THE MENINGES 
167 
suggestive history when no evidence of vertebral disease can be de- 
tected. 
Tuberculous pachymeningitis may present an identical clinical picture 
when there is no evidence of disease of the bone. The detection of 
tuberculous lesions in other parts of the body, an x-ray photograph of 
the spine, and the tuberculin test may be of value under these circum- 
stances. When pressure symptoms are due to a simple tumor of the 
meninges, they are often unilateral. 
Syringomyelia and amyotrophic lateral sclerosis may closely simulate 
hypertrophic pachymeningitis, but these conditions are unaccompanied 
by the severe pains in the neck and arms, which are so characteristic 
of the affection under consideration. The co-existence of syringomyelia 
and pachymeningitis has been noted. 
A thorough antisyphilitic course of treatment is called for in any case 
in which there is a possibility of syphilis. Counterirritation with the 
cautery over the lower cervical vertebrae is sometimes useful. The 
various analgesics may be used to relieve the pains, which will often, 
however, only yield to morphine. 
ACUTE CEREBROSPINAL MENINGITIS. 
Etiology.-Cerebrospinal fever, tuberculous meningitis, the acute 
affection of the meninges met with as an occasional sequel of syphilis, 
and the amicrobic serous meningitis of Quincke are described elsewhere. 
A group of cases, sometimes included under the comprehensive title of 
Acute Purulent Meningitis, remains for consideration. These may be 
subdivided according to their mode of production and with special 
reference to the causative agents upon which they depend. The causes 
are very yaried, and yet the distribution of the inflammatory process, 
its character, and the accompanying symptoms present no constant 
features distinctive of the individual infections. 
Leptomeningitis may be produced by many organisms, yet if we 
except the types due to the meningococcus, pneumococcus, streptococcus, 
and the tubercle bacillus, the general statement holds good that acute 
cerebrospinal meningitis is of rare occurrence. Councilman in 60 con- 
secutive cases of the disease found the following organisms: Pneumo- 
coccus (18), streptococcus (18), meningococcus (21), staphylococcus (2). 
Acute leptomeningitis may be of primary or secondary origin. The 
latter group of cases may be subdivided according to the conditions 
with which they are associated. 
Primary leptomeningitis, apart from cerebrospinal fever, is of very 
rare occurrence, and is almost always due to the pneumococcus. Thus, 
Marchal, quoted by Weichselbaum, found that the meningococcus was 
responsible for 50.5 per cent, and the pneumococcus for 42 per cent, of 
the primary cases of sporadic meningitis. The mode of entrance of the 
organisms in primary meningitis is a debatable question. Infection 
by way of the nose is very probable in some cases. It is to be remembered 
that two factors are necessary in order that a primary inflammation of 
the meninges may be produced: (1) the infective organism must be pres- 168 
DISEASES OF THE NEHVOUS SYSTEM 
ent, while (2) predisposing causes resulting in lowered resistance must 
also exist (Weichselbaum). 
Secondary meningitis may be associated with a variety of conditions. 
Trauma is a not uncommon factor; thus a leptomeningitis may be directly 
produced by a penetrating wound or a compound fracture of the skull. 
The manner in which the organisms obtain access to the meninges under 
these circumstances is obvious. Infection through some of the accessory 
sinuses may account for cases in which a meningitis follows a fracture 
of the skull in which the skin surface has not been broken. Extension 
from adjacent disease is responsible for many cases. An acute or chronic 
otitis is much the most common factor in this connection. The pus may 
pass by direct extension through the bone, or the infection may spread 
by way of the sheaths of the facial or auditory nerves, or be carried by a 
suppurative phlebitis. When the infective process stops short of the 
dura an extradural abscess or a serous meningitis may result. If it 
perforates the dura an encapsuled meningeal or brain abscess or a general 
purulent meningitis follows. Exceptionally meningitis is secondary to 
disease in the nasopharynx, orbit, or frontal sinuses. Erysipelas of the 
scalp or a carbuncle in this situation are occasional causes. A generalized 
meningitis may be set up by an abscess of the brain, even though rupture 
has not taken place. 
In the course of the infective fevers meningitis is met with as an occa- 
sional complication. It is to be remembered that a meningitis so occur- 
ring is not necessarily due to the same cause as the original disease, for 
mixed infections are not uncommon (Fiirbringer). Pneumonia is the 
acute fever in which meningitis is most often met with. Osler found 
meningitis in 8 of 100 autopsies, while among 253 cases of pneumonia 
examined after death, Aufrecht met with 7 in which meningitis was 
present. Musser and Norris found a postmortem record of meningitis 
in 180 of 4833 cases of pneumonia. An acute meningitis is sometimes 
a terminal process in a general streptococcal infection. Its occurrence in 
erysipelas is much rarer than was at one time supposed. Thus Anders 
in his analysis of 1674 cases of this disease does not refer further to it 
than to remark that it is an occasional complication. Typhoid fever 
is very rarely complicated by true meningitis. Cole has collected 21 
cases of meningism, serous and purulent meningitis, and 6 instances were 
present in the Johns Hopkins Hospital series of 1500 cases analyzed by 
McCrae. A few cases have been reported in which the meninges 
were affected without any other lesion. Dubois, in 1903, collected 11 
cases in which a purulent meningitis was due to Pfeiffer's bacillus. 
The rarity of influenzal meningitis may be gathered from the fact that 
of 55,263 cases of this disease occurring in the German army menin- 
gitis was only noted in four. There are eight cases of primary sup- 
purative meningitis in scarlet fever recorded in the literature (Teissier, 
Boudon and Duvoir, 1908). G. Henderson and W. T. Ritchie (1908) 
reviewed the reported cases of gonorrhoeal meningitis. Vennet (1908) 
collected the cases of meningitis occurring in association with mumps. 
The occurrence of meningitis in measles, smallpox, ulcerative endocar- 
ditis, rheumatic fever, actinomycosis, and anthrax has also been recorded. DISEASES OF THE MENINGES 
169 
A streptothrix meningitis has been described. Lastly, acute meningitis 
may be associated as a terminal infection with such diseases as chronic 
nephritis, arteriosclerosis, heart disease, gout, and the wasting diseases 
of children (Osler). 
Pathology.-Acute leptomeningitis is an inflammatory process which 
commonly implicates both the cerebral and spinal meninges. The degree 
and distribution vary; in some the base of the brain, in others the vertex 
is chiefly affected. The simple non-tuberculous meningitis of children 
is basal. Among Nauwerck's 27 pneumococcal cases the base alone 
was involved in 4, the convexity and base in 16, while in 7 the convexity, 
base, and cord were all implicated. When a leptomeningitis is secondary 
to adjacent disease, as, for example, otitis media, the process may remain 
for a time confined to the corresponding hemisphere, but in the great 
majority of cases, whatever the cause, the infection soon becomes general. 
The earliest visible change is a hyperemia of the pia arachnoid, which 
is soon followed by some cloudiness of these membranes. Accompanying 
this is an excessive secretion of cerebrospinal fluid at first clear, later 
turbid, and ultimately, it may be, purulent. Inflammatory exudation 
occurs along the course of the engorged vessels. The yellow lymph may 
gradually spread from the sulci over the convolutions. Finally, the 
surface of the brain may be covered, as is not uncommon in pneumococcal 
cases, with a thick layer of greenish pus. It must not be supposed that 
the underlying brain escapes. An inflammatory oedema of the cortex is 
almost always present, and may go on to a true encephalitis with hem- 
orrhages, hence the term meningo-encephalitis so often applied. Sooner 
or later a serous or seropurulent exudation occurs into the ventricles, 
the ependyma of which may be covered with lymph. When communi- 
cation between the ventricles and subarachnoid space is interfered with, 
a degree of ventricular distension develops, which, however, rarely 
reaches the marked hydrocephalus met with in the tuberculous and 
serous variety. 
The microscopic changes are practically identical with the appear- 
ances described under cerebrospinal fever. Engorgement of the menin- 
geal and cortical vessels, the walls of which are infiltrated with leukocytes, 
is the most striking feature. The inflammatory process extends along 
the course of the bloodvessels into the brain substance. The neuroglia 
commonly shows some increase, while very pronounced changes in the 
pyramidal cells of the cortex are commonly present (Voisin). An acute 
proliferative inflammation of the veins and arteries is often seen in 
the forms of meningitis due to the pneumococcus and streptococcus 
(Councilman). 
Symptoms.-The symptoms in acute purulent meningitis vary with 
the distribution, nature, and intensity of the process. Sometimes there 
is a premonitory stage in which such symptoms as irritability, restless- 
ness, somnolence, malaise, headache, and vomiting are complained of. 
When the meningitis occurs in the course of an acute disease, such as 
pneumonia, the earlier symptoms, if slight, may be obscured by those 
of the primary affection. Sometimes the first indication is intense 
headache, cerebral vomiting, or a general convulsion, followed, it may 170 
DISEASES OF THE NERVOUS SYSTEM 
be, by delirium and rapidly deepening coma. A rigor is not uncommon 
at the outset, especially when the ventrical meninges are attacked. 
Headache, frequently the earliest as well as the most distressing symp- 
tom, is rarely absent. It is, as a rule, widespread, although often referred 
especially to the frontal or occipital regions. When the meningitis is 
due to extension from adjacent disease, the headache may remain local- 
ized for a time. The pain is often very severe, the patient, even when 
in a semicomatose state, putting his hand to his head and crying out. 
"Cerebral vomiting," a common symptom, often develops at the outset 
and may persist. Occasionally it is absent. Delirium is often present 
and may appear at an early date, especially in vertical cases; it is some- 
times violent. The patient is in some cases, even from the first, in a 
somnolent state, which gradually passes into coma. Photophobia and 
hypersensitiveness are common early symptoms, while cutaneous and 
muscular hyperesthesia is almost invariably present at some stage. 
Rigidity of the neck, with pain on movement and perhaps head retrac- 
tion, the latter symptom being rarely, however, so extreme as the retrac- 
tion met with in the meningococcal form, is the rule in basal meningitis. 
When the meninges of the cord are prominently involved, rigidity of the 
back with opisthotonos may be observed. 
The pulse, although increased in frequency, is usually slow at the 
commencement of the illness and not uncommonly irregular both in 
time and force. Later it is often small and rapid. Considerable varia- 
tions in the pulse rate within a short period are often to be noted. A 
pulse rate which is unduly slow in comparison with the height of the 
temperature is a striking feature in some cases. The frequency of the 
respirations may be increased, and they are often irregular. Cheyne- 
Stokes breathing may be observed, especially in the event of an approach- 
ing fatal termination. 
Convulsions of the Jacksonian type are not uncommon, while fits, 
unilateral or generalized, are prone to occur in children, and especially, 
according to most authors, in cases in which the meningitis is vertical 
in distribution. It is interesting to note in this connection that convul- 
sions were not present in a single instance of the cases of penumonia, 
ulcerative endocarditis, or septicemia complicated with meningitis 
observed by Osler. Choreic movements are very occasionally seen. 
Paralysis is far from infrequent in the later stages, and would doubtless 
be more often recognized were it not for the coma which often obscures 
it. Reinhold in 53 cases found hemiparesis fourteen times and a mono- 
plegia on three occasions. The motor weakness may be associated with 
rigidity. Aphasia is an occasional symptom. 
Kernig's sign is, as a rule, present; thus, Netter found it in 41 of 46 
cases. This sign is by no means pathognomonic of a meningeal affection. 
Optic neuritis is not infrequent in basal cases, although pronounced 
papillitis, such as is seen in cases of intracranial newgrowth, is very rare. 
The pupils are often unequal, and are apt to vary much in size from time 
to time. In the early stages they are usually contracted, while at a later 
date dilatation is the rule. Reflex iridoplegia is sometimes observed. 
Strabismus and ptosis are frequent, nystagmus not uncommon. Con- DISEASES OF THE MENINGES 
171 
jugate deviation of the eyes is occasionally met with. Paresis of the face, 
often fugitive, and accompanied at times by tremor in the affected 
muscles, is sometimes noted. Trismus and trophic ulceration of the 
cornea indicating involvement of the fifth nerve are more unusual 
symptoms. Dysphagia is by no means rare. 
The tendon jerks, generally increased in the early stages, are usually 
lost later in the course. The plantar reflex may be of the extensor type. 
Evidence of vasomotor irritability is forthcoming in flushing of the skin 
and in the tache cerebrate, which is usually pronounced. Constipation 
is almost invariable. Sometimes there is retention of urine, while later 
there may be incontinence. Glycosuria has been recorded. Rapid 
emaciation is very often observed. The leukocyte count is commonly 
high although notable exceptions occur. 
Upon lumbar puncture a turbid fluid, which emerges under pressure, 
is usually obtained, even in the early stages; occasionally, however, 
the fluid is quite clear, very rarely it is so thick it will not flow through 
the needle. The fluid is found to contain large numbers of cells, 
the great majority of which are polymorphonuclears, with, in addi- 
tion, perhaps, the specific organisms which are responsible for the 
meningitis. 
Diagnosis.-Acute leptomeningitis may be suspected when cerebral 
symptoms, such as headache, "cerebral vomiting," irritability, general 
hyperesthesia, delirium, and convulsions, develop in association with 
the phenomena attendant on a febrile process, especially when these 
symptoms occur in conjunction with one of those conditions which are 
known to be of special causative moment. All these symptoms may, 
however, occur in the course of some of the specific fevers, notably 
pneumonia, typhoid, and influenza, as a result of congestion of the 
meninges. Rigidity of the neck and Kernig's sign increase the probability 
of meningitis, while the additional presence of local palsies of the limbs 
or cranial nerves affords strong corroborative evidence. 
Schultze holds that headache, rigidity of the neck, and hyperesthesia 
constitute a symptomatic triad, in the absence of which the diagnosis of 
meningitis is uncertain. Corroborative evidence is forthcoming if on 
lumbar puncture a turbid cerebrospinal fluid containing organisms is 
obtained. The various forms of acute generalized meningitis cannot be 
said to present symptomatic features, but the nature of a meningitis may 
be suspected when it occurs as a complication in the course of co-existing 
disease, and its pathology determined if specific organisms are detected 
in the cerebrospinal fluid. 
Meningism or pseudo-meningitis as a term applied to a symptom complex 
met with in the course of some of the infective fevers, which is charac- 
terized by symptoms indistinguishable from the general symptoms of 
meningitis, and yet when opportunity is afforded of examining these 
cases postmortem no inflammatory changes are detected. In some of 
these cases there may be a slight degree of meningitis actually present 
which is recovered from; in many there is probably merely a meningeal 
hyperemia; while in others the symptoms would appear to be due 
to the direct effects of a toxin upon the brain itself. Meningism 172 
DISEASES OF THE NERVOUS SYSTEM 
may be distinguished from true meningitis by the faet that although 
the cerebrospinal fluid may emerge under pressure, it presents normal 
appearances. 
Hysteria may simulate meningitis closely, and the diagnosis may be 
very difficult when hysterical symptoms occur in the course of an infec- 
tive disorder. Tuberculous meningitis is, according to Gowers, especially 
apt to be mistaken for hysteria, while the reverse mistake is seldom 
made. Such signs of organic disease as optic neuritis, ankle clonus, 
Babinski's sign, and nystagmus are of great importance, while a turbid 
fluid obtained on lumbar puncture will determine the point. 
Acute suppurative otitis sometimes presents a picture closely resem- 
bling that of acute meningitis. This is especially so in children, for 
delirium and convulsions, symptoms so apt to occur in early life in any 
acute febrile disturbance, may be accompanied by headache, vomiting, 
and giddiness due to the otitis. Here, again, lumbar puncture will be 
found to be of value. 
An intracranial abscess may give rise to symptoms very similar to 
those of meningitis. Further, abscess and meningitis may co-exist. 
An acute onset, with high temperature, rapid pulse, hyperesthesia, 
irritative phenomena, and a high leukocytosis are in favor of the latter 
affection, as are head retraction, rigidity of the spine, and Kernig's 
sign. Symptoms pointing to focal disease in the cerebellum or temporo- 
sphenoidal lobe, i. e., in the localities in which otitic abscess occurs, may 
indicate the presence of an abscess, but the absence of definite local 
manifestations does not exclude this possibility. When there is no 
co-existing ear disease the likelihood of abscess is small. In an uncom- 
plicated case of abscess the cerebrospinal fluid is clear and contains few 
cells. The symptoms of infective sinus thrombosis may closely resemble 
those due to an otitic meningitis. A pyemic temperature and the occur- 
rence of rigors are very suggestive of the first-named complication. 
Tuberculous meningitis sometimes gives rise to difficulty, and a dis- 
charge from the ear does not exclude tuberculous meningitis, for the 
ear disease is sometimes due to the tubercle bacillus. Tuberculous 
lesions are usually present elsewhere in the body, hyperesthesia is com- 
monly absent, and these cases, as a rule, run a more chronic course. 
The absence of a leukocytosis favors tubercle (Cabot found a leuko- 
cytosis in 32 of 43 cases of tuberculous meningitis). Choroidal tuber- 
cles when present make the diagnosis certain (Koplik found choroidal 
tubercles in 9 of the 46 cases in the first two days of the illness). 
The cerebrospinal fluid is often clear in tuberculous meningitis, and in 
contrast to the purulent form the increase of cells which is often present 
is found to consist almost entirely of mononuclear elements. Bernstein 
found the tubercle bacillus in the fluid obtained on lumbar puncture in 
100 of 102 cases in which he looked for it. 
The differential diagnosis of meningococcal meningitis from other 
forms of acute meningitis has been considered in a previous volume. 
The delirium of alcohol and the uremic state may resemble acute 
meningitis, although it very rarely happens that the diagnostic problem 
is attended with much difficulty. DISEASES OF THE MENINGES 
173 
Prognosis.-The prospect of recovery from an acute purulent lepto- 
meningitis is small. Death is almost invariable in cases with a sudden 
onset. In any form if the stage of coma has been reached, death is all 
but certain (Gowers). If the meningitis is localized the outlook is more 
favorable than when a general meningitis exists. The prognosis varies 
with the nature of the bacterial infection. The probability of recovery 
is greater in cerebrospinal fever than in purulent meningitis due to 
other causes. Recovery from pneumococcal meningitis, whether primary 
or secondary in origin, is most unusual. Thus of the 33 cases collected 
by Musser and Norris, 31 (93.3 per cent.) died, while only 1 of the 11 
cases described by Cantley recovered. Death occurred in all of Nau- 
werck's cases. The chances of recovery are greater in a staphylococcal 
than in a streptococcal infection. The duration of acute meningitis 
varies from a few hours to two or three weeks. Of the 65 cases collected 
by Netter, 54 died within four days. 
Macewen twenty years ago reported 12 cases of local otitic meningitis 
upon which he had operated, in 6 of which recovery took place. Cases 
of otitic meningitis, with pus and staphylococci in the cerebrospinal 
fluid, may recover after operative treatment of the purulent focus in 
the petrous bone (Korner, Alexander). 
Treatment.-The management of cases of meningitis consists in the 
adoption of certain general measures and the alleviation of symptoms. 
The patient must be kept absolutely at rest in a quiet, darkened room; 
a dose of calomel may be given with possible advantage if the case is 
seen early. The head should be shaved and an ice-bag applied to it. 
Counterirritation is generally considered to be of service, and with this 
object a blister may be applied to the back of the neck. Hexamethyl- 
enamine may be given in doses of five to ten grains every four hours. 
There appears to be no evidence to show that injections of collargol and 
other substances into the subdural space are followed by any beneficial 
effect. 
Headache and convulsions may be relieved by bromide and chloral. 
Morphine may be required when the headache is very severe. Vomiting 
is sometimes alleviated by a mustard leaf to the epigastrium or by giving 
the patient ice to suck. For general irritability, hyperesthesia, and 
delirium, warm baths are undoubtedly of value. When the temperature 
reaches an abnormally high point, tepid sponging should be employed. 
When the meningitis is secondary to trauma or ear disease, surgical 
measures should be promptly resorted to. 
Lumbar puncture is certainly of value in relieving the headache, and 
the patient may even be roused from a semicomatose state. The proce- 
dure is unattended with risk if care is taken not to lower the pressure 
of the cerebrospinal fluid below the normal. A marked amelioration of 
the symptoms may even follow the withdrawal of 10 cc. 
Chronic Leptomeningitis.-The chronic inflammations of the meninges 
call for passing notice. Syphilis is the most common cause. A chronic 
inflammation of the meninges is also met with in confirmed alcoholics 
and in general paralysis of the insane. Tuberculous meningitis some- 
times runs a chronic course. The duration in purulent meningitis is, 174 
DISEASES OF THE NERVOUS SYSTEM 
as we have seen, sometimes protracted, especially when the inflammation 
is localized, and necessarily in cases which ultimately recover. Posterior 
basic meningitis is a chronic disease, and in cerebrospinal fever the symp- 
toms may persist for many months before death or recovery takes place. 
A localized chronic spinal leptomeningitis occurs in connection with 
caries of the spine and other forms of pressure paraplegia. 
Symptoms.-These vary greatly and in the syphilitic and tuberculous 
forms often simulate closely those of tumor. The meningitis of chronic 
alcoholism may be suspected by its association rather than by any 
characteristic symptoms. The treatment varies with the cause. 
Localized Serous Spinal Meningitis.-This is an affection which recently 
has attracted some attention. Horsley, who reviewed the literature 
of this affection under the name of chronic spinal meningitis, states 
that he has operated on 21 cases. A circumscribed collection of 
subarachnoid fluid is found pressing on the cord. In some cases it 
would appear that the condition is related to an inflammatory process, 
trauma, or syphilis; Bruns, however, is strongly of opinion that it may 
result as a primary idiopathic affection apart from disease of the spine, 
membranes, or cord. The symptoms are indistinguishable from those of 
tumor. Krause has collected 8 cases in which the Brown-Sequard syn- 
drome and other symptoms led to laminectomy for the removal of a 
tumor, instead of which a localized serous meningitis was found. The 
same author, in 22 laminectomies performed for the removal of a sup- 
posed tumor, found in no less than six that a localized serous meningitis 
was the essential if not the only cause of the paralytic phenomena. In 
any case in which this condition is suspected an operation should be 
undertaken, since a subsidence of the symptoms usually follows. 
Lumbar Puncture and the Examination of the Cerebrospinal Fluid.- 
The operation is a very simple one, it causes little pain, and with due 
precautions is unattended with risk. The patient sits astride a chair, 
over the back of which he leans, or, better still, he lies on his side with 
his knees fully flexed upon his abdomen and his spine well curved. The 
fourth lumbar space is that usually selected for the puncture. The cord 
cannot be injured, since it terminates opposite the second lumbar vertebra. 
Further, there is more room between the vertebra* here than at a higher 
level, while another reason for choosing this situation is that the dura is 
more firmly adherent to the bone here than elsewhere, hence there is 
little chance of pushing the membrane in front of the needle. A line 
joining the highest point of the iliac crests passes between the fourth 
and fifth lumbar spines. The needle should be three and a half inches in 
length and made of steel or of platinum with an iridium point, the 
advantage of the latter being that it can be boiled. The skin should 
be very carefully sterilized. A general anesthetic is unnecessary except 
in the case of excitable patients, but ethyl chloride may be used locally. 
The needle is introduced midway between the fourth and fifth lumbar 
spines a quarter of an inch to one side of the middle line, and is passed 
almost directly forward with a slight inclination inward and upward. 
When the needle reaches the intervertebral ligament it receives a momen- 
tary check, but gentle, firm pressure sends it through the ligament and DISEASES OF THE MENINGES 
175 
dura, and its point then lies in the subarachnoid reservoir. The distance 
traversed by the needle before it reaches the dura varies from 2 to 7 cm., 
according to the age and development of the patient. Should the needle 
come in contact with bone, the point should be withdrawn for a short 
distance and passed in at a rather different angle. If after several 
attempts it is found impossible to avoid the bone, it is best to withdraw 
the needle altogether and reintroduce it in another situation. It not 
uncommonly happens that no fluid comes, even though the needle has 
been felt to pass through the ligament. Under these circumstances 
suction is not to be used, but a stilette should be passed along the needle, 
the lumen of which has probably been blocked on its way through the 
skin and muscles. Occasionally after this manipulation no fluid is 
obtained. This may be accounted for by the fact that the dura has been 
pushed in front of the needle, which has failed to penetrate it, or it may 
be that, as in some cases of hydrocephalus, the ventricular system has 
been shut off and there is no fluid in the subarachnoid space. Again, 
in some cases of purulent meningitis the fluid is so thick that it will not 
flow. Should the needle come in contact with a nerve root as it passes 
through the ligament, the patient complains of intense pain often radiating 
down the leg. The needle should then be withdrawn and reinserted. 
Unpleasant effects very seldom follow the operation, provided that 
only a small quantity of fluid is withdrawn. Headache, sickness and 
faintness are sometimes complained of, hence it is advisable that the 
patient should rest for an hour after the procedure. A rest of twenty- 
four hours after the operation, advocated by Nissl, appears in the writer's 
experience to be unnecessary. A number of fatal results have been 
recorded after lumbar puncture in cases of intracranial tumor in which 
there was a great increase of intracranial pressure. Almost all these 
fatalities took place before the importance of withdrawing only a small 
quantity of fluid was realized. Nevertheless, lumbar puncture should 
only be undertaken after very careful consideration in these cases. 
Under normal circumstances the fluid emerges drop by drop and is 
collected in a sterilized test-tube. The first few drops are sometimes 
turbid, owing to the fact that a little blood has been collected in the 
lumen of the needle in its passage through the muscles and skin; it is 
therefore advisable to allow this to escape. 
The pressure under which the fluid emerges is of some importance. 
When the pressure is raised, the fluid, instead of emerging in drops, may 
spurt out in a steady stream. Quincke states that the normal pressure 
equals on the average a column of water of 40 to 60 mm., but that it 
can only be regarded as pathological when it is above 150 mm. Krbnig, 
on the other hand, finds the average normal pressure in the horizontal 
posture to be from 100 to 150 mm., while in the sitting position he puts 
the average at 410 mm. According to the latter writer the pressure may 
rise to 700 mm. in pathological states. The pressure is often very greatly 
raised in cases of intracranial tumor and in the various forms of menin- 
gitis. It may also be increased in uremia and in certain of the acute 
infective fevers. According to Quincke moderate increase of pressure 
with severe pressure symptoms points to an acute, while a great rise of 
pressure with slight pressure symptoms indicates a chronic process. 176 
DISEASES OF THE NERVOUS SYSTEM 
Valuable information is often derived from the naked-eye appearance 
of the fluid. Thus in meningeal hemorrhage, and in hemorrhage into the 
ventricles, the fluid may be blood-stained. If such fluid is obtained on 
lumbar puncture, the question arises, Is the color of the fluid to be ex- 
plained by puncture of a bloodvessel during the passage of the needle? 
If the fluid is received in three different tubes, and it is found that the 
tint of the three is the same, probably the blood is not due to the puncture 
(Campbell). Again, when hemorrhage has occurred into the cerebrospinal 
fluid, after the fluid is centrifuged it often retains a yellowish color, 
whereas when the hemorrhage is accidental it presents its normal appear- 
ance (Mathieu). Hematoidin crystals may be found in cases in which 
the hemorrhage is of some standing (Kronig). The fluid may be puru- 
lent; indeed, in some cases thick pus is withdrawn. A purulent cerebro- 
spinal fluid means a purulent meningitis or a ruptured abscess. Even 
when there is a purulent meningitis the fluid obtained by lumbar puncture 
may be free from leukocytes and organisms, no doubt because communi- 
cation between the cerebral and spinal subarachnoid space has been 
interfered with. In abscess the fluid is clear unless a meningitis either 
localized or generalized co-exists. The presence of a turbid fluid does 
not insure a certain diagnosis between general meningitis and a localized 
inflammation of the meninges associated with brain abscess, sinus 
phlebitis, and purulent disease of the labyrinth, for in all of these cases 
leukocytes and bacteria may pass into the cerebrospinal fluid. The 
cerebrospinal fluid in tuberculous meningitis may be quite transparent, 
although it usually shows some turbidity. A yellow coloration of the 
fluid has been observed in jaundice. 
The chemical examination yields some information of diagnostic impor- 
tance. The normal fluid contains a trace of serum globulin, and when 
boiled becomes slightly cloudy. An abnormal excess of the albuminous 
content is met with in acute meningitis, tabes, general paralysis of the 
insane, and active cerebrospinal syphilis. The amount of albumin present 
may be estimated by special methods. Fibrin, although absent from 
the normal fluid, is present in acute meningitis. The reducing substance, 
which is constantly present in the normal fluid and in the examination 
for which at least 10 cc. should be used, is, according to Bernstein, 
always absent in purulent meningitis, while in the tuberculous variety, 
although present in small quantities at the commencement, it disappears 
in the later stages. Considerable quantities of sugar have been found in 
the cerebrospinal fluid in diabetes. Cholin, a product of myelin degenera- 
tion, has been detected in the blood and cerebrospinal fluid by Mott 
and Halliburton in acute degenerative diseases of the nervous system. 
The cytological examination yields valuable data. The same technique 
is to be constantly employed for comparative purposes. That originally 
recommended by Widal, Sicard, and Ravault is as follows: Three cubic 
centimeters of the fluid are withdrawn; this is centrifuged for ten minutes 
in an instrument that revolves 3000 times per minute; the tube is 
inverted for two minutes, the bottom of the tube is then scraped with 
a fine capillary pipette (a vaccine tube does excellently for this purpose), 
the fluid is blown on to a slide, the deposit is allowed to dry in the air, DISEASES OF THE MENINGES 
177 
it is fixed in equal parts of alcohol and ether for twenty minutes, and 
stained with methylene blue, eosin, Jenner's or Ehrlich's stain. Follow- 
ing this procedure it is found that under a magnification of 400 diameters 
the normal fluid shows not more than half a dozen cells to the field. 
If the normal fluid is examined in a counting chamber it is found that 
it does not contain more than two cells to the cubic millimeter. 
The observations of importance in the examination of the film are 
(1) the number of leukocytes, (2) the types of cells present and their 
relative proportions, and (3) the presence of other elements. Both in 
epidemic meningitis and in the group of cases included in the present 
article there is commonly a great excess of cellular elements, and the 
increase is found to be largely due to polymorphonuclear cells. In 
long-standing cases in which recovery is taking place the mononuclear 
cells are found to preponderate. Very exceptionally a fluid containing 
no excess of cells is met with in acute meningitis. 
Tuberculous meningitis differs from the other forms of cerebrospinal 
inflammation in that although the cells in the fluid are increased the 
increase is almost entirely due to an increase in the mononuclear leuko- 
cytes, and may be described as a lymphocytosis. Cases of acute tuber- 
culous meningitis are occasionally met with in which the polymorphonu- 
clear cells are in excess, but these are of rare occurrence. 
In general paralysis of the insane, in tabes dorsalis, and in cerebro- 
spinal syphilis a pronounced cerebrospinal lymphocytosis is almost 
invariable, and is usually most marked in the earlier stages of the disease. 
Thus, Purves Stewart found in eleven cases of general paralysis of the 
insane an average count of 127.5 lymphocytes per field, while in 22 tabetics 
the number in each field averaged 131.4. Very occasionally a case of 
tabes is met with in which there is no lymphocytosis. The presence of 
a lymphocytosis in an individual who has had syphilis points to implica- 
tion of the central nervous system, and is an indication for treatment. 
A pronounced diminution in the number of cells may occur during 
antisyphilitic treatment. In cases of syphilis in which the nervous 
system is not affected there is no cerebrospinal lymphocytosis. A lympho- 
cytosis has also been found to occur in cases of herpes zoster, very occa- 
sionally in cases of intracranial tumor, in chronic alcoholism, and in 
mumps. When present it is to be regarded as an expression of chronic 
meningeal irritation. Tumor cells have been detected in occasional 
cases of malignant tumor of the meninges. 
The bacteriological examination is sometimes of great value. The 
detection of microorganisms enables us to exclude conditions in which 
bacteria are not present, and also to distinguish between the various 
forms of meningitis. In tuberculous meningitis the bacillus may be 
found in the large proportion of cases if only sufficient care is used. 
The pneumococcus, streptococcus, and meningococcus are the organisms 
most often found in meningitis. A number of instances of mixed infec- 
tion have been reported. The Wassermann reaction in the cerebro- 
spinal fluid has come to be regarded as of great importance in the diag- 
nosis of nervous syphilis, for a positive reaction may be obtained in this 
fluid when that in the blood is negative. CHAPTER VI. 
DIFFUSE AND FOCAL DISEASES OF THE SPINAL CORD. 
E. FARQUHAR BUZZARD, M.D. 
1. THE LOCALIZATION OF NON SYSTEMIC DISEASE AFFECTING 
Segmental Localization.-In discussing the various morbid conditions 
which affect the spinal cord, it will be necessary to state on more than 
one occasion that the symptoms produced by a particular disease will 
depend upon the segmental level of the lesion. In order to avoid repeti- 
tion, it is expedient to review the data upon which the diagnosis of the 
segmental level of disease is based. A focal lesion may be regarded as 
one which is limited longitudinally to one, two, or three segments of the 
cord, but which may involve either a part or the whole of the transverse 
area of those segments. A diffuse lesion may be defined as one which 
affects in a confluent or discrete fashion a considerable length of the 
cord, amounting to many segments. In neither case have we to deal 
in this section with morbid processes confined to one or more neuronic 
systems. From a clinical standpoint, when a diffuse lesion involves a 
considerable length of the spinal cord it is often difficult and sometimes 
impossible to do more than define the upper limit of the affected area. 
For this reason a focal lesion limited to a single segment is the most 
convenient proposition upon which to base a discussion of segmental 
localization. 
Each segment of the spinal cord has two chief functions; in the first 
place, it forms a link in the chain of nervous paths which traverse it, 
the conduction of impulses along these paths being dependent upon 
the normal condition of the fibres within the limits of the segment; in 
the second place it is a centre for various activities of motor, sensory, 
vasomotor, trophic, or reflex character, all of which rely upon the integrity 
of the segment, and especially of the gray matter, together with the 
anterior and posterior roots, for their healthy expression. 
The Segment as a Link in the Chain of Nerve Tracts.-The result of a 
lesion affecting the conducting paths depends upon the extent of inter- 
ference with the function of these paths. 
Total Flaccid Paralysis.-If the injury to a spinal segment is so severe 
that there is complete severance of all impulses, a condition of total 
flaccid paralysis supervenes in all parts innervated from the spinal 
cord below the level of the lesion. The motor palsy is associated with 
absolute loss of appreciation of all sensory impressions arising from 
the skin and other tissues supplied by the same region of the cord, and 
with abolition of all tendon reflexes in the same parts, at any rate for a 
THE SPINAL CORD AND CAUDA EQUINA. 
178 PLATE IV 
Areas of Anaesthesia upon the Body after Lesions in the Various 
Segments of the Spinal Cord. 
The segments of the cord are numbered: C I to VIII, D I to XII, L I to V, S 1 to 5, 
and these numbers are placed on the region of the skin supplied by the sensory nerves of 
the corresponding segment. DIFFUSE AND FOCAL DISEASES OF THE SPINAL CORD 
179 
considerable period of time. The loss of tendon reflexes is combined 
with absence of superficial reflexes, with the exception that an extensor 
response may in some cases be obtained on stimulating the soles of the 
feet. It is not quite certain whether such survival of the plantar reflex 
denotes or does not denote an incomplete physiological discontinuity 
of all impulses at the site of the lesion. Visceral reflexes are also inter- 
fered with, and complete incontinence of urine and feces rapidly super- 
venes, although this may be preceded, especially in young subjects, 
by a short period during which reflex evacuation takes place. There 
is cessation of all sexual power or desire, with a tendency to incomplete 
priapism in certain male patients. The skin of the paralyzed parts is 
liable to trophic changes, and if the lesion is acute there is often a period 
of vasodilatation with oedema, which is, however, soon replaced by vaso- 
constriction and a dry exfoliative cutis. In the early stages the muscles 
supplied from the cord below the lesion, although completely paralyzed 
and flaccid, preserve their nutrition and their normal reaction to electrical 
and mechanical stimuli. When the complete transverse lesion is of long 
duration an atrophic process gradually attacks the paralyzed muscles 
which ultimately lose their faradic excitability. 
Spastic Paralysis.-If the interference is less profound, and yet suffi- 
cient materially to affect the conductivity of the chief motor and sensory 
tracts, the flaccid paralysis is replaced by a spastic paralysis of all muscles 
supplied by the cord below the level of the lesion. This spastic paralysis, 
which is sometimes known as "upper motor neuron paralysis," and which 
we attribute to changes in the pyramidal tracts, is characterized by the 
presence of some degree of tonic muscular spasm or rigidity, often asso- 
ciated with a tendency to involuntary reflex spasms of a painful type. 
The muscles retain their nutrition, size, and normal electrical excitability, 
but their increased tone (hypertonicity) offers some resistance to passive 
movements. The amount of voluntary power varies with the severity 
of the lesion, but the relationship of degree of spasticity to degree of 
paresis is one the laws of which are not yet perfectly understood. The 
tendon reflexes of all the spastic muscles are increased and there is a 
tendency to the production of clonus. On the other hand, the superficial 
skin reflexes are diminished or abolished below the level of the lesion, 
with the exception that a plantar response of extensor type is usually 
present on stimulating the sole of the foot, and a cremasteric reflex is 
occasionally obtainable. With spastic paralysis the sensory loss may 
vary from complete insensibility to one in which only very fine tests 
are capable of eliciting some evidence of anesthesia in the skin supplied 
by segments below the level of the lesion. The intensity of anesthesia 
is often not equal all over this area, but further reference will be made 
to this later on. The condition of the sphincters is also variable, the 
slight cases generally showing, after an initial retention, some hesitancy 
or precipitancy of micturition, with constipation of the bowels; the severe 
cases, retention or incontinence of urine, associated with constipation 
and rectal incontinence after aperients. At the same time the sexual 
powers of the male are diminished or in abeyance. 
When spastic paralysis of the lower limbs has been of long duration it 180 
DISEASES OF THE NERVOUS SYSTEM 
tends to the production of certain deformities, of which a pes cavus is 
the most common. Permanent contracture of muscles in the position 
of flexor spasm is another troublesome complication. 
The Segment as a Centre of Activity.-The destruction of the gray 
matter by a focal lesion gives rise to certain phenomena distinct from 
those which result from the destruction of the conducting paths in the 
white matter. Each segment contains anterior horn cells, from which 
originate the anterior root fibres. As a result of the degeneration of 
these ganglion cells the efferent nerve roots degenerate, and the muscles 
innervated by them are paralyzed and rapidly waste. Fibrillary tremors 
or twitchings and a tendency to the formation of contractures are addi- 
tional characteristics of what is known as "lower motor neuron paralysis." 
If the muscles are examined electrically during this process of atrophy 
they are found to undergo the reaction of degeneration, i. e., diminution 
and abolition of response to faradism, together with an altered slow 
response to galvanism. In this respect they differ from the paralyzed 
but spastic muscles supplied by nerves arising from segments below 
the focal lesion, which retain their size and nutrition and present no 
alteration in electrical excitability. The tendon reflexes of the muscle 
affected by the atrophic palsy disappear, as do all the reflexes subserved 
by the segment which is the seat of the disease. In the skin of the 
area innervated by the same segment, vasomotor and trophic changes 
are also to be noted, a moist, sodden surface, with atrophy of nails or 
other structures, being the usual result. Sensibility in the skin area 
from which the afferent fibres of the corresponding posterior root arise, 
is altered, but the anesthesia is less marked than the analgesia and is not 
so complete in the upper part of the root area as it is in the lower and in 
the areas subserved by the segments of the cord below the lesion. A 
zone of hyperesthesia may under certain conditions be present just 
above the anesthetic level. Should the segment involved by the focal 
lesion innervate the anal or vesical sphincter, the latter becomes toneless. 
Speaking generally, the results of a severe transverse or focal lesion 
of the spinal cord are atrophic paralysis of the muscles supplied by the 
segment in which the lesion is situated, together with a spastic paralysis 
of the muscles innervated by segments below the level of the disease. 
Alteration of sensibility is present as high as the area supplied by the 
affected segment. In order to apply the above data to the localization 
of a spinal lesion, it is obviously necessary to know which muscles are 
supplied by each segment of the cord, the skin area from which sensory 
impulses reach each segment of the cord, and the situation of certain 
centres subserving visceral and other reflexes. The large majority of 
muscles receive fibres from two or more spinal segments, and a knowledge 
of their peripheral nerve supply is therefore but a poor indication of 
their spinal supply. There is the same discrepancy between the nervous 
and spinal supply in the segmental sensory areas, which are entirely 
different from the areas of distribution of peripheral nerves. In addition 
there are factors in segmental localization which deserve discussion. 
Spinal Root Symptoms.-The recognition that certain symptoms may 
be dependent on a lesion of a spinal root rather tl1U of a spinal segment DIFFUSE AND FOCAL DISEASES OF THE SPINAL CORD 
181 
may be of immense clinical service, especially when the root concerned 
is a large one. It must be remembered that the result of an injury to 
the root may be much the same whether its site is close to the spinal 
origin of the root or at the point where it leaves the neural canal, a differ- 
ence, it may be, of several inches. Root symptoms are sensory, motor, 
reflex, trophic, and vasomotor in character, but it is only by means of 
the sensory and trophic phenomena that any marked distinction can be 
drawn between a lesion of a spinal segment and one which involves its 
anterior and posterior roots. 
In the first place, severe pain of a sharp, stabbing kind, referred to a 
segmental skin area, is suggestive of a root lesion, and this may be asso- 
ciated w'ith some degree of hyperesthesia in the same region. In the 
second place, a destructive lesion of a single posterior spinal root may 
give rise to slight disturbances of sensibility of a certain character. For 
instance, the section of a first dorsal posterior root will produce some loss 
of painful and gross thermal sensibility over a region on the ulnar side 
of the forearm, which is smaller than the whole area supplied by the 
first dorsal root. The slighter degree of tactile anesthesia in the same 
area is due to the greater overlapping in contiguous roots of fibres sub- 
serving touch compared to those which are concerned with painful and 
gross thermal sensibility. From a localizing standpoint the presence 
of herpes zoster within a segmental skin area denotes disease involving 
the corresponding spinal root ganglion, and affords a valuable1 guide to 
the site of the lesion in certain cases in which the spinal column or men- 
inges are affected. Sometimes a subcutaneous hemorrhage or other skin 
affection of similar distribution may appear as an indication of a root 
lesion. Motor and reflex activities are affected in a similar manner 
whether the anterior gray matter of the segment or its efferent roots are 
the seat of disease. 
Oculopupillary Symptoms.-A number of fibres pass down from a 
centre in the brain-stem through the cervical cord in order to join the 
cervical sympathetic system, leaving the cord by the eighth cervical, 
the first and second dorsal nerve roots. In some lesions of the cervical 
cord, but more often in focal lesions of the first dorsal segment, injury 
to these fibres causes diminution in size of the palpebral fissure and of 
the pupil of the same side. In such instances of sympathetic miosis 
and ptosis the pupil may not actively dilate to shade or to the influence 
of cocaine. Irritation of the same system of fibres produces a reverse 
condition of mydriasis and exophthalmos. 
Optic Neuritis.-In lesions of the cervical and upper dorsal cord the 
occurrence of a mild and occasionally of a severe degree of papillitis has 
been noted, but its mechanism has not been adequately explained. 
Vasomotor and Secretory Phenomena.-An acute transverse lesion often 
produces temporary cutaneous vasodilatation and hyperidrosis in the 
paralyzed parts, and this may sometimes be associated with oedema of the 
skin, the deeper tissues, and occasionally of the synovial cavities. The 
fact that this is rapidly followed by complete secretory paralysis has been 
utilized by Horsley for the purpose of demarcating the upper limit of the 
affected skin by means of a dose of pilocarpine. A successful result is 182 
DISEASES OF THE NERVOUS SYSTEM 
sometimes obtained in cases of not very recent origin. Paralysis of 
the cervical sympathetic, with hyperemia and hyperidrosis of the 
face and neck, is met with in lesions of the cervical and upper dorsal 
segments. 
Cardiac and Respiratory Symptoms.-Sympathetic fibres for the cardiac 
and pulmonary plexuses leave the upper four segments of the dorsal 
cord; lesions above or involving that region give rise to modification of 
cardiac and probably also of respiratory action, although the latter is 
difficult to estimate on account of the associated interference with the 
innervation of the respiratory musculature. It is important to remember 
that the diaphragm is innervated from the fourth cervical segment, 
and that lesions of that or higher segments affect the thoracic and 
abdominal respiratory muscles. 
Splanchnic Symptoms.-The superior splanchnic nerve arises from 
fibres leaving the dorsal cord from the fifth to ninth segments. Injuries 
to these fibres in their intramedullary course is attended as a rule with 
marked but often only temporary tympanitic distension of the hollow 
abdominal viscera. 
Vesical, Rectal and Sexual Reflexes.-The generally accepted view that 
there are definite centres in the lumbosacral enlargement of the cord 
connected with the vesical, rectal, and sexual reflexes has been strongly 
controverted recently on experimental and other grounds by Muller, 
who maintains that the central mechanism subserving these functions 
is situated in the sympathetic system. It would be out of place to 
discuss here the relative merits of these views, since the question is by 
no means settled; at the same time, the confession must be made that 
some of the clinical phenomena associated with the sphincters in spinal 
and cerebral disease have never received satisfactory explanation. Muller 
experimenting on dogs, found that after extirpation of the lumbosacral 
cord, periodic evacuations of the bladder and rectum were soon established 
after a short period of retention, and that in male animals erection of 
the penis, associated even with ejaculation, was still possible. The 
voluntary muscles of the external anal sphincter and of the higher parts 
of the urethra become toneless and flaccid when the lumbosacral cord 
is destroyed, but if the lesion only isolates this part of the cord from 
the higher centres the striated muscles retain their tone and also their 
reflex action, although it may be in a modified manner. According to 
Muller the lumbosacral cord is only necessary for the conduction of 
sensory impulses from the organs to the brain and for the conduction 
of voluntary impulses to the striated muscles just mentioned. Periodic 
contractions of the non-striated muscles of these parts are only dependent 
on the sympathetic system, just as normal emptying of the pregnant 
uterus will take place in complete motor and sensory paralysis of the 
lower part of the body. It is highly probable that the simplicity of the 
mechanism is complicated in human conditions by the unnatural supine 
position of the patient and the paralysis of other voluntary muscles 
which are normally concerned with the evacuation of the bladder and 
rectum, as well as by the common secondary changes in the vesical 
walls resulting from sepsis. DIFFUSE AND FOCAL DISEASES OF THE SPINAL CORD 
183 
Pain as a Localizing Symptom.-This is a subject of much importance 
and difficulty in actual practice. It is probably true that focal or diffuse 
disease of the cord never gives rise to pain directly, but frequently does 
so indirectly by involvement of the osseous or meningeal coverings or 
posterior nerve roots. For this reason it is obvious that pain is more 
often associated with morbid processes which attack the cord from with- 
out than with those which originate in its own substance. It is necessary, 
however, to distinguish carefully between several forms of pain. A dull 
aching localized pain in the back is often present in cases when the spinal 
cord and theca are undergoing steady, slow compression, or, more rarely, 
when an intramedullary growth is causing a focal expansion of the cord 
of considerable size. This form of pain may be increased by movement 
of the vertebral column, and it may spontaneously undergo paroxysmal 
exacerbations of a burning character. It is generally referred to a point 
only slightly below the level of the lesion. 
Sharp, shooting pains referred to particular regions, it may be at some 
distance from the spine, are characteristic of disease implicating the 
posterior spinal roots, and their accurate study may be of considerable 
value in determining the seat of injury. These pains may also be excited 
or intensified by movements, especially when the disease actually involves 
the vertebrae. In considering these root pains two important facts must 
be borne in mind; in the first place, the region to which the pain is referred 
will correspond to the cutaneous area supplied by that root; in the second 
place, the area of referred pain will be the same whether the root is 
affected near its medullary origin or in its course through the interverte- 
bral foramen, this being of great significance when the root is one of 
considerable length. In the latter case, associated symptoms will aid 
in localizing the injury to the root more exactly. Care must be exercised 
in investigating the character of so-called girdle pain, and also of the pain 
which is associated with flexor spasms. Girdle pain is in some cases 
a root pain distributed through one of the belt-like dorsal segmental 
areas; in others it is a sense of constriction, which is often marked at 
the upper level of spasticity and anesthesia. Pain and cramps, associated 
with flexor spasms of the hips and knees, are generally referred to the 
front of the hip and back of the knee, and must not be confounded with 
the pain originating from injury to the lumbrosacral roots. 
Disturbances of Sensibility.-Hitherto the disturbances of sensibility 
in transverse lesions of the spinal cord have been merely referred to as 
complete or incomplete anesthesia to various stimuli, extending over 
all parts of the body innervated from the cord below the site of disease. 
This may be sufficient when the lesion is really transverse, and influences 
equally all afferent paths as they traverse the affected segment, but it is 
totally inadequate to describe the various forms of sensory loss which 
may arise in connection with less complete and more patchy focal lesions, 
and especially those which are associated either with disease of the central 
gray matter or with unilateral morbid processes. For the proper under- 
standing of such conditions reference must be made to the work of Head 
and Thompson on the grouping of afferent impulses within the spinal cord. 
Head, in conjunction with Rivers, had previously shown that the 184 
DISEASES OF THE NERVOUS SYSTEM 
afferent mechanism in the peripheral nervous system consists of three 
systems: 
1. Veep Sensibility.-This conveys impulses excited by pressure and 
by all movements of joints, tendons, and muscles. Painful impulses 
derived from excessive pressure are also carried by this system. The 
patient in whom this system is intact is not only able to recognize move- 
ments of joints, but also the locality of the stimulus and the direction of 
the movement. The fibres which conduct these sensory impulses run 
mainly with the muscular nerves, and are not destroyed by division of 
all the sensory nerves to the skin. 
2. Protopathic Sensibility.-This system is capable of responding to 
painful cutaneous stimuli and to the more extreme degrees of heat and 
cold. The appreciation of these stimuli is diffuse and inexact as to the 
locality of the spot stimulated. 
3. Epicritic Sensibility.-To this system is due the power of perceiving 
and locating light touches, of discriminating between two points applied 
simultaneously to the surface, and of recognizing the finer grades of 
temperature, called cool and warm. 
Head and Thompson have shown that the impulses of these three 
different systems combine in new groups very soon after they enter the 
spinal cord. Some impulses cross to the opposite side immediately, 
others cross after running a short course on the same side, and others 
ascend to the upper extremity of the cord entirely on the side of their 
entry. This re-arrangement of afferent impulses and their rough position 
in their spinal paths may be briefly summarized in the following manner: 
1. Impulses of pain, whether excited by cutaneous stimuli or by 
excessive pressure, run together in the spinal cord and cross, probably 
early, to the opposite side. 
2. Impulses of temperature of all degrees are combined, cross to the 
opposite side, and are closely associated, but not intermingled, with 
those of pain. Impulses of heat are also separated from those of cold. 
3. Impulses excited by light touch and by pressure and those which 
subserve their localization accompany each other, cross to the opposite 
side, probably less rapidly and less completely than those of pain and 
temperature, and ascend in a path which is distinct from that of the latter. 
4. Impulses subserving the sense of passive position and movement 
are associated with those of tactile discrimination (compass points) 
in their ascent of the cord on the same side as their entry. These impulses 
probably pass up the posterior columns. 
5. Of the non-sensory afferent impulses, many ascend in the secondary 
system of the direct cerebellar tract to reach the cerebellum. 
Head and Thompson then state their belief that the recombination of 
afferent impulses into new groups takes place on the side by which they 
enter the spinal cord, and that all those impulses which cross to the 
opposite side are carried by systems of fibres belonging to a secondary 
or intramedullary level. On the other hand, the impulses subserving 
the sense of passive position and movement and of tactile discrimination 
remain in a system belonging to the primary or peripheral level in their 
ascent on the same side of the cord. DIFFUSE AND FOCAL DISEASES OF THE SPINAL CORD 
185 
In the light of their observations, the symptom complex which is 
known by the name of Brown-Sequard's paralysis may now be considered. 
He observed that a hemisection of the spinal cord was followed by motor 
paralysis and loss of muscular sense on the side of the lesion, and by loss 
of other forms of sensibility at the level of the lesion on the same side, 
and below the level of the lesion on the opposite side of the body. Well- 
defined local lesions of one-half of the spinal cord are of rare occurrence, 
but modified forms of Brown-Sequard's paralysis are not infrequent 
and those which have been examined bear out in the main the conclusions 
Fig. 13 
Diagram illustrating the grouping of afferent impulses within the spinal cord according to 
Head and Thompson. 
of Brown-Sequard. With improved methods of examination in relation 
not only to sensory, but to motor and reflex disturbances, a perfect 
hemisection of the cord should produce a condition which may be graphic- 
ally represented in the diagram (Fig. 14). 
If an exception be made for those phenomena marked "uncommon" 
or "not constant" in the diagram, the remaining symptoms present a 
picture which is by no means rare in focal diseases of the spinal cord. 
Apart from the sensory disturbances connected with unilateral lesions 
of the cord, there are one or two other points of interest. It is by no 186 
DISEASES OF THE NERVOUS SYSTEM 
means rare to find that the loss of cutaneous sensibility below an incom- 
plete transverse lesion of the cord is not equal in ah the skin areas which 
are affected. In the first place it may happen that when pressure is 
slowly applied to the spinal cord at the level, for instance, of the second 
dorsal segment, the sensory loss in the early stages may only be traced 
as high as the mid-dorsal segmental areas, or may only be complete up 
to that level and relative in those above that level. This is readily 
understood when it is remembered that the long secondary afferent 
paths lying in the periphery of the cord are first affected by pressure, and 
that the decussation of sensory impulses is a gradual process not completed 
in less than four, five, or six segments. 
Fig. 14 
In the second place, when the upper limit of sensory loss is clear and 
well defined, and corresponds to the level of the lesion, it may be found 
that some of the lower sacral areas have escaped the anesthesia of sur- 
rounding regions. Such a phenomenon can only be explained, as Head 
points put, by a lamellar arrangement of the segmental impulses as they 
pass up the spinal cord. 
Diagnosis of Disease of the Cauda Equina.-The cauda equina is 
contained within the vertebral canal from the second lumbar vetebra 
to the coccyx, losing a pair of roots at every intervertebral space on either 
side. In certain cases there arises a difficulty in distinguishing between 
lesions of the lumbosacral segments and those of the cauda equina. 
Certain principles must be remembered in this connection. In both 
segmental and root lesions the symptoms may be similar, the distribution 
of motor and sensory loss being of the segmental type. A lesion of a DIFFUSE AND FOCAL DISEASES OF THE SPINAL CORD 
187 
lumbar segment, however, produces symptoms relating not only to that 
segment, but to each segment below it, while a lesion of a lumbar root 
may be distinct from any involvement of the roots or segments above 
or below. 
It is clear that all the sensory and motor symptoms are of the spinal 
root type in lesions of the cauda equina, and that the presence of any 
spasticity, any increase in the deep reflexes, clonus, or an extensor plantar 
response, negatives the diagnosis of disease limited to that region. All 
muscles which are paralyzed are also wasted, have undergone the reaction 
of degeneration, and have lost their tendon reflexes. When the sphincters 
are involved, as they frequently are, they are relaxed and incontinent. 
Pain is nearly always present, is of a stabbing character, and is referred 
to a root area where, as a rule, it is ultimately replaced by anesthesia. 
It must be remembered, however, that there is sometimes a stage in 
which pain and anesthesia are co-existent in the same area. Modifica- 
tions in the sensibility of the affected parts follow the rules indicated 
in lesions of the peripheral nervous system, and do not conform to those 
of the spinal cord as laid down by Head and Thompson. Additional 
assistance in locating the lesion in the cauda equina may sometimes 
be derived from physical signs pointing to disease of the second lumbar 
or lower vertebne. 
II. THE DIFFERENTIATION BETWEEN THE INTRA- AND EXTRA- 
MEDULLARY ORIGIN OF FOCAL DISEASE. 
It not infrequently occurs that, the level of a particular spinal lesion 
having been diagnosed, it is necessary to come to a conclusion as to 
whether the lesion is produced by a morbid process originating within or 
without the cord. The decision of this question may, for instance, 
have an important bearing upon treatment and may indicate or contra- 
indicate the performance of a laminectomy. 
The early incidence of pain, and particularly root pain, to the characters 
of which reference has been made, is strong evidence of the extramedullary 
origin of the disease. This kind of pain may be increased by movement, 
and then suggests some involvement of the vertebral column, or it may 
be associated with local tenderness on pressure and even with deformity, 
which point in the same direction. Hyperesthesia in the cutaneous 
root area, followed by anesthesia of a dissociative type, is also found in 
extramedullary lesions in their early stages as the result of injury to a 
posterior spinal root. 
On the other hand, an atrophic paralysis of the muscles innervated 
from one or two segments preceding spastic paralysis at lower levels, 
raises suspicions of a primary intramedullary disease, especially if pain 
has been insignificant or absent during its development. In addition 
to these differences in local effects, an intramedullary may be distinguished 
from an extramedullary lesion in some instances by its remote effects. 
When symptoms below the level of the lesion point for a long time to 
the unilaterality of the latter, an intramedullary cause must be regarded 
as the more likely. Pressure from without may and does cause unilateral 188 
DISEASES OF THE NERVOUS SYSTEM 
symptoms, but usually for a short time only, as the effect of compression 
can, naturally, not long be limited to one side of the spinal cord. Early 
sensory loss, especially of a dissociative type, in regions innervated from 
below the level of the lesion, favors the intrinsic origin of the disease, 
and syringomyelia affords the most striking example of this condition. 
With regard to the action of the sphincters no definite rules can be 
laid down. Intramedullary disease, even of the cervical region, may 
sometimes produce bladder troubles before any disturbance of motion 
or sensation, but this is not a common phenomenon. With extramedul- 
lary disease the vesical and rectal symptoms are usually first noticed 
when the paralysis of one side is being followed by that of the other, or, 
in cases where the lesion is bilateral from the beginning, as soon as the 
legs show signs of weakness. In difficult cases skiagraphy may throw 
some light on the question at issue. 
In conclusion, it must be admitted that none of these rules are without 
exceptions, and that in some instances there is insufficient evidence to 
give a definite opinion without the aid of laminectomy. 
COMPRESSION OF THE SPINAL CORD. 
The possible causes of compression paraplegia are numerous. Fract- 
ures, dislocations and other injuries of the vertebral column belong to 
surgery rather than to medicine, and will not be dealt with here. Inas-r 
much, however, as the lesion produced by such injuries is of the focal 
transverse type, its symptomatology at any level can be readily deduced. 
The following are the chief causes of compression paraplegia: (1) 
Fracture dislocation and injuries of the vertebral column. (2) Spinal 
caries. (3) Newgrowths of (a) the vertebral column and surrounding 
tissues; (6) of the meninges; and (c) of the cord or its roots. (4) Aneurism 
of the aorta. (5) Parasites, particularly hydatids in the extrathecal 
space. (6) Arthritis deformans of the spinal column. 
Spinal Caries or Pott's Disease.-Etiology.-The tubercle bacillus is 
necessarily the most important factor, and spinal caries is often associated 
with tuberculosis of other organs, of which pulmonary tuberculosis is 
one of the most common. Tuberculosis of the vertebrae occurs most 
frequently in children and young adults. It may exist at birth, and 
has been seen in the fetus in utero. Injury is often said to precede the 
onset, and it is possible that a traumatic lesion may determine the site 
of infection or excite a latent focus. 
Pathology.-The favorite site of origin for tuberculous disease of the 
vertebne is in the spongy substance of the bodies, especially in their 
anterior halves. Occasionally the first focus is in the cancellous tissue of 
the laminae or, still more rarely, in the smaller articulations of the spinal 
column. From its origin in the body of the vertebra there is a tendency 
to invade the neighboring intervertebral cartilages and vertebrae and 
to extend posteriorly through the bone and periosteum, so as to invade 
the extradural space. Having thus reached a region of very limited 
resistance and considerable vascularity, fungoid granulations spread 
upward and downward as well as laterally, and finally may attack, DIFFUSE AND FOCAL DISEASES OF THE SPINAL CORD 
189 
and even, in rare cases, penetrate, the dura mater. If an abscess has 
formed within the bone, it tends to force its way backward or forward. 
In the latter case the pus will find a path along the anterior surface of 
the column beneath the anterior common ligament, and may give rise 
eventually to a psoas, a retropharyngeal, or other form of "gravity" 
abscess. In the former case it encounters the posterior common liagment, 
on one or both sides of which it projects into the lumen of the neural 
canal. In other instances the pus may find its way into the perispinal 
tissues, and present itself near the surface in the form of a lumbar abscess. 
It is the rule to find some degree of bony deformity; the collapse of one 
or more diseased bodies produces generally a kyphosis, a kyphoscoliosis, 
or, occasionally, only a lateral curve. It is obvious that a narrowing of 
the lumen of the canal may be occasioned (1) by the ingrowth of fungoid 
granulation tissue, (2) by the intrusion of an abscess, or (3) by displace- 
ment of bone. As a matter of fact, more than one of these causes may 
be at work at the same time. In those rare cases in which an acute 
compression of the cord is induced, either spontaneously or as the result 
of some slight injury, it is usually dependent upon a sudden bony displace- 
ment still further narrowing a canal which has already been largely 
encroached upon by granulation tissue. 
The injury to the cord is not, however, always a result of compression 
or of compression alone. According to Schmaus, the most important 
factor bringing about a loss of conductivity is an oedema of the medullary 
tissue. This is a consequence of the strangulation of bloodvessels, 
especially veins, in the surrounding granulation tissue. In the first place 
the chief effect is upon the myelin sheaths, but later, if the condition 
persists, the axis cylinders and nerve cells degenerate, and ultimately 
become destroyed and replaced by proliferated neuroglia. This is the 
process which earned at one time the name of compression myelitis, 
but which is more appropriately referred to passive congestion and 
oedema. Less commonly interference with the arterial supply to a segment 
or segments of the cord invokes a true evascularization and ischemia, 
and still more rarely the tuberculous process penetrates the dura and 
sets up a tuberculous meningomyelitis. The latter may remain localized, 
but occasionally a general tuberculous meningitis follows. Very uncom- 
monly a tuberculous intramedullary neoplasm may be discovered post- 
mortem in the neighborhood of the caries. 
Dry caries, or caries sicca, is the name used to denote that form of the 
disease in which a rarefying osteitis, rather than abscess or granulations, 
is the most conspicuous feature. The condition usually proves to be an 
infective myelitis or meningomyelitis rather than true compression. 
The macroscopic changes in spinal caries are very variable, and the 
cord, in particular, may be found in widely differing states. In one 
case it will be severely compressed, reduced to a thin ribbon consisting 
mainly of thickened membranes and sclerosed tissue. In another the 
caliber may be less diminished, but the consistence, that of cream, 
indicates the necrotic results of circulatory interference. In another 
there may be no evidence of compression, but an area, embracing a 
segment or two, in which swelling and oedema are the most conspicuous 190 
DISEASES OF THE NERVOUS SYSTEM 
features. Lastly, there are the instances of acute compression, displaying 
the bruising, softening, and hemorrhage usually associated with fracture 
dislocations of the vertebral column. Before alluding to the secondary 
changes it may be mentioned that central cavities of the cord, extending 
some distance beyond the site of the focal lesion, have been recorded. 
The spinal roots may be, and very frequently are, directly involved 
in the granulation tissue. On the other hand, the anterior roots may 
be atrophied as a result of the changes taking place in the central gray 
matter. Ascending and descending tracts of degeneration can be traced 
above and below the focus of disease in the cord, if it is of sufficiently 
long standing, and resemble those found in other transverse lesions of 
the same organ. Not infrequently a diffuse spinal caries may lead to 
compression at more than one level, in which case the resulting degenera- 
tions are all the more complicated. 
Symptoms.-Caries of the spine is most common in the dorsal, less 
common in the lumbar, and comparatively rare in the cervical vertebrae 
and sacrum. On the other hand, it is probable that paralytic cord 
symptoms are more often associated with cervical than with lumbar 
caries. In the majority of cases the first symptoms are those which are 
referable to the disease in the bones, although the results of pressure 
upon a spinal root, or upon the cord itself, do occasionally manifest 
themselves before the process in the vertebral column has been suspected. 
Localized pain referred to one part of the spine is usually the first symp- 
tom, and has certain characteristics. It is of a dull, boring kind, and 
is accentuated by movement, especially by pressure applied deeply and 
directly to spinous processes at its seat. It may also be increased by 
lateral movements exerted on the part involved, or by jarring the column 
as a whole. Localized rigidity of the spine next deserves attention. In 
early cases this is due to inhibition of painful movements, and is induced 
by a subconscious muscular fixation of the part. In later cases mechanical 
conditions due to displacement of bone or involvement of ligaments 
may take a share in the production of immobility. The rigidity may 
be apparent in every movement of the patient, especially when the 
cervical spine is the seat of disease, or only when he attempts to stoop. 
In other cases it is only observed in the course of a careful examination. 
Next in importance is the presence of a deformity. An acute angular 
curvature denotes a less extensive involvement of vertebrae than a more 
rounded kyphosis. The most prominent spinous process belongs usually 
to the vertebra immediately below that which has collapsed. The 
deformity varies from a slight prominence of one spinous process to a 
malformation in which the greater part of the vertebral column is directly 
or indirectly involved. A certain amount of scoliosis is a common 
accompaniment of, or sequel to, the kyphotic curve, and is often only of 
secondary origin. On the other hand, scoliosis may be the only form of 
curvature, in which case it will be distinguished from those of muscular 
or rachitic origin by the presence of some rigidity and by the failure to 
reduce it on stooping. Congenital malformations are only to be excluded 
by the use of skiagraphy. Deformities may be completely lacking, 
even in those cases of spinal caries which are associated with marked DIFFUSE AND FOCAL DISEASES OF THE SPINAL CORD 
191 
symptoms of paraplegia, but severe root pains should suggest the possi- 
bility of vertebral disease in spite of the absence of all curvatures. 
When a spinal root is involved certain symptoms are wont to appear. 
Of these, the most important are root pains, which must be carefully 
distinguished from the localized pains of bony origin. A root pain is 
generally sharp or stabbing in character, or may only amount to a constant 
sense of pressure'or tightness. In the case of the limbs the pain is often 
described as shooting into the periphery; in the case of the trunk it 
may give rise to one form of girdle sensation. If any anesthesia is present 
as the result of destruction of a single posterior root, it is of narrow 
limits, and affects the painful and thermal sensibility rather than the 
tactile. It is very unusual for this to be detected in the trunk areas. 
On the other hand, an eruption of herpes zoster may suggest that a spinal 
root ganglion has not escaped injury. When the anterior root fibres 
are involved, some atrophic paresis of certain muscles may be noted, 
but this again is slight when only one root is affected, and may be over- 
looked altogether on the trunk. 
In the usual order of clinical sequence, the root symptoms are followed 
by those which are referable to the spinal compression or to those lesions 
of the spinal substance which are at any rate induced by the surrounding 
disease. These are identical with those of any focal lesion of the cord, 
and are fully described elsewhere, for instance, in the discussion of trans- 
verse myelitis. The signs vary with the level of disease, and are referable 
in the first place to the destruction of certain spinal centres at the site 
of the lesion, and in the second place to the interference with the con- 
ductivity of efferent and afferent tracts. It is impossible to distinguish 
the signs of destruction of spinal centres from those of the destruction 
of corresponding roots, and these have already been described. In the 
case of cervical and upper or middorsal caries a varying degree of 
spastic paralysis is found below the level of the spinal compression, with 
a certain amount of sensory loss and of sphincter trouble. 
Since the compression is usually of gradual onset, it is well to point 
out that the earliest evidence is usually to be detected in the examination 
of the lower extremities. The patient may complain of being easily 
tired in walking or of a tendency to drag one or both feet, and the observer 
will find slight weakness of ankle dorsiflexion, brisk knee- and ankle-jerks, 
and extensor plantar responses. These early signs may precede any 
anesthesia, and may or may not be associated with precipitate or hesitat- 
ing micturition. When compression destroys all conductivity in the cord, 
either after a long time, or as the result of a sudden collapse in rare cases, 
a flaccid paraplegia will result, and then the deep and superficial reflexes 
also disappear. Toneless relaxation of the sphincters will also eventuate, 
as well as complete loss of all sensibility as high as the segmental area 
corresponding to the spinal lesion. 
When the compression involves the lumbosacral region of the cord, 
a mixture of atrophic palsy of certain muscles, with spastic paresis of 
others, forms the clinical picture on the motor side. The sensory disturb- 
ance follows the distribution of the lumbosacral roots, and the vesical 
and rectal difficulties are those which are associated with paralysis of 192 
DISEASES OF THE NERVOUS SYSTEM 
their respective sphincters. The preservation of the patellar and Achilles 
tendon jerks depends on the integrity of the third and fourth lumbar 
segments on the one hand and of the fifth lumbar and first sacral segments 
on the other. The plantar reflex is only obtainable if the lesion extends 
no lower than the first sacral segment, but is of the extensor type. 
Trophic or vasomotor disturbances are common when the spinal 
lesion is severe, at whatever level it is situated. Sexual impotence 
results from all but the slightest degrees of compression, and priapism 
is occasionally observed in the dorsal and cervical cases, with more or 
less complete paraplegia. Compression of, or above, the fourth cervical 
segment interferes not only with the intercostal, but also with the 
diaphragmatic respiration, and lesions of the upper dorsal cord are fre- 
quently responsible for distension of the hollow abdominal viscera as 
the result of splanchnic palsy. 
Some reference should be made to the mode of onset and the course 
of the spinal symptoms. Except in rare cases in which a sudden luxation 
causes severe bruising or destruction of the cord, the onset of paralysis 
is slow, taking many days, more often weeks or months, to reach its 
height. The advent of paralysis may, on the one hand, rapidly succeed 
the symptoms of vertebral disease, or, on the other, may take place 
after the latter have been in existence for years. It may be said that 
no case of spinal caries, of whatever duration, is free from the risk of 
compression paraplegia. 
Pyrexia of slight degree, with daily variations, is often present, but 
the disease sometimes runs an afebrile course, or rapidly becomes afebrile 
under rest in bed and other appropriate treatment. 
The course must necessarily vary with the treatment, the nature of 
the morbid process underlying the spinal lesion, and the severity of the 
damage, but the condition must always be regarded as chronic, one 
which is slow to develop and slow to disappear, even when the issue is 
favorable. Remissions and exacerbations are well known to occur, and 
may have no obvious relation to the progress of the bone disease. A 
trauma may induce a rapid accentuation of paralysis, or the spontaneous 
evacuation of an abscess may be associated with marked improvement. 
When a favorable turn is taken, the sensory disturbances usually disappear 
before the motor. In the unfavorable cases death may result from 
decubitus, cystitis, pyonephritis, or from tuberculosis of other organs. 
An intermediate course generally means a chronic spastic paraplegia, 
the length of life depending largely upon the circumstances and surround- 
ings of the patient. The occurrence of gravity abscesses is of surgical 
rather than medical interest, but it is well to recollect that a psoas or 
lumbar abscess may be the first indication of vertebral disease, and may 
indicate the origin of an otherwise unexplained paraplegia. 
Diagnosis.-From the point of view of diagnosis cases of spinal caries 
may be divided into two classes: (1) Those in which spinal deformity is 
present, and (2) those in which the spinal column displays no obvious 
signs of disease. 
1. When the curvature is extensive and prominent the diagnosis is 
usually easy and straightforward, but slight kyphotic curves are not DIFFUSE AND FOCAL DISEASES OF THE SPINAL CORD 
193 
uncommon in vertebral carcinoma and aortic aneurism. Prominence 
of a spinous process is, moreover, occasionally met with in intra vertebral 
tumor originating in the meninges or extrathecal space. 
Vertebral carcinoma is suspected when malignant disease is, or has 
been, present in other organs, especially in the breast. In such cases 
pain, although severe, is less often limited to one particular spot, and 
examination of the spinal column will probably reveal a loss of the normal 
curves and a general flatness, which may be combined with some diminu- 
tion in the patient's height. The age must be taken into account when 
either carcinoma or aneurism is being considered, and in the latter disease 
assistance may be forthcoming in the shape of the signs of intrathoracic 
or abdominal disease. A skiagram may be of the greatest value. 
The presence of a scoliosis without kyphosis does not exclude caries, 
but other causes must be eliminated. The reduction of the curve on 
extending the spine in the act of stooping, and the absence of rigidity 
in carrying this out, point to the common forms of scoliosis seen in children 
and young adults. In such cases there is no paralysis, but a well-marked 
scoliosis may be seen in syringomyelia, and be associated with spastic 
paraplegia. The characteristic sensory and trophic disturbances of 
syringomyelia will serve, as a rule, to differentiate it. Congenital abnor- 
malities of the vertebral column may simulate spinal caries, but a skiagram 
indicates the true condition. 
2. When no spinal deformity is present the inquiry is much more 
difficult. In the first place, it must be definitely ascertained that the 
lesion is a focal one and that there are no signs of any disease above a 
particular level. Having decided that the entire motor and sensory 
disturbance can be referred to a single focal lesion, and having thus 
excluded all the diffuse or disseminate scleroses of the cord, the diagnosis 
will rest between spinal caries, the various forms of intra- and extramedul- 
lary tumors, and other, more rare, causes of spinal compression. The 
differentiation between these conditions must depend, not upon the 
character and extent of the paralytic phenomena, which may be seen 
in any one of them, but upon considerations of a wider and accessory 
character. Tenderness and rigidity of the spinal column at a level 
corresponding to the signs of compression will, in young children, forcibly 
suggest vertebral caries. In other persons the same symptoms may be 
found in connection with vertebral or intravertebral tumors as well 
as with caries, whether it be of the moist or dry variety. A gumma or 
local syphilitic meningomyelitis must also be excluded. Here, again, 
the use of skiagraphy may clinch the diagnosis. It is usually stated 
that the root pains are more severe and precede by a longer period signs 
of compression in cases of tumor than in cases of spinal caries, but this 
is not the universal rule. On the other hand, paralysis more often com- 
mences unilaterally when a tumor is the cause of compression than when 
spinal caries is at work. The detection of tuberculosis in other organs 
must favor the presumption of spinal caries, but in a considerable number 
of cases the certain diagnosis must be arrived at by an exploratory lamin- 
ectomy. This should not be delayed too long in doubtful cases, and it is 
still more important to remember that the diagnosis of a chronic myelitis 194 
DISEASES OF THE NERVOUS SYSTEM 
to explain slow progressive signs of paraplegia is never admissible except 
in certain rare syphilitic cases, which can generally be excluded by the 
results of appropriate treatment or by examining the cerebrospinal 
fluid. 
The pain complained of by neurasthenic patients is often referred to 
the spine, which may be exquisitely tender. It is rare, however, for 
the pain and tenderness to be constantly localized at a particular level, 
and examination generally shows that the tenderness is more superficial 
than deep. Local rigidity is not observed in these cases, although the 
patient may hold the spinal column stiffly as a whole. 
The differentiation between a true compression paraplegia and a 
functional paraplegia may sometimes require attention. In the functional 
cases the legs are usually stiff and extended, without tendency to flexor 
spasms. The weakness is general throughout all the groups of muscles 
instead of being more marked in the dorsiflexors of the ankles and flexors 
of the knees, as it is in all cases of spastic paraplegia of organic origin. 
The characteristic interference with the sphincters is absent in the 
functional cases, and, although the tendon jerks are generally exaggerated 
and a form of clonus of irregular rhythm may be elicited, the plantar 
responses are either absent or flexor in type. That these points are of 
importance is evident when the possibility of a functional paraplegia 
supervening in a spinal caries is remembered. 
Prognosis.-There are few general rules which can guide one in giving 
a prognosis in any particular case of spinal caries which is complicated 
by paraplegia. Youth favors the outlook and age adds to the gravity. 
Oppenheim gives statistics from Billroth's clinic which show that out 
of 97 patients suffering from caries, 48 died, 22 were cured, and 11 were 
dismissed as incurable. Speaking generally, the more complete the 
paralysis the more serious is the case as regards life, on account of the 
complications which arise in connection with decubitus, cystitis, etc. It 
may be admitted that the cervical cases are more dangerous, on account 
of the interference with the respiratory musculature, than many of 
those in which the mid- or lower dorsal region is involved, but the writer 
has been favorably impressed by the power of recovery exhibited by 
some cervical cases, especially in young subjects. Tuberculosis in other 
parts naturally adds to the danger, but treatment may have as good 
results upon one focus as upon another, and the presence of pulmonary 
tuberculosis, for instance, is not an insuperable obstacle to recovery. 
Gowers truly says that there is no disease of the cord in which symptoms 
of equal gravity so often pass aw'ay. 
Treatment.-This is a question which may provoke considerable 
diversity of opinion, especially in regard to the advantages and disad- 
vantages of operative interference. 
Preparalytic Prophylaxis.-The earlier the disease of the vertebrae is 
recognized and treated, the less likely are cord symptoms to supervene. 
This is an axiom of importance, but it is not equivalent to a statement 
that paraplegia will not develop in cases which have had the advantage 
of early and good treatment. As soon as caries of the spine has been 
diagnosed the patient should be placed on his back, kept at complete DIFFUSE AND FOCAL DISEASES OF THE SPINAL CORD 
195 
rest for many months, and the general treatment for tuberculosis should 
be carried out. 
Rest and Extension -In certain cases of spinal caries in which para- 
plegia has supervened, especially in young children, the adoption of 
simple rest will suffice to restore power to the paralyzed limbs in a com- 
paratively short length of time. In others recovery will ensue after 
many months, even when no other remedial measures are superadded. 
The application of extension to the vertebral column may, and often 
does, make all the difference in those cases in which rest alone has proved 
of no avail. Extension should certainly be tried in all cases where 
displacement of bone is obvious and recent, but it is often useless and 
adds to the discomfort of the patient when the bony deformity is of 
long standing. It is probably more serviceable in cervical than in dorsal 
caries, and is of little value when the lumbar spine is the seat of disease. 
In all cases in which rest alone or rest and extension are being used every 
precaution must be taken against bedsores. 
The employment of plaster, poroplastic, and leathern jackets cannot 
satisfactorily replace recumbency in bed, but is necessary as a supple- 
mentary measure in order to maintain immobility for a time after recovery 
has taken place. In very young children the use of bandages and splints 
may be necessary to insure the requisite rest. Massage, passive move- 
ments, and perhaps electricity may be requisitioned while the patient 
is in bed in order to improve and maintain the nutrition of the paralyzed 
limbs, so long as no undue movement is imparted to the trunk. 
Operative Treatment.-This should be regarded as a supplementary 
measure, one not to be undertaken lightly and not to be regarded as 
replacing rest and open-air treatment. It may be resorted to before 
rest has been tried, when there are indications that no relief of pressure 
can be procured by the latter, or it may be employed when a course of 
rest has failed to produce any amelioration in the paraplegia. The 
evacuation of any superficial abscess must be carried out at once, and a 
deep abscess, if within reach, should be subjected to the same treatment, 
particularly when it is suspected of causing compression symptoms. On 
the other hand, a deep-seated abscess may sometimes dry up without 
surgical interference under favorable conditions. Laminectomy should 
not be postponed until secondary changes in the spinal cord render the 
relief of pressure ineffectual in restoring power to the paralyzed parts, 
and, speaking generally, it should be undertaken earlier in adults than 
in children. 
It is probable that surgical interference would enjoy a greater popularity 
if the cases were chosen with greater care and submitted to the surgeons 
at an earlier period. The indications and contra-indications for lamin- 
ectomy as usually cited are not to be relied upon. For instance, it is 
often stated that the involvement of several vertebree excludes the use 
of the knife. The writer has had an experience which directly contravenes 
such a view. A child, aged three years, with cervical caries, after five 
months of rest, during which period symptoms of paraplegia continued 
to progress, was operated on by I). Armour. A large abscess was evacu- 
ated, and what appeared to be the necrosed remains of several vertebral 196 
DISEASES OF THE NERVOUS SYSTEM 
bodies were extraeted. The abscess cavity was washed out and the wound 
closed. Within four months the child, with a shortened neck, was run- 
ning about without a sign of paralysis and without any need of artificial 
support for her head. 
In spite of the success which attends operation in some suitable cases, 
it must be remembered that spinal caries is not a condition with regard 
to which it may be said that an operation can do no harm. There are 
instances in which this course has appeared to excite the disease into 
renewed activity, and others in which a fatal issue from tuberculous 
meningitis has rapidly followed. It may justifiably be hoped that the 
use of skiagraphy will encourage and promote that careful selection of 
cases which is necessary to bring this treatment into the position it 
deserves. Equally important is it that emphasis should be laid on the 
after treatment, so that each may enjoy for a prolonged period the rest, 
fresh air, and good nourishment which are essential for satisfactory 
results. Finally, no hesitation should be felt in advising laminectomy 
when the patient's life is seriously threatened by increasing respiratory 
embarrassment, as is not infrequently the case in caries of the higher 
spinal regions. 
TUMORS OF THE SPINAL CORD. 
For the sake of convenience, although at the expense of terminological 
accuracy, the title "Tumors of the Spinal Cord'' is here, as often elsewhere, 
intended to include all tumors which habitually modify the functions of 
the spinal cord by pressure upon or invasion of that organ, as well as those 
which originate in its substance. The classification of spinal tumors 
followed by Bruns may be adopted here, but it is necessary to explain 
that the word tumor embraces, in this connection, granulomata and 
parasitic cysts as well as neoplasms proper. On the other hand, spinal 
gliomatosis is reserved for the article on syringomyelia. 
I. Tumors which, originating in its envelopes, secondarily affect the 
spinal cord. 
(a) Vertebral tumors arising from the spinal column or the soft 
tissues immediately surrounding it. 
(6) Intravertebral tumors, which may be divided into two classes 
in accordance with their relation to the dura mater. 
1. Extradural tumors originating in the periosteum of the 
vertebrae, the outer layers of the dura mater, or the fatty 
areolar tissue of the epidural space. 
2. Intradural tumors originating from the inner layers of the 
dura mater, the arachnoid, the ligamentum denticulatum, 
the spinal roots, or the pia mater. 
II. Intramedullary tumors of intrinsic spinal origin. 
Vertebral Tumors.-The bones of the spinal column, especially their 
bodies and less commonly their arches and processes, are frequently the 
site of malignant growths. 
Carcinoma in this region is always secondary and generally metastatic, 
although occasionally the vertebrae are directly involved by extension of DIFFUSE AND FOCAL DISEASES OF THE SPINAL CORD 
197 
disease from other organs. The primary focus may be in the uterus, 
stomach, intestine, prostate, thyroid, lung, kidney, or gall-bladder, but 
most commonly, by far, is in the mammary gland. This special connec- 
tion with mammary carcinoma and perhaps uterine cancer is sufficient 
to account for the greater incidence of the disease in females, although 
males are not exempt. Probably 70 or 80 per cent, of cases occur in 
women. The age which predisposes to vertebral carcinoma is that of 
carcinoma of other organs. Considering the rarity of bronchial carcinoma, 
secondary vertebral disease from this source is relatively frequent. 
The lumbar and dorsal parts of the column are more often involved 
than the cervical, and the disease may be limited to one or more foci or 
spread diffusely over many vertebrae. It is not uncommon to find the 
spine much more extensively affected after death than was suspected 
during life. The neoplasm is found to attack first the spongy portions 
of the bone, to destroy the medulla, and to fill the medullary spaces. 
The more compact bony cortex may survive as a thin shell for a time, 
but is ultimately also replaced by the expanding growth. Along with 
osseous resorption, a certain amount of calcification in portions of the 
neoplasm is not infrequently observed. When this osteoplastic process 
is excessive a compact and firm ankylosis of a large part of the vertebral 
column may ensue, and there may be a complete absence of any osseous 
deformity. On the other hand, it is more common for the softening 
produced by the infiltration of a spongy growth to allow the body weight 
to effect a general shortening of the column, a condition which is some- 
times described as "entassement." When the softening is local it is 
often described as carcinomatous caries, and, like the tuberculous variety, 
may lead to collapse and the formation of a curve like that of Pott's 
disease. In this manner the spinal cord may be compressed, but is 
only affected in comparatively few cases, the spinal roots being much 
more prone to suffer close to or within the intervertebral foramina. 
Nodules of carcinoma projecting from the inner surface of the vertebral 
bodies or arches may exert pressure upon the cord, and very occasionally 
the tissue of the epidural space becomes invaded by growth, with the 
result that the cord is surrounded by a kind of neoplastic cylinder. Very 
rarely does the disease penetrate the theca. 
Sarcoma of the vertebral column or of the soft tissues immediately 
surrounding it may be primary or secondary, may form a single well- 
defined tumor, or be diffused as multiple growths. The lumbar and 
sacral regions appear to be the favorite seat of the disease. The results 
of the infiltration of the vertebral column resemble those of the carcino- 
mata, and may comprise collapse and luxation, the formation of curva- 
tures, and the compression of nerve roots or of the spinal cord. The 
spinal cord compression is usually brought about by the presence of 
growths in the epidural space, but occasionally by the penetration of 
the dura along the course of a spinal root, and the formation of masses 
upon the surface of the pia. The secondary sarcomata of the spinal 
column either extend directly from the neighboring tissues or are metas- 
tatic deposits from more distant parts. The writer has seen a renal 
sarcoma invade the vertebral column along many of the spinal nerves, 198 
DISEASES OF THE NERVOUS SYSTEM 
and evoke symptoms both of root involvement and of compression 
paraplegia. Metastatic sarcoma is a rare growth in the spinal bones; 
according to Schlesinger secondary invasion of the vertebral column is 
most common when the primary sarcoma is of osseous origin. 
Myeloma occupies an intermediate position between the malignant 
and benign tumors of the vetebrse. Multiple myelomatosis is usually 
characterized by a wide infiltration of the bony tissue of many vertebrae, 
rendering large extents of the column soft and pliable, with the result 
that deformities are easily produced by the influence of weight or position. 
Less often one or more masses of growth are found in the form of definite 
tumors. Other bones, especially the ribs, sternum, clavicle, etc., are 
frequently involved at the same time. The spinal cord may suffer 
from compression, in consequence of deformities leading to narrowing 
of the neural canal or from a nodule of growth projecting from the internal 
surface of a vertebra. Myelomata are not invariably soft throughout; 
occasionally they indulge in ossification and productions of ivory-like 
character. The disease is confined to the osseous system, and affects 
males more often than females. 
The benign tumors of the vertebral column are very rare, and comprise 
osteomata and exostoses, chondromata and osteochondromata, myxo- 
mata, the so-called luxuriant callus formations, and the bony excrescences 
associated with arthritis deformans. The bony tumors only affect the 
nervous system when they grow in such a way as to encroach upon the 
neural canal or the intervertebral foramina. The cartilaginous growths 
may penetrate the intervertebral foramina and produce root palsies and 
sensory symptoms. The occasional erosion of the spine by aneurism 
of the aorta or by hydatid cysts may be mentioned, but it is still an open 
question whether the latter ever originate primarily from the bony 
envelopes of the spinal cord. 
Intravertebral Tumors.-1. Extradural.-Tumors of the epidural space 
are more often secondary than primary. Sarcomata and lipomata share 
the reputation of being the most common varieties, but fibromata, 
myxomata, chondromata, are also very occasionally seen in this situation. 
It is only rarely that the malignant growths penetrate the dura mater. 
Hydatid cysts are found most commonly in this region when they give 
rise to spinal symptoms. 
2. Intradural.-These originate from the inner layers of the dura mater, 
the arachnoid, the ligamentum denticulatum, the spinal roots, or the pia 
mater. Sarcomata may be diffuse or localized. The former variety, 
which may be termed pial sarcomatosis, is usually of the round-celled 
type, and characterized by its tendency to envelop more or less extensive 
portions of the spinal cord in a kind of cylindrical covering. It is a 
growth of the leptomeninges, and is usually less luxuriant on the ventral 
than on the dorsal and lateral aspects of the cord. Not infrequently it 
may cover the whole length of the cord and cauda equina and penetrate 
even into the cranial cavity. In some instances it never actually invades 
the substance of the cord; in others it infiltrates that organ as well as 
the spinal roots. In spite of the absence of direct invasion by the neo- 
plasm the cord is often softened owing to the interference with its vascular DIFFUSE AND FOCAL DISEASES OF THE SPINAL CORD 
199 
supply and perhaps to some degree of pressure. More rarely secondary 
growths from sarcomata of the vertebrae, the pleura, or peritoneum gain 
access to the subdural space and extend in a longitudinal direction upon 
the surface of the cord. Localized circumscribed sarcomata are also 
found in this situation and tend to exert mechanical pressure upon the 
spinal marrow as well as upon spinal roots. Endotheliomata arising 
from the theca, cylindromata (Billroth), psammomata, fibromata, 
lipomata (Gowers), lymphangiomata (Schlesinger) are less common. 
On the other hand, fibromyxomata or fibrosarcomata are frequently 
found in connection with the nerve roots. These are often single, but 
neurofibromata are generally multiple, and may be associated with more 
or less neurofibromatosis of the peripheral nervous system. 
Such vascular growths as angiosarcoma and true angioma are observed 
now and then within the dura, and cysticerci as well as echinococci are 
also recorded as present in the same cavity. Intradural tumors are 
usually situated laterally or posterolaterally, rarely on the anterior 
surface of the cord. The latter is often displaced to one side and com- 
pressed or indented. In the region of the cauda equina there is room 
for greater development in lateral dimensions than in the higher regions, 
and neoplasms may here reach a considerable size with relatively few 
symptoms. 
3. Intramedullary Tumors.-These comprise gliomata, sarcomata, angio- 
sarcomata, as well as tubercles and gummata. Cysticerci are rarely 
found in the substance of the cord. Gliomata are usually diffuse and 
conform to the type which is discussed under syringomyelia, but they are 
occasionally circumscribed, producing the ordinary changes and symptoms 
of an intramedullary growth. The sarcomata, solitary tubercles, and 
gummata arise in the first instance from the pial septa, and are more 
often single than multiple. Cases in which gummata have been found 
in the brain, in the cord, and on several spinal roots, are described. 
Pathological Processes in the Nervous Tissues Resulting from the Pressure 
of Spinal Tumors.-Meningeal tumors rarely occasion more than slight 
erosion of the bony parts, but their effects upon the nervous structures 
within the vertebral column are important. The spinal roots, although 
more resistant than the spinal cord, are usually the first to suffer. They 
may be directly involved in the growth, in which it is sometimes difficult 
to trace their course, or they may be compressed as they pass through 
the dura or even the intervertebral foramina. Degenerative changes 
follow in either case, and may be traced in the efferent fibres in their 
peripheral, and in the afferent fibres in their intramedullary course. 
The spinal cord may suffer in different ways. In most cases a certain 
amount of displacement is the first result, and this is followed by compres- 
sion. The early effects of the latter may be only mechanical, but sooner 
or later they lead to interference with the circulation and the production 
of oedema and ischemia. Venous stasis has usually been regarded as an 
important element in producing the oedema, and the arterial and capillary 
blood supply is modified in consequence of the latter. Superficial arteries 
may also be compressed, thus contributing to the local anemia. The more 
specialized nervous structures, particularly the ganglion cells, are the 200 
DISEASES OF THE NERVOUS SYSTEM 
first to show signs of nutritional change, and the myelin sheaths of the 
conducting tracts readily undergo degeneration. More pronounced 
degrees of ischemia lead to areas of softening, which may be associated 
with sclerotic changes in the neuroglia if the pressure is slowly exerted. 
The presence of secondary degeneration above and below the site of 
compression denotes definite changes in the axis cylinders at that level. 
Similarly degeneration of the anterior root fibres follows the atrophy 
of the cells of the ventral gray matter. The cord may be softened without 
direct compression, owing to the interference with the arteries, veins, 
and lymphatics on the surface. Direct infiltration of the cord by extra- 
medullary tumors is less frequent than might be supposed, the pia-arach- 
Fig. 15 
Gumma of third lumbar segment. 
noid affording a certain amount of protection. Intramedullary growths 
may be sharply defined or may present little in the way of differentiation 
from the nervous matter. Those which are circumscribed are often 
surrounded by an area of softening, and occasionally by evidences of 
inflammation or of toxic oedema. 
Incidence.-Schlesinger has shown that tumors affecting the cord are 
by no means common, and says that in 35,000 necropsies only 147 
instances were recorded. 
Age.-Starr collected 100 cases of intra vertebral tumor, and found 
that 70 occurred above and 30 under the age of fifteen years. The 
following table represents the age incidence in 82 cases collected by 
Armour from the records of the National Hospital (London). DIFFUSE AND FOCAL DISEASES OF THE SPINAL CORD 
201 
Vertebral 
Vertebral 
Spinal 
Medul- 
Intra- 
Extra- 
Year. 
carcinoma. 
sarcoma. 
tumors. 
lary. 
dural. 
dural. 
Both. 
Under 10 . 
. . 0 
0 
1 
1 
0 
0 
0 
11 to 20 . 
. . 0 
4 
10 
2 
3 
4 
1 
21 to 30 . 
. . 0 
1 
11 
3 
5 
3 
0 
31 to 40 . 
. . 1 
3 
12 
4 
5 
3 
0 
41 to 50 . 
. . 6 
1 
17 
2 
10 
4 
1 
51 to 60 . 
. . 2 
2 
5 
1 
4 
0 
0 
61 to 70 . 
. . 3 
1 
1 
0 
1 
0 
0 
71 to 80 . 
. . 1 
0 
0 
0 
0 
0 
0 
- 
- 
- 
- 
- 
- 
Total 
. . 13 
12 
57 
13 
28 
14 
2 
Nature and Site.-Of 400 cases collected by Schlesinger, 126 were 
intramedullary and 239 extramedullary. The extramedullary included 
151 intradural and 88 extradural growths. 
Cervical .... 
Medullary. 
Intradural. 
5 
Extradural. 
0 
Both. 
1 
Cervicodorsal 
0 
1 
1 
0 
Dorsal .... 
4 
13 
10 
1 
Dorsolumbar 
0 
1 
2 
0 
Lumbar .... 
3 
2 
o. 
0 
Cauda equina . 
0 
6 
1 
0 
Sacrum .... 
0 
0 
0 
0 
Diffuse .... 
1 
0 
. o 
0 
- 
- 
- 
Total 
13 
28 
14 
2 
Symptoms.-These can be conveniently divided into three groups. 
The first comprises those associated with disease of the bone, the second 
those which indicate involvement of the spinal roots, and the third those 
referable to derangement of the functions of the spinal cord. 
Vertebral Symptoms.-The earliest symptom is pain referred to some 
part of the back, dull, boring, or burning in character, and liable to 
exacerbations, either spontaneous or the result of pressure, movement, 
or concussion. Conscious of the increased suffering, which is excited 
by movements of the trunk or head, and particularly by those which 
involve rotation of the vertebral axis, the patient holds himself stiffly 
and avoids exposing himself, as far as he is able, to the possibility of a 
sudden jar. On examination the observer will detect limitation of the 
normal mobility of one or more parts of the spine, and will easily elicit 
evidences of tenderness in the same regions. Inspection and palpation 
of the back and even of the anterior surface of the sacrum by rectum 
may fail to reveal any mass, especially in carcinoma. On the other 
hand, obliteration of the normal spinal curves, shortening of the vertebral 
column, or difficulty in distinguishing the spinous processes by palpation, 
may afford indications. The most striking examples of loss of height 
are seen in vertebral carcinoma or myeloma, and may be inferred from 
the statement of the patient or from the obvious collapse and conden- 
sation of certain parts of the column. A narrowing of the normal space 
intervening between the lower ribs and the iliac crests, and the resulting 
prominence given to the abdomen, may afford some indication of this 
" entassement." While obliteration of natural curves is marked in some 
cases, the formation of abnormal kyphoses is equally characteristic of 202 
DISEASES OF THE NERVOUS SYSTEM 
others. A sharp-angled curvature is generally acquired, more or less 
rapidly, when the body of a vertebra becomes so softened as no longer 
to be able to bear the weight of those above. More rounded kyphosis 
is the result of a general and diffuse infiltration such as is seen in cases 
of multiple myelomata. 
The degree of prominence to which bone symptoms attain in any 
particular case varies considerably. On the one hand, pain and tender- 
ness of the back, deformities, and curvatures may monopolize attention 
throughout the course. On the other hand, pain and paresthesia referred 
to the extremities and disturbance of general health may fail to attract 
particular notice to the spinal column, and very extensive disease of 
that part of the skeleton runs the risk of escaping detection. 
A word is necessary upon the occurrence of vertebral symptoms in 
instances of intravertebral tumor in which the bones escaped involvement. 
It is not rare to observe limitation of movement, some local tenderness, 
and even the prominence of a spinous process beyond its neighbors; the 
occurrence of these physical signs must not be allowed to weigh too 
heavily against the diagnosis of a meningeal growth. 
Spinal Root Symptoms.-It is desirable to utter a word of warning 
against placing the symptoms and signs of the spinal tumors derived 
from this source in too important a position. From a positive point of 
view their occurrence is valuable, but it is unwise to hold that the absence 
of any phenomena associated with disease of the spinal roots in any way 
negatives the existence of a spinal tumor. Pain is by far the most 
common of root symptoms, and yet a painless or almost painless develop- 
ment of paraplegia is met with sufficiently often in intravertebral neoplasm 
to prove that this symptom has sometimes been held in too high esteem. 
The pain associated with disease of the posterior roots, whether in 
their intrathecal or intravertebral course, is usually severe, neuralgic, 
stabbing, and referred to the peripheral distribution of the afferent 
fibres. It may be unilateral or bilateral, and in the latter case may give 
rise to a painful form of girdle sensation. Exacerbations may be caused 
by movement, especially when the root is involved by grow'th in the 
intervertebral foramen, which is most frequent in vertebral carcinoma 
or sarcoma. The skin of the areas in which pain is felt is often hyper- 
esthetic, and later may become anesthetic and analgesic, although pain 
persists (anesthesia dolorosa}. Appreciable anesthesia and analgesia 
are rarely evident unless two or more consecutive roots are interfered 
with, although some loss of thermal and painful sensibility may be 
detected in the case of single root lesions in connection with the upper 
or lower limbs. When the spinal root ganglion is involved an eruption 
of herpes zoster may appear in the corresponding root area. 
The destruction of the anterior root fibres gives rise to physical signs 
which are difficult to separate from those of compression of the cord 
itself, and which often make their appearance at a time when the latter 
organ has begun to suffer. Thus, abolition of function in a single anterior 
root does not, as a rule, give rise to complete palsy or complete atrophy 
of any muscle, but a few muscles, partially innervated by the root 
involved, present some paresis, some wasting, partial reaction of degen- DIFFUSE AND FOCAL DISEASES OF THE SPINAL CORD 
203 
eration, and possibly fibrillary twitchings such as are seen in cases of pro- 
gressive muscular atrophy. These signs are much more readily detected 
in the limbs than in the trunk, where the overlapping of muscular root 
innervation is much more pronounced. When two, three, or more 
adjacent anterior roots are affected, the paralysis and wasting is more 
or less complete in certain muscles and partial in others, the contractures 
usually associated with lower motor neuron palsy following in the ordinary 
course of events. It is hardly necessary to add that the reflexes subserved 
by the affected spinal roots are abolished. Cases of tumor of the cauda 
equina afford the most striking examples of multiple root lesions uncom- 
plicated by symptoms of spinal cord origin. 
Spinal Cord Symptoms.-Sooner or later the neoplasm gives rise to the 
symptoms of a focal lesion of the spinal cord. In vertebral carcinoma 
death may occur before any compression has taken place, but in the 
majority of instances of intravertebral growth the spinal symptoms 
engross most attention before the disease has been very long in progress. 
It is characteristic of spinal tumors that their effects upon the cord are 
progressive, more characteristic, indeed, than the occurrence of pain. 
The symptoms attendant upon transverse lesions and the diagnosis of 
their level have already been fully discussed, but a few points, bearing 
upon the onset and course of spinal tumor, must be referred to here. 
With vertebral tumors signs of compression are mostly bilateral from 
the beginning, with intravertebral and particularly intradural growths, 
although they are frequently situated to one side of the cord, the symp- 
toms of paralysis are rarely unilateral for long. On the other hand, 
inequality in degree of disablement of the two sides is common enough, 
and it is not always the side first affected which is later the more pro- 
foundly paralyzed. True unilateral lesions of considerable duration are 
more commonly met with in cases of intramedullary tumors, and their 
symptomatology is that of Brown-Sequard's paralysis. 
In .extramedullary growths motion is usually affected before sensation, 
and the earliest symptoms of compression paraplegia in cases of tumor 
above the lumbosacral enlargement are easily induced fatigue in walking 
and a tendency to drag the toes. Examination at this time will probably 
reveal some paresis in the dorsiflexors of the ankle and flexors of the knees, 
as well as increased tendon jerks and the extensor type of plantar reflex. 
These signs may, however, only be found on one side at first. So long 
as the symptoms are unilateral there is rarely any interference with 
the sphincters, but the extension of the effects of compression to the 
other side is attended by precipitancy or hesitancy in micturition. 
Increasing spasticity and paresis in the muscles innervated from the 
segments below the site of disease mark the advance of pressure until a 
time comes when the lesion amounts to a complete physiological section; 
a flaccid paralysis then replaces the spasticity, tendon and superficial 
reflexes disappear, and the sphincters become quite incontinent. 
Similarly, on the sensory side paresthesia in the periphery of the lower 
extremities-a vague numbness or coldness of the feet-precedes the 
development of definite diminution of sensibility. When the latter is 
established it involves all forms of sensibility, perhaps in unequal degree, 204 
DISEASES OF THE NERVOUS SYSTEM 
and spreads rapidly upward toward the level of the disease. For some 
time the upper limit of diminished sensibility may correspond to a 
segmental area some distance below that of the site of compression, the 
decussation of tactile, painful, and thermal impulses being gradual, and 
their supradecussational paths lying more exposed to the effects of 
pressure than the more central areas in which the crossing takes place. 
It is common, however, to find that, while diminution in cutaneous 
sensibility only reaches a certain level, between that level and the area 
innervated from the segment which is being compressed, stimuli, tactile 
or painful, may be felt by the patient as altered in character, although 
they are perfectly appreciated. The more nearly the lesion approaches 
completeness, the more closely does the loss of sensibility approximate 
upward to the area subserved by the segment which is on the verge of 
physiological disintegration. Emphasis is laid upon this fact, because 
it is often of supreme importance to estimate accurately the upper limit 
of sensory change in order to localize the site of compression. In other 
cases, in which the afferent root is compressed, or the posterior gray 
matter suffers early at the level of the tumor, the local anesthesia in the 
corresponding segmental area is sufficient to guide the observer to a 
correct estimation of the height of the lesion. 
Sensory loss in cases of intramedullary growth tends to exhibit special 
features. Its early appearance, its frequent unilateral distribution, and 
dissociative characters are among the most important of these. The 
sensory phenomena associated with Brown-Sequard's paralysis have been 
described, but a duplication of these may sometimes be caused by a 
central tumor, with the result that the local and remote areas of dissocia- 
tive anesthesia are met with on each side of the body at the same time. 
Although this is characteristic of syringomyelia, it is not limited to that 
disease, and may occasionally be found with medullary tumors other 
than gliomata. 
Upper Cervical Region.-Tumors in this region tend to produce a 
picture of spinal hemiplegia or double hemiplegia which resembles in 
many respects that of pontine or cerebral origin. The absence of symp- 
toms pointing to involvement of the cranial nerves and the local signs 
in the region of the neck usually separate the two conditions. In these 
cases pain is referred to the neck and to the region of the occiput, extend- 
ing as high as the vertex, and is followed by the development of hemiplegia 
involving the arm, leg, and trunk on one side, less commonly by bilateral 
motor paralysis. In a case seen by the writer, pressure, exerted by a 
tumor at the level of the foramen magnum, caused hemiplegia on one 
side, shortly followed by a similar condition on the other, the side last 
affected being the more disabled at the time the patient came under 
observation. Although usually regarded as a dangerous site, in relation 
to life, it is worthy of note that the patient in this instance had suffered 
for over three years from symptoms referable to the presence of the 
growth. More profound paralysis of the respiratory musculature would 
have brought about an early fatal termination. 
Cervical Enlargement.-Owing to our fairly precise knowledge of the 
spinal motor and sensory innervation of the upper extremities, tumors 205 
DIFFUSE AND FOCAL DISEASES OF THE SPINAL CORD 
of the cervical enlargement can generally be readily recognized. An 
atrophic paralysis of the arm, with spastic paralysis of the parts below, 
associated with sensory disturbances and sphincter troubles, all steadily 
progressive, are characteristic. Oculopupillary symptoms and grave 
respiratory paresis may often be noted in the same connection. 
The Dorsal Region.-The chief difference between tumors of the dorsal 
cord and those of other levels lies in the absence of well-marked atrophic 
palsies as local symptoms at the level of the lesion. This is partly due 
to the overlapping of the muscular spinal innervation, but still more to 
the difficulty in distinguishing clinically how far separate sections of a 
large muscle supplied by many spinal segments are in a state of functional 
activity, or how large a share of a movement, such as inspiration, is 
carried out by each separate muscle, such as an intercostal, concerned 
in it. These difficulties are not so great if a careful examination is made, 
and there are certain points which are helpful. Paralysis of the lower 
half of the rectus abdominis is readily demonstrated by the upward 
movement of the umbilicus in the attempt to rise from a supine to a 
sitting posture. Palsy of the lower intercostals may be recognized if the 
hands of the observer are placed over the lower ribs during respiration, 
especially if the patient is not too well covered with fat. Perhaps the 
accurate testing of the superficial trunk reflexes from segment to segment 
affords more dependable evidence of the level of the lesion than any of 
the motor phenomena, but the accomplishment of this requires consider- 
able experience and is often difficult in obese subjects. If, for instance, 
the skin reflexes are obtained on either side above the umbilicus and 
are absent below that level, the indication is in favor of a lesion reaching 
as high as the tenth dorsal segment, and this may be confirmed by the 
movement of the umbilicus already referred to. On the whole, the 
disturbances of sensibility offer, in most cases of tumors of the dorsal 
cord, the most reliable indication of the height to which the disease has 
attained. Apart from the questions involved in their segmental locali- 
zation, tumors of the dorsal cord present no special clinical features if 
exception is made for the splanchnic palsy, occasionally associated with 
pressure on the upper half of this region. The distension of the abdomen 
in these cases is often only temporary, but, while it lasts, may be produc- 
tive of great discomfort and respiratory embarrassment, as well as of 
troublesome constipation. The sphincter troubles and the tendency to 
decubitus depend directly on the severity of the lesion and the amount 
of interference with the sensory tracts. 
The Lumbosacral Enlargement.-The short length of this part of the 
spinal cord and the fact that it can be easily exposed by the removal of 
two or three laminae minimizes the necessity for the accurate segmental 
localization which is so necessary in cases of tumor of other regions. 
On the other hand, it is most important to distinguish between lesions 
of this enlargement and those of the cauda equina. 
Pains and atrophic muscular paralysis of the regions innervated by the 
lumbar plexus, sensory disturbances over the whole of the lower extremi- 
ties, absence of patellar jerks and presence of ankle clonus and extensor 
responses, together with retention of urine and feces, form a symptom 206 
DISEASES OF THE NERVOUS SYSTEM 
complex characteristic of compression of the lumbar segments and roots. 
When the lesion also involves the sacral segments, the atrophic paralysis 
and anesthesia are more extensive, and absence of all reflexes in the legs 
is combined with incontinence of the sphincters. Under these circum- 
stances the ultimate picture may closely resemble that of a tumor of the 
cauda equina, and the diagnosis may depend upon a consideration of the 
development of the symptoms. A Brown-Sequard paralysis is occasion- 
ally met with in cases of lumbar tumor, more often with intra- than 
extramedullary growths. 
The Cauda Equina.-It must be admitted that tumors, apart from 
injuries, form the large majority of morbid conditions of the cauda 
equina. The first symptoms of some neoplasm attacking the lumbosacral 
roots is nearly always pain in one lower limb. The supervention of 
anesthesia and of atrophic muscular palsy and the gradual spread of 
these three signs in that order, from one part to another, together with 
the early involvement of the opposite side in a more or less symmetrical 
manner, point with certainty to a gross organic basis for the disease. 
The loss of sphincter control when the third and fourth sacral roots 
begin to suffer, and the abolition of the various reflexes, with the spread 
of the morbid process to the radicular paths, upon the integrity of which 
they depend, follow in the natural course of events. The question as 
to whether the growth is of vertebral or intravertebral origin is sometimes 
difficult to answer, but the solution may possibly be arrived at by a 
careful examination of the lower lumbar vertebrae and sacrum, supple- 
mented by investigation of the pelvis. 
Contra-indications to the diagnosis of tumor of the cauda equina are 
afforded by the presence of a Brown-Sequard's paralysis, of any spasticity, 
exaggeration of tendon reflexes or extensor type of plantar reflex. It 
may be mentioned here that neither the presence nor absence of fibrillary 
tremors, nor the character of the electrical reactions of the muscles, 
can be relied upon for the purpose of distinguishing between an affection 
of the anterior gray matter and of the anterior roots. 
Course and Result.-With few exceptions the course of spinal tumors 
is gradually progressive and their result fatal. The rapidity with which 
symptoms reach their greatest intensity differs considerably with the 
nature and seat of the growth, and the relative duration of the pre- 
paralytic and paralytic stages may present wide variations. Rare 
instances of apoplectiform paraplegia are afforded by the collapse of 
diseased portions of the spinal column and by the development of acute 
"myelitic" changes in the cord at the seat of slight pressure, but in the 
first cases the morbid condition of the bones has usually made itself 
obvious by other symptoms, and in the others premonitory signs of 
compression have afforded some clue to the presence of a tujnor, even 
if local or root pains have been absent. 
With regard to duration a sharp distinction may be drawn between 
cases of vertebral and intravertebral growths. In the former, whether 
of carcinomatous or sarcomatous origin, the inevitable fatal issue is 
generally reached within nine months; in the latter, life may be prolonged 
for two or two and one-half years, and in some instances for much longer DIFFUSE AND FOCAL DISEASES OF THE SPINAL CORD 
207 
periods. Isolated nodules of sarcoma, fibroma, or psammoma have 
been known to produce symptoms over eight, fourteen, and even twenty- 
five years. Remissions and exacerbations cannot be said to be marked 
or even usual, but, they are not by any means of great rarity. 
The primary intravertebral tumors are hardly ever responsible for 
metastases in other parts; it is less uncommon for intracranial growths 
to be associated with secondary deposits in the spinal cord or on the 
spinal roots. 
The mortality depends on the almost inevitable supervention of 
decubitus, cystitis, and renal disease, except in those cases in which 
respiratory paralysis leads to a rapid death. 
Diagnosis.-Three important questions have to be answered in this 
connection. In the first place, the differentiation between a spinal 
tumor and some other spinal or nervous diseases has to be established. 
In the second place, the precise situation of the neoplasm must be ascer- 
tained. Thirdly, the nature of the growth is a matter of some moment 
when the line of treatment has to be decided upon. 
1. The Diagnosis of Spinal Tumor.-This is not so difficult a task in 
the case of vertebral tumors, because, on the one hand, these are frequently 
secondary, and the presence or history of another growth may indicate 
the nature of the disease, and, on the other, spinal caries is almost the 
only morbid process with which they may be confounded, if an exception 
is made for vertebral aneurism and hydatids. No absolute rules can 
be laid down, but in a general way caries of the spine is associated with 
more marked curvatures and gibbosities, with less prolonged, less severe, 
and perhaps more symmetrical root pains, and often with definite skia- 
graphic appearances. Vertebral tumors are characterized by less sharp 
curvatures and more condensation of the spine, by prolonged and severe 
pains, often asymmetrical, and by a more rapidly progressive course 
and cachexia. 
Tuberculosis in other organs must always be looked for. In their 
early stages vertebral tumors are often overlooked because pain may be 
the only symptom and physical examination may yield no further help. 
To neurasthenia or "rheumatism" must not be too hastily assigned 
persistent pains in patients who are obviously suffering, but it is not 
often that a definite decision can be arrived at until some more objective 
evidence is available. 
With intravertebral growths there are certain diagnostic points of 
primary importance. The fact that all symptoms can be ascribed to a 
single lesion at a particular level in the cerebrospinal system serves to 
exclude such diffuse diseases as disseminate sclerosis, in which paraplegia, 
sensory and sphincter disturbances may have a more or less gradual 
and painless onset. When everything points to a single localized lesion, 
the fact that the onset is slow and the course of symptoms progressive 
practically excludes all forms of spinal disease except a tumor, a local 
meningeal cyst, and some rare forms of spinal syphilis. With the excep- 
tion of the latter there is no form of chronic myelitis which can simulate 
the insidious onset or the progressive course of a compression paraplegia. 
The rare cases of vertebral caries without deformities or evidence of 208 
DISEASES OF THE NERVOUS SYSTEM 
osseous disease must be remembered, although it is often impossible 
to diagnose these without exploration. Mention has just been made of 
local meningeal cysts with the object of emphasizing the difficulty of 
their differentiation from spinal tumors, this being the greater on account 
of our ignorance of their pathogenesis. That they are a real and not 
infrequent source of error is proved, although the mistake is of academical 
rather than practical or therapeutic importance. 
Hypertrophic cervical pachymeningitis may suggest the early stages of 
an intravertebral neoplasm, but the limitation of the symptoms to those 
of root or meningeal origin points in the direction of the true diagnosis, 
which may often be confirmed by the favorable course. 
As a matter of fact, there are few conditions with which a spinal tumor 
can be confounded if we exclude caries and meningeal cysts, and if we 
remember that a tumor produces a local lesion and that the onset of 
symptoms is slow and their course steadily progressive. 
When multiple tumors of the meninges or roots exist, there is usually 
much difficulty in forming a true estimate of the amount and situation 
of the spinal damage. Multiple neurofibromata and multiple sarcomata, 
although located on the roots, often evoke symptoms of spinal cord 
compression before giving rise to anything very definite in the way of 
radicular palsies or disturbances of sensation. The symptom complex 
may be so mixed in degree and height that a true conception of the 
anatomical condition is impossible. 
2. The exact situation of the tumor must be gauged from the data 
which have been given, but attention may be directed to certain general 
principles. In estimating the highest level of damaged spinal marrow 
the opinion must be arrived at by a careful comparison of the results of 
examination on the motor, sensory, and reflex sides, helped by any 
information which can be gained from investigating the spine of the 
patient manually, visually, or by the .r-rays. The most minute changes 
in sensibility should be noted, especially in case of tumor of the dorsal 
region, and when a segmental area shows well-marked anesthesia it 
should be concluded that at least one spinal segment above that by which 
the area is innervated is, in all probability, severely damaged. Thus, 
if definite anesthesia can be traced as high as the seventh dorsal segmental 
area, the sixth dorsal segment is compressed, and the removal of the 
fourth and fifth dorsal laminae will expose the seat of disease. The 
highest indication of any disturbance of function is nearly always the 
safest guide to follow. The downward extent of the lesion is generally 
impossible to estimate. 
3. It is hardly ever possible even to hazard a guess as to the histological 
characters of a primary intravertebral tumor until it has been exposed. 
It is therefore unwise to allow the question of its nature to influence in 
any way the consideration of treatment. On the other hand, a vertebral 
tumor, primary or secondary, or a secondary intravertebral growth, 
usually presents evidences or associations pointing to its nature. In the 
great majority of cases the knowledge thus acquired contra-indicates 
the hope of a radical cure. 
The diagnosis between extra- and intramedullary lesions of the cord DIFFUSE AND FOCAL DISEASES OF THE SPINAL CORD 
209 
has been discussed, and the principles there laid down can be applied 
to cases of spinal tumor. It is necessary to repeat the warning that 
there are not infrequent instances in which the question can only be 
positively answered by an appeal to exploration, and that the gravity 
of the outlook justifies that measure in any doubtful case. 
Prognosis.-In the large majority of cases of vertebral tumors their 
malignant character renders the prognosis hopeless and the fatal termina- 
tion a matter of months. With intravertebral growths the outlook 
depends entirely on the possibility of successful removal, the liability 
to recurrence and the condition of the spinal cord at the time the attempt 
to remove them is made. The prognosis must always be grave until 
these questions can be answered. The most favorable cases are those 
of localized benign or comparatively benign tumors in the extradural 
or intradural spaces, and the least favorable those of diffuse or multiple 
extramedullary neoplasms and all those of intramedullary origin. It 
is fortunate that compression of the cord may be prolonged and severe 
without destroying all hope that its functional activity can be restored 
if the source of pressure is removed. 
Treatment.-From the therapeutic point of view cases of spinal tumor 
may be divided into operable and inoperable classes. With those which 
are inoperable remedial measures must be directed toward the alleviation 
of pain and the prevention of complications, such as sepsis, bronchitis, 
pneumonia, cystitis, and pyonephritis. The alleviation of pain may be 
difficult, but less compunction need be felt about the administration of 
narcotics than in diseases with a more favorable outlook. Unfortunately 
the pain produced by pressure upon the afferent roots and the great 
discomfort evoked by flexor spasms of the lower limbs in the stage of 
advanced spastic paraplegia do not always yield even to opium. In 
this contingency the advisability of dividing the posterior roots for the 
relief of the more urgent sufferings may be well considered. 
The possibility of a syphilitic basis for the symptoms of spinal tumor, 
in the shape of an intramedullary gumma or a local gummatous meningitis, 
must not be forgotten, and antisyphilitic remedies should always be 
given a trial in doubtful cases. The examination of the spinal fluid should 
be resorted to for the purpose of setting these doubts at rest. 
A large number of cases of intravertebral tumor are suitable for surgical 
exploration, even if only a small proportion attain a really successful 
result. With our present knowledge it is usually possible to give an 
accurate diagnosis of the site of the lesion, but rarely possible to state 
the nature, size, origin or possibility of removal of the growth. Nothing 
short of exposure of the tumor can clear up these points, and there is less 
harm done by operating on cases of what prove to be ineradicable spinal 
neoplasms than by choosing unsuitable cases of intracranial growth for 
surgical intervention. An opportunity is afforded for relieving pain 
even when the disease cannot be arrested, and the operation in skilled 
hands is attended by no undue risk considering the gravity of the case. 
The surgeon who can stay his hand when a laminectomy exposes a 
hopeless condition will do no harm even if he effects no good. 
This is not the place to discuss surgical details, but one or two points 
vol. v-14 210 
DISEASES OF THE NERVOUS SYSTEM 
may be noted. It sometimes happens that on the removal of two or 
three lamime no tumor is found in the expected situation. The absence 
of pulsation in the theca at this level should encourage the surgeon to go 
higher, and he will probably find the source of pressure by removing 
another arch or two. It is less common for the laminectomy to be 
performed at too high a level, but in such a case the healthy pulsation 
of the theca may suggest the advisability of extending the wound down- 
ward. The removal of several arches is not attended by the disastrous 
results in the way of lack of support which might be expected. 
The after treatment of successful operations is that of convalescing 
myelitis. The restoration of function in paralyzed limbs may be hastened 
by the employment of massage and electricity, but attention to the general 
nutrition and health of the patient will do the rest. 
CAVITIES AND FISSURES OF THE SPINAL CORD. 
Under this title are included a number of conditions of little practical 
importance with the exception of syringomyelia. For some reason we 
are accustomed to term "cavities" in the spinal cord what would, perhaps 
more properly, be termed "cysts" in other organs. At any rate we mean 
by a cavity a space, with or without an organized wall, containing fluid. 
Cavities, continuing the abuse of this term, are the not infrequent terminal 
results of an acute inflammation, in the course of which portions of the 
spinal tissue have become necrosed and ultimately absorbed. An example 
may be found in the cord of a long-standing case of poliomyelitis, just 
as a cyst or porencephaly may represent an old encephalitis. Of very 
similar origin are the cysts or cavities resulting from an old focal hemor- 
rhage or thrombosis. These cavities have no definite lining membranes, 
their walls consisting of neuroglia, which may or may not be compressed, 
so as to give it a condensed laminated appearance. 
The term "fissure" is usually applied to a cavity which is narrow, and 
which may appear narrower after it has lost its fluid contents than it 
did before it was opened. Care must be taken not to regard as fissures 
those spaces which are seen in microscopic specimens as the result of 
hardening processes, and which are particularly liable to be artificially 
formed along the tracts of bloodvessels when the walls have been thickened 
by disease or when the embedding has not been skillfully carried out. 
Hydromyelia.-This name has been variously used, and it would cause 
less confusion if it were confined to cases of simple dilatation of the central 
canal without co-existing alterations in surrounding structures, apart 
from the results of simple stretching, and without clinical symptoms. 
The condition is most frequently found in infants with congenital hydro- 
cephalus, spina bifida, or other developmental abnormalities, but may 
also be present unsuspected in older persons in minor degrees. A satis- 
factory explanation of its production is difficult, but increase of fluid 
pressure due to mechanical causes, or physiological increase of fluid 
output by an overactive ependyma, may be of sufficient account in 
certain cases. The hydromyelic cavity is lined by ependymal cells, 
and its shape is usually round, but sometimes T-shaped, owing to a 211 
DIFFUSE AND FOCAL DISEASES OF THE SPINAL CORD 
diverticulum in the direction of the posterior median septum. The 
addition of some peri-ependymal gliosis or of ependymal proliferation 
with the formation of accessory canals is the first step toward the produc- 
tion of what is termed "syringomyelia," and is evidence of the very 
imperfect line of demarcation separating the two conditions. 
Syringomyelia.-Definition.-A chronic disease of the spinal cord 
characterized anatomically by the existence of one or more pathological 
cavities, and clinically, in its typical form, by the presence of dissociative 
anesthesia and trophic changes in muscles, skin, and bone, together with 
numerous other, but less constant, physical signs. It should be stated 
that Morvan's disease, first described in 1883, and for a time regarded 
as a separate disease, is included in this article only as a variety of syringo- 
myelia. This is justified by the prevailing opinion that there are no 
grounds for putting it in a distinct category. 
Etiology.- Distribution.-Syringomyelia can nowhere be regarded as 
a common disease, although it is now well recognized in all civilized 
nations. There is no reason to suppose that it is not universal in its 
distribution, and yet its literature appears to indicate that it is of more 
frequent occurrence in France and Germany than in America or the 
British Isles. Statistics are wanting, and it may be that the more careful 
investigation of the infirmary class of patients in the first-named countries 
accounts for the apparent difference in numbers. 
Sex.--It is usually stated that men are more frequently affected, and 
this is borne out by an analysis of 40 cases which have been in the National 
Hospital (London). Of these, 25 were men and 15 women. 
Age.-The second and third decades represent the most frequent time 
of onset. In the 40 cases just mentioned the first indication of any 
abnormality was observed on an average, at the age of twenty-four and 
one-half years. Among the males the average age was twenty-eight 
and among the females nineteen, a difference which would probably 
be less marked in a larger series. The latest date of onset (fifty-six) 
was in a man, and the earliest in a girl of seven. 
Heredity.-There is no evidence that heredity plays any important 
part, and, although its occurrence in more than one member of a family 
lias been recorded, it cannot be termed a familial disease. 
Congenital Anomalies.-The not uncommon presence of congenital 
anomalies in the victims of syringomyelia is important. A careful study 
will often reveal some skeletal peculiarities. The patient may be unduly 
small, much smaller than his brothers and sisters; he may be infantile 
in proportion, presenting a large head with small trunk and limbs. 
Another patient may have unusual deformities of the skull; for instance, 
bosses in the occipital or temporal regions; he may have, without other 
signs of acromegaly, very large hands and feet. Pronounced degrees of 
genu valgum or of pes cavus have been present from early infancy in 
cases of the disease, and examination of the spinal column may elicit 
signs of a spina bifida occulta. 
Trauma.-A history of preceding injury must be regarded with judi- 
cious suspicion in all diseases, and syringomyelia is no exception. It is 
safe only to say that more or less severe trauma to the head and spine 212 
DISEASES OE THE NERVOUS SYSTEM 
is by no means an infrequent antecedent, and that considerable impor- 
tance has been attached to this fact. The relationship of spinal hemor- 
rhage to syringomyelia is intimately connected with this question. 
Other Diseases.-Syphilis may be excluded as a direct cause, but its 
influence in producing meningitis and vascular disease must be taken 
into account. The common infective diseases do not appear to have any 
causal relationship, although the occurrence of one or other of them 
may coincide with the development of, or may intensify, its early symp- 
toms. The co-existence of conditions such as tabes, spinal caries, etc., 
is rare and probably unimportant; on the other hand, cases of combined 
acromegaly and syringomyelia have been recorded sufficiently often to 
make the association interesting and worthy of further investigation. 
Pathology.-The exposure of the spinal cord is generally sufficient to 
reveal characteristic changes. The organ is altered, often very irregularly, 
in shape; the cervical region, as a rule, is enlarged and flattened, the 
lateral dimension being proportionately more increased than the antero- 
posterior. The dorsal segments share in the change, or may be narrow 
with irregular thickenings. Usually the lumbosacral enlargement has a 
more normal appearance. The extension upward of the cervical deform- 
ity may cease high up in the cord, may occasionally involve the medulla, 
or very rarely the pons. Folds and furrows are not infrequently observed 
on the surface of the cord, especially if the fluid contents have drained 
away. The meninges, in the majority of cases, present no abnormality. 
Exceptions are found in those cases of spinal cavitation definitely associated 
with pachy- or leptomeningitis (often syphilitic in origin), a consider- 
able number of which have been recorded. The anterior spinal roots, 
particularly those of the cervical enlargement, may be atrophied and of 
a gray translucent color. On palpation the swollen parts are soft and 
usually fluctuate; the narrower regions may be normal in consistence or 
suggest the presence of a hard core lying within an outer ring of the 
medullary substance. A series of transverse slices will demonstrate 
striking changes in the interior of the cord, probably varying considerably 
at different levels. In the region of the greatest swelling, usually the 
cervicodorsal region, the section may at first sight appear to have tra- 
versed an area of necrotic softening, or even an abscess, but closer 
scrutiny will show that near the centre of the cord is a cavity, from which 
clear fluid may be oozing, and that this cavity is surrounded by an 
enveloping mass of gelatinous material either pale and translucent or 
yellowish brown, according to the amount of altered blood pigment it 
contains. The immediate wall of the cavity sometimes stands out as a 
more opaque pale yellow membrane which has been thrown into folds 
by the escape of the contents and collapse of surrounding parts. Fur- 
ther sections at different levels will discover the length of the cavity. 
In some cases it extends through a few segments only, in others through 
the whole length of the cord. Rarely the tube is prolonged into the 
fourth ventricle, although in the unique case described by Spiller it 
reached the right internal capsule. 
Frequently the cavity disappears at one level, the surrounding opaque 
tissue continuing for another segment or two, sometimes to develop DIFFUSE AND FOCAL DISEASES OF THE SPINAL CORD 
213 
another cavity beyond. Two cavities may be present side by side, but 
if these are traced farther it is usual to find that one is an offshoot of the 
other. The lumen varies both in size and shape at different levels. 
Position of Cavity.-To describe this accurately it is necessary first 
of all to know its shape and size at any particular level, but speaking 
generally, these tubes lie behind the anterior commissure, their centre 
of origin, if one may use the expression, being situated in the gray matter 
behind the central canal, either on the median line or laterally in the base 
of one or other posterior cornu. From this centre diverticula may 
extend in any direction, with resulting varieties in the shape of the cavity 
in transverse section, but more often than not some part of the posterior 
white columns is invaded at one level or another. Without doubt the 
posterior horns and posterior columns are more often involved than 
the anterior horns, while the anterolateral white matter is rarely en- 
croached upon directly by the cavity, although it may suffer indirectly 
from the effects of pressure. The shape of the cavity may be round, 
oval, crescentic, stellate, or irregular, and it is certainly unusual to find 
any great degree of symmetry in its relation to the two halves of the cord. 
In the bulb, as in the cord, the morbid process has a favorite site. 
Occasionally the spinal cavity may reach the fourth ventricle, but more 
often it ends below, the only evidence of the disease in the bulb being a 
fissure or fissures originating in the floor of the ventricle, a little to one 
side of the midline, and extending forward and outward in such a way as 
to cut off the restiform body from the central parts of the medulla. 
Such a fissure may destroy the descending root of the fifth nerve, the 
solitary fasciculus and some of the nuclei belonging to the vagoglosso- 
pharyngeal nerves, but it rarely extends as high as the facial nucleus. 
It is sometimes unilateral and sometimes bilateral. 
What has been said with regard to the position of the spinal cavity 
applies equally to the disposition of its surrounding tissue, which roughly 
follows the various diverticula when it does not form prolongations of 
its own, and which, it must be remembered, extends in most cases to 
levels beyond the ends of the tube. 
Deformities of the surface of the cord and of the outline of the gray 
and white matter are dependent upon, and present as many varieties 
as, the distortions of the cavity and its walls. 
Gliomatosis.-The glial tissue surrounding the cavity varies enormously 
in thickness not only in different cases, but at different levels in the same 
case. It may only present the characters of a thin lining membrane to 
a large cavity, or it may in other instances be so voluminous as to over- 
shadow completely the narrow slit-like cavity it contains. In fact, it 
may exist in the form of a hard core without cavitation through consider- 
able lengths of the cord. Whatever may be the prevailing theory as to 
its mode of production, there can be no doubt that it is essentially of 
neuroglial origin, the term gliomatosis being useful in that it suggests an 
overgrowth of that tissue and yet separates it from the true glioma which 
belongs to the group of malignant neoplasms. This gelatinous-looking 
material is mainly composed of fibres and cells whose relative proportion 
varies, but it may be stated roughly that there are more cells than in 214 
DISEASES OF THE NERVOUS SYSTEM 
normal neuroglia, and less cells than in a true glioma. At levels where 
no true cavity exists, foci of softening, to which the name granular disin- 
tegration has been applied, are occasionally present in the centre of the 
gliomatous formation. 
Central Canal.-The fact that the cavity may be lined or partly lined 
by ependymal cells has been noted, and this condition usually obtains 
when the cavity represents an enlarged central canal or when a cavity 
formed in the peri-ependymal tissue has fused with the central canal. 
Rarely, a cavity situated at some little distance from an intact central 
canal may be lined with ependymal cells, in which case the explanation 
is offered that the cavity has originated in a nest of embryonal tissue 
persisting from the time of the closing up of the posterior columns. 
In most cases the cavity is either obviously a dilatation of the central 
canal or appears to be quite independent of the latter. According to 
Schlesinger the independence is only apparent. At any rate it is quite 
a common thing to see the central canal, either open or obliterated, 
lying quite separate from the pathological cavity in a long series of 
sections. In other instances the cavity is fused with the central canal 
over several segments and separated from it in regions beyond. 
Vascular Lesions.-In a certain number of cases the vessels of the cord 
and its membranes are perfectly healthy, but in others the vessels have 
undergone definite alterations. For this reason French observers in 
particular have attached considerable importance to " les espaces vasculo- 
conjunctifs." Vessels radiating from the surface of the cord into the 
central glioma present the following changes: The vessel walls and the 
accompanying glial tissue are swollen, hyaline, and often thrown into 
folds. As a rule, the lumen is diminished or obliterated, especially when 
it reaches the glial mass, and there is no evidence of proliferation of the 
nuclear elements of the walls or sheath. In the neighborhood of the 
cavity the only remnant of a vessel may be an undulating ribbon of 
hyaline connective tissue which has been unfortunately called a papillary 
membrane, and this, owing to degeneration of neighboring tissues, may 
at times line the cavity or a portion of its wall. Schlesinger has observed 
these vascular changes in a case of syringomyelia without gliomatosis. 
In sections the central parts of the glial mass are usually poor in vessels, 
but the more peripheral zones contain a number, mostly running parallel 
with the long axis of the cord. It is stated that newly formed vessels 
are found in these parts, but the evidence of this does not appear to be 
very clear. Hemorrhages old and recent are by no means infrequent. 
Lesions of the Nervous Elements.-The gliomatosis and cavitation, 
after attaining a certain bulk, produce changes in the surrounding tissues 
partly by compression and partly by direct invasion. The latter method 
is the one by which the posterior columns are principally affected, the 
newly formed neuroglia eating its way, as it were, between the bundles 
and producing slow destruction. Closely associated with compression 
is the production of an oedema of the tissues surrounding the glial mass 
which is responsible for the rarefaction of the gray matter often seen in 
the anterior horns. The nerve cells become isolated and their processes 
disappear, with the result that a species of cavity may be formed to DIFFUSE AND FOCAL DISEASES OF THE SPINAL CORD 
215 
which the name perigliomatous, as opposed to endogliomatous, has been 
given. A still further loss of glial tissue may eventually lead to fusion 
of the cavities originating in these two different ways, and thus to the 
appearance of a large central canal surrounded only by a thin layer of 
white matter. 
The secondary degenerations which result from the lesions of the white 
matter and from the destruction of ganglion cells in the gray matter 
conform to the general laws governing secondary degeneration in the 
cord and peripheral nerves. Sclerosis of the pyramidal tracts, of the 
posterior columns, and of the ascending anterolateral tracts is of common 
occurrence, as well as atrophy of the anterior roots. 
Mention must be made of certain neuromata which have been described 
in the gliomatous tissue, white matter, intramedullary roots, and subglial 
tissue. These small tumors are composed of numerous fine medullated 
fibres running in various directions and accompanied only in some 
instances by bloodvessels. Their origin is uncertain, Raymond suggesting 
that they afford evidence of attempted regeneration, and Schlesinger 
that they are due to local irritation. Patoir and Raveirt have found 
neurofibromata in the spinal roots in a case of syringomyelia, and have 
regarded them as of similar origin to the neuromata of the cord and per- 
haps allied in their etiology to the interstitial changes occurring in the 
peripheral nerves. The pathological changes which occur in other 
tissues, in the nerves, muscles, skin, joints, and bones, must be all 
considered as secondary to the lesions of the central nervous system. 
They do not present features peculiar to this disease. 
The occurrence of syringomyelic cavities in association with marked 
pachymeningitis or leptomeningitis or with vertebral caries is not ex- 
tremely rare, although the exact relationship to the spinal condition has 
not been fully established. Still more common is the concurrence of some 
true neoplasm with the syringomyelia. At any level of the cord, com- 
monly in the cervical region, may be found a true tumor, involving more 
or less the entire transverse area, which has evidently originated from a 
part of the gliomatous mass. Such are commonly very cellular gliomata 
or very vascular angiogliomata, but may sometimes be of a more sarco- 
matous nature. The writer has seen neoplasms of this kind in the 
medulla, the cervical and sacral regions in different cases, all associated 
with syringomyelia. 
Pathogenesis.-Numerous theories have been put forward to explain 
the origin and progression of the morbid process. Their number suggests 
that many cases of spinal cavitation and gliomatosis, although resembling 
one another roughly in their clinical and anatomical characters, may 
yet have different modes of origin, and this view is finding more and 
more favor with those whose knowledge of the disease is most profound. 
While it is impossible to elaborate dogmatically a genetic theory applicable 
to every case, a consideration of certain developmental, anatomical, 
and pathological facts cannot fail to throw' some light on particular 
examples, and may serve as a basis for more scientific grouping. 
Developmental.-The medullary canal of early fetal life is only repre- 
sented clearly in the child by the central canal of the cord, a cylindrical 216 
DISEASES OF THE NERVOUS SYSTEM 
tube running throughout the length of that organ lined by ependymal 
cells of epiblastic origin. The central canal, however, represents only 
the anterior limb of the medullary canal, the posterior and lateral limbs 
disappearing in the coalescence of the posterior columns of either side 
along the posterior median fissures and in the process of forming the 
gray commissure and the bases of the posterior horns. Normally no 
cells resembling the ependymal cells of the central canal can be seen in 
the gray commissure, in the bases of the posterior horns, or in the walls 
of the posterior median fissure. Presumably, however, there are in 
these three contiguous parts embryonic remnants, which may take on 
renewed activity and once again produce neuroglia, the most primitive 
tissue of epiblastic origin in the central nervous system. On such an 
assumption the occurrence of a neuroglial hyperplasia appears to have 
a reasonable, if not complete, explanation and gliomatosis practically 
always originates either in the gray commissure, the bases of the posterior 
horns, or in the anterior third of the posterior columns. The occasional 
presence of groups of ependymal cells away from the central canal may 
be regarded as a developmental accident in otherwise normal cords, 
but it is possible that such groups may be the starting point of a peri- 
ependymal hyperplasia, just as the latter certainly originates in some 
instances from the walls of the central canal itself. 
Anatomical.-A consideration of the spinal vascular supply will bring 
out one important point. That part of the spinal cord which includes 
the gray commissure, the anterior third of the posterior columns, and the 
bases of the posterior cornua is the centre of the cord from the vascular 
point of view. It receives blood from various radiating vessels, of which 
the most important are those entering along the posterior roots and 
along the posterior median septum, as well as from some of the ter- 
minal branches of the anterior spinal artery. It is the central ter- 
minus of those various arteries, and contains, therefore, vessels of small 
caliber, some of which have a transverse and some a longitudinal 
course. 
This is of importance in two ways. In the first place, any general 
disease of the spinal arteries which reduces the muscularity and elasticity 
of their walls will produce its greatest effect on the vascularization of 
this central zone. In the second place, relying as it does very largely 
on the posterior spinal arterial system for its blood supply, any morbid 
process leading to pressure upon or strangulation of the pial vessels on 
the posterior surface of the cord will have a profound effect upon the 
same area. 
The lymphatic supply of this central spinal area must also be considered, 
the more so because theories suggesting an infective origin for the disease 
have been propounded. The central canal is possibly an important 
lymph channel, and the presence in it of an irritant agent capable of 
inciting ependymal proliferation and peri-ependymal gliosis has been 
suspected. More important is the fact that lymphatic infection of the 
cord from other organs generally takes place along the lymph channels 
of the posterior roots. On facts such as these, and experimental work 
in connection with lymphatic infections of the spinal cord through the DIFFUSE AND FOCAL DISEASES OF THE SPINAL CORD 
217 
peripheral nerves, some have attempted to isolate a group of syringo- 
myelias due to an ascending neuritis. 
The closure of the central canal by pressure at one level of the cord 
has been quoted as a sufficient reason to explain dilatation of that canal 
at other levels. The justice of this is by no means proved, and, in fact, 
may be questioned when it is remembered that in normal adult cords 
the canal may be obliterated in some segments and yet patent without 
dilatation above and below. 
There are certain pathological facts to be taken into account, of which 
the most important is the partiality of intramedullary hemorrhages and 
abscesses for particular paths in their progress through the cord. There 
is a great tendency for blood and pus to track from segment to segment 
along the tissues at the bases of the posterior horns or in the central 
gray matter, so much so that this area has been termed the zone of least 
resistance. It is evident that gliomatosis spreads along similar lines, 
and the close relationship existing between the two processes has led 
to the belief that many cases of syringomyelia have their remote origin 
in spinal hemorrhage occurring at birth. Cases have been recorded 
among adults in which the clinical history has suggested a traumatic 
intramedullary hemorrhage followed, perhaps years later, by the symp- 
toms of a progressive syringomyelic lesion. It is possible, therefore, 
that an old pathological process may after a lapse of time be the starting 
point for gliomatosis and cavitation. 
There are other forms of syringomyelic cavities apparently originating 
from a level of the cord, which has been compressed by meningitis, by 
vertebral caries, or by some other external cause. It is difficult to see 
how this comes about unless as the result of evascularization of the cord 
with the production of necrotic cavities in the central zone and the 
subsequent spread of disintegration along the favorite lines just men- 
tioned. The presence of gliomatous tissue surrounding the cavity so 
formed is not easy to explain, but the theory has been advanced that the 
products of disintegration are sufficiently irritating to incite glial pro- 
liferation. Such an explanation appears in a few cases more likely than 
one which assumes the necessary existence of a congenital abnormality 
as a predisposing factor. 
These developmental, anatomical, and pathological data, together 
with clinical observations, suggest that various influences are at work 
in the production of spinal cavities. Many examples of syringomyelia 
fall naturally into groups in which one or other of these influences plays 
a predominant part, while others are not so easily classified. Thus there 
are cases in which a congenital defect appears to carry great weight; 
others in which a morbid condition of the vascular supply seems too 
important to overlook, and others in which some source for a chronic 
lymphatic infection or a history of some traumatic hematomyelia require 
careful consideration. The fact that gliomatosis is always associated 
in greater or less degree with the formation of these cavities can only 
be explained on the supposition that the neuroglia of the central zone 
of the cord has an inherent tendency toward this form of overgrowth, 
the initiation of which may be due to a variety of agents, physiological, 218 
DISEASES OF THE NERVOUS SYSTEM 
chemical, mechanical, or pathological. The tendency exhibited by the 
gliomatous tissues after reaching a certain bulk to undergo central 
degeneration and then to help in the formation of cavities must be referred 
either to deficient vascular supply or to some inherent property. 
With regard to the ordinary cases of chronic progressive syringomyelia 
little hesitation can be felt in placing foremost some congenital anomaly 
of the central embryonal tissue, the latent activity of which is prone to 
display itself during the early years of adult life in the form of a slow- 
growing hyperplasia, having some of the characters of a benign neoplasm 
and strong tendencies toward the formation of cavities. As far as the 
other morbid influences we have referred to are concerned, they can 
only be looked upon as secondary exciting agents or, in very occasional 
instances, as of primary causal importance. 
Symptoms.-A disease so prolific in physical signs and symptoms as 
syringomyelia can never be portrayed by a serial enumeration of its 
manifestations. The detailed consideration of these must be postponed, 
therefore, until a picture has been drawn of an ordinary typical case 
which may serve as a basis for further remarks. A man, aged twenty- 
seven years, seeks advice on account of some paralysis of his hands. 
He says that the first symptom occurred about four years before, when 
a sensation of numbness in his left hand was followed before very long 
by some weakness and awkwardness of the same part. After some 
months he noticed that the hand was wasting, the leaders and bones 
standing out more prominently than on the other side. Little notice 
was taken of this until he became aware that similar alterations were 
taking place in his right hand and that he was beginning to find himself 
awkward in writing, buttoning his clothes, and other fine movements. 
The legs are perhaps more easily tired than formerly, and on one or two 
occasions the left toes have caught a step in going up stairs. He says 
that he has had little or no pain, an occasional "burning sensation" in 
the region of the neck, but nothing to worry about. 
The patient appears healthy and strong, and his face is natural, except 
that the left pupil is smaller and the left palpebral fissure narrower than 
the right, giving an appearance of slight ptosis on the left side. This, 
however, disappears on his looking up, both upper eyelids moving equally 
well. The pupils react to light, but do not dilate very readily to shade, 
nor do they react when the skin of the neck is pinched. The ocular 
movements are natural except that lateral deviation is associated with 
marked nystagmus. The observer is struck by the good muscular 
development of the shoulder and upper arm compared to the wasted 
condition of the forearm and hand. This is more marked on the left 
side, where the contour of the inner border of the forearm is completely 
altered and flattened. The left hand is noticeable for the " claw" position 
of its fingers, the flatness of its palm, and for the fact that the palmar 
surface of the thumb is parallel instead of nearly at right angles to that 
of the other fingers. There are an unusual number of scars on the hand 
and forearm, and the patient, on being questioned, admits to many cuts 
and burns. One long scar was the result of leaning his forearm on an 
oven door, the only knowledge of its having been burnt being conveyed DIFFUSE AND FOCAL DISEASES OF THE SPINAL CORD 
219 
to him by the discovery of a blister. The terminal phalanx of the 
middle finger is missing, and on inquiry it appears that three or four 
years ago, subsequent to a slight injury, a whitlow formed, which in 
spite of incision and scraping on two or three occasions ended after many 
months in the loss of the top of his finger. There was no pain with it. 
The skin of the hand is rough and hard, and the remaining nails are 
thick and striated. All his finger movements are feeble, and flexion 
of the wrist is much less powerful than extension. Except for the triceps, 
which may be a little impaired, the muscles about the upper arm and 
shoulder are quite as strong as their appearance would suggest. Turning 
the man around, the only thing to catch the eye is a slight yet definite 
lateral curvature in the dorsal part of the vertebral column, of which 
the patient is quite unaware. Inspection of the legs reveals nothing 
visibly wrong, although, if each group of muscles is tested against resist- 
ance, dorsiflexion of the feet is not so powerfully performed as it should 
be. A glance at his boots shows that the toes of each are unduly worn 
down, more noticeably on the left side. Tapping the patellar or Achilles 
tendon produces brisk jerks, and stimulation of the soles of the feet 
elicits an extensor response on either side. An attempt to obtain the 
abdominal reflexes is unsuccessful. The patient's sensibility to tactile 
stimuli is found to be perfect everywhere, but of painful and thermal 
stimuli his appreciation is practically absent over a large area, which 
includes both arms, the neck, and the thorax as far down as the xiphi- 
sternum on the left side and the third rib on the right. On the other 
hand, his sense of passive movement and position and his stereognosis 
are intact even in the upper limbs. 
The subsequent history illustrates the usual course of events. After 
a temporary improvement in the strength of the muscles of the upper 
limbs due to local teatment, the inevitable slow progress of the disease 
displays itself in the fact that several more muscles in each arm are 
affected and the area of analgesia and thermo-anesthesia extends lower 
down the trunk and over a great part of the left side of the face. Five 
years later the patient is found to be unable to work on account of the 
condition of his hands, and his walking has become seriously interfered 
with by the increasing spasticity of his lower limbs. The right shoulder 
has recently become the site of a large fluctuating swelling, a skiagram 
showing that the joint has become completely disorganized. The left 
side of the tongue is slightly wasted and the left palatal arch lags behind 
the right during phonation. Inquiry elicits information that the patient's 
control over his vesical sphincter is not so good as it was, and hesitating 
or precipitate micturition is the result. A few more years and the 
bedridden stage is reached, the length of which will depend very largely 
on the circumstances surrounding the sufferer and the care with which 
he is nursed. Some pulmonary complication or septic infection will 
end an illness which may have lasted anywhere from fifteen to thirty-five 
or even forty-five years. 
This is the usual progress, but there are exceptional cases, in which no 
increase in symptoms may take place for many years, and some in which 
the disease appears to become permanently arrested. Slow, insidious 220 
DISEASES OF THE NERVOUS SYSTEM 
progress is the general rule, although every now and then a case is observed 
in which a sudden increase in symptoms has been followed by some 
improvement, leaving the patient, however, worse off than he was before 
the sudden change. Such an event has generally been attributed to 
hemorrhage taking place into a syringal cavity. 
Such a history is typical of the majority of syringomyelic patients 
and these may be included in a class to which the name "amyotrophic" 
is applicable on account of the early appearance of progressive muscular 
atrophy and the prominent part taken by that symptom throughout?- 
The remaining cases may be divided into three other classes, making 
four altogether, viz.: (1) Amyotrophic, (2) spastic, (3) Morvan's type, 
and (4) spinal tumor type. 
This classification is useful, although quit^ artificial, and depends 
upon the predominance of certain clinical features. Thus, the spastic 
case ("forme spasmodique") is characterized by marked rigidity affecting 
all four limbs and producing attitudes and fixed positions which are 
worthy of note. The back is kyphotic, the head low between the shoulders 
and forced forward in a manner suggestive of paralysis agitans. The 
hands, too, adopt special attitudes, the three inner fingers flexed into 
the palm, and the index firmly pressed against the thumb. Such a 
case may and usually does present some muscular atrophy, although 
the latter is not so striking as in the amyotrophic class, in which spas- 
ticity is less marked and usually confined to the trunk and lower ex- 
tremities. 
The Morvan's type of case, at one time regarded as a separate disease, 
is remarkable for trophic changes in the extremities, especially for a slow 
necrotic dactylitis, in the course of which most of the small bones of the 
hands or feet may be lost and the parts deformed almost beyond recogni- 
tion. Amyotrophy and sensory disturbances are associated with the 
trophic changes in skin and bones. 
The fourth class includes a number of cases in which a diagnosis of 
spinal tumor is more often made than that of syringomyelia until operation 
or postmortem examination reveals the presence of a cavity associated 
with a local gliomatous or sarcomatous tumor, to which the symptoms 
and physical signs have been mainly due. In such cases the absence 
of the characteristic dissociation of sensation and of trophic disturbances 
may have rendered an accurate diagnosis difficult or impossible. 
Onset - In 17 of 40 cases this was marked by some disturbance of 
motion or sensation of one of the upper extremities. In at least half of 
them the patient first noticed some sensation of cold, heat, numbness, 
tingling, or pain in the hand or forearm, but in all the weakness and 
subsequent wasting either followed quickly or were the earliest to appear. 
At the time they came under observation all had some objective loss 
of sensation in the affected limb, and it may well be that this preceded 
the other signs, without its existence having come to the patient's notice. 
Some difficulty in gait was stated by 8 patients to be their first trouble, 
but it is remarkable how rarely any incoordination in walking is seen 
later in the disease. Subjective sensations in the lower extremities 
were noted before any other symptoms in three cases, one of whom DIFFUSE AND FOCAL DISEASES OF THE SPINAL CORD 
221 
described his right leg as cold and dead, and another his left leg as always 
hot when the rest of the body and the other leg were cold. 
Scoliosis preceded all other signs of disease in four cases, and painless 
whitlow in a like number. Pain in the occiput and neck (in two 
instances), difficulty in articulation, and difficulty in micturition were 
the other initiatory symptoms. 
Disturbances of Motion.-(a) Atrophic Paralysis.-This is more common 
in the upper extremities than elsewhere, usually beginning in the intrinsic 
hand muscles on one side and spreading up the arm in accordance with 
the segmental innervation of the muscles and not following the peripheral 
nerve distribution. Less often it commences in the muscles of the 
shoulder girdle and rarely in the muscles of the legs. Awkwardness 
or feebleness of finger movements usually precedes the appearance of 
obvious atrophy, but later on the amount of paralysis corresponds 
roughly to the destruction of muscle fibres. In consequence of the 
interosseal palsy and the preservation of the long extensors of the fingers 
the hand may present the "main en griffe" appearance. When vaso- 
motor and trophic changes are associated, the "main succulente" is the 
result. Any muscle which is undergoing atrophy may display fibrillary 
tremors, and its response to faradic and galvanic currents is propor- 
tionately diminished; in some instances true reaction of degeneration is 
observed. All atrophic muscles sooner or later lose their tendon reflexes. 
(6) Spastic Paralysis.-The degree of spastic paralysis depends upon 
the amount of degeneration in the pyramidal tracts and its extent upon 
the level at which the pressure upon, or the invasion of, the lateral 
columns occurs. Mention has already been made of the spastic form 
in which the affection of the pyramids extends at least as high as the 
upper cervical segments, with the result that all four limbs as well as 
the trunk are more or less rigid. In the more common amyotrophic or, 
speaking anatomically, cervicodorsal form the spasticity usually appears 
later and affects the trunk and legs only. In addition to their passive 
and active rigidity, severely spastic parts are subject to flexor spasms, 
sometimes of a painful character, which may occur spontaneously or 
as a more or less easily elicited reflex phenomenon. Associated with 
the spasticity is the form of flexed hand already referred to and a tendency 
to clubbing of the feet. All spastic muscles have increased tendon 
reflexes sometimes amounting to clonus. Occasionally a moderate 
degree of atrophy is associated with spasticity in the same muscle, which 
may preserve a tendon jerk of diminished excursion and power. More 
often than not the atrophic paralysis of one hand is accompanied by 
spastic paresis of the leg on the same side before the opposite limbs 
have become affected. In all spastic cases it is the rule to find diminution 
or absence of the abdominal reflexes and an extensor response to plantar 
stimulation. These constitute the earliest evidences of this form and 
are present before any definite paresis or rigidity can be detected. 
(c) Incoordination.-A marked degree is not a common feature, and 
the majority do not suffer from any great loss of sense of position and 
active movement. Some interference with this sense may be present 
in those patients who complain of reeling gait in the early stages. Tabes 222 
DISEASES OF THE NERVOUS SYSTEM 
and syringomyelia occurring in the same individual is not unknown, 
but the association is only accidental. 
(d) Spinal Curvature.-The common dorsal scoliosis must be regarded 
in most instances as of muscular origin. The asymmetrical character 
of the paresis affecting the spinal muscles and the legs is quite sufficient 
to account for its presence in the ordinary way. When the deformity 
is extreme or when combined with some kyphosis it is possible that 
osseous or arthritic changes may be additional factors. 
(e) Tremors -These are not common features, although fine tremors, 
resembling those of Graves' disease, and coarse "intention" tremors 
have been described. 
Bulbar Symptoms.-When the disease affects the bulb either primarily 
or as an extension from the cervical region of the spinal cord the clinical 
picture is often described as "syringobulbia." Atrophy of the tongue, 
unilateral or bilateral, paralysis of one or both vocal cords or palatal 
arches are among the more constant symptoms. 
Facial paralysis is more rare, and ocular palsies very exceptional. 
On the other hand, nystagmus is a common feature, and this may be 
lateral, vertical, or rotatory. The latter type was present in 2 out of 
40 cases, and the former was of frequent occurrence. Sensory disturb- 
ances in the area supplied by the trigeminal nerve are often present, 
even without other bulbar symptoms. Defects in articulation, 
deglutition, and phonation are the results of the various palsies 
mentioned. 
The only special senses to be interfered with in any degree of frequency 
are those of taste and hearing, and even these are usually intact except 
in severe examples of the bulbar form. Vision remains unaffected almost 
without exception, but H. S. Hutchinson has once observed degeneration 
of the optic nerves. 
Disturbances of Sensation.-(a) Thermo-anesthesia.-1This is one of the 
most constant symptoms, and is almost invariably present to some 
extent even in the earliest stages. The upper extremities or thorax are 
Usually first affected, extension of the area taking place with the progress 
of the disease. One patch of thermo-anesthesia is sometimes separated 
for a time from another by a healthy area, coalescence occurring later 
on. The defect in sensibility exists generally for both cold and heat 
and for all degrees of temperature, but occasionally cold is recognized 
and heat is not, or vice versa. It may happen that an area of complete 
thermo-anesthesia has a border separating it from the normal skin, in 
which cold only is appreciated. When the defect of sensibility is only 
partial, it would appear, according to Rosenfeld, that the power of 
appreciating heat or cold depends on the extent of the area stimulated 
rather than on the degrees of temperature. 
The association of thermo-anesthesia and analgesia together with 
complete preservation of tactile sensibility in the same cutaneous region 
is very characteristic, but tactile anesthesia may coincide with the other 
forms, especially in advanced stages. Although thermal sensibility 
may be absent from the skin and from the mucous membrane of the 
mouth and gullet, yet appreciation of the temperature of a liquid may DIFFUSE AND FOCAL DISEASES OF THE SPINAL CORD 
223 
be derived from the stomach. A syringomyelic patient may burn his 
mouth and be unaware of the heat of the food until after it is swallowed. 
(6) Analgesia.-More often than not analgesia is present in thermo- 
anesthetic areas. Frequently the distribution of the one is co-extensive 
with that of the other, but this is not always the case, and wide variations 
may be observed. Cutaneous analgesia is generally combined with a 
certain amount of deep analgesia (loss of sensibility to painful pressure), 
although their degrees may be unequal. Head and Thompson record 
a case in which an area of complete cutaneous analgesia was co-existent 
with deep hyperalgesia in the neighborhood of a painful arthropathy 
of the shoulder. "The lightest pressure stimuli caused pain, and the 
extent of the hyperalgesia could be marked out on the skin. Yet the 
superficial layers of the skin were analgesic to the prick of a pin when 
raised from the subcutaneous structures." 
(c) Distribution of Dissociative Anesthesia.-Thermo-anesthesia and 
analgesia are usually found first in the upper extremities and thorax, 
and spread thence all over the body. In rare instances they commence 
in the lower extremities or on the face. Their distribution is nearly 
always asymmetrical. The borders of the cutaneous loss are not sharp, 
but shaded off and correspond to the limits of spinal root areas. At 
the same time charts sometimes show regions of dissociative anesthesia 
which correspond laterally to one or more root areas, but do not cover 
their longitudinal extent. For instance, the sensory loss in one hand 
may be limited above by a line encircling the forearm, so as to give it 
the appearance of a glove distribution. Similarly on the face a central 
area, including the nose, mouth, and eyes, may preserve its sensibility 
intact, while the surrounding regions are completely insensitive to 
painful and thermal stimuli. The explanation of these limitations is 
somewhat difficult; the presence of a cavity running through several 
segments and interfering, in the gray matter of each, with corresponding 
systems of afferent fibres connected with one posterior root after another, 
may throw some light on the matter. 
(d) Tactile Anesthesia.--Sensibility to tactile stimuli and with it the 
power of localizing touches and distinguishing between the head and point 
of a pin is usually preserved until the later stages, when it is lost over 
areas of varying distribution. It is lost earlier, however, in that class 
of cases which has already been referred to as the spinal tumor class, 
in which no dissociation of anesthesia may be present. 
(g) Loss of Other Afferent Impulses.-The sense of passive position and 
movement is often preserved until late, but may disappear progressively 
from one joint after another in either the upper or lower extremities. 
According to the observations of Head and Thompson, the power of 
discriminating between the two points of a compass tends to disappear 
pari passu with the sense of passive movement. Other afferent but 
non-sensory impulses, for instance those destined for the cerebellum, 
may be interfered with and defective equilibrium be the result. 
(f) Subjective Sensation.-Subjective sensations occasionally form the 
initial evidence of disease, and may be of a thermal, painful, or tactile 
character. Lancinating pains have been described, and cramps of 224 
DISEASES OF THE NERVOUS SYSTEM 
considerable severity are not unknown. More curious is the subjective 
sensation of drenching sweat in a part which is dry. A girdle sensation 
occurs in this disease, as it does in many other affections of the spinal 
cord. 
Vasomotor and Secretory Disturbances.-These form a very large 
group of symptoms, many of which, although common in syringomyelia, 
are not characteristic. The appearance of an affected limb may vary 
from time to time, now dry and pale, then moist and hyperemic. Vaso- 
motor paralysis has the effect of making affected parts react in an exagger- 
ated way to the temperature of the environment, with the result that 
the skin is overwarm and moist in the summer, and overcold, perhaps 
cyanotic, and dry in the winter. A cutaneous tache is of common 
occurrence in analgesic areas, a pin prick producing a red spot with a 
central white area which may last for hours, or even days, and may 
resemble a parasitic bite. Urticarial rashes are very prone to make their 
appearance with little provocation. A vasomotor disturbance of the 
subcutaneous tissues is largely responsible for the "main succulente" 
first described by Marinesco. In this the whole of the dorsum of the 
hand and all the fingers may swell to an extent which renders their 
detailed anatomy unrecognizable, while the overlying skin is tense, 
glossy, and smooth. Pressure produces no marked pitting. Hyperi- 
drosis is another common feature, and often affects the analgesic areas. 
It may be spontaneous or easily elicited by the warmth of a hot bath. 
In this way half the head and neck, together with the corresponding 
arm and side of thorax, may be bathed in sweat while all the rest of the 
body is dry. Excessive lacrymation or salivation is sometimes observed 
in bulbar cases, and Guthrie has reported a unique instance of unilateral 
seborrhoea. 
Ocular Sympathetic Paralysis.-Owing to the frequent involvement of 
the first dorsal and eighth cervical segments by the gliomatous process, 
oculopupillary symptoms occur in about 85 per cent, of cases. As a 
rule, the paralysis of the sympathetic fibres is unilateral, or is more 
advanced on one side than on the other. On the affected side the pupil 
is smaller and the palpebral fissure narrower than on the other side. 
Owing to paralysis of Muller's unstriated muscle, the eyeball is often 
slightly retracted. The visible effect is one of false ptosis, which dis- 
appears at once when the patient looks upward. It is stated that 
paralysis of unstriated muscle fibres in both the upper and lower eyelids 
allows of their approximation. Further examination would show that 
the pupil does not dilate or dilates sluggishly when cocaine is applied or 
when the eye is shaded, and that no enlargement takes place when the 
skin of the neck on the same side is stimulated. 
Trophic Disturbances.-(a) Skin.-The glossy skin, or "peau lisse," 
represents the common atrophic variety of the cutaneous affections. 
Hypertrophy or thickening of the skin occasionally takes place, and this 
is particularly liable to lead to fissures, which deepen and ultimately 
ulcerate. All forms of ulceration tend to spread and perforate deeper 
subcutaneous structures, necrosis of bones eventually being produced. 
Healing is slow and sometimes impossible. In extreme degrees the DIFFUSE AND FOCAL DISEASES OF THE SPINAL CORD 
225 
panaris of Morvan's type of the disease is the result. Keloid has been 
known to develop in the cicatrices of these trophic ulcers. 
Among many forms of dermatitis which have been described, the 
eruption of vesicles or bullae is one of the most common. These may 
appear suddenly in the night, accompanied sometimes by fever, and are 
distributed generally over the arms or thorax. The superficial layers 
of the rete mucosum are only involved at first, but when the fluid has 
escaped deeper ulceration generally ensues. True pemphigus is a rare 
but occasional complication. 
Trivial injuries may lead to serious septic processes, owing to the 
analgesia preventing their early discovery and attention. The nails 
are generally thickened, striated, and brittle; sometimes they tend to 
drop off. Cases are on record in which nails grew in unwonted positions, 
while the natural nailbeds remained inactive. The hair may be altered 
in quality, becoming brittle and coarse, or it may disappear from large 
tracts of skin. A complete alopecia has been seen by the writer. The 
growth of hairs in unaccustomed sites has been noted. 
(6) Bones.-In addition to suffering in the general tissue necrosis, 
the bones, particularly of the extremities, are liable to undergo trophic 
changes in the form of rarefaction or increased density. Osseous excres- 
cences or exostoses, symmetrical in distribution, have been observed, 
and in at least one case suppuration has taken place in the newly formed 
tissue. Fragility of the long bones has given rise to spontaneous fracture, 
the occurrence of which is more common in the radius and ulna than in 
all the other bones of the body put together. General enlargement of 
parts of the skeleton occasionally produces a semblance of acromegaly, 
although it is often symmetrical and may not be associated with increase 
of the softer tissues. Chiromegaly is the most common example of 
this condition. The deformity described under the name of "thorax 
en bateau" by Marie and Astie is the result of osseous changes in the 
upper part of the sternum and upper ribs. In this there is a depression 
of these parts of the thoracic skeleton, which is associated with a forward 
and upward projection of the shoulders. It is not always combined 
with vertebral deformities, and therefore not dependent upon the latter. 
(c) Joints.-Arthropathies occur in 20 to 25 per cent., the incidence 
on particular joints being very different to that which characterizes 
tabes dorsalis. Thus, 80 per cent, of the arthropathies of syringomyelia 
are found in the upper extremities, and the most frequent joint to suffer 
is the shoulder. As a general rule, the joint troubles are unilateral, 
but occasionally bilateral and symmetrical arthropathies are observed. 
According to Schlesinger, males present this symptom more commonly 
than females in the proportion of two to one. While trauma has an 
undoubted influence in certain instances, it is equally certain that they 
may appear spontaneously, and probably do so in the majority of cases. 
The development of a Charcot's joint is often sudden, although it 
may be preceded by pains or by unpleasant sensations in the part about 
to be affected. The first evidence in some cases is an extensive fluid 
swelling involving the joint and surrounding tissues, in others a creaking 
or crepitus evoked by movement. The fluid is apt to disappear in the 
vol. v-15 226 
DISEASES OF THE NERVOUS SYSTEM 
course of a few days or weeks, leaving a relaxed and abnormally mobile 
joint. Fresh eflfusions may occur spontaneously or as a result of some 
slight injury, every such occurrence producing increased disorganization. 
The changes are of an atrophic or hypertrophic nature. Sometimes 
the two varieties are combined. The hypertrophic changes include 
thickening of the synovial membrane, ligaments, and capsule, with the 
appearance of osteophytes and cartilaginous masses often embedded in 
the softer tissues. Bony excrescences originating in the muscular inser- 
tions add to the deformities. Ankylosis has occasionally occurred. In 
the atrophic form the articular cartilage gradually disappears and the 
subjacent bone may be rarefied and destroyed so as to cause considerable 
shortening. 
In the majority of instances these arthropathies run a painless course, 
but this is not invariably so in their early stages when deep analgesia 
may not have developed. Suppuration occurs in a certain number, and 
this leads to increased disintegration and perhaps to a general septicemia. 
Operative measures may often be carried out without an anesthetic, 
although sinuses very difficult to close generally persist. 
Disturbances of Control over Excretory and Sexual Organs.-In the 
common type of cervicodorsal syringomyelia the interference with the 
control over the bladder and rectum varies with the degree of spasticity 
in the lower extremities. In the early stages there may be no abnormal- 
ity. With moderate degrees of spasticity it is usual to find precipitate 
or hesitating micturition and rather obstinate constipation associated 
with precipitate defecation after aperient medicine. In later stages 
retention and incontinence are experienced. In the more rare lumbosacral 
form of the disease sphincter trouble may be prominent from the begin- 
ning, and in this condition the sexual power is not preserved so long as 
it is in the more common type. In the spinal tumor type the symptoms 
of a complete transverse lesion may include priapism. 
Diagnosis.-Syringomyelia is one of the easiest and one of the most 
difficult diseases to diagnose. The majority of cases cannot escape 
recognition if a systematic examination of the nervous system is carried 
out. The minority may successfully simulate a number of other con- 
ditions for a time, and may even elude discovery until post mortem. 
The ordinary atrophic case will seldom be mistaken for progressive 
muscular atrophy if the patient's sensibility is carefully tested. Vague 
sensory disturbances with spastic palsies, nystagmus, and sphincter 
troubles may sometimes suggest disseminated sclerosis, but the absence 
of the remitting history of the latter and the presence of trophic disorders 
and perhaps of congenital anomalies point to syringomyelia. Spinal 
hemorrhage or hematomyelia may reproduce the symptoms and physical 
signs of syringomyelia exactly, but the history of the acute onset will 
make the diagnosis. At the same time there are cases in which the 
evidence points to hemorrhage occurring in a syringal cavity, and thus 
exciting a clinical condition which was merely latent, or others in which 
the track of an old hemorrhage may possibly be the starting point of a 
gliomatosis. 
By far the most difficult problems are presented by intramedullary DIFFUSE AND FOCAL DISEASES OF THE SPINAL CORD 
227 
tumors, on the one hand, and syringomyelia with localized neoplasms 
on the other. The signs of a cavity extending above the level of the 
transverse lesion are often vague and sometimes absent, while the exten- 
sion downward is generally entirely obscured by the paraplegia. For- 
tunately the distinction between these two maladies, equally incurable, 
is not a matter of vital importance. Extramedullary tumors less often 
cause confusion, but in any case of doubt it is better to explore than to 
allow any possibility of a removable growth being left. 
Prognosis.-There is no cure, and therefore the prognosis is never 
favorable. The chances of life are variable, and can only be estimated 
by an extensive observation of the rate at which the disease is progressing. 
The lower the disease the shorter the life is an axiom of some veracity. 
Fortunately the statement can be truthfully made to patients and their 
friends that it is not unreasonable to expect an arrest in the morbid 
process, and to hope that such an arrest may occur before the paralysis 
has reached the stage of complete disablement. 
Treatment.-Drugs have no power to arrest the course, and surgery 
cannot help us. The alleviation of pain, if there is any, and careful 
protection against all the septic processes to which these patients are 
so exposed comprise the chief treatment. In the earlier stages massage 
may temporarily increase the power of enfeebled muscles, but neither 
this nor electricity will do more. Antisyphilitic remedies should be tried 
when a history of lues is obtained. Belladonna and ergot will be found 
useful in relieving the early sphincter trouble and in diminishing painful 
spasms. Suppuration of bones and other tissues must be treated in the 
usual manner, amputation being resorted to with hesitation. 
Speaking generally the patient should be advised to lead a quiet 
healthy life, paying particular attention to the avoidance of all injuries, 
however trivial they may appear to be. 
MYELITIS.1 
Definition.-A non-systemic, diffuse, or focal affection of the spinal 
cord, of heterogeneous yet usually infective and extrinsic origin, discon- 
certing in the variety of its pathological processes, but characterized by 
a clinical picture, which depends for its details in individual cases more 
upon the segmental level than upon the exact nature of the underlying 
lesion. 
The vascular element in the production of spinal softening has gained 
well-deserved recognition in recent years, and as a result of this and the 
prevailing view that myelitis should only be applied to an inflammatory 
condition, the desire to introduce such names as spinal thrombosis and 
myelomalacia has found expression. Syphilis now takes such an impor- 
tant place in the etiology of so-called myelitis that controversy has arisen 
as to how far the preservation of the name in its wide sense is justifiable. 
1 Myelitis from a true etymological standpoint only signifies disease of the spinal 
marrow. The suffix has, however, for a number of years been regarded as denoting 
inflammation. Consequently, by analogy, myelitis is generally held to mean inflam- 
mation of the spinal cord, although it has been used with a great deal of license. 228 
DISEASES OF THE NERVOUS SYSTEM 
The difficulties in the way of a general agreement on this and other 
points may be discussed here because they are of fundamental importance 
in arriving at a true conception of the etiology and pathology of acute 
myelitis. In the first place, the result of an acute or subacute focal 
lesion of the spinal cord is a clinical picture, which varies with the site 
rather than with the nature of the morbid process. It will be much the 
same whether the latter is of inflammatory or vascular origin, and in 
many cases a discrimination between the two is only possible after death, 
and not always then if the disease is of long standing. For this reason 
the term "myelitis" is convenient from a clinical point of view, and has 
by long usage become the natural designation for cases of the kind. 
In the second place, thrombosis can never be regarded as the primary 
and sole factor in any of these cases. As a general rule, it occurs either 
as the result of disease of the bloodvessels or as one of the phases of an 
inflammatory process. In the latter case the primary factor is of such 
a character as to justify the term myelitis. In the former the disease 
of the bloodvessels may be either degenerative, as in certain forms of 
arteriosclerosis, or inflammatory, as in syphilitic arteritis. 
Syphilitic thrombosis has some claim to be called inflammatory, and 
the clinical analysis of the conditions influencing the production of an 
acute paraplegia must be uncommonly close in many instances if the 
observer is to give a truly scientific diagnosis. Further difficulty arises 
from the fact that in syphilitic cases it is often impossible to differentiate 
clinically a spinal thrombosis from an intramedullary gumma. 
The issue must also be considered from its histological side. Is it 
possible to distinguish, even in recent cases, a purely thrombotic from 
an "inflammatothrombotic" lesion? Singer, who has strongly upheld 
the importance of vascular disease in the genesis of myelitis, makes the 
presence or absence of a cellular infiltration the chief criterion upon 
which this question must be decided. He maintains that in cases of 
syphilitic thrombosis, although the larger vessels may show the usual 
perivascular small-celled infiltration, yet the necrosed areas are free 
from cellular infiltration and neuroglial proliferation. In conditions 
of simple inflammation, on the contrary, the excess of cells both in the 
perivascular lymph spaces and in the surrounding tissues is an essential 
and striking feature. The former condition was seen in the cases Singer 
described, and is undoubtedly present in many others. On the other 
hand, there are instances of acute spinal syphilis in which thrombosis 
and consequent necrosis play a very insignificant part, and in which 
gummatous infiltration of the tissues is so marked that many aspects 
of a true inflammation present themselves, with the result that the luetic 
origin of the lesion can only be gauged from the specific changes in the 
walls of the bloodvessels. Under these circumstances inflammation 
is at least as prominent as thrombosis in the microscopic field. If, 
moreover, the opportunity of examining the cord does not take place 
until a long time after the onset, it is more than probable that an accurate 
discrimination between its vascular or inflammatory origin will be impos- 
sible. Necrotic, sclerotic, or rarefied areas might be the result of either 
process, and a thickened, perhaps hyaline condition of the bloodvessels DIFFUSE AND FOCAL DISEASES OF THE SPINAL CORD 
229 
is as likely in the one as in the other. Nor do the points of similarity 
end here. In some cases of inflammation of bacterial origin areas of 
necrosis without cellular infiltration, and not dependent upon vascular 
occlusion, are met with, and are generally supposed to be due to toxic 
influences. 
In spite of the difficulties, there can be no doubt that at the present 
day thrombosis due to syphilitic arteritis, and occasionally phlebitis, is 
responsible for the symptoms of a large number of examples of acute 
myelitis. It is equally certain that the same criticism can be applied 
to cerebral syphilitic thrombosis, and it is to be regretted that the close 
analogy of the two conditions, differing chiefly in their site, is not pre- 
served in their terminology. 
Another point of interest now claims attention. It might be supposed 
that no difficulty would arise in comprising under the term myelitis 
all cases of spinal disease which are presumably due to the action of 
bacteria or their toxins, but this is not universally admitted. There 
are on record instances of paraplegia in which the clinical picture has 
suggested an acute or subacute inflammatory myelitis, but in which 
postmortem examination has revealed no interstitial changes, no cellular 
infiltration of the vessel walls, perivascular spaces, or surrounding tissues, 
but only degenerative changes in the more highly specialized ganglion 
cells and nerve fibres. The question as to how far toxic retrograde 
changes of the parenchymatous elements can be properly regarded as 
constituting evidence of myelitis is one which has raised considerable 
controversy. The two characteristics of this type of case are (1) oedema 
of the tissues and (2) degenerative changes in the nervous elements. 
Both may be present in the more truly inflammatory and infiltrative 
forms of myelitis, but they may be found in certain cases as the only 
changes. Storch and v. Kahlden consider that inflammatory oedema 
is sufficient alone to justify the use of the term "myelitis." Mayer, 
on the other hand, demands an acute infiltration of the vascular adventitia 
and perivascular lymph spaces with small round cells. 
It has long been the custom to describe as "compression myelitis" the 
altered condition of the spinal marrow which has resulted from the 
pressure exerted by tumors, thickened meninges, or displaced bone. 
In the absence of secondary infection by bacteria it is probably more 
correct to look upon these changes as due to evascularization and depen- 
dent upon the ischemia produced by slow vascular occlusion. The intro- 
duction of some term such as compression evascularization of the spinal 
cord is not, however, likely to be welcomed. The only course is to 
retain the word myelitis in its broader and, etymologically, more accurate 
sense, preserving distinctive characters by the use of qualifying adjectives. 
Acute or subacute myelitis will be considered under the following 
heads: (a) Acute infective (or infiltrative) myelitis; (b) acute toxic (or 
degenerative) myelitis; and (c) acute syphilitic myelitis. 
(a) Infective (or Infiltrative) Myelitis.-Etiology and Pathogenesis.- 
Infective myelitis is now, at any rate, a rare affection which has no definite 
relation to age or sex, although young adults are probably its most 
common victims. It does not occur in epidemic form, nor has it seasonal 230 
DISEASES OF THE NERVOUS SYSTEM 
or climatic proclivities. No real importance can be given to the influence 
of chill, trauma, strain, or sexual excess, although it is possible that 
Fig. 16 
Acute infective myelitis. Vessels engorged with blood and surrounded by cells in the 
white matter of the cord. 
Fig. 17 
Acute infective myelitis. Hemorrhage into the white matter. 
these may be of some predisposing or determining value in certain cases. 
On the other hand, the onset has not infrequently occurred in the course DIFFUSE AND FOCAL DISEASES OF THE SPINAL CORD 
231 
of one or other of the specific infections. Barlow and Primangeli have 
recorded fatal cases during or following an attack of measles. Auche 
and Hobbs and Marinesco and Oetlinger have described its association 
with variola. Lepine and Schiff have each seen myelitis occurring as a 
fatal complication of typhoid fever. Gonorrhoea has also been reported 
in a similar connection by Leyden, Gowers, and others. 
The incidence of myelitis upon pregnancy or the puerperium has been 
of sufficient frequency to attract attention, but in many such cases the 
description is not detailed enough to enable one to judge whether they 
shall be included under the inflammatory or the syphilitic groups. There 
is probably a special type of myelitis, which occurs in connection with 
childbearing, and which will be referred to among the "toxic" varieties. 
It may be presumed that the disease can arise in one of several ways: 
1. As an extension from neighboring inflammatory processes, particu- 
larly when the latter involve the meninges or vertebral column. A 
tuberculous form of myelitis occurs in connection with spinal caries and 
less frequently with tuberculous meningitis. . Some degree of myelitis 
is often present in meningitis due to pneumococci, staphylococci, strepto- 
cocci, and other organisms, but in these the symptoms of myelitis are 
largely overshadowed by those originating from the meningeal affection. 
On the other hand, infective myelitis practically never exists without 
some degree of meningitis caused by the same agent. It must then be 
regarded as an essential part of the disease. 
2. As the result of an infection of the spinal tissues through the blood 
stream. This may take place either as an incident in a general pyemia 
or as a purely local phenomenon. 
3. As the result of an infection through the lymphatics. The possibility 
of this is based more on the analogy of experimental pathology than on 
verified cases of human myelitis. The investigations of Homen and others 
prove that infection of the spinal cord may take place along the course 
of the lymphatics accompanying the spinal nerves, and that this may 
give rise to foci of intramedullary inflammation similar in all respects 
to those seen in the human cord. The paucity of clinical evidence on 
this point would be remarkable were it not for the difficulty attending 
their elucidation, but Marinesco has succeeded in tracing an infection 
of the spinal marrow by way of the neural lymphatics in a patient suffering 
from crural gangrene. Further investigation of such sources is urgently 
needed, and might throw light on the pathology of spinal disease following 
cystitis and other septic conditions of the pelvic or abdominal organs. 
The bacteriology of myelitis is only in its infant stage, and is hampered 
by the fact that many organisms appear to be very short-lived in the 
tissues of the spinal cord. Streptococci and staphylococci have been 
found in some cases, and a diplococcus was certainly the causal agent 
in a case reported by T. Buzzard and Risien Russell. Marinesco has 
shown experimentally that myelitis can be produced by several of the 
ordinary pyogenic organisms. 
Pathology.-The gross changes are generally striking and essentially 
the same in character, whether the extent of the lesion justifies the use 
of the terms, transverse, diffuse, or disseminated, in order to further 232 
DISEASES OF THE NERVOUS SYSTEM 
describe it. A transverse myelitis is one which is limited in the longitudi- 
nal direction to one, two, three, or, at most, four segments, and which 
affects the transverse area of the cord within these limits more or less 
completely. A diffuse myelitis involves a considerable length of the 
spinal marrow without definite interruption, and is often the anatomical 
equivalent of a clinical "ascending myelitis." The term "disseminated" 
implies the presence of two or more foci of the disease separated by 
comparatively healthy intervening tissue. 
The morbid appearances vary somewhat with the length of time which 
has elapsed since the onset. In early cases the affected area is softer 
than normal, sometimes diffluent, swollen, and oedematous and generally 
hyperemic throughout. Hemorrhages may give a dark red or brown 
color to the cut surface. The definition between the white and the gray 
substance is frequently lost. The soft meninges on the surface of the 
altered parts present dilated bloodvessels, and in some cases contain 
serous or even purulent exudation. The exudation, if present, is usually 
more profuse on the posterior than on the anterior aspects of the cord. 
In the later cases the softening is at least as marked, but is associated 
with shrinkage rather than swelling of the diseased portions. Hyper- 
emia is less evident, both superficially and on section, and the intramedul- 
lary tissue may be mottled with brown or yellowish patches. At levels 
distant from the seat of inflammation degenerative changes may possibly 
be detected in the ascending and descending tracts of the white column. 
In very severe lesions a segment or two of the cord may be reduced to 
a purulent fluid contained within the pia arachnoid membranes. 
At a still later period the consistence of the diseased area is more firm, 
even sclerotic, and a transverse section may reveal the presence of one 
or more cysts. The tissues are devascularized, in part opaque white, 
and in part gray or translucent in appearance. The secondary degenera- 
tions in the white matter are now very evident to the naked eye. 
Histology.-The features belong partly to the vascular tissues, partly 
to the neuroglia, and partly to the true nervous elements. In individual 
cases the relative importance may vary, but in all examples of this 
particular form of myelitis the vascular phenomena play some part. 
The two striking things are the dilatation of the bloodvessels and the 
excess of nuclear elements in their walls and the surrounding tissues. 
Vascular Changes.-There is general engorgement of all bloodvessels 
both of large and small caliber. The adventitial sheaths and the perivas- 
cular lymph spaces show a large excess of nucleated cells. Examination 
of the latter under a high power proves them to be chiefly of the lymphoid 
type. The majority resemble the lymphocytes of the blood, but plasma 
cells,- mast cells, and polymorphonuclear leukocytes are also present. 
The proportion of the latter to the total number of cells varies very 
considerably, and depends upon factors which are still obscure. If the 
lesion is of some days' duration, large cells filled with the granular products 
of disintegration may also be seen in the perivascular lymph spaces. 
The neuroglia usually presents marked alterations. It is the seat of 
an oedema, in the early stages, at any rate, and microscopic evidence of 
this is either negative or positive. In the former case empty spaces or DIFFUSE AND FOCAL DISEASES OF THE SPINAL CORD 
233 
lacunae represent the fluid which has been absorbed in the process of 
hardening; in the latter these spaces are filled with an amorphous granular 
material deposited from the albuminous fluid by which the tissues have 
been bathed. The neuroglial cells undergo great changes. Many swell 
to a large size and display two or more nuclei which may be vesicular. 
The cell plasma is liable to vacuolation, and the processes may be thick- 
ened or lost. In addition to variety in size and shape, the neuroglial 
cells are greatly increased in number, especially in the neighborhood of 
the dilated vessels and their lymphatics. Among them are also many 
migratory cells from the blood, which are not always easily distinguished 
from those of neuroglial origin. Hemorrhages into the tissues and masses 
of granular debris are also common in these situations. 
Changes in the Nervous Elements-'The nerve cells of the gray matter 
undergo changes which may be partly due to the oedema, but which are 
mainly the result of toxic influences. They become swollen, rounded, 
and homogeneous in appearance. The chromatin granules disappear 
and the nuclei lie excentrically or protrude from the cell periphery. The 
axis cylinder processes are swollen and often broken. The earliest 
changes in the white matter are those which affect the myelin of the 
nerve fibres. This swells and rapidly loses its power of staining deeply 
by the Weigert-Pal method. The Marchi method reveals the presence 
of fatty changes. The axis cylinders are also enlarged in transverse 
and irregular in longitudinal outline. In sections the myelin is apt to 
disappear in places, and the axis cylinders remain naked and unsup- 
ported. 
Three weeks after the onset a Marchi preparation shows that a great 
deal of fatty degeneration has taken place and that much of the fat has 
been taken up by large granular cells, which are probably of neuroglial 
origin. Sections from other parts of the cord show in a striking manner 
the ascending and descending degenerations in the white columns. 
At a later period these degenerations are visible only when the Weigert- 
Pal method is used, and by this time the acute changes in diseased areas 
have been replaced by those which represent attempted repair. The 
vessels have lost much of the perivascular cell infiltration, but their walls 
are thickened and perhaps hyaline in appearance. Proliferated neuroglial 
tissue has replaced the nervous elements which have been destroyed, 
and possibly cysts may indicate areas of necrosis. In the sclerotic zones 
the number and distribution of vessels may suggest that there has been 
some new formation of the latter. Secondary degeneration appears 
in the anterior spinal roots, the efferent fibres of the peripheral nerves, 
and in the muscles innervated by those segments of the cord in which 
the gray matter has been seriously involved. 
Symptoms.-These are dependent on the site and extent of the spinal 
inflammation, and vary accordingly. In the majority the onset is 
associated with some constitutional disturbance, pyrexia, anorexia, 
and perhaps vomiting. The more severe paralytic troubles are usually 
preceded by pain in the back of a burning character and by paresthesia 
in the limbs or in those parts about to be the seat of serious disorders 
of motion and sensation. 234 
DISEASES OF THE NERVOUS SYSTEM 
Transverse.-A focal lesion affecting more or less completely the whole 
transverse area of the cord produces a clinical picture which varies 
according to whether the cervical, dorsal, or lumbosacral regions are 
involved. 
(a) Cervical.-A focal myelitis high up in the cervical region is rare, 
and usually rapidly fatal, owing to paralysis of the entire respiratory 
musculature. More common are cases of myelitis affecting the cervical 
enlargement. In such a case there may be atrophic flaccid paralysis 
of the hand muscles and the flexors of the wrist and fingers, while the 
extensors of the wrist and the muscles moving the elbows and shoulders 
remain intact; at the same time there is partial or complete spastic 
paralysis of all muscles below the arms, including those of the trunk and 
legs with the exception of the diaphragm. Such a distribution of paralysis 
indicates a lesion of the eighth cervical and first dorsal segments, and 
is associated with diminution or loss of cutaneous sensibility in the area 
supplied by those segments and by all the segments below that level. 
Respiration is embarrassed on account of interference with the inter- 
costal muscles, and control over the sphincters is impaired. Absence 
of the abdominal reflexes, exaggeration of the tendon jerks in the lower 
limbs, clonus, and extensor responses are also observed. The incidence 
of the lesion on the eighth cervical and first dorsal segments would 
probably cause irritation or paralysis of the oculopupillary fibres and 
lead to widening or narrowing of the pupils and palpebral fissures. 
The above picture indicates a partial lesion. If the morbid process 
produces a complete discontinuity of function, the paralysis below the 
lesion is of flaccid type, and accompanied by loss of all tendon jerks, at 
any rate for a considerable time. Moreover, the anesthesia is complete 
for all forms of sensation, and trophic changes in the skin, especially 
bedsores, are readily excited. Total paralytic incontinence of urine 
and feces adds to the difficulty in preserving an intact skin. Splanchnic 
paralysis giving rise to distension of the hollow abdominal viscera is 
another result of any transverse lesion above the dorsal region. 
(6) Dorsal.-In this case the arms escape, and it is generally easier 
to locate the upper limit of the lesion from the sensory than from the 
motor disturbances. Paralysis of the lower half of the rectus abdominis 
may, however, give rise to drawing up of the umbilicus when the patient 
attempts to sit up. Examination would then reveal the presence of 
the epigastric and the absence of the hypogastric superficial reflexes, 
which, with the other observation, points to the ninth dorsal segment 
as the highest one involved. The atrophic paralysis due to destruction 
of the lower motor neuron elements is not so easy to detect in the trunk 
muscles as in those of the limbs. The paraplegia resembles that of the 
cervical cases; it is spastic with partial and flaccid with complete lesions. 
If the inflammation is limited to either the left or the right half of the 
transverse area of the cord, the signs are more or less those which have 
already been described under the name of Brown-Sequard's paralysis. 
It is, however, rare for an inflammatory lesion to be so limited as to 
reproduce exactly the symptom complex of an experimental hemisection 
of the cord. DIFFUSE AND FOCAL DISEASES OF THE SPINAL CORD 
235 
(c) Lumbosacral.-Myelitis of the lower parts of the cord may give 
rise either to a mixed spastic and atrophic or to a purely atrophic type 
of paraplegia. Rapid paresis, atrophy, and reaction of degeneration in 
groups of muscles associated with areas of impaired sensibility, loss of 
tendon reflexes, and paralytic incontinence of the sphincters are the 
characteristic features. If the disease is limited to the upper part of the 
enlargement, atrophic palsy of certain muscles of the hip and thigh may 
be combined with spastic paresis of those moving the ankles and toes. 
In such a case the knee-jerks may be absent, and yet ankle clonus and 
extensor plantar responses be easily elicited. A clinical picture of this 
kind can be readily understood if the segmental innervation of the muscu- 
lature is remembered. 
The conditions so far described represent the fully established disease 
in its early days. A certain number of cases run a rapid course to a 
fatal termination, others last a few weeks, but succumb finally to some 
complication. In those which survive, recovery of function modifies 
the clinical picture in varying degree. When the recovery is insufficient 
to allow of walking or standing, the resulting condition, in the cervical 
and dorsal cases, is one of spastic paraplegia in a bedridden patient who 
often suffers from painful flexor spasms of the lower extremities, from a 
sense of constriction at a level varying with the site of the lesion, and 
from retention or incontinence of urine and feces. Improvement in the 
cutaneous sensibility usually precedes and remains in advance of any 
improvement in voluntary power. Such a patient may live for many 
years and enjoy good general health if surrounding circumstances are 
favorable. In less severe instances a remarkable return of function 
may be witnessed and the patient may recover with a normal gait or more 
often with some degree of spastic paraparesis, necessitating, perhaps, 
the aid of a stick. Control over the sphincters may be entirely regained, 
or any deficiency in this respect may only be noticeable in a tendency 
to precipitate micturition or defecation after aperients, or after drinking 
much fluid. Sexual impotence is the rule in most cases of myelitis. 
Complete restoration of sensibility is general in such cases, and the last 
evidence of the disease to disappear is the extensor type of plantar 
response. In the lumbosacral type the prospect of complete regeneration 
of the atrophied muscles is not bright and the more serious interference 
with the sphincters adds to the gravity. Considerable degrees of recov- 
ery, resulting in a condition of partial disablement not unlike that which 
follows lumbar poliomyelitis, may be expected in patients who survive 
the early stages. 
Disseminated Myelitis.-This must be regarded more as a multiple 
of the transverse type than as a separate disease. It is very rare, and 
can only be recognized clinically when, for instance, a focal lesion in 
the dorsal region of the cord causing a spastic paraplegia is associated 
with an atrophic paralysis of one arm or with a patch of anesthesia 
above the level of that which is the result of the dorsal myelitis. Further 
evidence may be afforded by the appearance of some affection of one 
or other of the cranial nerves. 
More often dissemination of the patches of inflammation is only 236 
DISEASES OF THE NERVOUS SYSTEM 
revealed post mortem, the clinical picture having suggested a transverse 
myelitis corresponding to the highest focus in the spinal cord. In other 
cases the clinical course was that of an acute ascending myelitis, although 
examination of the spinal cord showed considerable dissemination of the 
lesions. Possibly there is no room for the two separate terms, dissem- 
inated and ascending, the one referring rather to the anatomical and the 
other to the clinical aspect of the same kind of case. 
Acute Ascending Myelitis.-This forms a morbid entity which can be 
readily recognized during life. It is characterized by a progressive 
paralysis of motion and sensation beginning in the lower extremities 
and more or less rapidly climbing up the body, segment by segment, 
until involvement of the respiratory musculature threatens life, and not 
infrequently produces a fatal termination. The extension of the disease 
to the brain-stem is occasionally indicated by the implication of cranial 
nerves before death, and the occurrence of optic neuritis has been recorded 
in non-fatal cases when all other symptoms have been referable to the 
affection of the spinal cord. With the more usual employment of lumbar 
puncture the bacteriology is likely to become better known. 
(6) Toxic (or Degenerative) Myelitis.-It has already been stated that 
infective or infiltrative myelitis is by no means a common disease. Toxic 
myelitis must be very rare, and the name can only be applied to a small 
proportion of all cases. Very little is known about its origin, although 
it appears from experiments that bacteria and their toxins are able to 
produce similar conditions in the lower animals. Moreover, it seems 
possible that the same organisms may at one time or under one set of 
circumstances produce an infiltrative and at another time or in other 
conditions determine a toxic form of myelitis. It is equally certain 
that the effect of some organisms upon the spinal cord is in part to excite 
inflammation in the interstitial tissues, in part to produce retrograde 
changes in the nervous elements. 
Speaking generally, toxic myelitis differs from infective myelitis in 
the less acute, more subacute onset and course, the small amount of 
constitutional disturbance, and perhaps the power of more complete 
recovery. This last point must necessarily be one rather of speculation 
than of actual fact, but it is natural to suppose that toxic changes, which 
are known to be capable of recovery, leave less permanent effects than 
inflammatory lesions, resulting as they usually do, in necrosis and sclerosis. 
Of the etiology, little can be said, because, as a rule, the toxic nature of 
the process is not discovered until after death. As an exception must 
be mentioned the cases of toxic myelitis associated with pregnancy. 
The close association is shown by the improvement in the myelitis at the 
birth of the child and by its recurrence in subsequent pregnancies. 
Probably some inorganic substances, lead, alcohol, and certain poison- 
ous gases, are capable of producing a toxic myelitis, but if so they are 
not commonly concerned in this connection, and the part they play is 
referred to elsewhere. Diphtheritic toxins are among the possible causal 
agents, but it is doubtful whether they ever excite a general toxic myelitis. 
Among the cases of unknown bacterial origin must be grouped those which 
for many years have gone by the name of Landry's paralysis. DIFFUSE AND FOCAL DISEASES OF THE SPINAL CORD 
237 
Pathology.-Gross changes are not very obvious, but some oedema of 
the spinal tissues with consequent softening may be observed. On 
cross-section some degeneration may be seen in the white columns. 
Microscopically, the chief changes consist in swelling of the myelin 
sheaths and of the axis cylinder processes, especially in the white matter. 
The change is generally patchy and shows no tendency to follow a sys- 
temic distribution. In addition there are toxic changes in the nerve 
cells as well as in those of the neuroglia. The ganglion cells swell, become 
rounded and chromatolytic with excentric nuclei. The neuroglial cells 
also become round and enlarged, but do not show the great increase 
in number characteristic of the infective form. In the early stages the 
Marchi stain demonstrates fatty changes in the myelin sheath; later on 
the disappearance of groups of these sheaths and the dilated spaces they 
leave give a somewhat "worm-eaten" appearance to the affected areas. 
Symptoms.-These depend upon the site of the disease and resemble 
those of the infective type in all respects. The onset and course are 
generally less acute, and the power of recovery is probably greater, but 
in the majority of cases it is quite impossible to differentiate the two 
conditions at the bedside. Motion, sensation, and sphincter control 
are all impaired and the relative amount of muscular spasticity and 
atrophy varies with the degree in which the upper and lower neurons 
are involved in the morbid process. In a case described by Rhein the 
clinical picture resembled that of a subacute ascending myelitis of the 
inflammatory kind. Post mortem, only cell changes and myelin degen- 
eration were found to account for the symptoms. The cases in pregnant 
women are also characterized by an ascending progress of their symptoms. 
In fatal instances bulbar symptoms followed those of spinal origin. 
(c) Syphilitic Myelitis.-Etiology.-Syphilis is by far the most im- 
portant factor in the production of myelitis, and for this reason cases 
of syphilitic myelitis easily outnumber those of other origin. Singer 
found that out of 19 cases of myelitis admitted in the course of two years 
into the National Hospital (London), 15 had a definite history of syphilis. 
In 12 of them the onset of myelitis occurred within three years of the 
primary infection. In the opinion of the writer these figures, roughly 
80 per cent., do not exaggerate the preponderating causal influence of 
syphilis. Males are much more frequently affected than females, and 
the greatest susceptibility prevails during the third, fourth, and fifth 
decades. It is probable that age has also some influence upon the rapidity 
with which myelitis follows the infection, the interval being shorter in 
the younger patients. This interval varies from a few months to many 
years, but the liability to the disease diminishes after three years have 
elapsed. Gummatous meningomyelitis may also be the result, although 
rarely, of congenital syphilis. In at least 75 per cent, of cases of syphilitic 
myelitis the incidence of the disease is upon the dorsal region of the cord 
and more particularly upon the middle third of that region. This may 
be explained by the fact that this area is more poorly supplied with large 
contributing root vessels than any other, and is in distinct contrast to 
the incidence of the infective type which favors the lumbar and cervical 
enlargements at least as frequently as the dorsal region. 238 
DISEASES OF THE NERVOUS SYSTEM 
It may be doubted whether any other factor than that of lues is of 
material importance in the etiology of this form, but it seems possible 
that great fatigue, exposure, and perhaps alcoholism may have an exciting 
influence. It is certainly common among soldiers. 
Pathology.-The influence of the syphilitic virus upon the spinal tissues 
is not a simple one, but is exerted in a number of different ways. Syphil- 
itic myelitis is really the manifestation of more than one morbid process, 
and each variety may be represented to a greater or less degree in one 
case of the disease. It may be said that the spirochaete produces its 
results in part by its power of causing changes in the walls of bloodvessels 
with consequent effects upon the local circulation, in part by the diffusion 
of toxic substances capable of exciting profound influence upon certain 
of the tissues, and in part by exciting a cellular infiltration of an inflam- 
matory or granulomatous character. 
Any or all of these results may be concerned in any particular case, 
but, as a rule, one or other has more or less preponderating importance. 
It must always be remembered that morbid changes may be silently at 
work for a considerable time before some sudden catastrophe, such as 
the occlusion of an important bloodvessel, provides a suggestion of 
acuteness to the illness to which it is in reality hardly entitled. For 
much the same reason it is the rule to find evidence of syphilitic changes 
in the bloodvessels and meninges over a much wider area of the cord than 
the segment or two forming the site of the lesion which has determined 
a disturbance of function. Although concerned for the moment with 
the intramedullary changes, the fact that these are often secondary to, 
or intimately associated with, syphilitic processes in the surrounding 
tissues, especially with those in the dura mater, must not be lost sight of. 
The incidence of syphilitic myelitis is much more frequent in the dorsal 
region than elsewhere, but wherever placed the focus of disease is generally 
well defined. The lesion is usually single and generally limited to a very 
few segments, so that it may be included under the term transverse 
myelitis. The diseased area is recognized by its soft consistence and 
possibly by some slight alteration in color. The pia arachnoid overlying 
it may be milky and contain distended veins, but the milkiness may not 
be more noticeable at the level of the softening than elsewhere. In 
long-standing cases there is marked narrowing in the caliber of the cord, 
as well as a wrinkled condition of the membranes covering it. Atrophy 
of anterior roots arising from the softened segments may be detected. 
Adhesions connecting the dura mater with the leptomeninges are of 
frequent occurrence. 
Histology.- The Bloodvessels.-The vascular changes may be divided 
into at least three chief types: (1) Moderate perivascular and adventitial 
cell proliferation with thickening of the intima of sufficient degree to 
produce diminution or complete occlusion of the lumen. This may be 
very general throughout the cord or confined to the area of softening. 
The proliferation of small round cells may only be slight, and yet the 
walls of the vessel may be very thickened and perhaps hyaline in appear- 
ance. This would suggest that the morbid process has been subacute 
or chronic, and that the acute onset of symptoms has been due essentially DIFFUSE AND FOCAL DISEASES OF THE SPINAL CORD 
239 
to a rapid ischemia originating in a local obstruction or following on a 
general lowering of blood pressure. (2) Excessive perivascular cellular 
infiltration with little or no endarteritis. In this case there may be no 
evidence of actual obstructive thrombosis, although slowing of the 
circulation and blood stasis is suggested by the excess of white corpuscles 
in the vessels. On the other hand, the changes in the neighboring tissues 
are probably due to a toxic necrobiosis or to an extension of the cellular 
proliferation from the immediate precincts of the vessels. (3) Paralytic 
vasodilatation with very moderate perivascular infiltration and with no 
obvious thickening of the vessels. Retardation of the blood stream with 
capillary thrombosis and profound alterations in the nervous elements 
are the characteristics of this change. The general appearance produced 
is one of hyperemia of the white as well as of the gray matter associated 
with retrograde changes in myelin sheaths and ganglion cells. 
The Neuroglia.-This offers the greatest amount of resistance to the 
vascular changes. In areas of complete necrosis it succumbs with 
everything else, the cell nuclei rapidly lose their staining reaction, and 
the cells as well as fibres taking part in the general liquefaction. In 
other regions the neuroglial cells survive when the more specialized 
structures are destroyed. Under these circumstances they are enlarged, 
rounded, and often multinuclear. In later stages they proliferate and 
take a leading part in the process of repair, sending out long interlacing 
fibres in all directions. It is not improbable that they give origin to at 
any rate some of the large granular cells, which act as scavengers and 
which, at first distributed in the tissues, are later collected in large num- 
bers in the perivascular lymphatics. With the neuroglial changes must 
also be mentioned the gummatous infiltration which occasionally goes 
hand in hand with the vascular phenomena. In some instances this 
forms the predominant feature, and a true neoplasmic gumma, with the 
usual central degeneration and caseation, occupies a considerable part 
of the transverse area of the cord. In others the aspect is that of a 
gummatous meningomyelitis, either involving the cord in the form of a 
ring or penetrating its substance at one or two points. 
The Nervous Elements.-Demyelinization of the nerve fibres and 
retrograde changes in the ganglion cells are the results either of the 
circulatory disturbances or of the diffusion of toxins. The myelin sheaths 
swell, disintegrate, and finally disappear, leaving axis cylinders in a naked, 
unsupported, but often swollen and perhaps varicose condition. The 
ganglion cells undergo the usual changes in shape, in granulation, and 
in nuclear displacement, which have been described. These alterations 
are local and do not extend into distant parts of the cord. 
The Leptomeninges.-Round-cell infiltration of the pia arachnoid is 
almost universal in the syphilitic form, and is usually associated with 
varying degrees of meningeal thickening. The arteries are often hyper- 
trophied, and the veins may display an obliterative phlebitis. The walls 
of the latter not infrequently present a laminated appearance, layers of 
round cells being separated from each other by strands of connective 
tissue, so that their bulk is as great as that of the arteries. 
The ultimate results resemble those of other forms of myelitis. The 240 
DISEASES OF THE NERVOUS SYSTEM 
processes of repair, of sclerosis, of new vessel formation, and perhaps of 
cyst or cavity production are essentially the same, and an examination 
of a focal lesion a year or more after the acute stage might reveal nothing 
which would enable one to speak with certainty as to its exact origin. 
The type of syphilitic myelitis which has been referred to as transverse 
is the common one, and it is rare to find a diffuse myelitis of acute or 
subacute syphilitic origin. Occasionally a few patches of disease scattered 
through a few segments may invite the term "disseminated," but the 
clinical picture even then is rather that of a transverse myelitis. On 
the other hand, it is not rare for a spinal syphilitic lesion to be associated 
with a cerebral one of the same nature. 
Symptoms.-Perhaps no disease has a more characteristic onset than 
syphilitic myelitis. A man in good health, for no reason that he can see, 
finds himself unable to empty his bladder, or finds one or both legs heavy 
and weak, numb and "prickly." He is reassured in a few hours by the 
disappearance of this trouble, and it may be two or three days or two 
or three weeks before anything further occurs. Then, perhaps after an 
unusually long day's work, or perhaps with no departure from the ordinary 
routine, he rapidly, sometimes suddenly, loses power in his legs, loses 
his ability to micturate, and is conscious of a numbness from his waist 
downward. Throughout this period he has experienced no feeling of 
illness, and unless he is anemic and sallow, as so many victims of recent 
syphilis are wont to be, has been regarded by his relations as strong and 
healthy. Even as he lies in his paraplegic condition his appetite and 
temperature are little disturbed. Such a history is common enough, 
but it may be varied by the absence of any premonitory symptoms or 
by the more gradual development of the paralysis. For instance, one 
leg may be paralyzed some hours or even a day or two before the other, 
and very occasionally the disablement is limited from the first more or 
less completely to one lower extremity, with the production of a modified 
form of Brown-Sequard's paralysis. Not uncommonly the patient has 
gone to bed in his usual health, and on getting up in the morning falls 
to the ground. Pain is not a prominent feature; it may be absent alto- 
gether, or the patient may complain of a burning sensation at a particular 
spot on the spinal column. A girdle sensation corresponding roughly 
to the upper border of paralysis may be present early or may supervene 
in the later stages, but paresthesia referred to the paralyzed limbs is 
frequently experienced. Such is the onset of a dorsal syphilitic myelitis, 
and the results of an examination will correspond more or less exactly 
to those alluded to under infective myelitis of the same region. Accord- 
ing to whether the physiological continuity of the spinal cord is completely 
or incompletely severed at the site of the lesion a flaccid or a spastic 
paraplegia will present itself. In the former case paralysis will be com- 
plete from some level on the trunk downward, the legs will be extended, 
toneless, and powerless; all deep and superficial reflexes will be abolished. 
The bladder may at first empty itself at intervals as a reflex involuntary 
act without the consciousness of the patient, but later will dribble more 
or less continuously or whenever any change of posture is attempted 
or the abdomen is pressed upon. The relaxation of the anal sphincter DIFFUSE AND FOCAL DISEASES OF THE SPINAL CORD 
241 
may be realized by the introduced finger; solid feces may be retained, 
but diarrhoea will lead to constant soiling. Incomplete priapism is 
sometimes observed. Loss of sensibility to all forms of stimuli exists 
up to the segmental area corresponding to the spinal disease, and a band 
of hyperesthesia may be present just above that level. The skin of the 
lower extremities is dry and sometimes cedematous. Of extreme and 
urgent importance is the liability to bedsores and cystitis. 
In the spastic cases the lower extremities are usually flexed at the hip 
and knee, and strong adductor spasm brings the limbs in apposition. 
The tendon-jerks are lively; ankle clonus and extensor responses are 
easily elicited. The mere handling of the limb or the movement of 
bedclothes suffices to excite painful clonic spasms, which may last for 
some little time. The relation of the sensory disorders to the paralysis 
is very variable, but sensation is nearly always less affected than motion. 
Constipation and retention of urine with overflow incontinence are the 
characteristic sphincter troubles in this type. 
In the more rare cervical and lumbosacral cases the symptoms are 
those already described under infective myelitis. 
A few words only are necessary for the purpose of indicating the course. 
A flaccid paraplegia may become spastic in the course of a few days or 
a week or two. Rarely does a case of the flaccid type survive long in 
that condition, a fatal termination being usual. The spastic cases, 
under appropriate treatment, display a general tendency toward improve- 
ment and the less complete the initial paralysis the more favorable is 
the ultimate outlook. Only a very few cases recover completely, but 
a considerable number attain a condition in which they are able to get 
about with more or less difficulty and with greater or less degrees of 
spastic gait. Not infrequently the sphincter troubles prove obstinate. 
Diagnosis.-This can be conveniently divided into two parts: (1) The 
diagnosis of acute myelitis from other forms of paralysis, and (2) the 
discrimination between the various forms of acute myelitis. 
1. The diagnosis of acute myelitis from other forms of paralysis is 
not attended, as a rule, with much difficulty in the simple transverse 
cases. The rapid onset of a paraplegia with a well-defined upper limit 
to the motor and sensory disturbances could only result otherwise from 
some sudden pressure exerted by displaced bone or by a tumor. In the 
very exceptional cases in which symptoms of compression are really 
acute there is usually some indication of the gross nature of the lesion. 
Moreover, pain is generally a more prominent feature in these cases. 
Compression paraplegia developing in a patient who is suffering from 
cancer may suggest acute myelitis if the possibility of compression is 
forgotten. More deceptive are the attacks of acute, although often 
incomplete, paraplegia which form part of the course of disseminated 
sclerosis, and which sometimes constitute the earliest symptom of that 
disease. The absence of a well-defined upper limit to the paralysis 
and the presence of some unsuspected symptom or physical sign above 
the level of the lesion will generally clear up any doubt. The observer 
should pay particular attention to the cranial nerves and keep a sharp look- 
out for nystagmus, diplopia, or pallor of the optic disks, the presence of any 
vol. v-16 242 
DISEASES OF THE NERVOUS SYSTEM 
one of which would point strongly toward the diagnosis of disseminated 
sclerosis. An acute form of multiple sclerosis has occasionally been 
described, and may require careful differentiation from acute dissemi- 
nated myelitis. A lumbosacral myelitis with its atrophic changes in the 
lower extremities may simulate an acute poliomyelitis until the presence 
of anesthesia and of severe sphincter troubles is recognized. 
Only a few years ago the distinction between myelitis and peripheral 
neuritis was a prerogative of the expert. Nowadays it is hardly necessary 
to draw attention to the differences between the two diseases. In the 
dorsal and cervical cases no mistake can arise. In the lumbosacral form 
of myelitis it must be remembered that the distributions of sensory and 
motor phenomena follow a segmental arrangement, and have not a 
special incidence upon the peripheral parts of the limbs as they have in 
neuritis. Furthermore, the sphincter disturbances of the myelitic, and 
the muscular tenderness of the neuritic cases help to distinguish the two 
conditions. Finally, a case of peripheral neuritis is not very often suffi- 
ciently acute to give rise to the suspicion of an acute myelitis. 
In all cases of myelitis lumbar puncture should be performed, and the 
examination of the cerebrospinal fluid will sometimes settle any doubts. 
Many cases of dorsal myelitis have been regarded in their first stage as 
functional or hysterical, especially when young women have been the 
victims. Loss of power and sensibility, very brisk tendon-jerks, pseudo- 
clonus, and even retention of urine may be found in functional paraplegia, 
but incontinence of urine, of feces and, above all, an extensor type of 
plantar reflex serve as infallible signs of an organic basis for the 
paralysis. 
2. A diagnosis of transverse myelitis having been arrived at, the next 
question to be answered really amounts to this: Is it syphilitic or the 
result of some other infective process? The history, or absence of his- 
tory, of syphilis must of course be allowed due weight, and when an 
undoubted luetic record exists it will necessarily indicate the line of 
treatment. In doubtful cases the mode of onset affords some guide. 
With those of syphilitic origin premonitory symptoms are often observed 
over a considerable period before the actual development of the disease, 
and the initial stage is generally associated with no constitutional dis- 
turbance. In the infective type, on the other hand, warnings are less 
common, and, when they do occur, precede by a few hours only the 
onset of paralysis. Moreover, some degree of general malaise, general 
pain, pyrexia, and perhaps vomiting are the rule rather than the excep- 
tion in these patients. The site of the lesion is not of very great diagnostic 
significance, but, ceteris paribus, the dorsal region favors the syphilitic, 
and the cervical enlargement the infective form. The examination of 
the cerebrospinal fluid may or may not be of assistance. The presence 
of organisms or the discovery of the Spirochoeta pallida would of course 
be more or less decisive if secondary infections could be excluded. A 
preponderance of polymorphonuclear leukocytes would point to a non- 
syphilitic virus, but a lymphocytosis does not afford definite proof of the 
luetic nature of the lesion. A positive Wassermann reaction in the blood 
associated with a positive or negative reaction in the cerebrospinal fluid DIFFUSE AND FOCAL DISEASES OF THE SPINAL CORD 
243 
is the most satisfactory evidence of syphilis, but a negative reaction in 
both fluids does not necessarily exclude it. 
If syphilis is suspected careful search should be made for corroborative 
evidence in other parts, and the pupillary light reaction in particular 
should be the object of investigation. A myelitis not infrequently repre- 
sents one feature or phase of cerebrospinal syphilis. The writer has seen 
tabes, myelitis, and cerebral thrombosis present in one subject. Equally 
important in the infective type is the discovery of some source for the 
secondary invasion of the cord, and the possibility of gonorrhoea as well 
as of septic processes must be diligently inquired into. 
The acute ascending types of myelitis, whether of infective or toxic 
origin, present a clinical picture, which may for a moment suggest a 
Landry's paralysis or an acute poliomyelitis, but the presence of sensory 
disturbances and of sphincter trouble suffice to exclude the latter diseases. 
Prognosis.-The general outlook has already been indicated in de- 
scribing the progress of the different forms, but certain principles may 
be deduced which should be of use in an individual case. The severity 
of the lesion is of importance both as regards the prospect of life and the 
hope of useful degrees of recovery. A complete loss of conducting power 
in the cord at the site of disease, although it may only be temporary, 
is a menace to life whenever it presents itself, and the higher the level 
of the lesion the greater the immediate danger. Interference with the 
respiratory musculature may lead to a rapid fatality, and even when 
the dorsal region is the part affected the tendency to severe decubitus 
and to cystitis with its renal complications constitutes a grave element. 
Should the patient survive these dangers, the prospect of good recovery 
in such severe cases is a very poor one. Instances of lumbar myelitis 
are particularly hopeless in regard to the possibility of regaining the 
power of walking, and their irremediable sphincter troubles usually 
determine an early death. Less complete paraplegias may be confidently 
expected to show marked improvement, but only after the effects of 
treatment, especially in the syphilitic cases, have been gauged, can any 
precise estimate of the ultimate result be formed. It must be stated that 
cases of spastic paraplegia resulting from myelitis are disappointing to 
themselves and to their physicians. Improvement sets in and goes on 
up to a certain point, and then progress is arrested and a stationary 
condition, obstinate to all forms of treatment, is maintained. 
Treatment.-Infective and Toxic Cases.-In the acute stage the patient 
must be given complete rest preferably, and often imperatively, on a 
water bed. Changes of posture are necessary for the prevention of sores, 
but cannot be seriously recommended for any influence they can exert 
upon the morbid process. The supposition that the volume of blood in 
the spinal cord is lessened by placing the patient on his face, or that 
such a depletion, if it occurs, is beneficial, does not appear to have any 
reasonable basis. On the contrary, the determination of blood to the 
site of infection must be regarded as an essential factor in Nature's 
effort of resistance and the effect of gravity in altering the hyperemia 
as insignificant. Immediate attention should be paid to the bladder, 
and the urine drawn off if there is retention. Free action of the bowels 244 
DISEASES OF THE NERVOUS SYSTEM 
should be secured at least every alternate day after an initial purge, 
and the greatest care must be taken to keep the skin clean. If pyrexia 
and pain are present, a diaphoretic mixture containing salicylates or 
quinine and a light fluid diet are indicated. There is no reason to believe 
that the application of heat or cold to the spine can be of use, and dis- 
turbing the patient for the purpose of cupping is probably more harmful 
than beneficial. A lumbar puncture should be performed at the earliest 
opportunity and an examination of the fluid carried out. As soon as 
acute symptoms have passed off all paralyzed parts should be rubbed 
and moved every day, and each joint prevented from becoming fixed. 
In the dorsal cases the spastic muscles need no treatment, but in the 
lumbosacral cases atrophied muscles of the lower extremities should 
receive electrical treatment, provided that this can produce contractions. 
The kind of current must be chosen accordingly. A careful watch must 
be kept upon the urine and any sign of cystitis combated by means of 
hexamethylenamine by the mouth and by irrigation of the bladder with 
some antiseptic solution. The sphincter trouble may in the less severe 
cases be favorably influenced by giving belladonna internally, and the 
same drug combined with extract of ergot may relieve the painful flexor 
spasms which are particularly liable to be troublesome at night, and 
when sleep is much disturbed by them the use of such drugs as veronal, 
sulphonal, and even morphine is not only justifiable, but urgent. 
As the patient gains in strength more energetic massage and passive 
movements may be carried out, and he should make every endeavor to 
perform movements on his own account. It is his duty to force impulses, 
as it were, through the block on the conducting lines or to find some other 
way round for the resumption of traffic. This side of the treatment is apt 
to be forgotten in the modern craze for massage and electricity. If the 
patient can be induced to attach less importance to the energy displayed 
by the rubber and to impart more energy into his own efforts at initiating 
voluntary movements, the medical attendant will have gained valuable 
cooperation. When the limit of improvement by these means appears 
to have been reached a change to some suitable health resort should be 
advised. 
Syphilitic Cases.-Antisyphilitic treatment can in most cases be 
commenced as soon as paralysis sets in, or, better still, during the pre- 
monitory stage. Both mercury and iodide of potassium should be given, 
the method of administration being of minor importance. Beginning 
with 5 grains (gm. 0.3) three times a day, the iodide of potassium should 
be increased rapidly until 20 to 30 grains (gm. 1.3 to 2) are taken in each 
dose. The mercury may be given by the mouth in the form of perchloride 
or the red iodide, by inunction, or by hypodermic injection, but it is not 
advisable to apply the inunction or the injection to anesthetic parts. 
This should be pushed to salivation and renewed after an interval of 
two or three weeks. Unless there are contraindications in the way of 
serious cardiac or renal disease, intravenous injections of salvarsan 
should be given early in addition to the mercurial treatment. Two or 
more injections may be given at intervals of a week, according to the 
effect produced upon the Wassennann reaction, and the condition of 245 
DIFFUSE AND FOCAL DISEASES OF THE SPINAL CORD 
the cerebrospinal fluid. Bearing in mind the fact that the victim of 
syphilitic myelitis is prone to other manifestations of cerebral or spinal 
syphilis, and that there is no criterion as to when that liability is suc- 
cessfully eradicated, the patient should undergo a course of mercurial 
treatment two or three times a year for an indefinite period. 
Further injections of salvarsan may be necessary if indications of 
further syphilitic lesions appear, or if the Wassermann reaction of the 
blood does not remain negative. 
CHRONIC MYELITIS. 
There are perhaps few facts more deserving of recognition in neuro- 
logical medicine than the extreme rarity of a primary chronic myelitis. 
One meets with patients who exhibit the results-generally a stationary 
spastic paraplegia-of a former acute or subacute myelitis, but rarely, 
if ever, does one encounter a case in which slow progressive symptoms 
of a spinal affection justify the diagnosis of a chronic myelitis. If such 
a diagnosis suggests itself every effort must be made to exclude (1) the 
various forms of systemic scleroses, such as subacute combined sclerosis, 
amyotrophic lateral sclerosis, etc.; (2) disseminated sclerosis; (3) syrin- 
gomyelia; and (4) compression paraplegia due to tumors or vertebral 
disease. It is to the exclusion of spinal compression that attention 
must especially be paid, because the life of the patient may depend on 
the accuracy of the opinion. 
It may well be asked whether, if the chronic stages of acute myelitis 
and the list of diseases quoted above are put on one side, there is any 
condition which deserves the name of chronic myelitis. The answer to 
this is in the negative if an exception is made in favor of certain ill- 
defined and rare cases of syphilitic spinal diseases to which reference will 
shortly be made. In other words, slowly progressive symptoms pointing 
to a lesion of the spinal cord indicate in the vast majority of cases one 
or other of the diseases above mentioned, and not a chronic myelitis. 
It is true that a chronic myelitis secondary to some infective process, 
such as tuberculous disease of the vertebrae and spinal membranes, 
may be induced, but the primary cause must be firmly established before 
the chronic myelitis can be legitimately inferred. 
In former days the term chronic myelitis was used as a cloak to 
ignorance, and many cases of multiple sclerosis, of subacute combined 
sclerosis, and particularly of compression paraplegia went under that 
designation. The importance of a general recognition of this is especially 
obvious in cases in which there is evidence of a focal transverse lesion 
of the spinal cord with symptoms increasing in intensity. These must 
not on any account be labelled chronic myelitis, but, speaking generally, 
must be submitted to surgical exploration in the hope that the removal 
of a tumor pressing upon the cord may change the prognosis of the 
case from that of a progressive incurable paraplegia to one which holds 
out good prospect of a complete or partial recovery. Emphasis having 
been laid upon the rarity of any spinal disease justifying the diagnosis 
of chronic myelitis, a condition which is known under the title of Erb's 246 
DISEASES OF THE NERVOUS SYSTEM 
syphilitic paraplegia may be briefly described, although the writer has 
some doubts as to how far a separate morbid entity answering to that 
name may fairly be recognized. 
Erb's Syphilitic Paraplegia.-In 1892 Erb sought to establish a type of 
disease to which he gave the name "syphilitic spinal paralysis." Ac- 
cording to his view the disease was a form of spastic paraplegia character- 
ized (1) by its syphilitic origin; (2) by the marked exaggeration of the 
tendon reflexes as compared to the moderate degree of muscular rigidity; 
(3) by bladder trouble, which might precede by a long time the other 
symptoms; (4) by distinct paresthesias of subjective rather than objec- 
tive character; and (5) by the gradual development of the disease and 
its tendency to improve under appropriate treatment. Rumpf had 
already shown that all these symptoms could be the result of syphilitic 
disease of the bloodvessels of the cord, though he had not laid so much 
emphasis on the activity of the reflexes in relation to muscular rigidity. 
At the present time it must be confessed that this point is not character- 
istic of Erb's type, because it may be observed in the late stages of acute 
myelitis and under other conditions. 
The occurrence of this form of spastic paraplegia with a gradual onset 
and progressive course must be very rare, but it is interesting, as it 
provides at any rate one possible form of chronic syphilitic myelitis. 
It would be suggested in any particular case by a history of syphilis, 
and its diagnosis would be readily confirmed by a lumbar puncture, 
with examination of the fluid, and also by the results of antisyphilitic 
treatment. In a case of the kind which came under the writer's obser- 
vation the bladder trouble anticipated the other spinal symptoms by 
many months, thus raising the suspicion that some urethral disease 
was responsible for the difficulties associated with micturition. 
Pathology.-Little is known about the anatomy of these cases, because 
the majority, which have been called examples of Erb's syphilitic para- 
plegia during life, have been shown postmortem to be really the subjects 
of a transverse syphilitic myelitis in the dorsal region of the cord, with 
the usual ascending and descending tract degenerations. Other cases 
are the result of diffuse syphilitic disease of the spinal vessels, sclerosis 
taking place in areas which are poorly nourished. Finally, it is stated, 
without much positive evidence, that the syphilitic toxins may induce 
a degeneration of the pyramidal tracts and of the long ascending tracts 
in the posterior and lateral columns without the intervention of any 
specific changes in the bloodvessels or meninges of the cord. 
Diagnosis.-This must depend on the progressive character of the 
symptoms and on the results of lumbar puncture and antisyphilitic 
treatment. The absence of pain serves to distinguish it from most, 
but not all, cases of compression due to tumors or vertebral disease. 
The definite limitation of motor and sensory disturbances to parts of 
the body innervated from below a certain level in the spinal cord must 
always suggest a focal lesion, and not a diffuse affection of the long 
tracts in the cord, such as is found in Erb's syphilitic paraplegia. 
Prognosis.-Appropriate treatment tends to ameliorate the spinal 
symptoms, and some improvement in the paraplegia may generally be DIFFUSE AND FOCAL DISEASES OF THE SPINAL CORD 
247 
anticipated, especially if an early diagnosis is made and acted upon. 
On the other hand, it is unusual to obtain a perfect recovery, particularly 
in regard to the vesical troubles. A patient may reach a stage in which 
he is able to perform all his ordinary duties, and even to walk many 
miles without great fatigue, but his control over micturition often 
remains very incomplete. 
Treatment.-What has already been said with regard to the treatment 
of acute syphilitic myelitis applies to this more chronic type of lesion. 
The danger of bedsores and of cystitis is not nearly so urgent, and 
complete rest is rarely indicated. If the bladder trouble proves intrac- 
table, regular daily catheterization, with due precautions against sepsis 
should be instituted, and the patient taught to carry this out himself. 
A portable urinal is sometimes a convenience and occasionally a necessity. 
LANDRY'S PARALYSIS. 
History and Introduction.-During the last half century this disease 
or its title, has enjoyed a chequered character. Every form of acute 
ascending paralysis which has borne the faintest resemblance to the 
case originally described by Landry has been called Landry's paralysis, 
whether the changes in the cord have been gross or insignificant, with the 
result that the literature has become hopelessly confused. E. W. Taylor 
says that " the tendency to consider cases of rapidly advancing paralysis, 
whether fatal or not, regardless of pathological findings, as Landry's 
paralysis, is very noticeable in much of the best recent work in America;" 
but this tendency is not confined to America, and may be found in any 
country of importance. 
This unfortunate confusion can be traced to three principal sources. 
In the first place, many cases of acute poliomyelitis of the adult have 
been mistaken for Landry's paralysis during life, and the diagnosis 
has not been revised when examination of the spinal cord has revealed 
the inflammatory changes characteristic of the former disease. In the 
second place, the occurrence of acute cases of polyneuritis of unknown 
toxic origin has led observers to believe that Landry's original case, 
in which the peripheral nerves were not examined, was only an example 
of this condition. In the third place, acute ascending myelitis presents 
features which have provoked the diagnosis of Landry's paralysis in 
certain instances. 
The writer desires to avoid any attempt, which can never be successful, 
to reconcile the various views which have been, and are, held upon this 
subject, or to classify the different morbid processes which have at one 
time or another carried the name of Landry's paralysis. It does not 
matter, and it will certainly never be known, what was the actual disease 
from which the cases recorded by Landry suffered. It is sufficient for 
our purpose that he called attention to a class of case in which the 
symptoms were those of a rapidly spreading motor paralysis, without 
atrophy or electrical changes in the muscles, with slight sensory pheno- 
mena and no sphincter disturbances, and in which he was unable by the 
methods at his disposal to detect anatomical changes. Does this class 248 
DISEASES OF THE NERVOUS SYSTEM 
of case still exist? The answer to this question is in the affirmative 
if allowance is macle for improved histological technique, and if it is 
not forgotten that Landry would certainly have been able to detect 
the changes of acute poliomyelitis or any other acute inflammatory pro- 
cess in the spinal cord. It is clear, however, that "acute ascending 
paralysis" and "Landry's paralysis" cannot be regarded as synonymous 
terms; the latter is only one variety of the former. 
It will be the object of this article to describe a morbid condition, 
which corresponds clinically to Landry's cases, in which the anatomical 
changes in the spinal cord would certainly have escaped notice with 
the methods of fifty years ago, and which presents no grounds for its 
inclusion in the group of the neuritides. There is nothing to be gained 
from imitating the course adopted by some writers, who describe a 
central and a peripheral form of Landry's paralysis. 
Etiology and Bacteriology.-There are but few points of etiological 
importance, if we exclude those cases which should properly be classed 
under the term myelitis. A recent history of some infectious disorder, 
such as gonorrhoea, influenza, or typhoid fever, is sometimes forthcom- 
ing, but not with sufficient constancy to merit attention. In the majority 
of instances the disease attacks healthy adults, and the period of life 
between twenty and forty appears to be the most susceptible. Men 
are more often affected than women, but it is difficult to say whether 
the preponderance is great or insignificant. Exposure to extremes of 
heat or cold may be a predisposing factor. Seasonal or climatic influences 
are not known to play any part, nor is there any evidence of the occur- 
rence of epidemics. During epidemics of acute poliomyelitis the earlier 
severe cases have often been mistaken for examples of Landry's paralysis 
until the opportunity for examining the tissues has revealed the true 
condition. 
The bacteriology is in an immature condition, and it is not possible 
to say whether the disease is specific or whether it may occur as a result 
of various forms of intoxication, bacterial or otherwise. The large 
majority of cases in which spinal lesions have been absent or insignifi- 
cant have given a negative response to bacteriological investigation. 
On the other hand, a few have provided findings of some interest. Roger 
and Josue recorded a case of acute ascending paralysis in which they 
found toxic changes in the cells of the lumbar enlargement. Sections 
stained for bacteria did not demonstrate the presence of any organism, 
but cultivation from the heart blood produced a diplococcus resembling 
the pneumococcus. Injections of the coccus into a mouse did not prove 
fatal, but a rabbit submitted to the same experiment died at the end 
of twenty-one days with symptoms of paraplegia, and the organism was 
recovered from its blood. 
The writer investigated a case of the disease which was in the National 
Hospital under the care of Sir William Gowers with the following results: 
(1) A micrococcus was isolated in pure culture from the blood of the 
patient after death. (2) An organism indistinguishable from that which 
was cultivated was found in large numbers in the loose vascular tissue 
forming the external layer of the spinal theca. (3) A subdural injection DIFFUSE AND FOCAL DISEASES OF THE SPINAL CORD 
249 
of the cultivated coccus into a rabbit produced after some days a rapidly 
spreading palsy. (4) The same organism was discovered in the theca 
of the rabbit, and isolated in pure culture from its blood. (5) In neither 
the patient nor the rabbit was the organism demonstrated in the spinal 
cord or the pia arachnoid, and in neither case were there inflammatory 
reactions in these tissues. Macnamara and Bernstein isolated from the 
blood and cerebrospinal fluid of a non-fatal case a coccus which bore 
some resemblance to that just described, but they were unable to obtain 
any positive results from experimental inoculations. 
Pathology.-To the naked eye the central and peripheral nervous 
systems present little that is remarkable, with the exception of some 
general hyperemia of the cord. It is particularly to be noted that this 
organ is always firm and natural in consistence, provided that post- 
mortem decomposition has not taken place. On cross-section the vas- 
cularity of the gray matter may be noticed, and sometimes it is possible 
to detect small hemorrhages in its substance. The soft meninges present 
no evidence of exudation, serous or purulent. 
The microscopic examination may, especially in cases which have run 
a rapidly fatal course, reveal practically no signs of disease beyond a 
few capillary hemorrhages, but if the Nissl and Marchi methods are 
employed a careful inspection will usually result in the discovery of the 
following changes: 
1. Cells.-A smaller or larger number of the spinal cord cells, especially 
those of the anterior horns and of Clarke's column, present either early 
pericentral chromatolysis, or more or less complete loss of chromatin 
granules and excentration of nuclei. The most intense cell change is 
found in those parts which, judging from the clinical symptoms, were 
earliest affected; in the majority of cases the legs are the first limbs 
to fail, and the most marked cell changes are detected in the lumbo- 
sacral enlargement. 
2. Myelin.-The myelin sheaths of the spinal cord nerve tracts, and 
to a less extent those of the peripheral nerves, often present a form of 
diffuse fatty change. In longitudinal sections the small droplets of fat 
are seen lying singly, or two or three together, along and between the 
nerve fibres, and do not fill the transverse area of the myelin sheaths. 
This appearance may be found in toxic states unassociated with paralysis, 
and does not therefore indicate necessarily any alteration of function on 
the part of the nerve fibres. 
3. Neuroglia and Vessels.-There is no evidence of neuroglical prolifera- 
tion, although some of the cells may appear to contain more protoplasm 
than normal. The vessels are engorged, but are free from changes in 
their walls or perivascular sheaths. Very rarely a slight excess of small 
round cells may be seen in the immediate neighborhood of one or two 
vessels. 
The above are the only morbid changes found in most cases at the time 
of death. Occasionally, when the fatal termination has been postponed, 
the Marchi method will reveal the presence of true Wallerian degenera- 
tion in the spinal cord and peripheral nerves, probably secondary to 
the cell changes. Early fatty changes in skeletal muscles are often 250 
DISEASES OF THE NERVOUS SYSTEM 
detected, but definite atrophy is not present until some weeks after the 
onset. 
Outside the neuromuscular tissues, the most constant findings have 
been an enlarged spleen, enlarged mesenteric glands, and some evidence 
of pulmonary or pleural complications, generally of a secondary character. 
Symptoms.-There is frequently a distinct prodromal stage lasting 
some hours, days, or even weeks, during which the patient may complain 
of various subjective sensations. Pain in the back is not common, 
although it does occur, but pricking, tingling, numbness, pins and needles, 
are usually described and referred to the peripheral parts of the limbs. 
A general sense of languor or fatigue and a feeling of heaviness in the 
legs and arms may also precede any definite loss of power. 
The development of paralysis takes place somewhat rapidly and 
smoothly or by definite stages. In the latter case there may appear to 
be stationary periods followed by rapid involvement of fresh areas. 
Usually the legs are affected first, but occasionally the arms and rarely 
the cranial nerves present the earliest signs. The paralysis spreads 
from one part to another, following roughly the lines of spinal inner- 
vation. The lower intercostals, for instance, are weakened before the 
diaphragm is involved if the disease is following an ascending course, 
and the peripheral parts of the arms are affected before the muscles 
of the shoulder girdle. When a whole limb has become paretic, however, 
the proximal muscles are just as powerless, or sometimes even more 
powerless, than those moving the hands or feet. The writer has on 
more than one occasion seen a patient lying perfectly helpless as regards 
any gross movement of the trunk or legs, yet able slowly to Hex and 
extend his toes. In this respect the condition affords a most striking 
contrast to many cases of multiple neuritis, in which it is the general 
rule to find the peripheral muscles more paralyzed than the proximal. 
From the beginning and throughout its course the paralysis is of the 
flaccid type, the muscles lacking their normal tone and presenting no 
opposition to passive movements of the limbs. No atrophy or diminution 
in electrical excitability can be detected in any muscle until two or three 
weeks after the onset, and even then the wasting is slight and the elec- 
trical alterations rarely exceed a lessened response to the faradic current. 
If the motor paralysis continues to ascend, the movements of the head, 
and later those of the tongue, larynx, pharynx, and palate, may be 
interfered with, but it is unusual for a patient to survive sufficiently 
long after the respiratory musculature has been seriously embarrassed 
to develop marked cranial nerve symptoms. The loss of the accessory 
muscles of respiration, difficulty in swallowing and in articulation, are 
the principal phenomena brought about by the upward extension of 
the disease. The complete paralysis of all trunk as well as all limb 
muscles renders the patient particularly helpless. 
In contrast to the severity of the motor symptoms everything else 
appears almost insignificant. The constitutional disturbance may be 
slight or even absent, and the temperature rarely exceeds 100° or 101°, 
unless it rises rapidly within a few hours of death. The intellect and 
memory remain clear so long as respiration is effectually carried on, DIFFUSE AND FOCAL DISEASES OF THE SPINAL CORD 
251 
and, in spite of dyspnoea, the patient is usually cheerful and sanguine 
concerning his condition. The paresthesia associated with or preceding 
the onset may continue for some time, and, in addition, complaint 
is often made of cramp-like pains in the immobile limbs, which may be 
relieved by change of posture. 
Tests for detecting loss of sensibility in the skin or deep tissues usually 
discover no change from the normal, unless it be a slight dulling in the 
cutaneous areas below the knees. On the other hand, a suggestion of 
muscle tenderness may be elicited by firm pressure. The sense of passive 
movement and of position remains unaffected. Every deep or super- 
ficial reflex disappears in the paralyzed parts, and is usually unobtain- 
able as soon as any decided paresis has developed. The plantar response 
remains flexor in type so long as it can be elicited at all. 
The rectal and vesical sphincters are unaffected, although, in conse- 
quence of the paralysis of the abdominal muscles, some difficulty may 
be experienced in emptying both the rectum and bladder. Incontinence 
does not occur, except as the result of a distended bladder, and the 
patient is aware of the natural calls to defecation and micturition. With 
ordinary care decubitus can be avoided, as there is no special tendency 
to the formation of trophic sores nor any deficiency in healing power. 
Vasomotor disturbances are slight, if present at all; the skin over the 
paralyzed parts is moderately moist, at any rate in the early days. A 
tache may sometimes be elicited. 
The pulse may continue of fair strength and regular rhythm long after 
respiration has become difficult. With a fatal termination respiration 
gives out for a considerable time before the circulation fails, and arti- 
ficial respiration may often preserve life for many hours. If life is pro- 
longed the most serious complications are pulmonary and bronchial 
catarrhs, with which the patient is hardly fit to cope. 
When neither the disease itself nor any complication proves fatal, the 
patient then enters upon the stage of recovery, and this presents some 
features of interest. The muscles often show a moderate degree of 
general wasting within three or four weeks of the onset, but the 
atrophy is diffuse, and not limited to particular groups. The response 
to faradism may be diminished, but is very rarely lost, and the galvanic 
current produces a normal or slightly sluggish contraction. 
Recovery in power and in nutrition takes place slowly and equally 
over the whole of the musculature, and it is rare for any particular 
set of muscles to hang behind the others. Occasionally those of the 
limbs do not respond to treatment as quickly as those of the trunk. 
Contraction and deformities are rarely seen, except as the result of 
unskilled attention. Remissions and recurrences are not known to occur. 
Prognosis.-Owing to the confusion which has existed, it is impossible 
to give statistics throwing any valuable light upon the mortality. In 
the opinion of the writer Landry's paralysis is a very fatal disease, and 
in this respect contrasts unfavorably with acute toxic polyneuritis and 
also with acute poliomyelitis. 
At the same time it is undoubtedly true that there are instances of 
true Landry's paralysis which do end favorably, and in such there is 252 
DISEASES OF THE NERVOUS SYSTEM 
no reason to expect anything but an exceedingly good or even perfect 
recovery of function. As will be readily understood, the gravity of any 
particular case depends almost entirely on the condition of the respira- 
tory musculature. So long as the diaphragm and the accessory respira- 
tory muscles are intact, or at any rate capable of considerable work, 
there is, in the absence of complications, no serious cause for anxiety. 
When the diaphragm and intercostals are both very feeble, and the 
respiration is being carried on largely by the sternomastoids and the 
other neck muscles, the appearance of cyanosis, the look of distress, 
and the fogging of the patient's intellect are danger signals of the gravest 
import. The most favorable cases are those in which, although mus- 
cular weakness is very general, the loss of power is nowhere complete. 
Pulmonary and bronchial troubles must always be regarded as serious. 
Diagnosis.-This is of importance not so much from the point of 
view of treatment (although it may not be many years before a correct 
diagnosis may be essential for this also) as for the purpose of giving an 
accurate prognosis while the disease is still in its acute stage. 
When a patient is suddenly seized with an acute form of paralysis 
involving a large part of his musculature, two questions may well be 
asked by his friends: Is he going to live? If he lives, will he be per- 
manently disabled? The medical attendant will ask himself, "Is this 
a case of acute ascending myelitis, of acute poliomyelitis, of acute toxic 
polyneuritis, or of Landry's paralysis?" Upon the answer to this 
question must the prognosis largely depend. For instance, it may be 
stated without hesitation that the diagnosis of acute toxic polyneuritis 
renders the prognosis bright as regards both life and fair recovery of 
power, that of acute poliomyelitis, on the other hand, hopeful as regards 
life, but very grave as regards return of activity. 
There should be no difficulty in distinguishing acute ascending myelitis 
from Landry's paralysis on account of the severe sensory loss and sphincter 
disturbance of the former condition, although the spread of motor palsy 
is often similar in the two diseases. The mistake of calling a case of 
acute and widespread poliomyelitis one of Landry's paralysis has been 
often made, but attention to the following general principles will assist 
toward a proper diagnosis. 
1. The constitutional symptoms in the severe cases of acute poliomyelitis 
are more marked than those in Landry's paralysis, the range of tempera- 
ture is higher, often reaching 102° to 104°, the malaise and anorexia 
more profound, vomiting and disturbance of the alimentary canal more 
frequent. In children convulsions are often associated with the former 
ailment. The older the patient the less likely is acute poliomyelitis to 
be present. Cases of acute toxic polyneuritis may, in many instances, 
run their course with no corresponding features. 
2. Sensory Phenomena.-In Landry's paralysis there may be, and often 
is, complaint of pain in the back, of sensations of numbness and tingling 
in the extremities before and during the onset of paralysis. The only 
sensation complained of in the paralyzed limbs is that of discomfort or 
of cramps, arising, partly at any rate, from their immobility; they may 
be handled without giving rise to anything more than slight tenderness DIFFUSE AND FOCAL DISEASES OF THE SPINAL CORD 
253 
on deep muscular pressure. In acute poliomyelitis the pains complained 
of are more urgent, and affect not only the back, head, and neck, but 
frequently the limbs as well. Paresthesia may be present, but the more 
prominent feature is the pain, sometimes very severe, elicited by passive 
movements of the affected extremities. In both diseases cutaneous 
sensibility is usually unimpaired. In acute toxic neuritis, numbness, 
pins and needles, and sharp pains are common in the extremities, and 
very marked tenderness of muscles, sometimes of nerves, is the rule. 
There may or may not be relative anesthesia in the glove and stocking 
areas. 
3. Motor Phenomena.-In both Landry's paralysis and acute polio- 
myelitis the paralysis is flaccid and may be general. The escape of a 
single muscle or of a group of muscles in a region where all the others 
are affected, or a marked asymmetry in the condition of corresponding 
muscles on the two sides of the body, is suggestive of acute poliomyelitis 
rather than of Landry's paralysis. Within a few days or a week of the 
onset in cases of acute poliomyelitis, if death has not occurred, it is usual 
to find rapid recovery in some parts and early atrophy and electrical 
changes in others. In Landry's paralysis recovery, if it takes place, 
is slow and evenly distributed, without marked atrophy. 
In acute toxic polyneuritis the limbs are more affected than the trunk, 
and the peripheral parts of the limbs more than the proximal. Atrophy 
and electrical changes, perhaps only slight, quickly make their appearance, 
especially in the dorsiflexors of the ankles and extensors of the wrists. 
In the same disease, the most marked incidence is generally upon the 
diaphragm, in which case respiration becomes entirely costal. Of the 
cranial nerves, those supplying the facial muscles are often picked out 
and may be the only ones to suffer. Occasionally the palatal or ocular 
muscles are affected. In Landry's paralysis the musculature is affected 
generally and evenly, the trunk and limbs presenting a degree of paresis 
which is more or less symmetrical and equal. In cases in which the 
paralysis first affects the lower extremities it is not uncommon to find 
these parts most paretic at the time of observation, but the spread of 
the disease will then be a progressive one in an upward direction, and 
the thoracic muscles may succumb before the diaphragm, or both may 
be equally weak. When the muscles innervated by the cranial nerves 
are involved, deglutition, phonation, articulation, and more rarely the 
movements of the face and jaws, may be impaired, but the fatal termina- 
tion generally takes place before any of these actions are abolished. 
The condition may be progressive over two, three, or more weeks, and 
yet be unassociated with any definite local atrophy of muscles. 
4. The deep reflexes are abolished in the affected parts in Landry's 
paralysis and toxic polyneuritis. The abdominal reflexes are often 
retained in acute toxic polyneuritis and nearly always absent in Landry's 
paralysis. The plantar reflexes are absent or flexor in type in both 
instances, and the sphincters are only temporarily, if at all, disturbed. 
In addition, it is interesting to note that relapses and recurrences are 
not infrequent in toxic polyneuritis, although they are extremely rare 
or unknown in poliomyelitis and Landry's paralysis. 254 
DISEASES OF THE NERVOUS SYSTEM 
Treatment.-No measures are known to have any definite influence 
on the course of Landry's paralysis. The patient must be placed at 
complete rest, the head slightly raised, and changes of posture allowed 
for the sake of comfort. There is little liability to the formation of 
sores provided ordinary care and cleanliness are exercised. The bladder 
may require catheterization in the first day or two. A purge should be 
given, and if there is any fever a diaphoretic mixture is indicated. Every 
effort must be made to prevent the excitement of bronchial catarrh by 
exposure to cold, and the administration of atropine or belladonna, 
together with strychnine, may relieve the respiratory distress by diminish- 
ing bronchial secretion when the intercostal muscles and diaphragm 
become involved. Oxygen and artificial respiration may prolong life, 
although the necessity for their use is generally a token that a fatal 
termination is impending. 
No active treatment of the paralyzed parts is indicated until the 
dangerous stage has passed and the disease has definitely taken a favor- 
able course. The employment of massage and electricity may then 
tend to hasten the recovery of power. Passive movements should be 
carried out daily in order to prevent arthritic adhesions. 
ABSCESS OF THE SPINAL CORD. 
Intramedullary collections of pus are very uncommon in the spinal 
cord as compared to the brain, and are not of great clinical importance. 
It is doubtful whether the fluid found in some of the early recorded cases 
was really pus or the result of necrosis following spinal thrombosis. 
Etiology.-Spinal abscess occurs most frequently as a secondary 
complication of distant septic processes, especially those involving the 
urogenital system, the lungs, and the heart. Two cases observed by 
Cassirer arose in connection with a suppurating dermatitis and an 
infective endocarditis respectively. 
In some cases, especially those of pulmonary origin, suppurative 
meningitis has been found in association with the central abscess, but 
it must not be too hastily assumed that the abscess was secondary to the 
meningitis. The frequency of suppurative meningitis and the rarity of 
spinal abscess are strongly opposed to this view. It is possible that the 
meningitis is sometimes a consequence of the abscess, and it is probable 
that in other instances the two conditions arise independently from a 
common cause. On the other hand, small abscesses are very occasionally 
seen within the spinal cord in cases of suppurative meningitis under 
circumstances which point to the dependence of the former upon the 
latter. In another class may be grouped instances of spinal abscess 
which have been grafted upon a cord already damaged by fracture, 
dislocation or caries or carcinoma of the vetebral column. Most of these 
must be regarded as secondary to a skin infection or to a purulent cystitis, 
the result of the initial lesion, the incidence of the metastatic process 
being determined by the evascularized and non-resistant condition of 
the spinal tissues at the seat of compression. Finally, a small focal DIFFUSE AND FOCAL DISEASES OF THE SPINAL CORD 
255 
abscess is sometimes found in the midst of a non-purulent infective 
myelitis. 
Pathology.-An abscess may be suspected when the cord presents a 
fluctuating swelling, especially if a purulent meningitis is apparent on 
the surface, but the organ must be opened in order to exclude a syringo- 
myelic or hematomyelic cavity. The purulent collection is always more 
or less central, occupying a position at the base of the posterior horns 
or in the posterior commissure so much favored by extravasations of 
blood and by gliomatous cavities. Occasionally the white matter, usually 
that of the posterior columns, is encroached upon. The pus is of a green- 
ish-white color, generally thick, and contains the remains of nervous 
tissue in the form of drops of myelin and fat. The abscess cavity may 
be focal or may extend through many segments of the cord. The walls 
in acute cases are friable; in more chronic cases a more definite lining 
may be formed by proliferated neuroglial tissue. Degenerative changes 
are found in relation to the nerve elements involved in the process. 
Symptoms.-Little is known of the symptomatology for several 
reasons. The evidence of abscess formation is often obscured by the 
signs of meningitis or myelitis, or the suppuration is frequently a ter- 
minal event, and occurs at a time when the patient's general condition 
does not allow of careful investigation. The process may run an afebrile 
course, or may be associated with rigors and an irregular pyrexia. The 
spinal symptoms are those of a focal or diffuse myelitis, and will depend 
upon the site of the lesion. Flaccid motor paralysis, disturbance of 
sensibility and of the sphincters, loss or exaggeration of the tendon 
reflexes, form part of the clinical picture, but the patient rarely survives 
long enough for muscular atrophy to take place. Occasionally symptoms 
pointing to multiple purulent foci present themselves. 
Diagnosis.-This is most likely to be arrived at in cases of pulmonary 
sepsis, although the symptoms may be ascribed to myelitis or meningitis, 
and the discovery of an intramedullary abscess on the postmortem table 
may come as a surprise. Metastatic abscesses in other organs are the 
chief indications of the spinal condition. In cases of compression para- 
plegia followed by cystitis, the upward spread of spinal symptoms may 
suggest the spread of a purulent focus, especially if it be accompanied 
by severe constitutional disturbance. 
Prognosis.-This is invariably bad and a fatal termination not often 
long delayed. 
Treatment.-This is only prophylactic and must be directed against 
the primary cause. 
VASCULAR DISORDERS OF THE SPINAL CORD. 
Anemia.-There is no proof that a general anemia is capable of pro- 
ducing organic lesions of the human spinal cord, nor does our knowledge 
permit us to attribute any spinal symptoms to that source. On the 
other hand, experimental results justify the supposition that the most 
specialized nervous elements react quickly to alterations in the blood 
state, and conduce to the belief that disorders of nervous function may 256 
DISEASES OF THE NERVOUS SYSTEM 
result from anemia of the spinal cord, without the presence of signs of 
organic change. 
In certain varieties of general anemia, particularly in pernicious 
anemia, it is not uncommon to find changes in the spinal cord, but it 
is highly probable that these are not the direct result of the altered 
blood state, and that they should properly be referred to the effect of 
some toxic agent, to which the anemia is also secondary. 
The paraplegia which results from excessive loss of blood has no known 
anatomical basis, and it is difficult, therefore, to say which part of the 
nervous system is chiefly at fault. Speaking generally, it may be quoted 
as an example of the vulnerability of the nervous tissues to anemia, and 
the question of its cortical, spinal, or peripheral origin left for further 
investigation. 
When the subject of local anemia is brought under consideration, there 
are more facts to guide us in forming conclusions. This may be partial 
or complete, and may have its origin in (1) thrombosis due to a blood 
state or to disease of vessels, (2) embolism, (3) strangulation of vessels 
by pressure of tumors, thickened meninges, or displaced bone, and (4) 
vasomotor constriction or spasm, or (5) dilatation. 
1. Little is known about thrombosis as the result of an abnormal blood 
state in the spinal cord, unless some of the changes in toxemic conditions 
can be accounted for in this way. Arteriosclerosis of the spinal vessels 
is by no means uncommon, but for some reason which is not obvious 
spinal thrombosis is far less common than cerebral thrombosis. Probably 
some of the cases which are usually termed senile paraplegia or senile 
myelitis have a pathological basis founded on softening of arteriosclerotic 
origin. If this is so, their symptomatology is more or less identical 
with that of cases of syphilitic myelitis. Arteritis due to syphilis, 
and more rarely to tubercle, is the most potent and frequent cause of 
thrombosis. 
2. Embolism in the spinal cord is a rare event, but cases have been 
described by von Leyden, Weiss, and Gowers which bear this interpre- 
tation. In all these instances the patients were suffering from mitral 
disease, and in two of them the postmortem examination showed the 
presence of spinal softenings. Experimental spinal embolism has been 
successfully produced by means of foreign particles introduced into the 
lumbar arteries. Hoche showed that foci of simple softening were 
generally found in the central gray matter, owing to the larger caliber 
of the anterior spinal artery. On the other hand, the use of irritating 
vegetable seeds resulted not only in simple necrosis, but in an inflam- 
matory reaction. The animals operated on became rapidly paraplegic. 
3. Spinal vessels are frequently narrowed or occluded by the pressure 
of tumors, thickened meninges, or displaced bone, with the result that 
areas of tissue are evascularized and sooner or later become necrotic. 
The term "compression myelitis" was formerly used to denote the 
condition. 
4. Vasomotor constriction or spasm of the spinal arteries is one of those 
phenomena the occurrence of which may be regarded as possible, but 
the proof of which is not yet forthcoming. Paroxysmal paraplegia or DIFFUSE AND FOCAL DISEASES OF THE SPINAL CORD 
257 
monoplegia in a person who presents no symptoms of spinal disease 
between the paroxysms may be interpreted on the hypothesis of vaso- 
motor spasm, and a similar construction placed upon cases of intermit- 
tent paresthesia of the extremities, but these are matters of speculation 
rather than of demonstrated fact. Vasodilatation can certainly be 
instrumental in slowing the blood stream, and so producing a relative 
anemia and possibly a thrombosis. Such an event may be the result of 
the vasomotor paralysis produced by toxic agents, and a special form 
of paralytic vasodilatation has been described with syphilitic myelitis. 
The effects of local anemia are dependent upon the degree to which the 
cutting off of the blood supply attains. A diminution in the blood stream 
may exert deteriorating influences upon the nerve cells of the anterior 
horns, while the rest of the tissues escape. A further diminution may 
destroy the myelinated nerve fibres, including their axis cylinders, the 
neuroglia still maintaining sufficient integrity to undergo proliferation, 
and thus to inaugurate a process of repair and sclerosis. Finally, when 
the evascularization is complete all tissues succumb in the affected 
area, and, according to Schmaus, the attempts at repair and the removal 
of debris are carried out by mesoblastic elements, with the result that 
a connective tissue scar, with or without the formation of a cyst, replaces 
the parts which have been destroyed. In this respect the effects of 
focal thrombosis in the spinal cord resemble those which obtain in the 
brain, although the common etiological factors are not the same. 
Hyperemia.-Hyperemia of the spinal cord at one time enjoyed a 
position of considerable importance in relation to the pathology of spinal 
diseases. Nowadays it has fallen from its pedestal, and, whether active 
or passive, is regarded rather as a concomitant phenomenon of physio- 
logical or morbid activities than as a primary factor capable of evoking 
clinical symptoms or anatomical lesions. It is sufficient to say that 
the spinal cord shares with other organs the power of attracting an excess 
of blood during functional activity and the inability to avoid plethora 
when it is the seat of inflammation or of passive congestion. 
HEMORRHAGE INTO THE SPINAL MEMBRANES. 
Extradural Hemorrhage.-Extravasation of blood may occur between 
the dura mater and the periosteum lining the inner surface of the vertebrae. 
This space contains, especially posteriorly, a considerable quantity of 
loose areolar and fatty tissue, in which lies a rich plexus of veins. Bleeding 
into this epidural region occurs in the large majority of cases as the result 
of trauma, fractures of the vertebrae, with or without laceration of the 
dural sheath, being the most common cause. Less frequently extrav- 
asation may be due to violent involuntary muscular contractions, 
such as those which are associated with tetanus, eclampsia, and infantile 
convulsions. The venous congestion of cardiac and pulmonary disease 
combined with the excessive action of the respiratory musculature in 
terminal dyspnoea may also be responsible. 
Finally, the bursting of an aortic aneurism into the extradural space 
will in rare instances produce a hemorrhage of a gross character. With 
vol. v-17 258 
DISEASES OF THE NERVOUS SYSTEM 
the exception of the latter cases, the amount of blood poured into the 
extradural space is rarely sufficient to produce symptoms, and its almost 
invariable occurrence within a few hours of death from other causes 
renders these symptoms likely to be overlooked. Only in exceptional 
instances, such as with aneurism, is pressure upon the spinal cord likely 
to be brought about. These hemorrhages are therefore usually recog- 
nized only postmortem. The blood may be limited to a small area or 
diffused throughout the length of the vertebral column, sometimes 
penetrating, with the spinal nerves, the intervertebral foramina. 
Intradural Hemorrhage; Hematorrachis.-In this the blood is effused 
into the arachnoid sac and is more or less mingled with the cerebrospinal 
fluid, to which it gives a hue proportionate to the size of the extravasation. 
As a general rule, the source of bleeding is in the vessels with which the pia 
mater is richly supplied. In other cases blood has made its way into the 
spinal theca from the cranial cavity, where it has escaped as the result of 
a hemorrhage of traumatic or non-traumatic origin. Laceration of the 
dura mater may also allow of extravasation into the arachnoid sac from 
surrounding tissues. Aneurism of a vertebral artery is a very rare cause 
of intradural hemorrhages. The most common origin of small hemor- 
rhages or ecchymoses on the surface of the pia is to be found in the 
fulminating forms of septic meningitis. In such cases numerous extrav- 
asations, sufficient to color the cerebrospinal fluid, are not infrequent. 
Hemorrhages are also met with in connection with tetanus, eclampsia, 
and epilepsy, as well as with purpura, scurvy, and hemophilia. Urgent 
dyspnoea as a result of pulmonary or cardiac disease or of respiratory 
paralysis is an occasional causal factor. 
In most cases there are no symptoms referable to the effusions of 
blood which can be distinguished from those of the primary lesion, 
whether it is of cerebral or spinal origin. In other instances, usually 
of traumatic, but very occasionally, of spontaneous origin, to which the 
term spinal apoplexy has been given, the amount of blood effused is suf- 
ficient to produce a recognizable symptom complex. 
Symptoms.-These are produced by pressure upon the leptomeninges 
and upon the spinal roots, and are characterized by the rapidity of 
their development. Sudden, violent pain referred to the back, associated 
with tenderness over a considerable length of the vertebral column, is the 
first indication. This may give rise to spinal rigidity or opisthotonos, 
and is quickly followed by root pains in the distribution of the spinal 
nerves, by various paresthesias in the extremities, and by involuntary 
spasmodic contractions in the muscles of the trunk, legs, and arms. 
Pain may be evoked by attempts at micturition and defecation or there 
may be temporary retention of urine and feces. 
Such are the symptoms of meningeal and root irritation. They may be 
short-lived or they may merge into more serious disturbances. Corre- 
sponding to the site of the hemorrhage, paralysis of the arms or legs may 
develop, and hyperesthesia of certain cutaneous areas may be followed 
by anesthesia and analgesia, both superficial and deep. This is particu- 
larly liable to affect the lumbar and sacral root areas, owing, probably, 
to the collection of blood in the lowest part of the thecal sac and conse- DIFFUSE AND FOCAL DISEASES OF THE SPINAL CORD 
259 
quent pressure upon the cauda equina. Abolition of the tendon reflexes 
and retention or even incontinence of urine and feces may be observed. 
The evolution of this clinical picture is usually associated with a 
certain degree of shock and in severe cases with loss of consciousness. 
The pupils are contracted at first and may be dilated later. The pulse 
is small and rapid, and there may be an initial fall of temperature. 
The disease reaches its height in the course of a few hours or a day or 
two, and it is during this time that life is most dangerously threatened 
either from shock, respiratory paralysis, or in very rare instances from 
extension of the hemorrhage into the cranial cavity. An early retrogres- 
sion of symptoms may be expected in those who survive, but the period 
of blood resorption is often attended by an exacerbation of the phe- 
nomena and by pyrexia and constitutional disturbances. This is not often 
fatal, although some days may elapse before definite evidence of progres- 
sive recovery from paralysis is forthcoming. From this time onward 
complications arising from decubitus or cystitis are the chief source 
of anxiety, and these may generally be successfully dealt with provided 
the spinal cord itself has not sustained irreparable damage. A consider- 
able degree of recovery is generally attained in the course of six or eight 
weeks, but residual troubles in the form of atrophic palsies, bladder 
disorders, and paresthesias may require many months to clear up, or 
may even be permanent in the worst cases. 
Prognosis.-This has already been indicated by reference to the 
chief sources of danger. The nature of the primary lesion in traumatic 
cases, and particularly the level of the cord, to which the symptoms 
point, must be taken into account. The confinement of symptoms to 
those of meningeal irritation is a favorable sign and generally denotes 
a rapid recovery. Signs of an intramedullary lesion render the outlook 
very grave from the point of view of recovery from paralysis and sphincter 
disorders. 
Diagnosis.--The diagnosis is rendered easy, as a rule, by the history 
of trauma immediately preceding the development of symptoms of 
meningeal irritation. A slow evolution of the latter without pain is a 
very exceptional occurrence, although it has been recorded. When there 
is no history of injury, meningitis may be suspected, and the differen- 
tiation will than depend on the slow onset of the latter condition, the 
more pronounced early pyrexia, and especially on the examination of 
the cerebrospinal fluid which may be blood-stained in either case. 
The diagnosis of hematorrachis from hematomyelia depends chiefly 
upon the early preponderance of paralytic over irritative phenomena 
and the more localized character of the pain in the latter disease. It 
must be remembered that intradural and intramedullary hemorrhage 
may co-exist in traumatic cases. 
Gowers refers to the possibility of mistaking spinal apoplexy for 
strychnine poisoning, and notes the case reported by Dixon, in which 
an extensive hemorrhage into the dural sac gave rise to violent paroxysms 
of muscular spasm and general pain. In such instances the question 
as to whether an effusion of blood into the arachnoid space is the result 
or the cause of muscular spasms may often be difficult to answer. 260 
DISEASES OF THE NERVOUS SYSTEM 
Treatment.-The first essential treatment is physical and mental 
rest, which is best promoted by placing the patient in bed and adminis- 
tering a hypodermic injection of morphine. It is generally assumed 
that the prone position exerts a favorable influence on hemorrhage or 
inflammation in the spinal tissues, but on what grounds this opinion 
is based it is difficult to see. The importance of securing the patient's 
repose and comfort probably outweighs any advantage that can be 
gained from invoking the help of the laws of gravity. The application 
of ice to the spinal column may be of service in arresting hemorrhage. 
Scarification of the surface over the spine at the seat of pain is suggested 
by Gowers as probably the wisest treatment at the onset. Calcium 
salts may be given by the mouth in order to avert further oozing, although 
they can hardly exert any influence on the initial hemorrhage. 
The after-treatment is that of atrophic palsies in general, and the per- 
sistence of symptoms indicating pressure on the spinal cord or cauda 
equina may eventually justify an exploratory laminectomy. 
HEMATOMYELIA (HEMORRHAGE INTO THE SPINAL CORD). 
Hemorrhages within the substance of the spinal cord are not infre- 
quently found postmortem in cases of spinal inflammation or spinal 
thrombosis, but the symptoms of these cases belong to the primary 
disease, and are not those of a true hematomyelia. Capillary hemor- 
rhages are also common in patients who have died with urgent dyspnoea, 
particularly in cases of pulmonary disease, myasthenia gravis, and in 
all instances of paralysis of the respiratory musculature. Here, again, 
there are no clinical symptoms referable to the hemorrhages. The 
term hematomyelia is reserved for cases in which a hemorrhage is mainly 
responsible for the clinical picture. 
Etiology.-Since sclerotic vascular change is one of the most important 
factors in the production of cerebral hemorrhage, it might be expected that 
similar changes in the spinal cord would determine the occurrence of 
spinal hemorrhage, but this is not the case. Sclerotic degeneration of 
spinal arteries is not uncommon, but it rarely gives rise to hematomyelia, 
and the comparative rarity of the latter condition as compared to cere- 
bral hemorrhage is sufficient evidence of the essential difference in their 
etiology. Possibly the circuitous course of the spinal vascular supply 
modifies the effects of high arterial tension. 
By far the most common factor is trauma or strain, although there are 
a few cases in which hemorrhage appears to have occurred without 
any obvious cause or predisposition. Included under the term trauma 
are cases of injury to the spinal column, with or without fracture or 
dislocation, cases of concussion due to falls on the head, on the feet, or 
upon the sacral region. Obstetrical injuries have also caused hemor- 
rhage into the child's spinal cord. The strain of childbirth and that 
of great muscular exertion may give rise to the condition, and excessive 
coitus has been cited by Gowers as a possible causal factor. Hemo- 
philia, congenital or acquired fragility of vessels, purpura, and other rare 
conditions have been assumed to exert an influence. DIFFUSE AND FOCAL DISEASES OF THE SPINAL CORD 
261 
Hematomyelia may occur at all ages, but is most common between 
twenty and forty years, the period of greatest physical exertion and 
exposure to injury. The laborious nature of their occupations renders 
men much more liable than women to the disease. The incidence of 
hemorrhage upon the gray matter is generally explained by the rich 
vascular supply of that tissue and the comparative looseness of its texture. 
Pathology.-In recent cases the appearance of the cord may be normal 
or the soft meninges may present some ecchymoses. Palpation will 
often detect a soft fluctuating swelling, and, if the hemorrhage is exten- 
sive in its transverse direction, the dark bluish-red color of the blood 
clot may be visible through the surrounding white matter. In more 
severe cases a large part or the whole length of the cord may form a 
tubular thin-walled, blood-containing sac. The most common site is 
the cervicodorsal enlargement; its occurrence in the dorsal or lumbo- 
sacral region is rare. 
A series of transverse sections shows that the extravasation may be 
limited to a segment or two, when it will have a round or oval shape, 
or it may extend through many segments in the form of tapering prolon- 
gations upward or downward, in which case it will have from the vertical 
aspect a more or less spindle-shaped contour. Closer scrutiny reveals 
the limitation of the blood to the gray matter at most levels, although 
at the seat of the original leakage the white matter may also be seriously 
encroached upon and torn up. The track pursued by the hemorrhage 
usually involves the bases of the posterior horns, but extends also into 
the anterior and lateral gray substance. 
Multiple foci are relatively often seen (in 10 out of 32 cases according 
to Doerr). The color depends on the age of the hemorrhage, red in the 
early cases, it becomes brown or ochre in those of longer standing. In 
very old cases the site may be marked by a serous cyst, or more com- 
monly by longitudinal cracks or fissures with well-defined walls in the 
posterior gray matter. It is stated that the blood rarely courses along 
the central canal. 
Histology.-The nerve substance surrounding the blood in recent 
cases is seen to be partially disintegrated by mechanical means and by 
the secondary oedema. Proliferation of the neighboring neuroglia soon 
takes place, and granular cells are seen in considerable numbers. These 
appearances have been regarded as inflammatory, but this is not correct 
if the word is used in its ordinary sense, and it seems a pity to assume a 
secondary myelitis in order to describe the process of repair. Secondary 
changes in the nervous elements comprise disappearance or atrophy of 
ganglion cells, degenerations in the ascending and descending spinal 
tracts, and atrophy of the anterior root fibres. 
Symptoms.-Onset.-Only in rare cases is this preceded by warning in 
the form of tingling or numbness corresponding to the site of the future 
lesion. As a rule, the development of symptoms is absolutely sudden, 
exceptionally occupying a few minutes to half an hour. A slower onset 
is suggestive of a secondary hemorrhage, except in those cases when a 
slight leakage is followed shortly by a more extensive extravasation. 
Abrupt paralysis involving all parts below a certain level is character 262 
DISEASES OF THE NERVOUS SYSTEM 
istic of the disease, and may or may not be accompanied by pain. The 
latter, when it is present, is often of an intense radiating character, or 
may be described as a burning sensation constricting a part of the trunk, 
usually the upper thoracic region. Consciousness is usually retained, 
except in the most severe hemorrhages, and no disturbance of general 
health is immediately noticeable. If the lesion occupies the cervical 
enlargement, examination at this period reveals a flaccid palsy of all 
four extremities, together with paralysis of the thoracic and abdominal 
muscles. Unless the fourth cervical segment has been involved, the 
diaphragm will continue to act. In addition to the motor paralysis 
complete retention of urine and feces supervenes. Interruption of the 
oculopupillary fibres leaving the cord at the level of the eighth cervical 
or first dorsal segments produces contraction of the pupils and narrowing 
of the palpebral fissures. Splanchnic palsy entails distension of the 
abdomen, which may add to the respiratory embarrassment occasioned 
by the absence of intercostal action. The pulse may be slowed or unal- 
tered. The temperature usually remains normal at first, and rises after 
a day or two. 
In the course of a few days, unless a fatal termination has ensued, a 
marked retrogression of symptoms usually takes place. The arms 
remain flaccid and present evidence of muscular atrophy, associated 
with changes in the electrical reactions. The lower extremities, on the 
other hand, become spastic, with increased tendon reflexes and extensor 
plantar responses. A certain amount of recovery in power is often 
exhibited at this stage both in the trunk and legs, but the abdominal 
reflexes remain absent. Incontinence of urine sets in and obstinate 
constipation, alternating with incontinence of feces, may be expected. 
Priapism has been noted, but is not always present. 
Cutaneous sensibility may remain generally impaired, but more fre- 
quently tactile stimuli become recognizable, while painful and thermal 
sensibility are still lost. Unless great care has been exercised bedsores 
may have rapidly developed in the first few days. 
Occasionally the symptoms quickly assume, or have from the begin- 
ning, a unilateral character corresponding to what is known as Brown- 
Sequard's paralysis. In that case one arm may present flaccid, and the 
corresponding leg spastic, paralysis, painful and thermal sensibility being 
lost in the affected arm and in the trunk and leg of the opposite side. 
Hemorrhage into the dorsal cord produces a similar picture, with 
the exception that the arms are unaffected and the oculopupillary fibres 
escape. When the lumbosacral enlargement is the site of disease the 
lower extremities are rendered powerless, all their deep and superficial 
reflexes are abolished, and the sphincters are relaxed and incontinent. 
In these rare cases the retrogression of symptoms is usually less marked, 
although a few muscles may regain power and complete sensory loss 
may be replaced by that of the dissociative type in certain areas. 
With hemorrhage into the conus medullaris there is paralysis of the 
bladder and rectum, impotence, and anesthesia of the perineal region, 
the anus and the genital organs, occasionally of the posterior surface 
of the thighs. Vasomotor and sensory phenomena may be observed in DIFFUSE AND FOCAL DISEASES OF THE SPINAL CORD 
263 
paralyzed regions, early vasodilatation and hyperidrosis being suc- 
ceeded by a pale dry skin in the later stages. 
The future course of the severe cases described is generally marked by 
continued improvement up to a certain point, but some muscular atrophy 
usually remains when the cervical or lumbar enlargements have been the 
site of hemorrhage, and some degree of spastic paraplegia when the 
cervical or dorsal regions have been the parts affected. In less extensive 
hemorrhages the injury may be confined to one side of the gray matter, 
when sensory and motor phenomena are limited to a single limb. More 
rarely an area of motor paralysis is observed without any sensory loss, 
or even a partial hemianesthesia without any degree of paresis. These 
mild cases may recover completely, although the occurrence of some 
muscular atrophy usually precludes such a favorable outlook. 
Diagnosis.-Hematomyelia is, as a rule, easily distinguished from other 
forms of spinal disease by reason of its abrupt onset and the rapid partial 
amelioration which supervenes on the resorption of extravasated blood. 
II emator rachis or meningeal hemorrhage is characterized by more diffuse 
pain along the whole length of the spine, by the preponderance of signs 
of irritation over those of paralysis, and by the still more rapid retro- 
gression of symptoms. Lumbar puncture may aid in the diagnosis, 
but it is contra-indicated in cases of hematomyelia. Acute infective 
myelitis is associated with marked constitutional disturbance and pyrexia, 
and a less rapid onset of symptoms. Syphilitic myelitis may have a 
very abrupt development, but the history of lues and the premonitory 
symptoms are usually sufficient for a correct diagnosis. 
In children hematomyelia may simulate an acute poliomyelitis, and 
in certain cases the differentiation may conceivably be impossible. The 
almost invariable occurrence of sensory and sphincter disturbances in 
the former and the pyrexia and malaise associated with the latter disease 
are safe guides in the majority of instances. 
The clinical picture of a case of hematomyelia may be identical with 
that of syringomyelia, in which circumstances the diagnosis can only be 
arrived at from a consideration of the history of onset and the absence 
or presence of evidence of progressive symptoms. 
Prognosis.-Life is threatened at the onset when the extravasation 
is at a high level or when it is severe and continuous. A few cases die 
rapidly, either from respiratory failure or from shock. The majority 
survive the early dangers, and the future outlook then depends upon 
the avoidance of pulmonary complications and of sepsis in connection 
with bedsores or cystitis. Complete recovery from paralysis can hardly 
ever be expected, and the prospects in this connection must be gauged 
from the atrophy and the electrical reactions of affected muscles. So 
long as a muscle responds to the faradic current the hope of its regenera- 
tion may be maintained. The disappearance of all response to elec- 
trical stimuli is a sure sign that the paralysis is permanent. The parts 
affected by spastic paresis may reasonably be expected to show continued 
improvement over a long period of time because the encroachment of 
the disease upon the white columns is usually only temporary and incom- 
plete. The return of sphincter control depends upon much the same 264 
DISEASES OF THE NERVOUS SYSTEM 
anatomical data, except in cases of lumbosacral hemorrhage, in which 
the sphincters are more likely to be permanently paralyzed. 
Treatment.-It is imperative to secure absolute rest. The patient 
should be placed in bed, on a water mattress if possible, and given a dose 
of morphine hypodermically. The prone position is generally advised, 
but is probably quite unnecessary and certainly not conducive to the 
patient's repose or comfort. Ice may be applied to the back. The 
bladder and skin should from the very beginning receive assiduous care 
and attention. The bowels must be relieved by aperients or enemata, 
or both. The patient should be warned that coughing, sneezing, and 
all movements are injurious, and his diet should be nutritious and non- 
stimulating. Insomnia may be treated by bromides, veronal, sulphonal, 
or paraldehyde, but the administration of sedatives must be regulated 
by the condition of the respiratory muscles and organs. 
Active treatment of the paralyzed parts should not be undertaken 
for six or eight weeks after the last sign of hemorrhage has been 
observed, and the first form allowable is electricity, which entails no 
active movements. This may be followed by massage and passive 
movements, and still later by ■ gymnastics, baths, and other remedies 
devised for those who have faith in them. Iron, quinine, and arsenic 
are preferable to strychnine and digitalis in the way of tonics, and a 
change of air and scene is probably of most value to a patient who has 
had a prolonged period of dull inactivity and confinement. 
The treatment of the cystitis and bedsores, which are sometimes 
inevitable in spite of all precautions, is the same as of those of other 
origin, if it is remembered that every care must be taken not to move 
the patient more than is absolutely necessary in the early days of the 
disease. CHAPTER VII. 
TOPICAL DIAGNOSIS OF DISEASES OF THE BRAIN. 
JOSEPH COLLINS, M.D. 
Introduction.-There are certain areas of the cerebral cortex which have 
definite, highly specialized function, to which the name centres has been 
given. Thus, we speak of the motor centre, the centres for the different 
varieties of sensation, and of the speech centres. Some of these areas are 
more closely delimited than others, such, for instance, as the motor area. 
The determination of the areas of the cortex, to which highly specialized 
function is confined, was one of the brilliant contributions to medicine of 
the nineteenth century. 
Topical diagnosis of brain disease is possible only when focal symptoms 
exist. The so-called general symptoms of disorder of the brain are of 
great importance in leading to a diagnosis of disease of the brain, but 
they are of no value in determining the part that is diseased. The focal 
symptoms are, first, those that manifest themselves through the emissive 
channels, twitching, spasm, convulsion, paralysis, dysarthria, anarthria, 
and second, those that manifest themselves through the percipient, 
apperceptive, and interpretative mechanism, such as disorder of tactile, 
thermal, pain, postural and spatial sensibility, apraxia, agnosia, astereog- 
nosis, aphasia, agenesia, and anopsia. 
The localization of diseases within the substance of the brain is depen- 
dent upon the existence of symptoms similar in a measure to those result- 
ing from disease of the cortex, because they are due to lesions of pathways 
leading to and from the cortex. In addition, there occur symptoms due 
to involvement of parts having more or less specific function, such for 
instance as the nuclei of some of the cranial nerves. 
The Motor Area of the Brain-cortex.-The centres for voluntary move- 
ment are situated in the anterior central, precentral, or ascending frontal 
gyrus. This gyrus extends on the mesial surface of the brain from the 
paracentral lobule to the operculum. It was to this area of the cortex that 
Fritsch and Hitzig first attributed representation for voluntary move- 
ments, and though a vast amount of clinical and experimental evidence 
was offered to show that motor representation was not narrowly confined 
to this area, yet the preponderance of the evidence today indicates 
that motor representation is confined essentially to this region. 
Many workers have conclusively shown that true motor responses are 
never obtained save from stimulation anterior to the Rolandic fissure. 
To different parts of this anterior central convolution are allotted centres 
for the different parts of the body. It may be said that a manikin, with 
its head resting on the operculum and its feet over the paracentral lobule, 
will adequately convey the representation, i. e., the centre for the face 
265 266 
DISEASES OF THE NERVOUS SYSTEM 
and its components occupies the lower third, that for the arm and the 
trunk the middle third, and that for the legs and feet the upper third. 
Formerly it was thought that there was considerable localization of 
function to the mesal surface of this area, the paracentral lobule, but 
this view has gradually lost ground. Notwithstanding the statements 
just made that motor representation of voluntary movement is confined 
narrowly to the anterior central convolution, it must be admitted that 
there is an area anterior to this convolution, and particularly in the pos- 
terior end of the second frontal convolution, irritation of which produces 
movement of the eyes. Moreover, irritation of the posterior end of the 
first frontal convolution produces movement of the head to the opposite 
side. 
The Focal Symptoms of Disease of the Motor Cortex.-The most strik- 
ing symptoms which disease of the cortex of the brain produces are motor 
symptoms, which manifest themselves in two forms: In expression of 
irritation-twitchings, spasms, and convulsions; and of obliteration- 
paralysis more or less complete. As a rule, lesions that develop slowly, 
such as new-growths of various kinds, and lesions which are not of suffi- 
cient severity completely to overwhelm, such as poisonous matters cir- 
culating in the blood, produce irritative effects, while rapidly occurring 
lesions, such as hemorrhage, acute inflammation, and traumatic destruc- 
tion, produce paralysis. In many instances, disease of the motor cortex 
is manifest first by irritative phenomena: twitchings and convulsions 
which, after a variable time-in one instance a few minutes and in 
another many months-are followed by paralysis. Occasionally paralysis 
follows immediately upon convulsions and is transitory in duration. 
In such cases the paralysis is interpreted as an acute exhaustion of 
capacity of the motor cells following an intense so-called motor discharge. 
The distribution of spasm due to irritation of the motor cortex depends 
largely upon the area that is involved, although it does not absolutely 
correspond to this. An irritative lesion of the motor area of the brain 
may manifest itself in twitchings and spasms, limited to the corresponding 
contralateral peripheral parts, such as the phenomena known as Jack- 
sonian epilepsy. It may, on the other hand, show itself first in these 
parts, but before the convulsive seizure has passed it may extend to the 
entire body. Spasm and convulsions caused by irritation of the motor 
cortex are usually followed by loss of consciousness, but in many in- 
stances, and especially in diseases that are of small extent and slight 
intensity, and those which develop very slowly, the twitching manifes- 
tations frequently occur many times (i. e., over a protracted period) 
without accompanying loss of consciousness. Sooner or later unconscious- 
ness becomes an accompaniment of the attack. 
The antithesis of spasm is paralysis. When the motor cortex no longer 
responds to irritation, lesion of it is evidenced by paralysis. Paralysis, it 
matters not how brief its duration, indicates abolition of function of the 
motor cortex. The intensity and distribution of the paralysis depend 
upon the nature and extent of the lesion that produces it. A slowly grow- 
ing tumor in the middle of the motor area may produce a slight, slowly 
increasing paralysis of one upper extremity, showing itself in a finger or TOPICAL DIAGNOSIS OF DISEASES OF THE BRAIN 
267 
thumb, and gradually, as the tumor increases in size and encroaches upon 
other parts of the motor area, the paralysis involves the face and the lower 
extremity. On the other hand, a cortical hemorrhage following trauma 
may produce a complete hemiplegia within a few minutes. Circumscribed 
encephalitis may be so narrowly confined in the cortex that only the face 
and shoulder may be paralyzed for any considerable length of time, 
although the oedema, which is secondary to the inflammatory process, 
may cause a disturbance of motor function over a much more extensive 
area. As a rule, paralysis of cortical origin is characterized by incom- 
pleteness and by definite clinical phenomena: slowness of development, 
spasticity, diminution or abolition of the plantar-jerk, exaggeration of 
the tendon-jerks, these phenomena having been preceded or accompanied 
by spasm or convulsion, and particularly by the fact that it is confined 
in the beginning at least to one member of the body. Naturally, a 
lesion that obliterates suddenly the functions of the motor cortex causes 
paralysis of abrupt onset. In such cases spasm and convulsions are 
likely to be added to the clinical picture later when the results of the 
reparative process (scar tissue, adhesions) act as an irritant. The per- 
manence of a paralysis depending upon a cortical lesion is intimately 
associated with the severity of the disease process. It is a common ex- 
perience to have hemiplegia follow surgical interference of, or in the 
vicinity of, the motor cortex, which disappears after a few days or weeks. 
The Focal Symptoms of Disease of the Sensory Cortex.-Knowledge of 
sensory localization in the cortex is much less definite than that of motor. 
The manifestations of lesion of the motor sphere are very striking, mainly 
objective, and not readily susceptible of misinterpretation. The mani- 
festations of sensory irritation, on the other hand, are frequently indefi- 
nite, subject to wide variation, often confined to one variety of sensibility 
and not affecting another, and in every instance subject to personal 
interpretation. 
For a long time it was believed that sensory representation in the cortex 
coincided very closely with motor, and this view, promulgated by Munk, 
is still held by many students of physiology, such as Horsley. It was 
thought that the Rolandic cortex was in reality sensorimotor or even 
kinesthetic (Bastian). Gradually, however, the advocates of the separate 
localization of the sensory and motor areas have gained ground, and the 
soundest teaching at present seems to be that the sensory area is confined 
largely to the posterior central convolution on the external surface of 
the brain, and to the falciform lobe on the mesal surface, particularly 
the central portion of the latter. On the external surface of the brain, 
sensory representation extends backward into the superior parietal 
convolution, to the anterior portion of which is allotted form-perception 
representation, while to the anterior part of the inferior parietal convo- 
lution is allotted muscular sensibility. Much effort has been made to 
give the various forms of common sensibility definite localization. The 
various sensibilities making up the "muscular sense" are thought to be 
located in the posterior half of the postcentral gyrus, the tactile, pain, 
and thermal senses in the anterior half of the same convolution. 
Mills maintains that the cortical representation of cutaneous and 268 
DISEASES OF THE NERVOUS SYSTEM 
muscular sensibility is subdivided into a mosaic of centres, each of which 
is correlated anatomically and functionally to one or more motor centres, 
and that the areas of sensibility are subdivided like the motor area. 
Although this view is not yet accepted generally, there is much evidence 
in favor of it, quite enough to justify the position here taken. 
Allochiria.-Allochiria (other hand) is the name given to a condition in 
which, though sensibility is retained more or less completely, the patient 
cannot tell which side of the body is being touched. The patient can feel 
the touch and can often describe the qualities of the object exciting it, 
but refers it to a spot on the opposite limb corresponding to that touched. 
E. Jones has also maintained that under the name of allochiria two 
fundamentally different conditions have been confused: (1) Part of a 
general defect in localization (alloesthesia), and (2) a specific defect inde- 
pendent of any error in localization (dyschiria). In other words, dyschiria 
of this writer, in reality, is allochiria of the majority of writers. Neither 
allochiria nor alloesthesia has any topical diagnostic value. All the evi- 
dence so far is in favor of the view that allochiria is a manifestation of 
hysteria, and does not occur with organic diseases. 
Astereognosis.*-Much clinical importance has been given to astereog- 
nosis and to the localization of the so-called "stereognostic sense." 
Great confusion exists because of the loose way in which the term has 
been used. Stereognosis means form perception, but most writers use 
it to mean the capacity to recognize objects by handling them, which 
incites a number of sense impressions. Burr contended that the "stereog- 
nostic sense" is not a sense but a judgment, and Prince emphasized this 
point. Unfortunately the term has come to have this application. In 
this sense stereognosis being an intellectual process, it cannot be localized 
any more than any other feature of the intellect. However, if it is limited 
to perception of form alone, tactual astereognosis may well have definite 
localization. The term agnosia, meaning inability to recognize objects 
because of lack of information about them, is a term that has a more 
legitimate usage. Tactual agnosia is the inability to recognize objects 
by touch. Such capacity may also have definite localization, insomuch 
as the various forms of tactual sensibility may have definite localization, 
but no more. Disturbance of stereognostic perception may follow injury 
of the motor area, the ascending frontal convolution, and in a few 
instances it has accompanied disease of the area; but at the present 
time the best teaching is that astereognosis, associated with other symp- 
toms of cortical involvement, indicates lesion of the superior parietal 
convolution, and especially its anterior portion in the vast majority of 
instances. 
"Soul Paralysis."-We sometimes encounter cases of motor paralysis, 
that is, loss of voluntary and spontaneous motor action, in which there 
is no disease of the cortical motor areas or of their projections. Phys- 
iologists do not all admit that unilateral motor paralysis may result 
from destruction of the avenues that convey common sensation alone, 
but the preponderance of opinion is in the affirmative. It is certain, 
however, that when the cortical sensory centres or the tracts connecting 
them with the motor areas are destroyed, ability to perform voluntary TOPICAL DIAGNOSIS OF DISEASES OF THE BRAIN 
269 
motor acts is lost and spontaneous motor power is impaired, occasionally 
wholly abolished. This condition, called "soul paralysis," "Seelenlah- 
mung" of the Germans, was described by Munk to indicate a bizarre 
state noticed in dogs after extirpation of the sensory cortical area. 
Like other conditions or symptoms thought to be new, we find on 
examination of the older writers that it had not escaped their notice. 
There is no doubt that Duchenne described it under the name of "perd 
de la conscience musculaire." It is usually associated with profound 
sensory disturbances, and its most striking feature is due to a break in 
the sensory or afferent section of the reflex arc. Voluntary acts are 
really reflex acts and are possible only when the reflex arc is intact. An 
act performed in response to an effort of the will calls for a revival of the 
sensory memories that accompanied similar performances in the past and 
the stimuli that pass from them to the motor centre produce the move- 
ment that the will desires to produce. These afferent stimuli pass from 
the areas in which such memories are "stored." In practically all cases 
of soul paralysis that have been investigated anatomically there has been 
found destruction of the cortex or of the subcortical region of the parietal 
lobes or of the sensory tracts. 
Soul paralysis is not always used in the sense here employed. Noth- 
nagel used it to designate loss of the memory pictures of movement for 
one extremity or for one-half of the body. This condition Meynert called 
motor asymbolia. This word, and agnosia, apraxia, and other designa- 
tions, which have been used with much latitude, are now coming to have 
very definite meanings attached to them. Their value in topical diagnosis 
has not been definitely assigned. 
Asymbolia.-The word asymbolia was first used by Finkelnburg to 
denote inability to understand certain conventional signs aside from 
speech signs. Wernicke used it in this sense, but enlarged it to include 
failure to recognize the images of objects, i. e., the loss of the "memory 
pictures" of the object. Thus, in Wernicke's sense, patients with asym- 
bolia can see, hear, feel, smell, and taste, but they cannot re-recognize 
objects by these means. That is, they do not call up the previously 
existing memory pictures. They cannot turn sense impre'ssions to 
account. The term asymbolia is therefore used by some in a sense not 
unlike that of agnosia. 
Agnosia.-This is a term used to indicate inability to recognize things 
for what they are. An object is perceived by the peripheral sense appa- 
ratus, but it is not seen by the "mind's eye." Thus, there may be as 
many kinds of agnosia as there are special senses: visual agnosia (mind 
blindness), auditory agnosia (mind deafness), tactile agnosia, gustatory 
agnosia, and olfactory agnosia. Tactile agnosia is not the same as astereog- 
nosis, although it is frequently confounded with it. Before an object 
can be used properly, it must be recognized; therefore, agnosia entails 
dyspraxia, often apraxia, but apraxia does not necessarily indicate 
the existence of agnosia, in fact, there are many cases on record in which 
it existed without agnosia. 
Apraxia.-This is the inability to perform purposeful familiar acts by 
a person who is not paralyzed, anesthetic, demented, or ataxic. It is a 270 
DISEASES OF THE NERVOUS SYSTEM 
commoner symptom of organic disease of the brain than is generally 
admitted. The fact that the localization of the lesion which causes apraxia 
is not so definite as that which causes other symptoms, spasm, for instance, 
explains why so little attention is given to it. The status of apraxia as 
a definite reliable localizing symptom is not yet satisfactorily established. 
Two varieties of it are distinguished-motor apraxia and ideational 
apraxia. In the first there is inability to translate the idea of a move- 
ment into a movement and in the second there is some defect in putting 
together the components of the idea of the movement. It generally 
involves both sides of the body, but it may be unilateral. Indeed, it 
may occur in one group of muscles only. Apraxia has been noted with 
cortical and subcortical lesions of various parts of the brain, but there 
is constantly increasing evidence to show that lesion of the anterior pole 
of the left hemisphere, especially the upper two frontal convolutions, 
will cause it, other portions of the brain cortex being normal. Lesions 
of the corpus callosum also cause it. v. Monakow is of the opinion 
that lesion of the gyrus supramarginalis causes apraxia. 
Apraxia is associated with lesions of the left hemisphere more often than 
with lesions of the right. It has not yet been found with lesions below the 
capsule. The apraxia associated with disease of the left hemisphere may 
be homolateral or heterolateral. Although the lesions with which apraxia 
are commonly associated are those of arteriosclerosis and brain tumor 
it is not infrequent in the confusional states following attacks of epilepsy, 
in the dementia of alcoholism, and other toxic states. Like all symptoms, 
it may be caused by hysteria. Its most frequent symptomatic association 
is with aphasia. 
Amnesia.-Although amnesia is the most constant symptom of disease 
of the brain, neither its intensity nor its variety has any relationship 
to topical diagnosis. 
Hemianopsia.-The centre of sight (the primary cortical visual area, 
so-called) is more definitely located than any other. It is easily acces- 
sible in animals, and its blood supply is often disordered in man. Despite 
this, there is no unanimity of opinion on the part of the physiologist 
and the clinician regarding its limitations. The best opinion seems to 
be that it is situated in the mesial surface of the brain, around the cal- 
carine fissure, in the cuneus of the occipital lobe. It occupies the cortex 
and the entire lingual lobe behind the junction of the calcarine and the 
parieto-occipital fissures, and the entire cuneus extending for half an 
inch or more on the external aspect of the occipital lobe. Destruction 
of this area in one hemisphere causes homonymous hemianopsia. It is 
now generally admitted that permanent visual disturbance occurs only 
when the entire visual area is destroyed. The primary cortical area 
for sight has two divisions, a peripheral and a central. The central is 
for the representation of the macula, and the peripheral for the remainder 
of the retina. The view of Henschen, that the centre for the macula is 
situated toward the apex of the cuneus, in the inferior extremity of the 
calcarine fissure, is the one that has received most substantiation. The 
lower quadrant of the field is represented in the upper half of the cuneus; 
the upper quadrant in the lower half of the cuneus. TOPICAL DIAGNOSIS OF DISEASES OF THE BRAIN 
271 
The secondary, or higher visual area (visuopsychic of Campbell) is an 
association area variously allocated in the posterior end of the inferior 
parietal convolution, particularly the angular gyrus, or a considerable 
part of the parietotemporo-occipital cortex. Its function may be that 
of a storehouse of visual memories and a part of the mechanism for the 
appreciation of things seen. 
The symptoms which disorder of the primary and secondary visual 
areas cause, which are available in topical diagnosis, are anopsia and 
hemianopsia, word blindness, and mind blindness. Cases of purely corti- 
cal anopsia and hemianopsia are extremely rare, for in the majority of such 
cases the optic radiations are found to be involved. Hemianopsia may 
be partial or complete, depending upon the extent of the primary visual 
area involved. It may also be double, as the result of second attacks of 
a bilateral homonymous hemianopsia. 
In attempting to distinguish hemianopsia due to lesion of the cortex and 
to lesions of the optic radiations from hemianopsia dependent upon disease 
of the optic tracts and of the basal ganglia, the peculiar pupillary reaction 
described by Wernicke is of no importance. Wernicke pointed out that 
when hemianopsia was dependent upon involvement of the cortex and 
optic radiation, the pupillary response was intact for the entire retina, 
while when the optic nerves and optic tracts were the seat of the disease, 
only one half of the retina responded. This has never been corroborated 
by neurologists or ophthalmologists. All that may be said with positive- 
ness concerning the attribution of hemianopsia to the cortex is that when 
it is not associated with other symptoms, such as sensory disturbances, 
pupillary disturbances, and aphasia, it is probably not of cortical origin. 
Further, quadrant anopsia is suggestive of cortical lesion. 
Cortical color blindness, or achromatopsia, is not a symptom of impor- 
tance in the diagnosis of focal disease of the brain. In other words, the 
distinction cannot be made clinically between color blindness due to 
disorder of different parts of the central ocular mechanism. 
Mind blindness is the name given to the condition in which an affected 
individual is unable to recognize familiar objects by the sense of sight. 
In other words, it is a condition in which visual memories of things pre- 
viously seen and comprehended cannot be reinvoked. Mind blindness 
is dependent upon disease of the cerebral cortex, or possibly of tracts 
which assist certain parts of the cortex. The term is here used to indi- 
cate object blindness, word blindness, letter blindness, and symbol 
blindness, with intactness of the peripheral ocular apparatus and the 
primary visual centre, and due to lesion or disturbance in function of 
the higher or secondary visual area, and possibly also to lesion of 
the tracts or association fibres which connect the higher visual field 
with the lower visual centre. 
The Centres of Hearing and Smell.- The centre for hearing is situated 
in the posterior transverse temporal gyri and the cortex of the first and 
possibly the second temporal convolutions. By virtue of the hemi- 
decussation of the secondary auditory fibres in the pons each centre has 
a bilateral representation. Permanent deafness would therefore not be 
expected from a one-sided cortical lesion. 272 
DISEASES OF THE NERVOUS SYSTEM 
Auditory disorder, aside from word deafness, is not an important symp- 
tom suggestive of topical diagnosis. Subjective auditory symptoms, 
auditory hyperesthesia, and variously described paresthesia may occur 
with tumor or other slowly developing lesion of the pons, or at the base 
of the brain encroaching upon the auditory nerve. Similar symptoms 
preceding an epileptic attack should suggest lesion of the temporosphe- 
noidal lobe of the opposite side. Deafness of some degree is the result of 
disease of the central auditory apparatus. When it is of the cortex, it 
is called word deafness (see aphasia); when of other part of the central 
auditory mechanism, nerve deafness. Instances of bilateral deafness 
from tumors of the quadrigemina encroaching upon the tegmentum have 
been recorded. 
The centre for smell is situated in the uncinate gyrus of the same side, 
and probably to a certain extent on the opposite side. Jackson and other 
clinicians have shown that this gyrus is diseased in cases of epilepsy at- 
tended with olfactory aura?, such as unaccountable smells. Disorder of 
the sense of smell, especially when associated with symptoms indicative 
of brain lesion, should suggest at least an involvement of the uncinate 
gyrus, but it has also existed with tumor of the cerebellum. 
The higher mental faculties, such as abstraction, introspection, judg- 
ment, comparison, reasoning, etc., are subserved mainly by the brain 
anterior to the motor areas, particularly in conjunction with association 
areas in other parts of the brain. So far nothing definite has been 
established concerning the subdivision of the prefrontal lobe into centres 
to which are allocated definite components of the higher intellectual 
functions, and, considering how complex the higher psychical faculties 
are, and that modern psychology views all mental faculties as arising 
from a complicated coordination or adjustment of movement to the 
environment, it is perhaps not legitimate to seek to localize them. 
Many investigators believe that the anterior part of the frontal lobe 
probably is concerned in selecting and coordinating the products of 
associative process of the post-Rolandic area and converting them into 
psychomotor manifestations or inhibiting them, while the posterior is 
purely a psychomotor area in which physical complexes necessary to 
give external expression to the results of cerebral association are developed 
and elaborated. 
There is much convincing evidence to show that Flechsig's anterior 
centre of association is more concerned with attention and other coor- 
dinations of psychic process than any other part of the brain. It is an 
area undeveloped in cases of idiocy and diseased in cases of secondary 
dementia. Although there is no centre of attention or thought or apper- 
ception or morality in the frontal lobes or elsewhere in the brain, the 
frontal lobes contain neural elements whose integrity must be maintained 
if the individual is to develop and persist in habits of attention, concen- 
tration of thought, balance of feeling, sound judgment, and moral conduct. 
Lesion of the frontal lobes, aside from the so-called ascending frontal 
(which in this article is referred to as the anterior central), and the foot of 
the third frontal convolution (Broca's convolution), produces no constant, 
definite, localizing symptoms. In the majority of cases, however, such 273 
TOPICAL DIAGNOSIS OF DISEASES OF THE BRAIN 
lesion is attended with distinct mental symptoms, such as inattentive- 
ness, lack of concentration, impaired judgment, emotionality, and 
impairment of the higher mental faculties, reaching even to imbecility. 
If the lesion is of the orbital surface of the frontal lobe, olfactory and 
optic tract symptoms often occur even in the early stages of the disease. 
Such symptoms followed by motor symptoms, disturbance of speech, 
monoplegia, and hemiplegia, are highly suggestive of lesion in this part 
of the brain. 
Grainger Stewart suggests that absence, diminution, or easy exhaustion 
of the superficial abdominal reflexes on the contralateral side and a fine, 
rapid, vibratory tremor in the limbs of the same side are suggestive of 
tumor of the frontal region of the brain, but I have seen these symp- 
toms in cyst of the post-Rolandic area. 
Summary.-Briefly to summarize the present-day teachings of localiza- 
tion of function in the cortex: The motor area and the sensory area are 
situated in the centroparietal region, the motor anterior to the fissure of 
Rolando, the sensory posterior. These areas are subdivided so that 
different portions of them serve different muscles and different varieties 
of sensation. An inverted manikin, parallel to the fissure of Rolando, 
will serve to remind one of the divisions of the motor area. The centres 
for cutaneous and muscular sensibility are in the posterior central convolu- 
tion; that for so-called stereognostic sensibility in the anterior end of the 
superior parietal and probably the adjacent posterior central. The centre 
for vision is in the occipital lobe, particularly in the cortex around the 
calcarine fissure, that for hearing in the middle third of the first temporal 
lobe, and that for smell in the hippocampal region. The left hemisphere 
contains the speech area in right-handed individuals, and vice versa; 
the part of the brain that subserves this function includes the foot of the 
third frontal, the superior temporal, and the posterior end of the inferior 
parietal convolutions. In addition to these centres, the cerebral cortex 
has special constituents serving for association processes, which are located 
by some in definite centres, but which are by the majority assumed to 
be scattered over the entire cortex. Speech is related both to the sensory 
areas and the associative areas. On the one hand, the word-component 
of ideation is dependent upon the presence of association fibres; on the 
other hand, it is based upon various sensations which have their seat in 
the sensory centres. Finally, the frontal lobes are necessary for the main- 
tenance of those processes which underlie the higher psychical faculties. 
If these areas have the functions that have been allotted them, disease 
of these areas should cause definite symptoms, and it is a fact that it does. 
It would seem, therefore, that topical diagnosis of brain disease should not 
be difficult, and it would not be were it not that (1) the disease process is 
not often confined narrowly to one of these areas; (2) the immediate 
and subsequent result of disease of these areas is disturbance of function 
of other areas both proximate and remote; (3) disease of one area may 
be but part of a widespread disease of the vascular system, the centre 
that apparently manifests the disease being either the one which is bearing 
the brunt of the lesion or is most tolerant of encroachment; and (4) in 
every case of topical disease of the brain there are general symptoms which 
vol. v-18 274 
DISEASES OF THE NERVOUS SYSTEM 
vary with the nature, seat, extent, and duration of the lesion. Such 
symptoms occur practically in every case, and are called the general 
symptoms. They are headache, dizziness, vomiting, convulsions, apathy, 
stupor, disturbance of gait and station, disorder of intellect usually mani- 
fest by sluggishness of the so-called psychical reflex, and disorder of 
the optic nerves. This, in conjunction with the fact that a considerable 
extent of the brain cortex and almost all of the brain substance beneath 
the cortex has no definite functional allotment, makes accurate topical 
diagnosis difficult. Occasionally when a lesion is confined narrowly to 
one of the areas whose function has been definitely determined, espe- 
cially when the disease process is small and of slow onset, so as not to 
cause obscuring symptoms, the diagnosis may be made with something 
approximating mathematical accuracy. 
Fig. 18 
Localization of function in the brain cortex. 
In every case the most essential thing for such diagnosis is careful, 
repeated physical examination, the results of which are noted and, after 
a sufficient number of them have been made, compared. If, then, there 
are persistent constant disturbances of function of one or more of the 
centres, which have special function, and likewise one or all of the gen- 
eral symptoms, a definite topical diagnosis is possible. 
Lesion of the Central Convolutions.-Lesion of the anterior central 
convolution causes spasm of a muscle or of functionally associated groups 
of muscles, such as those of the leg, the hand, and the face, of the oppo- 
site side, or paralysis, partial or complete, in similar parts. Such paraly- 
sis is nearly always spastic unless the lesion is traumatic and dependent 
upon extensive laceration or hemorrhage, when it may be flaccid. If TOPICAL DIAGNOSIS OF DISEASES OF THE BRAIN 
275 
the lesion extends backward into the posterior central convolution and 
the adjacent parietal convolutions, disturbance of muscular, cutaneous, 
postural sensibility and impaired recognition of the forms and uses of 
objects, so-called astereognosis, will be present. For instance, the occur- 
rence of a series of tonic or clonic spasms in the hand, followed or not 
by loss of consciousness and other manifestations of an epileptic attack, 
would immediately suggest lesion of the middle third of the motor area. 
In case such symptoms are followed by slowly developing monoplegia, 
and later by such sensory disturbances as impairment of the deep sen- 
sibility and astereognosis, the justifiable assumption is that the lesion 
starting in this area had extended posteriorly. 
A lesion confined narrowly to the posterior central convolution does not 
produce pure motor phenomena, but causes impairment of tactile sensi- 
bility and of muscular and articular sensibility, while lesion of the anterior 
end or the superior parietal convolution, possibly also of the posterior 
central convolution, produces tactile astereognosis, tactile asymbolia, 
hemiataxia, and abolition of the superficial reflexes, especially the plantar 
reflex, and in some instances the abdominal. Extension of the lesion 
from this area into the motor area is accompanied by motor manifesta- 
tions and exaggeration of the tendon-jerks, wrhile extension backward to 
involve the cuneus or in depth to implicate the fibres of Gratiolet is 
followed by hemianopsia. Lesion of this area is usually not attended with 
psychical symptoms or disturbance of speech until late in the disease. 
Lesion of the occipital area, and particularly of the cuneus, produces 
homonymous hemianopsia, which is often associated with word blindness 
in conjunction with the general symptoms of intracranial disease. This 
is often a most important localizing symptom. Visual hallucinations, 
transitory but recurring, are occasionally early symptoms of disease of 
the occipital lobe. Such are referred to one-half of the visual fields. 
Lesion of the Centrum Semiovale.-The white substance of the brain 
is made up largely of fibres that convey impulses to and from the cortex 
and fibres that connect different parts of the same hemisphere or one 
hemisphere with the other. Disease of it should therefore be evidenced 
by symptoms varying with the variety and number of the fibres involved, 
i. e., varying with the situation and size of the lesion. If the fibres coming 
down from the motor centres to pass through the internal capsule to the 
pyramidal tract are involved, and these only, the prominent symptom will 
be paralysis of the opposite side of the body. Lesion of this sort, unless 
close to the cortex, is not so frequently attended with convulsions, par- 
ticularly of the localized variety, as is lesion of the motor cortex itself. 
On the other hand, general epileptoid attacks often occur. If, again, the 
lesion interferes with or interrupts the sensory fibres radiating to the 
posterior central and superior parietal convolutions the symptoms will 
be predominantly disturbance of sensation with hemiataxia. If the 
lesion is in the posterior part of the white substance, then symptoms 
referable to vision or to the speech faculty are likely to predominate. 
A lesion of the white substance of the brain in the anterior pole cannot 
ordinarily be diagnosed or even suspected. As a rule, the general symp- 
toms are those of intracranial disease with no localizing symptoms. As 276 
DISEASES OF THE NERVOUS SYSTEM 
a matter of fact, lesion of the centrum semiovale, such as tumor, often 
gives rise to symptoms thought to be characteristic of remote parts of 
the brain, as the cerebellum. 
Lesion of the Corpus Callosum.-Disease of the corpus callosum, 
particularly tumor, causes symptoms which, although not pathogno- 
monic, are suggestive of invasion of this portion of the nervous system. 
Mental symptoms occurring early are most characteristic. These are 
peculiar delusional states, eccentricity of manner and action, impairment 
of memory, dulness, excitability, want of sequence in ideas coupled with 
an apparent conservation of the intelligence, followed by the general 
symptoms of intracranial disease, which may, however, develop very 
late. Apraxia has been recorded and irritative and paralytic phenomena 
due to encroachment upon the neighboring motor zones. An attempt 
has been made at a subdivision of localization in the corpus callosum, 
but this is an unnecessary refinement of diagnosis. 
Lesion of the Internal Capsule.-This is the avenue which impulses 
originating within the brain must traverse in order to be externalized, 
and likewise the avenue through which impressions coming from without 
must pass in order to be interpreted by the brain. In other words, it 
contains practically all the projection fibres of the cerebral cortex. 
Therefore, lesion of the internal capsule may produce symptoms that 
parallel those of lesion of the cortex. A small lesion in this area of the 
brain will be accompanied by symptoms which could otherwise be caused 
only by most extensive disease of the brain cortex. Complete destruc- 
tion of the internal capsule produces flaccid hemiplegia, hemianesthesia, 
vasomotor disturbances of the paralyzed side of the body, hemianopsia, 
and disturbance of hearing on one side. 
The internal capsule, which is in the form of an obtuse angle, is divided 
by the apex of the angle, or the knee, into two portions, an anterior and a 
posterior. The anterior part, or the lenticulo-caudate segment, contains 
the fibres from the frontal lobes, particularly those going to the thalamus. 
The posterior division, the lenticulo-optic and the retrolenticular seg- 
ments, contains, in its anterior part, the pathway of the lower third of 
the precentral convolution. These fibres are located in the anterior part 
of the posterior division. They pass from there into the ventral half of 
the pons, to end mainly around the nuclei of the facial, the motor nucleus 
of the trigeminal and hypoglossal nerves. A lesion of this portion of the 
internal capsule, it is maintained by many, especially by the school of 
Charcot, causes symptoms referable to these nerves, particularly paralysis 
of the muscles of the face which are under volitional control. The path- 
way made up of projection fibres coming from the other regions of the 
motor cortex, and known as the pyramidal tract, especially after it 
reaches the pons, is situated just posterior to the tract mentioned above. 
These two pathways or fascicles constitute the anterior two-thirds of 
the posterior limb. Adjacent to this, posteriorly, is the pathway of the 
general sensibility from the entire body, which, coming in from the teg- 
mentum of the crus, passes up through the internal capsule to go to the 
parietal convolutions. Capsular hemianesthesia of organic origin may 
be accompanied by hemianopsia, but not sensorial hemianesthesia. TOPICAL DIAGNOSIS OF DISEASES OF THE BRAIN 
277 
When alteration of the most posterior segment of the internal capsule is 
attended with hemiplegia accompanied by hemianesthesia and hemianop- 
sia, the optic tract participates in the capsular lesion. Between the 
pyramidal tract -and this fascicle, practically a portion of the former, 
Charcot, Raymond, and others place the "fascicle of hemichorea," i. e., 
the portion of the posterior limb of the internal capsule, irritation of 
which causes hemiathetosis, unilateral paralysis agitans, unilateral 
intention tremor, and hemiataxia. Finally, the visual tract passing from 
the pulvinar of the thalamus to the occipital lobe, and the auditory tract 
going from the auditory nucleus to the temporal lobe, complete the 
internal capsule. 
This being the relative composition, functionally, of the internal cap- 
sule, it follows that lesion of different parts of it produces different symp- 
toms. Clinically there is very little satisfactory evidence that such 
localization exists. Horsley and Beevor demonstrated, apparently to 
the satisfaction of physiologists, that such localization exists in the 
bonnet monkey, and in most of the recent standard works on patho- 
logical anatomy and neurology it is stated that it is true for man. But 
Marie maintains he is not able to corroborate such teaching and the 
writer's experience leads him to agree with this. The teaching that 
there is definite capsular localization has been largely the outcome of 
the acceptance of statements of Charcot and his school, and of homolo- 
gizing in man the results of experiments on the lower animals. It is 
impossible to say that there is a special territory of the internal capsule 
of man that is exclusively sensory, and even with intense capsular and 
cortical lesion, hemianesthesia may be absent. 
Marie maintains that the motor pathway occupies the entire posterior 
segment of the internal capsule as far as the level of the posterior angle 
of the lenticular nucleus. In this lenticulo-optic territory, in which 
descend motor fibres, it is impossible from clinical data to specify distinct 
territories for the fascicles that go to the arm, leg, foot, etc. A lesion of 
this part of the capsule produces the syndrome of hemiplegia, and that 
is all, and, conversely, a lesion limited to the internal capsule causes 
degeneration of the entire pyramidal tract at the level of the oblongata 
or the cord. Clinically one cannot establish any persistent segmentary 
localization in the internal capsule. Very small lesions of the posterior 
limb of the internal capsule may produce irritative symptoms manifest 
as contracture and irregular movements on purposeful effort. After a 
considerable vascular lesion has been partly absorbed, the destruction 
of the pathway will be manifest by hemiplegia and the irritative effect 
on the partially destroyed fibres may be evidenced by contracture. When 
the lenticulo-optic section of the internal capsule is diseased, there may 
be a flaccid, complete hemiplegia with, later, hemicontracture. Conju- 
gate deviation of the eyes associated with forced movements of the 
head is sometimes an initial symptom. If the part of the internal 
capsule just anterior to the knee is affected, there will be hemiplegia 
and dysarthria, or, if it is a small area in front of the knee, facial paralysis 
alone may be the symptom. 
Occasionally a focus in the lenticular nucleus or in the optic thalamus 278 
DISEASES OF THE NERVOUS SYSTEM 
encroaches upon the internal capsule in such a way as to produce a char- 
acteristic clinical picture, paralysis of the face and of the leg, while the 
arm is spared. In such cases the lenticulostriate and the retrolenticular 
section of the internal capsule are affected, while the lenticulo-optic sec- 
tion remains comparatively free. 
Double-sided lesion in the internal capsule, like certain bilateral cortical 
lesions, produces the condition known as pseudo-bulbar paralysis, and, 
varying with the encroachment upon the pyramidal tract, hemiplegia of 
one or both sides. 
Lesion of the Corpora Striata (Ganglia of the Cerebral Hemispheres); 
the Lenticular Nucleus and the Lenticular Zone.-The corpora striata, 
the greater part of which is embedded in the white substance of the hemi- 
sphere, are constituted of two parts, an intraventricular part, called the 
caudate nucleus, and an extraventricular, called the lenticular nucleus. 
The functions of this body have not been satisfactorily established. 
Dejerine denies that it has any connection with the motor and other 
portions of the cortex or that it plays a part in the control of movement 
or in the function of speech. Mingazzini, on the other hand, has proved 
conclusively that certain parts of the lenticula transmit motor and sensory 
fibres and have motor and sensory functions. A focus of disease, even 
of small size, involving only the lenticular nucleus, never fails to cause 
motor disorder, usually partial paralysis, to which, sometimes, are added 
irritative symptoms. Motor paresis of the entire half of the body, often 
of slight intensity and therefore overlooked, is the most frequent symp- 
tom. This often disappears, but a residue can always be found by careful 
examination. Disturbance of speech is also often associated with lesions 
of the left lenticula. The lesion in the majority of cases with the syndrome 
of hemiparesis has been softening of the putamen. The motor functions 
of the lenticula are supplementary to the pyramidal tract. There is no 
justification for regarding it as a vestigial organ; Mills and Spiller have 
furnished ample proof that destructive lesions of certain portions of the 
lenticula cause paresis of the limbs or face, which differs from that pro- 
duced by capsular lesions in so much as the impairment of power is not 
so severe, and from cortical lesions in that it is less likely to be dissociated, 
and Kinnier Wilson1 has described a well-defined clinical picture of Pro- 
gressive Lenticular Degeneration, due to bilateral progressive diseases of 
the putamen in particular. 
Progressive Lenticular Degeneration.-Clinically, this disease manifests 
itself in involuntary movements: tremor-dysarthria, dysphagia, muscular 
weakness, spasticity and contractures with progressive emaciation, 
usually associated with emotionalism and often with mental symptoms. 
It is a disease occurring in young people, often familial, but not congenital 
or hereditary. The course is progressive and the termination fatal. 
Pathologically, it is characterized by a bilateral symmetrical softening 
of the lenticular nucleus, especially of the putamen, while the globus 
pallidus is less affected. In addition, cirrhosis of the liver is constantly 
present. In pure cases the internal capsule is absolutely intact. In 
1 Brain, 1912, xxxiv, 295. TOPICAL DIAGNOSIS OF DISEASES OF THE BRAIN 
279 
other words the affection uncomplicated represents an extrapyramidal 
motor disease. 
Marie maintains that the lenticula and the area around it, which 
he calls the lenticular zone, play an important role in the causation 
of motor aphasia. There is no doubt that lesion of this zone causes 
disturbance of speech, the result of impairment of coordination of the 
complex muscular movements concerned in the exteriorizing of speech, 
and apparent motor aphasia must be considered a symptom of disease 
of the lenticular nucleus. 
Certain symptoms, mental and physical, accompany with much con- 
stancy disease of the globus pallidus (the inner part of the lenticular 
nucleus), due to illuminating gas, to which the lenticula is peculiarly 
vulnerable. These are vasomotor and trophic disturbances of any of 
the soft parts (discoloration of the skin, ulceration simulating bedsores, 
etc.), disturbed control of the bladder, very rarely of the bowels, amnesia 
or loss of memory, especially the variety known as retrograde, and a 
peculiar mental state, which shows itself most strikingly in the appear- 
ance of the patient, reminding one of catatonia. Such patients are 
fully oriented, apparently appreciative of all that goes on around them, 
but wholly indifferent, apparently uninterested, reflecting in their 
features and attitudes absence of initiative. 
Lesion of the Optic Thalamus.-It has been taught with much con- 
fidence that the optic thalamus is a region which manifests disease or 
encroachment upon it by motor, sensory, secretory, and emotional 
symptoms. These are hemiplegia, posthemiplegic choreic movements, 
intention tremor, hemiataxia, disorder of involuntary mimetic movements, 
hemianesthesia, hemianopsia, vasomotor and trophic manifestations in 
the controlateral extremities and pain. 
The "thalamic syndrome' has been rather widely accepted. Gradually, 
however, it has become obvious that there are two reasons why it should 
be subject to critical investigation: First, because destruction of parts 
of the optic thalamus by disease may occur without symptoms; and 
second, because many of the cases upon which the claim has been made 
that lesions of the optic thalamus caused such symptoms have been 
investigated only macroscopically, and therefore it is impossible to say 
that the subthalamic region, the red nucleus, and even the retrolenticular 
constituent of the internal capsule were not affected as well. Finally, 
many cases of thalamus lesion which did not produce the above-mentioned 
symptoms have been carefully studied. 
Lesions confined to the thalamus do not cause hemiplegia. Although 
hemiplegia occasionally occurs as an initial and transitory accompani- 
ment of thalamus lesions, its existence is to be interpreted as a coinci- 
dence, or as the result of a lesion at a distance. In other instances in 
which hemiplegia has been present with lesion of the thalamus, careful 
examination of the crura (cerebral peduncles) or the capsule has not 
been made. Certain other disturbances of motility occur in thalamic 
lesions, especially lesions of vascular origin. These are so-called post- 
hemiplegic choreic movements, intention tremor, ataxia of purposeful 
movements, athetoid movements, and disturbance of the involuntary 280 
DISEASES OF THE NERVOUS SYSTEM 
mimetic musculature. Lesion of the pulvinar and of the lateral geniculate 
body causes spasticity of the contralateral extremities and ataxia or 
tremor especially of the upper extremity. In some instances the move- 
ment which accompanies such lesion is more or less deliberate and rhyth- 
mical, known as athetoid. The term athetosis, meaning without fixed 
position, was introduced by Hammond to describe slow, apparently 
determinate systematic movement with a tendency to distortion of an 
extremity or part of an extremity, such as the hand or fingers. Athetosis 
is probably always the result of thalamic lesion and not of lesion of the 
corpus striatum. Choreic movements, ataxia, and intention tremor may 
result from lesion in the cortical motor pathways, but athetosis does not. 
In several cases of athetosis lesions of the red nucleus, the tegmentum, 
and the vicinity have been found. 
Disturbed innervation of the muscles that cause involuntary emotion 
display is perhaps the most constant symptom of lesion of the thalamus. 
It has long been established by clinical observation that there are patients 
with slight hemiplegia or hemispasticity and other symptoms indicative 
of thalamus lesion, such as paresthesia of one-half of the body, disordered 
muscle sensibility, choreic or athetoid movements, who have the pecu- 
liarity that they cannot innervate the emotional muscles of the face 
voluntarily, but they are able to smile and weep emotionally. Many of 
these cases have been carefully studied, and lesion of the thalamus is 
invariably found. This symptom constitutes an important feature of 
the thalamic syndrome. The occurrence of forced smiling and weeping 
is due to lesion of the corticothalamic fibres, the mimetic centre reacting 
convulsively to every stimulus whether from the periphery or from the 
cortex. It should be said that Roussy claims the mimetic capacity was 
not disordered in the animals in which he produced lesion of these fibres. 
The occurrence of sensory symptoms with lesion of the thalamus is by 
no means constant, although it would seem to be conclusively proved that 
sensory disturbances may be caused by experimental lesion of the thala- 
mus in the lower animals. Moreover, there are many cases of thalamic 
disease with sensory disturbances, but in these cases there has not 
been adequate investigation of the state of the surrounding parts, only 
possible by microscopic examination. For instance, Dejerine and Long 
are inclined to interpret the sensory disturbances attending thalamic 
lesion as evidences of co-existing lesion of the retrolenticular division of 
the internal capsule. Lesions of the basal portion and of the subthalamic 
region are undoubtedly attended with sensory disturbances more con- 
stantly than lesion of the thalamus elsewhere. The disorder of sensation 
is usually limited to certain qualities, such as the tactile. Pain and 
temperature sensibility are sometimes undisturbed. Another feature of 
the resulting tactile and muscle anesthesia is that they are not permanent. 
This feature explains in a measure some of the cases recorded in which 
lesion of the thalamus was not accompanied by sensory disturbances, 
and especially cases of tumor which are of slow growth and long duration. 
In such cases there may have been sensory disturbances. 
Vasomotor and trophic disturbances which occur with thalamic lesion 
are manifest particularly in the contralateral extremities. They are sub- TOPICAL DIAGNOSIS OF DISEASES OF THE BRAIN 
281 
jective and objective sensations of warmth or of cold, redness, swelling, 
and hyperidrosis. Such symptoms have been observed so often with 
thalamic lesions that many writers maintain the existence of vasomotor 
centres in the thalamus. Numerous cases have been reported of hemi- 
plegia with sensibility disturbances on the paralyzed side, in which there 
has been well-marked muscular atrophy on the opposite side, a condi- 
tion that cannot result from lesion of the pyramidal tract alone. Some 
interpret this atrophy as the manifestation of a trophic influence, others 
as a vasomotor disturbance. 
In brief, it may be said that the thalamic syndrome consists of (1) a 
persistent partial hemianesthesia, (2) slight hemiplegia, (3) ataxia and 
astereognosis of the contralateral half of the body, (4) pains, and (5) 
choreic and athetoid movements on the hemiplegic side. Head has 
called attention to another symptom, namely, an over-response to affec- 
tive stimuli which he believes pathognomonic of thalamic affection. 
Our knowledge of the symptomatology of thalamus lesions is indefinite 
because lesions limited closely to the thalamus are of rare occurrence, 
save tumors, and of such slow development and protracted course that 
compensation occurring meanwhile makes it difficult to say that the 
symptoms are, indeed, due to thalamic disease. Vascular lesions are 
rarely confined to the thalamus, and it is not at all improbable that many 
symptoms produced by them are due to simultaneous disease of parts 
of the brain adjacent to the cortex, von Monakow maintains that the 
thalamus constitutes an important station within the central nervous 
apparatus for sensory and cutaneous perception. The representation 
of individual sensory organs is present in it in a sharply localized way. 
The final relation of the anatomical connections must be deemed bilateral. 
Further than this, it must be accepted that other parts of the brain 
may functionate vicariously for the thalamus. In this way is to be 
explained the fact that symptoms of focal disease of the thalamus are 
transitory and that many instances of lesion of this region run their 
course without symptoms. 
Lesion of the Crura Cerebri and Tegmentum.-After the motor fibres 
pass through the internal capsule they leave the cerebral hemisphere by 
the crus, of which they occupy the middle three-fifths. In their downward 
course they supply contralateral fibres to the nuclei of the motor cranial 
nerves. Lesion of a crus may be limited to the crura or to the tegmentum. 
When confined to the crus, it causes a characteristic syndrome, paralysis 
of one-half of the body on the side opposite the lesion, and of one or 
more of the cranial nerves, usually the third, on the same side, the 
so-called paralysis alternans. 
Tegmental Syndrome.-1The tegmentum of the crus cerebri, which is 
made up of a continuation of the formatio reticularis of the oblongata and 
pons, the superior peduncle of the cerebellum, and other white and gray 
matter, is sometimes the seat of disease w7hich confines itself rather 
narrowly to it. Such disease produces fairly constant symptoms, which 
are said to constitute the "syndrome of the tegmentum." These are 
alternating sensorimotor hemiplegia, paralysis of the associated move- 
ments of elevation and depression of the eyes, of convergence, and 282 
DISEASES OF THE NERVOUS SYSTEM 
disorder of the internal musculature of the eyes with preservation of 
the associated movements of bilaterality. Such symptoms may be 
associated with involuntary movements (in rest and in action), hemi- 
ataxia, adiadokokinesia, hemihypoesthesia, and abolition of deep sensi- 
bility of the opposite side of the body. 
Gruner and Bertolotti described two typical cases of this affection. 
The sensory hemiplegia was more marked than the motor, but disorder 
of involuntary movement, incoordination and asynergia, which super- 
imposed themselves upon the hemiplegia, were well marked. To the 
hemiplegia was added bilateral and symmetrical paralysis of all the 
muscles innervated by the third and fourth pairs of cranial nerves. The 
only conjugate movements preserved were those of laterality. The 
lesion was a tubercle situated in the middle of the tegmentum at the 
level of the nucleus of the third nerve. It was bordered on its left side 
by a zone of softening which reached through the median lemniscus and 
totally destroyed the anterior quadrigeminal body. It had caused com- 
plete destruction of the gray column of the motor nuclei of the aqueduct 
extending from the level of the third ventricle into the pons as far as 
the level of the nucleus of the superior oblique. 
Lesion of the Quadrigeminal Bodies; Mid-brain.-Disease confined 
closely to the quadrigeminal bodies causes symptoms from which a 
localizing diagnosis may be made with some confidence, but such disease 
is comparatively rare, especially hemorrhage and softening, which are of 
common occurrence in other parts of the mid-brain and hind-brain, but 
rarely confined to these bodies alone. Tumor, however, is sometimes so 
confined; it is from consideration of the symptoms accompanying such 
lesion, and from experimental work, that the symptomatology of disease 
of this part of the mid-brain has been made out. Experiments on the 
lower animals seem to show that destruction of the entire roof of the 
corpora quadrigemina produces complete blindness, and that lesion of 
one-half of it produces hemianopsia, but in man and the higher animals 
this does not occur, although in man an important optic nerve bundle 
is in connection with the anterior corpora quadrigemina. Disturbances 
of vision, even complete blindness, occurring in affections of the corpora 
quadrigemina have been described by many authors, but the fact that 
there are a great number of negative cases and cases in which there is 
no disturbance of vision during the entire course makes it certain that 
other parts of the mid-brain than the quadrigeminal bodies were im- 
plicated in those instances in which vision was affected. Profound visual 
disturbance accompanying such lesions is indicative of associated lesion 
in the external geniculate bodies or in the optic tract. Ferrier and Turner 
concluded from their experiments on monkeys that the quadrigeminal 
bodies have only a subsidiary relation to the special senses of sight and 
hearing, although it is generally believed that lesion of the posterior 
quadrigeminal bodies causes defect or abolition of the sense of hearing. 
They are also of the opinion that the sensory auditory tract lies outside 
the quadrigeminal bodies, the posterior quadrigeminal body being merely 
an accessory structure and end-station of the fibres of the lateral fillet; 
clinically, disturbance of hearing is not noted with sufficient constancy TOPICAL DIAGNOSIS OF DISEASES OF THE BRAIN 
283 
to justify us in placing much reliance upon it as a localizing symptom of 
disease of the posterior quadrigeminal bodies. 
Much attempt has been made to distinguish between disease of the 
anterior and the posterior quadrigeminal bodies. A lesion confined to 
the anterior pair causes bilateral paralysis of all the eye muscles save 
the abducens, while if the posterior are affected alone, ocular palsy is 
not an important symptom, but deafness and ataxia are. 
Foci of disease of the quadrigeminal bodies adjacent to the tegmentum 
or the crura cannot be differentiated from lesions of these structures, 
but foci in the roof of the quadrigeminal body produce symptoms which 
are more or less characteristic. These are disturance of vision, pupillary 
disturbances, limitation of movements of the eyeball, i. e., some degree 
of ophthalmoplegia, especially of the oculomotor and trochlear nerves, 
sensory disturbances, and disorder of voluntary motion and gait. These 
symptoms vary with the extent of the lesion and with the implication 
of the surrounding bodies. This fact cannot be too strongly emphasized. 
According to Bach, double-sided complete destruction of these bodies, 
but more particularly of the roof, causes lesion of the pupillary light 
reflex on both sides, while unilateral destruction causes it only on one 
side. But from experiments on the higher animals it seems to have been 
conclusively proved that there is retention of iridomotor action after 
complete ablation of the quadrigeminal bodies. This seems to indicate 
that the pupillary fibres of the optic nerve reach the third cranial nucleus 
by some other route than through the anterior quadrigeminal bodies. 
The truth of the matter is that until a lesion of the corpora quadri- 
gemina extends basalward, involving the tegmentum or the central 
gray matter around the aqueduct of Sylvius, the location of the lesion 
can only be suspected, but when it does so extend, fairly characteristic 
symptoms occur, the most important of which is ophthalmoplegia. A 
lesion confined narrowly to the corpora quadrigemina, and causing no 
pressure around the surrounding parts, is not accompanied by paralysis 
of associated ocular movements. The many cases in which the corpora 
quadrigemina have been destroyed without causing disturbance in the 
movements of the eyeballs attest this statement. Complete ophthal- 
moplegia is rare, but partial limitation of the associated movements of 
the eyes, especially the upward and downward movements, is fairly 
common, as is also ptosis. Ophthalmoplegia in connection with ataxia 
resembling cerebellar ataxia constitutes a most important association of 
symptoms. When the ataxia develops first and is very severe, the lesion 
is very likely to be in the cerebellum; when ocular palsies develop first, the 
ataxia much later, the lesion is likely to be in the corpora quadrigemina. 
Limitation of the ophthalmoplegia to the oculomotor and the trochlear, 
speaks in favor of affection of the corpora quadrigemina. Involvement of 
the abducens may occur both in quadrigeminal and cerebellar lesions, 
but involvement of other cranial nerves from the abducens downward 
indicates an affection of the cerebellum. 
Paralysis of the trochlear nerve on one or both sides and disturbance 
of the chewing capacity have been described as symptoms of mid-brain 
disease, and especially of the posterior quadrigeminal bodies, the reticular 284 
DISEASES OF THE NERVOUS SYSTEM 
formation and the locus coeruleus. Such paralysis is manifest by diffi- 
culty of moving the eyes outward and downward and circularly. In 
the majority of these instances the paralysis is associated with hemi- 
ataxia, cerebellar ataxia, choreic movements, and oculomotor palsy. 
Its occurrence bespeaks extensive involvement of the central gray 
matter and of the thalamic region. Difficulty in chewing, pointing to 
involvement of the descending root of the fifth nerve, in reality a pons 
symptom, has been mentioned in a number of cases of quadrigeminal 
disease. Some writers speak of an association of symptoms consisting 
of atrophy in the region of the neck and shoulder, and later in the muscles 
of mastication, as the "Syndrome of Kojevnikoff," and maintain that 
it is dependent upon lesion of the posterior quadrigeminal bodies. 
Disease of the mid-brain involving the tegmentum, particularly a 
vascular lesion, disorder of the functions of the red nucleus, the fillet, the 
reticular formation, and other pontile connections, is always accompanied 
by ataxia of a cerebellar nature often confined to one side of the body. 
In fact, this symptom, in conjunction with irregular choreic movements 
and intention tremor occurring on the opposite side of the body, is per- 
haps the most characteristic combination of symptoms of mid-brain 
disease. The ordinary ataxia of movement is unquestionably dependent 
upon involvement of the fillet and of the reticular formation. The "cere- 
bellar" ataxia is not so easily explained and no satisfactory explanation 
has been given. 
A lesion, such as glioma, affecting the gray matter around the aqueduct 
of Sylvius at a point midway between the two corpora quadrigemina and 
the third ventricle, may produce forced movement with a constant ten- 
dency to fall backward or to bear backward against a support, loss of 
the upward movements of the eyeballs and upper eyelids, exaggeration 
of the knee-jerks, ankle clonus, patellar clonus, and general symptoms 
of brain tumor. 
Lesion of the Cerebellum.-The symptoms of disease of the cerebellum 
as a whole are often pathognomonic. The symptoms of disease of definite 
portions of it are equivocal. Symptoms of disease of the cerebellum vary 
profoundly with the suddenness of onset and the rapidity of progress of 
the lesion, and without full recognition of this fact the diagnostician is 
bound to be disappointed frequently. As Hughlings Jackson wisely said, 
"the symptoms are so different in different cases that it is nearly impos- 
sible to make general statements." 
Some of the chief functions of the cerebellum have long been estab- 
lished, but it is only recently that we know with any certainty that 
there is definite localization of function in it. The cortex is a massive 
recipient organ (Flourens, Jackson, Edinger, Horsley), the head ganglion 
of the proprioceptive system (Sherrington), to which different impulses 
from all parts of the body converge for rearrangement and coordination, 
so as to render them suitable for transmission and interpretation to 
the deep nuclei, which may be looked upon as stations interposed between 
the different cortical fibres and the rest of the nervous system. The 
relation of these nuclei to the cortex has been fairly well established by 
experimentation, but the relation of the nuclei to the extracerebellar TOPICAL DIAGNOSIS OF DISEASES OF THE BRAIN 
285 
nervous system is only now being determined. The chief role of the 
cerebellum is to collect and arrange the various afferent impulses outside 
consciousness for other parts of the nervous system. This is manifested 
in motor coordination, equilibration, and in the maintenance of muscular 
tonus. The cerebellum is also intercalated into sensory pathways which 
receive excitation from the periphery and from the higher centres, but 
its functional relationship to the sensory sphere becomes more doubtful 
the more experimentation forces the secrets of its purpose. The middle 
lobe is functionally more important than the lateral hemispheres, and 
removal of half of this lobe in lower animals causes disturbances similar 
to that caused by removal of a lateral lobe. The effects of unilateral 
ablation are manifest in disturbance of equilibrium, peculiar attitude, 
disordered sensibility, and ocular symptoms. In human beings injury 
and disease of the cerebellum cause similar symptoms and also disorder 
of speech. 
The results of experimental lesion and destruction of portions of the 
cerebellum are very soon compensated by the sensorimotor cortex, and 
after a few months animals that have been thus operated upon present no 
abnormalities unless very careful examination is made. If the Rolandic 
area of the brain, however, is destroyed, simultaneous compensation 
does not result, and the cerebellar symptoms persist. In the human 
being injury to the cerebellum early in Ife, and arrested development 
of the cerebellum, may not give rise to any symptoms during adult life, 
the functions having been assumed by other portions of the brain. 
The disturbance of equilibration caused by experimental destruction 
of the vermis of a hemisphere is manifest by a tendency of the animal 
to rotate on its own axis, the so-called circus movements. When the right 
half of the cerebellum is removed the rotation is toward the right, and 
vice versa. This phenomenon is not always present, but unsteadiness 
of gait and profound incoordination of all voluntary movement invari- 
ably result. No true paralysis occurs in cerebellar lesions, but despite 
this, in animals experimented upon, there seems to be a motor paresis 
of the limbs of the homolateral side. 
The so-called cerebellar attitude of the head is inconstant; and the 
rotation of the head whether forward or away from the lesion has been 
found to be variable (Batten). Bonhoeffer has reported a forward incli- 
nation of the head in several cases. Displacement of the eyes always 
occurs from unilateral ablation, both eyes being turned away from the 
seat of injury, the eye on the opposite side of the lesion usually being 
more displaced than the one on the same side, and nystagmus is invariably 
present. 
The symptoms of cerebellar disease may be divided into those due to 
injury or irritation of the cerebellum itself, those due to increased intra- 
cranial pressure, particularly pons and oblongata symptoms, and those 
due to sudden changes in the blood supply, which, perhaps, causes seizures. 
The major symptoms are motor asthenia, hypotonus, astasia, tituba- 
tion, asynergia, dysmetria, and ataxia (the latter having definite 
features which entitle it to the designation cerebellar ataxia), tremor, 
nystagmus, and disordered phonation and articulation. These symptoms 286 
DISEASES OF THE NERVOUS SYSTEM 
constitute a pathognomonic group of symptoms called the "cerebellar 
syndrome." The most important of these symptoms is the disorder 
of station and gait. The reeling, reckless gait is so familiar that 
description is unnecessary. The titubation or apparent constant bal- 
ancing while the patient is standing is nearly as familiar. Cerebellar 
asynergia is the most important symptom, as it is the basis of most of 
the others. Babinski has shown that volitional equilibrium must be 
considered in two aspects: first, static equilibrium, and equilibrium of 
the body in a state of movement and that a lesion of the cerebellum may 
apparently produce the paradoxical result of very materially diminishing 
kinetic equilibrium, while at the same time increasing static equilibrium 
(cerebellar catalepsy). A patient so affected lying upon his back can 
hold up one or both lower extremities for several minutes without trace 
of tremor or swaying, which appear within a minute in a normal person 
who essays to do it. Babinski has further demonstrated that persons 
with cerebellar disease may be unable to make successive movements 
rapidly, such as pronation and supination of the wrist. The rapid suc- 
cession of such movements, or of any movements for that matter, involves 
the faculty of arresting suddenly a motor impulse, i. e., a contraction in 
a muscle or group of muscles, as well as sending to other muscles a new 
impulse which will cause them to contract. This faculty is essential to 
kinesthetic equilibrium, and for some reason not yet explicable it is 
disordered in cerebellar disease. The best way to display this capacity 
is to have the patient lay his hand flat upon the table and then rapidly 
to supinate and pronate the hand. Patients with cerebellar disease do 
it very slowly or not at all (dys or adiadokokinesis). 
Tetanus-like seizures occurring in cerebellar disease, especially of the 
middle lobe, were first described by Jackson. They consist of exaggera- 
tion of the "cerebellar attitude" plus tonic spasm in certain groups of 
the bilateral muscles of the trunk and the extremities which comes on 
suddenly, lasts a variable time, and is not usually attended with loss 
of consciousness. But motor disorder, aside from asynergia in its various 
manifestations and dysmetria, is not an important sign of disease of 
the cerebellum. Cerebellar tremor has nothing characteristic to dis- 
tinguish it from other intentional tremors. Paralysis and contracture 
when they occur are the result of ventricular distension or neighborhood 
lesions. The tendon-jerks may be altered in cerebellar disease, but 
the alteration is not characteristic or constant. Cranial nerve paralyses, 
of commoner occurrence with lesion of the superior cerebellar peduncles 
than of any other part of the cerebellum, and which are caused indi- 
rectly, that is by extension of the lesion, by pressure, and by action at 
a distance, are usually on the side of the lesion, the facial and auditory 
nerves being oftenest affected. The sixth, ninth, tenth, eleventh, and 
twelfth nerves may indicate indirect affection through impaired func- 
tion. Disorder of sensibility and of the intelligence are not symptoms 
of uncomplicated cerebellar disease, although they are not infrequent, 
especially such symptoms as visual hallucinations and motor excitement. 
Cerebellar Localization.-A certain amount of differentiation of the seat 
of the lesion is justifiable, especially of disease of the middle lobe and the TOPICAL DIAGNOSIS OF DISEASES OF THE BRAIN 
287 
vermis from lesion of the lateral lobe. In lesion of the middle lobe and 
vermis the symptoms are equally distributed on both sides of the body; 
head retraction and opisthotonos are especially likely to occur. Such 
lesion is more frequently accompanied by paralysis of the seventh and 
eighth nerves, the result of indirect pressure. Sudden death in cerebellar 
disease from pressure upon the oblongata is more likely to occur with 
disease of the vermis. In lesion of the lateral lobes the muscular asthenia, 
cerebellar ataxia, hypotonia, asynergia, increase of static equilibrium, 
are present on both sides, but the symptoms are most pronounced on the 
side of the lesion. The head is inclined to the side of the lesion, and the 
face looks up away from the lesion. The patient inclines to the same side 
in walking and tends to fall to the same side. If rotation is a symptom, 
he rotates from the supine to the prone position over the side of the lesion. 
The Syndrome of Cerebellar Deficit.-Many writers have described an 
association of symptoms thought to be characteristic of cerebellar 
agenesis, atrophy, or sclerosis. The chief symptom is disorder of motor 
coordination, manifest in station, gait, and speech. The usual symptoms 
are ataxia of "cerebellar" characteristics, with relative integrity of indi- 
vidual movements of the limbs when the patient is supine. He stands 
with the feet widely separated and legs and trunk betraying oscillations, 
titubations, which are sometimes so intense that he falls. Ataxia of the 
upper extremities and general muscular asthenia often exist in these 
cases. Speech is often scanning or otherwise seriously disordered and 
nystagmus usually exists. There may be no symptoms whatsoever. 
Such instances are supposed to be examples of sensorimotor cortex 
compensation. 
Recently an attempt has been made to classify primary diseases of the 
cerebellum according to their pathological nature. Mangazzini distin- 
guishes the following varieties: (1) Agenesis and atrophy of the cere- 
bellum; (a) unilateral, and (&) bilateral; (2) Atrophy of the cerebellum; 
(a) in conjunction with disease of the brain, and (6) in conjunction with 
disease of the spinal cord. Holmes suggests the following: (1) Primary 
parenchymatous degeneration of the cerebellum; (2) olivopontocerebellar 
atrophy; (3) progressive cerebellar disease due to vascular or interstitial 
lesions; (4) acute cerebellar lesions, apart from tumor and focal lesions 
of vascular origin; (5) degeneration of the spinocerebellar tracts, the 
cerebellar being normal or small only; (6) cerebellar symptoms associated 
with a small central nervous system. Under the first are included familial 
and sporadic cases, occurring at any age and usually slowly progressive, 
with symptoms constituting the cerebellar syndrome. The morbid 
anatomy is a primary and progressive degeneration of the nervous ele- 
ments of the cortex of the cerebellum, especially of the Purkinje cells, 
and of the fibres springing from the cortex. 
Olivopontocerebellar atrophy, first described by Thomas, is characterized 
clinically by the cerebellar syndrome. It is neither hereditary, familial, 
nor congenital. It develops in late adult life and its course is progres- 
sive. It is characterized anatomically by atrophy of the cerebellar cortex 
and the bulbar olives, and of the gray matter of the pons; by total 
degeneration of the middle cerebellar peduncles, by partial degeneration 288 
DISEASES OF THE NERVOUS SYSTEM 
of the restiform bodies, and by relative integrity of the central nuclei 
of the cerebellum. 
Under the third caption are included those cases of cerebellar disease 
incident to arteriosclerosis and primary interstitial proliferation. There 
is no doubt that the cerebellar syndrome may be caused by sclerosis of 
the bloodvessels of the cerebellum, focal or general, and the consecutive 
changes in the parenchyma. There are cases of disseminated sclerosis 
in which the sclerotic tissue is limited to the cerebellum, that may be 
included under this heading. 
Under caption number four are included particularly acute cerebellar 
inflammation, hemorrhagic or otherwise, and hemorrhages into the cere- 
bellum. It comprises also those cases in which the cerebellar syndrome 
comes on after acute infectious diseases, especially in children with or 
without symptoms indicative of encephalitis. It is important to recognize 
this occurrence. The fact that it may end in recovery must also be kept 
in mind. The relationship of this form of cerebellar disease to alcoholism, 
primary anemia, and toxic states has yet to be established. 
Under the fifth heading are some of the cases (such as those of Sanger 
Brown), which Marie utilized to support the existence of hereditary 
cerebellar ataxia, and, whatever justification there is for adhering to the 
designation as a clinical convenience, it is given by these cases, in the 
majority of which there was no disease of the cerebellum. And as the 
hereditary feature is lacking in some cases, the sooner the designation 
is given up the better the outlook for interpreting disease of the cere- 
bellum. 
The Symptom Complex of Occlusion of the Posterior Inferior Cere- 
bellar Artery.-Occlusion of this vessel causes symptoms so sharply 
defined that the diagnosis can be made with much certainty. The symp- 
toms are manifold, usually coming on abruptly without disorder of con- 
sciousness. They consist of slight motor weakness of the limbs, analgesia 
and thermo-anesthesia in the limbs opposite the lesion, and in the dis- 
tribution of the fifth nerve on the side of the lesion, usually associated 
with pain or paresthesia in the area in which objective sensory disturb- 
ances exist. Tactile and postural sensibility are usually attacked. 
Ataxia in the limbs on the side of the lesion, which bespeaks involvement 
of the restiform body or the cerebello-olivary fibres, is of very constant 
occurrence. With this there is often an inclination for the head to drop 
to the side of the lesion, and in some instances the patient tends to fall 
toward the same side. In addition to this there are symptoms indicating 
disturbances of Deiters' nucleus, the vestibular, cochlear, and vagus 
nerves, such as Meniere's syndrome, vertigo, vomiting, and auditory 
hallucinations. Paralysis of the muscles of deglutition and of the soft 
palate on the side of the lesion, is of frequent occurrence in these cases. 
The larynx is sometimes paralyzed on the side of the lesion, which 
causes disturbance of phonation. The sixth and seventh cranial nerves 
are rarely affected on the side of the lesion. Symptoms indicating dis- 
turbances of the sympathetic nerve supply are smallness of the pupil, 
narrowing of the palpebral fissure, retraction of the eyeball, and anhi- 
drosis, all on the side of the lesion. Occasionally obstinate hiccough and TOPICAL DIAGNOSIS OF DISEASES OF THE BRAIN 
289 
tachycardia occur. Hemiasynergy and loss of the tendon reflexes on the 
side of the lesion may exist. 
Lesion in the Pontomedullocerebellar Space.-Lesion of the cere- 
bellopontine angle, or of the space above designated, usually a tumor 
springing from the eighth nerve, occasionally from the fifth, seventh, 
and other bulbar nerves, and sometimes the accompaniment of a central 
neurofibromatosis, produces more or less characteristic symptoms refer- 
able to a single cranial nerve, especially the eighth or fifth, which usually 
long antedate symptoms characteristic of disease of the brain stem or 
cerebellum. Such symptoms are tinnitus aurium, Meniere's syndrome, 
auditory paresthesia, occipital headache, pain in the domain of the 
fifth nerve, and rarely facial paralysis. These symptoms are in many 
instances indefinite and not infrequently exist for years without incapa- 
citating the patient. Encroachment upon neighboring organs is mani- 
fest by cerebellar ataxia, nystagmus, and occasionally paralysis of asso- 
ciated ocular movements, dysarthria and dysphagia. Hemiplegia may 
occur, usually on the side of the tumor, but it has been observed on the 
opposite side. The headache is by no means always occipital; frontal 
and crossed frontal headache have been noted. Circulatory, respiratory, 
and vasomotor disturbances have likewise occurred. 
Lesion of the Pons.-Disease of the pons, both acute and chronic, is 
of frequent occurrence compared with disease of other parts of the brain. 
Inflammatory processes, acute and chronic softenings, and new-growths 
occur there and produce symptoms interpretation of which leads to a 
topical diagnosis. The symptoms vary with the site of the lesion, its 
size, its intensity, and the rapidity with which it develops. A slowly 
growing lesion, such as tumor, often exists for a long time. 
The dorsal part of the pons, which contains the chief nuclei of origin 
of the sixth, seventh, and eighth nerves, the posterior longitudinal bundle, 
and not far from this ventrally, the superior olivary nucleus and the 
motor nucleus of the fifth nerve, and* toward the mid-line the fillet, is 
the commonest site of disease in this region. 
Disease of the pons is characterized by fairly definite symptoms, and 
in the books the "pontile syndrome" receives full consideration. It is 
true that lesion of certain parts of the pons produces a more or less typical 
symptom complex of crossed paralysis, i. e., paralysis of one side of the 
face, which may include the tongue, some of the eye muscles, the 
abducens and motor oculi, and of the other side of the body, but disease 
of the pons covering a considerable area occurs without any recognizable 
symptoms. 
The pons is the medium of transmission of many pathways, and the 
apparent origin of several cranial nerves and the termination of many nerve 
fibres (nuclei pontis) whose functions have not been definitely deter- 
mined. Although these constituents are contained within a relatively 
small area, foci of the disease may be microscopic and produce extremely 
limited symptoms or no symptoms at all. On the other hand, a lesion 
that affects one-half of the pons, extending from the dorsal to the ven- 
tral border to include the pyramidal tract, may produce symptoms 
indicative of implication of the third, fifth, sixth, seventh, and eighth 
vol. v-19 290 
DISEASES OF THE NERVOUS SYSTEM 
nerves, disturbance or paralysis of associated lateral movements of the 
eyeballs indicating lesion of the posterior longitudinal bundle, disturbances 
of sensation, which may reach complete hemianesthesia due to destruction 
of the fillet and the ascending root of the fifth nerve, and motor paralysis 
due to destruction of the pyramidal tract. In addition to such symp- 
toms, lesions of the pons adjacent to the oblongata are accompanied by 
profound vasomotor symptoms, flushing of the skin and hyperidrosis, 
which indicate that this part of the pons has an important representation 
for the sympathetic nervous system. Experimental destruction of half 
the pons in lower animals is accompanied by convulsions and coma, 
and similar lesions from disease in man produce similar symptoms. 
When we consider that in addition to such symptoms as those above 
enumerated, disease of the pons of slow growth, such as tumor, causes 
increased intracranial pressure resulting in headache, vertigo, nausea and 
vomiting, choked disks, and mental disturbances, and that acute lesions 
such as encephalitis and acute softenings are accompanied by symptoms 
indicative of disturbances in other parts of the brain in addition to that 
which manifests the focus of the disease, some idea will be gained of the 
difficulty of making satisfactory localizing diagnosis in many cases. 
The occurrence of alternating hemiplegia will naturally establish the 
diagnosis of pontile disease, but these localizable lesions represent only 
a small number of the cases of pons affection. 
An alternating hemiplegia is hemiplegia of one side of the face and the 
opposite side of the body. It is of two types, inferior and superior. When 
there is paralysis of the abducens or facial nerve on one side, and of the 
extremities on the opposite side, it is called the inferior type, whereas if 
the hemiplegia is associated with an oculomotor paralysis on the opposite 
side, it is spoken of as the superior type. Alternate hemiplegia was first 
described by Millard and by Gubler, and is frequently known as the 
Millard-Gubler type of paralysis. Alternating paralysis of the inferior 
type is sometimes complicated with involvement of the tongue, and dysar- 
thria is a conspicuous symptom. There is difficulty in articulation, 
and in some instances difficulty in swallowing. These symptoms con- 
stitute pseudo-bulbar paralysis. If disturbance in the associated move- 
ments of the eyes on one side is added to such a clinical picture, the 
topical diagnosis is fairly certain, because such symptoms indicate that 
the lesion involves the posterior longitudinal bundle. In such cases the 
lesion must be predominantly of the dorsal part of the pons. 
Absence of hemiplegia, be it alternating or otherwise, does not exclude 
lesion of the pons. Partial or complete hemianesthesia, hemiataxia, cere- 
bellar ataxia without hemiplegia, may occur with lesion of the pons, and 
may be deemed to be of such a nature when they.are associated with 
paralysis of one of the motor cranial nerves having their origin in the 
pons or the ascending root of the fifth nerve, and which cause alternating 
hemiplegia, insensitiveness of the cornea, and half-sided disturbances in 
the associated movements of the eyes. Lesion of the pons may cause 
motor hemiplegia with no other symptoms when the lesion is in the 
ventral part of the pons, of small size, and confined narrowdy to the 
pyramids. It is said that such pontile hemiplegia causes more profound TOPICAL DIAGNOSIS OF DISEASES OF THE BRAIN 
291 
involvement of the trunk muscles than capsular hemiplegia, and that the 
face is usually spared. 
A lesion developing ventrally in the pons may, by pressure upon the 
posterior part of the oculomotor nuclei, cause paralysis of associated 
upward movement as a result of injury to the nuclei over the superior 
rectus and inferior oblique muscles. Paralysis of downward associated 
movements depends on impairment of the inferior rectus and the superior 
oblique muscle, which have cells of origin in two distinct nuclei. A lesion 
of the nuclei of the inferior rectus muscles and of the fibres connecting 
them with the nuclei of the trochlear nerves causes paralysis of associated 
downward movements. 
Conjugate deviation of the eyeballs from pontile lesion is uncommon 
when the lesion is confined to one side of the pons. It has been stated 
repeatedly that conjugate deviations of the eyeballs away from the site 
of the lesion is common in unilateral pontile lesion. It is probable that 
in most cases the side of the pons opposite to the lesion is directly or 
indirectly involved in these cases. Pons lesions almost always cause 
pupillary symptoms and tonic convulsions in all extremities, also in the 
face, with the exception of those areas which are innervated by nerves 
coming from the pons whose angle or whose intrapontile course has 
been destroyed or disturbed by the lesion. 
Superior Pontile Syndrome.-Raymond and Cestan describe a superior 
pontile syndrome characterized by paralysis of the lateral associated move- 
ments of the eyes and paralysis of the arm and leg with the characteristics 
of a central paralysis, the inferior facial being affected on the same side. 
The motor symptoms were manifest particularly by trembling, athetosis, 
incoordination, and asynergia. All forms of sensibility, stereognostic, 
muscular, articular, and postural, were affected. The eyes were fixed 
straight ahead, and the pupils reacted, i. e., there was paralysis of the 
voluntary movement of the sixth pair and paralysis of the associated 
lateral movements of the eyes. This was associated with preservation 
of the power to converge and integrity of the movements of elevating 
and lowering the eyes, known in France as the "Syndrome of Parinaud," 
a quasi-pathognomonic sign of lesion of the superior part of the pons. 
The lesion was a tubercle of the right half of the pons affecting the 
median fillet, destroying the fibres going from the cortex to the sixth 
pair of the fibres which are supposed to unite the nuclei of the third 
and the sixth pairs. 
Pituitary and Pineal Glands.-Disturbance of the function of the 
pituitary gland is evidenced by a syndrome of which acromegaly, adiposity, 
stunted growth, and maldevelopment of the genital organs are the chief 
manifestations. The former is believed to be the result of hypersecre- 
tion, the latter of hyposcretion of the epithelial part of the gland. The 
commonest cause of disturbed function of the gland is tumor. Excessive 
growth of hair and deposition of fat, associated with abnormality of the 
genitalia, suggest disease of the pineal gland. Such symptoms followed 
by bilateral ocular paralysis, nystagmus, abnormality of the pupils, and 
ataxia constitute the pineal gland syndrome. CHAPTER VIII. 
APHASIA. 
JOSEPH COLLINS, M.D 
The most interesting association of symptoms in the topical diagnosis 
of disease of the brain is that known as aphasia. The externalization of 
thought in any form, and its communication to another is a psycho- 
physical act of great complexity. When this capacity is disordered or 
destroyed in an individual, the phenomena that accompany such dis- 
order or destruction are very striking. In attempts to determine the 
localization of the speech faculty we have had to rely entirely upon the 
mode of investigation known as the pathological method, that is the 
study of symptoms first and the seat of disease later. The result has 
been that more has been taken for granted on inadequate evidence or 
insufficient proof in the localization of the speech faculty than in the 
localization of any other distinctive function. 
Aphasia is a term used to indicate any disturbance or perversion of 
intellectual expression. It includes all defects or disorders of intellectual 
expression, whether the result of disarrangement or destruction of the 
receptive or of the emissive components of the speech mechanism, or of 
anything which may be employed as the substitute or equivalent of 
speech. It may be the result of conditions by which the patient is unable 
to part with the expressive equivalent of an idea which has been properly 
formed; it may be caused by any condition that interferes with the 
reception of impulses or stimuli that enter into the genesis of ideas to 
be used in the construction of internal or external language. As move- 
ment in some form is requisite for the manifestation of all expressions, 
defect of this is the condition to which the term motor aphasia or aphasia 
of emission has been and is still often applied. In the second form of 
aphasia the sufferer is unable to adapt receptive communications and 
make them fit the idea represented by the verbal symbol, auditory or 
visual; that is, he has lost the faculty of adapting the complement of 
the word to his own idea; it matters not whether these words be spoken 
or written, or communicated by some equivalent, such as music and 
pantomime. In a general way, this is aphasia of reception or sensory 
aphasia. 
Aphasia is a symptom; not a disease. It may be classified as: 1. True 
Aphasia.-Aphasia of apperception. Due to lesion of any constituent 
of the cortical speech area, constituting the zone of language. 
2. Sensory Aphasia. - Due to lesion of the central and peripheral 
sensory pathways leading to the zone of language. 
3. Motor Aphasia.-Due to lesion of the motor pathways, over which 
the motor impulses travel, in passing to the peripheral speech musculature. 
292 293 
APHASIA 
4. Compound Aphasia.-Any combination of two or more of these. 
If this classification were adopted, it would simplify the matter enor- 
mously, inasmuch as there would be in reality only one aphasia; more- 
over, it would harmonize with the clinical manifestations. It will be 
seen that the scheme is not unlike that which has been set forth by Marie. 
He denies No. 2 and calls No. 3 anarthria or aphemia. Some points 
in the history of aphasia facilitate an understanding of this difficult 
subject. There are three important epochs. 
1. The publication in 1861, by Broca, of an example of aphasia and a 
description of the brain of the individual, which seemed to prove that the 
lesion was of the posterior part of the third frontal convolution. 
2. The classical work of Wernicke, in 1874, which demonstrated that 
lesion of the first temporal convolution caused a definite symptom com- 
plex, from that time described as "sensory aphasia." Bastian had 
anticipated Wernicke in many of his contentions and conclusions. 
3. The papers of Pierre Marie, entitled "Revision of the Question of 
Aphasia" in 1906, and the studies of Liepmann, of Berlin, on apraxia. 
The Zone of Language.-This is the name given to the area of the 
brain in which are carried on the processes essential to speech and its 
components. This zone is not to be defined narrowly. It varies in indi- 
viduals and at different periods of life in the same individual, i. e., it 
is susceptible to phylogenetic, and to ontogenetic variation, the latter 
depending somewhat on the speech acquisition of the individual, and 
on the range and number of avenues by which he receives or has schooled 
himself to receive information. 
The speech area, or zone of language, is an area made up of neurons, 
some of which send their axons into the Rolandic region and into the 
frontal regions of the brain, while others confine their distribution to the 
speech area itself; and, as they do not pass outside of this area, they may 
be looked upon as intercentral neurons. 
The centres of speech in the area of language are three in number: 
a centre for auditory memories, for visual memories, and for articulatory- 
kinesthetic memories. The latter is often called the centre for motor 
memories. The validity of this teaching is under discussion. These 
three centres have definite localization. The centre in which are 
stored the memories of articulation is situated in the third frontal con- 
volution, immediately adjacent to that portion of the Rolandic cortex 
the cells of which give origin to the projection fibres going to the tongue, 
the lips, and the larynx. The centre in which are stored the visual images 
is situated in the posterior portion of the inferior parietal lobule. The 
calcarine cortex is the percipient cortical visual centre. It embraces part 
of the dorsal lip, the bottom and the ventral lip of the calcarine fissure 
from the region of the peduncle of the cuneus to the posterior lip of the 
retrocalcarine fissure. It extends on the lingual gyrus and on the lower 
part of the first occipital gyrus. The geniculocalcarine path is its afferent 
radiation. If we have in mind the afferent radiation or central pro- 
jections of the optic tract after they leave the external geniculate body, 
the inferior quadrigeminal body, and the pulvinar of the thalamus, until 
they reach the lingual and fusiform lobules bordering the calcarine fissure, 294 
DISEASES OF THE NERVOUS SYSTEM 
we shall appreciate that the angular gyrus is the most direct, the most 
adjacent, and the most elective place in which the visual images could 
be stored. In fact, its relationship to the primary visual centre and 
to the fibres that convey visual impulses, the radiations of Gratiolet, is 
analogous to the environmental relationship of the centre for articulatory 
memories and the Rolandic cortex that externalizes speech. 
The third centre, the auditory centre, the most important of all the 
speech centres, is situated between these two centres in the zone of lan- 
guage, and occupies the first temporal convolution, and particularly that 
portion of the cortex which surrounds the temporoparietal sulcus. The 
centre for the storage of auditory memories is in a definite part of the 
general auditory area, in the posterior part of the first temporal convolu- 
tion immediately adjacent to the gyrus in which are stored visual mem- 
ories. Thus, it will be seen that the auditory and visual memories, 
which are contributory to the development of speech and education in 
general, are immediately adjacent, constituting one continuous area. 
Marie maintains that there is no trustworthy clinical observation that 
substantiates the claim that the first temporal convolution is the centre 
of hearing, but his views on this phase of the matter cannot be accepted. 
Probably there is no fixed spot in the first temporal convolution that is 
the sole repository of auditory memories. The cortical auditory area 
may vary in different persons, but anatomical researches by Flechsig, by 
Brodmann, Meyer, and others, demonstrate conclusively that the trans- 
verse temporal gyri and the adjoining part of the first temporal convolu- 
tion are decidedly richer in acoustic fibres and have a distinct connection 
with the internal geniculate body. The economy of Nature is such that 
anatomical possession entails functional endowment. 
It is folly to speak dogmatically at the present time of the relationship 
of sensory aphasia and its various clinical components to lesion of the 
auditory receiving station. Until there is a record of many cases care- 
fully observed clinically, and until we are fortunate enough to encounter 
cases in which the symptoms carefully noted are found to depend upon 
small and single lesions, such discussion as that carried on by Moutier, 
defending the Marie theory, and Ladame and v. Monakow, leads nowhere. 
The relationship of the auditory word-perceiving centre to the word- 
elaborating area is probably a close one, but just what it is cannot be 
said dogmatically. Destruction of the left anterior transverse con- 
volution produces in right-handed persons word deafness. Whether it 
might be affected to such a slight extent as to cause partial word deaf- 
ness and such slight affection of internal and articulate language as to be 
detected only with difficulty seems quite probable. 
Clinical Classification.-It has been customary to divide aphasia into 
(1) motor aphasia, (2) sensory aphasia, (3) total aphasia. Each of these 
is further subdivided, the second into auditory and visual aphasia. 
The first two varieties have always been considered distinct, although it 
has been generally admitted that it is not always easy to distinguish one 
variety from another. Anatomically speaking, sensory aphasia might 
be defined as aphasia due to a lesion of the posterior part of the area of 
language, and motor aphasia as due to the anterior end of this zone. APHASIA 
295 
The subcortical forms occur when there is lesion of the pathways which 
carry impressions into and away from the zone of language. 
So-called Motor Aphasia.-This has been classified as (a) cortical, due 
to lesion of the foot of the third frontal convolution, which abolishes 
the "memory images" of articulation, and (6) subcortical motor aphasia 
(pure motor aphasia of Dejerine), due to lesions of the motor pathway 
over which speech impulses or messages travel to be externalized. Speech 
is properly formed, but it cannot be produced because of the hiatus that 
exists in the speech pathway. These two varieties may often be readily 
distinguished. But it must be said that the features, which allow us to 
distinguish the one from the other, are not quite so absolute and con- 
vincing as one might be led to infer from reading works on the subject. 
Proust and Lichtheim suggested a test for subcortical motor aphasia to 
prove that patients preserve the memorial motion of the word; that is, 
that they have in their minds the name of the object, which they are 
incapable of emitting. To test this deficiency of internal language, the 
patient who hears a polysyllabic word, or sees and recognizes an object 
indicated by that word, is asked to indicate by some movement the 
number of syllables; then to indicate by similar pressings the number 
of letters in the word and the number of letters in the syllables. 
Cortical motor aphasia, Broca's aphasia, is characterized by a loss of 
spontaneous and repeated speech, some disturbance of the capacity to 
read articulately, slight intellectual enfeeblement and by preservation 
of the capacity to comprehend articulate speech. In some instances 
interpretation of spoken speech is slightly impaired in true cortical 
motor aphasia. The peripheral speech mechanism-the tongue, lips, 
palate, and vocal cords-is in condition to functionate. The only justi- 
fication for the use of the word motor in this form of aphasia is that the 
images of articulation are called into being by movement and are external- 
ized by movement. In true cortical motor aphasia there exists the same 
inability to call into existence the sensory memories of articulation, and 
thus to make them a part of internal speech, as there is to externalize 
them in the shape of articulate words. Many of the cases of aphasia in 
the literature, which are considered to belong in this category, are not of 
this variety at all, but are examples of subcortical aphasia; that is, dis- 
turbance of speech dependent upon interruption of the projection tracts, 
which convey the articulatory impulses from the cortical area of the 
peripheral speech mechanism to the peripheral speech apparatus. 
Associated with this loss of spontaneous speech there is an inability 
to repeat words and to read aloud, but the patient comprehends spoken 
words, oftentimes imperfectly. There is inability to express thoughts in 
writing, because in writing the motor word representations are always 
revived by the impulse which travels from the percipient centre (which 
is either in the visual area of the brain in spontaneous writing, or the 
auditory speech area in writing from dictation) through the articulatory 
kinesthetic centre to that part of the Rolandic region which guides the 
part of the body holding the pen. Incapacity to write is proportionate 
to the amount of derangement of internal language, and it bears a definite 
relation to the amount of latent or actual visual amnesia of words which 296 
DISEASES OF THE NERVOUS SYSTEM 
every patient with cortical motor aphasia has. In most cases the capacity 
to write is limited to the name and a few other words that have been done 
so habitually, automatically, and frequently, that they form a part of 
patient's habitual acts, and are done almost reflexly. Writing volun- 
tarily and writing from dictation are practically impossible, yet the 
patient is able to write from copy. 
The patient is unable to call up promptly and vigorously auditory 
images; that is, he has some word deafness, and in many instances 
amimia. This word deafness becomes conspicuous when the patient is 
spoken to abruptly and rapidly. The capacity for articulate expres- 
sion which some motor aphasics retain is for a few words whose utter- 
ance partakes more of the nature of a reflex act or of an emotional pos- 
session than it does of a process of intellection. Although in Broca's 
aphasia the power to make voluntary expressions is usually entirely gone, 
the loss may be partial. When partial, the power of expression is limited 
as a rule, to monosyllabic words, usually verbs. Occasionally patients who 
are afflicted with complete motor aphasia are able to utter some words of 
the nature of an oath, which seem to escape from them in a rapid, un- 
controllable way, or to ejaculate words expressive of the feelings. Such 
expressions are not the product of cognition, but of the emotions, and 
partake of the nature of reflex action. Other patients repeat continually 
some expression or meaningless word or words. Such recurring utter- 
ances are distinctive features of cortical motor aphasia and not of the 
subcortical variety. 
Cortical motor aphasia, due to small lesions, is sometimes manifest 
merely by a loss of substantives, amnesia of the names of the things or 
objects of which the patient tries to speak, by inability to construct 
sentences correctly, and by paraphasia. 
The symptoms of subcortical motor aphasia are practically the same 
as those of Broca's aphasia, with two important exceptions; the patient 
retains the capacity to write and to read, and he responds to the Proust- 
Lichtheim test. In other words, the patient with subcortical motor 
aphasia retains the capacity to talk to himself, to speak without words. 
He hears, sees, writes, mimics, and in other ways gives evidence of 
intellectual integrity and internal language. lie is incapable only of 
articulate speech. This may not be entirely lost. Indeed, it varies 
through dysarthria, dysrhythmia, up to complete anarthria and arhyth- 
mia, and thus to complete speechlessness. In the conventional usage 
of the term this is aphasia. But it is not true aphasia, for true aphasia 
occurs only with lesion of the area of language, and is invariably attended 
with disturbance of internal language. 
Sensory Aphasia.-This is the designation given to the speech disturb- 
ances or imperfections of language due to lesion of the posterior part of 
the speech area, more particularly the temporal and angular gyri; that is, 
to lesion of the perceptive areas of the brain and the immediate incom- 
ing special sense and commissural pathways of such areas. The percep- 
tive centres by whose functioning speech is ontogenetically developed 
are the auditory and the visual, and sensory aphasia is thus practically 
auditory and visual aphasia. It may be defined as loss of the under- APHASIA 
297 
standing of words due to interference with the formation of associations 
necessary for complete perception. Sensory aphasia differs materially 
from motor aphasia. In the first place it is not usually accompanied 
by hemiplegia unless the underlying lesion is very extensive. It is the 
aphasia of comparative speechfulness in contrast with the speechlessness 
of motor aphasia. It is characterized by logorrhoea, motor aphasia by 
alogia. The course of sensory aphasia is rather typical. The patient 
starts with senseless loquacity and gradually his vocabulary shrinks, 
often reaching frank mutism when the auditory area is completely 
destroyed. 
When the lesion of the auditory centre is slight, the patient may 
seem to understand what is said to him but he cannot use words with 
their proper signification and paraphasia may be the distinctive symp- 
tom. Jargon-aphasia, or the production of a jumble of words all forged 
into one, the syllables of which may be articulated, but the words have 
no similarity to words as usually spoken, may be looked upon as an 
extension of paraphasia. 
The degree to which spontaneous writing may be preserved or lost in 
sensory aphasia varies with the patient and with the seat and intensity 
of the lesion. Incapacity to write is most striking when word blindness 
is prominent and total agraphia always accompanies destruction of the 
angular gyrus. If the lesion is predominantly of the auditory area, there 
is inability to write from dictation. Spontaneous writing may be pre- 
served to a considerable extent, but the output is usually senseless and 
disordered. The capacity to copy is preserved, but the patient makes an 
exact reproduction of what is before him. This is especially true of 
cases in which there is lesion of the angular gyrus. 
In sensory aphasia with word deafness there is inability to understand 
spoken words. This is dependent apparently upon the total loss of 
auditory verbal memories. It is one of the commonest forms of 
aphasia, and it rarely occurs independently, being frequently associated 
with some degree of word blindness or manifestations of Broca's aphasia. 
In sensory aphasia with word deafness the patient hears the voice with 
which the words are spoken, but they contain no meaning to him. He 
often recognizes the significance of other sounds, unless it be that the 
memory pictures of such sounds are also lost. Naturally these different 
degrees of word deafness depend upon the extent of the lesion or the 
destruction of the auditory area. The appearance of such patients is 
very significant. They are quiet and observant; their glance betrays 
unawareness or suspicion, and their demeanor is often one of restlessness. 
This alteration of manner, the inability to repeat what is said to them, 
and the profound diminution of spontaneous speech often cause them to 
be looked upon as dements. 
To the form of aphasia in which there is deafness for musical notes the 
designation tone deafness is given. Musical deafness is almost always 
associated with word deafness, but there have been a few cases recorded 
in which it occurred apart from the latter. Many attempts have been 
made to classify the clinical varieties of amusia, but to no purpose. 
One of the commonest manifestations of sensory aphasia is loss of the 298 
DISEASES OF THE NERVOUS SYSTEM 
verbal memory of written and printed symbols-word blindness. It is 
of constant occurrence when the lesion is of the posterior part of the 
area of language. The visual area is made up of a perceptive centre sit- 
uated on the mesal surface of the occipital lobe along the calcarine fissure, 
and of the centre in which is "stored" the visual memories of words, other 
graphic symbols and probably of objects, situated in the posterior portion 
of the inferior parietal lobule, the angular gyrus, and the adjacent margin 
of the supramarginal convolution, which curves over the posterior extrem- 
ity of the fissure of Sylvius. It must be admitted that the localization 
of the "visual word centre" is based largely on theory and slightly on 
facts. Destruction of this centre causes word blindness or alexia and 
agraphia, but no loss of visual acuteness. The primary visual area and 
the higher visual centre are frequently diseased simultaneously, and 
when this occurs homonymous hemianopsia due to lesion of the primary 
visual area is superadded to the word blindness. There are various degrees 
of intensity of word blindness in sensory aphasia. The patient may be 
unable to read words and yet retain the faculty of recognizing letters, 
or he may be able to recognize letters and be unable to join them in 
syllables-asyllabia it is called. The patient may not be blind to all 
forms of notation, graphic and symbolic representation. Thus there may 
be sensory amusia, sensory asymbolia, sensory amimia, etc. In many 
cases with word blindness there is preserved the capacity to recognize 
certain familiar words, such as the patient's name or words that he has 
been accustomed to see frequently. There are also cases in which num- 
bers are recognized, but letters are not. 
If the angular gyrus is completely destroyed the patient has agraphia. 
In those cases in which voluntary writing is preserved, the lesion involves 
the primary visual centre, and the patient has right homonymous hemi- 
anopsia; the patient begins to write at the extreme left side of the sheet 
and stops in the middle of the page. The patients, being unable to read 
what they have written, are unconscious of any errors in spelling or 
phraseology that they may make, although they put the words on paper 
in an orderly fashion. Patients with word blindness are sometimes able 
to read written or printed words by tracing the word with the index 
finger or a pencil, thus substituting kinesthetic for visual memories, 
which kinesthetic memories revive the mental concept of the word. 
There has been a very great amount of analysis of visual aphasia, as 
that form of sensory aphasia in which word blindness predominates is 
often called, and much speculation has been indulged in as to the patho- 
logical physiology and psychology of these subdivisions. There is a 
variety to which the name verbal amnesia or psychic blindness is given. 
The patient interprets letters as letters and words as wmrds, and he can 
read them and copy them, but they convey no meaning to him. When 
they are pronounced by someone else he hears and interprets them read- 
ily, but he has no idea that they are the same words that he has been 
reading or copying. The lesion which it is supposed produces such a 
condition is one that interferes with the pathway that conveys the sen- 
sation from where the memory of the printed word or object is stored to 
the place where the idea is formed, if there is any such special place. APHASIA 
299 
Another variety is that to which the name optic aphasia has been 
given by Freund. The patient, on looking at an object which he has 
previously seen or used, is unable to call up its name, although he is 
able to utter it when it is recalled for him. The lesion is supposed to be 
one that interrupts the pathways that unite the seat of cortical visual 
representation and the seat of cortical auditory memories. 
A third variety is that to which the name of psychic blindness or mind 
blindness is given. It is the Seelenblindheit of the Germans and the 
cecite psychique of the French. In this condition the patient not only 
does not recognize the significance of letters, but he loses the power 
to differentiate between objects or persons and to distinguish the use 
of things; in other words, it is word blindness plus apraxia. The word 
apraxia is coming to have, or it may be said has come to have, a wider 
application than was formerly given to it. The term was used to indi- 
cate inability to comprehend the usage of ordinary objects and things 
to which one has been accustomed. It was thought to be due to the 
abolition of the visual memories of objects which are stored in the cortex 
of the parietal lobe adjacent to the centre for verbal memories. The 
word apraxia is now used to indicate inability to perform certain familiar 
purposeful movements by one who has neither paralysis nor ataxia. 
Apraxia may occur without aphasia. According to the accepted doctrines 
of aphasia of today, aphasia may occur without apraxia, but if Marie's 
ideas of aphasia are accepted, so-called motor aphasia will be looked 
upon as a form of apraxia, that is, apraxia of the speech musculature. 
It is customary to describe a subcortical form of sensory aphasia anal- 
ogous to the subcortical form of motor aphasia, although its delineation 
is not so distinct. If we divide sensory aphasia into auditory and visual 
aphasia, we have to divide subcortical and sensory aphasia in the same 
way, for anything that causes an interruption of the visual and auditory 
pathways subcortically will give rise to these varieties of aphasia. Sub- 
cortical word deafness is characterized by inability to understand spoken 
words and by inability to write from dictation. It is distinguished from 
cortical auditory aphasia by the preservation of spontaneous speech and 
by the ability of the patient to read aloud, to copy, and to write. As in 
every other form of subcortical aphasia internal language is intact. 
The symptoms of subcortical visual aphasia vary somewhat with the 
seat of the lesion, that is, with its proximity to the angular gyrus. As 
a rule, there is word blindness, usually partial, associated with the right 
lateral homonymous hemianopsia. Spontaneous speech is usually well 
preserved. The patient is able to write voluntarily and from dictation, 
but he cannot read what is written either by himself or by others. There 
is very slight or no word deafness. 
Summary.-In brief, then, we have two distinct aphasias-motor 
aphasia (Broca) and sensory aphasia (Wernicke); two subdivisions of 
each of them, and total aphasia, when the two chief varieties occur simul- 
taneously. A patient with Broca's aphasia cannot speak, or is reduced 
to a word or two, he writes as badly as he speaks, and he often has some 
word deafness. He cannot talk to himself, and he cannot read to himself. 
The patient with sensory aphasia, on the other hand, is a babbler. He 300 
DISEASES OF THE NERVOUS SYSTEM 
uses words senselessly, jargon-aphasia, or in a preverse way, paraphasia. 
There is marked difficulty of comprehension of spoken or written language 
and profound disorder of reading and writing. In Broca's aphasia the 
trouble is predominantly of articulation and of writing, that is to say, in 
the omission of words; in sensory aphasia all the elements of language 
are disordered. 
Broca's aphasia is therefore the suppression or profound alteration of 
the speech not dependent upon paralysis, associated with trouble of 
internal language, of speech, and of writing, dependent upon lesion of the 
foot of the third frontal convolution, which lesion causes destruction of 
"motor verbal images," or upon interruption immediately beneath the 
cortex of the fibres or pathways that convey motor speech impulses. 
Sensory aphasia is due to the lesion of the posterior two-thirds of the 
zone of language, and especially the first temporal convolution and the 
angular gyrus. 
Revision of the Aphasia Question Proposed by Marie.-This, in brief, 
was the status of the aphasia question when Pierre Marie assailed it in 
190G. Marie maintains that the third frontal convolution of the left 
side does not play any special role in the function of language; that 
which is called motor aphasia or Broca's aphasia is anarthria plus aphasia; 
that the aphasia of Broca is not a disease, not a clinical entity, but a 
syndrome, a superimposition of aphasia upon anarthria, or, better, a 
simple juxtaposition of two distinct troubles, anarthria and aphasia. As 
to aphasia itself, Marie holds there is only one aphasia, which he pro- 
poses to call Wernicke's aphasia, and only one speech centre diffusely 
localized in the left temporoparietal lobe, and that this centre is a region 
of intelligence specialized for language, not a centre of sensory images. 
The clinical splitting up of the aphasia of Broca into two elements, 
anarthria and aphasia, Marie maintains is verified by autopsy. One 
finds constantly lesion of the lenticular zone associated with lesion of 
the zone of Wernicke. 
Marie's description of his lenticular zone is as follows: If in a hori- 
zontal section of the brain one carries a line in a transverse direction 
from the anterior fissure of the island of Reil as far as a corresponding 
point in the lateral ventricle, and another transverse line from the poste- 
rior fissure of the insula to a corresponding point in the lateral ventricle, 
a region is circumscribed having nearly a quadrilateral outline and 
containing in its territory the caudate nucleus and the lenticular nucleus, 
the external capsule with its different parts, the cortex of the island of 
Reil, and the internal capsule. It is in this territory very distinctly 
separated from the third frontal convolution that the lesion which deter- 
mines anarthria especially is situated. This territory the writer designates, 
for the sake of brevity, the lenticular zone. The superior and inferior 
limits of this zone it is not yet possible to define. 
Anarthria (or aphemia, the term Marie is now willing to use) is char- 
acterized clinically by the loss of speech with preservation of the under- 
standing of words, of reading and writing. It is produced by a lesion 
in the lenticular zone, interfering with the coordination of movements 
required for the phonation and articulation of words, without introducing APHASIA 
301 
true muscular paralysis. Broca's aphasia is produced by the combina- 
tion-the proportion varying with the case-of the lesion of anarthria 
with a lesion of Wernicke's zone or the fibres coming from this zone. 
The third left frontal convolution plays no special part in the function 
of speech. The true speech centre is the zone of Wernicke; which 
must be considered not a sensory centre, but an intellectual centre. 
Wernicke's zone consists of the supramarginal and angular gyri and the 
feet of the first two temporal convolutions. Lesion of this centre deter- 
mines in proportion to its extent, and in addition to the disturbances of 
speech, deficient understanding of spoken words, inability to read and 
write, as well as the disappearance of certain concepts of a didactic char- 
acter. The foot of the first temporal convolution cannot be said to con- 
stitute a sensory centre for the auditory image of words. Pure deafness 
does not exist. Pure alexia (pure word blindness of authors) does not 
occur clinically. The lesion producing it is a lesion of the posterior cere- 
bral artery, not of the sylvian artery, as in the other aphasias. It is 
useless and inaccurate to drag in the angular gyrus, which cannot be 
recognized as the centre of visual word images. 
Marie maintains that there is no reason to preserve the classification 
of aphasia into cortical and subcortical forms. As a matter of fact, 
aphasia due to focal lesions is never exclusively cortical. It is, moreover, 
advisable at present not to refer to the cerebral cortex the entire patholog- 
ical physiology of aphasia, since the subjacent white matter seems to 
play a part of perhaps greater clinical importance than the gray matter. 
If one insists upon classifying aphasia, the varieties of which are connected 
by a scale of innumerable transition forms, the best division would be 
into (1) intrinsic aphasia, in which Wernicke's zone or the fibres coming 
from it is directly and considerably affected by the lesion (Broca's aphasia, 
Wernicke's aphasia), and (2) extrinsic aphasia, in which Wernicke's zone 
with its fibres is not directly involved. 
For Marie there is only one aphasia, the aphasia of Wernicke. The 
term sensory aphasia, he thinks, should disappear. The aphasia of Wer- 
nicke has for its foundation lesion of the zone known by his name. 
Intrinsic aphasia is accompanied by trouble of the internal language 
and by intellectual deficit. The other alterations of language, anarthria, 
and pure alexia, are extrinsic syndromes. Pure alexia is dependent upon 
lesion of quite another vascular territory than aphasia. Aphasia is 
dependent upon lesions of the sylvian artery, and alexia upon oblit- 
eration of the posterior cerebral artery. He maintains that in autopsies 
upon patients with Broca's aphasia there is a double lesion. One causes 
the anarthria, and the other the trouble of internal language, of reading, 
and of writing. The terms Broca's aphasia, ataxic aphasia, total aphasia, 
designate the progressive degrees of the same syndrome. 
The three important factors in the discussion are: (1) Is Broca's 
area (the foot of the third frontal convolution), the centre in which are 
stored the memories of phonetic speech, articulatory kinesthetic mem- 
ories? In other words, is motor aphasia or Broca's aphasia real aphasia 
at all? If there is a well-defined, though not sharply marked, syndrome 
to which the name of Broca's aphasia is given, upon what is it dependent 302 
DISEASES OF THE NERVOUS SYSTEM 
anatomically? (2) Do lesions of the so-called lenticular zone give rise 
to a symptom complex parallel to that of so-called subcortical motor 
aphasia, the anarthria or aphemia of Marie? (3) Is the area of language 
the area in which memories and words seen and heard are stored, and 
from which alone they can be evoked by peripheral stimuli, or is the 
storage of such memories a function of the anterior pole of the brain 
(the so-called psychic sphere) or of the whole brain itself? 
It must be granted that there is no adequate clinical or anatomical 
evidence for considering Broca's centre to be the seat of memories of 
articulation. There does not exist in the literature a case of Broca's 
aphasia clinically in which the lesion was confined narrowly to the foot 
of the third frontal convolution, nor does the brain upon which Broca 
bases his original thesis, which is now preserved in the Musee Dupuytren, 
which the writer has examined carefully, show it. Although there are 
some cases recorded in which macroscopically lesions seem to be closely 
confined to Broca's convolution, macroscopic examination is entirely 
inadequate in these cases, save that it gives an accurate idea of the extent 
of the lesion. Serial sections alone can be relied upon. 
It may be stated that our present conception of the zone of language 
must be modified in so far as denying the existence of the storage of 
articulatory or kinesthetic memories in the anterior pole, the foot of the 
third frontal convolution. 
It is admitted by everyone that there is an aphasia, which is clinically 
distinctly characterized, to which the name motor aphasia or Broca's 
aphasia is given. Its symptomatic features have been described. Is it 
sensory or Wernicke's aphasia plus anarthria or aphemia? If it is true 
that the foot of the third frontal convolution is not the seat of phonetic 
memories, then Marie's explanation is probably the correct one. Marie 
admits that Broca's aphasia and Wernicke's aphasia are clinically two 
distinct varieties. The distinction between the two is not always very 
sharply drawn, but, as a rule, certain characters permit the distinction 
to be made. Those who have contended that the symptomatology of 
the two forms of aphasia was very unlike have pointed out that it is a 
mode of copying that distinguishes motor aphasia from the sensory 
aphasia. It is admitted by everyone that the motor aphasic has some 
word deafness and some word blindness, but it is maintained that he 
copies print in script while the sensory aphasic copies servilely. But 
there has been important testimony to show that this is not true (Souques). 
In regard to the mental defect of aphasic patients, whether they have 
sensory or motor aphasia, this has received due consideration in every 
treatise on aphasia, and there can be no doubt of its existence; consider- 
ing the lesion of the brain in the majority of cases of aphasia, the wonder 
is that the mental defect is not more pronounced. 
That lesion of the so-called lenticular zone gives rise to the symptom 
complex parallel to that of so-called subcortical motor aphasia there can 
be little doubt. The preservation of internal speech is the distinctive 
feature in each of them. Whether the loss of speech capacity or destruc- 
tive speech capacity which results is called anarthria or aphemia is not 
of prime importance. APHASIA 
303 
In the discussion of aphasia, Marie concedes the word aphemia to 
express speech defect of Broca's aphasia, which he first proposed to call 
anarthria, making sacrifice of the word, not of the fact. The writer 
agrees with Marie that the term aphasia should be reserved for those 
cases in which internal speech is disordered. Those cases in which the 
internal speech is not disordered and in which there is inability to speak, 
pure motor aphasia (Dejerine), should be classified as cases of anarthria 
or aphemia. This anarthria may exist from the onset of the patient's 
illness, that is, there may never be any true aphasia associated with it. 
The answer to the third question cannot be given positively at this 
time. It will be appreciated that the phrase "storage of memories," 
which is constantly used symbolically in writing on aphasia, is not to 
be taken literally. We do not know how memories are created, invoked, 
reinvoked, or localized, or indeed that they are localized at all. 
The position taken by Marie is not so revolutionary as has been com- 
monly supposed. In the first place, it has never been seriously denied 
that there is a certain amount of dementia in practically all cases of 
true aphasia. This mental defect varies in different cases, but it has 
been generally recognized. In the second place, the anarthria or aphemia 
of Marie is in reality the same thing as the subcortical motor aphasia 
of Wernicke and the pure motor aphasia of Dejerine. The important 
contribution that Marie has made is that the foot of the third frontal 
convolution (Broca's convolution, so-called) is not the seat of articulatory 
kinesthetic memories, and is not the integral part of the zone of language, 
and that, therefore, destruction of it does not cause aphasia. It is not 
enclosed in the quadrilateral area called the lenticular zone by Marie. 
It is probable that Marie is correct in assuming that the third frontal 
convolution is not an area in which are stored memories of articulation. 
The criticism that may justly be made on Marie's revision of aphasia is 
that he has torn down without building up. When he attempts to define 
his quadrilateral or lenticular area, we see that, although he gives an 
anterior and a posterior border to it, it includes in reality an enormous 
amount of brain, especially in the superior and inferior direction, and 
Dejerine's criticism that it is not a localization at all is a very just one. 
Whether we accept the classic teaching or not (and certainly it cannot 
be fully accepted after all the evidence has been considered), we must 
admit that Marie's service has been very great, for it has put us on 
toward fact and away from theorizing. 
Diagnosis.-The speech faculty consists of two parts, the receptive and 
the emissive; either may manifest the predominance of aphasic symp- 
toms. In true aphasia, that is, aphasia dependent upon lesion of the 
zone of language, neither can be the sole medium of manifestation of 
the speech defects. Emissive speech is manifest by articulation, by writ- 
ing, and by pantomime. Integrity of the receptive side of language is 
commensurate with the interpretation of visual and auditory stimuli. 
The attitude, the demeanor, the conduct of the patient may be of the 
greatest service from the very beginning of the examination. The manner 
and expression of one with sensory aphasia (Wernicke's aphasia) are 
frequently those of a person who has lost interest in his surroundings, 304 
DISEASES OF THE NERVOUS SYSTEM 
and his attitude is that of a deaf person slightly demented. Moreover, 
patients who have this form of aphasia are often garrulous, and on the 
slightest provocation, or without provocation, emit a string of sounds 
that convey no meaning. This is especially true of recent cases. Patients 
with motor aphasia (Broca's aphasia) and with subcortical motor aphasia 
(anarthria or aphemia of Marie) are very different. The aphemic is often 
absolutely silent, but watchful, and the intensity with which he holds 
every move of persons around him is very striking. He may be abso- 
lutely speechless, yet capable of understanding all that goes on about 
him and within his hearing and vision. 
A number of schemes have been devised to facilitate the examination 
of aphasic patients, and the following simple plan is found most service- 
able. After securing a general history of the patient's life and of his 
previous illness from some member of the family, and in this way getting 
information of the character of the disease of which the aphasia is a symp- 
tom, the patient's ability to express ideas, to receive and interpret infor- 
mation through every avenue should be tested. The mental processes, 
apprt from the manifestation of mental states, and the mental capacity 
for the reception of sensory stimuli, then should be examined. Although 
a number of these may be determined simultaneously, it is best to take 
each one separately, such as attention, memory, orientation, capacity 
for retaining impressions, for deliberation, for reasoning, and his tem- 
perament or mood. 
In approaching a patient with aphasia, it is natural that endeavor 
should be made to elicit information by speaking to him. It becomes 
necessary, therefore, to determine if the patient takes note of what is 
said to him orally, and secondly, if he understands what is said. In other 
words, does spoken speech awaken in his auditory centre and in the 
auditory interpretative area corresponding memories? This can be done 
ordinarily by taking some simple question, as, "How long have you 
been sick?" or by addressing to him some simple command, such as, 
"Give me your hand." Care must be taken not to employ too conven- 
tional questions or commands, such as, "What is your name?" and "Put 
out the tongue." The patient may have lost the auditory apperceptive 
faculty and still make reasonable reply to such questions merely from 
association and habit. Naturally, the patient should not get any infor- 
mation of what is being asked through any other avenues than those of 
hearing. Such patients are quick to grasp, particularly if they have been 
aphasic for some time, the significance of even slight emotional expres- 
sion or pantomime on the part of the interlocutor. If the patient does 
not reply to such questions or commands, there may be trouble with the 
receptive or with the emissive speech faculties. If he is word deaf (that 
is, if the trouble is one that prevents the sound of the word from reaching 
the centre in which the memories of previous word sounds are said to be 
stored), the patient will not endeavor to respond by word or act, though 
in some instances he does so. Nor will the face show the slightest 
response or indication of comprehension. If he does respond, the diag- 
nostic feature is that his answer, even though it be made up of articulate 
words, has no pertinency or bearing on the question. If the patient is APHASIA 
305 
not word deaf, he will make some movement, be it of the head, hand, 
or features, to indicate that though he understands, he cannot reply. 
Generally, this gesture is very significant. It consists of a despairing 
expression of the countenance and a touching of the lips or the throat 
with the fingers. Oftentimes the question can be decided very quickly, 
if there remains some doubt even yet, by asking some absurd or ludicrous 
question and noticing how the patient receives it. If, in reply to the 
question, "Are you one hundred years old?" he solemnly says "Yes," or 
if he does not see the ludicrousness of a request to turn a somersault 
when he is obviously paralyzed, it is rather convincing proof that such 
speeches do not awaken the proper responses in his mind, and if there 
be no dementia, it is suggestive evidence that the patient is word deaf, 
and the examination should then proceed from that standpoint. It must 
be borne in mind that there is a varying degree of word deafness in cases 
of motor aphasia. Thomas and Roux suggest a method for eliciting it. 
The patient is shown an object and the examiner pronounces several 
syllables of the name of the object, the first, last, or intermediate syllable. 
In minor degrees of word deafness the patient recognizes some of the 
syllables, not all. 
Although all the speech centres may be simultaneously disorganized, 
the symptoms attributable to lesion of one usually dominate the speech 
defect. If the examination so far seems to suggest the existence of the 
word deafness as the leading feature of the sensory aphasia, it should then 
be determined to what degree of completeness this exists, and the extent 
and kind of disturbance that it causes in the externalization of language. 
The amount of diminution of the patient's vocabulary, the degree of 
inappropriate usage of words, the imperfections of sequence and rhythm, 
should all be noted. The patient should be tested for his power of recog- 
nition of simple words, short sentences, and long sentences. As he may 
react to conventional questions, uncommon requests should be made. 
The ability of the patient to interpret sounds should then be noted. 
Do sounds evoke previous memories of similar sounds, and do they incite 
the auditory centre to revive the name of the object from which sounds 
proceed ? When a bell is sounded, or a watch is held behind the ear and 
apart from the stimulation of any perceptual avenue other than hearing, 
can the patient say "bell" or "watch?" 
Then an attempt should be made to determine the various degrees of 
indentification of sounds and words which the patient hears, his capacity 
for repetition, and his capacity for spontaneous thought and action. 
His capacity to write spontaneously and from dictation, to read to him- 
self, should then be tested, and then his ability to name objects and to 
designate persons and things. 
Finally, the existence of any disturbance of bone or aerial conductivity 
should be demonstrated or excluded. 
If word deafness can be excluded and the patient still makes no reply 
-that is, if he remains completely speechless-the examination should 
be made to determine whether or not internal language is defective, for 
the question has then narrowed itself to a determination of whether or 
not the aphasia is Broca's aphasia, or whether it is subcortical motor 306 
DISEASES OF THE NERVOUS SYSTEM 
aphasia. In other words, is the inability to speak due to a lesion of 
Broca's area, or is it due to lesion of the neurons that conduct the motor 
word impulses from the Rolandic area to the parts that externalize the 
word? The essential thing, then, is to determine if the patient is in full 
possession of the internal language. If the internal language in any of 
its components is disordered, then the patient has true cortical motor 
aphasia. If, on the other hand, there is no such disturbance, the lesion 
is elsewhere than in the zone of language. In some patients the differ- 
entiation will be an easy one. On the other hand, however, the task is 
oftentimes extremely difficult. It is particularly so because the test to 
determine if the legitimate idea of words can be evoked in the internal 
language (the test of Proust and of Lichtheim) is not one of universal 
application, because in the first place many patients have no sufficient 
scholarship to know anything of syllables or word construction. In the 
second place, there is very often associated with aphasia a slight degree 
of dementia. In such patients it is often extremely difficult to make 
them understand just what is meant by telling them to press the physi- 
cian's hand as many times as there are syllables in a word. Nor is the 
substitute of asking the patient to make voluntary expiratory effort 
as many times as there are syllables or letters in a word more applicable. 
But even when we cannot get the patient to respond to these tests, there 
is a general atmosphere about the patient with subcortical motor aphasia 
(aphemia, anarthria) which enables one to recognize that he is in full 
possession of his intellect and internal speech. The only shortcoming 
of the subcortical motor aphasic is inability to articulate. He under- 
stands everything that is said to him; he interprets information received 
through the visual sphere; he is capable of expressing his thoughts 
fully, easily, and correctly by writing and by pantomime, or, at least, he 
would be if it were not that the right half of the body is usually paralyzed, 
and he is obliged to portray mental states by the pantomime activity of 
the left, the less dextrous half of the body. 
Difficulty is often found in properly assigning cases of cortical motor 
aphasia, because the patient is still able to articulate some words. If it 
be kept in mind that the patient with Broca's aphasia need not be abso- 
lutely deprived of the power to articulate words; that he frequently 
retains the ability to say one or several words, which he uses at all times 
and under all conditions, and that frequently these words take the form 
of recurring utterances; that there is always agraphia, which is usually 
proportionate to the aphasia; that it is manifest in voluntary writing 
and in writing from dictation, but not in writing from copy, and that the 
patient in copying, copies print in script and script in print, showing 
that the copying is not a mechanical, but an intellectual act; and that 
there is defective internal speech, then the diagnosis of Broca's aphasia 
will not be so difficult. 
After having tested the patient's capacity to perceive and interpret 
words through the auditory apparatus, he should be examined with a 
view to determining if there is any disability of acquiring and interpret- 
ing information through the visual apparatus. To do this requires 
patience and circumspection. In the first place, it should be established APHASIA 
307 
that the patient has no trouble with the peripheral apparatus. This 
can be done by an ophthalmoscopic examination. Tests should be made 
to determine the existence of hemianopsia. This is not an easy matter 
to do if the patient is aphemic or if he has word deafness; in fact it is 
extremely difficult to do satisfactorily. With a patient who can under- 
stand what is said to him and who can indicate when he perceives the 
entrance of an object into the visual field, who can tell when the indicator 
of a perimeter passes beyond the range of vision, testing for hemianopsia 
is a simple matter. If the patient is word deaf, and if he has visual 
blindness, which, of course, he is apt to have if he has hemianopsia, one 
finds himself unable to convey to the patient by written or spoken word 
that which one wishes him to do or to observe. In such cases one must 
content himself with the information that is to be derived from forcibly 
and suddenly thrusting some object into the visual fields, from the right 
side (for right-handed patients invariably have right lateral homonymous 
hemianopsia when they have any), and taking note whether or not the 
patient blinks, as he should do if the object be perceived. If he does 
not, it is rather certain that he has hemianopsia. 
To determine the integrity of the visual mechanism, one may show 
him familiar objects. If, having normal ocular apparatus, he does not 
recognize them, or shows by act or deed that he comprehends their uses 
or purposes, the lesion is probably of the occipital cortex. Such an 
individual may obtain information through the medium of other special 
senses, such as the tactile, gustatory, etc., that will enable him to recog- 
nize the object, the person, or the thing. If he is shown familiar objects, 
and he recognizes them, knows what they are for, but cannot name them, 
then he may have either an interruption in the pathway leading to the 
higher visual centre or in the centre of the intellect specialized for visual 
language. If it be the former, internal language will be preserved and 
spontaneous speech may be intact, although there is usually some para- 
phasia and jargon aphasia, and this preservation is shown most con- 
clusively by the retention of ability to write. He may write easily and 
moderately well, not only voluntarily, but from dictation, but is unable to 
read what he writes. If the aphasia be of the latter character and com- 
plete, the patient will be absolutely agraphic. This agraphia is to be 
considered a part of the disorder of internal language; there is inability 
to arouse the visual image of the word. In such a case, an arousal must 
precede the transmission to the part of the Rolandic cortex that inner- 
vates the member holding the pen. 
We shall understand aphasia better after we have interpreted the 
significance of apraxia, and in every case of aphasia the existence or 
absence of apraxia should be established from examination of the patient's 
spontaneity and initiative and by a study of movements conditioned by 
visual, auditory, tactile, and kinesthetic stimuli. It should be specifically 
noted what the patient does when objects are held up before him, what 
his responses are, and how they are expressed to appeals of any nature 
made to him through his vision, his hearing, or his senses of touch and 
posture. Apraxia may exist without aphasia, and vice versa, and when 
they occur separately the interpretation of each is much easier. CHAPTER IX. 
TUMORS OF THE BRAIN AND MENINGES. 
HARVEY CUSHING, M.D. 
Introductory.-Under the caption "Brain Tumors" it is customary 
to group all neoplasms which encroach upon the intracranial space. 
In this broad sense the variety of new-growths which symptomatically 
compromise the brain, whether by displacement from pressure or by 
actual destruction, is multitudinous. They include not only the tumors 
originating from the tissue components of the brain and its various 
appendages, but those arising from the meningeal envelopes and even 
from the cranium as well; they include metastatic growths whose tissue 
elements may be foreign to the brain, and also certain infectious lesions- 
cysts and granulomata-which elicit the familiar pressure symptoms of 
tumor, though, strictly speaking, they are not neoplasms. 
The characteristic symptom complex of an intracranial tumor may 
therefore be produced by lesions which originate from a cranial or extra- 
cranial source and implicate the brain secondarily through metastasis 
or invasion; although lesions of this type are numerous, for the sake of 
brevity they will be given only passing consideration. One must dis- 
tinguish clearly between the growths which primarily occur within the 
substance of the brain and those which arise from its appendages or 
meningeal coverings.1 
Incidence.-Despite their supposed rarity, the brain is actually one 
of the most common seats of tumor growth-their seat of predilection 
according to Oppenheim; and Bruns affirms that when a general prac- 
titioner says he has never encountered an instance it is an acknowledg- 
ment that patients with tumor have passed through his hands unrecognized. 
Though during the past decade physicians have largely awakened to 
this fact, it is still true in all probability that by far the larger number 
are incorrectly diagnosed unless at a terminal stage of the disorder. 
Furthermore, as the progress of the disease is often a lamentably slow 
one, it is with comparative infrequency that patients thus afflicted 
end their days in a general hospital. Hence it is unusual for them to 
figure largely in pathological records. In certain hospitals large numbers 
of these cases have been observed. Thus in the National Hospital, 
London, there were 500 cases between 1902 and 1911 (Tooth).2 The 
writer's series comprises almost an equal number.3 
1 From the enormous literature there are a few comprehensive monographs which 
should be consulted: L. Bruns, Die Geschwiilste des Nervensystems, 2te Auflage; S. 
Karger, Berlin, 1908; II. Oppenheim, Die Geschwiilste des Gehirns, NothnageVs Spec. 
Path. u. Ther., Wien, 1896, Bd. ix; H. Duret, Les Tumeurs de I'Encephale, Felix Alcan, 
Paris, 1905; E. Poggio, Diagnostic di Sode dei Tumori Intracranin. Unione Typo- 
graphica Editrice di Torino. 
2 Brain, 1912, xxxv, 64-108. 3 Journal of the Amer. Med. Assoc., 1915, Ixiv, 189 
308 TUMORS OF THE BRAIN AND MENINGES 
309 
In the autopsy reports of the Johns Hopkins Hospital to January 1,1909, 
among 3150 autopsies there were 55 cases of brain tumor (1.7 per cent.). 
This is a larger percentage than that given by Siedel, who found, in 
Munich, 1.25 per cent., and by v. Beck, who found, in Heidelberg, 0.8 
per cent. There are many elements to be considered, such as the average 
age of hospital patients, the character of the service, and the interest 
paid to neurological cases. Bruns states that 2 per cent, of all patients 
classified as neurological suffer from brain tumor, and Blackburn's 
figures (29 tumors in 1642 autopsies, nearly 2 per cent.) show an equal 
percentage for asylum patients. 
In the medical wards of the Johns Hopkins Hospital, in the first 
25,000 admissions, there were in the neighborhood of 100 cases diagnosed 
as brain tumor, making an average of one tumor in every 250 patients. 
In the surgical service during the same period the number of patients 
with the diagnosis of brain tumor rose from 0.06 per cent, in the first 
5000 admissions, to 0.2 per cent, in the second 5000, to 0.3 per cent, in the 
third 5000, to 0.75 per cent, in the fourth 5000, and to 1.3 per cent, in 
the following 3000 cases. In the subsequent 1000 surgical admissions 
there were 40 tumor cases, showing the rapid increase when particular 
attention is given to them. This is further emphasized by the experience 
at the Peter Bent Brigham Hospital where in the first 2500 surgical 
admissions there were (including cases readmitted) approximately 200 
cases (8 per cent.) of brain tumor or presumed brain tumor; this number 
is exclusive of the growths associated with the pituitary body. 
Etiology.-Sex.-There is a general opinion that tumors occur with 
greater frequency in the male than in the female-twice as often, accord- 
ing to the following figures: Bruns found, in 63 autopsy cases, 40 males 
and 23 females; Gowers, in 650 clinical cases, found 440 males and 210 
females. An analysis of the author's first 130 patients with tumor or 
presumed tumor showed 87 males and only 43 females. 
Age.-More than half the cases occur between the ages of twenty and 
forty years. Tumors are said to be rare at the extremes of life, if tubercles 
and congenital growths of infancy be excluded. This, however, must 
be taken with a certain reservation, for in the 22 cases in the first decade, 
there was one dermoid cyst and one tubercle, the others being mostly 
cystic gliomas. Gowers limits the relative immunity of infancy to the 
first five months. Grouping the aforementioned 130 cases according to 
decades, there were 22 in the first, 13 in the second, 28 in the third, 37 
in the fourth, 20 in the fifth, 8 in the sixth, and 2 in the seventh decade. 
Trauma.--The relation between trauma and the first appearance of 
symptoms is a coincidence that occurs too often to be ignored, though it 
is possible that blows on the head may serve merely to bring a preexisting 
lesion into symptomatic prominence; for, in consequence of the blow, 
the rupture of a bloodvessel may occur in a vascular glioma or concussion 
oedema may accentuate symptoms which were previously vague. The 
point of injury may even indicate the situation of the lesion and this 
would appear to be true even of the meningeal endotheliomata. It is 
more often the case, however, with the granulomata, for here we are 
dealing with a simple infection at a spot of lessened tissue resistance. 310 
DISEASES OF THE NERVOUS SYSTEM 
Indeed, the latent tumor itself, through an epileptic attack or faint, may 
have been the cause of the fall producing the cranial injury. Thus 
trauma may not actually occasion, but may only serve to bring into the 
open, the symptoms of a previously obscure growth. This is a debated 
question, and naturally one of the arguments advanced in its favor is the 
greater frequency of tumors in the adult male, whose occupations and 
sports engender blows on the head. Shaving the scalp preparatory to 
operation often reveals an unsuspected and significant scar. 
Pathology.-The comments on the frequency of brain tumors indicate 
how misleading collective evidence may be; and this applies even more 
to the past statistics relative to their histological nature. Few pathologists 
have had opportunity or inclination to make a comprehensive study 
of a large series of cases, for a complete postmortem investigation entails 
three factors: (1) The gross relations and physical character of the 
tumor in so far as it disturbs the brain in general as well as its circulation; 
(2) the histological nature of the lesion itself; and (3) the secondary 
degenerations. The first requires the careful fixation of the tissues 
in situ by carotid injections-a precaution which is rarely taken. The 
second necessitates absolutely fresh tissues, for the postmortem changes 
which occur rapidly in the central nervous system militate against 
the perfect fixation essential for fibrillar stains. The third object of 
study-the degenerations-can only be of contributory value in excep- 
tional cases when lesions are small, uncomplicated, situated in such areas 
as to have involved paths whose degenerations are profitable for study, 
and finally when the pathological findings can be correlated with an 
exhaustive clinical record. One or another of these is usually lacking. 
Mallory's studies1 have shown that it may be impossible to make 
positive statements in regard to the cellular structure of many tumors, 
particularly of those arising in the nervous system, without operative 
removal and immediate tissue fixation. This goes to show with what 
caution one must accept earlier statistics on the frequency of glioma and 
sarcoma. A matter of equal importance has been emphasized by Adolf 
Meyer and Charles J. Lambert, namely, the desirability of a histological 
study of the entire growth in situ. The section of an entire growth will 
oftentimes betray its nature, owing to a certain characteristic arrange- 
ment of the cells which cannot be appreciated by the survey of a small 
fragment. This applies particularly to the recognition of certain gliomata. 
The histological diagnosis in our early (Baltimore) series of 130 cases 
was authenticated both by Dr. Mallory and by Dr. Lambert, so that with 
the exception of one or two tumors of very obscure nature we may speak 
of them with some certainty. The tissues became available either through 
operation or autopsy in 69 cases. Of these, 44, or 66.6 per cent., proved 
to be gliomata or gliomatous cysts (11 cases, in 3 of which a demonstrable 
shell of tumor was still present), or gliopsammomata (3 cases). Many 
of these in the past would have been classed with sarcomata, of which 
there was but a single instance (metastatic) if we exclude the 5 endothe- 
liomas. There were 4 tubercles and 3 syphilomas: with the rest of the 
1 Jour, of Med. Research, 1905, viii, N. S., p. 113. TUMORS OF THE BRAIN AND MENINGES 
311 
field scattering. In Allen Starr's much quoted table,1 based on 600 
cases gathered from various sources, at a time when histological differ- 
entiation was impossible, there were 91 gliomata, only 15 per cent.; 
whereas there were 120 cases, or 20 per cent., tabulated as sarcoma. 
Hence sarcoma was long regarded as by far the commonest tumor in the 
adult. In 70 certified lesions in our more recent (Boston) series the 
diagnosis was as follows: glioma and gliomatous cyst, 27 (38.5 per cent.); 
endothelioma, 7; angioma, 2; sarcoma, 1; carcinoma metastatic, 1; syphil- 
oma, 4; tuberculoma, 4; osteoma, 1; ecchinococcus cyst, 1; adenoma, 17 
(hypophyseal 15, pineal 2); interpeduncular and mixed tumors, 5. Exclud- 
ing the pituitary cases the gliomas would represent about 50 per cent, of 
the series. In Tooth's studies2 of the cases at the National Hospital, in 
258 certified cases 127, or 49.2 per cent., were gliomas. So that one may 
say that one-half of all brain tumors are gliomas of one kind or another. 
Gliomata.-These were first shown by Virchow to represent specific 
growths of the neuroglial tissue framework of the central nervous system; 
and since the discovery of a specific stain for neuroglial and ependymal 
cells, Mallory3 and Stroebe have demonstrated that they may arise from 
the ependyma as well. A glioma consequently may arise in remote 
situations, as the coccygeal region, from vestiges of the primitive neural 
canal. Christian has shown that ependymal epithelium and neuroglia 
are common constituents of teratoid tumors of ovarian origin. Thus, 
a glioma is a growth of ectodermal origin, whereas a sarcoma arises 
invariably from the mesenchymal tissues. A true combination of 
these forms is rare, although the difficulty of distinguishing them 
without special stains and close study has led to the misuse of the term 
"gliosarcoma." 
Cells are found in gliomata showing all gradations from the undiffer- 
entiated embryonal type to the characteristic neuroglial spider cells 
on the one hand and toward ganglion cells on the other. These growths 
doubtless have an embryonic origin, and thus a single cellular anlage 
suffices to explain all the types of cells which are found-neuroglial, 
ependymal, or ganglionic. The growth may be made up almost entirely 
of round, undifferentiated cells, possessing no fibrillae, so that only by 
some characteristic arrangement of these cells, or by the finding of a 
small group which has begun to assume a more adult type, can such a 
tumor be distinguished from certain forms of sarcoma. Lambert is of the 
opinion that there is a fairly uniform tendency for the cells to arrange 
themselves more or less radially around a central core, which, on cross- 
section shows a characteristic rosette-shaped figure-the satisfactory 
disclosure of which may require a section through the entire growth. 
The variable differentiation of the primitive cells into those of a 
ganglionic type or into ependymal cells has led to the terms neuroglioma 
ganglionare, ependymal glioma, etc. The neuroglioma of Klebs, in which 
the nerve fibres themselves participate in the new-growth, becomes a 
1 Brain Surgery, Wm, Wood & Co., N. Y., 1893, p. 202. 
2 Brain, 1912-13, xxxv, 61-108. 
3 Jour, of Med. Research, 1902, iii, N. S., p. 1. 312 
DISEASES OF THE NERVOUS SYSTEM 
somewhat doubtful lesion in view of the known persistence of normal 
preexisting fibres which traverse many of these tumors. 
A glioma is usually a solitary tumor which may occur in any part of 
the cerebrum, cerebellum, or brain stem. There is often an extensive 
gliosis which spreads widely over the nervous system with but a single 
area which in gross is evidently a neoplasm. In a few of the primary 
cerebellar gliomas in the author's series the gliosis had in time extended 
throughout the length of the entire spinal cord. Though such a gliosis 
is a diffuse process the tumor spreads by extension and in a strict sense 
does not form metastases, although regionary extensions which resemble 
them may occur. They may be of cortical or basal origin, or may arise, 
although less frequently, from the ependyma. Gliomata supposedly 
originating in the corona radiata are common and may attain a large 
size. The ependymal gliomata which arise from the subependymal 
layer of the ventricles, possess unusual characteristics. Ten out of the 
twelve published cases, according to Martens and Seiffer,1 originated, 
as did their own, in the fourth ventricle, which seems to be their seat of 
predilection. The striking feature of these growths is their multiple 
nature, for they have a tendency to spread, so that small, metastatic 
nodules may be found throughout the ventricles, resulting, in rare cases, 
in an extensive diffuse growth-ependymgliomatosis. Their symptom- 
atology is obscure and they seem never to have been diagnosed clinically.2 
There have been two cases in the author's series in both of which the 
tumor had extended down on the dorsum of the spinal cord. 
In color, gliomata resemble the grayish red of cortical tissue, and 
their consistence is not unlike the normal brain, so that in the absence of 
pigmentation from hemorrhages, from cystic or other structural meta- 
morphoses, it may be difficult to distinguish them from the surrounding 
tissues when exposed during an operation or even at autopsy. This is 
particularly true of certain forms of diffuse gliomatosis, often mistaken 
(Gowers) for hypertrophies of the brain. Some of the largest recorded 
intracranial tumors have been gliomata. A gliopsammoma in our series 
weighed 496 grams. 
Gliomata are usually described as infiltrating tumors with an indefinite 
boundary which shades off into the normal tissues without clear demar- 
cation; but this is not always the case, for they may be encapsulated. 
By their character of growth it frequently occurs that the form of the 
brain remains unchanged, with complete absence of pressure symptoms, 
and also that normal functionating nerve fibres are found traversing them.3 
This explains the latency of many gliomatous growths and also shows 
how their extirpation may greatly accentuate focal symptoms which were 
slight before their operative removal. The pia usually serves to check 
their extension, although this is not invariable, for we have seen a number 
of gliomata which have broken through into the subarachnoid spaces and 
spread over the surface in the manner of an infective process. 
Gliomata are often exceedingly vascular (gliomateleangiectodes), and 
1 Berl. klin. Woch., 1908, xlv, 1477. 
2 Bassoe, Archives, of Int. Med., 1908, ii, 194. 
3 Byrnes, Jour, of Nerv. and Ment. Dis., 1909, xxxvi, 129. TUMORS OF THE BRAIN AND MENINGES 
313 
trauma, congestion, or operation with dislocation of position, may lead 
to hemorrhages from rupture of the thin-walled vessels. A spontaneous 
hemorrhage simulating apoplexy may occur and be the first symptom 
of an unsuspected growth; or repeated small hemorrhages may occur, 
with marked fluctuation of symptoms. Degenerative processes with 
cyst formation (cystogliomd) are common, and the entire growth may 
seemingly become replaced by a single cyst, although a careful search 
will often reveal vestiges of the original growth on the cyst wall. Areas 
of calcification-gliopsammoma-or even ossification, may occur. 
Certain tumors of mesenchymal origin (sarcomata) can only be distin- 
guished from some of the gliomata by stains of absolutely fresh and 
properly prepared tissue. Gliomata were claimed by Strobe1 to be dis- 
tinguishable from sarcomata by their gross appearance and manner of 
growth, for the latter are encapsulated, are sharply distinguishable from 
the brain, and easily enucleated. This distinction is not to be relied upon, 
for a number of gliomata in our series have shown all the features, except 
histological ones, thought to characterize sarcoma. 
Endotheliomata.-Next to the gliomata these tumors, often misnamed 
psammoma, sarcoma or fibroma are probably the most frequent of all 
true intracranial tumors (14.3 per cent., Tooth; 11.1 per cent., Cushing), 
though they take their origin from the endothelial lining of the meninges 
much more often than from an endothelial source, vascular or lymphatic, 
within the substance of the brain itself. Hence, they are apt to be extra- 
cerebral or extracerebellar, as the case may be, rather than intracerebral 
or cerebellar, and in a strict sense are not brain tumors. They are com- 
monly called dural endotheliomas, but it is the author's belief that they 
arise from the clusters of mesothelial cells of the arachnoid villi or 
tufts which project into the dura and which are the points of outlet 
for the cerebrospinal fluid.2 
In the statistical tables of the past they have been included with 
sarcomata or fibromata, for their spindle-shaped cells resemble those 
characterizing these growths. Presumably all of the so-called benign 
fibrosarcomata originating either from the cerebral or spinal meninges 
have been of this nature, and the diagnosis of fibroma of the brain is 
open to doubt. Mallory has demonstrated that fibrils do not occur in 
these tumors, so that they can be thus differentiated histologically from 
fibromata or sarcomata. 
These tumors are intrinsically benign, but ultimately cause pressure 
symptoms by slow indentation or moulding of the cerebral substance. 
They do not invade the cerebral tissues, but can be readily dislocated from 
their cavity or nest, leaving a smooth surface. They grow slowly and 
are often of long duration. In two of our cases the lesion had doubtless 
been present, giving local symptoms, for thirteen and nine years respec- 
tively before pressure disturbances of serious import, due in each to an 
obstructive hydrocephalus, became pronounced. Blackburn's figures 
indicate that a considerable proportion of the asylum cases showing 
tumor after death have this lesion. 
1 Ziegler's Beitrage, 1895, xviii, 405. 
2 Studies on the Cerebrospinal Fluid, Cushing and Weed, Jour. Med. Research, 1914. 314 
DISEASES OF THE NERVOUS SYSTEM 
Endotheliomata may attain a large size (one tnmor in the author's 
series weighed 246 grams on removal) and they may occur in almost 
any situation over the hemisphere. They, however, have certain seats of 
predilection, a very common source of origin being from the arachnoid 
villi along the sagittal sinus. They not infrequently cause an osteoma- 
like dome-shaped thickening of the overlying skull. They may, on the 
other hand, cause a pressure absorption of the adjacent skull. Another 
supposedly common seat of these tumors-possibly due to the unmis- 
takable symptoms elicited-is the cerebellopontine recess. Occasionally 
bilateral tumors may be found in this situation. Owing to their presumed 
orgin from the sheath of the acousticus and the early onset of auditory 
symptoms, they are often spoken of as acoustic fibromata. They are, how- 
ever, to be distinguished from the intracranial neurofibromata of von 
Recklinghausen's disease, which are apt to be symmetrical and may be 
multiple, involving other nerves than the auditory. Endotheliomata 
sometimes undergo degenerative processes, and large gelatinous or cystic 
areas may be found. Of all tumors, they are the most favorable for 
operative removal. 
Sarcomata.-The so-called gliosarcomata are in all probability gliomata 
made up in part of undifferentiated cells; the fibrosarcoma of the past 
is, in truth, a meningeal endothelioma. Still, if one is to include all 
actual sarcomata which serve directly or indirectly to implicate the 
encephalon, they may perhaps continue to represent a form of cerebral 
growth which is common enough, even though they no longer out-number 
other tumors. They are nowise specific of the nervous system, and 
although they may originate there, are more often invasive or metastatic 
in origin. They may be intracranial or extracranial growths. 
Although varying greatly in consistency, being in general of a firmer 
structure than most gliomata, they nevertheless are sharply distin- 
guishable from the brain tissue in which they lie more or less definitely 
encapsulated and from which they are easily shelled out. There is usually 
a zone of softening of the surrounding brain due to vascular disturbances. 
This would make them favorable for surgical removal were it not counter- 
balanced by the frequent presence of secondary metastatic nodules and 
the tendency to involve adjacent tissues. 
These tumors, often of cranial origin, include the alveolar sarcomata, 
growths which, without fibrillar stains, it may be impossible to distinguish 
from endotheliomata or from true epithelial tumors. They are often of 
diploetic origin, the temporal bone constituting their favorite seat 
(Weisswanger). Osteosarcomata likewise take their origin from either the 
cranial vault or base and, penetrating the skull, lead to pressure symptoms. 
Endotheliomata arising from the meninges may also come to involve 
the bone and assume certain outward characteristics of an osteosarcoma 
from which they must be carefully differentiated. These so-called per- 
forating sarcomata may arise from either surface of the dura, from diploe 
or either table of the skull, and as they infiltrate the normal tissues and 
even absorb the bone in their process of growth, they are apt in time to 
give external evidence of their situation. As the bony absorption takes 
place slowly, the growths are apt to assume an hour-glass shape, the TUMORS OF THE BRAIN AND MENINGES 
315 
intracranial portion of the tumor indenting, but usually not invading, 
the cerebral cortex, which remains protected by pia-arachnoid. When 
the scalp has become broken down the condition known as fungus du res 
matris results. The brain is occasionally involved secondary to a cranial 
lesion by the growths designated as chloroma or myeloma. 
Sarcomata found within the substance of the brain are usually of metas- 
tatic origin and show the character of the original tumor. They may be 
cystic, or may undergo cystic degeneration (cystosarcomata)-a process 
which occurs possibly less often than is the case with gliomata. They 
also may become oedematous and the cells assume a mucoid character-- 
myxosarcomata. Other degenerative processes may lead to necrosis or 
caseation, when they may bear a close resemblance to tubercle. Deposits 
of calcification may occur within them, giving rise to psammosarcomata 
or angiolithic sarcomata. Intracranial growths with psammomatous 
bodies, whether gliomatous or sarcomatous, can occasionally be localized 
by the a'-rays. Pigment-containing sarcomata are said to occur in the 
brain only in metastatic form and the metastases, as a rule, are numerous. 
The single indubitable sarcoma in our series was a large, coal-black, 
encapsulated melanosarcoma removed at operation from a subcortical 
situation in the parietal lobe. At the time there was no obvious 
extra-cerebral source of origin. The patient died a year later, and a 
primary melanotic growth of the ovary was found with numerous 
small, visceral metastases and two other nodules occupying silent areas 
in the brain. 
Diffuse Sarcoma.-There is a particular form of diffuse sarcoma 
(pachymeningitis interna sarcomatosa, Orth) in which a primary sarcoma- 
tous thickening of the meninges may occur almost over the entire central 
nervous system-brain, cord, and nerves often being encased by the 
nodular masses; it would seem as though a direct implantation of tumor 
cells had occurred through the medium of the cerebrospinal fluid. Oc- 
casionally a primary growth seems to have burst into one of the lateral 
ventricles, leading to the inoculation, as it were, of the cerebrospinal fluid 
with wide dissemination of the infective agency. The process in less 
extensive form is more commonly met with about the base of the brain, 
often with bilateral involvement of the cerebral nerves, and not infre- 
quently of the cauda equina as well. 
Carcinomata.-Like the foregoing these tumors occur for the most part 
as metastases, being found most often as secondary nodules in cases of 
mammary cancer. The nodules occur usually in the substance of the 
brain, are frequently multiple, and show a marked tendency to cystic 
degeneration. Epithelial tumors occasionally arise from a cranial or 
extracranial source and invade the cranial chamber secondarily. We have 
seen such a tumor apparently of labyrinthine origin. 
Epitheliomata and Papilloma.-Primary epithelial tumors may arise 
in the brain from the secreting cells of the choroid plexus (Ziegler), and 
one of the tumors in our series, difficult to classify, is a large, seemingly 
benign epithelial growth arising in the occipital ventricle and containing 
cysts with gelatinous contents, apparently of this origin. These tumors 
are regarded by some as a simple hyperplasia or adenomatous papilloma 316 
DISEASES OF THE NERVOUS SYSTEM 
of the plexus; 38 analogous cases have been collected from the literature.1 
Epithelioma of the ependyma has been described, and the hypophysis 
is naturally a not infrequent seat of epithelial as of other growths. These 
are commonly a form of malignant adenoma, into which a pituitary 
struma has become transformed much as a thyroid struma may undergo 
malignant changes. 
Cholesteatomata.-Sometimes called "mother-of-pearl" tumors in con- 
sequence of their peculiar glistening external appearance, these growths 
have their seat of predilection in the basal meninges. They appar- 
ently never arise primarily in the brain substance itself, although this 
becomes excavated and compressed as the growth advances. There is 
no unanimity of opinion as to their histogenesis. Virchow, Frank, and 
others thought that the cells of the arachnoidal meshwork multiply and 
distend the pre-formed endothelial spaces, producing a sort of complex 
retention cyst rather than a true tumor growth. A later view, advanced 
by Ziegler, Bostrcem, and others, favors a congenital anlage of misplaced 
epidermoidal cells, supported by the fact that the cells become cornified 
and contain keratohyalin. Bostroem has demonstrated that a close 
relationship exists between these and the more usual forms of dermoid 
tumors. Possibly both epithelial and endothelial forms may exist. 
The otitic cholesteatoma of older writers, following chronic suppura- 
tions of the middle ear, apparently consists of epidermoidal masses which 
cause pressure absorption of the temporal bone and invade the cranial 
chamber secondarily. These tumors usually possess a more or less 
irregular flat form, modified as the growth pushes its way into the clefts 
and recesses between the bone and base of the brain. The surface is 
often covered with warty irregularities. On section these tumors consist 
of a grayish, friable, more or less laminated mass, made up t)f layers of a 
closely packed mosaic of flat, polygonal cells-really a dead tissue con- 
taining no bloodvessels. They are of slow growth and may run a symp- 
tomless course or give definite general and focal symptoms of a basilar 
tumor with cerebellar, cerebellopontine, or trigeminal symptoms. 
Dermoid Tumors.-These are among the rarest of intracranial growths. 
There is a single instance in our series, the cyst, containing hair, having 
been successfully extirpated. Bostroem considers that various grades 
are recognizable, from a simple epithelial-lined sac, which he would 
regard as a cholesteatoma, up to the most complex embryoma which may 
contain examples of many tissues of the body. These tumors date from 
the third to the fifth week of embryonic life. They are usually basal, 
commonly mesial, grow slowly, and are not likely to produce symptoms. 
Another variety is connected with the formation of dura and bone, and 
it is not uncommon for them to connect, through a cranial defect, by a 
stalk with an extracranial dermoid. 
There are other lesions, possibly somewhat less rare than the foregoing, 
which deserve passing mention to complete the enumeration. 
Teratomata.-These have been found, chiefly in the neighborhood of 
the interpeduncular space, arising doubtless from some embryonal 
1 Boudet et Clunet, Arch, de Med. Experiment el d'Anat. Patholog., 1910, xxii, 379. TUMORS OF THE BRAIN AND MENINGES 
317 
vestige with faulty development of the pituitary gland. They are apt 
to be accompanied by symptoms of hypopituitarism and by the char- 
acteristic local symptoms of tumor in this situation.1 
Chordomata.-These tumors arise from an anlage at the cephalic end of 
the primative notochord. They are usually small but may undergo 
malignant transformation and reach a large size as in the case recorded 
by Jelliffe and Larkin.2 They are situated in the interpeduncular space 
and the resemblance to cartilage possibly leads to a diagnosis of chon- 
droma or teratoma, and this may account for their presumed rarity. 
Lipomata.-These are rare and those described have usually been 
small, pea-sized, basal growths in which bone is often present. Zucker- 
mann has collected 50 such cases. A larger form is known which has 
especial predilection for the upper surface of the corpus callosum. Hecht 
has described a lipoma of the Sylvian Fissure. 
Fibromata.-These are occasionally found in fairly pure form, arising 
from the periosteum of the cranial base or from the nerve sheaths- 
the latter being more properly classified as a manifestation of von Reck- 
linghausen's disease. It is presumable that the endothelial cells of an 
endothelioma may become fibroblastic, and thus many so-called fibromata 
really belong to the endothelioma group. 
Neuromata.-The same is true of the so-called neuromata, which arise 
from the perineurium of the cerebral nerves, and consequently are extra- 
cerebral tumors. They usually surround the entire nerve, giving it a 
spindle-shaped form, and may undergo sarcomatous or myxomatous 
change. They are particularly common on the acousticus, but other 
nerves may be involved (multiple neurofibromatosis). 
Neurocytoma or Neuroblastoma.--These terms have been introduced 
by J. H. Wright3 to cover a group of tumors the essential constituents 
of which are undifferentiated nerve cells (neurocytes) and the fibrils 
of which resemble those occurring in the anlage of the sympathetic 
nervous system and chromaffin tissues. There may be a simultaneous 
involvement of adrenal, liver, and brain. The single example identified 
in our series had been regarded as a glioma. 
Enchondromata.-These have been found arising from the choroid 
plexus or meninges. One especial variety, arising from the cartilaginous 
vestige of the Clivus Blumenbachii (Virchow), has its seat of predilection 
near the pituitary fossa (cf. chordoma and teratoma). 
Angiomata.-Angiomata, whether simple, telangiectatic, or cavernous, 
are congenital and usually cortical. As a rule, they produce no serious 
symptoms, barring the possible accident of hemorrhage or thrombosis. 
Typical pressure symptoms were elicited in one of our cases by a large, 
cavernous angioma of the temporal region. The angioma racemosum 
may occur alone or with congenital vascular lesions of the scalp or face 
(Kalischer; Emanual; Cushing). Cavernous angiomas are rare.4 
1 H. Cushing, Jour, of Nerv. and Ment. Dis., 1906, xxxiii, 704; D'Orsey Hecht, 
Jour. Am. Med. Assn., 1909, liii. 
2 Jour, of Nerv. and Ment. Dis., 1912, xxxix, 1. 
3 Jour, of Exper. Med., 1910, xii, 556. 
4 Astwazaturoff, Erankf. Zeitschr. f. Pathol., 1910, iv, 482. 318 
DISEASES OF THE NERVOUS SYSTEM 
Psammoma.-This is a term given to a number of tumors, glioma, 
sarcoma, and endothelioma, in which areas of calcification have taken 
place, making them gritty on section. Bruns is inclined to restrict the 
term to certain growths which arise in the pineal gland or choroid plexus 
where, normally, calcareous bodies-the so-called "brain sand"-occur. 
Cysts.-We meet with (1) congenital interpeduncular (pituitary) cysts; 
(2) simple serous cysts which presumably are degenerated tumors; (3) 
tumors containing cysts, as cystosarcoma, cystoglioma, etc., possibly 
on the way to the above; (4) blood cysts, the result of cerebral or cerebellar 
apoplexy; (5) cysts the result of softening after embolism; (6) the cystic 
widenings or diverticula of the fourth ventricle; (7) dermoid cysts; (8) 
parasitic cysts; and (9) gas cysts. 
The congenital interpeduncular or suprasellar cysts, the Jlypophysen- 
erganggeschwulste of Erdheim, arise from a pharyngeal anlage associated 
with the development of the pituitary body. The lesion is not an uncom- 
mon one and is usually, although not always, associated with evidences 
of pituitary insufficiency together with optic atrophy with bitemporal 
blindness. Characteristic outgrowths of squamous (pharyngeal) epithe- 
lium arise from the walls of these cysts. 
It is probable that, in this part of the world at least, the greater number 
of so-called simple cysts are actually degenerated tumors (gliomata and 
sarcomata), although it may be difficult to demonstrate this conclusively. 
Since surgical explorations have become frequent, it has often been found 
that tumors originally solid have become cystic at a later exploration 
or at autopsy; and one occasionally sees a tumor in what seems to be a 
process of cyst formation with a large area of semifluid, degenerated tissue. 
Unlike those which may form as the result of absorption of an apoplectic 
extravasation, and also unlike the porencephalic cysts of childhood, these 
degenerative cysts may continue to give pressure symptoms almost as 
severe as those of the original growth. On evacuation they tend to 
refill. Their fluid content is often of a yellowish or brownish tinge, and 
the fluid, rich in fibrin, clots on exposure to the air. Their seat of predi- 
lection is the cerebellum. Scholz1 has made an elaborate study of 75 
published cases. There have been many cysts in our series, the larger 
percentage of them cerebellar; and we have seen a number of borderline 
conditions in which a glioma seemed to be in the process of total cystic 
degeneration after a decompressive operation. 
There are certain obscure conditions, often seen in association with an 
actual tumor growth, in which a large cyst of the hemisphere has an 
apparent communication with a lateral ventricle. 
Arachnoidal Cysts.-These cysts, a consequence of trauma, may 
give pressure symptoms and focal signs simulating tumor. They may 
follow lesions which leave a defect in bone and dura over a given area, 
for an accumulation of the fluid which exudes through the arachnoidal 
membrane may be under tension, giving focal pressure symptoms. 
Parasitic Cysts.-The Cysticercus celluloses shows an especial predilec- 
tion for the central nervous system. Thus in 87 cases in Berlin the cysts 
1 Mitleil. a. d. Grenzgeb. d. Med. u. Chir., 1906, xvi, 745. TUMORS OF THE BRAIN AND MENINGES 
319 
in 72 instances were cerebral (Bruns). The hooked embryos attach 
themselves to the meninges, cortex, or ventricular wall-often of the 
fourth ventricle-where the larva develops as an isolated bladder-like 
structure, rarely exceeding a centimeter in diameter. The cysts are apt 
to be multiple, 111 of them having been counted by Delore and Bon- 
homme in a single patient. A particular form sometimes occurs in the 
basal meninges and a condition of so-called "cysticercus meningitis" 
may result. Ventricular cysts usually lead to a great increase of fluid 
and a distinct ependymitis may result (Lloyd). 
The larvae may be long-lived, although it is usual for them to shrink 
and become calcified in from three to six years. An especial study has 
been made by Stern1 of the 68 known cases of fourth ventricle involve- 
ment. The recent presence of an adult taenia solium in the intestine 
should arouse suspicion of the nature of the case. 
Echinococcus or Hydatid Cysts.-These may likewise occur in the 
central nervous system. The cysts are more apt to occur in the brain 
substance than in the meninges or ventricles. They are said to be usually 
single though in the only case in our series three were removed at operation 
from one hemisphere. They may reach a large size, and show an especial 
tendency to absorb the overlying skull and thus to appear under the 
scalp. Suppuration may occur, or the worm may die and the contents 
containing the free booklets become gelatinous and thick. The symp- 
toms are those of a slowly growing tumor; and on exposure a differential 
diagnosis from other forms of cyst can at times be assured only by the 
demonstration of head or booklets, or by the peculiar structure of the 
cyst wall. 
Infectious Granulomata.-Formations resembling tumors are the 
frequent result of tuberculous or syphilitic, and occasionally of actinomy- 
cotic, processes. Although not neoplasms in the same sense as some of 
the foregoing, custom calls for their consideration with tumors. 
Encephalic Tuberculomata.-Of encephalic tuberculomata two 
forms are recognized: (1) Focal tuberculous meningo-encephalitis, and (2) 
the so-called solitary tubercle. Tuberculosis of the central nervous system 
is usually-perhaps always-a complication of tuberculosis elsewhere. 
Focal Tuberculous Meningo-encephalitis.-This form consists of a local 
plaque of disease made up of a conglomeration of more or less caseated 
tubercles, usually situated on the surface of one of the cerebral hemi- 
spheres, less often a cerebellar or a basilar process. Although of men- 
ingeal origin, more or less involvement of cortical tissue is inevitable, 
and with the advance of the process the characteristic focal symptoms 
of tumor may occur, although general pressure phenomena are usually 
absent until meningitis supervenes. 
Solitary Tubercle.-The solitary tubercle behaves, on the other hand, 
in all respects like a brain tumor. According to Starr's tables, this 
should be the most common form of growth (32 per cent, if all ages are 
included), representing in the first two decades 50 per cent, of all cases, 
falling to 14 per cent, in adults. In Tooth's table tuberculoma occurred 
1 Zeit. f. klin. Med., 1907, Ixi, 64. 320 
DISEASES OF THE NERVOUS SYSTEM 
in only 5.4 per cent, of the cases and this corresponds closely with our 
own experience (5.7 per cent.). A tubercle has been found at the early 
age of twenty-three days in a case described by Demme. 
The term "solitary" is somewhat misleading, for these individual 
growths are composed of many fused tubercles. "Solitary tuberculoma" 
is a better term, or "conglomerate tubercle," as Schmaus suggested. 
The lesions are multiple, at least at an early stage, in about 50 per cent, 
of all cases (Bruns), becoming fused later into an irregular conglomerate 
mass. This is the more likely since the originally discrete foci, in evidence 
of their embolic origin, are apt to be distributed on a single branch of the 
arterial tree. Their form is roughly spherical, unless modified by fusion 
or other especial influence. Their favorite seat seems to be in the cere- 
bellum or brain stem. The individual lesions vary greatly in size, from 
a millet seed to a hen's egg on the average, although in rare cases by 
fusion of many isolated foci almost one entire hemisphere may become 
transformed into a tuberculous mass. 
Coagulation necrosis with caseation takes place as the lesions enlarge, 
and on section the mass presents a dry, yellowish, crumbling centre, with 
a grayish-red, peripheral growing zone where are located viable bacilli 
and actively growing tubercles. As in tuberculous processes elsewhere 
central liquefaction with abscess formation may occur; less often the 
process dies out and calcification occurs. When the lesions are active the 
surrounding encephalic tissues are usually oedematous, and this tends to 
increase the pressure effects and constitutes a mechanical difference 
between a tuberculous and a sarcomatous tumor; for the latter, as a rule, 
only affects the immediately adjoining zone by pressure softening. 
In no one of our several cases of extirpation of a cerebellar tubercu- 
loma has the recovery been permanent. All have succumbed in the course 
of a few months to the effects of other lesions of like nature. 
Syphilitic Processes.-Syphilitic processes giving a tumor symptom 
complex occur in the central nervous system in two especial forms: (1) 
As a local spreading meningo-encephalitis, and (2) as a solitary granuloma 
-the so-called gumma or syphiloma. To these may be added the basilar 
meningitis gummosa, a process often arising in the middle cranial fossa 
where, with notable fluctuation of symptoms, it implicates one after 
another of the nerves which pass through the region. Vascular lesions 
are common, and sudden hemiplegia may occur from occlusion of the 
middle cerebral artery. This basilar process is apt to spread over the 
sylvian region, and, as the cortex usually is more or less involved, it 
hardly deserves to be distinguished from the more common meningo- 
encephalitis syphilitica of the convexity, except for its peculiar local 
manifestations. 
Bruns, in agreement with Oppenheim and Gowers, regards syphilis as 
the most frequent of the conditions producing the tumor symptom 
complex in adults. This opinion is based naturally on the subsidence of 
symptoms after antisyphilitic therapy-not an infallible test-and is 
contrary to postmortem statistics. It is doubtless on the same clinical 
basis that the cerebral cortex and the paracentral convolutions in par- 
ticular are regarded as the favorite seat for gumma, owing to the definitely TUMORS OR THE BRAIN AND MENINGES 
321 
localizing symptoms of the lesion when in this situation. The well- 
recognized association of these lesions with trauma is possibly a sufficient 
explanation for their frequent occurrence under the exposed vault. 
Syphiloma.-Unlike tubercle, syphilitic brain lesions are largely 
confined to adult life, and are said to occur above the tentorium more 
often than below. In the majority of cases, furthermore, they are surface 
lesions, whereas tubercles are more common in the substance of the brain. 
For the most part, the solitary lesion does not reach a large size, and never 
equals the enormous, fused tuberculoma. Somewhat like an early 
tubercle, however, the gumma consists of a vascular, grayish-red, granu- 
lation tissue mass of round, epithelial, and giant cells. In this tissue 
mass changes soon occur; areas of coagulation necrosis appear, or through 
fibrosis a dense, scar-like tumor may be left-the typical syphiloma. 
This change is particularly common in the meningeal gummata. 
In an advanced stage, cortex, meninges, and even overlying bone may 
become adherent in a dense cicatrix which resists absorption under 
the most vigorous antiluetic measures. Such a granuloma rarely under- 
goes the process of softening characteristic of the tuberculous lesions, 
although, on the other hand, disturbances of the surrounding cerebral 
tissue through accompanying vascular disease are much more common. 
Nevertheless, it is often difficult to distinguish the processes pathologically 
without microscopic demonstration of the agent-bacillus or spirocheete. 
One histological difference lies in the presence throughout the gumma of 
the bands of tissue undergoing fibrous change. We have occasionally 
experienced difficulty in distinguishing between a cortical gumma with 
spreading meningo-encephalitis and a superficially placed glioma which 
had invaded the pia-arachnoid. In all cases the serum reaction should 
be tried. 
Actinomycomata.-These are rare and have usually been secondary 
or metastatic lesions, although Bollinger regarded his cases as primary. 
Howard has made a thorough study of these conditions.1 
Pathological Physiology and Symptoms.-The clinical aspects of a 
brain tumor hinge far more upon matters relating to the physiology of the 
intracranial space than upon its mere structural features. Indeed, in 
view of their extraordinary variability in size, form, consistence, manner 
of invasion, rapidity of growth and situation, not only in so far as the 
latter relates to the various nerve tracts and centres, but also to the blood 
supply and channels of outlet for the cerebrospinal fluid, it is perhaps 
remarkable that any recognizable symptom complex can be accepted 
as in the main characteristic of brain tumors. 
There are, however, in the majority of cases, two processes whose 
phenomena may be regarded as typical of the greater number of intra- 
cranial new-growths. The first embraces the symptoms produced 
within an intact skull by a more or less generalized increase of tension 
which affects the brain chiefly by upsetting its normal vascular and 
cerebrospinal fluid circulations-the so-called underlying or general 
symptoms; and the second, the symptoms produced by irritation or 
1 W. T. Howard, Jour, of Med. Research, 1903, iv, 301. 322 
DISEASES OF THE NERVOUS SYSTEM 
destruction of nerve tracts or centres of known function-the so-called 
localizing or focal symptoms. 
When both of these processes are in full operation-a thing which is 
to be expected eventually in most cases-the presence of the lesion, and 
probably its situation also, is readily recognized. Unfortunately, at 
the stage when the diagnosis may be of greatest value, one or both of 
these elements are wanting, and owing to the fragmentary sketch instead 
of the finished picture, the condition may long fail of recognition even by 
an expert. For example, the general pressure symptoms might naturally 
be expected to bear some relation to the size of the growth, and yet a 
small tumor compressing the aqueduct of Sylvius so as to obstruct the 
ventricular outflow may lead to symptoms out of all proportion to its 
size. On the other hand, a large infiltrating glioma may be present with- 
out eliciting general or focal symptoms; for it may invade the normal 
tissues in such a way as not to increase tension and it may be traversed 
by persisting fibres which can transmit impulses. Again, a small tumor 
too insignificant to produce pressure phenomena may, nevertheless, 
lead to definite focal symptoms, shown, for example, by irritative move- 
ments if it happens to be situated near the precentral gyrus; whereas a 
similar lesion over a silent area need give no indications whatsoever of 
its presence. Thus, the situation may be far more vital than the size. 
It can be easily seen that tumors which happen to arise in a so-called 
silent area of the brain may remain long dormant, and that the first 
symptoms may be those of general pressure when, in the course of time, 
the lesion has considerably augmented in size. Thus patients may 
present themselves, with (1) tumors which give absolutely no recognizable 
evidence of their existence and are found post mortem; (2) tumors which 
present focal symptoms alone with no evidences of a general increase 
in pressure; (3) tumors which give general symptoms alone with no 
focal manifestations, when they happen to occupy a silent area; (4) tumors 
which give typical symptoms of general pressure together with definite 
focal symptoms; and (5) the symptom complex (general and local) of 
tumor, which in the absence of a growth is brought about most often by 
oedema from one source or another. 
With full realization that practically all tumors at their onset fall under 
the first of the groups cited above, and also that many brain tumors may 
exist for years before their symptoms become sufficiently pronounced to 
attract any serious attention, we, nevertheless, may wisely retain as 
characteristic in general of most tumors at some stage of their progress 
the so-called underlying or general pressure symptoms - headache, 
vomiting, and choked disk-to which we would like to add the external 
evidences of intracranial venous stasis, the tendency to herniate through 
the cranial foramina or through operative openings and a few other 
possibly less characteristic signs of tension. 
The General Manifestations.-That the general symptoms are primarily 
the consequence of pressure against the brain within the.closed skull is 
now fairly conclusive, for they may be completely set aside by making 
a purposeful cranial opening, just as nature may occasionally alleviate 
them in the young by a general diastasis of the cranial sutures. TUMORS OF THE BRAIN AND MENINGES 
323 
Dislocation from Pressure and Tendency to Herniate.-Physio- 
logical studies, particularly those of Leonard Hill, have shown that the 
brain itself is practically as incompressible as water, and consequently 
that any considerable encroachment by a new body on the intracranial 
space must greatly increase the cerebral tension through the medium of 
its fluid contents. One of the earliest consequences of this is an engorge- 
ment of the venous circulation, leading not only to the subjective sensation 
of fulness, but to certain objective signs. In its most familiar aspects 
this appears as a dilatation and tortuosity of the retinal vessels-the 
premonitary features of a choked disk. There are, however, other 
external evidences equally characteristic, although less often commented 
upon, which occur; for, as in the case of the retinal veins, the veins of the 
scalp, and particularly the smaller venules of the eyelids, often become a 
tell-tale of the intracranial venous stasis. 
The cerebrospinal fluid is normally under low tension, and if there is 
no obstruction to its escape by usual channels the encroaching lesion at 
an early stage will drive the normally small amount of fluid out of the 
arachnoidal spaces, so that when exposed they will, relatively speaking, 
be found dry. Usually, however, the fluid becomes pent up in certain 
areas-in the interpeduncular arachnoidal space, for example, and hence 
the distension of the optic sheath; or in the ventricular cavities of the 
brain, and hence the obstructive hydrocephalus-a factor of great 
moment in accentuating, sometimes abruptly, the pressure symptoms 
which were previously inconspicuous. The fluid, which backs up in 
the spinal meninges, may be demonstrated under tension by a lumbar 
puncture, although its complete withdrawal from this source is dangerous. 
It may occasionally escape through the nares-cerebrospinal rhinorrhoea. 
Another element, in addition to the mere size of the growth itself, must 
be taken into consideration, namely, the tendency of the brain itself to 
become oedematous-a condition which may lead to a sudden and marked 
increase of intracranial tension. These areas of oedema are often macro- 
scopically evident on section of the brain, and are due either to vascular 
stasis or to local changes in osmotic pressure from chemical alterations 
of the cerebral fluids. They occur most frequently in the neighborhood 
of the growth, although they may be found at a distance, as Collier1 
has shown, and greatly confuse the regional diagnosis. Now these 
pressure disturbances, whether from swelling of the brain itself or from 
the encroachment of an enlarging tumor, affect the tension locally more 
than at a distance, although all portions of the intracranial cavity feel 
them to a greater or less extent. The cerebral chamber is partitioned in a 
measure into three subdivisions, by falx and tentorium, and a tumor in 
one of these compartments increases pressure locally to a greater extent 
than in the others, unless the tension is more or less equalized through 
the medium of cerebrospinal fluid obstruction. As a result of the unequal 
tension, distortion of the partitioning membranes, particularly of the 
falx, is common, with more or less dislocation of the hemispheres. 
The brain, being a semifluid mass capable of certain alterations in 
1 Brain, 1904, xxvii, 490. 324 
DISEASES OF THE NERVOUS SYSTEM 
form, tends to escape-to herniate, in other words-through any opening 
which may preexist or be made in the dense enveloping dura. As Wol- 
bach1 has shown, small, actual hernias of the cerebral substance are found 
over the surface of the brain, corresponding with the arachnoidal tufts- 
particularly along the walls of the sinuses, although by no means limited 
to this situation, for the cerebral surface over the temporal lobes may 
become dotted with these minute protrusions. There is but a single 
normal opening of any size in the dura; namely, at the foramen magnum, 
and here, particularly in case the growth originates below the tentorium, 
a more or less marked foraminal hernia of the medulla and surrounding 
fringe of cerebellum occurs with dislocation of these structures downward. 
Sudden implication of the bulbar circulation from this cause is supposed 
to explain the unexpected fatalities which characterize some cases of cere- 
bellar tumor; and a lumbar puncture with the removal of the supporting 
column of spinal fluid is particularly apt to lead to this accident, due to 
the wedging of the medulla in the foraminal ring. 
This tendency of the brain under pressure to herniate may sometimes 
by natural processes serve to relieve the general pressure symptoms, for 
occasionally a superficially placed tumor, especially one of dural origin, 
may, through pressure absorption, destroy the overlying bone and allow 
tumor and brain to protrude under the scalp. This is apt to occur in 
areas where the bone is thin, as the squamous portion of the temporals. 
Changes may occur in the skull other than diastasis of the sutures and 
local absorption. Deepening of the Pacchionian depressions is often a 
marked feature accompanying the formation of Wolbach's hernias. The 
entire inner surface of the cranium at times becomes thinned and rough- 
ened, so that it has a sand-paper feel, and the stasis in the cranial vessels 
may lead to great diploetic channels. 
A word must be added concerning the respiratory and blood-pressure 
reactions. An intracranial growth, owing possibly to its relatively 
slow advancement, rarely shows the same respiratory and vascular 
reaction that characterizes a sudden or abrupt encroachment on the 
intracranial space, as in apoplexy, traumatic hemorrhage, or oedema. 
The slow pulse, rise in systolic pressure, and rhythmic respiration accom- 
panying these states occur in their typical form in tumor cases only when 
there has been a hemorrhage into the growth or sudden oedema or hydrops 
ventriculorum. Otherwise one rarely sees the symptoms of acute com- 
pression in these long-drawn-out conditions except as a terminal event, 
for the rise in blood pressure and primary respiratory failure are the 
common phenomena antecedent to death. 
Headache.-We are here confronted with a problem very similar to 
that in reference to abdominal pain. The brain itself, like the liver, 
spleen, and intestines, is insensitive, as is also its immediately investing 
membrane, the pia-arachnoid. The cranial cavity, however, is lined with 
and partitioned by an outer sentient membrane-the dura-the afferent 
fibres of which, trigeminal and vagal, are capable of demonstration by 
dissection. The fact that patients after trigeminal neurectomy-an 
1 Jour, of Med. Research, 1908, N. S., xiv, 153. TUMORS OF THE BRAIN AND MENINGES 
325 
operation which produces anesthesia of the dura as well as the face- 
no longer suffer from the sensation of headache on the corresponding 
side, would favor the view that the dura plays a part at least in these 
subjective discomforts; but in a number of instances in which cranial 
operations have been performed upon conscious patients without anes- 
thesia it has been found that the dura is actually insensitive to touch or 
incision and that a sensation of pain is elicited only when traction is 
made upon it. It would seem, therefore, that distortion of falx or 
tentorium and the consequent stretching of the membrane are actually 
the important factors. 
A good illustration of headaches which may be particularly insufferable 
are those which occur in association with tumors or enlargements of the 
pituitary gland which distend its enclosing pocket of dura; and it has 
been found that these discomforts may cease as soon as the capsule of 
the gland has been incised, even though the growth be not removed. 
It is even better known that the headaches of tumor may oftentimes be 
completely relieved by a successful "decompressive" operation, and that 
even when a certain amount of discomfort persists after such a measure 
its severity varies with the degree of tension evident in the protruding 
area of brain. Indeed, many headaches not associated with tumor, 
such as those accompanying traumatic oedema, nephritis, and possibly 
even many cases of supposed migraine, may similarly be due to 
pressure.1 
Headaches are not invariably present, for certain tumors need not be 
associated with an increase of pressure. This is notably true of many of 
the lesions which occur below the tentorium, such as the familiar cerebello- 
pontine tumor, which may give merely local symptoms possibly for 
years with no notable increase of tension until it reaches such a size that 
it obstructs the aqueduct of Sylvius, when a rather abrupt onset of pressure 
symptoms may occur. 
When present, headache may be persistent or occur only at fairly 
definite hours of the day or over certain periods of time, to be followed 
by an interval of partial or total remission. The sensation may be nothing 
more than a feeling of general fulness, or it may be agonizing. Head- 
aches are rarely definitely localized to any particular points except when 
a lesion immediately underlies and possibly has caused thinning of the 
adjacent cranium, when local pain with associated tenderness may be 
present. As a rule,- patients refer them indefinitely to the frontal or 
occipital region or to the vertex more often than to one side or the other. 
Discomforts primarily referred to the occiput are suggestive of a sub- 
1 It must be admitted that many points relating to headache are not entirely 
explained by assigning pressure as the sole cause. Thus, lumbar puncture with the 
withdrawal of cerebrospinal fluid from a healthy individual-a condition that is 
naturally supposed to bring about a lessening of the normal tension-may occasion 
intracranial discomfort, and the definite reflex headaches of gastric or ocular origin 
are difficult to reconcile with this view. Perception of pain is unquestionably a cere- 
bral function, and yet the brain itself (with the possible exception of the basal ganglia), 
so far as we know, is insensitive to the stimuli which give pain at the periphery. Thus 
faradization of the supposed sensory cortex in two conscious patients, although 
giving vague tactual impressions referred to the periphery, provoked no local sensa- 
tion.-Boston Med. and Surg. Jour., 1909, clxi, 71; also Brain, 1909, xxxii, 44. 326 
DISEASES OF THE NERVOUS SYSTEM 
tentorial lesion, although too much faith cannot be placed upon this, for 
a posterior lesion may give frontal headaches, and a frontal lesion dis- 
comfort referred to the occiput. 
Vomiting.-Whether there is a centre in the medulla for this act- 
possibly vagal-can only be conjectured, but the symptom is thought 
to be more common in lesions involving the bulb. It is inconspicuous 
or entirely wanting in two-thirds of the cases, even after they have 
reached such an advanced stage that the evidences of pressure are pro- 
nounced. The typical so-called projectile or cerebral vomiting is common 
only in those cases in which headaches are severe, and is rarely seen until 
a late stage when pressure symptoms are pronounced; it hardly deserves 
the prominence given it as a cardinal symptom. When present it is often 
an early morning occurrence or brought on by some sudden change in 
position. The act may be unassociated with nausea. Unlike the other 
striking symptoms of pressure, it rarely occurs alone, whereas headache, 
choked disk, or vascular disturbances may exist almost as the sole pressure 
manifestation. 
Choked Disk.-This, the most striking objective sign, is at the same 
time the only one of the general pressure symptoms which lends itself to 
experimental reproduction. Many terms have been employed in desig- 
nation of the process and many views advanced to explain the causal 
agency: of the two most important, one has attributed the lesion to 
purely mechanical agencies, the other to toxic or inflammatory ones. 
Hence a terminology has risen which is confusing; and "optic neuritis" 
or "papillitis," introduced by von Leber, as opposed to "choked disk" 
(Allbutt's adaptation of the German Stauungspapille) and "papill- 
oedema," are variously used by different writers to express the whole 
process or to indicate its different stages.1 
Influenced by recent clinical and operative experiences, as well as 
by newer experimental investigations, the pendulum of opinion has 
swung away from the toxic toward the mechanical view. It seems in all 
probability a stasis oedema from the forcing of the cerebrospinal fluid 
into the meningeal sheath which invests the optic nerve. The sheath 
becomes distended, the nerve head oedematous, venous stasis occurs, 
and the ophthalmoscopic picture shows at this early stage tortuosity of 
the retinal veins with injection of the disk and more or less haziness 
of its outlines-the nasal margins usually being the first to become 
obscured.2 Shieck has modified this mechanical theory in so far as to 
point out that the fluid leaves the meningeal sheath and passes into 
the optic nerve by way of the perivascular spaces which accompany the 
central vessels of the nerve. 
1 Excellent summaries in regard to the causation of choked disk have been given 
by Th. Kocher in Nothnagel's Specielie Pathologic und Therapie, 1901, ix, 206, and 
by Alfred Sanger in the Wien. med. Woch., 1904, liv, No. 47, pp. 2201, 2231, 2258. 
Gower's Medical Ophthalmoscopy remains to this day the best monograph in English 
on this and allied subjects. Cf. Cushing and Bordley, Jour. Am. Med. Assn., 1909, 
lii, 353, also Paton and Holmes, Brain, 1911, xxxiii, 389. 
2 This stage is usually miscalled by ophthalmologists an "optic neuritis" if it be 
recognized at all as pathological and sharply distinguished from "choked disk," 
the term reserved for a later stage, when there is a measurable swelling of the papilla. TUMORS OF THE BRAIN AND MENINGES 
327 
Marcus Gunn1 divided this process, for which he suggested the unfor- 
tunate name "tumor papillitis," into the following stages: 
Stage 1. The earliest ophthalmoscopic signs are increased redness of 
the disk, loss of definition in its edges, slight prominence of its surface, 
and narrowing of the physiological pit. 
Stage 2. At a rate which varies much in different cases and which seems 
to bear a decided relation to the degree of intracranial tension, the 
swelling of the papilla increases, the physiological pit disappears, and 
the disk edges become quite obscured; along with these signs there is 
now slight haziness of the surrounding retina and the retinal veins show 
evidence of retarded circulation. 
Stage 3. In an advancing case the next alteration consists in further 
swelling of the papilla, so that it becomes more prominent and occupies a 
larger fundus area, the venous distension becoming very marked; fine folds 
not infrequently appear in the oedematous retina, particularly between 
the disk and macula, and there may be retinal hemorrhages. 
Stage 4. The papilla becomes more opaque and sometimes more 
prominent, the hemorrhages increase in size and number, and there are 
inflammatory exudations on the disk and surrounding retina. At this 
stage vision has become impaired. 
Stage 5. The next change consists in a gradually decreasing vascularity 
of the papilla, parts of its surface becoming paler than normal, while the 
prominence either persists or slowly subsides. At this time also we first 
note a change in the branches of the central artery, in the form of dimin- 
ished breadth-the state of atrophy with inevitable blindness. 
There is every reason for ascribing all these stages to a single process; 
and there are reasons for regarding the process as mechanical. For a 
similar change occurs in the neuroretinal tissue in cases of cerebral 
oedema following trauma, and although this rarely advances beyond 
Gunn's Stage 2, still we may see the process advance even to the stage 
of atrophy in persisting cerebral oedemas such as those which occur in 
nephritis. Then, again, it is a frequent experience to see the choked 
disk of tumor subside after a modern palliative operation, even though 
the tumor be not removed, so that the toxic elements, if any exist, should 
still be at work. 
Choked disk, when present, is doubtless one of the most valuable 
signs of tumor, but it must constantly be borne in mind that it is absent 
in all cases at an early period, in most cases until a late period, and that 
many tumors have a long life. The discussion as to the percentage of 
cases in which choked disk occurs seems therefore to be beside the mark, 
for, as the diagnosis is made earlier, doubtless it will be absent in an 
increasingly greater proportion; and we feel a certain pride in having 
successfully removed a tumor in a number of cases before any evidence 
even of congestion of the disk had set in. Tumors allowed, uninterrupted, 
to run their full course will all probably show a certain stage of choked 
disk before the end. 
The advent of a choked disk may be long delayed and yet may progress 
1 British Medical Journal, 1907, ii, 1126. 328 
DISEASES OF THE NERVOUS SYSTEM 
rapidly when the intracranial conditions lead to its formation. It is not 
uncommon to see the lesion develop while the patient is under obser- 
vation, and hence frequent examinations are requisite in all suspicious 
cases. In one of our patients, coincident with a hemorrhage into a glioma, 
a swelling of four or five diopters occurred within a few hours-the 
condition being comparable to the experimentally induced acute lesion 
in animals.1 These acute oedemas of the neuroretinal tissues may 
subside after pressure has been relieved almost as rapidly as they occur; 
it is only in the case of a long-standing lesion that subsidence of the 
swelling is delayed, owing to the naturally slow absorption of the new- 
formed tissue which fills the physiological cup. 
Choked disk may be of certain localizing value, for it is apt to make 
its appearance, as Horsley emphasizes, earlier on the side of the lesion. 
Later, or when both eyes have become involved, the mere measurable 
height of the swelling is not of equal moment, for the older and more 
advanced process may project from the level of the retina less prominently 
than the more recent one. Attempts have been made likewise to use 
choked disk as an evidence of a cerebral or cerebellar lesion, the view 
being taken that the subtentorial growths in a large percentage of cases 
have a high grade of choked disk; but this, it seems, is merely a question 
of the time at which the diagnosis has been made, for in the majority 
of the cerebellar cases the symptoms, often of long standing, are rarely 
thought to be due to tumor until the advent of the neuroretinal change. 
One particularly important fact, properly accredited to Hughlings 
Jackson, is the retention of visual acuity even in an advanced stage of 
the process. When once vision begins to fail, owing to atrophy of the 
nerve from the contraction of the new tissue, the loss of acuity may take 
place rapidly, usually with a concentric contraction of the visual field. 
Vision, indeed, may be suddenly lost. It is this which speaks urgently 
in favor of the modern palliative operation; for if the process has advanced 
to Gunn's Stage 4 the prognosis for permanent vision after operation 
is bad, and in Stage 5 almost hopeless. 
Choked disk is a sign by no means limited to intracranial tumors, for 
any condition which similarly and in equal degree increases pressure will 
naturally lead to a like neuroretinal oedema. There are certain reasons, 
indeed, for believing that the so-called albuminuric retinitis is merely 
a modified form of the same lesion,2 and the same may be said of the 
neuroretinal change which accompanies cerebral concussion or contusion, 
apoplexy, thrombosis, and a number of other intracranial conditions. It is, 
comparatively speaking, rare in congenital hydrocephalus and meningitis. 
Choked Labyrinth.-This condition-the Stauungslabyrinthe of Stein- 
brugge-thought to bear the same relation to the auditory that choked 
disk bears to the optic nerves, has been much debated. It is a conjectural 
lesion which has not been subjected to conclusive histological demon- 
stration or reproduced experimentally. Although many cases show a 
diminution of auditory acuity, there are no definite clinical means of 
1 Cushing and Bordley, The Johns Hopkins Hospital Bulletin, 1909, xx, 95. 
2 Byrom Bramwell, Clinical Studies, 1907, N. S., v, 1; Bordley and Cushing, Am. 
Jour. Med. Sc., 1908, cxxxvi, 484. TUMORS OF THE BRAIN AND MENINGES 
329 
testing for the lesion-at least nothing equivalent to the ophthalmoscope 
in choked disk. Batten and Collier think that stasis may affect the spinal 
cord and the posterior roots, especially of cervical and lumbar regions. 
Degenerations have been demonstrated in the entrance zone of the roots, 
and the condition is said to occasion pains in the root areas and loss of 
the tendon reflexes. 
Dyschromatopsia (interlacing and inversion of the color fields).-Since 
Charcot, this has been regarded as a condition peculiar to functional 
or hysterical states. The perimetric examinations by Bordley and 
Heuer in some of the early cases of the author's series led us to believe 
that color interlacing was a common and early expression of an increase 
of intracranial tension, even before the appearance of ophthalmoscopic 
changes in the disks. Though this view received some support by a 
number of other observers, perfected methods of perimetry, particularly 
in regard to the character of the color peripheries, have led us to believe 
that our early interpretation was erroneous and that the interlaced 
fields were due to a combination of inattention on the part of the patient 
and faulty technique on the part of the observer. 
Other Pressure and General Symptoms.-Other less important general 
pressure symptoms are vertigo and dizziness, possibly more common with 
subtentorial lesions than with those of the cerebrum and possibly also 
related in some way with the labyrinthine disturbances which have been 
described; drowsiness, with repeated yawning, is not infrequent when 
pressure is considerable and the patient in an apathetic state; convulsions 
are occasionally seen irrespective of those characterizing involvement 
of the precentral gyrus; and certain psychic disturbances with intellectual 
dulness are to be made out in most cases of tumor, even with no direct 
involvement of the frontal lobes. Rapid loss of weight is common, although 
the reverse may occur in tumors primarily or secondarily affecting the 
hypophysis; and disturbances of pulse rate, respiration, body temperature, 
and urinary secretion may also be regarded as general symptoms in cer- 
tain cases. In some rare cases, in the absence of demonstrable vascular 
lesion, a loud systolic bruit may be heard over the cranium. 
The Focal Manifestations.-In evidence of the gaps in our knowledge 
of neurological physiology, we must still refer to large areas of the brain 
as "silent." Fortunately for the regional diagnosis of tumors, these 
areas are becoming more and more constricted. The function not only 
of the cortex, but of deeper parts as well, is becoming sufficiently well 
determined to be of great diagnostic service. Irritation or paralysis, 
whether from simple pressure or from destruction of the various centres 
or paths of known function, gives symptoms which are available for this 
purpose, whether the lesions occur in the motor or sensory spheres, in 
those related to the special senses for taste, smell, vision or hearing, in 
the more highly differentiated area associated with the speech or stere- 
ognostic mechanism, in the psychic sphere itself, or involve the cerebral 
nerves in their peripheral intracranial course.1 However, the matter 
may not be so simple as this for symptoms at a distance; false localizing 
1 For illustrative cases of tumors in the various areas Beevor's Lettsomian Lectures 
may be consulted, Trans, of the Med. Soc. Lond., 1907, xxx, 150 to 233. 330 
DISEASES OE THE NERVOUS SYSTEM 
signs which greatly confuse the primary focal and neighborhood mani- 
festations1 are apt to occur, especially late in the history of tumor. 
These distant symptoms are apt to be especially confusing when the 
growth occupies a fairly silent area, but leads to oedema elsewhere. 
We have been misled by false signs in a number of instances, some illus- 
trations of which may be given. Thus in one case a tumor of one frontal 
lobe so greatly indented the other as to make the latter appear to be the 
primary seat of trouble; a tumor of one occipital lobe in a child blind 
from optic atrophy, so that hemianopsia was absent, gave definite 
cerebellar symptoms; medullary symptoms have been far from infrequent 
in cases when there is a large intracranial growth situated almost any- 
where; hydrocephalus has often effectually masked practically all local 
symptoms. A growth may become so large in a young child as to simu- 
late essential hydrocephalus; a basilar process, particularly when luetic, 
may lead to vascular thrombosis with distant symptoms; nerves situated 
far from the lesion may be stretched and paralyzed. This is particularly 
common with the sixth cerebral nerve, which often becomes constricted 
in cases of cerebellar tumors through pressure of one of the lateral branches 
of the basilar artery, thereby causing diplopia. 
It was made apparent in discussing the general pressure manifesta- 
tions that one or another of these phenomena may possess elements of 
localizing value. Thus, persistent suboccipital headache and vertigo are 
characteristic of a cerebellar lesion; choked disk is apt to occur first upon 
the side of involvement in lesions of the cerebral hemispheres;2 hemi- 
achromatopsia, wdiich may foretell a total hemianopsia, likewise may point 
to the hemisphere involved; and any unusual disturbances of pulse and 
respiration, to a lesion in the neighborhood of the medulla. These, 
however, are not commonly classified under focal symptoms, which 
concern more specifically the disturbed function of definite lobes, centres, 
or paths which can be conveniently taken up seriatim under the heading: 
Regional Diagnosis.-This can be aided by ways other than the 
mere study of neurological phenomena. Thus auscultatory percussion 
of the shaved scalp may occasionally be helpful, the audibility of the 
transmitted sound of the tuning fork being lessened over the tumor, par- 
ticularly if it be a solid growth or one placed near the skull. The Itbntgen 
rays may prove of value in locating growths which have undergone 
calcification or have eroded or displaced the cranial bones-particularly 
true of tumors which deform the sella turcica-or in disclosing teratoid 
tumors which contain bone themselves or in demonstrating an internal 
hydrocephalus by the presence of pressure digitations on the bone and 
of separated sutures. The Neisser-Pollack method of puncture through 
the intact skull (IIirnpunktiori) is not to be commended. 
Lumbar puncture as a diagnostic measure has been much abused. 
On the sudden withdrawal of the supporting column of spinal fluid the 
1 Collier, Brain, 1904, xxvii, 490. 
2 To quote from Marcus Gunn: "Double optic neuritis with surrounding retinal 
change coming on quickly suggests the cerebellum; a one-sided neuritis or marked 
difference suggests the cerebrum, and on the whole is in favor of the tumor being 
on the same side as the excess of neuritis." This also is Horsley's view. Leslie Paton's 
studies of the same series of cases disagree with these views. (Brain, 1909, xxxii, 65.) TUMORS OF THE BRAIN AND MENINGES 
331 
pressure from above, which continues unabated, serves to wedge the 
medulla and surrounding fringe of cerebellum into the cone of the fora- 
men, with resultant anemia of the vital centres and sudden respiratory 
interruption. Three of the fatalities in the unoperated cases in the author's 
series promptly followed this measure, and the literature records many 
other similar experiences. The procedure in tumor cases is therefore 
hazardous and of no particular diagnostic value; for mere measured 
tension of the spinal fluid is of no significance, nor is a cytological study 
of the fluid or a Wassermann test of great moment in cases in which 
a high grade of increased intercranial tension is evident. 
Frontal Lobe.-Lesions in this situation lead to mental disturbances, 
which in a way are comparable to the symptoms one meets in paresis- 
indifference, unpunctuality, mental enfeeblement, loss of memory and 
power of attention, change in disposition with more or less marked irri- 
tability or taciturnity or obstinacy or jocularity, etc., often a rambling 
speech, lack of realization of the illness, and change in the general conduct 
of life with habits of untidiness. These, in greater or less degree, charac- 
terize most of the cases, although it is often astonishing to find how incon- 
spicuous the symptoms may be with a very extensive growth. They 
may often be of rather abrupt onset and not until the situation of the 
lesion is definitely disclosed and careful interrogation made into the pa- 
tient's previous mental state is it possible to learn that in all probability 
some mental alteration has been of long standing. 
There is undoubtedly a great difference between the two sides, the 
symptoms being more pronounced in left frontal lesions in the right- 
handed. It is to be noted that a tumor of one side may markedly affect, 
by indentation or compression, the opposite hemisphere. The symptoms 
also depend somewhat on the situation of the growth, whether it affects 
the prefrontal region, in which case they are said to be more pronounced, 
or whether it lies at the base or upon the external surface of the frontal 
lobe. Disturbances of the speech mechanism and of the motor activites 
of the contralateral side of the body may be expected when the posterior 
part of the lobe is affected. Vocal aphasia is common, and a good instance 
of the rarer agraphia has been recorded by McConnell.1 There may be 
cortical epilepsy with movements of head and eyes to the opposite side; 
automatic movements are also described, and a form of frontal ataxia. 
Grainger Stewart2 has shown that a homolateral tremor with loss of 
abdominal reflex on the contralateral side of the body is of frequent 
occurrence. Lesions here are also accompanied by relatively early 
nutritional disturbances. 
Temporal Lobe.-With the exception of the uncinate area this con- 
stitutes a relatively silent region. Large tumors may give such vague 
symptoms that the first local sign of trouble may be the tenderness and 
bulging which fortells perforation and atrophy of the squamous wing. 
It is not uncommon in the usual subtemporal decompressive operation 
to expose an unsuspected temporal growth; this has occurred many 
times in our series. Left temporal lobe tumors in right-handed people 
1 Univ. of Penn. Med. Bull., 1905, xviii, 156. 
2 Rev. of Neurol, and Psych., 1906, iv, 809. 332 
DISEASES OF THE NERVOUS SYSTEM 
may lead to disturbances of motor speech when they encroach on the 
frontal area, and hemilingual or facial weakness may occur should the 
growth implicate the lower precentral gyrus. It is unusual for them to 
cause auditory disturbances, although inability to apprehend spoken 
language is supposed to follow a lesion of the transverse temporal gyrus 
of Hesche, with more or less upsetting of the entire speech mechanism.1 
Cortical lesions at the tip of the temporal lobe which implicate the unci- 
nate and possibly the hippocampal gyri are known, largely through the 
papers of Hughlings Jackson, to occasion certain peculiar seizures known 
as the uncinate group of fits. Purves Stewart, in 1899, was able to find 
reports of six typical cases, and Mills2 collected as many more. The 
condition, must be of fairly common occurrence, for we have had many 
examples in our series and Kennedy3 has reported a number of others. 
A tumor which occasions these symptoms may either arise primarily 
in the temporal lobe or may originate elsewhere, as in the interpedun- 
cular space, and involve the uncinate gyrus secondarily. The uncinate 
seizures are characterized by subjective sensations of smell or taste- 
the impression usually being a disagreeable one-and often by an epigas- 
tric aura. Together with these sensations, movements often occur such 
as those of the acts of tasting or smelling, chewing or swallowing. Saliva- 
tion is not unusual. Consciousness is retained, as a rule, in these attacks, 
and they are not infrequently replaced by or associated with so-called 
"dreamy states," described as a distressing sense of unreality of surround- 
ings. Respiratory phenomena have been noted. Neighborhood symp- 
toms occur. In one of our patients there were secondary hypophyseal 
disturbances; in another, a fluctuating contralateral hemianesthesia and 
homonymous hemianopsia due to pressure against the adjoining crux 
and visual pathway were present-a combination of symptoms which 
had led to a diagnosis of hysteria, for the tumor proved to be a benign 
one and had been present for many years. We have found in temporal 
lobe lesions that disturbances of the visual fields are common, some- 
times as an upper quadrantal homonymous defect. Hemianopic visual 
hallucinations may occur.4 
Paracentral Convolutions.-For evident reasons by far the greater num- 
ber of tumors which have been localized in the past have involved the 
central motor and sensory gyri. A small growth which implicates the 
surface of this part of the brain almost invariably leads to irritative 
symptoms, beginning either with a primary motor or sensory aura in 
leg, body, arm, neck, face, or tongue, as the case may be. These focal or 
Jacksonian seizures may for a long time be the only manifestations; 
they may remain limited to the part involved or may spread with a 
typical "march," so as to involve the entire body in convulsive move- 
1 The rarity with which word-deafness occurs in cases of involvement of the superior 
temporal gyrus by tumor or by the protrusion of the lobe through a palliative defect 
makes the cortical representation of this primary word centre a matter which would 
seem to deserve as careful consideration and possible revision as has been given to 
Broca's vocal speech centre since Marie's recent criticism; cf. Adolf Meyer, Jour, of 
Psychol, and Neurol., 1908, xiii, 203. 
i Jour. Am. Med. Assn., 1908, li, 879. 3 Arch. Int. Med., 1910, viii, 317-350. 
4 A. Pick, Am. Jour. Med. Sc., January, 1904. TUMORS OF THE BRAIN AND MENINGES 
333 
ments with loss of consciousness. As the lesion enlarges it is charac- 
terized by irritative disturbances to be followed by paralytic ones, shown 
first in the musculature of the part in which the attacks originally began. 
The more superficial the lesion the more circumscribed may be these 
paralyses; the deeper the lesion and the nearer the internal capsule the 
more extensive they are. 
It must be borne in mind that the cortex of the central fissure dips 
down to a depth possibly of an inch; also that, as Sherrington and Griin- 
baum's observations have shown, the motor strip extends to the depth 
of the fissure, so that a lesion truly cortical may nevertheless lie at a con- 
siderable depth below the surface of the hemisphere. Sensory anesthesia 
is much less likely to follow primary sensory fits than in motor palsy to 
follow either sensory or motor seizures; for although the cortical terminals 
for common sensation have with some definiteness been placed in the gyrus 
centralis posterior, still there are several relays between periphery and 
central end-stations, and a fairly extensive cortical or subcortical lesion 
is necessary before definite areas of anesthesia become demonstrable. 
The most characteristic symptom of tumor in this situation-if one 
wishes to delay intervention until it occurs-is the gradual transference 
of a local convulsive movement into a local paralysis of movement. The 
paralytic features occur first as a mere exhaustion after the attack in the 
affected muscular territory and then as an actual paralysis coincident 
with destruction of the centre. After this, convulsions no longer occur 
in these muscles, but in those whose centres are adjoining. The first 
convulsion is usually local, rarely a general one, although soon local and 
general convulsions become interchangeable. Not infrequently between 
the attacks continuous twitching-"epilepsia continua"-may occur. 
It is the usual rule for all objective sensory disturbances to be wanting 
in the paralyzed area, although it is not rare to find certain disturbances 
of tactual sense or of the sense of localization. 
The Parietal Lobe.-A lesion of the superior lobule, particularly if sub- 
cortical, leads to a contralateral disturbance of the stereognostic sense.1 
If the subcortical lesion be large and encroach on the postcentral gyrus, 
it may occasion additional sensory disturbances, such as loss of muscle 
sense, of position in space, or even of some forms of common sensation. 
Consequent upon the sensory disturbances certain irregularities of moment 
appear (the motor apraxia of Leipmann). The arm, for example, although 
not in a strict sense paralyzed, for the patient when observing the member 
can make powerful though somewhat awkward movements, yet cannot 
be used for any purposeful act, particularly if the patient be blindfolded. 
More or less muscular rigidity accompanies the condition, although it is 
unassociated with any direct implication of the pyramidal tract. 
One characteristic symptom elicited by a tumor involving the left 
angular gyrus in the right-handed is the inability to appreciate written 
language or even to read letters-word-blindness (alexia). A deep- 
1 Although much discussed, astereognosis seems to be a fairly definite and reliable 
symptom of lesions of the superior parietal lobule. A most typical instance in a case 
of stab wound-not tumor-has been recorded by the writer in the New York Med. 
Jour., 1907, Ixxxv, 161. 334 
DISEASES OE THE NERVOUS SYSTEM 
seated lesion of either lobule may involve the fibres of the optic radiation 
and lead to a half-blindness of the corresponding halves of both retinas, 
either for colors alone or for form also-homonymous hemiachromatopsia 
or hemianopsia. 
The right parietal lobe represents a comparatively silent area, formerly 
regarded by Sanger as the most favorable for decompressive operations. 
The Occipital Lobe.-A crossed homonymous hemianopsia is the char- 
acteristic, and may persist for a long time as the only, symptom in addi- 
tion to those incidental to general pressure. If optic atrophy secondary 
to a choked disk has occurred, and there is no history of half-blindness 
before the loss of vision, a regional diagnosis may be impossible. Occa- 
sionally the fields for color may be affected before1 those for form suffer 
any restriction, and cases have been recorded in which quadrantal blind- 
ness has been present, although these fractional defects in the field are 
possibly more characteristic of implication of the visual pathway farther 
forward as it traverses the temporal lobe. 
Homonymous hemianopsia, of course, is characteristic of a unilateral 
lesion of the visual pathway in any part of its course, from occipital lobe 
to chiasm, and neighborhood symptoms must be depended upon to 
determine the seat of the lesion. When the occipital cortex, particularly 
the more important region of the calcarine fissure, is involved, the loss of 
half-vision may be ushered in by certain visual hallucinations, such as 
scintillating scotoma. Involvement of the optic radiation itself on the left 
side may be accompanied by some of the parietal lobe symptoms just 
enumerated-alexia or optic aphasia. Lesions farther forward, which 
include the fibres from the oculomotor nuclei on one side and affect the 
arc of the pupillary light reflex (Plate V), lead to the hemianopic loss 
of this reflex on the blind side (Wernicke)-a condition which is wanting 
in pure occipital lesions. Occipital lobe tumors which have reached 
a great size may produce pontine disturbances or even symptoms indi- 
cating some implication of the corresponding cerebellar hemisphere. 
Centrum Semiovale and Basal Ganglia.-Needless to say, lesions in any 
of the situations heretofore considered involve to a greater or less extent 
the subcortical areas, and it is often difficult to determine from the symp- 
tomatology alone whether a tumor is entirely central or whether it actually 
reaches the cortex. This is the more so since the cortical areas repre- 
sented by the insula and by the deeper fissures dip down well into the 
central parts of the hemispheres, and some cortical manifestations may 
be present in many of these lesions, even though they lie far beneath the 
actual exposed surface of the brain. It is chiefly by the relatively large 
extent of the contralateral paralyses that one is enabled to conjecture 
the approximate depth of the lesion. Particularly in the left half of the 
brain the deeper lesions of the centrum lead to very extensive disturbances 
not only in movement and sensation but in psychic activity, in the speech 
mechanism, and in vision. 
Tumors of the basal ganglia, particularly those of the thalamus, are 
often capable of localization, the so-called thalamic syndrome having 
received much attention of late. The most characteristic symptom of a 
thalamic lesion is a contralateral disturbance of movement, either in the PLATE V 
Diagram to Show various Forms of Visual Disturbance following 
Lesions in Different Portions of the Left Visual Pathway. TUMORS OF THE BRAIN AND MENINGES 
335 
nature of a coarse tremor or of choreic or athetoid movements, often 
accompanied by painful subjective paresthesias. Indeed, this is the only 
area of the brain a lesion of which seems to produce subjective sensations 
of discomfort referred to the periphery. Roussy1 outlined this syndrome 
as follows: The patients present (a) persistent hemianesthesia, more or 
less marked in modalities of touch, pain, and temperature, but always 
pronounced in regard to deep sensation; (&) slight hemiplegia of a tran- 
sient nature without contractures; (c) slight hemiataxia and more or less 
complete astereognosis; (d) persistent paroxysmal pains on the affected 
side, often intolerable and unrelieved by any analgesic remedy (Aem- 
plegie douloureux); and (e) athetochoreic movements on the affected side. 
In addition to these, the most characteristic phenomena, neighborhood 
symptoms occur, such as contralateral hemiplegia; oculomotor palsies, 
such as crossed pupillary dilatation and ptosis; emotional palsy of the 
face; hemianopsia; deafness from involvement of the lateral geniculate 
bodies, and so on. 
In the hemiplegias which are due to lesions of the cortex or subcortex 
exaggeration of the deep reflexes and a persistent Babinski sign, together 
with contractions and epilepsy, are to be expected, with an absence of the 
athetochoreic movements and subjective painful sensations. 
Corpus Callosum.-Bruns found only 26 recorded cases of tumor pri- 
marily involving this structure, and it is unusual for them to give 
symptoms sufficiently definite to insure a local diagnosis, although this 
has been correctly made in a few instances.2 In addition to the usual 
pressure symptoms, these lesions are accompanied by a slowly progressive, 
bilateral, though not necessarily symmetrical, palsy of both extremities, 
without paralysis of the cerebral nerves, the latter being regarded by 
Raymond and others as an important negative symptom. Particularly 
when the anterior portion of the corpus callosum is involved, intellectual 
disturbances are marked,3 often with apraxia and alterations of the speech 
mechanism. There is apt to be a profound stupor, and the palsy of the 
extremities affects movement of the legs more than of the arms. Sensory 
disturbances are absent and the reflexes normal. The sphincters are 
uncontrolled. Tumors which involve the median portion of the corpus 
lead to more marked disturbances of motion, and a form of callosal 
ataxia has been described. Tumors which involve the splenium are apt 
to implicate adjoining structures with neighborhood symptoms. 
The Brain Stem (Crura, Corpora Quadrigemina, Pineal Gland, Tegmentum 
and Pons.)-It is only natural that distant and neighborhood symptoms, 
as well as complex local ones, occur with lesions in this situation-the 
"cross-roads" of the nervous system. Thus pressure upon or involvement 
of the internal capsule or cerebral peduncles produces a more or less 
marked contralateral hemiplegia, possibly with some degree of hemi- 
anesthesia; and if the growth extends outward from the mid-brain hemi- 
1 La couche optique (etude anatomique, physiologique et clinique); le syndrome 
thalamique. Steinheil, Paris, 1907, p. 371. • 
2 Putnam and Williams, Jour, of Nerv. and Ment. Dis., 1901, xxviii, 645. 
3 More than with tumors of the frontal lobes themselves, according to Knapp, 
Brain, 1906, xxix, 35. 336 
DISEASES OF THE NERVOUS SYSTEM 
anopsia may occur, and likewise the muscular sense and stereognosis may 
be affected. The deep reflexes may thus be increased with absence of the 
superficial ones-this being particularly true of the Babinski phenomenon. 
Any growth in this situation will in the course of time lead to an internal 
hydrocephalus, which greatly complicates the symptom complex, in 
consequence of the widely distributed pressure against the hemispheres. 
Several examples of tumors, often a solitary tubercle, of the crura 
have been recorded1 the characteristic symptoms being a contralateral 
hemiplegia, with homolateral or bilateral oculomotor palsies. 
Involvement of the corpora quadrigemina leads to a staggering gait with 
unsteadiness and deviation sometimes to one side or the other in progres- 
sion, and an especial tendency to fall directly backward; also to a failure 
of sight and hearing when, as the growth spreads to the side, the lateral 
geniculate bodies become invaded or compressed; to nystagmus; to per- 
sisting palsies of the ocular movements, particularly those in a vertical 
direction,2 with preservation of lateral movements; and possibly to loss 
of the pupillary reflex to light and even to convergence as the centres for 
the oculomotor nerves become involved. The pupils may be irregular; 
and Bielschowsky describes a form of nystagmus with clonic twitching. 
Thus a combination of ocular paralyses and ataxia (Nothnagel) especially 
characterizes lesions in this situation, whether they arise in the pineal 
gland or corpora quadrigemina. 
Pineal tumors occur chiefly in the young and are characterized by 
internal secretory disturbances which manifest themselves by a pre- 
cocious adolescence, the secondary characters of sex often being 
acquired at an early age-at two years in one of the author's cases. 
At the same time adiposity and skeletal overgrowth often occur. In 
addition the usual symptoms of a tumor of the neighborhood appear, 
oculomotor palsies, deafness, ataxia and so on, together with an internal 
hydrocephalus from occlusion of the iter. 
When the tegmentum is implicated, particularly the nucleus ruber and 
the superior cerebellar peduncle leading to it (the cerebello-rubral system), 
a particular syndrome is apt to be called out in addition to the above, 
characterized by a peculiar coarse, oscillatory tremor, described by 
Gordon Holmes.3 If the red nucleus of one side is involved the tremor 
is contralateral. We have had one or two striking examples of the 
condition. In addition to this tremor, which Holmes likens to that of 
paralysis agitans, a mask-like appearance of the face occurs; and the 
oculomotor palsies above mentioned, with staggering gait, etc., commonly 
occur. The disorders of movement with posterior thalamic lesions are 
more apt to be of an athetoid character than in tegmental lesions. 
The pons is not an uncommon seat, particularly of tubercles and 
gliomata. The latter may continue to be traversed by intact fibres, 
1 J. H. W. Rhein, Jour. Am. Med. Assn., 1914, Ixiii, 1662. 
2 "Persisting paralysis of associated lateral movement indicates a lesion of the 
posterior longitudinal bundle; of associated vertical movement, a lesion in the vicinity 
of the oculomotor nucleus; paralysis of associated movement does not result from 
lesions of extracerebral fibers." Spiller, Paralysis of Associated Movements of the 
Eyeballs (Blicklahmung), Jour, of Nerv. and Ment. Dis., xxxii, p. 417. 
3 Brain, 1904, xxvii, 327. TUMORS OF THE BRAIN AND MENINGES 
337 
so that they may reach an astonishing size without producing such 
symptoms as would be expected of a lesion here. The characteristic 
feature, however, of most pontine tumors is the well-known alternating 
or crossed hemiplegia, with nuclear cerebral palsies on the side of the lesion 
due to direct implication of the centres, and a contralateral palsy of the 
extremities with the usual evidences of pyramidal tract degeneration. 
Various forms are recognized, possibly the most common combining a 
homolateral facial palsy with a crossed involvement of tongue and extremi- 
ties. The facial territory may be the first to be affected, the brow often 
escaping; or, on the other hand, the palsy of the tongue and extremities 
may be primary, depending on the seat of the lesion. In another type 
the trigeminus may be affected on the side of the lesion with anesthesia 
of one side of the face, and there may be a coincident crossed anesthesia, 
pure or dissociated, affecting the paralyzed limbs. Another form may 
show itself, possibly in the absence of facial or hypoglossal disturbances, 
as an abducens palsy, crossed with a motor involvement of the limbs; 
and in these cases there is a characteristic paralysis of conjoint ocular 
movement toward the side of the lesion. Abducens palsies must be 
scrutinized carefully, however, as they are the commonest of all cerebral 
nerve lesions, and may, from pressure or from stretching through disloca- 
tion, accompany a tumor in almost any situation. 
When a pontine growth involves the main conducting paths or the 
nuclei on each side, these typical crossed paralyses will be wanting, and 
some evidence of bilaterality of the lesion may in many cases be expected 
before the end. We consequently may have a multiplicity of com- 
binations with unilateral or bilateral disturbances of facial, trigeminus or 
abducens, and it is even possible to have a coincident cerebral paralysis 
of all four extremities and both hypoglossi. The variations are too many 
to enumerate, although Bruns brings them into four main categories: (1) 
Bilateral paralysis of the cerebral nerves and extremities; (2) bilateral 
cerebral nerve with unilateral paralysis of the extremities; (3) unilateral 
cerebral nerve and bilateral paralysis of extremities; (4) bilateral cerebral 
nerve paralysis without involvement of the extremities. 
Neighborhood symptoms are shown, if the growth presses forward, by 
oculomotor symptoms; if backward, by affections of the medullar 
nuclei, evidenced by disturbances of deglutition or phonation-the 
bulbar palsies. Salivation, albuminuria, polyuria, and extreme degrees 
of pyrexia have been described in association with pontine tumors. In 
pontine lesions a choked disk is notably late in its appearance or possibly 
may seem to be so owing to the fact that the diagnosis of a lesion in this 
situation is apt to be made early. 
The Medulla Oblongata.-The complexity of paths and centres crowded 
here into a small space naturally makes the symptom complex most 
variable; nor are the lesions sufficiently common to enable us to more than 
sketch their clinical picture. The centres for the cerebral nerves, from the 
eighth to the twelfth, may be affected so that deafness or Meniere's 
syndrome, palsies of the palate and pharynx with dysphagia, vocal cord 
palsies, disturbances of cardiac or respiratory activity, dysarthria from 
weakness or paralysis of the tongue, and trigeminal anesthesias (ascending 338 
DISEASES OF THE NERVOUS SYSTEM 
root) may occur in combination with motor and sensory, crossed or 
uncrossed disturbances of the extremities. The medulla may be affected 
as a distant symptom of most intracranial tumors, owing to the crowding 
of the medulla into the foramen. 
Tumors of the fourth ventricle occasionally give symptoms sufficiently 
characteristic to justify a focal diagnosis. In the greater number of cases 
observed the lesion has been due to a cysticercus, less often to a glioma, 
this being the usual seat of the ependymal glioma already described. 
Stern1 collected 72 cases of the former and about 50 cases of tumor. The 
symptoms are suboccipital pain, a peculiar bowed position of the head, 
a periodic occurrence of general pressure symptoms, often of abrupt 
onset, accompanied by severe headache, vomiting, and cervical rigidity. 
In some patients a changed position of the head may cause a sudden 
accession of symptoms (Bruns); this is supposed to indicate an unattached 
and movable cysticercus. An interval of complete freedom from symp- 
toms may follow. Sudden death is apt to occur during some one of the 
periods, with a slowed pulse and respiratory failure. Choked disk is rare. 
Polyuria, polydipsia, or glycosuria may be present. The fluctuating 
intensity of the symptoms is presumably due to a varying degree of 
obstructive hydrocephalus. 
Cerebellum.-Inasmuch as the symptoms which they elicit bear a close 
relationship, all tumors situated beneath the tentorium which affect one 
or the other cerebellar hemispheres or the vermiform process, either 
through pressure or by direct involvement, will be considered together. 
According to the classification of Stewart and Holmes, in their paper2 
based on a series of 40 cases observed at the National Hospital, of which 
number the diagnosis was confirmed in 22, these growths are considered 
under the headings (1) of extracerebellar, and (2) of intracerebellar tumors. 
Tumors here, as in other parts of the brain, lead first or last to the 
cardinal symptoms of pressure-headache, vomiting, and choked disk 
-and very frequently to vertigo or dizziness. The headache is apt to 
be suboccipital, often on the side of the lesion, and not infrequently is 
associated with tendernesss on pressure. The choked disk is supposed 
to be found in a much higher percentage than is the case with growths 
elsewhere, probably due to the fact that in the course of time a cerebellar 
tumor will almost certainly lead to an obstructive hydrocephalus, in 
consequence of which a neuroretinal oedema is inevitable and may occur 
abruptly, often with a rapid augmentation of general pressure symptoms. 
In the absence of this complication, and with a slowly advancing benign 
lesion, choked disk may be absent for years. Vertigo is more frequently 
associated with subtentorial than with supratentorial tumors. When 
marked, the patient experiences a sensation of the movement of self or 
objects. According to Stewart and Holmes, in both extracerebellar and 
intracerebellar tumors the patient often has the impression that objects 
are moving in a direction away from the side of the lesion: whereas he 
feels himself revolving, in the case of an intracerebellar tumor, in the same 
1 Deut. Zeil. f. klin. Med., 1907, Ixi, 64; Deut. Zeit. f. Nervenheilk., 1908, xxxiv, 195. 
2 Brain, 1904, xxvii, 522. TUMORS OF THE BRAIN AND MENINGES 
339 
direction with the objects; in the case of an extracerebellar tumor he 
appears to be turning in the opposite direction. 
A tumor arising in the substance of the cerebellum (intracerebellar) 
leads to symptoms which affect the musculature on the side of the body 
homolateral to the lesion, and occasionally to the peculiar convulsive 
seizures described by Hughlings Jackson and known as "cerebellar fits." 
A tumor arising from the meninges and compressing the cerebellum 
secondarily (extracerebellar), of which the most characteristic type is 
the meningeal endothelioma originating in the lateral cerebellopontine 
recess, leads to more or less pressure disturbance of the cerebral nerves 
on the side of the lesion, in addition to the symptoms of an intracere- 
bellar growth. 
A large number of these extracerebellar tumors are supposed to grow 
from the flocculus or from the sheath of the auditory nerve, and as they 
enlarge they destroy and press to the side the pons and markedly indent 
the anterior and lateral portion of the adjoining cerebellar lobe. Conse- 
quently the acousticus is the nerve most commonly affected, and either 
subjective noises or complete nerve deafness may occur. The z-ray 
may show an enlargement of the internal auditory meatus. In view of its 
close proximity to the eighth nerve the facial may likewise be affected 
with weakness of its musculature on the side of the lesion. Even in the 
presence of a large tumor, however, it may be found stretched out to 
double its normal intracranial length, with little more evidences of 
weakness than is shown by a less complete closure of the eye in winking. 
A particular triad of symptoms has been pointed out by Oppenheim, 
namely, disturbance of hearing, paralysis of winking, and diminished 
corneal reflex. The trigeminus is occasionally affected, more especially 
its sensory portion, with pain or tingling or dysesthesia over its particular 
skin field. The abducens may likewise suffer and more rarely the glosso- 
pharyngeal, vagus, or hypoglossus. In intracerebellar tumors it is, 
relatively speaking, unusual for these nerves to be affected. 
Disturbances of the body musculature are common in both intra- 
cerebellar and extracerebellar forms. Tremor, loss of muscular tone, 
even paresis of the limb and trunk muscles, weakness of the conjugate 
movements of the eyes, nystagmus, and staggering gait with tendency 
to fall, oftentimes toward the side of the lesion, and alteration of the deep 
reflexes, all occur. These symptoms are most pronounced on the side 
of the lesion. The ataxia, which may be very marked, is a coarse move- 
ment accentuated when the patient attempts to perform some delicate 
act; unlike the ataxia of tabes it does not increase when the eyes are 
closed-an evidence of its central origin. There is an associated con- 
dition, which Babinski termed adiadokokinesis,1 in which the patient 
cannot repeat rapidly executed movements, such as pronation and sup- 
ination of the forearm, owing to the defective coordination. Loss of tone 
in the muscles (asynergia) may be present, so that the arm and the leg 
may be completely flaccid with retention of the deep reflexes. 
The ocular movements are also affected in ways characteristic of 
1 Rev. neurologique, 1902, x, 1013. 340 
DISEASES OF THE NE EV OUS SYSTEM 
cerebellar disease. There is apt to be difficulty in forcing and retaining 
conjugate movements of the eyes toward the side of the lesion, and the 
attempt usually elicits nystagmoid movements. It is not infrequent 
for nystagmus to be present on looking toward as well as away from the 
presumed site of the lesion, but as Stewart and Holmes pointed out, 
the jerking movements are apt to be more rapid on one side than the other, 
the slower rhythm being usually upon the side of the lesion. Nystagmus 
may nevertheless be wanting. It was absent in 11 out of 44 consecutive 
certified tumor cases in our series. 
The position of the head is apt to be toward one side, usually facing 
away from the lesion, and tilted back so as to relieve the strain on the 
cervical muscles. Alfred Gordon asserts that forcible movement of the 
head toward the lesion increases the headache and vertigo in a char- 
acteristic way. Irregular tremors are often present in the extremities. 
They are accentuated with the patient erect, his arms and hands held 
horizontally extended and in a forced position. A staggering gait is 
common, with a tendency to deviate, totter, or actually fall toward one 
side-more often toward the side of the lesion. A method of determining 
the affected side is to have the patient stand on one leg or the other, the 
chief disability appearing on the side of the lesion. Unsteadiness is 
equally characteristic of a tumor involving the middle lobe or vermis and 
one involving the hemispheres, although with the former there is a greater 
tendency to fall directly backward or forward. 
The reflexes are apt to be affected, disparity between the two sides 
being of chief importance, for there is considerable difference of opinion 
as to whether they are increased or are diminished on the side of the 
lesion. It is not uncommon for them to be diminished at one time, and 
at a later period increased, depending in all probability upon the degree 
of advancement of the process. The superficial reflexes are not greatly 
altered, although occasionally, in association with an increase of the 
deep reflexes, an extensor toe phenomenon may occur. 
Though the localizing diagnosis of a tumor in the cerebellum is possibly 
the easiest of all, nevertheless there are cases, even when a large tumor is 
present, in which few if any of the characteristic signs and symptoms can 
be elicited. 
The Cranial Base.-A variety of tumors, either of meningeal or cranial 
origin, may arise from one or another of the cranial fossae and produce 
definite focal symptoms, owing chiefly to the involvement of the cerebral 
nerves, in addition to the evidences of general pressure. Syphilitic pro- 
cesses are common here. Bony or cartilaginous tumors originating in the 
accessory nasal sinuses may invade the cranial chamber. Epithelial 
tumors may grow inward from the deeper ear; such a case occurred in 
our series, with the unilateral involvement of all the cerebral nerves 
from the sixth to the twelfth inclusive. The cavum Meckelii is not an 
uncommon seat for tumors which may give all the pain of a severe 
trigeminal neuralgia during the process of stretching or destruction of the 
Gasserian ganglion; and keratitis is a frequent accompaniment of the 
process. But of all the basal and extracranial lesions, those of chief 
interest and moment arise in the neighborhood of and implicate the- TUMORS OF THE BRAIN AND MENINGES 
341 
Hypophysis Cerebri.-This is a favorite seat of tumors, both congenital 
and acquired. Two separate factors must be considered with tumors in 
the interpeduncular region, commonly grouped as hypophyseal tumors. 
First, what is their effect, if any, on the gland itself; and second, what 
are the chief neighborhood symptoms? The former has heretofore 
received scant attention, but the neighborhood symptoms are well 
recognized and have the following characteristics: Any mesially placed 
tumor in the interpeduncular space naturally presses upon the optic 
tracts or chiasm, leading in the majority of cases to partial amblyopia 
and to primary optic atrophy. This in most striking form occurs as a 
bitemporal hemianopsia, although by no means is this the only form of 
perimetric defect, for an homonymous hemianopsia may occur, or one 
nerve may suffer much more than the other; so that, for example, total 
blindness in one eye may be associated with normal vision or some stage 
of hemianopsia in the other. 
Choked disk is usually absent, as there commonly is no great increase 
in intracranial tension, although it may occur later should the growth 
enlarge so as to push its way into the third ventricle and obstruct 
the foramina of Munro. Headache is often a common and distressing 
symptom, due rather to the distension of the dural pocket of the gland 
than to any general increase of the tension. The arrays are particularly 
helpful in the diagnosis of these cases, for in many of them there is a 
characteristic deformation of the sella turcica; or the growth itself, 
if a teratoma, may cast a shadow. 
The glandular symptoms are quite another matter. Since Marie's 
discovery of the frequent association of hypophyseal enlargements with 
acromegaly there have been innumerable reports of cases in which a 
tumor of the gland or its neighborhood has been unaccompanied by 
evidence of "Marie's disease." These findings have usually been inter- 
preted as contradictory of his views. An especial group of these cases, 
as shown by Frohlich,1 is characterized by a peculiar tendency to the 
deposition of fat with sexual infantilism, hypotrichosis, etc. 
The hypophysis has been proved by Paulesco to be a gland essential 
to the maintenance of life, its total removal leading to fatality, with a 
peculiar symptom complex-cachexia hypophyseopriva. In reading Paul- 
esco's experiments Crowe, Homans, and the writer observed that partial 
removal of the gland in adult animals led to adiposity and to atrophy 
of the ovaries or testes,2 whereas in puppies skeletal and sexual infantilism 
persisted. Thus the syndrome of Frohlich was shown to be due to a 
condition of hyposecretion (hypopituitarism). On the other hand, 
acromegaly, as shown by the improvement in the condition after hypophy- 
seal operations with partial removal of the gland (Hochenegg; Cushing), 
would appear to be due to an oversecretion (hyperpituitarism). 
Both of these conditions may be associated with tumor, although it is 
probable that in varying degrees of hyper- or hypo-activity they are of 
common occurrence-as common as the over- or underactivity of any 
1 Wien. klin. Rundschau, 1901, Nos. 47, 48. 
2 H. Cushing, Jour, of the Am. Aled. Assn., 1909, liii, 249 342 
DISEASES OF THE NERVOUS SYSTEM 
ductless gland, the thyroid for example. In the case of overgrowth 
(gigantism when starting early in life; acromegaly when in adult age), 
hypertrophy or adenoma of the gland itself is usually found. In the cases 
characterized by "adiposogenital degeneration" (showing persistence of 
sexual infantilism when originating before adolescence; and a tendency 
to revert to this condition when beginning in adult life) the tumor may 
either implicate the gland directly or may be situated above it in the 
infundibular region and lead to a pressure atrophy. There are other 
symptoms caused by these interpeduncular tumors, such as polyuria, 
glycosuria, amenorrhoea, impotence, psychic disturbances and so on, 
which in all likelihood are bound up with the activities of the other 
ductless glands. It is not unlikely, in view of Herring's discovery, that 
the hyaline secretion of the posterior lobe normally discharges into the 
cerebrospinal fluid of the third ventricle, that disturbances of hypophyseal 
activity may occur first or last in many cases of intracranial tumor. To 
this may be attributed the adiposity so often accompanying cerebellar 
tumors, particularly in children. 
Differential Diagnosis.-The three main questions which must be 
answered by the clinician when confronted with a suspected case of 
intracranial tumor in the order of their relative importance are: (1) Is 
a growth actually present? (2) If so, what is its presumable situation? 
(3) What is its nature? It cannot be emphasized too strongly that a 
brain tumor is rarely diagnosed in general practice until the onset of the 
major symptoms of pressure-the familiar triad of headache, vomiting, 
and choked disk. The ophthalmologist is too often the first to suspect 
a tumor. It is important to appreciate the fact that tumors in the retro- 
spect may prove to have been of years' duration before the onset of this 
unmistakable general pressure syndrome-an indication of the attention 
which must be paid in the future to the premonitory symptoms. 
The early mistakes in diagnosis make a most disconcerting professional 
record. In our early series the majority of the cases gave a history of 
having undergone treatment for long periods for an astonishing variety 
of presumed maladies, and, it may be added, inevitably for syphilis. The 
usual mistakes are naturally attributable to an effort to ascribe the more 
or less vague disturbances antecedent to the general pressure symptoms 
to some cause other than the unsuspected intracranial lesion. Diagnoses 
of hysteria, of psychoneurosis or neurasthenia, or of gastric or ocular 
reflex headaches are particularly common. 
Hysteria.-Certain of the so-called functional disturbances are inevit- 
ably superimposed on every organic lesion, and when the primary seat 
is obscure, and particularly if it affects the nervous system, the functional 
superstructure may so overtop the basal trouble as to effectually conceal 
it. One of our patients-the wife of a physician-for twenty years had 
suffered from irregular headaches, periods of amenorrhoea, intermittent 
grades of hemianesthesia and hemianopsia, inversion of the color fields 
and peculiar dreamy periods of semiconsciousness. Although seen by 
many during this period there was but one diagnosis-hysteria-until a 
terminal choked disk betrayed the nature of the lesion-a benign tumor 
of the right temporal lobe and uncinate region. TUMORS OF THE BRAIN AND MENINGES 
343 
In an excellent paper on brain tumor diagnosis Russell1 says that " the 
signs of organic disease cannot be confounded with anything that hysteria 
is able to furnish." This is true of a late period in the disease, not of an 
early one. The presence of organic changes, shown by a choked disk, 
cerebral nerve palsies, loss of the pupillary reflex, absence of the knee- 
jerks, an extensor plantar response, typical Jacksonian fits, and the like, 
serves to eliminate hysteria; but in the absence of these definite symptoms 
and in the presence of the so-called functional ones, brain tumor is often 
most difficult to rule out. Hysterical hemiplegia may at times be 
distinguished from organic disease by Hoover's test, which consists 
of an involuntary downward pressure of the supposedly paralyzed 
limb when the patient attempts to raise the sound member against 
resistance. 
General Paralysis of the Insane.-This is especially liable to be con- 
founded with frontal lobe tumors, particularly when the optic nerves 
have become atrophied, although a choked disk is said never to occur. 
The symptoms of tumor are usually progressive, those of general paralysis 
fluctuating; and the Argyll-Robertson pupil and characteristic defects 
of articulation are usually conclusive. 
Disseminate Sclerosis.-This may simulate tumors of the cerebellum 
and mesencephalon. There may be headache and choked disk; optic 
atrophy is common. The vertigo, vomiting, cerebral nerve palsies, 
nystagmus, incoordination and spastic condition of the extremities as 
well as the dysarthria may occur in both. 
Vascular Lesions.-These are often most difficult to distinguish. Press- 
ure symptoms may be present, and local signs are equally common. 
In arteriosclerosis the retina often proves a tell-tale should vascular 
disease, in the absence of choked disk, be marked in the retinal arteries. 
When a combination of tumor and cerebral arteriosclerosis is present a 
diagnosis may be very difficult. In our series there was a patient aged 
seventy-two, with transient aphasia and focal seizures involving the 
face, in whom existent vascular disease could well have accounted for 
the symptoms, but an exploration revealed a small primary glioma of 
the lower precentral region. 
Cerebral Thrombosis.-This may be difficult to distinguish from tumor 
when the process is slow, or, on the other hand, when tumor symptoms 
happen to be of fairly rapid onset. The vascular lesion leads to areas 
of softening which become oedematous and thus increase tension, so that 
headaches and choked disk, with the local manifestations, make the 
intracranial symptoms indistinguishable. This is especially true of the 
fairly acute forms of syphilitic encephalitis with thrombosis. 
Apoplexy.-Here, also, the characteristic sudden onset of symptoms 
speaks against tumor, although it must always be borne in mind that 
the hemorrhage may have occurred in the substance of a vascular growth. 
"Stroke" in a young individual with no evidence of general vascular 
disease and particularly when there has been a history of headaches, 
must arouse a suspicion of tumor. 
1 Brit. Med. Jour., 1907. ii, 1120. 344 
DISEASES OF THE NERVOUS SYSTEM 
Aneurisms.-Aneurisms of the larger vessels, particularly of the internal 
carotid, basilar, and middle cerebral, are not exceptionally rare, and may 
reach a considerable size without symptoms; on the other hand, they may 
closely simulate tumor. This is particularly true of carotid aneurisms 
which are found in the interpeduncular space near the chiasm, which 
may lead to hemianopsia, optic atrophy, or oculomotor palsy, in addition 
to the severe headaches simulating those of pituitary tumors. They 
occasionally may be recognized by a murmur, but this is less characteristic 
of the simple saccular aneurism than of the easily recognized arteriovenous 
variety. They may occur at any age, even in childhood, although 
naturally they are more common in adult life. Usually small, they may, 
as in Bramwell's case, reach the size of an orange. Their blood-content 
tends to clot, and may organize with spontaneous healing. Gowers gives 
the following series in order of their frequency: Sylvian, basilar, internal 
carotid, artery of the corpus callosum, posterior and anterior communicat- 
ing, vertebral, posterior cerebral, inferior cerebellar artery. 
When headache is present it is apt to be severe, and is increased by 
straining. Convulsions and vomiting may appear, but choked disk is 
rare, and a bruit is exceptionally uncommon except in the arteriovenous 
form, which could hardly be mistaken for tumor. The only patient in 
our series in whom a subjective and objective cranial bruit was present 
proved to have a contralateral glioma, and there was no aneurism. 
A bruit should be regarded as aneurismal only after the most careful 
consideration, for it may be produced by a vascular tumor, whether from 
its own circulating blood or by pressure against one of the large basal 
vessels. They are rarely diagnosed during life, and usually terminate 
in rupture with sudden death. In only 37 per cent, of the 555 cases 
studied by Beadles1 did aneurism give symptoms during life, and in 67 
per cent, death was preceded by an apoplectic seizure. 
Nephritis.-The symptoms of intracranial oedema secondary to renal 
disease may be indistinguishable from tumor. In both, headaches, 
vomiting, and neuroretinal changes may be present, and focal paralyses 
or convulsions may accompany the nephritic oedemas-for the process 
is apt to be more or less restricted and need not be general in character. 
The so-called "albuminuric neuroretinitis" is regarded by Bramwell,2 
Bordley, and the writer as a process due largely to the same factor which 
produces choked disk, namely, pressure. We have seen the characteristic 
stellate figures due to exudates in many cases of tumor; on the other hand, 
they may be completely wanting in nephritis. Uremia may have many 
features in common with the late stages of a new-growth. 
Abscess.-Wanting a clear history of an infected cranial wound, of 
otitis media or infection of the accessory nasal sinuses, a differential 
diagnosis may be difficult in the extreme. On three occasions we have 
mistaken abscess for tumor. Chronic abscesses have thick walls, and 
when exposed may be easily shelled out from the brain. They may be of 
long duration, with no antecedent history, and give symptoms the exact 
counterpart of a new-growth. Even a high grade of choked disk, which is 
1 Brain, 1907, xxx, 285. 
2 Byrom Bramwell, Clin. Studies, N. S., 1905, iii, 183. TUMORS OF THE BRAIN AND MENINGES 
345 
thought to be rare, may accompany them. In the retrospect, however, 
it becomes apparent that the symptoms are more apt to be fluctuating 
than is the case with tumor. Leukocytosis and fever may be absent, 
even though viable organisms can be recovered after some months from 
the cavity of the more or less dormant abscesses. 
Serous Meningitis or Ependymitis.-Serous meningitis or ependymitis 
leading to hydrocephalus may give a definite tumor symptom complex, 
although after the rather acute onset the symptoms are more apt to be 
fluctuating in severity, often showing long periods of abeyance. In one 
of our cases an obstruction of the left foramen of Munro occurred from 
ependymal inflammation, with symptoms indistinguishable from a tumor 
of the left hemisphere. Doubtless many of these peculiar cases are 
capable of spontaneous recovery, and should be included in the group 
of false or pseudo-tumors. It may be particularly difficult to distinguish 
a subtentorial tumor from circumscribed meningitis serosa. In addition 
to the pressure symptoms the latter may show nystagmus, paresis of 
an external rectus muscle, usually the right (Oppenheim), an inactive 
corneal reflex, tinnitus, and diminished hearing and cerebellar ataxia. 
It is in this latter group of cases that a differential diagnosis between 
cerebellar pontine tumor, labyrinthine disease and chronic serous arach- 
noiditis is difficult. Barany's1 method of accentuating symptoms by 
intra-aural caloric injections may be useful. 
Blastomycotic Meningitis.-This chronic form of meningitis may closely 
simulate a tumor with no localizing symptoms. We have had two 
recent examples both mistaken for neoplasm, the cerebrospinal fluid in 
both having been normal aside from greatly increased tension. 
Hydrocephalus.-It seems hardly possible that a tumor could be mis- 
taken for chronic or essential hydrocephalus, but such is the case, not 
only in the presence of a slowly progressive cerebellar lesion, but even 
with large tumors arising above the tentorium which happen to obstruct 
the ventricular outflow. Diastasis of the sutures occurs early in the 
cerebellar tumors of the young, and the cranial enlargement is sym- 
metrical: in tumors of the hemispheres an asymmetrical configuration 
of the head is more apt to occur, although even in these cases an associated 
ventricular hydrops may lead to a condition with a symptom-complex 
similar to simple hydrocephalus. A careful analysis of the incidents 
of onset will usually serve to distinguish the two conditions. 
A low grade of choked disk, rare in simple hydrocephalus, is almost 
always present with the tumor cases; and the latter are more apt to 
present rigidities with exaggeration of the reflexes and ataxia of movement 
than are the former. In one of our cases diagnosed by a number of 
neurologists and surgeons as hydrocephalus consequent upon ependymitis, 
an enormous psammoma of the right hemisphere was found; another 
child treated by ourselves for a year as a simple hydrocephalic proved 
to have a cystic glioma of the cerebellum. 
Hematoma of the Dura Mater.-Many conditions of lesser moment must 
be differentiated at times from tumor, such as hematoma of the dura 
1 Barany, Wien. med. Woch., August 27, 1910. 346 
DISEASES OF THE NERVOUS SYSTEM 
mater, characterized by a neomembrane of inflammatory origin with 
increasing layers of bloody extravasation. This may be found to cover 
the entire convexity of one hemisphere or may be merely a local process. 
It is usually accompanied by symptoms of progressive general paralysis. 
Traumatic Cysts.-Such cysts or even those of congenital origin may 
simulate tumors or be actually associated with tumors. 
Lead Encephalopathy.-This, according to Bramwell,1 may present 
neadache, vomiting, convulsions, often epileptiform in character, and 
double optic neuritis, and must be carefully distinguished from tumor 
when there is any suspicion of lead poisoning. Ue affirms that the two 
conditions so closely resemble one another that he never commits himself 
to a positive diagnosis of intracranial tumor without previously excluding 
lead poisoning. 
Pseudo-tumors.-Under this caption Nonne,2 Oppenheim, and Hoppe 
nave reported a number of instances in which a definite tumor symptom 
complex has completely subsided under specific or general medical 
treatment, and others in which, on postmortem examination, there was 
absolutely nothing to account for the definite pressure and focal symptoms 
present during life. Needless to say, hysterical conditions are excluded 
by the presence of definite symptoms of organic disease, often with a 
choked disk. We have had two cases of supposed tumor with death 
following operation in which no cerebral lesion was disclosed; and it is 
possible that a number of the patients in whom all evidences of pressure 
completely and permanently subsided after decompression have been 
cases of "pseudo-tumor cerebri." 
Certain of the oedemas of one origin or another, meningitis serosa and 
like conditions, are very difficult to recognize after death, and they 
represent states which lend themselves favorably to operation. The 
surgical cases which recover after simple decompression, even when they 
have been of sufficiently long standing and severe enough to have caused 
optic atrophy from choked disk, may in some instances represent healed 
tubercle, abscess, or cystic metamorphosis of a glioma. Hoppe calls 
attention to the possibility that some of the pseudo-tumors with negative 
postmortem findings may be due to the rare chronic cerebritis or cerebral 
hypertrophy described by Rokitansky. 
Course and Prognosis.-Generally speaking, an intracranial tumor 
represents one of the most serious of maladies, dreaded by victim and 
physician alike, in view of the suffering to be endured by the one and the 
incapacity of the other to relieve. As the diagnosis is rarely certified 
until pressure symptoms have become full-blown, the duration of life 
from that time rarely exceeds six months or a year and is often preceded 
by mental deterioration and blindness. Speaking of the lesions in par- 
ticular, on the other hand, in all their multiplicity of structure, manner 
of growth, situation, and possible complication, no definite statement can 
possibly be made. Their onset is always insidious, but it is self-evident 
that of tumors of the same size and type, one which occupies a silent 
area remains symptomatically dormant much longer than one which 
1 Clin. Studies, 1908-09, N. S., vii, 91. 
2 Deut. Zeit. f. Nervenheilk., 1907, xxxiii, 317. TUMORS OF THE BRAIN AND MENINGES 
347 
arises, for example, primarily in the precentral gyrus, or which early 
in its course has involved one of the cerebral nerves. 
One thing is certain, that tumors are of much longer duration than is 
generally supposed, for the retrospect will often show that even malignant 
gliomata have doubtless been present for months or years before a 
diagnosis is made coincident with the appearance of general pressure 
symptoms, after which their course may be rapid. The symptoms from 
the onset are usually progressive, although there are notable exceptions, 
for tumors may enter a stationary phase and undergo retrograde metamor- 
phosis. A tubercle may become quiescent, a glioma cystic or calcified; 
and some of the benign growths, as the endotheliomata, are of years' 
duration, leading in some instances to "autotrepanation" with relief of 
pressure, or they may even be a totally unexpected postmortem finding. 
There are other exceptions to the usual rule according to which symp- 
toms slowly progress from bad to worse. Thus in cases with sudden 
or apoplectiform onset or when symptoms come on abruptly after a fall 
the entire story is apt to be a short one. Then, too, death is not uncom- 
monly abrupt and unexpected, owing to a sudden acute oedema or to 
medullary anemia from pressure, so that prognosis as to duration of life 
is most uncertain. 
There remain "the unsolved riddles of pseudo-tumors," in the language 
of Bruns, so that the prognosis, in the presence even of a typical tumor 
symptom complex, need not necessarily be utterly pessimitic. 
Treatment.-Medicinal Measures.-So little has treatment been able 
to offer that the physician has heretofore turned to the one possible 
therapeutic resource-antiluetic measures-on the chance that the 
lesion might be syphilitic. The length of time which can justly be given 
over to such treatment in a case of brain tumor has been much debated. 
The period required for definite results has been placed by some as high 
as six months, and the dosage has been enormous. In this interval 
or even in a much shorter one if the growth happens not to be luetic, 
the patient may rapidly fail and his choked disk, if one be present, go 
on to the atrophic stage. Even a person in health, put upon a similar 
treatment, would suffer greatly from nutritional disturbances, and much 
more the individual already upset by headache and vomiting. 
Indeed, as a diagnostic test antisyphilitic treatment may be most 
misleading, for two reasons: one because with certain gliomata there 
may be a temporary amelioration of symptoms under these measures; 
and the other because the fibrous syphiloma, which gives the most 
characteristic tumor syndrome of all syphilitic processes, is exceedingly 
resistant even to massive doses of the usual drugs. Hence, even when 
the history and a positive serum reaction assure the diagnosis, the lesion 
must in the end be removed by operative methods if it be localizable, 
or if not, a palliative operation is necessary before the pressure symptoms 
are sufficiently relieved to allow the patient to receive full benefit from 
the drug administration. This too is the more urgently demanded in 
case a high grade of choked disk exists. 
The typical basilar gummatous meningitis is relatively easy to diagnose 
and yields much more quickly to treatment than does a syphiloma. 348 
DISEASES OF THE NERVOUS SYSTEM 
Fortunately the Wassermann or Noguchi reaction will usually serve to 
spare these patients much discomfort and their attendants long anxiety. 
The tests should always be made at the earliest moment. 
To state this matter briefly, the writer would say that in the presence 
or absence of a positive serum reaction or a definite history of lues, anti- 
syphilitic treatment deserves only a brief vigorous trial; if pressure symp- 
toms have been outspoken and do not become distinctly ameliorated in 
the course of a few days, a palliative decompression should be performed 
and the treatment subsequently resumed should the diagnosis remain in 
doubt. One valuable criterion of the effect of the treatment and the 
need of early operation must rest with the condition of the eye-grounds, 
which should be observed daily, for a choked disk may continue to 
advance, even though subjective discomfort is lessened. In our series 
a fibro-syphiloma has been found and successfully extirpated in a number 
of instances in patients who had been subjected to a long although 
unavailing course of treatment and were much run down. 
The general treatment should be directed toward the best possible 
preservation of the nutrition by frequent small amounts of digestible 
food; for, apart from the incidental vomiting many of these patients 
tend in later stages to lose weight rapidly. Cerebral congestion is to be 
avoided by proper position of the head, by ice caps, and by free daily 
evacuations. Alcohol is not tolerated. There is practically no effectual 
way of warding off the explosive attacks of vomiting; the headaches 
when insufferable may require large doses of narcotic drugs, antipyrine, 
phenacetine, codeine, combined with chloral, trional, or veronal for the 
night, and the hypodermic use of morphine as a last resort. Convulsions 
must be combated by bromides, or in desperate conditions by chloral 
or morphine-even by inhalation narcosis. Occasionally a threatened 
Jacksonian attack may be aborted by the prompt inhalation of a few 
drops of chloroform. 
Surgical Measures.-The first therapeutic indication for a tumor 
anywhere in the body which occasions, or is likely to occasion, sympto- 
matic disturbances, is removal, provided this can be accomplished without 
risk and without leaving undesirable consequences; for the mere prolon- 
gation of life, whether through medicines or operation, provided it is not 
made worth the living, is, of course, undesirable. The second indication 
is the alleviation of symptoms, and inasmuch as the conditions in the case 
of intracranial growths are almost entirely mechanical, they demand, for 
anything more than temporary palliation, mechanical means of relief. 
The first ray of hope in regard to the operative therapy for brain tumors 
was dimmed by von Bergmann's widely heralded views as to the practical 
hopelessness of these measures, owing to his long series of early surgical 
failures. But within the past few years new elements have served to 
put the subject on an entirely new and much sounder basis. Among 
these have been the greater precision in diagnosis and a greater perfection 
in technical operative methods. A further element of no little significance 
is the development of surgeons trained in neurological pathology and 
diagnosis-in other words, of operating neurologists; for it is inconceivable 
that these matters of handicraft can advance far or fast when the surgeon TUMORS OF THE BRAIN AND MENINGES 
349 
is merely the hand directed by the neurologist-a relationship comparable 
to that of the pre-Vesalian anatomist and his prosector. 
If medicine is still to be widely separated from surgery a system of 
medicine is no place for a discussion of surgical procedures. Nevertheless, 
so long as the physician continues to be the one first called upon to 
determine the future course of treatment for a patient with brain tumor, 
it is the more needful that he shall understand something of the operative 
methods employed and the results which should be obtained thereby. 
It is astonishing to learn how rapidly the number of these operations 
has increased. Allen Starr, in 1893, succeeded in collecting 97 cases; 
Chipault, in 1894, 135 cases; Oppenheim, in 1896, 140 cases; whereas 
today these by no means represent individual figures. Thus in the two 
years since the opening of the Brigham Hospital there have been 199 
admissions for " Brain Tumors," and, in all but a few of these, operative 
procedures were employed which gave a promise of relief or cure. 
Surgical measures have two main objects: (1) To expose and remove 
the growth if possible; (2) to palliate the symptoms of pressure in case 
(a) of an impossible regional diagnosis, (5) of a lesion in a recognizable 
though surgically inaccessible region, (c) of irremovability on exposure, 
whether from excessive size or undue vascularity. 
Tumor Extirpation.-Practically all of the successful cases before the 
year 1900 were confined to tumors involving the paracentral convolutions, 
but during the past ten years the number of successful removals from 
frontal, temporal, parietal, or occipital lobes and from the cerebellum 
has rapidly increased. The exposure of the hemispheres is no longer 
carried out by simple trephining, but by the reflection of a large osteo- 
plastic flap with its base hinging on the thin temporal region. Thus, a 
wide area erf exposure is secured through which the necessary manipula- 
tions can safely be carried out. As the making of the flap itself, from 
loss of blood, may be something of an ordeal, a two-stage operation, as 
advocated by Horsley, is often advisable. The tumor, if exposed, 
should not be scooped out, but should be removed,.if possible, by careful, 
slow dissection, with painstaking hemostasis. A bone-flap operation 
is undesirable in cerebellar cases where a bilateral suboccipital exposure 
with permanent removal of the bone is essential. Subtentorial tumors 
are often favorable for removal. 
Tumor Palliation (Decompressive Operations).-The unexpected 
relief which followed many of the earlier unsuccessful explorations gave 
birth to the idea of making a purposeful defect in the cranium and dura 
to give relief to pressure symptoms. Owing to the tendency of the brain 
under pressure to herniate through a defect and of the protruding portion 
to lose its function, it is desirable to make the opening over a silent area. 
When it is desirable to end a fruitless exploration of the hemisphere with 
a palliative decompression, the osteoplastic flap may be removed in toto, 
or the subtemporal area of bone alone removed. In the case of presumed 
cerebellar lesions the palliative operation differs in nowise from that 
necessary for exploration. 
These successful palliative measures have in many ways represented 
our chief advance in the past few years, for they are comparatively simple 350 
DISEASES OF THE NERVOUS SYSTEM 
and free from risk. Their chief value lies in the prevention of blindness, 
which too often is the penalty of procrastination in cases of brain tumor. 
Certain lesions included under intracranial tumors, as well as actual 
tumors themselves, may prove to be more or less self-limited, with a 
tendency toward spontaneous healing, but unhappily loss of vision 
through optic atrophy is apt to occur in the interval of their activity; 
and to offset this danger, decompressive operations are particularly 
well adapted. 
With the exception of tumors which have produced an internal hydro- 
cephalus, alleviation of the pressure symptoms without increase in focal 
manifestations may be expected in most cases; and the subtemporal 
protrusion, if well protected by muscle, is very unobtrusive. Occasionally 
a callosal puncture (van Bramann) may serve to alleviate the symptoms 
of a secondary obstructive hydrocephalus. Tumors situated in the 
pontine region, with or without hydrocephalus, are also an exception 
to this general rule, for the change of position of the parts, either after 
the establishment of a subtemporal or a suboccipital defect, is apt to 
exaggerate preexisting local symptoms. 
Puncture of the brain (IIirnpunktion) and of the lumbar meninges as 
therapeutic measures, although successful in an occasional rare instance 
of draining an unsuspected meningitis serosa (Quincke) or the exceptional 
aspiration of a simple cerebral or cerebellar cyst, are nevertheless attended 
with grave risks. 
Parts of the brain, like the interpeduncular region, heretofore thought 
forever secure from surgical approach, have proved to be accessible in a 
number of ways, and tumors involving the hypophysis, as well as primary 
hypertrophies of this gland, are regarded as surgical maladies.1 
Notable advances have been made during the past decade in perfecting 
the technical methods of operations for brain tumor and the mortality 
of these procedures has fallen to a very low figure2 compared with that of a 
few years ago. Nevertheless success in this particular field of surgery 
requires a special training the foundation of which must be laid in the 
neurological clinic and experimental laboratory for neuropathology. 
1 H. Cushing, The Pituitary Body and its Disorders, J. B. Lippincott Co., Phila., 
1912; also Surgical Experience with Pituitary Disorders, Jour. Am. Med. Assn., 1914, 
Ixiii, 1515. 
2 Concerning the Results of Operations for Brain Tumors, Jour. Am. Med. Assn., 
1915, Ixiv, 189. CHAPTER X. 
HYDROCEPHALUS. 
HARVEY CUSHING, M.D. 
A pathological increase in the amount of cerebrospinal fluid in the 
cranial chamber doubtless always represents a symptom of disease, 
not a disease of itself; although it must be confessed that pathology is at 
a loss to explain the undue accumulation of fluid in a certain group of 
cases, which, therefore, have been styled "essential" hydrocephalus. It 
may occur as an acute or chronic process, congenital or acquired, due to 
inflammatory or purely mechanical causes; and finally the hydrops 
may be confined to the ventricular spaces or be found on the external 
surface of the brain. 
As the cerebrospinal fluid, through retention or overproduction, is the 
factor chiefly concerned, some account of its source and physiological 
action is essential to such a crude understanding of the condition as we 
possess. Although possibly suspected by some of his predecessors, 
nevertheless Magendie, in 1842, first established beyond doubt the 
normal presence and rapid reformation of the fluid after withdrawal. 
Notable recent additions to our knowledge have come, first, through 
the reawakening of interest in the fluid, as the result largely of Leonard 
Hill's physiological and Halliburton's clinical studies in the nineties, and 
second, through the introduction and widespread use of Quincke's lumbar 
puncture. Hill expressed the opinion that the fluid should be regarded 
as the lymph of the brain, but as Halliburton1 showed, it has marked 
chemical differences from a transudation, and must be regarded as a 
true secretion, for the normal fluid is clear and colorless-like water- 
slightly alkaline in reaction, and of low specific gravity, 1005 to 1010; 
containing a mere trace of proteid (globulin) coagulable by heat, and also 
a reducing substance which has been shown to be glucose.2 Lymph is 
like diluted blood plasma, with a specific gravity of 1012 to 1022, giving 
a test for sugar, containing proteids (albumin) in large amounts and 
coagulating spontaneously. Only in diseased conditions, such as 
inflammation and general paresis, does the usual character of the cerebro- 
spinal fluid become greatly altered, with an increase of proteid, higher 
specific gravity, and in paresis a new substance, choline, due to the 
presence of degenerated nerve cells (Mott and Halliburton). 
The fluid, in large part at least, is presumably the product of secretory 
activity of the ependymal cells lining the vascular choroid plexuses, 
although the precise function of these peculiar organs has not been 
1 Biochemistry of Muscle and Nerve, Philadelphia, 1904, p. 70. 
2 An elaborate monograph entitled Le liquide cephalo-rachidien normal el patho- 
logique, Paris, 1912, W. Mestrezat, has fully considered the subject. 
351 352 
DISEASES OF TflE NERVOUS SYSTEM 
conclusively established. Certain experiments have shown that their cells 
may be definitely activated; and the plexuses doubtless possess an 
actual' glandular function.1 The secretion appears to be a fairly active 
and more or less continuous one, for the fluid, under certain circumstances, 
may form in large amounts. This is well illustrated by the rapid refilling 
of the hydrocephalic ventricles after tapping, but perhaps is best seen 
when, by accident or intent, an external fistula communicating with the 
subarachnoid space has been established, or in the remarkable cases of 
cerebrospinal rhinorrhoea such as are described in St. Clair Thomson's 
monograph-a condition characterized by a spontaneous escape of 
cerebrospinal fluid from the nose. No symptoms save loss of weight may 
attend such an escape of fluid continued over months, and an amount 
equal to half a liter may be collected in twenty-four hours. There may 
be a double source for the fluid, and although the larger portion comes 
through the ventricles, this may be added to by the cerebral lymphatics, 
which are said to follow the pial vessels and empty into the arachnoid 
spaces. 
It would seem, therefore, that there must be a definite circulation for 
the fluid; and presumably forming in large part in the lateral ventricles, 
it passes thence by the foramen of Munro to the mid-ventricular system 
and escapes into the subarachnoid spaces by way of the foramina of 
Magendie and Luschka. From this point it bathes cord and brain, con- 
fined chiefly in the subarachnoid spaces, for only in certain pathological 
conditions does fluid appear in any considerable amount in the subdural 
space. From the subarachnoid spaces the fluid seems to escape directly 
into the circulation by way of the dural sinuses, rather than through 
lymphatic channels with the intermediation of glands before it reaches 
the venous system-another striking difference from true lymph. Some 
of it may possibly escape along the meningeal sheaths of the cerebral and 
spinal nerves-particularly the optic (cf. the condition in choked disk) 
and olfactory, but this is but a drop to the rapid escape by way of the 
sinuses. It was the view of Key and Retzius that the Pacchionian 
granulations played a certain role in this escape, but though it pre- 
sumably occurs at the points where they are situated, fluid may escape 
freely, by way of the arachnoidal connections with the dura, in infants, 
in apes, or in the canine, in whom no Pacchionian granulations are present. 
Recent studies by Weed2 made it clear that it is the arachnoid villi or 
tufts, which project into the dura, which represent the points of exit 
for the fluid. 
Even with our scant understanding of the cerebrospinal fluid circulation, 
it nevertheless can be seen how ventricular hydrops, the more important 
of the conditions we are to consider, can be due to obstruction of the 
channels of outlet from the ventricles; how infectious processes may 
easily occlude the small perforations in the roof of the fourth ventricle, 
or the arachnoidal points of entry of the fluid into the sinuses; how 
thrombosis of the sinuses may lead to cerebrospinal fluid stasis, apart 
1 Meek, Jour, of Compar. Neurol, and Psych., 1907, xvii, 286. 
2 Studies on the Cerebrospinal Fluid, Jour, of Med. Research, 1914, xxxi, 1-176. PLATE VI 
Hydrocephalus Externus in an Infant with Normal Development 
of Skull and Meninges, but Defective Brain. HYDROCEPHALUS 
353 
from the possible actual increase in the amount of fluid through venous 
congestion; how it is that the source of obstruction may be easily over- 
looked; and lastly, why many of the operations suggested for the relief 
of the condition have been based on erroneous principles of drainage. 
Hydrocephalus Externus.-An abnormal collection of fluid outside of 
the ventricular cavities is rare, if we are to exclude certain pathological 
states accompanied by oedema of the brain and leptomeninges. It is 
misleading to refer to the "wet brain" of alcoholism, to the traumatic 
or angioneurotic oedemas, or to some of the cerebrospinal disturbances 
grouped under serous meningitis, as external hydrocephalus, although 
there is an excess of fluid in the meningeal spaces. It is confusing 
also to include here the hydrops ex vacuo or "compensation hydro- 
cephalus," which is merely the collection of fluid required to fill the 
intracranial chamber when atrophic processes have led to a shrinkage of 
the brain. 
A true external hydrocephalus, however, may be found in association 
with congenital anomalies, when an undeveloped brain is found filling, 
in part only, a cranial chamber of normal configuration (Plate VI); 
and in rare instances it may occur in combination with a ventricular 
hydrocephalus when, through spontaneous perforation, the fluid has 
escaped into the subdural space. This, however, on physical principles 
would not be expected to occur very often, and surgical experience has 
shown that the distended and thinned hemisphere will not necessarily 
collapse and allow fluid to become extracerebral, even if a large opening 
be made into the ventricle through its thinned wall. 
Hydrocephalus Internus.-It is customary to distinguish several 
varieties, particularly the so-called idiopathic form, regarded as a primary 
hydrocephalus, from those in which the condition is palpably secondary 
to some manifest lesion-the acquired forms. It would seem, however, 
that if there is any form of hydrocephalus which may justly be considered 
as a primary hydrocephalus, it is represented by the acute collection of 
fluid associated with the meningitis serosa of Quincke or with ependymitis; 
for here it is possible that we may be dealing with a condition in which 
fluid is excreted in excess of what may readily escape from the cranial 
chamber by the normal outlets. Nevertheless the resultant thickenings 
of ependyma when the acute process has subsided often lead to a persistent 
hydrops, so that even these states are customarily included with acquired 
or secondary hydrocephalus. 
Essential or Idiopathic Hydrocephalus.-Etiology.-This sometimes 
occurs as a familial type, several members of a family being afflicted, so 
that hereditary influences play a part. The condition has been ascribed 
to fetal syphilis or rachitis, its association with congenital lues having been 
frequently observed. It is deserving of note, however, that the lower 
animals, especially those in captivity, may have hydrocephalic offspring. 
Presumably in most cases there exists some obstacle to the normal 
outflow of fluid from ventricle or cranial chamber, whether due to anomaly 
or disease. Heinecke believes that the hydrops is produced by an ab- 
normal increase in the amount of fluid secreted. Both factors possibly 
may be at work. 354 
DISEASES OF THE NERVOUS SYSTEM 
Pathology.-Meningeal thickenings suggestive of some prenatal inflam- 
mation are occasionally found matting together the structures, par- 
ticularly about the roof of the fourth ventricle. More often, however, 
such an evident source of obstruction is absent, and the fluid from the 
ventricles can be withdrawn from the subarachnoid spaces by lumbar 
puncture, showing that if the process is actually an obstructive one 
the stasis in the fluid circulation must occur elsewhere, possibly at the 
foramina of entrance into the sinuses. The nature of these foramina 
has been made clear by Weed's studies and as the fluid escapes through 
the delicate membrane lining the arachnoid tufts by slow seepage an 
inflammatory process of mild degree may easily occlude these structures. 
Ventricular hydrocephalus is the usual accompaniment of the structural 
anomalies, cephalocele and spina bifida, and it is a natural conjecture that 
these developmental defects have been produced by some early disturb- 
ance with the normal circulation of cerebrospinal fluid which may have 
prevented the proper closing in by the mesoblastic tissues of the primitive 
nervous system. This could easily be brought about by some abnormal 
increase in tension of the fluid before closure was complete. Only when 
the obstructive lesion, whatever it may have been, has been recovered 
from or overcome by some operative measure can the spina bifida or 
cephalocele be removed without considerable augmentation in the degree 
of hydrocephalus already present. 
" The appearance of the skull is striking, the large, thin, flaring cranial 
leaflets being perched on the small facial bones like the petals of a single 
water lily on its calyx. The bones themselves are thinned and atrophied 
in places, so that pericranium and dura may meet (craniotabes). There is 
apt to be an abnormal number of Wormian bones. The frontal, temporal, 
and occipital wings, instead of arising vertically from the base, overhang 
so that the squamous wing of the temporal, for example, becomes almost 
horizontal and overlies the zygoma in such a way as to almost obliterate 
the temporal fossa. The irregularities of the three basal fossse are slowly 
pressed out, so that the sphenoidal and petrosal ridges separating them no 
longer project as prominent watersheds. The base, however, does not 
participate in the general enlargement which the other bones undergo. 
In extreme cases the brain itself becomes ballooned out by the gradual 
increase of fluid, so that in places it is of paper thinness. Not only the 
ventricles, but the communicating passages also are all widely dilated; the 
iter is distended, and the foramen of Munro may become large enough to 
admit three fingers (Plate VII, Fig. 1). The corpus callosum may be 
stretched into a thin sheet and the much distended septum lucidum often 
gives way, leaving a direct communication between the lateral ventricles. 
The commissures between the basal ganglia usually remain intact, 
although they may be drawn out to a length of 2 cm. or more" (Plate 
VII, Fig. 2). 
The amount of fluid may be enormous-three liters or more in extreme 
cases. It shows no abnormality in saline, albuminous, or cellular 
elements. 
Course and Symptoms.-In its more familiar and striking form the 
condition has been evident from birth, or at least has set in with insidious PLATE VII 
Advanced Grade of Chronic Internal Hydrocephalus, with extreme thinning of hemi- 
spheres, except at pyramidal tract (X)„ Note enormous size of foramen of Monro. 
Circumference of head, 70 cm. 
Moderate Degree of Ventricular Distention in Chronic Hydrocephalus. Note rupture of septum 
lucidum; also great widening of iter, which is crossed by elongated commissure. HYDROCEPHALUS 
355 
onset in early infancy. Owing to the ready distensibility of the sknll at 
this early age the condition when full-blown leads to external appearances 
which are unmistakable; "a cranial chamber enlarged out of all propor- 
tion to body and face; the enormous and tense fontanelles; the cranial 
bones of the vault widely separated, even when they should be together, 
so that the midparietal, midfrontal, parieto-temporal and parieto-occipital 
sutures, bridged over by tense membrane, may all be open for 1 cm. or 
more; the bulging of the frontal temporal, and occipital bones; the thin 
scalp, with its sparse hair and dilated vessels, which are thrown into 
prominence, on account of the intracranial venous stasis; the expressional 
characteristics due to the tilting of the ears, with their wide intertragical 
notch and slitlike meatus; of the eyes, which are displaced by the down- 
ward bulging of the orbital roof and are covered chiefly by the lower lid, 
owing to the upward pull of the tight scalp upon the palpebral angles. 
The palpebral cleft is narrow, and the upper lid, when smoothed out, 
shows a network of dilated vessels." 
When the enlargement is extreme (Plate VIII, Fig. 1) these patients 
are unable to turn or raise the head, and pressure decubitus may result 
unless changes of position are frequent. The circumference may often 
reach 70 and occasionally 100 cm. and the circumferential form is variable, 
depending upon the degree of tension and the habitual position assumed 
by the head, whether on the side, back, or at an angle. 
The patients are, for the most part, feeble and poorly nourished, and 
even if the process becomes stationary after it has reached a high stage 
they have little resistance and are prone to succumb to intercurrent 
troubles. Should they live for some years they are apt to retain an 
infantile aspect, as well as infantile mental characteristics. Blindness, 
from optic atrophy, may occur, but this is not usual, and, indeed, even a 
low grade of retinal oedema is rare, for a pressure sufficient to produce an 
advanced degree of choked disk cannot well occur, as the cranium is so 
easily distensible. In some instances, even of the extreme type, adult life 
may be reached, with a certain feeble intellectual capacity combined with 
a traditional good nature. Rarely the process seems to have become 
arrested at an early age, with subsequent normal mental and physical 
development. Indeed, certain individuals with notable intellects are 
said to have shown evidences, post-mortem, of a low grade of hydro- 
cephalus, presumably congenital. 
Diagnosis.-There may be a close resemblance of a low-grade process 
to the rachitic head; the conditions of course may co-exist. When hydro- 
cephalus is uncomplicated by this nutritional disturbance the diagnosis 
usually presents little difficulty. For though the cranium is large, cranio- 
tabes present, the fontanelles open, and the lines of the sutures flexible 
in rickets, the square shape of the head, the absence of signs of extra- 
cranial venous stasis, a fontanelle which is not tense, and rachitic changes 
in the bones elsewhere tend to distinguish the process. The chief difficulty 
lies in the differentiation of this so-called idiopathic hydrocephalus from 
the acquired form secondary to definite lesions. This is largely a matter 
of history; for in the essential form the process has been present from 
birth and is often associated with other anomalies; in the acquired form 356 
DISEASES OF THE NERVOUS SYSTEM 
careful inquiry will show that a normal development has seemingly been 
interrupted in consequence of a cranial injury, a febrile illness, or some 
other unusual incident. A choked disk and the hydrocephalic rigidities 
are much more common in the acquired form. 
Treatment.-The indications are purely mechanical ones and should 
be directed toward the establishment of some form of permanent drainage, 
whether by the channels intended by nature to carry away the fluid or by 
the establishment of new ones. There are a great number of operative 
procedures in which the attempt is made to drain the fluid into the sub- 
arachnoid space, into the subaponeurotic space of the scalp, directly into 
the sinuses, into the abdominal cavity, into the retroperitoneal spaces, and 
so on. None of them have given particularly brilliant or uniform results, 
although there have been occasional recoveries even in advanced cases. 
Whatever method is used, it is necessary to determine first of all where the 
obstruction has taken place, for many of the older operative measures 
are futile if the ventricular and subarachnoid spaces freely communicate. 
Most of the older operations have been conducted on the view that 
occlusion of the neighborhood of the foramen of Alagendie is the 
single possible lesion. Temporary withdrawal of the fluid by ventricular 
or lumbar puncture leads, as a rule, to its rapid reaccumulation, at times 
under greater tension than before, and is rarely of any lasting benefit. 
Acquired or Secondary Hydrocephalus.-This differs from the essential 
or primary form only in so far as it is apt to have a definite onset sub- 
sequent to birth, often in association with a manifest causal agency. 
Etiology.-Any form of obstruction to the normal circulation of the 
fluid or any condition which increases its production in excess of what 
may escape through the normal channels of outflow will produce this form. 
The more common agencies are tumors, meningeal or ependymal inflam- 
mations, and stasis of the venous circulation through extensive sinus 
thrombosis, or obstruction of the venae Galeni. 
Tumors.-Tumors, particularly those confined in the subtentorial 
region, whether intrapontine, extrapontine, or cerebellar, may obstruct 
the ventricular iter or the foramen at the roof of the fourth ventricle, 
leading to a more or less symmetrical dilatation of third and lateral 
ventricles. When these conditions arise in early infancy there is a rapid 
secondary enlargement of the head, so that the usual pressure phenomena 
of tumor may be wanting, with external appearances the exact counter- 
part of those described under essential hydrocephalus. A tumor, a cyst, 
or a tubercle may be an unsuspected postmortem finding (Plate VIII, 
Fig. 2). Diagnosis may be impossible without a local exploration-and 
not always then. A most valuable aid is the history of a previously 
normal state before symptoms began to appear. 
The outward manifestations of idiopathic hydrocephalus, with 
separated sutures, wide fontanelles, etc., need not be limited to the 
secondary hydrocephalus of subtentorial lesions, for tumors of the cere- 
brum may occlude one or both foramina of Munro and produce a sym- 
metrical cranial enlargement with general increase of pressure which 
serves to mask all focal symptoms. The tumors of themselves, under 
these circumstances of a yielding skull, may reach an enormous size. PLATE VIII 
FIG. 1 
Idiopathic Hydrocephalus. 
An unusual degree of cranial enlargement. Circumference, 40 inches. 
(Dr. Willard Knowlton.) 
FIG. 2 
Unsuspected Gliomatous Cyst of Cerebellum in an Infant. 
Producing enormous ventricular distention from obstruction; regarded during 
life as idiopathic hydrocephalus. PLATE IX 
FIG. 1 
Showing Separation of Sutures and Widely Opened Fontanelle 
Containing Isolated Island of Bone. 
An infant with hydrocephalus secondary to meningitis; viable organisms in ventricles. 
FIG. 2 
Section of Brain of Fatal Case of Capsular Apoplexy. 
In which a chronic hydrops ventriculorum with granular ependymitis was an 
unexpected postmortem finding. HYDROCEPHALUS 
357 
Tumors which arise after the normal closure of the sutures and fonta- 
nelle has occurred may, through hydrocephalus, lead to a reseparation or 
diastasis of the sutures in the young, and even after adolescence, with an 
appreciable enlargement of the head, although never to such a degree as 
is seen in the infantile conditions. The "cracked-pot" percussion note 
which characterizes these conditions is an unmistakable evidence of a 
secondary hydrocephalus. Hbntgenological changes in the skull are also 
very characteristic. The sutures are apt to show significant separations, 
but the most striking tell-tales of hydrocephalus are the pressure digita- 
tions or convolutional markings on the inner surface of the skull. There 
is only one other condition, namely, oxycephaly-which gives this 
picture in a degree more striking than hydrocephalus. 
The abrupt onset of general pressure symptoms, in many cases of tumor 
which have been more or less dormant, is usually due to the occurrence 
of an obstructive hydrocephalus. This is particularly true of such benign 
growths as the cerebellopontine endotheliomata. The pressure symptoms 
are not necessarily constant, but may vary in intensity from time to 
time, depending on the degree of completeness of the obstruction. 
Inflammations.-A basilar inflammation, provided the exudate 
serves to occlude the minute foramina of exit in the roof of the fourth 
ventricle, leads inevitably to a hydrocephalus-the usual antecendent of 
death in these processes. Were it not for this mechanical obstruction 
and the resultant pressure phenomena meningitis would doubtless often 
be a self-limited disease. 
Cerebrospinal Fever.-Attention has been called to the fact that in 
meningitis a pyo-hydrocephalus is often the terminal incident, for with 
the ventricular spaces not only infected but occluded the fatal termination 
of the malady is not remote. In infants, however, the ventricular dis- 
tension need not give profound pressure symptoms, for the cranial 
chamber may rapidly enlarge (Plate IX, Fig. 1) and the process con- 
tinue for months, organisms remaining viable in the cavities after they 
have died out from the meningeal spaces.1 Naturally in these conditions 
serum introduced by the lumbar route does not have access to the infected 
surfaces, and introduction by ventricular puncture is indicated. 
Posterior Basic Meningitis.-In the longer enduring forms of infection, 
as in Barlow's posterior basic meningitis, these occlusions are common, 
and the disease may drag on for months with slowly enlarging head, 
persistent retraction of the neck and hydrocephalic rigidities. Thus, 
it is not uncommon for the cases to run a chronic course, with more or 
less fluctuation of the symptoms according to the ease with which the 
pent-up fluid may escape. The occluded foramina of exit may become 
rechannelled in some cases, with no further advance in symptoms, although 
even with recovery a ventricular hydrops of this sort is apt to persist in 
some degree. 
The ventricular complications of tuberculous meningitis differ nowise 
from those due to other infectious agencies. The patients usually die 
with, if not from, the secondary ventricular dilatation. 
1 Cushing and Sladen, Jour, of Exper. Med., 1908, x, 548. 358 
DISEASES OF THE NERVOUS SYSTEM 
Ependymal Inflammation.-A primary ependymal inflammation may 
obstruct the passage of fluid by inflammatory closure of the interventric- 
ular foramina. This mysterious condition characterized by the presence 
of a granular thickening of the lining ventricular membrane occurs more 
often in the adult than in the young. The condition has no distinct 
symptomatology aside from that of an intracranial infection with definite 
pressure symptoms. It is not infrequently recovered from, judging from 
the number of instances in which traces of its former occurrence (a 
dilatation of the ventricle with ependymal granules) without history 
are unexpectedly brought to light when death has occurred from other 
causes (Plate IX, Fig. 2). 
Meningitis Serosa.-The meningitis serosa of Quincke is a condition 
which lumbar puncture has enabled us to recognize. It is an acute 
process, with onset very similar to that of infective meningitis, but with- 
out the association of organisms-at least of those which can be cultivated. 
The condition may arise spontaneously or in the course of infectious 
diseases or intoxications. It is a particularly common sequel of otitis 
media and may be difficult to distinguish from infective meningitis or 
abscess. There is an excess of fluid under tension and usually a ventric- 
ular hydrops. A choked disk of low degree is commonly present in this 
as well as in the many definitely obstructive forms of ventricular hydro- 
cephalus. In the absence of fever tumor may be closely simulated. 
Lumbar puncture shows the fluid to be under great tension, and its 
withdrawal may completely change the clinical picture for the better. CHAPTER XI. 
ACUTE ENCEPHALITIS AND BRAIN ABSCESS. 
E. E. SOUTHARD, M.D. 
ENCEPHALITIS. 
Synonyms.-Inflammation of the brain; acute red softening of brain; 
brain fever; cerebral fever; cerebritis; phrenitis; phrenesia; phrenetiasis; 
phrenicula; cerebral phlegmasia; calenture; febbre perniciosa frenetica; 
suppuratio occulta cerebri. The terms brain fever, cerebral fever, and 
the like frequently refer to meningitis. The term encephalitis has 
been used freely in the past for entirely obscure conditions, so that I 
am assured by the United States Census Office that their figures for 
encephalitis are quite unreliable. 
Definition.-Encephalitis means inflammation of the encephalon or 
brain. The best usage includes under the term encephalitis exudative 
inflammations of the brain. The place of encephalitis as here discussed 
may be indicated in the following scheme: 
ENCEPHALOPATHY 
TRAUMATIC 
VASCULAR 
encephalomalacia) 
DEGENERATIVE 
(e. g., saturnine, 
alcoholic) 
EXUDATIVE 
(encephalitis) 
PRIMARY? 
Most of the work on encephalitis preceding the era of Virchow's work 
on thrombosis and embolism (1846) can be neglected for modern purposes. 
Virchow's work began to have wide currency in 1856, but, although it 
cleared the way for a definition of focal softenings (focal encephalo- 
malacia), the status of encephalitis was no better than before. Nor can 
it be said that Virchow's contribution of encephalitis neonatorum (1867)1 
aided systematic work, since it was at once opposed by Hayem2 (1868) 
and Jastrowitz (1870), and cannot yet be said to be a solved problem. 
Hayem (1868) developed the idea of non-suppurative or hyperplastic 
encephalitis. Various contributions followed in which the infectious 
character of encephalitis became probable. Wernicke,3 in 1881, described 
the disease (fpoliencephalitis acuta hemorrhagica superior), which bears 
his name, as an acute inflammatory process about the Sylvian aqueduct 
region, and compared the process with acute anterior poliomyelitis. 
1 Arch. f. path, anat., etc., Berlin, 1867, xxxviii. 
2 Etudes sur les diverses formes d'encephalite, Paris, 1868. 
3 Lehrbuch der Gehirnkrankheiten, Kassel, 1881, Band ii, Th. 3, S. 229 to 242. 
359 360 
DISEASES OF THE NERVOUS SYSTEM 
Strumpell1 (1884) constructed a very productive hypothesis-that many 
cases of cerebral palsy in children are due to encephalitis of the motor 
areas. In 1893 Macewen's work on Pyogenic Infective- Diseases of the 
Brain and Sinnal Cord appeared, in which the surgical importance of 
brain abscess and inflammation of otitic origin was developed. In 
1898 Councilman, Mallory, and Wright described encephalitic lesions 
due to Weiehselbaum's meningococcus, and in all cases co-existent with 
frank meningitis. 
The infectious character of several types of exudative inflammation 
of the brain is well established. The interest of the present day is in 
the bacteriology (parasitology?) of these processes and in the possibility 
of differentiating cases into groups according to their infectious origins. 
Clinically, we are as yet unable to define groups with precision according 
to the different known infective agents. For some years, in Boston, an 
effort has been made to correlate these points of view. This work 
started out from the report of Councilman, Mallory, and Wright on 
"Cerebrospinal Meningitis," and has taken the shape of studies dealing 
with the histopathological and clinical effects upon the brain of the 
Micrococcus aureus (Southard and Sims,2 Southard and Keene3), pneu- 
mococcus (Bullard and Sims,4 Southard and Keene5), streptococcus 
(Southard and Stratton6), and Bacillus typhosus (Southard and Rich- 
ards7). This work has shown the association of Staphylococcus pyogenes 
aureus and of the pneumococcus with acute hemorrhagic encephalitis, 
uncomplicated by meningitis. 
Classification.-We distinguish the acute from the subacute and chronic 
forms of encephalitis. In the acute form we find characteristically the 
cardinal signs of inflammation. Of these signs, redness and swelling 
are prominent postmortem features of acutely inflamed brain tissue. 
The pain which sometimes accompanies encephalitis is due not to com- 
pression of the cerebral tissue itself, but to the pressure incidentally 
exerted upon the trigeminal endings by the swelling of the brain. Altera- 
tions of temperature can be demonstrated under surgical or experimental 
conditions only. More prominent and important than these signs is 
the so-called fifth sign of inflammation, or functio loesa, which may so 
affect consciousness at some stages as to remove the feature, pain, which 
is striking in inflammation elsewhere. 
In the subacute and chronic forms we meet a variety of reactions which 
have, until recent years, been little understood. Both the histological 
findings and the clinical pictures in these latter forms have much in 
common with the degenerative class of encephalic disease. The terminal 
effect of repair in several classes of encephalitic disease (inflammatory, 
traumatic, degenerative) is atrophy or induration (sclerosis, gliosis), 
or even porencephaly, which may offer few or no features to determine 
1 Jahrb. f. Kinderh., Leipzig, 1884, vol. xxii. 
2 Jour. Am. Med. Assn., 1904, xliii, 789. 
3 Am. Jour. Med. Sc., 1905, cxxix, 474. 
4 Boston Med. and Surg. Jour., 1904, cli, 647. 
5 Jour. Am. Med. J.ss., 1906, xlvi, 13. 
6 Ibid., 1906, xlvii, 1271. 
7 Jour, of Med. Research, 1908, vol. xix. ACUTE ENCEPHALITIS AND BRAIN ABSCESS 
361 
the nature of the original disease. These points partly explain why 
there has been so much confusion as to what the term encephalitis pre- 
cisely denotes.1 Encephalitis means, strictly speaking, an inflammation 
of brain substance; but we habitually include also inflamed brain tissue 
in which there is little or no sign of parenchymal change and the chief 
sign of injury is perivascular infiltration. Within the brain substance, 
we might well distinguish between (and do so denominate certain diseases) 
poli(o)-encephalitis (inflammation of gray matter) and leuh(o)-encephalitis 
(inflammation of white matter). 
Chronologically, we distinguish acute encephalitis (of which the types 
produced by staphylococci, streptococci, and pneumococci have been most 
studied), subacute encephalitis (of which the best examples are seen in 
syphilis and in tuberculous meningitis), chronic encephalitis (e. g., healed 
cases of epidemic meningitis), and progressive encephalitis as in dementia 
paralytica, now thought due to spirochsetes. 
Since the differentiation of thrombotic from inflammatory softenings, 
the development of entities in encephalitis has been as follows: 
Encephalitis congenita, Virchow, 1867. 
Spontaneous hyperplastic encephalitis, Hayem, 1868. 
Poliencephalitis acuta hemorrhagica superior, Wernicke, 1881. 
Acute infantile poliencephalitis (infantile cerebral palsy), Strumpell, 
1884. 
Acute primary encephalitis (adults), Strumpell, 1890. 
Pyogenic infective disease of the brain, Macewen, 1893. 
Encephalitis (Bacillus influenza), Nauwerck, 1895. 
Acute hemorrhagic encephalitis (Staphylococcus pyogenes aureus, pneu- 
mococcus), Southard and Keene, 1905 and 1906. 
The most extensive experimental work on the pathogenesis has been 
by Friedmann. The most influential systematic treatise since Huguenin, 
1876, has been Oppenheim's chapter in NothnageVs Handbuch, 1897, 
now in its second edition (Oppenheim and Cassirer, 1907). 
The picture of acute hemorrhagic encephalitis seems to have been 
produced by salvarsan (Fischer, Almkvist2) and even by mercury (Plotzl 
and Schuller). 
The etiological basis has, so far as possible, been adopted in the classi- 
fication which follows: 
Etiologically Well Characterized.-Influenzal Encephalitis. Pyogenic 
Encephalitis. (Hemorrhagic Superior Poliencephalitis, alcoholic form) 
Etiologically Doubtful, Clinically Definite.-Acute Hemorrhagic Encepha- 
litis, Strumpell type, often pyogenic (?), sometimes influenzal (?), or 
unknown in origin. Acute Bulbar Myelitis. Acute Encephalomyelitis. 
Babic Encephalitis (not considered in this article). 
Etiologically and Clinically Obscure.-Acute Non-suppurative Encepha- 
litis, Hayem type. Encephalitis in the Newborn. 
To these must be added other forms of such rare occurrence as to 
forbid systematization. Thus, Bacill.us typhosus (as well as other 
1 Nove-Josserand, These de Lyon, 1909-1910. 
2 Munch, med. Wochenschr., 1011, lix, 1803, 1809. 362 
DISEASES OF THE NERVOUS SYSTEM 
typhacese), Bacillus anthracis, and Trichinella spiralis have been found 
in encephalitis. The encephalitis following scarlet fever, measles,1 
and other exanthems is probably of pyogenic origin. The relation of 
encephalitis to gonorrhoea, mumps, and whooping-cough is still doubtful 
etiologically. 
Influenzal Encephalitis.-The great pandemic of influenza in 1889 to 
1890 led a few writers to suspect the influenzal origin of encephalitis.2 
The influenza bacillus was cultivated by Nauwerck from a case of enceph- 
alitis in 1893-94. He found the bacilli in section from a soft hemorrhagic 
spot in the cerebellum and in the sediment of the ventricular fluid. The 
organisms were cultivated from the ventricular fluid. Trouillet and 
Esprit3 published in the same year a report of pure cultures from the 
ventricular fluid in a disease which they term Meningo-encephalopathie 
grippale. It was suggested that influenza toxins might prepare the tissues 
for other organisms, notably for the pneumococcus, which might be 
recovered in pure culture from the meninges or brain substance. 
The atrium of infection in brain influenza may be: (1) The blood; 
(2) the nasal mucosa and the cribriform plate; and (3) the lymphatics of 
the middle ear. The nasal mucosa is now considered to be the most 
frequent route of infection, but it is probable that influenza is frequently 
a blood infection. Pfuhl4 found three cases of influenzal encephalitis 
in an epidemic of 93 cases of influenza in the barracks at Hanover. 
Encephalitis is not, as a rule, part and parcel of an attack of typical 
influenza, but follows recovery or partial recovery from such an attack. 
Sometimes there is an interval of several weeks before cerebral symptoms 
set in. In fact, the previous attack of influenza may be quite forgotten 
or considered as a cold in the head. Oppenheim remarks that the pre- 
monitory symptoms of influenzal encephalitis may be hard to interpret, 
since the headache, vertigo, nausea or vomiting, apathy, drowsiness, and 
general weakness may be regarded as merely incidents in convalescence 
from ordinary influenza. In other instances the onset may be acute. 
If there is anything which distinguishes this form of encephalitis, it is 
a gradually increasing loss of consciousness, taking several hours or a day 
for its completion. The patient can, as a rule, be roused by sharp 
stimuli, and the pupillary reflexes are normal. The projection system is 
not characteristically involved at the onset. Meningitic symptoms, 
such as stiffness of neck and opisthotonos, are exceptional. There is 
irregularity in the temperature, which is rarely high until the cerebral 
symptoms are well under way. Some of the febrile temperatures are 
perhaps due to intercurrent infections (Oppenheim). But these state- 
ments were made before the question of influenzal septicemia had engaged 
attention. The pulse is likely to be slow at first, but before death 
quicker, small, and irregular. 
The focal symptoms develop, as a rule, after coma has set in, not 
apoplectically, but one by one (Stadelmann's case with onset suggesting 
1 Marsh, Brit. Jour. Children's Diseases, London, 1910, vii, 20. 
2 Putnam, On Multiple Neuritis, Encephalitis, and Meningitis after Influenza, 
Boston Med. and Surg. Jour., 1892, vol. cxxvii. 
3 Sem. Med., 1895, No. 21. 4 Zeit. f. Hyg. u. Inf., 1897, Band xxvi. ACUTE ENCEPHALITIS AND BRAIN ABSCESS 
363 
cerebral hemorrhage is a rare exception). Moreover, in a limb about to 
become paralyzed there are often premonitory symptoms, such as weak- 
ness, numbness, or convulsions. Sometimes the convulsions involve 
the whole side of the body, when the eventual loss of power is to be a 
monoplegia. The paralyses may be quite masked by coma, or a difference 
in the two sides may be detected by choreiform movements on one side 
only. The variety of onset and course is so great that Leichtenstern 
felt that there are limiting cases in which a hemiplegia may develop 
without coma on a truly influenzal basis. Almost every possible focal 
symptom has been described in cases of encephalitis developing after 
influenza; but motor symptoms far outnumber sensory ones. 
It was formerly thought that the prognosis was grave, but Oppenheim 
is inclined to agree with Leichtenstern and Fiirbringer that many patients 
recover. The relation of influenzal encephalitis to spastic infantile 
hemiplegia cannot yet be stated; probably many of these cases are the 
relics of early coccal infections following exanthems. 
Pyogenic Encephalitis.-Macewen states that pyemic abscess of the 
brain may arise from infective embolism originating from pneumonic 
areas, fetid bronchitis and empyema, fetid pericarditis, infective com- 
pound fractures, rarely acute infective periostitis, and occasionally 
infective ulcers of the intestines and abdominal cavity. In his experience 
abscesses were rarely multiple except when originating in pyemia. 
Macewen makes mention of purulent encephalitis, red softening, and white 
softening. He describes purulent encephalitis as consisting of a swelling 
of the cerebral substance with extensive serous and leukocytal exudation 
and extravasation of red blood corpuscles, which he recognizes to be of 
very frequent occurrence in acute inflammations of the brain. The 
coalescence of minute hemorrhages gave rise to red softening according 
to Macewen. He made the observation that the pressure occasioned 
by the oedema and swelling of the brain "may aid in producing the pain 
experienced by the patient at the very outset of the encephalitis, especially 
when the ganglia of the fifth and its recurrent meningeal branches are 
included in the area of pressure." 
White softening is described by Macewen as due to infective embolism. 
He regarded microorganisms as the chief cause of intracranial pyogenic 
processes, and emphasizes the middle ear as an atrium of infection. 
He found the Streptococcus pyogenes and the Staphylococcus aureus 
most frequently in brain abscesses and in suppurative leptomeningitis. 
In pyogenic encephalitis there is an invasion (whether primary or 
secondary) of pyogenic bacteria. Although the pyogenes in the broad 
sense include numerous bacterial species, in the writer's experience only 
the Staphylococcus pyogenes aureus and (in a few instances) the pneu- 
mococcus have produced pure examples of hemorrhagic encephalitis. 
Encephalitis complicated by meningitis or meningitis complicated by 
encephalitis (conditions usually united under the term meningo-encephali- 
tis) is produced by the pneumococcus and streptococcus. In the 
writer's eight cases of pyogenic encephalitis the encephalitis was produced 
in six instances by Staphylococcus pyogenes aureus and in two .instances 
by the pneumococcus. A possibility in the pathogenesis is an acute 364 
DISEASES OF THE NERVOUS SYSTEM 
inflammatory lesion of the arachnoid villi of the longitudinal sinus.1 
If this could be shown to be the rule, one reason could be assigned for the 
frequent affection of the central gyri in this disease.2 
All the writer's cases of Staphylococcus pyogenes aureus brain infections 
have shown multiple hemorrhages in the brain. In two instances a 
condition which might be termed bacterial apoplexy has been produced 
in which rupture of a vessel and solution of surrounding brain tissue has 
been attended with intraventricular blood. 
The pneumococcus, although cultivated somewhat more frequently 
from the brain and meninges than the staphylococcus, is less likely to 
produce hemorrhagic encephalitis. 
In general, it may be stated that this disease occurs at all ages. Cases 
ranging from five to fifty years occur in the writer's series of pure hemor- 
rhagic encephalitis; cases of meningo-encephalitis range from four months 
to ninety years of age. Alcohol is an occasional antecedent factor. In 
children the exanthems frequently precede encephalitis, but encephalitis 
may appear to be a late complication of an exanthem. Arterial disease 
appears to have little or no relation to pyogenic encephalitis. The onset 
is more apt to be acute than gradual. The cases of gradual origin in the 
writer's series were more apt to be in older patients. The course is short, 
although variable, ranging from twenty-eight hours in a fulminant pneu- 
mococcus case to two weeks. The diagnosis of hemorrhagic encephalitis 
was not made in the writer's cases. The symptoms pointed to lesions of 
the brain in all cases except two, in which diagnoses of septicemia and of 
acute disease of unknown origin were made. Meningitis was considered 
in two of the Staphylococcus pyogenes cases, and in one of these suspicion 
of poisoning was raised. In the cases of meningo-encephalitis the diag- 
nosis of meningitis was frequent, and in 1 case a definite diagnosis of 
meningococcus meningitis was made. In 3 cases of meningo-encephalitis 
no other diagnosis than that of severe pneumonia was made. In the cases 
due to Staphylococcus pyogenes aureus the onset was sudden in 3 cases 
(hemiparesis and unilateral convulsions in 1; chilly feelings and frontal 
headache followed by ecchymoses in 1; malaise, manifold pains, ecchy- 
moses, and cerebral symptoms in 1). In 1 case the symptoms gradually 
supervened upon a pleurisy and orbital suppuration. 
The syndromes, as a whole, were of pronouncedly cerebral type in 2 
cases, of meningitic trend in 2 cases, of septicemic character in 1 case. 
Septicemia could be suspected in 3 cases in which superficial ecchymoses 
or suppuration became prominent. The temperature varied for the 
various cases; at times 103° and 104° for 2 cases; the temperature in 3 
cases was never high and frequently subnormal. The pulse was in no 
case subnormal, and, as a rule, ranged from above 100. The respira- 
tions were, as a rule, increased. 
The duration of the encephalitic symptoms cannot, in all cases, be 
closely reckoned; in 1 case three days; 1 case one week (after development 
of erysipelatoid condition) or three weeks (after injury to eye); 1 case, 
eight days; 1 case two weeks; 1 case fifteen days; 1 case unknown. The 
1 Southard and Sims, Jour. Am. Med. Assn., 1904, xliii, 789. 
2 Grasset et Rauzier, Maladies du systeme nerveux, 1894. ACUTE ENCEPHALITIS AND BRAIN ABSCESS 
365 
cases in which the cerebral syndromes were most clearly marked were of 
longer duration. 
The distribution of the brain lesions was as follows: Four cases showed 
areas of frank and somewhat voluminous hemorrhage, as a rule involving 
primarily the subcortical region (the vertex in 1, the occipital lobes in 2, 
and in 1 the pons). Three cases showed multiple ecchymoses and small 
abscesses of the cortex or subcortical region, with or without gross evidence 
of surrounding oedema. In 3 cases oedema of the pia mater was noted. 
In 2 cases an effusion of blood into the lateral ventricles was noted; in 
these cases the convolutions were flattened. In 2 cases, in which the 
temporal lobe was involved, the middle ear showed no lesion. 
A remarkable association of encephalitis with focal pulmonary lesions 
was made out in this series. Pulmonary lesions were either grossly 
prominent or found microscopically in all six cases. It is possible that 
this association is a mere matter of coincidence. 
Hemorrhagic Superior Poliencephalitis (Wernicke's Disease; Total Oph- 
thalmoplegia; Nuclear Ocular Palsy; Alcoholic Ophthalmoplegia).-First 
described by Gayet, 1875,1 as affection encephalique (encephalite diffuse 
probable), localized in the upper parts of the cerebral peduncles and in 
the optic thalami, as well as in the floor of the fourth ventricle and in the 
lateral walls of the third ventricle, the disease owes its name to Wernicke, 
whose cases were published in his Lehrbuch in 1881. The entity was 
further strengthened by Boedeker, who in 1892 summed up the findings 
in 11 cases (3 apparently non-alcoholic, and 8 alcoholic). The resem- 
blance of the disease to Korsakoff's polyneuritic psychosis was brought 
out by Bonhoeffer (1901).2 Schroder has shown that the disease, although 
a definite entity, is not a true encephalitis in the exudative sense. 
Wernicke defined the disease as an acute inflammatory disease of the 
nuclei of the eye muscle nerves, leading to death in from ten to fourteen 
days. The disease was characterized by palsy of eye muscles of rapid 
onset, quickly progressing to almost complete ophthalmoplegia (sphincter 
iridis and levator palpebrarum excepted). The patient's gait recalled 
that of alcoholism, showing a combination of stiffness and ataxia. Som- 
nolence was a striking feature, either at onset or toward the close. Where 
somnolence set in late, the disease at first recalled delirium tremens. 
Optic neuritis occurred in all Wernicke's cases. The course is not always 
so acute, for Gayet's case lasted five months, and other writers3 described 
cases with recovery. 
(a) Hemorrhagic Superior Poliencephalitis, Non-alcoholic.-These cases 
are very few. In Gayet's case the symptoms followed three days after a 
boiler explosion, in which, however, the patient, a man, aged twenty-eight 
years, sustained no bodily injury. Paraphasia and paragraphia were the 
first symptoms. General weakness, apathy, and marked drowsiness 
followed. The face became mask-like, and there was double ptosis with 
a general atony of the facial musculature, so that in speech there were no 
expressive facial movements. General weakness was such that the 
1 Archives de Physiologic, 1875, vol. vii. 
2 Monatsschr. f. Psych, u. Neur., 1899, v, 265. 
3 Wolfe, Jour, of Nerv. and Ment. Dis., 1894; Wiener, Brain, 1897, xx, 458. 366 
DISEASES OF THE NERVOUS SYSTEM 
patient could scarcely stand or grasp objects. Pupils, vision, and all 
kinds of sensation were quite normal. There was almost complete 
paralysis of both oculomotor nerves. There was later transitory hemi- 
plegia, which disappeared, leaving the patient in a state of excitement, 
with more facial expression and pain in the right leg. Conditions varied 
thereafter. Intelligence was maintained, and there were never any 
considerable changes in the eye grounds. Emaciation followed with 
incontinence and decubitus; death occurred five months after the 
accident. 
Morbid sleepiness also characterized Wernicke's first case, a young girl, 
who had attempted suicide with sulphuric acid. The sleepiness, ataxia, 
optic disturbances, and vertigo came on two months after the suicidal 
attempt. Oculomotor palsy, disorientation, and hebetude were the main 
features of the attack, which led to death twelve days after the onset. 
Salomonsohn's case showed loss of knee-jerks and slowing of the pulse, 
in addition to other characteristic symptoms. Luce's case was incidental 
in miliary tuberculosis. Murawieff's case followed three months after 
influenza, and showed also sarcoma of one precentral gyrus. 
(&) Hemorrhagic Superior Poliencephalitis, Alcoholic.-This is closely 
allied in symptomatology with Korsakoff's disease and with alcoholic 
polyneuritis. Some writers believe that the symptoms in the three 
conditions scarcely represent entities, but are all incidents in chronic 
alcoholism. If this be true, it is doubtful whether Wernicke's disease 
is truly encephalitis in the exudative sense, and, in fact, Schroder was 
able to find in a typical case, merely, (a) destructive lesions due to 
minute hemorrhages, and (b) reparative changes (phagocytic cells, 
vascular and neuroglial changes) like those following any non-inflamma- 
tory injury. Schroder inclines to the belief that alcoholism sets up some 
kind of vascular change such that, under conditions of unknown character 
involving severe brain injury, capillary hemorrhages ensue in various 
parts of the brain. The region beneath the corpora quadrigemina is 
the locus of election for such hemorrhages. It is possible that this 
tendency is related to the characteristic hemorrhages in anaphylaxis. 
Subjects having this symptom complex are often brought to a hospital 
as cases of delirium tremens. The victims are usually male whisky 
drinkers of middle age, who have been suffering for months or years from 
chronic alcoholism. For some days or weeks, headache, pains in various 
parts of the body, vertigo, and vomiting may precede the ophthalmo- 
plegia. As a rule, the ophthalmoplegia is incidental in a characteristic 
alcoholic delirium, which otherwise differs from the ordinary delirium 
tremens in the well-marked mental and bodily weakness of the patient. 
The ophthalmoplegia is not absolute nor always an associated palsy. 
Sometimes the pupil and the levator palpebra? superioris are involved as 
well. Nystagmus is frequent. Wilbrand and Sanger1 pointed out that 
bilateral ptosis occurs in about one-half the cases, although it is not an 
essential feature. One case in six shows normal vision and unaltered 
eye grounds. The eye ground changes when found, consist either of a 
1 Die Neurologic des Auges, Handbuch, 1900, vol. i. ACUTE ENCEPHALITIS AND BRAIN ABSCESS 
367 
slight neuritis with retinal hemorrhages, or of a pallor of the temporal 
halves of the papillae (sign of an alcoholic amblyopia due to chronic 
neuritis of the papillomacular fibre bundle of the optic nerve). It is 
difficult to distinguish the rest of the picture from that in chronic alco- 
holism. Muscular weakness, indistinct schlaftrunken (Wernicke) speech 
and a gait recalling the cerebellar gait are characteristic. 
In the differential diagnosis brain tumor (especially of the posterior 
cranial fossa, particularly in the cerebellum) is suggested by the gait and 
the eye-ground changes. The course is perhaps more likely to suggest 
apoplexy or focal encephalomalacia. Not only the gait and the non- 
febrile course, but also the alterations of consciousness may support such 
a diagnosis; and, in the light of Schroder's histological observations, the 
disease may perhaps be best regarded as a peculiar kind of apoplectic 
disease in which multiple minute hemorrhages affect a particular area. 
The optic neuritis and the symmetry of the ophthalmoplegia eliminate 
any question of the classical forms of apoplexy. The ocular palsies of 
botulism (bilateral paralysis or paresis of accommodation, mydriasis, 
ptosis) present some resemblances to those of Wernicke's disease. 
Acute Hemorrhagic Encephalitis, Strumpell Type.-It is probable that 
many of the cases of acute hemorrhagic encephalitis which Strumpell 
predicted in 1884 and described in 1890 are of pyogenic origin. Strum- 
pell's first cases showed at autopsy a bilateral bronchopneumonia and a 
markedly enlarged spleen, in addition to the encephalitis. Cultures 
from the brain lesions proved negative. Strumpell, after dismissing the 
hypothesis that his cases belonged to the epidemic meningitis group, 
assumed that there must be a specific agent for what he conceived was 
a primary local infection. 
It seems justifiable to put all primary cases of unknown etiology 
provisionally into this group, which further investigation will undoubtedly 
resolve. The newer ideas about septicemia have so altered our point 
of view that one naturally hesitates to classify any encephalitis as primary, 
on the ground that it may result from a blood infection. Since Land- 
steiner and Popper, and Flexner have successfully transferred anterior 
poliomyelitis from man to monkeys, there is a possibility that not only 
anterior poliomyelitis, but also acute encephalitis and Landry's paralysis, 
may be due either to an ultramicroscopic organism or to protozoa. 
Noguchi reports successful cultivation of minute organisms. 
Orazio D'Allocco described six cases of acute poliencephalitis in children 
(one with autopsy). The onset in these cases was sudden, attended with 
general infectious symptoms. There was a continuous generalized tonic 
spasm of all the muscles, including those of respiration and of the head, 
with continual uniform almost rhythmical spasms. The limbs were 
affected symmetrically, as a rule, but the spasm may be confined to one 
limb. Spasms are more marked near the head. Muscular excitability 
is increased. Anatomically, an endophlebitis and an endarteritis leading 
to vascular occlusion and degenerative or inflammatory lesions were 
found. Henning, 1914, brings evidence of four groups of atypical cases, 
(a) epileptiform, (&) pseudotumor, (c) resembling multiple sclerosis, (d) 
psychotic. 368 
DISEASES OF THE NERVOUS SYSTEM 
Acute Bulbar Myelitis (Acute Inferior Poliencephalitis; Acute Inflammatory 
Bulbar Palsy). - Acute inferior poliencephalitis has more verbal than 
substantial resemblance to acute superior poliencephalitis, since the non- 
inflammatory hemorrhagic character of the latter (Schroder) is replaced 
in the inferior form by acute inflammatory lesions. These lesions recall 
closely those of anterior poliomyelitis; in fact, many cases of anterior 
poliomyelitis show bulbar involvement, so that some writers believe that 
acute inferior poliencephalitis is a disease closely allied to anterior polio- 
myelitis-possibly an identical infection with an unusual localization. 
W. Pasteur's1 epidemic of seven cases of paralysis occurring in children 
(ranging from eighteen months to eleven years of age) in one family 
is often cited in this connection, since some of the cases were cerebral 
and some spinal. At all events, whether the analogy of anterior polio- 
myelitis is accepted or the frequent association of bulbar myelitis with 
influenza epidemics be taken as a hint at the etiology, the disease acute 
bulbar myelitis is very different from Wernicke's disease both in site and 
in character. Histologically, there is evidence of acute destruction of 
nerve tissue, not at all confined to the gray matter, together with vascular 
congestion and lymphocyte accumulations in the perivascular sheaths. 
Not only do these lesions affect white matter as well as gray, but the 
meninges are frequently involved. 
The onset is rapid, but not apoplectiform. General malaise, headache, 
vomiting, and sometimes fever precede the local signs of bulbar involve- 
ment. The characteristic delirium or sleepiness of Wernicke's disease is 
not found. As a rule, in from three to seven days there is difficulty in 
swallowing, with paralysis of the tongue and palate, facial paralysis, and 
paralysis or paresis of vocal cords. 
The course depends upon the locus of the inflammatory foci, which 
may directly involve a greater or smaller number of the gray'nuclei 
(nuclear form of palsy), in which there is least hope of recovery, or may 
affect the nerves in their intrabulbar course (intranuclear form), in which 
the prognosis is less grave. On account of the ill-defined limits of the 
inflammatory process, any given case is somewhat likely at its height to 
show a combination of nuclear, intranuclear, and even supranuclear 
involvement. If the subject does not become unconscious, the course is 
said to be more favorable. The prognosis is not so unfavorable as was 
formerly thought; death occurs in about one-half the cases. The possible 
relation of certain cases of recovery to multiple disseminated sclerosis 
has been mentioned (Oppenheim). 
In differential diagnosis, syphilis, hemorrhage, and softening of the 
bulb must be strongly considered, whereas tumor, tubercle, and abscess 
are less likely to cloud the issue. Syphilis is ordinarily attended by basal 
meningitic symptoms. Hemorrhage and softening have a sudden or 
rapid onset which is not characteristic of encephalitis. Some cases with 
persistent bulbar phenomena have been attributed to encephalitis; thus, 
for example, Huet and Lejonne2 attribute a facial palsy and hemiatrophy 
1 Clin. Soc. Trans., London, 1897, xxx, 143. 
2 Rev. Neurol., Paris, 1906, xiv, 105. ACUTE ENCEPHALITIS AND BRAIN ABSCESS 
369 
of the tongue to the effects of a former acute inferior poliencephalitis 
attacking the nuclei of the facialis and hypoglossus of the right side. 
Acute Encephalomyelitis and Poliencephalomyelitis.-These names have 
sometimes been given to cases of anterior poliomyelitis complicated by 
lesions higher than the spinal cord; but there are other cases in which the 
element of anterior poliomyelitis is not so prominent. Possibly cases of 
Wernicke's disease with a number of hemorrhages below the level of the 
quadrigemina have yielded so complex a picture as to deserve this name. 
Cases of acute bulbar myelitis have been not frequently complicated by 
lesion higher than the bulb. A tendency of the symptoms to descend, 
possibly beginning with ophthalmoplegia, going on with bulbar symptoms, 
and ending with limb disorders, has been noted. There is great irregu- 
larity in the degree to which the various portions of the involved apparatus 
are diseased. Whereas total ophthalmoplegia and glossopharyngolabial 
palsy, associated with spinal palsy, are the symptoms of the disease in 
its full development, yet in cases in which the ophthalmoplegia is complete 
the bulbar trouble may be limited to slight facial palsy; and in cases in 
which the glossopharyngolabial palsy is complete there may be no more 
than ptosis to express the suprabulbar lesion. 
When the cerebral white matter and the included nerve paths are 
involved, it seems better to name the disease encephalomyelitis. The 
disease has foci so widespread in some instances as to lose its usual purely 
motor character (Taylor1 and Oppenheim). These cases vary extremely 
in outcome, some dying, some recovering, and some remaining with 
defects. The probable course of several weeks or longer should possibly 
align them rather with the slower degenerative processes than with acute 
encephalitis. General cerebral symptoms are few or confined to the 
drowsiness and weakness characteristic of Wernicke's disease. Fever 
sometimes occurs. The facies is said to be characteristic (Hutchinsonian; 
eyeballs immobile, ptosis, elevated eyebrows), together with relaxation 
of the lower face, flattening of nasolabial folds, loose-hung limbs. 
A case of Combe's2 illustrates the peculiar combinations of symptoms 
possible in encephalitis with multiple foci. It is said to have followed 
influenza. That form of alternate paralysis known as Webers syndrome, 
viz., right hemiparesis and left ophthalmoplegia, appeared in a boy, aged 
two years. Weir Mitchell's posthemiplegic choreiform movements followed 
on the right side, but associated with spasmodic movements in the field 
of the left facial and abducent nerves, amounting to an alternating 
hemichorea. Later there was weakness of the right oculomotor nerve. 
Such hemiparesis with crossed paralysis of the oculomotor nerve with 
tremors of the paralyzed parts corresponds with Benedikt's syndrome 
(due to a lesion of the cerebral peduncle). But the involvement of the 
left facial and abducent nerves, together with the right-sided hemiparesis, 
amounts to the Millard- Gubler syndrome. The patient, therefore, suffered 
from a combination of Benedikt's syndrome and the Millard-Gubler 
syndrome due to a lesion of the pons near the left pyramidal tract (this 
new syndrome Combe proposes to term the Inferior Benedikt syndrome}, 
1 Boston Med. and Surg. Jour., 1903, cxlviii, 634. 
2 Syndrome de Benedikt inferieur, Rev. Mens. d. mat. de I'enfance, 1904, I, xxii, 1, 
vol. v-24 370 
DISEASES OF THE NERVOUS SYSTEM 
but in addition showed a complication by incomplete ophthalmoplegia, 
due to a separate lesion situated in the oculomotor nuclei. 
The diagnosis of such multiple circumscribed lesions and the determina- 
tion of their encephalitic nature is a matter for the future. At present 
the general diagnosis must be made largely by elimination of vascular 
and other focal possibilities and on the ground of the course of the disease. 
The poliencephalitis here mentioned evidently has considerable alliance 
with the degenerative diseases of these loci. E. W. Taylor distinguishes 
the following in this field: Encephalitis, encephalomyelitis, acute ophthal- 
moplegia, acute superior and inferior poliencephalitis, acute or apoplec- 
tiform bulbar myelitis, poliencephalomyelitis, and poliomyelitis. 
It may be mentioned at this point that Batten1 proposes to group 
anterior poliomyelitis with encephalitis, and neglecting the preemption 
of the term "superior poliencephalitis," or Wernicke's disease, Batten 
classifies as follows: Acute poliencephalitis superior, including cases 
with frontal, central, occipital, and cerebellar localization; acute polien- 
cephalitis inferior, including cases in which cranial nerve nuclei are 
affected; acute poliomyelitis anterior. Batten regards both brain and 
cord disease as due to thrombosis of fine terminal vessels due to an 
unknown organism or toxin. Batten's classification is not yet proved, 
although there are some cases in which there is a thrombosis of the'fine 
terminal vessels (a prominent feature in pneumococcus and streptococcus 
brain infections). It is not proved that thrombosis plays any part in 
the disease anterior poliomyelitis. 
Acute Non-suppurative Encephalitis (Acute Hyperplastic Encephalitis, 
Hayem).-Among non-hemorrhagic forms, Hayem's hyperplastic type is 
least well recognized and is probably very rare, except in the sense that 
hyperplastic or proliferative phenomena are the invariable concomitants 
of all not too severe destructive injuries in the central nervous system. 
Encephalitis in the Newborn (Encephalitis Congenita; Encephalitis Inter- 
stitialis Congenita; Encephalitis Neonatorum; Steatose Interstitielle Diffuse 
de 1'Encephale).2-This occurs at times as the result of septic infection of 
gastro-intestinal or pulmonary origin (Fischl). A case is on record in 
which a pathogenic strain of Staphylococcus pyogenes albus was grown 
from the focal lesions (Fischl). Encephalitis may complicate the picture 
of syphilis in the newborn, although spirochaetes are seldom demonstrable 
in the nervous system, even in congenital syphilis. 
Aside from pyogenic, syphilitic, or other recognized forms of encephali- 
tis in the newborn, there is another form, the encephalitis congenita of 
Virchow, which is still sub judice. Virchow believed that he had learned 
the cause of death in a considerable proportion of stillborn and newborn 
infants, attributing it to encephalitis and myelitis of congenital origin. 
The only analogies for this form of encephalitis were findings in the brains 
of children of variolous and syphilitic mothers. In general, he regarded 
congenital encephalitis as a brain disease analogous with the parenchy- 
matous changes of the liver and kidney in the exanthemata. 
Hayem, 1868, promptly criticised Virchow's findings, noting the 
1 Lancet, 1902, vol. ii. 
2 Archives de Physiologic, 1868, vol. i. ACUTE ENCEPHALITIS AND BRAIN ABSCESS 
371 
extreme frequency of fat-laden cells in the brains of the newborn. In the 
same year Parrot published a piece of work, since largely neglected, on 
what he termed diffuse interstitial steatosis of the encephalon, and offered 
clinical histories which proved to Parrot's satisfaction that these changes 
were due to lack of food. Parrot's results were reprinted without marked 
alteration in his monograph on athrepsia in 1877.1 
Jastrowitz regarded the fatty cells figured by Virchow and by Parrot as 
incidental in the development of the brain. From an extensive material 
(65 cases), Jastrowitz stated that these cells are found in small numbers 
in the third month of fetal life, in greater numbers in later months, but as 
a universal finding in the cerebral white matter only in the newborn. 
The locus of election for the granule cells is the white matter, and 
especially the corpus callosum and its processes. The occurrence of 
groups of granule cells outside these usual loci, e. g., in the gray matter of 
the cortex or in the basal ganglia, was regarded by Jastrowitz as patho- 
logical. The pathological fat-cell groups were thought by Jastrowitz 
to be due rather to infections and various maternal diseases than to 
developmental errors or atrophy (Parrot). 
Modern opinion inclines to the view that the focal form, in which not 
only masses of fat-laden cells, but also injury of nerve fibres, can be 
demonstrated is the true congenital encephalitis. A case of the writer's 
has shown how streptococci may bring about in the newborn a reaction of 
the mononuclear cells rather than of the as yet ill-developed polynuclear 
cells in the meninges. Possibly this finding explains some of the cellular 
peculiarities of congenital encephalitis. 
Summary-There is a form of encephalitis in the newborn which is 
at times a cause of stillbirth or of death soon after birth, and is due to 
various factors (sometimes to pyogenic infection, sometimes to syphilis, 
and sometimes to unknown intoxications), perhaps more often maternal 
than non-maternal. The disease is histologically characterized by focal 
lesions of the cerebral white matter (sometimes visible in the gross) 
showing nerve fibre alterations and accumulations of fat-containing cells. 
Protracted cases may yield a neurological picture of irregularly dis- 
tributed convulsions and pareses, respiratory rather than cardiac disorder, 
and initial, not continued, fever. 
Experimental Encephalitis.-The field of experimental encephalitis has 
not been thoroughly worked.- Coen2 demonstrated mitotic figures in the 
nerve cells surrounding the region of necrosis. Friedmann described 
varying phenomena of experimental encephalitis, which in 1904 he 
summed up by saying that "former distinctions of encephalitis into 
'forms' are unnecessary," since sufficiently strong stimuli (meningococcus, 
influenza bacilli, embolism, aseptic trauma, corrosive substances or heat) 
can bring about all the different conditions which have been termed 
encephalitis. In the more severe cases, according to Friedmann, all 
histological combinations of encephalitic processes may be found ranging 
from small-cell infiltrations to areas of softening with granule cell deposits 
1 Clinique des nouveau-nes. L' Athrepsie. Lemons recueillies par le Dr. Troisier, Paris, 
1877. 
2 Ziegler's Beitr. z. pathol. Anat., 1887, Band ii. 372 
DISEASES OF THE NERVOUS SYSTEM 
and extensive areas of proliferation with giant cells. Various stages and 
degrees of these processes have been hemorrhagic, parenchymatous, and 
hyperplastic encephalitis. 
After small traumatic lesions fibrillary sclerosis may follow; after more 
extensive lesions cellular proliferation is prominent. In cases of hema- 
togenous origin, Friedmann emphasizes a primary infection of the small 
bloodvessels. These have been shown by Southard and Keene, and 
Southard and Stratton, to characterize pneumococcus and streptococcus 
infections of the brain. Koeppen1 described one end result as a kind 
of etat crible, in which the fibrillar gliosis has been insufficient to fill the 
space of the destroyed tissue. In other cases a dense sclerosis follows. 
Experimental work upon bacterial encephalitis scarcely admits sys- 
tematization. Work upon the peripheral nerve roots and the method 
of invasion of the central nervous system by bacteria has been done by 
Homen and Laitinen, and by Orr and Rows.2 It is evident from this 
work that more attention must be paid to the lympahtic route of possible 
infection. The writer and associated workers have produced experi- 
mental brain infections in a variety of ways, using several varieties of 
pyogenic bacteria. They have produced meningitis, encephalitis, and 
ependymitis, but so far neither a pure encephalitis nor the important 
lesions of the small vessels found in many cases of adult encephalitis has 
been produced in the guinea-pig. The time relations of the experimental 
brain infections in the guinea-pig are: Staphylococcus pyogenes aureus, 
intrapulmonary injections, after six hours, focal polynuclear exudation 
in the meninges; after twelve hours, ependymitis; after twenty-four 
liours, slight polynuclear leukocytosis in the meninges; after forty-eight 
hours, subependymal leukocytosis and moderate encephalitis (leukocytes 
in apposition with nerve cells). After three days meningitis, encephalitis, 
and ependymitis at their height; after four and five days lymphocytosis 
and phagocytosis; after six days, phagocytosis for leukocytes; after nine 
days very slight polynuclear leukocytosis, slight evidence of phagocytosis. 
After one month the tissues are negative, except for slight fibrillar gliosis, 
pigment-bearing phagocytes, lymphoid and plasma cells, and eosinophilic 
polynuclear leukocytes. 
In work upon the pneumococcus, postorbital inoculations were 
employed. Many strains of pneumococcus gave no lesion. Polynuclear 
leukocytosis was noted as early as six hours. The exudate is at its height 
in three, four, or five days, and is largely confined to the meninges. Some 
of the strains produced lesions in which mononuclear cells were more 
prominent than polynuclear cells. Work with the streptococcus produced 
results analogous to those with the pneumococcus. 
Southard and Richards investigated guinea-pig brain tissues after 
postorbital inoculation of Bacillus typhosus. In contradistinction to the 
early crisis of the aureus brain inflammations (three days) and the pneu- 
mococcus inflammations (four days), it was found that the Bacillus 
typhosus produced a critical phase from the fifth to the seventh day. 
1 Arch. f. Psych., 1898, Band xxx. 
2 Brain, 1904, p. 460; Review Neurol. Psych., 1906, iv, 25; Brit. Med. Jour., 1907, 
i, 987; Review Neurol. Psych., 1907, v, 345. ACUTE ENCEPHALITIS AND BRAIN ABSCESS 
373 
Mononuclear elements appear in the pial meshes in the typhosus inocula- 
tions by the seventh day. By the tenth day fresh exudative cells have 
ceased to appear. By the fourteenth day the disease has virtually 
passed and traces only of the old exudate are found. 
Special work has been done by Cantani on the effect of influenza bacilli 
on the central nervous system of rabbits. He was able to kill rabbits 
by brain injection of influenza bacillus in proper amount and of proper 
virulence. The first symptoms appeared nine to ten hours after injection. 
Paraplegia of crural type was gradually followed by paralysis of all 
extremities and convulsions. The autopsies showed evidence of severe 
intoxication throughout the trunk, as well as meningitis, encephalitis, 
and ependymitis, with demonstrable influenza bacillus. Injections of 
killed bacilli into the brain in larger amounts produced the same results. 
In connection with experimental encephalitis more work is desirable 
in animals. Encephalitis in horses has been long recognized; but it 
is commonly held that equine encephalitis has a very manifold etiology. 
Diagnosis.-The diagnosis of acute encephalitis is not often success- 
fully made, and often not made at all. Perhaps the diagnosis is rendered 
more accurately in children's clinics than elsewhere, owing to the interest 
attaching to the Strumpell type. Nevertheless, in the majority of cases 
the course is either so acute as to offer little chance of diagnosis, or else 
is such as to warrant a diagnosis of " cerebral disease." In a large number 
of the writer's series of cases of encephalitis and meningo-encephalitis the 
diagnosis of cerebral disease was made without attempt to differentiate 
between hemorrhagic encephalitis, massive cerebral hemorrhage, men- 
ingitis, and brain syphilis. The attempt of Oppenheim to systematize 
encephalitis has been so successful that the diagnosis will undoubtedly 
be made more frequently in the future. 
There are few practical difficulties connected with the diagnosis of 
hemorrhagic superior poliencephalitis, alcoholic form, since the history of 
alcoholism, the acute onset, the associated ocular palsy, the somnolence, 
delirium, the general weakness (and, as a rule, optic neuritis), stamp the 
disease. Although Oppenheim sought to reduce hemorrhagic superior 
poliencephalitis, alcoholic form; hemorrhagic encephalitis, Strumpell 
type; and influenzal encephalitis to a single entity on clinical grounds, 
it does not appear that this position is warranted. Bonhoeffer's clinical 
claims and Schroder's histological observations go far to show that 
Wernicke's disease is a rare but perfectly definite disease. 
Although Wernicke's disease presents few or no difficulties in diagnosis, 
the Strumpell type of acute hemorrhagic encephalitis, the form termed in 
this article pyogenic encephalitis, and influenzal encephalitis give con- 
siderable difficulty. The endeavor should be made in all doubtful acute 
"cerebral" diseases to establish or eliminate an influenzal origin. 
Influenzal encephalitis, it must be remembered, occurs after rather than 
during an attack of influenza. Cases occurring in epidemics are more 
frequently diagnosed than sporadic cases. Lumbar puncture proves of 
considerable aid. The data of Cohen's work on septicemic meningitis 
must also be considered.1 
1 Annates de VInstitut Pasteur, 1909. 374 
DISEASES OF THE NERVOUS SYSTEM 
Since many of the cases may turn out to be of pyogenic origin and of 
pyemic nature, it is probable that blood cultures will aid extremely 
in the diagnosis. The etiological data of cultures from lumbar puncture 
fluid and from the blood will not point unequivocally to encephalitis. 
On theoretical grounds the positive lumbar puncture culture will point 
rather to meningitis or to meningo-encephalitis than to a pure encephalitis. 
Close attention must be paid to all the clinical signs. If we group 
together influenzal encephalitis, pyogenic encephalitis, and hemorrhagic 
encephalitis of unknown origin (termed in this article the Strumpell 
type), the chief conditions to be considered in differential diagnosis are: 
(1) Other intracranial infective diseases, such as meningococcus menin- 
gitis, tuberculous meningitis, pyogenic meningitis, and sinus thrombosis; 
(2) brain tumor and brain syphilis; (3) cerebral hemorrhage and focal 
encephalomalacia; (4) certain conditions of cerebral intoxication resemb- 
ling meningitis but anatomically negative; and (5) rarely acute poisoning, 
hysteria, and other diseases. 
The differential diagnosis in the first group, intracranial infective 
diseases, is greatly aided by lumbar puncture with examination of the 
cells and bacteriological examination. This, in doubtful cases, should 
include an attempt to grow the influenza bacillus on blood media. The 
finding of blood in the lumbar puncture fluid may suggest chronic internal 
hemorrhagic pachymeningitis and cerebral hemorrhage as well as acute 
hemorrhagic encephalitis. Nor will every case of hemorrhagic enceph- 
alitis show blood or leukocytes (Lucas and Southard) in the cerebrospinal 
fluid. A careful cytological examination should be performed. Examina- 
tions for the tubercle bacillus or meningococcus and other pyogenic 
bacteria should be made. A lumbar puncture fluid without leukocytes, 
blood, or bacteria must not be considered as excluding the possibility of 
hemorrhagic encephalitis. 
Stiff neck, retraction of the abdomen, and hyperesthesia are not 
common in encephalitis. Herpes has not been reported. Paralysis of 
eye muscles is exceptional. Nevertheless, on account of the extensive 
variation in foci affected by the multiple lesions of hemorrhagic enceph- 
alitis, it is improbable that there is any sign of meningitis which may not 
also occur in some cases of hemorrhagic encephalitis. The diagnosis 
frequently depends on the association of a monoplegia or hemiplegia 
with successive involvement of the paralyzed parts or aphasia coming on 
early. Similar phenomena in meningitis are frequently complications 
or sequelae rather than central factors. The acute onset, the drowsiness 
or tendency to coma, vomiting, convulsions, and optic neuritis offer no 
points in differential diagnosis against meningitis, nor can much weight 
be placed upon the condition of the pulse or respiration. 
As against sinus thrombosis the stasis of blood in the veins of the face 
has been mentioned by Macewen, but this is not always present. Sinus 
thrombosis runs a very acute course. The differentiation often fails, 
except in middle-ear disease. 
With respect to the differential diagnosis from brain tumor, the onset, 
the fever, and rapid course are important. Hemorrhagic encephalitis 
rarely shows an altogether characteristic tumor syndrome. Oppenheim ACUTE ENCEPHALITIS AND BRAIN ABSCESS 
375 
especially warns against the immediate diagnosis of brain tumor in cases 
showing attacks of cortical epilepsy. Similar remarks apply to brain 
syphilis when it takes the form of gumma. Brain syphilis in the form of 
chronic meningitis also runs a quite different course from that of typical 
hemorrhagic encephalitis. Those rare cases of brain syphilis in which the 
disease is essentially an encephalitis must give rise to difficulty, except 
when a history of syphilis can be established. Since these cases occur in 
secondary syphilis, the differential diagnosis should not prove difficult. 
Cerebral hemorrhage and focal encephalomalacia were formerly fre- 
quently diagnosed in cases of hemorrhagic encephalitis. In young persons 
with healthy cardiovascular systems the diagnosis of cerebral hemorrhage 
or thrombosis may not come into question; but, since hemorrhagic 
encephalitis occurs in older persons, there is a possibility of confusion. 
The cases termed by the writer bacterial apoplexy occurred in subjects 
twelve and twenty-two years of age. In such cases the differential 
diagnosis between hemorrhagic encephalitis and cerebral hemorrhage 
would be impossible on theoretical grounds. Practically the paralyses 
which characterize hemorrhagic encephalitis do not occur simultaneously 
with the onset of drowsiness or coma. The signs of severe cerebral 
disease are obvious long before paralyses or convulsions have set in. 
The paralyses of hemorrhagic encephalitis may increase in intensity from 
time to time in the course of several hours or a day, presumably owing to 
the gradual development of inflammatory lesions with oozing of blood. 
The absence of fever and of other signs of sepsis will indicate rather a 
cerebral hemorrhage or focal encephalomalacia due to arterial disease. 
Cerebral intoxication without anatomical signs (the so-called pseudo- 
meningitis of Krannhals1) gives rise to the diagnosis of encephalitis, 
although more often to the diagnosis of meningitis in some cases. Krann- 
hals' cases occurred in the influenza pandemic of 1889 to 1890. The 
occurrence of focal symptoms would ordinarily exclude this diagnosis. 
Poisoning was considered in one of the writer's cases (Staphylococcus 
pyogenes aureus) of bacterial apoplexy, which ran an acute course of 
three days. Attention to the acute infective feature of encephalitis will 
usually exclude the diagnosis of hysteria with certainty. Uremia and 
acetonemia may need to be considered. 
Flachs2 sums up points in differential diagnosis as follows: 
Encephalitis: Localizing symptoms (monoplegia, hemiplegia, aphasia), tendency to 
recovery. 
Meningitis: Retraction of abdomen; hyperesthesia; eye-muscle paralysis, persis- 
tent rigidity of neck. 
Tumor: Headache, optic neuritis, slowed pulse, slow course. 
Sinus Thrombosis: Stasis phenomena, acute course. 
Pseudo-meningitis: Severe symptoms; high fever with nerve symptoms. 
Encephalitis in Advanced Years.-There is neither predisposition nor 
lack of predisposition to hemorrhagic encephalitis in old age. The 
diagnosis as against focal encephalomalacia or cerebral hemorrhage is 
naturally difficult, especially since febrile conditions (bronchopneumonia) 
1 Deut. Arch. f. klin. Med., 1895, liv, 89. 
2 Fortschr. d. Med., 1910, xxviii. 376 
DISEASES OF THE NERVOUS SYSTEM 
may apparently lend infective features to focal encephalomalacia. 
Hoppe-Seyler1 mentioned hemorrhagic encephalitis as a rare complication 
of pneumonia in the aged, and Siemerling, in the same Lehrbuch, mentions 
difficulty in differential diagnosis between sinus thrombosis and enceph- 
alitis. Lhermitte has described a "paraplegic dementia" on the basis 
of chronic changes, apparently degenerative and not exudative.2 
Encephalitis in Infancy and Childhood.-The possible medicolegal 
significance of congenital interstitial encephalitis was mentioned by 
Virchow. Possibly this question should be raised more often in cases of 
"overlying." Pediatrics practically distinguishes acute encephalitis in 
infants and a form of encephalitis in older children which shows more 
tendency to recovery. Acute and frequently fatal encephalitis of infants 
is regarded as a primary encephalitis with origin unknown except in 
certain pyogenic cases. These cases show high fever, stupor or coma, 
convulsions at the onset as well as later, superficial breathing with 
occasional Cheyne-Stokes episodes, a rapid, feeble pulse, and sometimes 
bulging of the fontanelles. The neck and limbs are stiff and flexed. 
Strabismus sometimes occurs. The occurrence of slight paresis or mono- 
spasm of a limb or of the facial nerve is characteristic. The endeavor 
should be made to determine or eliminate a pyogenic origin. Lumbar 
puncture findings are helpful as against a meningococcus meningitis. 
The encephalitis of older children is often secondary to exanthemata, 
diphtheria, or pertussis, and has an insidious origin sometimes suggestive 
of brain tumor. Recovery is the rule in these cases, which sometimes 
precede the development of frank epilepsy. The child is immobile 
except for tetanic spasm; the neck is stiff, and opisthotonos may suggest 
meningitis. Tremor of the limbs, facial palsy, monoplegia, aphasia, 
convergence or conjugate deviation of the eyes, and optic neuritis may 
occur. James Taylor3 has given valuable data, and distinguishes the 
following varieties of infantile cerebral palsy: 
Hemiplegic forms, resulting from enceph- 
alitis, vascular lesions and injury. 
Acute encephalitis, infantile hemiplegia, 
infantile monoplegia, idiocy with cranial 
asymmetry, choreiform hemiparesis, 
hemiathetosis. 
Generalized rigidity, idiocy with general 
rigidity, paraplegic rigidity, spastic 
facial diplegia, amaurotic family idiocy, 
bilateral athetosis, choreiform diplegia, 
epileptiform myoclonus. 
Diplegic forms, resulting from death of 
cortical cells. 
Only the diseases under hemiplegic forms are considered by Taylor 
to be encephalitic in origin, and of these, infantile (cortical) hemiplegia 
is the most important. The conditions found, in order of frequency, are 
acute encephalitis, thrombosis (arterial or venous), hemorrhage, and 
embolism. Alzheimer called attention to the relation between idiocy 
and encephalitis. According to Taylor the victims of infantile hemiplegia 
1 Schwalbe's Lehrbuch der Greisenkrankheiten, 1909, p. 236. 
2 Sem. Med., Paris, 1910, xxx, No. 50, 585. 
3 Paralysis and Other Diseases of the Nervous System in Childhood and Early Life, 
1905. ACUTE ENCEPHALITIS AND BRAIN ABSCESS 
377 
are healthy and without hereditary stigmata at onset, and undergo 
between the ages of one month and six years severe symptoms, consist- 
ing of fever, convulsion, and often vomiting and coma. After a period 
varying from a day to a week the attack becomes less severe, but hemi- 
plegia comes on first in the face, then in the arms, and lastly in the leg. 
This hemiplegia is flaccid for a few days and afterward spastic. The 
paralysis clears up to a considerable extent, but, as a rule, not completely. 
Such are the primary cases. A second group is incidental in certain 
specific fevers, and probably corresponds to the group of pyogenic 
encephalitides. Taylor holds that thrombosis is found in certain cases, 
and calls attention to the possibility of embolism from endocarditis. 
Obstetrical injury or other forms of trauma and mechanical rupture of 
vessels in paroxysms of whooping-cough or in convulsions and meningeal 
hemorrhage occurring at birth are less important factors. The prenatal 
cases of infantile hemiplegia are likely to be of thrombotic origin as a 
result of maternal syphilis. The intrapartum cases are due to obstetrical 
injury, and the postnatal cases are in general encephalitic. 
According to Taylor the following anatomical changes have been 
found in cases of infantile hemiplegia: Circumscribed atrophic sclerosis 
(in cases where the nerve elements perish, but the neuroglia survives), 
cyst formation (in cases where both nerve elements and neuroglia are 
destroyed), superficial shrunken patches resembling wet wash leather 
with adjacent atrophic sclerosis (of thrombotic origin), and porencephaly. 
The neurological phenomena of surviving cases (persistent facial paresis, 
stunting of growth of arm, choreiform and athetotic movements of arm, 
characteristic brachial contracture, distortion of bones and joints, post- 
hemiplegic disorders of voluntary and involuntary movements, trophic 
disturbances and arrest of development, and epileptic attacks) will not 
be more particularly considered here. About one-half of all cases of 
infantile hemiplegia are said to be subject to recurrent attacks of epilepsy 
which begin long after the onset of the hemiplegia. 
Prognosis.-W. P. Lucas and the writer have studied the outcome in 
29 cases of clinically probable encephalitis, finding 3 cases of epilepsy, 
which developed after an apparently normal interval. Over one-quarter 
of our cases showed mild but distinct evidence of mental defect, and a 
number of others showed "nervousness." Strabismus, developing in 
the disease, has persisted in 2 of the 29 cases. A special study of the 
hereditary relations of the psychopathic subjects does not show that they 
have a higher index of taint than the rest of the population. 
Treatment.-The febrile nature of hemorrhagic encephalitis indicates 
certain general measures. Although the hemorrhages are, as a rule, of 
an oozing character, and the progress of focal symptoms depends rather 
upon spread of inflammatory lesions than upon massive hemorrhages, it 
is desirable to avoid any drug treatment which may stimulate the heart. 
The patient should rest in bed with the head higher than the trunk, and 
be shielded from light and noise. In the somnolent or comatose condition 
especial attention must be paid to the administration of food and the 
evacuation of the bladder and intestine. Incontinence occasionally 
occurs. Cold compresses or ice bags may be applied to the head. Except 378 
DISEASES OF THE NERVOUS SYSTEM 
in cases of chlorosis, venesection or leeches in the temporal or mastoid 
region are said to have given good results (Oppenheim). Drug treat- 
ment is unsatisfactory. Restless patients may require various narcotics 
or moist packs. Oppenheim warns against surgical treatment and even 
against lumbar puncture. It appears that there have been few surgical 
attempts at alleviation of this disease. It might seem that, were accurate 
localizing diagnosis possible in certain cases of massive hemorrhage, 
surgery would be helpful. In general, surgeons are doubtful of the value 
of surgical intervention in encephalitis. Operation in meningo-encephalitis 
(Macewen's case) has been done, and Oppenheim calls attention to Hahn's 
case (1896), in which trephining was performed under the impression that 
abscess or hemorrhage of traumatic origin was present. 
Since in the future our diagnoses of encephalitis must improve in the 
line of etiology, it is possible that especially in the influenzal and pyogenic 
groups the possibilities of immunization1 and opsonic treatment may be 
exploited. The somewhat rare chance of a syphilitic encephalitis must 
be considered.2 
BRAIN ABSCESS. 
Brain abscess is a focal destructive disease of brain tissues, character- 
ized by the presence of pus, and is ordinarily distinguished from tubercle, 
gumma, neoplasm, and cyst of softening, despite the fact that cavities 
with puriform contents may be found in these conditions. As in the 
rest of the body, artificial aseptic abscesses can be produced by chemical 
or physical means within the cranium. Non-experimental abscesses 
are now generally stated to be invariably due to microorganisms. It is 
useful to bear in mind, that even in man a variety of destructive aseptic 
lesions can be produced by violence from falls or blows, and that only 
months or years later these quiescent lesions may be occupied by bacteria. 
Knowledge concerning the frequency of bacteriemia giving no clinical 
sign has explained the otherwise mysterious occurrence of latent traumatic 
abscess. 
Etiology.-A great variety of organisms has been demonstrated in brain 
abscess, not all of which are surely concerned in the original production 
of the lesion, but may settle in preexistent lesions. Malinowski, 1891, 
showed that suppurative processes could sometimes be induced in the 
normal brain of the dog by injections of pyogens, but constantly in brains 
that had been injured ten days previously. 
Pathology and Pathogenesis.-Abscesses of the brain occur in all 
sizes from approximately half the size of a hemisphere down to the barely 
visible. The smallest abscesses are, as a rule, pyemic in origin and 
multiple; they intergrade with encephalitis, the non-suppurative form of 
which may be so named merely because no pus is visible, whereas, poly- 
nuclear leukocytes may be easily demonstrable microscopically. Never- 
theless, there is good reason to distinguish such interstitial inflammatory 
conditions from frank abscess, as in other organs and tissues. The larger 
abscesses are usually single and are frequently objects of surprise at 
1 Watson, Lancet, London. 1912, i, 1469 (influenza vaccine). 
2 Laache, Ztschr.f. Klin. Med., 1911, Ixxii, 203. ACUTE ENCEPHALITIS AND BRAIN ABSCESS 
379 
autopsy. The larger single abscesses are apt to be subcortical rather 
than cortical, and are likely to break through to the surface or into the 
ventricles late in the disease. 
The pathogenesis is by extension from external infected wounds (acute 
traumatic abscess), by extension from sinus thrombosis, by extension 
through diseased bone or by lymphatic paths (otitic, rhinogenic, orbital), 
by blood infection (pyemic abscesses, abscesses metastatic from bronchi- 
ectasis, empyema). The pathogenesis of latent brain abscess is dubious, 
although there can be no doubt of the part played by blood-borne infec- 
tion. Occasionally there is an actual demonstration of pigment derived 
from lung lesions in the metastatic cases. 
The abscesses contain, as a rule, a green or yellowish-green, or yellowish 
pus, generally watery, and in long-standing cases sometimes almost clear. 
The abscess wall after some days (five or six, according to Friedmann's 
experimental data, eight or ten days in man) begins to show a membrane. 
This membrane shows a large amount of collagenous tissue, intermingled 
with neuroglia in the outer zones. The development of mesodermal 
reaction in these abscesses is greater than in most other intraneuraxial 
lesions (solitary tubercle excepted), but for all that there is no tendency 
to obliteration of the cavity. Such obliteration must be the rarest of 
events. The investing membrane may be so dense and coherent that the 
whole abscess may be stripped out, especially from a brain somewhat 
softened by postmortem change. Frequently death, from rupture into 
the ventricles with generalized pressure symptoms or rupture into the 
meninges with a terminal leptomeningitis, intervenes before such mem- 
brane formation. 
Symptoms.-The general symptoms are those of heightened intra- 
cranial pressure, and in the main resemble those of brain tumor. Deep- 
lying abscesses tend to heighten intracranial pressure more than superficial 
abscesses. Subtentorial abscesses produce a general heightening of 
pressure by means of hydrocephalus. The general symptoms are: 
1. Headache. It is rare for a case of brain abscess to run a complete 
course without headache. Even in the latent period of chronic abscess 
headache may occur periodically. The onset of fever, attacks of coughing 
and sneezing, the use of alcohol and coffee are likely to increase the pain. 
The headache is apt to be of the dull, bursting type, and is inclined to 
localize itself upon the side of the abscess. Cerebellar abscesses yield, 
as a rule, a regionary headache, but there is a surprising number of cere- 
bellar cases with frontal headache. A certain number of cases, perhaps 
the majority, show localized sensitiveness upon pressure or percussion 
over the seat of the abscess. During the period of encapsulation the 
headache of abscess is perhaps less severe than that of tumor; but 
rapidly spreading abscesses yield headaches of maximal intensity. 
2. Vomiting. This occurs-in practically all cases, as a rule at the 
time when headache is most marked, without relation to the condition 
of the stomach, and often apparently excited by a change of position. 
Subtentorial abscesses are perhaps more prone to show this symptom. 
3. Vertigo. Vertigo, unlike headache and vomiting, is inconstant in 
brain abscess, and perhaps characterizes only a minority of cases. More- 380 
DISEASES OF THE NERVOUS SYSTEM 
over, since the majority of cases with abscess symptoms give at least a 
suspicion of being otitic in origin, the vertigo runs a chance of confusion 
with aural phenomena. 
4. Slowing of Pulse. At the height of the disease the pulse is usually 
subnormal (perhaps 50 to 56). Pulse rates of 40 and 30 occur, and rarely 
it may diminish to 10. A slow pulse is especially helpful in the differentia- 
tion of brain abscess and local ear disease and sinus thrombosis. 
5. Respiratory disorder, usually with tendency to slowing, is not infre- 
quent, particularly in cerebellar abscesses. 
6. Eye-ground Changes. These are less common and severe in brain 
abscess than in tumor. They are found, as a rule, when the other 
symptoms of brain abscess are pronounced. 
Generalized convulsions occur in a minority of the cases. Cerebral 
abscesses in children appear to cause convulsions more often than such 
abscesses in adults. Late in the disease and in cases in which the pus 
bursts into the ventricles convulsions are more common. Hebetude, 
sleepiness, indolence, and an appearance of fatigue are characteristic. 
Absolute coma occurs late and may occur repeatedly in a given case. 
Episodic attacks of restlessness, confusion, or excitement may set in at 
any time and are characteristic of the initial stage in cases having an 
acute onset. They are apt to accompany attacks of fever. Cases 
suggestive of the psychiatric diagnosis of delirium are rare. The latent 
cases may suggest melancholia. 
Temperature.-Normal temperature may persist throughout, and 
periods of subnormal temperature characterize some cases. The initial 
stages of cases having acute onset usually exhibit fever. As Oppenheim 
suggests, the insidious onset of many cases and the subsidence of fever 
before the physician is called in many others have led to the idea that 
brain abscess is characteristically non-febrile. Naturally the ventricular 
and meningeal complications of the terminal stages may lead to fever. 
Macewen's data show that the majority of cases throughout observation 
show normal or subnormal temperatures. Perhaps the latent encap- 
sulated abscesses of a chronic type are those which most characteristically 
fail to show fever. Certainly a persistent high temperature or a typically 
intermittent fever is not characteristic of uncomplicated brain abscess, 
that is, abscess without meningitis or otitis. 
Focal Symptoms.-Although cases sometimes fail to yield focal symp- 
toms, yet the majority yield fairly characteristic symptoms which often 
betray the nature and origin of the lesions. Thus the occurrence of 
motor symptoms is most characteristic in traumatic and metastatic 
abscesses; whereas frontal abscesses are characteristically rhinogenic; 
temporal and cerebellar, otogenic. A few metastatic and traumatic 
abscesses have been found localized in the occipital region. Abscesses 
of the pons, the medulla, and the brachium conjunctivum are decidedly 
uncommon. Local variations in the relatively normal tissue surrounding 
abscesses are commonly stated to account for rapid modifications in the 
symptom picture and the immediate diminution in symptoms following 
surgical drainage of abscess cavities. ACUTE ENCEPHALITIS AND BRAIN ABSCESS 
381 
1. Focal Motor Symptoms. These are: (a) Epileptic symptoms of the 
Jacksonian type whose distribution may easily serve to indicate the spread 
of the abscess. These phenomena naturally occur more often with the 
cortical than with deeper lesions. The possibility is to be borne in mind 
that the convulsions are due not to destructive cortical lesions, but to 
encephalitic or meningitic changes overlying abscess. (6) Paralytic 
symptoms tend to be monoplegic, and, if hemiplegia eventuates, the parts 
are successively and not simultaneously involved. Disorders of sensation 
are often masked by the patient's mental state. 
2. Frontal Symptoms. These are frequently absent or hard to differ- 
entiate. The vicinity of the motor zone permits, in the midst of indeter- 
minate symptoms, the development in later stages of brachial or facial 
symptoms. Conjugate deviation of head and eyes toward the side of 
lesion has been noted in connection with epileptic attacks. The 
mental symptoms wTich one might expect and which occasionally arise 
in the brain tumors so localized have generally failed of demonstra- 
tion. 
3. Temporal Symptoms. Temporal abscess is practically never found 
unexpectedly at autopsy in clinical cases properly observed. The data 
concerning contralateral and homolateral disturbances of hearing are still 
equivocal, both on account of the sensorial state of the patient and on 
account of the frequent involvement of the peripheral auditory apparatus 
in these cases. Aphasic symptoms are characteristic in left-sided lesions. 
The sensory aphasia of Wernicke does not ordinarily develop from the 
temporal lobe abscess as it usually occurs, since the cortex and white 
matter of the superior temporal gyrus are not usually involved until late. 
Oppenheim pointed out that the lesion characteristically cuts the associa- 
tion paths rather than the auditory centres themselves, so that an amnesic 
aphasia supervenes in which the patient speaks little and has lost com- 
mand of many words. The understanding of terms for simple things 
remains intact. Slight paraphasia and inability to name objects, with 
maintenance of power to repeat wrnrds, are shown. The abscess may 
disconnect the auditory and the optic centres and thereby effect a partial 
word-deafness with maintenance of the understanding of words without 
visual content. Hemianopsia may be produced by deeper lesions. Con- 
tralateral motor phenomena are paralytic rather than epileptic in char- 
acter, and are probably best regarded as due to involvement of the 
internal capsule. Muscular rigidity, increased deep reflexes, diminution 
in the abdominal reflexes, development of the Babinski reflex occur. 
Macewen and Kbrner pointed out that oculomotor palsy is frequently 
found on the side of the lesion. Oculomotor palsy and contralateral 
facial and brachial monoplegia, with other abscess symptoms, point to 
temporal localization. 
4. Cerebellar Symptoms. The most important cannot be precisely 
spoken of as focal; but cerebellar ataxia, Babinski's cerebellar asynergy, 
general muscular weakness, occasionally hemiataxia, Babinski's adia- 
dokokinesis, forced movements, peculiar attitudes of head and body, 
vertigo and vomiting, nystagmus, convulsions, and various effects of 
compression of lower structures may be found. Pain in the back of the 382 
DISEASES OF THE NERVOUS SYSTEM 
head and nuchal region, retraction of the head, and stiff neck are more 
characteristic of cerebellar abscess than of other types. 
5. Occipital Symptoms. Beyond hemianopsia and possibly word- 
blindness there are few characteristic symptoms from occipital abscess. 
Further localizing points are mentioned under the diagnosis of abscesses 
classified below as of various origin: (1) Traumatic. (2) Otitic. (3) 
Metastatic. (4) Rhinogenic. (5) The so-called idiopathic brain abscess. 
Diagnosis.-Traumatic Brain Abscess.-Traumatic brain abscesses form 
about one-quarter of reported cases (Church and Peterson). They are 
likely to be superficial and situated directly below the locus of trauma, 
and naturally affect the convexity, particularly the frontal and parietal 
regions, more often than other parts of the brain. In contradistinction 
to localized traumatic leptomeningitis, which may develop very shortly 
after the trauma, traumatic brain abscess begins to show symptoms only 
after several days (acute traumatic brain abscess') or after a long latent 
period of weeks to years (latent traumatic brain abscess). 
In the acute traumatic form, the interval between injury and develop- 
ment of frank symptoms of abscess may be occupied by signs of brain 
laceration or by certain meningeal symptoms, followed by a somewhat 
sudden development (several days to two weeks after trauma) of the signs 
of heightened brain pressure with fever. Whereupon, with the develop- 
ment of focal signs, the picture of abscess becomes complete. Involve- 
ment of motor parts of the brain is obviously more often the rule in 
traumatic than in other forms of brain abscess, so that both the history 
of trauma, with local appearances, and the course of the disease, with its 
focal signs, somewhat simplify the diagnosis. Nevertheless, it may not 
be easy to tell such cases from cases of suppurative meningitis. It is 
said that muscular hypertonus and generalized hyperesthesia are less 
apt to develop in abscess cases. The cortical epilepsy or monoplegic 
symptoms, which are not infrequent in encephalitis or with cortical 
cysts of softening, sometimes occur in traumatic brain abscess, but 
speedily develop into hemiparesis or hemiplegia, possibly with aphasia. 
Sometimes the hemiplegia comes on suddenly. Again, there are rather 
characteristic short remissions which indicate that the process of abscess 
development is by no means so free as that of a meningitis of similar 
localization. Oppenheim states that the focal signs of brain abscess are 
less likely to be masked by general signs than are the focal signs of 
meningitis, an observation which probably signifies that abscess may 
proceed for many hours or days without interfering with intracranial 
pressure. The fever which may characterize the initial onset of pressure 
symptoms need not be a prominent feature in the later course. In fact, 
absence of fever as well as slow pulse are commonly thought to favor the 
diagnosis of abscess when the symptoms of the disease are fully developed. 
The frequent co-existence of abscess and localized meningitis, as empha- 
sized by Huguenin, must not be forgotten. In the absence of surgical 
interference, death may ensue within three weeks. 
In the latent traumatic form we find a similar preference for frontal, 
central, or parietal localization, but, although these abscesses are certainly 
related to overlying local trauma, yet they are not due, in the opinion ACUTE ENCEPHALITIS AND BRAIN ABSCESS 
383 
of most writers, to direct infection from superficial injured tissues. 
Possibly subinfection with organisms, circulating in the blood but derived 
from some quite different source, may account for the lighting up of an 
acute inflammation in the mechanically injured brain tissue. These foci 
would otherwise, and doubtless often do, gradually heal and in the end 
produce quite imperceptible functional changes. A careful inspection of 
the latent periods in such cases may often reveal suggestive symptoms, 
as a rule, of an irritative or "functional" character. The frank onset of 
the disease is attended with headache, perhaps localized and accompanied 
by pain on percussion of the adjacent bone. Vomiting, vertigo, tremors, 
slow pulse, are characteristic, and a large number of cases fail to show 
fever, but may even exhibit a subnormal temperature. Such abscesses are 
consistent with but slight alterations of consciousness, but investigation 
usually discovers some change, if nothing more than unusual somnolence; 
such changes when observed are often variable. Changes in the eye- 
grounds are not constant. Emaciation and gastro-intestinal disorders 
are frequent. There is great possibility of confusing such latent abscesses 
with brain tumor; in the differential diagnosis we are aided by the occa- 
sional febrile attacks, the rapid progression, and the lack or late develop- 
ment of eye-ground changes in abscess. 
An instance of blood-borne infection of intracranial abscess with 
Bacillus typhosus in a traumatic case under treatment for typhoid fever 
has been reported by Gurd and Nelles.1 
Otitic Brain Abscess.-One-third to one-half of all cases of brain abscess 
are said to follow purulent otitis media. Of course, only a small propor- 
tion of such otitides give rise to brain abscess, and then often many years 
after the onset. Mastoiditis, temporal bone disease, and the so-called 
cholesteatoma (of the otologists), and cases of otitis media which do not 
secure free drainage (either naturally or surgically) are especially danger- 
ous. Less frequently brain abscess follows immediately upon an acute 
otitis media. A somewhat rare complication is brain abscess ensuing 
upon the temporal bone disease found in diabetes. Otitis abscesses of 
the brain are rare in cases which have never undergone perforation. 
Like the traumatic forms, otitic brain abscess is usually solitary. Its 
characteristic locus is the temporal lobe, and if in the right temporal 
lobe it may yield no localizing sign, but if in the left, may yield word 
deafness or sensory aphasia, permitting accurate local diagnosis. Nat- 
urally, the general symptoms may hinder the detection of this feature. 
Let such an abscess spread inward, and a spastic hemiparesis, possibly 
associated with hemianesthesia and hemianopsia, will ensue, as a result 
of pressure upon, or destruction of, the underlying white paths. Oculo- 
motor and abducent disorders, especially ptosis, may be associated with 
the other symptoms through pressure upon the corresponding nerves. 
Temporal lobe abscess is about twice as frequent as cerebellar abscess 
following ear disease (Heimann,2 428 temporal, 198 cerebellar; Neumann,3 
336 temporal, 196 cerebellar). Cerebellar otitic abscesses in about nine 
cases out of ten (Neumann) produce symptoms. One group of symptoms 
1 Annals of Surgery, 1908, xlvii, 4 to 9. 2 Arch. f. Ohrenh., 1905, Ixvi, 67. 
3 Der otitische Kleinhirnabszess, 1907. 384 
DISEASES OF THE NERVOUS SYSTEM 
(vestibular nystagmus, vertigo, and vestibular ataxia) is dependent upon 
involvement of Deiters' nucleus. Inasmuch as labyrinthitis and cere- 
bellar abscess often co-exist, the diagnosis of vestibular nystagmus may 
be difficult. The ataxia of cerebellar otitic abscess is, according to 
Barany, a characteristic compound of vestibular and cerebellar features. 
Peculiar correlations between the direction of nystagmus, the position 
of the head and trunk, and distribution of ataxia have been described. 
Nystagmus to the diseased side, described as Neumann's sign, has been 
confirmed in three of five cases of cerebellar abscess by Ilegener. Another 
group of symptoms (hemiparesis and hemiataxia) depends upon inter- 
ruption of white paths in the cerebellar tissue. Rapid emaciation and 
anemia have characterized many cases (Okada). Uncomplicated cases 
are apt to run an afebrile course. A slow pulse, without correspondence 
with such fever as may occur, as in other processes, shows heightened 
intracranial pressure. Occipital pain is not especially characteristic of 
cerebellar abscess; on the contrary, frontal pain is not infrequent (spread 
of irritation from a recurrent branch of the ophthalmic division of the 
trigeminal nerve, supplying the tentorium, to other branches of that 
division, Krause). 
Temporal lobe abscess and cerebellar abscess are often difficult to 
distinguish. Hemianopsia is uncommon in cerebellar abscess. The 
speech disturbances of cerebellar abscess are rather bulbar than cerebral 
in character. The hemiparesis of cerebellar abscess is homolateral; of 
temporal abscess, contralateral. Palsies of isolated eye muscles are the 
rule in cerebellar abscess, whereas ptosis, external strabismus, and oculo- 
motor palsy characterize the temporal cases. The pain of temporal 
lobe abscess cases is temporal or parietal, as against the occipital or 
frontal pain of cerebellar abscess. Nystagmus, stiff neck, and sensitive- 
ness of neck tissues are characteristic of cerebellar cases. 
The law of Toynbee, according to which disease of the tympanic cavity 
gave rise to cerebral abscess, whereas disease of the external meatus 
threatened rather the lateral sinus and the cerebellum, and labyrinthine 
disease was likely to lead to bulbar involvement, has been much modified 
by more recent knowledge as to possible paths of infection. Huguenin 
pointed out that the cerebellum could be involved directly along the 
facial and auditory nerves. 
Cases of Stokes, of Habermann, and of Bloch and Hechinger indicate 
that the sense of smell should be tested in this group of cases. Twice the 
anosmia was homolateral with abscess, once contralateral. Dench1 
published an analysis of 102 recorded cases of cerebellar abscess (33 
recoveries) and 100 cases of cerebral abscess (52 recoveries) of otitic 
origin. 
Metastatic Brain Abscess.-The diagnosis of metastatic brain abscess 
(about 10 per cent, of all cases, Gowers) must take into account the pre- 
dominant sources for embolism. Bronchiectatic abscesses, pulmonary 
gangrene, lung abscess, pleural empyema, ulcerative endocarditis, pyemic 
processes of very various origin, often permit the production of brain 
1 Otitic Brain Abscess, Amer. Jour. Med. Sc., 1907, cxxxiv, p. 692. ACUTE ENCEPHALITIS AND BRAIN ABSCESS 
385 
abscesses, and, as in other hematogenous brain diseases, the territory 
of the Sylvian arteries is especially prone to involvement. It is also 
stated that the left Sylvian region is the locus of election for metastatic 
abscess. Metastatic brain abscess is more often multiple than single 
and the symptoms more closely resemble those of the acute traumatic 
form than those of otitic abscesses. Embolic in origin, this disease is apt 
to have an apoplectiform onset, but the effects of the septic emboli are 
likely to continue and create sudden fresh attacks, due either to extension 
of local processes or to the accession of more emboli. The emboli are 
usually small and carried to the small branches supplying the cortex 
cerebri or its subjacent white matter. 
The Sylvian predilection of such emboli secures a predominantly 
motor set of symptoms for these cases. But in some instances the foci 
are so many that localization is impossible. Eye-ground changes usually 
fail to develop in the acute course of these cases, nor are vomiting and 
vertigo such prominent symptoms as in most other forms of brain abscess. 
Rhinogenic Brain Abscess.-Gerber1 collected from the literature 66 
cases of brain abscess following frontal sinus disease. In the majority 
of cases the posterior wall of the sinus was diseased, and the abscesses are 
usually found involving the gyri of the orbital surface, and vary in size 
up to that of an orange. The characteristic absence of focal symptoms, 
the occurrence of frontal sinus symptoms, and the complicating presence 
of meningitis symptoms render the diagnosis difficult. Eye-ground 
changes are not uncommon, so that a diagnosis as against brain tumor has 
to be guarded. If, after frontal sinus operation, the headaches continue 
or increase, and fever, loss of appetite, alteration of general attitude, 
slowness of speech, restlessness alternating with apathy set in, orbital 
brain abscess may be suspected (Gerber). Later, several of the general 
symptoms of brain abscess may set in. 
Idiopathic Brain Abscess.-Huguenin was forced to the opinion that 
idiopathic suppuration of the brain does not exist. Gowers states that 
in about one-sixth of the total number of cases no cause for the abscess 
can be discovered. He believes that forgotten trauma is an important 
feature of some of these cases, but regards the whole matter as an open 
cpiestion. Oppenheim also suggests forgotten trauma, and thinks that 
infectious diseases may at times produce suppurative disease in the brain 
without foci elsewhere (possibly through an otitis?). 
Course and General Diagnostic Features. - The symptoms of brain 
abscess may all occur separately with meningitis, sinus thrombosis, and 
brain tumor, especially if these be complicated by extension of purulent 
or destructive processes. Moreover, syphilis sometimes yields a similar 
picture, and the difficult differentiation of symptoms of labyrinthine and 
central origin may often leave the nature of a case in doubt. In the 
absence of ear disease, disease of the nose and its communicating sinuses, 
bronchiestasis, empyema, or other peripheral source of metastasis, the 
chances are against the diagnosis of brain abscess. The history of 
trauma is unfortunately often dubious of interpretation. 
1 Beitr. zur Anat. Path, und Klinik der Stirnhohlen, 1909. 386 
DISEASES OF THE NERVOUS SYSTEM 
As against meningitis, abscess more often produces hemiplegia, mono- 
plegia, or aphasia, whereas irritative symptoms are more common in 
meningitis. Cerebellar abscess, however, may produce the same retrac- 
tion of the head and stiffness of the neck as meningitis. The absence of 
fever is against meningitis; its presence favors neither diagnosis. 
As against sinus thrombosis, the lack of high fever, of rapid pulse, and 
of tenderness and swelling over the internal jugular vein at its origin may 
be decisive for abscess. The occasional co-existence of the two lesions 
must be remembered. As against brain tumor, double optic neuritis is 
less frequent in abscess. Bramwell states that he never commits himself 
to a positive diagnosis of intracranial tumor unless suppurative ear and 
nose disease can be excluded. 
In the majority of cases three or four stages may be distinguished: 
(1) The initial stage (lasting a day to a week), characterized by headache, 
vomiting, slow cerebration, confusion, delirium, fever, and rapid or slow 
pulse, with possibly stiffness of the neck, pupillary differences, convulsive 
phenomena, and the relative absence of focal symptoms. (2) The 
latent period (lasting weeks, months, or years, on the average one to 
three months), either absolute, when the symptoms are completely 
lacking, or relative, characterized perhaps by episodes of headache, 
vomiting, vertigo, and convulsions, and failing to show any constant 
phenomena save, possibly, depression. (3) The stage of abscess symp- 
toms properly speaking (lasting some days or weeks), characterized by 
the signs of heightened intracranial pressure and the extension of suppura- 
tion. (4) The terminal stage. 
Prognosis and Treatment.-Left to itself, brain abscess kills, despite 
the extraordinary periods of latency often reported. It may kill through 
heightening of intracranial pressure, perhaps effected by spreading of 
suppuration into the ventricles or the meninges. The treatment is 
essentially surgical. Oppenheim counsels opening the skull and searching 
for suppuration in all cases in which the diagnosis of traumatic brain 
abscess is certain or even probable. The indications for operation in 
otitic cases are scarcely less decisive. Investigation of the cerebral 
and cerebellar conditions in the course of otological operations must be 
left to the judgment of the surgeon. Every large clinic has cases in which 
an abscess cavity in these regions has been "just missed" by the operator. 
Oppenheim goes so far as to state that purulent pachymeningitis, sinus 
thrombosis, or even beginning pyemia do not contra-indicate operation. 
The onset of coma does not prove that surgery would fail. Obviously 
operations upon metastatic abscesses are less likely to prolong life, and 
multiple pyemic abscesses are beyond our present treatment. But 
experience shows that metastatic abscesses are not seldom single and 
warrant operation upon diagnosis (Krause). 
The mechanical increase of intracranial pressure either directly or 
through the effects of drugs or foodstuffs upon the vascular system should 
be avoided. Therapeutic measures against headache (narcotics, morphine, 
ice bags) and attention to the possibility of bedsores and the condition of 
the bladder and rectum, to say nothing of general nursing and hygienic 
measures, are obvious indications. CHAPTER XII. 
DISEASES OF THE CEREBRAL BLOODVESSELS. 
HENRY M. THOMAS, M.D. 
Anatomy.-The blood reaches the cranial cavity by four arteries, two 
on each side, and these also connect with the rich plexus about the spinal 
cord, which in its turn receives blood at various levels by arteries which 
enter the spinal column along with certain of the nerve roots. There 
are then three arterial systems supplying the brain-the internal carotids, 
the vertebrals, and the spinal plexus. The relative importance of these 
varies a great deal, especially that of the spinal plexus, which is of com- 
paratively slight importance in man. 
The vertebrals which arise from the subclavian arteries pass through 
the canals formed by the foramina in the transverse processes of the 
upper six cervical vertebrae, and pierce the dura mater between the 
atlas and the base of the skull. After entering the subdural space they 
converge along the lateral and anterior aspects of the medulla oblongata 
to the median line, where near the inferior border of the pons they unite 
and form the basilar artery. Each vertebral, in its intradural course, 
gives off a varying number of branches, which pass over the lateral to the 
dorsal aspect of the medulla. Any one of these branches may be much 
larger than its fellows and form the posterior inferior cerebellar artery 
which reaches the inferior surface of the cerebellar hemisphere, over which 
it ramifies. The lower vertebral branches are connected intimately with 
the spinal arterial plexus, and more or less with the branches above. 
The posterior spinal arteries rise from this network of arteries formed 
from the branches of the vertebral, or directly from one branch, as the 
posterior inferior cerebellar artery. Shortly before the vertebral arteries 
join to form the basilar artery each gives off the relatively large twig 
which passes over the ventral aspect of the medulla to join and form the 
unpaired anterior spinal artery. 
The basilar, in its course over the pons, gives off numerous small 
branches, and from it also arise the anterior inferior, or middle, and 
superior cerebellar arteries. When the basilar reaches the superior 
border of the pons it divides in a T-shaped manner, and may be said 
to end in the two posterior cerebral arteries, which, passing above the 
tentorium, apply themselves to the inferior and posterior aspects of 
the cerebral hemispheres. 
It is evident that the vertebral arteries entering the posterior fossa of 
the cranium supply all the important structures lying in this fossa. This 
vertebral system anastomoses freely below with the arterial system of the 
spinal cord, and ends above in the posterior cerebral arteries, which form 
the posterior pair of the three great arteries of the cerebral hemispheres. 
387 388 
DISEASES OF THE NERVOUS SYSTEM 
From each of the posterior cerebral arteries, soon after its origin, is given 
off a vessel, the posterior communicating artery, which unites the vertebral 
system with that of the internal carotids and helps in the formation of the 
circle of Willis. 
The internal carotids enter the cranial cavity through the carotid 
foramen. Each pierces the dura mater and is continued directly into 
the middle cerebral artery, which is connected with the posterior cerebral 
artery by the posterior communicating artery. It also gives off the 
anterior cerebral artery, which passes forward and toward the middle 
line to be supplied to the medial surface of the cerebral hemisphere. 
Just in front of the optic chiasm the two anterior cerebral arteries are 
united by a short trunk, the anterior communicating artery, and thus 
the circle of Willis is completed. 
There is the freest communication between the large arterial trunks at 
the base of the brain, and the circulation of the tw'O sides is intimately 
connected. It must be borne in mind that there is considerable variation 
in the size of the communicating branches, and that the circle of Willis 
is not so regular as the diagrams would indicate. 
The blood for all parts of the brain comes from a common arterial 
plexus or reservoir, situated in the middle line at the base of the brain. 
The vessels which nourish the structures at the base of the cerebral 
hemispheres, the basilar nuclei, etc., are given off directly from this 
plexus, or from large arterial trunks shortly after they leave it. These 
nutrient basal arteries penetrate the brain to a greater or less extent, and 
appear to be end arteries. The cerebral cortex gets its blood by the 
anterior, middle, and posterior cerebral arteries. Each of these supplies 
a more or less definite area, but these are not absolutely distinct, as they 
overlap and there are actual anastomoses by arterial branches of fair size 
(from 0.5 to 1 mm.) between the vessels of neighboring areas. 
The nutrient vessels of the cortex, so-called cortical vessels, are not 
given off from the larger arterial twigs, but from the smallest vessels of 
the pia mater, which have arisen by division of the larger branches. In 
the rich arterial mesh of the pia one is able to find occasional anastomoses 
between arterial twigs of fair size, but there does not appear to be a true 
pial plexus in the sense of Huebner. The nutrient cortical vessels enter 
the cortex at right angles and descend to a greater or less depth. They 
give off branches to the cortex and the white matter beneath it, and seem 
to be practically end arteries, although anastomoses between the branches 
of neighboring vessels have been described. 
The vessels of the cortical system which pass to the white matter almost 
reach to the basal nuclei. They, however, make no anastomoses with 
the system of basal nutrient arteries, and the two systems seem quite 
independent. Beevor's1 injections show as never before the area supplied 
by the different cerebral arteries, and his work settled many disputed 
points. The drawings of the arterial supply of the basal nuclei by Aitken2 
are also most instructive. 
1 Phil. Tr. Lond., 1908, cc, 1; Brain, 1907, xxx, 1. 
2 Boston Med. and Surg. Jour., 1909, clx. DISEASES OF THE CEREBRAL BLOODVESSELS 
389 
The blood, after having passed through the capillaries, is collected by 
the small veins and taken to the surface of the brain, where they join in 
the formation of the venous plexus, which empties its blood by the 
larger veins into the venous sinuses. The veins on the surface of the 
brain are less numerous than the arteries, but are of larger size and have a 
more superficial course. The various sinuses are large venous reservoirs, 
lying in folds of the dura mater. Most of them are protected on one side 
by the skull itself. They are intimately connected among themselves 
and all empty into the internal jugular veins. The intracranial venous 
plexus also connects with the veins of the rest of the body by other ways. 
Chief among these is the anastomosis between the cavernous sinus and 
the veins of the orbit, which, in their turn, connect with the superficial 
veins of the face. The other less important connections need not be 
mentioned, except to point out that there are numerous anastomoses 
between the veins which run in the diploe and the intracranial system 
on one side and the veins on the outer surface of the skull on the 
other. 
Physiology.-Within the skull we have the brain with its membranes, 
blood, and cerebrospinal fluid. There is much dispute about the exact 
conditions which regulate the circulation of the brain. We may regard 
the skull as a closed box, protecting its contents from the direct action 
of atmospheric pressure. The total amount of blood within the skull 
at any instant varies but little from that at any other. This is true 
because the brain substance itself is incompressible, and the small amount 
of cerebrospinal fluid is the only thing that can vary. Although the total 
amount of blood varies but little, the proportion between the arterial 
and venous blood may and does vary greatly. 
The arteries of the brain have well-developed muscular walls, and 
contain numerous nerve fibres, and it seems certain that they do at times 
change their caliber independently of the general arterial pressure. 
But it is generally stated by physiologists that there is no definite proof 
that the cerebral vessels are under effective vasomotor control, and the 
view of Leonard Hill, that the cerebral circulation passively follows 
changes in the general circulation, is widely accepted. Most experi- 
menters failed to get any evidence of direct vasomotor action until 
Muller and Siebeck1 reported experiments in which they cut and stimu- 
lated the cervical sympathetic, and obtained evidences of definite vascular 
changes in the brain; and Wiggers2 has shown that the cerebral vessels, 
as well as the renal vessels, react to various drugs in very characteristic 
ways, which he believes is due, at least in a large part, to the effect of the 
drugs on the nerve endings. It takes a much stronger solution of adren- 
alin, for instance, to cause the arteries of the brain to contract, than is 
effective on the vessels of the kidney. Surgeons have noticed the pial 
vessels change their caliber under the influence of drugs (Cushing). 
Therefore, it is probable that the cerebral vessels are under a more or 
less effective vasomotor control, but much must yet be done to establish 
1 Zeitsch.f. Exper. Path. u. Therap., 1907, iv, 57. 
2 Amer. Jour. Phys., 1907, xx. 390 
DISEASES OF THE NERVOUS SYSTEM 
this on a satisfactory basis, and in any case the cerebral circulation as a 
whole does follow passively that of the general circulation. When the 
blood-pressure is raised, the blood enters the skull with increased force, 
and a greater quantity passes through the brain in a given time. 
Consciousness depends upon a sufficient amount of blood reaching the 
brain, particularly the cortex, and life itself depends upon arterial blood 
passing through the medullary centres. To the vasomotor centre is 
delegated the function of maintaining an adequate blood supply to the 
other vital centres and to the brain in general. When there is a lack of 
arterial blood reaching this centre, it is thrown into strong activity and 
causes a contraction of the arterioles in the splanchnic and skin areas; 
the general blood pressure is raised, and the blood enters the brain with 
augmented force and floods the capillaries with arterial blood. 
The intracranial pressure, that is, the force which the brain exerts 
against the skull, depends upon the blood-pressure, and is equal to the 
pressure of the blood in the venous sinuses, which, under normal circum- 
stances, is the same as the general venous pressure. If the intracranial 
pressure be raised above the general arterial pressure, no blood can 
enter the skull, and the animal will die. In Cushing's1 experiment it 
was shown that when this pressure became so great as to embarrass the 
medullary centres there was a corresponding rise of general arterial press- 
ure. If the intracranial pressure was again increased the vasomotor 
centres answered again with a rise of arterial pressure. This could be 
repeated until the vasomotor centres were exhausted. 
When this regulating mechanism is disturbed, various results follow 
as, for instance, when the sudden removal of something that has caused 
continuous pressure on the abdominal vessels, as ascitic fluid, is followed 
by alarming symptoms. In this case the vasomotor influences controlling 
the splanchnic area have not been called on for some time, and the centre 
itself is weakened, so that when a sudden demand is made to compensate 
for the accustomed external support to the bloodvessels, it is unable to 
respond, the blood collects in the splanchnic vessels, the patient becomes 
unconscious and may die. 
While under ordinary circumstances the vasomotor mechanism and 
the tonicity of the muscles of the abdominal walls compensate perfectly 
for the change from the horizontal to the upright position, i. e., for the 
effect of gravity upon the column of venous blood from the heart to the 
feet, in asthenic states, as after severe illness, the compensation may be 
very imperfect. When such is the case, if the patient stands, or, at times, 
even if he sits up in bed, his heart beats more rapidly, he becomes giddy, 
and may faint. The change in the pulse rate with a change in position 
is a fair indication of the vasomotor control, for the heart endeavors 
to make up for this incompetence. 
Under various conditions the heart itself may become so weak as to be 
unable to keep the brain properly supplied with arterial blood. The 
ordinary fainting fit is, at least in part, an example of this. Under the 
influence of emotion the heart's action becomes weak, the vasomotor 
1 Mitt. a. d. Grenz, d. Med. u. Chir., 1902, ix; Am. Jour. Med. Sc., 1902, cxxiv. DISEASES OF THE CEREBRAL BLOODVESSELS 
391 
centre is inhibited, and, in consequence, the abdominal bloodvessels 
become dilated, blood-pressure falls, and the heart is no longer able to 
drive the blood back to itself against the force of gravity; the blood 
accumulates in the abdominal veins, the heart empties, cerebral circula- 
tion fails, and unconsciousness occurs. The sudden loss of conscious- 
ness in epilepsy has also been ascribed to a transient paralysis of the 
heart (Russell). In Stokes-Adams disease the cerebral symptoms, 
attacks of unconsciousness, convulsions, and apoplectiform seizures, are- 
due to cerebral anemia, caused by the temporary cessation of the ventric- 
ular systole. The extreme example of this cardiac weakness is paralysis 
of the heart muscle from failure of the coronary circulation, which is the 
usual cause of sudden death. 
Movements of the chest in respiration have an important effect upon 
the circulation in general and on that within the skull in particular. 
With every inspiration the blood is sucked into the heart from the veins, 
and the descent of the diaphragm, by increasing the pressure on the 
abdominal veins, tends to force the blood into the heart. During 
expiration, the intrathoracic pressure ''s increased, and the entrance of 
the blood into the heart is impeded. Arterial blood-pressure is influenced 
but slightly by the respiratory movements. The unconsciousness, or 
indeed, death, which at times follows forcible compression of the chest, 
is due to failure of the cerebral circulation caused by the inability of the 
heart to fill itself with blood, as well as to the damming back of the blood 
into the venous sinuses. 
Intracranial pressure has been shown to be equal to the venous blood- 
pressure within the sinuses and to follow every change in this. The 
column of venous blood between the brain and the superior vena cava 
is uninterrupted by competent valves, and every change of pressure in 
the cava is transmitted directly to the sinuses and veins of the brain. 
The brain dilates with each pulse beat, but much more with each expira- 
tion, that is every rise in venous pressure. Intrathoracic pressure is 
increased in expiration, and this causes an increase in the pressure of the 
cava, the jugular, and the brain sinuses. During prolonged expiratory 
efforts, the venous blood is, as it were, dammed back into the brain, 
intracranial pressure rises, and, as less arterial blood can pass through 
the cerebral circulation, the symptoms of anemia may follow, as when 
a crying child holds his breath until he becomes unconscious. 
Intrathoracic pressure makes it more difficult for the heart to fill itself 
with blood, and affects the circulation also in this way. Strong respira- 
tory efforts against an obstruction may cause very marked changes in 
intrathoracic pressure. If the glottis be closed and forced expiration 
be attempted, a normal, negative pressure of the thorax becomes markedly 
positive, and may far exceed normal pressure in the intrathoracic veins. 
This is conveyed directly to the cerebral veins and capillaries, and it is 
not uncommon to have hemorrhages within the brain substance following 
such an effort. These conditions accompany a strong effort, as straining 
at stool, lifting a heavy weight, or coughing severely. The importance 
of preventing, so far as possible, any obstruction to respiration during 
the course of apoplexy follows directly from what has been said. 392 
DISEASES OF THE NERVOUS SYSTEM 
The venous outlets from the skull are so large and the anastomoses 
so free that they must all be obstructed to cause venous congestion of 
the brain. It is for this reason that thrombosis or ligature of one of the 
sinuses is not necessarily followed by any symptoms, and, indeed, the 
superior vena cava itself may be obstructed, as by a tumor, without 
symptoms. In this last case the blood from the vein reaches the heart 
through the craniovertebral veins and anastomoses through paths to 
the inferior vena cava. If all the veins of the neck are compressed as 
by a tight band or by strong flexion of the neck, the circulation may 
be impeded, and this is important under pathological conditions. 
Hyperemia.-The brain is extremely sensitive to changes in the amount 
of blood which it receives. Many symptoms have been ascribed to anemia 
and hyperemia of the brain, but there is little agreement as to the symp- 
toms which depend upon these two conditions, and, indeed, upon just 
what is meant by them. Hyperemia is sometimes divided into passive 
and active; passive hyperemia meaning the damming back of blood into 
an organ by impeding the outflow from the veins, in fact, a venous 
hyperemia and an arterial anemia. The symptoms which follow must 
be largely due to a lack of arterial blood, and are, therefore, quite similar 
to those which depend upon anemia. 
Active hyperemia of the brain is due to anything that causes an increase 
of flow' through it, i. e., anything that makes the difference between 
arterial and venous pressure greater; for instance, an increase of the 
general arterial pressure or a lowering of the venous pressure in the 
sinuses. Such changes must be occurring all the time, and we know of 
no symptoms due to them, and it is hard to believe that any harm follows 
this increase in the supply of arterial blood. 
Anemia.-This may be general or local, i. e., the brain as a whole 
may take part with the rest of the body in a decrease of the total amount 
of blood, and it may be effected by a change in its quality. On the other 
hand, the circulation of the brain itself may be altered as a result of local 
conditions, by ligature of one of the vessels that enter the skull, or by 
disease of any of the intracranial vessels. 
The symptoms due to. anemia associated with general loss of blood 
make up most of the clinical picture following hemorrhage, and were 
the symptoms upon which the older physicians relied when bleeding was 
a common therapeutic measure. Since this procedure has become so 
infrequent the interest in the symptoms of hemorrhage has been largely 
confined to the surgeons, who study them as the chief part of surgical 
shock. When the loss of blood is sudden and of sufficient quantity, the 
patient faints. He first experiences a sense of vertigo, may have ringing 
in the ears, dimness of vision, and a rapidly increasing insensibility. 
The breathing becomes shallow and intermittent, more or less of the 
Cheyne-Stokes type; the pulse is feeble and rapid; the extremities are 
cold and covered by profuse perspiration. At times, especially when the 
bleeding has been rather slow, the patient may have general convulsions 
before loss of consciousness occurs, or, indeed, he may die suddenly 
without any marked premonitory symptoms, having retained conscious- 
ness until the end. This accident is more apt to occur when the patient, DISEASES OF THE CEREBRAL BLOODVESSELS 
393 
who has lost a considerable quantity of blood in a horizontal position, 
stands or sits up; or even without any change of position, when the 
hemorrhage has been great enough to seriously embarrass the heart. 
When the central nervous system is supplied with blood of a poor 
character, as in grave anemias, symptoms of malnutrition, more or less 
pronounced, are apt to follow. Headache, vertigo, tinnitus, and mus- 
cular weakness may, at least in a large part, be thus accounted for. On 
the other hand, it is remarkable how well and how long at times the 
nervous system performs its functions without apparent difficulty, even 
when the blood which nourishes it is far below the normal standard. 
In most of such cases, however, it will be found that the immunity from 
symptoms is only relative, and that under stress the brain gives evidence 
of its insufficient blood supply. 
Many symptoms are ascribed to anemia of the brain due to spasm of 
the vessel walls in circumscribed areas. Thus the transient paralyses in 
arteriosclerosis, etc., are so explained. This depends upon the assumption 
of an active vasomotor control, and we have seen how little definite proof 
there is of such an action. Should it be demonstrated, we may learn to 
recognize a group of phenomena now little understood. 
Cerebral Arteriosclerosis.-The vessels of the brain may be diseased 
either alone or in association with the rest of the bloodvessels. The 
local occurrence of arterial disease has not as yet been thoroughly worked 
out, but enough has been done to establish the fact that there may be 
marked degeneration in one system alone, and that the condition of 
those vessels wdiich can be easily examined, such as those in the arms 
and legs, is no absolute criterion to that which may be present in some 
other system, for instance, the brain. 
Etiology.-So long as our knowledge as to the production of arterio- 
sclerosis remains incomplete, we can not know why, in one case, the 
vessels of the brain should be first affected, w'hile in another it is those of 
some other vascular area. Given the general causes, the wrear and tear 
of life, the acute infections, the intoxications, and the conditions which 
keep up an increased blood-pressure, we may perhaps assume that 
those organs upon which the greatest amount of stress is brought would 
be those in w'hich vascular changes would first appear. We might expect, 
therefore, in individuals whose activities have been largely intellectual 
to find the cerebral vessels affected more often than in others whose 
work has been almost entirely physical. In a general way this appears 
to be true, although there are so many modifying factors that it is of 
very little practical value. The brain does not function only during 
so-called intellectual processes, but takes part in most of the bodily 
activities, and as cerebral circulation follows passively the changes in 
that of the rest of the body, the cerebral vessels are under constant 
strain, and almost as much so in the laborer as in the student. 
Certain infections, syphilis for instance, appear to have a peculiar 
affinity for the cerebral vessels. Syphilitic endarteritis is, indeed, usually 
distinguished from other alterations of the vessel wralls, and is apt to be 
associated with a gummatous involvement of the meninges. Syphilis, 
with the other infectious diseases, does also seem to predispose to the 394 
DISEASES OF THE NERVOUS SYSTEM 
ordinary arterial changes, and arteriosclerosis in infants and children is 
believed to be frequently due to hereditary infection. 
Age.-In the brain, as in other parts of the bodv, degeneration of the 
arteries may occur at almost any time of life. In hereditary syphilis early 
arteriosclerosis is common, and at times it follows acute infectious dis- 
eases. But generally arteriosclerosis is a rarity before the fortieth year, 
when, from that time on it becomes more common with every succeed- 
ing decade, but extensive arterial disease may occur before this period. 
Pathology.-Anatomically, fairly clear-cut changes in the brain, asso- 
ciated with alterations in the vessels, have been described. These consist 
in focal areas of necrosis, which may be very small, but are at times 
large enough to be seen by the unaided eye. These are essentially infarcts 
due to obliteration and sometimes to rupture of the capillary vessels. 
They are most frequently seen in the gray matter of the cortex, but may 
be found scattered anywhere in the brain. Associated with these one 
finds the ordinary large areas of disease due to obliteration or rupture 
of one of the larger arteries, which explain the occurrence of focal symp- 
toms. The cortical changes are distinguished from those in general 
paresis by being focal and unaccompanied by the special histological 
elements seen in this disease. The changes in senile dementia are much 
more diffuse and have their own special characteristics. 
Marie1 and his scholar Ferrand2 pointed out the importance of small 
areas of softening (lacunae) secondary to arterial sclerotic changes, as 
the cause of hemiplegia in elderly people. These were often associated 
with hemorrhage into the brain, at times with softening, but in more 
than half of the cases they occurred alone. The symptoms which he 
associates with these changes are very similar to those described under 
arteriosclerosis. 
Symptoms.-The anatomical changes in the arteries alter their make-up 
and render them less elastic, more brittle, and prone to gross alterations, 
such as the formation of aneurisms. The walls may encroach on the 
lumen and decrease the capacity. As a result, the organ supplied by the 
diseased vessels receives its blood through vessels of a smaller capacity 
and with walls which are changed. Thus the amount of blood reaching 
the brain is decreased, and the interchange between the blood and the 
nervous tissue is altered. This is entirely aside from the actual plugging 
of an artery or the rupture of its walls. 
Nature provides lavishly, and it is probable that under normal circum- 
stances much more blood passes through the brain than can be used. 
Certain it is that in not a few cases the cerebral vessels may be markedly 
altered and the brain continue to perform its functions so that no defect 
is obvious. This is due not only to the utilization of the normal surplus 
of food, but also to the reserve force of the circulatory system itself, 
which tends to compensate for anything that impedes the flow of blood. 
On the other hand, there are cases in which, although the vessels are 
much less altered, we ascribe the pronounced symptoms to a change in 
the circulatory condition; but we cannot explain why symptoms occur 
1 Rev. de Med., 1901, xxi, 281. 
2 Thesis, Paris, 1902. DISEASES OF THE CEREBRAL BLOODVESSELS 
395 
in these and not in the other cases. There is no considerable evidence 
upon which to base conclusions. The anatomical investigation is apt 
to be limited to the larger arteries at the base of the brain, and the 
clinical notes are far too often so meagre as to make even this of little 
use in correlation with the symptoms, and to render superfluous in this 
relation a minute study of the smaller and nutrient vessels of the brain 
substance. It may well be that these smaller vessels are at times much 
altered, when the larger vessels are but little affected, or the reverse may 
be true, and also the arteries of the different areas of the brain may be 
unequally diseased. 
One would expect that as the brain began to feel the decreased blood 
supply the symptoms would at first, if noticed at all, be indefinite and 
transient, and that they would differ with the varying distribution of 
the vessels most diseased. Until our knowledge is much more complete 
any statement as to the symptoms of beginning cerebral arteriosclerosis 
must be indefinite. There is a growing tendency to explain a great variety 
of symptoms as primarily due to an altered blood supply. The following 
are the more important symptoms usually so explained. 
Neurasthenia.-In certain cases in which arteriosclerosis has reached 
a relatively high degree, and which present definite symptoms referable to 
cerebral vascular lesions, a history is given of recurrent attacks of neuras- 
thenia dating from a relatively early period. The conclusion that these 
were themselves evidences of beginning arterial disease is attractive; 
and when, on the other hand, one finds men of about forty, or older, 
suffering from the protean symptoms which we class under neurasthenia, 
and the physical examination reveals well-marked arterial changes, we 
may be excused if we explain one condition by the other. 
One chief characteristic of the neurasthenic is that he is easily tired 
after any effort, and that even the slightest physical or mental work is 
followed by distressing symptoms. If the brain is nourished by an 
inadequate blood supply or receives its blood through vessels whose walls 
are so altered as to interfere with the free interchange of the blood and the 
tissues, one would expect that it would show some such evidences of 
distress under activity. The fact that most cases of neurasthenia can 
be cured by appropriate measures indicates that the condition of the 
brain is usually only a part of the general bodily state. Even if there 
be a beginning cerebral arteriosclerosis, the changes may be so slight 
as to be entirely compensated when the general level of bodily vigor is 
raised. Cerebral activities are important factors in this general uplift, 
and one may perhaps thus understand some of the good results of mental 
therapeutics, even in cases of neurasthenia which seem to have been due 
to acute organic changes, as proved by the subsequent course. 
However, the practical indication is clear that we must keep in mind 
the possibility, in our cases of neurasthenia, even in young individuals, 
that the chief underlying factor may be a beginning arteriosclerosis, and 
we should not neglect to examine carefully for this condition, and if 
found we should not overlook it in our treatment, no matter how 
enthusiastically we regard the perhaps more alluring field of psychical 
therapeutics. 396 
DISEASES OF THE NERVOUS SYSTEM 
Headache.-This is often classed as one of the most common and most 
persistent symptoms in cerebral arteriosclerosis, and, indeed, it does 
occur; but the writer has been struck by the relative infrequency of this 
symptom, and its complete absence occurs under conditions when it might 
be expected. Thus, a number of elderly patients, although suffering from 
undoubted general arteriosclerosis, with marked involvement of the 
retinal vessels, and in whom the blood-pressure remained persistently 
high, rarely falling to 200 mm. Hg., have entire freedom from any 
headache. Walton and Paul, in their study of 100 patients, all the sub- 
jects of arteriosclerosis, found headache in only 22 per cent., and in 
this connection they refer to the well-known fact that individuals who 
have been subject to headache in their youth, often become immune 
with advancing years; whereas the opposite would be expected were 
arteriosclerosis in itself a common cause of headache. 
On the other hand, one is at times consulted by patients suffering from 
intense headache, who give a history of having been entirely free up to 
late middle life, and it is in these cases that we should carefully examine 
the vascular condition; but even in them it may be the associated 
changes that are at fault, such as in the kidneys. When arteriosclerosis 
has advanced so far as to give focal cerebral symptoms, the onset of this 
is very generally associated with pain and other distressing sensations 
referred to the head. 
Vertigo.-Nearly all patients complain of a sense of insecurity of equi- 
librium, of dizziness, or of actual vertigo. This may be more or less con- 
stant, but is more commonly transient and brought on by exertion or 
change of position. Some patients when placed in a certain attitude 
suffer at once from a sharp attack of vertigo, as in two patients who 
could not be shaved in the usual manner, for the instant the barber 
tilted back the chair they had a vertiginous attack with the sense of 
impending loss of consciousness and death. In each of these cases this 
distressing symptom subsided under hygienic and tonic measures. Sub- 
jective auditory sensations, as well as true tinnitus, are occasionally 
associated with vertigo or may occur alone. The presumption is that 
there is some involvement of the middle or internal ear, but if such is 
the fact it escapes demonstration. It may be that some cases in elderly 
people, which we are in the habit of designating aural vertigo, are really 
to be put in this category. Vertigo, like headache, is commonly asso- 
ciated with the transient cerebral attacks which are very frequent in 
certain cases of cerebral arterial disease. 
Apoplectiform Attacks.-It is not uncommon to have sudden attacks 
due to more or less closure of some one of the cerebral bloodvessels, or, 
indeed, to their rupture. The resulting paralysis may be permanent, 
or it may last only a short time. In the first case we believe that there 
has been a destruction of nervous tissue, but in the second the loss of 
function is so transient that we assume it to be due to a passing stoppage 
of the circulation, from the effects of which the brain recovered. It is 
these latter cases in which we are at present interested, although they 
are relatively much less common. These patients suffer from repeated, 
and in some cases very many, attacks of vertigo and headache, associated DISEASES OF THE CEREBRAL BLOODVESSELS 
397 
with aphasia, or a more or less extensive paralysis of one side or the 
other, from which they recover completely. These attacks may come 
on spontaneously or after exertion. They have been brought into 
relation with the intermittent loss of function which under stress occurs 
in other organs, whose bloodvessels are diseased-intermittent claudica- 
tion, angina pectoris, etc., and the assumption of a transient spasm of 
the walls of the vessels is given as an explanation. Even the term claudi- 
cation has by some authors been extended to include these cerebral 
attacks, but this seems a too free use of the term. That the cerebral 
arteries during these attacks are in a state of spasm is an inference 
which receives strong support from observations on the retina in certain 
cases of transient blindness when the actual constriction and relaxation 
of the sclerosed central artery is to be noted (Zentmayer). 
Convulsions.-Convulsions, either general or focal, may be associated 
with the apoplectiform attacks, but perhaps more frequently the subject 
of cerebral arteriosclerosis suffers from convulsions which have the 
usual characteristics of epilepsy. The occurrence of such seizures in 
individuals who have reached middle life always makes one suspect the 
presence of this condition. Typical Jacksonian attacks at times recur for 
years after the occurrence of a cortical lesion. 
Mental Symptoms.-If the mental states which so frequently accom- 
pany neurasthenia are included under this heading-the inability to 
continue mental effort for any length of time, the forgetfulness, the 
irritability, the depression of spirits, etc.-we may say that mental 
changes occur early in cerebral arteriosclerosis. Usually, however, it 
is not until the disease has progressed much farther that the mental 
changes are so pronounced as to constitute a real alienation. On the 
other hand, it has been suggested that in manic-depressive insanity, the 
varying mental states, with their associated toxic condition, are par- 
ticularly favorable for the occurrence of arteriosclerosis. 
The other types of mental disease which occur late in life, the devolu- 
tional psychoses (Farrar1), are often associated with vascular changes, 
and it is difficult at times to determine how much of the symptomatology 
is actually due to the altered circulatory condition. From these psychoses, 
occurring during the latter part of life, there has been differentiated a 
distinct type which is believed to be due to cerebral arteriosclerosis, 
and the symptoms have been brought into relation with fairly definite 
anatomical changes. Such patients who may have passed through pre- 
vious attacks of neurasthenia begin to appreciate that they can no 
longer accomplish their accustomed mental work, that they tire easily, 
are unable to undertake new problems, often notice that their memory 
fails them, that they are unable to recall names, and frequently miss 
the right word. These symptoms are often associated with attacks of 
vertigo and other manifestations of arteriosclerosis described above; later 
definite vascular lesions occur, which, although they may be recovered 
from, tend to leave the patient on a lower mental level. In other cases 
these seizures may be the first indication of the process. 
1 Rev. of Neurol, and Psychiat., 1906. 398 
DISEASES OF THE NERVOUS SYSTEM 
As the mental failure increases the patient becomes unable to carry on 
his work, is usually apathetic, but his mood may show marked variability, 
and changes quickly from deep depression to well-being. It is uncommon, 
however, for this to reach an actual state of exaltation, such as is seen 
in general paresis. Most patients have a remarkably clear appreciation 
of their condition, and this is an important help in distinguishing them. 
The condition may progress to almost complete dementia, but is more 
likely to be terminated by a vascular accident. 
The clinical p'cture at times closely resembles that of general paresis, 
and it may also simulate senile dementia. The diagnosis rests largely 
upon the presence of the more or less characteristic mental conditions, 
associated with definite symptoms of arteriosclerosis, and the absence of 
the objective symptoms which point to general paresis, or the typical 
picture of the senile dement. The age is also of importance as general 
paresis occurs, as a rule, during the full vigor of maturity, while senile 
dementia occurs particularly in old age. It is between these periods that 
the insanities due to arteriosclerosis are apt to develop. The diagnosis 
may be impossible, and cerebral arteriosclerosis may modify the picture 
of general paresis and of the psychoses of the devolutional period. 
Diagnosis.-The cardinal points-(1) thickening of the peripheral 
vessels; (2) signs of hypertrophy of the left ventricle; (3) heightened 
blood-pressure; and (4) a slight and variable amount of albumin in the 
urine-should be kept in mind and looked for when the nervous symptoms 
suggest cerebral arteriosclerosis. The condition of the retinal vessels is 
of special significance, for although it cannot be said that alterations in 
them are always indicative of changes in the cerebral vessels, their con- 
dition furnishes the best objective evidences that we have. The ophthal- 
moscopic picture is quite characteristic. 
Treatment.-The treatment is that of general arteriosclerosis, but it is 
essential that the condition should be recognized early, and that treat- 
ment should be directed at once to the vascular condition. If the causes 
are known, measures should be instituted to remove or counteract them 
as far as possible. Thus, when syphilis is an etiological factor the indi- 
cation is plain. Usually, it is impossible to determine what has brought 
about the alteration in the vessel walls; whether the high arterial tension 
is a cause or effect, and if it be a cause, upon what it depends, and whether 
the alterations in the kidney are primary or secondary. 
Aneurisms of the Larger Cerebral Arteries.-True aneurisms of the 
larger cerebral arteries are relatively uncommon. In Crisp's list, as 
quoted by Osler (vol. iv, p. 480), among 501 aneurisms, intracranial 
aneurisms occurred only 7 times. Beadles1 collected the records of 555 
cases of such aneurisms found after death. He classified these in four 
groups: "(1) Those in which the first indication of a cerebral lesion 
has been an apoplectic attack due to rupture of the aneurismal sac. (2) 
Those in which a fatal apoplexy has been preceded by symptoms sug- 
gesting a cerebral tumor or other cerebral lesion. (3) Those in which 
there have been indications of a cerebral tumor only. (4) Those that 
1 Brain, 1907, xxx, 285. DISEASES OF THE CEREBRAL BLOODVESSELS 
399 
gave rise to no symptoms whatever during life, and the aneurism was 
discovered accidentally after death." 
In over one-half of the cases the first symptoms noted were those of 
apoplexy, and even in those cases in which symptoms did occur these 
were often trivial, or such as might have been attributed to cerebral 
arteriosclerosis, such as dizziness, headache, mental weakness, and even 
insanity. Indeed, not a few cases of cerebral aneurism have been 
unexpectedly found at autopsy in patients dying in insane hospitals. 
As aneurismal dilatations depend upon structural changes in the walls 
of the vessel, arteriosclerosis, or syphilitic disease, similar changes are 
apt to be present in the walls of the other vessels of the brain, and, as 
would be expected, not infrequently other lesions are present due to these 
vascular changes but entirely independent of the aneurism, and it is 
to these that many of the symptoms may be due. 
The general symptoms of brain tumor, violent headache, vomiting, 
and choked disk, are at times present, but the frequency of their absence 
is remarkable. Headache particularly seems to be a rare symptom, and 
choked disk has been only occasionally noted, but Beadles found but 
few notes of an ophthalmoscopic examination having been made. Aneu- 
risms of the basilar artery give rise more often to focal symptoms than 
those on any of the other arteries at the base, these occurring in almost 
one-half the number of cases. This is due to the fact that aneurisms in 
this situation may implicate the cranial nerves or the important nervous 
paths which run close to the surface in the pons and medulla. Aneurisms 
from the posterior communicating, posterior cerebral, and intracranial 
portion of the internal carotid, and the middle cerebral, have given symp- 
toms with the relative frequency in the order named, but these are 
almost never so characteristic as to allow of a local diagnosis. It is 
only in rare instances that the diagnosis of a tumor can be made, and 
that such a tumor is an aneurism is a deduction that is practically not 
justified. The classical symptoms supposed to indicate an intracranial 
aneurism, the subjective noises in the head, and a vascular murmur 
which could be heard through the skull, are so rare as to be of little 
value. Beadles was able to find only about "a dozen cases recorded in 
which some more or less definite sound has been heard in the head of the 
patient." In none of these was there any record of the head having been 
examined for an objective murmur, and he was able to find only two cases 
of " uncomplicated, true, intracranial aneurism in which a murmur had 
been heard by the examining physician. One of these was an aneurism 
of the vetebral, and the other an aneurism of the cavernous portion of 
the internal carotid." The fact that it is so rarely possible to make an 
accurate diagnosis of an aneurism may perhaps be partially explained by 
the small size which they usually reach. An aneurism larger than an 
ordinary marble is rare; one reaching the size of a small hen's egg is a 
very large one; and Beadles, in speaking of an aneurism, which he so 
describes, remarks that there have been probably less than a dozen larger. 
On the other hand, many aneurismal dilatations have ruptured relatively 
early, when they were no more than small, soft sacs and produced little 
pressure. It is the slow-growing, thick-walled aneurisms that have 400 
DISEASES OF THE NERVOUS SYSTEM 
usually given symptoms. In 339 of Beadles' 555 cases, death occurred 
following a rupture of the aneurism, and it is apparent that their chief 
importance is in relation to apoplexy. 
Obliteration and Rupture of the Cerebral Vessels.-Thrombosis and 
Embolism.-The subject of thrombosis and embolism has been fully 
considered (vol. iv, p. 526). The reader is also referred to Welch's 
article in Allbutt's System of Medicine, and only points which are of 
especial interest in the present connection will be referred to here. 
Etiology (Thrombosis).-The occurrence of thrombi in the vessels of 
the brain, as in the vessels of the body generally, is conditioned by the 
state of the vessel walls and by the character and rate of flow of the 
circulating blood. 
The changes in the vessel walls are of prime importance, and are believed 
by many investigators to be essential factors in the production of thrombi. 
These changes are usually those of arteriosclerosis, and are particularly 
active when they have affected the intima of the artery, as in the forma- 
tion of an atheromatous plaque. In arteriosclerosis the walls of the 
vessels may be thickened, and the lumen so encroached upon, that the 
formation of even a small thrombus obliterates it. This, however, is 
more apt to occur in that type of arterial disease associated with syphilis. 
Biochemical alterations in the blood may predispose to thrombosis, 
but whether this occurs in a vessel whose walls are normal is a disputed 
question, and just what these chemical changes are is not known. In 
the acute infections of childhood, in typhoid fever, septic infections, etc., 
thrombosis of the cerebral arteries has occurred. In many of these, 
however, the thrombi have contained the specific microorganisms of the 
infection, which also have been found in the vessel walls themselves, 
so that even here the primary change may have been in the arterial 
walls. The blood may also carry larger particles about which, when 
lodged, for instance, at the bifurcation of a vessel, thrombi may develop. 
The altered condition of the blood in chlorosis seems to predispose to 
thrombosis, but the thrombi in this condition usually affect veins, partic- 
ularly the cerebral sinuses. The change in the rate of flow of the blood is 
of importance, for when the other conditions favoring the formation of 
thrombosis are present, a slowing of the blood current may be the deter- 
mining factor. Thrombi occur especially in those localities where the 
shape of the channel causes eddies or little whirlpools in the blood cur- 
rent. Such places may depend on the anatomical arrangement, but are 
more apt to be due to the changes in the vessel walls, for instance, those 
which produce local dilatations or definite aneurisms. Cerebral throm- 
bosis is generally stated to be more frequent when the circulation as a 
whole is feeble, due to weakness of the heart or to some other cause. 
Age of Onset.-The age at which we may look for cerebral thrombosis 
depends upon the conditions which have been stated. As it at times 
occurs in association with the acute infections, we may find it in this 
relation at any period of life, and it is probable that many of the cases 
of infantile hemiplegia are due to this factor. Thrombosis due to arterio- 
sclerosis is uncommon before the fortieth year, but in later life the 
conditions favorable to its occurrence are almost constantly present. DISEASES OF THE CEREBRAL BLOODVESSELS 
401 
Syphilis is a disease usually acquired in early adult life, and the involve- 
ment of the cerebral arteries, leading to their occlusion, may occur at 
almost any time after the primary infection. The writer has seen it occur 
in the first six months; and it appears that more cases develop in the 
first than in any other year after the primary lesion, and that they 
decrease in frequency with every following year. Cerebral thrombosis 
due to this cause is, therefore, a disease of the prime of life, and most 
cases occur between twenty and forty. 
Etiology (Embolism).-Emboli which lodge in the brain are, in the great 
majority of cases, derived from somewhere between the beginning of the 
pulmonary veins and the arteries of the cranial cavity. The most common 
source is the heart itself, as from vegetations on the mitral valves, and 
they are more frequently associated with mitral constriction than with 
any other cardiac condition. Emboli are also dislodged from the aortic 
valves or from thrombi on the arterial walls, and at times, although 
more rarely, are formed of bits of calcareous matter detached from 
an atheromatous plaque. If we include microorganisms circulating in 
the blood as emboli, they may enter the circulation at any point, 
and pass through the relatively large capillaries of the lungs to be 
lodged in the capillaries of the brain; but these are not usually so 
considered. 
Age of Onset.-There is no particular age at which cerebral emboli are 
apt to occur. This depends upon the time of life in which the conditions, 
favorable to their origin, are present. These are for the most part those 
conditions which produce valvular heart disease, and often occur early 
in life, but may be present at any time. Embolism is perhaps, on the 
whole, most frequent between late childhood and middle life. 
Etiology (Cerebral Hemorrhage).-The rupture of a cerebral vessel 
depends primarily upon the weakness of its walls, but to some extent 
upon the pressure of the blood within them. Under normal conditions 
the vessel walls are competent to withstand any strain put upon them, 
and it is probable that they must be diseased before they rupture. Various 
changes may so weaken the wall of a vessel that it gives way, and in rare 
instances hemorrhages have taken place from arteries affected with 
syphilitic disease-a process more apt to give rise to thrombosis. 
Since Charcot and Bouchard, in 1864, called attention to the presence 
of miliary aneurisms on the cerebral vessels in cases of apoplexy, it 
has been generally taught that it was the rupture of these structures 
that caused the hemorrhage. They are little dilatations, many of which 
can just be seen with the unaided eye, having a diameter from 0.1 to 1 
mm., and very many may be present, scattered along the nutrient vessels 
of the brain, but particularly along those which nourish the basilar 
nuclei. There has been much dispute concerning the exact pathological 
process upon which they depend, which coat of the artery is first diseased 
and their relationship to arteriosclerosis. Dilatations of various kinds 
occur on the cerebral vessels, as Virchow showed, and von Monakow1 
points out that it is more or' less a matter of choice what dilatations are 
1 Gehirnpathologie, second edition, Vienna, 1905, p. 1105. Full literature. 
vol. v-26 402 
DISEASES OF THE NERVOUS SYSTEM 
to be considered as miliary aneurisms, and that it is not always possible 
to distinguish them sharply from other dilatations. Rupture may occur 
in vessels whose walls have undergone a hyaline degeneration, or those 
which have undergone alterations secondary to a mechanical injury. 
All these conditions he considers as exceptions, and concludes: "By far 
the most common and perhaps the only certain cause for spontaneous 
cerebral hemorrhage must, according to my view, be sought in the 
bursting of miliary aneurisms. These begin with a degeneration of 
the muscular coat; an endarteritic process is not essential for their 
formation." 
A. G. Ellis1 studied by modern histological methods the arteries in 
31 cases of spontaneous cerebral hemorrhage, and Pick2 examined 11 
other cases by a special method to determine the occurrence of miliary 
aneurisms and the source of hemorrhage in each case. These authors 
conclude that aneurisms of true miliary size are very generally, although 
not constantly, present in cases of cerebral hemorrhage. Pick was unable 
to associate them with the hemorrhages. These came from the rupture 
of large aneurismal swellings, or generally from diseased vessels without 
such swellings. These aneurisms, the smallest as well as those of larger 
size, are all, they believe, either dissecting or false aneurisms. The 
changes in the coats of the vessels, that permit of their occurrence, 
differ histologically in no respect from those seen in arteriosclerosis, the 
primary lesions being apparently in the elastic layer of the intima. 
All the effective causes in the production of arterial disease may be 
included among the predisposing causes of cerebral hemorrhage. It is 
interesting to note how some families show a strong tendency to the 
occurrence of cerebral hemorrhage at a relatively early period of life. 
This hereditary weakness of the vascular system may be shown by other 
vascular accidents-the early occurrence of general arteriosclerosis, 
angina pectoris, and the frequency of sudden death--even when apoplexy 
has not been particularly common in the family. Renal disease with 
hypertrophy of the left ventricle and an increased arterial pressure is 
frequently present in cases of cerebral hemorrhage. The increased arterial 
tension is believed to be an important agent in the production of the 
vascular changes, and is also a factor in the rupture of the vessel. Any 
great increase of the pressure of the blood within a vessel with weakened 
walls would tend to cause a break, and so increased arterial pressure is 
given as a common, exciting cause of cerebral hemorrhage. This increase 
may be produced during a sudden muscular effort or a fit of anger-con- 
ditions which increase the activity of the heart. If the outflow of the 
blood from the cranial cavity be impeded, as when there is a great rise 
of pressure on the venous side of the circulation, as during strong expira- 
tory effort, the pressure within the veins throughout the brain is increased, 
and this increase is extended to the capillaries and even to some extent 
into the arterioles, and may produce their rupture. This is still more 
likely to occur when combined with an increase of arterial pressure, as 
1 Proc. Path. Soc., Philadelphia, September, 1909, p, 197, 
2 Berl. klin. Wchnschr., February 21 and 28, 1910. DISEASES OF THE CEREBRAL BLOODVESSELS 
403 
during strong muscular effort. Such conditions are believed to be present 
during the intense respiratory spasms of whooping cough, when intense 
muscular efforts are made, the breath being held, as in lifting heavy 
weights, etc.-circumstances under which cerebral hemorrhage is said 
to be common. It will, however, be shown later how infrequently one 
is able to discover any such exciting cause. 
Age of Onset.-Cerebral hemorrhage is uncommon before the fortieth 
year, but may occur at almost any time in life. It is not an infrequent 
complication of difficult births, but these cases may, for the most part, 
be considered traumatic, resulting from the pressure on the child's head. 
True cerebral hemorrhage, however, does seem to occur during the first 
years of life, and the liability to it increases with each decade. 
Pathology (Cerebral Softening, Encephalomalacia, Thrombosis, Embo- 
lism).-The effect of the occlusion of a cerebral vessel depends upon how 
completely the blood is shut off from that portion of the brain supplied 
by the vessel implicated. There is an extremely rich anastomosis between 
the vessels at the base of the brain, and theoretically the obstruction of 
a vessel emptying into the circle of Willis should have no effect, but 
unfortunately the channels which connect the various parts are not 
always present, or if present are not always of the normal size. Even 
when the anatomical relations have been quite normal, certain of the 
connections may be partially or completely obstructed. It is, therefore, 
impossible to be quite sure of the effect of occlusion, either by disease or 
ligature of the large vessels. On dogs it has been possible to tie both 
internal carotids and both vertebrals, provided a sufficient time has 
intervened between the various ligatures. In these cases the brain 
receives its arterial blood from the anastomosis with the spinal system. 
In man, the ligature of one internal carotid is a procedure attended with 
considerable risk. Serious brain symptoms have occurred in about 25 
per cent, of the cases, and death has followed in nearly 10 per cent. 
(Jordan1). Certain of these results may possibly be avoided if the 
closure of the vessel is done slowly so as to allow the collateral circulation 
to be established, and it might be possible to ligate safely both common 
carotids in man if this procedure were followed and sufficient time 
elapsed between the two ligatures (Leonard Hill), but abnormalities of 
the circle of Willis must always be taken into account. 
The larger arteries after leaving the circle of Willis supply different 
areas of the brain, but there is more or less free communication between 
the vessels and it is quite possible to inject the whole brain from any 
one vascular trunk. The ease with which this is done depends upon the 
normal condition of the arteries, and in brains of older people, when 
arterial changes become more common, it may be impossible to do so 
completely, and the occlusion of one of the larger arteries, at its origin 
from the circle of Willis, is usually followed by more or less softening. 
Cases have been recorded in which such occlusion has occurred without 
any softening having followed. When a thrombus extends, as it com- 
monly does, so as to implicate the origin of the nutrient vessels which 
1 Verhandl. der Deut. Gesellschaft f. Chir., 1907, xxxviii, 83; Becker, ibid., p. 623. 404 
DISEASES OF THE NERVOUS SYSTEM 
enter the base of the brain, the softening constantly occurs in the gray 
nuclei which they nourish, for these vessels are end arteries. 
The changes which occur in the brain substance, following a closure of 
its nutrient vessels, have given rise to much discussion. The highly 
differentiated nervous tissue is probably more susceptible to a decrease 
in its vascular supply than any other tissue and at times a destruction 
of this tissue occurs from the blocking of an artery, which is apparently 
connected with other vessels by free anastomosis, as, for instance, one 
of the pial vessels. In general, necrosis occurs whenever the vessels which 
enter the brain substance, the so-called nutrient vessels, are blocked. 
The area of ischemic necrosis which follows varies in position and size, 
depending upon the number of vessels occluded. 
If the brain be examined shortly after the vascular occlusion has 
occurred, the necrosed area will be found to have a white or a reddish 
appearance, being more apt to be red when situated in the cortex where 
the vascular supply is particularly abundant. There is usually marked 
oedema which may extend beyond the boundaries of the softened region, 
and be so great as to cause a definite swellmg of a considerable portion 
of the brain. In certain cases this oedema is the only objective finding 
to account for the marked symptoms which have followed a vascular 
occlusion, as in death after ligature of the common carotid. 
The microscopic appearance of the necrosed area is characteristic. 
It is only in its centre that there is a complete disintegration and here one 
finds a diffluent mass Composed of the detritus of the nervous elements, 
and every now and then a compound granular corpuscle. Around 
this the tissue shows more or less marked irritative reaction, the blood- 
vessels are prominent, due particularly to proliferation of the cells of their 
walls and the collection of leukocytes in the surrounding tissue. Com- 
pound granular corpuscles are extremely common; newly formed neu- 
roglial elements are present, and there is a greater or less amount of 
blood, either in the vessels or free in the tissue; or there may be actual 
small hemorrhages. The color of the infarct depends upon the number 
of these red blood cells. The nervous elements in this region are found 
swollen and in various stages of destruction. If examined after one or 
two weeks the processes of repair are evident. The red blood pigment 
is becoming altered, the phagocytes, compound granular corpuscles, 
leukocytes, etc., have taken up and are removing the destroyed tissue, 
and there is a distinct increase of the supporting tissue, the neuroglia, 
and the true connective tissue. If the area is a small one this process 
may entirely replace it by tissue which usually contains minute cavities 
scattered through it. In larger areas there is a tendency to cystic forma- 
tion with more or less definite walls. The process which appears to have 
the most clinical bearing is the oedema, for it is to this that many of the 
symptoms may be due. It may be so great as to cause a dangerous 
increase in the intracranial pressure and account for definite pressure 
symptoms. To this also may be due many of the initial focal symptoms 
from which the patient recovers. It is a process which occurs frequently 
in the nervous tissue, and different theories have been advanced to DISEASES OF THE CEREBRAL BLOODVESSELS 
405 
account for it. Cannon,1 in a study of the effects of trauma on the brain, 
has paid particular attention to this point, and comes to the conclusion 
that the process depends upon the altered osmotic relations due to changes 
in the nervous tissue, secondary to vascular disturbances. 
When the thrombi which occlude the vessels are infected with 
pyogenic microorganisms there may be the production of an abscess 
in the brain. 
Pathological Anatomy of Hemorrhage.-When a hemorrhage occurs 
from the rupture of an artery of fair size, blood is thrown out under press- 
ure, nearly equal to that of arterial pressure and much above the cerebral 
pressure, which is about equal to venous pressure. The surrounding 
nervous tissue is broken up by the mechanical impact of the blood. 
The extent of the destruction varies with the amount of blood thrown 
out and the character of tissue into which it is thrown. The white 
matter is more easily disintegrated than the gray matter, and a hemor- 
rhage in it is apt to produce a larger area of destruction than when 
confined to the gray substance. The size of an intracranial hemorrhage 
varies greatly. It is usually no larger than a walnut, but it may at 
times occupy almost the whole of the cerebral hemisphere, or be very 
small. 
In the tissue about the actual clot there are usually minute hemorrhages 
which give it a blood-stained appearance, and a hemorrhage of any 
considerable size is nearly always surrounded by an area of more or less in- 
tense oedema. This oedema may be an important factor in the production 
of the symptoms, and it is not at all uncommon to find softened areas in 
the neighborhood of a hemorrhagic focus. If the patient survives the 
initial shock, processes of repair begin very quickly. There is an endeavor 
to absorb the clot from the periphery and the formation of a cyst. At 
first the walls of the cyst are formed of a loose tissue, but this tissue 
becomes firmer as it gets older, and in cysts of a year or more standing 
it is a firm connective-tissue capsule, which has on its inner surface a 
layer of cells of various kinds, stained with the remains of blood pigment. 
The rapidity of this cyst formation varies greatly, but even in relatively 
small foci the blood clot can usually be found up to four or five weeks, 
after which time the cyst formation becomes more and more definite. 
APOPLEXY. 
The symptoms following acute vascular lesions of the brain, whether 
the process be the rupture of a vessel or its occlusion, are in many respects 
identical; and clinically it is often impossible to determine w'hich process 
has been effective. For this reason the symptomatology of these different 
processes is considered together under the general term of apoplexy. 
The word apoplexy has a most interesting history. It was used by 
the Greek and Latin medical authors, and originally meant "to strike off 
or be disabled by a stroke." It appears in middle English, and Chaucer 
1 Amer. Jour, of Phys., 1901, vi, 91. 406 
DISEASES OF THE NERVOUS SYSTEM 
uses it near the beginning of his "Nonnes Priestes Tale." It was in 
general literary use in the time of Queen Elizabeth, and Shakespeare 
makes Falstaff (Henry IV, Second Part, Act I, Scene II) use it as an 
interesting subject of conversation. 
Apoplexy was used in medicine to signify a set of symptoms-a more 
or less sudden and complete abolition of consciousness with loss of feeling 
and motion, respiration being maintained. As Sydenham has it: "Pro- 
found sleep, utter loss of sense and motion, with the exception of that 
necessary for respiration." It came to be used almost synonymously 
with coma, and Abercrombie constantly uses such expressions as "The 
patient was found in complete apoplexy." The apoplectic attack was 
often spoken of as a fit or a stroke-the ictus. After it became generally 
recognized that the cause of apoplexy was commonly a hemorrhage in the 
substance of the brain, the pathological condition itself began to be 
called apoplexy, and in the early part of the last century the term was 
extended to signify any sudden interstitial hemorrhage, as "pulmonary 
apoplexy," " renal apoplexy," etc. Todd and most of his contemporaries 
used the term apoplexy as indicating hemorrhage into the brain, but 
Trousseau insisted on its original symptomatic meaning. At the present 
time the best English usage is reverting to the original meaning, although 
the word even yet bears with it a distinct idea of hemorrhage. In the 
present article apoplexy is used more nearly in its original symptomatic 
sense, and made to include the cases due to obstruction of the cerebral 
vessels as well as those following a rupture. 
Occurrence.-The frequency of apoplexy may be approximately judged 
from a study of mortality statistics. The Seventh Annual Report of 
the Census Bureau gives the figures for 1906. These cover those States 
whose mortality reports are deemed sufficiently reliable (a population of 
40,996,317, almost one-half the population of the United States). There 
were 658,105 deaths from all causes, a death rate of 16.1 per thousand. 
Among these 29,434 were ascribed to apoplexy, and 6933 to paralysis. 
Many of these latter cases must have been due to previous attacks of 
apoplexy, although some were probably of an entirely different character. 
They are, however, considered together, and the total of 36,367 has prob- 
ably as much chance of being below as above the right figures, for there 
are many cases included in the mortality statistics under the heading of 
softening of the brain and a general paralysis of the insane, which should 
properly be classed among the apoplexies and are not included in these 
figures. The total of 36,367 deaths from apoplexy and paralysis gives a 
death rate of 88.7 per 100,000 of the population. 
In Baltimore, with a population of 533,699, there were during the year 
544 deaths from apoplexy and paralysis. The expected deaths among 
such a population would be 490. The increased death rate depends upon 
the number (124) of deaths in colored individuals from these causes 
(29.5 per cent, of the deaths were in negroes, whereas the negro population 
forms not quite 19 per cent, of the whole population of Baltimore). 
This greater liability of the negroes to die from apoplexy and paralysis 
is quite parallel with their greater liability to diseases of the circulatory 
system. The Census Report shows that among 100,000 of the population DISEASES OF THE CEREBRAL BLOODVESSELS 
407 
of Baltimore there were 161.3 deaths from such causes among the whites 
and 327.4 among the blacks. 
Etiology.-Predisposing Causes.-These are the same as those in the 
primary vascular lesions. Whatever tends to produce these lesions has a 
bearing on the production of apoplexy. 
Race.-The figures from the Census Report indicate that there is a 
distinct tendency in the negro race to diseases of the circulatory system, 
and also a greater liability to apoplexy. In the writer's series there were 
152 negroes among 674 individuals suffering from hemiplegia in whom 
the race was noted. 
Sex.-Men are somewhat more liable to apoplexy than women. The 
proportion among 1000 of the aggregate population within the Registra- 
tion States was in 1900, 505.8 men and 494.2 women, while among 1000 
deaths from apoplexy and paralysis in 1906 there were 504.5 men and 
495.5 women. The tables (1913) compiled by the association of the life 
insurance medical directors show a slightly greater liability of the male 
policy holders to apoplexy. It is the most common cause of death in 
men who have taken their policies out after forty-five years of age, while 
in women of a corresponding age it stands fourth. In the records of the 
Johns Hopkins Hospital there is a marked preponderance of males, 
there being 489 males and 251 females, but in this hospital many more 
men are treated than women. 
Build.-The popular belief that stout individuals, with short, thick 
necks, are more liable to apoplexy is confirmed by the insurance tables, 
for the ratio of deaths due to apoplexy among 10,000 lives exposed to risk 
is nearly four times as great in the overweight as in the underweight group 
and about twice that for the standard lives. 
Age.-The vascular changes upon which the apoplexy depend are in a 
large proportion of cases conditioned by incidents of the life of the 
individual, and increase in frequency with each succeeding decade, and 
the frequency of apoplexy also increases with each decade. 
In the mortality statistics a large number of apoplexies are recorded 
as occurring under one year of age. These must have been composed 
largely of traumatic apoplexies occurring at the time of birth, and give an 
undue preponderance to this time of life. The following tables, condensed 
from the Census Report, show the occurrence of apoplexy-in the various 
decades, and give a fair idea of the relative frequency of apoplexy at 
the different periods. The frequency is, of course, not the same thing 
as the liability, for the number of cases w'hich occur at any period of 
life must depend upon the number of people alive at that age. The 
estimated population in the Registration States in 1907 was distributed 
among the decades according to the ratios found in the census of 1900, 
and compared with the number of deaths in each decade which were 
reported during 1907. The error introduced cannot be great. The 
relative liability in the different decades is expressed in proportion, taking 
the liabilitv in the second decade as 1. 408 
DISEASES OF THE NERVOUS SYSTEM 
Table I.-Deaths from Apoplexy and Paralysis. Census Report for 1907. 
Decade. 
Cases. 
Proportion per 1000 of 
deaths. 
First 
670 
20.1 
Second 
121 
3.6 
Third 
370 
11.1 
Fourth 
998 
30.0 
Fifth 
2,250 
67.7 
Sixth . 
4,836 
145.4 
Seventh 
8,491 
255.3 
Eighth 
9,877 
297.0 
Ninth 
5,100 
153.4 
Tenth 
544 
16.4 
Total 
33,257 
1000.0 
Table II.-Table Condensed from the Census Report for 1906. 
Population of Registration States in 1907, 
distributed according to ratios found 
in 1900. 
Deaths from apo- 
plexy and par- 
alysis in 1907. 
Liability per 
100,000 
population. 
Relative 
liability. 
First decade . 
6,984,883 
670 
9.6 
5 
Second decade 
6,204,278 
121 
2.0 
1 
Third decade 
6,260,922 
370 
5.9 
3 
Fourth decade 
5,171,628 
998 
19.3 
10 
Fifth decade . 
3,760,575 
2,250 
59.8 
30 
Sixth decade . 
2,550,957 
4,836 
189.6 
95 
Seventh decade . 
1,579,307 
8,491 
537.6 
269 
Eighth decade 
734,016 
. 9,877 
1,345.6 
673 
Ninth decade 
180,990 
5,100 
2,817.8 
1,409 
Tenth decade. 
13,406 
544 
4,057.9 
2,029 
. 
- 
The cases in each decade differ somewhat as to their etiology, and 
each decade will be considered separately. In the Johns Hopkins 
Hospital there have been 292 cases of apoplexy, i. e., patients suffering 
either from the acute condition or the subsequent paralysis, in the 
medical wards among 23,048 medical admissions. In the Out-patient 
Department 448 cases of hemiplegia and other results of acute lesions 
were seen. The cases registered in both hospital and dispensary were 
included only among the hospital cases. The following table shows their 
occurrence in the various decades: 
Table III. 
Decade. 
Males. 
Females. 
Total. 
First 
58 
77 
135 
Second 
16 
9 
25 
Third 
43 
19 
62 
Fourth 
73 
29 
102 
Fifth 
88 
49 
137 
Sixth 
99 
44 
143 
Seventh 
86 
19 
105 
Eighth 
20 
5 
25 
Ninth 
6 
0 
6 
■ 
■ - 
■ 
Total 
489 
251 
740 409 
DISEASES OF THE CEREBRAL BLOODVESSELS 
Decade I.-The cases of hemiplegia in the first decade are of especial 
interest, and in large part are the result of processes peculiar to that 
period. In the Census Report for 1906 a large proportion of cases of 
death from apoplexy and paralysis in the first decade occurred in the 
first year, 621 among a total of 961. These must include those dying 
shortly after birth with symptoms indicating injury to the brain. In the 
Johns Hopkins Hospital records there were 135 cases of hemiplegia which 
had appeared in the first decade. Of these, 32 are classed as congenital, 
and in 16 the onset occurred during the first year. This can be only 
relatively accurate, as it is often impossible to determine from the history 
whether the paralysis was due to conditions present at birth or to those 
which occurred later. When the parents believed the paralysis to have 
been always present, and when nothing in the history indicated a later 
onset, it has been classed as congenital. In other cases the history is 
so clear that little doubt can be possible. 
Congenital hemiplegias are in most instances due to accidents occurring 
at birth. As the head of the infant passes through the pelvic canal of the 
mother it is subjected to great pressure, which in the vast majority of 
cases the head is well adapted to withstand, but at times the pressure 
is great enough to injure the brain, either directly or, as more often, by 
means of a hemorrhage within the skull. Very rarely the paralysis is 
the result of a prenatal process, i. e., something that has affected the brain 
of the fetus before labor has begun. Prenatal pathology in so far as 
it relates to the central nervous system is an obscure subject. Bal- 
lantyne,1 in his large work, has but little to say about it. Gross defects 
of development in the brain and spinal cord have, indeed, been frequently 
described. These may be so great as to lead to the entire absence of the 
whole central nervous system, or of the brain or of a large part of it. 
The brains of newborn infants show at times relatively small defects, 
even when they may be otherwise normally developed. These consist of 
cavity formation involving to a greater or less extent the cortex and the 
white matter beneath it (porencephaly), atrophy of the convolutions 
(microgyria), and sclerosis in certain portions of the cortex or tracts 
within the central nervous system, the pyramidal tracts especially. 
Quite similar conditions are frequently found at autopsies performed on 
cases of infantile cerebral palsies, both in those which are presumably 
congenital and also in those which appeared during the first years of life. 
It is to the study of these end results of various initial lesions that our 
knowledge of such conditions is mostly due. 
The term porencephaly has been used to describe a great number of 
conditions. It was first introduced by Heschl, in 1859, but it was not 
until 1880 that especial attention was drawn to it by Kundrat, from whose 
monograph our knowledge may be said to date. Kundrat and most 
other writers believe that these cavity formations are usually due to 
some prenatal condition, especially to some disturbance in the circulation. 
These defects occur most often in the central convolutions, and the blood- 
vessels supplying this region have often, but not always, been found 
1 Antenatal Pathology and Hygiene, The Fetus, Edinburgh, 1902, p. 388. 410 
DISEASES OF THE NERVOUS SYSTEM 
diseased. The vascular obliteration has been ascribed to thrombosis 
and embolism during fetal life, but there do not seem to be many facts to 
substantiate this. Inflammation of the brain substance, with secondary 
softening, is also given as the starting point of the process. That this 
is actually a cause in prenatal cases seems also to be based on very few 
facts. Seitz1 after an analysis of the cases adduced in proof of this belief, 
first advanced by Virchow2 in 1867, came to the conclusion that the 
lesions described under the heading of congenital encephalitis have 
resulted from cerebral hemorrhage, or are extra-uterine from specific 
infections, possibly also intoxications. He concludes that congenital 
encephalitis in the sense that Virchow used it has not been proved to 
exist. On the other hand, he believes that porencephaly found at birth 
may be due to a cerebral hemorrhage during fetal life and records the 
case of a baby who died five hours after an easy birth. At the autopsy 
a large defect in the anterior and middle parts of the cerebrum was found. 
The white matter was replaced by a cavity which involved also the 
cortical substance. The basal ganglia, the pons, cerebellum, and medulla 
oblongata, were all normally formed. Microscopic examination of the 
walls of the cavity gave evidence of a former hemorrhage. The mother 
of the child had received a severe trauma to the abdomen when four 
months pregnant, which Seitz believed caused an intracerebral hemor- 
rhage in the fetus. 
The occurrence of hemorrhage within the fetal brain is certainly rare. 
Seitz was unable to find in the literature any case quite similar to his, 
and Ballantyne, in proof that such a condition may occur, refers to only 
one case, that recorded by Osler.3 It was in the fetus of a woman six 
months pregnant, who died of typhoid fever. The hemorrhage was in 
the left cerebral hemisphere and had broken through into the ventricle. 
There was also general oedema of the brain. 
Gibb4 records a case of a stillborn child who had a contracture of the 
left arm and leg and in whose right cerebral hemisphere an old clot was 
found above the lateral ventricle. The mother of the child had received 
a severe blow on the right side of the abdomen three months before labor. 
These cases show that intracerebral hemorrhage does occur during fetal 
life, both following a trauma to the abdomen of the mother and during 
the course of an acute illness. Freud refers to a clinical case recorded 
by Gaudard, in which the mother received a severe blow on the abdomen 
in the sixth month of pregnancy. The child was born with paralysis 
of the right arm. 
The intra-uterine causes of microgyria and lobar sclerosis are not well 
understood, but these conditions are generally believed to follow some 
disturbance of the blood supply to the developing brain. Lack of develop- 
ment of the pyramidal tracts, which is included among the prenatal causes 
of the cerebral palsies, appears to be the result of the cerebral defect, and 
not an independent condition. 
As has been stated, the great majority of congenital cerebral palsies 
are due to accidents during birth. The association of difficult labor, 
1 Arch. f. Gyn., 1907, Ixxxiii, 701. 2 Virchows Archiv, 1867, xxxviii, 127. 
3 Teratologic, 1895, ii, 13. 4 Lancet, 1858, ii. DISEASES OF THE CEREBRAL BLOODVESSELS 
411 
asphyxia, and premature birth with the spastic paralysis of children was 
pointed out by Little,1 but that these paralyses were due in most cases 
to hemorrhage within the cranium was first clearly shown by Sarah J. 
McNutt.2 Little was well aware of the frequency of intracranial hemor- 
rhage at the time of birth, but, as at that time the cortex was not known to 
have anything to do with motion, he believed that the spastic paralysis 
was due to hemorrhage about the spinal cord. McNutt showed that 
the hemorrhages incident to birth were apt to be most intense about the 
fissures of Rolando, and she assumed that the atrophy of the central 
convolutions was due to a bilateral hemorrhage limited for the most 
part to this region. 
In most instances these cases are associated with abnormal labor, but 
large and even fatal intracranial hemorrhages do occur when the birth is 
apparently normal and easy. Marked changes of pressure in the circula- 
tion of the infant must take place during labor, especially when placental 
circulation ceases and that through the lungs is established. When the 
child is asphyxiated the circulatory conditions present are particularly 
favorable for the rupture of vessels, a fact on which Little laid stress. 
Small capillary hemorrhages within the various tissues of the infant 
are an almost constant accompaniment of birth. Seitz,3 in speaking of 
this fact, refers to an investigation by Paul, who made an ophthalmoscopic 
examination on 200 newborn children, and found retinal hemorrhages in 
a large proportion of cases-in 50 per cent, of those in which the mother's 
pelvis was contracted, in 40 per cent, when the labor was protracted and 
complicated, in 40 per cent, when the labor was premature but otherwise 
normal, and in 20 per cent, of normal labors. 
Undue pressure on the child's head, due either to a disproportion 
between its size and that of the mother's pelvis, or to the faulty applica- 
tion of forceps, may cause direct injury to the brain, or, as is much more 
common, result in intracranial hemorrhage. The bleeding is usually 
from a rupture of one of the venous sinuses, or of veins emptying into 
them. The vessels can be injured by the bones of the skull being forced 
one under the other, or by being implicated in a tear of the teptorium. 
Beneke4 and a number of others have pointed out how frequently 
the tentorium is torn during difficult labor, due, they think, to stretching 
of the dura caused by compression of the head. They regard this as by 
far the most important cause of the birth hemorrhages. Seitz5 although 
confirming the frequent occurrence of injury to the tentorium, insists that 
this can account for at most but half of the cases, and cannot be the cause 
of clots confined to the convexity of the hemispheres, and which are due, 
without doubt, to rupture of the longitudinal sinuses or its veins. 
Some idea of the frequency of these meningeal hemorrhages during 
birth can be gathered from the records of autopsies done on stillborn 
babies and on those which died shortly after birth. Peterson6 refers to 
1 Trans. Obstet. Soc. London, 1861, iii, 293; also Treatise on Deformities, 1853. 
2 Am. Jour: Med. Sc., 1885, clxxvii, 58; Am. Jour. Obst., 1885, xviii, 73. 
3 von Winckel, Handbuch der Geburts., 1907, iii, 49. 
4 Munchen, med. Wchnschr., October 11, 1910, p. 2125. 
& Zentralbl. f. Gynak., 1912, xxxvi, 1. 
6 Nervous and Mental Diseases, Church and Peterson, 1908, p. 894. 412 
DISEASES OF THE NERVOUS SYSTEM 
the figures given by Litzmann, who found 35 cases of meningeal hemor- 
rhage among 161 stillborn children. Parrot, in 34 autopsies of the new- 
born, found 5 with blood in the arachnoid cavity and 26 with hemorrhage 
into the subdural space. H. R. Spencer, in 130 autopsies on stillborn 
children, found 53 instances of hemorrhage from the pia and arachnoid. 
Holt and Babbitt1 in their analysis of 10,000 confinements at the 
Sloan Hospital in New York, found among 9318 living births that 291 
infants died during the first two weeks. The cause of death was given as 
intracranial hemorrhage in 19 cases, and as injury to the head in 10. 
The 429 stillbirths were not analyzed in this relation, but among the 
196 ascribed to accidents of labor, many intracranial hemorrhages must 
have occurred. 
Although the hemorrhages occurring during labor are in the great 
majority of cases meningeal, they do occur at times within the brain 
substance. Weyhe, in an analysis of the records of the Pathological 
Institute at Kiel, in 959 autopsies in young infants found 122 cases in 
which intracranial hemorrhages had occurred. In many cases there 
were hemorrhages in more than one location. A clot was found 80 
times in connection with the dura and 56 times with the arachnoid; in 
35 instances it occurred within the substance of the brain, and in 21 
within the ventricles. There were 32 instances of relatively large hemor- 
rhages; 5 of these large hemorrhages were intracerebral, and in all but 
one case it seems fair to assume that the rupture took place at birth. 
Postnatal Cases.-Hemiplegia in children due to causes effective after 
birth may occur at any age, but is much more frequent in the first two 
years. This is well shown by Table IV, which is compiled from that 
given by Freud,2 with the addition of the writer's collected cases. Of 
the 607 cases recorded in the first decade, 344 (56.7 per cent.) were in the 
first two years, and 516 (85 per cent.) in the first half of the decade. 
Table IV. 
Congenital. 
Up to 1. 
Up to 2. 
Up to 3. 
Up to 4. 
Up to 5. 
6 
o 
Osler 
15 
45 
22 
14 
1 
3 
9 
Wallenberg 
19 
35 
29 
17 
9 
9 
30 
Gaudard 
11 
17 
7 
5 
8 
2 
14 
Lovett 
7 
5 
12 
Strumpell 
.. 
7 
8 
4 
5 
Sachs and Peterson 
22 
27 
17 
16 
4 
4 
8 
Gowers 
23 
23 
14 
10 
10 
Freud-Rie 
2 
3 
11 
9 
3 
3 
2 
J. H. H. cases 
32 
16 
37 
17 
8 
7 
18 
Total 
108 
178 
166 
96 
48 
28 
91 
The mortality statistics of the United States Census Report for 1906 
show the same thing more strikingly, for of the 961 deaths recorded as 
due to apoplexy and paralysis in the first decade, 882, or nearly 92 per 
1 Jour. Am. Med. Assn., 1915, Ixix, 287. 
2 Die Infantile Cerebralldhmung, Nothnagel, 1897, p. 44. DISEASES OF THE CEREBRAL BLOODVESSELS 
413 
cent., occurred in the first five years. Although these figures are exclusive 
of stillborn children, they probably include many children who died 
shortly after birth from accidents incident to it, for 621 died within the 
first year and only 127 in the second. 
It is also probable that a considerable number of the cases of hemiplegia 
included in Freud's table as developing in the first and second years were 
really congenital. For instance, of the 16 Hopkins cases in the first 
year, the histories of 5 merely stated that the paralysis was noticed at 
varying times during the infancy: once when the child was one month 
old, once when four months, twice when six months, and once when eight 
months; in none of these cases could the parents be sure that the condition 
had not existed since birth. In 5 of the 37 cases in the second year the 
disability had become apparent when the time arrived for the children to 
begin to walk, and here again no definite onset could be determined, so 
that these 10 cases might fairly have been added to the 32 congenital ones. 
Etiology.-The cerebral changes which underlie the hemiplegias 
developing after birth and during infancy are, in general, the same as 
those which are present at later periods of life, but are influenced by special 
factors. The vascular system of a child is much less apt to show per- 
manent changes, and on this account the spontaneous rupture or the 
occlusion of a bloodvessel is less apt to occur. On the other hand, acute 
infections seem especially prone to affect the brains of the young. Table 
V represents the etiology of the cases in the first decade. These are 
distributed by years in the first half of the decade and in the last half are 
considered together. 
Table V. 
Congenital, 32; probably congenital, 10; total, 42. 
Postnatal cases. 
Year of onset. 
Total 
1 
2 
3 
4 
5 
5 to 10 
Acute infectious diseases: 
Whooping cough 
1 
1 
1 
1 
1 
1 
6 
Typhoid fever 
1 
1 
1 
2 
5 
Measles 
2 
1 
1 
4 
Diphtheria 
1 
3 
4 
"Sore throat" 
1 
1 
Croup 
1 
1 
Gastro-intestinal trouble 
1 
1 
1 
3 
Marasmus 
1 
1 
Pneumonia 
1 
1 
Vaccination 
1 
1 
"Fever, brain fever," etc 
After convulsion without other cause: 
4 
4 
3 
1 
1 
2 
15 
With marked brain involvement . 
1 
3 
1 
5 
Convulsions at onset .... 
2 
12 
3 
1 
1 
19 
Developed in course of epilepsy 
1 
1 
Congenital syphilis 
1 
1 
2 
4 
Brain tumor 
*3 
3 
Trauma 
3 
2 
1 
6 
Heart disease 
1 
1 
2 
Poisoning 
1 
1 
Hysteria 
1 
1 
Without determined cause .... 
2 
3 
1 
1 
2 
9 
Total 
11 
32 
17 
8 
7 
18 
93 414 
DISEASES OF THE NERVOUS SYSTEM 
Hemorrhage.-The rarity of intracerebral hemorrhage in childhood is 
commented on by most writers. Meningeal hemorrhages are more 
common, but even these, when not associated with trauma or with one 
of the acute infectious diseases, are rare. Sachs1 thinks that hemorrhage 
occurs more frequently than most authors admit, and seems to regard it 
as the most common cause of these paralyses, even of the cases that 
develop after birth. The bleeding, he believes, is from a cortical vessel, 
although it may be from a vessel within the depths of the brain. 
Alterations in the vascular walls may occur in childhood as an early 
degeneration, or more frequently as the result of some acute infection. 
Sachs cites von Recklinghausen's statement that fatty degeneration of 
the cerebral vessel is not uncommon in children. The rupture of such a 
diseased vessel must account for some exceptional cases of apoplexy 
of the first decade, and a very few of the writer's cases may best be so 
explained. However, there is no record in the Johns Hopkins Hospital 
of the occurrence of a postnatal intracerebral hemorrhage in a child under 
ten years. This hospital admits relatively few children, and the rarity 
of such cases can be better judged from other records. Holt says that 
he has seen but one case. Among 830 autopsies at the Babies' Hospital 
in New York, the records of which the Staff assisted me in examining, 
there is no such case, and Dr. John Howland informed me that in 1416 
autopsies at the Foundling Hospital there was just one intracerebral 
hemorrhage. Sidney Phillips2 reports the occurrence of a spontaneous 
hemorrhage into the pons Varolii of a girl, aged fourteen months. The 
child, who was apparently well when put to bed at 3 p.m., was found 
comatose an hour afterward, and remained so for the fourteen days which 
she lived. No cause for the hemorrhage was discovered. 
Among the 93 postnatal cases there are 9 classed as developing without 
known cause, i. e., so far as could be discovered; the paralysis developed 
more or less suddenly in children who were not sick at the time, very 
much as we are in the habit of seeing an apoplexy occur in the late decades. 
The history of the onset in five of these cases is so incomplete that some 
or all of them may well belong to other classes. Two of the four remaining 
cases are of particular interest. The first was a girl, aged six years, who, 
while in apparent health, fell, paralyzed on her right side, but was not 
unconscious for any considerable time. The paralysis improved, but 
intense athetosis followed in the arm, for which she was admitted on 
six different occasions, the last time when she was twenty-five. The 
other case occurred in a girl, aged nine years. She was at her desk in 
school when she fell unconscious, and remained in a more or less dazed 
condition for nearly two weeks. The paralysis was on the left side and 
cleared up well. When twelve she had a second attack, which followed a 
right-sided headache, and came on while she was walking around her 
room. She fell, paralyzed again on her left side, but without loss of 
consciousness. The power again returned, but rather more slowly and 
athetosis followed. The onset in these cases was so characteristic of a 
vascular lesion that it was difficult to think of any other explanation. 
1 The Nervous Diseases of Children, second edition, 1905. 
2 Lancet, 1909, i, 1680. DISEASES OF THE CEREBRAL BLOODVESSELS 
415 
No sources of emboli were found, and we have been led in each case to 
assume the occurrence of either a rupture or a thrombus of one of the 
arteries in the substance of the brain. 
In the other two cases the paralysis came on at the eighteenth or nine- 
teenth month. In one case the onset was sudden; the baby was uncon- 
scious for ten days and was paralyzed on the right side. In the other 
case the onset was rather gradual, in the course of one or two days. 
Hemorrhage has been the assigned pathological condition in many of 
the cases of hemiplegia associated with definite etiological factors. This 
would seem justified in certain of the cases developing after injury to the 
head when the skull had not been fractured, although the possibility of 
an encephalitis must not be forgotten. With fracture of the skull, 
hemorrhages, particularly from the meninges, are common, no matter at 
what time of life the accident occurs, and even when the fracture is not 
apparent it cannot always be excluded. In only one of the traumatic 
cases was the skull known to be injured, and in that there was a perforat- 
ing wound.1 In one case the fall may have been merely coincident with 
the attack of paralysis. This was in a girl, aged two years, who had 
been sick with some fever and who fell from a little cart. When picked 
up she was paralyzed on the left side. In the other four cases the falls 
were of sufficient gravity not to be overlooked as etiological factors. 
Cerebral hemorrhage during infantile convulsions has been assumed as 
the cause of many of the early hemiplegias. The fact of the sequence of 
convulsions and paralysis is most striking, and the supposition that blood- 
pressure changes, incident to the fit, may have caused rupture of the 
bloodvessels is attractive. That the vessel walls, on the other hand, are 
in the vast majority of cases amply able to withstand any increase of 
pressure incident to the most severe convulsions, is shown by the rarity 
of their rupture in cases of epilepsy. Capillary hemorrhages are, indeed, 
frequentlv found in patients dying in the status epilepticus, but these do 
not appear to be large enough to account for paralytic symptoms. In 
none of the cases of this series did the paralysis follow a fit in an epileptic, 
unless two cases in the first decade may be so considered. The more 
striking of these was that of a hemiplegic idiot seen at seven, who began to 
suffer from convulsions at eight months, which recurred every two or 
three weeks. When two years old the patient had a severe attack lasting 
four hours, after which his mother noticed that he was paralyzed on the 
left side. He did not walk again for more than a year. A single convul- 
sion recurred six months after the attack of hemiplegia, and there was then 
an interval of four and a half years, after which he had numerous seizures. 
Including these cases, there wTere 54 of the 93 postnatal cases in which 
the paralysis was ushered in by convulsions, but in no other of these was 
there any record of the child having had convulsions previously. Among 
these 54 cases many developed in association with various etiological 
factors; 14 with acute infectious diseases and 10 from a variety of causes, 
congenital syphilis, brain tumor, trauma, etc., and the primary cause of 
the cerebral lesion must have varied much-hemorrhage, thrombosis, 
1 Had I analyzed the surgical records, without doubt many more cases of infantile 
hemiplegia due to trauma would have been added. 416 
DISEASES OF THE NERVOUS SYSTEM' 
embolism, and inflammation. In the remaining 30 the cerebral involve- 
ment, as judged by the sudden onset of the convulsions in apparent health, 
seems to have been the primary lesion. In 14 of these there was either 
more or less prolonged fever, or definite evidence by protracted coma, etc., 
to show that the brain was seriously involved from the first. The dis- 
cussion as to the pathology of infantile hemiplegia has, in a great measure, 
centred around cases similar to these, i. e., those cases in which the 
paralysis developed acutely in apparently .healthy children, with more or 
less fever and evidence of involvement of the brain. The convulsions 
are either complete or, for the most part, unilateral, and one must assume 
a cause that acts more or less locally from the first, and it is obviously 
unsatisfactory to explain the paralysis as the result of the convulsions 
without giving an adequate cause for these, especially as there is reason 
to doubt the efficiency of convulsions in themselves to cause permanent 
gross lesions. Gowers assumes, as the most satisfactory explanation of 
these cases, " a sudden occlusion of a small surface vein by clot, with the 
consequent intense congestion and hemorrhagic softening of the region 
of the cortex." 
The many points of similarity between the onset of these cases of 
infantile cerebral hemiplegia and those of acute poliomyelitis led Strum- 
pell, in 1884, to assume as their anatomical basis a lesion in the brain 
analogous to that in the cord, which was believed to underlie infantile 
spinal paralysis, i. e., an inflammation confined to the gray matter of 
the motor cortex of the brain (polioencephalitis) corresponding to the 
inflammation limited to the ventral horns in the cord. This theory was 
advanced at a time when the anatomical data in regard to spinal infantile 
paralysis were very limited and when there were practically none concern- 
ing inflammation of the brain, so that in spite of its great attraction it did 
not receive general acceptation. Since then a great deal of work has 
been done on the inflammatory conditions of the central nervous system, 
and the views in regard to these conditions have been very much broad- 
ened. We now believe that infantile spinal paralysis is due to a specific 
organism which manifests itself for the most part by a diffuse inflammation 
about the vessels of the meninges and central nervous system, involving 
particularly, but by no means exclusively, the anterior gray matter of the 
spinal cord. 
Many cases of infantile hemiplegia, which develop acutely with fever, 
convulsions, etc., and which are presumably due to an encephalitis, 
are independent of any such diseases. It is an attractive and obvious 
thought that certain of these cases may be due to a specific organism, 
analogous to, if not identical with, that of poliomyelitis. That the brain 
is involved in many cases of poliomyelitis is manifest clinically, and in 
practically every fatal case some inflammation of the pia mater at the 
base of the brain can be demonstrated. 
However, an encephalitis of the degree that would entail a hemiplegia 
seems extremely uncommon, for very few such cases have been reported 
during the recent epidemic of poliomyelitis. At present then we should 
regard the organism of poliomyelitis as only one of the causes, and 
probably a rare one, of infantile hemiplegia. DISEASES OF THE CEREBRAL BLOODVESSELS 
417 
Among the 93 postnatal cases there were 26 instances in which the 
paralysis developed in association with one of the acute infectious diseases, 
6 in relation to whooping cough, 5 with typhoid fever, 4 with measles, 
4 with diphtheria, 3 with gastro-intestinal disturbances, and the other 4 
distributed among various conditions. The cerebral lesions in these 
cases are by no means always the same. Inflammatory processes must 
account for a number of them, but hemorrhage, thrombosis, and embolism 
have been found with varying frequency. 
Whooping cough is accountable for more intracranial hemorrhages than 
any of the other conditions. A great increase in venous pressure during 
a paroxysm of coughing is the usual explanation, but Neurath believes 
that changes in the vessel wall are more important than increased pressure. 
The hemorrhage is thought to be from a meningeal vein. Rhein, who 
analyzed the autopsy findings of 21 cases of pertussis, found the hemor- 
rhage when present most frequently in the substance of the brain. In his 
case the lesion was inflammatory, and Neurath refers to a number of such 
cases and believes that hemorrhage is less common than is supposed. 
Typhoid fever we believe to be accountable for 5 of our cases, although 
this seems a large number when we consider the relatively few cases of 
hemiplegia associated with this disease. Smithies was able to collect 
only 40 cases. The age of 32 of these cases is interesting; 9 were in the 
first decade, 8 in the second, 13 in the third, and only 2 beyond that time. 
When one considers how relatively infrequent typhoid fever is in the first 
decade, it is surely remarkable to find more than one-quarter of the cases 
of hemiplegia occurring in children under ten. Smithies was able to find 
the record of 5 autopsies, only one of which was in a case in a child, and 
the examination was made twelve years after the onset of the hemiplegia. 
Two of the other 4 cases occurred among our records, and have been 
reported by Osler. In all of the cases occlusion of an artery was found, 
so that from the evidence at hand we must conclude that cerebral throm- 
bosis is the cause of most cases of hemiplegia developing during typhoid 
fever. It is probably secondary to changes in the artery walls. 
Diphtheria is accountable for 4 of our cases, or, indeed, 5, if we include 
the case in which the history simply recorded a severe sore throat. If 
hemorrhage is the most common cause of the hemiplegias associated with 
pertussis, and thrombosis the most common cause in relation to typhoid 
fever, it seems that embolism plays this part in the hemiplegias developing 
during diphtheria. Thus, Rolleston,1 in his collection of 65 cases, found 
15 which were completed by autopsies. Among these, hemorrhage was 
found in 1, thrombosis in 2, embolism in 10, embolism and thrombosis in 
1, and atrophy of one hemisphere in 1 case. 
Measles is represented in this list by 4 cases, but there is not any case 
which occurred in association with scarlet fever. Concerning this latter 
disease, Rolleston2 speaks of the rarity of hemiplegia in relation to it, 
but he was able to find the records of 57 cases, 8 with autopsy. He 
concludes that it is usually due to cerebral embolism, but may follow 
1 Rev. Neurol, and Psychiat., 1905, p. 722. 
2 Ibid., 1908, p. 530. 418 
DISEASES OF THE NERVOUS SYSTEM 
thrombosis, hemorrhage, or acute encephalitis. Under this last heading 
he thinks uremic hemiplegia may probably be placed. 
Except in the instance of encephalitis, the involvement of the brain 
during the course of an acute infectious disease is secondary to involve- 
ment of the vascular system. The association between emboli and 
endocarditis is evident, and that the vessel walls may be involved in 
children during the course of acute infectious diseases has been shown 
by a number of authors, particularly by Wiesel,1 and that the occurrence 
of thrombosis is secondary to this involvement cannot be doubted. 
The tendency to rupture of an artery, as in whooping cough, is probably 
brought about by the weakening of the vessel wall through this process. 
This arterial disease appears in most instances to be entirely recovered 
from, but it does at times leave the artery wall permanently damaged, 
and so acute infectious disease during infancy may be the predisposing 
cause of later vascular accidents. 
Embolism is not uncommon in association with acute endocarditis 
occurring in the course of the acute infectious diseases (diphtheria, scarlet 
fever, etc.), but it is remarkable how rarely it occurs in chorea, a disease 
in which the heart is so generally affected. There is but one case in the 
series, although we have seen a large number of cases of chorea with 
involvement of the heart. The case was that of a little girl, aged ten 
years, who had had chorea a year and a half before, which was followed 
in the next year by rheumatic fever and endocarditis. She had been up 
about four weeks after this attack, when she fell from a bench, had 
repeated convulsions, and was paralyzed on the right side with loss of 
speech. The only other case in which it seems reasonably certain that 
the hemiplegia was due to an embolus was in a little child, aged one year, 
who had congenital abnormality of the heart. This organ was on the 
right side. The sounds at the apex were clear, but over the right second 
costal cartilage there was a grating systolic murmur. Four weeks before 
we saw the patient the mother discovered, on taking him up in the morn- 
ing, that his right arm and right leg were completely paralyzed, the 
attack having come on during sleep. 
The importance of hereditary syphilis in the causation of infantile 
hemiplegia has given rise to a great deal of discussion. Sachs believes 
that it has very little influence, but other authors give it prominence. 
In 4 cases of this series it appeared to be the most likely etiological factor 
and in 1 case there was hardly a doubt. This was in a child whose brother 
and sister had been treated for congenital syphilis, and who, himself, 
when twenty-one months old, had marked manifestations of the disease. 
When four years old he had an attack of right hemiplegia, with loss of 
speech, choked disk, and retinal hemorrhages. He was at this time 
passing through whooping cough, but this case is not included among 
those occurring in whooping cough. He improved under specific treat- 
ment, but the left optic nerve became atrophied. When five years old 
he had a second attack, this time on the left side, and speech was again 
affected. The paralysis improved, but there was a marked mental 
1 Ztschr. f. Heilk., 1906, xxvii, 262. DISEASES OF THE CEREBRAL BLOODVESSELS 
419 
defect. There was another case in which the history seems clear. The 
father had syphilis, and a specific eruption developed in the baby when 
it was two months old. The child was given antisyphilitic treatment. 
When four years old convulsions developed, followed by right-sided 
paralysis. When seven the child had a painful swelling of the anterior 
border of the tibia, which disappeared under specific treatment. The 
vascular lesion in these cases was presumed to be an occlusion, sec- 
ondary to syphilitic changes in the vessel walls. 
During the course of brain tumor hemiplegia is not uncommon, and three 
such cases occurred on the medical side; but in these the paralysis was of 
rather slow development, and so probably did not depend upon a hemor- 
rhage into the substance of the tumor. 
Hysteria may cause a hemiplegia at almost any age, and we have the 
record of one case, that of a little girl seen in her tenth year, suffering 
from her second attack. The first attack had been a year before. She 
had paralysis of the right arm and leg with typical sensory disturbances. 
The disability cleared up at once under suggestion and faradic stimulation. 
Cerebral Diplegia.-Closely allied to the hemiplegias which develop 
early in the first decade are the infantile cerebral diplegias. The same 
etiological factors, congenital abnormality of the brain, injury to the bead 
during birth, hereditary syphilis, inflammatory conditions, etc., apply 
to both. In the one case the lesion is unilateral, while in the other both 
sides of the brain are affected. The two sides of the brain may be 
unequally involved, and it is not infrequent to find one side of the body 
more affected than the other, and only portions of the hemisphere may 
be injured and corresponding symptoms result. Not infrequently the 
upper portions of both hemispheres are most involved, and the symptoms 
are largely confined to the legs (cerebral spastic paraplegia). The lesions 
occurring before or at the time of birth are more frequently bilateral, 
and diplegia is the common result of these processes. Later in the decade 
the reverse is true, and hemiplegia is then much more frequent. 
Among 100 cases of infantile cerebral palsies in the Neurological Depart- 
ment of the Johns Hopkins Dispensary, and in which the process was 
definitely congenital, 68 were diplegia and 32 hemiplegia. There are 
records of only 10 cases of diplegia which developed after birth. Five 
of these were in the first year, 3 in association with acute illnesses, measles, 
pneumonia, whooping cough, and the others with indefinite illnesses. 
Of those developing after the first year, 2 were with fever, 2 are said to 
have followed falls, and in 1 no cause could be given. Besides these 
78 cases, there were 4 others in which the early history was unknown, 
and in all the processes may have been congenital. 
The various symptoms of infantile spastic diplegia (Little's disease) 
are, for the most part, those of hemiplegia, which will be described in 
detail later, only in this case both sides of the body are involved. Certain 
symptoms are so pronounced that they require special mention at this 
time. These are particularly those depending upon the lack of develop- 
ment of the brain, for it is obvious that when the brain has been severely 
injured early in life it cannot develop normally, and so these children 
show every degree of idiocy. The degree of mental retardation is, in a 420 
DISEASES OF THE NERVOUS SYSTEM 
general way, proportionate to the motor defect, but not always so, and 
the intelligence of certain patients who have never been able to walk or 
even to sit up is surprising. It has been suggested that in such cases the 
lesion is not in the brain itself but in the upper part of the spinal cord. 
The disparity is more common in the other direction, and one often sees 
children who show only a slight degree of spastic paralysis, but whose 
mentality is very low. Indeed, every combination occurs. 
As in certain cases of congenital hemiplegia, the disability may not be 
noticed until it is time for the child to begin to walk, but usually there are 
symptoms which should attract attention. Directly after the difficult 
birth there is often trouble in establishing respiration, and not infrequently 
the child has muscular twitchings or even convulsions. The increased 
intracranial pressure may be shown by a tense non-pulsating fontanel 
and a general lack of animation, a higher grade of choked disk than usual, 
and death may occur rapidly. In less severe cases the baby seems to 
recover and to perform all of its functions normally. These, it must be 
remembered, are largely reflex, depending on the lower centres. Later, 
when the time arrives for the baby to show certain spontaneous activities, 
as holding up its head, sitting up, learning to walk, and to talk, the parents 
are distressed to see that these are not acquired. The spastic condition 
of the legs is often first noticed when the child is several months old by 
a resistance in the adductor muscles as the mother changes the child's 
napkins. When the child is held in the standing position this adductor 
spasm comes out more clearly and the legs are held in extension and firmly 
together. Indeed, at times the spasticity can be brought out only in 
this manner. In other cases it is so pronounced that it attracts attention 
very early (congenital spastic rigidity). Such babies become rigid upon 
the slightest cause, when handled or when startled by a noise. 
Even when the spasticity is not excessive these children learn to walk 
late; not usually before the third year, and often even much later. The 
walk is awkward and spastic, and frequently shows the typical crossed 
leg progression, each leg as it is advanced being thrown across the other 
by the strong contraction of the adductor muscles. The finer movements 
of the arms and hands are learned, if at all, with difficulty, and even the 
coarser movements are performed with marked incoordination. Such 
children may be entirely unable to learn to talk, and when they do, the 
articulation is apt to be very inexact, even when the mental development 
is of such a grade as to make speech possible. 
In infantile cerebral palsies, both of the hemiplegic and diplegic type, 
abnormal spontaneous movements are common; indeed, they are rarely 
seen in cases which develop later in life. The characteristics of these 
conditions, athetosis, postparalytic chorea, etc., will be discussed later. 
Athetosis in its classical form occurs in diplegias, and the condition 
described as double athetosis is confined to this state. Lewandowsky1 
insists that double athetosis is not simply the involvement of both sides 
of the body with typical athetosis, but that it is a different condition 
and depends upon excessive associated movements. Such patients may 
1 Deutsche Ztschr.f. Nervenh., 1905, xxix, 339. 421 
DISEASES OF THE CEREBRAL BLOODVESSELS 
be quite quiet when undisturbed, but when their attention is attracted, 
or when they endeavor to perform any voluntary act, the muscles of the 
face are thrown into the most startling grimaces, the arms are moved 
about in quick excessive choreiform movements, which suggest strongly 
those seen in Huntington's chorea. Double athetosis may occur in its 
most pronounced form when there is but little motor paralysis. 
Epilepsy not infrequently develops, although perhaps this is somewhat 
more common in the hemiplegic than in the diplegic type. The affected 
limbs may be retarded in their growth, and the whole physical develop- 
ment be stunted. This is not always so, and even in a severe case the 
body may reach its normal growth. 
Decade II.-Fewer cases of hemiplegia occur in the second decade 
than at any other time of life, except extreme old age, and even here the 
liability to these vascular accidents is much greater. The difference 
between the first and second decades depends largely upon the great 
number of cases in the first three years of life due to accidents at birth, 
and the liability of the infant's brain to inflammatory processes. The 
25 cases falling in this decade were divided as follows: 6 were due to 
trauma, and 6 occurred in the course of acute illness, diphtheria 2, men- 
ingitis 2, typhoid fever 1, and undetermined illness 1. One other case, 
that of athetosis, developed after a disease that was said to be meningitis. 
Two were in relation to brain tumors and 2 were hysterical. The remain- 
ing 8 cases are of particular interest, for they developed suddenly in 
apparent health, and presumably were due to primary vascular lesions. 
Similar cases occurred in the first decade, but their relative frequency is 
much greater in the second. This is what would be expected from the 
increasing number of individuals who must have damaged vascular 
systems from congenital weakness, acute illnesses, etc. As none of the 
cases was completed by autopsy, the pathological basis for the paralysis 
must be largely a matter of inference. 
Embolism.-Embolism seems almost surely to account for 3 of the 8 
cases. The first was in a girl, aged eleven years, who had frequent attacks 
of sore throat and arthritis. Her paralysis came on suddenly while she 
was at work in the kitchen; her left leg gave way so suddenly that she 
fell on the stove. She did not lose consciousness, and was able to keep 
about for a few hours, but the next morning she noticed that her left arm 
and leg were weak. There was a typical left-sided hemiplegia, and the 
examination of the heart revealed stenosis and insufficiency of the mitral 
valve. The second case was quite similar. The third case was that of a 
colored boy, aged thirteen years, who was awakened early one morning 
be intense pain in the stomach, vomited, had to be assisted back to bed 
on account of staggering, went to sleep, but was awakened three hours 
later by another attack of vomiting, after which he became unconscious 
and was brought to the hospital the same day. He was comatose, the 
right arm and leg were motionless, and the left arm and leg were in almost 
constant, restless movement. The head w'as turned to the right, and the 
right leg was stiff. There was mitral insufficiency. The ophthalmo- 
scopic examination showed evidences of increased intracranial pressure, 
and a decompression was done. Dr. Cushing found the left side of the 422 
DISEASES OF THE NERVOUS SYSTEM 
brain red and dry, resembling a hemorrhagic infarct. The boy recovered 
with right hemiplegia and aphasia.1 
Hemorrhage.-This appears to account for at least one of the 5 remain- 
ing cases, which was in a girl, aged sixteen years, who, since she was nine, 
had been seen a number of times suffering from attacks of Henoch's 
purpura. While on her way home from a friend's house she had difficulty 
in speaking, and that evening found it hard to eat. There was, however, 
no weakness of the limbs until the next morning, when the right arm was 
affected. On the second day she had convulsions, and was admitted to 
the hospital aphasic and weak on the right side. She had recurrent 
convulsions, beginning in the right hand; the head and eyes turned to 
the right, the right leg was next involved, and the convulsions then became 
general. After each convulsion she was more comatose, and new purpuric 
areas appeared on various parts of the body. Dr. Cushing operated and 
found the dura tense and the brain and subpial space of a uniform cherry- 
red color. Nothing but decompression was attempted. The patient 
died on the day after the operation, and four days after the onset of the 
cerebral symptoms. No autopsy was permitted. 
Hemorrhage may have accounted for the other 4 cases, although 
thrombosis cannot be excluded. In none of them could a source for an 
embolus be found. They occurred suddenly, in 2 instances the paralysis 
being discovered in the morning, after an undisturbed sleep, and one 
case, a girl, aged fourteen years, while working in a very hot schoolroom, 
felt nervous and fell unconscious on her desk. This was followed by 
paralysis on her right side with speech disturbance. The examination 
nine months afterward showed the signs of a typical organic hemi- 
plegia. In this case the heart was normal and no arteriosclerosis was 
discovered. 
A most interesting specimen was brought to the Pathological Depart- 
ment. This was the brain of a school boy, aged fifteen years, who seemed 
perfectly well upon going to bed. At 1 a.m. he aroused his room-mate 
by loud screams, and complained of intense pain in the head. He quickly 
became unconscious, and died at 8 a.m. The autopsy showed an enlarged 
thymus, lymphatic hyperplasia of the intestine, lymph glands, and spleen, 
with oedema and congestion of the lungs. The brain showed a large 
hemorrhage in the right temporal lobe. A careful microscopic examina- 
tion failed to reveal any disease of the cerebral vessels. 
The cases which were associated with acute illnesses differ only from 
those occurring in the first decade in the age of onset, except the two 
cases associated with meningitis. In one of these the type of meningitis 
could not be determined, but the other case was proved by lumbar punct- 
ure to be acute cerebrospinal meningitis. Hemiplegia is a rare complica- 
tion of epidemic cerebrospinal meningitis. Castaigne and Rivet2 report 
a case in a boy, aged seventeen years, and could find the record of but one 
other case. They ascribe the complication to encephalitis. 
1 In connection with these three cases 1 may refer to a case already reported in 
The Johns Hopkins Hosp. Bull., 1901, vol. xii, of embolism of the central artery of 
the retina in a girl, aged sixteen years, who had chorea and mitral insufficiency. 
2 Bull, et mem. Soc. med. d. hdp. de Paris, 1909, xxvii, 900. DISEASES OF THE CEREBRAL BLOODVESSELS 
423 
Decade III.-The liability to apoplexy in the third decade is about 
three times as great as in the second. This is due to the influence of 
syphilis and to the increased number of cases depending upon cardio- 
vascular changes. Pregnancy and the puerperal state account for a 
certain number of cases, and a few are in association with various patho- 
logical conditions (pernicious anemia, hemophilia, etc.). 
Syphilis was recorded as having occurred in 20 of 62 cases of this 
series, and there can be no doubt that to this number others should be 
added. Of 12 doubtful cases, 6 were in negroes-5 men and 1 woman- 
in whom syphilis is extremely prevalent, but in whom it is always difficult 
to get a history of the infection, and there were 2 white women, both of 
whom were probably syphilitic, for one had many miscarriages and the 
other was a prostitute. The cerebral complication may follow the 
primary lesion quickly, indeed within the first year, or it may be delayed 
for a long time, ten or more years. One case occurred at five and one at 
seven months, and 4 others at about one year after the infection. The 
average time was approximately four and a half years. 
Thrombosis.-Thrombosis with consequent softening of the nervous 
tissue, is the lesion upon which practically all the cases of apoplexy due 
to syphilis depend. This depends upon a specific change in the artery 
walls, the so-called syphilitic arteritis. Under thrombosis should prob- 
ably be grouped most of the cases occurring with acute infectious diseases, 
and the cases occurring shortly after childbirth. The hospital list contains 
5 of the former and 3 of the latter in this decade. Of the 5 cases occurring 
during the course of an acute infection, 4 were with typhoid fever, and 
one with pneumonia. Two of the typhoid fever cases occurred in the 
hospital, and both died. The autopsy on one revealed thrombosis of 
branches of the middle cerebral artery. The case which developed 
during the second week of a severe double pneumonia was possibly 
due to an embolus, for when the patient was examined six months later 
his heart showed evidences of serious organic disease. Hemiplegia is an 
extremely rare complication in pneumonia. 
Hemiplegia and other focal manifestations of cerebral lesions occur in 
association with pregnancy and childbirth. These cases naturally fall 
in the third and fourth decades, and in this series 3 of the hemiplegias of 
the third decade were of this character. They developed after childbirth, 
one on the fourth day, one on the ninth, and one a month afterward. 
The lesions in such cases vary a great deal and hemorrhage, thrombosis, 
and embolism have all been found, von Hosslin1 has reviewed this 
subject. Hemorrhage is the most common lesion, particularly in those 
cases associated with marked albuminuria or symptoms of eclampsia. 
In this association, a number of cases have been reported in which the 
brain showed only oedema. 
Women with diseased arteries from any cause run special risks during 
pregnancy and confinement. An illustration of this was a woman who 
died in the obstetrical ward. She was thirty-five years of age, and 
when thirty-two had a left-sided hemiplegia, which had no association 
1 Arch. f. Psychiat., 1904, xxxviii, 730, and 1905, xl, 445. 424 
DISEASES OF THE NERVOUS SYSTEM 
with pregnancy. When thirty-five she became pregnant, and at the 
eighth month she fell unconscious, apparently in uremia. She was 
brought to the hospital, delivered immediately, but died soon after. 
Autopsy showed a softened area due to the old lesion, and a large hemor- 
rhage into the pons Varolii. The arteries at the base showed marked 
sclerosis. The liver did not show the changes characteristic of eclampsia. 
Hemorrhage does occur during parturition in women whose arteries show 
no general disease. 
Embolism.-von Hosslin found embolism the least common cause 
of apoplexy during pregnancy, although Meyer-Huegg believes that it 
is the most common-. The source of the emboli is endocarditis. This 
may be acute, developing spontaneously, or as an exacerbation of a 
chronic process; or a chronic endocarditis may be the source of an 
embolus. Embolic occlusion of a cerebral vessel is the reasonable explana- 
tion of the 12 cases of hemiplegia which occurred between the twentieth 
and the thirtieth year in individuals suffering from organic heart disease. 
If syphilis be excluded this is by far the most common cause of the 
hemiplegias in the third and fourth decades. Three of these 12 cases 
developed while in the hospital; 2 in association with ulcerative endo- 
carditis, and the third in a case of rheumatic fever with cardiac involve- 
ment. The other 9 cases occurred while the patients were about their 
usual vocations, and when the cardiac condition had become chronic. 
Hemorrhage.-This is uncommon before the fifth decade, but even 
though syphilis may account for the majority of the 12 cases in the third 
decade, in which no cause could be determined, still some of them are 
hard to explain in this manner. For instance, a man, aged twenty-one 
years, who admitted only one exposure to venereal infection and who 
denied all knowledge of any such accident, fell while working and was 
paralyzed on his right side. A physical examination revealed no lesion 
of the heart, and in such cases, without an autopsy, it is impossible 
to exclude cerebral hemorrhage. However, in 3 of the 62 cases occurring 
in this decade, there can be little doubt that cerebral hemorrhage did 
occur, and in one this was proved by autopsy. The patient was a 
woman, aged twenty-two years, who had nephritis, and who died eleven 
days after an operation for decompression. At autopsy a large capsular 
hemorrhage was found. The other two cases were associated with condi- 
tions which make hemorrhage almost certain. In one of these, a white 
man, aged twenty-eight years, with pernicious anemia, a right hemiplegia 
apppeared nineteen days before death. The autopsy revealed hemor- 
rhages in the various organs of the body, but unfortunately the brain 
was not examined. The other case was in a hospital orderly who came 
from a family of bleeders, and who was himself a hemophiliac. 
Trauma.-Trauma accounts for 2 of our 62 cases. One of these is of 
interest, as the head was not directly affected. The patient, a brakeman, 
aged twenty-one years, was injured while coupling cars; he was crushed 
about the chest and right hemiplegia developed, which may have been 
due to a hemorrhage secondary to great venous congestion. 
Hysteria.-This accounted for 5 of hemiplegias in the third decade. 
Four of these were in women and one was in a man. DISEASES OF THE CEREBRAL BLOODVESSELS 
425 
Decade IV.-In this decade the liability to vascular lesions is again 
greatly increased, being about three times as great as that in the third 
decade. The influence of syphilis is still marked in the fourth. Of the 
102 cases, 32 gave a definite history of syphilis, which almost equals that 
in the third decade, being 31.3 per cent, in the former and 32.2 per cent, 
in the latter. But the group of cases in -which no definite cause could 
be determined is much larger, making 44.1 per cent. In this group many 
cases must be included which are due to syphilis, although this has not 
been recognized or not recorded. As other conditions which cause 
arterial degeneration are constantly increasing with advancing age, the 
proportion due to syphilis naturally becomes smaller as the age becomes 
greater, and, therefore, in this decade, this group must contain a rela- 
tively smaller number of unrecognized syphilitic cases. 
The time .between the syphilitic infection and the occurrence of the 
apoplexy in the cases which fall in the fourth decade is, as would be ex- 
pected, somewhat longer than that which was found for the cases of the 
third decade. In this series, as accurately as could be determined, it 
was a little more than eight years. Of the 32 cases, all but 1 occurred in 
men. This, of course, does not mean that the condition does not occur 
in women, but only illustrates how difficult it is to obtain a syphilitic 
history in women. 
Acute infectious disease accounts for only 2 cases among the 102 in this 
decade. In these the hemiplegia developed during typhoid fever, and was 
presumably due to thrombosis of a cerebral artery. In this connection, 
a private case, not included in this analysis, seen in consultation with 
Dr. Chrystie, of Bryn Mawr, Pa., may be noted. It occurred in a woman, 
aged forty-two years, during the course of a protracted fever, the nature 
of which was never definitely determined, the diagnosis resting between 
a mild attack of typhoid fever and an unusual influenzal attack. On 
the fourteenth day the patient had a convulsion affecting the right side, 
which lasted about eight minutes, and which left her paralyzed on the 
right side and aphasic. The left eye was blind, due to obliteration of the 
central retinal artery. In this case the lesion was almost surely a throm- 
bosis of the internal carotid, implicating its ophthalmic branch as well 
as the middle cerebral artery. The hemiplegic attack had practically 
no influence on the temperature, which continued to vary between 
98.5° and 101° until the twenty-second day of the disease, after which 
it remained practically normal. 
There is one case in the fourth decade which occurred twelve days 
after the birth of the eighth child in a woman, aged thirty-nine years; it 
was associated with a normal puerperium. Two other interesting cases 
were seen in private practice.1 These were associated with evidences of 
toxemia, and both had hemianopia. In one there was a transient left 
hemiplegia with permanent sensory disturbances. Cerebral thrombosis 
was the most probable lesion in these cases. 
Bilateral capsular softening, due to thrombosis, was found at autopsy 
1 Both of these were seen with Dr. Hiram Woods, and have been reported by him, 
Jour. Am. Med. Assn., 1908, li, 204. 426 
DISEASES OF THE NERVOUS SYSTEM 
in a colored woman, aged thirty-nine years, who presented a typical 
picture of pseudobulbar paralysis. 
Embolism, associated with heart disease, was the probable lesion in 
11 cases-6 men and 5 women. This was demonstrated by autopsies 
in 3 cases. 
Hemorrhage within the cranium was found in 4 cases at autopsy. Two 
were in negro men who had had syphilis nine or ten years before death, 
and are included among the syphilitic cases, although this factor was 
probably but one of the causes of the vascular disease. They were both 
markedly alcoholic. In one the intracranial hemorrhage was associated 
with pachymeningitis and arteriosclerosis, and there was evidence of old 
cortical hemorrhages. In the other cases there were marked cardio- 
vascular changes and a large clot in the occipital lobe, which had broken 
through into the subdural space. The other 2 patients were brought to 
the hospital unconscious, and very little history could be obtained. In 
one the hemorrhage had taken place in a glioma, which apparently 
had given no symptoms. The other was in a man, aged thirty-nine years, 
who had an enormous hemorrhage and general arteriosclerosis. 
Spontaneous cerebral hemorrhage is, in fact, generally recognized as 
being rare before the fifth decade. Von Monakow says that before the 
fortieth year it is extraordinarily uncommon. However, this lesion 
was probably present in some of the 45 cases in which the history gave 
no very definite indication as to the cause of the cerebral lesion. In one 
case of hemiplegia, which had developed rather gradually, a brain tumor 
was found at autopsy, and in 2 cases the paralysis was definitely hysterical. 
Both of these occurred in men. 
Decade V.-Apoplexy becomes much more common in the fifth decade, 
the liability to it being three times as great as that in the preceding ten 
years, and more than twice as many cases occur. In the present analysis 
there are 137 cases within this period. By the time an individual has 
reached the age of forty many things may have occurred in his life that 
make for vascular accidents, and it is often difficult or impossible to 
determine which has been to blame in a particular case. 
Syphilis, such an important factor in the third and fourth decades, plays 
a much less outspoken part in the fifth. There are, indeed, 27 cases in 
which there is a definite history of a previous infection, but this, in most 
instances, took place many years before the apoplectic attack, the average 
time being between sixteen and seventeen years. In many of these 27 
cases other conditions were present which may have been effective, and 
may or may not have been related to the specific infection. This is 
illustrated by the only case which came to autopsy. The patient, aged 
forty-seven years, was infected fifteen years previously and had been in 
the wards on two occasions suffering from symptoms of arteriosclerosis 
and chronic nephritis. After staying in the hospital three months he 
became unconscious and a left hemiplegia appeared. The autopsy 
revealed chronic nephritis, hypertrophy of the heart, acute pericarditis, 
and thrombosis of the right internal carotid artery. 
Cerebral softening was found at autopsv in 7 cases dying between forty 
and forty-nine. It was not always determined whether the occlusion of 427 
DISEASES OF THE CEREBRAL BLOODVESSELS 
the bloodvessels was due to an embolus or a thrombus, but in one case, 
that of a man, aged forty-six years, who died during typhoid fever with 
evidence of left-sided paralysis, there seems to be no doubt that throm- 
bosis of the middle cerebral artery was the cause of the necrotic area 
in the right internal capsule. This is the oldest case of hemiplegia 
associated with acute infectious disease of which we have record, and is 
the only one in the fifth decade. In 2 other cases which were associated 
with chronic nephritis and arteriosclerosis, the multiple areas of cerebral 
softening were believed to be thrombotic, and there were numerous cases 
in which the most probable clinical diagnosis was thrombosis. 
In a case of diabetes mellitus a left hemiplegia developed two days 
before death. At autopsy pulmonary tuberculosis, chronic nephritis, 
acute endocarditis of the aortic and mitral valves, and infarcts in the 
spleen and kidney were found. There was slight sclerosis of the vessels 
at the base of the brain, but no macroscopic lesion was found other than 
a hyperemic condition of the pia. The probable explanation of the 
cerebral symptoms was an undetected embolus, which for some reason, 
an unusual freedom of collateral circulation for instance, had not pro- 
duced a definite softening. We must also think of the possibility of the 
case being one of toxic hemiplegia in diabetes mellitus. 
Embolism.-The heart was noted as definitely affected in 15 cases, and 
was probably involved much oftener than this. That the hemiplegia was 
in all these cases due to embolism is, of course, not a just inference, as 
there were often associated conditions that might have led to thrombosis 
or hemorrhage. Cerebral hemorrhage was found at autopsy in 4 cases 
dying in this decade. Two cases of hemiplegia, aged forty and forty-two 
years, occurred after childbirth. 
In 80 cases the hemiplegia developed without determined cause, 
although in some of them arteriosclerosis was noticed, and evidences of 
cardiac and renal involvement. 
Decade VI.-In this period the liability to apoplexy and also the fre- 
quency of its occurrence again increases over the fifth decade. It is 
interesting that the increase in both these relations is almost exactly 
the same as that of the fifth over the fourth decade, being three times 
for the first and twice for the second. In the present series, however, 
there are only 143 cases as against 137 in the preceding ten years. There 
were 30 deaths against 19 in the fifth decade. In this decade it is more 
difficult to determine the etiological factors, and 94 cases are included 
in the group of undetermined etiology. Most of these showed definite 
evidences of arteriosclerosis, which may have followed a variety of causes. 
No apology seems necessary for arteriosclerosis at this time of life. 
There were 11 cases in which it was definitely stated that the patient 
had suffered from syphilis. This, of course, is an evident understatement 
of the fact. Men between fifty and sixty have forgotten that they were 
infected in their early youth, or, believing that such an infection could 
have no possible bearing on their condition, conceal it; and, on the 
other hand, the examining physicians, knowing that an early specific 
infection is apt to be only of slight importance, have not inquired into 
this feature of the cases as diligently as they should. In these 11 cases 428 
DISEASES OF THE NERVOUS SYSTEM 
the average time of infection preceded the apoplexy by 21.7 years, and 
it is probable that in most of them syphilis acted only as one of the causes 
in producing arteriosclerosis. That syphilis may play its accustomed 
part at any period of life is shown in 2 of the 11 cases; in one, a man, 
aged fifty years, a slight hemiplegia appeared while the patient was 
being treated for tertiary syphilis, he having been infected two and a half 
years previously. In the other the hemiplegia developed at fifty-five, 
a year after an undoubted syphilitic infection. 
In the only case which came to autopsy there was a history of syphilis 
twenty years previously. He had marked arteriosclerosis, chronic 
nephritis, and dilated heart, for which he had been treated in the hospital 
two months before. He was admitted in a comatose condition, and died 
in twenty-four hours. An extensive hemorrhage was found in the left 
cerebral hemisphere, the vessels in the brain being markedly sclerotic. 
There was no evidence of syphilis at the autopsy. 
There were 17 cases in which the heart was noted as being evidently 
diseased, and in which presumably embolism accounted for the apoplexy. 
In 4 other cases among 7, in which intracranial softening was found at 
autopsy, there was a condition of the heart which could have been the 
source of an embolus. That it is not safe to assume that an apoplexy 
in an individual who presents the evidence of a cardiac involvement is 
always due to embolism, is shown by 3 cases dying of intracerebral 
hemorrhage, in all of which the autopsy showed valvular endocarditis. 
Cerebral softening was found in 9 autopsies on patients dying in this 
decade. Two of these, however, were in cases which had developed in the 
fifth decade, one due to embolus and one to a thrombus. 
Hemorrhage was found in 12 cases. Three of these have been referred 
to as being associated with endocarditis, and one occurred in a syphilitic 
patient, although there was nothing found at autopsy to suggest this 
infection. In one other case, in which the history gave no indication of 
any infection, the autopsy showed what was believed to be a definite 
syphilitic lesion of the abdominal viscera. In the other cases there was 
the ordinary vascular degeneration. 
Decade VII.-In the seventh decade we have 105 cases for analysis, 
a considerable falling off from the preceding period. The liability, judged 
from the mortality statistics, is greater, being increased more than two 
and a half times, and the actual number of registered deaths in Baltimore 
was 95 for the seventh decade and 69 for the sixth. 
At this period it is impossible to make any satisfactory division of the 
causes which led up to the vascular accident. Fourteen cases did, indeed, 
give a history of syphilis, but the average time of infection was about 
thirty years previous, and its influence could have been only problematical. 
In 2 cases syphilis did seem to have been the predisposing cause. In 
these, aged sixty and sixtv-five years, there was an undoubted infection 
about three years before the onset of the hemiplegia. In neither case 
was there any other probable cause determined. 
In the great majority of the other cases, evidences of general arterio- 
sclerosis were found, with associated cardiac and renal conditions, states 
in which thrombosis and hemorrhage are common. In 6 cases the heart 429 
DISEASES OF THE CEREBRAL BLOODVESSELS 
gave such evidences of severe valvular involvement that emboli might 
have been expected; but in the onlv case which camp to autopsy marked 
endocarditis, general arteriosclerosis, and a hemorrhage into the left 
internal capsule were found. Hemorrhage w'as found at autopsy in 4 
cases. In the 2 cases in which cerebral softening was found it was believed 
to be due to embolism, once from an aneurism of the aorta and once from 
some unknown source, the embolism being demonstrated on section. 
Decades VIII and IX.-In the eighth and ninth decades, although 
the liability to apoplexy continues to increase, the frequency of its occur- 
rence decreases markedly in the last part of this period, owing to the 
relatively small number of individuals alive between eighty and ninety. 
In the present analysis there are 25 cases in the eighth decade and 6 in 
the ninth. There are 3 autopsies recorded in the eighth and 2 in the 
ninth. In all 3 of the eighth decade and one of the ninth, cerebral soften- 
ing was found. One was due to thrombosis of the right internal carotid, 
secondary to embolism of the brachial artery. The other 3 appear to 
have been primarily thrombotic. The one case of cerebral hemorrhage 
occurred in a colored man, aged eighty-one, and was associated with 
intense degeneration of the whole vascular system. 
Symptoms.-Preceding.-Slight cerebral symptoms may occur many 
months before apoplexy. These depend upon the vascular changes which 
lead up to the rupture or the occlusion of a vessel, and are important as 
indicating that such changes have taken place. It is obvious that an 
embolus whose origin is from some distant part of the circulatory system, 
can give rise to no cerebral manifestations until it lodges in an artery 
of the brain. But if the occluded artery be small, or one in which collateral 
circulation can be established, the symptoms may be slight or transient 
and premonitory of those following a subsequent larger embolus. 
The liability of an artery to rupture depends upon some weakness of the 
vessel wall, usually upon the presence of small aneurisms. These dilata- 
tions on the smaller vessels themselves give rise to no symptoms that 
can be distinguished, and so in many, probably most cases of cerebral 
hemorrhage there are no premonitory symptoms. The associated cerebral 
arteriosclerosis may, however, cause symptoms for a long time before 
the occurrence of apoplexy, due to rupture or thrombosis. 
The rupture of a larger aneurism of one of the arteries at the base of the 
brain is a rare cause of cerebral apoplexv. These dilatations usually give 
no symptoms until their rupture, but at times they cause the manifesta- 
tions of cerebral tumors. When such symptoms are present, associated 
with subjective noises in the head, and the presence of a vascular murmur 
heard through the skull, the diagnosis of an aneurism is justified. This 
association appears to be so rare as to have very little practical impor- 
tance. Much more frequently the aneurismal dilatations are associated 
with widespread arterial changes and we are apt to have the symptoms 
of cerebral arteriosclerosis. Hemorrhage may occur into the substance 
of a brain tumor, and so the symptoms of such a growth may at times be 
premonitory of cerebral hemorrhage. 
The great majority of cases of cerebral thrombosis occur in the last 
half of life, and are due to more or less chronic changes of the cerebral 430 
DISEASES OF THE NERVOUS SYSTEM 
vessels-cerebral arteriosclerosis. The most significant symptoms due 
to this condition are a lowering of the mental strength, giddiness, slight 
cerebral attacks, convulsive seizures, transient aphasias, slight weakness 
of the various limbs, paresthesia and pain, etc., when associated with 
evidence of sclerosis in the peripheral arteries, especially in those of the 
retina, and the signs of general arteriosclerosis. 
Thrombosis of a cerebral vessel, in the first half of life is most commonly 
due to the effects of syphilis, or is in association with acute infectious dis- 
eases. That due to syphilis may be associated with the symptoms which 
are recognized as indicating specific involvement of the brain, intense 
periodical headaches, paralysis of various cranial nerves, etc., and these 
we may in the present relation regard as premonitory. There are usually 
no premonitory symptoms of thrombosis of a cerebral vessel occurring 
in the course of acute infectious diseases. 
Tho nervous symptoms which precede an apoplexy, if cerebral syphilis 
be - excluded, are practically all due to arteriosclerosis. They more 
commonlv precede thrombosis than hemorrhage, but are not exceptional 
even with the latter. 
Exciting Causes.-An apoplectic attack may occur under all conditions 
of life-during sleep, while awake in bed, upon getting up, at table, at 
work, at play, or while occupied in any other manner. Overexertion does 
not often play its assigned important role in exciting the attack, and, 
indeed, it is rarely possible to discover from the history any evident 
relation of the occupation to the stroke. 
In the analysis of the cases in which the onset was after the first decade, 
and excluding the traumatic cases, there were 379 in which the circum- 
stances of the attack were definitely noted. Among these 125 occurred 
while the patients were in bed and in most instances asleep. Fifty-four 
of these occurred during the course of some other illness, and 37 while 
the patients were in the hospital. In 36 instances the attack was noticed 
when the patient got out of bed, and some of these may have occurred 
during sleep. In contrast to this it is interesting to note that but 4 cases 
occurred while patients were preparing for bed. Fifty-nine attacks 
developed while the patients were sitting. A number of these were while 
the patients were eating a meal, but in most instances they were quietly 
reading, sewing, etc. In 3 instances they were playing cards, and it is 
possible that emotional effects may have been operative here and in 
some other cases. This seemed certainly so in one instance, that of a 
colored midwife, who became very angry on account of the refusal of 
the authorities to admit her patient to the hospital. She fell unconscious, 
and was herself taken into the ward, and died within twenty-four hours. 
This was the only case in which anger seems to have played a part. Ten 
attacks occurred while the patients were on more or less protracted 
sprees, and 54 while the patients were simply walking about. 
This analysis includes apoplexies due to all causes, and it might well 
be that those cases which took place during rest, included, for the most 
part, those due to thrombosis, and that the cases of hemorrhage were 
among those occurring with more or less bodily activity. Theoretically, 
anything that increases the blood-pressure within weakened vessel DISEASES OF THE CEREBRAL BLOODVESSELS 
431 
walls would tend to cause their rupture, and we should expect to 
find many cases of apoplexy due to hemorrhage occurring during 
muscular exertion or those emotional states associated with increased 
action of the heart. Increased cardiac action might also be assumed 
to favor the detachment of an embolus, and so similar causes might be 
expected to be active in this class. Thrombosis, on the other hand, is 
favored theoretically by quite opposite circulatory conditions, and the 
predisposing causes, changes in the vessel walls or in the blood, being 
present, slowing of the blood current should act as an accessory factor, 
and might determine the time of the occlusion. 
Clinically the state of affairs is not so simple, and is modified by 
many circumstances which cannot be determined, and it is very unsafe 
to assume the character of the lesion from the state of activity of the 
patient at the time of the stroke. The study of a large number of cases 
in which the lesions were determined after death would show the real 
relation of these factors. Unfortunately, the figures available from this 
series are but few, but they may help to swell the total from other sources. 
There were 25 cases of cerebral hemorrhage in which the onset was given. 
Twelve of the attacks took place while the patients were at rest, 10 while 
in bed, and 2 while sitting quietly; 6 of the 12 attacks occurred in the 
hospital wards, as the terminal event of various conditions; 3 other 
apoplexies terminated alcoholic debauches; 1 of these patients was sitting 
at a table, and the others were probably not very active, although as to 
this we have no definite data; 4 patients were struck down while walking 
about, and 5 while at their every-day work. In only 1 case did there 
seem any great amount of physical exertion, and that was in a man, 
aged thirty-nine years, who, after a long bicycle ride, was found at 3 
a.m. on the steps of a house, paralyzed and aphasic. The onset was 
noted in 23 cases of cerebral softening, and in 16 of these the patients 
were in bed, all but one being confined by some severe illness; 14 of them 
were in the hospital at the time of the onset. Of the other 7 attacks, 
4 took place while the patients were at work, and one while occupied 
in each of the following ways-sitting quietly, walking about, and during 
an alcoholic spree. 
It will be seen that of the 26 cases in which cerebral lesions developed 
while the patients were in bed, only 10 proved at autopsy to fie hemor- 
rhage, but that of the 19 apoplexies developing during the more or less 
bodily activity (including the 4 cases which occurred during alcoholic 
debauches), 13 proved to be due to cerebral hemorrhage. 
A number of authors have interested themselves as to the importance 
of bodily activity in the production of the various cerebral vascular 
lesions. Ernest Jones1 analyzed the records at University College 
Hospital, London, in relation, among other things, to the time of onset of 
the various vascular lesions. These records covered sixty-five years, and 
the available material consisted of 160 lesions, 123 cases of hemorrhage, 
24 of thrombosis, and 13 of embolism. None of his cases occurred during 
excessive bodily activity. He compared his results with those of various 
1 Brain, 1905, xxviii, 527. 432 
DISEASES OF THE NERVOUS SYSTEM 
other authors, and concluded: "Rest in bed, and especially sleep, protect 
to some extent against cerebral hemorrhage. This would seem a more 
accurate statement than that rest and sleep predispose to thrombosis, 
as I can find no evidence in favor of this, although in actual practice the 
two statements have a similar bearing on diagnosis. Severe exertion and 
time of day appear to have had too much stress laid on them in the past. 
A miliary aneurism that is going to rupture rarely needs the aid of severe 
exertion for this consummation. Time of day is only of interest when the 
habits of the patient's blood-pressure at different hours are known." 
The Apoplectic Attack.-Throughout the present article the word 
apoplexy has been used more in its original sense as meaning an acute 
cerebral attack, but it has been stretched to include not only those seizures 
of sudden loss of consciousness with paralysis of sense and motion, but 
also those in which the consciousness is affected later, or, indeed, not at 
all. Trousseau used the word strictly as signifying an absolutely sudden 
loss of consciousness, and so pointed out that "apoplexy" was rarely, 
if ever, the initial symptom in cerebral hemorrhage, and that if the 
patients wrere under observation at the time of the onset, symptoms due 
to cerebral lesions could always be noted before consciousness was lost. 
Indeed, he states that he had never seen a man struck down as by a blow 
and dropping instantly in a state of unconsciousness. 
Loss of consciousness as the initial symptom is indeed rare, and in the 
vast majority of cases patients usually complain of headache, vertigo, 
tingling or weakness in some part of the body, as the indications of the 
attack. The loss of consciousness, if it occurs, may be delayed only a 
few minutes, or for several hours, or, indeed, days. The manner of 
occurrence of the different lesions-thrombosis, hemorrhage, and embol- 
ism-differs, and one would expect the development of the symptoms 
to be somewhat characteristic; thus, the closure of an artery by a throm- 
bus is a more or less gradual process, and so one would expect the symp- 
toms to develop more or less gradually. The rupture of a bloodvessel 
is a sudden occurrence, and the resulting symptoms should make their 
appearance quickly. The occlusion of a bloodvessel by an embolus may 
be absolutely sudden, and in connection with it we might expect apoplexy 
to develop in its classical manner. 
These.considerations are, in general, true, but are often so modified 
that conclusions based upon them cannot be accepted absolutely. Al- 
though it is true that thrombosis is not in its entirety a sudden process, 
still the final closure of a vessel may occur suddenly, and it is not at all 
uncommon to have the symptoms develop with great rapidity. Jones, 
in this connection, says, " There is a large group of thrombosis cases, the 
onset of which is apoplectiform." The percentage has been put as high 
as 58 by some authorities. There are a number of such cases in this 
series. For instance, a woman, aged fifty-two years, who had one previous 
attack from which she recovered, while in apparent health and trans- 
acting business with a lawyer, fell unconscious. After her death, three 
weeks later, cerebral thrombosis was found. 
Although the rupture of a vessel is an instantaneous process, the tear 
at first may be very slight, and only a small quantity of blood may escape. DISEASES OF THE CEREBRAL BLOODVESSELS 
433 
The focus may increase in size more or less gradually, destroying more and 
more of the nervous tissue, and many cases of cerebral hemorrhage have 
the onset typical of thrombosis. A man, aged fifty-nine years, while 
sitting with his family after supper, complained of intense headache. 
Ten minutes later it was noticed that his face jerked, and that when 
spoken to he did not answer questions. In half an hour he got up, closed 
the house, went up stairs, and moved about his room. He gradually 
became unconscious during the night, and was completely paralyzed 
on the right side. After his death, one week later, a large hemorrhagic 
clot was found in the left cerebral hemisphere. Another man, aged 
sixty-five years, with definite arteriosclerosis, gradually lost power on 
the left side in the course of twenty-four hours, with increasing dulness 
but no actual coma. He improved somewhat, but died in eleven days 
from bronchopneumonia. At autopsy the brain showed marked arterio- 
sclerosis, multiple areas of softening, and, in the right hemisphere, a 
large clot, 7 cm. in diameter. 
The stoppage of an embolus is always sudden, but if an embolus lodges 
at the bifurcation of a vessel, the blood current is not completely inter- 
rupted until the formation of the thrombus around the embolus occludes 
one or the other of the arterial branches. This secondary thrombus 
formation may extend and cut off branches that had previously received 
blood by collateral circulation. An embolus may be caught in an artery 
that has free collateral circulation, or there may be more than one 
embolus, the first plugging a relatively small vessel and a subsequent 
one a more important artery. It is obvious that if any of these circum- 
stances occur the symptoms following an embolus of the cerebral vessels 
may develop more or less gradually. 
Consciousness.-Even though it is true that an absolutely sudden loss 
of consciousness is uncommon in association with any of the cerebral 
vascular lesions, still consciousness is often disturbed with all of them. 
In the records of 401 apoplectic attacks in which the state of consciousness 
was noted, there was no loss of consciousness in 202 cases, but in 199 it 
was either lost or markedly disturbed. In 107 of these it was reasonably 
certain that the disturbance of consciousness occurred early in the 
attack. As but few came to autopsy they are of no value in determining 
the relative frequency of loss of consciousness in relation to the different 
lesions. Jones analyzed his own cases, together with those which he had 
been able to collect from the literature, and found that among 201 cases 
of embolus, consciousness was lost in 47.7 per cent., and was affected 
in nearly 60 per cent., while in 235 cases of cerebral hemorrhage conscious- 
ness was lost in nearly 75 per cent. He gave no collected figures for 
thrombosis in this relation, but in his own 22 cases there was loss of 
consciousness in 45.5 per cent. 
The mechanism of the loss of consciousness is not thoroughly under- 
stood, but since consciousness depends upon an adequate supply of blood 
to the cerebral cortex, a disturbance of this is the most obvious cause 
for the loss. It is easy to understand how the sudden closure of one of 
the large vessels would produce such an anemia as to disturb conscious- 
ness, and, indeed, an embolus lodging in and occluding the middle 434 
DISEASES OF THE NERVOUS SYSTEM 
cerebral or the basilar artery, both favorite seats for such an occurrence, 
would offer the ideal conditions for a sudden loss of consciousness. 
Saveliew,1 who analyzed all the cases of cerebral emboli in Virchow's 
laboratory from 1856 to 1893, states that a sudden loss of consciousness 
is the common onset in cerebral embolism. When the artery is slowly 
occluded there is more opportunity for collateral circulation; primary 
cortical anemia is less apt to occur, and consciousness when lost is apt 
to be lost late. This late loss of consciousness must be due to secondary 
causes, and in association with occlusion of a vessel is probably dependent 
upon the oedema which accompanies and surrounds the anemic area. 
This secondary oedema causes, among other things, an increase in 
intracranial pressure, which may be so great as of itself to produce a 
cortical anemia. Destruction of the brain tissue itself must have an 
effect on consciousness, for it depends upon the association of various 
cerebral activities, and must be affected by anything that injures the 
structure upon which these depend. We do not know just how great the 
disturbance must be to abolish consciousness or the relative importance 
in this relation of the different parts of the brain. 
The loss of consciousness accompanying cerebral hemorrhage, although 
perhaps rarely the initial symptom, very commonly occurs early, and 
appears to be more frequent and usually of longer duration with this 
lesion than with either of the other two. The explanation is not clear, 
and many theories have been advanced. Gowers,2 in speaking of the 
loss of consciousness, says: "It is probably first due to irritative inhibition 
of the cortex, which is maintained by increased intracranial pressure. The 
laceration causes powerful irritation, and when the hemorrhage is into 
structures that have a wide relation to the cortex, as in the pons, a small 
extravasation, which can have little effect on intracranial pressure, 
causes early loss of consciousness, which endures." von Monakow 
analyzed the various effects caused by a hemorrhage into the substance 
of the brain, and divided them into the following groups: (1) The primary 
local destruction of the nervous tissue; (2) the oedema in its neighborhood; 
(3) the decrease in the amount of blood in the vessels in the neighbor- 
hood of the clot; (4) accompanying circulatory changes in the cortex; 
(5) the transmission of the mechanical shock caused by the sudden 
escape of blood into the brain in all directions throughout the intracranial 
cavity, this shock, besides its physical action, acting as a physiological 
stimulus to various parts of the brain; especially, he thinks, it may 
stimulate the vasomotor centres and cause a reflex contraction of the 
cortical vessels; (6) increase in intracranial pressure; (7) interference 
with circulation of the cerebrospinal fluid. In the last analysis he believes 
that the disturbance of consciousness depends upon a sudden cerebral 
anemia, and that this is brought about by some or all of the various 
factors enumerated above, but that probably the mechanical stimulation 
of the vasomotor centres "plays the lion's part." 
The occurrence and degree of coma does not depend upon the size of 
the hemorrhage, but seems to have some definite relation to its posi- 
1 Virchows Arch., 1894, cxxxv, 112. 
2 Brit. Med. Jour., London, 1907, ii, 1. DISEASES OF THE CEREBRAL BLOODVESSELS 
435 
tion. The exact relationship has not been definitely determined, but von 
Monakow states that after analyzing a great number of cases it appears 
to him that coma is more apt to occur the nearer the lesion is to the central 
gray matter of the third ventricle, or the nuclei of the optic thalamus, 
and that it is much less apt to occur when the white matter of the cerebral 
hemisphere is involved. When the hemorrhage breaks through into the 
ventricle consciousness is almost always lost, and Jones found that it 
was lost in 85.5 per cent, of this class of cases, while in the non-ventricular 
hemorrhages the percentage of loss was only 69.4 per cent. 
The duration of the coma varies greatly, and may be very short or 
persist until death. It is apt to be shorter when associated with em- 
bolism and longer with thrombosis and hemorrhage; but even in hemor- 
rhage von Monakow gives the average duration as only from a half 
hour to four hours. Oppenheim puts the duration somewhat longer, 
from one to four hours for the lighter cases and one to two days for the 
more severe ones. From the actual coma, patients not infrequently 
pass into a state of dull consciousness, during which they have very 
little recollection of what passes around them, and it is not uncommon 
for them to insist that they were unconscious for a week or even longer. 
Pulse and Blood-pressure.-During an attack of apoplexy th*3 condi- 
tion of the pulse varies markedly. A slow pulse, particularly with a high 
blood-pressure, indicates an increased intracranial pressure, and is usually 
associated with a cerebral hemorrhage. Thus, in an old colored woman, 
brought to the hospital directly after the attack, and who died within 
four hours from a large cerebral hemorrhage, the pulse on admission 
was only 48, and the blood-pressure w'as above 350 mm. Hg. In a man 
admitted on the third day after his stroke, the pulse rate was also 48, 
and the blood-pressure between 330 and 340 mm. Hg. A large capsular 
hemorrhage was found. These cases are exceptional and the rate of 
the pulse is often not much lowered, and is at times accelerated. 
The character of the pulse is usually of more importance, for in asso- 
ciation with cerebral hemorrhage it is, as a rule, hard and full, and the 
blood-pressure may be very high. Such an excessive elevation, although 
characteristic of acute increase in intracranial pressure, and perhaps 
never found clinically except with cerebral hemorrhage, is exceptional 
even with this condition. The blood-pressure more usually ranges be- 
tween 150 and 200, and even in rapidly fatal cases may be quite normal 
as in a woman, aged forty-three years, brought to the hospital shortly 
after an attack, in whom the pulse was 100 and the blood-pressure 125 
mm. Hg. She died within twenty-four hours, and a large hemorrhage 
was found in the right hemisphere. 
A blood pressure in the neighborhood of 200 is frequently found in 
association with arteriosclerosis, and as this condition often precedes 
for a long time the occurrence of an apoplexy due to either rupture or 
thrombosis of a cerebral vessel, the significance of a high blood-pressure 
during an apoplectic attack depends largely upon our knowledge of 
the previous conditions. Although it is generally stated that when an 
apoplexy has been due to thrombosis the pulse is quick, intermittent, and 
of low tension, it is not at all uncommon to find a high blood-pressure 436 
DISEASES OF THE NERVOUS SYSTEM 
associated with this condition. Janeway1 says: "Thrombotic softening 
is the common cause of hemiplegia in our patients, even when they have 
granular kidneys and high blood-pressure." 
Changes in blood-pressure are more significant than the actual height, 
and a rapidly rising pressure indicates an increasing cerebral compression, 
while a falling pressure is one of the most definite signs of failure of the 
vasomotor centres and of approaching death. A good illustration of the 
first condition is the case of a man brought into the ward shortly after 
having had a capsular hemorrhage. His blood-pressure was then 220 
mm. Hg. and the pulse about 90. The pressure rose steadily, and the 
next day reached 250, when an operation was undertaken. As the dura 
was opened the blood-pressure fell to 180 and subsequently even lower. 
It arose again, but never reached 200 during the three days which he 
lived. A most impressive example of the second sequence of events 
was in a man whom we had observed for a number of months. He showed 
the typical picture of cerebral arteriosclerosis, and had suffered from 
several vascular attacks. He was in the ward for nearly a month before 
the final accident. During the first few days of his stay in the hospital 
his blood-pressure ranged between 230 and 240, but subsequently about 
215, with a pulse rate of 95. One afternoon he complained of intense 
headache, for the relief of which morphin had to be given. At 6 p.m. 
the blood-pressure was 200. In the middle of the night he was found in 
coma. His blood-pressure at 3.30 a.m. was 240; pulse, 145. At 5.30 
the pressure was 200; at 11 it was 180, and then fell quickly, reaching 
80 at 1 p.m. and 60 at 1.15 p.m., the pulse remaining between 120 and 
130. Death took place at 1.40 p.m. At autopsy a large cerebellar hemor- 
rhage was found. 
Respiration.-The respiration is usually slow and deep; it is often 
stertorous and irregular, and at times shows the Cheyne-Stokes type. 
The noisy stertorous breathing is usually due to the fact that during 
coma the mouth has a tendency to fall open and the tongue to fall back. 
Bowles2 points out the importance of keeping patient on his side. 
Cheyne-Stokes breathing during apoplexy is usually a symptom of the 
embarrassment of the respiratory centres in the medulla as the result 
of anemia, secondary to increased intracranial pressure, and is an im- 
portant danger signal. The vasomotor centres tend to keep the blood- 
pressure above intracranial pressure, so that the medullary centres may 
still be supplied with blood. When the intracranial pressure remains 
constantly high, the blood-pressure may show periodic waves (Traube- 
Hering waves), varying from a little below to a little above the level of 
the intracranial pressure. Experimentally, when such variations occur, 
respiration becomes intermittent. In general, it ceases when the blood- 
pressure is relatively low and begins and increases when the blood-pressure 
rises above intracranial pressure. If careful tracings of the blood-pressure 
and respiration be made in a case of apoplexy showing the Cheyne-Stokes 
respiration, quite analogous conditions will be seen to be present. Eyster,3 
1 The Clinical Study of Blood-pressure, 1907, p. 189. 
2 On Stertor, Apoplexy, and the Management of the Apoplectic State, 1891. 
3 Jour, of Exper. Med., 1906, viii. No. 5. DISEASES OF THE CEREBRAL BLOODVESSELS 
437 
from a study of such cases, concludes that this type of Cheyne-Stokes 
breathing is due to the centre of respiration being intermittently 
supplied with blood; for when the centre has been deprived of arterial 
blood, it ceases to be capable of responding to its normal stimulus, i. e., 
an increase in its supply of carbon dioxide and a reduction in oxygen, 
and so when the arterial pressure falls below intracranial pressure the 
medulla becomes anemic and respiration ceases. Anemia of the medulla 
excites the vasomotor centres to renewed activity, blood-pressure is 
again raised, circulation is reestablished through the medulla, the respi- 
ratory centre regains its irritability, responds to stimuli, and respiration 
commences. The respiratory efforts vary in force in accordance with 
the varying relations between the irritability of the centre apd the force 
of the stimulus that excites it. 
There are often other phenomena due to the varying blood supply 
of the brain. The pulse varies in rate, being slow during the period of 
apnoea, as a result of the stimulating effect which anemia has on the 
cardio-inhibitory (vagus) centre. This inhibition is relaxed when respira- 
tion begins and the pulse rate is increased during the period of inhibitory 
activity. The mental activity also varies, and the patient, who is com- 
pletely unconscious when the respiration ceases, can be aroused and may 
speak voluntarily, during the period of respiratory activity. At times 
the pupils show a periodical change in their size, dilating at the. beginning 
of apnoea and contracting when respiration returns (Cushing). The 
importance of this type of periodic respiration, especially when accom- 
panied by these other phenomena, is as an indication of an increase of 
intracranial pressure to such a degree that the vasomotor centre has 
difficulty in maintaining the blood-pressure above the intracranial 
pressure, and is a symptom of the utmost gravity. 
Ophthalmoscopic Examination.-This may give important information, 
both as to the state of the bloodvessels and as indicating the condition of 
intracranial tension. Bordley, who examined many of the cases in the 
hospital, states that in most cases no other fundus changes were seen 
except those which preceded the stroke, i. e., the changes indicating 
degeneration of the bloodvessels. In certain patients, however, particu- 
larly in those who were profoundly unconscious and showed Cheyne- 
Stokes respiration, undoubted choked disk was present. This was often 
of low grade, and with more dilatation and tortuosity of the veins than 
is usually seen with such a degree of choked disk, but it was seldom asso- 
ciated with retinal hemorrhages and exudates. In the cases of traumatic 
intracerebral hemorrhage, in which there was rapid increase of intra- 
cranial pressure, choked disk was constant. 
Temperature.-When an apoplexy is due to cerebral hemorrhage there 
is usually an initial fall of from 1° to 2°. This may last only an hour or 
two, to be followed by a gradual rise, and may be entirely missed unless 
the patient is under observation at the time of the stroke. It is said that 
with thrombosis this initial fall does not take place. The writer has not 
sufficient exact data of cases in which the lesion was proved at autopsy 
to express any definite opinion. The cases of thrombosis which occurred 
in the hospital were in sick patients who usually had more or less fever 438 
DISEASES OF THE NERVOUS SYSTEM 
and the changes in the temperature at the time of the stroke were neither 
constant nor remarkable. 
The temperature almost always rises within the first twenty-four hours, 
and in rapidly fatal cases may reach great heights. Gowers associated 
an immediate elevation of temperature with hemorrhages into the pons 
or medulla. This hyperpyrexia is of great significance, and whenever 
it occurs is usually a sign of approaching death. In a patient already 
referred to, who died in about twelve hours from a cerebellar hemorrhage, 
the temperature rose rapidly to 100.5°. The attack took place in the 
night, and the initial fall of temperature, if it occurred, was not deter- 
mined. On the other hand, in certain quickly fatal cases the temperature 
continues to fall from the time of the attack, and may reach remarkably 
low levels. A rectal temperature of 95° has been recorded. The cause of 
these extreme temperatures seems to be a disarrangement of the heat 
regulating mechanism, and is one of the signs of failure of the cerebral 
functions. 
The ordinary rise of from 1° to 2° in the first few days after an apoplexy 
has no especially grave significance. It is the so-called fever of reaction, 
and is believed to depend upon some inflammatory process about the 
hemorrhagic focus. This fever usually lasts for only two or three days, 
after which the temperature remains normal. Should the fever be 
higher than usual or last longer, it becomes of grave significance, and at 
any time if the temperature rises rapidly to unusual heights the prognosis 
becomes very grave. 
Signs of Local Injury to the Brain.-The symptoms so far considered are 
general, but even during the height of an apoplectic stroke there are in 
most cases signs of local injury. These depend somewhat upon the 
location of the lesion, but for the most part indicate that the functions 
of one cerebral hemisphere have been disturbed. These symptoms are 
usually paralytic but may be irritative, when they are revealed by an 
excess of muscular activity. The limbs on the side of the body opposite 
to the lesion may be in a state of hypertension (early rigidity) with the 
eyes and head deflected toward the side of the spastic limbs and away 
from the lesion-spastic conjugate deviation; or the affected limbs may 
be the seat of clonic convulsions, and if the lesion is in the motor cortex, 
or near it, typical Jacksonian epileptic attacks may follow. 
Symptoms of paralysis are much more common, and all the movements 
represented on one side of the brain are usually more or less affected. 
During coma, when the patient is making no spontaneous movements, 
this is often shown by a decrease in the muscle tone on the side opposite 
to the lesion. The limbs are flaccid, they fall like a dead weight when 
raised and released, while those on the opposite side may show a certain 
amount of resistance. Heilbronner1 points out that this lack of muscular 
tone may be determined by inspection, for if the patient be on a firm 
support the muscle mass on the affected side, particularly that of the 
thigh, acts like a sac filled with a semi-solid, and takes the shape deter- 
mined by gravity. It is broadened and flattened, while that on the normal 
1 Deutsche Ztschr. f. Nervenh., 1905, p. 1. DISEASES OF THE CEREBRAL BLOODVESSELS 
439 
side has a more rounded contour. The lack of tone of the muscles of the 
face on the affected side is shown by the cheek being puffed out more than 
on the other side during forcible respiration, the air escaping from the 
corner of the paralyzed side of the mouth. At times the nostril on that 
side, instead of being expanded, is contracted during inspiration. 
Not infrequently the patient exhibits a tendency to lie with the head 
turned toward the non-paralyzed side and with the eyes deflected strongly 
toward that side, i. e., the patient looks toward his lesion. This is 
paralytic conjugate deviation, and is due to the fact that in each cerebral 
hemisphere movements are represented which turn the eyes and head to 
the opposite side and a destructive lesion paralyzes them. The normal 
position of the eyes depends upon the delicately adjusted activity of 
their muscles, and so when the tone of the muscles associated in a definite 
movement is decreased, the tone of the muscles causing the movement 
in the opposite direction deflects the eyes. Thus a lesion in the right 
hemisphere causes a decrease of the activity of the muscles which turn 
the eyes to the left, i. e., the left external rectus and the right internal 
rectus, together with the muscles active in the closely associated move- 
ment of the head to the same side, and it is the unbalanced tone of the 
muscles which opposes these movements which causes the deflection of 
the head and eyes to the opposite side. 
Muscle tone depends, for the most part, upon reflex stimulation, and it 
seems probable that conjugate deviation in apoplexy is, to a large extent, 
due to a disturbance of the reflex mechanism. At any rate, the symptom 
is usually transient, occurring during coma, and lasting from a few 
hours to one or more days, and is coincident with disturbances of other 
reflexes. When it persists for a longer time it is often associated with 
hemianopia, a condition in which all the visual stimuli come from but 
one-half of the patient's surroundings and tend to keep the eyes turned 
toward that side. The mechanism is a complicated one, and many 
stimuli must be active in maintaining the tone of the centres. That the 
visual impressions, although normally perhaps the most important, are 
not the only ones concerned, is shown by the interesting case reported 
by Dejerine and Roussy,1 in which conjugate deviation of the head and 
eyes occurred in an old woman who had been blind since infancy. 
Pupils.-During the apoplectic attack these are usually equal and 
about normal size. They may be dilated, contracted, or unequal, with 
the larger pupil on the side of the lesion when this is in a cerebral hemi- 
sphere, but on the opposite side when the focus is in the brain stem. 
During coma the light reflex is absent. 
Reflexes.-During absolute coma all reflexes are abolished, but very 
generally the coma is not of that grade, and one often is able to demon- 
strate a difference in the reflex activity of the two sides. In general, it 
may be stated that a sudden severe vascular lesion in one cerebral 
hemisphere has a tendency to abolish the reflexes on the opposite side 
of the body. The early conjugate deviation of the eyes and the lack of 
1 Rev. Neurol., 1905, xiii, 161; Weisenburg, Jour. Am. Med. Assn., 1907, xlviii, and 
Debray, Jour, de Neurol., October 5 and 7, 1907, have given interesting reviews 
concerning this subject. 440 
DISEASES OP THE NERVOUS SYSTEM 
muscle tone on the paralyzed side must, in a large measure, be due to this 
effect. The reflexes from the skin and mucous membranes, as well as the 
deep reflexes, may also be lost or altered, and when such a change is 
demonstrated on one side it is an important indication of a unilateral 
cerebral lesion. Since Babinski pointed out the varying response of the 
great toe to plantar stimulation under different conditions, this reflex 
has assumed the utmost importance. If a lesion has occurred which 
implicates the pyramidal tract, the great toe, instead of being turned 
down, moves in the opposite direction, i. e., in extension, or more properly 
dorsal flexion. This abnormal response (Babinski reflex) may appear 
directly after a cerebral lesion, and may be the first positive indication 
that such a lesion has occurred. The writer has noted this on several 
occasions, and in a patient whose attack occurred in the ward it was 
marked within twenty minutes. 
As the coma lessens the reflexes return, first on the non-paralyzed 
side, and according to von Monakow in the following order: first the 
conjunctival reflex, then the pupillary light reflex, the abdominal and the 
other skin reflexes, and finally the deep reflexes. The return of the 
reflexes on the paralyzed side may be delayed for a considerable time, 
even, it is stated, for two or three weeks. In our experience, however, 
the deep reflexes return quickly, and may even be exaggerated within 
a short time, although the corneal and the abdominal reflexes may remain 
absent. The importance of the absence of these two skin reflexes in 
association with hemiplegia has been insisted upon.1 
During coma the bladder and rectum are apt to empty involuntarily, 
but there may be retention, and as the patient recovers there is not infre- 
quently some difficulty in completely emptying the bladder. 
Paralysis of Voluntary Motion.-Unless the coma is absolute, observation, 
in the great majority of cases, reveals that the patient has lost certain 
movements. It will be noted that one arm and one leg will lie perfectly 
quiet, while the limbs on the other side may be moved restlessly about; 
that when the patient is moved in bed or irritated in any other manner, 
the movements which result are only one side of the body. This lack 
of motion may also affect the face on the same side, i. e., the patient 
has hemiplegia. At first, and in severe cases, all the unilateral movements 
are affected, but certain movements show a tendency to recover quickly 
or to be spared when the process is less intense. This selective paralysis 
in hemiplegia is of great interest and will be discussed in a subsequent 
section. The limbs on the non-paralyzed side are not infrequently 
moved restlessly about; the patient picks at the bedclothing, moves 
the hand to the head, draws up the leg, and may persist in these 
movements so constantly that at times they may suggest convulsive 
phenomena. 
The movements of the chest during respiration are generally recorded 
in our histories as being equal and we have endeavored to confirm the 
common statement that the movements on the paralyzed side are less 
than on the opposite side. 
1 Milian, Le Progres med., May, 1909. DISEASES OF THE CEREBRAL BLOODVESSELS 
441 
Diagnosis.-The diagnosis of coma due to a cerebral vascular lesion 
is usually made without difficulty, as when a patient who has shown 
evidences of the circulatory conditions which predispose to rupture or 
occlusion of an artery has symptoms of local involvement of the brain 
(numbness and weakness on one side of the body, unilateral muscular 
twitchings, etc.) and then passes into unconsciousness, during which 
definite evidences of hemiplegia are present. At times the diagnosis 
may be difficult, and, indeed, impossible, as when the coma occurs sud- 
denly without premonitory cerebral symptoms, or when the physician 
sees the patient for the first time during the attack, the onset of which 
has been unobserved. 
Coma may occur under a variety of circumstances, and is the frequent 
terminal state of all fatal illnesses. It is often an evidence of the toxemias 
associated with nephritis (uremia) and diabetes. It occurs as a result 
of poisoning by alcohol, opium, and its derivatives, illuminating gas 
(carbon monoxide), chloroform, ether, etc. It may be associated with 
sudden disturbances of the general circulation, if they are sufficient to 
produce cerebral anemia, as in the ordinary fainting fit, the attacks of 
unconsciousness with Stokes-Adams disease, the rupture of a large 
aneurism, etc. It follows an epileptic attack and may be purely hysterical. 
It happens occasionally in malaria and heat stroke, and it is a frequent 
result of trauma. 
In most cases the history will exclude many of the conditions in which 
coma may occur, and the mistakes are apt to happen when little or nothing 
is known of the patient or of the onset of the attack, as in hospital and 
police practice. Every such case should be regarded with suspicion, 
and the diagnosis of one of the less serious states should be deferred until 
the indications are clear and unmistakable. 
When the coma is so deep that all special activities of the brain are in 
abeyance, when no movements occur and no reflexes can be elicited on 
either side of the body, evidences of a focal lesion of the brain are, of 
course, absent, and as it is upon these that the diagnosis of a cerebral 
lesion must largely depend, it may for a time be quite impossible to 
determine the cause of the coma. 
Uremia.-In the absence of unilateral cerebral symptoms the diagnosis 
must depend upon the previous history, the occurrence of chronic neph- 
ritis, convulsions ushering in the coma, which usually develops rather 
slowly, etc. Objectively, the condition may be remarkably similar to 
apoplectic coma. The high arterial tension, Cheyne-Stokes respiration, 
and the ophthalmoscopic picture, may be quite similar in both condi- 
tions, and when it is remembered that transient focal cerebral symptoms 
are not uncommon in uremia, and that apoplexy frequently occurs in 
patients suffering from chronic nephritis, it will be seen how complicated 
the diagnosis may become. Eyster pointed out that in the periodic 
respiration of uremia the relation between the respiratory phases and the 
changes in the blood-pressure differs from that in Cheyne-Stokes respira- 
tion secondary to increased intracranial pressure, as in increasing cerebral 
hemorrhage, and it is probable that careful tracings of the respiratory 
and blood-pressure changes may give important indications. Simple 442 
DISEASES OF THE NERVOUS SYSTEM 
choked disk may be present in both conditions, but in nephritis the 
characteristic picture of albuminuric retinitis is much more frequent. 
Coma occurring in pregnant women at about the time of labor is usually 
due to toxemia (uremia or eclampsia), but exceptionally it depends upon 
a cerebral hemorrhage, which, unless accompanied by focal symptoms, is 
very likely to be overlooked. 
Diabetic coma generally causes but little confusion. The history of 
the preceding illness is usually obtained. The coma develops gradually, 
being preceded by lassitude, headache, great restlessness, and other 
symptoms. The pulse is apt to be rapid and of low tension, and the 
respirations show "air hunger," being full and noisy, but quite regular 
and usually not increased in frequency. The breath has a fruity odor, 
and so may the urine, which shows the presence of sugar, diacetic acid 
and acetone. Very rarely optic neuritis is present. 
It must be remembered that the deep reflexes are often lost in diabetes, 
and their absence should not be given undue significance. Confusion 
may also arise from the fact that hemiplegia, in which no anatomical 
lesions were found, has been noted in diabetes. On the other hand, sugar 
has been found in the urine as the result of a cerebral vascular lesion. 
It is always safer to assume a definite focus when the symptoms indicate 
its presence. 
Alcoholic Coma.-The mistake of regarding a case of apoplexy as 
one of acute alcoholic intoxication has been made innumerable times, 
and whenever this question arises the patient must be given the benefit 
of every doubt. A history of former alcoholism, and a marked alcoholic 
odor of the breath and vomitus have significance, but it must be remem- 
bered that alcohol is universally administered as a medicine under all 
conditions, and may have been swallowed at the onset of the attack, 
and also that apoplexy not infrequently occurs during an alcoholic 
debauch. The coma due to alcohol is almost never absolute, and the 
patient can usually be aroused to show some evidence of activity. This 
fact permits the discovery of paralytic symptoms, and it is the absence 
of these in such a patient, with the history, that justifies the probable 
diagnosis of drunkenness. 
Opium Poisoning.-In acute opium poisoning the coma simulates 
intense drowsiness; the patient can be aroused by energetic stimulation, 
but lapses again into unconsciousness as soon as he is undisturbed. The 
pupils are contracted to an extreme degree; the respirations are stertorous, 
very slow, and may be regular, but at times occur in groups, as in the 
Cheyne-Stokes type. The skin is cold and of a livid hue; the pulse is 
rapid and of small volume. This picture is so characteristic that even 
without the history of administration of the poison mistakes are not apt 
to occur. It must not be forgotten that very small pupils sometimes 
occur with a hemorrhage into the pons or mid-brain, and that they may be 
associated with some preexisting condition, such as tabes and general 
paralysis, in which apoplexy is not uncommon. 
Poisoning by other agents rarely gives rise to much confusion, but 
where nothing is known of the history of the patient one should be on 
the alert not to overlook them. The burning of the mouth and the intense DISEASES OF THE CEREBRAL BLOODVESSELS 
443 
gastro-intestinal disturbances, associated with symptoms of collapse, 
are usually sufficient to indicate the presence of carbolic acid poisoning. 
In unconsciousness from carbon-monoxide poisoning the history is 
almost always obtained. In doubtful cases the blood must be examined. 
Its bright cherry-red color and the characteristic reactions of carbon 
monoxide are conclusive. 
Coma following changes in the general circulation is clearly distinguished 
by the associated conditions, and rarely gives rise to confusion. The 
ordinary fainting attack (syncope) is so well known as to require no 
comment. The attacks of unconsciousness in heart block are transient, 
and are so definitely associated with a pause in the ventricular pulse 
that mistakes should not occur. Rapidly occurring coma from the 
rupture of an aneurism, which persists until death, may, at times, be 
confused with that due to a large cerebral hemorrhage. The history 
and physical signs are usually sufficient to indicate the presence of an 
aneurism, and death is, in such cases, more rapid than in even the most 
severe apoplexies. 
Absolutely sudden death, which is so often recorded in death certificates 
as due to apoplexy, is usually associated with a stoppage of the heart due 
to an obliteration of one of its coronary arteries. Apoplexy, even in the 
most quickly fatal cases, lasts a considerable time. Death within one or 
two hours is a rare occurrence, and the case recorded by Abercrombie 
nearly one hundred years ago, in which death occurred in five minutes, 
has not so far as the writer knows, been equalled. The quickest death 
in our records occurred in a little less than an hour, and in this case, 
besides the cerebral hemorrhage, there was a ruptured mesenteric 
aneurism. 
Epilepsy and Hysteria.-The stupor following an ordinary isolated 
epileptic attack is usually easily recognized. The history of previous 
attacks, the characteristic convulsion which immediately preceded the 
coma, and its relatively short duration are significant. A case seen for 
the first time in the status epilepticus might easily be mistaken for one 
of convulsions and coma, following a cerebral vascular lesion, and the 
diagnosis would have to depend upon the subsequent course or the results 
of an autopsy. Unilateral epileptic convulsions are often followed by 
evidences of transient paralysis-a slight hemiplegia-even when there is 
no gross cerebral lesion; and, on the other hand, in patients past middle 
life, Jacksonian epilepsy is not infrequently the result of a previous cortical 
vascular lesion; and, indeed, such attacks may be the signs of a cerebral 
arteriosclerosis and the premonitory symptoms of a gross vascular lesion. 
In general paresis convulsive seizures and transient paralytic phenomena 
are not uncommon, but as cortical lesions often occur in this disease, one 
must be cautious in estimating the importance of an individual attack. 
Hysteria at times introduces most puzzling and interesting questions 
of diagnosis. It should always be remembered, what is so often forgotten, 
that a hysterical patient has the same liability to organic disease that any 
other similarly situated individual has, and that in a predisposed individ- 
ual hysterical symptoms may be so added to those which are dependent 
upon an organic lesion as to seriously confuse the picture. The definite 444 
DISEASES OF THE NERVOUS SYSTEM 
evidence of organic disease has the same significance in a hysterical patient 
as in any other condition. 
Hysterical coma has the appearance of deep, normal sleep, which is 
unduly prolonged or which has occurred during the daytime after some 
psychical shock. The patient can usually be aroused, at least for a time, 
by painful stimuli, as a faradic brush, pressure over the supra-orbital 
nerve or the eyeballs, etc., or by the determined command of the physician 
or the suggestion of some disagreeable procedure. The pulse and respira- 
tion are usually not markedly altered, although at times the respiration 
shows most bizarre changes with entire absence of regularity. It may 
have an extremely rapid rate, suggesting the panting of an animal, or 
it may be so infrequent as to give rise to the fear that the patient is about 
to die from suffocation; and in very rare instances it shows the periodicity 
of the Cheyne-Stokes type. Persistent hiccoughing may be an alarming 
and confusing symptom, as this is also a symptom of an organic brain 
lesion. The temperature, unless in exceptional cases, is quite normal. 
When such a condition has been ushered in by a typical hysterical 
convulsion one may feel reasonably sure of the diagnosis. The con- 
vulsions, however, may be absent or far from typical, and the condition 
so obscure that it is only after a detailed and fruitless search for some 
evidence of an organic lesion that a tentative diagnosis of hysterical coma 
is justified. The diagnosis of hysteria, especially where symptoms, 
apparently grave, are present, should never be more than a provisional 
one, and should stimulate to repeated examinations. The paralyses 
associated with hysterical coma are to be distinguished by the absence 
of the distinctive characteristics of an organic type of paralysis. 
Malaria and Sunstroke-The sudden loss of consciousness that occurs 
exceptionally in malaria, and, as a rule, in heat stroke, may simulate 
the coma of apoplexy, and in malarial districts the diagnosis is often 
difficult. High fever is common in both malaria and heat stroke, and in 
the latter it may reach a very high point, 108° or even more. The dis- 
covery of the malarial parasite in the blood is conclusive of that disease, 
and the blood should alwTays be examined in cases of coma where there 
is a possibility of malaria being the cause. The diagnosis of sunstroke 
depends upon the history of the onset under conditions that may induce 
it, and the absence of the evidences of paralyses characteristic of apoplexy. 
Trauma.-In coma following injury to the skull the history of the blow 
is usually clear, but in a case in which the history is unknown, or the 
circumstances arouse suspicion of foul play, a careful examination of 
the head must be made. Many of the symptoms following injury to the 
head are due to hemorrhage within the cranium and give rise to the same 
localizing symptoms found in spontaneous apoplexy. It is in these cases 
of hemorrhage, usually from a meningeal vessel, that the symptoms 
of an increasing intracranial pressure are most characteristically shown. 
Such evidences, however, do not always mean an increasing hemorrhage, 
for they may be due to oedema which follows the trauma. 
From what has been said it follows that it is often difficult to determine 
at once whether coma is due to a cerebral vascular lesion or some other 
cause, but if the inquiry be made carefully and in such a manner as to DISEASES OF THE CEREBRAL BLOODVESSELS 
445 
reveal the presence of the various conditions most likely to cause mistakes, 
and the conclusion not too hastily made, grave errors can usually be 
avoided. The history must be gone into carefully and confirmed in 
every way possible, the patient examined for the evidence of any bodily 
injury, the urine obtained and examined, the bodily temperature, respira- 
tions and pulse watched carefully, the blood examined, and the ophthal- 
moscopic examination must not be omitted. 
Important as these procedures are, the proof that there has been a 
local injury to the brain and that the coma is associated in some way 
with the cause of the lesion, depends upon the discovery of some irregu- 
larity in the nervous activities pointing to such a lesion. These symptoms 
have been considered but it is at this stage in the examination that the 
necessity for a systematic search for them must be kept in mind. 
The attitude of the patient must be observed. Is he quiet or restless? 
If restless, are the movements confined completely or largely to one side 
of the body? Are the movements purposeful or not? If convulsions 
occur, it is of the utmost importance to note the movements which are 
first involved; their march, extent, and duration. Is there any difference 
in the muscle tone of the two sides of the body? If there is such a differ- 
ence, are the limbs abnormally flaccid or unusually stiff? 
Does the patient tend to take any particular attitude? Is the head 
turned to one side and held there? Are the eyes in the median line or 
turned to one or the other side? Are they steady, or do they move and 
to what extent? What are the conditions of the pupils as to their size 
and reaction to light? Is there any asymmetry of the face during respira- 
tion? Do the two nostrils dilate equally on inspiration? Is one cheek 
blown out more than the other during expiration? Are the visual 
apertures equally wide? Are the wrinkles on one side of the face more 
pronounced than on the other? 
The movements of the chest and abdomen during respiration should 
be carefully watched to see whether there is any difference between the 
two sides, and this should be observed both in quiet and forced breathing. 
Does the patient respond to stimuli upon the two sides of the body 
equally, i. e., is there any evidence of disturbed sensation? Care must be 
taken not to mistake simple reflex responses for the more elaborate move- 
ments that we believe to depend upon the activity of the cerebral cortex. 
If the patient is sufficiently conscious, tests should be made to determine 
whether he sees objects on both sides, so as to exclude hemianopia. 
The reflexes must be carefully investigated, particularly as to the 
relative activity on the two sides. The reflex movements that follow 
stimulation of the cornea, the nasal mucous membrane, the skin of the 
abdomen, and, in man, of the inner side of the thigh, are the most impor- 
tant of the so-called skin or superficial reflexes. Babinski's reflex and its 
modifications must be tested for with great care. The deep reflexes must 
be noted. 
The condition of the skin of the patient is important. Is there any 
difference in the two sides as to the color of the skin, its temperature, 
and the amount of visible perspiration upon it? 
When unilateral symptoms are demonstrated the presence of a local 446 
DISEASES OF THE NERVOUS SYSTEM 
lesion of the brain is strongly suggested, and when the symptoms have 
developed in a sudden apoplectiform manner the diagnosis of some one of 
the vascular lesions is usually justified. At times, however, confusion 
may be caused by hysterical paralysis. In this there is often complete 
paralysis of the limbs on one side of the body, with marked disturbances 
of sensation, which may have developed suddenly after a period of real 
or apparent unconsciousness. At times, even with a thorough under- 
standing of hysteria, together with a knowledge of the characteristics of 
organic paralysis, the diagnosis may be extremely difficult. Usually, 
however, the hysterical nature of the paralysis is manifest. The attack 
occurs in a hysterical subject after some psychical shock. It usually 
develops somewhat slowly and without loss of consciousness. The loss 
of motion is confined, in the great majority of cases, to the arm and leg, 
and spares the face and the other muscles supplied by the cerebral nerves. 
In the limbs affected all movements are completely lost, and the paralysis 
does not show the selective distribution which follows a cerebral lesion. 
The paralyzed limbs are often quite flaccid, but contractures do not 
infrequently occur. There is very generally a loss of cutaneous sensation 
which is apt to involve the whole affected side, including the head. 
The anesthesia also involves the mucous membranes, and may include 
the nerves of special sense. 
In sharp contrast to this absolute loss of function, depending, as we 
believe, upon some abnormality in the field of consciousness, is the quite 
normal activity of the lower nervous centres. There is no disturbance 
in the functions of the bladder and rectum, and the pupils react normally 
to light. The deep reflexes, although at times very active, are not so 
exaggerated as to cause a clonus, and are usually equal on the two sides 
of the body. This is also true of the reflexes from the skin. Plantar 
stimulation gives the normal plantar flexion of the great toe. 
The hysterical loss of function is not infrequently influenced by sugges- 
tion. This is particularly true of the anesthesia which at times can be 
dispelled or transferred to the opposite side by various procedures. 
The paralysis may be similarly influenced and absolutely disappear after 
tetanization of the muscles by the application of the faradic current to 
the nerve trunks. 
Under normal conditions the muscular activity involved during any 
strong voluntary effort spreads far beyond the muscles primarily con- 
cerned, i. e., there is a synergistic action of many other muscles. This 
may consist of contraction or relaxation, and may even involve move- 
ments of the opposite side of the body. It seems to depend upon an 
automatic action of the lower centres,1 and should not be affected in 
hysteria. Hoover's2 observation seems to be an illustration of this. 
He has shown that when a normal person lying on his back endeavors 
to lift the extended leg from the couch, the opposite leg is pressed down- 
ward, and that in an organic hemiplegia this same pressure occurs when 
the patient tries to lift the paralyzed leg. If in such a case the patient 
lifts the unparalyzed leg the downward pressure of the paralyzed leg is 
1 Sherrington, Quart. Jour. Exper. Phys., 1909, ii, 109. 
2 Jour. Am. Med. Assn., 1908, li. DISEASES OF THE CEREBRAL BLOODVESSELS 
447 
proportionate to its remaining strength. If the hemiplegia is due to 
hysteria the action is different. When the patient raises the non-affected 
leg the opposite leg, totally paralyzed for voluntary effort, is pressed 
strongly into the couch, while if he be told to endeavor to raise the 
paralyzed leg there is no movement on the non-paralyzed side, indicating 
that no outgoing stimuli have left the cerebral cortex, just as if he were 
consciously simulating the paralysis. Risien Russell1 called attention 
to a somewhat similar phenomenon in hysteria, but in this case it concerns 
the lack of relaxation in the antagonistic muscles. 
Hysterical hemiplegia was formerly believed to affect the left side much 
more frequently than the right, but Ernest Jones,2 in an analysis of 277 
cases, found no marked difference between the two sides; indeed, the right 
side was a little more frequently affected than the left, the percentage 
being 54.2 on the right side and 45.8 on the left. 
It is often stated that hysteria may simulate every type or form of 
organic paralysis, but this simulation is usually superficial and disappears 
before a detailed examination. Mistakes are more commonly made by 
overlooking what is at least equally true, that many organic diseases may 
simulate hysteria, and that it is not at all uncommon to have a combina- 
tion of the two conditions. This was well illustrated in the case of an 
unmarried woman, aged forty-five years, who after a long and severe 
emotional shock had an attack of dizziness associated with paralysis 
of the left arm and leg. She was admitted to the hospital in a highly 
nervous state, and the diagnosis of hysteria was made. The paralysis 
of the arm cleared up rapidly under suggestion, but that in the leg devel- 
oped the typical signs of an organic paralysis. The distribution was 
characteristic, as was the spastic condition -with well-marked ankle 
clonus. The patient was subsequently under my care for a number of 
years, and passed through numerous hysterical storms, but the paralysis 
of the leg remained constant and resisted all forms of suggestive treatment. 
Diagnosis of the Vascular Lesion.-After the diagnosis of apoplexy has 
been made, i. e., that the patient is suffering from the effects of a sudden 
vascular lesion, it becomes of great importance to determine, if possible, 
whether there has been a rupture of an artery and blood has escaped into 
the brain, or whether an artery has become plugged by a thrombus or by 
an embolus, causing cerebral softening. The diagnosis of the cause of 
the apoplexy in any individual case can be made only with a certain 
degree of probability. This degree may be so high as to almost reach 
certainty, or, on the other hand, so low as to amount to practically little 
more than a guess. Unfortunately, in the majority of cases the diagnosis 
has to remain very uncertain. 
The brain lesion is the result of a vascular state and it is this that must 
be kept in mind. The vascular conditions liable to occur at the various 
periods of life differ, and so the age of the patient is an important point 
in diagnosis. In childhood and youth the spontaneous rupture of a 
cerebral vessel is rare, and apoplexies at that time of life, when not 
dependent upon inflammation, are very generally due to cerebral softening 
1 Brit. Med. Jour., 1908, i, 608. 
2 Rev. Neurol., 1908, p. 193. 448 
DISEASES OF THE NERVOUS SYSTEM 
and are in association with some definite cause, acute diseases, syphilis, 
etc., for thrombosis, and endocarditis for embolism. 
The diagnosis of cerebral embolism depends upon the determination 
of the source of the embolus, and when such is found the inference is 
usually correct. Gowers points out that the presence of endocarditis 
is not excluded by normal heart sounds, and under conditions in which it is 
common, chorea, for instance, it should be suspected even when it cannot 
be determined. In the later decades, when degenerative changes in the 
vessels have become common, the association between valvular heart 
disease and apoplexy has less significance as indicating embolism, but 
even in early life one is occasionally surprised to find at autopsy a rupture 
of a vessel when its occlusion was confidently expected. 
Between twenty and forty most cases of apoplexy are due to thrombosis 
secondary to syphilitic arterial disease, and then, or even before and 
after this period, when the cerebral accident occurs within a few years of 
the primary syphilitic infection, thrombosis is the most probable. 
Cerebral hemorrhage, in spite of its rarity in the first half of life, should 
be suspected whenever the apoplexy has the distinctive characteristic 
of such an accident, especially when it occurs in apparent health in a 
subject in whom syphilis is unlikely or in conditions in which hemorrhages 
are common, leukemia, pernicious anemia, eclampsia, etc. 
In the latter or degenerative period any one of the vascular conditions 
may occur. It would be important to know their relative frequency, but 
accurate knowledge could be obtained only from mortality statistics in 
large communities in which the cause of death was determined by autopsy. 
In the cases dying in the Johns Hopkins Hospital softening was more 
common than hemorrhage, there being 30 of the former and 26 of the 
latter. Table VI shows the occurrence of these lesions in the various 
decades at the time of death. Ludlum,1 who analyzed the material in 
Spiller's laboratory found a still greater frequency of softening, his figures 
being 69 of such lesions to 24 of hemorrhage; but Cadwalader,2 using the 
same material and also that of the Pennsylvania Hospital, collected 
72 cases of hemorrhage and 50 of softening. In the Royal Victoria 
Hospital, Montreal, the figures of which Dr. Adami put at my disposal, 
the relative frequency of the lesions was about that found at the Johns 
Hopkins Hospital, being 35 of softening and 29 of hemorrhage. These 
and other figures are given in Table VII. 
Table VI.-Johns Hopkins Hospital.-Fatal Cases. 
Decades. 
Total. 
1 
2 
3 
4 
5 
6 
7 
8 
9 
Cerebral softening 
1 
0 
3 
3 
8 
9 
2 
3 
1 
30 
Hemorrhage 
0 
0 
1 
4 
4 
12 
4 
0 
1 
26 
1 Jour. Nerv. and Ment. Dis., 1909. 
2 Jour. Am. Med. Assn., 1914, Ixii, 1385. DISEASES OF THE CEREBRAL BLOODVESSELS 
449 
Table VIL 
Hemorrhage. 
Softening. 
Johns Hopkins Hospital 
26 
30 
Dr. Ludlum (Philadelphia Hospitals) 
24 
69 
Royal Victoria Hospital, Montreal .... 
29 
35 
Montreal General Hospital, Dr. Duval . 
53 
44 
Boston City Hospital, Dr. Mallory .... 
132 
78 
Presbyterian Hospital, New York .... 
112 
48 
University College Hospital, London 
123 
37 
Total 
499 
341 
From these figures hemorrhage appears to be considerably more fre- 
quent, but as Gowers points out this is probably due to the fact that as 
hemorrhage is more often quickly fatal than softening, more such cases 
would be expected to die in hospitals. He thinks it probable that 
softening is in general the more common lesion. Clinically, the probable 
diagnosis of cerebral softening, in the writer's experience, has been much 
more often justified than that of hemorrhage. 
The character of the apoplectic stroke itself gives some indication as to 
its cause. Premonitory symptoms when they have been present for two 
or three days, especially if there is a history of several slight previous 
cerebral attacks, suggest the gradual, onset of thrombosis. In cerebral 
hemorrhage slight symptorns not infrequently precede the culmination 
of the attack, but do so for a much shorter period, from a few minutes 
to a few hours, and there may be the history of a previous apoplexy of 
considerable severity, but not commonly of repeated slight attacks. 
Hemorrhage may occur, however, after several thrombotic attacks. 
The occupation of the patient at the time of the stroke may give some 
indication, for hemorrhage is to be expected when the circulation is 
active and thrombosis under the opposite conditions, when the circulation 
is depressed. Although it is uncommon to find a history of severe physical 
effort at the time of a cerebral hemorrhage, still more cases occur during 
the active hours of the day, and rest in bed and sleep seem to protect 
to a certain extent against this occurrence. Thrombosis is more common 
during sleep and rest in bed than hemorrhage, and this probably accounts 
for the preponderance of cerebral softening found at autopsy in hospitals 
where old chronic cases are treated. If one could determine exactly 
the state of the circulation at the time of the apoplexy it would be a great 
help in the diagnosis between these two lesions. 
The essential thing to know is the circulatory condition at the site of 
the lesion, but the condition of the peripheral circulation is an imperfect 
index of this, especially if nothing is known of the patient previous to the 
attack. High blood-pressure is a frequent accompaniment of advanced 
arteriosclerosis, and may precede and accompany thrombosis as well as 
hemorrhage and should not be given too much weight. On the other 
hand, a feeble, soft pulse, with a weak, irregular heart, points strongly 
to thrombosis. 450 
DISEASES OF THE NERVOUS SYSTEM 
Coma is much more frequently absent in thrombosis than in hemor- 
rhage, and when it develops rapidly it strongly suggests the occurrence 
of the rupture of a vessel. This is particularly so when the focal symp- 
toms are not pronounced. On the other hand, a widespread paralysis, 
which has developed with slight or transient disturbances of consciousness, 
is usually due to thrombosis. However, there are many cases of throm- 
bosis which have the typical development of hemorrhage, as well as a 
group of hemorrhage cases that have the onset typical of thrombosis. 
A marked fall in the bodilytemperature immediately after the apoplectic 
stroke speaks for hemorrhage, as do a rapidly rising blood-pressure, Cheyne- 
Stokes respiration, and the other signs of increased intracranial pressure. 
Convulsions, particularly if repeated, point to an irritative lesion 
in or near the cortex, and are much more frequently associated with 
softening than with hemorrhage, and more often with embolism than 
with thrombosis. Cerebral softening bears the same relation to localized 
paralyses-monoplegias, the different types of aphasia, hemianopia, etc. 
Hemiplegia is the usual result of hemorrhage, but is also common with 
softening. 
Hemorrhage, in the great majority of instances, is from some one of 
the central nutrient arteries, and involves the basal ganglia and internal 
capsule. This was true in 19 of the 27 lesions analyzed by the writer. 
The other lesions were situated as follows: twice in the frontal, twice in 
the occipital, and once in the temporal lobe. The cerebellum was 
involved twice, once with the pons, and in one instance the clot was in 
the pons and medulla. In Ellis' 31 cases it was in the region of the 
basal ganglia 26 times, not designated once, and was once each in the 
following situation: occipital lobe, parietal lobe and postcentral gyrus, 
cerebellum and pons. Hemorrhages into the pons are more frequent 
than those in the cerebellum (v. Monakow). True cortical hemorrhages 
are rare and are usually small. 
Softening frequently involves the cerebral cortex; 20 of my 30 cases 
were so localized, and the basal ganglia and internal capsule showed 
softening 8 times, and in 2 instances the focus was in the lateral aspect 
of the medulla. Ludlum's experience was different. He found among 
69 areas of softening only 9 in the cortex, all the others being in the 
neighborhood of the internal capsule. Thrombosis may occur anywhere, 
but is more frequent in the distribution of the middle cerebral artery and 
in that of the posterior cerebral. Emboli, in the great majority of cases, 
are lodged in the middle cerebral artery and its branches. 
Therefore it appears that when the symptoms indicate a capsular 
lesion the probability is somewhat, although not much, greater that it is 
hemorrhage, but when they indicate a lesion in some other situation, 
the cortex particularly, it is apt to be softening. The examinations 
of the blood serum and spinal fluid may help to distinguish hemorrhage 
from softening. Thus a positive Wassermann reaction and the other 
signs of syphilis speak for thrombosis and softening. Blood or a yellow 
or reddish-brown color in the spinal fluid indicates hemorrhage.1 P. 
1 Bigelow, Cleveland Med. Jour., 1913, xii, 265. DISEASES OF THE CEREBRAL BLOODVESSELS 
451 
Marie and Leri1 noted that in cases of hemorrhage the blood serum has a 
fluorescent, greenish appearance not seen in softening. This appears 
shortly after the stroke and may persist for a week. 
Prognosis.-The diagnosis of a cerebral vascular lesion always entails 
a serious prognosis, not necessarily as to the outcome of the present 
attack, but very generally as to the underlying vascular condition, which 
must remain a source of constant danger. The chief exceptions are those 
accidental cases occurring in the young in relation to acute infectious 
diseases, parturition, etc., and of certain cases due to syphilitic endarteritis 
which is amenable to treatment. 
The great majority of apoplectic attacks are recovered from. In my 
own list there are the histories of 740 patients, most of whom had recovered 
from the attack and applied for relief of the residual paralysis. There 
were 448 of these. The fatal cases in the present list were among those 
treated in the hospital, and number 97 out of a total of 292 hospital cases. 
This includes a number in which the apoplexy was a contributory and 
not a direct cause of death. These figures give no idea of the relative 
frequency of death. The dispensary cases were all, of course, drawn from 
those that recovered, and there is no means of ascertaining the fatal 
cases of apoplexy occurring in the same community. The percentage 
of deaths (33.2 per cent.) among the hospital patients must be much 
greater than that which obtains in general, for it applies to a special 
group composed largely of the more severe cases or those complicated by 
other conditions. 
In many cases previous attacks had occurred; usually one previous 
attack, not infrequently two and in several instances there were histories 
of three or more definite strokes. The time between the attacks may be 
short. It is usually estimated in months, and often there is an interval 
of years. The longest interval was sixteen years between the first and 
last attack. In this case the second attack occurred in two years, but 
there was an interval of thirteen years between the second and third and 
two between that and the fourth. The idea that the third attack is 
fatal has often been refuted. Among our cases, 79 died in the first, 10 in 
the second, 4 in the third, 1 in the fourth, and 3 after several attacks. 
The gravity of the prognosis of any attack increases with the suddenness 
with which the coma has occurred, with its depth and with its duration. 
If the coma shows no sign of lessening after twenty-four hours the progno- 
sis is very grave and becomes more and more grave the longer the coma 
persists. Patients have been known to recover from coma which has 
lasted for a long time, even up to six weeks. The writer saw a woman, 
nearly eighty years old, who had been in coma for three days, return 
suddenly to perfect consciousness as from sleep, much to the surprise 
of her watching relatives and to the chagrin of her physicians, who had 
given an absolutely hopeless prognosis. If the coma, at first slight, 
becomes later more pronounced, or returns after the patient has regained 
consciousness, it is of more serious import that the same degree of uncon- 
sciousness at or shortly after the onset of the attack. 
1 Bull, de I'Academic de Med., 1914, Ixxi, No. 22. 452 
DISEASES OF THE NERVOUS SYSTEM 
When the initial fall of temperature, common in cerebral hemorrhage, 
persists and increases, it is of very grave import. On the other hand, 
when the temperature rises quickly, or the fever lasts longer than the first 
two or three days, the outlook is grave, and if at any time the temperature 
reaches great heights, it is almost always a signal of approaching death. 
Marked changes in the respiration, particularly when it assumes the 
Cheyne-Stokes type, are of grave importance as indicating embarrass- 
ment of the respiratory centre. Cheyne-Stokes respiration, however, 
does not always mean immediate death; it may persist or come and go 
during many days. It may also have been long present before the 
apoplexy in association with cardiac or renal disease. 
Rapidly rising blood-pressure as indicating increasing intracranial 
pressure is, of course, a serious symptom, but a constantly falling blood- 
pressure from a previously high level is one of the surest signs of approach- 
ing death. Cardiac failure has its usual significance. Any symptoms 
pointing to involvement of the lungs are very important, for many cases 
of apoplexy die of a terminal pneumonia. 
An increasing swelling of the optic nerves is a serious symptom as 
indicating an increasing intracranial pressure, but it may be in association 
with uremic oedema of the brain and may entirely subside. 
Any estimate as to the duration of an attack is very uncertain. The 
most rapidly fatal cases usually live for several hours, and a patient may 
survive a large hemorrhage for several days, even when it has ruptured 
into the ventricle. The time of death varies, among other things, 'with 
the character of the lesion, and Jones1 gives an interesting table compiled 
from cases proved by autopsy: 
Lesion. 
Number of 
cases collected. 
Percentage dying 
within twenty- 
four hours. 
Within a 
week. 
Within a 
month. 
Hemorrhage 
. . 828 
30.4 
63.8 
79.4 
Thrombosis 
. . 158 
15.8 
38.0 
74.7 
Embolism . 
. . 273 
8.0 
35.5 
56.0 
In our experience the average time of death in all fatal cases when 
this could be determined was about eight days. For the cases proved 
by autopsy it was 6.2 days; for cerebral hemorrhage, 5.3 days, and for 
cerebral softening, 7.5 days. The few' cases in which the thrombosis 
was secondary to embolism had a somewhat longer duration-nine days. 
The age and general state of health have also an influence on the 
prognosis. Young, strong individuals often survive a severe cerebral 
shock that would be rapidly fatal at a later period of life. On the other 
hand, in elderly people whose arteries are badly diseased, usually with 
associated cardiac and kidney trouble, a very slight stroke may be the 
beginning of a spreading thrombosis, and the patients become duller, 
lapse into coma, and die after a protracted illness. 
Treatment.-Prophylactic.-This should begin with the prevention 
of the conditions liable to bring about vascular degenerations. As our 
knowledge increases as to the cause of arteriosclerosis, it is probable that 
1 Brain, 1905, xxviii, 546. DISEASES OF THE CEREBRAL BLOODVESSELS 
453 
we will know better how to ward off or delay its occurrence. The enor- 
mous part that syphilis plays in the etiology of the apoplexies of early 
adult life makes the importance of its prevention and treatment too 
obvious for comment. 
When vascular conditions are present that make the rupture or occlu- 
sion of an artery probable, we can, unfortunately, do but little to restore 
the integrity of the vessel wall, except when the change is syphilitic. 
When there is any evidence of involvement of the cerebral arteries in 
individuals under forty, syphilis should be suspected and thoroughly 
treated, even when there is no history and its possibility seems unlikely. 
Patients with damaged bloodvessels should avoid excesses of all kinds, 
not only those of alcohol and tobacco, etc., but also excessive mental or 
physical labor. The diet should be light, the bowels kept freely open, 
and the other measures recommended for arteriosclerosis should be 
employed. If such measures do not cause a reduction in the blood- 
pressure when it is high, it is doubtful whether vasodilators are of any 
permanent value. Apoplexies occur during every conceivable activity 
of daily life, and so it is inadvisable to restrict the patient within too 
narrow limits. 
Apoplexy.-Here as elsewhere effective treatment must depend upon 
an accurate diagnosis. In this instance similar symptoms may be due 
to vascular lesions of exactly opposite character-on the one hand the 
rupture of an artery, and on the other its occlusion, conditions which 
indicate quite different procedures if they are to be met directly. It is 
impossible in many cases to be certain which of these lesions is present, 
and the treatment must be such as would be applicable in either condition. 
It is often of the utmost importance to decide whether active measures 
should be employed in the endeavor to influence directly the initial 
lesion, especially whether the patient should be bled or undergo a sur- 
gical operation, or whether the treatment should be conservative. 
Bleeding.-If the patient be seen immediately after the stroke, and 
the symptoms all indicate the bursting of an artery (a rapidly developing 
coma, lowered bodily temperature, a strongly acting heart, with a full, 
bounding pulse), and we have reason to believe that the high blood- 
pressure preceded and did not follow the hemorrhage, bleeding from a 
peripheral vessel might be indicated. This w'ould be done in the endeavor 
to so lower the pressure inside of the ruptured vessel that the hemorrhage 
would stop and the extravasated blood be allowed to clot and further 
destruction of the nervous tissue be prevented. It must be remembered 
that the symptoms of apoplexy are not all dependent upon the local 
effect of the extravasated blood, and that many of them, coma itself 
among the rest, are often believed to be due to reflex effects, among 
which anemia of the cortex is of great importance. If this be true, lower- 
ing of the arterial pressure could hardly be expected to help. 
High blood-pressure is by no means of itself an indication for blood- 
letting, for this may be a result and not a cause of the hemorrhage, as 
when the blood-pressure continues to rise, and there are other evidences 
of undue pressure on the medulla (Cheyne-Stokes respiration, etc.). 
Under these circumstances bleeding would seem to be absolutely contra- 454 
DISEASES OF THE NERVOUS SYSTEM 
indicated. If the apoplexy be due to the occlusion of a large bloodvessel, 
and this be associated with high blood-pressure, there can hardly be an 
excuse for venesection. 
When we remember how rarely it is possible to be certain that we are 
dealing with a ruptured and not with an occluded vessel, and even if we 
be sure that a hemorrhage has occurred, how uncertain the indications for 
the rapid lowering of the general blood-pressure are, and, indeed, how 
incomplete our knowledge is of the effects of bleeding on the cerebral 
condition, we will see how seldom we have a definite indication for 
bloodletting in apoplexy. 
All the recent authors refer to bleeding, but there is very little agree- 
ment as to the benefit which follows it, and it is difficult to find exact 
observations, von Monakow finds little reason, theoretically or prac- 
tically, for its employment, and Cushing speaks strongly against its use, 
but Gowers, Starr, Oppenheim, and others recommend it more or less 
strongly. Goldscheider,1 after considering the theoretical objections, 
concludes that these have been pushed too far, that they do not stand 
on a firm basis of known facts, and that although it must be acknowledged 
that permanent good effects are extremely rarely seen after bleeding in 
severe apoplexies, still he believes that it is indicated when the diagnosis 
of hemorrhage is certain and when the face is congested and the pulse is 
full and hard. He would bleed not only at the onset of the attack, but 
even later if coma had persisted with symptoms of hyperemia, or if the 
symptoms, which were at first slight, became more pronounced. In our 
own cases in which it has been used the writer has not been able to see 
evidence of the least permanent benefit. Even the blood-pressure, 
although it is reduced during the operation, rises rapidly again to near 
its original height. Only the most urgent cases were bled, however, and 
this may account for the lack of good results. 
Surgical Interference.-Spencer and Horsley suggested, in 1889, that 
hemorrhage from the middle cerebral artery might be controlled by 
compression of the carotid artery, this being applied by digital pressure, 
either through the skin or upon the exposed vessel, or by permanent 
ligature of the vessel. This apparently has never been carried out. 
Cushing,2 in 1903, urged that the clot be dealt with directly, and gave 
details of the surgical procedure. He bases this on the theory that the 
hemorrhage occurs rapidly, has ceased when the physician sees the 
patient, and that the immediate symptoms are, for the most part, due to 
increased intracranial pressure, which can best be relieved by opening 
the cranial cavity and removal of the clot. Cushing has operated on a 
number of desperate cases of capsular hemorrhage, and in several instances 
certain of the alarming symptoms improved markedly, at least for a 
time. The most certain effect was perhaps on excessively high blood- 
pressure when present. At times the change in blood-pressure is not so 
marked, and, indeed, a decompression may have little or no effect. This 
is particularly so when the blood-pressure has accompanied arterio- 
sclerosis and preceded and not followed the apoplexy. That the clot itself 
1 Deutsch, med. Wchnschr., 1907, xlviii, 1977. 
2 Am. Jour. Med. Sc., 1903, cxxv, 1017; Keen's System of Surgery, iii, 215. DISEASES OF THE CEREBRAL BLOODVESSELS 
455 
can be successfully evacuated without subjecting the brain to more 
trauma than is justified, awaits demonstration, although isolated cases 
have been reported that seem to suggest this possibility. A decompres- 
sion is clearly indicated when the patient's life is endangered by rapidly 
increasing tension. Marie and his co-workers1 called particular atten- 
tion to this, and recommend that the decompression be done over the 
sound hemisphere, for they believe that coma occurs only when the 
sound hemisphere is compressed, and that it is much less dangerous to 
operate on this side than on the other. They consider as favorable 
cases for the procedure those in which the coma and other signs of increas- 
ing intracranial pressure develop after twenty-four to forty-eight hours. 
A dangerous increase in intracranial pressure may also follow throm- 
bosis and embolism, and a decompression is equally indicated in these 
cases. Cushing has operated on a number of such cases, in several of 
which the procedure seems to have saved the patients' lives. Cushing's2 
brilliant example in operating on certain intracranial hemorrhages of 
the newborn has been followed successfully by a number of surgeons, 
particularly in France and Germany. K. Henschen3 reviews the subject 
and recommends as a combined diagnostic and therapeutic procedure, 
lumbar puncture with puncture of the anterior fontanel. If a subdural 
clot is found and can not be aspirated, the major Cushing operation is 
indicated. He refers to the reports of 16 cases operated on by Cushing's 
method, of which 7 were cured. 
The physician usually sees the apoplectic patient after the damage has 
occurred, and far too often hears the history of procedures that could 
only have done harm. The belief is very general among the laity that 
the early symptoms of a cerebral lesion, drowsiness, tingling or weakness 
in the hand or leg, etc., can best be combated by physical exercise, and 
that to give way to them is to court disaster. Even physicians do not 
seem always to appreciate the importance of absolute rest to a patient 
who shows the evidences of a beginning hemorrhage or other vascular 
lesion. 
If the patient be conscious he should be cautioned to make as little 
voluntary effort as possible, not even to speak, this especially if there are 
evidences of aphasia. No endeavor should be made to arouse an uncon- 
scious patient, and the physician should be careful to make his examina- 
tion with as little disturbance to the patient as possible. The patient 
must not be moved more than is absolutely necessary, and it may be 
wise not even to transfer him to another room. Absolute tranquillity 
of the patient is insisted upon; he should be in a room protected from 
noises and sources of irritation. Only those necessary for the care of 
the patient should be permitted to enter. He should be in bed with 
the head slightly raised, special care being taken that the neck is not 
bent and that nothing interferes with the return of blood from the head. 
When the patient is profoundly unconscious and the breathing is stertor- 
ous, and, indeed, perhaps in general, he had better be placed on his 
1 Bull, de I'Acad, de Med., 1913, No. 36; Presse med., June*6, 1914, p. 429. 
2 Amer. Jour. Med. Sc., 1905, cxxx, 563. 
3 Zentralbl. f. Gynak., xxxvii, 9, 925. 456 
DISEASES OF THE NERVOUS SYSTEM 
side, so that the tongue does not fall back and impede respiration. 
Bowles insists that the apoplectic patient should always be kept in the 
lateral position, the paralyzed side being down. 
The state of the circulation is of utmost importance. If the heart be 
feeble and weak, it must be regulated. Stimulants, such as nitrous ether, 
camphor, caffeine, and small doses of digitalis, are recommended, and 
even alcohol in habitual drinkers. The opposite conditions, a strongly 
acting heart, high blood-pressure, etc., which may indicate bleeding or 
surgical interference, have been considered. 
When a hemorrhage is reasonably certain, active purgation is almost 
universally employed, and even in thrombosis it is the custom, although 
here milder purgation is recommended. Goldscheider speaks strongly 
against this practice, for he doubts whether the good derived from the 
slight lowering of the blood-pressure, even if this occurs, counterbalances 
the danger from the disturbances incident to the procedure, and during 
the first day or two he would simply endeavor to relieve the lower bowel 
by the use of enemata, care being taken not to disturb the patient more 
than is necessary. There is often incontinence of urine but there may 
be retention, and the condition of the bladder must be carefully watched. 
If retention occurs the bladder should be emptied two or three times a 
day by a catheter. 
Ice to the head has probably no influence on the circulation-within the 
skull, and so the common habit of applying an ice cap is harmless, and as 
it appears to be grateful in certain cases it may be used. In hemorrhage, 
agents that increase the coagulability of the blood are indicated and in 
thrombosis those which have the opposite effect may be used (Gowers). 
When the patient is in convulsions or very restless he must be kept 
quiet by the use of bromide, chloral, veronal, and other hypnotic drugs. 
Morphine, on account of its depressing effect on the respiratory centres, 
should be given only when absolutely necessary. 
In all cases when there is a possibility that the lesion is thrombosis due 
to syphilitic disease of the bloodvessels, active measures must be employed 
-salvarsan intravenously, mercury by injections, and later mercurial 
inunctions, and iodide of potassium in increasing doses. 
Directly after an apoplectic stroke the patient should not be disturbed 
by attempts at feeding. The lips may be moistened with water and the 
mouth cleared with a swab of gauze, saturated with some cleansing mouth 
wash. If the patient remains unconscious for a long time and appears 
to be becoming weak for lack of nourishment, nutrient enemata may be 
given. If these are not retained, infusions of salt solution may be given. 
When the patient is able to swallow (and this ability should be carefully 
determined by the physician) liquids in small quantities may be given 
after the first few hours. Even in the mildest cases the diet must be 
very light for the first few days, and then increased very gradually. 
In slight attacks it is always difficult to keep the patient quiet, but 
even in the mildest cases the physician must insist upon a rest in bed for 
at least a week, and it would be safer for two. 
Sequels.-Desfruction of any part of the brain entails some loss of 
function, and when the focus is large, or so situated as to interfere with DISEASES OF THE CEREBRAL BLOODVESSELS 
457 
an easily recognized function, we have symptoms. Such focal symptoms 
commonly follow vascular lesions within the brain, and not infrequently 
make up most of the clinical picture, and instead of apoplexy we have 
palsy, as the older writers would say. The symptoms vary with the 
position of the focus, and a knowledge of cerebral localization is necessary 
for a clear understanding of this relationship. Local symptoms follow 
hemorrhage more frequently than softening within the brain. This is 
due to the fact that hemorrhage usually occurs in the region of the 
internal capsule where it causes destruction of important tracts, whereas 
softening may be anywhere, of small extent involving a silent area. 
In most cases of apoplexy from all causes, however, focal symptoms 
are present. Such may be the first sign or evident only when conscious- 
ness begins to return. These symptoms may be so transient that they 
can hardly depend upon a destruction of tissue, but must represent a 
loss of function due to some passing state-anemia or oedema. 
After an attack of apoplexy the patient may show little or no intel- 
lectual weakness, but usually a careful inquiry will reveal a distinct lower- 
ing of mental vigor. The patient is not the man he was before the stroke, 
but tires more easily, is petulant, emotional, and often depressed. He is 
often unable to control the expression of his emotions, and weeps tumul- 
tuously upon the slightest cause, or even when he may not be conscious 
of any sad feeling. Less commonly his laughter may be uncontrolled. 
Similar symptoms due to arteriosclerosis are often present in a less 
degree before the stroke, which appears to have merely intensified them 
and to have reduced the patient to a lower intellectual level. When the 
lesion is a large one, or associated with widespread arterial disease, the 
defect may amount to almost complete dementia. On the other hand, 
especially in young subjects, a severe stroke may be survived with 
little effect on the mental functions. Pasteur, who for days was thought 
to be dying from an apoplexy when forty-six years old, subsequently did 
much of his brilliant work, and lived to be seventy-three. 
If the lesion be so situated that it involves the speech area, aphasia 
follows and may be permanent. This is often associated with a marked 
lowering of the intellect, but at times this is not evident. 
The physical defect that most often remains is hemiplegia, which results 
whenever the pyramidal tract is implicated, as in the common lesion of 
the internal capsule, or in any other part of its course. In severe cases 
the paralysis is widespread, affecting generally all of the movements of 
the opposite side of the body. The arm and leg are motionless, and 
often the facial muscles are powerless, and the common statement that 
one side of the body is completely paralyzed seem justified, A closer 
examination shows that this statement is far too general, and that there 
are many movements on the affected side which are little, if at all, 
implicated, and that those affected are so to an unequal extent. This 
attracted the attention of the older observers, and various hypotheses 
were advanced to explain it. The most widely accepted one was that 
which Broadbent1 advanced in 1866, when he pointed out that the 
1 Brit, and For. Med.-Chir. Bev., reprinted in Selections from the Writings, Medical 
and Neurological, of Sir Wm. Broadbent, London, 1908. 458 
DISEASES OF THE NERVOUS SYSTEM 
muscles which escaped were those which acted more or less constantly 
in association with corresponding muscles of the opposite side of the 
body, i. e., bilaterally acting muscles, and that the degree of paralysis, 
when present, corresponded to the degree of their unilateral activity. 
This he explained by conceiving a more or less intimate association 
between the lower spinal nuclei. When muscles acted always together 
the connection was so close as to practically make one nucleus, which 
could be stimulated equally well from the right or left cerebrum, i. e., 
its movements, composed of both right- and left-sided muscles, were 
represented on both sides of the brain, and a lesion on one side of the 
brain would not abolish them. When muscles, although usually acting 
together, could act unilaterally, the connection between the lower nuclei 
was less intimate, and their bilateral cerebral representation less com- 
plete, so that a unilateral lesion would paralyze them more or less, 
depending upon the degree of their unilateral activity. As constant 
bilaterally acting muscles, Broadbent considered those of the eye, back, 
neck, and chest. He regarded the upper facial muscles as being largely, 
but not completely, bilaterally acting muscles; the lower facial muscles 
he thought were almost entirely unilaterally acting. The muscles of 
mastication and of the tongue he also classed among those which act 
both symmetrically and unilaterally. Subsequent investigations have 
confirmed Broadbent's observations and his explanation is generally 
accepted as fundamentally true. 
That movements and not individual muscles are represented in the 
brain (Hughlings Jackson) and that it is these which are affected in hemi- 
plegia, is nowhere better seen than in relation to the external muscles of 
the eye. These muscles are never individually paralyzed, but the conju- 
gate movements to the right or to the left are not infrequently affected, 
particularly in the early stage of a hemiplegia. In each of these move- 
ments muscles on the tw'o sides of the body are involved. The rapid 
recovery of these movements cannot easily be explained on Broadbent's 
hypothesis, for although using muscles on the two sides of the body, the 
movements are themselves unilateral. It may be that these movements 
are represented in more than one part of the cortex, anteriorly in the 
motor cortex and posteriorly in the occipital lobe, and therefore one 
lesion would not be likely to destroy the pyramidal tract and the path 
from the occipital lobe. 
The condition of the muscles of the eye in hemiplegia has been care- 
fully reviewed. Mirallie and Desclaux tested the strength of the muscles 
by their power to overcome prisms, and came to the conclusion that in 
every case of organic hemiplegia, in which the face was the least involved, 
there was a weakness of all the muscles of the eye on that side, and also 
to a less extent on the sound side. S. A. K. Wilson,1 working with Marie's 
material at the Bicetre, and employing the same methods, came to 
exactly the opposite conclusion, i. e., he was unable to determine any 
weakness of the external muscles of the eye in cases of hemiplegia beyond 
that which was often found in healthy individuals. 
1 Rev. Neurol, and Psychiat., 1904, p. 265. DISEASES OF THE CEREBRAL BLOODVESSELS 
459 
Ptosis due to weakness of the levator palpebrse superior is at times 
observed with lesions of the cerebral hemisphere, and the involvement of 
this muscle in hemiplegia is cited as an exception to the general exemp- 
tion of the muscles supplied by the third nerve. Ptosis is, however, 
rare from a lesion within the cerebral hemisphere, and Wilbrand and 
Saenger, in their careful analysis of the literature, were able to find the 
records of very few satisfactory cases. When the lesion is in the brain 
stem and can directly implicate the nuclei of the ocular muscles, their 
paralysis is, of course, a common symptom. 
The movements of mastication on the paralyzed side are more or less 
weak. Mirallie and Gendron found that the weakness was particularly 
of the masseters and pterygoids, and more marked the more recent the 
case of hemiplegia. 
Although Broadbent recognized that all the facial muscles were 
implicated in varying degrees in the paralysis of hemiplegia, those 
about the eye being less affected, later observers applied his hypothesis 
more strictly, and the statement that the upper facial muscles were 
completely spared in a lesion of the brain, received general acceptation. 
A review of the question by a number of writers has confirmed Broad- 
bent's original statement in a remarkable way. In early cases, when 
the face is at all implicated, it is usually so in its entirety. The move- 
ments of the upper facial muscles, in a great majority of cases, recover 
rapidly, and after a few days their weakness is revealed only by careful 
examination. It may be noticed that the eyebrow on the paralyzed side 
is a little lower than on the other, that the movements of the forehead, 
although present, are weaker than those on the other side, and that 
the eye cannot be closed so quickly and firmly. The patient is unable to 
close voluntarily the eye on the paralyzed side without at the same time 
closing the opposite eye, even when he may have been able to do so before 
the stroke, and this may be the only remaining evidence of the weakness 
of this group of muscles (the orbicularis sign). The paralysis of the 
muscles about the mouth (the lower facial group) is much more per- 
manent, and in some cases a slight drooping of the corner of the mouth 
during voluntary effort, may be the last evidence of a hemiplegia. 
Although this relation between the degree of paralysis of the upper and 
lower facial muscles holds good for the majority of cases, exceptional 
cases have been reported in which the condition is the reverse, the upper 
group being more paralyzed than the lower. 
The movements of the larynx are as typically bilateral as any in the 
body. Horsley and Beevor found that stimulation of the cortex or the 
internal capsule of either hemisphere caused movements of both vocal 
cords. In hemiplegia these movements are not permanently affected, 
although some involvement of them probably takes place in the difficulty 
in speech so common in the first few days after an apoplexy. 
The movements of the soft palate are rarely referred to in detail in 
relation to hemiplegias, but Tetzner1 studied this question and finds that 
the condition varies greatly. In certain cases there is complete paralysis, 
1 Neurol. Centralbl., 1909, p. 520. 460 
DISEASES OF THE NERVOUS SYSTEM 
and in others the palate moves only during gagging and then usually 
symmetrically. Tn a number of cases, fifty-two, the palate moved 
during phonation, and in those he found the movements symmetrical, 
the soft palate in front and above the anterior arch being drawn back- 
ward and toward the paralyzed side, making the picture of a tent, whose 
point was directed toward the paralyzed side. In a few cases this 
movement of the palate was the only remaining sign of the paralysis. 
The tongue when protruded not infrequently deviates toward the 
paralyzed side. This is usually explained by the statement that the 
muscles of that side, being weak, are overbalanced by their opponents, 
and when the tongue is protruded it is pushed over toward the weak 
side. Beevor1 showed that the condition is not so simple, and pointed 
out that stimulation of the cortex reveals two separate centres for the 
movements of the tongue, one in the upper part of the tongue area, 
which causes deflection of the tongue toward the opposite side, and 
another situated lower and more anterior, the stimulation of which 
causes protrusion of the tongue in the mid-line. This latter affects equally 
the movements on the two sides of the tongue. It is some disturbance 
of this movement, protrusion of the tongue in the median line, that 
causes the deflection of the tongue toward the hemiplegic side, and not 
the voluntary movement of turning the tongue toward that side. This 
latter is a unilateral movement, and Beevor showed that it is affected 
in hemiplegia, the patient having more difficulty in putting the tongue 
into the cheek on the paralyzed side than in moving it in the opposite 
direction. 
The explanation of the deflection of the tongue when the patient 
endeavors to protrude it straight is not clear, but Beevor advanced 
the tentative theory that although each hemisphere contains the move- 
ments for both sides, still for a perfectly balanced movement the action 
of both hemispheres is required, and a lesion in one causes a greater 
amount of weakness in the movements of the opposite side. This 
amounts to an elaboration of the common statement.2 
The movements of the mouth, pharynx, larynx, tongue, etc., are 
used in many most important complex functions, deglutition and speech, 
for instance. A single lesion above the medulla does not often per- 
manently disturb the power of swallowing, nor, unless in the speech 
sphere, abolish the movements underlying speech, although in rare 
instances such permanent effects occur and result in a typical pseudo- 
bulbar paralysis. Panski3 refers to such cases as well as to some other 
unusual symptoms in hemiplegia. Pseudo-bulbar paralysis usually 
follows two apoplectic strokes, first on one side and then on the other, 
implicating the fibres from both cortices to the bulbar nuclei. The 
lesions are most often in the internal capsule. 
The movements of respiration are bilateral, and so little affected in 
hemiplegia that in the records of the routine examination they are very 
1 Brain, 1906, xxix, 487. 
2 The movements of the tongue following stimulation of the twelfth nucleus, root 
and nerve, are the subject of a study by Mussen, Brain, 1909, xxxii, 206. 
3 Deutsch. Zeitschr.f. Nervenh., 1914, li, 1. DISEASES OF THE CEREBRAL BLOODVESSELS 
461 
generally noted as being equal. In a number of cases where special 
attention has been called to this point no difference in expansion of the 
two sides of the chest could be determined. At times, however, there 
is a marked difference, the movements on the paralyzed side being less 
than those of the opposite side. Hughlings Jackson1 thought that the 
condition differed in quiet and forced breathing; during quiet breath- 
ing the movements on the paralyzed side being greater, while in volun- 
tary or forced breathing they were less than those on the opposite side. 
This was confirmed by a number of investigators, but West2 found no 
such difference. In his carefully studied case the movements were less 
on the paralyzed side during every character of respiration, and the 
chest on that side actually bulged during the strong effort of coughing. 
The muscles of the trunk act both bilaterally, as in forward and back- 
ward flexion of the body, and also unilaterally, in the movements toward 
the right and left. These movements are affected differently in hemi- 
plegia, those acting bilaterally being but little affected, the muscles on 
the paralyzed side acting nearly as strongly, although somewhat later 
than their fellows. The lateral movements may be quite differently 
affected. Beevor3 reported a case of left hemiplegia which again shows 
that movements and not muscles are represented in the cerebrum. 
When sitting, the patient could not lean to the left with as much force 
as to the right, although he showed a tendency to fall toward the left, 
i. e., there was a weakness both in the movements of the muscles which 
bend the body to the left (left-sided trunk muscles), and also those which 
keep it from falling in that direction (right-sided trunk muscles); both 
these movements Beevor considered as left-sided movements. These 
same muscles acted strongly in right-sided movements, i. e., the patient 
could bend himself strongly to the right (right trunk muscles) and could 
from that position bring himself with normal power to the upright posi- 
tion (left trunk muscles). 
All movements of both limbs are abolished at first in a severe hemi- 
plegia, but as the patient begins to recover, or from the first, in slight 
cases, the tendency for the arm to be affected more than the leg is 
apparent, and movements return first in the lower limbs. If the lesion 
be sharply localized well back in the internal capsule or involve the leg 
centres in the cortex, the paralysis is, of course, most marked in the 
leg; but the above statement is true for the great majority of hemiplegias, 
and it is a very uncommon case in which there is no return of motion in 
the leg, and generally there is some return of movement in the arm. 
As the movements begin to return there is evidence of a certain selec- 
tion. This has been the subject of a number of investigations, and here 
again we find that it is rarely individual muscles that are paralyzed in 
hemiplegia, but movements which require the action of many muscles. 
Of these muscular mechanisms there are some which are more apt to 
remain paralyzed and others which show a tendency to recover. In the 
leg the groups that are most affected are, in a general way, those which 
advance the leg during walking, i. e., the flexors, abductors, and external 
1 Lancet, 1905, i, 476. 2 Quart. Jour. Med., 1907-1908, i, 448. 
3 Brit. Med. Jour., 1909, 881. 462 
DISEASES OF THE NERVOUS SYSTEM 
rotators of the hip and the flexors of the knee and the dorsal flexors 
of the ankle; whereas the opposite group of muscles is little affected and 
recovers first. In the arm the condition seems to be more complicated. 
The movement which opposes the thumb to the little finger is most apt 
to remain paralyzed. Opening of the hand and outward rotation cf the 
forearm are more affected than closing the hand and inward rotation. 
Extension of the elbow is more affected than flexion. The movements 
forward and backward of the upper arm and all the movements of the 
shoulder girdle are usually lost. The disability is not always proportionate 
to the return of muscular strength, and at times one finds an arm in 
which there is little or no decrease in the force of the individual move- 
ments, but which is practically useless for all its finer activities. 
It is apparent that the varying degrees of paralysis following a cerebral 
lesion cannot be entirely explained by Broadbent's simple hypothesis of 
the close association of the lower centres for bilaterally acting muscles. 
The condition is much more complicated; the association may be between 
the cerebral centres themselves, as seems to be the case for the trunk 
muscles, which, although unilaterally represented in the cortex, as shown 
by simulation experiments, act symmetrically and are little paralyzed 
from a unilateral lesion. Forster1 believes that the selective paralysis of 
the limbs is due to an innervation of the retained muscles by auxiliary 
fibres which arise from the homolateral motor cortex and run as direct 
pyramidial fibres both in the anterior and lateral tracts of the cord. 
Rothmann,2 in a review of the subject finds no proof of the existence of 
such fibres, but believes that the retained movements are due to the 
renewed activities of phylogenetically old, sub-cortical, motor centres- 
chiefly the red nuclei and spinal centres-as modified by the upright 
position in man. A full explanation must wait for a more complete 
knowledge of the physiology of motion. 
The movements on the non-paralyzed side are usually somewhat 
affected, i. e., their force is reduced. Brown-Sequard called attention to 
this in 1882, and Sternberg3 has made a study of the question. Often the 
reduction in the power is not great, but at times it is marked. The force 
with which a movement can be executed on the paralyzed side is generally 
increased, if at the same time the patient makes the corresponding move- 
ment on the opposite side. The effect of simultaneous movements on 
the normal limbs varies but it is never very great. 
Allied to this reinforcement during simultaneous movements of the 
two sides are the associated movements which are often seen in cases of 
hemiplegia. When a strong effort is made with the non-paralyzed side 
it is very usual to see some involuntary movement of the corresponding 
paralyzed limb. During efforts to make a definite localized movement 
on the paralyzed side, as closing the hand, flexing the ankle, etc., the 
whole limb may be brought into play, and, indeed, the other limb on the 
same side may be moved. It is also not very infrequent to see associated 
symmetrical movements of the opposite non-paralyzed side. These 
phenomena are particularly well seen in infantile hemiplegia. Strumpell 
1 Deutsch. Ztschr. f. Nervenh., 1909, xxxvii, 349. 2 Ibid., 1914, 1, 406. 
3 Ibid., 1908, xxxiv, 128. DISEASES OF THE CEREBRAL BLOODVESSELS 
463 
pointed out that if a hemiplegic, or, indeed, any patient in whom the 
pyramidal tract was affected, be placed in a sitting position and asked to 
flex the paralyzed hip while the observer resisted the effort by making 
downward pressure on the knee, there will be a strong involuntary action 
of the tibialis anticus muscle, even though this muscle may be paralyzed 
to voluntary effort (Strumpell's tibialis phenomenon). This is a good 
example of an associated movement, and illustrates the inability of a 
hemiplegic to make isolated movements. During certain involuntary 
or reflex movements the paralyzed limbs may be moved in a surprising 
manner; thus, during yawning the arm may be forcibly raised and drawn 
across the chest. 
Many explanations have been advanced to account for these associated 
movements. They are obviously due to a spread of the impulse beyond 
the centres which control the intended movements. This is believed to 
be due either to a hyperexcitability of the lower centres or to an increase 
in the stimulus, i. e., voluntary effort, the direction of the spread depend- 
ing upon associated paths between these centres. These connections 
may be the remains of primary paths laid down in the early evolutional 
history at a time when all movements were bilateral, and which have 
persisted to a greater or less extent during development (phylogenetically 
old mechanisms). 
In newborn children the tendency to associated movements is very 
great, and becomes less as the facility to make coordinated movements 
is acquired. Later in life, when new, highly specialized movements are 
being learned, writing, dancing, etc., at first many more movements 
are brought into play than are required for the action. During practice 
the unnecessary movements are inhibited, the necessary ones are 
emphasized, and finally, when the movements are perfectly learned, only 
necessary muscles are moved, and only with the required amount of 
energy. The processes underlying this must be very intricate, but an 
essential factor is the cerebral control, acting, for the most part, through 
the pyramidal tracts, and an interruption of one of these tracts, as in 
apoplexy, causes a loss of the finely coordinated movements, and a 
reversion to a more fundamental type. The importance of the sensory 
factor is obvious. 
The muscular tension in the paralyzed limbs is, in the great majority 
of cases, at first very much reduced, but if the lesion is situated in or near 
the cortex, and is irritative, the limbs may at once become rigid, or be 
the seat of recurrent convulsions. This is the early rigidity to which 
Todd called special attention. These irritative phenomena usually pass 
off in a few days, but are at times said to persist and to pass over into 
the state of late rigidity with permanent contractures. 
In practically every case of hemiplegia in which there is a certain degree 
of recovery a condition of muscular rigidity develops in the paralyzed 
limbs (late rigidity of Todd). This begins when there is some evidence 
of returning voluntary power, and at a time when the deep reflexes have 
already become exaggerated. The degree of contracture is not exactly 
proportionate to either of these factors. It is most marked in hemiplegias 
in which the return of voluntary power has been but slight. The limbs 464 
DISEASES OF THE NERVOUS SYSTEM 
become rigid and tend to assume definite positions. The arm is adducted, 
flexed at the elbow, the thumb and fingers being flexed into the palm. 
The leg is straightened, i. e., the contracture is most marked in the 
extensors of the hip and knee and in the plantar flexors of the hip. The 
opposite condition, the arm in extension and the leg in flexion, is very 
exceptional. The muscles most contracted are, in a general way, those 
in which the recovery of voluntary power has been greatest, and this 
relative overstimulation of certain muscles has been given as an explana- 
tion of the contracture. The condition, however, is far more compli- 
cated, and various theories have been advanced to account for it. Here, 
as in associated movements, we have the action of a portion of the central 
nervous system from which an important controlling influence has been 
taken. 
In the contractures of hemiplegia there is a condition of increased 
muscular tension, i. e., of heightened muscular tonus. Muscular tone 
depends on many things, but peripheral sensory impressions are neces- 
sary, i. e., it is essentially a reflex phenomenon. That the cerebral hemi- 
sphere has a modifying influence on these centres is well established, and 
the remarkable effect upon the muscular tension of the withdrawal of 
these influences is beautifully illustrated by Sherrington's "Decerebrate 
Rigidity." In animals whose cerebrum is cut away the muscles show a 
remarkable state of hypertension, being maintained in a flexed position 
against gravity. If, however, in such an animal the posterior roots which 
contain the sensory fibres from a leg be cut, the limb becomes perfectly 
flaccid. This seems quite analogous to the fact that contracture does 
not occur in a hemiplegic with tabetic involvement of the posterior roots 
and gives a physiological basis for Forster's procedure of cutting the 
posterior roots as a treatment of the hemiplegic contractures. 
The positions that the limbs assume in the hemiplegic with late rigidity 
appear to depend, at least in part, upon the positions in which they are 
maintained, and can mechanically, by the application of splints, be 
altered. The attitude of the leg in bed, as determined by gravity, etc., 
is an important factor in its extensor contracture. When the origin and 
insertion of a muscle are, either actively or passively, brought closer 
together or separated, there is normally a reflex tendency to maintain 
the muscle in that position (fixation reflex). Sherrington studied this 
plastic tonus in animals during decerebrate rigidity and under other 
conditions, and gives evidence to show that it depends upon definite 
reflex phenomena. This tendency is much exaggerated when the cere- 
bral control has been withdrawn, so that when a hemiplegic contracts 
a muscle there is a marked tendency for the contraction to be main- 
tained, and this explains why the contracted muscles are, as a rule, 
those muscles in which voluntary power has to some extent returned. 
At first the contracture is entirely active and subsides during sleep and 
under an anesthetic, but after it has been maintained for a certain length 
of time anatomical changes occur in the muscles, so that they become 
permanently shortened and the contracture becomes fixed. 
The walk of a hemiplegic patient is very characteristic, and is con- 
ditioned by the loss of voluntary power and the occurrence of contrac- DISEASES OF THE CEREBRAL BLOODVESSELS 
465 
tures. The paralyzed leg is held rigidly in extension and usually rotated 
inward. As it is advanced the weight of the body is thrown over the 
sound limb and the paralyzed leg is swung forward in a curve, the toe 
often scraping the ground, the movement depending largely upon the 
muscles of the trunk. With every such movement there may be asso- 
ciated movements in the contracted arm. 
Exaggeration of the deep reflexes is an early sign of the abnormal con- 
dition of irritability of the lower subcortical centres which follows inter- 
ruption of the pyramidal fibres, and may occur soon after the apoplectic 
stroke. It is a constant accompaniment of the later hemiplegic state. 
On the paralyzed side the knee- and ankle-jerks are excessive, and 
clonus at both places can usually be obtained. An active abductor 
reflex of the thigh is common, and can be elicited, not only directly, 
but when the blow is struck on the opposite healthy leg, and it often 
accompanies the knee-jerk on that side-crossed adductor reflex. On 
the non-paralyzed side the deep reflexes are usually increased, but not 
to the same degree as those on the hemiplegic side. In the paralyzed 
arm there is an analogous exaggeration, the biceps and triceps reflexes 
are intense, the scapula reflex is marked, and at times a definite clonus 
at the wrist may be brought out. When contractures are extreme they 
may so mask the reflexes that it is difficult to elicit them. 
The skin reflexes vary. Those from the conjunctiva and the abdominal 
wall usually remain absent. The reflexes from the skin of the leg and 
foot may be excessive, any irritation causing a widespread movement of 
the paralyzed leg. This is usually in flexion at the hip, knee, and ankle, 
and the marked dorsal flexion of the great toe appears to be a part of it. 
This latter movement, Babinski reflex and its modifications, is justly 
regarded as a most important evidence of a lesion in the pyramidal 
tract, and is very generally, although not constantly, present in cases 
of hemiplegia. Crocq,1 who has for years studied the question of tonus, 
reflexes, and contractures, has given an interesting summary of his 
present view, and Walshe2 investigated the reflexes clinically in the light 
of Sherrington's findings and showed how clearly they correspond. 
Many voluntary movements of the paralyzed limbs are lost, but even 
those which are retained are, as a rule, performed in an awkward, inexact 
manner. At times, when there is but little loss of actual muscular 
strength, the inability to perform the finer movements is very marked, 
and there may be almost complete loss of function with but a slight 
decrease of muscular strength. Ataxia in its more limited sense occurs 
in those cases with marked and permanent loss of the muscle sense. 
Movements of the affected limb may be accompanied by a definite 
tremor, which at times has the characteristics of that seen in paralysis 
agitans, and at times has more the volitional type, approaching the 
tremor of multiple sclerosis. Not infrequently, and more especially in 
cases early in life, the paralyzed limbs are in more or less constant involun- 
tary motion. These movements are at times confined to the hand and 
foot, and consist in a slow, worm-like twisting and bending of the fingers, 
1 L'Encephale, 1914, ix, 147, 197, 293. 
2 Brain, 1914, xxxvii, 269.- 466 
DISEASES OE THE NERVOUS SYSTEM 
associated with flexion and extension of the wrist, analogous move- 
ments occurring in the foot. Although in a typical case of athetosis, as 
this condition is called, the movements do not involve the whole limb, 
the muscles of which, however, are usually spastic, still not infrequently 
the rest of the limb may be affected by other spontaneous movements 
and may at times be jerked violently about. 
Very similar movements may occur alone, and we then have a condi- 
tion that has been called posthemiplegic chorea. Many authors make no 
distinction between these two conditions, and it is undoubtedly true 
that it is often difficult to decide in just which category a given case 
should be placed. Typical, uncomplicated, posthemiplegic chorea is 
much less frequently seen than athetosis, but when present it has marked, 
distinguishing characteristics. The movements are widespread, affect- 
ing the face, the body, and all the segments of the extremities. They are 
manifold, violent, and have a quick, trembling character. Contractures 
are not common. Both occur more frequently in infantile hemiplegias, 
and usually in those cases in which there has been a considerable return 
of voluntary power. Indeed, the limbs affected may show a little or no 
decrease in power. Various explanations have been given to account 
for the condition. It appears to occur most frequently when the lesion 
is in the neighborhood of the basilar nuclei, particularly of the optic 
thalamus or the subthalamic region, and the most probable explanation 
seems to be that it is some disturbance of the afferent paths, particularly 
those from the cerebellum, which run through the red nucleus,1 and that 
lesions of the motor cortex and pyramidal tracts have but little to do 
with it. 
In hemiplegia following apoplexy the nutrition of the muscles is, as a 
rule, not much affected, and never to the degree that is constantly present 
when the lesion is in the lower motor segment. When the attack has 
occurred in early youth there is generally a considerable retardation in 
the growth of the paralyzed side, and later in life the contrast between its 
size and that of the normal side is striking. This general wasting or 
retardation in the growth of a paralyzed limb is regarded by many as 
depending upon disuse, and is distinguished from the degenerative 
atrophy which depends upon a disturbance of the lower motor neurone. 
Others regard it as a trophic disturbance, and point in confirmation to 
the lack of development of the hair and breast which occasionally occurs 
on the paralyzed side. 
A number of observers have pointed out that it is not very uncommon 
to see such a degree of muscular wasting occurring so quickly, following 
a cerebral lesion, as to suggest the degenerative type of muscular atrophy. 
Indeed, Steinert asserts that some degree of this type of atrophy occurs 
with every hemiplegia. It usually affects the muscles of the arm more 
than those of the leg, and reaches its maximum in a few weeks, after 
which it may recede, the wasted muscle regaining most of its lost volume. 
Associated with the atrophy he found a definite decrease in the elec- 
trical excitability of the nerves and muscles, some slowness of the response, 
1 Gordon Holmes, Brain, 1904, xxvii, 327. DISEASES OF THE CEREBRAL BLOODVESSELS 
467 
and a tendency for the excitability to disappear after repeated stimulation 
(myasthenic reaction). 
The bladder, the disturbance of which is so common during the 
apoplectic stroke, usually regains its function quickly, but in certain 
cases some permanent disability remains. The patient may have great 
difficulty in starting the flow or may be unable to restrain the bladder 
from emptying soon after the desire is felt. 
Loss of sensation is much less common than motor paralysis as a 
permanent effect of a cerebral vascular lesion. In many cases the dis- 
turbance, if present, is so slight that it escapes notice, but it must be 
acknowledged that far too often the examination has been too super- 
ficial to discover the slighter grades of sensory loss. We have the record 
of 70 cases in the Johns Hopkins Hospital in which definite sensory dis- 
turbances were noted, and of 56 cases in which it was stated that there 
was no such loss. It is probable that in the majority of the other cases, 
where the records are silent in this regard, at least rough sensory tests 
were made and no disturbance found. Individual observers who have 
made special inquiry, have found some sensory loss in most cases of 
hemiplegia. Sandberg1 examined 31 cases in Strumpell's clinic and 
in only 10 was he unable to discover any objective sensory disturbance, 
and among these were a number who complained that the impressions 
were not so acute from the paralyzed as from the normal side. 
At times the apoplexy has been due to a focus so situated that the 
sensory loss is the chief, or the only symptom of a local destruction of 
the brain. Such cases are uncommon, and von Stauffenberg2 was able 
to find in the literature only 8 cases of hemianesthesia without motor 
paralysis which were completed by autopsy. He included lesions of all 
kinds. The studies of Head and Holmes3 have done much to clarify 
our knowledge of the subject. The sensory loss from cerebral lesions 
varies with the level of the sensory path involved. Below the optic 
thalamus the primary sense qualities of touch, pain, heat and cold, and 
those underlying postural and spacial discrimination, are subserved by 
the fibres of the fillet, and may all be disturbed by a lesion of this tract. 
In apoplexy such a lesion is usually near the thalamus, and very generally 
this structure is also involved, in which case there will result a marked 
lowering of sensation in some or all of its qualities, "deep" or muscular 
sense being constantly affected, combined with a peculiar, painful res- 
ponse, characteristic of thalamic lesions, i. e., when any sensation is 
aroused it is accompanied by a most disagreeable, painful feeling, and, 
indeed, this may be the only response, the patient being quite unable to 
distinguish the character of the stimulus. The pain may be apparently 
spontaneous, occurring in paroxysms of great severity for many years. 
This is the distressing feature of the "syndrome thalamique" of Dejerine 
and Roussy. 
In the optic thalamus there is a re-grouping of the sense qualities, and 
in it fibres arise to pass to various regions of the cerebral cortex. Lesions 
of the sensory cortex, post-central and parietal convolutions particu- 
1 Deutsch. Ztschr. f. Nervenh., 1906, xxx, 149. 
2 Arch. f. Psychiat., 1909, xlv, 683. 3 Brain, 1911, xxxiv, 102. 468 
DISEASES OF THE NERVOUS SYSTEM 
larly, if large enough, cause sensory loss. The defects are largely of 
judgment, and do not usually involve fundamental sense qualities as 
touch, pain, and temperature, to any marked degree. The recognition 
of the position of a limb or its passive movements (muscular sense) is 
most frequently disturbed. Not infrequently a patient who is conscious 
of the most delicate touch is entirely unable to localize it, or to form an 
idea of the size, shape, weight or structure, of an object placed in his 
hand (astereognosis). 
A lesion may involve the optic tract and the fibres which are leaving 
it, and then there will be combined sensory loss of the cortical type with 
the painful phenomena of a thalamic lesion. 
A sharply localized lesion in the brain stem, as that following an 
occlusion of the posterior inferior cerebellar artery, frequently gives a 
dissociated sensory disturbance of the spinal type, absolute loss of pain 
and temperature, with perfect retention of touch and muscular sense. 
The distribution of the sensory defect varies greatly. It may involve 
the whole side, but the mucous membranes and the skin of the face are 
usually spared, and even when most widespread the degree of loss differs, 
the distal portion of the limbs being more affected, and the hand being 
usually more involved than the leg. The anesthesia may be entirely 
confined to the distal portions of the limbs or of one limb or may be 
found in more or less isolated areas of the affected side. The division 
between normal and affected skin areas is not sharply marked, but one 
passes more or less gradually into the other. It has been shown that the 
areas of sensory loss, following a cerebral lesion, may have the distribu- 
tion of those seen in lesions of the spinal cord.1 This is particularly so 
when the lesion is in the cortex, and Russell and Horsley2 suggest that 
this depends upon a re-representation of the spinal segments in the 
sensory cortex. 
Hemianopsia of the opposite side occurs when the visual path is 
involved in a cerebral lesion, and is a frequent accompaniment of organic 
hemianesthesia. Total blindness of one eye due to optic atrophy, secon- 
dary to occlusion of the thalamic branch of the internal carotid artery, 
is a symptom of thrombosis of this vessel. The blind eye is on the side 
of the lesion, and not of the paralysis. 
Hearing is not much impaired by a unilateral lesion. Impressions 
from each ear go to both sides of the brain, although probably in greater 
intensity to the opposite side. One would expect a certain degree of 
deafness in the opposite ear as compared with its fellow when the audi- 
tory centre or its afferent path is implicated. Such a loss has been 
frequently found from a lesion in the region of one of the posterior 
corpora quadrigemina. 
Vasomotor and Other Sympathetic Effects.-During the apoplexy and 
directly afterward the paralyzed limbs are not infrequently warmer than 
those of the other side, and it is said they may be swollen and oedematous. 
Often the patients sweat profusely, but except in lesions of the brain 
stem there does not seem to be any difference between the two sides. 
1 Goldstein, Neurol. Centralbl., 1909, p. 114. 
2 Brain, 1906, xxix, 137. DISEASES OF THE CEREBRAL BLOODVESSELS 
469 
von Bechterew1 and others have described unilateral abnormalities in 
the secretion of sweat following cerebral lesions, and have shown experi- 
mentally that definite effects in this secretion can be obtained by stimu- 
lating the cerebral cortex. In the chronic stage the paralyzed limbs are, 
as a rule, cooler than their fellows. 
There appears to be no doubt that the cerebrum influences sympathetic 
activities, but little has been proved as to the manner and extent of this 
influence. The most striking case seen by the writer was in an infant 
sixteen months old, in whom there was a most remarkable pallor and 
coldness of the entire left side, which had been paralyzed during an acute 
cerebral attack. The vasoconstrictor symptoms and the paralysis cleared 
together in about six weeks. 
In several cases, in which there was every reason to believe, and proved 
in two by autopsy, that the lesion was a softening of the lateral aspect of 
the medulla, following an occlusion of the posterior inferior cerebellar 
artery, there were marked evidences of paralysis of the sympathetic 
functions on the same side-slight ptosis, a pupil that dilated poorly in 
dim light, and an absence of sweating on that side of the body. 
Bedsores.-In patients who are long in bed, especially when there is lack 
of control of the sphincters, bedsores tend to develop. Charcot believed 
this to be a definite trophic disturbance, but most observers regard it as 
the result of pressure, lack of cleanliness, and disturbed nutrition. 
Treatment of the Hemiplegia.-The management of a patient after he 
has recovered from the cerebral attack must, in the first place, be directed 
against the occurrence of a new attack. These measures have been 
considered under the prophylactic treatment of apoplexy. 
In the treatment of the hemiplegic state itself, the paralyzed limbs 
should be rubbed, moved passively, and the arm supported at the elbow 
in the endeavor to keep the shoulder from sagging, and so prevent the 
pain which is so often referred to this joint. The galvanic current 
applied directly through the joint sometimes is a benefit. Systematic 
massage, passive movement of the joint, and electrical stimulation of 
the muscles appear, at times, to be of use in reestablishing motor power. 
When voluntary power begins to return this should be encouraged by 
practice, and the patient should have regular gymnastic exercises. When 
the tendency to contracture is very pronounced, one should endeavor 
to see that the limbs are stiffened in the most convenient positions, and 
fortunately this is the attitude that is most often assumed-the arm 
in flexion and the leg in extension. The usual methods employed are 
massage, passive movements, and at times the application of splints, 
but other methods have been suggested, and Criegern2 exercises the 
arm after the blood has been driven from it by the use of the Esmarch 
bandage, the limb held above the head. 
In cases in which the contractures in the limbs have become so intense 
as to prohibit their use, as not infrequently occurs in the diplegias of 
infants, Forster3 devised, and Tietze executed, a most interesting surgical 
1 Arch. f. Anat. u. Physiol., 1905, p. 297; Neurol. Centralbl., 1907, p. 187. 
2 Fortschr. d. Med., 1909, xxvii, 2. 
3 Mitteil. aus d. Grenzgeb. d. Med. und Chir., 1909, xx, 493. 470 
DISEASES OF THE NERVOUS SYSTEM 
procedure. This is based on the theory that the spasticity is a reflex 
phenomenon, and depends largely on the afferent impressions which the 
lower centres receive from the limbs, and that if these could be abolished 
the limbs would become flaccid, and if they could be decreased in amount 
the spasticity would be lessened. Forster, considering the overlap in 
the distribution of the posterior roots, each area of the skin being supplied 
by at least three, and each muscle by two and usually three roots, con- 
cludes that two posterior roots supplying a definite muscle group might 
be safely cut if they were not neighboring roots, and that in this way the 
spasticity might be decreased with slight, if any, sensory loss. Stoffel, 
on the other hand, divides some of the fasciculi of the motor nerves going 
to contracted muscles, thus reducing their strength. The subsequent 
reeducational treatment is, however, a most essential part in both of 
these procedures, and must account in great measure for the results. 
Other more usual orthopedic measures of cutting and transplanting the 
tendons are sometimes useful. CHAPTER XIII. 
DISEASES OF THE CEREBRAL NERVES. 
E. W. TAYLOR, M.D. 
Introduction.-The title, "Diseases of the Cerebral Nerves," is some- 
what misleading, first, because of the relatively brief statement necessi- 
tated by space, and secondly, because it is not possible to consider 
affections of the cranial nerves with any degree of completeness, irre- 
spective of the general diseases of which they so often form a part. The 
attempt has been made in this section to consider only those disturb- 
ances which are characteristic of the individual nerves, and not to infringe 
on the domain of the ophthalmologist, otologist, laryngologist, or the 
broad field of the general physician or surgeon. Such inconsistencies 
as may occur are necessitated by the difficulty of classification. It is 
evident that many general diseases are in part manifested by disturbance 
on the part of various cranial nerves. An attempt has been made to allude 
to these frequent associations without entering into undue detail. 
OLFACTORY (FIRST) NERVE AND TRACT. 
Etiology.-Disturbances of smell may arise from lesions in any part of 
the course of the olfactory nerves or tracts from the peripheral distribu- 
tion in the nose to the ultimate terminations in the brain. If, for example, 
the olfactory mucous membrane of the nose is affected through local 
irritation, swelling or any obstruction of the passage leading to this area, 
the sense of smell of necessity suffers, even though the nerve be not 
affected. Local disease may destroy the nerve endings in the olfactory 
area, naturally leading to a greater or less degree of anosmia. Actual 
injury of the olfactory nerves at the base of the brain, where for a short 
distance they lie in close relation to the bone, may produce a like disturb- 
ance. Such a lesion, though probably often overlooked, undoubtedly 
occurs as a result of contusion of the brain, or in extensive fractures of 
the base involving the frontal region. Loss of the sense of smell has been 
observed after an attack of influenza and in senile conditions. A con- 
genital anosmia and a rare hereditary form have also been described. 
Various perversions of smell are not uncommon. A condition of increased 
sensitiveness, hyperosmia, has been described in hysteria, usually asso- 
ciated with altered appreciation of odors. This condition is, no doubt, 
analogous to the extreme acuity of vision occasionally met with in that 
disease. Subjective sensations of smell, parosmia, are, on the whole, rare, 
but undoubtedly occur in disease of the brain, or from irritation of the 
nerve in its extracerebral course. Olfactory hallucinations occur in cer- 
tain forms of insanity, and may possibly be regarded as having some 
localizing significance. As an epileptic aura, also, such perversions of 
471 472 
DISEASES OF THE NERVOUS SYSTEM 
smell may be significant. Gowers speaks of a case, reported by Urben, 
in which parosmia occurred in the course of tabes dorsalis. 
Pathology.-The pathological anatomy of affections of the olfactory 
nerves is closely associated, on the one hand, with local disturbances in the 
nasal cavity, bearing merely a secondary relation to the nervous system, 
and, on the other hand, to traumatism of the cranial walls or the brain. 
Infections, notably meningitis, may include the olfactory apparatus in 
their spread, but usually in such cases other symptoms so far predominate 
that special disturbances of smell are overlooked. It should be remem- 
bered that a possible source of infection of the brain meninges may be 
through the cribriform plate of the ethmoid bone, particularly if it has 
sustained a fracture. Primary disease of the olfactory apparatus, apart 
from the conditions mentioned, is rare. 
Symptoms.-The sense of smell, broadly considered, includes the 
recognition of odors through the anterior nares, stimulating the olfactory 
area, and the recognition of flavors through the posterior nares, also 
through the mediation of the olfactory nerve. Whether or not the fifth 
nerve contains elements which subserve the special sense of smell is open 
to doubt. Inasmuch as only a small part of the mucous membrane of 
the nose, namely, the so-called olfactory area, is supplied by the olfactory 
nerve, and the remainder, which is by much the larger part, by the fifth 
nerve, it is necessary to separate substances used for testing the sense of 
smell into those which stimulate the olfactory nerve and those which 
affect only the fifth nerve. If sensations of smell are blended with those 
of taste proper, such as bitter, sweet, salt, sour, a mixed sensation, termed 
a flavor, is produced. If the sense of smell be lost, the perception of 
flavors is also lost, but in spite of this fact, volatile substances, such as 
ammonia, acetic acid, or mustard, may so stimulate the nasal mucous 
membrane supplied by the fifth nerve that a distinct perception is aroused. 
Lachrymation may thereby be produced, due wholly to the irritation of 
nasal mucous membrane through the mediation of the fifth nerve. This, 
however, is not a true sense of smell. Bearing these facts in mind, tests 
for smell must be made with non-irritating substances, such as pepper- 
mint, oil of cloves, or asafetida, rather than with substances which 
irritate the mucous membrane. 
Diagnosis.-After determining the fact of loss of smell by proper tests, 
it is essential to exclude disease of the mucous membrane, a purulent 
discharge, or other local cause for the defect. The retention of the 
ability to recognize flavors, with a loss of the sense of smell, is very 
frequently due to such lesions, owing no doubt to the fact that the 
expiratory stream is stronger and impinges more directly upon the 
olfactory area than the inspiratory stream. The importance of a local 
examination cannot be overestimated. In the examination, apart from 
the tests to which allusion has already been made, electricity has been 
locally applied. This procedure gives rise to a phosphorus-like odor, but 
the test is painful and at best of doubtful value. In making a routine 
examination account must be taken of the intelligence of the patient, and 
one nostril should always be closed when the other side of the nose is being 
examined. As a further complication it should not be forgotten that 
disease of the fifth nerve may at times cause considerable anosmia, DISEASES OF THE CEREBRAL NERVES 
473 
probably indirectly through the general nutrition of the mucous mem- 
brane, including the olfactory area. 
Prognosis.-The outcome of affections of smell naturally depends 
essentially upon the character, persistence, and possibility of removal of 
the cause. Temporary loss of the sense of smell, due to local irritation of 
the nasal mucous membrane, as, for example, in ordinary rhinitis, is a 
matter of common experience. The function of smell is restored with the 
removal of the irritation. In man the sense of smell may be regarded as 
a luxury rather than a necessity. Hence its permanent loss, especially if 
unassociated with the loss of appreciation of flavors, is often not deeply 
felt, and in some cases no doubt not appreciated by the patient. Apart 
from injuries and infections of the nervous system the prognosis and 
treatment depend on the local conditions within the nasal cavity. 
OPTIC (SECOND) NERVE AND TRACT. 
The optic nerve is to be regarded rather as an outgrowth of the brain 
than as a nerve properly so-called. As in the brain and cord, the axones 
running in the optic nerve are myelinated but devoid of a connective- 
tissue sheath. As in other sensory nerves, the primary neurones originate 
for the most part, in ganglion cells of the retina. Passing backward 
toward the brain a large number of fibres, presumably twice as many 
as uncrossed, cross at the chiasm. This crossing is partial in man, mon- 
keys, and many mammals, and complete in fishes, amphibians, reptiles, 
and birds. A possible explanation for this irregularity of crossing is to be 
found in the fact that those animals whose vision is wholly monocular 
have a complete crossing, whereas those in which binocular vision has 
been established have developed a partial crossing. Behind the chiasm, 
the optic tracts, made up of an external fasciculus uncrossed, a middle 
fasciculus crossed and an internal fasciculus (Gudden's commissure) ex- 
tend to their temporary termination in the lateral geniculate bodies, the 
pulvinars of the thalami and the superior corpora quadrigemina, con- 
stituting neurones of the first order. The inferior corpora quadrigemina 
are not to be regarded as a part of the visual system. The macular 
bundle presumably has bilateral cerebral connections, as shown by the 
fact of the frequent preservation of central vision in hemianopsia. The 
internal fasciculus of the optic tracts, known as Gudden's commissure, 
connects the twro internal geniculate bodies, and is not to be regarded 
as immediately concerned with the visual act, except through possible 
reflex relations. From the dorsal portions of the thalmi the external 
geniculate bodies and the superior corpora quadrigemina fibres pass 
through a secondary neurone system to the cortex of the occipital lobes 
in the region of the cuneus, constituting the so-called optic radiations. 
The visual representation in the cortex is considerably greater on the 
mesial aspect of the hemisphere than externally.1 There is evidence to 
1 Much work has been done on the cortical localization of the visual centres. For 
summaries see Spiller, The Cortical Centres of Vision, p. 8, and Mills, Position and the 
Subdivisions of the Primary Cortical Visual Area, p. 75, in The Eye and Nervous System, 
Posey and Spiller, Lippincott, 1906. The exhaustive treatise by Wilbrand and 
Saenger, Die Neurologie des Auges, Wiesbaden, 1904, should be consulted in all matters 
relating to the ocular nerves. 474 
DISEASES OF THE NERVOUS SYSTEM 
show that certain fibres, originating in the cortex, pass to the retina 
through the superior corpora quadrigemina. A lymph space, known as 
the supravaginal space, presumably of importance in its relation to the 
results of intracranial pressure, surrounds the optic nerve and its sheath. 
The intervaginal space of the nerve communicates directly with the 
intracranial meninges (Schwalbe). 
Fig. 19 
Diagram of the optic system. R., retina; N., optic nerve; C., optic chiasm; G., Gudden's commissure; 
T., optic tract; P., pulvinar of thalamus; E.G., external geniculate body; S.Q., superior quadrigeminum, 
the three ganglia constituting the primary visual centres; O.R., optic radiations; O.C., occipital cortex; 
III, nucleus of third nerve. Note crossing of internal fibres at the chiasm; also that most of the fibres 
originate in the retina, a lesser number in the cortex. 
Lesions of the Retina.-An ophthalmoscopic examination often throws 
light on general diseases, and may be a most important element in 
diagnosis. Such an examination should not be omitted in ordinary 
routine examinations. 
Retinitis.-Alterations of the retina, usually manifested as an inflam- 
mation, occur in a large number of conditions, a few of which only may 
with propriety be considered briefly here. The general characteristics 
of retinitis, as seen by the aid of the ophthalmoscope, are diffuse cloudi- 
ness, particularly of the more central portions of the fundus, due to a loss 
of transparency of the retina and veiling of the choroid. The optic papilla DISEASES OF THE CEREBRAL NERVES 
475 
shows more or less congestion with loss of clearness of outline, leading to 
a somewhat striated appearance. The veins are enlarged, tortuous, and 
engorged. Hemorrhages may occur with exudation, ultimately leading 
to a whitish appearance of the parts involved, through secondary changes. 
The color of hemorrhages, whether bright red, dark, or almost black, 
depends upon their age. If the nerve head or the choroid is involved in 
addition, the condition becomes a neuroretinitis or choroidoretinitis. 
Inasmuch as a retinitis is usually due to a constitutional infection, both 
eyes are commonly involved. The combination of the foregoing signs, 
together with certain characteristic groupings, make possible the recog- 
nition of somewhat definite forms of retinitis. 
(a) Syphilitic Retinitis (or syphilitic choroidoretinitis).-Syphilis 
manifests itself by somewhat varied lesions in the retina. The retinitis 
is usually associated with concurrent disease of the uveal tract, with 
frequent involvement of the choroid. The affection may be divided into 
two main types, one diffuse and the other circumscribed. In the diffuse 
type there is widespread but slight opacity of the retina with the occa- 
sional appearance of small grayish spots. The gradual disappearance of 
this opacity is accompanied by progressive changes in the pigment 
epithelium. The pigment cells occasionally wander into the retinal layers. 
In the circumscribed form, on the other hand, a characteristic feature is 
a yellowish-white exudate, occurring either in the macular region or 
along one of the larger vessels. When this occurs the ophthalmoscope 
will often disclose disease of the vessel wall. In the course of time these 
exudates are replaced by scar tissue, which predisposes to retinal detach- 
ment. Hereditary syphilis may likewise lead to a retinitis which is 
ordinarily observed only in the atrophic stage. There may be either 
small whitish areas, pigment clumps, or the connective-tissue remains of 
former exudates. In late cases vision is affected, and various scotomata 
and defects appear in the visual field. Night blindness is a somewhat con- 
stant feature, and occasionally micropsia (apparent diminution in the 
size of objects) and metamorphopsia (distortion of the outlines of objects) 
may occur. The treatment is of the underlying cause, syphilis. 
(b) Albuminuric Retinitis.-The ophthalmoscope discloses hyperemia 
and swelling of the papilla and of the surrounding retina. Hemorrhages 
may occur in the nerve head and in the nerve-fibre layer of the retina. 
Irregular white patches may often be seen in the retina at a considerable 
distance from the papilla. The macula is not infrequently surrounded 
by a star-like arrangement of white dots, apparently converging toward 
it. An accompanying papillitis may be extreme, even suggesting the 
existence of a brain tumor. The white areas, the result of fatty degen- 
eration, at times become confluent, forming single large white plaques. 
The hemorrhages vary widely both in size and number. An affection of 
the fundus occurs in albuminuric conditions, which is entirely unchar- 
acteristic. For example, simple retinal hemorrhages, retinitis hemor- 
rhagica, or even optic neuritis and choked disk may occur. 
Retinitis may occur in anemia, leukemia, and many other general and 
constitutional diseases which come rather within the province of the 
ophthalmologist than the practitioner of internal medicine. 476 
DISEASES OF THE NERVOUS SYSTEM 
Lesions of the Optic Nerve.-Inflammation, in general limited to the 
optic disk, producing a so-called optic neuritis or papillitis, or, in extreme 
degrees of swelling, choked disk, is important to recognize. Although this 
sign may occur in a great variety of conditions, its usual cause is increased 
intracranial pressure from disease within the cranial cavity. Of such 
causes the most frequent are tumors, the location of which, rather than 
their character, is important in the production of a neuritis. Optic 
neuritis may also occur in other structural diseases of the brain, such as 
meningitis and abscess (rarely according to Bordley and Cushing) idio- 
pathic hydrocephalus, and syphilis. In these conditions, in the absence 
of marked pressure, the mechanism of the production of the neuritis is 
often not easy to determine. Many cases of inflammation of the nerve 
head have been described in influenza, malaria, erysipelas, scarlet fever, 
and other general infections. It has also been observed occasionally in 
neuritis, anemia, and in intoxications from alcohol and lead. Certain 
deformities of the skull, and particularly localized disease of the orbital 
region, more or less directly involving the nerves, may naturally lead to 
their inflammation. 
Mechanism of Papillitis, or Choked Disk.-Various theories, no one of 
which is entirely satisfactory, have been suggested to account for the 
so-called inflammation of the nerve head. Von Graefe held to the view 
that the condition was due to a venous stasis, resulting from an obstruc- 
tion to the return flow of blood from the cavernous sinus (Stauungs- 
papille). On the basis of Schwalbe's work on the communication between 
the sheaths of the optic nerve and meninges, Manz and Schmidt-Rimpler 
concluded that mechanical pressure led to an oedema of the disk. Meehan 
regards as the important elements, oedema, congestion, and inflammation, 
due to a dropsy of the optic nerve lymph space induced by increased 
intracranial pressure. Parinaud considers choking of the nerve head to be 
due to an oedema of the optic nerve itself. A somewhat widely accepted 
theory, of which von Leber is an advocate, bases the optic neuritis upon 
the supposed presence of an irritant in the fluid distending the nerve 
sheath, produced in some way by the presence of a tumor or other source 
of irritation within the cranial cavity. A significant investigation by 
Bordley and Cushing1 tends strongly to support the so-called mechanical 
theories. Of the various terms used to describe the condition of the 
nerve they prefer "choked disk" to designate all grades of the neuroretinal 
oedema. The view expressed is that early injection with stasis of the 
retinal vessels, oedema with elevation of the papilla, and final cellular 
infiltration with new tissue-formation leading to atrophy are stages of the 
same process, due essentially to the distension of the sheath of Schwalbe 
by obstructed cerebrospinal fluid. It will be seen that the toxic element 
plays small part in this conception. The results upon the optic disk of 
decompressive cranial operations lead to the assumption that choked 
disk from practically all causes, including the changes occurring in 
nephritis, are due to mechanical rather than toxic causes. .The evidence 
in this article is strongly in favor of the mechanical theory. It must, 
1 Jour. Am. Med. Assn.. 1909, lii, 353. DISEASES OF THE CEREBRAL NERVES 
477 
however, still be regarded as possible that different causes may lead to 
similar ophthalmoscopic appearances. A true neuritis behind the bulb 
Fig. 20 
Optic neuritis (choked disk). From a case of orbital tumor. Very slight oedema of subvaginal 
lymph space. (Reproduced through the courtesy of Dr. Frederick H. Verhoeff.) 
Fig 21 
(Edema of subvaginal lymph space of optic nerve. With slight swelling of the disk, from a case of 
tuberculous meningitis. (Reproduced through the courtesy of Dr. Frederick H. Verhoeff.) 
has also been observed in certain cases, and an engorgement oedema is a 
somewhat characteristic accompaniment of the typical picture of choked 478 
DISEASES OF THE NERVOUS SYSTEM 
disk. If, on the other hand, a simple inflammation is the predominant 
factor, the elevation of the disk is less marked, and the retina is more 
apt to be involved. A marked swelling of the nerve head is in general 
to be regarded as an indication of pressure behind. The term optic 
neuritis is rather an unfortunate one. Papillitis more nearly describes 
the condition, but both terms are bad if it can be shown that inflamma- 
tion plays no part. The term choked disk is useful as indicating a char- 
acteristic ophthalmoscopic appearance, associated with much swelling, 
but it may properly be given a much wider significance. 
Appearance of the Optic Disk in Papillitis.-A somewhat artificial dis- 
tinction may be made between a first stage of congestion with oedema 
and a second stage of true neuritis or papillitis. The general appearances 
are as follows: The nerve head early shows a deepening of color, with a 
gradual loss of outline. As the process goes on the optic disk swells, with 
a final complete loss of its form, so that ultimately its position can only 
definitely be determined by the convergence of bloodvessels toward its 
centre. The height of the swelling is often several millimeters. The 
appearance of the vessels is perhaps most characteristic. The arteries 
are small, and frequently concealed by swelling; the veins are dark, 
distended, tortuous, and appear divided, owing to infiltration of the 
disk. Small flame-like hemorrhages are frequent, but not an essential 
part of the picture. Vision may, for a long period, be unaffected, and the 
optic neuritis, therefore, go unnoticed. The visual fields show an irregular 
concentric contraction. There is often an increase in the size of the 
normal blind spot and loss of appreciation of red and green. In brain 
tumors, particularly, such a papillitis may become what has been well 
described as a choked disk. A condition known as descending neuritis 
is associated with moderate swelling, and considerable exudation, pro- 
ducing discoloration and opacity of the papilla, but not limited to it. 
The retina may likewise be involved, leading to a neuroretinitis. 
Optic Atrophy.-A degeneration of the optic nerve may occur either as 
a primary process or as secondary or consecutive to a papillitis. The 
general appearances of atrophy of the optic nerve when well marked are 
as follows: Alteration in color, varying from gray to greenish-gray to 
clear white. Slight variations in color are extremely difficult to determine, 
and their significance should not be ventured except by those of wide 
experience in ophthalmoscopy. There is sinking of the centre of the disk, 
often with a characteristic appearance of the lamina cribrosa. The 
margins of the disk are usually distinct, but in secondary atrophy follow- 
ing papillitis they may be veiled for a long period. A broadening of the 
scleral ring indicates a shrinkage of the disk. The arteries, particularly 
in consecutive atrophies, are narrowed, in contrast with the veins which 
often retain their tortuosity. There is, however, finally an atrophy of 
the veins as well, with secondary connective-tissue changes. It is evi- 
dent that the transition from papillitis to atrophy is extremely gradual, 
and that the foregoing appearances are clearly defined only when the 
atrophy has progressed to a considerable degree. 
In contrast to papillitis, vision always suffers in optic atrophy. There 
are various defects in the field and development of central scotomata with DISEASES OF THE CEREBRAL NERVES 
479 
concentric limitation. The loss of the color field is usually greater than 
that of the form field. The pupillary light reflex suffers in proportion to 
the degree of atrophy. 
Primary atrophy of the optic nerve occurs in tabes dorsalis, paralytic 
dementia, and, much less commonly, in multiple sclerosis. In the latter 
disease a pallor of the temporal half of the disk, as pointed out many 
years ago by Uhthoff, is not an uncommon appearance, presumably 
induced by partial lesions of the chiasm. Primary atrophy may likewise 
occur in chronic malaria, diabetes, syphilis, arteriosclerosis, and through 
the toxic action of certain drugs. A curious and inexplicable atrophy of 
the optic nerve on a hereditary basis has been described, usually appear- 
ing between the eighteenth and twenty-third years. A stage of oedema 
and congestion, gray discoloration and pronounced atrophy, may be 
distinguished in this affection. 
Secondary atrophy is usually consecutive to an optic neuritis, but may 
occur through external causes leading to compression of the tracts and 
fibres of the nerve. The pathological appearances of optic nerve atrophy 
are similar to those of the degeneration of fibres in general. 
Diagnosis.-Although an optic neuritis may often be a determining 
point in diagnosis, its occurrence in unexpected conditions and its not 
infrequent failure to develop in well-defined, intracranial disease should 
lead to hesitation in its interpretation. Lesions of the spinal cord, for 
example, may occasionally give rise to a well-defined optic neuritis. 
Weisenburg and Thorington1 reported a definite optic neuritis in a case of 
syringomyelia, and the writer has observed the development of a neuritis, 
rapidly leading to blindness, in a case of primary tumor of the lower por- 
tion of the cord extending upward.2 In both these instances the optic 
neuritis is, no doubt, to be attributed to a blocking back of cerebrospinal 
fluid leading to hydrocephalus. Optic neuritis occurring in the course 
of a myelitis has been described, and presumably is to be attributed to 
the action of the toxic agent leading to the cord disease.3 On the other 
hand, cases of brain tumor are in a considerable proportion of cases 
unaccompanied by optic neuritis, and especially in those in which pres- 
sure is not a marked symptom. The occurrence of optic neuritis in the 
course of intracranial syphilis is not infrequent, particularly in those 
cases in which the symptoms develop in acute fashion. Cases might easily 
be multiplied to illustrate the uncertainty of optic neuritis in various 
serious intracranial conditions. The fact remains that the ease of its 
demonstration, and its definite significance when present make a study 
of the eye-grounds in all cases a matter of the utmost importance. 
Course and Prognosis.-The course and outcome of affections of the 
optic disk are wholly dependent upon the character of the cause, whether 
it acts temporarily or permanently and whether it is capable of removal. 
Retrobulbar Neuritis.-An acute form of retrobulbar neuritis has been 
described, among other causes, in influenza, multiple sclerosis, and poison- 
ing by wood alcohol. In this condition there is rapid loss of sight in one 
1 Am. Jour. Med. Sc., 1905, cxxx, 1019. 
2 Taylor and Collier, Brain, 1901, xxiv, 532, discussed this in detail. 
3 Kernhensteiner, Munch, med. Woch., 1906, liii, 802. 480 
DISEASES OF THE NERVOUS SYSTEM 
eye, occasionally in both, with the development of a central scotoma and 
pain in the orbit. At first there are no changes in the optic nerve; later 
an optic neuritis may appear passing into atrophy, or the atrophy may 
apparently be primary. Complete blindness is rare, but a defective vision 
may remain. A chronic form of retrobulbar neuritis is more frequent. 
This also is rapid in onset; objects are veiled; the acuteness of vision 
is reduced; there is loss of color sense, with the development of a central 
scotoma, particularly for colors, usually oval in form. In extreme cases 
a central amblyopia may develop with complete loss of central vision. 
This is usually binocular, and often unobserved by the patient. Recovery 
is partial or complete. The special causes are excess in alcohol, tobacco, 
or both, hence it is more frequent in men than in women. The ophthalmo- 
scopic appearances are hyperemia of the papilla, loss of outline, with the 
development of white stria1 along the vessels. Following this temporary 
phase there is apt to be a pallor of the temporal side of the disk, the 
nasal portion remaining normal. Rarely the whole papilla undergoes a 
so-called white atrophy. The essential treatment is total abstinence 
from alcohol, tobacco, or other causal agents. 
Amblyopia and Amaurosis without Ophthalmoscopic Changes.-A great 
variety of conditions affecting sight may occur entirely without objective 
evidence in the fundus. Some of the more important of these are as fol- 
lows: A congenital disturbance of vision for colors, or total color blind- 
ness; an amaurosis due to trauma, uremia, diabetes, malaria, from loss 
of blood, from the abuse of drugs, of which methyl or so-called wood alco- 
hol may be taken as an example; various disturbances of vision in 
hysteria with inversion of color field; monocular diplopia; conjugate 
deviation and general narrowing of the visual fields are the more impor- 
tant. Night blindness (nyctalopia), day blindness (hemeralopia), red 
vision (erythropsia), reduced size of objects (myopsia), and increased 
size of objects (megalopsia) are some of these disturbances of vision. 
Vision.-Apart from the ordinary tests by means of charts, it is often 
useful and essential to diagnosis to determine the degree of eccentric 
vision and its possible limitation. Such an examination may be made 
roughly by means of the hand or any other object brought into the field 
of vision from various directions. Gross defects may be determined by 
carefully observing the point at which the patient first sees the object. 
Hemianopsia may easily be so determined. A somewhat more accurate 
method is to require the patient to look at a definite mark on a blackboard 
while seated at a distance of from one and a half to two feet. An object, 
as, for example, a piece of chalk, brought into the fields from various 
points, when recognized by the patient as a moving object, serves as an 
indication of the extent of the visual field in that direction, and may 
be so indicated on the board. In making such examinations one eye 
should be closed while the other is being examined. Colors are less 
easily seen in the order from without inward, blue, green, red. A peri- 
metric examination is essential for an accurate determination of the 
visual fields. 
Disturbances of Vision.-The general disturbances are: (1) Concentric 
narrowing of the fields; (2) scotomata, or defects of vision elsewhere than DISEASES OF THE CEREBRAL NERVES 
481 
on the periphery; (3) hemianopsia. Mention has been made of concentric 
narrowing and various scotomata. Hemianopsia is a disturbance of 
more importance for our present purpose, inasmuch as it indicates not 
only disturbance in peripheral vision, but also is of very great importance 
in determining the location of lesions within the cranial cavity. 
Affections of Optic Chiasm and Tracts.-Hemianopsia.-Destructive 
lesions of the optic nerves, ventral to the chiasm, naturally give rise to 
Fig 22 
Diagram of lesions producing hemianopsia. T.F., temporal field; N.F., nasal field; R., retina; N., 
optic nerve; T., optic tract; P.V.C., primary visual centres; O.R., optic radiations; O.C., occipital cortex; 
a.a., lesion producing unilateral blindness; b., lesion producing unilateral hemianopsia; b.b.', lesions 
producing binasal hemianopsia, very rare; O., at centre of chiasm, lesion producing bitemporal hemi- 
anopsia; c.c., d.d., lesions producing homonymous hemianopsia. Hemianopic pupillary reflex in lesions 
anterior to primary visual centres, and behind the chiasm. 
unilateral or bilateral blindness and need no further mention. Owing 
to the partial crossing in the chiasm, however, partial disturbance of 
vision develops when the lesion lies either at that point or in the course 
of the optic tracts to their central terminations, characterized by losses 
of portions of the visual field in each eye. The usual lesion of the chiasm 
occurs in its central portion, leading to disturbance in the nasal portions 
of the retina with consequent affection of vision in the temporal field. 
vol. v-31 482 
DISEASES OF THE NERVOUS SYSTEM 
This condition is known as bitemporal hemianopsia. Naturally, if the 
process in the chiasm extends, total blindness may result, or total blind- 
ness in one eye with retained half vision in the other. The occurrence 
of binasal hemianopsia, through symmetrical lesions limited to the 
outer chiasm bundles, is exceedingly unusual, as well as lesions of the 
chiasm producing inferior and superior hemianopsia. Syphilis, tumors, 
particularly of the hypophysis, and hydrocephalus are the more frequent 
causes of disturbances in the chiasm leading to hemianopsia. 
Affections of the optic tract lead to homonymous bilateral hemianopsia, 
due to the fact that each optic tract contains fibres supplying the temporal 
side of one retina and the nasal side of the other, the fibres to the nasal 
side being crossed, and to the temporal side being uncrossed. The same 
visual disturbance is produced by any lesion either of the primary optic 
centres or of the occipital lobe of one side. With the exception of the 
so-called Wernicke hemianopic pupillary reaction the localizing 
significance of homonymous hemianopsia is relatively slight, apart from 
coincident lesions of neighboring portions of the brain or cranial nerves. 
Affections of the visual area in the occipital cortex, produced by tumor, 
areas of softening or injuries, may lead to isolated hemianopsia. The 
same is true of lesions of the optic radiations, which, however, if affected 
on the left side, usually are accompanied by aphasia. Certain facts of 
importance regarding the location of the lesion producing the hemianopsia 
may be derived from a detailed study of the visual field, particularly 
with reference to the dividing line between the seeing and the blind 
areas. In complete lesions of the optic tract the hemianopsia is usually 
complete, by which is meant that it passes vertically through the fixation 
point. Such a complete hemianopsia is less frequent in disease of the 
external geniculate body, and occurs only exceptionally in affections of 
the cortex. The incompleteness of the hemianopsia in lesions above the 
optic tracts must be regarded as due to the separation of the fibres and 
the probable separate representations of the visual fields in the cerebral 
cortex. The frequent sparing of the macula, for example, may be 
explained on the basis of a separate cortical localization for that group 
of fibres. 
The association of homonymous hemianopsia with concentric narrowing 
of the visual field is, no doubt, often to be explained, as Uhthoff has 
pointed out, by a complication of organic disease with a functional 
disturbance. Affections of the color fields, appearing in hemianopic 
form, have been described, and various minor anomalies of the more 
definite picture which is here presented. Bilateral hemianopsia is 
essentially identical with blindness, except that in several cases central 
vision has not been destroyed, and the light reflex of the pupil is retained, 
provided the lesion lies beyond the reflex arc. 
Hemianopic Pupillary Reaction.-A further possibility of localizing the 
lesion producing hemianopsia is afforded by the so-called hemianopic 
pupillary reaction, to which Wernicke has called attention. If the lesion 
lies within the reflex arc from the retina to the central visual centres, 
illumination of the amblyopic half of the retina will lead to no pupillary 
reaction, owing to the severance of the reflex arc. Such illumination DISEASES OF THE CEREBRAL NERVES 
483 
applied to the seeing half of the retina will produce the ordinary reaction. 
If, however, the lesion lies behind the reflex arc between the optical centres 
and the visual cortex, illumination of the blind side of the retina will 
produce a normal pupillary response. This test is of value, but its 
successful performance demands much care, and the isolated illumination 
of one retinal half is difficult of accomplishment. 
Mind Blindness.-In the consideration of the disturbances of the optic 
nerve the condition of so-called mind blindness demands mention. This 
state is not to be confused with optical aphasia. In this latter condition 
objects are both seen and their significance recognized, but cannot be 
designated in words through the medium of the sense of sight. In such 
cases the proper designation may, at times, be aroused through another 
sense, as, for example, a key may be recognized as a key, but cannot be 
named until it is put into the patient's hand and a demonstration of its 
use requested. 
OCULOMOTOR, TROCHLEAR, ABDUCENS (THIRD, FOURTH, SIXTH) 
NERVES. 
The three nerves, third (oculomotor), fourth (trochlear), and sixth 
(abducens), which control the movements of the eyeball and pupil, may 
be best grouped, although the function of each nerve is distinct and 
demands individual consideration. The relations of the three nerves 
governing the movements of the eyeball are extremely close, which 
naturally is a development of the function of binocular vision. It is also 
of interest and of very great clinical importance to recognize the connec- 
tion between these three motor nerves and the sensory eighth nerve. 
The preservation of equilibrium is undoubtedly dependent upon this 
relationship, with the complex reflex connections of the eighth nerve with 
the cerebellum and other portions of the central nervous system. 
Symptoms of Paralysis of Ocular Nerves.-A complete paralysis of 
all the muscles providing for the movements of the eye gives rise to the 
condition known as total ophthalmoplegia. This may develop partially 
either as an internal opthalmoplegia, in which the pupillary muscles are 
chiefly affected, or as an external ophthalmoplegia, in which those gov- 
erning the globe are alone involved. 
Under normal conditions the external rectus muscle (sixth nerve) 
moves the bulb directly outward. The internal rectus (third nerve) 
moves it directly inward; the superior rectus (third nerve) upward and 
inward, also with rotation of the upper axis slightly inward; the inferior 
rectus (third nerve) draws the globe downward and slightly inward. 
The superior oblique (fourth nerve) moves it downward and outward 
with inward rotation of the upper end of the vertical axis. The inferior 
oblique finally draws it upward and outward, with outward rotation of 
the upper end of the vertical axis. 
A defect of the lateral action of the eyes, normally brought about by 
the internal rectus of one eye and the external rectus of the other, is 
usually easily observed. If the movement of both bulbs to one or the 484 
DISEASES OF THE NERVOUS SYSTEM 
other side is interfered with, the condition is known as conjugate deviation 
or associated paralysis (Blickliihmung). The paralysis of eye muscles 
leads to the following conditions: (1) Loss or limitation of special move- 
ments of the bulb; (2) diplopia; (3) secondary contracture of the antago- 
nists; (4) secondary deviation of the sound eye; (5) false projection of the 
visual field and abnormal position of the head. 
From the general clinical standpoint the development of double 
vision is particularly important as an indication of more or less serious 
intracranial disease. It should be borne in mind that in congenital 
conditions or in persons in whom a strabismus has existed for a long time, 
a diplopia must exist, although often not recognized by the patient. 
This is due to the fact that through 
training and habit the retinal 
image of one eye is ignored, after 
the manner of a person looking 
through a microscope with both 
eyes open. On the other hand, in 
a diplopia coming on suddenly the 
double images are at first annoy- 
ing to the patient, often leading 
to the semivoluntary closure of 
one eye to overcome the confusion 
of a double image. It is well to 
remember this in arriving at a 
conclusion regarding the length of 
time a diplopia has existed. A 
simple examination, asking the 
patient to fix the moving finger 
of the examiner with both eyes, 
is often enough to determine the 
existence of double vision. The 
patient should be directed to state 
the exact moment at which two 
pictures come into the field. It 
is sometimes useful to use a colored 
glass before one eye in order that 
the two images may be sharply 
distinguished. The image seen by 
the sound eye is spoken of as the 
true image, that by the affected eye as the false image. If the false image 
is on the same side as the eye by which it is seen, the diplopia is spoken 
of as homonymous, otherwise it is spoken of as crossed. If, for example, 
the right image disappears with the closure of the right eye, the diplopia 
is homonymous. In general, convergent strabismus is accompanied by 
homonymous, divergent strabismus by crossed diplopia. The position 
of the head is often altered in order to overcome the annoyance produced 
by a diplopia. Such altered position of the head is strikingly seen in cases 
of conjugate deviation, in which it is impossible to bring the eyeballs into 
the median line. The curious condition of so-called monocular diplopia 
Fig. 23 
Paralysis of left sixth nerve. Occurring in the 
course of tabes. (Massachusetts General Hos- 
pital.) DISEASES OF THE CEREBRAL NERVES 
485 
or polyopia is of rare occurrence. It has been observed and adequately 
described in hysteria (Janet, Prince). 
Paralysis of Special Muscles.-External Rectus.-This muscle is fre- 
quently paralyzed through lesion of the sixth nerve at some part of its 
long course from the pons forward. A convergent squint is produced by 
this paralysis, which is increased through secondary contracture of the 
internal rectus. The diplopia is homonymous, with a widening of the 
space between the upright images as the object is moved outward toward 
the side of the paralyzed muscle. 
Internal Rectus.-Isolated paralysis of this muscle gives rise to a defect 
of inward movement with consequent divergent squint. There is a 
crossed visual image in that part of the field corresponding to the un- 
affected eye. The space between the images increases as the object is 
moved toward the side of the sound eye. 
Superior Rectus.-Limitation of upward movements with rotation of 
the bulb, due to the tension of the inferior oblique, is induced by paralysis 
of this muscle. There is a crossed double image in the upper half of 
the field. The upper or false image is oblique and separates from the 
true image on looking upward. 
Inferior Rectus.-Paralysis produces downward limitation. The eye 
is turned outward and also is rotated outward in the effort to look down 
(superior oblique). The diplopia is crossed in the lower half of the field. 
The false image is lower than the true and the distance between them 
increases downward. The images are oblique. 
Inferior Oblique.-Alovements upward and outward are limited. 
On looking upward the bulb is rotated inward. There is homonymous 
diplopia in the upper half of the field. The images are oblique. 
Superior Oblique.-Paralysis of this muscle produces slight disturbance 
of motion. On looking downward there is a slight turning inward of the 
eye, with homonymous diplopia in the lower half of the field and con- 
vergent strabismus. In this paralysis on going down stairs the steps 
often appear to be double. 
A slight exophthalmos is produced by a paralysis of all the external 
muscles of the eye, which, on account of the immobility of the globe, is 
not likely to be confounded with the exophthalmos of Graves' disease, 
hydrocephalus, or postorbital tumors. The paralyses of convergence 
and divergence which occasionally occur have been supposed to be due 
to disturbance in a special cortical centre which presides over these 
associated movements. Such paralyses are apt to be accompanied by ver- 
tigo. Conjugate deviation of the eyes, due either to spasm or paralysis, 
is a sign of considerable diagnostic importance and is strongly indica- 
tive of a lesion within the pons having relation to the dorsal longitudinal 
bundle through which the associated movements of the third and sixth 
nerves are brought about. Spiller1 has discussed this matter in detail, 
and reaches the following conclusions: Persisting paralysis of associated 
lateral movement indicates a lesion of the posterior longitudinal bundle. 
Persistent paralysis of associated upward or downward movement 
1 Jour, of Nerv. and Ment. Dis., 1905, xxxii, 417. 486 
DISEASES OF THE NERVOUS SYSTEM 
indicates a lesion in the vicinity of the oculomotor nucleus. Such par- 
alyses are not the result of a lesion of extracerebral nerve fibres. Cortical 
lesions may produce such paralyses, but they are temporary unless due 
to a bilateral brain lesion. In hysterical states such associated paralyses 
may likewise occur and also as a result of inflammatory lesions or others 
produced by alcohol and syphilis; tumor is a common organic cause, and 
if such associated paralyses persist operation can never be regarded as 
more than palliative. 
Nystagmus is commonly met with in conditions of muscular inequal- 
ity, in multiple sclerosis, and as one of the signs of lesions in the posterior 
fossa, particularly in relation to the eighth nerve. Although the cause 
may be varied, the general explanation must be sought in a loss of coor- 
dination between the various muscles of the eye. Depending upon the 
movement which the eyeball makes, the nystagmus is spoken of as hori- 
zontal, vertical, or, more rarely, rotatory. The movements are very 
much more marked on lateral fixation, and it should be borne in mind 
that under these conditions considerable oscillation of the bulbs must 
be regarded as within normal limits. Nystagmus is often conspicuous 
in the blind, with whom fixation is not possible, and also in persons, 
especially children, with defective vision. 
Intra-ocular Muscles.-The muscles of the iris, associated with alteration 
in the size of the pupil, are the so-called constrictor and dilator pupillte. 
The ciliary muscle, presumably important in bringing about the act of 
accommodation, consists of unstriped longitudinal and circular fibres 
forming a circular band about one-eighth of an inch in breadth between 
the choroid and the iris. The nerve of these muscles is through the nasal 
branch of the ophthalmic division of the third nerve and the short ciliary 
nerve from the ciliary ganglion. The circular fibres of the iris receive 
their innervation from the third nerve and the radiating fibres from the 
sympathetic, hence paralysis of the third nerve leads to dilatation of the 
pupil, and paralysis of the sympathetic to its constriction. Naturally 
the opposite results occur when the lesion of the nerves involved is irrita- 
tive rather than destructive. 
The pupils narrow physiologically under the following conditions: 
Light on the retina; consensual reaction or the phenomenon of pupillary 
constriction of one eye when light is thrown into the other; narrowing 
of the pupil for accommodation for near objects. The path by which 
the reflex action of the pupil is determined is still in dispute. The 
so-called reflex centre has been variously placed in the ciliary ganglion, 
in the oculomotor nucleus, the corpora quadrigemina, the upper portion 
of the spinal cord, and in the oblongata. Experimental and clinical 
studies give no uniformity of result as to the exact location of this reflex 
centre. Spiller is inclined to think that there is a limited area in the 
oblongata near the respiratory centre which is inhibitory in function, 
that cerebral to this there is a subordinate centre, and that the complete 
reflex is, therefore, conditioned by one centre having its location in the 
corpora quadrigemina and another at the spinal end of the fourth ven- 
trical. It is probable that the optic nerve contains special fibres which 
are not identical with those fibres which subserve sight, but which con- DISEASES OF THE CEREBRAL NERVES 
487 
stitute a part of the reflex arc partially crossing in the chiasm. In rare 
instances these fibres have apparently retained their reflex function when 
the optic nerves have been extensively diseased. 
Pathological Variations in the Pupil.-The ordinary pathological 
alterations may be summarized as follows, after Uhthoff, quoted by 
Weeks: (1) Loss of pupillary light reflex with retained convergence 
and accommodation (Argyll-Robertson pupil); (2) loss of convergence 
and accommodation with retained light reaction; (3) loss of pupil reflex 
for light and accommodation; (4) loss of all reflex movements of the 
pupil (ophthalmoplegia interna); (5) loss of direct light reflex in an 
amaurotic eye and consensual light reflex in the fellow eye due to loss of 
vision from a lesion peripheral to the chiasm; (6) loss of sensory or psychi- 
cal reflex action; (7) abnormal miosis with retention of light and conver- 
gent reflexes arising from stimulation of the sphincter or paralysis of the 
dilatator; (8) abnormal dilatation of the pupil (spastic mydriasis) due 
to stimulation of the dilatator or paralysis of the sphincter with retention 
of light and convergent reflexes; (9) difference in size of the pupils (aniso- 
coria); (10) changing anisocoria; (11) irregularity of pupil outline; (12) 
change in the size of the pupil under uniform illumination (hippus); 
(13) paradoxical pupillary reflex or dilatation where contraction would 
ordinarily be expected; (14) hemianopic pupillary reaction. 
Of these conditions several only require special comment here. From 
a practical standpoint the Argyll-Robertson pupil is of very great impor- 
tance. In testing for this reaction it should be remembered that in 
elderly persons a light reaction is naturally slow, that there are many 
transitional conditions between a normal pupillary reflex and an immobile 
pupil, and that an accompanying convergence or accommodative reaction 
must not be mistaken for a light reaction. Although opinions differ, 
syphilis is possibly not always the cause of this peculiar pupillary phe- 
nomenon. It is most often met with in the syphilitic disease, tabes. In 
166 cases Uhthoff found an Argyll-Robertson pupil associated with tabes 
in 67.6 per cent., with dementia paralytica in 8.8 per cent., in syphilis 
without tabes in 8.17 per cent. In other cerebral diseases it occurred 
in only 5.87 per cent., and the remaining very small percentage in other 
disorders of the nervous system. In a statistical summary by Siemerling, 
made on the basis of 1639 cases of Argyll-Robertson pupil, 94.7 per cent, 
occurred in persons suffering from general paralysis, tabes, or syphilis.1 
As previously stated, the location of the lesion producing this sign 
remains in doubt. Its frequent occurrence in tabes had led to the 
supposition that the cord was primarily responsible, but the evidence 
for this is insufficient. Internal ophthalmoplegia, due to the paralysis 
of the ciliary and sphincter muscles, is in many cases presumably depen- 
dent on a nuclear lesion, with syphilis often conspicuous in the etiology. 
Slight differences in the size of the pupils are not to be regarded as 
pathological. If pronounced, however, suspicion should be aroused and 
attention directed toward the possibility of tabes, dementia paralytica, 
tumor of the neck, or tumor of the upper portion of the cord. Irregularity 
1 Bach, Pupillenlehre, Berlin, 1908, p. 144. 488 
DISEASES OF THE NERVOUS SYSTEM 
of pupillary outline, unless due to local disease of the eye, is to be attrib- 
uted in most cases to lesions of the nerves innervating the iris. Such a 
condition of the pupils is often an early sign in tabes and dementia para- 
lytica, and may precede the development of an Argyll-Robertson pupil. 
Special Conditions of Paralysis of Ocular Nerves.-Owing to the 
wide distribution of the third nerve to both the external and internal 
muscles of the eye, its paralysis gives rise to the most striking signs. 
If all branches of this nerve are affected by the lesion, the paralysis induced 
gives rise to external squint, ptosis, dilated and inactive pupil, and 
certain other less conspicuous defects in the movement of the eye from 
involvement of other muscles than the internal rectus. Owing also to 
the wide distribution of the nerve, individual muscles are often the 
seat of paralysis, explained in some instances at least by the separation 
and distinct representation of the individual muscles in the nuclei of 
origin. Such partial palsies are, in fact, more often met with than the 
complete paralysis, and occur notably in tabes, basal gummata, or men- 
ingitis, and in other conditions within the cranium which lead to pressure 
or partial destruction of the nerve. Among the common more or less 
isolated paralyses of muscles supplied by the third nerve, ptosis takes 
the first place. A great variety of conditions may produce this dis- 
turbance. It may, for example, occur as a congenital condition in which 
there is from birth a certain incapacity to open the eye widely through 
weakness of the levator. This affection appears therefore to have a 
hereditary basis.1 Acquired ptosis may arise from traumatism, vascular 
disorders, increase of intracranial pressure, and notably in myasthenia 
gravis and in Gerlier's disease. Ptosis is extremely frequent as part of 
the more general involvement of the third nerve. In relatively young 
persons this sign, often associated with paralyses of other ocular muscles, 
is significant of syphilitic infection. The sixth nerve supplying the 
external rectus is likewise frequently involved in paralytic conditions, 
giving rise to internal squint. An association of polyuria with paralysis 
of the sixth nerve has been described. The causes of its paralysis are, 
however, in general similar to those affecting the third nerve. Paralysis 
of the fourth nerve is often difficult to determine and is of small practical 
significance. It is rarely involved alone. 
Recurrent Oculomotor Palsy.-This extraordinary condition, as its name 
implies, is a recurrent palsy of one third nerve and always the same 
nerve, occurring usually at regular intervals, lasting days, weeks, or 
months, and then disappearing in whole or in part. Children are usually 
affected. The paralysis is accompanied by pain in the head or through 
the eye on the side affected, also by nausea and vomiting, in this respect 
having a certain analogy to migraine. Unlike migraine, the headache 
and vomiting may continue for a week. Often there is headache more 
or less severe at intervals of about a month, at times accompanied by 
oculomotor paralysis. In typical cases the whole supply of the oculo- 
motor nerve is affected; in others, individual muscles. A somewhat 
doubtful distinction has been made between those cases in which there is 
1 A family type of ptosis has been described (E. W. Taylor) occurring in persons 
over fifty, associated with progressive paralysis of deglutition. DISEASES OF THE CEREBRAL NERVES 
489 
an interval entirely free from paralysis and those in which a certain 
amount of paresis persists. Oppenheim, following Charcot's theory, 
Fig. 24 
Fig. 25 
Paralysis of left third nerve. Case of opthal- 
moplegic migraine. (Dr. G. A. Waterman.) 
Same as Fig- 24. Attempt to open eye. 
Showing action of frontalis muscle. (Dr. G. A 
Waterman.) 
Fig. 26 
Double ptosis. An operation to raise the lid has been attempted on the right side. (Massachusetts 
General Hospital.) 490 
DISEASES OF THE NERVOUS SYSTEM 
finds the most probable explanation in a vasomotor disturbance allied 
to ordinary hemicrania. In general, the relations of the condition to 
ordinary ophthalmic migraine1 and to the less frequent ophthalmoplegic 
migraine is a matter of very great interest, a discussion of which is not 
in place here. A similar periodic abducens and trochlear paralysis has 
been described. Some of the cases are apparently progressive, others 
stationary. The prognosis is doubtful, and the treatment uncertain, 
owing to the lack of exact knowledge of the predisposing causes. 
Ophthalmoplegia Interna.-This, on the whole, unusual condition, is due 
to a paralysis of the sphincter pupilke and ciliary muscles, and is usu- 
ally unilateral. Rarely one of these 
muscles may be involved without the 
other, and not infrequently the pro- 
cess may extend to the extrinsic 
muscles of the eye, to involvement of 
other muscles supplied by the third 
nerve, as well as to those supplied 
by the fourth and fifth nerve. The 
lesion is presumably nuclear. Syphi- 
lis is apparently the most frequent 
cause, but such a paralysis has also 
been observed in diphtheria. 
Ophthalmoplegia Externa. - More 
commonly a paralysis of the external 
eye muscles, with or without a co- 
incident disturbance of the intrinsic 
muscles, occurs in the form, if the 
onset is acute, of a so-called superior 
encephalitis, or, if the course is from 
the outset progressive, as chronic 
ophthalmoplegia. 
Superior Encephalitis (Wernicke). 
-In 1881 Wernicke2 described a 
symptom complex which he called 
acute hemorrhagic superior polien- 
cephalitis. Alcohol was regarded as 
playing an important part in the 
etiology in two of the three cases 
originally described, and inflammatory 
changes were found in the neighbor- 
hood of the third nerve as the pre- 
dominant lesion. The essential symptoms were paralysis of ocular 
muscles, of quick onset and rapid progress, leading finally to a practically 
total paralysis of the muscles involved, death resulting in from ten to 
fourteen days, with certain general cerebral disturbances suggestive of 
Fig. 27 
External ophthalmoplegia. Involvement 
of both third nerves and of left sixth nerve. 
All movements of eyeballs imperfect except 
of the right outward. Normal pupillary re- 
actions. (Massachusetts General Hospital.) 
1 Walton has advanced the idea that ordinary migraine is an occupation neurosis, 
involving the visual centres, centre of accommodation, intrinsic and extrinsic muscles 
of the globe and certain muscles outside the orbit, Jour. Am. Med. Assn., 1908, li, 200. 
2 Lehrbuch der Gehirnkrankheiten, 1881, Sec. 47, 229. DISEASES OF THE CEREBRAL NERVES 
491 
alcoholic intoxication. Further observation has shown that Wernicke's 
conception must be widely extended, and that a fairly well-defined 
group of conditions exists, due apparently to infection in the broad sense, 
which are characterized by a great variety of paralyses of ocular muscles, 
both external and internal. The pathological anatomy is not uniform, 
but various nuclear and peripheral degenerations have been found. 
There is increasing evidence to show that the relationship between this 
so-called superior encephalitis, poliomyelitis, and various other acute 
inflammatory conditions of the central nervous system is an exceedingly 
close one, if,, in fact, this whole group of conditions is not identical.1 
Chronic Ophthalmoplegia.-Although this condition is hardly to be 
regarded as a disease sui generis, its striking symptomatology justifies 
its separate consideration. In most cases this usually progressive form 
of paralysis of eye muscles forms part of a more general process, and is 
often associated with bulbopontine degenerations of motor nuclei and 
occasionally with degenerative cord lesions. The symptom-complex, 
originally described by von Graefe, is characterized by a slow develop- 
ment and long course, inasmuch as no centres essential to life are involved 
in its progress. The affection usually begins with involvement of a 
single muscle, often the levator palpebrse, followed by gradual extension 
to the external muscles of both eyes, often sparing the accommodative 
mechanism. The character of the paralysis points strongly toward 
nuclear degeneration as a cause. Finally, the eyeball may become 
entirely immovable, with involvement of the orbicularis oculi. The 
disease is usually progressive and ultimately fatal. The usual lesion 
found postmortem is a chronic nuclear degeneration entirely analogous 
to progressive bulbar palsy and progressive muscular atrophy. 
TRIGEMINAL (FIFTH) NERVE. 
Ophthalmic Branch.-The first or ophthalmic branch supplies the eye- 
ball and lachrymal glands, the conjunctiva, except that of the lower lid, 
the skin of the forehead and scalp up to the vertex, the mesial part of the 
skin of the nose and the mucous membrane of the upper part of the nasal 
cavity. There are also afferent pupil dilating fibres derived from the 
cervical sympathetic. 
Superior Maxillary Branch.-The second, or superior maxillary division, 
is connected with Meckel's ganglion in the sphenomaxillary fossa which 
gives off the vidian nerve, important in relation to the sense of taste. 
The ultimate supply of the nerve is to the skin of the upper lip, side of 
the nose and adjacent part of the cheek, the lower eyelid, and part of the 
temple, also to the conjunctiva of the lower lid, the upper teeth, mucous 
membrane of the upper lip, upper jaw, uvula, tonsil, nasopharynx, the 
middle ear, and lower part of the nasal cavity. 
Inferior Maxillary Division.-The third, or inferior maxillary division, 
so far as it is sensory, supplies the skin of the posterior side of the temple, 
adjacent part of the pinna, anterior and upper wall of the external auditory 
1 E. W. Taylor, Boston Med. and Surg. Jour., 1903, cxlviii, 634. 492 
DISEASES OF THE NERVOUS SYSTEM 
meatus, including the anterior part of the drum, a part of the cheek, 
lower lip and chin, the lower teeth, gums, and tongue as far back as the 
circumvallate papillae, the floor of the mouth, the inner surface of the 
cheek, and salivary glands. The motor portion of the third division 
supplies the muscles of mastication, the masseters, temporals, both 
pterygoids, also the tensor tympani, the mylohyoid, and the anterior 
belly of the digastric. One of the most important anatomical relations 
of the fifth nerve is with the chorda tympani, through which the special 
sense of taste in the anterior two-thirds of the tongue is subserved. 
As Cushing1 pointed out, a valuable means of determining the exact 
distribution and function of the trigeminal nerve in all its branches is 
to be derived from clinical study, particularly through the frequent 
operations on the Gasserian ganglion or more peripheral portions of the 
nerve. Cushing's studies are of the greatest value, and determined with 
much accuracy the fields of anesthesia 
in the distribution of the nerve. 
Special Pathology.-Owing to the 
long course and complicated ramifica- 
tions of the fifth nerve, together with 
the fact that from origin to termina- 
tions it passes through several bony 
canals, it is natural that it should be 
damaged in various injuries or diseases 
of the brain and skull. It is, however, 
shown from Thomas'2 statistics that the 
nerve is infrequently involved in frac- 
ture of the skull. In a series of 69 
cases he found it affected but once, 
whereas the facial nerve, either alone 
or in combination with other nerves, 
was involved in 44 cases. In disease 
of the pons, occasioned by hemorrhage, 
softening, tumor, multiple sclerosis, or 
abscess, the fifth nerve is naturally 
involved. Extensive affection of the 
base of the brain is likewise often a cause of disturbance in the dis- 
tribution of the fifth nerve. In the periphery a true primary neuritis 
of this nerve is exceedingly rare, and a neuritis secondary to disease in 
the neighborhood is also infrequent. Involvement of the nerve is natu- 
rally frequent in traumatism of various sorts, but it is rarely damaged in 
ordinary skull fracture. The pathological anatomy underlying the fre- 
quent neuralgia of the nerve is as yet wholly indefinite. Its association 
with arteriosclerosis, particularly in the neighborhood of the Gasserian 
ganglia, no doubt has some significance, but certainly in many cases is 
an insufficient and incomplete explanation of the pain. The studies 
of Spiller, Barker, Rusk, and Cushing have shown certain alterations in 
removed Gasserian ganglia, but not sufficiently characteristic to permit 
Fig. 28 
Cutaneous distribution of fifth nerve. S.O., 
supra-orbit^l division; S.M.. superior max- 
illary division; M., mandibular division. 
1 Jour. Am. Med. Assn., 1905, xliv, 773. 
2 Ibid., 1908, li, 271. DISEASES OF THE CEREBRAL NERVES 
493 
of their definite association with neuralgia. The situation regarding 
the pathological anatomy of a somewhat frequent herpes occurring in 
the branches of this nerve is more definite. There seems little question 
that an involvement of the ganglion is the direct cause of the eruption 
in this as in other sensory nerves, and that the somewhat fanciful term 
posterior poliomyelitis is not wholly misplaced. Barker's and Cushing's 
experiments, with the Nissl method, showed that distinct groups of cells 
may be involved leading to localized herpes in the same sense that groups 
of muscles are involved in poliomyelitis of the motor type. 
Symptoms.-By all means the most important affection of the fifth 
nerve from the practical standpoint is the so-called tic douloureux, or 
neuralgia in other forms. In the general symptomatology of the affections 
of the nerve primary symptoms of irritation, manifested by pain, should 
be recognized. A more serious involvement of the nerve leads to anes- 
thesia in parts supplied, including certain mucous membranes. In such 
an anesthesia of one nerve, for example, a cup from which one is drinking 
feels as if broken. Food collects within the anesthetic cheek in spite of 
the fact that the motor power may be unimpaired. Taste is impaired 
over the anterior two-thirds of the tongue and somewhat at the back 
of the tongue, but does not remain wholly lost. The sensory loss to 
the facial muscles causes a peculiar awkwardness, due to an abolition, 
partial or complete, of the muscle sense. This disturbance not infre- 
quently, particularly after neurectomies, may closely simulate a Bell's 
palsy. Cushing quotes such an instance in one of his cases of removal 
of the Gasserian ganglion. Such a condition is temporary and is easily 
determined by the persistence of normal electrical reaction. In consider- 
able injury of the nerve the secretion of tears, of nasal mucus and of saliva 
is decreased, resulting in dryness with possible secondary trophic changes. 
Under these conditions the sense of smell may be lost finally through such 
changes in the Schneiderian membrane. The corneal, lachrymal, and 
palatal reflexes are lost, and the tongue lies on the paralyzed side. The 
teeth are anesthetic and tend to drop out. The condition of neuro- 
paralytic ophthalmia, after operation on the fifth nerve, has been regarded 
as a trophic change. This Cushing is inclined to doubt, since he has 
found that a partial neurectomy is more often a cause of this ophthalmia 
than a complete one, and that if the anesthetic eye be properly protected, 
such an ophthalmia does not occur. He attaches, therefore, particular 
importance to external sources of irritation. 
Taste.-A wide difference of opinion exists regarding the course of the 
taste fibres and the part played by the fifth nerve in the transmission of 
gustatory sensations to the brain. There is general agreement that the 
glossopharyngeal nerve supplies the posterior portion of the tongue, 
and that the chorda tympani presumably subserves the same function 
for the anterior two-thirds. A difference of opinion arises as to the 
connection between the chorda tympani and the brain, whether it passes 
by way cf the trigeminus or by the facial or glossopharyngeal route. 
The theory that the fifth nerve is the course over which the taste fibres 
pass has had a large following. According to this theory the course of 
the fibres is through the lingual, the chorda tympani, and the facial to 494 
DISEASES OF THE NERVOUS SYSTEM 
the geniculate ganglion, thence by the great superficial petrosal and 
sphenopalatine ganglion to the second division of the fifth nerve, and so 
to the Gasserian ganglion and the brain, or possibly through the small 
superficial petrosal and otic ganglion to the third division of the fifth 
nerve. Krause and Cushing arrive at somewhat different conclusions 
from their neurological studies of operative cases. This no doubt is 
due to a number of causes, among which, as Cushing points out, the failure 
to make adequate preliminary tests for taste, uncertainty regarding total 
extirpation of the ganglion, faulty methods of making the tests, or, finally, 
a failure to take into consideration the loss of common sensation in the 
part of the tongue under examination, are important. In thirteen cases 
which Cushing reported in 1903, he found that in only one was there a 
failure to reestablish taste perception, and this was found to be due to 
the fact that the chorda tympani had been divided at a previous operation 
and had not reunited. From this evidence it appears somewhat conclu- 
sive that the taste fibres do not, or do not exclusively, enter the skull 
along with the trigeminal nerve. The other alternative is that the fibres 
of the chorda tympani, after passing through the geniculate ganglion, 
reach the brain by way of the pars intermedia along with the facial 
nerve, constituting its sensory portion. The question is further com- 
plicated by the fact that lesions of the facial nerve involving the pars 
intermedia between the geniculate ganglion and the pons usually at 
least do not lead to affections of the sense of taste. The evidence, 
therefore, remains conflicting. The whole subject has been discussed 
by Davies.1 The posterior third of the tongue and palate is presumably 
supplied in great measure through the glossopharyngeal nerve. Even 
from this part of the tongue, however, some of the fibres apparently 
pass to the brain by way of the fifth nerve, since disease of the Gasserian 
ganglion has caused impairment of taste, both in the anterior and posterior 
part of the tongue. The route of these fibres, therefore, has been pre- 
sumed to be from the glossopharyngeal nerve to the fifth, through the 
tympanic plexus (Jacobson's nerve) to the small superficial petrosal, 
and so through the otic ganglion to the third division of the fifth. Cassirer 
reported a case of complete loss of taste from disease of the glossopharyn- 
geal nerve. From this observation the possibility is suggested that the 
glossopharyngeal may be the special nerve of taste. It must, furthermore, 
be borne in mind that individual differences may exist and that the taste 
fibres may conceivably take different courses in different persons. 
Motor Portion.-Paralysis or disturbance of the innervation of the 
motor portion of the nerve naturally occurs only in involvement of the 
third division. In extirpation of the Gasserian ganglion the motor 
division is necessarily destroyed, but without leading to serious conse- 
quences. Paralysis of the muscles of mastication takes place on the 
affected side-masseter, temporal, and pterygoids. Paralysis of the 
mylohyoid and anterior belly of the digastric do not lead to significant 
disturbance of function. Chewing is in general not seriously interfered 
with, owing to the preserved action of the corresponding muscles of the 
1 Brain, 1907, xxx, 219. DISEASES OF THE CEREBRAL NERVES 
495 
other side. The reaction of degeneration occurs in these muscles, but is 
rarely tested. Atrophy later supervenes. The lower jaw can be moved 
only toward the injured side, either on opening or on attempted lateral 
movement. There is also interference with wide opening of the mouth 
(digastric, mylohyoid). The paralysis which occurs in violent trigeminal 
pain is naturally merely apparent and due to pain inhibition. Paralysis 
from central lesion is rare, and the definite location of a cortical centre 
is as yet uncertain. 
Trismus.-Tonic and clonic spasm of the jaw muscles, known as trismus, 
is rather a symptom than a specific affection. In the tonic variety the 
jaws are closely approximated. This occurs as a symptom of tetanus, 
in meningitis, seldom in tetany, as a temporary symptom in the epileptic 
seizure, also in certain diseases of the pons, and possibly as a result of 
irritation of cortical centres. It is also not very uncommon in hysteria, 
and may occur as a reflex from carious teeth or periostitis of the jaw. 
The clonic form of trismus manifests itself in rhythmic movements of 
the jaw, usually in a vertical, less often in a horizontal direction. It 
occurs in general convulsions, hysteria, epilepsy, and paralysis agitans. 
Extensive involvement of the central motor tracts may lead to a jaw 
clonus similar to that often obtained in the ankle. The prognosis of 
trismus is, as a rule, good, provided the underlying source of the affection 
can be alleviated or removed. 
FACIAL (SEVENTH) NERVE. 
Facial Paralysis.-Etiology.-A paralysis of the seventh nerve is 
more frequent than that of any other nerve in the body. In the large 
proportion of cases it is unilateral, and the lesion producing it is usually 
in its peripheral distribution. Among the apparent causes of the ordinary 
type of peripheral facial paralysis exposure takes a prominent place. 
It has been estimated that 70 per cent, of all cases show this etiological 
factor. Due largely to the investigations of Reik1 in this country, 
attention has been called to the relationship between non-suppurative 
otitis media and facial paralysis of the refrigeratory type. In Reik's 
view a middle-ear disturbance always precedes this variety of facial 
paralysis, causing the paralysis through direct inflammation of the 
nerve, or by pressure from exudate. Other suggestions have been made 
and theories of an infectious process, a neuritis, or a primary degeneration 
of the nerve within the Fallopian canal, all have advocates. In this 
connection a pathological report by Andre Thomas,2 in which he was 
able to study three cases, is of interest. The cases were all of peripheral 
paralysis of the seventh nerve, two of them showing hemispasm. In 
each of these cases an examination of the nerve and of the nucleus was 
made. In the first case death resulted in eighteen days and the nerve 
showed a parenchymatous degeneration downward in the aqueduct 
1 Johns Hopkins Hosp. Bull., 1902, xiii, 83; Trans. Am. Otolog. Soc., 1904, viii, 
365; Internal. Clinics, 1909, ii (19th Series), 221. 
2 Rev. Neurolog., 1907, xv, 1273. See also A General Discussion and Bibliography 
on Facial Paralysis, A. Fuchs, Arb. a. d. Neurolog. Inst. a. d. Wien. Univ., Band xvi, 
Part 2, 245. 496 
DISEASES OF THE NERVOUS SYSTEM 
of Fallopius from the first bend, with almost entire loss of the axones. 
Above the geniculate ganglion the axones were less altered. The cells 
Fig. 29 
Complete right peripheral facial paralysis. Following operation for sarcoma of the mastoid region, 
with unavoidable destruction of the seventh nerve. Impairment of taste, anterior portion of the 
tongue on the left. Face at rest, showing slight deformity. (Massachusetts Charitable Eye and Ear 
Infirmary and Massachusetts General Hospital.) 
Fig. 30 
Fig. 31 
Same as Fig. 29; attempt to show teeth. 
Paralysis of zygomatici and other muscles 
concerned in raising the lips. 
Same as Fig. 29; attempt to close eyes. 
Right side of face immovable. 
of the facial nucleus were swollen and showed chromatolysis and eccentric 
nuclei, whereas the cells of the opposite nucleus were normal. In the DISEASES OF THE CEREBRAL NERVES 
497 
second case, following an otitis media, there was total left facial paralysis, 
with complete reaction of degeneration and occasional muscular spasm. 
Examination of this nerve showed chronic perineuritis below the first 
bend of the aqueduct of Fallopius. The nerve showed a neuroma of 
regeneration. The geniculate ganglion was somewhat atrophied, the 
facial nucleus normal. The value of this case is impaired by the pre- 
existing otitis. In the third case there was almost complete right facial 
paralysis with occasional spasm. Examination of the nerve showed 
signs of regeneration. There was swelling of the nerve at the first bend 
of the aqueduct of Fallopius. The nucleus was normal. In all of these 
cases the initial lesion in the aqueduct of Fallopius appeared at the first 
bend of the facial nerve. 
The attempt has been made to hold a neuropathic tendency responsible 
for facial paralysis, but statistics do not support this. There are, how- 
ever, instances in which cases have occurred in one family, and others 
in which recurrences have taken place. An emotional shock has been 
held responsible in occasional cases. Gowers reported the case of a 
woman who underwent a facial paralysis immediately after watching 
the dressing of a breast cancer, and Waterman has made somewhat 
similar observations. Constitutional diseases, such as gout, diabetes, 
leukemia, and syphilis, have all been held responsible. It has also 
occurred during the puerperal state and following diphtheria. It is 
rare as an accompaniment of a general polyneuritis, and if it occurs in 
this or in allied conditions, paralysis is apt to be double-sided, as the 
writer has observed in several cases. A facial paralysis may also, no 
doubt through a nuclear lesion, occasionally occur in poliomyelitis. One 
such case was observed in a child, in whom the only other involvement 
was a slight paralysis of the anterior muscles of one leg. We are on much 
more definite etiological ground in considering the relation of trauma. 
Owing to its long course through the Fallopian canal, and its close prox- 
imity to a cavity which often suppurates, paralysis of a facial nerve from 
carious and suppurative ear disease is extremely common. Basal fract- 
ures likewise show a facial paralysis in a large proportion. 
Naturally disease of the base of the brain, meningitis, new-growths, 
and affections of the medulla and pons, may readily lead to involvement 
of one or both facial nerves. The occasional palsy of a branch of the 
facial nerve supplying the orbicularis oris in cases of bulbar palsy, pre- 
sumably through the degeneration of fibres passing out with the twelfth 
nerve, is of interest. • The observation which Cushing has made, that 
certain of the cases of supposed paralysis of the seventh nerve, following 
extirpation of the Gasserian ganglion, are due rather to loss of muscle 
sense in the fifth nerve than to an actual paralysis of the seventh, is 
likewise worthy of mention. In an analysis of 335 cases of facial par- 
alysis, exclusive of those due to ear disease or trauma, G. A. Waterman1 
arrives at the following conclusions relative to the etiology and course. 
He finds that sex plays no significant part in the incidence; that hereditary 
influence is doubtful; that exposure to cold is the most definite etiological 
1 Jour. Nerv. and Ment. Dis., 1909, xxxvi, 65. 
vol. v-32 498 
DISEASES OF THE NERVOUS SYSTEM 
factor as yet determined; that the affection is no more frequent in winter 
than in summer; that it may occur at any period of life, but is much more 
frequent before forty and during the third and fourth decades. Thirty- 
six per cent, of all these cases occurred between twenty and thirty. 
The disease is more severe, but less frequent in later life. Preliminary 
pain is not prognostic, and secondary contractures are not affected by 
galvanic treatment, as has at times been maintained. 
Onset and Symptoms.-The onset of an ordinary peripheral facial 
paralysis is usually rapid and at times sudden. Naturally the signs are 
produced more slowly if the paralysis is due to a gradually increasing 
disease of the middle ear, or to a progressive tumor growth. Pain is a 
not unusual prodromal symptom, located in the ear, behind the ear, in 
the mastoid region, or in the neck, but is of small diagnostic or prog- 
nostic significance, according to Waterman's statistics. The explanation 
of this preliminary pain is not easy. It may be due either to coincident 
involvement of sensory branches or possibly to a neuritis which may 
be regarded as an accompaniment or cause of facial paralysis. At times 
the facial paralysis is ushered in with fever and signs of infection, especially 
in children. In the absence of a slowly progressive cause, a gradual 
onset of the paralysis is extremely unusual. The signs of facial paralysis 
are naturally conspicuous. The most striking is a loss of movement, 
often complete, of the side of the face affected, more marked in elderly 
persons than in young, no doubt due to the presence of wrinkles in advanc- 
ing years, which when obliterated by paralysis increase the contrast 
between the normal and affected side. The eyelid remains open, to a 
certain extent even in sleep, owing to the paralysis of the orbicularis 
oculi. In an attempt to close the eye the globe is rolled upward, showing 
the sclerotic and often leading to the false idea on the part of the patient 
that the eye is completely closed. The partial closure of the eye often 
observed in the presence of very complete paralysis of other branches 
of the nerve is due to relaxation of the levator rather than to any active 
contraction of the orbicularis. In giving expression to the emotions, 
as in smiling, the mouth is drawn toward the unaffected side through the 
unopposed action of the zygomatici. The tongue appears to be pro- 
truded to one side, due to the fact that the position of the mouth is 
altered by the paralysis. The tongue is in reality not affected. The 
lips cannot be properly apposed, so that whistling is not possible. Drink- 
ing is likewise interfered with, and speech may suffer from the impossibil- 
ity of properly making use of the labial sounds. From the paralysis 
of the buccinator, food often remains between the teeth and in the cheek 
until mechanically removed. The muscles of the external ear are par- 
alyzed, as well as the platysma, so that the lower lip cannot be drawn 
down. The palate is not affected. 
In considering the point at which the nerve is involved, a somewhat 
artificial distinction may be made regarding five positions (Fig. 32): 
1. If the lesion lies below the exit of the nerve from the stylomastoid 
foramen, taste will not be affected, inasmuch as the chorda tympani 
joins the nerve above this point. 
2. If the lesion be between the stylomastoid foramen and the geniculate DISEASES OF THE CEREBRAL NERVES 
499 
ganglion within the Fallopian canal, taste in the anterior two-thirds 
of the tongue is affected through coincident involvement of the chorda 
tympani, which accompanies the facial nerve between these points. 
In addition there is also occasional slight loss of general sensibility, with 
possible subjective taste sensations and changes in the secretion of saliva. 
The tongue may be furred in consequence of the altered salivary secre- 
tions. If the nerve to the stapedius muscle given off from the trunk 
of the facial nerve, between the geniculate ganglion and its exit from the 
Fic. 32 
Diagram of facial nerve. F., facial root; G., geniculate ganglion; G.S.P., great superficial petrosal 
nerve; S., nerve to the stapedius muscle; C.T., chorda tympani nerve; S.F., stylomastoid foramen; 
T.T., taste fibres; T.'T.', possible course of taste fibres through the pars intermedia. The arrows represent 
the probable course of these fibres. Lesions of the nerve from the geniculate ganglion to the point at 
which the chorda tympani is given off lead to disorder of taste, with probable impairment of hearing. 
skull, be involved, hypersensitiveness to muscial notes of low pitch has 
been observed. 
3. If the nerve is affected between the geniculate ganglion and the 
pons, there is in general the same symptomatology as in the ordinary 
peripheral type. There is no involvement of taste, which has been used 
as a somewhat forcible argument against the theory that the pars inter- 
media carries the taste fibres. Deafness is often associated with lesion 
of the facial nerve at this point from the fact that the auditory nerve 500 
DISEASES OF THE NERVOUS SYSTEM 
accompanies it. A deficiency of tear secretion on the affected side has 
been described in many cases, and possibly an involvement of the 
stapedius. 
4. If the lesion is in the pons itself, taste and hearing are unaffected, 
and there are also almost invariably lesions of other nerves, notably 
of the sixth, accompanying the facial paralysis. 
5. If the central course of the facial nerve be involved, the symptoma- 
tology differs from all those previously considered, inasmuch as no changes 
in electrical reactions occur and the upper branch of the nerve is very 
much less involved than the two lower. 
Fig. 33 
Fig. 34 
Left facial paralysis of long standing. Show- 
ing inability to close eye and laxity of muscles, 
especially evident about the mouth. (Massa- 
chusetts General Hospital.) 
Spasm of left orbicularis oculi. Photograph 
shows an attempt to open the eye, which tends 
merely to increase the spasm; girl, aged twelve 
years. (Putnam and Waterman. Studies in Neu- 
rological Diagnosis. Massachusetts General Hos- 
pital.) 
The facial nerve is a peculiarly easy one to examine with the electrical 
current, and the information gained from this source is of the greatest 
possible value in arriving at a diagnosis. The presence or absence of 
electrical changes may be the determining factor as between a lesion 
peripheral to the pons or above the pontine nuclei in the central course 
of the nerve. Altered electrical reactions naturally occur only in lesions 
of the peripheral neurones. In slight degrees of paralysis the electrical 
reactions may be unaffected. In severe cases a complete reaction of 
degeneration may be established in two weeks. Between these two 
extremes forms of partial reactive degeneration may occur, and upon the 
information thus given depends very largely an estimate of the duration 
of the attack. DISEASES OF THE CEREBRAL NERVES 
501 
Diagnosis.-It will be seen that the diagnosis of the approximate 
position of the lesion is usually easy. It is especially important often 
to distinguish between a central disturbance in the nerve and one due 
to a peripheral cause. The essential points in making this differential 
diagnosis are as follows: In the central lesion there is no change in 
electrical reactions; the upper branches of the facial nerve are slightly 
involved, either due to the fact that there are special cortical centres 
for different portions of the nerve, or, more probably, because muscles 
which ordinarily act together are presumably innervated from both 
sides of the brain. The facial paralysis is usually accompanied by a 
homolateral hemiplegia. A crossed or alternating hemiplegia indicates 
a lesion in the pons below the crossing of the central facial fibres and above 
the crossing of the pyramid. The reflex conditions of the nerve are 
theoretically increased, but for practical purposes may be regarded as 
unchanged. In contrast to this series of conditions, lesions of the per- 
ipheral nerve, irrespective of special location, are, in general, as follows: 
Electrical alterations are always present, except in the mildest cases. 
All branches of the nerve are equally involved, and reflex activity is 
diminished, with ultimate muscular atrophy, if recovery does not take 
place. A modification of the foregoing general statement must naturally 
be made in those instances where through trauma a special branch of 
the nerve has suffered. 
Course and Prognosis.-The outcome, as well as the course, varies 
within wide limits. Slight cases may last not more than one or two weeks, 
and give very little physical evidence of their presence, either by inspection 
or on physical examination. Other cases may continue for weeks, months, 
or even become permanent. As already suggested, the electrical con- 
ditions are the most important determining factor in relation to prognosis. 
A complete reaction of degeneration is by no means inconsistent with 
final recovery, but a reaction of degeneration persisting for months, 
and finally giving place to a quantitative diminution of electrical excita- 
bility, must always be regarded as a grave prognostic sign, indicative of 
permanent changes in the nerve, usually no doubt due to a greater or 
less degree of destruction of the pontine nucleus. A somewhat artificial 
distinction may be made between a mild, more severe, and a severe form 
of paralysis, depending upon whether the electrical reactions are normal 
or very slightly affected, with recovery in two or three weeks, or, in the 
second case, whether a partial reaction of degeneration is present, with 
recovery in from four to eight weeks, and finally, in the severe form, 
in which the reaction of degeneration is complete, with a duration of 
paralysis from three to six months. Naturally there are certain cases 
which do not conform to this classification, as, for example, those in 
which the paralysis lasts many months, or those in which the electrical 
reactions do not correspond with the apparent degree of paralytic defect. 
In the ordinary case, after a varying period of complete reaction of 
degeneration, the electrical alterations change for the better in that the 
reaction of degeneration is no longer complete. The cathodal contraction 
increases over the anodal, and the faradic irritability of nerve and muscle 
is gradually reestablished. Along with this improvement in electrical 502 
DISEASES OF THE NERVOUS SYSTEM 
reactions voluntary control of the muscles is gradually regained until 
the face is restored to an essentially normal condition. 
Secondary Contractures.-In giving a prognosis in peripheral facial 
paralysis the possibility of secondary contractures should always be 
borne in mind. Even in the cases of moderate severity a certain amount 
of defect may persist in the movements of the face on the affected side 
and in the tendency of the muscles to overact. Such secondary con- 
tractures usually come on from four to six months after the onset, and 
not infrequently lead to a false conclusion as to the affected side. Inas- 
much as the tendency is toward overaction, the affected side of the 
face shows a somewhat general contracture, the aperture between the 
lids is diminished, and on smiling or other emotional expression the 
affected side of the face is much more actively used than the unaffected. 
A further annoyance is the onset of spontaneous twitching of the facial 
muscles, often with isolated spasmodic contractions, especially of the 
zygomatici. This condition often persists for years, and, in fact, is not 
infrequently permanent, and may well prove to be a very much more 
disturbing condition than the original paralysis. Operation for its relief 
has been attempted with some degree of success. The cause of these 
secondary contractures has been a subject of much discussion, and is not 
yet definitely determined. A favorite theory has been that in any serious 
facial paralysis the nucleus of the pons is of necessity involved, the cells 
suffer and degenerate beyond the point of restitution, thereby interfering 
permanently with adequate innervation. Nerve impulses are conceived 
as spreading from the damaged nucleus in an irregular and somewhat 
uncontrolled manner, so that an excessive degree of a normal phenomenon 
is produced in the contractured parts. 
A lessened resistance between the cells of the nuclei has also been 
suggested. A plausible theory also is that which regards the contractures 
as due to permanent alterations in the muscles of such a character that 
the fibrillar substance is imperfectly reproduced, whereas the sarcoplasm 
maintains the muscle in a state of exaggerated tonus. 
In other than the ordinary form of facial paralysis, which has been 
especially considered, prognosis depends essentially upon the underlying 
cause, and is usually a relatively insignificant matter in view of the more 
serious condition of which it is a sign. In tumor, for example, and 
similar conditions, except for its localizing and, therefore, diagnostic 
value, a lesion of the seventh nerve naturally depends for its outcome 
upon the course of the growth which produced it. Certain cases have 
been described as recurrent, not unlike periodic oculomotor palsy. Sev- 
eral cases have also been described as occurring in one family, suggesting 
some hereditary predisposition. Both of these circumstances are too 
infrequent to alter the general statement that peripheral facial palsy of 
the so-called rheumatic type is a benign affection of good prognosis. 
Treatment.-This consists in various measures of assisting nature in 
what may be regarded as a natural process of cure. Not too much, 
therefore, should be expected of any definite course of medical treatment, 
although as an adjuvant such treatment is unquestionably often useful 
and at times demanded. In view of the fact that a neuritis within the DISEASES OF THE CEREBRAL NERVES 
503 
Fallopian canal may be regarded as a pathological basis of the affection, 
fomentations and blisters may be applied in the mastoid region to serve 
as counter-irritants. If Reik's view be accepted that an otitis media 
always precedes a paralysis of the so-called refrigeratory type, an early 
paracentesis of the drum membrane should lead to rapid cure, and this 
has been the result in cases reported by him. The bowels should be 
kept free, the salicylates may at times be administered with advantage, 
and, if there is reason to regard syphilis as an etiological factor, the iodides, 
salvarsan, and mercury are indicated. The chief reliance in the treatment 
of this paralysis has been the use of electricity, and in spite of its recog- 
nized limitations, this form of treatment remains in popular favor. It is 
doubtful whether the electrical current has any specific effect upon the 
nerve, but it cannot be questioned that the stimulation of muscles incap- 
able of movement through an act of the will is a reasonable and logical 
procedure. Oppenheim believes that in early, and often in older, cases 
striking results may be obtained by the use of electricity. In treatment 
by this means, presuming that a reaction of degeneration is present, 
the galvanic current should be employed, using over the affected muscles 
the pole which produces the more marked contraction. Not more than 
4 to 5 milliamperes should be used, and the muscles should be stroked by 
an electrode for a period not exceeding ten minutes. As the faradic 
irritability returns, this form of electricity may be substituted for the 
constant current, but it is well to discontinue any form of electrical 
treatment when the tendency to contracture and spasm of the muscles 
becomes apparent. There is no adequate evidence to show that the 
application of electricity plays any part in the production of secondary 
contracture, but the treatment becomes more and more superfluous 
as voluntary control over the muscles increases, and may well be discon- 
tinued before such voluntary control becomes complete. 
By surgical intervention intractable cases of facial paralysis may be 
very greatly benefited, if not completely cured. The first method, 
suggested by Ballance in 1895, was the anastomosis of the facial with the 
spinal accessory. In this operation a part or a whole of the spinal 
accessory was sutured into the divided facial, or an implantation of the 
facial nerve made into the split spinal accessory, the object being to 
facilitate the growth downward of spinal accessory fibres in the path of 
the functionless facial. Following Ballance, cases of this operation were 
reported by Faure, Kennedy, Cushing,1 and Hackenbruch. The imme- 
diate result was a more or less permanent paralysis of the trapezius and 
sternomastoid muscles. The facial condition improved, but any sudden 
voluntary movement of the face was accompanied by a distressing 
involuntary movement of the shoulder and vice versa, which proved to 
be a decided disadvantage. To obviate this the hypoglossal nerve was 
selected for the anastomosis. Inasmuch as the hypoglossal nerve is 
closely associated physiologically with the facial, and since the cortical 
localization of the two nerves is near together, it seemed more rational 
to use the hypoglossal than the spinal accessory and a considerable 
number of such operations have been done. 
1 Annals of Surgery, 1903, xxxvii, 641, with historical and bibliographical notes. 504 
DISEASES OF THE NERVOUS SYSTEM 
The importance of operating as soon as possible after injury of the 
facial nerve can hardly be too strongly insisted upon, inasmuch as the 
progressive disintegration of muscle may easily go on to a point where 
recovery is no longer possible, however completely the nerve may regen- 
erate. In carrying out this operation it is desirable not to divide the 
hypoglossal completely, owing to the consequent difficulty of mastication, 
deglutition, and phonation. Indication for the operation is the assurance 
that the facial nerve is divided or so completely injured that spontaneous 
recovery is impossible. Its field, therefore, lies essentially in those cases 
in which the nerve has been divided through trauma, particularly resulting 
from accidental injuries in operating on the ear, and from destructive 
suppurative processes within the middle ear. 
Facial Spasm and Tic Convulsif.-Irrespective of the postparalytic 
contracture and spasm, the facial nerve is peculiarly prone to spasmodic 
affections, due, no doubt in part, to its complex reflex relations. There 
is, in the first place, an exceedingly close relation between the mental 
condition and the muscles of expression, and in the second place the 
reflexes which subserve this relation are naturally highly complex. A 
localized irritation of the nerve may lead to spasm, which later, when 
the cause has been removed, may persist as a fixed habit. The direct 
irritation of the nerve stem, as by a tumor, or the occasional irritation 
from a localized brain lesion, may produce more or less persistent facial 
spasm. In most of the cases no definite anatomical basis is demonstrable. 
Brissaud and especially Meige have distinguished between what they 
regard as true spasm and one of psychogenic origin. Of the latter, are 
those aroused emotionally, which later have become habitual. Such 
are various reflex spasms, the so-called tic convulsif. As distinguished 
from this type the true facial spasm is not dependent upon a mental 
state, but is due rather to material changes in the reflex arc either in the 
sensory trigeminus, facial nucleus, or the facial stem. The first type 
bears close relation to the psychic life, and may be externally influenced 
through diversion of the attention, and by similar means. The second 
type of spasm usually occurs in the whole distribution or in definite 
portions of the nerve, and is usually one-sided, and presumably is not 
influenced by suggestive measures. This type of spasm is that artificially 
induced by an electric current. A true cramp of the facial nerve may 
likewise occur, either tonic in character or alternating. 
Patrick1 made clear the fundamental difference between true facial 
spasm and tic. He states the distinction as follows: "Spasm may be 
said to be an anatomical, tic a physiological, disorder. A good picture 
of facial spasm may be obtained by faradization of the facial nerve. 
Tic is always the replica of a perfectly natural (though may be unusual) 
and physiological movement. Looking at the distortion caused by 
faradization of the nerve no one would mistake it for voluntary contraction 
of the facial muscles. On the other hand, having seen only one individual 
contraction of facial tic, no one could say that the movement was not a 
perfectly normal and natural one-possibly unusual, possibly overdone, 
1 Jour. Nerv. and Ment. Dis., 1909, xxxvi, 1. DISEASES OF THE CEREBRAL NERVES 
505 
but the legitimate result of a casual cause. Voluntary imitation of facial 
spasm is practically impossible. The patient himself can always repeat 
or imitate his tic movements, and another person can nearly always do 
so." In so-called blepharospasm, one of the commonest of the spasmodic 
affections, the subjective disturbance may be extremely slight or wholly 
lacking, especially in its milder forms. 
In so-called tic convulsif there are often signs of other nerve lesions 
and even of a definite underlying hysteria. The course of these affections 
is chronic, the prognosis doubtful but never hopeless. 
Treatment.-This consists in the removal of any reflex cause. Drugs 
are, on the whole, useless, except as palliatives. Nerve stretching is 
possible, but should only be resorted to in very intractable cases. Injec- 
tions of alcohol into the facial nerve near its exit from the skull have been 
used in intractable cases of facial spasm. Patrick reports three such 
cases, with excellent results in two and with a failure in one due to faulty 
technique. The treatment consists in the injection of a 40 to 75 per cent, 
alcohol solution into the trunk of the facial nerve. If the injection is 
successful, an immediate paralysis of the nerve results, naturally with a 
cessation of the spasm. Recovery takes place from the paralysis, and 
there is reason to believe that the spasm will not return even after a 
complete restoration of the normal function of the nerve. 
Occasionally a spasm of the platysma may develop. Such a case has 
recently come under personal observation in which the patient, a man, 
for two or three years has been annoyed by a distinct spasm of the right 
platysma when talking, without apparent involvement of other muscles 
supplied by the facial nerve. 
Herpetic Inflammations of the Geniculate Ganglia.-Under this 
general heading J. Ramsay Hunt1 has brought together a group of cases 
which he regards as constituting a hitherto undescribed clinical entity. 
He regards the facial as a mixed nerve, the nerve of Wrisberg being its 
sensory portion, with the geniculate as its ganglion of origin. It follows 
that herpes may occur in its sensory distribution in common with other 
ganglia of this type, as, for example, the Gasserian ganglion of the fifth 
nerve or the various spinal ganglia. Hunt points out that the zoster 
zone for the geniculate ganglion lies in the interior of the auricle and in 
the external auditory canal. On the clinical side the affection manifests 
itself in the characteristic way, with slight febrile prodromata, neuralgic 
pains in the neighborhood of the ear, followed on the third or fourth day 
by the typical herpetic vesicles, very rarely, according to Hunt, localized 
on the tympanic membrane. This area retains its sensation after extirpa- 
tion of the Gasserian ganglion and the second and third cervical ganglia, 
from which it is assumed that it represents the cutaneous distribution 
of fibres of the geniculate ganglion. 
Herpes zoster of the auricle has long been recognized, but had heretofore 
been regarded as belonging to the trigeminal area, and hence due to 
involvement of the Gasserian ganglion. Hunt's position seems well 
taken that this localized manifestation of herpes may be more properly 
1 Jour. Nerv. and Ment. Dis., 1907, xxxiv, 73; Am. Jour. Med. Sc., 1908, cxxxvi, 226. 506 
DISEASES OF THE NERVOUS SYSTEM 
explained by an involvement of the nerve of Wrisberg and its ganglia. 
The cases of herpes in and about the ear are uncommon. Nevertheless, 
in a very large collection of cases from special hospitals a certain small 
number have been reported for many years. Hunt described three clinical 
types: (1) Uncomplicated auricular herpes; (2) auricular herpes with 
facial palsy; and (3) auricular herpes with facial palsy and auditory 
symptoms. The latter group of cases is perhaps the most obscure and 
difficult of classification. On the basis of 60 personal observations and a 
somewhat limited pathological investigation, Hunt feels justified in 
establishing a characteristic syndrome divided into the three foregoing 
clinical groups. He also makes the statement that neuralgic affections 
of the geniculate ganglion and its divisions productive of an otalgia 
may properly be regarded as due to disorder in the sensory mechanism 
of the facial nerve, giving rise to earache of non-inflammatory origin. 
Anatomically, the geniculate ganglion stands in relation to the interior 
of the auricle and external auditory canal, to the tympanic plexus, to the 
second and third divisions of the fifth nerve, to the glossopharyngeal, 
and to the vagus. It is evident, therefore, that ear pain may be due to a 
variety of reflex causes. Hunt is convinced that, although many of the 
neuralgic affections of the ear are no doubt dependent upon involvement 
of the trigeminal and occipitocervical nerves, there exists also an otalgia 
due entirely to involvement of the sensory branch of the facial nerve. 
Orbison1 takes issue with some of Hunt's conclusions and makes a still 
further division of zoster zones on the basis of a study of herpes of the 
tympanic membrane. He believes that herpes of this area is due not, 
as tacitly assumed by Hunt, to involvement of the zoster zone of the 
seventh nerve, but rather to zoster conditioned by disease of the petrosal 
ganglion of the glossopharyngeal. 
AUDITORY (EIGHTH) NERVE. 
The Cochlear Nerve.-The function of hearing is almost undoubtedly 
subserved wholly by the cochlear division of the auditory nerve. Hearing 
may be affected through lesions anywhere in the course of the nerve, from 
the cortex through the various sensory nuclei to the labyrinth, but such 
disturbance is far more frequent in actual disease of the internal ear and 
its structures and in affections of the terminal branches of the nerve than 
in more centrally localized lesions. 
1. The cortical centre for hearing is presumably localized in the superior 
convolution of the temporal lobe, and unquestionably stands in close 
relation with the cortical area for the perception of language. There is, 
however, still disagreement regarding its exact position. The reason 
that word deafness does not lead to sound deafness is that the auditory 
nerves naturally have relations with both hemispheres, whereas the more 
highly specialized function of the appreciation of language is confined 
wholly to one hemisphere. The observation has been made that when 
both superior temporal convolutions are diseased, sound deafness has 
1 Jour. Nerv. and Ment. Dis., 1908, xxxv, 500. DISEASES OF THE CEREBRAL NERVES 
507 
also resulted. Inasmuch as this latter lesion is extremely unusual, it 
follows that deafness rarely results from cortical lesions. 
2. Lesions of the auditory nerve from the cortex to its subsidiary 
nuclei may also lead, under certain circumstances, to deafness which, 
on the practical side, is indistinguishable from deafness due to laby- 
rinthine disease. Lesions of the corpora quadrigemina and geniculate 
bodies, tumors of the substance of the pons, also certain tabetic conditions 
and multiple sclerosis, may lead to deafness in one or both ears through 
involvement of the auditory mechanism in its course through the brain 
stem. More practically important are lesions in the peripheral course 
of the nerve. Inflammatory processes, notably meningitis, are likely to 
involve the nerve with others at its exit from the pons, often spreading 
along the nerve to the ear itself. A primary neuritis of this nerve and 
subsequent atrophy, also the so-called rheumatic paralysis similar to 
that frequently observed in the facial nerve, are exceedingly rare con- 
ditions, if, in fact, they exist. A condition of importance, to which 
special attention has been drawn, is that of tumor growing in the cere- 
bellopontile angle. Such tumors are not infrequently neurofibromatous 
in character, and may under certain circumstances be removed. They 
are most frequently localized on the eighth nerve, but may also involve 
the trigeminus and vagus. Tumors lying in this region naturally give 
rise to auditory symptoms, which may aid in determining the location. 
Aneurism is at times a cause of deafness through pressure on the 
nerve. 
3. In the great majority of cases the lesion inducing the deafness 
or other disturbances in the function of the auditory nerve lies in the 
internal ear, either as a primary process or resulting from the extension 
of disease from the middle ear. Causes of such disturbances are hemor- 
rhage, inflammations, sclerotic processes often on the basis of acute 
infectious disease, particularly mumps or syphilis, certain constitutional 
diseases, as, for example, nephritis, diabetes, and pernicious anemia. A 
very common cause of labyrinthine disease is epidemic cerebrospinal men- 
ingitis, -which not infrequently results in permanent deafness. Hysteria 
not infrequently shows a definite loss of hearing. 
Symptoms.-These may be classed in two groups-symptoms of 
irritation and symptoms of defect. Irritative symptoms or hyperesthetic 
conditions are unusual, but have been observed in certain abnormal 
functional states of the nervous system, notably in hysteria. In this 
condition sounds may be distinctly appreciated which are not ordinarily 
heard. Such hyperacuteness of hearing may also be observed at the 
onset of certain acute and general diseases, and undoubtedly in these 
instances may be attributed to a general hyperexcitability of the nervous 
system. In certain disorders of the conducting sound mechanism, as, 
for example, in paralysis of the stapedius muscle often occurring in 
conjunction with a general facial paralysis, low notes may be heard 
with unusual distinctness, due presumably to the relaxation of the 
stapedius muscle. The condition of so-called dysacusis, in which sounds 
ordinarily innocuous cause distinct discomfort, should also be mentioned. 
Under these conditions pain, as, for example, headache, is apparently 508 
DISEASES OF THE NERVOUS SYSTEM 
actually increased by noise. Undoubtedly this affection, which is not 
an uncommon one in healthy persons, is due to a temporary general 
lowering of the nervous tone or to disturbance in the middle ear. 
Treatment.-This must depend largely upon the underlying condition. 
If, for example, the stapedius muscle is involved conjointly with the 
facial nerve, the difficulty, which in any event is a trifling one, will pass 
off with the correction of the facial paralysis. The general nervous 
conditions which underlie the abnormal auditory phenomena must be 
treated. The bromides are undoubtedly useful, but much more important 
is a consideration of the general nervous and physical health. 
Tinnitus Aurium.-Under this general heading are included various 
subjective sound sensations referred to the ear, the character of which 
may be roaring, hissing, ringing, ticking, or whistling. This affection 
in its varied forms is extremely common and often most distressing. Its 
causes are manifold. In consideration of the normal sensitiveness of the 
auditory mechanism, it is, in fact, remarkable that subjective sounds 
are not constantly being heard. One reason for this undoubtedly is 
that we come to ignore many sounds, which, if our attention were directed 
to them, would become exceedingly annoying. To city dwellers at any 
rate noise is practically constant, which is, for the most part, ignored. 
More in detail, some of the more important causes of tinnitus are: 
Any morbid process in the ear may cause subjective sounds, but 
especially disease of the labyrinth, which is accompanied by more or 
less nerve deafness. Among these are alterations in circulation due to 
anemia, abnormal pulsation in the carotid artery, intracranial aneurism, 
or possibly vasomotor palsy of the labyrinthine vessels. Osler speaks 
of a bruit in the ear, with systolic intensification, which was supposed, 
for a time, to be due to an aneurism. Cerumen in the external meatus 
and various affections of the middle ear may be operative in producing 
tinnitus. The actual involvement of the nerve endings may also be 
responsible, in which case irritative processes or clonic spasm of the 
internal ear muscles may be operative. Violent stimulation of the 
auditory nerve by loud and particularly by continuous sounds may 
produce a persistent tinnitus. Workmen in boiler factories, foundaries, 
and similar places of employment are not uncommonly so affected. 
Finally, in various general nervous conditions-hysteria, neurasthenic 
states, in the aura of epilepsy, or in migraine-subjective sounds may 
be a prominent feature. A further elaboration of these simple forms of 
tinnitus leads often to very elaborate subjective sounds, which must 
then be assigned to the interposition of higher centres and probably 
are attributable to the action of the cerebral cortex itself. Such are the 
auditory hallucinations not infrequently observed in the insane. In all 
such conditions the relation of the mental attitude to the physical con- 
dition in the ear is naturally of very great interest and importance in 
modifying and keeping alive the condition. It is certainly true in this 
as in other neuroses that a definite cause in the ear leading to simple 
tinnitus may well produce a habit with which ultimately the original 
cause has nothing to do. If the tinnitus is double-sided the prognosis 
is much less favorable than in the unilateral affection. DISEASES OF THE CEREBRAL NERVES 
509 
Diagnosis.-In this very little information is to be gained from the 
character of the sounds. Although it is true that sounds synchronous 
with the pulse are probably directly or indirectly due to arterial pulsation, 
nevertheless such sounds may also be intermittent. It may, in general, 
be said that sounds of arterial origin are more or less continuous, but 
here again it is necessary to be on one's guard, since, for example, cerumen 
has been responsible for a constant tinnitus. The apparent location of 
the sound is also most varied. It may be referred to the ears, be bilateral 
or unilateral, or be referred to the head either at the occiput or vertex. 
The relation to deafness is also extremely vague, since at times the tinnitus 
may decrease with increasing deafness, or may persist unchanged. 
It is evident that a definite diagnosis is often extremely difficult. 
If the sounds are elaborate it is usually safe to assume that they are 
of central origin, or at least that the cortex takes part in the general 
condition. The further question of the exact relation of the labyrinth 
to various subjective sounds must naturally be determined by expert 
otological examination. It is, however, well to note whether continuous 
sounds are increased or diminished by the recumbent posture. If the 
tinnitus is the result of venous hyperemia, it is ordinarily increased by 
lying down, but if due to simple anemia, it is relieved thereby. It should 
also be remembered that certain drugs, notably quinine and the salicy- 
lates, may produce a tinnitus, and that persons with middle-ear or laby- 
rinthine disease are particularly susceptible. A pulsating tinnitus 
due to arterial congestion, may often be relieved by pressure over the 
carotid artery, and this may serve as a diagnostic hint. 
Treatment.-This is usually unavailing unless its cause may be dis- 
covered, which is not infrequently impossible, and when possible the 
condition is often such that there is no available relief. The bromides 
and counter-irritation behind the ear may at times serve a useful purpose. 
If syphilis is suspected as a cause a vigorous treatment directed against 
that disease should be instituted. Tinnitus due to so-called functional 
disorders of the nervous system may be helped by the use of galvanism, 
using the anode as the active pole, and also by various suggestive 
measures. Recently Frazier1 has reported a case of successful intra- 
cranial division of the auditory nerve for tinnitus, as suggested by Mills 
in 1908 for vertigo. This somewhat radical procedure should find favor 
in selected cases but only in the hands of specially skilled operators. 
Nerve Deafness.-This term is applied to the inability to hear, con- 
ditioned by an affection of the cochlear nerve with accompanying laby- 
rinthine involvement. The details of this subject lead directly into 
the field of the otologist, but certain tests must be made by the practitioner 
which will throw light on the general character and location of the dis- 
turbance leading to the deafness. For this purpose a watch with a fairly 
distinct tick, or, better, a middle-range tuning fork, is essential. In 
making such an examination it is first necessary to determine whether 
the external auditory meatus is clear of wax and the drum membrane 
intact. Having determined these points, one ear should be closed, the 
1 Jour. Am. Med. Assn., 1913, Ixi, 327. 510 
DISEASES OF THE NERVOUS SYSTEM 
patient directed to close his eyes, and the watch brought from a distance 
toward the ear, the exact point being noted at which the tick is first 
distinctly heard. It should be remembered that the tick is not heard 
at so great a distance under these conditions as when the watch is grad- 
ually removed from the ear. The results obtained should be compared 
with the other ear and with a normal control. Considerable variation 
will be observed, but relatively speaking the test is sufficient to demon- 
strate the presence or absence of conduction by air. Should such an 
examination be negative, it indicates simply that the external ear, the 
internal ear, and the sound-appreciating apparatus are intact; in other 
words, it forms the most general relative test of hearing. Under certain 
conditions the conduction through the air and through the internal 
ear to the cochlea may be interfered with by disease in such a way that 
sounds no longer penetrate by way of the air. It is, therefore, necessary 
to examine bone conduction. Under normal conditions a sounding 
tuning fork placed either over the mastoid or other part of the skull in 
contact with the skin will be heard in both ears. Inasmuch, however, 
as the conduction through the bone to the internal ear is less direct 
than through the air, it happens that the tuning fork, when no longer 
heard over the mastoid, if placed before the ear will again be heard. 
This is the usual course of events under normal conditions and in nerve 
deafness. If, on the contrary, the sound-conducting apparatus is diseased 
through a plugging of the external meatus or through disease of the middle 
ear the sound will naturally not be heard aerially after it has ceased to 
be heard through the bone. If, therefore, there is an affection of the 
auditory nerve leading to so-called nerve deafness, a tuning fork is not 
heard through the bone, inasmuch as the receptive mechanism of sound 
in the cochlea is diseased. Under these conditions it may or may not be 
heard aerially. This is known as Rinne's test. This may be summarized 
as follows: In labyrinthine disease both air and bone conduction hearing 
are diminished and bone conduction is usually lost; whereas, in middle- 
ear disease air conduction is diminished and bone conduction is increased. 
As a further associated test of that devised by Rinne, the Weber test 
is ordinarily employed. Under normal conditions, if a tuning fork be 
placed on the forehead in the middle line and one meatus closed, the sound 
is best heard in the closed ear, since the aerial conduction is prevented on 
that side, whereas bone conduction is preserved and intensified through 
the conversion of the ear into a closed chamber. If, therefore, the sound- 
conducting mechanism is affected, the tuning fork is best heard on the 
affected side. This is known as a positive Weber test. If, on the other 
hand, nerve deafness exists from involvement of the labyrinth or the 
auditory nerve, the fork when applied to the forehead can no longer 
be heard on the affected side, inasmuch as the percipient mechanism is at 
fault. This is known as a negative Weber test. The Weber test, in 
general, is much less dependable than the Rinne test. These two tests 
are supplementary, and through their use a more definite knowledge 
may be obtained of the location of disease either in the sound-conducting 
mechanism, external auditory canal and middle ear, or in the sound- 
perception apparatus-labyrinth and cochlea. It is an interesting fact DISEASES OF THE CEREBRAL NERVES 
511 
that in nerve deafness hearing is usually worse in the midst of noise, 
whereas in deafness from chronic disease of the middle ear hearing is 
improved by external sounds. 
Vestibular Nerve.-Disease of this branch of the auditory nerve, so 
far as it is separable from affections of the cochlear nerve, leads essentially 
to disturbances of coordination, of which vertigo, including Meniere's 
complex, is the most important. Owing to the relations which the 
vestibular branch bears to other cranial nerves and to the spinal cord 
various incoordinations of the muscles of the head, neck, and eyes may 
result from a lesion of the nerve. Nystagmus, for example, has been 
observed as one of these symptoms. Tinnitus, due to disturbance in the 
special sense of hearing, is often difficult to separate clinically from 
vertigo, of which it is often a symptom. The two conditions are very 
frequently associated, but it is nevertheless best to consider vertigo as a 
distinct disorder of equilibration in connection with the vestibular nerve. 
Vertigo.-This may be defined as a sensation of giddiness and uncer- 
tainty resulting from a defect of equilibration, which is a complex act of 
muscular innervation, regulated ultimately by the cerebral cortex through 
the vestibular nerve and its connections in the ear and in the central 
nervous system. Experimental and other evidence goes strongly to 
show that the cerebellum is important in this function and that fibres 
of the vestibular nerve passing from the semicircular canals to the cere- 
bellum are of great significance in the preservation of equilibrium. 
It is also clear that the semicircular canals, standing as they do in close 
relation to the vestibular nerve, are to be regarded as an essential part 
of this complex reflex. Finally, the cerebral connections, directly and 
indirectly, of the vestibular nerve bring about the cerebral control, without 
which preservation of equilibrium is impossible. It is perhaps too much 
to assume that the vestibular nerve is invariably concerned directly 
in the production of the symptom of vertigo, but that it indirectly takes 
part in this condition through its various reflex connections is, in our 
present state of knowledge, hardly to be questioned. The relation 
of the vestibular nerve to the ocular nerves and muscles is likewise of 
great importance, and accounts for the vertigo frequently observed 
in connection with defects in ocular innervation. The production of 
vertigo through affections of the larynx and various portions of the 
gastro-intestinal tract is no doubt anatomically explainable through 
connections with the vagus group of nerves. 
Vertigo is described as subjective when the movement seems to be in 
the patient himself, and objective when external objects appear to be in 
motion. The usual result of a feeling of vertigo is the phenomenon of 
staggering. Inasmuch as vertigo occurs under many conditions, it is 
often a matter of extreme difficulty to arrive at an estimate of its sig- 
nificance in any given case. On the one hand, vertigo may be one of the 
symptoms of organic changes in the ear or central nervous system of 
gravest character, or, on the other hand, may indicate a nervous disturb- 
ance of the slightest sort. As Oppenheim pointed out, a distinct sensation 
of vertigo may be brought about in perfectly normal persons by closing 
the eyes, standing on one foot, and directing the attention to the general 512 
DISEASES OF THE NERVOUS SYSTEM 
uncertainty of this position. This fact, however, should not lead us to 
minimize its possible importance, particularly if associated with other 
symptoms of more or less grave character. In tumor of the brain, 
and particularly of the cerebellum, vertigo is a diagnostic point of much 
significance, particularly if the staggering tendency is persistently in one 
direction. As an example of the relative ease with which vertigo may be 
induced, it has been observed that a galvanic current passed through the 
head leads to a distinct tendency to fall toward the side of the positive 
pole, whereas a breaking of the current tends to induce falling toward 
the negative pole. That this phenomenon has direct relations with the 
semicircular canals is not to be questioned. 
It is not possible to give a complete list of the causes and conditions 
which may induce vertigo. As a general statement, it may be said that 
anything which alters our normal spatial relations may be productive 
of vertigo. Some of the more important of these conditions are diplopia, 
rapid changes of position, especially in a rotary direction, looking from 
high places, and, more indirectly, through immediate or secondary 
involvement of the vestibular nerve, general increase of intracranial 
pressure, notably through tumors especially of the cerebellum, disturb- 
ances of circulation, particularly through arteriosclerosis, acute anemia, 
multiple sclerosis, migraine, epileptic attacks. Other causes may be 
attributed to disorders of the stomach, intestinal tract, nose, larynx, 
and to certain intoxications. Affections of the ear itself are naturally 
a prolific cause. Among the most commonplace and practically impor- 
tant of these is a collection of cerumen in the external auditory canal. 
Auditory Vertigo.-In chronic middle-ear catarrh a type of disturbance 
may occur characterized at times by sufficient vertigo to cause a fall, 
accompanied with nausea, vomiting, and increase of tinnitus and deafness. 
Such attacks have been given the name Meniere's symptom-complex, 
to distinguish them from true Meniere's disease, which is presumably 
a labyrinthine affection, and which should in general be somewhat 
sharply distinguished from various other forms of vertigo which may under 
certain circumstances simulate it. So far as possible it is well to establish 
a pathological basis in the various forms of vertigo, and this seems possible 
in true Meniere's disease. 
Meniere's Disease.-In 1861 a French physician drew attention to the 
group of symptoms which have since passed under his name. The 
terms auditory1 and labyrinthine may be regarded as essentially synony- 
mous with Meniere's disease. The words, at least, are used interchange- 
ably. In limiting the application of this affection it is desirable to realize 
that it is through the immediate disturbance of the semicircular canals 
of the labyrinth that the symptom is produced. It should not, for 
example, be applied to vertigo manifestly due to distant conditions in 
the nervous system or organs which may, in a secondary way, affect 
the vestibular nerve. The onset of a typical attacks of auditory vertigo 
is, in general, as follows: It comes on suddenly, often sufficient to throw 
1 Dr. Clarence J. Blake has made a very useful distinction between "auditory 
vertigo," due to disease of the true auditory mechanism of the internal ear, and 
"aural vertigo," due to affections of other portions of the ear. DISEASES OF THE CEREBRAL NERVES 
513 
the patient to the ground. He is for a varying time stunned and possibly 
at times loses consciousness momentarily. The vertigo may be either 
objective or subjective in type. Vomiting and nausea quickly supervene, 
often persisting for hours, with headache. In these cases there is usually 
diminished hearing, with reduced bone conduction and almost always 
tinnitus. Occasionally nystagmus, on looking toward the affected side, 
diplopia, and facial palsy may supervene. The attacks vary widely in 
frequency, they may occur daily, weekly, monthly, or at times become 
practically constant (Status Meniere). In the earlier descriptions of the 
disease the onset was regarded as apoplectic in origin in a previously 
healthy ear. This view has been broadened to the extent of applying 
the term "aural vertigo" to cases with recognized ear disease. 
Pathological Anatomy.-Considering the affection in its narrower 
aspect, the lesion may be regarded as a primary disturbance in the 
labyrinth, usually hemorrhage, occurring in the course of syphilis, leu- 
kemia, gout, and similar constitutional affections or from injury or from 
local inflammation. Disturbance in the function of the endolymph is 
causative of the ensuing symptoms. Deafness naturally results from a 
lesion of the cochlea, whereas the vertigo depends upon the invasion or 
disturbance of function of the semicircular canals. Parkes Weber, quoted 
by Osler, has made the following classification which is rather more 
general and inclusive than the limited conception of Meniere's disease 
as due only to disease of the labyrinth would justify. It is, however, 
of value as a comprehensive statement of the main causes of vertigo. 
" (1) The apoplectic form, due to hemorrhage into the labyrinth, as in 
leukemia, follow'ed, as a rule, by complete deafness in one or both ears. 
(2) The cases associated with progressive inflammatory disease of the 
labyrinth. (3) Associated with organic changes in the auditory nerves, 
as in tumors, sometimes in tabes, and in cases of aural vertigo associated 
with facial paralysis on one side. (4) Cases in which a paroxysm of 
epilepsy is preceded by an auditory aura. (5) The moderate attacks 
which are associated with the various middle-ear affections, with wax 
in the meatus, with violent syringing of the ears, etc., all of which are 
probably due to increase in the intralabyrinthine pressure. Meniere's 
symptoms may occasionally be due to temporary excessive increase in 
the perilymph, possibly of angioneurotic character." 
Diagnosis.-When occurring in typical form and when associated with 
manifest disturbance in the auditory function this is not difficult. In 
any condition of vertigo, the ear should be subjected to a careful and 
expert examination, to determine the range of hearing and the possible 
affection of the internal ear, even if the patient's attention has not been 
especially directed to it through tinnitus or other symptoms. The other 
conditions which may be indirectly the cause of vertiginous attacks should 
likewise be carefully investigated. It is not uncommon to attribute to a 
disordered stomach conditions which are due primarily to labyrinthine 
disturbance and of which the stomach disorder is an effect. 
The greatest diagnostic difficulty lies in a sharp distinction between 
Meniere's disease and epileptic seizures. In the presence of marked 
labyrinthine disturbance manifested through deafness or tinnitus, the 514 
DISEASES OF THE NERVOUS SYSTEM 
differentiation should not be difficult. When these symptoms, however, 
are slight or possibly lacking, Meniere's disease may very closely simulate 
an epileptic attack. For example, aural vertigo may be and often is 
extremely sudden in onset, extremely short in duration, may unquestion- 
ably be accompanied by temporary loss of consciousness, may occur 
during sleep, and finally, which naturally renders its separation from 
epilepsy impossible, may be actually associated with that disease. All 
the foregoing conditions are met with in epileptic seizures in essentially 
the same form. Evidently, therefore, the points of distinction may be 
extremely vague, at least in exceptional cases. The violent sense of 
impulsion, as if being "hurled to the ground," is no doubt characteristic 
of aural vertigo, and does not occur in the same form in the epileptic 
seizure. Convulsive movements naturally do not occur in aural vertigo, 
nor is the onset so sudden that the patient is likely to injure himself. 
Gowers1 has described in much detail the extraordinary difficulties which 
may sometimes arise in the differential diagnosis between these two 
affections. Stress should always be laid upon the existence of ear symp- 
toms in a supposed Meniere complex. Such symptoms naturally may 
occur as a complication of a true epilepsy, but if epilepsy is excluded 
point strongly to the labyrinth as the primary source of the affection. 
In certain debilitated states of the nervous system, symptoms closely 
resembling, if not identical with Meniere's disease, may occur. It has 
been suggested that under these conditions vasomotor disturbances in 
the labyrinth may actually lead to a true auditory vertigo. 
Prognosis.-The outcome of Meniere's disease is always problematical. 
Experience has shown that with the increase of deafness the vertigo often 
decreases, and finally, when deafness is complete, may entirely disappear. 
Treatment.-It is natural that in a condition of this sort a wide range 
of treatment has been suggested. From what has been said it is evident 
that attention should primarily be directed to the condition of the ear. 
It is found, however, that simpler methods of treatment, such as middle- 
ear inflation, often fail of definite result, making a resort to surgical 
intervention almost imperative. Eugene A. Crockett, of Boston, has 
drawn attention to the operation of removal of the stapes for the relief 
of vertigo. He regards this, however, as justifiable only in those cases 
in which ordinary treatment has proved futile. Less radical operations 
on the ossicles are suggested as preliminary measures of relief. The 
still more radical operation of removal of the semicircular canals necessi- 
tates the almost certain destruction of the cochlea as well, with resultant 
complete deafness. Even this operation, however, may in certain 
intractable cases be justified. The following general methods of treat- 
ment are at times serviceable: The bowel movements should be kept 
perfectly free, warm foot baths may be given, a residence at Karlsbad 
or Marienbad and similar watering places may be advised. Iodide of 
potash may be used to combat a possible syphilitic cause. Ocular and 
nasal defects should be corrected. Charcot advised quinine in spite 
of its known effect on the auditory nerve. The salicylates have also 
1 The Borderland of Epilepsy, 1907. DISEASES OF THE CEREBRAL NERVES 
515 
been prescribed and the bromides have undoubtedly been found useful 
at times. Nitroglycerin is indicated in high arterial tension associated 
with arteriosclerosis. Pilocarpine injections have been extensively 
used, 5 to 8 drops of a 2 per cent, solution every second day, often con- 
tinued for weeks, or | grain subcutaneously or by the mouth. Lumbar 
puncture, advised by Babinski, in which from 3 to 20 cc. of fluid are with- 
drawn in one or several sittings, is a somewhat doubtful procedure. 
Paralyzing Vertigo (Gerlier's Disease).-Although vertigo is but one 
of the symptoms of this extraordinary affection and its relation to the 
auditory nerve is not understood, nevertheless it may properly be dis- 
cussed at this point. Gerlier,1 a Swiss physician, first described the 
affection in 1887. In Europe the affection has been observed only in 
Collex, a canton of Geneva, and in certain surrounding hamlets. Cow- 
herds and workers in the fields alone appear to be its victims, and they 
only during the summer months and under poor hygienic conditions. 
Vigorous young men are particularly likely to be attacked. 
Symptoms- Pain in the occipital region, muscular weakness, and 
disorders of the ocular nerves constitute the characteristic symptoms, 
combined in various ways and in varying degrees of intensity. Vertigo 
is a somewhat less conspicuous symptom. The pain in the neck is 
described as a sense of constriction often radiating into the back. The 
muscular weakness affects the voluntary muscles and particularly the 
extensors. The neck muscles may be unsymmetrically involved. An 
unequal ptosis is a common symptom not accompanied by paralysis of 
external or internal ocular muscles; the visual fields are at times dimin- 
ished, but the fundus shows nothing characteristic. At the onset of an 
attack and preceding the ptosis a certain clouding of vision with vertigo 
often occurs. A typical attack may be described as follows: "The 
patient, previously well, is suddenly attacked by pain in the neck and 
back; his sight is clouded to the point of temporary blindness; ptosis 
develops rapidly along with general weakness of neck and body muscles; 
standing erect becomes difficult; he reels and has all the appearance 
of drunkenness. After a period not exceeding ten minutes complete 
recovery takes place. During an attack the tendon reflexes are preserved, 
at times increased; the skin sensibility is intact, false trismus occurs, 
swallowing is often impossible, and general motor weakness dominates 
the clinical picture. The head often falls forward on the chest, due 
to weakness of the neck muscles, which, with the ptosis cutting off 
vision, renders the patient for the time wholly helpless. Nausea does not 
occur. Many attacks are slight and merely momentarily incapacitate 
the patient, but the foregoing symptoms invariably are developed in 
some degree." 
Etiology.-The cause remains undetermined. Considering the con- 
ditions under which the affection is most likely to occur, Gerlier presumed 
that it was due to an infection derived from stable soil and active only 
in the warm months of summer. The malady does not occur in cold 
weather, and the attacks are usually in the afternoon, ceasing shortly 
1 Rev. med. de la Suisse romande, 1887, vii, 1, 260; ibid., 1888, viii, 22, 86; ibid., 
1891, xi, 201, 260. 516 
DISEASES OF THE NERVOUS SYSTEM 
after sunset. Other explanations which have been offered, including 
hysteria, are even less satisfactory than Gerlier's suggestion. Although 
this type of vertigo, so far as known, occurs nowhere else on the Western 
Continent, a similar affection was described by Miura in the northern 
portion of Japan, to which was given the name " Kubisagari." 
Treatment.-Improved hygiene appears to be the most rational form 
of treatment. The affection is never fatal, and occurs only in summer. 
GLOSSOPHARYNGEAL (NINTH) NERVE. 
Symptoms.-Isolated disease of this nerve is not definitely known, or 
at least the cases are too few upon which to base conclusions of value. 
It follows, therefore, that exact knowledge of its function is vaguer than 
that of any of the other cranial nerves. Clinical investigation and experi- 
ment have established beyond much doubt that the dorsal portion of 
the tongue and neighboring parts of the pharynx are supplied with taste 
fibres through it. The question has, however, been raised that possibly 
even these fibres finally reach the brain through the fifth nerve. Others 
are of the opinion that the chorda tympani fibres finally enter the central 
glossopharyngeal. This and similar disputed points are difficult of 
determination on account of the rarity of affection of this nerve without 
coincident involvement of its neighbors. Cassirer, working with Oppen- 
heim, has reported a case which, in their opinion, demonstrates the fact 
that in certain instances all the taste fibres may run in the glossopharyn- 
geal. In addition to the innervation through the glossopharyngeal, 
parts of the posterior portion of the tongue and neighboring parts are 
also innervated by the fifth nerve, which adds still further to the difficulty 
of exact determination of the boundaries of the two sensory supplies. 
It may, however, be accepted that the chief innervation of the pharynx 
is from the glossopharyngeal. So far as this nerve is concerned with 
the act of swallowing and with the motor innervation of the oesophagus 
in general, its relations to the vagus presumably must be held responsible. 
Its direct relation to salivary secretion is probable. The nerve is very 
frequently involved in disease of the bulb, but rarely alone, as would 
naturally be inferred from its extremely close central relations, particularly 
with the vagus and accessory nerves. Disease external to the bulb in 
the posterior portion of the skull, aneurism, tumors, thrombosis of the 
jugular vein, may implicate the nerve, but here again rarely alone, 
nor are injuries any more likely to affect it and not others. Oppenheim 
describes an interesting case following protargol insufflation, in which 
the symptoms were slight paralysis of the palate and throat muscles, 
with loss of taste in the parts supplied by the nerve. This condition 
he thought due to chemical action on the nerve endings. Degeneration 
of the nerve may occur in tabes. Disturbances of taste in affections of 
the middle ear due to involvement of the tympanic plexus and increase 
of salivary secretion have been observed. DISEASES OF THE CEREBRAL NERVES 
517 
PNEUMOGASTRIC (VAGUS, TENTH) NERVE. 
Etiology.-Although the vagus nerve is seldom affected by a primary 
isolated neuritis, it is easily involved through other causes because of 
its extremely long course and wide distribution. There is evidence to 
show that the nerve may take part in a general neuritis or analogous 
conditions. For example, the nerve may be affected in a general alcoholic 
neuritis, and its involvement in diphtheria, as described by J. J. Thomas1 
and others, is recognized as possibly one of the causes of sudden death 
in that disease. The nerve may also be affected in other infectious 
diseases and also in intoxications through lead and arsenic. Tumor, 
aneurism of the vertebral artery, meningitis, and similar disturbances 
external to the oblongata may injure the nerve, but in this case, as with 
the glossopharyngeal, neighboring nerves are usually affected at the 
same time, among which the ninth, eleventh, and twelfth are the most 
frequent. Sclerosis of the vessels of the posterior fossa may lead to 
disturbance in the function of the nerve, and it is not infrequently injured 
in operations for tumors of the neck and in tying the carotid artery. 
Conditions affecting the bulb itself, as, for example, softening, hemor- 
rhage, bulbar paralysis in its various forms, tabes, and multiple sclerosis, 
are responsible for lesions of central origin. A noteworthy lesion is the 
involvement of the nerve in tabes, which presumably leads to the crises 
characteristic of that disease. In many of the hysterical conditions on 
the part of the gastro-intestinal, respiratory, and circulatory systems 
the nerve plays an important part. 
Symptoms.-The symptoms produced by affections of the vagus nerve 
are naturally varied because of the possibility of wide location of the 
lesion producing them. A total paralysis of the nerve is rare, but may 
occur through extensive destructive processes in the posterior fossa. 
In this case, however, lesions of other nerves are a usual accompaniment 
as already suggested. In one-sided paralysis the symptoms of unilateral 
weakness of the palate and larynx are conspicuous. The soft palate is 
flaccid and hangs away from the affected side, not moving perfectly with 
phonation. Speech is nasal and there is more or less difficulty in swallow- 
ing. The vocal cord on the affected side does not take part in phonation 
or respiration. There may also be partial anesthesia of the pharynx 
and larynx. In unilateral lesions of the nerve the condition of the heart 
is not constant. At times there is slowing of the beat, but more often 
an increase. Respiratory disturbance occurs usually only with a double 
lesion. Other general symptoms due to irritation or paralysis of the 
vagus are vomiting, bulimia, loss of appetite and thirst, pain in the 
epigastrium, and, conceivably, production of a diabetes. 
Pharyngeal Branches.-The innervation of the pharynx is through the 
vagus and pharyngeal nerves by way of the pharyngeal plexus. It is 
difficult to determine the exact part played by each nerve in the paralyses. 
The symptoms of disturbance in this field of innervation are difficulty 
in swallowing, inasmuch as the food is not properly passed on or enters 
1 Med. and Surg. Reports, Boston City Hospital, Ninth Series, 1898, p. 52. 518 
DISEASES OF THE NERVOUS SYSTEM 
the larynx, or, if the soft palate be involved, passes into the posterior 
nares. The so-called nasal voice is a common accompaniment. This 
group of symptoms is characteristic of lesions of the nuclei of origin, as 
seen in bulbar paralysis, in which death commonly results from actual 
starvation. The involvement of the pharynx with good prognosis is also 
common in postdiphtheritic paralysis. The difficulty in swallowing 
is very slight or negligible in one-sided paralysis. Spasmodic affections 
of the pharynx often occur without known structural basis, and are at 
times important as part of a more general neurosis, as, for example, 
hysteria. A pharyngeal spasm may at times be induced by exaggerated 
self-consciousness. It occurs also as one of the symptoms of hydrophobia. 
Laryngeal Branches.-The importance of a laryngeal examination in all 
cases where there is a suspicion of the involvement of this region cannot 
be exaggerated. A palsy of the larynx may exist entirely without 
objective symptoms. For example, in common abductor paralysis there 
is not necessarily any disturbance in vocalization, but the quality of the 
voice is usually slightly changed. 
The innervation of the larynx is through the superior and inferior or 
recurrent laryngeal nerves. The superior laryngeal nerve supplies 
sensation to the larynx and motor fibres to one pair of muscles, the 
cricothyroids, which act as tensors by separating the points of attachment 
of the vocal cords on the thyroid cartilage from those on the arytenoids. 
Paralysis of this nerve, therefore, leads to relaxation of the cord or cords, 
which, therefore, become slack on phonation. The voice is not lost, 
but is weak and easily tired. The paralysis is not well marked. Paralysis 
of the superior laryngeal nerve is rare because of its short course, but 
may be produced by wounds, tumors, or enlarged glands. It is also often 
obscured by the much commoner paralysis of the muscles of the cords. 
The other muscles of the pharynx proper are wholly supplied by the 
recurrent laryngeal nerves, which also give sensory fibres to the mucous 
membrane below the cords. The left recurrent nerve curves around 
the arch of the aorta and the right passes under the subclavian artery. 
It is natural, therefore, that these nerves are peculiarly liable to pressure 
from aneurism and also on account of their long course through involve- 
ment by tumors, glands, and other processes in the upper thoracic region. 
Varied palsies of the laryngeal muscles may result, giving rise to a wide 
diversity of symptoms and to a varied laryngeal picture. 
In conditions of health the edges of the vocal cords are straight on 
phonation, parallel and nearly in contact. Abduction takes place on 
deep inspiration and there is slight movement in quiet breathing. Rest- 
ing, the glottis is wider than when in the cadaveric position or in complete 
paralysis, due to the slight tonic action of the abductors. In gradual 
degeneration of the recurrent laryngeal nerves the paralysis of the muscles 
follows a definite order (Semon's law). Abduction is first affected through 
disturbed action of the posterior crico-arytenoids. The thyro-arytenoids 
are next involved, and then the lateral crico-arytenoids. The arytenoid, 
unpaired, has a bilateral nerve supply, and is, therefore, not affected in 
unilateral cases. The analogy in this law to paralysis of skeletal muscles 
of the arm and leg is interesting. DISEASES OF THE CEREBRAL NERVES 
519 
Abductor Paralysis.-In unilateral abductor paralysis the affected 
cord lies in the middle line. On phonation the sound cord abducts and 
the larynx looks normal. The palsy becomes evident on inspiration. 
The voice may not be affected. Owing to the reduction of the aperture 
between the cords, dyspnoea occurs on exertion, but in adults ordinary 
quiet breathing is not affected. The symptoms of dyspnoea are more 
marked in children, owing to the relatively small size of the glottis. 
In bilateral abductor paralysis both cords lie near the middle line. On 
inspiration, owing to the shape of the cords, they act as a valve and are 
drawn closer together, causing marked inspiratory dyspnoea with stridor. 
In this form of paralysis sudden death is always possible from a cutting 
off of the air current. On failure of the thyro-arytenoids, the cords are 
no longer tense and hoarseness ensues. 
Total Unilateral Recurrent Paralysis.-In this type of paralysis the cord 
lies in a position between abduction and adduction, the so-called cadaveric 
position. On inspiration the sound arytenoid is abducted and lies behind 
the other. On phonation the sound cord moves across the middle line, 
and lies at a slightly higher level than the other. The voice is not lost 
but is hoarse and easily tired. 
Bilateral Recurrent Paralysis.-The severe dyspnoea which occurs in 
bilateral abductor paralysis does not occur when the paralysis is more 
complete, but the voice is completely lost. Both the cords lie in the 
cadaveric position, but slight inward movement from the partially active 
arytenoid may occur, presumably through its possible supply from the 
superior laryngeal nerve. 
Diagnosis.-The diagnosis of these various forms of paralysis is made 
chiefly from the laryngoscopic appearances and from conditions of 
phonation and respiration. 
Functions of the Larynx.-There are two main functions of the larynx- 
(1) phonation, and (2) respiration. The adductors are essential to the 
former and the abductors to the proper performance of the latter. Pho- 
nation, being a later acquired function, is under direct cerebral control, 
the centres controlling it presumably lying in the lower part of the 
precentral area, with further connections with the areas presiding over 
the general function of speech. Stimulation of the cortical centre for 
phonation in either hemisphere leads to adduction of both cords, demon- 
strating, as is usually the case in muscles which act under normal cir- 
cumstances together, that the innervation is not entirely from one 
hemisphere. Respiration, which is the more fundamental function, and 
which in a certain sense is antagonistic to phonation, persists after the 
destruction of both cerebral hemispheres, as experimentally demonstrated. 
These facts are of interest in connection with the observation that so-called 
functional disorders lead to paralysis of the adductors, whereas abductor 
paralysis is due to structural changes. In lesions lying above the bulbar 
nuclei, if unilateral, paralysis of the cords does not ensue, as, for example, 
in hemiplegia, even though it be associated with aphasia. A bilateral 
lesion of the central tract, on the other hand, causes an adductor paralysis. 
Direct paralysis of the recurrent laryngeal nerve may result from lesions 
in the oblongata, at the base of the brain, in the vagus nerve, or in the 520 
DISEASES OF THE NERVOUS SYSTEM 
recurrent laryngeal itself. In nuclear disease affection of this nerve is 
usually combined with that of others, often associated with a persistent 
rapidity of the pulse. Syphilis, meningitis, or other disturbances in 
the posterior fossa may lead to laryngeal paralysis. A primary neuritis 
analogous to that of the seventh nerve is unusual, but has been described; 
paralysis from toxic agents is far less unusual. The vagus nerve in the 
neck may be variously injured. The frequency of laryngeal difficulty in 
the course of tabes, although not definitely understood, is of interest 
particularly in relation to the laryngeal crises. Anesthesia and various 
paresthesias of the larynx are not infrequent in this affection. 
The condition of the larynx in tabes has been carefully studied by 
Dorendorf, of Berlin, in 1903, and by D. Crosby Greene,1 of Boston, 
who found in 00 cases of tabes that 15 per cent, showed laryngeal com- 
plications, 10 per cent, paralysis of one or both vocal cords, and 12 per 
cent, were affected with laryngeal crises, figures which differ rather 
widely from those secured by Dorendorf on a basis of 245 cases. The 
type of paralysis in Greene's cases was always abductor, and this much 
more often unilateral than bilateral. The reason given for this fact is 
that the lesion is presumably a progressive degeneration of the fibres of 
the recurrent laryngeal nerve, and inasmuch as the abductors are the 
weaker group, they are the first to succumb. Greene further found that 
laryngeal crises are among the earliest laryngeal manifestations of the 
disease, from which the general practical conclusion is drawn that tabes 
as an etiological factor of certain laryngeal difficulties should be carefully 
considered. In certain cases, undoubtedly, the diagnosis of the more 
generalized disease may be made from the laryngeal signs. 
Perhaps the most usual causes of the involvement of the nerve are 
the trauma of surgical operation, the presence of aneurism and enlarged 
glands, cancer, goitre, effusions in the pericardium, and mediastinal 
disease. The involvement of the recurrent nerve is unilateral or bilat- 
eral, depending upon the extent of the causative process. Aneurism is, 
no doubt, the most important single cause, but tuberculosis is also often 
operative. Involvement of the right cord should, in fact, suggest the 
possibility of tuberculosis of the lung, because of the fact that the 
nerve in its course downward lies close to the pleura and may. easily be 
involved. 
Treatment.-This comes almost wholly within the province of the 
laryngologist. The practitioner, however, is often called upon to treat 
the general condition, of which a laryngeal affection is one of the symp- 
toms. Constitutional disease, for example, such as syphilis and tuberculo- 
sis or a general neuritis, must be treated by appropriate means irrespective 
of the laryngeal condition. The application of faradism to the affected 
laryngeal muscle under guidance from the laryngoscope is, at best, a 
doubtful procedure, and would certainly not be undertaken by any but a 
specialist. Surgical intervention in the various conditions which may 
lead to compression of the nerve in the neck and mediastinum falls within 
the province of the surgeon. 
1 Boston Med. and Surg. Jour., 1906, cliv, 97. DISEASES OF THE CEREBRAL NERVES 
521 
Adductor Paralysis.-As has before been said, adductor paralysis 
leading to difficulty or abolition of phonation forms a distinct class of 
functional paralysis. The great part which hysteria plays in these 
paralyses has been a subject of much discussion. It is probable, however, 
as pointed out by Cartaz, Janet, and others, that in hysterical conditions 
there is not much disturbance in the vocal cords, and that, as a matter 
of fact, the paralysis is apparent rather than real. In this, as in other 
hysterical paralytic conditions, the source of the paralysis is unquestion- 
ably central, and lies rather in a central incapacity for proper innervation 
than in any disorder of the cords themselves. In other words, in hysteri- 
cal mutism, if the patient were able to utter the sound which would 
lead to the proper action of the cords, the cords would no doubt function- 
ate properly. The movement of the cords, however, cannot be made 
simply because the words or proper sounds cannot be framed, and this 
naturally is purely a mental process. In general, it is probable that the 
so-called hysterical origin of adductor paralyses has been exaggerated, 
inasmuch as such disturbances are very frequently due to local disease 
immediately affecting the proper use of the laryngeal muscles. It may 
be said that adductor paralysis is never due to organic disease in the 
passage of the nerve from the bulbar nuclei to the larynx, but is either a 
pure neurosis or due to local disease. These myopathic paralyses in 
some degree are frequent in various forms of laryngitis. Although 
aphonia may be and often is a manifestation of hysteria, most of the cases 
are not purely hysterical in the proper sense of that term. Any state, 
for example, which requires greater effort than normal for proper phona- 
tion, as, for example, debilitated conditions in general or laryngeal 
catarrh, naturally predisposes thereto. Difficulty in phonation is 
especially common in tuberculosis on account of the frequent combination 
of extreme debility and laryngitis apart from immediate involvement of 
the nerve. In hysterical conditions whispering is usually possible, 
but even this may be at times lost, leading to the condition of so-called 
hysterical mutism. 
Adductor paralysis is almost always bilateral and seldom complete. 
It usually consists merely in an imperfect apposition of the cords. There 
are three sets of adductor muscles-the lateral crico-arytenoids, the 
thyro-arytenoids, and the unpaired arytenoid. These muscles may be 
affected together or irregularly. 
Diagnosis.-Adductor paralysis may be mistaken for total recurrent 
paralysis. In neither do the cords come together on phonation. In 
incomplete adductor palsy the cords separate widely on deep inspiration, 
whereas in complete paralysis of the adductors the cords lie in a position 
of extreme abduction, which should prevent confusion of interpretation. 
Treatment.-This falls within the special province of the laryngologist 
except when the affection is due to a general neurosis. The neurologist, 
however, is often called upon to treat the aphonias and mutisms which 
depend wholly or in part upon mental, hysterical causes. Suggestion 
variously applied, particularly by means of a strong faradic current 
applied over the larynx, will frequently restore speech. Such superficial 
methods of treatment, however, are usually temporary in effect and 522 
DISEASES OF THE NERVOUS SYSTEM 
relapses are the rule. A much more rational treatment is a painstaking 
investigation of the past life of the patient, with particular reference to 
the cause or causes which may have induced the speech defect. 
Spasm of the Laryngeal Muscles.-This leads to adduction of the 
cords, as, for example, artificially induced by stimulation of the cortex. 
The abductors, if affected, are overpowered by the stronger adductors. 
Such spasmodic affections are usually bilateral, even if due to irritation 
of the vagus fibres on one side. The causes are irritations of the nerve 
in its central or peripheral course, and naturally precede the paralytic 
conditions due to a destruction of the nerve. Therefore, any pressure 
of mild degree on the nerve in its peripheral course, tumors in early stages, 
slowly growing aneurism, and local disease of similar sort may lead to 
the condition of spasm. A structural central lesion may likewise be a 
cause, and the condition has been observed in tabes, general paralysis 
and hydrophobia. In hysterical conditions, instead of a loss of function 
of the laryngeal muscle, there may likewise be an overaction leading 
to spasm. In children the condition known as "laryngismus stridulus" 
is of practical importance. 
Phonic Spasm (Spastic Dysphonia).-This may be classified as a rare 
occupation neurosis allied to writer's cramp and similar affections. It 
occurs particularly in voice users, and gives rise to a variety of spasmodic 
affections similar to those just described. 
Sensory Neuroses.-Anesthesia of the larynx, complete or partial, 
may result from injury of the nerve through disease, notably diphtheria, 
or from bulbar affections of varied character. Except in purely functional 
conditions, the motor portion of the nerve is ordinarily involved as well. 
The result is that food easily enters the larynx, often resulting in a fatal 
deglutition pneumonia. The prognosis is unfavorable in cases of total 
bilateral anesthesia. The treatment must be of the underlying condition 
so far as is possible. Special care must be taken in feeding the patient 
to prevent the passage of food into the larynx. In unilateral cases the 
patient should be directed to swallow very slowly, lying on the sound side 
during the process. In complete sensory paralysis the oesophageal 
tube is necessary, the strictest care being taken not to pass it into the 
insensitive larynx. Hyperesthesia and paresthesia of the larynx are not 
uncommon in certain neuroses and in tabes. 
Cardiac Branches.-The cardiac plexus of nerves is formed from branches 
of the inferior laryngeal nerve from the main trunk of the vagus and from 
the sympathetic system. By these means the motor, sensory, and 
trophic functions of the heart are subserved. In one-sided lesions of the 
cardiac branches the symptoms on the part of the heart are not constant. 
At times there is slowing of the beat, but more often an increase in its 
rapidity. Such results have been observed both in experimental com- 
pression and in section of the nerve. In unilateral section of the vagus 
at times no symptoms whatever are produced, the laryngeal muscles 
even not showing the effect of the lesion. This no doubt is explained 
by the fact that muscles which ordinarily act bilaterally are innervated 
from both sides of the brain. The phenomenon at times observed of 
voluntary control of the heart is of interest in this connection. It DISEASES OF THE CEREBRAL NERVES 
523 
occasionally happens through some peculiar aptitude that a person is 
able, through an act of the will, to regulate within certain limits the 
rapidity of action of his heart. 
Sensory Symptoms.-A. consideration of the various cardiac neuroses 
dependent upon the disordered function of the vagus nerve would lead 
beyond the limits allotted to this section. It is one of the best recognized 
facts in medicine that the heart is peculiarly subject to functional dis- 
orders. Some of the conditions which may be attributed to a disorder 
of the sensory mechanism of the heart are palpitation, arhythmia, tachy- 
cardia, bradycardia, angina pectoris and so-called pseudo-angina. 
Pulmonary Branches.-Unless due to actual disease of the larynx or to 
organic disease of the lungs themselves, respiratory disorders are usually 
attributable to central causes or to bilateral lesions of the nerve. The 
evidence, however, thus far secured regarding the exact part played by 
the lesions of the nerve in the respiratory act is somewhat conflicting. 
Slowing, rapidity, and irregularity of respiration have all been observed. 
Oppenheim speaks of a case in which slowing of the pulse was associated 
with increase of respiration, attributable to vagus disease. In tabes, a 
slowing of the respiration to three or four in a minute has been observed 
and has been supposed to be due to a double vagus lesion. A permanent 
disturbance of respiration does not occur in unilateral vagus disease. 
It is possible that the motor fibres controlling the bronchial vessels are 
concerned in the production of asthmatic conditions, and possibly also 
are responsible for a form of emphysema. 
(Esophageal and Gastric Branches.-Branches of the nerve to the 
oesophagus are rarely diseased alone. A not infrequent condition, which 
must be classed among the functional disorders, is spasm of the oesophagus 
unassociated with organic stricture. A case under observation for 
many years in an elderly man without organic disease has shown marked 
improvement through treatment by faradism. In this instance, any 
carelessness in swallowing or any lack of proper mastication is liable to 
excite a violent spasmodic contraction of the lower end of the oesophagus, 
leading often to regurgitation and expulsion of food. The vagus nerve 
must be regarded as the sensory nerve for the stomach, through the local 
irritation of which the sensation of hunger is presumably induced. In its 
motor function to the stomach, its section lessens contractions. Vomiting 
is to be regarded as a sign of undue irritation, occasioned either by irrita- 
tion from the presence of a foreign body, from overmuch food, or indirectly 
from the effect on the nerve of cerebral pressure. The gastric crisis in 
tabes is a phenomenon entirely analogous to the laryngeal crisis. 
Vagal Attacks.-Under the heading "Vagal and Vasovagal Attacks," 
Gowers described seizures of peculiar type which he regards as due to 
disturbances of some of the functions of the pneumogastric. The symp- 
toms of these seizures are, for the most part, sensory, and include sub- 
jective, gastric, respiratory, and cardiac discomfort, at times accompanied 
by cardiac pain and a sense of impending death. In addition to these 
symptoms there is frequently a slight accompanying mental change 
with vasomotor constriction of peripheral vessels. Slight tetanoid spasm 
and some sensory impairment complete the general clinical picture of 524 
DISEASES OF THE NERVOUS SYSTEM 
a somewhat vague but no doubt important symptom-complex. Seiz- 
ures having the foregoing symptoms in varying degrees have a duration 
of ten minutes to one-half hour or more, occurring at varying intervals 
often for months or years. Gowers calls attention to the fact that a 
condition somewhat similar to these seizures has been described by 
Nothnagel and others, who have, however, not regarded the group of 
symptoms as features of a definite attack. The effect of compression 
of the vagus nerve in the neck, as shown by Waller many years ago, is, 
first, difficulty in breathing, followed by labored inspiration, uneasiness 
over the precordial region, and gastric discomfort at times amounting 
to nausea. If the pressure is continued, syncope results. These symp- 
toms were not considered as due to compression of the carotid. In 
Gowers' experience women suffer more frequently from these vagal 
attacks than men, but he repudiates the idea that the seizures are to 
be regarded as manifestations of hysteria.1 
SPINAL ACCESSORY (ELEVENTH) NERVE. 
The eleventh or spinal accessory nerve is purely motor in function. 
The portion of the nerve accessory to the vagus has already been con- 
sidered. The spinal portion may be considered independently. 
Etiology.-There are many conditions leading to involvement of the 
spinal accessory nerve and consequent disturbance in the functions of 
the two large muscles which it in part supplies. Lesions of the cervical 
cord itself, with destruction of the cells of origin of the nerve, are not 
infrequent. The more important of these are cervical myelitis, pro- 
gressive muscular atrophy of spinal origin, gliosis and syringomyelia. 
Disease of the bone, notably tuberculous processes in the upper cervical 
vertebrae, pachymeningitis of tuberculous or syphilitic character, are 
likely to involve this nerve. Injuries of the nerve in the neck, particularly 
in operations for tumors, and of late the voluntary injury of the nerve 
to provide an anastomosis with the facial in cases of intractable facial 
paralysis, naturally lead to its partial or complete paralysis. A primary 
neuritis has also been described. In most instances, especially when the 
lesion lies outside the cord, the affection of the nerve is one-sided. 
Symptoms.-The symptoms of paralysis are purely motor and consist 
in paralysis of the sternocleidomastoid and partial paralysis usually of 
the trapezius. Under ordinary conditions of rest a paralysis of one 
sternomastoid muscle does not lead to noticeable deformity. The 
defect, however, is brought out when the head, better against resistance, 
is turned toward the side away from the affected muscle, with slight 
raising of the chin. In paralysis of the right sternomastoid such a 
movement toward the left will be incomplete and no contraction of the 
1 "Women suffer more frequently, but these attacks are also met in men. This 
and the fact that the pneumogastric and vasomotor systems are readily influenced 
by emotion have probably led to the frequent submergence of these attacks beneath 
the vague conception of hysteria, a conception which conceals whatever it covers. 
We must rescue from it whatever we wish to study. It can be put back again after- 
ward if desired."-Borderland of Epilepsy, p. 19. DISEASES OF THE CEREBRAL NERVES 
525 
muscle will take place. If the sternomastoid is paralyzed on both sides, 
the head tends to fall backward, and in a horizontal position cannot 
easily be raised. The attachments of the trapezius are such that a 
certain distinction may be made between the paralysis of its three por- 
tions. Under normal conditions the muscle, through its attachments 
in the neck and throughout the thoracic region of the spine to the upper 
and outer portion of the scapula, tends to raise the shoulder and approach 
the scapula to the middle line. The upper portion of the muscle from 
the occiput to the outer third of the clavicle draws the head backward 
and turns it slightly toward the opposite side. The middle portion 
from the ligamentum nuchse and the three upper thoracic vertebrae 
to the acromion and outer portion of the spine of the scapula elevates 
the scapula. The lower portion from the fourth and subsequent thoracic 
vertebrae to the inner half of the spine of the scapula draws the scapula 
toward the middle line. In paralysis of the clavicular portion of the 
muscle the shoulder does not move in respiration, but the failure of 
backward motion of the head is inconspicuous because of the continued 
action of deep neck muscles. This upper bundle of the trapezius is very 
apt to be spared in paralytic conditions. Paralysis of the middle portion 
leads to a sinking of the acromion through lack of fixation, which the 
trapezius ordinarily gives it, and through the continued action of the 
levator anguli scapulae. The shoulder is therefore depressed forward 
and cannot be raised. The raising of the arm suffers somewhat in 
consequence. Paralysis of the lower portion leads to an increased distance 
of the inner border of the scapula from the middle line, with increased 
prominence of the scapula as a whole. 
Prognosis.-The prognosis of lesions of the spinal accessory nerve 
from the causes given above naturally depends upon their individual 
curability. In syphilitic conditions and those resulting from transient 
inflammations a favorable outlook may be anticipated. In general, 
if the nerve be given opportunity, it will rapidly regenerate without 
special treatment. In hopeless paralysis much comfort and improved 
action of unaffected muscles may be obtained by the application of 
apparatus to elevate the shoulder. 
Spasmodic Affections of the Neck Muscles.-Spasm of the neck 
muscles, including the innervation of the spinal accessory nerve, is 
perhaps the most distressing form of this type of disturbance. The 
variety of spasm which may develop in this general group of muscles is 
so great that merely an artificial classification may be made. Although 
the sternomastoid and trapezius frequently take part in these spasmodic 
affections, and often predominate in the clinical picture, we are usually 
not justified in regarding the spasm as wholly under the control of the 
spinal accessory nerve, inasmuch as other muscles supplied from other 
sources are almost invariably involved. Among these other muscles, 
the splenius, scaleni, and deep muscles of the neck, and at times the 
platysma and omohyoid, are of particular importance. The fact should 
be recognized that, although for purposes of clinical convenience we regard 
certain common spasmodic affections of the neck as due to disturbance 
in the spinal accessory innervation, the affection is usually much more 526 
DISEASES OF THE NERVOUS SYSTEM 
widespread and shows a distinct tendency to pass to other muscles. 
The importance of this in relation to treatment will receive attention later. 
Torticollis.-Torticollis, or wryneck, occurs in various forms, some of 
which are quite independent of the nervous system. Of these the 
following may be mentioned: Rheumatic torticollis, the ordinary stiff 
neck, has no relation with a true spasm. A fixed torticollis due to con- 
genital or other shortening of the muscles supporting the head is 
likewise not a form of spasm. A type of acquired torticollis, usually 
of slight degree, which may lead to confusion in diagnosis, is that asso- 
ciated with carious processes in the upper vertebrae in the absence of 
external deformity. An abnormal and rigid position of the head, espe- 
Fig. 35 
Torticollis. Showing spasm of the right sternocleidomastoid muscle, and characteristic position of the 
head at the height of the spasm. (Massachusetts General Hospital.) 
cially when associated with an otherwise unexplained exaggeration of the 
deep reflexes, should lead to the suspicion of a tuberculous or other 
process in the upper cervical region. The rigidity of the neck often 
seen in meningitis and other conditions which lead to pressure on the 
outgoing nerves is usually easy of diagnosis. 
Spasmodic Torticollis.-The most practically important variety of 
spasm of the neck muscles is the so-called spasmodic torticollis, or wry- 
neck, in which the muscles innervated by the spinal accessory nerve play 
a predominant but by no means exclusive part. 
Etiology.-1This is vaguely understood, hence a great variety of causes 
has been given, few of which are explanatory of the fundamental under- DISEASES OF THE CEREBRAL NERVES 
527 
lying condition which actually induces the spasm. Oppenheim, among 
others, is very strong in the conviction that the affection is observed 
particularly among persons with hereditary unstable nervous systems, 
although direct inheritance is exceedingly rare. Frequent association 
of torticollis with other nerve disorders, either neuroses or psychoses, 
lends a certain weight to this view, as does also the fact that many of 
the cases yield most satisfactorily to treatment by psychotherapy. To 
make the statement, however, that the cases occur primarily in an 
otherwise unstable nervous system does little to explain the exact nature 
of the mental or physical process which makes possible this special 
Fig. 36 
Spasmodic torticollis. Head drawn backward and chin up, due to involvement of the right trapezius, 
probably with certain deep neck muscles, in addition to the manifest spasm of the sternomastoid. 
(Massachusetts General Hospital.) 
spasmodic manifestation. In view of the fact that there is no known 
pathological anatomy, we are entirely justified in assuming that the 
cerebral cortex plays an important part in the production of the spasm, 
whether or not it be on a previous neuropathic basis. Various somewhat 
fanciful explanations of the affection have been submitted which cannot 
be regarded as of universal application. Irritations in the distribution 
of the trigeminal and occipital nerves, localized peripheral irritations as 
from too small a collar, disorders in the symmetrical innervation of the 
two sides of the body, eye-strain, organic disease of the brain, intoxications 
of various sorts, even astigmatism, are some of the causes which have 
been assigned. Perhaps in the majority of cases no adequate exciting 528 
DISEASES OF THE NERVOUS SYSTEM 
cause whatever is to be found, and recourse must be had to the explanation 
that mental perturbation, overaction of the muscles, and habit are 
responsible. 
Symptoms.-The form of spasm varies in different cases from slight, 
hardly noticeable involuntary movements of the head to violent spasmodic 
contractions, painful to the patient and extremely conspicuous to the 
onlooker. Clonic spasms usually associated with tonic muscular con- 
tractions are the characteristic forms in which the affection manifests 
itself. In those cases in which the spasm is clonic in character over 
long periods of time, one is naturally not justified in speaking of the 
torticollis as of the form here under consideration. The exact type of 
spasm depends upon the degree of involvement of individual muscles and 
upon the number and position of the muscles affected. The picture, 
therefore, becomes a very complex one, and it is often a matter of extreme 
difficulty to determine the precise extent of the muscular involvement. 
The common form and that most closely related to the spinal accessory 
nerve consists of a clonic and tonic spasm of the sternomastoid of one 
side, often accompanied by a similar spasm, particularly of the upper 
portion of the trapezius. In this type the chin and face are moved 
away from the affected side and the head bent slightly backward, due to 
contraction of the sternomastoid. The coincident contraction of the 
upper part of the trapezius likewise leads to a turning and backward 
bending of the head away from the affected muscle, thereby increasing 
in a general way and supplementing the action of the sternomastoid. 
Other forms of spasm, as, for example, of the splenius, rhomboids, and 
deep muscles of the neck, do not now concern us, particularly since they 
bear no immediate relation to the spinal accessory nerve. It is, however, 
very important to bear in mind that although a torticollis may begin 
with the spinal accessory innervation, it is likely to spread to other 
muscles. As suggested above, the extent and intensity of the spasm vary 
widely in different cases, and are unquestionably affected for the worse 
by unfavorable emotional conditions. Associated psychical disturbances 
are of interest particularly in their etiological relationship, but it should 
be borne in mind that a coincidental disturbance of a mental sort is 
perhaps particularly likely to arise in this distressing condition. 
Pathology.-The attempt to locate the lesion productive of torticollis 
of the spasmodic type has been wholly unsuccessful. To say that it is a 
condition of irritation in the nerve nuclei is of small help, since we are 
ignorant of what such irritations may consist. The most reasonable 
hypothesis is that the cerebral cortex, particularly in the area concerned 
in the movements of the neck, is primarily at fault. 
Prognosis.-The outcome is always uncertain, but never entirely 
hopeless. The unexpected not infrequently happens. In one instance, 
in which the condition had become so grave that an operation had 
practically been decided upon, a preliminary sea voyage afforded entire 
relief. In general, however, the affection is stubborn, resistant of 
treatment, and shows a tendency to spread to other groups of muscles, 
oftentimes with a substitution of a practically tonic spasm for the 
alternating clonic spasms of the earlier stages. DISEASES OF THE CEREBRAL NERVES 
529 
Treatment.-As in many affections the exact cause of which is not 
understood, treatment for spasmodic torticollis has been and remains 
largely empirical. It is not necessary, therefore, to enter into detail 
regarding the great number of drugs which have been advocated. Expe- 
rience has unquestionably shown that, apart from their influence upon 
the nervous system in general, there is no agent that exerts the slightest 
specific action upon the spasm. The bromides and opium may be 
given, and as general sedatives may have an effect, but few would venture 
the prescription of the opium preparations unless as a last resort and under 
most unusual conditions. Drugs unquestionably play a very small part 
in the rational treatment of this affection. Electricity has been used in 
certain cases with good results. It has been suggested that in the 
application of the electrical current it is desirable to stimulate the sound 
muscles rather than those affected with the spasm in order to overcome, 
so far as possible, the overaction of the latter. However theoretically 
justified such treatment may be, it has relatively little practical sig- 
nificance. Hydrotherapy is, no doubt, useful as a general tonic measure, 
but it has no further significance. 
The use of mechanical appliances is of interest, particularly from the 
fact that their effect in different cases varies widely. An arrangement 
by means of which the head is held absolutely fixed is never to be advised. 
Such constriction is extremely apt to excite the muscular spasm in an 
even greater degree, leading to a distressing situation for the patient, 
even although the head is mechanically prevented from moving. On 
the other hand, it has frequently been shown that a supporting apparatus 
which is suggestive in its operation rather than providing an absolute 
fixity, is often of great benefit. The common experience that in the 
midst of the most violent spasm the head may be restored to its normal 
position by gentle pressure applied to the chin is suggestive. The hint 
which these facts gives regarding rational treatment is that the mental 
side of the affection must always be seriously taken into account. The 
spasm is, in fact, semi-involuntary, at least in its earlier course, and 
should be treated accordingly. Following out this idea, the most useful 
treatment is certainly that which combines mental and physical means, 
and this is best attained through cobrdinative gymnastic movements. 
Such movements have as their underlying principle the possibility 
of substituting in a voluntary muscle a voluntary for an involuntary 
movement. The aim, therefore, is to educate the patient to again 
perform voluntarily movements over which he has lost control, or, 
otherwise expressed, to break up a bad neuromuscular habit. System- 
atic movements of the head are therefore advisable, and best carried 
out under the instruction of a skilled teacher. A purely psychothera- 
peutic treatment in these cases is, on the whole, not efficacious, nor is 
it necessary. Hypnotism is undesirable, certainly as a general procedure. 
A wide range of surgical operations has been suggested and surgery 
would appear to be at first sight clearly indicated. Results have shown 
that surgical intervention is rarely curative and nearly always discour- 
aging in its results. The reason for this is two-fold: first, that a surgical 
operation does not relieve the cerebral condition which undoubtedly 530 
DISEASES OE THE NERVOUS SYSTEM 
lies at the basis of the spasm; and, in the second place, that more than 
one muscle is apt to be affected, with a strong tendency to spread to 
still other muscles or groups of muscles. Simple resection of the spinal 
accessory nerve is of small value, owing to its rapid regrowth. Resec- 
tion with evulsion is a more rational procedure. The operative pro- 
cedure may go farther and resect upper cervical nerves if the increasing 
spasm demands. The muscles themselves may also be cut, thereby 
diminishing the strength of their contraction, and even more radical 
operations on nerves and muscles of the various structures about the neck 
may in rare instances be advisable. The results of operations have been 
such, however, that the unpleasant suggestion arises that the section of 
one nerve with cessation of spasm of the muscle to which it goes rather 
increases the tendency to spread. If the affection is primarily psycho- 
genic in origin it necessarily follows that operation in itself is unlikely 
to effect a complete cure. 
HYPOGLOSSAL (TWELFTH) NERVE. 
Etiology.-This nerve is very commonly affected in disease involving 
the bulb, notably in the various forms of bulbar paralysis, acute and 
chronic. In its central course from the lower portion of the cortical 
motor area to its secondary nucleus in the oblongata also it is not infre- 
quently involved. Although not particularly noticeable on account of 
the action of the nerve of the opposite side, one hypoglossus is ordinarily 
affected in cerebral hemiplegia. This lesion, however, is less conspicuous, 
because unaccompanied with atrophic changes of the tongue. This 
fact, together with unchanged electrical reactions, is an important and 
simple means of differentiation between lesions of the central and per- 
ipheral neurone, being merely an example of a general principle. Lesions 
in the oblongata itself, however, naturally involve the cells of origin of 
the peripheral axones and lead to a characteristic atrophy of the tongue. 
Lesions external to the oblongata itself, as, for example, meningeal 
exudates, tumors, or hemorrhages at the base of the skull, may involve 
the nerve in its peripheral course, but usually in conjunction with others, 
and particularly the vagus. Aneurism of the vertebral artery and 
syphilitic processes may involve the nerve, but these are rare occurrences. 
As of other cranial nerves, an isolated neuritis is rare, although such a 
condition has been described by various writers. Congenital atrophies 
of the tongue, lingual hemiatrophy, and degenerations should also be 
mentioned in this connection. 
Symptoms.-Disease of one or both hypoglossal nerves gives rise to 
signs not easily mistakable and of much diagnostic importance. These 
signs are perhaps particularly noticeable when the lesion involves but 
one nerve. As the tongue lies on the floor of the mouth there may be no 
deviation of its tip, but if moved, the tendency is for it to be projected 
toward the paralyzed side. The explanation of this constant phenomenon 
is not simple, owing to the complicated relations of the tongue muscles. 
The attempt to move the tongue within the mouth cavity is imperfect 
toward the diseased side, which naturally interferes somewhat with the DISEASES OF THE CEREBRAL NERVES 
531 
contact of the tongue on that side with various portions of the mouth 
cavity essential to proper enunciation and mastication. This defect, 
however, is so far compensated by the normal side that it leads to slight 
inconvenience. The defect becomes more apparent in the attempt to 
thrust out the tongue. This can be accomplished if but one side is 
paralyzed, but if the tongue is protruded the tip points strongly toward 
the affected side, its raphe describing a semicircle with its convexity 
toward the sound side; this deviation is due to the loss of contraction of 
the genioglossus. When the paralysis of both nerves is complete the 
tongue remains helpless and immovable in the mouth, and it becomes 
impossible for the patient to speak except in a most imperfect way. 
Mastication and deglutition are likewise interfered with. This condition 
is met with in the late stages of progressive bulbar paralysis. There is 
no sufficient evidence, as maintained by Gowers, that the muscles of the 
lips are innervated through the hypoglossal. 
Fig. 37 
Unilateral atrophy of the tongue. Protrusion toward the affected side, associated with movement 
of lower jaw toward the same side, owing to coincident involvement of the motor branch of the left 
fifth nerve. From a case of poliencephalomyelitis. 
Atrophy is the constant accompaniment of peripheral lesion of this as 
of other motor nerves. The tongue loses its normally smooth appearance, 
and is thrown into folds and ridges due to the wasting of the underlying 
muscle bundles. To the touch it is soft and non-resistant, shows altera- 
tions in electrical reaction, and is pervaded by a fibrillary tremor. 
Treatment.-The treatment of paralysis of the tongue, either of the 
spastic or atrophic type, lies almost wholly in that of the underlying 
condition. Electricity applied to the atrophying tongue muscle is a 
practical impossibility. In many conditions of unilateral disturbance 
of the tongue the discomfort is not sufficient to call for treatment, even 
if it were possible, and in the sudden or gradual involvement of the nerve 
from bulbar or more peripherally lying disease the affection of the tongue 532 
DISEASES OF THE NERVOUS SYSTEM 
constitutes a relatively insignificant part of the clinical picture and 
demands no special treatment. The partial resection of the nerve which 
has lately been practised in faciohypoglossal anastomosis for facial 
paralysis leads naturally to immediate disturbance in its function char- 
acterized by a tendency to atrophy and slight speech defect, but this is 
quickly overcome, with complete restoration of function. 
Spasmodic Affections.-The occurrence of spasm in the muscles 
supplied by the hypoglossal nerve is common in association with more 
general spasmodic affections, as, for example, epilepsy, hysteria, and 
chorea. The impossibility of controlling the tongue observed in general 
athetosis, often leading to a complete loss of power of articulate speech, 
is a somewhat analogous condition, but is not properly included under 
spasmodic affections. Much more rarely the tongue muscles are affected 
with spasm which either does not extend beyond them or only in the 
immediate neighborhood. Such spasms may be either tonic or clonic 
in character. If tonic, the tongue is apparently reduced in size and is 
pressed against the teeth or other parts of the mouth cavity. Under 
these conditions speech and swallowing are affected. Associated or 
independent of these tonic spasms a clonic condition of the tongue muscles 
may be developed. Under these conditions the tongue is rapidly thrust 
out or withdrawn, or makes various involuntary excursions within the 
mouth cavity. Such spasms may be either bilateral or unilateral in 
distribution, the latter being less frequent. The relation of these cramp 
forms to various speech defects known as stammering and stuttering is 
of interest. Under certain conditions the difficult speech is apparently 
induced by the incapacity of the person to move the tongue properly, 
with a result that the word or syllable is not enunciated, or in an explosive 
manner. However complicated the ultimate explanation of such forms 
of speech disturbance may be, the relation to spasmodic affections in the 
distribution of the hypoglossal nerve should always be borne in mind. 
The muscle spasm or tic is rarely constant, but occurs rather in the form 
of attacks, varying widely in frequency and not always ceasing during 
sleep. The causes are somewhat vague, but may in general be attributed 
to sources of irritation in the neighborhood, on the basis of a neuropathic 
tendency. The relation of the mental state to cramp attacks is undoubt- 
edly important. They may, for example, be aroused by emotional 
disturbances of one or another sort. The prognosis is, on the whole, 
favorable, even though the tendency to spasm may have existed for 
years. The treatment consists essentially in the removal of any exciting 
cause and psychotherapeutic procedures, with such muscle training as is 
possible. Drugs and electricity play no essential part in the treatment. CHAPTER XIV. 
DISEASES OF THE PERIPHERAL NERVES. 
GORDON M. HOLMES, M.D., F.R.C.P. 
The symptoms of disease of the peripheral nerves may be classified as 
they result from disturbance of the functions of those fibres which convey 
impulses to the muscles, of those which carry the sensory impressions 
centralward, and finally, of the fibres which are more immediately con- 
cerned with the nutrition of the tissues, either directly or as part of the 
mechanism for the local regulation of the blood stream. There are, how- 
ever, but few nerves in the body, excepting the cranial nerves, which are 
either entirely sensory or entirely motor. The consequence is that when any 
nerve is severely injured, both sensory and motor symptoms and probably, 
in addition, nutritive or trophic changes result. It is by the extent and 
distribution of these symptoms that the localization of the disease can 
be determined. If, however, the lesion or the disease of a mixed nerve 
is partial or incomplete, the functions of the different sets of fibres may 
suffer unequally; the general experience is that in incomplete lesions, 
as those due to compression, the sensory suffer much less than the motor 
fibres, or sensory symptoms may be absent, although the muscles supplied 
by the nerve are completely paralyzed. Liideritz showed experimentally 
that the conductivity of the motor fibres is lost earlier than that of the 
sensory fibres when a mixed nerve is subjected to slowly increasing press- 
ure. Evidence of trophic or vasomotor disturbance is, as a rule, little 
apparent in partial lesions. 
Motor Symptoms.-The symptoms which immediately follow the com- 
plete interruption of the motor nerve fibres that supply a muscle are 
complete paralysis of both reflex and volitional contraction of that 
muscle, loss of its tone, and later, atrophy, changes in the character of its 
response to electrical stimulation, and finally, if recovery does not take 
place, contracture owing to secondary fibrosis. These features distinguish 
lower motor neurone or spino-muscular paralysis from paralysis due to 
disease in the upper motor or cerebro-spinal neurones. In the latter 
condition movements, not muscles as such, are paralyzed, and the dis- 
tribution of the paralysis does not correspond to the distribution of 
one or more peripheral nerves; secondly, the tone of the paralyzed 
muscles is increased, not diminished or lost, that is, the paralysis is 
spastic and not flaccid, and the reflexes which are dependent on the tone 
of the muscle, as the knee-jerk, are more active than normal instead of 
being abolished; thirdly, the paralyzed muscles do not atrophy, or 
atrophy only to a much less degree; and finally, there is no change in 
the nature of their response to electrical stimulation. 
533 534 
DISEASES OF THE NERVOUS SYSTEM 
The amount of loss of power in the muscles naturally depends on the 
degree of the injury of the nerve; when there is complete interruption of 
its structure or loss of its function the paralysis is necessarily complete. 
When the lesion of the nerve is incomplete another factor must be con- 
sidered, namely, the rate of its evolution, as when the disease is of sudden 
or rapid onset the symptoms may be considerably greater, for a time at 
least, than those produced by a similar lesion which has developed slowly. 
A muscle that receives its motor fibres from more than one nerve is 
not completely paralyzed by even a complete lesion of one of them. 
The normal tone or tension of muscles is dependent on the integrity of 
the peripheral reflex arc, which consists of the afferent fibres from the 
muscle that enter the cord by the dorsal spinal roots and terminate by 
synapsis around the cells of the corresponding motor neurones in the 
ventral horn, and of the peripheral motor neurones. When this arc is 
broken in any place the muscles immediately lose their tone. The 
atonia may be recognized by loss of the normal contour of the muscles, 
if this is easily visible; by their softness and flabbiness to touch, and by 
the lack or diminution of the resistance which they normally offer'to 
stretching, and consequently the excess of mobility to passive movement 
of the joint at which they act. 
The deep reflexes or tendon-jerks are dependent on the maintenance of 
the muscle tone, and it is by these so-called reflexes that this variety of 
tone in the muscles is most easily measured. Their disappearance qr 
abolition is consequently an indication of the diminution or disappearance 
of the tone of the muscles concerned and not of their paralysis alone. 
The knee-jerks may disappear early in the toxic affections of the peripheral 
nerves, before there is any demonstrable evidence of either motor or 
sensory paralysis; and during recovery from such conditions, or after 
regeneration of degenerated nerves, they may be absent when the recovery 
of power is apparently complete. The diminution or loss of tone as 
measured by the deep reflexes is the most sensitive guide to any inter- 
ference with the normal function of the peripheral nervous system. 
Atrophy of the muscles supplied by the affected motor fibres is one of 
the most prominent symptoms; this is due to degeneration or regression 
of the muscle fibres owing to loss of the trophic influence which the motor 
nerves normally exert on them. It is recognizable by a diminution in 
the size of the muscle, and by its soft and structureless consistence. 
The latter symptom is frequently neglected, but it may prove valuable, 
especially in children, in whom it is not easy to determine the distribution 
of an atrophic paralysis by merely noting the movements which cannot 
be performed; here by touch alone we may pick out the affected muscles. 
Muscular atrophy is generally recognizable within two or three weeks 
after a complete interruption of a motor nerve, and increases rapidly 
from this time until few if any fibres remain. When the nerve lesion 
is incomplete the atrophy corresponds closely with the degree of paralysis; 
in other words, the conducting power and trophic functions of the per- 
ipheral motor nerves are lost together. When the lesion of the nerve is 
very slowly progressive the muscular atrophy is generally less prominent 
than the paralysis. DISEASES OE THE PERIPHERAL NERVES 
535 
Coincident with the atrophy of the fibres of the wasting muscle an 
increase and proliferation of its connective tissue occur. If the muscular 
atrophy attains severe degrees, and if regeneration does not soon set in, 
this new connective tissue slowly undergoes fibrosis and the muscle is 
converted into a firm, inelastic band. The contracting fibrous tissue may 
produce contractures and deformities. Further, for the recovery of 
function it is necessary that the muscle fibres should regenerate, and 
regeneration must be evidently seriously interfered with by the presence 
of dense and contracted fibrotic tissue. The condition of the muscles 
and the changes they undergo are therefore as important as the changes 
in the affected nerves. 
One of the most valuable signs in disease of a peripheral motor nerve is 
change in the electrical excitability of the nerve and of the muscles it supplies. 
Muscular contraction can be normally obtained by either faradic or 
galvanic stimulation of the motor nerve fibres which supply it. Two or 
three days after section of a nerve the excitability of its peripheral portion 
is diminished, and after a period of six to eight days the excitability is 
lost with the secondary degeneration of the nerve. The most character- 
istic changes are observed on direct stimulation of the muscle; they 
compose what is known as the reaction of degeneration (R. D.) and usually 
appear from the eighth or tenth day after section of the nerve. It must 
be mentioned that according to Sherren1 increased excitability to the 
galvanic current is found only when the lesion of the nerve is incomplete. 
After some months, if recovery has not set it, the reaction of the muscles 
to the galvanic current diminishes slowly and finally disappears. 
In every case of peripheral paralysis it is important to test the electrical 
excitability of every portion of the nerve and muscle that can be reached. 
It occasionally happens when there is a local lesion in the nerve that 
muscular contractions can be obtained by stimulation below the lesion, 
but not from above it; the lesion may block the conduction of impulses 
without leading to secondary degeneration of the peripheral portion of 
the fibre. On the other hand, regenerating nerves may be excitable only 
from above the lesion; the regenerated portion is for a time inexcitable 
to stimuli which it can conduct. This may depend on the absence of 
a myelin sheath (Erb). When the lesion is unilateral the excitability 
of both nerve and muscle may be compared with those of the normal 
side; when both sides are paralyzed the irritability should be compared 
with those of a normal person, or the tables prepared by Stintzing to 
show the normal limits of excitability may be used. 
Ghilarducci's distal reaction is also a valuable test; the indifferent 
electrode is placed on the neck or back, the other distal to the insertion 
of the degenerated muscle; then on closure of the current a contraction 
of this is obtained by a weaker current than suffices for the unaffected 
muscles. 
Symptoms of irritation of motor fibres are much less frequently met with 
in diseases of the peripheral than of the central nervous system. Spasm, 
either tonic or clonic, and cramps, when due to irritation of the peripheral 
injuries of Nerves, London, 1908. 536 
DISEASES OF THE NERVOUS SYSTEM 
neurones, are generally reflex in origin and arise from excitation of the 
sensory fibres. Occasionally, however, intermittent or tonic spasm 
may result from irritation of a motor nerve by a neighboring focus of 
inflammation or by a foreign body, but even here it is difficult to exclude 
its reflex origin. True reflex spasms are much more frequent; the 
involuntary facial movements which often accompany trigeminal neu- 
ralgia, and even irritation of the cornea, are of this nature. The fixation 
of painful joints by the tonic contraction of the surrounding muscles 
is also due to irritation of the sensory fibres which reflexly excite the 
corresponding motor neurones to excessive tonic activity; the sensory 
impulses may, if sufficiently intense, spread to wider reflex centres in 
the cord. The potency of sensory impulses from the periphery in the 
causation of spasm is illustrated by the severe spasms met with in 
strychnine poisoning and in tetanus; both these poisons only trans- 
mute an inhibitory effect into an excitation effect in the spinal reflex 
centres (Sherrington), and the spasms or convulsions are always directly 
due to a stimulus from the periphery. Cramp of a muscle may be occa- 
sionally due to influences that affect its fibres directly, as poisons like 
veratrine; venosity of the blood may also predispose to or cause spasms. 
Some forms of muscular atrophy are characterized by the occurrence 
of fibrillation or intermittent, more or less rhythmical wave-like contrac- 
tions of some of the fibres of a muscle; this is more probably due to 
direct stimulation of the fibres than to excitation of the motor nerves. 
Myokymia, a condition characterized by constant undulating or wave- 
like contractions of the muscle fibres which changes from place to place, 
has been regarded as a symptom of abortive neuritis, but is rarely seen. 
After complete division of a nerve and primary suture the time neces- 
sary for the return of power depends on the distance of the lesion from 
the periphery; Sherren lays down the rule for the upper extremities, 
that when a nerve is divided at the wrist perfect power may be regained 
within a year; but if at the elbow or in the plexus, not for two years. 
The muscles nearer the lesion regain their functions earlier than those 
distant from it. Recovery is slower after secondary than after primary 
suture. The muscular functions return much more rapidly after incom- 
plete nerve lesions. 
Sensory Symptoms.-When a sensory nerve or the sensory fibres of a 
mixed nerve are cut across we might expect to find absence of all sensation 
in the cutaneous and deep structures which are supplied by that nerve; 
but by the ordinary methods of testing this is rarely found, as the sensory 
loss which is revealed is generally much less extensive than the anatomical 
distribution of the nerve. This has been generally explained by assuming 
that the nerves overlap or anastomose, or that the neighboring end 
organs take up sensibility from the anesthetic region. But the explana- 
tion has been afforded by the brilliant work of Head and his colleagues.1 
These have shown that the afferent fibres may be divided into three 
systems. The first system subserves deep sensibility, which is conveyed, as 
Sherrington2 has shown, by the afferent fibres that run from the muscles, 
1 Brain, 1905, xxviii, 99. 
2 Phil. Trans. Royal Society, 1896. DISEASES OF THE PERIPHERAL NERVES 
537 
tendons, and joints, and which escape when only cutaneous sensory 
nerves are injured. Even when the muscular branches are involved this 
form of sensibility may not be quite abolished, as its fibres have wide 
anastomoses and often join the tendons and muscles high up in the 
limbs. Its function is the appreciation of pressure, of stimuli that pro- 
duce deformation of structure, and of any change in the position and 
condition of the joints and muscles. It is the system which subserves 
the sense of position. It is owing to the persistence of deep sensibility 
that the statement is often made that no sensory loss ensues on the 
section of a cutaneous nerve, as the area of its distribution remains sen- 
sitive to even light pressure by a finger or pencil point. 
The second system, to which the name protopathic is given, conveys 
painful cutaneous stimuli and the appreciation of the major degrees of 
temperature. But in the absence of the third system, the pain produced 
by a pin-prick or other means is not localized, but radiates widely over 
the affected area and causes an unnatural amount of discomfort and an 
almost uncontrollable desire to withdraw the part from the stimulus. 
Although it is through this system that major degrees of heat and cold 
are appreciated, minor degrees of temperature cannot be recognized 
when it is alone present, and the appreciation of degrees of heat and cold 
is lost. The third system, which has been called epicritic, responds to 
light touches and to the minor degrees of temperature, which produce 
the sensations called "warm" and "cool." It is only when epicritic 
sensibility is present that the point of skin touched can be accurately 
localized, and that two points at a normal distance apart can be dis- 
criminated when applied simultaneously. 
In the sensory disturbances that result from section of a peripheral 
nerve the different varieties of sensibility are lost according to their 
arrangement in these three systems, that is, sensibility to light touch 
and the minor degrees of temperature disappear together, and the appre- 
ciation of the major degrees of temperature with insensibility to painful 
cutaneous stimuli. 
When the condition of sensation on the hand is examined after section 
of the ulnar nerve it will be seen that there is complete loss of all forms 
of sensation only on the little finger and on a variable, but small, area on 
the ulnar border of the hand; that is, it is only here that both the proto- 
pathic and epicritic sensibilities are absent (Fig. 38), but on the rest of 
the cutaneous distribution of the ulnar nerve light touch and the inter- 
mediate degrees of temperature, the epicritic sensibilities, cannot be 
be appreciated, and although painful stimuli may be recognized they 
cannot be accurately localized, but radiate widely and give rise to un- 
natural discomfort. It is evident that though there may be a consider- 
able overlap between the fibres of the ulnar and median nerves which 
conduct protopathic sensibility, the loss of epi critic is practically limited 
by the anatomical boundary of the nerve distribution. With other 
nerves there may be an overlapping of epicritic sensation too. When 
the fibres are injured in a plexus the area of loss of protopathic sensibility 
may* almost equal in extent that of the epicritic, while if due to lesion 
of the dorsal spinal roots, the area insensitive to a pin-prick may actually 538 
DISEASES OR THE NERVOUS SYSTEM 
exceed that insensitive to light touch. The more closely a peripheral 
nerve represents the supply of one or more posterior roots the more 
nearly will the loss of protopathic coincide in distribution with the loss 
of epicritic sensibility. Head's conclusions have been contested by 
Trotter and Davies1 and others. 
When a nerve is not completely divided, or if only its functional con- 
tinuity is affected, as by bruising or compression, the condition of sen- 
sation is very variable. Sometimes every form of sensibility is lost for 
a time, but often pain perception remains intact and loss of epicritic 
sensibility may be the only sign of the injury; or after slight injuries 
there may be no absolute loss of sensation, though the patient may be 
conscious of an altered sensibility in the area of the nerve. 
The recovery of sensibility in the area of a divided nerve which has been 
sutured and is regenerating takes place in a definite and constant man- 
ner. The first change, generally observed after about two to three 
Fig. 38 
A, loss of sensation produced by division of the ulnar nerve; B, that due to division of the 
median nerve. The areas of complete cutaneous insensibility are marked black; the skin insen- 
sitive to light touch and the intermediate degrees of temperature are enclosed by the line. 
(After Head and Sherren.) 
months, is a gradual diminution of the area of total analgesia commencing 
in the proximal parts of the area, and recovery of appreciation of major 
degrees of temperature, while the area of insensibility to light touch 
remains unaltered. At this stage, generally completed in about six 
months, there is still absolute loss of epicritic sensibility, although all 
the forms of protopathic sensation have recovered. The diffuseness 
and radiation of pain produced by a pin-prick, or rough handling, or 
a blow is so unpleasant at this stage that the complete absence of sen- 
sation may appear preferable to the patient. The return of sensibility 
to light touch and the minor degrees of temperature, as well as the 
power of discriminating between one and two points and of accurately 
localizing stimuli, rarely commences earlier than six months, no matter 
how favorable the nerve union may be, and is seldom complete within 
a year; but even for years careful examination may detect abnormalities. 
1 Jour, of Physiol., 1909, xxxviii, 134. DISEASES OF THE PERIPHERAL NERVES 
539 
After incomplete nerve lesions, on the other hand, the appreciation 
of pain and of light touch return at approximately the same time; it may 
be taken as an absolute rule that if the simultaneous recovery of proto- 
pathic and epicritic sensibility is observed within a few months of the 
injury the nerve has not been completely divided. Recovery of function 
is also much more rapid than after complete division of the nerve. It 
commences at a date which varies with the distance of the injury from 
the periphery, from about three weeks at the wrist to six months in the 
plexus, and also with its degree. 
Symptoms of irritation or perversion of function of sensory fibres are 
often a prominent feature of disease or injury of the peripheral nerves, 
especially when the lesion is only partial or when the central stump of 
a divided nerve is involved in scar tissue. Pain is the most important 
of these subjective symptoms. Irritation of a nerve by pressure or 
disease may give rise to severe pain; this is undoubtedly due to the 
excitation of the sensory fibres by the lesion; the abnormal excitation 
thus produced is conveyed by the ordinary paths to the higher sensory 
centres of the cerebral cortex, where it reaches consciousness; its origin 
is, however, misinterpreted and is referred to the sensory end-organs 
of the irritated fibres, even although the area in which they lie be com- 
pletely anesthetic-anesthesia dolorosa. Pain of this origin is often 
paroxysmal, and is generally sharp, burning, or darting; at times, how- 
ever, it is of a dull aching or boring character; it may be referred to the 
skin or the deeper structures of the limbs. 
Hyperalgesia is met with in regions in which epicritic sensation is 
lost, while protopathic persists, but it often follows partial lesions where 
there is little or no disturbance of sensation. It not infrequently extends 
over the boundary of the anatomical distribution of the injured nerve, 
and is often associated with severe spontaneous pain. 
Weir Mitchell gave the name causalgia to a condition of severe spon- 
taneous pain associated with hyperalgesia and tenderness, and often 
with trophic disturbances. It is most frequently seen after bullet 
wounds; it never results from complete interruption of the continuity 
of the nerve, and always disappears immediately the nerve is cut across 
for secondary suture. In these cases it is probable that normal stimuli 
of peripheral origin are augmented in their passage through the injured 
region, or it may be that the sensory end-organs become hyperexcitable 
owing to a disturbance of their connection with their trophic centres. 
Hyperalgesia appears rarely if ever immediately after the nerve injury, 
generally not for a period of a few weeks. 
Pain is uncommon after nerve injuries by modern high velocity bullets; 
Olconomakes1 saw it very rarely accompanying peripheral palsies in 
the Balkan wars. 
Various paresthesias, or a feeling of loss of feeling, or of weight or 
pressure, or, it may be, a sense of warmth or coldness, are frequently 
due to affection of the peripheral nerves which supply the region to which 
they are referred; they are generally, but not always, associated with 
1 Neurolog. Centralblatt, 1914, xxxiii, 486. 540 
DISEASES OF THE NERVOUS SYSTEM 
some loss of sensibility. They are, on the whole, met with more fre- 
quently in the toxic degenerations of nerves than after traumatic or 
local lesions; they are probably due to a slight but persistent irritation 
of the sensory fibres. The most frequent form is generally described as 
a numbness or deadness of the part, but from the discomfort it causes 
it is evident that it is more than the consciousness of loss of feeling; 
it is rather an abnormal positive symptom, which may, even when there 
is no objective sensory disturbance, seriously interfere with the functions 
of the part. Even when paresthesias are due to the local injury of a 
nerve, they are rarely referred to the whole area of its anatomical 
distribution; in the limbs they tend to be more intense peripheralward. 
Occasionally the irritation of one of the terminal branches of a nerve 
gives rise to pain extending over its whole protopathic distribution, or 
that of its roots, which may be accompanied by hyperalgesia, or by 
spasm or contractures of the muscles supplied by it. Neurotomy of 
the irritated branch gives instant relief. 
Trophic and Vasomotor Symptoms.-The nutrition and conservation of 
all tissues of the body are to a certain extent dependent on the integrity 
of their connection with the central or autonomic nervous systems, and 
when this is interrupted the isolated tissues may undergo certain changes. 
The most prominent of these is the atrophy of the muscles which follows 
the degeneration of the motor nerve fibres which innervate them, but 
the skin, subcutaneous tissues, and even bone may also undergo struct- 
ural alterations. 
It was for long discussed whether the influence of the nervous system 
on the tissues is exerted through special trophic fibres, or through the 
ordinary motor, sensory, or vasomotor nerves. The muscles are cer- 
tainly dependent only on the integrity of their motor fibres, and the 
existence of special trophic fibres to other tissues has not been proved. 
The vasomotor nerves must, however, have a considerable influence 
on nutrition and growth; paretic vasodilatation follows section or injury 
of a nerve, and although there may be hyperemia and a slight elevation 
of the local temperature for a time, the part soon becomes cold, congested, 
and even cyanosed, owing to the slow circulation through the dilated 
vessels; and its inactivity leads to lymph stasis. 
It seems probable that the trophic centres for the skin and other tissues 
lie in the spinal ganglia, and that the centrifugal conduction of trophic 
influence is a function of the sensory fibres; or more probably that the 
normal nutritional equilibrium is in some way determined by the sensory 
nerves. The strongest evidence of the trophic function of the spinal 
ganglia is that the acute changes in the skin which characterize herpes 
zoster are directly due to disease of them. The innervation of the blood- 
vessels and of the sweat and sebaceous glands takes place through the 
sympathetic system; in the limbs, at least, these sympathetic fibres 
are intimately associated with those of motion and sensation. 
Trophic changes in the skin vary with the rate of evolution and the 
degree of the nerve lesion; the more acute it is the more likely are trophic 
changes to be prominent. They may be met with either in areas of total 
anesthesia, or associated with only partial disturbance of sensation. In DISEASES OF THE PERIPHERAL NERVES 
541 
areas of total analgesia the skin becomes thin and atrophic, and, especially 
in the hands and feet, inelastic and tightly stretched over the part; owing 
to the cessation of sweat and sebaceous secretion it is usually very dry 
and may be either glossy and shiny, or scaly when desquamation of the 
superficial layers of the epidermis is delayed. In this condition it is 
very liable to injury, and ulcers often develop, which, as a rule, first 
appear as blisters or bullae; these often appear to be the result of the 
trophic disturbance alone, as they may occur apart from any assignable 
local cause, or follow a slight bruise. Owing to the loss of sensation the 
skin is liable to suffer from neglected or unobserved injuries, and to this 
many of the trophic changes have been attributed. Head1 has observed 
that these affections are generally co-extensive with the area of complete 
analgesia, and disappear with the return of protopathic sensibility. 
Another form of cutaneous trophic disturbance which follows incom- 
plete lesions of nerves, and is always associated with spontaneous pain 
and hyperalgesia (causalgia), was first accurately described by Paget2 
and WeireMitchell,3 and by the former termed "glossy skin." It is seen 
chiefly on the fingers and hands; the skin becomes "smooth, hairless, 
almost devoid of wrinkles, glossy pink or ruddy, or blotched as if with 
permanent chilblains" (Paget). The subcuticular tissues shrink, and 
the skin, which appears tightly drawn over them, is often cracked and the 
epithelium is partially lost, so that the cutis is exposed in places. Accord- 
ing to Weir Mitchell, this condition is often attended with vesicles. 
Changes in the growth and condition of the nails often follow nerve 
injuries. Retardation of growth has been frequently described, but 
Head has shown that this is independent of senspry disturbance, and 
that want of movement owing to paralysis or the fixation of the limb 
is the main factor. Weir Mitchell observed remarkable alteration in the 
nails associated with glossy skin, which consisted of curving on their 
long axes, extreme lateral arching, and sometimes a thickening of the 
cutis beneath their extremities. 
When several nerves of a limb are damaged the bones are liable to 
become fragile, and if it occurs in early life their growth may be retarded. 
Acute and extensive nerve lesions may lead to swelling of and effusions 
into joints in the paralyzed regions. Another form of more chronic 
joint change occurs chiefly in the fingers and wrists; it commences with 
pain and periarticular thickening, and may ultimately produce ankylosis. 
The Treatment of Local Nerve Lesions.-This naturally depends on 
the nature and the degree of the lesion; but there are certain general 
lines which should be invariably followed. In every case the chief aims 
should be to maintain the nutrition of the parts, to keep the paralyzed 
muscles relaxed, and to prevent the occurrence of contracture. 
If the nerve has been completely divided, its two ends should be 
sutured together at once. The prognosis when primary suture is pos- 
sible is good, but it depends to a certain extent on the nerve injured, 
the nature of the lesion, the condition of the wound, and the distance 
of the injury from the periphery; the muscular functions generally 
1 Erain, 1905, xxviii, 251. 2 Medical Times and Gazette, 1864, i, 331. 
3 Gunshot Wounds and other Injuries of Nerves, Philadelphia, 1864. 542 
DISEASES OF THE NERVOUS SYSTEM 
return within nine months, but sensation is rarely perfect for two or 
three years. Often, however, the nerve injury is not observed, or the 
patient does not come under treatment until after a considerable time, 
then secondary suture must be performed. The outlook in these cases 
is not so favorable as after immediate suture, but the time after the 
injury at which the operation is undertaken, certainly up to three years, 
seems to have little influence on the recovery (Sherren). 
Much depends on the condition of the muscles; when thefee have 
entirely lost their galvanic irritability, complete recovery is probably 
impossible, and according to Warrington and Jones/ when the paralysis 
has lasted several weeks return of full power cannot be expe'cted unless 
paralyzed muscles have been kept relaxed. It is important that not 
merely should all scar tissue around the ends of the nerves be removed, 
but that these should be freshened up and brought into accurate apposi- 
tion. It is occasionally impossible to bring the divided ends together; 
then Assaky recommended imposing catgut threads between ^hem to 
form a scaffold along which the regenerating axis-cylinders jjiay £row, 
and Vanlair has introduced the method of interposing a tube of decalci- 
fied bone or a piece of a small artery or vein for the same purpose. Nerve 
transplantation, however, seems to give more favorable results in such 
cases, but, as Merzbacher5 has shown, the portion of nerve inserted can 
take an active part in regeneration only when it is taken from the same 
animal, or an animal of the same species; if taken from another species, 
it immediately necroses and can consequently at the most act only as 
a scaffold. In other cases it is preferable to anastomose the peripheral 
end of the paralyzed nerve into a neighboring healthy trunk, or end to 
end with a flap raised from the sound nerve. In every case the exact 
condition of the injured nerve should be examined under an anesthetic 
if there is complete reaction of degeneration in the muscles it supplies 
after fourteen days. 
Tendon transplantation is occasionally necessary, especially when there 
is such complete degeneration of the paralyzed muscles that recovery 
after secondary suture of the nerve cannot be expected. The results 
obtained are often favorable. It is interesting that after nerve anasto- 
mosis and tendon transplantation nerve centres in the cord can acquire 
new functions or adapt themselves to new conditions. 
Whatever surgical treatment is adopted it is important to keep the 
paralyzed muscles relaxed and prevent shortening and contracture of 
their antagonists. If the extensors of the wrist and fingers are paralyzed, 
their joints must be kept fully extended by a splint along the forearm and 
hand; if it is the flexors of the forearm, the elbow should be held flexed 
in a sling. But the splint or other apparatus used should be removed 
frequently, and massage and passive movements carried out. 
The next aim should be to maintain the paralyzed muscles in as good 
a state of nutrition as is possible, and for this electrical treatment is 
usually employed. If the paralysis is not complete, and if some excita- 
bility to the interrupted current remains, faradism may be employed, 
1 Lancet, 1906, ii, 1644. 
2 N eurologisches Centralblall, 1905, xxiv, 150. DISEASES OF THE PERIPHERAL NERVES 
543 
but it is of little use if the muscles will not react to it, although, according 
to Mann,1 regular treatment raises the excitability of both nerve and 
muscle and increases the flow of blood and lymph. Galvanism is more 
effective when the muscles will not react to faradism, but the current 
employed should not be too strong. 
Systematic and energetic massage is probably more effective than any 
form of electrical treatment, and should be combined with it, but the 
region of the nerve injury should not be forcibly handled. Passive move- 
ments are always advisable; and the patient should make efforts to use 
the paralyzed muscles as soon as any return of power sets in. 
DISEASES OF THE SPINAL NERVES. 
The Cervical Plexus.-Owing to its deep position among the muscles 
of the neck, the cervical plexus is rarely injured or affected by disease. 
The branch which is of most clinical importance is the phrenic nerve. 
Its paralysis may be due to a lesion in the ventral horns of the cord at 
the level of its origin, to an intraspinal hemorrhage or tumor, or to 
syringomyelia. Duchenne first described it in progressive muscular 
atrophy. It is most frequently caused by involvement of the third and 
fourth cervical roots in meningeal or vertebral disease, especially in 
spinal caries and syphilitic pachymeningitis. Owing to its deep position 
the nerve trunk is relatively immune from trauma, but it is occasionally 
injured by wounds or operations. It has been repeatedly observed after 
local anesthesia of the brachial plexus.2 Within the thorax it may be 
compressed by tumors, aneurisms or enlarged glands. Unilateral 
paralysis can be often attributed only to a local neuritis, perhaps fol- 
lowing exposure to cold. Bilateral palsy sometimes occurs in multiple 
neuritis, especially in that form which follows the acute infective dis- 
eases, in lead poisoning, and in tabes dorsalis (Gerhardt). 
The diaphragm is one of the most important of the respiratory muscles; 
when the patient is at rest its inactivity may give no trouble, but dyspnoea 
is easily produced by exertion or when respiration is interfered with 
by disease of the lungs or pleura. If bilateral paralysis sets in suddenly 
there may be considerable dyspnoea and even cyanosis for a time, but 
it is quickly relieved by the activity of the accessory muscles. Several 
cases have been recorded in which accidental injury of one phrenic 
nerve during operations in the neck has led to a fatal result, but Schroder 
and Green,3 who analyzed these cases, come to the conclusion that death 
was generally due to some other cause. 
When there is complete bilateral paralysis the upper part of the abdo- 
men is no longer protruded with each inspiration, but sinks in as the 
diaphragm is. drawn upward by the negative pressure in the thorax; 
on palpation the descent of the liver and spleen can be no longer felt, 
and the patient is unable to expand the abdomen by taking a deep breadth. 
The movements of the thorax are consequently often increased, and the 
1 Centralblatt f. Nervenheilkunde, 1897, xx, 1. 
2 Klauser, Centralbl. f. Chir., 1913, xl, 595. 
3 Am. Jour. Med. Sc., 1902, cxxiii, 196. 544 
DISEASES OF THE NERVOUS SYSTEM 
excessive movement of the lower part of the thoracic cage may draw the 
abdominal wall tense; this must not be mistaken for the effect of the 
descent of the diaphragm. If the paralysis is not complete the protru- 
sion of the abdomen in inspiration can be easily resisted by pressure. 
Owing to inability to take a deep breath there is difficulty in coughing, 
and the patient cannot spit out with the normal force nor sneeze properly, 
and there may be difficulty in defecation. Pulmonary symptoms are 
the most important complication, owing to the relative immobility of 
the bases of the lungs they may have become dangerously congested. 
The symptoms of unilateral paralysis are generally slight and fre- 
quently escape observation, but the deficient movement of one side 
can be definitely determined by a radiograph, or by the direct observa- 
tion of the movements. The electrical excitability of the phrenic nerves 
may be examined; they are easily stimulated in the neck between the 
sternomastoid and scalenus anticus muscles, and above the omohyoid. 
The phrenic nerves also convey sensory fibres to the pleura, pericardium, 
and diaphragm, but sensory symptoms have been rarely referred to 
their disease; in a few cases, however, patients have complained of pain 
in the mediastinum and in the region of the diaphragm. 
The diagnosis of diaphragmatic paralysis is not always easy; the 
examination of the electrical excitability of the phrenic nerves may be 
very important when other signs are not conclusive. In the majority 
of the cases the condition is only part of a general peripheral neuritis, 
or of poliomyelitis. Otherwise, bilateral paralysis is generally due to 
some disease in the spinal cord or meninges; it can then be rarely an 
isolated symptom. The vertebral column should be carefully examined, 
and the existence of tumors in the neck and mediastinum excluded. 
Paralysis must be distinguished from immobility of the diaphragm owing 
to diaphragmatic pleurisy or peritonitis, or to large pleural effusions. 
Or the diaphragm may be weak owing to secondary degenerative changes. 
Acute fatty degeneration of its muscle fibres frequently occurs in diph- 
theria, and may enfeeble, but rarely paralyzes the action of the muscle. 
Treatment.-This should be directed to the removal of the cause if 
it is possible, but it is rarely so unless it is due to an operable tumor 
either in the vertebral canal or neck. Otherwise the chief aim should be 
to avoid all pulmonary complications and save the patient from exertion. 
If there is reason to suspect a local neuritis, warm fomentations and 
counter-irritation may be applied over the lower part of the anterior 
triangle of the neck. Electricity, especially the faradic current, has been 
employed with apparently some effect; the one electrode should be 
placed over the phrenic nerve immediately behind the sternomastoid 
muscles in the neck, the other over the epigastrium. 
Long Thoracic Nerve.-Isolated paralysis of this nerve is rare, Stein- 
hausen1 was able to collect records of only 29 pure cases, it is more 
frequently found in association with palsy of other muscles of the shoulder- 
girdle, and it is often seen in progressive muscular atrophy and in the 
muscular dystrophies. The nerve may be injured in the neck by blows 
1 Deutsche Zeitschr. f. Nervenheilk., 1900, xvi, 399. DISEASES OF THE PERIPHERAL NERVES 
545 
or perforating wounds, or by direct pressure from a heavy weight carried 
on the shoulder; and it occasionally happens that it is bruised by the 
forcible contraction of the scalenus medius muscle through which it 
passes, or by excessive stretching of the nerve when the arm is raised 
above the head, as in painting a ceiling, climbing hand over hand, or 
hanging suspended by the arms. In the axilla it may be injured by a 
perforating wound or by operation. Paralysis of the serratus magnus 
has also been observed after infective disease, and with acute arthritis of 
the shoulder-joint. In some cases it has been apparently due to a local 
neuritis following exposure to cold. It is much more frequent in strong 
muscular men than in women and is more common on the right side. 
Its paralysis produces very little deformity while the arm is at rest; 
the scapula may stand slightly higher than normal, with its inferior 
angle slightly approximated to the vertebral column and separated from 
the chest wall. When the arm is moved forward into the horizontal 
position or pressed forward against resistance, the scapula, no longer 
held against the thorax by the serratus, is rotated on its vertical axis, 
so that its vertebral border projects backward and appears winged. 
This deformity is almost pathognomonic of serratus palsy. There is 
also difficulty in raising the arm above the horizontal, as while the arm 
is normally abducted from the side as far as the horizontal level by the 
deltoid alone, its further elevation is brought about by rotation of the 
scapula, chiefly by the serratus magnus. The latter part of the movement 
is consequently lost when this muscle is paralyzed, but not invariably, 
as it can be sometimes carried out by contraction of the middle fibres 
of the trapezius. There is often diminished muscular power in the whole 
arm, which disappears when the scapula is firmly bound to the trunk. 
Slight scoliosis is frequently observed in cases of serratus palsy; it is 
probably due to an attempt to reestablish the equilibrium upset by the 
malposition of the shoulder. When the paralysis is due to a neuritis 
its onset is often accompanied by severe neuralgic pains in the supra- 
clavicular region, which may radiate up the neck, behind the scapula 
and even into the arm, but no loss of sensation results from an isolated 
paralysis of the long thoracic nerve. 
Treatment.-This must be conducted on the usual lines; it is important 
to prohibit work or exercise which may produce pressure or strain on 
the nerve. When the palsy is incurable the humeral attachment of a 
portion of the percoralis major may be inserted into the serratus magnus. 
The Suprascapular Nerve.-Isolated paralysis, of it is very rare; 
Fischler1 was able to collect'only 14 cases. In half of these it was due 
to direct or indirect trauma, in others apparently to a local neuritis; it 
may be caused by the pressure of a heavy weight carried on the shoulder, 
or by a fall on the shoulder or the outstretched arm. Its most prominent 
symptom is flattening of the infraspinous fossa and weakness of out- 
ward rotation of the humerus owing to the atrophy and palsy of the 
infraspinatus; this movement is not, however, completely absent, as 
the teres minor and the posterior fibres of the deltoid can execute it, 
1 N eurologisch.es Centralblatt, 1906, xxv, 444. 
vol. v-35 546 
DISEASES OF THE NERVOUS SYSTEM 
but only very feebly. According to Duchenne, the chief function of 
the supraspinatus is to act as an elastic ligament in keeping the head of 
the humerus in close apposition to the glenoid cavity; when it is paralyzed 
the humerus falls away and the movements of the shoulder-joint are 
impeded, especially abduction and elevation in the sagittal plane. In 
every case of its paralysis the patient has complained of weakness and 
fatigue in the shoulder, and of the inability to carry weights. No definite 
disturbance of sensation has been detected with suprascapular palsy, 
but its onset may be accompanied by pain in the shoulder-girdle. 
The Circumflex Nerve.-Paralysis is most often due to such injury as 
a blow or fall on the shoulder, dislocation of this joint, or fracture of the 
upper end of the humerus. Occasionally it is caused by the pressure of 
a crutch, or by lying for long on the shoulder in deep sleep or in an 
unconscious state; in those cases in which paralysis follows prolonged 
operations under anesthesia the lesion is evidently due either to com- 
pression or undue stretching of the nerve. Similarly, paralysis of the 
circumflex has been frequently observed in miners who work lying 
constantly on the left side. Local neuritis may be due to exposure, or 
to extension of inflammation from arthritis or from disease in the axilla; 
neuritis has also been observed in infectious diseases and in diabetes. 
It is noteworthy that Bernhardt and Buzzard have observed isolated 
paralysis of the circumflex nerve in lead poisoning. 
A complete lesion of the nerve leads to complete paralysis of the deltoid, 
except of a small number of its anterior fibres, which are supplied by 
the anterior thoracic nerves. Abduction and elevation of the arm in 
any plane are consequently impossible, except by rotation of the scapula 
by the trapezius and serratus magnus. The supraspinatus may also aid 
in this movement. The shoulder-joint becomes relaxed, and owing to 
the wasting of the deltoid its shape is altered. The paralysis of the teres 
minor, which depresses the arm and rotates it outward, is less prominent. 
The onset is generally accompanied by much pain. Objective sensory 
disturbances are not constant, but in typical cases the area of anesthesia 
to light touch occupies an oval area on the outer side of the arm extend- 
ing from the level of the acromion process rather more than half-way to 
the elbow; the loss of sensibility to pain and the extremes of temperature 
is less extensive. When the paralysis lasts for long there is a danger of 
adhesions forming in the shoulder-joint, and ankylosis may occur. In 
obstinate cases good results have been obtained by transplanting the 
clavicular portion of the trapezius and pectoralis major into the deltoid, 
and by grafting the subscapular nerve into the circumflex.1 
A little care and the examination of the electrical reaction of the 
muscle serves to distinguish primary joint disease with the secondary 
wasting of the muscle from circumflex paralysis. 
The Musculocutaneous Nerve.-It is rarely paralyzed alone. Bern- 
hardt2 collected only 14 cases, but as it is not infrequently associated 
with lesions of other nerves it is important to recognize its symptoms. 
It is generally due to a blow on, or compression of the arm, or to fracture 
1 Langfellner and Frohse, Med. Klinik, 1909, v, 1270. 
2 Die Erkrankungen der Peripherischen Nerven, Wien, 1902. DISEASES OF THE PERIPHERAL NERVES 
547 
or dislocation of the humerus. When the lesion is complete the biceps 
and coracobrachialis are absolutely paralyzed, as well as the greater 
portion of the brachialis anticus. Flexion of the elbow is consequently 
impossible when the forearm is supinated, but it can be carried out 
feebly and in limited range by contraction of the supinator longus when 
the forearm is pronated. Sensory disturbance is limited to the radial 
side of the forearm and the thenar eminence. 
The Musculospiral or Radial Nerve.-This is probably paralyzed 
more frequently than any other nerve in the arm. It may be injured 
in the axilla by dislocation or fracture of the upper end of the humerus, 
or involved in callus formation or by the pressure of a crutch. It is 
occasionally compressed by the head of the humerus when the arm is 
kept fully abducted and extended during operations under anesthesia 
(Braun). But it is much more frequently injured during its course 
round the humerus, very often by the pressure to which it is exposed 
when a person sleeps on a hard or uneven surface with the arm beneath 
his body, or with the weight of the head resting on the outer surface of 
the arm. This occurs so often in a drunken sleep that is has been as- 
sumed that chronic alcoholism predisposes to it by lowering the vitality 
of the nerve (Oppenheim). Gowers has pointed out that it may be injured 
as it passes through the triceps by a sudden violent contraction of this 
muscle. Its paralysis has followed the use of an Esmarch's bandage on 
the arm, and it sometimes results from the injection of ether. A local 
neuritis due to cold is occasionally assumed to be the cause. When palsy 
develops during an acute illness it is more probably due to pressure as 
the patient lies in a semiconscious or delirious state. The affection of 
some of its fibres is a characteristic feature of lead palsy. 
Symptoms.-These depend on the site and severity of the injury. 
When the lesion is in the axilla all the muscles supplied by it are paralyzed 
and the patient is no longer able to extend the elbow and wrist, or the 
fingers or thumb at their basal joints, or to supinate the forearm except 
by the biceps. If the nerve is injured, as it is most frequently, on the outer 
side of the arm, the extensors of the elbow and more rarely the supinator 
longus escape, but wrist-drop, which is the characteristic feature of 
the palsy, is present. The thumb cannot be abducted or extended, 
but its other movements are intact. Although the flexor muscles are 
not affected, the hand-grasp is considerably weakened owing to the 
mechanical disadvantage at which they work when the wrist is not 
held straight by the normal action of their antagonists; with the hand 
passively extended its grasp is normal. As the arm hangs by the side, 
the forearm is generally pronated, and becomes more fully so when the 
hand grasps any object, owing to the unresisted pronation action of 
the flexors. The power of supination with the elbow extended is com- 
pletely lost, but when it is flexed the biceps can supinate the forearm. 
If the nerve is injured in the forearm, the supinators and even the exten- 
sors of the wrist may escape. Paralysis of the supinator longus produces 
slight weakness of flexion of the elbow. 
Sensory symptoms are very variable; with the onset there may be sub- 
jective sensations of numbness and tingling in its cutaneous distribution, 548 
DISEASES OF THE NERVOUS SYSTEM 
generally most pronounced on the radial border of the hand. In incom- 
plete lesions, the sensory fibres are, according to the general rule, much 
less affected than the motor, and even with complete paralysis of the 
muscle there may be no loss of sensibility; when present the anesthesia 
is generally most marked over the radial branch. Trophic changes are 
rarely prominent. In the pressure palsies there may be little or no 
atrophy of the paralyzed muscles. Occasionally a prominence develops 
on the dorsum of the hand, which is due either to the swelling of the 
sheaths of the extensor tendons (Gubler) or to overflexion of the carpus. 
There may be slight effusion into the carpal joints, and adhesions may 
form in them. The electrical reactions of the nerve and the muscles 
it supplies are extremely important; in the pressure palsies the nerve 
may be inexcitable from above the lesion, while in the portion below it 
and in the muscles it supplies normal responses can be obtained; the 
conduction of volitional and electrical impulses is thus interrupted by 
a lesion which is not of sufficient intensity to damage the continuity or 
vitality of the nerve fibres. If the lesion is more severe there must be 
partial or complete reaction of degeneration in the muscles it supplies. 
Paralysis of this nerve is generally easily recognized, but, as Gowers 
points out, the fact that it produces loss of extension of the limb at all 
its joints may lead to error, as palsy limited to a single function suggests 
central disease; the absence of sensory loss, and of change in the elec- 
trical excitability of the muscles may increase the risk of error. In 
lead palsy it is the musculospiral groups of muscles which are chiefly 
involved, but the affection is almost invariably bilateral, the onset is 
usually slow and unconnected with trauma, the supinator longus, as 
a rule, escapes, and the reaction of degeneration appears early in the 
paralyzed muscles. 
Treatment.-This must be conducted on the usual lines. In the 
pressure palsies the application of the galvanic current for twenty to 
thirty minutes at a time is of value; the cathodal electrode should be 
placed over the seat of injury, the anode distal to it, and the strength 
of current slowly increased until the patient feels it distinctly. 
The Median Nerve.-Owing to its deep position among the soft tissues 
of the arm this nerve is much less liable to injury than the musculo-spiral. 
In the axilla and arm it is usually injured by fractures and dislocations of 
the humerus and occasionally by the pressure of a crutch, but the most 
frequent cause of its paralysis is a wound on the palmar surface of the 
wrist. Occupation palsies frequently involve some or all of the hand 
muscles supplied by the median nerve; it has been repeatedly observed 
in laundresses, joiners, milkmaids, and cigarette makers. The exact 
nature of the lesion is doubtful; often it is undoubtedly due to a neuritis 
set up by pressure, but in other cases the muscular palsy and atrophy 
seem to be the direct result of the stress of overwork. Drummer's palsy 
affects chiefly the thumb muscles, but seems to be generally due to rup- 
ture of the tendon of the extensor longus pollicis (Heinicke1). 
When the nerve is damaged above the elbow the power of pronating 
1 Deutsche Zeitschr. f. Nervenheilk, 1913, xlvii, 229. DISEASES OF THE PERIPHERAL NERVES 
549 
the forearm is lost; flexion of the wrist is feeble and incomplete, and as 
it can be performed only by the ulnar flexor, the hand is strongly deviated 
to the ulnar side. Flexion of the interphalangeal joints is also lost except 
that of the distal phalanges of the two ulnar fingers, which can be still 
bent by the unparalyzed part of the flexor profundus. The flexion of 
the fingers on the metacarpus is unaffected, as it is performed by the 
interossei. The unopposed extensor action of the latter muscles at the 
interphalangeal joints may lead to their hyperextension. The thumb 
is kept extended and adducted by the muscles which remain, and it 
cannot be opposed nor its distal phalanx flexed; its metacarpal bone 
comes to lie in the same plane as that of the fingers, like the thumb 
in the ape. Owing to paralysis of these movements of the fingers and 
thumb there is considerable difficulty in firmly grasping any object and 
in employing the hand in any work. When the lesion affects the nerve 
in the forearm, after its branches to the pronators and flexors have 
been given off, pronation and flexion of the wrist and fingers may be 
intact; then it is chiefly the thumb movements which are lost. Bern- 
hardt and Head have pointed out that the branch which supplies the 
muscles of the hand may leave the main stem of the nerve in the lower 
part of the forearm and thus escape injury when the wound is at the 
wrist. When the lesion of the nerve is severe, prominent atrophy of 
the muscles of the thenar eminence results, and of the flexor surface of 
the forearm if it is above the elbow. There may be considerable pares- 
thesia in the cutaneous area of the nerve in earlier stages of the paralysis; 
the occupation palsies and neuritis are generally accompanied by trouble- 
some pain. A complete lesion of the nerve may lead to troublesome 
trophic disturbance of the skin and nails in the area where protopathic 
sensibility is lost, and to vasomotor paresis and cessation of sweating in 
the radial part of the palm. (Fig. 38 B, p. 538.) 
The Ulnar Nerve.-This may be injured alone or with other nerves in 
the axilla or upper arm by dislocation or fractures of the humerus, or in- 
volved in callus formation; occasionally it suffers in crutch palsy. It 
is much more frequently damaged at the elbow-joint by dislocations 
or fractures; occasionally ulnar paralysis develops slowly at long periods 
after elbow injuries owing probably to the pressure of fibrous adhesions 
or excess of callus on the nerve, and in a few cases it has been due to 
traumatic or synovial cysts in this region. More rarely the curious 
condition of dislocation of the ulnar nerve from its groove on the pos- 
terior surface of the internal condyle is met with; it probably occurs 
only when the internal condyle is badly developed. Paralysis due to 
direct pressure from without is rare; it is occasionally due to pressure 
on the elbow during sleep, especially in emaciated subjects, or during 
infective illness. According to Braun, however, these sleep palsies are 
to be attributed to pressure of the head of the humerus on the nerve 
in the axilla when the arm is abducted and extended. But undoubt- 
edly wounds in the wrist, which may either injure this nerve alone, or 
the median nerve and flexor tendons as well, are the most common cause 
of ulnar palsy. Primary neuritis is rare; a few cases of syphilitic 
neuritis are described, and leprosy has a predilection for this nerve. 550 
DISEASES OF THE NERVOUS SYSTEM 
When the nerve is injured at or above the elbow the power of flexion 
of the hand is very feeble, and when attempted the hand is deviated radial- 
ward by the radial flexor; the wrist becomes hyperextended when the 
fingers are straightened, owing to palsy of its ulnar flexor. The move- 
ments of the little finger are lost, the middle and ring fingers cannot be 
flexed at their distal joints, and owing to the paralysis of the interossei 
the basal phalanges of all the fingers cannot be flexed or the middle or 
distal phalanges fully extended. Adduction and abduction of the fingers 
are also impossible. When the injury is situated in the lower part of 
the forearm the fibres to the flexor profundus digitorum escape and the 
interossei and thumb muscles are alone paralyzed; then owing to the 
unopposed contraction of the long extensors and flexors the hand becomes 
claw-like-main en griffe-with the first phalanges hyperextended and 
the interphalangeal joints flexed. As the first two lumbricales escape, 
this deformity is generally not so pronounced as it is in progressive mus- 
cular atrophy (Gowers). Adduction of the thumb is also lost. The 
muscular atrophy which follows severe lesions of this nerve is very 
typical; the hypothenar eminence disappears, the palm becomes hollow, 
and the interosseal spaces sink in. When the nerve is severely 
damaged there is complete loss of sensation in the little finger and over 
a variable extent of the ulnar border of the hand; on the ring finger and 
the rest of the ulnar cutaneous distribution sensibility to light touch and 
the intermediate degrees of temperature are alone affected. As the 
dorsal cutaneous branch separates from the nerve in the middle third 
of the forearm it may escape in wounds at the wrist. Dupuytren's 
contracture of the palmar fascia has been observed after neuritis of the 
ulnar nerve, and de Leon1 has described contractures of the ulnar portion 
of the flexor profundus digitorum, owing to which the three ulnar fingers 
were bound down in the maximal flexion position. 
The chief danger of error in diagnosis is of confusing central and per- 
ipheral lesions, as the ulnar nerve contains almost all the root fibres of 
the eighth cervical and first thoracic segments of the cord. 
Paralysis of the Brachial Plexus.-The brachial plexus is formed by 
the anterior primary divisions of the lower four cervical and the first 
thoracic roots; it contains all the fibres which supply the muscles of the 
shoulder girdles and upper extremities, as well as the sensory fibres to 
almost the whole of the arm. By division and secondary anastomosis 
of these roots the three main cords of the plexus are formed. The outer 
cord receives the ventral trunks of the fifth, sixth, and seventh cervical 
roots; the inner contains the ventral trunk of the eighth cervical as 
well as the whole of the first thoracic root, and the posterior cord is 
made up of the dorsal trunk of the four lower cervical roots. This is 
the most common form of the brachial plexus, but it is liable to varia- 
tion, for the fibres concerned in any single function do not constantly 
leave the cord by the same roots, but although fibres may alter their 
position relative to the vertebral column, they always maintain their 
position in relation to other fibres (Herringham). The whole plexus may 
1 Nouvelle Iconographie de la Salpetriere, 1901, xiv, 409. DISEASES OP THE PERIPHERAL NERVES 
551 
be shifted up-high or prefixed type-or downward-low or post-fixed 
type-and the extreme variations between the high and low form of 
plexus may amount to nearly a whole root (Harris).1 Paralysis may be 
due to injury or disease of the nerve fibres in the roots, in the plexus, 
or in the nerve trunks and branches. Two types of plexus paralysis 
merit special description. 
Duchenne-Erb Palsy, or the Upper Arm Type.-In this form the deltoid, 
biceps, brachialis anticus, and supinator longus are generally paralyzed 
together, and frequently also the supinator brevis and supra- and infra- 
spinati; more rarely other muscles of the shoulder-girdle, and the radial 
extensor of the wrist and the pronator radii teres. The lesion to which 
it is due involves the fifth and sixth cervical roots either before or imme- 
diately after their union, or occasionally the fifth root alone; when 
Fig. 39 
A diagram of the brachial plexus and its connections with the spinal cord. A, represents the 
usual seat of the lesion in the upper arm (Duchenne-Erb) type of plexus paralysis; B, that in the 
lower arm or Klumpke type. Symp. is the Ramus communicans from the first thoracic root which 
carries the oculo-sympathetic fibres. 
caused, as it frequently is, by a blow or fall on the shoulder, it has been 
assumed that these roots were compressed between the clavicle and the 
transverse processes of the lower cervical vertebrae, or the first rib, but 
it seems more probable that the lesion is a rupture of some or all of the 
fibres of this portion of the plexus owing to excessive stretching and 
tension. In many cases at least the root fibres themselves are ruptured, 
or are torn out from the spinal cord. This may be due to any cause 
which increases the distance between the shoulder and the head and 
neck. The vulnerability of the two upper roots of the plexus to this form 
of trauma is explained by the fact that they bend downward imme- 
diately on their exit from the spine, so that the extravertebral portion 
1 Journal of Anatomy and Physiology, 1904, xxxviii, 399. 552 
DISEASES OF THE NERVOUS SYSTEM 
of each root forms an angle open downward with the portion which lies 
in the intervetebral foramen, while in the lower two roots this angle is 
open upward and in the seventh root there is little or no bend. (Fig. 39.) 
In severe cases the arm hangs by the side and cannot be abducted 
because of the paralysis of the deltoid, or rotated outward at the shoulder 
if the infraspinatus is affected. Flexion of the elbow is impossible owing 
to the paralysis of the biceps, brachialis anticus and supinator longus, 
or it can be effected through a small range by the pronator radii teres 
and the flexors of the wrists. Supination of the forearm is always 
weakened by the loss of power in the biceps and it may be impossible 
if the supinator brevis is also affected. Sensory symptoms are absent 
in the slighter cases, but there may be paresthesia and loss of sensation 
on the radial side of the arm and forearm. Even complete section of 
the anterior primary division of the fifth, and sometimes of the fifth and 
sixth roots may produce no sensory loss (Sherren). Erb has shown that 
all the muscles paralyzed in this type of plexus palsy may be made to 
contract by electrical stimulation over a point in the neck 3 cm. lateral 
to the sternomastoid and the same distance above the clavicle; the 
loss of excitability of the nerve trunks from this point is an important 
sign of the upper arm type of plexus palsy. 
Klumpke Palsy, or the Lower Arm Type.-This form of paralysis, in 
which the eighth cervical and first dorsal roots are involved, is generally 
met with only as the residue of a more extensive lesion, but it may be 
due to compression by a tumor in the neck or a growth in connection 
with the lung or vertebral column. Owing to the deeper position of 
these roots they are rarely injured by trauma. The palsy is characterized 
by an atrophic paralysis of the intrinsic muscles of the hand and generally 
of some of the forearm muscles, especially the flexors, with a certain 
amount of loss of sensation on the ulnar fingers and the ulnar border of 
the hand and forearm. Frequently, too, there are pupillary symptoms 
due to injury of the sympathetic fibres which leave the cord in the first 
thoracic root; but as these branch off from the root immediately outside 
the intervertebral foramen, they are affected only when the inner portion 
of the root is damaged. When they are injured, the palpebral fissure 
is narrowed, the pupil contracted, and there may be a slight degree of 
exophthalmos on the same side as the arm palsy. Vasomotor paresis 
on the same side of the face occurs probably only when the second and 
third thoracic roots are injured (Klumpke). When the small hand 
muscles are alone paralyzed, the typical deformity of claw-like hand 
results; if the flexors of the fingers and wrist are also powerless, the 
wrist may be hyperextended owing to contracture of the extensors. 
These traumatic plexus palsies are frequently followed by severe 
spontaneous pain in the arm, probably owing to the constriction of the 
nerve fibres in scar tissue, and often by trophic disturbances. 
Birth Palsies.-Birth palsies due to injury of the brachial plexus gener- 
ally belong to the Duchenne-Erb or upper arm type, and the limb 
cannot be adducted or rotated outward at the shoulder, or flexed or 
supinated at the elbow. The paralysis is often more extensive, and occa.- 
sionally it is one or more of the lower roots which is damaged. The 553 
DISEASES OF THE PERIPHERAL NERVES 
causation of these birth palsies has been much discussed; for the purpose 
of treatment it is important to recognize the nature of the lesions and 
to what they are due. Fracture or separation of the epiphysis of the 
humerus, dislocation of the shoulder, and fracture of the clavicle are 
occasionally found associated with birth palsies, but they are rarely the 
direct cause. In some cases injury of the plexus may be due to the 
direct pressure of the accoucheur's finger in the axilla or supraclavicular 
fossa, or to the blade of the forceps in the latter region; or the roots 
are compressed between the clavicle, which is drawn backward by the 
elevation and retraction of the shoulder, and the transverse processes 
of the lower cervical vertebrae or the first rib. It is most probable that 
the majority of these birth palsies are due to laceration or rupture 
of the fibres of the nerve trunks or roots by excessive stretching and 
tension or by the forcible deviation of the head and neck away from 
the shoulder which is temporarily fixed (Fairbank1). Clarke, Prout, 
and Taylor2 found rupture of the sheath and hemorrhages into the 
nerve, with secondary cicatricial contraction, in cases which were operated 
upon; complete rupture of the fifth root alone has been observed, but 
in most cases the lesion is found at the junction of the fifth and sixth 
roots. The sensory disturbances are slight and generally unimportant, 
but marked trophic changes may result. The paralyzed muscles atrophy 
quickly and lose their electrical excitability. 
Prognosis.-The outlook in the plexus palsies naturally depends very 
largely on the nature of the lesion, but it is much less favorable than in 
disease of the peripheral nerves. Only six of the twenty-three cases 
recorded by Bruns recovered, and eleven of the thirty reported by War- 
rington and Jones.3 On the other hand Fairbank believes the majority 
of birth palsies recover. Many of the cases of neuritis, but these are 
rare, and of those in which the loss of conductivity of the fibres is due 
to pressure or bruising, improve or make complete recoveries; but when 
a root or trunk is torn or ruptured the prognosis is very grave unless 
surgical treatment can be successfully adopted. The ruptured fibres be- 
come enveloped in cicatricial tissue, and the cells of origin of the fibres 
in the ventral horns of the cord are liable to degenerate completely when 
their axis-cylinders are forcibly torn out. The prognosis is consequently 
less favorable in cases of rupture due to indirect violence than when the 
plexus is damaged by a penetrating wound, and the chances of recovery 
diminish the nearer the lesion is to the spinal cord. 
Brachial Neuritis.-This is a form of neuritis limited to the brachial 
plexus which is closely analogous to sciatica; it seems relatively rare. 
It occurs generally in late life and in females more often than in males. 
The pain, which is often sudden in onset, is generally of great severity 
and is at first referred to the back of the scapula, the forearm or hand, 
or to the region of the plexus itself above the clavicle or in the axilla. 
As a rule, it is at first intermittent, but soon becomes more continuous 
with proxysmal variations, which occur spontaneously or are induced 
by movement. When fully developed the pain may spread over the 
1 Lancet, 1913, i. 2 Am. Jour. Med. Sc., 1905, cxxx, 670. 
3 Lancet, 1906, ii, 1644. 554 
DISEASES OF THE NERVOUS SYSTEM 
whole arm; it is usually dull and aching, but in the severer attacks it 
is a sharp, lancinating or stabbing. It may be accompanied by undue 
sensitiveness or hyperalgesia of the skin, and perhaps with tingling and 
numbness, but definite objective change in sensation is rare. The muscles 
may present slight wasting and loss of power, but it is more apparent 
than real, as the patient is reluctant to move the limb owing to the pain 
which ensues. Adhesions often develop in the joints and trophic changes 
in the skin and subcutaneous tissues. The condition can be mistaken 
for neuralgia; the chief points of distinction are the persistent tenderness 
of the nerves, and the influence of movement on the pain. There seems 
to be occasionally a danger of mistaking brachial neuritis for pain of 
angina pectoris or that due to an aneurism, and especially for arthritic 
changes in the shoulder-joint. The chief aim in treatment should be 
to avoid movement or anything which may excite pain. The disease is 
obstinate, and complete recovery cannot be always expected. 
Treatment.-In neuritis counter-irritation may be applied over the 
plexus. It is much more important to maintain the nutrition of the 
paralyzed muscles by electrical (galvanic) treatment and massage, and 
to prevent the formation of adhesions in the joints and of contracture 
in the muscles. Warrington has laid emphasis on the importance of 
preventing the overstretching of the paralyzed muscles by the unre- 
strained contraction of their antagonists, and Fairbank recommends 
strongly the use of a splint by which the arm may be held abducted from 
the side and elevated. When there is evidence that the nerves have 
been ruptured, or that the damaged fibres are enveloped in cicatricial 
tissue operative interference becomes necessary. If there is no return of 
power within nine or twelve months of the injury in a case which has 
had proper treatment, it is justifiable and generally advisable to explore 
the plexus. The results of surgical treatment have been less favorable 
in cases of birth palsy than in the traumatic palsies of the adult, but a 
considerable number of cases of both varieties have been reported in 
which more or less complete recovery has followed secondary suture or 
grafting of the nerves. Winnen1 found that recovery or some improve- 
ment occurs in 70 per cent, of the operated cases. 
Symptoms Due to Cervical Ribs.-Supernumerary cervical ribs are 
not rare; they are generally bilateral but in the majority of the cases 
the symptoms they produce are limited to one side. It has been gener- 
ally assumed that these are due either to abnormal stretching, or to 
compression of the lower roots or the lower cord of the plexus as these 
pass over the rib, but Sargent2 believes that the nerves are in closer 
relationship to the fibrous band which connects the free end of the rib 
with the sternum or with the first thoracic rib, and that they are damaged 
by this as the rib rotates in the respiratory movements. Movements 
of the arm may also increase the compression of the nerves by this band. 
Many persons with cervical ribs never develop any characteristic symp- 
toms, probably due to the fact to which Eisler has drawn attention, that 
cervical ribs are generally associated with a plexus of the prefixed type 
1 Deutsche Zeitschr. f. Chir., 1911, cxxiii, 903. 
2 Proc. Roy. Soc. of Med., 1913, vi, Clin. Sect., 117. DISEASES OF THE PERIPHERAL NERVES 
555 
which receives few or no fibres from the first dorsal root. On the other 
hand cases with the characteristic symptoms of cervical ribs occur in 
which no supernumerary ribs exist, and in which the clinical disturb- 
ances are due to the pressure of a normal or underdeveloped first thoracic 
rib on a postfixed plexus. The determining factor is consequently a 
disturbance in the relative development of the rib elements and the 
formation of the plexus (Wood Jones1). 
The vascular symptoms are also generally assumed to be due to com- 
pression, elevation and stretching of the subclavian artery by the rib, 
but Todd2 believes they must be attributed to compression of the sym- 
pathetic vasomotor fibres in the lowest cord of the plexus. 
The shape and direction of growth of the rib are of more importance 
than its size; those that grow directly outward are not so liable to cause 
symptoms as those that project downward and forward into the pos- 
terior triangle of the neck, and which may consequently seem relatively 
insignificant on a-ray examination. 
Fig. 40 
To show wasting of the thenar muscles in a case of cervical rib. 
The symptoms are much more common in females and generally appear 
first in early adult life though the ribs exist from childhood; this late 
development depends on the fact that as growth proceeds the shoulders 
drop, and more in females than in males, and that consequently the 
tension on the affected roots increases. The more energetic use of the 
upper limbs in adult life is also an important factor. The more frequent 
occurrence of the symptoms in the right arm than in the left, and the 
considerable relief that is often obtained by raising the shoulder, are to 
be explained by this factor. 
The first symptom, and the only one in many cases, is almost invari- 
ably pain in the arm, which is generally located in the ulnar border of 
the forearm, hand and fingers, but is occasionally referred to the radial 
aspect of the limb or to all the fingers; it is always increased by vigorous 
or continued movement of the limb. It may be associated with numbness 
and tingling, or a feeling of coldness in the same areas, usually most 
pronounced in cold weather. In the majority of the cases some loss of 
1 Proc. Roy. Soc. of Med., 1913, vi, Clin. Sect., p. 97. 
2 Jour. Anat, and Physiol., 1911, xlvii, 250. 556 
DISEASES OF THE NERVOUS SYSTEM 
sensation eventually appears in the distribution area of the first thoracic 
root, or on the areas of the first thoracic and eighth cervical roots, that 
is on the ulnar border of the forearm and hand, and in the two ulnar 
fingers. As is characteristic of root lesions, there is generally a certain 
amount of dissociation of the various forms of sensation, the tactile loss 
being less pronounced than the analgesia, while the thermal sense seems 
to suffer most severely. In many cases there is little or no objective 
sensory disturbance. 
A feeling of weakness or uselessness of the limb is often one of the 
earliest symptoms, but definite motor paralysis is much less common than 
the sensory disturbances. After a variable period, during which the pain 
has been more or less constant, the hand generally becomes weak and 
its intrinsic muscles, especially those of the thenar eminence, atrophy 
and lose their normal electrical reactions. In other cases there is a general 
wasting of the small hand muscles, and the hand may thus assume the 
main en griffe position. The flexors of the fingers, and to a less extent 
those of the wrist, may be also affected. Spasm of the flexors of the 
fingers and probably of other muscles frequently occurs when the palsy 
is not advanced. Clonic twitching has been observed. 
The almost constant absence of oculo-pupillary symptoms, despite 
the paralysis of the first thoracic nerve, indicates that the lesion is 
situated outside the intervertebral canal where the sympathetic fibres 
branch off from the root. Sympathetic ocular symptoms have, how- 
ever, been observed. The vascular symptoms, which are rarer, are 
inequality of the radial pulses, cyanosis and coldness of the limb, but 
arterial thrombosis and even gangrene of the fingers have been observed. 
The cervical rib may be often palpated in the neck, and there is generally 
a point of tenderness to pressure over it. Cervical scoliosis convex toward 
the rib was present in 22 of 61 cases collected by Schonebeck. 
The diagnosis must depend largely on a radiographic examination, 
by which the presence of abnormal ribs may be easily revealed; but it 
must be remembered that cervical ribs often produce no symptoms and 
that other nervous diseases are occasionally associated with them, espe- 
cially syringomyelia, which has been found in several cases. Cervical 
ribs should be suspected in every case of isolated paralysis of the 
first thoracic, or of paralysis of the first thoracic and eighth cervical, 
roots, which cannot be otherwise explained. The presence of vascular 
symptoms, as inequality of the radial pulses, is of considerable value. 
The only rational treatment is the removal of the supernumerary rib; 
the operation is attended with little danger, though more extensive 
paralysis has resulted from operative interference with the plexus. Of 
87 cases collected by Streissler1 77 per cent, recovered and an additional 
13 per cent, improved. The pain is almost invariably relieved by re- 
moval of the rib, but it may disappear if the limb is supported or kept 
at rest. The recovery of the muscles is slower and frequently incomplete. 
Nerves of the Lower Limbs.-These are much less frequently involved 
in injuries or affected by disease than those of the upper extremities. 
1 Ergeb. d. Chirurg. u. Orthoped., 1913, v, 280. DISEASES OF THE PERIPHERAL NERVES 
557 
The Anterior Crural Nerve.-Isolated palsy of this nerve is rare, but 
it may be due to compression by abdominal growths or by a psoas abscess, 
or to injury by fractures of the upper end of the femur or of the pelvis, 
or disease of these bones. Primary local neuritis is seldom seen, but 
occurs occasionally in diabetes. This nerve, either alone or with the 
obturator, may be injured during parturition; this probably occurs 
with greater frequency than is recognized, owing to the rapidity with 
which it recovers from slight compression and to the fact that its symp- 
toms may not be noticed while the patient is confined to bed. Ernst 
has observed 30 cases in 800 births. The psoas muscle is paralyzed only 
when the nerve is damaged in the immediate neighborhood of the lumbar 
plexus; when the lesion is situated here the thigh cannot be flexed on 
the abdomen, and if paralysis is bilateral the trunk cannot be flexed 
on the thighs when these are fixed; the patient is consequently unable 
to rise from the supine position. When the lesion is in the intra-abdomi- 
nal portion of the nerve the iliacus alone is paralyzed, and flexion of 
the hip is only wTeak. The most prominent symptom is paralysis of the 
extensors of the knee, and the absence of the knee-jerk in the affected 
limb. Paralysis of these muscles does not make standing or walking 
impossible, but contraction of its flexors must be avoided, as the patient 
cannot resist flexion or actively straighten the joint. The paralysis of the 
pectineus and sartorius does not produce any other prominent symptom. 
Its sensory branches arise in the upper part of the thigh; the middle and 
internal cutaneous are distributed to the lower two-thirds of the front and 
inner side of the thigh, the internal saphenous to the front and inner side 
of leg, and the inner side of the dorsum of the foot. Disturbance of sen- 
sation, paresthesia, or radiating pains may be present over these areas 
when the nerve is injured at or above the level of the groin. 
The diagnosis is, as a rule, evident; the only risk is of confusing the 
marked atrophy of the quadriceps extensor group on the front of the 
thigh w'hich occasionally results from inflammation of the knee-joint 
with atrophy of these muscles, due to a nerve lesion; but in the former, 
although the electrical excitability of the muscles may be diminished, 
there is no reaction of degeneration. The ordinary lines of treatment must 
be followed; good results have been obtained by grafting the tendon 
of one of the flexors of the knee into the tendons of the quadriceps 
extensor when the paralysis is permanent. 
The Obturator Nerve.-This is still more rarely injured alone. It may 
be injured during parturition, by intra-abdominal or pelvic growths, or 
by an obturator hernia. When the muscles it supplies are completely 
paralyzed the limb cannot be adducted, and although it can be raised 
by flexion of the hip, it cannot be thrown across its fellow when the patient 
is seated on a chair. Owing to paralysis of the obturator externus, 
outward rotation is enfeebled, and inward rotation of the thigh is also 
weak, as the adductor magnus is paralyzed. Gait is not seriously inter- 
fered with. The disturbance of sensation is limited to a small area on 
the inner side of the lower half of the thigh. 
Meralgia paresthetica is a condition characterized by paresthesia and 
pain, usually with slight objective disturbance of sensation, in the region 558 
DISEASES OF THE NERVOUS SYSTEM 
supplied by the external cutaneous nerve on the front and outer side 
of the thigh. It occurs chiefly in middle-aged men, less frequently in 
women. Its etiology is obscure; in many cases there has been a history 
of trauma; probably the long course of the nerve through the fascia 
predisposes to its injury. In one case a localized perineuritis was found 
but in other specimens there was no abnormality. In about one-sixth 
of the cases reported the condition was bilateral. In 1900 Schlesinger1 
analyzed 122 recorded cases, and in the same year Musser and Sailer2 
added ten- personal observations in a valuable contribution. The con- 
dition has been associated with flat foot. The symptoms vary greatly 
in intensity but the most common complaint is of abnormal sensations, 
of numbness, coldness, or tingling, on the front and outer surface of 
the thigh. In other cases pain is the chief symptom; it may be very 
severe, but is usually felt only after walking or standing; it is probably 
due to constriction of the nerve as it passes through the deep fascia 
when the latter is tense. Occasionally it persists even when the patient 
is lying down. In the majority of the cases there is a point of tenderness 
just below the anterior superior iliac spine where the nerve pierces the 
fascia. Objective sensory disturbances are very variable, but often 
considerable; occasionally there is hyperesthesia. The symptoms are 
very intractable; rest may be necessary when there is much pain, and 
massage and the faradic brush over the course of the nerve may give 
relief. Resection of the nerve has cured some cases, but in others the 
pain has returned (Bramwell). 
Similar symptoms have been occasionally observed in the distribution 
of the middle cutaneous branch of the anterior crural nerve, but generally 
associated with meralgia paresthetica. Lasarew,3 who has found the 
condition isolated, has given it the name meralgia paresthetica 
anterior. 
Gluteal Nerves.-Isolated paralysis of the superior gluteal nerve is 
uncommon; the muscles it supplies are the chief abductors and inward 
rotators of the thigh, and when they are paralyzed these movements 
are weak or lost. As the posterior fibres of the glutei rotate the limb 
outward, this movement becomes weak. The inferior gluteal nerve is 
rarely paralyzed alone; when this occurs the thigh cannot be forcibly 
extended, nor the trunk straightened on the thigh when the lower limbs 
are fixed. Standing and walking on a level are not seriously interfered 
with, but the limb is of little use in ascending steps, and the patient 
has difficulty in rising from the sitting position. 
The sciatic nerve supplies motor fibres to the ham-strings and to all 
the muscles below the knee, as well as the skin in the outer side of the 
leg and the whole of the foot except a small part of the inner portion of 
its dorsum. Its main terminal branches are the external popliteal or 
anterior tibial nerve, and the internal popliteal or posterior tibial nerve; 
these generally separate in the popliteal space, but are sometimes dis- 
tinct from their origin in the plexus. 
1 Centralbl. f. d. Grenzgeb. d. Med. u. Chir., 1900, iii, 241. 
2 Journal Nerv. and Ment. Dis., 1900, xxvii, 16. 
3 Deutsch. Zeit. f. Nerven., 1908, xxxiv, 154. DISEASES OF THE PERIPHERAL NERVES 
559 
The External Popliteal Nerve may be injured by direct trauma in any 
part of its course, but it is especially liable to be bruised by a blow 
or by pressure as it bends round the fibula. It has been occasionally 
torn or ruptured by violent extension of the limb, and paralysis has 
been frequently observed in laborers who work in a kneeling or crouch- 
ing position; there it is probably due to compression of the nerve between 
the fibula and the tense tendon of the biceps cruris. A primary neuritis 
of this nerve is by no means rare, and even in a general neuritis its fibres 
seem especially liable to degeneration. Lead palsy is occasionally limited 
to its distribution, especially in children (Putnam), but in these cases 
the tibialis anticus escapes as a rule. Isolated paralysis of this nerve 
is sometimes seen in tabes dorsalis. 
The whole limb must be unduly raised, as it is brought forward in 
walking, to enable the toes to clear the ground. Inversion and eversion 
of the foot are also weakened when there is a total paralysis. When the 
tibialis anticus is alone paralyzed the foot can be still flexed by the long 
extensors of the toes, but it is at the same time abducted; while if the 
function of the latter muscle alone is lost the active tibialis anticus inverts 
and adducts the foot. As Bernhardt points out, it not infrequently 
happens that the tibialis anticus escapes when the other muscles of this 
group are paralyzed. The paralysis of the peroneus longus is most evident 
when the ankle is extended, as the foot is then so strongly inverted by 
the unopposed action of its extensors that its outer border rests on the 
ground, and, as the inner part of the foot is not supported during exten- 
sion, flat foot may develop. When all these muscles are paralyzed talipes 
equinus gradually develops, owing to contracture of their antagonists; 
if the paralysis of all the muscles is not equal in degree the foot may be 
at the same time either inverted or everted, according to the degree of 
the paralysis of the muscles with the opposing function. The toes may 
be permanently flexed by the contracture of the unopposed flexors and 
interossei. The sensory loss which results from a lesion of this nerve is 
limited to the outer side of the leg, the dorsal surface of the foot, and 
the dorsum of the first phalanges of the toes. 
The Posterior Tibial or Internal Popliteal Nerve.-Owing to its deeper 
course it is less liable to injury than the external popliteal, and its 
isolated paralysis is consequently much rarer. A few cases have been 
recorded in which it has been injured by the tendons of the flexors of 
the knee when these muscles are forcibly contracted (Oppenheim), 
and it may be compressed by or involved in tumors, aneurisms, or 
inflammations. 
The most prominent feature is inability to extend the foot or flex the 
toes, so that the patient can no longer stand on tip-toe or spring from 
the forepart of the foot in walking; if the paralysis is of long duration 
the unantagonized action of the flexors of the ankle produces talipes 
calcaneus, while the unopposed action of the peroneus longus leads to 
eversion of the foot and increases the plantar arch. Flexion of the distal 
and middle phalanges of the toes is no longer possible, owing to paralysis 
of the long flexors, while the loss of the interossei and of the adductors 
and abductors of the great and small toes makes lateral movements 560 
DISEASES OF THE NERVOUS SYSTEM 
impossible. The unopposed contraction of the long extensors may lead 
to permanent overextension of the basal phalanges. 
When the conduction of sensory impressions is completely interrupted 
there is loss of sensation on the outer side and back of the lower third 
of the leg, on the outer border of the foot and on the sole and plantar 
surfaces of the toes, as well as in the dorsum of the distal phalanges. 
There may be trophic disturbances and ulcers may form. 
Paralysis of the Main Trunk of the Sciatic Nerve may be produced by 
fractures of the pelvis or of the upper end of the femur, or by dislocations 
of the hip-joint; or the nerve may be compressed by tumors in the pelvis 
or invaded in the extension of septic processes from the surrounding 
tissues. Some or all of the fibres may be paralyzed during parturition, 
but the lesion is then generally situated in the lumbosacral plexus; the 
sciatic nerve of the child may be injured by traction on the leg in breech 
presentations. The symptoms of complete paralysis by a lesion near the 
sciatic notch are those of paralysis of its terminal branches, the internal 
and external popliteal nerves, with, in addition, palsy of the flexors of the 
knee. When the latter are powerless the limb must be held extended 
at the knee in walking, and it can be used only as a stilt; there is no 
power of movement at the ankle-joint. As all the sensory fibres enter 
its terminal branches, the loss of sensation in a complete sciatic palsy 
includes the outer side of the leg and the whole of the foot except a small 
area in the inner side of its dorsum. 
The Lumbar and Sacral Plexuses.-Isolated paralysis of the lumbar 
plexus is extremely rare and merits no further reference, but palsy of 
the whole or part of the sacral plexus is occasionally met with. It may 
be due to invasion or compression of some or all of its roots by tumors 
or inflammation, or the roots may be injured by pressure from the fetal 
bead during birth. In the latter case it is generally only the fibres which 
enter the external popliteal nerve which suffer. This, it has been shown 
by Hiinermann1 and Thomas,2 is due to the fact that the higher roots 
of the plexus, from which this branch receives the majority of its fibres, 
lie directly on the bone as they pass over the brim of the pelvis and are 
consequently more liable to suffer from compression than the sacral 
roots which are separated from the bone by the pyriformis muscle. For 
the same reason the superior gluteal nerve is often injured at the same 
time. But probably the most common causes of paralysis of these roots 
are malignant tumors of the pelvis, or tuberculous caries, sarcomata 
or metastatic carcinomata of the sacrum, which either compress or 
invade these roots in the intervertebral canals. The symptoms are 
generally those of an incomplete sciatic paralysis, but if the upper roots 
are involved the outward rotators of the hip and the gluteal muscles 
are in addition paralyzed; or if the lower, there will be probably sensory 
loss in the distribution of the small sciatic nerve on the back of the 
thigh and on the buttocks and perineum. 
Diagnosis.-The diagnosis of disease of the nerves of the lower limbs 
is, as a rule, easy, but different conditions with which a partial or complete 
1 Archiv f. Gyn., 1892, xlii, 489. 2 Johns Hopkins Hosp. Bull., 1900, xi, 279. DISEASES OF THE PERIPHERAL NERVES 
561 
paralysis of the sciatic nerve and its branches may be confused needs 
further consideration. The diagnosis is often greatly dependent on the 
history of the mode of onset of the paralysis and of its course; when it 
immediately follows an injury in the region of the nerve there can be 
little room for doubt, if the symptoms correspond to the portion of the 
nerve injured. Disease of the sciatic nerve and its branches must be 
distinguished from: 
1. Lesions of the Sacral Plexus and of the Extradural Portions of the 
Lumbosacral Roots.-When the disease is situated in the sacral plexus, 
muscles other than those supplied by the sciatic nerve are paralyzed, 
as the glutei, the obturator interims, the gemelli, and the quadratus 
femoris; and the anesthesia may extend to the back of the thigh and to 
the buttocks if the lower portion of the plexus is involved. A careful 
examination of the pelvis may reveal the presence of a tumor or of other 
disease. The extradural portions of the sacral roots are most frequently 
involved by tumors or disease of the sacrum; at first, as a rule, only 
one root is affected and the earliest symptom is generally pain, which 
is often extremely severe, referred to the peripheral distribution of its 
sensory fibres, and paresis of the muscles supplied by it. The neighbor- 
ing roots are subsequently paralyzed, and if the disease extends across 
the middle line, motor and sensory symptoms may develop in the oppo- 
site limb. The distinguishing feature, as contrasted with a plexus or 
nerve paralysis, is that the motor and sensory symptoms correspond 
in extent with the distribution of the root fibres. Further, if the lower 
sacral roots are involved before they give off their visceral branches 
the bladder and rectum are paralyzed; true sphincter paralysis never 
results from disease of the nerves. 
2. From lesions of the cauda equina, the paralysis of the nerves which 
spring from the sacral plexus may be distinguished by the fact that the 
symptoms in the former are always of radicular and not of nerve dis- 
tribution, that they are almost invariably bilateral, and that when the 
disease has advanced sufficiently far all the roots below the level of the 
intrathecal disease are generally involved. The sphincter functions too 
are almost invariably affected. It is more difficult to distinguish be- 
tween disease of the cauda equina and of the extradural portions of 
the spinal roots; in the latter condition, however, the symptoms are 
often uniradicular for a considerable time, as the sacral disease to which 
they are most commonly due will generally involve only one root at 
first. Another point of distinction is that all the roots below the level 
of the affected one are not paralyzed in the latter condition, no matter 
how long the disease lasts, unless the sacral tumor extends into the 
vertebral canal and compresses the cauda equina; while when this 
is primarily affected by tumor or meningitis, all the roots which pass 
through the level of the disease are, as a rule, compressed. 
3. From disease of the sacral segments of the cord the diagnosis is easier. 
The symptoms, as a rule, develop more rapidly; they are almost invari- 
ably bilateral and are typically segmental in distribution, but all func- 
tions represented in the segments below the upper level of the disease 
are interfered with. If, however, the lower segments are not involved, 
vol. v-36 562 
DISEASES OF THE NERVOUS SYSTEM 
the paralysis of the muscles which they supply is not associated with 
atrophy or change in the electrical reactions. The severe radiating 
pains which are an almost invariable symptom of root lesions are absent, 
and anesthesia develops earlier. The sphincter functions are generally 
seriously affected. 
Sciatica.-This term is commonly applied to all affections of which the 
chief symptom is pain in the distribution of the sciatic nerve. Such 
pain may be of the nature of a neuralgia and unassociated with any 
disease of the nerve, or it may be due to a neuritis, or to compression 
of the nerve or its roots by tumors or by fibrous adhesions secondary to 
inflammation. It is unfortunate that the one term should be used for 
the symptoms of these different conditions, but it is, indeed, often diffi- 
cult to differentiate between them. It is, however, important to separate 
the cases in which there is pain without any evidence of organic disease 
in the nerve, from those in which sciatic pain is associated with symp- 
toms of a nerve lesion, as anesthesia, atrophic muscular paresis, change 
in the electrical reactions and loss of the Achilles tendon-jerk. 
Etiology.-Males are affected much more frequently than females, 
in about the proportion of 5 to 1. It occurs more frequently in middle 
life, and very rarely, if ever, under fifteen years of age. It has been 
attributed to almost innumerable causes, but exposure to wet and cold 
is generally the only apparent exciting factor; it may follow sleeping 
in a damp bed, or sitting on a wet or cold seat. Gowers insists that 
many cases develop on a gouty diathesis; others undoubtedly follow 
spondylitis. The disease may be also due to trauma to the nerve, as 
by continuous pressure on the edge of a chair, a fall on the buttock, or 
injury in the neighborhood of the hip-joint. Occasionally an attack 
sets in after severe muscular exertion, but probably only in those pre- 
disposed to the disease. It occurs frequently in anemic and badly 
nourished subjects, and in the course of chronic intoxications (alcohol), 
without any apparent exciting cause; and often after infectious diseases. 
Sciatica may be a symptom of diabetes, and is then usually bilateral. 
Quenu has shown that the pain may be due to the pressure of varicose 
veins on the nerve in the neighborhood of the sacrosciatic foramen; this 
generally occurs only in those who work all day standing erect. 
Sciatic pain may be also due to the presence of tumors or inflammatory 
processes in the pelvis, or to a loaded rectum, which may either directly 
compress the nerve or affect the nutrition by the venous stasis it produces. 
Finally, pain in the course of the sciatic nerve may be due to affection 
of its roots by disease of the sacrum or lesions of the cauda equina. 
Hysterical sciatica has been described. 
Symptoms.-The chief symptom is pain along the course of the nerve, 
or limited to one of its chief branches. The onset is occasionally sudden 
and associated with slight pyrexia and constitutional disturbances, but, 
as a rule, it sets in gradually with pain in the buttock or back of the 
thigh in movements or in postures which make the nerve tense or cause 
pressure upon it. In other cases the onset of the typical severe pain 
is preceded by slighter diffuse pain or a feeling of discomfort during 
walking or after exercise. The pain increases gradually in severity; DISEASES OF THE PERIPHERAL NERVES 
563 
it may be either gnawing and burning, or sharp and darting in character. 
As a rule, it is constant, but severer paroxysms occur, either spontaneously 
or excited by movement of the affected limb, and its intensity generally 
increases at night. It may be at first limited to one portion of the nerve, 
generally that in the upper portion of the thigh, but as the disease de- 
velops it usually extends along the whole length of the sciatic trunk and 
its branches. Often bouts of pain occur which shoot from the buttock 
down the limb; such attacks may be described by the patients in similar 
terms to the lancinating pains of tabes dorsalis. It is usually most 
intense in certain points, as over the sciatic notch, in the middle of the 
thigh, in the popliteal region, below the head of fibula, and behind the 
external malleolus; more rarely it is referred to the region of the postero- 
superior iliac crest, or is most severe in the foot. ' The seat of the chief 
pain is often, however, variable in any case from day to day. It is 
generally more or less accurately limited to the course of the nerve, but 
in other cases it is referred to its whole cutaneous distribution. The 
most comfortable posture is lying on the back or on the affected side, 
with the thigh slightly flexed and the knee considerably bent, and when 
sitting the patient generally rests only on the tuber isebii of the unaffected 
side with the hip-joint of the painful limb extended as much as possible. 
In walking the hip and knee are held in moderate flexion with the foot 
extended at the ankle-joint, and only its toes and forepart touching the 
ground. Any sudden movement may bring on an attack of pain. 
The disease is further characterized by the extreme tenderness of the 
nerve to pressure, rarely absent, except, according to Edinger, in those 
cases in which the sciatica is due to compression of the nerve by dis- 
tended veins, and in which the spontaneous pain disappears when the 
patient lies at rest. In some cases the muscles of the limb are also tender 
to pressure. It is characteristic of the pain that it can be invariably 
produced by stretching the nerve; this can be most easily done by 
flexing the thigh with the knee extended, or by extending the knee when 
the hip is flexed-Lasegue's sign, or, as Gowers has shown, by pressure 
on the nerve in the popliteal space as the patient sits in a chair with the 
knee flexed to a right angle; the pain which is produced by either of 
these means is felt not only at the point of pressure, but along the course 
of the nerve in the back of the thigh. Bechterew has pointed out that 
full flexion of the opposite hip when the limb is extended at the knee 
also often produces pain, but believes this indicates disease of the roots 
or of the cauda equina. 
The pain is usually associated with paresthesia. The muscles supplied 
by the sciatic nerve and its branches often become flabby and undergo a 
slight degree of general wasting, even in cases which are not due to any 
organic lesion, when the disease is of long duration; but in this class of 
cases there is no change in the electrical excitability of the muscles. 
Severe reflex spasms of the limb and cramps in some of its muscles, 
especially in the calves, are not infrequently observed. 
Slight trophic and vasomotor disturbances are occasionally met with, 
but usually only pallor, dryness, and coldness of the skin. In other 
cases there may be an increase of the surface temperature, and of sweat 564 
DISEASES OF THE NERVOUS SYSTEM 
secretion. Herpes has been observed. Scoliosis of the lumbar spine 
convex to the affected side is often seen, but its immediate cause is in 
dispute; it may be due to an attempt to spare the painful limb by tilting 
the centre of gravity toward the opposite side, or to a relaxation of the 
lumbosacral muscles of the affected side. All explanations meet with 
the difficulty that the scoliosis is occasionally in the opposite direction, 
concave to the side affected. Kyphosis also occurs, but only rarely. 
In addition to the pain and tenderness, evidence of organic disease is 
present in a considerable proportion of the cases. There may be diminu- 
tion of cutaneous sensibility, but, as a rule, it is nothing more than a 
blunting of tactile sensation on the back of the leg and on the foot. 
When there is an organic nerve lesion the hamstrings and leg muscles 
and occasionally the glutei may be found not merely flabby, but dis- 
tinctly wasted and weak; but the feebleness of movement due to a true 
paresis must be distinguished from the reluctance of the patient to exert 
full power owing to the fear of pain. The most certain indication is the 
presence of qualitative changes in the electrical reactions of Ahe wasted 
muscles, which must be regarded as proof of the existence of an organic 
lesion of the nerve. The third sign of the existence of organic disease 
in the nerve is the absence of the Achilles tendon-jerk. It seems very 
doubtful if this reflex ever disappears in the purely neuralgic cases; in 
fact, in many such cases it is very brisk or even exaggerated; as both 
the afferent and efferent paths of the reflex arc are contained in the 
sciatic nerve, its diminution or disappearance is one of the most delicate 
signs we possess of the presence of organic disease of the nerve; probably 
this may be excluded in all cases in which the reflex is undiminished. 
The reflex seems to be absent in about 30 to 40 per cent, of the cases 
which are clinically regarded as sciatica (Strasburger1). 
In the great majority of the cases sciatica is unilateral, but it is occa- 
sionally bilateral, in about 7 per cent, of all cases according to Gibson's2 
statistics, though Hyde3 found it in 33 per cent, of his cases. This, as 
a rule, indicates a general and not a local exciting cause; it occurs fre- 
quently in diabetes, and may be a part of an incomplete general neuritis. 
Sciatica has been regarded by some recent authors as a disease of the 
dorsal roots of the cauda equina. Dubois4 pointed out that the hypo- 
esthesia, when there is any, may correspond in extent to the distribution 
of one or more of the sacral roots, and Lortat Jacob and Sabareanu5 
confirmed his observations. The sensory loss, according to these ob- 
servers, most commonly coincides with the cutaneous areas of the last 
lumbar and the upper two sacral roots. In at least two of their six cases, 
however, there was an earlier syphilitic infection, and in one of these 
a lymphocytosis of the cerebrospinal fluid made probable the existence 
of a syphilitic meningitis. The fact that the small sciatic nerve, and 
more rarely the anterior crural and the perineal nerves, are occasionally 
affected simultaneously indicates that the disease is then not limited 
1 Deutsch. Zeil. f. Nerven., 1900, xvii, 306. 
2 Lancet, 1893, i, 860. 3 Ibid., 1896, i, 1281. 
4 Correspondenzblatt f. Schweizer-Aerzte, 1902, xxxii, 366. 
5 Revue de Med., 1905, xx, 917. DISEASES OF THE PERIPHERAL NERVES 
565 
to the sciatic but probably affects the lumbo-sacral plexus or the spinal 
roots. In the latter case there is usually an excess of lymphocytes in 
the cerebrospinal fluid, and according to Bonola1 the fascia lata reflex, 
which is unaffected in sciatica, is lost. 
Diagnosis.-The term sciatica is applied by custom to cases in which 
the symptoms are due to an organic affection of the nerve, as well as 
to those in which there is no evidence of structural disease. The first 
step must be, however, to separate these two classes, simple sciatica or 
sciatic neuralgia, in which there is spontaneous pain and tenderness of 
the nerve to pressure and to tension, but no pronounced sensory dis- 
turbance, degenerative atrophy of the muscles, or diminution of the 
Achilles tendon-jerk; and organic sciatica or sciatic neuritis, in which 
some or all of these signs of disease of the nerve are present. Some 
author's, as Gowers, regard all cases of sciatica with persistent tenderness 
of the nerve as neuritic, and consequently make simple sciatica or 
sciatic neuralgia very rare; but in facial neuralgia, in which there is 
certainly, as a rule, no disease in the nerve, its trunks may be quite as 
tender to pressure as the nerve in sciatica. Oppenheim and others have 
insisted that tenderness of the nerves is not a differential sign between 
neuritis and neuralgia. The pain of hip disease may radiate a short 
distance down the thigh; from sciatica it may be distinguished by the 
absence of tenderness in the nerve to pressure and tension, and the 
occurrence of pain on movement of the hip-joint, and on pressure on 
the trochanter. Disease of the sacro-iliac synchondrosis may be more 
difficult to recognize. 
Although many cases of sciatica are due to a neuritic or morbid process 
in the nerve, the presence of marked sensory disturbance and degenera- 
tive atrophy of the muscles, with the absence of the Achilles tendon- 
jerk, should always raise the suspicion of more serious disease. If it 
is due to the compression of the nerve or its roots by a tumor in the pelvis, 
the sciatic trunk is not tender to pressure. 
When the sacral roots are involved in disease of the sacrum or of the 
cauda equina, the symptoms are generally bilateral and more irregular 
in distribution, and the functions of the sphincters are, as a rule, affected; 
the pain is also generally referred to the cutaneous distribution of the 
affected fibres; the sensory or motor disturbances correspond to root 
distribution, and the nerve trunk is not tender. 
In the early stages of tabes dorsalis the shooting pains may be limited 
to the sciatic distribution, but a careful examination of the case will 
generally reveal characteristic signs; in it the pain is almost invariably 
bilateral, and the nerves are not tender to pressure or stretching. 
The pain of intermittent claudication occurs only after exercise, and is 
generally most intense in the distal segments of the limbs and is not 
limited to the course of the nerves. 
Prognosis.-In simple sciatica this is good as regards ultimate recovery, 
but it is extremely difficult to predict the duration, although, as a 
general rule, it is proportional to the severity. Cases in which pain is 
1 Rev. Neurol., 1912, xxiv, 324. 566 
DISEASES OF THE NERVOUS SYSTEM 
associated with signs of an organic nerve lesion are less favorable than 
the uncomplicated neuralgic cases. The outlook is less favorable in 
cases of long duration and where adequate treatment is not possible. 
Where the sciatic pain is due to some lesion extrinsic to the nerve which 
compresses or injures it, the prognosis is naturally dependent on the 
nature of the primary disease. Relapses are unhappily not infrequent. 
Treatment.-The first essential in all cases is rest. Even in mild cases 
this should be made as absolute as possible for some days at least, and 
if it is adopted early severe cases may be often converted into slight 
ones. The patient should be confined to bed and all movements of the 
affected limbs should be restricted as far as possible, if necessary by 
a long splint. This treatment is applicable not merely to recent cases; 
obstinate and protracted cases which have resisted all other treatment 
often yield readily to it. It is often necessary to continue it four to six 
weeks, or even longer without break. Where it is not possible to obtain 
such complete rest an effort should be at least made to avoid all move- 
ments which give pain and cause stretching of or pressure on the nerve. 
Constitutional conditions which are often predisposing causes should 
receive adequate treatment. In some of the acute cases the salicylates 
relieve the symptoms; iron and arsenic often prove useful in cases with 
anemia. The rectum should be emptied, and constipation avoided. 
In acute stages the application of hot poultices along the course of 
the nerve may ease the symptoms. Counter-irritation by the application 
of the cautery or by blisters over the course of the nerve is very generally 
employed, and often seems to be of distinct value. Baths, douches, 
and especially the hot-air bath undoubtedly give relief and often in- 
fluence the course very favorably, especially in its most chronic stages. 
When there is acute pain, symptomatic treatment may be forced into 
the first place. Antipyrine, phenacetin and such drugs often give relief 
for a time. Occasionally deep injections of morphine or cocaine into 
the nerve may be necessary, but they are only temporary measures 
and, owing to the nature of the disease, there is always a considerable 
danger of a drug habit. Morphine is the most effective, but cocaine in 
doses of from | to | of a grain may abolish all pain for hours. 
Favorable results have been obtained by the injection of relatively 
large quantities of normal saline solution (50 to 100 cc.) into the sheath 
of the nerve. The injection is made in the upper part of the thigh; 
severe pain referred peripheralward, paresthesia, and muscular spasm 
of the limb, indicate when the needle enters the nerve. The acute pain 
disappears rapidly when the injection is commenced, which should be 
made slowly. More than two or three injections are rarely necessary, 
and often one is sufficient. Bum1 recorded the result of this in 73 cases; 
he obtained a complete cure in 42, and 14 were much improved. 
Half a gramme of antipyrine dissolved in an equal weight of distilled 
water has been also recommended as a local injection into the nerve. 
Excellent results have been also obtained by the injection of 10 to 15 
cc. of physiological salt solution, or 1 per cent, cocaine or 4 per cent, 
stovaine, into the epidural space by Cathelin's method (Heile2). 
1 Wien. med. Presse, 1907, xlviii, 1660. 2 Neurol. Centralbl., 1912, xxxii, 596. DISEASES OF THE PERIPHERAL NERVES 
567 
The galvanic current is often useful in the later stages; one large elec- 
trode should be placed over the nerve in the upper part of the thigh, the 
other on the leg over one of its branches or on the foot, and a constant 
current of 3 to 5 milliamperes employed for five to twenty minutes. 
It is rarely advisable to use massage in acute cases, but when the 
muscles become flabby or atrophy, it is of service, but pressure or tension 
of the nerve must be carefully avoided. Acupuncture may be tried; 
a series of six or more sterilized needles are thrust in to a depth of about 
two inches along the course of the nerve in the upper half of the thigh, 
and left there from twenty minutes to an hour. Many of the needles 
may pierce the nerve, but if they are inserted from above downward, 
only the first one causes much pain. 
Nerve stretching has also fallen out of fashion, and probably rightly, 
but it may be necessary to have recourse to it in very obstinate cases. 
Bardenheuer has suggested cutting away the brim of the sciatic notch 
where the nerve passes over it, in order to leave the latter embedded in 
the soft tissues and free from pressure. 
Diseases of the Spinal Roots.-The spinal roots may be affected by 
disease either inside the dura mater, or in their extrathecal course before 
they anastomose to form the plexuses; the lesions of the latter portions 
have been considered under the plexus lesions, and the diseases of the 
cauda equina are dealt with in another section. The disease of the roots 
may be primary, or the symptoms may be caused by compression or 
extension of disease from the surrounding parts. 
Primary root lesions are extremely rare. One root alone may be injured 
by a tumor in the neighborhood of the vertebral column as frequently 
happens in malignant disease in this region, or spinal caries. Super- 
numerary ribs may produce an isolated palsy of the first thoracic nerve. 
But as Dejerine and his. pupils1 have pointed out, root lesions are more 
frequently secondary to intradural disease, and especially to compres- 
sion and the invasion of them by a local tuberculous or syphilitic menin- 
gitis. A single root, either sensory or motor, only may be affected, but 
more commonly several roots are involved; there are frequently signs 
of associated disease of the spinal cord. 
The distinguishing feature of all root lesions is the limitation of the 
symptoms to disturbance of the functions of the motor or sensory fibres 
of the root or roots affected; this distinguishes root lesions from lesions 
of the peripheral nerves, which almost invariably contain fibres of two 
or more roots. The onset, which may be either acute or slow, is gener- 
ally with pain and paresthesia referred to the peripheral distribution 
of the sensory fibres involved; at this stage the skin of this region may 
be hyperesthetic. The pain, which is generally at first paroxysmal but 
may become continuous, is, as a rule, very severe and of the same dart- 
ing or shooting character as the pains of tabes dorsalis. If it is the 
intradural portions of the roots which are affected, sneezing or coughing 
may produce very acute pain in the affected root areas-signe de Veter- 
1 Camus and Sezary, La Presse med., 1907, xv, 537; Camus, "Les Radiculites," 
Paris, 1908. 568 
DISEASES OF THE NERVOUS SYSTEM 
nuement-owing to the effect of the sudden increase of the intradural 
pressure on the irritable fibres. After a variable time the pain gradually 
diminishes, and the skin which was previously hyperesthetic become 
hypesthetic as the sensory root fibres degenerate or are destroyed. 
When only one root is involved this diminution of sensibility may escape 
notice owing to the considerable overlap of the adjacent root fibres; 
it is largely dissociative in character, the loss of pain sensation being, 
in contrast to the condition found after peripheral nerve lesions, more 
extensive than the insensibility to light touch (Head). Simultaneously 
with the appearance of hypesthesia, the muscles supplied by fibres from 
the affected ventral root or roots become paretic, and if the lesion is 
sufficiently intense they atrophy, and changes in their electrical reac- 
tions develop; but as almost all muscles receive fibres from two or more 
roots the paralysis of any muscle is rarely complete if the lesion is uni- 
radicular. Cutaneous trophic changes, have been observed in the area 
of sensory distribution of the affected roots, probably in cases in which 
the root ganglia have been involved, and the ocular symptoms of irrita- 
tion or paresis of the cervical sympathetic fibres may appear when the 
lower cervical and upper dorsal roots are affected. 
Diagnosis.-This depends on the essentially radicular distribution of 
the symptoms. Spinal diseases, as tabes dorsalis, syringomyelia, and 
local lesions, may produce symptoms of this distribution, but these can 
rarely offer any difficulty. From local neuritis of a peripheral nerve 
it may be also distinguished by the fact that the nerve trunks are never 
very tender to pressure. When the primary disease is a meningitis which 
invades or constricts the roots, lumbar puncture may aid. 
Treatment.-This should be directed to removing the cause; surgical 
intervention may be successful in the case of tumors, and if there are 
other symptoms of meningitis vigorous antisyphilitic treatment should 
be adopted if syphilitic infection cannot be excluded. In cases with 
persistent pain the intradural section of the dorsal roots may be necessary. 
NEUROMATA. 
The peripheral nerves may be compressed and destroyed by tumors 
in the tissues through which they pass, or invaded by the infective 
granulomata, or by metatases of malignant tumors. The name neuro- 
mata was for long applied to all tumors seated in nerves, regardless of 
their histological structure; Virchow separated those which are com- 
posed of nerve substance proper, which he called true neuromata, from 
those which develop from the connective tissue of the nerves and contain 
no newly formed nerve fibres, false neuromata. 
True neuromata, as defined by Virchow, are, however, exceedingly 
rare; they are found practically only in connection with the sympathetic 
system in the 'thoracic or abdominal cavities. They contain, as a rule, 
ganglion cells and non-myelinated nerve fibres. Although benign, 
they may infiltrate the surrounding tissues. They produce nervous 
symptoms only by compression of nerves, and are not tender to pressure. DISEASES OF THE PERIPHERAL NERVES 
569 
Two cases have been recorded, by Knauss1 and Kredel-Benecke,2 in 
which there were multiple true neuromata in the subcutaneous tissues, 
but apparently most of the others described have been solitary. They 
are met with only in early life. Krauss3 observed one in the lower 
lid. 
False neuromata, which develop from the connective tissue of the 
nerves, are much more common. Their etiology is obscure; they gener- 
ally occur in the first half of life, and in many instances they have been 
congenital. Occasionally they are hereditary; Petren4 found that in 
10 of 60 cases of multiple neuromata there was definite evidence of the 
existence of similar tumors in the ascendant or collateral lines, while 
in 25 per cent, of all the cases there was an hereditary neuropathic 
taint. The appearance of neuromata has followed trauma of a nerve, 
but the false neuromata must be sharply separated from the bulbous 
swellings in the proximal ends of nerves which have been divided. Other 
cases have been attributed to infections and to arsenical poisoning. 
Pathology.-As these tumors grow from the fibrous tissue of the nerves 
they are generally fibromata and of a benign nature, but they occasionally 
undergo myxomatous or cystic degeneration. More rarely they become 
sarcomatous; this is frequently observed when part of a tumor has 
been removed by operation or otherwise injured. Gliomata and lipo- 
mata have been described. The neuro-fibromata may originate from 
the epineurium, in which case the tumor generally lies on one side of 
the nerve and scarcely interferes with it, or from the perineurium or 
endoneurium; in the latter case it penetrates between and splits up 
the nerve into its separate bundles. The structure of the nerve fibres 
which pass through the tumor is, as a rule, unaltered, but there may 
be partial or complete disappearance of the myelin sheaths, while the 
axis-cylinders remain intact. Neuro-fibromata may be found on any 
of the spinal or cranial nerves, or on the intramedullary portions of 
the nerve roots. They vary in size from that of a pin-head to several 
inches in diameter; the smaller are generally spindle-shaped and elon- 
gated in the axis of the nerve from which they grow. There may be 
only one solitary growth on one nerve, or thousands may be distributed 
on all the somatic and visceral nerves; they are relatively common on 
the acoustic nerves and are then frequently bilateral. 
Solitary Neuromata.-Symptoms are more frequently produced by 
solitary and isolated than by multiple neuromata, but many never cause 
inconvenience to the patient. The most prominent feature is pain 
which radiates peripheralward from the tumor along the nerve and its 
branches; it is generally intermittent, but may become continuous and 
so severe as to merit Smith's description of it as "agonizing torture.'' 
Movement of the limb and palpation of the tumor, which is generally 
tender to pressure, may induce an attack of pain, and, as in neuralgia, 
the influence of the weather may be sometimes observed. It has been 
known for long that compression of the nerve involved proximalward 
1 Virchows Archiv, 1898, cliii, 29. 2 Deutsch. Zeil. f. Chir., 1902, Ixvii, 239. 
3 Zeitschr. f. Augenheilk., 1912, xxviii, 110. 
4 Nordiskt Med. Arkiv, Axel Key Festband, 1899. 570 
DISEASES OF THE NERVOUS SYSTEM 
to the tumor can relieve the pain. Cases have been described in which 
epileptic convulsions have been attributed to the irritative symptoms 
of neuromata, and in which the fits have ceased after their removal. 
Paresthesia occasionally occurs in the distribution of the sensory fibres 
which are affected, but loss of sensation and muscular paresis are rarely 
due to simple neuro-fibromata; reflex muscular spasms are sometimes 
seen. When palpable, the tumors are firm and evidently circumscribed, 
and they are movable in the transverse but not in the longitudinal axis 
of the nerve on which they are situated. Neuromata of the sheath of 
the optic nerve produce loss of vision, exophthalmos, limitation of the 
ocular movements, and pain in the eye; and tumors on any of the cranial 
nerves may give rise to the symptoms of complete or incomplete lesions 
of these nerves. 
The only satisfactory treatment is removal of the tumors which produce 
inconvenient symptoms. This is rarely a serious operation, but resec- 
tion of the nerve and primary suture of its divided ends may be neces- 
sary. Rapid growth, adhesion to or infiltration of the surrounding tissues, 
and evidence of acute destruction of nerve fibres indicate malignancy; 
amputation of the segment of the limb involved may be then necessary. 
The spontaneous regression of neuro-fibromata has been observed. 
Tubercula Dolorosa.-Tubercula dolorosa, or the painful subcutaneous 
tubercles of Wood, may be either solitary or multiple; they are generally 
very small and lie directly under the skin. They grow slowly and are 
always benign. As a rule, they are neuro-fibromata, but are occasionally 
lipomata or sweat-gland adenomata which develop in connection with 
the smaller cutaneous nerves. They are generally exquisitely tender 
to pressure and may give rise to spontaneous pain. When troublesome 
they should be excised. 
Multiple neuromata or general neuro-fibromatosis is a condition in which 
a large number of tumors is found on many or all the peripheral nerves, 
and even in the sympathetic system; R. W. Smith1 recorded a case in 
which 450 were counted in one limb and over 2000 were present in the 
body. They are nearly always simple fibromata, but may become 
malignant. The condition is distinguished from the solitary neuromata, 
of which there may be more than one on a nerve, by the presence of a 
diffuse hyperplasia of the connective tissue of the nerves between the 
tumors, so that they can be felt or seen through the skin as thickened 
and beaded structures. They are rarely tender to pressure, and do not, 
as a rule, produce pronounced symptoms. Occasionally they give rise 
to local, or to vague and wandering pains, but they scarcely ever cause 
paralysis or serious sensory loss. Petren observed one case, and col- 
lected others in which there was motor incoordination, probably second- 
ary to loss of deep sensibility. These cases consequently resemble 
progressive interstitial hypertrophic neuritis. As a rule, inconvenience 
results only from the size of the tumors, although when seated on the 
spinal roots they may compress the spinal cord or the cauda equina, 
and within the skull they may give rise to the symptoms of intracranial 
1 A Treatise on Neuroma, Dublin, 1849; New Sydenham Society, London, 1898. DISEASES OF THE PERIPHERAL NERVES 
571 
tumor. All forms of neuro-fibromatosis are frequently associated with 
cretinism, idiocy, or other mental deficiencies. 
Plexiform Neuromata.-In this condition there is a diffuse hypertrophy 
of the connective tissue of all branches of a nerve, and the whole being- 
embedded in fat or loose connective tissue gives rise to the appearance 
of a tumor. Occasionally they are pedunculated and hang in a sac of 
skin. They occur most frequently in the head and neck and are often 
congenital. They are not tender to pressure, and as they rarely produce 
nervous symptoms they belong rather to the domain of surgery. 
Molluscum fibrosum, or von Recklinghausen's disease, is another form of 
neuro-fibromatosis which is characterized by numerous sessile or pedun- 
culated cutaneous nodules which project from the surface. They are 
very variable in size, and may be either soft or hard, and are occasionally 
tabulated. The nodules are generally most numerous in the trunk and 
scalp, and are very rarely seen in the hands or feet. As von Reckling- 
hausen first demonstrated, the nodules are neurofibromata of the ter- 
minal branches of the cutaneous nerves, but the fibrous tissue of the 
cutaneous vessels and glands may thicken and take part in their forma- 
tion. There are rarely nervous symptoms, and the nodules are not tender. 
The disease is generally congenital, and is most frequently seen in degene- 
rate types. It is often associated with naevi and skin pigmentation, 
and there are generally neuromata on the deeper nerves. 
In the condition known as elephantiasis neuromatosa there is, in addi- 
tion to a fibromatosis of the cutaneous nerves, a diffuse thickening of 
the skin, subcutaneous tissues, and even of the bone, of some part of 
the body, generally of one lower limb. Symptoms rarely result from 
affection of the nerves. The condition is usually congenital, and is 
frequently associated with pigmentation of the skin and other abnor- 
malities. It may be distinguished from true elephantiasis by the absence 
of oedema and by the thickening of the nerves which are palpable. 
HERPES ZOSTER. 
Definition.-Herpes zoster is an affection characterized by the appear- 
ance of erythema and cutaneous vesicles on the cutaneous distribution of 
the fibres of one or more dorsal roots. 
Etiology.-The researches of Head and Campbell have made it prob- 
able that idiopathic herpes is an acute specific disease of the nervous 
system. This view is supported by the fact that it is occasionally epi- 
demic and may have a seasonal prevalence, but no specific organism has 
been isolated. It occurs occasionally during the course of general infec- 
tious disease and in diseases in which there is an increased susceptibility 
to infection, as in general paralysis of the insane. It may also result 
from invasion of the dorsal root ganglia by tumors, or from the extension 
into them of a tuberculous process from spinal caries, or of inflammation 
from the meninges; it has been repeatedly observed in the course of 
acute cerebrospinal meningitis. In one case of herpes of the fourth 
thoracic segment the writer found a small metastatic abscess in the 572 
DISEASES OF THE NERVOUS SYSTEM 
corresponding intervertebral ganglion. Herpes has been attributed to 
general intoxications, including arsenical poisoning. It may occur in 
the early stage of acute poliomyelitis. 
Pathology.-Barensprung, in 1861, first demonstrated changes in the 
intervertebral ganglia in a case of herpes zoster, but it was Head and 
Campbell1 who definitely established the pathology. They showed 
that the herpetic eruption is always associated with acute interstitial 
inflammation of the dorsal root ganglia, or of their homologues on 
the cranial sensory nerves. In the earlier stages the affected ganglion 
is found infiltrated by small round cells; later part of the ganglion tissue 
is generally destroyed by the intensity of the inflammation, and many 
of its cells undergo acute necrosis; hemorrhages almost invariably 
occur in the inflamed area. The sheath also is generally involved, and 
the whole ganglion is swollen and hyperemic. When the inflammatory 
products are absorbed the disease may be represented only by a small 
area of scar tissue, or there may be cyst formation, but in the slighter 
affections there may be no permanent changes in the ganglion. The 
affection is not, as a rule, so localized as the eruption would indicate; 
in one case, although the eruption was limited to the third sacral root 
area, I found cellular infiltration not merely in the corresponding gan- 
glion, but in all the neighboring ones of the same and of the opposite 
side. The inflammation may extend a short distance into the peripheral 
nerves or into the dorsal roots. When cells of the ganglion are destroyed 
their processes in the peripheral nerves and dorsal roots naturally undergo 
secondary degeneration. There is a striking similarity between the 
changes in the intervertebral ganglia in herpes and the affection of the 
spinal cord in acute poliomyelitis. 
Symptoms.-Idiopathic herpes often sets in with fever and general 
malaise, which may last for three to five days, but the rise of temperature 
is, as a rule, slight. Occasionally, there are gastric disturbance and other 
constitutional symptoms, and enlargement of lymphatic glands has been 
observed. The eruption, which generally occurs on the third or fourth 
day of the disease, is often preceded by pain and hyperalgesia in the 
affected area. The skin becomes erythematous and finally vesicles 
develop, either while there is still fever, or as the temperature falls. 
The special characteristic of the disease is the limitation of the vesicles 
to the cutaneous distribution of one dorsal root, or more rarely of two 
or more adjacent roots. The eruption is not, however, uniform over 
this region, but is made up of a series of outbursts which follow the 
course of the small cutaneous nerves; there is a variable extent of over- 
lap between adjacent herpetic areas. Herpes is much more frequent on 
the trunk along the course of the intercostal nerves than on the limb; 
according to Head those ganglia are most often affected which receive 
visceral sympathetic fibres. After a time the vesicles dry up and dis- 
appear, but their distribution is generally permanently marked by 
small, slightly depressed scars in the epidermis. On the face herpes is 
most common on the cutaneous distribution of the ophthalmic division 
1 Brain, 1900, xxiii, 353. DISEASES OF THE PERIPHERAL NERVES 
573 
of the trigeminal nerve; when it occurs here vesicles may form on the 
cornea and lead to troublesome scarring and faceting. 
The most prominent symptom is pain referred to the region of the 
eruption. The pre-herpetic pain which usually ushers in the attack 
is generally of a burning or stabbing nature; it may be extremely severe, 
but generally abates with the appearance of the rash. The post-herpetic 
pain or neuralgia is much more serious; it is also referred to the region of 
the eruption, but frequently spreads to adjacent segments; it is so severe 
and so intractable to treatment that it has driven the subject to suicide 
more than once. It occurs almost invariably only in old subjects. In 
a large proportion of the cases some loss of sensibility in the affected area 
follows an herpetic eruption, but in others sensation is unaffected. 
Petren1 has shown that there may be marked or even complete loss of 
pain and temperature sensibility, with tactile sensation unimpaired; 
and he has drawn attention to the fact that post-herpetic neuralgia 
occurs chiefly in those cases in which there is considerable or complete 
analgesia. The sensory loss is generally uniform over the whole of the 
affected area. A not uncommon complication is muscular paralysis of 
radicular distribution. Doucet2 has collected 40 such cases. The palsy 
generally occurs in the neighborhood of the eruption, and is probably 
due to involvement of the ventral spinal root in the inflammatory 
process as it lies beside the affected ganglion; or occasionally perhaps 
to central lesions of toxic or infective origin. The facial nerve is perhaps 
the most frequently affected, generally in association with trigeminal 
or occipito-cervical herpes; it is, however, probably due to compres- 
sion of the nerve in the aqueduct of Fallopius by a swollen geniculate 
ganglion which is simultaneously involved by herpes (Ramsay Hunt).3 
Paralysis of the ocular nerves has also been observed. In a few cases 
slight paraplegia has developed, as in a case published by Bruce;4 it 
is probably due to extension of the disease to, or to the appearance of 
an independent focus in, the spinal cord. 
Treatment.-The pain may be relieved by drugs in the earlier stage 
of the disease, but post-herpetic neuralgia is very intractable to all 
treatment; relief can be promised only by section of the dorsal spinal 
root corresponding to the ganglion affected, or by extirpation of the 
Gasserian ganglion where pain follows trigeminal herpes. 
MULTIPLE NEURITIS. 
Multiple neuritis, peripheral neuritis, and polyneuritis are the terms 
applied to a group of diseases which are due to affections of the peripheral 
nerves, or rather of the peripheral motor and sensory neurones. These 
conditions are distinguished from paralysis limited to a nerve, or a 
group of nerves in close anatomical relation, by the fact that several 
nerves are affected simultaneously or in rapid succession (therefore 
1 Zeit.f. klin. Med., 1907, Ixiii, 91. 
2 Le Zona assode aux paralysies et aux amyotrophies, These de Paris, 1906. 
3 Jour. Nerv. and Ment. Dis., 1907, xxxiv, 71. 
4 Rev. of Neurology and Psychiatrie, 1907, v, 885. 574 
DISEASES OF THE NERVOUS SYSTEM 
multiple neuritis), that the condition is always bilateral and more or 
less symmetrical, and that the longer fibres of the nerves which extend 
to the periphery of the limbs suffer more severely than the shorter fibres 
which are distributed to the proximal segments, or to the muscles and 
sensory structures of the trunk (therefore peripheral neuritis). The 
disease is characterized by the fact that it is limited to the peripheral 
neurones, while the central nervous system is intact, or any changes 
which may occur in it are merely a coincident effect and of no significance 
in the production of the clinical picture. 
Classification.-A pathological classification is insufficient, as there are 
only two distinct types of pathological change; in the one the primary 
and essential process is a degeneration of the parenchyma or functional 
elements of the peripheral nerves; in the other it is an inflammatory 
or simple hypertrophic process of the connective tissue in which the 
fibres are ensheathed. But primary interstitial neuritis is extremely 
rare, and exists practically only as part of an infective disease-namely, 
leprosy-while the great majority of the cases are instances of paren- 
chymatous neuritis, and it is impossible to subdivide this enormous 
group, as there are no essential differences in the neural changes in 
varieties due to different causes or characterized by special symptoms. 
The classification by the clinical symptoms is equally unsatisfactory. 
These may consist only of motor phenomena, or of disturbance of sen- 
sation, or vasomotor symptoms may be the most prominent feature; 
but we rarely find that there are only motor, or sensory, or vasomotor 
symptoms; in fact, there is almost invariably disturbance of all the 
functions of the nerves affected, and affection of any of these functions 
may be the dominant symptom in different cases due to the same cause. 
It is, therefore, inadvisable to classify cases as of the motor, sensory, 
or vasomotor type. 
An etiological classification is usually adopted and is the most useful, 
as it brings into prominence the cause of each case, and when multiple 
neuritis has been diagnosed the next step is to ascertain its cause. But 
an etiological classification cannot express the prominent features of 
a case, for though certain poisons may have an affinity for nerve 
fibres with certain functions, such a selective action is not always 
apparent. 
Etiology.-The wide distribution of the affection and its symmetrical 
regularity indicate, as Gowers points out, that multiple neuritis must be 
directly due to an altered blood or lymph state which has equal access to 
all parts of the body, and, indeed, its cause is invariably some poison 
which is introduced into the body from without, or produced within it 
by infective microorganisms or by a derangement of normal metabolism. 
Many of these poisons, especially those of inorganic nature and of simple 
chemical constitution, seem to have a selective action on the peripheral 
nerves and may produce disturbance of function in no other organ of 
the body; motor palsy, for instance, may be the only symptom of chronic 
lead poisoning. But in the majority of the cases which are due to bac- 
terial toxins or to poisons of complex chemical nature the symptoms of 
multiple neuritis are only part of the result of a general infection or DISEASES OF THE PERIPHERAL NERVES 
575 
intoxication, or, as is the rule in the acute specific fevers, the neuritis 
may appear when the symptoms of the infection are on the wane. 
There is a group of infective diseases in which a peripheral neuritis 
may be the only or the most prominent symptom. Beriberi is the best 
known example, but there are undoubtedly other, as yet ill-defined or 
scarcely recognized, infectious conditions which may manifest themselves 
only by the symptoms of a primary multiple neuritis. 
It not infrequently happens that there are two or more factors at 
work, predisposing as well as directly exciting causes. Neuritis occa- 
sionally develops in the course of pulmonary tuberculosis, and although 
it is probable that it may be at times due to the action of the tubercu- 
lous toxin alone, it is very rarely that the influence of alcohol can be 
excluded. Similarly, quite a series of cases of neuritis have been ob- 
served after the administration of phosphate of creosote to patients with 
phthisis;1 here the tuberculosis was probably the predisposing, the drug 
the exciting, cause. In the extensive epidemic of peripheral neuritis in 
the north of England, which was caused by the contamination of beer 
with arsenic, it is probable that there were also two distinct but coinci- 
dent etiological factors, namely, alcohol and arsenic. Finally, there can 
be no doubt that most marasmic and cachectic conditions make the 
nerves unduly vulnerable to noxious agents. 
1. Neuritis Due to Poisons Introduced into the Body from Without.-These 
poisons may be either: (a) Metallic or inorganic substances, as lead, 
arsenic, mercury, copper, carbon monoxide, or bisulphide of carbon; 
or (b) organic substances, as alcohol, dinitrobenzene, various aniline com- 
pounds and derivatives, sulphonal, and other drugs; finally, the poison 
may be of more complex constitution, as the toxins of ptomaine poison- 
ing. Neuritis due to any of these causes, except perhaps carbon mon- 
oxide, is generally associated with chronic intoxication and rarely follows 
a single administration of the poison, no matter how large the dose. 
2. Neuritis Secondary to, or Associated with, Diseases Due to Toxins 
Produced Within the Body.-These toxins may result from: (a) Bacterial 
infections, most frequently diphtheria, smallpox, typhoid, and scarlet 
fever. The toxins produced by septic processes may also give rise to a 
multiple neuritis, as is the case in puerperal neuritis; (6) derangement 
of metabolism as in diabetes and other constitutional diseases. There 
is very little evidence that gout produces a polyneuritis, but rheumatism 
may certainly be a cause (Schulhof).2 As a rule, these conditions act 
more as predisposing factors than exciting causes. 
3. Toxins Produced within the Body Which Have a Primary Action on 
the Peripheral Nerves.-A variety of neuritis which sets in with malaise 
and fever and more or less severe constitutional symptoms is not infre- 
quently observed, and probably comes into this group (acute febrile 
polyneuritis). It is often called post-influenzal, and although the influ- 
enza bacillus or its toxins may be occasionally the exciting cause, it 
seems doubtful if it is often so. These cases are distinguished by the 
severity of the neuritis as contrasted with the slight constitutional 
1 Huet, Neurol. Centralbl., 1907, xxvi, p. 60. 
2 Med. Klinik, 1913, ix, 952. 576 
DISEASES OF THE NERVOUS SYSTEM 
disturbances which precede its onset, or the complete absence of pro- 
dromal symptoms. Occasionally exposure to cold is the only apparent 
exciting cause in cases which probably belong here. 
4. Neuritis associated with cachexia or malnutrition, such as occurs in 
anemia, tuberculosis, syphilis, malignant disease, and in old age. These 
may be directly due to intoxication, or in some cases, as in tubercu- 
losis, to the action of the bacterial toxins which produce the cachexia. 
In other cases the general malnutrition merely leads to an increased 
vulnerability of the nerves; or the nutrition of the nerves may be 
directly interfered with, as in severe peripheral arteriosclerosis. 
5. Neuritis Due to the Invasion of the Peripheral Nerves by Bacteria.- 
Leprosy is the classical example of this type. Nerves involved in septic 
wounds may be invaded by pyogenic bacteria which occasionally extend 
along the trunk and may involve adjacent nerves--ascending neuritis- 
but the picture of multiple neuritis can scarcely arise. Gonorrhoea may 
also lead to interstitial multiple neuritis (Frisco), or to acute neuritis 
either with or without arthritic disease, and Eberth's bacillus has been 
found in the nerves in typhoid neuritis. 
In certain cases it is impossible to determine the direct cause, as in 
the neuritis of pregnancy (Dustin1) and that which occurs associated 
with gastric ulcers (Klippel and Pierre-Weil2). 
Incidence.-Multiple neuritis is essentially a disease of adult life. It 
occurs rarely in childhood, and then practically only after the acute 
specific fevers, especially diphtheria. It may follow the treatment of 
chorea with "heroic" doses of arsenic. Polyneuritis is also uncommon 
in advanced age, and when it occurs is usually slight in degree. Heredi- 
tary influences are rarely observed. The disease is more common in 
females, probably due to their greater liability to alcoholic neuritis. 
There are many factors which may determine the localization or inci- 
dence of the disease. Perhaps the most important of these is the effect 
of overwork or exhaustion, especially when the nutrition is subnormal 
or when the metabolic equilibrium is otherwise upset. Thus Edinger 
explains the predominant paralysis of the extensors of the wrist and 
fingers in lead palsy in painters; in compositors, on the other hand, 
who not infrequently suffer with lead palsy, the paralysis is often limited 
to the small hand muscles with which they chiefly work. Occasionally 
the mode of the infection determines the type of the palsy; the soft 
palate is generally the first organ affected in diphtheritic neuritis fol- 
lowing pharyngeal diphtheria, while the abdominal muscles were the 
earliest and most severely paralyzed in a few cases in which the seat 
of infection was the umbilicus in infants. 
Pathology.-The changes in multiple neuritis differ from those of 
local neuritis not only in their distribution, but also in their nature. 
In the latter, the primary changes affect the interstitial tissues, and 
are generally of an inflammatory nature; the condition produced is 
consequently a local interstitial neuritis. Multiple neuritis is the result 
of degenerative, parenchymatous changes in the peripheral nerves. 
1 Nouv. Iconographie de la Salpetriere, 1909, xx, 349. 
2 L'Encephale, 1909, iv, 417. DISEASES OF THE PERIPHERAL NERVES 
577 
The amount of structural change is very variable; it depends not 
only on the intensity of the disease, but also on its cause and nature, 
and particularly on its duration. To the naked eye there is rarely any 
definite abnormality, although in the acuter cases in which the primary 
disease has lead to a reaction in the interstitial tissues, the nerves may 
appear red and swollen, and there may be a distinct hyperemia of their 
sheaths. These changes, when present, are generally most distinct in 
the smaller peripheral branches. In chronic cases, the nerves may be 
reduced in size and unusually firm and opaque. Microscopic examina- 
tion shows that the myelin sheaths are broken up and disintegrated 
into irregular masses which stain black with osmic acid; in cases of 
longer duration this fatty degeneration product may have been already 
removed. In the early stage the axis-cylinders may be intact; more 
commonly they are swollen and varicose and stain feebly, or if the neuritic 
process is further advanced they may be broken or completely disinte- 
grated. The protoplasm around the nuclei increases in amount and 
penetrates between the fragments of the disintegrating myelin, and the 
nuclei proliferate by direct division. Some of these neurilemmal nuclei 
form independent cells which assume phagocytic functions or become 
fibroblasts and lead to the formation of an excess of connective tissue; 
but others fuse end to end to form those nucleated strands of undiffer- 
entiated protoplasm into which, when regeneration commences, the new 
outgrowing axis-cylinders penetrate. 
Even in parenchymatous neuritis there is frequently some evidence 
of inflammatory or reactionary changes in the interstitial tissue; the 
vessels become congested and there is often some serous effusion, and 
occasionally a small round-cell infiltration. 
These neuritic changes differ from secondary or Wallerian degenera- 
tion of nerve fibres in many important particulars. In the first place, 
they are always most advanced in the most distal segments of the nerves 
-multiple neuritis has been consequently aptly called peripheral neuritis 
-and the intensity of the changes always diminishes steadily proximal- 
ward; indeed, it is the rule that except in the severest cases the larger 
nerve trunks, as the sciatic, contain a much smaller proportion of degen- 
erated fibres than their peripheral muscular or cutaneous branches, while 
in the dorsal and ventral spinal roots there may be no degenerated 
fibres. Secondly, the morbid changes are much less uniform than in 
fibres which are undergoing secondary degeneration. In almost all 
cases of toxic parenchymatous neuritis there are patches of that form 
of segmental periaxial neuritis which was originally described by Gom- 
bault, in which the disease is limited, or practically limited, to a seg- 
ment of the myelin sheath; in its slighter forms the axis cylinder is 
not interrupted and the portion of the fibre distal to the lesion does not 
undergo secondary degeneration, and may be perfectly normal. Numer- 
ous transition stages may be found between these local lesions and the 
diffuse degenerative changes. Thirdly, not only is the affection of any 
one fibre irregular and diffuse, but the number of fibres affected in any 
nerve is extremely variable; even in the severest cases it is rare to find 
all the fibres of even the smaller nerves degenerated. 578 
DISEASES OF THE NERVOUS SYSTEM 
The interstitial changes are not without importance; the extreme 
tenderness of the nerves has been ascribed to this, but it seems extremely 
doubtful if it is so, as the affected nerves are almost invariably tender 
to pressure, while the presence of such inflammatory changes is very 
inconstant. When, however, they are pronounced they may lead to 
the formation of an excess of connective tissue, which ultimately re- 
places the degenerate fibres and may offer a serious obstacle to their 
regeneration, or may even interfere with the vitality of those which 
were originally unaffected. 
Changes are occasionally present in the central nervous system; when 
the dorsal roots are involved degenerated fibres will be naturally found 
in the dorsal columns of the spinal cord; but multiple neuritis is essen- 
tially due to toxic causes, and the dorsal column fibres are extremely 
liable to degenerate in all forms of general intoxication. More import- 
ant and much more constant are the changes in the large ventral horn 
cells; they were present in some degree in every case of multiple neuritis 
examined by the writer. They may be described as chromatolysis of the 
cells. Similar changes may be generally found in the cells of the dorsal 
root ganglia, and not infrequently in other parts of the central nervous 
system, as in the cerebral cortex; the association of mental disturb- 
ances with multiple neuritis may be the clinical manifestation of the 
affection of the latter region (Bonhoeffer). Small inflammatory foci and 
minute hemorrhages have been described in the spinal cord, but have 
been much too slight and inconstant to play any part in the production 
of the symptoms. They can be only attributed to the action of the toxin. 
The nature of the marked alterations in the large motor ventral horn 
cells, and their relation to the neuritic changes, has been much discussed. 
There are evidently three possibilities: In the first place that the cell 
changes are the primary effect of the action of the noxious agent which 
produces the neuritis, and that the degenerative lesions in the nerve 
fibres are secondary to the affection of their trophic centre; this hypoth- 
esis seems scarcely tenable, as there is no constant relation between the 
cellular and fibre lesions; in fact the former are occasionally absent, and 
it is well known that the cell disease must be very acute to produce an 
acute degeneration of its axis cylinder. Further, the nerves are so dif- 
fusely and irregularly affected, and the disease is so frequently limited 
to their distal extremities that it is extremely improbable that it can 
be secondary to an acute cell lesion. In favor of this view it has been 
argued that the portions of the neurone most distant from their trophic 
cells must be the first portions to degenerate when the vitality of the 
cell is lowered. Even though this view may not give sufficient support 
to the hypothesis that the cell changes are the primary lesions in multiple 
neuritis, it explains very satisfactorily the chief incidence of the disease 
on the peripheral segments of the nerves. 
The second possibility is that the cell changes are secondary to the 
degeneration of the fibres-that they are comparable to the retrograde 
chromatolysis which may be seen in any cell after its axis-cylinder has 
been cut across. This may be an important factor, but it is improbable 
that it is the only one, as it must be recognized that there is no close DISEASES OF THE PERIPHERAL NERVES 
579 
parallel between the neural and the cellular changes, and that the cellu- 
lar alterations do not always conform to the type of retrograde chro- 
matolysis. The third possibility, and the most acceptable, is that all 
portions of the peripheral motor and sensory neurones are simultaneously 
affected, but the most prominent alterations are found in their least 
resistant portions, namely, the segments of the fibres furthest removed 
from the cell body. 
The importance of the muscular changes must be remembered. The 
degree of change depends on the severity and the acuteness of the 
neuritis, but in addition to the degenerative or regressive changes, such 
as succeed section of a nerve, there is also often fatty, granular, or waxy 
degeneration of the muscle fibres, a result of the direct action of the 
toxin. There is frequently, too, evidence of inflammatory processes in 
the interstitial tissues of the muscles, an interstitial myositis. 
Symptoms.-It seems advisable to describe fully one form in which all 
symptoms may be found in various combinations; other varieties of the 
disease will then need only short references. 
Alcoholic neuritis is much the most frequent form-in Great Britain, 
at least, it is probably much more common than all the other varieties 
put together, and may be taken as a type. In most cases all the func- 
tions of the peripheral nerves are affected, but occasionally the symp- 
toms are limited to either motor or sensory disturbances, or incoordina- 
tion of movement may be the most prominent feature; the latter type 
has been called neuro-tabes peripherica, or alcoholic pseudo-tabes. Alco- 
holic neuritis occurs practically only in middle adult life, but a few cases 
have been recorded in the first decade; in these the disease has gen- 
erally developed after a single large dose of the poison. In England 
and America it occurs much more frequently in women than in men, 
according to Gowers in about the proportion of 3 to 1; in Germany, 
from the statistics of Remak, it seems to be more common in males, 
The social habits of the people must largely determine its incidence, 
but women undoubtedly seem more vulnerable to the poison. 
It usually results from the use of the stronger forms of alcohol, espe- 
cially from spirits, but it is seen not infrequently in beer drinkers, and 
especially in constant and steady tipplers. Undoubtedly, it affects 
chiefly those who habitually take a considerable amount of alcohol, but 
are rarely drunk; occasionally, however, an attack may follow a severe 
bout of drinking or delirium tremens. There is frequently some excit- 
ing or predisposing cause in addition to alcohol, as a general infection 
or insufficient nutrition; pulmonary tuberculosis is found in a consider- 
able proportion of the cases, at least of those which come to a fatal 
termination. Lindl1 found some evidence of local or general neuritis in 
214 of 300 drunkards. The slighter forms often escape recognition. 
Onset.-In some cases premonitory symptoms occur which may be 
either the result of the alcoholic intoxication or a part of the disease. To 
the former group belong general constitution disturbances, as morning 
vomiting and other symptoms of disorder of the digestive system, or 
1 Der Alkoholismus, 1093, Neue Folge, Heft i. 580 
DISEASES OF THE NERVOUS SYSTEM 
dyspnoea and symptoms of affection of the circulatory system. In other 
cases loss of memory or slight mental impairment may be the first indi- 
cation that anything serious is wrong. But the true prodromata, when 
there are any, are numbness and tingling of the fingers and toes, vaso- 
motor disturbances in the extremities, painful cramps of the muscles, 
especially in the calves, and vague pains. These are undoubtedly part 
of the disease and constitute its first symptoms, but as they often exist 
for long periods before the onset of classical signs, it is important to 
recognize them as premonitory of a more serious affection. In this 
early stage there is often some fever. 
The onset of the neuritis is nearly always gradual, often slow and 
insidious; as the patient's memory is frequently impaired, the earliest 
symptoms may be overlooked or attributed to the effects of drinking, and 
it may be difficult to ascertain the duration or the mode of onset. An 
acute onset is rare, but cases have been recorded in which a sudden loss 
of power in the limbs apparently occurred. But these acute attacks are 
generally more apparent than real; they are usually seen only after 
acute illnesses or a bout of drinking, during which the earlier symptoms 
were not observed. In the common forms the first definite symptom is 
usually disturbance of sensation with paresthesia in the hands and feet, 
vague diffuse pains which are often very severe and always increased by 
movement, and tenderness of the muscles to pressure; these are soon 
followed by motor symptoms. The symptoms are almost invariably 
bilateral and symmetrical from the onset and more prominent in the 
lower than the upper limbs, but in acute cases one limb or the functions 
of one nerve may be unduly affected at first. The further evolution 
is variable; in the severer forms the patient is generally bedridden in 
a few days, but, as a rule, the disease progresses more slowly and reaches 
its acme in three or four weeks. Occasionally its course is much more 
chronic; it may progress slowly for months in mild cases when alcohol is 
not withheld. The ataxic variety has often this slow and chronic course. 
Motor Symptoms.-Paralysis may involve either the upper or lower 
limbs, more commonly both, but it is invariably more marked in the 
distal than in the proximal segments. The legs are almost always more 
affected than the arms, often they alone are involved. The trunk muscles 
escape frequently, and any considerable degree of palsy in the muscles 
supplied by the cranial nerves is rare. It must always be borne in mind 
that true muscular paralysis may be simulated by the reluctance of the 
patient to perform movements with the full amount of power which is 
possible to him, owing to the pain which forcible contraction of the 
tender muscles produce; a careful examination of the electrical reactions 
of all muscles may be necessary to ascertain the extent of the affection. 
In the usual type the first symptom is a slight difficulty in walking, 
especially on uneven surfaces or in ascending stairs, owing to the patient's 
inability to raise the toes from the ground. Dorsiflexion of the feet is 
very feeble or impossible, and the foot when unsupported hangs in the 
extensor position with its dorsum almost in the line of the axis of the leg. 
With this, some malposition of the toes is generally associated; in the 
earlier stages they are generally hyperextended at their distal and flexed DISEASES OF THE PERIPHERAL NERVES 
581 
at the interphalangeal joints, except the great toes, which are often 
extended at all their joints; later all the toes are generally fully flexed 
and curled into the soles. 
To avoid the constant danger of the toes cataching in any obstacle, 
or even tripping him on level ground, owing to the ankle-drop, the 
patient raises his feet unusually high by flexion of the hips, and throws 
them forward with each advancing step; the gait has been consequently 
called steppage gait. It is almost characteristic of peripheral neuritis. 
It occasionally happens that all the muscles of the anterior tibial groups 
are not equally affected-the tibialis anticus often escapes when the 
peronei and extensors of the toes are paralyzed. 
The symptoms of palsy are, however, very rarely limited to the muscles 
on the front and outer aspects of the legs; those which are usually next 
affected in both time and degree are the calf muscles. Walking is then 
more seriously interfered with, as the patient cannot spring from his 
toes or even fix the ankle-joints properly. The muscles of the thighs 
and hips suffer later and, according to the general rule, being proximal 
muscles, to a considerably less degree; indeed, the muscles which move 
the hip-joints are much affected only in the severest forms, and by that 
time the patient is invariably bedridden. The paralyzed muscles are 
soft and flabby to touch, and evidently hypotonic; when the paralysis 
has lasted more than two or three weeks they begin to waste, and the 
atrophy corresponds approximately to the degree of the paralysis. In 
the latter stages, however, when the atrophied fibres become replaced 
by connective tissue, the muscles may be firm and fibrous to touch. 
In the upper limbs the paralysis, as a rule, first expresses itself by 
affection of the extensors of the wrist and fingers. Rarely, however, does 
the extent of the paralysis coincide with the nerve distribution; in the 
arm the supinator longus and the extensor ossis metacarpi pollicis often 
escape, or are less affected than the long extensors. This type of par- 
alysis and the wrist-drop that results, although most characteristic of 
lead palsy, is not infrequently seen in alcoholic neuritis. The loss of 
power in the extensors of the wrist and fingers leaves the arms almost 
useless; the patient cannot grasp any object firmly owing to the mechan- 
ical disadvantage at which the flexors of the fingers must work when the 
extensors of the wrist are paralyzed. But the flexors of the wrist and 
fingers rarely escape, and in the severer cases may be as powerless as 
the extensors. Occasionally the intrinsic muscles of the hand are affected 
early; according to Gowers those of the thenar and hypothenar groups 
always suffer later than the interossei. Of the muscles above the elbow, 
the triceps generally suffers more severely than the biceps, while those 
which move the shoulder-joints are rarely much affected. In the atrophic 
stage the wasting is generally most prominent on the dorsal surfaces 
and ulnar borders of the forearms; in the hands it may be indicated 
by flattening of the thenar and hypothenar eminences and by hollowing 
of the interosseal spaces. 
The muscles of the trunk are involved in severe cases, most commonly 
those of the abdominal walls; this impairs the power of expelling the 
contents of the bladder and rectum, and coughing and sneezing. If the 582 
DISEASES OF THE NERVOUS SYSTEM 
flexors of the thighs are weak as well as the recti abdominis, the patient 
becomes unable to raise himself from the recumbent position. There 
is considerable palsy of the muscles of the back and neck only in the 
severest cases. Paralysis of the diaphragm is occasionally observed; res- 
piration is consequently severely implicated, as the intercostal muscles 
rarely escape completely, and a fatal termination is therefore probable. 
The functions of the cranial nerves are sometimes involved; lingual, 
palatal, or pharyngeal palsies may develop in very severe cases, generally 
when they are progressing to a fatal termination; laryngeal paralysis is 
very rare. The tachycardia, which is often a prominent symptom, is 
generally attributed to disease of the pneumogastric nerves; Dejerine 
has demonstrated parenchymatous degeneration of them. 
Bilateral facial palsy, which is not infrequently seen in infective 
multiple neuritis, is rarer in the alcoholic form. So also are ocular pal- 
sies, though many cases in which they have occurred have been recorded; 
they are probably due to hemorrhagic polioencephalitis superior (Wer- 
nicke's form), which develops most often in the subjects of chronic 
alcoholism, and not to a neuritic affection of the ocular nerves. Nys- 
tagmus, however, is not infrequently observed. The pupillary reactions 
are very rarely altered, but the Argyll-Robertson phenomenon has been 
observed in alcoholic neuritis and in chronic alcoholism (Nonne1). Optic 
neuritis has also been described. 
The state of the electrical reactions of the nerves and muscles is very 
variable. The excitability of the nerves which are affected diminishes 
rapidly and generally disappears completely; it is interesting that little 
or no reaction may be obtained from nerves the functions of which are 
almost intact; this is evidently the result of incomplete lesions. No 
contraction can be obtained, as a rule, by the direct application of the 
faradic current to muscles which are much paralyzed; in the less affected 
the reaction may be incomplete, as a small proportion of the fibres may 
respond. With the galvanic current all forms of the reaction of degen- 
eration may be obtained, but the current necessary to produce any 
contraction is generally greater than normal. In severe cases the muscles 
often eventually lose their galvanic excitability. 
Although paralysis is invariably the most prominent motor symptom, 
there is occasionally evidence of motor irritation or hyperactivity. The 
tonic spasms or cramps of calves and other muscles which occur as fre- 
quent premonitory symptoms occasionally persist in slighter cases 
throughout. Fibrillation, too, is occasionally seen, and irregular tremor 
of the hands, tongue, and facial muscles is a well-recognized symptom. 
These phenomena are probably due to irritation of undegenerated fibres. 
Still more important is the reflex or voluntary contraction of relatively 
intact groups of muscles, by which the limbs are held in the positions 
which are most comfortable, and by which the patient resists passive 
movements which give him pain. Thus the knees are almost always 
semiflexed by contraction of the hamstring muscles; the shortened 
muscles may become permanently contracted. 
1 Neurol. Centralbl., 1912, xxxi, 6. DISEASES OF THE PERIPHERAL NERVES 
583 
Sensory Symptoms.-The tingling, numbness, and other subjective 
sensations in the distal segments of the limbs generally persist throughout 
the course but the pains which are an almost constant symptom are 
a more serious cause of suffering. They may be aching, burning, or 
shooting in character, and are often very severe. They are generally 
more diffuse and less intermittent and lancinating than those of tabes 
dorsalis, and, as a rule, are referred to the deeper tissues of the limbs or 
joints, rarely to the course of the nerves or to the skin. They may 
occur spontaneously, but are excited or increased by movement, either 
active or passive, of the limbs; severe paroxysms often occur during 
the night and deprive the patient of sleep. Not infrequently there is 
constant and very troublesome neuralgic pain in the soles of the feet. 
But the most prominent and constant sensory symptom of multiple 
neuritis is the extreme tenderness of the muscles to pressure; this is 
probably never absent and is almost a pathognomonic sign. It is usu- 
ally greatest in the calves and in the regions in which there is consider- 
able paralysis, but muscles which are in no degree paralyzed may be very 
tender. In the severer cases the limbs cannot be touched without elicit- 
ing cries of pain, and even the weight of the bed-clothes may be intoler- 
able. It becomes agonizing for the patient to lie long in any position, and 
yet he dreads being moved, owing to the pain which any change of 
posture produces. The affected nerve trunks are also often very sen- 
sitive to pressure, but this is neither so constant nor so prominent as the 
muscular tenderness. Occasionally the superficial nerves may be felt 
slightly enlarged or swollen. 
Objective disturbances of sensation are nearly always much less 
marked than the motor palsies, but they are rarely quite absent. They 
are most marked in the distal segments of the limbs and diminish grad- 
ually proximalward, ceasing in the legs, as a rule, about the middle of 
the thighs and rarely marked in the arms above the elbows; but there 
is never a definite boundary line between the normal and anesthetic 
skin, and it is characteristic of the hypesthesia that it does not corre- 
spond in extent to the distribution of either the peripheral nerves or the 
dorsal roots. Usually the most definite loss is to light touches, which 
may not be perceived in the feet or legs and felt only unnaturally on 
the thighs. Sensibility to pain is less commonly lost, but there is not 
infrequently some delay in perception of painful stimuli. The appre- 
ciation of temperature also may be diminished or absent in the extrem- 
ities, but this is far from constant even in the severer cases. But more 
characteristic of neuritis is a curious combination of hyperesthesia and 
hyperalgesia; in areas of skin which are more or less insensitive to 
light touches a pin-prick or a scrape may cause intense pain, and even 
washing or rubbing the skin may give rise to much discomfort. Occa- 
sionally the sense of position is more affected than cutaneous sensibility. 
Another symptom which results from loss of sensation, but expresses 
itself by disturbance of motion, is ataxia or incoordination. It is not 
frequent although occasionally it may be the most prominent feature. 
Incoordination becomes a prominent symptom when the afferent nerves 
from the muscles and joint are unduly affected. Dejerine has given 584 
DISEASES OF THE NERVOUS SYSTEM 
this condition the name neuro-tabes peripherica, owing to the similarity of 
its symptoms to those of tabes dorsalis; but in his two original cases, as 
well as in others, the dorsal spinal roots and the dorsal columns of the 
cord were intact, and the disease was limited to the peripheral nerves. 
The ataxia is generally apparent only in the movements of the lower limbs, 
and especially in walking. The gait may closely resemble that of tabes 
dorsalis, and the similarity is increased by the fact that the patient is 
more unsteady in the dark or when his eyes are closed. Ataxia of this 
type must be distinguished from the disorders of movement which may 
result from paresis alone; it may be observed in cases in which the 
muscular power is intact or relatively little affected. In some cases the 
ataxia precedes the development of paralysis; in others it may remain 
the chief symptom. In the latter class it often sets in rapidly, and is 
usually accompanied by at least some diminution of cutaneous sensibility 
and frequently by severe pains and muscle tenderness. The sense of 
position and of movement is diminished in the majority of these cases. 
Reflexes.-Loss of the tendon reflexes-including the knee- and ankle- 
jerks in the lower limbs, the biceps and triceps jerks in the arms-is one 
of the cardinal signs, and the most delicate test we possess of disease of 
the peripheral nerves. The knee-jerks may be absent not only when 
there is definite palsy or loss of sensibility, but also in chronic alcoholism 
when there is yet no suspicion of neuritis. Their absence is, however, 
not an absolute rule; they persist in rare cases, despite the presence of 
considerable paralysis, and they have been found definitely exagger- 
ated in the early stages. This is difficult to explain; one theory assumes 
that it is due to the co-existence of a spinal lesion, probably also the 
result of the intoxication that produces the neuritis; another attributes 
it to a state of excessive irritability of the sensory fibres which leads to 
such an exaggeration of the afferent impulses to make it possible for 
them to overcome the obstacle offered by a slight degeneration of the 
motor fibres of the reflex arc. Absence of the tendon-jerks is often the 
most persistent sign of neuritis in cases which improve; they may not 
reappear for years after complete disappearance of the other symptoms. 
The cutaneous reflexes are generally diminished or abolished, but they 
occasionally persist, even when there is considerable motor palsy, es- 
pecially in cases with marked hyperalgesia. The plantar reflexes, when 
they do not disappear, are invariably of the flexor type. 
Sphincters.-The functions of the bladder and rectum are, as a rule, 
unaffected, although in cases in which there is much mental disturbance 
the patient may pass urine involuntarily or have symptoms of retention. 
Occasionally, however, dysuria and retention have been observed; such 
sphincter affection has been generally attributed to the co-existence of 
spinal disease, but this has not been demonstrated, and it is possible 
that the neuritic affection may extend to the nerves of the bladder. 
Trophic and Vasomotor Changes.-Apart from the atrophy of the par- 
alyzed muscles trophic changes are not a prominent feature. In chronic 
cases the skin, especially of the hands and fingers, loses its natural wrinkles 
and becomes thin and shining, but true "glossy skin," which is frequently 
seen after severe' local nerve lesions, is rarely met with in multiple neu- DISEASES OF THE PERIPHERAL NERVES 
585 
ritis. That of the legs is generally dry, rough, and covered with scales 
of desquamating epidermis. The hair and nails become dry and brittle, 
and the latter may be furrowed longitudinally and may grow irregularly. 
Bedsores are rare, and severe trophic changes are almost never seen. 
Of the vasomotor change, oedema is the most prominent; it occurs in 
a considerable proportion of the severer cases. It is generally localized 
and is most common on the dorsa of the hands and feet, and about the 
ankles. In the majority of the cases it certainly develops independently 
of vascular and renal disease, and is probably due to an affection of 
the vasomotor fibres. Sometimes it does not become apparent until 
the patient begins to walk or to sit up with his feet dependent; in other 
cases it may be an early or the initial sign, as it so frequently is in beri- 
beri. Local anemia is occasionally observed, and Ross has reported a 
case of alcoholic neuritis with the symptoms of Raynaud's disease. 
Profuse sweating is common in the early and acute stages, especially 
in the extremities of the limbs. 
Several cases have been recorded in which neuritis set in with or im- 
mediately after an attack of polyarthritis; most probably the two con- 
ditions were here due to the same cause, but in other cases one or more 
joints have been observed to swell and become painful during the course 
of neuritis; the changes may ultimately produce ankylosis. More 
chronic arthritic changes, with pain and peri-articular thickening and 
ultimately limitation of movement, are not uncommon. 
Mental Symptoms.-Psychical disorders are so common in multiple 
neuritis, especially in the alcoholic form, and so frequently of the same 
type, that they may be regarded as part of the disease; in fact some 
authorities speak of these mental symptoms as the polyneuritic psycho- 
sis. Korsakow first carefully analyzed and described them in their most 
common form (see Vol. II, p. 427). 
Course.-This is very variable; it depends largely on the cause, more 
on the stage in which the patient comes under treatment and when the 
action of the poison is arrested. As a rule, the more severe and acute 
the symptoms the longer will be their duration; they generally increase 
for a time after the alcohol has been stopped, and then remain station- 
ary until improvement sets in. But this can be rarely expected within 
one or two months. The first sign of improvement is usually diminution 
of the pains and cutaneous hyperalgesia, but tenderness of the muscles 
often persists until recovery is almost complete. The disturbances of 
cutaneous sensibility generally disappear before there is much return of 
power. This occurs first in the muscles which were last and least affected, 
that is, in those which move the proximal joints of the limbs, and pro- 
gresses distalward, but only very slowly; in even the most favorable 
cases it requires months for muscles that were definitely paralyzed to 
regain their functions. On the other hand, complete return of power in 
all muscles may be expected eventually in almost all cases which improve, 
provided that the patient does not revert to the abuse of alcohol. As 
the paresis disappears the wasted muscles slowly regain their bulk, but 
may not attain their normal size for months. The most serious obstacle 
to complete recovery is the shortening and contracture of the antagonists 586 
DISEASES OF THE NERVOUS SYSTEM 
of the most affected muscles. It is very frequently seen in the calf 
muscles; the ankle is then more or less fixed in the extensor position 
and the foot cannot be dorsiflexed, so that the patient is unable to get 
his heel to the ground on attempting to walk. The absence of the 
tendon-jerks, and alterations in the electrical reactions are usually the 
last persisting evidence of the disease in these favorable cases. 
The course of alcoholic neuritis is not always so favorable. In cases 
with acuter onset, in which the limbs become powerless within one or 
two weeks, the paralysis may spread to the muscles of respiration and 
produce a fatal result; and not infrequently death occurs from cardiac 
failure in the acute stage of the disease, owing either to paralysis of the 
vagi nerves, or to direct affection of the heart muscles. Cases have been 
recorded which have come to a fatal termination within a week, but 
probably most of these were cases of Landry's paralysis. 
The chief complications are the effects of alcohol on other organs, as 
the heart, liver, and gastro-intestinal tract. Pneumonia and bronchitis, 
which occur not infrequently, are serious complications. Pulmonary 
tuberculosis is perhaps the most frequent of the serious complications 
and probably the most common cause of death. Its relation to the 
neuritis may be twofold; on the one hand, the tuberculous toxin may be 
an etiological factor, especially when combined with other causes, as 
with alcohol; on the other hand, alcoholic subjects are very liable to 
tuberculosis, or latent foci may become active when the patient is con- 
fined to bed with neuritis. 
Diagnosis.-When the essential features are recognized this can present 
no difficulty in the majority of cases. The wide distribution of the 
affection of both motor and sensory functions, its rigid symmetry, and 
above all its greatest intensity in the distal segments of the limbs, char- 
acterize the disease in the typical cases. It is, however, very important 
to remember how variable the symptoms may be in different forms, and 
even in cases due to the same cause. 
The condition with which it may be most easily confused is perhaps 
acute poliomyelitis; it is little over a quarter of a century since a definite 
distinction was drawn between them. The difficulty is chiefly present 
in cases which occur in early life, as then multiple neuritis is relatively 
rare, while infantile palsy is common. The onset of poliomyelitis is, 
however, more acute, and is generally accompanied by severer constitu- 
tional disturbances; the paralysis is rarely symmetrical, and is often 
more marked in the proximal than in the distal segments of the limbs; 
further, complete paralysis of the abdominal and trunk muscles is not 
uncommon, while in multiple neuritis it is exceptional. Sensory dis- 
turbances and the severe and persistent neuritic pains are absent, and 
although the paralyzed limbs may be at first painful to pressure or move- 
ment this soon disappears and the nerve trunks are not unduly tender. 
Neuritis is generally progressive for a few weeks, while poliomyelitis 
usually comes to a standstill in a few days and the muscles least affected 
recover quickly; then the irregular and random distribution of the 
paralysis is evident. 
The differential diagnosis betweefl multiple neuritis and Landry's 587 
DISEASES OF THE PERIPHERAL NERVES 
paralysis is more difficult. The course of the latter is more acute, and, as 
a rule, death results within ten days or a fortnight; the constitutional 
symptoms are generally slight and the sensory phenomena are limited 
to pain in the back and occasionally slight numbness in the extremities; 
definite anesthesia does not occur, and neither the nerves nor muscles 
are tender to pressure. But its most characteristic feature as contrasted 
with polyneuritis is that the muscles of the trunk and of the limbs are 
equally affected, and that the paralysis ascends the trunk from the legs 
to the arms, and does not appear in the hands and feet simultaneously 
or successively and involves the musculature of the trunk last, as does 
multiple neuritis. Finally, the paretic muscles rarely waste and the 
cranial nerves are usually affected only when the limbs and trunk are 
more or less completely paralyzed. 
The ordinary form of multiple neuritis, in wdiich there is widespread 
palsy, can be scarcely mistaken for tabes dorsalis, but in the cases in 
which sensory loss and ataxia are the most prominent features diagnosis 
may be extremely difficult. Neuritis is, however, distinguished by its 
more rapid evolution, and by the absence of lightning pains, of sphincter 
disturbances, optic atrophy and alterations in the pupillary reactions. 
On the other hand, in tabes there is, as a rule, no true paralysis, and 
when muscular .atrophy does occur it is usually limited to one group 
of muscles, and is rarely symmetrical from the beginning. But the most 
•valuable diagnostic sign is that while in neuritis the nerves and muscles 
are invariably, in some degree at least, tender to pressure, in tabes the 
pain reaction on squeezing the nerves and on deep pressure on the muscles 
is almost always diminished and frequently quite absent. The history 
of infection, an excess of lymphocytes and globulin in the cerebrospinal 
fluid, and a positive Wassermann reaction are in favor of tabes. 
The symptoms of trichiniasis may resemble those of polyneuritis, but 
the acute onset with fever and gastro-intestinal symptoms, the oedema 
about the eyelids, and the swelling and tension of the affected muscles, 
as well as the character of the pain in them, are fairly characteristic. 
The eosinophilia and the early affection of the functions of the cranial 
nerves are also distinctive features. 
With general oedema there is often a certain degree of flaccid paresis 
of the limbs, especially of the legs, and loss of the tendon-jerks. In some 
of these cases there may be a slight peripheral neuritis due to a general 
toxic state, but the rapidity with which the paresis disappears, and the 
early return of the tendon-jerks as the oedema subsides, makes it more 
probable that the paresis is a direct mechanical or nutritional effect of 
the effusion into the muscles. 
Acute myositis may be diagnosed from polyneuritis by the swelling 
of the extremities owing to inflammatory oedema of the subcutaneous 
tissue and muscles, the rigidity of the muscles, the absence of sensory 
disturbances, and the erythematous rash which is nearly always present 
over the affected parts. Finally, in myositis the knee-jerks often per- 
sist. A form of paraplegia in old people due to contractures following 
chronic myositis, which has been described by Lhermitte1 and others, 
1 Nouv. Iconographie de la SalpNri'ere, 1906, xix, 251, These de Paris, 1907. 588 
DISEASES OF THE NERVOUS SYSTEM 
may be confused with polyneuritis. But it rarely occurs before the age 
of seventy-five, and generally only in feeble persons confined to bed. 
Like neuritis, it may set in with pains, cramps, and weakness of the legs, 
but it can be easily distinguished by the persistence of the knee-jerks, 
the normal electrical reactions of the muscles, and the firm contracture 
of all the muscles which are affected. 
Prognosis.-The outlook naturally depends on the acuteness and 
severity of the attack. In the early stages, when the paralysis is spread- 
ing rapidly, prognosis must be guarded, as there is always the danger 
that the affection may involve the cardiac or respiratory apparatus and 
lead to sudden death. But it is often surprising how patients in whom 
the diaphragm is paralyzed, or the heart dilated, irregular, and rapid, 
recover. The disease almost invariably progresses for a time after the 
poison has been withdrawn, but when its course once becomes station- 
ary there is very little danger of relapse, even though improvement is 
slow. The chief danger to life results from the complications, especially 
from tuberculosis; if these can be excluded, and the cardiac and respira- 
tory functions are not disturbed, the outlook as regards life is very favor- 
able and the chances of ultimate complete recovery are excellent. The 
restoration of function is always slow and even in cases of moderate 
severity four or six months must elapse before the more -severely affected 
muscles have fully regained their power. The electrical reactions are a 
valuable guide in estimating the duration of the muscular paresis. 
Treatment.-The first essential is to discover and remove the cause, 
or stop its action as soon as possible. In dealing with alcoholic neuritis 
this is often not easy, especially with female patients who take every 
possible means to deceive the medical attendant and obtain further 
supplies of alcohol. Frequently it is necessary, and when possible it 
is always advisable, to place the patient in a home or hospital under 
the care of trained and reliable nurses; if he remains in his own home 
he will probably succeed, even if bedridden, if it is in any way possible, 
in procuring stimulants. Many authorities recommend that alcohol 
should not be suddenly stopped, owing to the danger of collapse; but 
this is rarely to be feared except when the heart is very feeble, and it 
is certainly inadvisable to continue the administration of a drug which is 
producing such serious symptoms. 
Rest in bed is always advisable, except perhaps in the mildest cases; 
the patient is thus most easily protected from exposure to cold, which 
may not only have an unfavorable influence on the neuritis, but also 
make him more liable to pulmonary complications. Complete rest is 
also a matter of great importance, as movement often seems to intensify 
the changes in the degenerating nerves, and it certainly increases the 
pain. In severe cases the use of a water or air bed is generally advisable, 
as on it the necessary pressure is more uniformly distributed, and the 
patient does not suffer so much as when his limbs lie on a firmer and ill- 
adapted surface. Much relief can be given by removing the weight 
of the bed-clothes by bed-cradles. 
There is no specific treatment; it can be, therefore, in the acute stages 
at least, only symptomatic, and the most urgent and prominent symptom DISEASES OF THE PERIPHERAL NERVES 
589 
is usually pain. Often it is necessary to have recourse to the use of 
drugs; antipyrine and phenacetin are the most effective, and there is 
rarely any contra-indication to their use; when the patient is restless 
and sleepless, bromides may be combined with one or other of them. 
Occasionally the pains are so severe and constant that morphine is 
necessary, but it is evidently advisable to avoid its use in a person already 
addicted to a drug habit. Local applications over the chief seats of 
pain often give considerable relief, especially warm fomentations. Mills 
recommended the alternate application of very warm and cold water 
with a large sponge over the limbs, and Pospischill cold packs. It often 
suffices to keep the limbs wrapped up in a thick layer of cotton wool. 
Oppenheim has obtained excellent results by diaphoretic treatment; 
warm baths may give relief if the patient is strong enough to be moved 
from bed, but this should not be attempted if the heart is feeble. Local 
hot air baths, which can be used in bed, are sometimes very effective, 
and if they are not available warm packs may be employed. 
Another important aim should be to prevent the development of con- 
tractures. The greatest danger is -that the feet become fixed in the 
ankle-drop position, or the hips and knees in semiflexion. From the 
first, steps should be taken to keep the feet at about right angles to the 
legs, by the use of a large sand-bag at the foot of the bed, against which 
the soles of the feet may press, or by a broad, padded board placed 
vertically across the bed. If the calf muscles are already shortening, 
their contraction may be overcome by the constant tension of rubber 
cords stretching from the toes of shoes which the patient wears to a 
band around the limb at about the level of the knee. In the acute stages 
the knees are nearly always held semiflexed, but this should never be 
permitted, as the danger of shortening of the hamstrings is very great. 
Of the many drugs, salicylate of soda and iodide of potassium are 
probably the most frequently used, but there is no evidence that they 
have a specific effect. Attention is better directed to general tonic 
treatment; strychnine in fairly large doses is certainly of considerable 
value when there is any tendency to respiratory embarrassment or car- 
diac failure. Arsenic should be rarely given, and only in small doses and 
under constant supervision, as it itself may be a cause of neuritis. Diet 
needs some attention; it should be as light and nourishing as possible. 
During the stationary stage and when improvement is setting in 
galvanism may be employed, but it should not be adopted in the early 
stages when tenderness of the muscles is still very great. Large electrodes 
should be used, and the current should be the weakest that can produce 
a definite contraction of the muscle fibres and yet is not strong enough to 
give pain. Massage also may be of considerable service, but it should not 
be resorted to in the acute stages or while the limbs are still very tender. 
At first it should be as gentle as possible. 
The patient should be encouraged to move the affected limbs as much 
as possible, when movement does not give him any acute pain, as vol- 
untary movement is undoubtedly a more effective stimulus to nutrition 
than either galvanism or massage, and voluntary effort probably exerts a 
favorable influence on regeneration. If contractures have been allowed 590 
DISEASES OE THE NERVOUS SYSTEM 
to develop, tenotomy may be necessary, but they can be generally over- 
come by systematic passive movements and extension. 
Acute Infective Polyneuritis.-The group of cases included under 
this title has been variously described as rheumatic, infective, or spon- 
taneous neuritis, as it is rarely possible to ascribe the attack to any 
definite cause; or as febrile polyneuritis, since the disease usually sets in 
with febrile symptoms. Many cases follow or are attributed to cold, 
exposure, or overexertion, but it is difficult to see how these factors can 
directly produce such widespread and progressive symptoms, or the exten- 
sive parenchymatous neuritis to which they are due. It is very probable 
that the polyneuritis is directly due to some bacterial infection, which 
may not be always of the same nature. Its onset is frequently preceded 
or accompanied by general malaise, pains in the back or limbs, a rise of 
temperature to perhaps 104° F., gastro-intestinal symptoms, and loss of 
appetite. In the severer cases the attack may be ushered in with a rigor; 
swelling of the spleen, profuse sweating and albuminuria, as well as 
vomiting and diarrhoea, have been observed. Its infective nature is 
further supported by the occasional occurrence of small epidemics, for 
which no definite cause can be discovered. 
Generally, as the febrile symptoms are subsiding the patient begins 
to complain of numbness, tingling, or other subjective sensations in the 
fingers and toes, and occasionally of sharp or burning pains, but these 
are rarely so severe as in alcoholic neuritis. Within a short time some 
loss of muscular power appears, generally in the legs before the arms, 
but it is more or less symmetrical and greater in the distal than in the 
proximal segment of the limbs. In rare cases the arms are affected 
before and more severely than the legs. 
The disease is a typical parenchymatous neuritis. Its clinical features 
are practically identical with those which have been described under 
alcoholic neuritis, for no set of symptoms is exclusively related to a 
single cause; but there are a few characteristic points. The paralysis 
is, as a rule, more generalized than in alcoholic neuritis of corresponding 
severity, the trunk muscles are usually relatively more affected, and even 
complete bilateral flaccid facial palsy is by no means uncommon. Cases 
may be seen in which, though the limbs are not very severely affected, 
the patient is unable to close his eyes, his articulation is indistinct owing 
to loss of the labial letters, and as he attempts to drink, fluids dribble 
from the corners of his mouth owing to the palsy of the lips. Not in- 
frequently, too, the palatal and pharyngeal muscles are weakened in 
some degree, so that swallowing becomes difficult and food may regur- 
gitate through the nose. Laryngeal paresis, producing aphonia, is rarer. 
There is also, as a rule, less sensory disturbance than in the alcoholic 
cases; spontaneous pains may be slight and even occasionally absent, 
and although the nerves and muscles are always tender to firm pressure, 
they are rarely as extremely so as in alcoholic paralysis. 
This form generally runs a fairly rapid course, often reaching its acme 
in a week or ten days, and then, after a stationary period in favorable 
cases, the patient begins to improve. Fatal terminations, which are not 
infrequent, generally result from paralysis of the respiratory muscles or DISEASES OF THE PERIPHERAL NERVES 
591 
from heart failure. Acute cases which die within ten days may so closely 
resemble Landry's paralysis that distinction is difficult if there is but 
little sensory change, or if the evolution of the disease has not been 
observed. There is, however, generally more constitutional disturb- 
ance than with the onset of Landry's disease, and the early affection 
of the cranial nerves in the neuritis is an important point in the diagnosis. 
Recurring Polyneuritis.-Under this term Mary Sherwood1 described 
two cases in 1891, although one of them, in which only the nerves of one 
arm were affected, certainly does not belong to this class. 
These cases are characterized by a marked tendency to repeated 
attacks of multiple neuritis for which no definite cause can be ascer- 
tained; cases in which the successive attacks are due to renewed expos- 
ure to some poison, as alcohol or lead, are naturally excluded from this 
group. H. M. Thomas,2 who reviewed the subject in 1898, was then able 
to collect only seven cases, and Hoestermann,3 who published three 
original cases, could only add three additional records. The one ob- 
served by Thomas is most typical: A man, aged twenty-eight years, 
otherwise healthy, who did not indulge in alcohol to excess and was 
apparently not exposed to any poison, developed each June for five 
years an attack of typical multiple neuritis, from which he slowly recov- 
ered in four to six months. Remak and Fletan4 point out that the disease 
is often atypical, and that one or more of the cranial nerves have been 
frequently involved. It is difficult to say whether the repeated attacks 
are the result of an unusual predisposition, or if the first attack leaves 
the nerves more liable to a second. Dejerine-Klumpke and Bernhardt 
have observed recurrent lead palsy apart from renewed exposure to the 
poison. One of these cases reported by Barnes5 as "Toxic Degeneration 
of the Lower Neurones," and that recorded by Williamson6 under the same 
title, probably belong to this class. 
1 Virchorvs Archiv, 1891, cxxiii, 166. 
2 Phila. Med. Jour., 1898, i, 885. 
3 Deutsche Zeitschr. f. Nervenheilk., 1914, li, 116. 
4 Nothnagel's Spezielle Pathologie und Therapie, 1900, Band xi, 3te Teil. 
5 Brain, 1902, xxv, 479. 
6 Ibid., 1903, xxvi, 206. CHAPTER XV. 
EPILEPSY. 
L. PIERCE CLARK, M.D. 
Synonyms.-Epilepsy was derived originally from the Greek epilepsia 
(epi, upon, and lepsis, seizure), a disease of seizures. The word persists 
with the original significance in the Latin, epilepsia, French, epilepsie, 
Italian, epilessia, etc. The Romans designated the affection as morbus 
commitialis (the attendant malady) and also as morbus caducus (falling 
sickness); in the latter form it persists in most European languages- 
German, fallsucht, fallende sucht; Italian, mal caduco; English, falling 
sickness, etc. 
Definition.-Epilepsy is a chronic disorder of the central nervous 
system characterized by more or less frequently recurring convulsive 
attacks attended by a loss of consciousness. Both phenomena may vary 
much in duration and intensity. 
Introduction.-The number of individual elements to be considered 
in epilepsy is very large, and includes the seat of the disease, which is the 
entire brain, the motor and psychical phenomena being referable to the 
cortex. The overexcitable brain and a peculiar psyche may exist without 
active manifestations (latent epilepsy). Local lesions in the brain, 
peripheral irritation, may gradually produce the epileptic brain. The 
seizure types include the major convulsive paroxysms (grand mal), 
usually complete, but at times incomplete or atypical, and petit mal, 
or the abortive seizure. In the typical major seizure there should be 
prodromes (aura), loss of consciousness, convulsions, and a post-paroxys- 
mal state of exhaustion, stupor, and confusion. 
While we speak of focal epilepsy or Jacksonian spasms as distinct 
from genuine epilepsy, being of organic origin, the latest authors tend 
more and more to break down the wall between. It is still convenient, 
however, to distinguish between true, genuine or idiopathic epilepsy, and 
seizures due to actual macroscopic lesions known as organic or focal. 
The interpar oxystic state and epileptic mental deterioration-which super- 
vene surely as a result of the injury inflicted by years of convulsions 
-are allied subjects which belong to the psychic expressions of the disease. 
The so-called isolated psychic equivalent, the existence of which is disputed 
by some authors, cannot readily be separated from post-convulsive 
confusion, and may be considered under the same head. Confusion, 
with hallucinosis, leads the patient to perform foolish, immoral, and often 
criminal acts, and forms an important feature of forensic medicine. 
In no country is the exact number of epileptics known. It is probably 
not far from 1 in every 300 to 500 of the general population in the United 
States. In regard to sex, figures show much variation. Gowers finds 
592 EPILEPSY 
593 
women to outnumber men (43 per cent., male, 57 per cent., female; also 
Morselli, 46 per cent, male, 54 per cent, female). In most reports the 
males are in excess, either slight or considerable (Binswanger, 62 per cent, 
male, 38 per cent, female). The ages are no doubt responsible for want 
of parallelism, as the sex frequently varies with the decades. The age 
at the outbreak of the disease varies. According to Gowers about one- 
eighth of all cases begin in the first years of life. 
As regards deaths in epileptics: The status epilepticus is the cause of 
at least 20 per cent, of the deaths to be directly ascribed to accidents of 
the disease itself, while 20 per cent, are due to oedema, congestion of the 
lungs, aspiration pneumonia, and the like. Asphyxia in attacks shows 
approximately 4 per cent, of the deaths. The epileptic deaths in Hahn's 
statistics stand in relation to the general deaths as 343 to 541 (63.4 per 
cent, of the whole deaths). Of deaths not ascribed to epilepsy we note a 
relatively low incidence of tuberculosis, influenza and lobar pneumonia, 
cancer, nephritis, cirrhosis of the liver, diabetes, etc., which appears to 
show that the epileptic either per se or as a result of segregation shows a 
remarkable immunity to certain world plagues. Deaths by means of the 
heart, vessels and lungs are very numerous. 
The mortality of epileptics is not much above that of the general 
population and is much lower than that of the insane as a class. How- 
ever low the mortality among epileptics is, their longevity as a class is 
not as great as the average man. The epileptic insane perish nearly ten 
years earlier than the epileptic not so designated. Mortality increases 
both with the menace to life from the disease and the increasing age. 
The menace to life is in part due to accidents. The not very great increase 
in mortality over sound subjects is to be traced to the number of con- 
vulsions and cumulative or progressive character of the disease, and this 
is offset in part by robustness of constitution. If the dangerous accidents 
could be eliminated, the result would favorably influence the percentages. 
This is largely realized in colony life. The mortality of colonized epileptics 
is naturally much less than in uncontrolled epileptics. 
Etiology.-Predisposing Causes.-The most striking fact in connection 
with the hereditary nature of epilepsy is that at least 20 per cent, of 
epileptics present no evidence of neuropathic ancestry or indeed of any 
other causation. That epileptic children should be born of neuropathic- 
degenerative stock, including alcoholics, is almost a foregone conclusion; 
but in most cases epileptics do not beget epileptics. From a common 
stock we see develop neuropathy or psychopathy, degeneracy, imbecility, 
insanity-expressed very largely in antisocial units like paupers, drunkards, 
vagrants, prostitutes, etc. In other words, an epileptic tends to be born 
from such stock. But the epileptic may at times descend directly from 
the epileptic, and in any case, the epileptic brain is not merely a psycho- 
pathic brain, but a something added. Literature abounds in statistics 
of heredity. Moreau noted years ago that one-sixth of his epileptics 
were of epileptic parentage. 
Nothing has as yet superseded Echeverria's statistics. He was able 
to trace the progeny of 136 married epileptics (one or the other parent) 
who procreated 553 children. Of this number 78 were epileptics while 594 
DISEASES OF THE NERVOUS SYSTEM 
185 died of infantile convulsions. This author, by using all available 
statistics, concluded that 14.10 per cent, of all epileptics have direct 
epileptic ancestry-a figure doubtless too small, if we reckon some of the 
victims of infantile convulsions as epileptic. Familial epilepsy is not 
rare. The best examples of this are to be found in myoclonus epilepsy. 
Why the ovum is of such constitution as to develop a psychopathic 
or epileptic brain is, of course, impossible to answer. Hence there has 
been much speculation as to the part played by conception while intoxi- 
cated, and the excessive use of alcohol and other race poisons after con- 
ception-upon the supposition that the highest faculties suffer first and 
chiefly. 
The closest equivalent of epilepsy is imbecility. The two constantly 
occur in relatives or in the same subject. Children begotten at times when 
a large proportion of a community is presumably intoxicated, rather 
tend to imbecility than epilepsy, although the latter disease may show 
some increase. In alcoholism in the parents continental statistics usually 
show an unfavorable influence upon the ovum; but in some figures 
relating to England quoted by Turner, children of alcoholics showed no 
especial tendency to the disease, even the reverse. 
The influence of " race poisons"-minerals and toxins of disease, upon 
the embryo and fetus (without special regard to syphilis), has shown 
ever that such children are born small and of low vitality, nutrition, 
etc., but without any especial blight on the brain. That is, these sub- 
standard children may reach complete development, but are small and 
puny and with low vital capacity. The children of victims of saturnism 
are said to have convulsions. 
The question of maternal impressions, i. e., violent shock to the preg- 
nant mother, seems of some minor importance. The women will chiefly 
be found of neuropathic stock. This origin, however, applies to all 
forms of psychopathy and degeneracy. Violent mental or mental and 
physical shock in a gravida may be followed by the birth of a degenerate 
child, a "black sheep" so-called in a sound stock. 
Birth traumatisms, in addition to prenatal lesions, are doubtless able 
to "epilepticize" a brain, especially if the stock is tainted. Molecular 
changes may exert the same power (Binswanger). Another aspect of 
epilepsy in the infant is its relationship to simple eclampsia infantum. 
It is only recently that the labors of pediatrists like Theimich and Birk 
have shown that there is no actual connection between the two although 
they may both develop upon the same soil. Infantile convulsions in the 
first weeks of life are as a rule a sign of incapacity for life. Theimich 
and Birk followed 53 such cases up to the ninth to twelfth year and 
not one became epileptic, although not a few showed mental inferiority. 
Authorities like Redlich and Potpeschnigg have good arguments to 
the contrary, such as the occurrence of the Chvostek sign in epilepsy, 
and the not infrequent passage of spasmophilia into epilepsy. In a 
particular series of cases infantile convulsions led up surely to epilepsy. 
When infantile convulsions are carefully analyzed, they will be found 
to differ notably from an epileptic paroxysm. There is always some 
reflex irritant and the temperature rises. The cerebrum is involved EPILEPSY 
595 
very deliberately. A preliminary stage consists either of general motor 
unrest or stupor with staring eyes and dilated pupils. Convulsions then 
begin in the facial muscles. Loss of consciousness is a later development. 
Tonic contractions appear in the lower jaw, larynx, pharynx, and tonic- 
clonic movements in the extremities. 
A degenerative stock is but slightly in evidence in the first six months. 
There may be only increased reflex excitability. Other authorities see 
a proved connection between infantile convulsions and nutritive dis- 
turbances. Aside from these, certain irritable zones, etc., give rise to 
reflex convulsions; these include the gums, stomach, urinary organs, and 
others. In addition to all these causal factors one may invoke an heredi- 
tary factor in about one-half of all cases. 
It seems reasonable to infer that about one-half of all epileptics owe 
their disease to antenatal factors, which eventually lead up to the typical 
epileptic brain. The disposition is there and the causes which bring 
it out may be mild or severe. 
In analyzing the subject of etiology into its original elements, we find 
that intoxications and infections play a leading role. This naturally 
leads us to the part played by the acute and chronic infectious diseases, 
with especial reference to tuberculosis, syphilis, alcohol, and all other 
world plagues. Marie and others lay much stress on such diseases as 
scarlatina, pertussis and typhoid of childhood as conditions which pre- 
dispose to epilepsy. Binswanger and others have written at great 
length on the complete relationship of epilepsy and syphilis. As already 
stated, very few cases of the former react positively to the Wassermann 
test. This small number does not include the paretics who as a class are 
subject to convulsions. There is but a single established type of syphilis- 
epilepsy, to wit, the Fournier, due to focal syphilitic lesions in the brain. 
In rare instances of the Fournier type, no gross lesions are present. 
There may be local spasms due to lesions in one or both hemispheres. 
The brain then may undergo the special epileptic alteration and general 
convulsions develop. Recent syphilis may light up epilepsy in the pre- 
disposed. 
Binswanger, who after infinite pains is admittedly unable to solve the 
riddle of syphilitic epilepsy, states that the latter expression is merely 
a collective term for any kind of spasm occurring in syphilis. There are, 
despite all adverse criticism, two distinct forms of syphilis of the brain: 
(1) para- or meta- or post-syphilis, and (2) the tertiary gummous 
syphilis. The former, despite what is said adversely, may occur at any 
period of syphilis, beginning with the secondary. Paresis and tabes are later 
developments. The tertiary gummatous syphilis may be placed under the 
focal or Jacksonian type. The subject of syphilitic epilepsy is at best unsat- 
isfactory to deal with. Why should not an epileptic youth contract syphilis? 
Why should he not develop paresis? Why should not a syphilitic infant 
become an epileptic of the ordinary sort when we consider his usual 
ancestry? But after all, when but 2 or 3 per cent, of epileptics taken at 
random give the Wassermann reaction, why does not this small coinci- 
dence of the two diseases show that the subject is not one of prime 
importance ? 596 
DISEASES OF THE NERVOUS SYSTEM 
Convulsions of various kinds occur in users of spirits, but in ordinary 
alcoholism epileptic attacks are not very frequent. It is admitted 
that neuro- and psychopaths and epileptics usually offer but little resist- 
ance toward alcohol. Alcoholic indulgence often causes relapses. 
In the convulsive and delirious reception-ward at the Berlin Charite 
there was a notable association between these conditions. The figures 
showed that from 36 to 40 per cent, of deliriants were epileptic. In 
alcoholic mental disturbances there was about 10 per cent, of epilepsy. 
Fiirstner found that nearly one-third of delirium tremens cases were 
epileptic. 
Trauma, etc.-Nothnagel has seen a case with positive clean history 
become a confirmed epileptic after a blow on the head. A somewhat 
different type exists in which the injury causes fractures of the skull and 
wounds of the brain, with scar formation. The injury causes both shock 
and irritation. These stand in some relation to Jacksonian as well as 
ordinary epilepsy. The relationship of injuries producing simple con- 
cussion to epilepsy has caused authorities to suspect that many cases 
of epilepsy dating from early childhood may have originated in over- 
looked falls-a favorite view of the laity. 
Injuries and other sources of irritation at a greater or less distance 
from the brain, have been held responsible for a type of epilepsy familiarly 
known as "reflex epilepsy." The confusion here is very great; even 
lesions within the cranium may act peripherally on the cortex. The 
subject is best studied when cortical foci alone are present. The action 
upon these of the "summation of irritations" might gradually extend 
from the foci to the entire cortex until an epileptic brain results. But 
within late years the distinction between true and partial epilepsy is 
gradually disappearing. The infracortical, partial and Jacksonian spasms 
are considered to be of the same nature. From the cortex both local and 
general convulsions arise; from the infracortical ganglia there arise tonic 
spasms, shivering or trembling spasms, entirely nonepileptic in character. 
In the narrower sense of the term "reflex epilepsy" we refer to epileptic 
convulsions associated with injuries to peripheral nerves. There may 
be convulsions of this origin which are by no means epileptic. Merely 
regional spasms may be produced by severe irritation of the nerves; 
among these are included tremulous and shivering movements. In such 
cases there is extension to other muscle groups until the entire body is 
involved, but these convulsions never extend above the cord, and seldom 
cross from one side to the other. The cortical symptoms are sensory- 
vertigo, confusion, and complete loss of consciousness. 
That attacks of epilepsy may begin with certain peripheral irritation 
and continue until the latter is removed is attested by very many cases. 
That caries of the teeth may be the cause of the paroxysms, which may 
subside or disappear, is vouched for by Fere. An epileptic who had not 
had an attack for ten years developed one on the breaking through of a 
wisdom tooth, and had consecutive attacks. Many similar cases have 
occurred in the nasopharyngeal and aural regions. 
We may make three groups of reflex epilepsy: (1) Where the relation- 
ship is obscure; where trauma causes ordinary epilepsy, and where EPILEPSY 
597 
connection between wounds and spasms is not absolute. (2) Where the 
patient may have had a strong inborn tendency to epilepsy, and there 
may be no direct evidence that the injury caused the spasms. (3) Where 
the relationship between scars and convulsions may be demonstrated 
at will. Members of the latter class possess the so-called epileptogenous 
zones, chiefly scars. A convulsion may be caused by pressure on a scar. 
If we review these epileptogenous zones they appear to consist of two 
types. In one the zone lies in the distribution of the injured nerve. 
In the second type there is no apparent connection between the epileptic 
zone and the injured nerve. The convulsions do not appear to be epi- 
leptic, but local and psychogenic. 
Psychic Influences.-These are well known for their power to pre- 
cipitate individual attacks, as a rule only the first one. According to 
Gowers this holds good for 75 per cent, of cases. The emotions con- 
cerned are not necessarily those of anger or terror, for they are at times 
joyful. In such cases, however, the patient will be found neuropathic, 
probably with a labfle vasomotor system. Fright seems to be active 
at puberty, especially in girls. According to Gowers, there is always an 
interval between the fright and the fit, but Binswanger finds many 
exceptions. 
Psychic infection-the spread of a case to bystanders-has often 
been seen. In the Middle Ages, some towns officially regarded epilepsy 
as contagious. No doubt these were examples of what is now termed 
affect epilepsy, the number and variety of which are constantly increas- 
ing. The subjects are psychopaths, and exhibit seizures which in many 
cases can be differentiated only with great difficulty from true epilepsy. 
The seizures are chiefly psychogenic in character and naturally resemble 
hysteria. In institutions seizures may occur at any friction with officers 
or other inmates. Fever, great heat and alcohol act like psychic insults. 
The so-called epileptiform convulsions due chiefly to poisoning of any 
kind, whether exogenous or endogenous, are no longer separated radically 
from epilepsy, because when not mortal in character they are able at 
times to set up genuine epilepsy; of crises of any kind, fits of laughter, 
coitus, etc., the same may be said. 
Many external factors, such as hypertrophy of the heart, poorly re- 
fracting eyes, etc., while not in evidence as causing crises, have much to 
do with their production. Thus in 61 per cent, of cases of epilepsy there 
are errors of refraction and other anomalies of vision.1 Properly fitted 
lenses often cause improvement and sometimes recovery. The influence 
of the heart is so marked in certain cases that the condition is termed 
cardiac epilepsy. In such cases cardiac sedatives form a necessary in- 
gredient in the therapy. 
Despite all the preceding evidence in regard to the first attack, in a 
third of the cases submitted to statistics there were absolutely no causal 
factors discoverable (Lomer). 
Special forms of true epilepsy which may be considered from the 
etiological standpoint are not numerous. We have a few exceptions, as 
1 Schon and Thorey, Arch. f. Psych., vol. xxxix. 598 
DISEASES OF THE NERVOUS SYSTEM 
nocturnal and menstrual. The influence of the weather-attacks appear 
more readily during any change of air pressure-gives us nothing by which 
to name a class. While mental overwork is hardly enough to determine 
attacks, it is of undoubted influence as a contributory element. There 
is no doubt that dreams have determined many first attacks but such 
instances are more properly classed with so-called affect epilepsy. 
In speaking of prodromes we have to consider not only the near but 
the so-called remote type. This, being volatile and vague, is not often 
seen; we do not know how often it occurs. This question is, however, 
of great forensic value. Several days before the paroxysm a distinct 
psychical and nervous alteration is perceptible. The disposition becomes 
irritable; small occurrences cause anger, contrariness, and suspicion. 
The patients feel injured, insulted, suppressed, and yield to impulses of 
violence. They also feel depressed, ill, uncomfortable, and apprehensive, 
with a sense of tension or pressure in various localities. Fearful dreams 
and insomnia are powerful factors in the psychical prodromes. As the 
hours wear on headache becomes worse, there is confusion, vertigo, and 
inability to do manual labor. The limbs are tired and heavy, the skin 
has burning and creeping sensations, and there are nausea, yawning, and 
hiccough. The part played by the vasomotor system is marked, and may 
affect nearly all the tissues, notably the mucosa?. 
The "epileptic psyche" is fully in evidence in epileptic idiots and 
degenerates and old epileptics. While it is true that chronic epilepsy 
is usually complicated with some other mental state, old or new, there 
is a class in which the brain was evidently intact before the disease 
developed. Yet with intact brain there were nervous peculiarities. The 
patient might be termed temperamental in certain directions; he is a 
neuropathic, exhibiting many of the severe phenomena of this con- 
dition. The epileptic psyche is perhaps best seen in children. They 
present an odd mixture of confusional states, frequently accompanied 
by excitement and violence. They often vary their states of irritability 
with cruelty (that shows strong sadistic factors) and dullness and apathy. 
The rapid and seemingly unaccountable change of temperament and mood 
is strangely misinterpreted until the epileptic state is fully disclosed by 
paroxysmal phenomena. 
Pathology.-This subject is comprised under three heads, viz., gross 
anatomical changes (per se), minute anatomical changes (per se), and 
finds which connect organic with genuine epilepsy (Binswanger). 
Gross Changes. - These are found in great number and variety in 
epilepsy but in general are non-specific in character, for they occur also 
in idiocy and infantile cerebral palsies. They represent congenital errors 
of development or lesions apparent soon after birth and in earliest infancy. 
We have to distinguish between simple arrest of development and actual 
pathological processes. Thus numerous conditions coming under the 
head of scleroses are really examples of the former (tuberous form, 
diffuse and circumscribed sclerosis, the so-called stadium verrucosum 
cortex). Porencephaly, a defect of fetal origin, is occasionally present. 
Of especial interest is a form of lobar sclerosis limited to Ammon's horn 
which is so often seen (50 per cent.) in epilepsy. It has been a subject of EPILEPSY 
599 
debate for decades, and opinions still differ greatly as to its nature and 
significance. The cornua are firm and anemic to the naked eye. Lobar 
sclerosis in general is due to the condition of numerous diffuse foci. The 
whitish appearance distinguishes them from the normal gray matter. 
They may be extremely shallow (plaques) or of various degrees of depth, 
smooth or scarlike. 
Passing to the other group which are not due to mere arrest of develop- 
ment and which may develop during the fetal and early infantile period, 
we find chiefly the results of inflammatory processes-encephalitis and 
meningitis with resulting hemorrhage and softening-conditions which 
are, in fact, the essential causes of infantile cerebral diplegias; the 
traumas of birth, which stand also in direct relationship with the latter 
and which comprise depression of the skull, laceration of the intracranial 
tissues and their consequences. Much more rarely are encountered 
finds which are not inflammatory or traumatic, as chronic hydrocephalus, 
cysts, and tumors. But these which we associate with the newly born 
or very young child do not differ radically from those dating later in 
life, which include trauma of all kinds, the results of intracranial inflamma- 
tions, tumors, granulomata, cysts and in later years arteriosclerosis. 
Minute Changes.-So constant is the association of gliosis with epilepsy 
that with some writers the one implies the other. Alzheimer found the 
condition termed by him marginal gliosis in 40 per cent, of all cases. 
The excessive proliferation of the glia, however, is clearly secondary to 
changes in the neurones. Some of the nerve fibres and cells disappear 
altogether. Such changes have been noted in comparatively recent cases 
and in status epilepticus. Alzheimer (confirmed by Clark and Prout) 
sums up the combined changes briefly as follows: proliferation of blood- 
vessels, large amoeba glia cells, division of nuclei in glia cells, regressive 
changes in ganglion cells, disintegration of axis cylinders. In a much 
larger percentage of cases Alzheimer evidently found no marginal gliosis, 
and in fact nothing to throw light on the disease. Sclerosis of Ammon's 
horn, so often present in genuine epilepsy as to be almost typical, he 
appears to regard as secondary and degenerative. Finally in a very small 
fraction of cases the findings consisted of atrophy of a few convolutions 
with marked disappearance of nervous elements and some increase of 
glia. It should be remarked that the (secondary) gliosis of epilepsy is in 
a sense specific, and quite unlike any other type of gliosis. Recent studies 
of the finer anatomy of sclerosis of Ammon's horn show hypergliomatosis 
associated with actual disappearance of the nerve cells, most strikingly 
seen in the pyramid cells. The two were complemental, varying directly 
with each other. 
Relations Between Structural Changes and Epilepsy.-This subject has 
been foreshadowed in preceding paragraphs and is discussed in other 
sections. It can hardly be dealt with save in a cursory manner. The 
original belief that Ammon's horn is a powerful motor centre having 
been dispelled, lesions of this body can no longer be regarded as causes of 
epilepsy. Marginal gliosis is looked upon by Alzheimer as standing in 
direct relation to mental deterioration and imbecility, which is one of the 
most striking components of epilepsy. Focal lesions in very young 600 
DISEASES OF THE NERVOUS SYSTEM 
children are believed to have the same force as congenital defects, since 
they lead to arrest of development. Such lesions may readily associate 
themselves with typical genuine epilepsy. It is impossible to detect 
any relationship between these lesions and any particular type of true 
epilepsy. Marginal sclerosis is almost never associated with sclerosis of 
Ammon's horn. In theory porencephaly should be an ideal anatomical 
condition for association with epilepsy, but few cases of this association 
occur. The epilepsy which accompanies infantile cerebral palsies is 
genuine. Jacksonian epilepsy does not as a rule belong to autopsy finds. 
Symptoms.-The aura is the first portion of the epileptic seizure, 
but does not appear in over one-third of all cases. It is frequently purely 
sensorial and remembered by the sufferer, which shows that it antedates 
the practically constant loss of consciousness and fall. The more rapid 
and explosive the attack, the less the opportunity for an aura. In such 
cases, on the other hand, the remote prodromes already described are 
relatively common, acting as an aura equivalent. Gowers makes no less 
than seven types. These, however, may be condensed to five: 
1. Psychic Auras. These are emotional or intellectual, the former 
characterized by fear or anxiety, euphoria, joy, etc., appearing suddenly 
or after painful expectation. In the latter all the mental faculties or 
the memory alone may be suppressed. In rare cases of absence of con- 
vulsions petit mal is closely simulated. 
2. The sensorial auras affect the eye and ear and rarely the other 
special senses. The eye may see as if through a fog or behold formless 
colors, sparks, scintillating scotomas, etc.; there may be hallucinations 
of all kinds. The ear may hear loud noises, reports, musical sounds, and 
there may be otic hallucinations. 
3. Auras of bad smells and tastes, disconnected from the presence of 
sapid bodies, occasionally occur. 
4. Sensible awas differ from sensorial in that they affect common 
sensibility. They appear as paresthesias or actual pain. Occurring in the 
toes, the sensation may march up the limb. 
5. The motor aura is varied, less often seen and is related chiefly to 
focal lesions of the cortex. It consists of convulsions, locomotor move- 
ments, and automatism. The cloni are mere twitchings of muscle groups. 
Tonic spasms may be bilateral, affecting the so-called "spastic" muscles 
and following organic brain lesions. They may be ranked under partial 
epilepsy. The locomotive aura is rare per se but not uncommon in noc- 
turnal attacks. They may be unilateral, bilateral, or simply confined to 
one extremity. The automatic aura appears to be voluntary, as in all 
automatism. Thus the patient may seem to be trying to abort an attack. 
The automatism is psychic in origin. The patient is really conscious, 
and the movements may be recollection pictures. The cursive aura, 
which is not to be confounded with epilepsia procursiva, represents 
automatic locomotion a few steps forward, before the fall and loss of 
consciousness occur. The motor aura may take the shape of an inhibitory 
paralysis. It may also begin with sneezing, yawning, coughing, etc.; 
or with pains and paresthesia in the abdomen and pelvis. 
The vasomotor aura is relatively common both as a local or general EPILEPSY 
601 
angiospasm which begins suddenly. Angioparesis also occurs. Local 
angiospasm is expressed by pallor, coolness, and sensory disturbances 
which occur chiefly in the digits. Angioparalytic lesions are seen as 
blotches, redness of the face, neck and trunk, hemorrhages, etc. When 
the vasomotor aura is general there is usually a chill, pallor, and burning 
sensations everywhere, with increased excretion of saliva, tears, and sweat. 
Such vasomotor phenomena may occur in epileptics independent of 
seizures, for vasomotor lability is more or less characteristic of the disease. 
A single type of aura is not as a rule constant for the patient, who may 
show a difference with each convulsion. In reflex epilepsy it may be 
possible to abort the seizure. It is evident that there is no essential 
difference between certain auras and petit mal. 
Seizure Types.-Following immediately upon the aura when one is 
present we see two essential phenomena of ordinary major epilepsy, 
namely, loss of consciousness and falling to the ground. The patient falls 
prone in an instant, with all his weight, often on his face. Being uncon- 
scious he feels no pain. (There are rare exceptions to the rule of uncon- 
sciousness, as shown in a series of my cases in which though the attacks 
were of a severe grand mal character the individuals were fully conscious 
throughout the attacks. Most frequently, however, the tonic clonic 
spasms in such grand mal attacks have not an instantaneous order of 
muscular involvement in all parts of the body at the same time.1) The 
number and variety of injuries from striking the ground or floor are legion. 
The convulsive stage is the most striking and characteristic phase. 
With the patient on the ground or floor, and perhaps in part before he 
lands on the latter, all the voluntary muscles are thrown in a tonic spasm. 
The muscles are chiefly synchronously involved, not seriatim. The 
epileptic cry, which occurs only in about one-half of cases, is bound up 
with the tonic stage but its mechanism is obscure. Since it may precede 
this stage it cannot be due inevitably to forced expulsion of air. In 
tonic spasms the head is usually thrown back; eyes widely open, staring, 
and expressionless; face muscles all tense, the jaws locked together; the 
trunk rigid and immobile, in opisthotonos, or more rarely emprostho- 
tonos; the arms in tetanic extension spasm, rotated inward; fingers 
enclosing thumb, made into a fist. The lower extremities are likewise 
in forced extension and inward rotation, thigh abducted, toes spread 
out and extended or maximally flexed. The cycle of tonic spasms lasts 
as a rule, and upon an average, about thirty seconds. Relaxation of 
muscles is not synchronous-as a rule the lower extremities first, trunk 
next, and head and neck last. 
While the tonic phase shows some order, the next or clonic phase is 
irregular, planless and tending to begin in the lower extremities as the 
tonic spasm wears off. The limbs are flexed and extended in the clonic 
phase. The variety of clonic spasms is such that they can hardly be 
described. Then often succeeds simple flexion and extension, inward 
and outward rotation, etc., the limbs are hurled in every direction, the 
movements suggesting blows, kicks, stamping, treading, etc. The head 
1 See "Conscious Epilepsy," L. Pierce Clark, Am. Jour. Insan., 1909, Ixvi, No. 2. 602 
DISEASES OF THE NERVOUS SYSTEM 
and trunk are also hurled about and the latter is often made to rotate 
back and forth. The cloni of the face are the most shocking. In addition 
to the deformity, these movements cause destructive acts, notably the 
biting of the tongue. The foaming lips result because the cloni of the 
chewing muscles churn the increased saliva into a frothy mass. The 
stomach and intestines fill with gas, followed by much belching. Occasion- 
ally there are seminal ejaculations, and involuntary expulsion of urine 
and feces may occur. A stream of urine may be thrown a distance of 5 
to 10 feet at the onset of a severe attack. 
The duration of the clonic phase is considerably longer than the tonic 
phase-from one-half to three minutes. The classical attack is usually 
bilateral, one-half of the body being involved directly after the other. 
In atypical and rudimentary attacks fewer muscles are involved. In 
typical cases we find those violent cloni which cause severe injuries, 
-fractures and luxations. Cloni are for the most part continuous; 
exceptionally we see relaxation, followed once more by cloni. The two 
kinds of spasm overlap in certain seizures. 
Psychic Epilepsy.-The most striking symptom of this is an alteration 
of consciousness, which is termed dreamlike. The very mildest phases, 
which resemble somewhat petit mat, are characterized by a brief con- 
fusional state, accompanied by a few automatic activities. Cases differ 
extremely and it is difficult to describe psychic epilepsy in brief, straight- 
forward text. Thus a subject may suddenly run out of doors, return at 
once, and perform any number of purposeless, automatic acts, some of 
which may injure himself and others; while acts simple in themselves 
may lead to arson, suicide, and homicide. A strong sexual bias is often 
present. The emotional sphere does not enter into these acts, which 
resemble those of the intellectual aura, or intellectual petit vial. 
Psychic attacks as just described are really relatively rare in occurrence. 
Their apparent frequency in many medical reports is due largely to 
insufficient analysis of such seizure symptoms. The more closely psychic 
epilepsy is studied the more one finds that the attacks are really preceded 
by a very slight local spasm of muscles in some one part of the body 
perhaps, which because of its very transitory and casual nature eludes 
observation, thus forcing many instances of psychic epilepsy into the 
medical type of petit mal followed by a prolonged train of obvious mental 
symptoms which characterize the many phases of automatism. So excel- 
lent an authority as Hughlings Jackson believed that no form of epileptic 
attacks occurred without some muscular spasm, however slight. 
Epileptic Psychic Alteration.-Statements as to the number of epileptics 
who retain their psychical integrity do not exist. Those accessible to 
us are institutional cases in which the often associated idiocy shows the 
frequency of an intellectual defect. With advancing experience we now 
believe that in many chronic epileptics the mind is intact. By further 
knowledge of their personalities, various features are seen which point 
to epilepsy-a certain pedantry and "matter of factness" in all things, 
extravagance in speech and writing, a distinct egotism or hypochondria, 
over-appreciation of self, exaggerated sensitiveness-but nothing which 
points to mental failure. In patients who have attacks years apart we EPILEPSY 
603 
see no affect phenomena of a chronic character which suggest the epileptic 
alteration, nor do we see any characteristic defect of intelligence. At 
times there is a transitory psychic alteration, in relation to affect life 
(irritability, alterations in temper, outbursts of anger). 
The patients who are most in danger of mental degeneration are (1) 
those who become epileptic at or before puberty; (2) those who have 
serial attacks, and (3) those who have had numerous abortive attacks. 
Of very great interest and importance is the intellectual foundation- 
the men of great intellect or high spirits who throw off the fits and their 
results, both readily and without injury. A few world celebrities belong 
here. Aside from these there are many parallel cases. 
In elderly people petty causes seem to be sufficient to cause senile 
epilepsy, which, with ordinary senile involution already present, rapidly 
precipitates the latter. There is no general rule applicable to these cases, 
for I have seen a case of an old man who at the age of sixty-two began 
to have rapid epileptic attacks. At the age of sixty-eight, after six 
years of epilepsy he showed no intellectual defects. 
The psychic degeneration of epileptics possesses certain characteristic 
traits, which certainly depend upon the underlying disease and affect 
disturbances due to the attacks. The intellectual weariness and exhaus- 
tion symptoms, which at first follow individual attacks, become protracted 
in the course of time. Finally a period for recuperation becomes no 
longer possible. The patients are seen to gradually lose the capacity to 
receive new impressions. The memory pictures of the newly happened 
become more scanty and full of gaps. Forgetfulness extends more and 
more toward the past. We see complete oblivion of certain experiences 
while for others memory shows no depreciation. The greater the diffi- 
culty in receiving impressions the less readily are they reproduced; so 
that the trend of thought becomes monotonous, while speech expression 
is awkward and dull. Next comes epileptic dementia, characterized 
chiefly by attacks of anger. The gracious patient bursts into anger and 
attacks those about him. Sexual extravagances are common. 
Post Paroxysmal Psychosis (Temporary).-The transitory, psychosis 
which follows a motor paroxysm is comparable with psychic equivalents, 
and pre-paroxysmal phenomena (remote prodromes). There appears 
to be in certain cases a single continuous disturbance of consciousness. 
But a third of epileptic major seizures are known to have had auras, 
while the majority have a post-convulsive stuporous stage. One may 
designate four fairly distinct post-paroxysmal conditions in epilepsy: 
1. The more common stuporous state characterized by complete 
inactivity of mental and physical performances, lasting an hour or so. 
2. Attacks followed by a more or less orderly and systematic autom- 
atism usually lasting a few minutes to several hours. 
3. A fairly systematized series of hallucinations, dream-like in forma- 
tion. 
4. Severe and prolonged states of excitement with pronounced in- 
coherence and violent impulsive activities which may last for several 
days. These elemental types are frequently combined in many ways. 
A protracted, stuporous condition, which may last for days and weeks, 604 
DISEASES OF THE NERVOUS SYSTEM 
is more common as post-paroxysmal state than as psychic equivalent. 
The relationship between post-epileptic psychoses and attacks is in- 
telligible; the more frequent the latter (until they become serial) the more 
severe the post-paroxysmal psychoses. The shorter the intermission 
between individual attacks, the more frequently will the condition be 
distinguished by a protracted, confused or impulsive, excitable condition. 
In a second rare group of cases the transitory psychical disturbance 
follows individual fully or partially developed convulsions, but at a 
protracted interval. This sort of post-epileptic psychosis is often of 
toxic origin; for example, after alcoholic indulgence a number of hours 
may elapse before the attack sets in. The same is true of an affect and a 
subsequent attack. 
The worst species of post-paroxystic psychoses are those which follow 
abortive attacks. It is not easy to state whether the latter are psychic 
epileptic equivalents with pronounced aura, or simple motor auras, 
or brief isolated abortive attacks of true epilepsy, or some other known 
manifestation; whatever the type, the paroxysm is an individualized, 
brief, self-limited process, be it an attack of vertigo, a brief lapse of 
consciousness, or a period of violence. Some transitory psychoses do not 
last over two or three minutes, while the more protracted may last for 
hours and days, even weeks. These cases of mental cloudiness of relatively 
brief duration require to be very carefully analyzed in order to fix their 
status. Let us take cases of "absence." The entire duration of a special 
case is but one minute with retrograde amnesia. 
In simple feeblemindedness, there is spasmophilia of high degree. 
But mental defectives after a certain interval of freedom from epilepsy 
may develop typical attacks of the latter from trifling causes-slight 
use of alcohol, of certain drugs, fever, or psychic trauma. The spasmo- 
philia or tendency to convulsions in the feebleminded has been ascribed 
to infantilism. A spasmophic imbecile may later develop epilepsy from 
trivial causes. Difficult as it is to understand the relationship between 
epilepsy and idiocy in these cases, it is far more difficult to explain cases 
in which the child was born normal and developed epilepsy in late child- 
hood or puberty. At this very period the intellectual growth is either 
halted or actually retrogrades. 
Petit Mal.-A generation ago, medical text-books spoke of petit mal 
as if it were little more than a mere "nodding" or "losing oneself," which 
differed from the ordinary form only through the fact that speech, work 
or other activity was resumed just at the point where the seizure caused 
the inhibition. Amnesia was present, the patient having no recollection 
of what had happened. Another characteristic point is that the features 
instead of being flaccid, are either made tense like a mask or undergo 
grimaces, chiefly of the ocular and buccal muscles. The condition as a 
whole became recognized as a form of epileptic seizure, not less deadly 
in the long run than the severe convulsive attacks; and with this dis- 
covery it become known as petit mal. 
From another viewpoint petit mal maintains its independent character 
because the simple movements of the facial muscles may be greatly 
expanded, affecting muscular groups here and there. It is as if when the EPILEPSY 
605 
inhibition is not so powerful as to cause loss of consciousness, certain 
motor equivalents set in. The presence or absence of an aura in petit 
mal is of much significance. In theory petit mal is one form of an abortive 
grand mal attack. They comprise attacks which consist only of an aura, 
which are extremely rare and are not followed by a motor discharge, 
although there are auras which consist solely of certain motor mani- 
festations. Next comes a form of petit mal, in which there is a brief loss 
of consciousness and at times motor phenomena; finally, with loss of 
consciousness and face to the ground, we still see irregular and imperfect 
manifestations of a major convulsion. It is not strange that situated 
in the midst of abortive attacks petit mal shades into other manifestations. 
The space given by Gowers and Binswanger to all these phenomena and 
their mutual relations shows the complexity of this subject. 
The subjects of petit mal as a rule remember certain phenomena, 
chiefly of motor character, which represent auras. In Gower's analysis 
of 155 cases 45 showed nothing whatever of prodromes. There were not 
even the premonitory feelings of swoon or drowsiness. In 25 cases 
there was preliminary vertigo; in 17 shock or tremor of the limbs, and 
again 17 of eye prodromes-either excessive sensibility or loss of sight. 
Other elements include mental states (sudden phobias); a unilateral 
peripheric sensation or twitchings; epigastric sensibility; sudden tremor 
-and so on down to single causes with but one case to illustrate 
them. 
All these remembered experiences tend to show the great part played 
by auras in petit mal. The most common feature is vertigo or a feeling 
of lost equilibrium which does not quite amount to vertigo. About 
20 per cent, belong here. But this number can be increased if we include 
cases which begin as half flexion of certain muscles. As petit mal was 
first known to the French as epileptic vertigo we can appreciate the value 
of this connection. Gowers has on several occasions heard the epileptic 
cry with petit mal, although in grand mal it is usually associated with 
the fall. The simple loss of consciousness of typical petit mal, with 
resulting amnesia, represents but a certain number of these cases; others 
have retention of consciousness. This is likely to be noted in very brief 
attacks and is usually the result of some physical experience. 
It is thus evident that any brief seizure, lasting for a few seconds up 
to half a minute or more is an example of petit mal; an abortive or other- 
wise mitigated equivalent of the major attacks. Petit mal stands in 
near relationship with auras, especially a motor aura. The forms are 
very numerous - a brief vision or hallucination, leading to defensive 
motions; twitching of the eye muscles, or of those of one side of the face, 
consciousness being retained; isolated vertigo may belong here. In a 
single chronic epileptic, one may see every form of seizure-monospasms 
with or without loss of consciousness, rudimentary attacks, typical com- 
plete major attacks and status epilepticus. A point of importance, 
according to Gowers, is the great tendency of association of petit mal 
with vasomotor aura. 
Incomplete, Rudimentary Attack.-This rare form is known as the 
apoplectic, because of the clinical parallelism. It is thought to be a 606 
DISEASES OF THE NERVOUS SYSTEM 
severe form of tonic crisis, which hampers breathing and the cerebral 
circulation. The post-paroxysmal state suggests an apoplectic stroke. 
Paradoxical types occur in isolated cases in which motor auras are 
closely paralleled. Their culmination is seen in epilepsia procursiva. Its 
locomotor component may be due to the activity of the infra-cortical 
ganglia: A man, aged twenty-six years, had had seizures of a peculiar 
character for fifteen years. The first evidence of the attack was the cry but 
instead of falling he ran forward at great speed, unconsciously paying no 
attention to the small objects in his way. In a few seconds he stood still, 
becoming conscious and at the same time very red-faced. He had amnesia 
for facts but recalled some dream-like image of his experience. A second 
seizure followed, in which the patient fell but rolled himself over the 
floor, crying out continually for about twelve seconds. Conditions 
resembling this have also been described (epilepsia rotatoria).1 
Phenomena Which Accompany or Succeed Immediately Upon Major 
Crises.-Many authors devote much space to the vasomotor disturbances 
during a seizure; they are indeed next to the convulsions the most 
essential component of the latter. The vasomotor attack is short and 
quickly replaced by exhaustion, the face becoming cyanotic. This 
phenomenon, however, may also be explained by compression of the 
veins of the neck by the rigid neck muscles. The picture shown by the 
face in severe cases is that of strangulation. With vasomotor spasm 
there is naturally a sudden and marked rise of blood pressure. As a result, 
minute hemorrhages often occur, and since these might persist for days 
often supply evidence of an attack. These occur in the skin over the 
mastoid process as fine punctiform hemorrhages. Bleeding may occur 
from nearly all of the mucosae, but profuse hemorrhages are rare. The 
very wide divergence of the symptoms of this type overthrows any theory 
that epilepsy is per se a vasomotor affection, while still showing that this 
symptom plays an important part. 
In regard to the pupillary phenomena during and after a seizure there 
is no complete uniformity, save that dilatation begins with the tonic 
stage. There is no response to light; after the seizure the pupils usually 
become normal. 
In the fully developed atypical attacks the remote prodromes and aura 
show no peculiarities in comparison with grand mal in general. The 
differences begin in the loss of consciousness and the order of the motor 
movements. As a rule the tonic spasms are complete before the slowly 
progressive inhibitory discharge of the cortex has led to a complete 
elimination of the psychic functions. This slow onset, aided perhaps 
by the warning of an aura, gives the patient time to seat himself or lie 
down on the floor. He often has good recollection of the tonic stage 
but then loses consciousness. In this form of attack the tonic stage 
does not develop instantaneously throughout the entire musculature 
but may begin on one or the other side of the body or in groups of muscles 
of both sides, and may simulate for the moment coordinate movements. 
1 See "Status Epilepticus," Clark and Prout, Am. Jour. Insanity, lx and Ixi; also 
"A Clinical Contribution to the Irregular and Unusual Forms of Status Epilepticus," 
Clark, Am. Jour. Insan., 1913, Ixx, No. 2. EPILEPSY 
607 
The variety of these irregular tonic spasms is infinite. No absolute 
borderline can be erected in these cases between tonic and clonic spasm, 
for at the close there is still some tonus left in the muscles. The post- 
paroxysmal stupor in such cases is likely to be attended by psychic 
excitement, but is usually short and followed by deep sleep. From 
certain facts one may believe that such attacks occur only on a hereditary- 
degenerative base and that the epilepsy represents but part of it. 
The so-called epileptoid conditions of Griesinger may be noted here. 
Most of these may be accounted for under other heads but a few remain 
to which the title may be given. We designate by the term epileptoid 
only those recurring paroxysms of disease-phenomena, non-convulsive, 
with or without consciousness, which have been seen in connection with 
undoubted epileptic insults, and also appear independently as a sole, 
unique sign of a discharge. There belong under this head secretory, 
trophoneurotic, and vasomotor disturbance. Thus a patient may or may 
not have vertigo at first; but there is a seizure type, in which with no 
apparent cause a local hyperidrosis appears. Attacks of oedematous 
swelling may occur as an epileptoid condition, just as in hysteria. This 
swelling is painful and tender. A third form is sudden darkening of the 
integument in certain localities of the body. Attacks of urticaria have 
also acted as epileptoids. 
Exhaustive or Stuporous Stage.-At the close of a grand mal seizure, 
the patient sighs deeply and assumes an attitude of extreme prostration 
and deep slumber. This lasts but a few minutes, when the patient wakes, 
sometimes suddenly, and makes immediate efforts at orientation. He 
may jump up and look about, but as a rule his exertions are passive, 
as if he were trying to collect his thoughts. In a short time, however, 
the state of exhaustion again overcomes him and he settles down to a 
condition of sleep or stupor, or at times a sort of delirium, in which 
dream hallucinations may or may not cause motor activities of various 
kinds. Various disturbances of speech are especially known. This 
delirium is really a part of the epileptic psychopathology. The post- 
convulsive sleep may last from a quarter of an hour to several hours, 
and almost always there is complete amnesia. The patient is left in a 
high degree of fatigue, incapable of mental activity, and is sad, irritable 
and hypochondriacal. He has pains in the muscles and joints, nausea, 
vomiting, and anorexia. Many subjects feel themselves better off for 
having had the fit. 
The local forms of exhaustion are numerous and not necessarily of 
much importance. As in other convulsive affections, the muscles may be 
in a state of paresis. This is also seen in Jacksonian epilepsy. In a few 
individuals repeated exhaustion of the same muscles leads to an increasing 
disability. The subject of cortical deterioration, muscular exhaustion 
after prolonged seizures, and deterioration of the function of the muscles 
is a complex one. Such lesions as concentric narrowing of the visual 
field, disturbances of color vision, deafness, etc., exist for a short time 
as exhaustive manifestations. But little is known of anosmia and 
ageusia. Much also has been written concerning post-paroxysmal 
analgesia and anesthesia, together with other disturbances of sensation. 
These have no diagnostic value and occur without any regularity. 608 
DISEASES OF THE NERVOUS SYSTEM 
Rationale of Major Seizures.-We know the phenomena of seizures from 
the clinical standpoint, and we know something of the nature of the 
epileptic brain; but back of all such knowledge there must be a theory 
or a rationale to account for the why and wherefore of the attack. We 
have partial ideas of the mechanism of the interruption of a crisis, so that 
numerous abortive clinical forms occur and they aid us in establishing 
a theory of the complete major crisis; but it is the latter in its entire 
cycle which we wish to consider. We also are interested in knowing 
whether true convulsions can be produced artificially. 
We know that the entire brain must be altered in some manner for 
its owner to become epileptic. We have no word to express this state, 
because alteration is a vague term, and applies equally to the mental 
changes which are due directly to the great number of seizures. There 
should be some term corresponding to spasmophilic to describe the 
sensitized brain of the epileptic. Many authors contend more or less 
successfully that the original irritability of the brain differs in no wise 
from that of extreme cases of neurasthenia (including psychasthenia). 
Some phenomena predominate in one state, others in the other, but there 
is no line of demarcation. Other authors, including myself, state that 
the epileptic predisposition is something altogether peculiar.1 We are 
inclined to lean to a dualistic viewpoint, for if an epileptic brain is only 
a psychasthenic brain, it will be impossible to distinguish radically 
between true and affect epilepsy. The change in the entire brain manifests 
itself in cortical disturbances merely because the cortex is the most 
highly developed portion. All manifestations of general seizures are 
linked with the cortex. 
We cannot take it for granted that the general irritability of the brain 
is of much help to us in explaining a seizure. Mere irritability must 
be a factor in all psychoneuroses. We have to account for the sudden 
character of the seizure and especially for its periodical return. There 
is a suggestion of petty insults which very slightly irritate the brain, 
but are not at first manifested. As these insults slowly increase, a period 
comes in which the brain is comparable to a strongly charged cell. A 
state of saturation is impossible. Finally a so-called explosion or dis- 
charge occurs. In this respect the affection is quite parallel with migraine 
or any other periodically occurring neurosis. This explosion, at least 
in so far as it represents migraine, was termed many years ago a " brain 
storm." The typical epileptic brain, now clear, begins the cycle anew. 
We cannot, of course, claim that the discharge is complete when frequent 
or serial attacks make up the disease. 
This overloading or discharging causes two types of manifestation, 
best styled respectively as inhibitory and exciting. The first inhibit 
the activities of numerous functions, while the second cause an excess of 
function. Even the stage of post-paroxysmal exhaustion is not unphysio- 
logical; general convulsions cause general exhaustion, local convulsions 
cause local exhaustion. The first epileptic attack differs from the 
subsequent ones in being an affect manifestation, due usually to fright. 
1 Clark, Am. Jour. Med. Sc., 1914, cxlviii, 729. EPILEPSY 
609 
The typical convulsions proceed not only from the cortex but from the 
infracortical centres, including the large basic ganglia, quadrigemina, 
and medulla. Certain mild attacks (incomplete) probably concern 
the cortex alone. It is assumed that the discharge, whether strong or 
weak, always begins in the cortex. Those convulsions of cortical origin 
which follow organic disease, injuries, etc., are not at first epileptic but 
doubtless readily become so. 
Experimental Seizures.-The subject has been summed up as follows: 
When a circumscribed area of the motor-excitable cortex is subjected 
to a mild, electric current, clonic twitchings of the corresponding muscles 
appear. Nearby muscles also become involved through superficial 
extension of the irritation in the cortex. After the electrode is with- 
drawn, the spread of the irritation may reach the cortex of the opposite 
lobe and cause convulsions therein. In certain animals, and with very 
strong currents, we may also see tonic spasms and automatic activity 
(locomotion), evidently from projection to the infracortical motor centres. 
These experiments show analogy with human epilepsy, and help us to 
understand some of the rationale, both of general and local attacks. 
In man himself there has been much experiment upon epileptics, with 
particular regard to so-called reflex epilepsy. Experiment may be 
justified in cranial surgery, in order to aid in the localization of certain 
motor areas, and there have been many operations performed in the 
hope of curing organic epilepsy, some of which have experimental signi- 
ficance. But no results obtained can contradict what we already know 
about epilepsy. An epileptic is not immune to other forms of convulsions 
such as do not depend upon his peculiar brain texture. He has been 
known to simulate his own attacks, may have hysterical and psychas- 
thenic crises-in a word, he may have any one of the old so-called epilepti- 
form convulsions. The results of experiment, however they may show 
the presence of a mechanism also visible in epilepsy, must not be made 
co-equal with epilepsy as a disease. 
Status epilepticus is not recognized as a special type of seizure, for it 
differs only in degree from serial attacks. The latter, if anything, marks 
any innate peculiarity of seizure. This serial seizure occurs as a rule 
after prolonged freedom from attacks, whether spontaneous or due to 
bromides. Another peculiarity of serial attacks is that we find them 
most frequently after abortive and rudimentary attacks, or the type 
of the individual seizures may be a picture of all the forms, including 
grand mal. The immediate consequence of the serial seizures is that the 
subject has little time to recover from the usual exhaustion and stupor 
following a single attack-hence when the series ends he finds himself 
often in a state of prostration and stupor which may last for months. 
Status epilepticus instead of resulting necessarily from intensive 
serial attacks, may set in spontaneously without apparent cause. For 
a short time individual attacks may be seen but it is not long before we 
have to deal with one continuous convulsion. In twenty-four hours 
the subject goes through the equivalent of from 10 to 30 or more separate 
attacks. The amount of muscular activity, as in the case of tetanus, 
may send the temperature up to 42° C. or more. A constantly ascending 610 
DISEASES QF THE NERVOUS SYSTEM 
temperature ends fatally and the temperature continues to increase 
after death. It can be seen that in status epilepticus seizures of all kinds 
may succeed one another. The blood pressure is high, the face cyanotic 
and the subject perspires freely. The condition may simulate rabies 
in several ways, necessarily in difficult deglutition. The convulsive 
period lasts from one to three days. 
This prolonged convulsive stage is followed by a prolonged period of 
exhaustion or stupor. Various acts and movements made during this 
period show that the condition is akin to busy, muttering delirium. The 
face is very expressive and excited, the gaze wanders, patients toss on 
the bed and even leave the latter. All the while they mumble parts of 
words. The patient soon presents a typhoid picture and dies of oedema 
of the lungs, heart-failure or decubitus. 
In patients who are destined to recover we see profound exhaustion, 
which may last for days. We have here to watch the temperature and 
should this begin to decline rapidly the patient will recover, if he does 
not develop the excited hallucinatory period. Recovery may occur 
under the most unfavorable conditions, a prolonged convulsive state 
(twelve days) followed by days of excitable delirium, etc., and fever. 
Older writers spoke of fragmentary or serial attacks which never led 
to status epilepticus. Thus 2500 attacks have been experienced in a 
month by a boy of fifteen (Delasiauve). It is not impossible that these 
were really psychogenic fits. 
Nocturnal Attacks.-All are familiar with day attacks, but nocturnal 
seizures may occur and pass undetected for years. It seems at present 
that the nocturnal attack is much more common than the diurnal, and 
that they may precede the latter, perhaps for years. Diurnal attacks, 
in the belief of some, do not develop until the disease is deep rooted. 
Thus nocturnal attacks at puberty may not be discovered until marriage 
or until a period of military service is required. 
Miscellaneous Seizures.-An epileptic may exhibit other than epileptic 
paroxysms, which are best termed affect epilepsy. Bratz and numerous 
others have described this form of epilepsy under various terms. The 
subjects comprise degenerates, young criminals, etc. Affect influences 
cause serial attacks, with no tendency to status epilepticus or epileptic 
deterioration. Such patients have until very recently been interned in 
epileptic asylums as genuine epileptics. They profit nothing from the 
hygienic treatment of epilepsy and are never free from pallor. A labile 
psyche appears to furnish a foundation. Vasomotor lability and spas- 
mophilia for the most part co-exist. Not only sharp affects, but chronic 
depression, moroseness, etc., furnish the substratum for these attacks. 
Hysteroid Seizures.-The new field of affect epilepsy has cut deeply 
into the old material of hysteria and hystero-epilepsy. Some authorities 
insist that hysterical and affect crises are one and the same. As in epilepsy, 
"hystero-epilepsy" occurs on a degenerate soil. The manifestations of 
the latter are very numerous, and an epileptoid convulsion is but one of 
them. Oppenheim's psychasthenic convulsions, which are usually ranked 
under affect epilepsy, are placed as a "third affection" or "bridge" 
between hysteria and epilepsy, and certainly differ in some ways from EPILEPSY 
611 
Bratz's syndrome. The latter exhibits a large number of serial fits 
at long intervals, while Oppenheim's disease shows but one or two attacks 
in a lifetime. True hystero-epilepsy can only be a distinct combination 
of both diseases in one subject, each pursuing its own course. 
Psychical Anomalies of Epileptics.-The psychical phenomena are 
very manifold. There are two principal forms, transitory and permanent 
or chronic. These stand in no absolute relationship to each other. First 
there is the association of idiocy and epilepsy. Statistics show that but 
one-fourth to one-fifth of all epileptics have a normal psyche. In severe 
child epilepsy, the convulsions produce an arrest of development, depend- 
ing somewhat on the age, and but few escape idiocy. 
Epileptics are unable to absorb and retain impressions because of 
dulled perception. The prolonged bad temper has been ascribed to the 
fact that the results of scanty perception are offset by a much longer 
period of sinking in of impressions than is the case with the normals. 
There may be a direct connection between dulled perception and dis- 
ordered comprehension. Perceptions are often even at the outset falsified 
by deception of the special senses. Hallucinations and psychic auras 
before convulsions may be cited first, but in the interval and even in the 
post-paroxysmal stupor we see the same phenomena and, in the interval 
state, must often regard them as equivalents. Hallucinations lead up to 
the dream state and mental confusion or so-called twilight state. 
The paranoid states which are seen in combination with epileptic 
attacks are chiefly manifested by hallucinations. These states are 
regarded by some as equivalents. Ziehen describes a chronic epileptic 
hallucinatory paranoia. 
Consciousness is the last and highest step of the process of perception. 
Hence a change in consciousness may mark the very essence of the 
disease. In severe epileptic seizures consciousness is lost entirely. The 
epileptic sequence is as follows: dulled perception, and resulting de- 
pression of comprehension; spurious perception hallucinations; interrup- 
tion of perception; loss of consciousness. In twilight states, amnesia is 
not necessarily present; and in wanderlust consciousness is now known 
to be partly retained in certain cases-imperfect amnesia. 
In measuring the degree of intelligence in average men we find in the 
epileptic a constant diminution of the various faculties. The typical 
epileptic disturbance of thought is seen in the hazy mental state. Asso- 
ciation of ideas is difficult. Inhibition and incoherence are the chief 
tokens. There is distinct dissociation in the course of thought. The 
subject performs unexpected acts. Psychical continuity is interrupted. 
Disturbance of affect life is common in the course of epilepsy. Excita- 
bility in dealings with others becomes marked. The least possible offense, 
real or imagined, causes an outburst of rage. This is especially the 
case if a little alcohol has been taken. The same outbreaks may occur 
in stupor, after an attack; others may be regarded as equivalents. 
The disposition of the epileptic is manifold. He may be depressed, 
gloomy, preoccupied. This combination of hypochondria and melan- 
cholia may last for hours and days. Since they recur periodically they 
are called equivalents by Kraepelin. They come on without cause, 612 
DISEASES OF THE NERVOUS SYSTEM 
suddenly, as a general melancholia with its self-reproach and suicidal 
tendency. Numerous somatic accompaniments appear to be of vaso- 
motor origin. Intensive variations in disposition also occur in connection 
with the convulsive seizure. The hypochondro-melancholic behavior 
may occur before the paroxysm and also after recovery from the latter. 
The opposite picture, that of mania, is rare. We sometimes see in the 
post-paroxysmal period a combination of euphoria, increased self- 
conceit, etc. The psychic aura is at times accompanied by a most 
intensive feeling of happiness or good fortune. 
An epileptic is a readily excitable, sensitive man, whom the slightest 
occasion may cause to explode into violent acts. As a rule he presents 
a gloomy, closed-in nature. He is suspicious, jealous and vindictive 
toward others, and is but little interested in the outer world, is dull, 
indifferent, idle, and performs his duties with difficulty. His makeup 
in the presence of others is that of an obstinate pedant. His behavior 
is without form. The smallest trifles he regards as serious, and his 
opinion cannot be changed. He is notable for piety; is a typical hypocrite, 
egoist, self-satisfied, and even has marked family pride. 
We finally find disturbances in the field of the will and behavior. A 
depressed will may be regarded as a psychomotor inhibition, which often 
becomes a habit. The reaction time is greatly retarded. Inhibition plays 
a large role in the epileptic mood. Extreme disturbances of behavior 
include arson, theft, etc., occurring in the twilight-state. 
Speech difficulties occur in several types; there is a struggle to express 
what he would say, there is a sort of "plateau speech,-a type of non- 
melodious voice (Clark-Scripture)-a habit of coining words, and of 
suddenly changing the subject. The difficulty of expressing himself 
is perhaps the reason why the epileptic loves bombastic speech, which 
is the easiest way of self-expression. 
It is very difficult to comprehend or define the epileptic psyche in 
toto. Powerful contrasts are present. We may see the highest intellect 
and the deepest dementia; the greatest reserve and the greatest desire 
to be attentive to others, especially women; depression and excitement; 
inaccessibility and hypocritical devotion in contrast with intense egotism 
(especially in religious matters); fearsome shyness and blind rage (as 
affects); apathy and extreme irritability. And all this is "epilepsy." 
The epileptic psyche shows a distinct and complete injury to the 
intellect. Perception, retention, formation and association, reflection, 
etc., are all retarded or rendered difficult-and to such a degree that 
falsification occurs, so that hallucinations and fantastic ideas result. 
With a further degree we obtain the "twilight state" (dissociation of 
Ziehen). In many epileptics, however, we find normal intellect, while 
complete terminal dementia is rare. 
The relation of psychical epilepsy to the convulsive crises and to other 
psychoses requires much research. The chief division of psychoses 
(already given) is into acute and chronic. Are these both phases of one 
condition? The acute form is characterized by periodicity. This seems 
to set up a barrier between acute and chronic forms. 
"Is there really a psychical epilepsy?" "May not the psychical mani- EPILEPSY 
613 
festations occur in non-epileptics?" This question has not yet been 
decided. 
Diagnosis.-This involves study of seizures, and determination of 
their visible, transitory, and permanent results. We must also study 
the extraparoxysmal features, the psychic behavior and the somatic 
concomitants in order to facilitate the recognition of the disease epilepsy. 
Convulsive seizures, even when rudimentary, are readily recognized, 
although epileptiform convulsions may be caused by tumors of the brain, 
pachymeningitis, acute poisonings, progressive paresis, etc. These are 
no longer regarded as pictures of epilepsy. Here we come into contact 
with rudimentary (unilateral), or generalized convulsions proceeding 
from fresh or old lesions in a circumscribed portion of the cortex. It 
was Fere in his work on partial epilepsy, who first insisted that such 
convulsions represented true epilepsy as a result of organic brain disease. 
The greatest importance in this phase of diagnosis is bound up in operative 
treatment. If convulsions are really epileptiform and nothing more, 
the indication for operation is much more strict. 
Focal phenomena in epilepsy form a link between organic and genuine 
epilepsy. The focal symptoms of infantile epilepsy and the same in 
adults present all shades of transition. In the first we see a paretic 
component, while in the last this is absent. One explanation is that in 
the grown subject a healed focus starts up convulsions but no paresis. 
Focal paresis is a most striking phenomenon, and may perhaps be expressed 
in the adult by minimal paresis or perhaps by a slight motor component 
(some cases of left handedness have even been attributed to such a causa- 
tion). In post-paroxysmal exhaustion, etc., the evidence of such foci 
may be seen. Unilateral interparoxysmal increase of tendon reflexes 
and abolition or weakening of the cutaneous reflexes are to be included 
under exhaustion phenomena. Such are extremely important, if the 
unilateralism be associated with focal affections (not necessarily in the 
gross organic sense). Unilateral spasms cannot always be referred to a 
focus. In Muller's "status hemiepilepticus idiopathicus" with con- 
tractions in the Jacksonian sense, no organic basis whatever could be 
found. Hence there is a chance for differential diagnosis here. A 
number of data may be obtained from a comparison between certain 
focal phenomena of genuine epilepsy and Jacksonian epilepsy which 
would tend toward confusion. Thus several of the auras are unilateral, 
seen chiefly in rudimentary and abortive attacks. These auras are 
motor, sensible, and sensorial. Now one of these unilateral motor auras 
bears a close resemblance to a Jacksonian spasm, chiefly one which 
represents the circumscribed form, limited to one segment of a limb or 
one group of muscles. In this case the spasm is clonic. A distinction 
can be made, i. e., we can only exclude the presence of an organic lesion, 
after a full examination of the interval period-motor weakness, depres- 
sion of tactile sensibility, disturbance of coordination, etc.-the absence 
of which excludes the organic element. The motor aura of genuine 
epilepsy lasts but a few moments and is distinct from loss of consciousness. 
In organic cases, the motor aura is slower in development and appears 
only when the clonic spasms extend to other muscles. Post-paroxysmal 614 
DISEASES OF THE NERVOUS SYSTEM 
paresis in the muscles first involved in the spasms speaks for organic 
epilepsy. The motor aura is practically uniform in each attack. Focal 
phenomena during a paroxysm do not as a rule indicate the source of the 
attack. The greatest difficulty in regard to the significance of focal 
phenomena is offered in cases of genuine epilepsy in which focal lesions 
of traumatic or syphilitic origin appear in the course of the disease. 
As a rule there is no difficulty in the diagnosis of petit mal at the hands 
of an experienced practitioner. Sometimes confused with it are cases 
of vertigo arising from several causes (ear, stomach), migraine and syn- 
cope. Seen by themselves, these phenomena might impose themselves 
as petit mal but a little investigation will show the presence of something 
further of epileptic nature. 
The fully developed type of hysteria (Charcot) is easily distinguished 
from epilepsy, and while the linking together of the manifestations of the 
seizures correspond, there is a marked difference in the motor component. 
The complete hysterical attack is very rare and its passionate attitudes, 
etc., represent a stage of delirium. Fere agrees with the Germans in 
that the clownish and passionate manifestations are extremely rare among 
the rank and file of hystericals; when present they may make the diagnosis 
of hysteria. Very different is it when these manifestations are absent 
or in the rudimentary state. Here it is extremely difficult to distinguish 
between hysteria and epilepsy. Fere's method of stopping an attack 
by pressure on the ovaries, as indicative of hysteria, is not infallible, 
as he himself admits. In doubtful cases we should study the inter- 
paroxysmal symptoms, which may at times point to hysteria, but often 
to the contrary-so that some special criterion is still in demand. This 
can no longer be found in the pupillary reaction, which is shown to be 
similar in individual cases for each disease. Neither is the temperature 
test of any differential value. 
The "twilight state" in each disease is difficult to distinguish from 
its fellow, for parallelism is the evidence throughout. The chief point 
of differences lies in the fact that hysterical delirium is largely affective- 
"a delirium of recollection." In this, past experiences are sometimes 
re-enacted. 
To sum up, the most distinguishing trait is as follows: Epilepsy in 
serial attacks (these also occur in hysteria) leads to pronounced psychical 
alterations in the sense of a defect of intelligence; and even in milder 
attacks injures the sound freshness and elasticity of the mind. Hysteria 
in every form and degree never causes any recognized decrease in the 
mental powers. In neurasthenia we may see affectively determined 
psychical inhibitory phenomena in considerable variety (irritation and 
deficiency symptoms) which closely resemble abortive attacks of epilepsy. 
That convulsions may appear upon the foundation of neurasthenia, 
which are neither epileptic nor hysterical, has been shown by Oppenheim. 
The patients were heavily tainted, and after birth promptly showed 
evidence of psychopathic constitution, which crystallized within a short 
time into a psychasthenic character. The subjects exhibited tics, states 
of fear, phobias, imperative concepts and vasomotor disturbances. 
Upon this substratum arose, almost always as a result of affects (dis- EPILEPSY 
615 
turbances of temper, overwork, alcohol, etc.), a condition difficult to 
distinguish from an epileptic seizure; it was accompanied by unconscious- 
ness, escape of urine and feces, convulsions with tongue-biting and 
pupillary rigidity. Between the paroxysms occurred congestions, vertigo 
and phobias, with consciousness retained. 
This tendency to convulsions vanishes under favorable hygienic 
conditions through general cures directed to combating neurasthenia 
and to avoidance of ordinary affective experiences. As in affect epilepsy 
and also affect spasms the causes are antagonistic to those of true epilepsy. 
This type (Oppenheim) may occur but once or twice in a lifetime, and 
intelligence and memory do not suffer. These cases are regarded by 
Oppenheim and Binswanger as representing a "tertium quid," a partition 
between psychasthenia and epilepsy which must always suggest the 
possibility of organic lesions. 
There are many points of agreement between epilepsy and migraine. 
We see in each, periodicity, prodromes, aura phenomena (visual and 
auditory paresthesia), motor and sensory phenomena during a crisis, 
transitory psychotic states, etc. Migraine has been known to pass into 
epilepsy, the headache component being replaced by "scintillating 
scotoma." The transitions between the two are sometimes explained 
by the substitution of migraine for epilepsy; it appears also that migraine 
may mitigate epilepsy. Mobius has isolated a migraine which is merely 
a symptom of epilepsy, but as a rule migraine is an equivalent, not a 
symptom of the latter. It could well be shown that a migrainous attack 
might run its course in such atypical forms that to distinguish between 
it and an atypical form of epilepsy would be almost impossible. We 
may see migraine without headache or vomiting, expressing itself solely 
as a visual or paresthetic aura (with or without speech disturbances). 
Vasomotor epilepsy bears a close resemblance to migraine, since in both 
we may see darkening of the visual field, foggy state of consciousness, 
paresis; occurring in the same subject no distinction could be made. 
There are two other subjects of especial interest under diagnosis: 
(1) To identify a man who has once been an epileptic and (2) to recognize 
a malingerer. In the first class the signs of a recent attack are readily 
recognized-such as bitten tongue, ecchymoses behind the ear or on 
the brow and ocular region. In case of remote attacks there should be 
scars on some part of the buccal mucosa, as well as on the tongue. The 
question of diagnosis in the interparoxysmal state is very difficult, 
coinciding largely with neurasthenia, which is practically the same state. 
Not until a seizure has occurred can a positive diagnosis be made. 
In regard to simulation, it is easy to master the salient points and so 
impose on the non-expert, but the pallor of the face, dilatation of the 
pupil and its insensitiveness to light, the change in the pulse-rate and the 
cyanosis cannot be simulated. Magnan states that the simulator is 
unaware of the physiological spasm of the sternocleidomastoid and never 
simulates it. In not a few cases the simulators are themselves epileptics, 
which makes detection the more difficult. 
Treatment of the Epileptic Attack.-The first and immediate measure 
is to determine what shall be the emergency treatment of an attack. 616 
DISEASES OF THE NERVOUS SYSTEM 
If it may not be obviated it is still often possible to mitigate its severity; 
failing in this one may still be able to prevent injury to the patient. 
A constant aura acts as an aid. Localized sensory or motor auras 
hold out the best hope, especially when, as usually is the case, they are 
localized in an extremity; when violent compression, flexion and extension, 
pulling, etc., often inhibit the attack. Forcing back the flexed thumb 
is in this category. But most attacks in which these phenomena occur 
are by nature partial. 
There are certain so-called "inhibition points," pressure upon which 
may check an attack, such as the points of exit of the supra- and infra- 
orbital nerves. Certain "shock" measures are said to abort attacks, 
such as flogging or lashing, a blow between the shoulder blades, causing 
the patient to swallow a spoonful of salt, calling sharply by name. Fere, 
who aborted an attack by severe compression of the auricle, believed 
he had, so to speak, neutralized the discharge just as one paroxysmal 
affection may sometimes act as equivalent to another. 
The practice of giving remedies to lower blood tension, to produce 
nausea (amyl nitrite, apomorph in) is to be condemned because they 
often aggravate attacks. 
During a paroxysm all tight clothing is loosened, the head and chest 
raised, mucus removed from the mouth, and a piece of wood or rubber 
placed between the jaws. Nocturnal epileptics if in institutions should 
be watched; side pieces may be added to the bed to prevent the patient 
from rolling out. It is of great importance that the patient should 
lie undisturbed during the post-paroxysmal period. The exhaustion is 
made good only by rest. If awakened too soon he suffers from headache 
and vertigo. We should give the mind and psyche a long rest. 
In serial attacks we can often arrest them in part by chloral enemata 
(or amylene hydrate). The same holds good for status. In cyanosis 
one may advise venesection. In the first stage of status cold to the 
head and purgatives may be given; in coma, cold affusions, or warm 
packs and analeptics. 
Treatment.-1. Hygienic-Dietetic.-These methods are by far the 
most important. Such methods coincide in part with prophylaxis. 
The principles can be laid down in general terms only; one needs to 
individualize according to the age and social status of the patient. In 
the beginning one should seek out any sources of errors of living and 
whatever calls forth the frequency and intensity of the attacks, and 
endeavor to correct these. Special inquiry should be made, for instance, 
whether there be too much mental effort and stress and if such act as 
undue irritants. In all severe and frequent epilepsies all mental strain 
and school studies must be suspended at once, the criteria being such 
neurasthenic symptoms as headache, rapid fatigue, insomnia, and general 
malnutrition. In their absence one may not rightly infer that the daily 
mental stress or school work is not harmful, and therefore the cessation 
of such activities is desirable in all cases showing severe or frequent 
grand mal, at least during a few months of careful observation and study 
of the individual case. Afterward a gradual increase of educational 
study may be allowed for two or three hours at home There should EPILEPSY 
617 
be, however, long periods of rest intervals even then. Often the plan 
of study should be prescribed or supervised by the physician. 
Not a little advantage gained may be definite information of the 
manner and character of mental work the patient performs. At first 
the schedule of study should be systematic and fairly cover the field of 
general education. Later the plan of study may be more specific, 
according to the liking and trend of interest of the individual and at the 
same time the scheme may be made to disclose the particular line of 
work or occupation the patient may follow in after life. The whole 
life of such individuals should be considered as a plan of comprehensive 
school training and treatment of his malady. If such a viewpoint is 
adopted many relapses after apparent arrest may be obviated. On the 
other hand, if epilepsy begins in adolescence (16 to 20 years) or even 
earlier and the disorder is not brought under subjection by treatment, 
many individuals may have to give up an ambitious career and conform 
their occupation to their disease. They should shun intellectual pursuits 
on general principles for even though the mind may not deteriorate, the 
disorder though not of a severe grade rarely permits an intellectual 
occupation to be pursued with great mental vigor. In those epileptic 
individuals who show congenital mental enfeeblement a certain industrial 
training should be prescribed. Those suffering even marked feeble- 
mindedness are benefited by the simpler sorts of industrial education. 
The schooling must be arranged on lines of physical labor only. 
However, the presence of frequent (daily) severe grand mhl attacks 
at the outset or when the attacks are psychic, rudimentary or nocturnal 
in character, naturally reduces the question to one of active treatment 
with no immediate thought of education as such. 
The choice of occupations is agriculture, horticulture, simple gardening, 
etc. These pursuits are, however, for those patients who are strong, 
muscular subjects. Men more highly educated and refined may take 
up the arts, carving, weaving, drawing, modeling, etc. Such occupations 
often permit the patient purely voluntary employment, to be on half 
time or able to come and go at will, which is the ideal work in consideration 
of the uncertain nature of his malady. Many of those less highly gifted 
and without private means cannot hope to become self-supporting and 
must seek institutional care and treatment where steady practical periods 
of varied employment are commonly supplied. Unfortunately only 
the larger and better endowed institutions of this sort or those under 
generous state supervision and maintenance supply the better class of 
epileptics the proper degree of classification which according to social 
demands and usage they crave. The herding together of the less severe 
epileptics with those imbecile or having serial nocturnal seizures in the 
public institutions cannot be too severely deprecated. If State care 
of epileptics is to be much further advanced careful segregation and 
classification of the young and unfavorable epileptics must be provided. 
Then, too, every means must be provided to furnish such properly 
arranged institutions in which recovery is the dominant idea and not 
merely custodial care. To this end these institutions should be furnished 
with a complete outfit for the treatment of nervous affections. This 618 
DISEASES OF THE NERVOUS SYSTEM 
is a better plan than the reception of epileptics in the great neurological 
institutes where diverse and discordant mental and nervous disorders 
are brought into harmful association. Epileptics should be isolated 
for their own as well as for the good of others. Unfortunately neither 
in this country nor abroad are there a sufficient number of well regulated 
private institutions for the acute cases. Economic conditions probably 
prevent this. The best that can be hoped for at present is to gain privacy 
in a well regulated family nearby some of the large cities in which the 
patient and his nurse may carry out such regulations in treatment and 
work as may be found desirable. If several such individuals are grouped 
in the same neighborhood the expense and labor of providing a common 
source of tutoring, workshops and amusements may be curtailed. Youth- 
ful epileptics may be sent to moderate altitudes (1000 to 2500 feet above 
sea level), which is decidedly beneficial. Travel should never be thought 
of as beneficial in the active treatment of epilepsy. Even when a fair 
degree of arrest has obtained relapses may be caused by such a course. 
Bed treatment is of advantage in the severer grades of epilepsies 
(several attacks daily). It is not only a protection but it permits even 
better study and observation of the case than a larger freedom. 
The dietetic treatment has always stood in the foreground and 
deservedly so, as at least three-fourths of all epileptics have some gross 
functional defects of the gastro-intestinal tract. As in other phases, a 
strict individualization of the dietetic regime of each patient is necessary. 
Often patients have a very poorly balanced ration, living on a single 
class of foodstuffs or what is more often encountered, over- or under- 
feeding. As a rule one has to deal with young fullblooded subjects who 
have a pronounced congestive habit. Thin anemic subjects are excep- 
tional but in both classes excesses in diet cause coated tongue, foul breath, 
meteorism, borborygmi, constipation, diarrhoea, and many types of 
visceral pains. If possible studies in metabolism should precede active 
treatment. Whatever the previous diet may have been it should be 
transformed. All dietetic articles of an irritant nature such as shellfish, 
alcohol, tea and coffee should be eliminated. As a general rule the more 
or less complete exclusion of meats will be found advantageous. A 
purin free diet does not seem to be an asset. The habits of eating should 
be supervised. A fairly common diet card is as follows: 
Breakfast.-Allowed.-Cup of cocoa, with cream and sugar. Wheat cereal 
with cream and little sugar. Two soft boiled eggs, or minced chicken. Rolls, toast, 
pulled bread, or zwieback. Maximum amount of butter, preferably unsalted. 
Strained honey. Orange marmalade or Dundee jam. Plenty of stewed fruit. No 
hot bread. 
10 to 11 a.m. Choice of glass of milk (top), or milk and crackers. Cup custard, 
junket, egg-shake or raw eggs, chicken sandwich, malted milk, buttermilk, or lactone 
milk. 
Luncheon.-Allowed.-Choice of fresh fish, chicken, lamb, mutton. Fowl of 
all kinds, except domestic duck or goose. Peas, beans, spinach, samp, rice, macaroni, 
spaghetti. Any vegetable that may be put through a puree sieve, such as turnips, 
carrots, etc. Boiled potatoes may be taken occasionally, and thoroughly masticated. 
Tender beets and oyster plant stewed. Choice of rice pudding, farina, corn starch, 
blanc mange, prune souffle, tapioca, ice cream, but no fruit ices. Baked apple with 
cream, stewed figs, and prunes. Cream cheese, Camembert, pot cheese. 
Allowed Occasionally.-Asparagus tips. Salad with French dressing. EPILEPSY 
619 
Not Allowed.-Soups; liquids restricted to less than one glass. Steak, roast 
beef, pork, salt fish, or shell fish. Sweetbreads or kidneys. Radishes, raw celery, 
anchovy. Oranges, grape fruit, raw apples. 
4 p.m. Same variety as at 11 a.m. with the additional choice of cocoa with sugar, 
or a farinaceous dessert, such as on luncheon list. 
Dinner.-Same variety as for luncheon. 
While liquids are restricted at meals, water may be taken freely between meals. 
No alcohol allowed. 
It has been more or less definitely proved that the use of bromine as a 
remedy disturbs the chlorine metabolism, therefore salt may be reduced 
in the diet when the former is given. Numerous plans of diet have been 
constructed upon the foregoing idea. A sample is 1000 gms. of milk 
daily, 50 gms. of sweet butter, three eggs and from 300 to 400 gms. of 
bread with sodium chloride replaced by the same quantity of sodium 
bromide. The results from such a diet are often striking but it is difficult 
outside of special institutions to get such diets properly and consistently 
administered; besides, it is insipid to the average patient and is refused 
in the course of a few days' use. It is better so far as possible to select 
salt-free foods, but one should then guard carefully against bromism 
which under such a dietary often supervenes with remarkable celerity. 
It is a paradoxical behavior that often upon a salt-poor diet a patient 
becomes very sensitive to sodium chloride, so that a small dose, as in 
colonic flushing with normal salt solution, causes a seizure. Moreover, 
in such dietetic treatment a small addition of bromide precipitates an 
attack of bromism. Many confusing symptoms often supervene in 
the administration of salt-free diet and are far from being explained. 
It should, however, be tried in every case in which bromides are employed. 
Hydrotherapy.-Morning bathing in lukewarm water for the delicate 
and cool baths (70° to 80° F.) for the robust patient followed by good 
frictional rubs are of essential benefit in treatment. If warm or lukewarm 
baths are employed a cool shower (60° to 80°) should always be given 
afterward. Alternating warm and cold showers of a variation of 15° 
to 20° may be employed when the circulation needs to be aroused, which 
is especially needed in all bromide therapy. Strong cold douches alone 
are not advised. 
Electrotherapy has little or no place in the therapy of epilepsy. Gym- 
nastics and massage are always valuable in increasing muscular strength 
and overcoming anemia when present. Packs and bed rest are often 
advisable in the overactive and too energetic epileptic. 
The mmtaZ treatment is of first importance, not alone that he may be 
encouraged to renewed efforts to carry out the daily routine (a sample 
of which will be given later) which, because of its very monotony is most 
frequently abrogated in a few weeks if not thoroughly supervised, but 
that the patient may be taught right principles of mental hygiene. 
Often enough epileptics have had no proper bringing up either because 
of parental neglect or in the belief that their dilapidation of character 
is an essential part of the disease and which, if corrected, may entail 
more frequent and severe seizures. Hence the ideal treatment of all 
youthful epileptics should begin by removing them from the home and 
all its surroundings. They should be placed if possible in small colonies 620 
DISEASES OF THE NERVOUS SYSTEM 
where some fellowship with others may be enjoyed. At home they are 
often shunned by their social equals and this causes great depression and 
more frequent attacks. 
Suggestion or hypnosis and their various modifications play little 
role although the occasional improvement noted when placed under 
secret remedies shows plainly that some suggestion may work to advan- 
tage. Psychoanalysis, although of greatest value in psychogenic convul- 
sions (Bratz type), has yet to be carefully employed in genuine epilepsies. 
When this is done we may expect more light upon the exact mental 
training required in the treatment of the individual epileptic. 
Drug Treatment.-The number of remedies which have been used 
is incalculable. This is natural both because it is such a stubborn 
disorder and because a cessation of attacks frequently follows any plan 
of treatment, by coincidence, suggestive effects, or because the remedy 
may possess a certain degree of sedative power necessary in the individual 
case. Before taking up such a list of drugs one should consider some of 
the conditions under which the disease improves or eliminates itself with 
the use of any drug. This includes not alone the plan of hygienic- 
dietetic regimen but the conditions under which spontaneous recoveries 
occur in young epileptics. In many such the diagnosis has been at 
fault or the employment of slight principles of corrective living have 
been sufficient to bring about amelioration or cure. 
One may rightly ask if there are any anti-epileptic remedies per se 
that have a special or exclusive action on the disorder independent of 
their general utility as nerve sedatives. Valerian was the mainstay 
of the profession for ages or until the introduction of belladonna. Atropin 
is still greatly employed but usually in combination with other sedative 
drugs, and especially when vasomotor crises are prominent either in 
conjunction with grand mal attacks or interspersed between frankly 
convulsive seizures. In view of the fact that the number of individual 
attacks is usually so speedily reduced by the exhibition of bromides, even 
to the entire suppression of them, the hope that the drug is a real curative 
agent in the disease has been and still is widely held. So great has been 
the reliance upon the bromides that real scientific therapy has actually 
been delayed by this erroneous empiricism. Until a few years ago the 
almost wholesale use of the drug made it possible for a public clinic to 
count the cases showing bromide poisoning as the rule rather than the 
exception. Happily the recognition that there is an acquired art in the 
use of bromides is removing this reproach. Further, the sudden sup- 
pression of all the convulsive manifestations of epilepsy after prolonged 
use of bromides or by an abrupt withdrawal of the same is often followed 
by a serial or status period in which attacks occur continuously or are 
overlapping, in which a primary mortality of 50 per cent, obtains. 
It is a safe rule never to begin the steady use of bromides until all 
other hygienic-dietetic plans have been tried. Personally I employ 
bromides in less than 10 per cent, of my cases. Probably the most 
convincing proof of the successful use of the bromides is furnished by 
the statistics of institutions in which the drug is presumably used with 
rigor and at the same time with caution. In these institutions all modi- EPILEPSY 
621 
fications and combinations of bromides have been tested with the idea 
of the economy of dose, elimination of toxicity, and especially the use 
of adjuvants. The ordinary limit of tolerance of the drug is not far from 
60 to 80 grains daily while the average dose should not be much above 
30 to 40 grains daily. If these doses are not effectual the patient is not 
suited to bromide therapy. Usually the attacks are suppressed in greater 
part by such medication, for a time at least, until too great reliance 
is placed upon the drug and the main hygienic-dietetic regimen is allowed 
to fall into loose observance and the attacks recur as severely and as 
frequently as before. Thus one says the "drug is outworn," when 
in fact the other and more important principles of treatment have been 
disregarded. It is best to impress the patient at first, and constantly 
thereafter, that the real value of the treatment is non-medical in character 
rather than the reverse. One may thus obtain the attention of the 
patient to all the matters of routine of diet and hygiene in the absence 
of any specific drug medication. Then the bromide may be gradually 
increased by 1 grain a day, beginning at 10 to 15 grains daily as the 
minimum of effective medication. Some of the symptoms of bromide 
poisoning especially to be looked for are undue fatigue, drowsiness, 
abolition of sexual desire and the pharyngeal reflex, coated tongue, 
bromide rash and fetor of the breath. The undue prominence of any 
or all of these symptoms should cause one to lower or withdraw the 
bromide. It is generally held that some slight manifestations of many 
or all of the evidences of bromide ingestion as above outlined are necessary 
to show that the system is in a state of saturation, at which point the 
most effective use of the bromides is obtained. Personally, I prefer 
never to obtain the symptoms of bromism. I always employ the bromides 
as but secondary and adjuvant aids in the treatment of any case when 
sedatives are necessary. In many instances the judicious modification 
and use of baths, mouth cleanliness, colonic flushings, massage and 
physical gymnastics and dietetic principles (eggs, fruits, milk and vege- 
table foods) may cause the rapid disappearance of the first symptoms of 
bromism without altering the dose of the drug. In many severe cases 
of epilepsy in which exceptionally very high dosage of bromides may be 
desired it is possible by extra attention to the details of treatment under 
strict medical and nursing supervision to give patients as high as 180 
or 200 grains daily without marked and untoward physical depression.1 
The whole plan of treatment including the bromides should be continued 
for years even though attacks should cease. Some authorities regard 
the plan of successful routine a necessary plan of life henceforth; its 
long continuance after attacks have ceased (for several years at least) 
seems to upbuild and make for a permanency of the restored normal 
psyche. In the absence or partial abrogation of the continued treatment 
one most frequently may successfully seek for the cause of relapses. 
Epileptic children having a better tolerance than the adult to sedatives 
the dose may be held to 25 to 60 grains daily during the active phases of 
treatment. An epileptic, it must be remembered, does not have a greater 
1 Clark, Am. Jour. Med. Sc., 1908, cxxxv, 94. 622 
DISEASES OF THE NERVOUS SYSTEM 
tolerance to sedatives than the normal individual, despite the common be- 
lief to the contrary. The bromides having a selective action for the cerebral 
cortex, one may note its sedation here first, in diminished time reaction 
and memory defects. The emotional change in irritability is also to be 
noted. One must remember, however, that the latter is usually more 
marked in the frankly established disorder to begin with and one must 
therefore not unduly accuse the drug. Often a moderate irritability 
may be increased to attacks of rage and even assaults of violence by 
sedatives,-a sort of bromide intoxication thus supervenes. Not infre- 
quently in highly excitable individuals in whom the attacks have marked 
psychic expressions, the outburst of temper alone requires the with- 
drawal of sedatives. Now and then one not only experiences an increased 
number of attacks accompanied by insomnia, a tendency to hallucinate, 
but actual sensory illusions and a sort of manic excitement which usually 
disappears as the bromides develop their usual pronounced sedation. 
While the absence of corneal reflex is characteristic of the full action of 
bromide sedation its appearance in epilepsy may be late and vary much 
in its persistence, therefore it loses much of its guiding value on the 
exact amount of sedation at a given time. A wide variation in the time 
and degree of bromism shown in bromic acne also renders it not so trust- 
worthy for the finer gauging of the action of the drug. The long detailed 
accounts of the severe forms of bromic cachexia have diminished in 
importance and significance in the literature of epilepsy since more 
watchful and observant plans of treatment have obtained. Suffice it to 
say in addition to the slighter symptoms heretofore detailed that there 
are stupor, loss of reflexes, ataxia, tremors and inability to write, anorexia, 
suppression of urine, severe constipation, slow and shallow respiration, 
and difficulty of swallowing. Death from aspiration-pneumonia or 
broncho-pneumonia has often supervened in such poisoned individuals. 
Obviously on the slightest suspicion of these grave symptoms all sedation 
should be discontinued. Contrary to what is often held the exhibition 
of bromides never causes permanent deterioration of the mental faculties. 
Whenever the bromides are not effective, which as a rule is true sooner 
or later in the majority of cases, one may try the various substitutes 
including belladonna, digitalis, iron, opium, camphor, chloral, or their 
various combinations with bromides. The opium-bromide treatment is 
highly recommended and employed but has been of little permanent 
use in my hands. In patients with a positive Wassermann reaction it 
is not easy to reach the seat of the disease for there may be incurable 
arterial lesions. As a special resource a combination of bromides and 
iodides may be used with profit, preceded perhaps by an opium cure. 
Organotherapy as yet offers no prospect of relief in epilepsy. 
A sample of the daily routine under an attendant's care for a youthful 
individual suffering from an epilepsy of moderate severity is as follows: 
Rise at 6 a.m. The patient to take either a glass of hot water or a cup 
of hot malted milk before or just after rising. Patient should then take 
five to ten minutes' active gymnastic exercises, with dumb-bells, medicine 
ball, Indian clubs, or simple setting-up (military) exercises. EPILEPSY 
623 
6.30 a.m. Cold bath, plunge, or cold douche standing in warm water. 
Rub down with coarse towel and dress. 
7.30 a.m. Breakfast as per dietary. 
8.00 to 10.00 a.m. Two hours' physical exercise such as cutting 
wood, tennis, golf, horseback riding, bicycling, etc. 
10.00 a.m. Light lunch as per dietary. 
10.30 to 12.30 a.m. Study, reading, sedentary work, letter writing, etc. 
12.30 to 1.00 p.m. Rest lying down, dressed. 
1.00 to 2.00 p.m. Dinner as per dietary. 
2.00 to 2.30 p.m. Rest sitting or half reclining. 
2.30 to 4.30 p.m. Physical exercise either indoor or outdoor, shop, 
farm or garden. 4.30 p.m. Luncheon as per dietary. 
5.00 to 6.00 p.m. Colon flushing alternating with cold pack in summer 
and warm in winter. 6.00 to 6.30 p.m. Rest. 
6.30 p.m. Supper as per dietary. 7.30 to 8.00 p.m. Rest. 
8.00 to 10.00 p.m. Games, sports, etc., in which patient may do as he 
pleases. 10.00 p.m. Warm bath or sponge and to bed. 
In an ideal environment the patient lives away from home in a small 
country town with his companion-attendant in a small family. He is 
permitted to smoke in moderation if the habit is already contracted; 
no alcohol is allowed. A short attendance at religious services may be 
allowed once a week. Fishing, hunting and boating may be allowed 
under close supervision. 
Operative Treatment.-Surgical measures are indicated only in so-called 
reflex epilepsy, partial Jacksonian and general traumatic epilepsy. 
The results here have not fulfilled the claims put forward. Temporary 
improvement with occasional remissions are seen often enough, but there 
is only a small fraction of permanent cures. We should, however, not 
discourage operation whenever any purely organic epileptoid condition 
appears to threaten the development of the epileptic alteration. All 
such operations are self-indicated without reference to epilepsy. Irritable 
scars should be excised and lesions of the skull, meninges and cortex 
remedied whenever found, on general principles. CHAPTER XVI. 
NEURASTHENIA. THE TRAUMATIC NEUROSES AND 
PSYCHOSES. 
CHARLES W. BURR, M.D. 
NEURASTHENIA. 
The word neurasthenia is used with both an indefinite and a precise 
meaning. Unfortunately it is frequently used to designate very unlike 
conditions, sometimes as a cover for ignorance, and sometimes as a 
euphemism for a more serious word-insanity. It is often confused 
with hysteria and sometimes regarded as mere laziness. It may be so 
severe as to cause permanent disability or so slight as to be recovered 
from in a few weeks. Its symptoms are numerous, and its true primary 
cause is still a matter of dispute. The difficulties of its study are, there- 
fore, manifold, and many of the apparent differences of opinion among 
writers arise from using the same word to designate different things. 
Notwithstanding the frequent use, indeed abuse, of the word, primary 
neurasthenia really is infrequent, and the diagnosis is becoming more 
rare as the methods of study improve, as our knowledge of the chemistry 
of disease grows, and as we examine patients with greater and greater 
thoroughness. Yet it is a really existing thing, and not a mere word. 
Strictly defined it includes only a condition of pathological weakness 
without discoverable lesion, showing itself by too rapid and too great 
fatigue, physical or mental, or both, emotional unbalance, and undue 
irritability (too great response to stimuli) of the nervous system. It 
means disability or inability more than perversion of function. The 
machine runs too slowly and weakly rather than wildly or pervertedly, 
although this also happens. 
One thing always to be remembered is that there are two distinct 
types of the condition, one a primary disease and the other a secondary 
state. From the examination of the patient on any one day, and in 
ignorance of his past history, it is often impossible to determine which 
of the two we are dealing with. Time will always show, but it is impor- 
tant to make the correct diagnosis early and not late, and therefore it is 
important to learn the patient's personal and family history. It is more 
than probable that future discoveries in biological chemistry will decrease 
the number of cases diagnosed as primary and increase the number 
diagnosed as secondary, but there will remain a certain number prenatal 
in origin and not due to external causes arising after birth. 
Etiology.-We shall first consider the cause of primary neurasthenia 
and then some of the diseases causative of secondary neurasthenic 
conditions. Primary neurasthenia is prenatal in origin and, in the 
624 NEURASTHENIA 
625 
majority of cases, inherited. These two words, prenatal and inherited, 
are usually used by medical men as synonymous, but the former includes 
the latter. Hereditary influences are those carried by the germ cell 
and the sperm cell, from generation to generation, and are to be kept 
separate from the influences arising and acting after fusion of the cells. 
Prenatal influences include the hereditary plus those which, arising 
after conception, act upon the fetus from without. We know little about 
the effects of pathological prenatal influences, save in the case of gross 
disease, and nothing about the mechanics of heredity. We know but 
little of the late results (results appearing in childhood or later) of mild 
illness of the mother or of the embryo on the future mental and physical 
condition of the offspring. It is very possible, for example, although 
it has never been proved, that an acute febrile attack in a pregnant 
woman, not severe enough to cause abortion or anything noticeable at 
the child's birth, may yet so weaken it that the power of resistance to 
the stress of life will be greatly decreased. It is possible that alcoholic 
intoxication of either parent, otherwise healthy, at the time of conception, 
may have a permanently ill effect on the offspring which yet may not 
show itself till long after birth. It has been claimed that this has been 
clinically proved by the exclusion of all other possible causes, but we do 
not know, nor can we learn, whether acute alcoholism can affect human 
spermatozoa and ova, and to exclude all other causes is, to say the least, 
difficult. It has been claimed to have been experimentally proved that 
acute intoxication does affect the spermatozoa in certain lower animals. 
Chronic alcoholism may, by the widespread and serious tissue changes 
it produces in the parent, with consequent disturbance of function, 
undoubtedly affect the offspring. 
The fundamental symptom of neurasthenia is inability to withstand 
the normal amount of stress without breakdown, shown by excessive 
fatigue and irritability. Stress is the sum of all the forces which act 
upon the individual organism and its constituent cells, and therefore 
includes disease, the wear and tear of life, everything affecting the 
emotions, mechanical work, and intellectual effort. In the primary 
form of neurasthenia this weakness is congenital; we come into the 
world with unequal powers of resistance to emotional, intellectual, 
physical, or chemical stress. We are only beginning to know something 
of the causes of inequality of ability to resist, and what we do know 
concerns almost altogether chemical immunity in mycotic disease. 
Of the causes of congenital muscular or mental weakness apart from the 
cases in which there is manifest gross disease we know nothing. It is 
possible that congenital abnormalities of function of one or several 
of the ductless glands may be at the root of much neurasthenia rather 
than any disease arising primarily in the nervous system itself, but 
today this is a theory rather than a proved fact. This is certain, that 
while the great majority of people are able to successfully resist the 
ordinary stress of life, there are a number who succumb, whatever the 
reason may be, under a burden which the majority carry well. The 
chief predisposing cause seems to be, in fact is, a bad heredity. In 
almost all cases of serious primary neurasthenia, if the family history 626 
DISEASES OF THE NERVOUS SYSTEM 
be known, there will be found "a bad strain in the blood.'' By this, of 
course, is not meant moral viciousness, but biological weakness. There 
is present some nervous instability in the family. It is a tendency which 
is inherited, not neurasthenia itself. Thus, neurasthenia does not neces- 
sarily beget neurasthenia, nor hysteria hysteria, but nervous or mental 
instability produces either itself or some allied weakness. 
Further, since in nature there is always an endeavor (the limitations 
of language compel us to personify nature and speak of it as if it willed) 
to regain the normal, a very bad ancestry may beget a good posterity. 
Most neurasthenics, then, show something wrong in the family history. 
It may be'general nervousness, eccentricity, or genius, at least pseudo- 
genius, or it may be some definite disease, as hysteria, insanity, epilepsy, 
gout, or alcoholism. General nervousness or mere invalidism is a more 
frequent ancestral trait than the various neuroses, psychoses, or organic 
nervous disease. This may be merely because invalidism is more frequent 
in general than serious organic or functional nervous disease, i. e., the 
greater frequency may be merely arithmetical and not one of percentage. 
The writer is well aware that there is a tendency among certain writers 
and in the popular mind to minimize the effects of inheritance and to 
magnify those of environment, to believe that education and training 
can overcome all inherited evil tendencies, and that all of us could be 
well, strong, brilliant, and altogether normal if only we had been taught. 
Education can do much, and a bad environment may injure the good just 
as a good environment may help the bad, but an inheritance of health 
is still better than riches. 
Some primary neurasthenics break without any discoverable exciting 
cause; in most, however, some unusual external stress is necessary, 
however slight. The average age of onset of marked symptoms is late 
adolescence or early maturity, but some subjects retain fair health till 
middle life and a few succumb in early childhood. Puberty is a critical 
period. Some of the boys and girls who in childhood appear bright 
and strong, in every way normal, at about fourteen or fifteen begin to 
complain of headache, backache, and mental tire, no longer do well at 
school, cease to take pleasure in active muscular play, and are fretful and 
peevish. They are really entering on a life of neurasthenia. Some die 
of senility while they are still mere children by the calendar. Only the 
healthy man's age is measured by the courses of the sun, the weakling's 
clock runs more quickly. The amount and nature of the stress necessary 
to cause breakdown vary with the individual. Some can carry a burden 
almost equal to the average, others reach the straining point far short 
of this. Mental work, even if carried to excess, provided it is not accom- 
panied by worry, is not a frequent cause, but when worry is present, 
neurasthenia is frequent, and often the first symptom is inability to think 
consecutively and continuously. Many intellectual men are neurasthenic, 
but not often because of their work, and much of the world's work has 
been done by invalids. Much pessimism is due to what one may call 
neurasthenic dyspepsia, and the reader, especially if he be young and 
ingenuous and take the printed book too seriously, may, in turn, get a 
mental dyspepsia leading to physical neurasthenia. It is probable that NEURASTHENIA 
627 
the reading of books of a certain type has caused more neurasthenia than 
the writing of them. 
Emotional stress is a much more important factor than physical over- 
work. In women the countless little troubles of life and family care are 
very important. The common notion that only the rich and socially 
well placed among women have neurasthenia is absolutely false. It is 
more common among working women than among those who are relieved 
of the burden of work. This is becoming more noticeable since women 
have invaded the work formerly monopolized by men. School teachers 
and workers in stores and factories are especially susceptible. The mill 
demands and gets an awful tribute in the broken health of countless girls. 
It is not alone, not so much, the actual work that causes breakdown as 
the whole manner of life and the associations of the average mill girl. 
A few, one had almost written a great many, physicians have objected 
to the so-called higher education of women on the ground of injury to 
health, but they are regarded as old fogies and no one listens to them. 
Perhaps they will be listened to some time in the future. Today it is 
wisest not to endeavor to oppose the current of popular opinion and 
desire, for that is impossible, but let us not try to make Portias out of 
common mortals nor forget that motherhood is rather more important 
than science, literature, or even politics. Professional life, especially 
the practice of medicine, has done but little good to many of the women 
who have gone into it. 
Child-bearing should in no way be injurious to normal women, but 
among those under par it is an occasional cause, as are the infectious 
fevers. Women are more prone to primary neurasthenia than men, 
but secondary, symptomatic cases seem more frequent in men. This is 
probably because men are still exposed to more stress of different kinds. 
Bad education is a great predisposing cause. The most important 
purpose of education, far more important than book learning, is to teach 
self-control, and none needs to be taught this as much as the offspring 
of the weak. Often, on the contrary, the neurasthenic that is to be 
receives the poorest education; he often learns unconsciously, from his 
elders in the family, to whine, to grumble, to be unwisely selfish. His 
ego is hardened, his altruism destroyed. He is petted and scolded and 
spoiled and soon made worthless. His little aches and pains are made 
much of, and obedience is unknown to him. The writer is not preaching 
brutality to children, and has no sympathy with the puritanical dogma 
that pleasure is sin and happiness a preparation for damnation, but one 
must learn to obey before he can command himself. Undue severity 
and lack of sympathy may do as much harm as the reverse. There 
are children, and often they are of the best, who are so out of tune with 
their human surroundings that they are driven in on themselves and live 
their own lives in a subjective world all of their own creation. This, 
however, more often leads to insanity than to neurasthenia. 
Occupations are important causative factors. Those causing great 
emotional stress are most dangerous. Thus, actors, business promoters, 
speculators (financial, not philosophical), and clergymen of the intense 
type are especially liable to break down. The fault is not altogether 628 
DISEASES OF THE NERVOUS SYSTEM 
with the work. It is partly due to the fact that emotionalists are prone 
to be drawn to it. Climate, or rather the change from a temperate 
to a hot country, has an influence. Woodruff, for example, has described 
the neurasthenia occurring in white people living in the tropics. Race, 
too, is important. The American negro never suffered from neurasthenia 
till recently. But now it is of not infrequent occurrence, especially in 
the half-breeds. Alcohol, syphilis, and moral degeneration partly 
account for this, but more important is the fact that a lower race is trying 
to compete with a higher, and is unfit to do so. The strain is too great, 
and, although it seems a hard saying, the fittest to survive in the moral 
sense are often the unfittest in the biological sense. Apart from organic 
disease and bad morals, the mere struggle of the best specimens of an 
inferior race to attain the plane of a superior leads often to their downfall. 
Among Caucasians the Jews seem to be the most prone to neurasthenia. 
Of course in them it is not a question of racial inferiority, rather the 
reverse. The usual explanation given is that years of oppression have 
at last affected their stamina, but the cause, whatever it may be, is deeper 
than that. It certainly plays no part in this country, because several 
generations of living here does not seem to decrease the tendency. Those 
of us who read the Old Testament can recall some evidence that even in 
the days recorded there the disease was not unknown among them. 
Much has been written to prove that some one thing is the cause of 
primary neurasthenia, indeed of all neurasthenia-floating kidney, 
ptosis of one or all of the abdominal organs, diseases of the genitalia in 
women, aberration of the sexual function, even lateral curvature and 
eye-strain, have each been upheld as the one and only cause of the con- 
dition. All these things are evil, and any one may be an exciting cause 
of nervous symptoms, and each should be looked for and if present 
attended to, but no one is a primary cause. That is inherent in the 
patient himself. The influence of trauma as a cause will be discussed 
elsewhere. Probably the most frequent exciting cause is the mere 
burden of life, the worries and frets, the great griefs and responsibilities, 
the dull routine, the lack of ability to get interested in the game of life, 
and, on the other hand, playing the game too hard. If we could be 
taught, and could obey the lesson, that everyone must live his life within 
the limitations of strength nature has put upon him, much neurasthenia 
would be prevented, but unfortunately few are taught till too late, and 
many who know are so enmeshed in the net of adverse circumstance 
that they cannot obey the lesson. 
In secondary or symptomatic neurasthenia the cause can usually be 
found if sought for, but not a few serious organic diseases may, for a time 
at least, hide their own proper symptoms and show only weakness. 
The writer well remembers a man under his care when a hospital resident. 
The chiefs, distinguished and skilled men, diagnosed his case as neu- 
rasthenia. lie complained of great general weakness and nothing more. 
After a rest cure he was persuaded and mildly browbeaten into taking 
more and more gentle exercise, longer and longer walks. He himself 
thought he was getting better. One day he told me he was too tired and 
weak to get up, and I, with the superior air of the young interne, rather NEURASTHENIA 
629 
scolded him. He died before I had walked the length of the ward. 
The necropsy showed marked chronic myocarditis. He was not a 
neurasthenic, but died because his heart muscle could no longer do its 
work. Another patient, a man beginning to be old, lost his wife, to whom 
he had been devoted. He began to complain of pain in the back of his 
head and neck, and of general weakness. He slept poorly, ate capri- 
ciously, lost interest in all things, and began to pass large quantities of 
urine. He then took to his bed. Examination revealed nothing. He 
improved a great deal for several months, when a sarcoma of the thigh 
appeared, ran a very rapid course, and he died. The sarcoma had nothing 
to do with the early symptoms, the association of the neurasthenia and 
the sarcoma was purely accidental, but it was hard for the family doctor 
to make the patient's people believe so. 
The later stages of syphilis may, for quite a long time, present nothing 
but neurasthenic symptoms, and, without a history of chancre or of 
previous organic symptoms, an accurate diagnosis is impossible. Given 
a man who presents symptoms of weakness without manifest cause, 
especially if his previous life has been healthy and he has borne stress 
well, it is important to inquire very closely into the possibility of luetic 
infection, and if there be any evidence whatever it is well to put him 
to the therapeutic test. The following is an example: A young man 
complained of loss of memory and inability to fix his attention, with 
general weakness. Examination revealed no sign of organic disease. 
His history, however, cleared the matter up. He had had a chancre 
about five years before, and later suffered from a transient apoplectic 
attack with aphasia, still later an epileptiform seizure with a temporary 
left-sided hemiplegia, and at another time an ocular palsy lasting only a 
short time. It is remarkable that such serious symptoms should have 
occurred and left no trace in the way of residual palsies, disturbance of 
the reflexes, etc., but such was the fact. His symptoms were not 
neurasthenic but due to a diffuse brain degeneration. 
Sometimes, and with increasing frequency since the appearance of the 
present custom of surgeons in getting patients out of bed at the earliest 
possible moment after an operation, severe neurasthenia follows operation. 
The writer has not infrequently seen months lost out of the lives of 
patients and much suffering ensue because surgeons were stingy of a few 
more weeks in bed. This is true not only of women, but of men patients. 
Profound neurasthenia often follows typhoid fever and influenza, and in 
the latter especially there seems to be no relation between the severity 
of the primary infection, or rather of the symptoms during the fever, and 
that of the neurasthenia. It is notorious that two or three days of 
influenzal fever may be followed by weeks, even months, of convalescence, 
the patient feeling as miserable and being as weak as if he had passed 
through some long and dangerous illness. 
There is a distinct type of neurasthenia, commoner among young men 
than young women, due to vicious sexual habits in adolescence. Not 
only does the habit itself do harm, but a large part of the literature about 
it does more harm. There is scarcely a book on the subject, and they are 
all sold to and read by youths, which does not do the boy reader harm 630 
DISEASES OF THE NERVOUS SYSTEM 
and, if he is neurotic, irreparable injury. Many a boy has been per- 
manently injured in mind by fright after being made to believe, by 
reading pseudoscientific literature, that he is congenitally perverted, 
when really he is only the victim of a habit which can be cured. Some 
authors claim that sexual abstinence may be a provocative cause, but 
there is no foundation for such belief. 
Symptoms.-It is very difficult to give a clear picture of the symptoma- 
tology of neurasthenia, because cases vary so in intensity and severity 
and because what symptoms or group of symptoms predominate depends 
so much on the personality of the patient. While the cardinal symptoms 
of rapid fatigue and undue response to stimuli are always present, the 
organs which show the irritability vary most markedly in different 
patients. This variability is so great that it has even been proposed 
to differentiate spinal, cerebral, gastric, and many other kinds of neu- 
rasthenia, but this is too great refinement in diagnosis, or rather it 
confuses the place of manifestation of symptoms with the place of origin 
of the disease. One organ may present the greatest number of symptoms, 
but the disease is general. It is impossible to give an account of the 
disease that will cover and include all cases. The best which can be 
done is to relate the symptoms and indicate the commonest combinations. 
An attack is never sudden in onset, although there may be an acute 
outburst of severe symptoms. The physician may be told that the 
illness came on acutely, but investigation will always show that really 
the onset was slow. The great symptoms are physical and mental tire. 
The patient finds that she, for the disease is more frequent in women, 
is very easily fatigued. Molehills are mountains and the grasshopper 
a burden. Things that she could do before without effort now become 
burdensome. At first she does not think that there is much the matter. 
If the condition is severe she becomes unable to make any continuous 
muscular effort, and soon is bedridden. At the same time mental effort 
becomes difficult. In the beginning there is no difficulty in starting a 
train of thought, but in carrying it on for any length of time and in keeping 
the attention fixed. Finally, continuous thought becomes impossible. 
There are rare cases in which the weakness is entirely physical and not 
at all mental. There are men and women who can do continuous mental 
work when physically they are bedridden. But no matter how difficult 
it may be to think, there are never any profound perversions of thought. 
There are no delusions of grandeur, of self-accusation or of persecution, 
or, indeed, of any kind. The patient may be depressed and even fearful, 
being startled by the inability to think, and fearing it is the precursor 
of mental breakdown. Frequently there are morbid fears-a sense of 
impending evil, fear of closed or open places, etc. 
There is usually some emotional depression; the patient is, as a rule, 
unhappy, but this is a natural though exaggerated result of the weakness. 
It arises occasionally from hyperconscientiousness, the patient feeling she 
is neglecting her duty, that she is not properly looking after her children 
or caring for her husband, that she ought to rise up and do her work, 
and that it is her own fault she does not do so. Or if she is of a different 
temperament she becomes peevish, fretful, fault-finding, and supremely NEURASTHENIA 
631 
and childishly selfish and unreasonable. When depression becomes 
profound and unsurmountable the patient is bordering on, if she has not 
reached, melancholia. A few patients maintain emotional equilibrium 
throughout a long attack. There is never any serious change in the moral 
sense, and the neurasthenic is never led by illness to acts of shameless 
crime, or even to slight infraction of the moral law. In severe cases which 
have lasted a long time there is frequently a great increase in selfishness, 
and this is not to be wondered at, because there are few Jobs in the world. 
Pain or rather unusual feelings (paresthesias) are always present in the 
head. Occipital ache is the most usual complaint. This may come on 
apparently spontaneously or follow muscular effort, thought, or worry. 
There may also be brow ache or pain in the vertex. Some patients 
complain only of queer, indescribable sensations in the head. They 
speak of them as dragging, drawing, or straining feelings, or compare 
them to a tight band around the head. Slight vertigo is common. There 
may be hyperesthesia of the scalp, and pain may be referred to the hair. 
After the head the spine is the most frequent seat of pain. It may be 
localized or diffuse, and, as a rule, a mere touch on the skin over the 
backbone causes more pain than deep pressure. Although there is never 
anesthesia, the patient frequently suffers paresthesias in the hands and 
feet-pins and needles, a feeling of a tight glove, cold or hot flashes. 
Curiously enough, pain due to organic disease, e. g., gallstone colic, may 
be fairly well borne. Paresthesias are often more distressing than pain. 
Circulatory disturbances are always present. The hands and feet are 
cold, moist, and bluish or bluish-red, or the skin is dry and scaly. There 
may be attacks of cardiac palpitation, a choking sensation as if the 
heart were in the throat, or the heart may seem to stop beating for an 
appreciable time. Sudden movement may cause vertigo. Although 
the skin is washed out and pasty looking, and the lips have lost their 
color, there is no true anemia; the circulation, not the blood, is at fault. 
There may be, and probably is, a decrease in the total quantity of blood 
in the body-that is a thing that cannot be measured-but there is never 
any great decrease in the hemoglobin percentage or in the number of 
red and white corpuscles. Leukocytosis is never present unless there is 
some complication. The explanation of the cases of apparent plethora, 
the red-cheeked patients, probably is a reduction in the quantity of the 
plasma of the blood, causing an increased blood count from concentration. 
Loss of appetite may be present from the onset, come on later, or the 
appetite may be capricious-today ravenous and tomorrow gone. The 
sight of food may produce nausea, and its presence in the mouth may 
fail entirely to stimulate the salivary glands to action. This is one of the 
causes of difficulty in swallowing in neurasthenics. Food in the stomach 
may almost entirely fail to excite the gastric glands to secrete. After 
eating there may be a sense of weight in the epigastrium, a feeling of 
smothering and even pain. The intestines may be distended with gas. 
Sometimes eating increases the headache. The bowels are, as a rule, 
constipated, or constipation may alternate with diarrhoea. Pain may 
be referred to the appendix in the absence of any inflammation in it. 
Often there are annoying feelings in the rectum after a stool, as if the 632 
DISEASES OF THE NERVOUS SYSTEM 
bowel had not been emptied. The gastrie atony may go on to catarrh 
and eating be followed by vomiting. When anorexia and indigestion 
are marked, emaciation always follows, and may be so great as to reduce 
the patient to a mere skeleton and make one fear the existence of some 
malignant disease or visceral tuberculosis. 
Sleep is, as a rule, poor; either there is more or less insomnia or the 
patient dreams much and nightmare is common. The patient does not 
awake rested and refreshed, but weary and depressed. The morning 
is the worst period of the day with all neurasthenics. In mild cases there 
is no disturbance of menstruation, but in the more severe amenorrhoea 
occurs and leucorrhoea is common. There may be nervous irritability 
of the bladder and consequent frequency of micturition, and, on account 
of the habit of neurasthenics of taking too little liquid, the amount of 
urine voided may be below the normal and its specific gravity too high. 
Of course, the presence of albumin and casts indicates something other 
than neurasthenia. Endeavors have been made to find - distinctive 
substances in the urine which would throw some light on the origin of the 
disease, but so far without success. 
The deep reflexes are, as a rule, increased, but genuine, persistent 
ankle clonus can never be obtained, although quite often there is an 
abortive or spurious clonus shown by a few coarse and irregular vibrations 
of the foot when it is passively dorsally flexed. The existence of a per- 
sistent and rhythmical ankle clonus means organic disease. A tap on 
the knee often causes a start of the entire body. Although perhaps 
increased in amplitude, the knee-jerk is never spastic. It often diminishes 
under repeated stimulation, and when the patient is extremely weak it 
may be so slight as to be obtainable only on reinforcement. If the knee- 
jerk is absent it is well to look for other signs of organic nervous disease, 
for surely the absence is not caused by neurasthenia. Its absence is 
congenital and significant of nothing in one person in five hundred. 
Vision and hearing are the two special senses most often affected. 
True deficiency of vision is rarely present at the beginning of an examina- 
tion, but retinal tire soon comes on. The patient can read for a short 
time, but soon the print becomes blurred and she complains also of 
pain in the head and mental fatigue. Sometimes there is retinal hyper- 
esthesia, and the patient desires to be kept in a dark room, saying light 
causes intense pain. Reversal of the fields of vision is a symptom of 
hysteria, not of neurasthenia, but rapid contraction of the fields as the 
examination proceeds, caused by retinal exhaustion or possibly by 
inability to hold attention acutely enough to appreciate slight stimuli, 
is not uncommon. The auditory symptoms are somewhat similar. 
Either the patient appears to be somewhat deaf, which is really a matter 
of lack of attention, or she is extremely sensitive to sounds of all kinds. 
There are no visual, auditory, or other hallucinations. Ringing in the 
ears is not very infrequent, but the subjective sound is never interpreted 
as bells or voices. 
Diagnosis.-The diagnosis is largely by exclusion. It is necessary 
to exclude all possible gross organic disease. One of the diseases most 
often confused with neurasthenia and frequently associated with it is 633 
NEURASTHENIA 
hysteria. The error is of no great practical importance from a therapeutic 
point of view when it concerns physically weak hysterics, because the 
same methods of treatment are then useful in both diseases. As a matter 
of intellectual accuracy it is, however, well to keep them apart. Hysteria 
is as much abused a word as neurasthenia, and it would be well if it were 
used to designate only that group of symptoms including certain palsies, 
convulsive attacks, anesthesias, and respiratory and circulatory dis- 
turbance, with or without a certain type of purely mental symptoms. 
Convulsions, paralyses, and anesthesias are not symptoms of neurasthenia 
under any circumstances, and their presence indicates some other disease. 
The early stage of chronic insanity, especially paresis, may look very 
much like simple neurasthenia. By the time a paretic comes under the 
care of an alienist, or is so ill that the question of sending him to a hospital 
is being considered, the diagnosis is usually not difficult, but in the early, 
the prodromal, stage it may be impossible. In the cases of paresis in 
which there are physical signs of organic disease from the outset, inequal- 
ity of the pupils, abnormality in the iridic reflexes, scanning speech, 
spastic or absent knee-jerks, and intention tremor, the diagnosis is 
easy, because none of these occur in neurasthenia. Tremor is often 
seen, but it is an emotional tremor and not like that present in paresis. 
When the grandiose state is present the problem is easy, because no 
neurasthenic could be grandiose, but some paretics are depressed and 
even hypochondriacal. Moral degeneration, as shown by shamelessness 
or criminal acts, always excludes neurasthenia. The neurasthenic is often 
very selfish, but never morally degenerate. That is to say, the disease 
never causes moral degeneration. It may have existed before, and of 
course the neurasthenia will not cure it. In paresis there is a great 
lack of power of intellectual judgment even very early. In neurasthenia 
there is not so much lack of judgment as inability to form judgments 
continuously for any length of time. So far as the neurasthenic judges 
at all, she is prone to judge correctly. Notwithstanding all these points 
of differentiation, it may need several weeks' study before a correct 
opinion can be formed. When distinct delusions of any kind persist 
in a patient, he or she is insane and not merely neurasthenic. The 
best rule to follow is to regard every patient who shows pupillary and other 
reflex abnormalities as seriously ill. 
Alild hypochondria is sometimes called neurasthenia, but when there 
are delusions, when the patient believes he is suffering from some non- 
existent disease, he is mentally diseased, and needs treatment appropriate 
for that and not for neurasthenia. It is sometimes very difficult to 
differentiate the very early stage of adolescent insanity from neurasthenia. 
Indeed, the onset is often so insidious that it is impossible to foretell 
whether the patient is going to be seriously ill or not. Always, however, 
there is some distinct mental perversion even at the start. The patient 
is a little queer, his manner changes, his behavior alters, his ideas show 
perversion, and there occurs some manifestly insane act. When hallu- 
cinations and delusions appear, the diagnosis is certain. Cases of mild 
melancholia may seem to be neurasthenia at the outset, but the emotional 
depression is always greater than the physical weakness. There is always, 634 
DISEASES OF THE NERVOUS SYSTEM 
even in the mildest cases, a sense of personal unworthiness. So soon as 
delusions of self-accusation are established there is no doubt the patient 
is insane. Certain cases of Graves' disease may be mistaken for neuras- 
thenia. The classical case, with exophthalmos, goitre, and rapid heart, 
is easy enough to diagnose, no matter how many neurasthenic symptoms 
may be present, but cases occur in which the weakness seems to be 
before careful examination, the only symptom. 
One of the difficult problems in medicine is the diagnosis of neurasthenic 
headache. There is a tendency among some physicians to jump to the 
conclusion that if a woman-with men they are more careful-complains 
of constant persistent headache it is not so severe as she imagines and is 
caused by a slight neurasthenia or "a run-down system." This is rather 
dangerous, because, while head pain, or at least some strange feeling in 
the head, is common in neurasthenia, yet headache from other causes 
is much more frequent. Counting all kinds of headache, that from 
neurasthenia is not common. There is little in the headache itself 
which is characteristic except that it is apt to be described rather as a 
dull, queer, or strange feeling than as a sharp pain. It may be located 
in any part of the head, but most frequently in the occiput, the base of 
the brain the patient will say, and the nape. The next most frequent 
seat is the frontal region. When at all severe it is apt to greatly decrease 
ability to think and to bring on emotional depression, but these same 
effects follow headache from other causes. The eyes should always be 
examined, not only for refractive errors and muscular trouble, but also 
for optic neuritis and albuminuric retinitis. The writer has more than 
once seen a brain tumor in the early stage mistaken for neurasthenia. 
Typical migraine is easily diagnosed, but aberrant cases may require 
careful study. The nose should always be examined, for disease of 
it is a not very infrequent cause of headache, although not of paresthesia. 
Headache should never be regarded as neurasthenic unless all other 
causes are excluded and other symptoms of neurasthenia are present. 
It is possible at first sight to mistake myasthenia gravis pseudo- 
paralytica for neurasthenia, but in it the muscular weakness is at first, 
if not throughout the course of the disease, local. It begins with drooping 
of the eyelids, weakness of the muscles of mastication, and trouble in 
swallowing. The later picture is definite. 
Prognosis.-The severity of neurasthenia varies greatly, and so much 
depends upon whether the patient can afford proper treatment and is 
so situated as to regulate and control the manner of her life that it is 
impossible to make any general statements as to prognosis. Taking all 
neurasthenics together, the outlook as to both life and cure is good. 
Many men and women are mildly neurasthenic for many years, and yet 
not only live, but do their duty in the world and not infrequently a great 
work. Much of the world's work has been done by invalids, many of 
them congenital neurasthenics. Throwing out the mild cases and 
counting only the severe ones, the patients who become bedridden and 
need strict rest cure, the danger to life is not great, but the likelihood of 
recurrence is extreme. If the patient be so situated that she can control 
the manner of her living, it is often possible for her to lead a fairly happy NEURASTHENIA 
635 
and quite useful life by living according to rule, but should stress come to 
her, and sometimes even without stress, she may break again. Again, 
if the stress which caused the attack was very severe and the patient 
reacts well under treatment, the danger of recurrence is much less than 
if the stress was slight and the reaction slow. Thus, many busy, hard- 
working, and much worried men pass through a very severe attack, 
recovering completely and remaining well. The prognosis in secondary 
neurasthenia depends upon the nature of the primary disease. Cases 
following the acute infectious fevers, even though symptomatically 
severe, are usually entirely and permanently recovered from. 
Treatment.-The means used to combat neurasthenia must vary with 
the severity of the attack. Some cases are so slight as to require nothing 
more than a change of scene, outdoor play, and, if possible, removal of 
the exciting cause, while others are so severe that they test the skill of 
the wisest physicians, who sometimes fail utterly in bringing about a 
cure. For patients seriously ill the Weir Mitchell rest treatment is 
the best, indeed, the only, method, and it will be described in detail. 
Its important elements are absolute rest in bed, food, massage, isolation, 
baths, and electricity. The patient is best treated away from her own 
home, in order to get rid of all association with the worries of housekeeping 
and family life. A well-regulated rest house, where there are but few 
other patients and where all the servants and nurses are well trained, is 
the ideal place, but it is also the most expensive. A not too large special 
hospital is the next best place, and home, as a rule, the worst. For work- 
ing people, in the narrow meaning of the term, a bed in a ward of a 
general hospital is infinitely better than home. A very, small number 
of patients, those in whom, although the physical weakness is great, 
emotional control is good, can be successfully treated at home. A well- 
trained and sensible nurse, not a chatterbox or a scandal-monger, but a 
woman of brains, tact, and strength, is in any event a necessity, because 
amateur or family nursing of a neurasthenic always spells failure. She 
must perform not only the official duties of a nurse, but by her presence 
relieve the monotony of isolation, for to leave a seriously neurasthenic 
person entirely alone for hours at a time is disastrous. To have ever 
with her a nagging nurse, who in her wisdom thinks neurasthenia a 
polite name for laziness, is still more disastrous. A nurse who is not 
loyal to the physician in charge is, to use the language of one of the 
newer pseudosciences, a potent source of "malignant animal magnetism." 
Her psychological influence is evil. Even a good nurse and admirable 
woman may be ill adapted for a particular patient. If the patient takes 
a dislike to her, even though to do so be entirely irrational, her usefulness 
is done. Friction between patient and nurse makes cure more difficult. 
Patients who need treatment for a long time should, for several reasons, 
have a change of nurses after a few months. The nurse, no matter how 
good, is prone to lose interest after a time or become "stale" or even 
nervous herself, for the work is hard and the strain severe. She may get 
too well acquainted with the patient, and sometimes, but this is extremely 
rare, an unwholesome friendship arises. Having obtained the nurse and 
got the patient to the rest house or hospital, the treatment begins. At 636 
DISEASES OE THE NERVOUS SYSTEM 
first rest should be absolute. The patient should not leave the bed 
except to go to the toilet. Very rarely the muscular weakness is so great 
that the bed-pan must be used. The bed should be neither too soft nor 
board-like in hardness. Feather beds are the worst, woven wire beds 
with hair mattresses the best. The patient should be put on a couch 
for an hour each forenoon while the bed is aired and freshly made up. 
Food is an important element in the treatment. Such patients very 
soon after treatment is begun need large quantities of easily digested 
food, but at first it is best to use skimmed milk alone, giving the first 
day a few ounces, say three, every two hours from seven in the morning 
till nine at night. Next day the dose may be increased to four ounces, 
and soon the patient should be given two or two and a half quarts of 
milk daily. Many patients do not like milk, or believe that it makes 
them bilious, but a tablespoonful of limewater in each glass will often 
overcome this, or, if need be, it may be predigested. No positive rule 
can be laid down as to how long milk is to be the only food. Some 
patients thrive on it, and can be kept upon it for several months, but the 
majority need some other food after two or three weeks. It is then well 
slowly to enlarge the diet. The seven o'clock milk may be replaced by 
a glass of cocoa and at 8.30 she may have a breakfast consisting of a 
soft-boiled or poached egg, bread and butter, fruit, and milk, and after 
a few days dinner, in the middle of the day, never at night, may be 
added. It should consist of soup, some hot meat, vegetables, bread 
and butter, and some simple dessert. About a week later a light evening 
meal may be added. By this time the daily dietary consists of three 
meals, the mid-day one being quite large, and a glass of cocoa in the early 
morning and milk in the middle of the forenoon, middle of the afternoon, 
and at bedtime. The kind of bread used is important. Fresh bread 
and hot, moist rolls should never be taken by a neurasthenic. Bread 
a day old, toast, the crusty part of crisp rolls, or zwiebach are the best. 
Water is important and as milk is withdrawn should be supplied in its 
place. Red meat should not be eaten more than once a day. Eggs 
may be eaten raw, poached, or boiled, but not fried. In fact, all fried 
food should be prohibited. Coffee and tea should be prohibited in the 
earlier period of the treatment. Later, one cup of coffee with cream 
may be allowed at breakfast. Alcohol in all forms is harmful to all 
neurasthenics, and should not be given in any dose at any time. 
The next important element in treatment is massage. If a neurasthenic 
is put to bed and given no physical exercise, evil is bound to result. 
She will become constipated and digestion, even if fairly well performed 
before, will be disturbed. The great good of massage is that it gives the 
patient exercise, and the muscles work without any fatigue on the patient's 
part. Of course, the work done is nothing like so great as in active 
exercise, but there is no need that it should be so. Massage also does 
good in increasing the peripheral capillary circulation and possibly in 
actually squeezing poisonous products out of the muscles. The best time 
to give it varies in different patients, but the treatment should never 
begin or end within an hour of a meal. Some women who sleep badly 
are helped to sleep by it, and with them it should be given just before NEURASTHENIA 
637 
settling down for the night. For most patients about 3 p.m. is the best 
time. Whatever time is chosen, it should be the same hour every day, 
and not one day in the forenoon, the next in the afternoon, and the next 
at night. While the treatment is being given the patient should, during 
the first weeks, at any rate, remain quiet and not talk to or be talked to 
by the nurse. After the treatment is over she should remain quiet for 
one hour and not be disturbed for any reason. Some masseuses like to 
use cocoa oil or some other substance during massage. They are harm- 
less, but of little if any benefit. There are a few women whom rubbing 
makes intensely nervous. Some have an abhorrence of being handled 
by anyone, and some do not dislike the treatment but get cold hands 
and feet and bluish lips after it. Oftentimes after a few days and by 
beginning with only a perfunctory rubbing abhorrence can be overcome. 
Rarely the treatment must be stopped altogether. At the beginning 
the treatment should not last more than half an hour, later on forty-five 
minutes to an hour. At first it should be very gentle, later more severe. 
Toward the middle of the course resistive movements should be added, 
and when the patient is up several hours a day she should begin light 
gymnastics. It is better to have massage given by some one other than 
the nurse, because she has enough to do in other ways. 
Electricity is useful but not imperative. It, like massage, gives muscle 
exercise without work on the patient's part and has some good effect 
on the circulation. It is a mild stimulant to some patients, but this 
effect is largely, if not altogether, psychical. A few patients have such 
a nervous fear of it that it cannot be used to advantage. It should be 
given several hours after or before massage. A good way is to give it 
at eleven in the morning and massage in the afternoon. An ordinary 
nurse's faradic .battery is all that is required. The two electrodes should 
be freshly covered with absorbent cotton for each treatment, wet with 
warm water, placed only a few inches apart over the bodies of the muscles 
of the arms and legs, and, the slowly interrupted current being turned 
on, each muscle be made to contract three or four times. The electrodes 
should not be placed on the tendons or the bony prominences. Care 
must be taken not to use a current so strong as to cause pain. The entire 
body should be gone over in about forty-five minutes. Sometimes the 
patient is made to feel much better by ending the treatment by using 
the rapidly interrupted current to the neck and heels. A small electrode 
is put on the nape of the neck and an electrode large enough to cover both 
feet placed on the soles. The rapid current is then turned on for ten 
minutes. 
Isolation is imperative in all severe cases, but not necessary in the 
milder ones. Mental rest is needed quite as much as physical, and a 
neurasthenic is injured by the anxious faces of near relations and the 
useless sympathetic talk of friends who too often discuss her troubles 
with her. The kind friend who tells her how well she is looking and that 
she will recover right away if she will only make an effort is a very dis- 
turbing factor. The ills of life and petty annoyances which to her are 
great burdens are brought constantly before her by family talks. Some- 
times more serious troubles in the home life right themselves after a time 638 
DISEASES OF THE NERVOUS SYSTEM 
by a judicious absence of talk and argument about them. Sometimes 
more wholesome marital relations, which have been lost, may be regained 
by the temporary separation of husband and wife. Sometimes people 
who care a great deal for each other react very badly on each other 
when one or the other is sick. For a shorter or longer time, then, it is 
best for the patient to see only the nurse, the doctor, and the masseuse. 
Notwithstanding the isolation, there is no need for the patient, as a 
rule, to lead a silent and entirely passive life. Soon the nurse should 
read to her for a little while each day. Later they may play games, 
and when she begins to get up she may read, Write letters, see her friends, 
sew, embroider, make baskets, and so gradually resume her life. 
At first she should have a warm sponge bath every day, preferably 
in the morning after the cocoa and before breakfast. Each extremity 
and the trunk should be bathed separately. Later, she can be given a 
warm and still later a cool plunge bath daily, and later she may bathe 
herself. In women the hair should be shampooed twice monthly. This is 
a little thing, but it gives comfort, and comfort hastens cure. All possible 
causes of so-called reflex irritation should be removed. Ocular troubles 
especially should be remedied. Local treatment of the genitalia, unless 
there be real disease present causing local symptoms, usually does more 
harm than good. Vaginal examination in young, unmarried women in 
the hope of finding some hypothetical cause for the disease is never 
justifiable. Such an examination is a great shock, and should never be 
made unless local symptoms imperatively demand it, and then it should 
be done under an anesthetic. 
Drugs play little part in the rest treatment. Sometimes insomnia is 
so great as to require an hypnotic. Veronal is probably the best, although 
sometimes trional or sulphonal does better. Hyoscine in doses of 
gr. at night is sometimes useful, but, with the frequent perversity of 
active and powerful drugs, it occasionally excites instead of quieting the 
patient. A warm pack is often of service. Opium and its derivatives 
are always to be avoided. The old compound sumbul pill three times 
daily often quiets the nervous unrest. When there is much gastric 
atony ascending doses of nux vomica, beginning with 10 drops before 
meals, are useful. When intestinal indigestion and catarrh are present 
a pill of nitrate of silver, gr. T^, immediately after meals is of great use. 
Constipation is often overcome by abdominal massage. When it fails 
the fluid extract of cascara in 15 minim (1 cc.) doses three times a day, 
or a single larger dose at night, is useful. Phosphate of soda before 
breakfast or a corresponding dose of the effervescent salt sometimes 
acts better. The combination of senna leaves stewed with prunes is 
homely but useful. Enemata are sometimes required. Many distressing 
abdominal symptoms are relieved by a binder. 
Let me say one word more about the remedying of peripheral irritation 
in neurasthenia, and first as to the pelvic organs. Any serious disease 
should be treated in the same way as if the patient were not a neurasthenic. 
Surgical treatment of an infantile uterus will do no good. It is not a 
cause of the neurasthenia, but simply one of the signs of an ill-developed 
organism. Tears of the perineum and cervix should be repaired if they NEURASTHENIA 
639 
cause local symptoms, but no immediate amelioration of symptoms is 
to be expected. Even the wildest doctrinaires have ceased to remove 
normal ovaries. If the ovaries are seriously diseased and in themselves 
need surgical treatment, they must be removed, but such removal 
usually has but little effect on the neurasthenia and sometimes is followed 
by very serious nervous symptoms lasting months or even years. This, 
or course, refers to operations before the menopause. After the pelvic 
organs the eyes are the organs most treated in neurasthenia. Any disease 
of them should be treated. It is undoubtedly true that eye-strain in 
persons prone to neurasthenia may cause many annoying and even dis- 
tressing symptoms which can be remedied by proper treatment. Whether 
a floating kidney should be operated upon depends upon the circumstances 
and the symptoms in each particular case. 
How long should a rest treatment last? No definite time can be set, 
for it depends upon the progress. There certainly should be some 
improvement in six weeks. The good signs are increase in weight or, 
if the patient has been fat always, a hardening of the fat and firmness 
of the muscles, and improvement in the color of the skin and mucous 
membranes. The pasty, oily, dry skin becomes clear, a little pink, and 
the oiliness passes away. The tongue becomes clean and the breath 
ceases to be foul. The appetite improves, and, the strength returning, 
the patient wants to get out of bed and do things. When the patient 
begins to get out of bed it should at first be only for a half-hour, then an 
hour, and every few days thereafter a longer time. She should gradually 
do more and more for herself and should be interested in something 
outside herself and outside the serious affairs of life. She should also begin 
to go out of doors, and finally take up her duties again. 
There is one type of woman whom a rest cure ruins. Through very 
wilfulness and indolence such women want to stay indefinitely in bed, 
not because they are sick, they are not, but because they enjoy it. They 
are lazy and selfish, and love the luxury of having doctors and nurses. 
They enjoy the importance of being ill, and are too empty-headed to 
want to do anything in life except exist. They must be forced to get up- 
they never should have been put to bed-and slowly resume ordinary 
life. It is as hard, as Weir Mitchell said, to get some patients out of 
bed as it is to make others remain there. Each needs what the other 
wishes. 
Recently there has arisen, or, rather, is now arising, an opinion among 
physicians that rest treatment is not as useful as many of us believe. 
The reason for this is not hard to discover. After it became popular 
it began to be used in many entirely unsuitable cases, and often was not 
carried out in proper detail. Sometimes the physician forgot that there 
is necessarily a psychic element in all treatment, and thought that all 
his duty was done when he mechanically set the machinery of treatment 
in order and then ceased to exercise any personal influence in the matter. 
Really the personality of the physician is a very important element in 
success. He needs to be a wise man, to know much, and to have great 
sympathy with the frailties of human nature. He must know when 
to be stern and when to be sympathetic, and, above all, he must never 640 
DISEASES OF THE NERVOUS SYSTEM 
lose or, at any rate, show that he has lost interest in the case. It is 
sometimes dangerous to show too much interest in the patient. Another 
reason is that in therapeutics, as in everything else, the pendulum of 
opinion is apt to swing first to one extreme and then to another, never 
reaching a place of stable equilibrium. Hence today there is coming 
into vogue a work cure for neurasthenia. It has a place and a large 
place in the treatment of nervous people. It has long been used by 
many men as the last stage of the rest treatment (Weir Mitchell so used 
it for years), and with many patients is a most excellent thing. Its 
fundamental idea is that much nervous disturbance depends upon the 
loss of interest in life and that work outside the things the patient has 
been in the habit of doing and along new lines will resurrect that interest 
and produce a saner, healthier view. All this is undoubtedly true. 
The question at the outset, then, is, Does the patient need rest or work? 
The answer depends largely upon the physical strength or weakness of 
the patient and upon the character of the symptoms. When there is 
great neuromuscular weakness, when all physical effort causes rapid and 
great fatigue, when there is emaciation and serious digestive disturbance 
and any mental effort causes distress, the first, the imperative need is 
bed rest. On the other hand, when without much or any true physical 
weakness there is mere loss of the joy of living, mere boredom of life, 
with some, even great, nervous irritability and a little mental queerness, 
some doubt as to the affection of near relations, not very severe mental 
tire, and some but not severe gastric and intestinal disturbance, then work 
in the therapeutic sense is much more needed than rest. A total change 
in environment and the development of new interests are very necessary. 
Cases a little more severe need increased rest but not rest treatment. 
On the contrary, one hour of rest each afternoon is often quite sufficient. 
The important thing is to re-arouse interest in life, and this can best be 
done by inducing the patient to take up some new line of work, giving 
her at the same time much outdoor exercise and compelling her to take 
enough food at regular times. Finally, there is a large class of neu- 
rasthenic patients who are not severely ill, but yet suffer a great deal and 
need only modified rest treatment. Removal from home is almost always 
necessary for cure. No absolute rules can be laid down for the conduct 
of every patient. The matter must be left to the wisdom and discretion 
of the attending physician. 
In the last few years a great deal has been written concerning the 
so-called psychic treatment of functional nervous diseases, and claims 
have been made that neurasthenics can be cured by such means. Indeed, 
the word psychasthenia is coming more and more in use to replace neu- 
rasthenia. Now, if neurasthenia be made to include all kinds of nervous 
symptoms and several types of mental aberration, if it means palsy of 
the will, and laziness and general nervousness, then certain cases of it can 
be cured by purely mental treatment. But the writer has not been dis- 
cussing that sort of thing, but a serious and well-defined disease, the 
causes of which are deeply rooted in the patient. What part, then, can 
so-called psychotherapy play in its cure? Both much and little. No 
physician can help a neurasthenic if the patient doubts his skill or thinks NEURASTHENIA 
641 
he is treating her in a merely mechanical way without human interest. 
He must have her confidence to get good results. She must believe he 
can cure her or, at any rate, help her. This is, of course, purely a psychical 
matter dependent upon his personality. But when we come to the 
technical side of psychotherapy the question is different. For example, 
the use of hypnotism, as a therapeutic means is in the writer's mature and 
deliberate opinion productive of more harm than good, not only in 
neurasthenia, but in almost all diseases. It is a great shock to a woman 
to learn that anyone can make her unconscious and, so she believes 
and has learned by reading popular books and articles, subservient to 
another's will. If often repeated it certainly tends to weaken will power. 
Quite recently suggestion under hypnotism has been replaced by sugges- 
tion without hypnotism. This is really not such a mysterious process 
as some of the papers written about it would lead one to suppose. It 
really is nothing more than the acceptance on the patient's part of the 
truthfulness and accuracy of what the physician says and willingness to 
be led by him. Susceptibility to it indicates only ingenuousness and 
child-likeness of mind. One form of psychotherapy consists in an appeal 
to the religious sense of the patient. No harm and much good may and 
does come from this when properly and conservatively used, but unwisely 
and emotionally employed, and to the exclusion of proper medical 
treatment, it must do harm. It sometimes leads to very serious results 
because there are certain people who need not stimulation of the religious 
sense, but sedation. Dabbling in psychology, the occult, and the mys- 
terious has injured a great many people and made not a few insane. 
It is wiser and safer for physician and priest each to keep within his own 
sphere. Somewhat allied to religious psychotherapy, but advised by 
men of a very different kind, is the treatment founded on the theory that 
neurasthenia and hysteria are based on the sexual function, and that if a 
patient be induced to make a confession of errors and sins in the long ago, 
she will be helped by the confession and speedily recover. 
Neurasthenia in youths resulting from sexual abuse is not helped 
by the rest cure. As a rule, they are injured by it. Even though they 
seem to be physically very weak, it is far better to compel them to take 
outdoor exercise, cold baths, the simplest of food and not too much 
of it, than to put them to bed, stuff them, and give them time to day- 
dream about sexual pleasures. Often the hard life of a ranch among men 
who will not sympathize too much with them is the best means to a cure. 
They always need encouragement, and a quiet sensible talk does them a 
great deal of good. 
A great deal of neurasthenia would be prevented if people were taught 
how to live properly. The robust and healthy, hard fibred, and with 
good ancestry, can get through this world wdth little care and less training, 
but those who come into the world handicapped need much of both. 
Although we suffer from the despotism of our ancestors, we can to a degree 
avoid their rule if we learn our own weakness and what we can do and 
what to avoid. So far environment may overcome heredity. The weak- 
fibred boy or girl can often escape neurasthenia if he or she be well 
environed and taught self-control and sensible habits of living; how much 
vol. v-41 642 
DISEASES OF THE NERVOUS SYSTEM 
to work, how much to play, what responsibilities to assume and what 
to decline. Further, good training may strengthen a weakling so that 
he can do more and better work and stand more and more stress. 
THE TRAUMATIC NEUROSES AND PSYCHOSES. 
The use of steam and more recently of electricity in industrial life has 
created a new cause of disease, or, rather, has made an old cause, acci- 
dental injury, much more frequent. Traumatic neuroses and psychoses 
are not new diseases; it is probable that some of the slaves suffered from 
them at the building of the pyramids, but the tremendous development 
of machinery has increased the frequency of accidents and the horrors 
accompanying them. The rapid growth of the very modern belief in 
the responsibility of employers and the increase in the legal duties of 
common carriers toward their passengers and the general public have 
drawn great attention to these diseases and led to their close study. 
We shall not consider the effects of severe direct trauma to the brain, 
spinal cord, and nerves, but only the nervous and mental conditions 
resulting from accidents in which, although there may or may not have 
been injury to some other part of the body, the nervous system has 
escaped gross trauma. We shall exclude discussion of injury and fright 
as exciting causes of paralysis agitans, exophthalmic goitre, and Syden- 
ham's chorea, and consider only traumatic neurasthenia, hysteria, and 
insanity not ascribable to serious local cerebral injury. We shall include 
the late results of concussion of the brain and strain of the lumbar muscles. 
Any discussion of treatment is unnecessary, because it is the same whether 
the illness be caused by trauma or not. 
A great deal has been written and several theories propounded to explain 
the mechanism of the traumatic neuroses and psychoses. It is pretty 
generally accepted that they are in some way connected with disturbances 
in the central nervous system. Some metaphysical authors have assumed 
that mind exists independently of the brain, but works in association 
with it, the doctrine of parallelism, and attribute the nervous and mental 
disorders to psychic, as distinguished from cerebral, shock. Other 
writers hold that mind is merely a function of the brain, but that the 
brain is affected purely in its function without organic change, and still 
others believe that there are organic changes present but so minute 
that we cannot see them even under the microscope. But, although 
much has been written, we do not yet know what happens to and in the 
brain to produce traumatic neuroses and psychoses; we know nothing 
of their morbid anatomy. It may be assumed that whatever lesion does 
exist is in the brain. There are probably other, secondary, lesions in the 
abdominal viscera in neurasthenia, but they are hypothetical. 
The possibility of fraud on the part of the person alleged to be ill makes 
the study of the traumatic neuroses and psychoses more difficult than 
that of most diseases. In ordinary practice the question of malingering 
is rarely important; in illness following an accident it is always to be 
thought of. Another difficulty is due to the fact that some writers are, 
unconsciously of course, biased in their opinions, because most of their THE TRAUMATIC NEUROSES AND PSYCHOSES 
643 
work consists in examining claimants for defendant corporations or the 
clients of lawyers who have claims. Sometimes the bias is not altogether 
unconscious, and, indeed, might be called by another name. Apart 
from downright fraud, there may be exaggeration of really existing 
symptoms, and a pessimistic physician may scare the patient to his 
increased hurt. Indeed, it has been claimed that there are no traumatic 
neuroses or psychoses, but that the conditions so named are the direct and 
sole result of suggestion on the part of the physician treating or examining 
the patient. It is not, how'ever, the prevalent opinion, and is not likely 
to become such. A last difficulty is the fact that there is no complex 
of symptoms pathognomonic of trauma as a cause. There is no clinical 
picture from which one can conclude, knowing nothing of the history of 
the case and judging only from the physical examination and the patient's 
account of his symptoms at the time of the examination, that the illness 
is caused, and only can be caused, by trauma. In every case other causes 
might have produced the same effect. We can only say that the shock 
of an accident can produce this condition and cannot produce that. 
The accidents most liable to be followed by serious results are those in 
which the accompanying circumstances are horrifying and those which 
occur suddenly, unexpectedly, and without any warning. Accidents 
which cause very severe physical results very often are followed by less 
severe results than others in which the physical injury is slight, and those 
occurring during sleep, and especially during drunkenness, are the least 
likely to be followed by serious so-called functional nervous troubles. 
Traumatic neurasthenia is probably the most frequent of all the nervous 
conditions which follow accidents. It may be mild and transitory or 
severe and permanent. Its symptomatology differs in no way from that 
of neurasthenia from any other exciting cause. A frequent history is 
about as follows: The patient has fallen from a car which started as she 
got off or on, or has been knocked about in a collision, or thrown to the 
ground from a wagon or cart. She is dazed for a moment, or even made 
unconscious for a short time. There are also some bruises, or, it may be, 
a broken rib, a cut scalp, or a broken arm. She goes, or is taken, home 
and finds herself much shaken up, but with no fear of any serious results. 
The physical injuries heal well, but she finds that there is something wrong 
with her. She sleeps badly, her appetite is capricious, and digestion 
poor. Attacks of cardiac palpitation occur and the peripheral circulation 
is sluggish. She may at times faint, or fear she is going to do so. She 
is downhearted, cries easily, is peevish and fretful. She was a useful 
and sensible housewife; now she is fit for nothing. She tires very easily, 
and has lost interest in everything. She may blame herself for this 
loss of interest and struggle against it, or she may become wholly selfish 
and demand to be treated better by her family. She gets the feeling 
of being a martyr and ill used. Power of mental concentration goes. 
Headache comes on, especially the curious occipital ache which patients 
often describe as a feeling of unbearable strain rather than as pain. 
She becomes physically weak, and can scarcely drag herself around, and 
finally, it may be, is bedridden. The onset, instead of being slow, may 
be ve y acute and date from immediately after the accident. If she is a 644 
DISEASES OF THE NERVOUS SYSTEM 
working woman or a workingman's wife, her illness often leads to great 
financial difficulties in the family, and brooding over this and comparing 
it with former times increase her illness. If the case is of a kind that 
leads to a lawsuit, the expectation of appearing in court, the interviews 
with lawyers, the examination by experts all add to the illness. Many 
patients are improved or, indeed, cured soon after a verdict, no matter 
what it may be, not because the patients were malingerers, but because 
the horror of the trial is over. Some cases are made more severe by the 
long delays which occur before legal settlement is reached, and sometimes 
among the poor, in cases in which there is no doubt of the genuineness 
of the symptoms, proper treatment cannot be carried out because of the 
lack of money. Some patients present great physical weakness and 
rapid mental fatigue with little or no emotional unbalance. 
Some cases which are commonly classified as traumatic neurasthenia 
ought rather to be designated by the term general nervousness. The 
patients suffer from general nervous unrest rather than real weakness. 
They often describe their condition by saying they "feel in a hurry all 
over." They fidget and fret and cannot get anything done because they 
do not know where to begin. The condition is, of course, not serious, 
but it is annoying and may last a long time. 
Traumatic lumbago, which logically should not be studied here because 
it is caused by direct muscular strain, and hence is neither a neurosis 
nor psychosis, may be, because it so frequently accompanies and, indeed, 
aggravates neurasthenia and hysteria. It really is the result of strain 
of the muscles and ligaments of the back caused by violent and involun- 
tary muscular effort made to save one's self in falling or being thrown. 
Force directly applied, as by a blow or by striking an object, is a less 
important causal factor. The pain of traumatic lumbago must not be 
confounded with the painful points in the spine which occur in hysteria. 
The lumbago involves more or less of the whole mass of lumbar muscles 
on one or both sides, and is shown objectively by rigidity when the patient 
twists upon the spine, or bends to either side or backward. In a severe 
case the attitude is characteristic. The spine is held stiff and straight, 
or bent forward from the pelvis. The lumbar muscles are in more or 
less rigid spasm. The patient moves slowly, not only in walking, but in 
using the hands or moving the head. On rising from a chair he supports 
the back by placing the hand on the thigh. The gait is slow and hesitat- 
ing, the feet being dragged on the ground or barely raised from it. In 
very severe cases the pain is so great that the patient cannot walk, and 
this may lead to a suspicion of palsy from spinal cord disease. But there 
is no true palsy; the bladder and rectum are under control; there is no 
anesthesia; indeed, there are no signs of cord disease. 
Hysteria.-Any one of the possible combinations of hysterical symp- 
toms seen in non-traumatic cases may follow and be caused by trauma. 
No one combination proves a traumatic origin. It must never be for- 
gotten that the presence of hysterical symptoms does not disprove the 
existence of organic cerebral or spinal disease. This association of 
hysteria and organic disease, whether it is a mere coincidence or whether, 
as is thought by some, a gross lesion may be an exciting cause of hysteria, THE TRAUMATIC NEUROSES AND PSYCHOSES 
645 
is seen apart from trauma. Thus, often in brain tumor, in women 
especially, in the early stages, hysterical symptoms, or symptoms taken 
to be hysterical, are so pronounced that the organic disease is entirely 
overlooked. It is especially important, for therapeutic and prognostic 
reasons, to carefully exclude organic disease before the diagnosis of 
hysteria is made. To diagnose cerebellar disease as hysterical astasia- 
abasia might lead to serious though different evils to patient and doctor. 
The most frequent type of serious hysteria following an accident is 
that of palsy with anesthesia, but any type may develop. The palsy 
may be monoplegic, hemiplegic, or paraplegic. Sometimes the symptoms 
remain unchanged throughout the course of the illness, sometimes new 
ones are added, until finally the patient presents or has presented all or 
almost all the symptoms of the disease. A hysterical fit or series of fits 
may begin the illness or appear during its course. Sometimes the patient 
complains of only one symptom, but in such a case great care must be 
used in diagnosis, because monosymptomatic hysteria is very rare. Thus, 
the tremor of fright may become permanent. Tremors of this kind are 
sometimes followed by, or are the first symptom of, paralysis agitans. 
Among the rarer symptoms are the hysterical breast, bleeding breast, and 
true hysterical insanity. More or less neurasthenia is present in a great 
many hysterical patients, although many neurasthenics are not hysterical. 
A common history is as follows: After an accident, in which the 
patient has been shocked, and sometimes quite a little time after, the 
patient finds trouble in walking or using the hands. There is associated 
with this headache, especially in the occiput, backache, points of tender- 
ness along the spine, and a general feeling of weakness. The motor 
disability may go on to complete inability to use the affected parts. 
The palsy, instead of slowly increasing, may come on suddenly imme- 
diately or quite a little time after the accident. On examination the 
ordinary signs of organic palsy are not found. There is no true ankle 
clonus, or spasticity of the knee-jerk, but there is frequently anesthesia, 
it may be to slight touch only, or all sensibility may be absent. The 
anesthesia may involve one entire side, the palsied one if there is hemi- 
plegia, and stop abruptly at the middle line, or both legs, or be in scattered 
spots of more or less regular geometrical shape, but having no relation 
to the distribution of the sensory nerves, or involve the hand or foot 
alone, having a clear-cut boundary anywhere above the wrist or 
ankle. 
Sometimes single symptoms are complained of or are so predominant 
that the patient pays little attention to others which medically may 
be more important. For example, a woman was thrown to the floor of a 
car during the excitement following the blowing out of a fuse. She was 
taken to a hospital, where she was found to be dazed and confused, but 
not unconscious. She was bruised on the head and arms. After a few 
hours she went home by herself and went to bed. When seen by the 
writer, some months later, she complained only of loss of the sense of 
taste and smell, which she dated as arising after the accident, but could 
not remember how long after. Careful examination revealed no organic 
cause for the loss, and the general examination of the nervous system 646 
DISEASES OF THE NERVOUS SYSTEM 
brought to light no other signs of disease. More frequently anosmia and 
ageusia are merely part of a complex symptomatology. 
A bleeding breast in a man who had had a penetrating wound of the 
chest seemed to have been caused by some slight local trouble in the 
nipple rather than by hysteria. When examined he complained of pain 
at the seat of the old wound, saying that the pain caused by bending 
forward prevented him doing manual work. Many months after the 
accident he suddenly became numb in the right arm and leg. During 
the examination, on milking the left breast, or rather pinching the nipple, 
about a quarter of a dram of blood spurted out. He had no other sign 
of anything more than general nervousness. A jury gave him $5000 
damages, but were said to be more influenced by the fact that he had 
actually sustained a penetrating wound of the chest than by his nervous 
complaints. Vomiting of blood, especially immediately after the acci- 
dent, is much more frequent than bleeding from the breast. It may 
recur at intervals for some time, and is probably caused by rupture of 
some of the small veins of the oesophagus. Malingerers sometimes suck 
blood from cut gums to simulate this symptom. 
Mental Disturbances.-Several types of mental trouble may follow 
accident. In fact, in a sense, there are always some mental symptoms 
associated with the nervous, because, even in neurasthenia, there is 
always rapid mental fatigue, an inability to do any continuous mental 
work, and oftentimes some, and sometimes very marked, decreased 
ability to form correct mental judgments, with disability or inability 
to decide any question. As to hysteria, it is a mental affection having 
physical symptoms, although it is not a true insanity. Under this 
heading, therefore, are considered the purely mental symptoms that may 
follow accident. The most frequent, probably, is mere mental dulness, 
resembling congenital stupidity. It may occur alone or be accompanied 
by the physical symptoms of neurasthenia. Alone it constitutes a true 
psychasthenia. There may be all degrees of severity. The man or 
woman who, before, was mentally quick, alert, and accurate in thought 
is now slow, dull, and inaccurate. The attention cannot be fixed for any 
length of time. There is marked mental inertia. The man who read 
much and high class literature now does not read at all or very little. 
The clerk who could rapidly add up a column of figures, calculate percent- 
age quickly, almost automatically, now can scarcely do any arithmetic, 
not because he cannot do it if he tries hard enough, but because he cannot 
try hard enough for more than a few minutes. All grades can occur, 
from the slightest to the profound dementia in which mental power is 
lost entirely, for the time being, at least, and the patient must be cared 
for. The extreme cases are very rare, unless there has been some gross 
organic lesion resulting from direct and serious injury to the brain or 
skull, which, of course, puts them out of the class of psychoses. The 
milder cases are very common. Frequently it is not so much the direct 
result of the accident as a secondary consequence dependent upon the 
worry and grief resulting from inability to work. It may be unaccom- 
panied by true physical weakness, although the mental inertia may 
render it impossible for the patient to make muscular effort for more than THE TRAUMATIC NEUROSES AND PSYCHOSES 
647 
a few minutes. There is sometimes added to the mental dulness emo- 
tional depression, which may be slight or severe. 
Let us now take up the insanities in the more limited and restricted 
sense of the word. What types of insanity can and what cannot be 
caused by the shock of accident? Of course, the accident is merely 
the exciting cause; the predisposing cause is in the man, else everyone 
would become insane after an accident that would make anyone insane. 
The predisposing causes are the soil, the exciting causes the seed which 
grows in it. One of the most frequent questions which comes up is whether 
a given case of paresis has resulted from an accident. Now, of course, 
the classical cause of paresis is the combination of alcohol, syphilis, and 
strain and stress of life. There is no doubt that, given these as already 
existing, and given a concussion of the brain or a fracture of the skull, 
the brain injury may precipitate the occurrence of symptoms which did 
not exist before and would never have come into being had not the 
patient been subjected to some unusual stress. The question is rather 
different in those cases in which no gross injury to the skull or brain 
has occurred, but it appears that a severe shock to the nervous system 
can light up the disease. A patient with an already existing paresis, 
but in the early stage, may surely be made worse by an accident. In the 
later stages no harm is done because the mental powers are already too 
low to be influenced by shock. It is very important to determine whether 
the patient had the disease established before the accident. When a 
period of many months of good health has elapsed between the accident 
and the very beginning of the symptoms of paresis, and there has been 
no gross injury to the brain, the probabilities are that the two have no 
correlation. 
When elderly people have sustained an accident the claim is often made 
that it has resulted in senile dementia. As a rule, elderly people do not 
stand sudden physical shock well, and frequently there is permanent 
loss of mental alertness in consequence of it. There is no doubt that 
occasionally it is the exciting cause of a true senile dementia. The 
cerebral arteries, although thickened, may be healthy enough to nourish 
the brain under ordinary circumstances, and the kidneys, although 
sclerosed, may also excrete fairly well and yet both fail, never to recover, 
under the stress of shock. There may be serious difficulty, however, 
in determining the causal relation of the accident. It is frequently not 
easy to discover what was the mental state of the patient before. 
Hysterical insane states may be caused by accidents, as they may be 
caused by shock of any kind. The melancholic condition spoken of 
above may, in a susceptible person, pass into a true melancholia. States 
of acute confusional insanity and dementia prsecox also occur. Drunk- 
ards bear shock worse than any other class of people, and may have an 
acutely oncoming insanity in consequence. So much for the positive 
side. Now as to the negative. True congenital paranoia is never 
caused by accident. It is a purely developmental disease and needs no 
external exciting agent for its production. It is doubtful also if true 
acute mania is ever so produced. The cases of epileptic insanity usually 
follow some direct injury to the brain, although the convulsions may not 648 
DISEASES OF THE NERVOUS SYSTEM 
occur for a long time after the accident, but there are usually some 
physical signs of the injury even at the beginning. This is not always 
the case, or rather at the beginning the physical signs may be slight. 
Thus, a man in a railroad car was struck on the side of the head with 
the metal end of a bell rope, which had snapped off when the car parted 
from the rest of the train. He was knocked down by the force of the blow 
and was senseless a few minutes. He recovered consciousness quickly 
and complained only of the pain in the bruise at the spot where he had 
been hit. The bruise quickly healed, but he continued to have pain 
in that region. Some months later he began to have general, not local, 
epileptiform convulsions. They were alleged by the railroad company 
to be hysterical, but his family physician claimed they were organic, 
and he was trephined at the seat of the injury and the inner table of the 
skull was found fractured, a spicule of bone pressing against the dura. 
There was a marked adhesive inflammation of the dura in the neighbor- 
hood. Whenever epileptiform, not hysterical, fits follow an accident it is 
always well to hunt for a local cerebral trauma. 
Diagnosis.-The diagnosis of accident as a cause of nervous and 
mental disease is difficult, because in so many cases the question arises, 
Is the patient a malingerer? There is no short and easy way to a deter- 
mination of the question. Mental alertness, knowledge, and skill on the 
part of the examiner, and care in investigation will always uncover 
fraud. Bias on the examiner's part may lead to great injustice, and this 
is a very important matter. The man who believes that no attention 
should be paid to the mere complaints of the claimant, the merely sub- 
jective symptoms, and that a diagnosis should be based solely on the 
objective signs, will often reach a different conclusion from him who 
thinks the mere statement of the claimant should be accepted at its 
face value. The middle path is always the safest. Undoubtedly there 
are many fraudulent claims, and just as undoubtedly perfectly legitimate 
claims are sometimes opposed in ways that are not fair. Some defendants 
are as tricky as some claimants. 
Before a correct diagnosis can be made all the important facts in the 
case must be known. Especially important are the state of health of 
the claimant just before the accident and the nature of the accident. 
When a merely trifling accident has occurred, unaccompanied by any 
cause for shock, it is doubtful if the accident is the cause of the illness. 
For example, a man, about thirty-three years of age, had undoubted 
hysteria, which he claimed was due to striking his head against the roof 
of a buss which gave a sudden jolt, because of an inequality in the road. 
The man made no complaint at the time, continued his journey, attended 
to his affairs for about a month, and then had a hysterical convulsion 
followed by persistent hysterical symptoms. It was found that he never 
had had any permanent occupation, never had kept at any work in any 
one place for more than a few weeks, had caused his family, who were in 
good circumstances, much trouble by his laziness and shiftlessness, and 
had always excused himself on the plea of illness, making hypochondriacal 
complaints all the time, while, as a matter of fact, he was well nourished 
and strong. The trifling nature of the accident, together with his tern- THE TRAUMATIC NEUROSES AND PSYCHOSES 
649 
perament and character, made one disbelieve that the accident and the 
hysteria had anything to do with each other. 
The next question is to determine the presence or absence of other 
causes, for example, preexisting physical disease. Thus, a man com- 
plained that a few days after being violently thrown from a trolley car 
and sustaining many bruises his left knee suddenly gave way under him 
and he could not walk. He claimed, and no doubt honestly believed, 
that it was caused by the fall. As a matter of fact, he had locomotor 
ataxia, and the knee trouble was due to a Charcot joint. Although he 
had very slight ataxia of motion and station, the knee-jerks were absent, 
Argyll-Robertson pupil was present, and he said he had had slight pains 
in his legs for several years. Of course, the degeneration of the spinal 
cord necessary to produce all these symptoms could not have taken 
place in a week, no matter how severe the injury and shock of the accident 
might have been. The question sometimes arises in the case of men, 
whether their illness is not really caused by alcohol or syphilis, but 
both conditions usually present quite definite objective signs. 
flow long a time may elapse between the accident and the illness and 
yet the illness be the result of the accident? No time can be arbitrarily 
set down. Many persons who at first suffer little, later develop very 
serious symptoms, but the time between the onset of symptoms and the 
accident is never very long. This, of course, applies only to the neuroses 
and psychoses and not to the late effects of serious physical injury, such 
as concussion or fracture of the skull. In both these conditions there 
may be apparent recovery, and a long time after symptoms may arise 
as a direct consequence of a disease process in, say, the meninges, the 
direct result of the primary trauma. External conditions may bring 
on severe and sudden symptoms in these cases of old and quiescent injury. 
For example, a severe concussion may make it impossible for a man to do 
work in the heat, although in a cool place he may work very well. Trau- 
matic epilepsy may begin years after the injury to the skull. 
Certain diseases may come on suddenly at the time of the accident, 
and yet not be caused by it. For example, an old man fell as he was 
getting off a car. The evidence was that it was motionless, but nobody 
could tell whether he had stumbled or not. When he was picked up he 
was unconscious. When consciousness returned he was paralyzed on 
the right side and aphasic. He was old, his arteries very hard, his urine 
contained tube casts and a trace of albumin, and was of low specific 
gravity. He had no local signs of fracture of the skull, and the writer's 
opinion was that he most probably fell as a result of an apoplexy. In 
cases similar to the above, in which death occurs and an autopsy is held, 
if there is no fracture of the skull and there is a hemorrhage from the 
striate artery, it is very strong evidence that the patient fell in an apo- 
plexy. On the other hand, the shock of an accident may, itself, produce 
an apoplexy. 
The differential diagnosis between senility and the effects of trauma 
is often difficult. Shock may produce a premature senility, and, given 
a man who presents the signs and symptoms of senility, the diagnosis 
must depend upon the history of his condition preceding the accident. 650 
DISEASES OF THE NERVOUS SYSTEM 
If it be shown that, notwithstanding the existence of arteriosclerosis, 
the man was in good condition, and that dating from the accident mental 
and physical decay began, it is reasonable to suppose that the injury was 
the exciting cause. 
Can an accident without severe physical injury to the head affect the 
mental development of a child? Now and again it is claimed that such an 
accident has caused imbecility. A nervous child is often dull and stupid 
and emotionally apathetic or unbalanced for some time after an accident 
in which there has been a large element of fright, but in the great majority 
of cases there is no permanent ill effect, the patient regaining emotional 
and mental equilibrium after a variable period. Permanent mental 
impairment may result in already pathologically high-strung children, 
but is very rare. When there has been serious concussion of the brain 
very severe and permanent effects may follow, but even in cases of con- 
cussion the natural resiliency of youth tends to bring about complete 
recovery. Slight concussion surely causes no permanent ill effects in 
healthy children. 
Assuming that the patient is ill, and ill because of an accident, the 
next question is the exact nature of the illness. Is it a pure neurasthenia? 
If so, the patient will present more or fewer of the symptoms described of 
that disease. For our present purpose the most important thing is to 
exclude organic disease of the abdominal and thoracic viscera. This 
can only be done by careful and, it may be, repeated examination. 
Defendants sometimes claim in court that all the symptoms are subjective 
and that no one can tell how ill the claimant is. This is not altogether 
correct. A physician can tell much by the behavior, conduct, and appear- 
ance of a person as to whether he is ill or pretending. 
The differential diagnosis between hysteria and gross organic diseases 
is important. A patient has convulsions, palsy, ataxia, or neurasthenia. 
Are the symptoms hysterical or organic? The classical hysterical fit 
is not hard to recognize. The absence of the initial cry, the unbitten 
tongue, the retention of control of the sphincters, with, after a fit is 
over, the passage of a large quantity of clear urine, the preservation of 
consciousness or rather its alteration, an aberration of consciousness 
without its abolition, the state in which the patient knows more or less 
well what is going on but is unable to communicate with anyone, the slow 
onset of the fit, permitting the patient to reach a place of safety, the 
absence of momentary rigidity before the clonic spasm comes on, the 
dramatic element, the seeming purpose in the movements, the long 
continuance, the absence of deep sleep after, the presence of signs of 
hysteria between the attacks, all point to hysteria. 
The diagnosis of hysterical palsy may be difficult. This may be 
paraplegic, monoplegic, or hemiplegic. Authorities differ as to which 
is the more frequent. In the writer's experience brachial monoplegia, 
or rather hemiplegia, with much greater involvement of the arm than of 
the leg, has occurred most frequently, and paraplegia least. Although 
at first sight it may look like organic disease, the picture is never perfect. 
The condition of the reflexes is important. Hysteria never causes a 
loss of the knee-jerk. As a rule, it is much increased, but it does not THE TRAUMATIC NEUROSES AND PSYCHOSES 
651 
possess the qualities of the increase present in disease of the motor cortex 
or lateral tract. There is not the spasticity, and when the patellar 
tendon is tapped the whole leg is jerked, often the whole body, and the 
patient becomes excited. True ankle or patellar clonus is never present. 
There may be a few irregular and unequal to-and-fro movements of the 
foot when the examination is made, but true clonus, with the regularly 
periodical, equal, and continuous vibration of the foot, never occurs. 
The plantar-jerk may be absent or very marked even in an anesthetic 
foot; the true Babinski reflex is never present. 
Anesthesia is an important diagnostic symptom. It is sometimes 
claimed to be non-existent because the patient did not know of its exist- 
ence till examined, when, it is alleged, it has been brought into being by 
the suggestion of the physician. Now, anesthesia may be due to sugges- 
tion, but the explanation of many cases is that it is so slight as not to 
have made it difficult for the patient to feel objects. A patient may have 
tactile anesthesia sufficient to make him insensitive to a wisp of cotton 
or the light touch of a match stick, and yet feel perfectly when an object 
is pressed against or put in his hand. There are then multitudinous 
points of contact which may reinforce each other. A pretended analgesia 
can easily be discovered by sudden and unexpected pricks with a 
sharp instrument. This should not be done during the formal sensory 
examination, but before it and while the patient is off guard. Examiners 
sometimes forget that there may be preservation of the pain sense with 
tactile anesthesia. To jab a patient with a pin and because he says it 
hurts conclude he necessarily has no tactile anesthesia is to form a hasty 
judgment on insufficient evidence. The Mannkopff test, dependent upon 
the fact that a sudden painful stimulus frequently increases the pulse 
rate, is of some value in testing sensibility to pain, but sudden fright 
from fear of pain may do the same, and the amount of pain necessary 
to affect the pulse varies with the individual. 
Contractures are frequent and may be lasting. The only muscular 
wasting is that from disuse. It is always diffuse, and there is never any 
change in the electrical reactions. When marked local wasting is present 
there is organic disease. It is necessary to be on guard not to mistake 
the general wasting of disease for trophic changes. The contractures, 
especially if the patient is very thin, may make the joints, notably the 
knee and elbow, so prominent as to create a suspicion of trophic joint 
trouble. Such changes do not occur, but long-continued immobility 
of the limb may cause a kind of adhesive inflammation around the joint 
and in the muscles, causing stiffness in it and shortening of the muscles. 
In such a case the contracture does not relax under the most profound 
anesthesia. The hysterical joint, which curiously is usually the knee, 
is swollen and painful, but shows no other sign of inflammation. There 
is no local heat, redness, or swollen tortuous veins. It comes on suddenly 
and may pass away as suddenly. Hysterical joints are sometimes claimed 
to be cases of tuberculous arthritis due to direct injury, and hence the 
importance of a differential diagnosis. 
The palsy in hysteria may be either flaccid or rigid. In organic 
hemiplegia the patient in walking lifts the leg from the hip and swings 652 
DISEASES OF THE NERVOUS SYSTEM 
it; in hysteria the leg is dragged. Hoover has studied a sign of great 
value in differentiating the two. In organic hemiplegia, if the patient, 
lying supine, attempts to lift the palsied leg, the other is pressed down 
against the bed, if he lifts the non-palsied leg, the other is pressed down. 
This does not occur in hysteria. There is never in hysteria, a true palsy 
of the bladder and rectum, but temporary retention is common. 
The visual symptoms are interesting. Contraction of the fields of 
vision with reversal, although common, is not pathognomonic. It occurs 
in brain tumor, fracture of the spine, and, indeed, not a few other diseases. 
A gradual contraction of the fields during the examination is characteristic 
rather of neurasthenia and states of exhaustion, but tubular vision 
apparently occurs only in hysteria. Monocular diplopia is hysterical 
unless due to dislocation of the lens or some other local trouble, but it 
must be remembered that unintelligent people often complain of monocu- 
lar diplopia when really they mean they have dimmed or blurred vision 
in one eye. Hysterical blindness can only be diagnosed by exclusion, 
and must be separated from mind blindness. Since the patient avoids 
obstacles in walking, he may be accused of hysteria or even fraud. The 
condition is always accompanied by other signs of brain disease. 
Hysterical ataxia is differentiated by the absence of the signs which 
accompany organic ataxia. Occasionally it is mistaken for cerebellar 
disease, the examiner being misled by the fact, common in the latter 
disease, that the patient moves the legs well in bed. 
Hysterical mutism must be separated from aphasia and fraud. As 
a rule, the presence of other symptoms makes the diagnosis quite easy. 
The hysterical mute, as a rule, manifestly makes no real attempts to 
speak; the aphasic, no matter how speechless, always tries. In armies, 
soldiers are cured of pretended mutism by the inhalation of chloroform. 
When mutism is the only symptom, careful watching will soon decide. 
Prognosis.-The danger to life is very small in any of the traumatic 
neuroses and psychoses. Pure neurasthenia never in and by itself 
leads to death, nor does it seem to increase very much the patient's 
susceptibility to infection if he is so situated that he can take proper 
care of himself. There is some dispute as to whether hysteria ever ends 
fatally. A few cases have been reported, but the probability of there 
having been an error in diagnosis is quite great. Myasthenia gravis 
and Landry's paralysis are sometimes mistaken for it, especially in the 
early stages, and occasionally patients who have had severe hysterical 
paralysis for years develop sclerosis of the cord and die from its secondary 
consequence. Whether a larger percentage develop such lesions than 
can be accounted for by mere arithmetical chance, or whether long- 
continued hysteria does predispose to sclerosis, is an unsettled question. 
If it does, then hysteria may indirectly be a fatal disease. Personally, 
if a hysterical patient should die under my care, unless from some 
intercurrent disease, my belief would be that there had been an error 
in diagnosis. 
The outlook as to the duration of any given case of traumatic hysteria 
or neurasthenia depends on many factors. The age of the patient, the 
severity of the illness, the length of time it has already existed, the THE TRAUMATIC NEUROSES AND PSYCHOSES 
653 
ability of the patient to obtain proper treatment, are all important 
elements. Although some cases of hysteria are recovered from suddenly 
and the cure of neurasthenia is never sudden, the latter disease probably 
has a somewhat higher permanent recovery rate than the former, because 
hysterical patients are very prone to relapse when subjected to any 
stress or even without it. In general, old age is of bad prognostic import, 
because when the arteries begin to thicken and the kidneys are sclerosed 
the patient's power of resistance to shock decreases. Usually youth 
increases the chance of recovery, but hysteria may so upset the mental 
and nervous equilibrium as to lead to permanent disease or a life of semi- 
invalidism. This is, of course, especially true in those patients in whom 
there is a strong hereditary or congenital predisposition to hysteria. 
One of the most important elements in prognosis is ability of the patient 
to get proper treatment early in the illness, especially if it be neurasthenia. 
It is rarely safe to venture any prophecy as to how long illness will last. 
It is never safe to do so unless the physician knows all about the case. 
The duration of traumatic lumbago is very variable. Enough pain 
usually lasts after the acute stage is over to give the patient some trouble 
for a long time. 
The prognosis of abnormal mental states is the same as when not 
caused by shock, except that, in general, in any mental trouble directly 
resulting from an external cause the patient has a better chance for 
recovery, other things being equal, than if it had arisen without such 
external cause. CHAPTER XVII. 
HYSTERIA. 
SMITH ELY JELLIFFE, M.D. 
The synonyms for this affection are legion. The term hysteria was 
in common use in the time of Hippocrates; Pliny spoke of suffocatio 
mulierum, Willis and Sydenham of affectio hysterica, Lorry of melancholio 
nervea, Sauvage and nosologists of his time of hysterie, Van Helmholt 
of asthma uteri, Piorry of neuropallie, while Babinski's pithiatism is 
the latest term proposed. 
Etiology and Psychopathology.-No abnormal manifestation of human 
conduct is so well documented as that which is usually termed hysteria. 
From the very earliest times to the present, it has been recorded, in some 
of its phenomena, in all of the forms of human expression. It entered 
into legends and folk lore before historic records were known; it can be 
traced in the most ancient books of the East and West, in the poems and 
plays of the latter lyrical or dramatic writers, in the records of historians 
of events, chroniclers of action, and the makers of laws; can be seen 
preserved in marble in the works of the Greeks and Romans, in wood 
in the early Christian centuries, and in the forms of decorative art of 
the middle ages to the present. Priests, lawyers, philosophers, and 
physicians have occupied themselves with it in all ages and in all lands, 
and it still remains the great enigma of human personality. At no time 
in the past can it be said that any unanimity of opinion was reached 
concerning its essence; every age has known conflicting opinions, not 
only concerning its causes, but also its manifestations. Current notions 
of the times and customs of the different countries have each in their 
turn contributed to give it a varying presentation, but its underlying 
features have remained the same throughout. 
Down through historical records one can trace in almost unbroken 
succession the development and modifications of the germ idea of an 
unsatisfied uterine longing as bearing a genetic relationship to hysteria, 
which, in the modern expressions of Freud and his disciples receives its 
most refined and psychologically subtle rendering as the "psychic con- 
version of forgotten sexual trauma." The uterine etiology died a linger- 
ing death, and had its advocates as late as the middle of the eighteenth 
century when the nervous system of the uterus became the chief criminal 
in the new pursuit. 
Humoral Doctrines.-These doctrines were held by Galen, but a later 
humoralism was prevalent in the eighteenth century. Naturally there 
were many with mixed humoral and uterine doctrines. Auto-intoxication, 
gastric, intestinal, and dental, are the modern descendants. 
654 HYSTERIA 
655 
Nerve Theories.-To consider the nerves as a primary seat of hysteria 
was a natural outgrowth, and Boerhaave sought to explain the hysterical 
crises by a sort of intoxication of the abdominal nerves, due to a stagna- 
tion or obstruction of the corrupting humors. Boerhaave was in reality 
one of the earliest suggestors of the splanchnic origin of hysteria. He 
did not give any better or worse definitions of these corrupting humors 
than many modern auto-intoxication advocates. He did not believe 
that the uterus was the site of the manufacture of these humors. 
Although, for English-speaking students, the works of Sydenham 
are justly regarded as offering a remarkable picture of hysteria, yet he 
introduced ideas of retrogression, the influence of which is seen even at 
the present day. It has been noted that Galen may have believed in 
hysteria in the male, but, if so, such a belief was forgotten for many hun- 
dreds of years, and only about Piso's time did the presence of hysteria 
in the male obtrude itself upon the thoughts of clinicians. How to 
handle it, in view of the uterine humoral theories, was evidently difficult, 
and Sydenham cut the knot by saying that hypochondria was for the 
male what hysteria was for the female. Sydenham may also be regarded 
as the forerunner of the doctrine that there was no such disease as 
hysteria; hypochondria was everything in this century. 
Cerebral Localization Hypothesis.-Cesbron makes the rather startling 
statement that from the days of Hippocrates to the nineteenth century 
practically only four writers located hysteria as an affection of the brain. 
Carolus Piso, in 1618, said that all the symptoms of hysteria came from 
the head and not from the uterus or intestines, and he also was one of the 
first to describe cutaneous anesthesia, deafness, blindness, hysterical 
aphonia, and hysterical tremors. 
Modern Period -With the development of the Charcot school the 
hysteria hypotheses commenced to take on more definite shape, and in 
the past thirty years the views show a marked similarity even though 
couched in different terminologies. These modern hypotheses may be 
grouped about three general centres, the psychological, the physiological 
and the biological, in all of which the psychogenic factor is prominent. 
The chief psychological hypothesis, namely, that of dissociated person- 
alities, received its first great impulse from Charcot himself, and has 
been most attractively elaborated and made popular by his pupil Janet, 
and even more intricately analyzed and extended by Freud and his 
school. For Charcot, hysteria was a psychosis, and Gilles de la Tourette 
is largely responsible for the prominence of the factor of suggestion in 
the aftercoming presentations; the extreme position of this feature alone 
having been advocated by Babinski, which author would seek to dis- 
member hysteria further and give us a new grouping within this large 
medley, a task that Janet has also attempted, as well as Freud. Pithia- 
tism, psychasthenia, and the anxiety neuroses are the new entities partly 
separated out of the hysteria conglomerate, partly from the neurasthenic 
mass, and partly from the initial stages of the more frank psychoses. 
Of the Charcot followers, Mobius suggested the line of many later 
definitions. He called those morbid phenomena hysterical which were 
induced by ideas, and the physical as well as the psychical reactions 656 
DISEASES OF THE NERVOUS SYSTEM 
had a common psychogenic origin. For him every one was more or 
less hysterical. Every one has hysterical small coin in the bank of his 
personality. Gilles de la Tourette's large monograph, published in 1891, 
is the most faithful elucidation and amplification of the Charcot doctrine. 
It, with Briquet's classic, has served as the modern fount of sympto- 
matology. At present it is recognized that the Charcot teachings were 
too fixed; they regarded hysteria in the light of an indivisible entity, 
almost in the formal light of a " species" within the limits of which were 
dragged a vast cohort of symptoms. Charcot described an entity- 
a morbus-where present-day psychiatry sees a cohors morborum, 
having really little in common, save a tendency to similar emotional 
reactions. The stigmata of Charcot are not alone the appanage of a 
definite disease, they are very widely distributed. 
Probably no studies of hysteria in modern times have attracted so 
much attention as those of Pierre Janet, who has been so very prolific 
that it becomes almost impossible to give a short resume of his standpoint. 
His own abstract, given at the Amsterdam Congress in 1907, is perhaps 
the best available. The study of somnambulism is his starting point. 
For him there exists in consciousness a region below, if such a term be 
permissible, the normal waking or personal consciousness, which is called 
the subconscious. Groups of ideas may exist in this, so to speak, twilight 
region without being at all clearly perceived by an individual, in fact, 
without being known at all, and yet they may operate to produce results 
very much as if they were the subject of voluntary attention. 
The hysteric in an access of delirium lives through fancied experiences 
about which he knows nothing when he "comes to"-he has an amnesia 
for all of these events. The hysterical amnesia does not confine its mani- 
festations to such conditions, but invades the details of life. The person 
who is sent on an errand forgets what she is sent for before she gets half 
way to her destination. This is a simple but common example. Janet 
would explain this by a disorder of attention. The directions are imparted 
to the patient but are not acutely attended to, and drop at once into the 
region of the subconscious and are forgotten by the waking, personal 
consciousness. The anesthetic arm is so because the patient does not 
attend sufficiently to the sensations from the arm to perceive them. 
There is a narrowing of the field of personal consciousness, which is but 
another way of expressing the defect in attention. 
The synthesis of mental processes into a coherent whole constitutes 
the personality or ego, and the hysterical process causes a splitting, a 
disintegration, or a doubling of the personality. Janet's definition is 
that "hysteria is a form of mental depression, characterized by the 
retraction of the field of personal consciousness and by the tendency to 
the dissociation and the emancipation of systems of ideas and of functions 
which by their synthesis constitute the personality." For Janet the 
hysterical and the hypnotic states are identical, based upon the common 
factor of suggestibility. 
The theories of Sidis, of Breur and Freud, and those of the Freud 
school are modified dissociation theories in which one finds more stress 
laid upon etiological factors. This is practically true of Freud's ideas, HYSTERIA 
657 
which are of paramount interest to the student of mental problems since 
this author's work with that of Vogt's has proved a great stimulus leading 
to the interpretation of mental mechanisms. 
Freud's hypothesis presupposes three features: (a) The role of psycho- 
genesis, (6) Janet's ideas upon dissociation and psychical automatism, 
and (c) Binswanger's formulation of the etiological importance of the 
affect. As early as 1880 Breur advanced the interpretation that the 
individual roots of the hysterical symptoms were to be sought in ideational 
complexes with marked feeling tone which came about as a result of 
psychical or physical trauma, and in 1893 to 1895 Breur and Freud 
further formulated the idea that the psycho-neurotic symptoms originated 
from these complexes either by a process of (a) conversion, whereby the 
emotional (affect) excitement brought about abnormal physical innerva- 
tion-this caused hysteria; or (6) by transposition of the affect through 
indifferent ideas (anxiety neuroses). The principle of overdetermination 
expressed the heaping action of an affect sufficient to determine a symp- 
tom. This same overdetermination is more concretely and physiologic- 
ally expressed by Cajal's concept of avalanche action. 
The ground of the conversion or the transposition lies in the immisci- 
bility of the traumatic complex with the personality. The patient 
refuses to accept it, and instead of ab-reacting-and thus normally 
disposing of the complex-converts it or transposes it. The affect thus 
remains shut in or hidden in the subconscious. 
Gradually Freud's attention became centred, and at first somewhat 
one-sidedly, upon a trauma of a genital nature. His general hypothesis 
is extremely intricate, and no short abstract does justice to the results 
obtained by his psycho-analysis, yet his present attitude may be expressed 
somewhat as follows: There develops, usually on a constitutional basis, 
in the infantile period definite sexual activities which are mostly of a 
perverse nature. These activities do not, as a rule, lead to a definite 
neurosis up to the time of puberty, which in the psychic sphere appears 
much earlier than in the body, but sexual fantasy maintains a perverse 
constellated direction by reason of the infantile sexual activities. On 
constitutional (affect) grounds the increased fantasy of the hysteric 
leads to the formation of complexes which are not taken up by the 
personality and by reason of shame or disgust remain buried. There 
therefore results a conflict between the characteristic normal libido and 
the sexual repressions of these buried infantile perversions. These 
conflicts give rise to the hysterical symptoms. It is in his contributions 
to the sexual theory that Freud develops his later thoughts of the sexual 
origin of the hysterical reaction. By sexual it is important to remember 
Freud is not speaking of sensual, and that sexual means the instinct of 
race propagation. 
Inasmuch as these sexual traumata are forgotten, buried in the uncon- 
scious, it becomes necessary to dig them out by the process of psycho- 
analysis, either using Freud's method, or by the association tests so 
minutely elaborated by Jung and Riklin particularly. In practice it may 
take months to fully analyze some hysterical cases. When fully analyzed 
the patients become cured-the analysis has been a catharsis, 
vol. v-42 658 
DISEASES OF THE NERVOUS SYSTEM 
White1 has expressed the whole matter very clearly. " The character- 
istic of the psychic traumata that produce hysteria is their large content 
of painful affect. A painful affect, fully reacted to at the time, may 
produce no harm, but if for any reason reaction fails, the feelings become 
repressed and the possibilities of dissociation are created. Failure of 
reaction may be due to the failure of conditions that make efficient 
reaction impossible, as for instance, an insult "is swallowed," a dear 
friend or parent is lost; and no compensation is possible. This gives 
rise to "retention-hysteria." Again, ideas usually of a sexual nature, 
which are incompatible with the personal consciousness, are repressed- 
ab-reaction is not permitted, no effectual catharsis takes place. This 
condition produces the "defense" hysteria. Finally, experiences occur 
in a hypnoid state-i. e., in a split-off, dissociated, or dreamy state. 
They produce the so-called "hypnoid-hysteria." 
"The final principle of the Breur-Freud hypothesis is the principle 
of conversion. The strangulated affect, the unreacted-to emotion, belong- 
ing to the dissociated state which has been repressed, finds its way into 
bodily innervation, thus producing the motor phenomena of hysteria. 
In this way the strong idea is weakened by being robbed of its affect- 
the real object of conversion." 
It is premature to pass judgment on Freud's ideas. They have their 
warm advocates and bitter opponents-they suggest the psychical 
archeologist working among the fragments of old, crumbling, and muti- 
lated memories with a work of reconstruction in mind. The method 
of the archeologist who reconstructs the entire animal from a single 
tooth receives the approval of the archeological world, and experience is 
accumulating rapidly to show that the psycho-analytic method of Freud 
is on a sound basis in the interpretation of hysterical states.2 
It has already been intimated that the superstructure built on the 
Charcot foundations had become a little top heavy, and the time had 
arrived for a more searching critique. There have been hosts of destruc- 
tive critics, and the dismemberment of hysteria has been going on pari 
passu with its growth. Of the more recent serious studies looking toward 
a limitation of the concept and a sharpening of the diagnostic boundaries, 
the monograph of Steyerthal and the articles by Babinski are the most 
noteworthy. Steyerthal's position is extreme. For him there is no 
such thing as hysteria. The vast majority of the cases may be reduced 
to neurasthenia and mental inferiority. The stigmata of Charcot, 
Janet, and others are nothing but signs of tire or exhaustion. Steyer- 
thal's exposition is ingenious and scholarly, and he has detected a number 
of historical errors which have been repeated for many years, but his 
general standpoint is too narrow. Babinski's chief position is equally 
restricted, but in another direction. His dismemberment of hysteria 
1 Current Conceptions of Hysteria, Interstate Medical Journal, January, 1910. 
2 Freud's ideas are available in Brill's very excellent translation, Selected Papers 
on Hysteria and Other Psychoneuroses, Nervous and Mental Disease Monograph 
Series, No. 4, New York, 1912. The method of Psycho-analysis by Association 
Tests is given best in the same writer's translation of Jung's Psychology of Dementia 
Praecox, same series, No. 3, 1909. Also in Payne's translation of Hitschmann- 
Freud's Theories of the Neuroses and Psychoneuroses, same series. HYSTERIA 
659 
is the most complete contribution that he has given to date.1 He has 
seen the hysterical problem grow, and with many another, he has sought 
to carve away from the mass the trivial and unessential and present 
some definite entity. This new grouping of old facts under a new head 
he has christened pithiatisme; it consists of those hysterias of tradition 
which respond to his formula, namely, those patients whose ills are 
brought into existence by the power of suggestion, and the cure of which 
takes place by persuasion. And suggestion, for Babinski, should express 
the action by which one endeavors to make another accept or realize 
an idea which is manifestly unreasonable, while persuasion ought to be 
applied to ideas that are reasonable, or which, at least, are not in opposition 
to good sense. All that class of phenomena which falls within the limits 
of this concept are truly pithiatique, or, using the old term which Babinski 
would avoid, are hysterical. 
Babinski has attacked the unconscious and great extension of the idea 
of hysteria. The reasons for this are numerous, but may be conveniently 
assembled in three groups as follows: They are due to (1) errors in diag- 
nosis; (2) to defective observation and the including in the hysterical 
camp of a number of simulated symptoms which do not belong there, 
and finally, (3) the confusion of nervous states which should be considered 
separately--neurasthenia, hypochondria, etc. 
While all of the errors in diagnosis cannot be reviewed, Babinski calls 
particular attention to hemiplegia and hemianesthesia. The plantar 
reflex of Babinski, the combined movements of trunk and thighs, the 
Hoover thigh phenomenon, the sign of Grasset, the hypertonicity of 
paralyzed muscles, the phenomena of pronation, have come to aid in 
diagnosis. As for hemianesthesia, if the examination be sufficiently 
artful, it disappears entirely. If the more careful methods of Babinski, 
Stern, and Ziehen are employed it would appear that Babinski's conten- 
tion is fairly true. Babinski further has pointed out that in the mistakes 
have been included hemoptysis, hematemesis, hematuria, and fever. 
The phenomena of his second group, simulation and deceit, are difficult 
of classification. These, again, may be subdivided. One sub-group 
consists of the imitators of true pithiatic signs, those who simulate true 
hysteria-these show the various accidents such as paralyses, contract- 
ures, anesthesia, etc. This sub-group is one of the most difficult to 
understand, and it cannot be said that Babinski has thrown any real 
light on it. These have been dealt with under the heading of aggravation 
and exaggeration by the followers of the more classical schemes. Inas- 
much as persuasion does not seem to cure them, they are, ergo, not 
pithiatiques, but simulators. Surely a very weak position. A second 
sub-group comprises those patients whose phenomena are fictitious and 
cannot be induced by suggestion. Fever and anuria are examples. 
A third sub-group comprises real phenomena but consciously brought 
about for purposes of deception. These are the erythemata, phlyctenulse, 
ecchymoses, ulcerations, sphacelee, oedemas, etc. This group Dupre 
has already termed "mythomanies." 
1 La Semaine Medicale, January 6, 1909, No. 1, p. 1. 660 
DISEASES OF THE NERVOUS SYSTEM 
Finally, in discussing the third category, Babinski narrows down his 
conception to those cases which can be influenced by suggestion, and in 
a significant line he asks of the symptomatic aspect of the phenomena 
which suggestion can give rise to: Is it specific; does it belong to troubles 
related possibly to an organic affection, or to a functional malady having 
a mechanism different from suggestion? Here Babinski begs the whole 
question. For if we have a suggested chorea so like the real thing as 
to be inseparable by present methods, it hardly seems that his criteria 
help us much. Babinski in his third group separated the true pithiatiques 
from the "emotives"-in whom moral shock can cause phenomena to 
appear or disappear. He contends that in these cases an imitative factor 
is greater than the emotional one; finally, he erects a third sub-group 
in which exaggerated tendon reflexes, vasomotor phenomena, dermo- 
graphia, etc., are present and usually allied with hysteria. 
True hysteria then falls into three large sub-groups-the " pithiatiques," 
the "emotifs," and the "reflex," and these constitute a well-limited 
neuropathic state distinguished from all other neuroses. One can deduce 
that the hysterical or pithiatique phenomena depend essentially in their 
appearance, duration, form or disappearance, on the psychic milieu of 
suggestible subjects whose predisposition is susceptible of being put in 
play by such and such a spectacle, or such and such a proposal. 
Babinski concludes that: (1) A physician should guard himself 
from maladroit suggestions and poor methods of examination. (2) 
Knowing the influence that surroundings play with these patients, these 
should be so modified as to get the desired effect. (3) One must avoid 
contrasuggestive treatment. (4) One should limit one's self in diagnosis 
and not make too many promises, especially in the combined cases, 
i. e., where there are hysterical signs due in reality to organic disease. 
(5) From a medicolegal standpoint it is important to differentiate hysteria 
from simulation in order not to deprive those really suffering from-just 
recompense on the one hand, and secondly, to limit the abuse of accident 
litigation in fraudulent claims. 
Physiological Theories.-The modern physiological hypotheses of 
chief interest are those of Sollier, Binswanger, and the English school. 
They hardly do more than restate the fundamental psychological 
hypotheses in physiological terms. Sollier defines hysteria as a psychical 
functional disturbance of the brain, consisting of a torpor or a sleep of 
the cerebral centres, localized or generalized, temporary or permanent, 
and manifesting itself consequently according to the centres affected by 
vasomotor, trophic, visceral, sensory, motor, and final psychic disturb- 
ances, and according to its variations, its degree, and duration, by tran- 
sitory crises, permanent stigmata, or by paroxysmal accidents. Con- 
firmed hysterics are only vigilambulists whose state of sleep is more or 
less profound, more or less extensive. This is a dissociation hypothesis 
pure and simple, and inasmuch as little is known of sleep physiologically, 
one makes no advance by defining hysteria in terms of sleep. 
Binswanger may be reckoned among the adherents of a physiological 
hypothesis. For him, although he claims it to be true that all hysterical 
phenomena can be influenced by psychical processes, yet there is no proof HYSTERIA 
661 
that hysteria originates without exception by means of psychical processes 
exclusively. The disturbances of sensation and of motion are not solely 
of psychical origin. The same holds true for many of the visceral dis- 
turbances. The trend of recent study tends to confirm Binswanger's view. 
The hysterical change consists in the disturbance of the orderly correla- 
tion between the psychical and material processes, and in a double 
direction. On the one hand for a definite series of material cortical 
stimuli, the parallel psychical processes are either lacking or only incom- 
pletely awakened, while, on the other hand, an excess of psychical accom- 
plishment results in response to minimal cortical stimulation, producing 
a variety of back actions on the entire innervation processes which 
originate in the cortex, or are ruled by it. The pathological super- 
abundance of psychical work consists not only in an increase of emotional 
reactions, in a sharpening of the sensations, or in the propping up of 
overvalued ideas, but also in the very striking facility by which the 
psychical processes, above all, ideas, can influence function to produce 
hysterical reactions. 
It must be considered that in individual functional areas of the cortex, 
entirely different variations from the normal conditions of stimulation, 
both quantitatively and qualitatively, exist. At times over-stimulation, 
at times under-stimulation come into play, or dissimilar activities may 
show side by side. Furthermore, it is to be borne in mind that sharply 
localized disturbances of the cortex secondarily involve the capacity of 
other functional cortical areas, either enhancing or depressing their 
activities. Such functional dependencies show themselves most markedly 
in the disturbance of the corticomotor regions, where, for example, a 
"falling out" of the functional capacity of the path from cortex to bulbar 
or spinal motor centres causes paresis or paralysis, the incoming stimuli, 
influenced by anesthesia or analgesia, not being sufficiently active to 
cause the requisite nerve discharge leading to motor activity. This 
disturbance may extend to the entire nervous system, showing itself 
particularly in correlated physiological functional groups. 
Binswanger further holds that similar dynamic disturbances are found 
in epilepsy, hypochondria, neurasthenia, etc., but that the distribution 
of the disturbed mechanism varies, and, although the entire group may 
be thrown into one, the psychoneuroses, yet each has its distinguishing 
features notwithstanding overlapping, combination, and mixed forms. 
Such a physiological statement does not go far enough, however; 
practically the same defects may be postulated for the psychoses in 
general, certainly for dementia prsecox at least. According to Binswanger, 
however, the hysterical changes are characterized by the abnormal 
activity of psychical influence on the innervation processes. Propor- 
tionately slight, of themselves almost negligible, cortical stimuli set up 
intense sensations, with pathologically increased feeling tone (pain) 
and conversely corticoperipheral stimuli processes, in spite of moderately 
strong peripheral stimuli, do not give rise to the parallel psychical pro- 
cesses. The intercortical or association processes show analogous 
disturbances. The importance of the pathological affect action has 
received much extended analysis and Binswanger adopts Freud's general 662 
DISEASES OF THE NERVOUS SYSTEM 
method of interpretation, especially with reference to the mechanism of 
conversion, laying less stress upon sexual traumata as the exclusive agents 
in the production of the reaction. 
Vasomotor Hypothesis.-Such a view has been adopted by numbers of 
English neurologists, the most recent expression of which is that of 
Savill. For this author hysterical syncope is one of the cardinal symp- 
toms in the disease. It represents a hysterical cerebral attack, and is 
due to the activity of a special reflex vasomotor centre situated in the 
solar plexus of the abdominal sympathetic. This is usually started 
into activity by an emotional stimulus from the brain, but may be 
started to activity and produce exactly the same kind of attack by 
pressure upon the groin, the stimulus passing through the iliohypogastric 
nerve, or by other peripheral stimulus from other areas in the skin or 
mucous membrane. Hysterical cerebral attacks may also be induced 
by variations in the vascular condition of the sphlanchnic area. 
It is a further feature of Savill's hypothesis that the vascular conditions 
within the abdomen, skin, and brain oscillate among themselves, and 
he further drags in a modern humoralism by assuming that the symptoms 
are conditioned by the state of the purity of the blood. Hysterical 
motor disorders, paralyses, etc., are due to localized or general vascular 
changes in the brain; they differ from organic lesions only in extent, 
duration, and intensity, and in the fact that they are primarily conditioned 
through the sympathetic. Other sensory symptoms, hyperalgesia, etc., 
are due to peripheral vascular lesions, and psychogenesis may play a 
leading role. 
As to the psychogenic element, Savill admitted that the mind plays a 
prominent part in many hysterical phenomena, but hysteria is a psychosis 
only in the following respects: 
1. Hysterical persons throughout life present certain inherent peculiar- 
ities of mind-e. g., a tendency to mental abstraction, to autohypnotism, 
to mental dissociation, and to dual consciousness-which render them 
more liable, especially on the occurrence of any disturbance of the cerebral 
circulation or nutrition, to exhibit abnormal mental phenomena. 
2. A certain proportion of hysterical symptoms are purely mental; 
the mental faculties are unstable and easily disturbed, particularly the 
various commemorative faculties; various tricks and habits of body and 
mind are constantly arising. 
3. The emotional side of the mind is strongly developed, and emotional 
outbursts which we call "hysterics" are frequent. Emotion is also a 
frequent determining cause of other hysterical disorders by producing 
vascular changes. By the well-known influence of the emotions on the 
vasomotor centres, vasomotor phenomena are common; and since the 
vasomotor centres are themselves unstable, many surprising effects 
and symptoms are produced as the indirect effect of emotion. 
4. The mind plays an important part in exaggerating symptoms which 
have a slight physical basis, such as a vascular derangement of a part. 
5. The mind also plays an important part in the perpetuation of symp- 
toms after the physical basis which had initiated them has passed away. 
Thus, Savill comes to a poly-etiological conclusion. No single lesion HYSTERIA 
663 
or hypothesis is capable of explaining all the symptoms. The vasomotor 
system, however, plays the most important role, and Savill states that 
fully 90 per cent, of all of the symptoms are conditioned by instability 
of the vasomotor centres throughout the body and a want of coordination 
among these, associated with more or less emotional instability. 
Although much is said of emotional instability, Savill makes no attempt 
to clear up the conception of the role of the emotions in this disease- 
a conception which practically all agree is of primary importance. If 
we are to succeed in analyzing hysteria we must approach the subject 
from the side of emotional reactivity. This is best considered here, as 
its final analysis will probably come from the physiological and patho- 
logical data furnished by the study of certain cellular alterations found 
in those psychoses with prominent disorder in the affective sphere. 
A logical mode of approach is hardly possible, but in considering some 
of the well-known psychical reactions which accompany organic visceral 
disease, a breach may be made in the walls of the problem. For some 
it is true such phenomena are not hysterical, but if the ground is so 
shifted, the significance of the word is lost. These types of phenomena 
furnish a clue to the nature of similar phenomena. 
Head has clearly presented certain mental changes which accompany 
visceral disease; the more patent of these may be dismissed from present 
consideration. In considering a special type of reflex pains of visceral 
disease and the influence that they consciously or unconsciously play in 
conduct, one approaches very closely, if not enters, the hysteria confines. 
Many of the phenomena called hysterical are almost constant accom- 
paniments of organic disease of the viscera. Hallucinations of sight, 
of hearing, of smell, haptic hallucinations, rarely as completely developed 
as seen in the major psychoses, are extremely frequent. Many of these 
partake more of the nature of illusions, but the difference is at times an 
artificial one, in which connection Mobius' term of pain illusion as descrip- 
tive of the psychogenic pains of hysteria is to be considered. Moodiness 
is constant and leads to sleeplessness. Restlessness, attacks of crying, 
ideas of impending ill, feelings of exaltation, impulses to suspicion, 
though rarely approaching the grades usually termed delusions of refer- 
ence or of observation, are extremely frequent. In all of these cases 
there is a type of pain which is produced by impulses passing from the 
affected organ up the fibres of the sympathetic, through the ganglion 
of the posterior root, into the central nervous system. From this result 
the reflex pains of the peripheral distribution of continuous segments 
so well described by Head. The intensity and duration of these visceral 
reflected pains are factors of considerable importance in the production 
of mental changes. Slight and evanescent pains rarely have any reaction, 
but the continuous, oftentimes only slight pains, are more likely to lead 
to the psychical effects. Women are more prone to these reflex vis- 
ceral pains, and menstruation increases the severity of the mental 
phenomena. 
Individuals thus afflicted often acquire a depressed moodiness which 
is, to use Head's phrase, non-projected, by which he seeks, and rather 
inadequately, to distinguish the state from an emotional state by calling 664 
DISEASES OF THE NERVOUS SYSTEM 
the latter projected. Consciousness is dominated by a feeling tone, but 
the reasoning state is not projected. The opposite side of this moodiness 
is an exaggerated sense of well-being. The moodiness of organic dis- 
orders may be associated, or even conditioned, by the ill-defined feelings 
of suspicion already mentioned. Such feelings are usually under the 
influence of persuasion, in which respect, again, their relation to like 
hysterical states is evident. 
A further consideration concerns changes in attention and memory. 
Their analysis is extremely difficult by reason of the complexity of the 
phenomena. Head has reported classical examples in mild and advanced 
tuberculosis and the literature of organic disorders allied with hysterical 
reactions is replete with similar cases; some of Binswanger's unusually 
ample histories in his great monograph on hysteria are instances in point. 
Notwithstanding the difficulty of analysis, several points stand out. 
Fatigue is one, and the presence of the feeling due to the visceral sensations 
is another. Thus, attention is divided, and memory is less complete. 
The individual capacities for reproducing-i. e., by associated processes 
to bring up in consciousness memory images of feeling tone-are extremely 
variable, and here visuals, auditives, olfactives, affectives, etc., follow 
their own laws of association. 
While these considerations may, at first sight, seem to have but 
indirect bearing upon the subject of hysteria proper, it is very evident 
that they do bear directly upon the mechanism by which many manifesta- 
tions, usually termed hysterical, are brought into existence, and further- 
more they are of importance in any attempt to sift out those cases which 
depend upon some definite organic factor, either from the standpoint 
of a visceral irritant factor in producing mental states of altered adapta- 
tion, or as causative in reducing mental resistance. The significance of 
Babinski's insistent critique of hysterical phenomena is amply justified 
by these considerations. 
The next step is to ascertain how far such similar mechanisms may be 
set in play by lesser and lesser amounts of organic change and greater and 
greater amounts of mental presentation of the same class of phenomena. 
For it is practically coming to be conceded that the term hysteria should 
be restricted to the more purely psychogenetically induced disturbances 
of these mechanisms just considered. Not that these cover the entire 
series of phenomena ranged as hysterical, but they are those least capable 
of being understood as possibly arising as purely psychical reactions. 
No attempt is made to present the theories of the emotions, but it is 
accepted that inadequacies to emotional reactions are primary factors 
in the phenomena of hysteria. 
Stated in very bald and primitive form, when one is brought face to 
face with an experience-say the oncoming of an excited animal-one's 
primary sensations of recognition and feeling tone of fear cause the 
adaptive motor mechanism of self-protection. In the healthy animal 
it may be to flight, but an inadequate reaction may result in inability 
to do anything, i. e., in a paralysis, the individual components of which 
cannot be here analyzed. One may reason that so long as the reaction 
remains inadequate the individual suffers from an emotion, fear, and the HYSTERIA 
665 
recognition of the inadequacy, i. e., the paralysis, constitutes the emo- 
tion. Thus the emotion considered biologically is an evidence of mal- 
adaptation. 
Head's work seems to point in the direction of the localization of the 
affected mechanisms, i. e., the sympathetic, but turning to Sherrington's 
recent discussion of the proprio-receptive system one finds indications 
that the question is a much wider one and progress is being made that is 
eminently suggestive, at least, in its bearings on the hysteria problem. 
Biological Hypotheses.-One terms these biological largely for the sake 
of convenience. They regard the hysterical reaction in the light of an 
adjustment to experience, or interpret it in biological terms, or perhaps 
in the terms of comparative psychology. The hypotheses mentioned 
do not conform to hard and fast classifications; thus Freud's latest inter- 
pretations could as well be classed here as under the head of psychological 
hypotheses. Here one can conveniently place the views of Dubois, and 
more particularly his pupil Schnyder, Hellpach, and Claparede, not to 
mention a host of others. Schnyder regards hysteria as the persistence 
in adult life of the childish type of reaction to the facts of life. It is a 
mode of reaction in persons of naive, simple, and elementary mentality. 
It is a mentality lacking in development and defective in judgment and 
critique. Such mentalities, when placed in new environments to which 
they cannot adapt, or adapt with difficulty, develop the hysterical 
reaction. They go back to primitive methods of escaping difficulties. 
Schnyder also speaks of a racial hysteria as a manifestation of the 
infancy of a nation, of people who are primitive and simple. It is a 
commonplace to note that conduct which for a white man would be called 
hysterical is normal for the colored race, and Kraepelin's observation of 
the great amount of hysteria among the natives of Java is in line with 
this general argument. 
Modern methods of expression, the press, strikes, boycotts, unions, 
etc., are the safety valves of much that otherwise would be retained 
and repressed, and in Freud's terms "be converted" into hysterical 
phenomena. 
Claparede lays special stress upon the fact that the hysteric shows a 
marked resistance to the recall of painful memories. This, viewed 
biologically, he considers to be a defense reaction. Suggestibility is 
also a protective adaptation against excessive development of personal 
peculiarities and tendencies, which, should they be given free play, would 
isolate the individual from the social world in which he lives. Instead of 
yielding to these impulses the suggestion of another is followed because 
adaptive. Suggestion thus becomes a biologically rational process- 
not, as Babinski would have it, a response to irrational thoughts. Clap- 
arede's ideas are interesting in view of Bleuler's very clear-cut presenta- 
tion of suggestibility in the light of an adaptive mechanism with nega- 
tivism as its physiological corrective, producing severally in its excess 
hypersuggestibility and hypernegativism in both the hysterical and 
dementia prsecox reactions. 
An attempt at a biological definition may be hazarded. Thus under- 
stood, hysteria is the carrying over into adult life of a primitive adaptive 666 
DISEASES OF THE NERVOUS SYSTEM 
type of nervous and mental reaction to psychical influences due to 
congenital anomaly, acquired development, or diminished resistance. 
A few words may be said concerning the anatomical and pathological 
data. Here, although definite correlations have not yet been affected, 
there are indications which show that the factors of congenital anomaly 
and diminished resistance may have some demonstrable anatomical 
substratum. The analysis of cortical function and cellular structure is 
yielding results. Flechsig's data have been partly verified, corrected 
and amplified by the systematic study of structural variations in the 
cortex by cytological methods. Criteria are being established for the 
determination of the physiological value of cell layers and cell 
groups. 
The layers of cells functioning for the reception of different types of 
stimuli are becoming fairly well delimited, but perhaps more important 
than all of these, pathological studies of the psychoses, dementia prtecox 
particularly, show us, though as yet but faintly, that certain definite 
cell groups are affected. Here there is a most profound disturbance of 
the affective states with relative integrity of sensation, motion, memory, 
and association processes. The work of Cajal on the functional physi- 
ology of different neurones is giving more point to the ideas of dynamic 
polarization and some tangible anatomical details are coming to hand 
which give warrant for the terms "condensors" and "accumulators" of 
nervous energy. There is some hope of explaining the mechanism of 
increased discharge, lengthened discharge, and avalanche action on 
anatomical grounds, and problems which have always been considered 
as purely functional seem to offer some hope of solution by these more 
tangible and objective procedures. 
Symptoms.-Before attempting a systematic presentation of the symp- 
toms of the hysterical reaction a few remarks may be made regarding 
the value of this motley collection of observations in which real fragments 
are mingled with those born of credulity and mental laziness. The golden 
period of hysteria which reached such a high point as a result of the 
stimulus of the Charcot teachings has been followed by one of analysis 
in which the careful sifting of the enormous material has become an 
imperative necessity. One of the first striking facts of this reexamination 
is the astonishing frequency of mistakes in diagnosis. 
Thus, the tubercle bacillus has shown the real character of many 
heretofore diagnosed hysterical hemoptyses; gastric chemistry has simi- 
larly relegated many intestinal disorders into their proper position; 
microscopic and cryoscopic methods enable one to determine the essential 
features of supposed hysterical hematurias; methods of blood examination 
have reduced the number of hysterical fevers almost to a minimum. 
In the field of the paralyses, hemiplegias, monoplegias, paraplegias, 
etc., mistakes have been especially frequent. With the newer signs of 
organic involvement of the nervous system many organic disorders are 
recognized which, heretofore, were called hysterical, and are even called 
so at the present time in their early stages. Literally thousands of sick 
individuals suffer from complaints which a lazy diagnosis dubs hysteria. 
The falsity would be revealed by a searching and intelligent analysis. HYSTERIA 
667 
Another feature concerning the revaluation of symptoms and one of 
the most difficult to adequately present, is simulation. Unconscious 
simulation, if there be such, and aggravation are not included here. 
These are features of what must be termed the hysterical mentality. 
Simulation as here restricted is probably rare, yet it is constant and 
serves to discourage careful study of many deserving patients. 
What symptoms are due to deception and deceit in the non-hysterical 
and in the hysterical? It is highly probable that many of those who 
practise such deceptions, seen from the psychiatric view point, are either 
debiles or mild precocious dements. As for deliberate frauds, their 
symptomatology may be disregarded. There still remain a large number 
of symptoms which are held to be characteristic of hysteria, and which, 
however, are variously estimated by different observers. Here one is 
on the most difficult ground of the hysteria problem and each individual 
observer looks at the symptoms from entirely different points of view. 
Each reflects certain aspects of subjective truth rendering a combination 
impossible, hence the hopelessness of a definition. 
The imperfect scales have been many. Thus, restricting the discussion 
purely to modern symbols, the striking motor phenomena of the "crises 
des nerfs," the major attacks, are the only real phenomena which should 
be considered as hysterical, according to the present view of Bernheim. 
All others are epiphenomena and are the results of education. This 
point of view would considerably simplify the symptomatology. 
The subdivision of symptoms into two groups such as stigmata and as 
accidents has long been current, but it is certain that in the word stigmata 
is found a relic of the methods of the middle ages in their search for trace 
of the presence of the Devil in accused individuals. Whereas theo- 
logians, magistrates, and medical men were in practical accord in the year 
1600, at the present time there are hardly two neurologists agreed as to 
what shall be considered as stigmata or essential features of the hysterical 
phenomena. One knows that for Janet, anesthesias, amnesias, abulias, 
paralyses and changes of character represent the stigmata, while under 
the head of accidents, he groups hysterical attacks, somnambulism, 
subconscious acts, and fixed ideas, whereas Babinski claims that careful 
examination shows that anesthesia never exists, and between these one 
finds all possible variations. Some authors lay great stress on restriction 
of the visual fields, others practically deny its existence. 
Thus for almost every symptom these conflicting attitudes of mind 
are found. A few uphold the absolute lack of existence of many phe- 
nomena, while others are in supreme discord as to the interpretation of 
phenomena which to them are subjectively true. It becomes, therefore, 
a difficult matter to know just how to present the symptoms. We have 
chosen the purely clinical method, grouping the observed symptoms 
according to the methods of clinical neurology and clinical psychiatry. 
Inasmuch as anatomy teaches the absolute interrelation of nervous struct- 
ures within the entire human body, it is an idle distinction to say that 
hysteria is a psychosis or a neurosis. Splitting the difference by calling 
it a psychoneurosis simply implies that the nervous adjustments of the 
different parts of the body, one to another, as well as the adjustment of 668 
DISEASES OF THE NERVOUS SYSTEM 
the individual as a whole to his environment, show various types of 
disturbances called hysterical symptoms. 
Hysteria is a general tendency to certain reactive expressions. The 
difficulty in description is an evidence of the instability of the concept, 
its width, and its fluctuating outlines. There lies in every person the 
possibility of the hysterical reaction, and it all depends either on the 
stimulus or the change in the resistance to bring it out. Pitt's satirical 
comment that "every man has his price" has its analogue in the truth 
that every individual has his hysterical Jack-in-the-box. Whether it 
will go off or not depends on the strength of the spring and the force put 
on the lid. When one finds a malady due to thousands of causes, it makes 
one conclude that either none of them has anything whatever to do with 
it, or that each may play a minimal role, and so it would seem with the 
hysterical personality. It may take the loss of a child to unloose the 
hysterical mechanism of one, while the death of a parrot is sufficient to 
keep some women in bed a month with an attack of hysterical paraplegia. 
The aphorisms of a people reflect the aspirations of their affective 
lives. In the one to whom the phrase "Still waters run deep" applies, 
it will take a severe shock to unhinge the mechanism of affective control. 
So it is with the many small difficulties of life, by the one digested and 
forgotten, by another ever rising like the garlic of a bygone dinner and 
producing its disturbance in the affective sphere. 
The difficulty that all students of the hysteria problem have encount- 
ered is to pick out those phenomena which are of the most significance, 
and which offer the most tangible lines of explanation. The various 
theories represent these master keys, which have seemed, for their 
authors at least, to have unlocked all the doors of the hysterical crypt. 
Hysterical Character.-For didactic purposes, the symptoms of hysteria 
may be divided into two main classes. The foundation is a psychically 
abnormal character-the hysterical character, hysterical temperament- 
engrafted upon this are a variety of nervous and psychical phenomena 
due to a pathological suggestibility. Suggestibility is used here in the 
biological sense, not in the restricted sense as used by Babinski. 
The hysterical character is chameleon-like in its variations, appearing 
in all classes, in the intellectual as well as the weak-minded, and baldly 
expressed, shows remarkable similarities to the childish type of mind. 
Leaving aside the minor variations, it shows itself chiefly in emotional 
instability or liability, in its ability to be influenced readily, in negativism 
and impulsiveness, in a tendency to make sensations, a remarkable 
egotism, desire to confabulate, to fabricate, and to simulate. Seen from 
the standpoint of the psychiatrist, these mental attributes stand in the 
foreground; the neurological manifestations are dependent upon them, 
and the tendency of modern analyses of hysteria is to push the psychical 
anomalies forward. 
In the child, one finds the prototype of this character, hence some 
authors speak of hysterics as grown-up children, others, again, as educated 
savages. Students of comparative psychiatry find these features wide- 
spread in lower races. Naturally in the weak-minded one looks for 
much the same types, just as one expects to find them in those grown-up Il Y ST ERI A 
669 
individuals widely classed as degenerates or, better, as Walton suggests, 
deviates. The female represents in the main the physiological type of 
such character summaries, and Janet has made a lasting contribution 
to the subject in advocating the idea of a "defective synthesis," as a 
word picture of what occurs in those who show these traits to excess. 
From this standpoint the advent of hysterical signs in comparatively 
healthy individuals under the influence of fatigue, of old age, disease, 
intoxications, of shock, and of trauma becomes comprehensible. Here, 
again, Janet's phrase "reduction of the mental level" is illuminating. 
Under such circumstances synthesis, which has been adequate to meet 
most of the exigencies of life, breaks down-becomes dissociated by reason 
of a sinking of the mental level, and permits physiologically younger and 
simpler types of reaction to dominate conduct. The use of the concept 
subconscious seems unnecessarily confusing. 
Naturally the complete exhibition of these features is rarely seen in 
any individual case, save in the severe degenerative hysterias, and those 
well on the road to a hysterical psychosis. Practically one may encounter 
patients with well-developed hysterical symptoms, with almost complete 
absence of the features just enumerated, barring perhaps the element of 
heightened suggestibility. 
Emotional Instability.-Excessive lability of the emotional life is most 
striking. The mood is constantly changing. They are readily hurt, 
and break into tears on the slightest reproof; again, they show a passionate 
scorn and relieve themselves by violent bursts of anger. Gaiety changes 
into depression, and prolonged periods of happiness are followed by 
equally moody depressions. This changeability of mood shows itself 
in their activities. They are happy and busy or discontented and idle, 
sympathetic one moment and antipathetic the next; their mood can 
rarely be counted on. Smiles are succeeded by frowns and friends and 
relatives are at a loss to explain this change, or are constantly on their 
guard to avert its consequences. Thus many such patients develop into 
"spoiled darlings." Sydenham expressed it well: "All is caprice. They 
love without measure those whom they will soon hate without reason." 
In male hysterics particularly, one observes a greater tendency to 
prolonged depressions, which anomaly undoubtedly had its effect in 
influencing earlier writers to term these patients hypochondriacs. 
Suggestibility.-Volumes have been written analyzing the pathological 
suggestibility of hystericals and science is in practical accord in acknowl- 
edging this trait as one of the most fundamental in the genesis of this 
mental state. It is well known that many claim it to be the master key 
to the whole problem, and the description of Babinski is founded upon 
this feature alone. True pithiatism (hysteria) for Babinski consists in 
those conditions which can be brought about by suggestion and cured 
by persuasion. 
This peculiarity is to be sought in a special type of idea associations 
so well studied by Jung and Ricklin of recent years. In the foreground 
of the hysterical reaction type-for one can speak of general types only- 
are to be found more or less independent active idea complexes of great 
affect value whose development is enormously greater than in normal 670 
DISEASES OF THE NERVOUS SYSTEM 
individuals. The association test reactions are riddled through and 
through with disturbances due to these complexes, many of which are 
only slightly suppressed by apparently harmless reactions. 
Up to the present time no adequate notion of what is meant by 
suggestion and suggestibility has been presented. Fortunately, psychiatry 
had freed itself from the notion that it is due to Zeus, Juno, Apollo, or 
other deity; the middle-age witchcraft and devil possession has dis- 
appeared, that is, in so far as the name is concerned. The essential 
ideas, however, are still compelling in the minds of the populace, but 
set in phrases, such as telepathy, mind influence, absent treatment, 
and the like. It is almost a commonplace to remark that 90 per cent, 
of the populace (certain cynics put it higher) do not and cannot think 
for themselves; they must, therefore, be led by the few, and adopt the 
phrases, formulas, and ideas of the few. This simply means that practi- 
cally all people are more or less easily influenced or led by the suggestions 
of others. What is common to so many is found greatly exaggerated in 
a fewT-these are, for the most part, the hystericals, but it is only to 
certain types of suggested action that they are suggestible. Janet 
speaks of them as showing the type that leads to dissociation as a result 
of the narrowing of the field of consciousness. 
Suggestion and suggestibility pervade the entire hysteria problem 
in terms of varying vagueness that are inexhaustible. 
Hysterical Negativism.-Many studies in so-called hysterical negativism 
have concerned themselves with the graver psychosis, dementia prsecox, 
but the same mechanism is met with in the hysterical. One meets 
it in the frequent antipathic moods, when the patients are mute, or give 
only monosyllabic answers, when they refuse to eat at the table, or only 
toy with their food, but eat large quantities of sweets in their rooms, or 
steal down to the cupboard late at night. One sees it in an exaggerated 
spirit of Christian resignation and renunciation. The extreme capricious- 
ness and impulsiveness of the fully educated hysteric shows most exquisite 
negativistic phenomena.1 
Hysterical Sensationalism.-To be constantly in the limelight, be it 
for purposes of attracting attention or to invite pity, is another feature. 
Romantic accusations, sensational confabulations, self-mutilations, and 
refined theatrical attempts at suicide-hysterical dream state suicidal 
accidents are not meant here-these have their motive power in this 
desire to be the observed of all observers. Lies, slandering, disfigurations, 
and stealing are not too much to bring this about. In the present state 
of journalistic enterprise in certain countries, opportunities are not 
wanting to gratify these ambitions. The exaggerated ego of the hysteric 
has always given rise to comment from the earliest times. The attention 
is fastened pathologically on the ego, and the constant search for sensa- 
tions within the patient's own body on which to hang complaints is 
pursued with a definite pleasure and refined meditation. The slightest 
sensation is caught hold of and magnified. Somatic pains, for instance, 
are not created by the neurosis, but are merely utilized, aggravated, 
1 Schizophrenic Negativism, Nerv. and Ment. Dis. Monograph Series. HYSTERIA 
671 
exaggerated, and retained by it. Careful examination, psycho-analysis, 
as well as physical, of so-called hysterical pains will nearly always find a 
nucleus of organic foundation. Freud makes the acute observation 
that it is always the common, the most widespread pains of humanity 
that seem to be most frequently called upon to play a part in hysteria. 
The pains of others, however, excite but very little interest and fre- 
quently one sees jealousy and envy arise if others' pains are considered of 
moment in comparison. To be sick and the centre of the stage becomes 
a lifework, a theatrical apprenticeship, which with masterly tutelage by 
many physicians, usually produces a finished artist in the end. The 
negativistic phase, martyrdom, is a self-evident corollary. 
Special attention should be directed to the tendency to confabulation. 
Originating in day dreaming, in the delights of fantasy, poetic fiction 
gradually passes through the stage of unconscious warping of truth to 
deliberate falsification. The will to believe becomes pathological, and 
a facile imagination soon supplies all of the gaps of actual observation 
even if it does not supply amnesia itself. Ganser's syndrome and 
Korsakoff's psychosis afford opportunities for studying the mechanism 
of hysterical confabulations of the most pronounced types. 
Motor Disturbances.-Attacks.-The convulsive attacks have always 
attracted great attention and contributed the most striking phenomena. 
The capacity for this type of conduct has existed from the earliest days 
and it is one of the most remarkable and persistent of the features of 
hysteria. This transmission down through the ages, of precisely the 
same positions, attitudes, cries, and contortions, shows that fundamental 
human mechanisms are being played upon by the most primitive agents. 
The striking major hysteria attacks are, however, the exception rather 
than the rule, and have varied so little in their manifestations that little 
can be added to the older descriptions. The matter of absolute frequency 
is a question that cannot be resolved, since there are so many factors as 
to make generalizations hazardous. Some physicians never see any 
such phenomena. It is well known how German students maintained 
the absence of major hysteria, while the Charcot school was richest in 
observations. The studies on hysteria from Germany are triple in number 
those of France of today. Epidemics come and go, local agitations 
and general excitements give rise to the proper milieu for major hysterical 
manifestations; the possibilities seem always there in all peoples; the 
environment factors may only be lacking. The religious revivals of 
one people are considered as manifestations of major hysteria by another 
and there are plenty of contemporary movements that offer excellent 
opportunities for studying the manifestations of major hysteria. 
Statistics are notoriously unreliable in matters of this kind. Briquet, 
who made the first large enumeration, found that 72 per cent, of his 
hysterical patients (practically all women) had major hysterical attacks. 
Pitres, who has given a similar numerical summary, found 81 per cent, 
of 69 women and 22 per cent, of 31 men had major attacks. These were 
in the great Charcot times and under the influence of active imitative 
and suggestive factors. Binswanger's later figures show that 25 per 
cent, of 52 male patients and 54 per cent, of 80 female patients with 672 
DISEASES OF THE NERVOUS SYSTEM 
clearly defined hysterical symptoms suffered from convulsive attacks. 
Gotz in his 75 patients found 3 per cent, to be affected. Voss gives higher 
figures for the men, 65 per cent, of 22 men, and 57 per cent, for 101 women. 
On the surface of things Slavic and Celtic races are more often affected 
but in any such summary due recognition should always be given to the 
things compared. A polyclinic population, for example, will show 
absolutely different statistics from a clinic or hospital population, and 
conditions of education, of environment, and emotional stress have more 
relation to these manifestations than questions of race. Behind all 
such statistical summaries there is a marked lack of critique of the essential 
determining factors, and at present there are no trustworthy factors 
regarding the racial relations to hysteria as seen in major hysterical 
attacks. The Jewish race has always shown a higher incidence than 
other races, but the explanation is not apparent. Personal statistical 
studies do not confirm the usual idea so far as the frequency of major 
hysterical phenomena are concerned. 
During periods of intense excitement the curves of incidence of certain 
affections of the nervous system are bound to show marked fluctuations. 
Famine, war, local or general disturbance, financial distress-all of the 
social factors which make an impress upon the emotional nature of man, 
cause great perturbations in the incidence of major hysteria. 
With the beginning of major hysterical phenomena in an individual 
the tendency to recurrence is very strong. Data are wanting as to the 
number of attacks, but with young individuals daily, weekly or monthly 
(menstrual) attacks are not uncommon. There is a natural tendency 
for such major manifestations to gradually become less common with 
advancing years. Weeks or months and finally years will go by-even 
in untreated individuals-with few attacks and usually only the weak- 
minded or trained convulsionists remain. Other manifestations may 
grow up, constituting the interparoxysmal or postparoxysmal modes of 
the same capacity for reaction. 
Notwithstanding the persistence in type of the grand attacks there 
are countless modifications. Those accompanied by contortions may 
be estimated as among the severest. Many authors with the example 
of the epileptic convulsion in mind have sought to erect a similar march 
for the major hysterical attack. Such attempts are not in accord with 
the clinical factors, and lack of uniformity is universal. If purely general 
lines are sought the hysterical attacks may be described as showing three 
stages-a prodromal stage, one of muscular convulsions/and a poststadium. 
The prodromal stage-one of extreme multiplicity, often, though badly, 
termed as aura-is most frequently one of mental unrest, and is shown 
either by restlessness, irritability, crossness, or depression; there is 
frequently some mental as well as motor retardation and a feeling of 
general distress, or a sense of tension which may lead to crying, fits of 
anger, or moodiness. With the fuller development a certain increase 
in intensity in a more- restricted number of these phenomena takes place, 
anxiety increases, the sense of tension localizes itself more definitely, 
as clutchings at the throat, as palpitations, as the feeling that the skin 
might burst, the stomach explode, and similar feelings of tense constric- HYSTERIA 
673 
tion. Things dance before the patients' eyes, they hear sounds in the 
ears, become dizzy, and then begin to show convulsive movements. 
All of this may take a few days or a few minutes to develop; as a rule, 
the development of the earlier major attacks is longer than the later 
attacks, when, at times, apparently the slightest cause will precipitate 
an attack. In some patients the development is slow and apparently 
very quiet. They will lie very still for a long time-perhaps all day- 
in bed with the head in the pillow, and then sudden respiratory spasms, 
yawnings, passing of gas, etc., will indicate the presence of the severe 
muscular spasms. Many apparently-to-be major hysterical attacks 
only get to the period of the motor outbreak. They are all aura and 
never show the discharge phenomena. 
Convulsive Stage.-Here the usual initial phenomena are those of 
extreme muscular tension, which is tonic in character. It is most fre- 
quently a slow extension movement, the head being thrown back, the 
arms extended, fingers extended or in a tight fist, legs straight and stiff, 
and toes extended. The face, rarely mask like, gives more often the 
impression of one in a day dream or phantasy, and the general tonus of 
the whole musculature is in marked contrast to that of an epileptic 
convulsion. The position of the body often assumes certain very char- 
acteristic attitudes, and the crucifix position is extremely common, 
especially among those whose associations have been extremely devout. 
The development of the position in point of time also shows considerable 
variation. Very frequently there is a progression from the head and 
shoulders, then to the waist, and, finally, the lower extremities, of a sort 
of snake-like extension that is like a gigantic overdrawn stretch. Local 
groups of muscles may show isolated extension positions. Some attacks 
consist entirely of these sinuous movements of extension with slow 
relaxations, and then slow extensions. The ancients, and many moderns, 
always interpreted these as having a strong sexual element. 
In others the extension period is of but a few seconds' duration. Then 
the muscles relax, and a period of irregular contortional movement 
follows. It is not a true clonic spasm, the movements are too irregular, 
with rolling, tossing, kicking, and frequently screaming, with frequent 
recurrences of the tonic position, with opisthotonos, and then a recurrence 
of the rolling and individual clonic muscle movements. The tonic phases 
are much more coordinated than in those of relaxation-clownism is 
the proper application. Here a vast variety of individual muscular 
positions are manifest, boring of the head into the pillow, shaking it 
from side to side, threshing the arms backward and forward, rocking the 
hips, sudden sitting up and rocking backward and forward, or side to 
side, with hands tightly clasping the strongly flexed knees. Sometimes 
again the patient stands up and hops from foot to foot, or glides about, 
sometimes slowly and dreamily, with eyes widely opened, turned upward 
and arms extended, or suddenly breaks into a wild dance. In most of 
these cases the dramatic or theatrical character of the impersonation is 
very remarkable. The individual variations are too numerous to men- 
tion. Some of the antics of dementia pnecox (katatonic) patients have 
been described as being those of major hysteria. 
vol. v-43 674 
DISEASES OF THE NERVOUS SYSTEM 
The facial expression often shows in these various phases a similar 
changeability, sometimes devoid of any expression, again it takes on the 
character of one in great joy or hatred, or anger; it leers and squints, 
pouts and disdains. It is dramatic or ecstatic, repulsive or enticing, and 
psycho-analysis often reveals that the idea associations passing in the 
mind are such as are expressed by such facial movements. 
This period shows extreme variability not only in the form of muscular 
expression, but also in point of time. There is constant change in many 
patients, while in others monotony is the characteristic stamp. Some 
attacks come and go, others are over with in one explosion, but most 
attacks last some time-rarely less than fifteen to thirty minutes, more 
often one to two hours, frequently several hours, and in rare instances, 
days. Some hysterical dream states extend over months-if psychiatry 
is advanced enough to assure the differentiation from some abortive 
forms of dementia prsecox or certain confused manic-depressives. 
Notwithstanding the statements of many of the Charcot school that 
the major hysterical attack begins with a cry, it would appear that such 
a mode of onset is comparatively rare. It probably was an imitative 
phenomena in France when the great cultivation of hysteria was in 
progress. The hysterical cry has rarely the same sound as that of the 
initial cry of the epileptic attack. Accidents not infrequently happen in 
the stormy attacks; the tongue may be wounded and a cyanotic color 
of the lips is frequent. Not much dependence can be placed on the 
presence or absence of mucus. Absolute increase in the production of 
saliva is rare. 
Ordinary hysteria usually stops here. The patient gradually quiets 
down, the movements become less and less assertive, and a crying fit 
or a sudden scream may terminate the affair. After the attack there is 
usually a condition of great fatigue and depression. Post-paroxysmal 
mutism is a frequent sequel, and mild aphasic or paraphasic disturbances 
are frequent. Voss reports the finding of a myasthenic reaction. 
A neurological examination during a quiet stuporous interval, or post- 
paroxysmal stupor, usually reveals no marked changes. The pupils 
are usually widely dilated and react to light for the most part; Voss 
and others report slow reactions; loss of pupillary light reaction is known; 
Redlich's studies show considerable variability; sensory tests are obviously 
impossible, analgesia being usual. Signs of organic paralysis are absent, 
and the deep reflexes are not markedly altered, but the skin and mucous 
reflexes may show marked diminution. In rare instances only will 
one find the patient passing urine and fecal matter. 
Postconvulsive Period.- Dream States.-The multiplicity of paths 
along which the major hysterics will travel is enormous, but about one- 
half of them, according to Pitres, less according to Binswanger's and 
Voss's observations, will pass into a condition of lethargy, in which they 
may remain for hours, days, in some instances, weeks. These are the 
patients concerning whom one reads so much in the yellow press-the 
"living dead" and "buried alive" cases. 
These patients lie with closed eyes, immobile. The respiratory 
movements are hardly appreciable, and they often go for days without HYSTERIA 
675 
attending to the wants of nature. Careful examinations shows that 
the urine leaks away little by little in some patients; passing the feces 
in bed is also reported. In others there is a condition of more active 
delirium; they talk, coherently or incoherently, to imaginary personages 
about them, a sort of semi-dream, spoken aloud, abounding in phantasy, 
and showing on analysis much of the characteristics of a dream, at times 
a typical delirium. Some patients in hysterical dream states show almost 
all of the characteristics of a katatonic dementia prsecox, while, again, 
others behave like mildly confused manic-depressives. 
The varieties of mental disturbance which may be arbitrarily arranged 
in this third group are countless. The attack often serves as the point 
of departure for many of the interparoxysmal phenomena. The patients 
wake up, lost, as it were, to their surroundings. They may have gone 
away from home in a somnambulistic state, and have little memory for 
what has happened, or only very hazy or isolated bits of memory concern- 
ing their actions. Patient analysis may bridge over the gaps and recon- 
struct a history of the mental wanderings, but unless the most severe self- 
critique is invoked, many of these attempts will reflect the subjective 
romantic dispositions of the investigator more often than they do the 
memories of the investigated. 
It was a common experiment in the Paris hospitals in the days of 
Charcot, to press upon the ovarian region, mammary region, etc., to 
produce such major hysterical attacks, and as a result an extensive 
literature collected regarding these so-called hysterogenic zones; Charcot 
had his patients so trained that pressure on an ovary would cause an 
attack, pressure of the mamma would bring it to a close, and pressure 
on the two areas at the same time would cause no reaction. All this was 
nothing more than pure training. Scientifically one calls it suggestion; 
a great deal of it was humbug; not a malicious sort of deception, but a 
type of superabundant, uncritical faith shared in by both physician and 
patients. Hysterogenic zones there are none, if one restricts the meaning 
to the direct influence of definite peripheral irritations as capable of 
including hysterical attacks without the influence of psychical, suggestive 
factors on the part of the investigator. Again, it has been denied that 
major hysterical attacks could be consciously induced by the mere willing, 
but there is evidence that negatives this view. 
Course and Length.-No two major hysterical attacks are alike. In 
general they are prolonged, usually lasting an hour or so, occasionally 
days, while lethargic states are known to persist several weeks. Some 
attacks are as short as a minute or two. Status hystericus lasting for 
several days is described. 
Varieties of Attacks.-Kaleidoscopic varieties are to be expected. 
Monophase attacks are seen in those that show only the contortions, 
or others who fall directly into a lethargy or narcolepsy; more rarely 
mono-epileptoid phases are observed, and in these differentiation is 
difficult. Isolated cataleptic phases were frequent in the earlier descrip- 
tions; most of these are now relegated to the katatonias of the dementia 
prsecox group, although apparently true hysterical catalepsies with 
flexibilitas cerea and hallucinatory ecstatic postures are known. 676 
DISEASES OF THE NERVOUS SYSTEM 
Rhythmic Movements.- Tremors.-The full description of the innumer- 
able varieties of tremor in patients with complete or fragmentary hysteri- 
cal reactions is due to the labors of the Charcot school. The three-fold 
classification that they offer (a) trepidation, (&) vibratory, and (c) inten- 
tional tremors has the merit of simplicity even if incomplete. Simple 
tremor when the hands are at rest is extremely frequent. Static tremor, 
when the fingers are strongly extended and spread apart, is less often 
seen, while intention tremor is comparatively rare. Mixed forms are 
the rule, and the polymorphic character of the tremor is diagnostic. All 
these types of tremor are usually rhythmic, but irregular, ataxic, choreic- 
like tremors are observed. The localization is manifold, eyelids (Rosen- 
bach), shoulders, fingers, legs, tongue, mouth, etc. When involving the 
muscles of the mouth or tongue one obtains the characteristic stuttering, 
or irregular pseudo-paralytic speech disturbances. Horizontal axis 
tremors of the head may resemble early paralysis agitans tremor. 
In the hands they may be unilateral or bilateral, local or general, 
quick, vibratory tremors-8 to 12 per second, slower, 5 to 12 per second 
(pseudo-mercurial), or even slower, 4 to 5 (paralysis agitans like). From 
another point of view the tremors may be peripheral, radicular, or spino- 
segmental. The chief diagnostic feature of the hand tremors is that they 
rarely conform to a single type; they are polymorphous. Hysterical 
tremors are absent during sleep. Attention directed to these hand 
tremors and attempts at restraining them usually result in their increase, 
and sudden emotional shock usually increases them greatly. In the act 
of eating they usually diminish or disappear. Isolated attacks of tremor 
are of further interest; such may persist for a week or so. 
Intention tremor undoubtedly takes place in hysterical affections. 
It may resemble that of multiple sclerosis very closely, and is not infre- 
quently met with in traumatic cases, especially in litigated ones. The 
combination of absent tremor in the supported hanging hand with 
intention tremor in hysteria is seldom met with. It is not to be forgotten 
that true multiple sclerosis tremor is all too frequently diagnosed as 
hysterical. The coincidence in similar localization of tremor with changes 
in sensibility is diagnostically especially significant. 
Further rhythmic motor phenomena are hysterical hiccough, hysterical 
coughing, often extremely confusing, abdominal spasms, and hysterical 
shaking (shakers). Hysterical stuttering is a particularly obstinate 
type. Hysterical asthma, spastic hysterical aphonia (laryngismus 
hystericus) hysterical tachypnoea, dyspnoea, all fall within the rhythmic 
affections of the respiratory apparatus. Just which of these may be 
attributable to the influence of suggestion and which fall in the group 
of faulty diagnosis in Babinski's dismemberment time will decide. 
Coordinated Impulsive Movements.-Hysterical crying and laughing 
are among the most ordinary explosions of hysteria, occurring for the 
most part in young women about the time of puberty. The middle 
grades of the hysterical temperament are prone to this type of expression, 
and in the histories of most hysterics one reads that such emotional 
expression was common in the earliest phases. 
Binswanger offers certain diagnostic and prognostic signs concerning HYSTERIA 
677 
these attacks and the severity of the hysterical neuropsychosis in general. 
In one class one observes them after emotional shock in the absence of 
other factors. They are then to be considered as the expression of a 
pathologically emotional irritability, and the explosion is out of all 
proportion to the affect shock both with reference to its intensity and its 
duration. They betoken constitutional inferiority, usually on a definitely 
hereditary basis, particularly of the same type of disturbance in the 
ascendants. Binswanger is inclined to regard them, when symptomatic, 
of less grave importance than those of a second group in which an acquired 
or congenital neurasthenia lays the foundation. In the former group 
they represent perhaps the only disease feature of the personality, while 
in the second they seem to indicate a general instability and are more 
frequently accompanied by a more complete hysterical symptomatology. 
Other types of coordinated impulsive movement attacks are described, 
a number of which are characterized by great uniformity; grimacing, 
hopping, etc. The trend of modern investigation is to ally many of 
these cases with the milder and even severer dementia prsecox reactions. 
A characteristic failure of many of the earlier investigations of these 
movements was in not keeping the patients under observation for a 
sufficient number of years. Had this been done the development of 
dementia prsecox would have been recognized. The symptomatology 
of hysteria might have been poorer, but a more correct understanding 
of the process would have been gained. 
Arhythmic Movements.-Choreiform.-While the tremors are usually 
rhythmical, irregular, incoordinated, unwilled, and large, movements of 
the choreic type are occasionally met with, which offer particularly 
difficult diagnostic problems. This is all the more striking since combina- 
tions of hysteria with chorea are observed. Imitative choreas, which 
may attain epidemic proportions, are matters of history. Charcot 
complicated the whole question by assuming the essential relationship 
of chorea and hysteria, a view that has support only on superficial grounds. 
In all choreiform affections the important factors of infection or 
excessive growth with mental or physical fatigue should not be over- 
looked. To call a chorea hysterical in the absence of rheumatic fever is 
nonsense. Hysterical hemichorea is known. All patients with chorea 
are entitled to a rigid neurological examination, especial stress being 
laid upon the reflexes, and the more recent symptoms of involvement of 
the cerebellar and cerebrospinal systems. Spinal puncture should not 
be omitted in the severe cases. Chorea hysterica in the face of rigid 
neurological and psychiatric examination is becoming rarer and rarer. 
Myoclonus.-Here the movements are larger, involving whole, function- 
ally related muscular groups, and the contractions are rapid, tic-like 
in character. Whether there is a hysterical myoclonus is as difficult 
to decide as to assume practically all myoclonic reactions to be hysterical. 
To refer them to the tics is simply to push the psychogenic factor farther 
back in the past. This entire group of affections needs revision. 
Tetany.-The difference between a true tetany and hysterical tetany 
reaction is difficult to state. The presence of Chvostek's, Erb's, and 
Trousseau's phenomena, with the special factors of occupation and of 678 
DISEASES OF THE NERVOUS SYSTEM 
etiology, go far to establish the diagnosis of essential tetany. The test 
of calcium therapy affords further information. From the clinical 
point of view the signs known are only generally indicative; they are 
not positive, not even Erb's phenomena. In many hystericals electrical 
hyperexcitability of the muscles is marked. 
Akinetic Motor Phenomena.-Paralyses.-Weakness or loss of muscular 
power is among the most frequent of the more marked manifestations. 
There is the greatest variety, with reference both to extent and grade of 
akinesis. For many years clinical neurology was without definite 
criteria of the psychic nature of these pareses and paralyses, and it has 
been only within recent times that the essential differences between 
organic and functional paralyses have been definitely formulated. Statis- 
tically considered, about 25 per cent, of hysterics show definite akineses. 
In Briquet's series there were 125 in 405. Landouzy reports 40 paralyses 
in 370, while Pitres' figures are less than 17 in 100 with paralyses of the 
extremities. Binswanger reports 4 in 109, remarking that the paralyses 
are less frequently seen in Germany than in France. Voss, working 
largely with Russians, finds nearly as many as did Briquet, 27 in 123. 
Percentages will depend largely upon the type of clientele, and the special 
suggestive factors. Personal figures drawn from three types of clientele 
show less than 5 per cent, in what might be called private practice, 
perhaps 10 per cent, in hospital practice, and over 60 per cent, in litigation 
railroad accident work. Statistics are largely fortuitous. 
Muscular weakness, myasthenia, of varying grades is found in the 
majority of hysterics; it may remain without developing farther, or be 
but the beginning of a paralysis. Maladroit suggestions can convert 
a paresis into a paralysis with surprising facility. These myasthenias, 
which are purely of psychogenic origin, due to the sense perhaps of 
helplessness, of anxiety, of dread, fear, displeasure, pain, etc., which 
affect influenced psychical processes and exert a depressing effect upon 
the motor innervation, are to be distinguished from the neurasthenic 
myasthenias. Clinically considered, however, they are mostly hystero- 
neurasthenic in character. Difficulties in diagnosis from organic 
myasthenias are often extreme, especially in early multiple sclerosis and 
certain obscure spinal-cord lesions, small circumscribed myelitis, slowly 
developing tumors, low-grade pressure neuritis, certain intoxications, etc. 
Of the most outspoken paralyses, hemiplegia, paraplegia and mono- 
plegia are the more striking; mixed types, are also met with. 
Hemiplegia-This occurs in about one-half of the total paralyses 
(Ziehen). It may~be incomplete-diplegic, arm and leg without cranial 
nerve involvement-the commoner form-or rarely with cranial nerve 
additions, or mixed hemiplegic with the opposite arm or leg. Still more 
rare are the quadriplegias, and alternate hemiplegias are curiosities. The 
onset of the hemiplegia is nearly always acute, often accompanied by 
a sense of weakness or giddiness, and almost invariably accompanying 
an affect shock; many develop after a major hysterical attack. The 
paralysis is at once manifest, and only rarely advances slowly. Occasion- 
ally pain, nausea, and vomiting accompany the paralysis. 
Clinicially considered, these paralyses lack the signs of organic disease. HYSTERIA 
679 
The absence of involvement of the cerebral neurone is demonstrable by 
the absence of pathologically increased tendon reflexes, clonus, increased 
tonus, of Babinski's phenomena, of Oppenheim's reflex, or modification 
of Schaffer's paradoxical reflexes, of Van Gehuchten's inguinal cutaneous 
reflex, the femoral reflexes of Remak, of Grassett's, Peaucier's, Hoover's, 
and Babinski's hip-thigh, associated movement signs, etc. The integrity 
of the peripheral neurone is evidenced by the presence of knee-jerks and 
of the Achilles jerk, the absence of atrophy (until contractures may 
develop after long disuse), no nerve tenderness (unless psychogenic), 
or unaltered electrical excitability, hypotonus, trophic disturbances, etc. 
These are the usual diagnostic signs separating functional from organic 
cases, yet a review of recent literature emphasizes the need of excessive 
caution in attaching too much or too little importance to variations 
which undoubtedly exist. Thus while it is clearly realized that the 
tendon reflexes should be normal, Babinski maintains always, the knee- 
jerks are usually very active. Ankle clonus, called by some pseudo- 
clonus, has been reported by Binswanger, Sternberg, Westphal, and 
Voss. Oppenheim describes foot tremor, and Dejerine has demonstrated 
that patellar and foot clonus are exceptionally present in hysterical cases. 
Of recent years considerable discussion has centred about the possibility 
of the presence of inferior cutaneous reflexes, particularly Babinski's 
phenomena in hysteria, heretofore considered absolutely diagnostic 
of organic involvement of the pyramidal tracts. Roth, Van Gehuchten, 
and others, however, report the occasional presence of a Babinski toe 
phenomenon. Since modern conceptions of the many factors which 
may cause a Babinski toe phenomenon have been so amplified, definite 
judgment concerning this is premature. 
Involvement of the motor cranial nerves seldom occurs in hysterical 
palsy. The cases reported of oculomotor and abducens paralysis are 
extremely doubtful (Borel, Parinaud), and the differentiation between a 
paresis and a spasm is extremely difficult. The cases of ophthalmoplegia 
externa should also be judged with the idea of hysterical contractures in 
view. Hysterical ptosis is not infrequent. 
Facial palsy is of particular interest as a hysterical sign. All three 
branches may be involved, although participation of the supra-orbital 
branches is exceedingly rare. Special attention must be directed clinically 
to the danger of seeing a palsy on one side when there is in reality a 
hysterical contracture on the opposite side. 
Glossolabial involvement is reported (Remak), and considerable 
variation exists regarding the position of the tongue and the palate. 
Most of the anomalous cases seem to consist of a mixture of spasm and 
of paresis. In the hysterical aphonias the movements of the palate 
have been observed to be less ample. Inferior alternate paralyses with 
crossed facial are reported. They need to be carefully weighed (Voss). 
Laryngeal palsy may also be reckoned among the paralyses, more 
frequently bilateral than unilateral, it is rarely complete. Laryngo- 
scopically, the picture is usually negative. 
Paraplegia.-Paraplegia superior is extremely rare, while paraplegia 
of the lower extremities is more frequent, although statistics concerning 680 
DISEASES OF THE NERVOUS SYSTEM 
its occurrence are not available. Inferior paraplegia is found either as 
a typical paralysis of the lower extremities, with inability to move the 
limbs, complete or in part, or as an abasia (astasia-abasia), in which there 
is conservation of the power to move the limbs while in a horizontal 
position, but inability to use them in the vertical position. Both the 
flaccid and spasmodic types of paraplegias have their analogues in the 
hysterical paraplegias, and contractures with these paraplegias are 
comparatively often met with. Transitional types are the rule. 
Trauma, especially in young girls, or even children, is the most frequent 
exciting cause. Railway accident hysterical paraplegias are of com- 
paratively frequent occurrence. The onset is, as a rule, brusque, although 
its gradual development is observed, and Voss states that excessive tire 
of the lower extremities in anemic young girls may be considered as a 
contributory cause. The distribution is apt to be extensive, although 
restrictive movements may be preserved. The patellar reflex is apt to 
be exaggerated in both types, but the absence of the signs mentioned in 
the discussion on hemiplegia is indicative of the psychogenic nature of 
the disturbance. The functions of the bladder and rectum are usually 
normal, although several authors note involuntary defecation and urina- 
tion. Mistakes in diagnosis, or imperfect observation, are here to be 
particularly guarded against, since the symptoms from small spinal-cord 
hemorrhages are not infrequently diagnosed as hysterical. 
The distribution of the anesthesia will largely depend upon the method 
of examination. A maladroit examiner can find almost anything he 
wishes, and since the usual methods for examining for sensory disturbances 
are largely tinctured with clumsy suggestions, the beautiful figures of 
the Charcot School students are better evidences of such methods than 
scientific documents. In examining for anesthesia, the real point should 
always be guarded. The patient should never be asked if she feels, but 
should be questioned to distinguish between kinds of sensations. A 
consistent anesthesia to all the types of possible test has never been 
encountered. The classical representations show a sensory distribution 
practically coinciding with the extent of the motor loss, but a great 
variety of irregular distributions has been figured. 
Atrophy may follow contracture, but without reaction of degeneration, 
although Dubois has well shown that a diminution of both galvanic 
and faradic irritability in the majority of cases is possible. After years 
of inactivity, paraplegic contractures may be absent. 
Astasia-abasia.-This is an irregular incomplete type of paraplegic 
disturbance, first describedBlocq, in 1888, differing from true para- 
plegia briefly in that there is no paralysis or ataxia of the limbs in the 
sitting or reclining, position, but only when the patient tries to stand or 
to walk. It is undoubtedly of psychogenetic origin, and, hence, should 
be considered as a hysterical complication of/the other psychoneuroses, 
or an anxiety neurpsis^jieurasthenia, etc.'***' 
Pseudo-tabes.-Pitres has given a full analysis of this allied disturbance. 
Pains, incoordinated ataxic movements, disturbance of the eyes, analgesia, 
etc., make a differential diagnosis extremely difficult at times. It is an 
interesting fact that Charcot made a diagnosis of true tabes in a patient HYSTERIA 
681 
who went to Lourdes and recovered. Hysterical symptoms in a tabetic 
are frequent, but the diagnosis should not be difficult. 
Monoplegia.-Hysterical monoplegias are extremely frequent, and very 
irregularly distributed. Mention has been made of the paralyses of the 
third, fourth, or sixth nerves, of the facial and of the muscles of the 
pharynx and larynx. Even individual muscles of a nerve innervation 
group may be involved as in hysterical ptosis, single, or double, spastic 
or flaccid, internal or external rectus, etc., with nystagmus. Brachial 
monoplegias are perhaps the most frequent, leg monoplegias are rarer. 
Disturbances of Sensation.-A searching study of hysterical anesthesia 
is lacking. Believing it to be premature to accept in toto the dictum of 
Babinski, that there is no such thing as hysterical anesthesia, a brief 
recapitulation of the knowledge concerning these phenomena is desirable.1 
Disturbances of Touch.-These consist of anesthesia, hypesthesia, 
and hyperesthesia. Anesthesia may be general, unilateral, isolated in 
spots or patches, or involving symmetrical regional areas. Hypesthesia, 
i. e., incomplete anesthesia, is much commoner than anesthesia. Total 
general anesthesia is probably extremely rare. Pitres' statement that 
20 per cent, of the cases show it, is one of the extravagances of the Charcot 
period. Binswanger considered only six of the many reported cases to 
be valid, and Voss added six from the literature as probably authentic, 
and two of his own observation. Some of these patients showed "dream" 
state conditions, and it is difficult to estimate their value. 
Hemi-anesthesia or hemi-hypesthesia is not infrequent, although the 
usually accepted teachings of Charcot are certainly defective. The left 
side appears to be much more often involved, and considerable variation 
in intensity exists in the different portions of the affected side. Crossed 
anesthesia, i. e., face of one side, and body of opposite side, is also reported. 
Bizarre groupings, such as involvement above the waist on one side, and 
below the waist on the opposite side, are known. It is not impossible 
that some of the crossed anesthesias have been really due to minute 
thalamic lesions. Patch-like anesthesia or hypesthesia is common. The 
breast and forearm are especially involved, but the irregularity in position, 
form, size, constancy, etc., is bewildering. 
Geometrical distribution, the well-known stocking-and-glove type, 
which rarely corresponds to any anatomical nerve area, is classic. The 
border of the anesthetic area is apt to be very indefinite, not only during 
an examination, but particularly on comparison with preceding examina- 
tions. The patch-like anesthesias show a similar, if not greater, variabil- 
ity. As a rule, the patient is unaware of the anesthesia, and in but few 
cases does it cause any inconvenience. Other patients complain of pain 
(anesthesia dolorosa) or pricking, or compare the sensation to the crawling 
of ants, or the feeling of a limb that has gone to sleep. 
Anesthesia develops suddenly, as a rule, either following a major 
attack, or without such a precedent. It would appear that few old 
hysterics are free from anesthetic concomitants. The increasing oppor- 
tunity for suggestive action is here manifest. 
1 See Voss (Schmidt's Jahrbucher, February, 1909) for a critical summary of Babin- 
ski's teachings concerning anesthesia. 682 
DISEASES OF THE NERVOUS SYSTEM 
Temperature Sense-Loss of ability to distinguish heat and cold 
appears to follow the modification of tactile anesthesia, but cold nearly 
always has a striking effect upon the hysteric, especially when applied 
en masse, as by a shower bath or cold pack. A few, not truly convincing, 
cases of dissociation of temperature and tactile sense are on record, also 
cases of dissociation of warm and cold. Patients with loss of temperature 
sense rarely burn themselves, although burning in hysterical therman- 
esthesia is recorded. There are no complete studies in accord with Head's 
teachings regarding epicritic and protopathic sensibility. 
Deep Sensibility.-In a few isolated instances deep sensibility seems 
to be affected, but it does not necessarily impose its pathological corol- 
laries-ataxia, etc. Hypesthesias and hyperalgesias are more frequent 
than deep anesthesias. Dissociations with intact tactile and lost deep 
sensibility, or vice versa, are among the hysterical anomalies recorded 
that require more corroboration and more exact observation. 
Mucous Membrane.-The sensibility of the mucous membranes may be 
modified in a manner quite analogous to that already noted for the skin. 
Thus anesthesias of the mucous membrane of the mouth, nose, urethra, 
rectum, ear-drum, epiglottis, etc., are recorded. Thaon makes the state- 
ment that in one-sixth of all hysterics there is anesthesia of the epiglottis, 
yet respiration pneumonia does not occur as a result. 
Pain Sensibility.-Analgesia and hyperalgesia are both present. 
General diminution of pain sensibility, hypalgesia, is about as frequent 
as general hypesthesia, and is found under similar conditions. Absolute 
analgesia is found in a few hysterical dream ecstatic states. Hemi- 
analgesia and hemi-hypalgesia run pari passu with the hemi-anesthesia 
and hemi-hypesthesia, and crossed varieties are described. The usual 
pupillary response to painful stimuli is obtained in many of these cases, 
thus the integrity of the reflex mechanism is assured even if there may be 
repression of the psychical pain associations. 
Hyperalgesia is a common hysterical symptom. In one form or another 
it enters into almost every hysterical history. Hysterical points or 
zones of tenderness on deep pressure, usually unilateral, are extremely 
frequent. The most common of these are the iliacal, or so-called ovarian, 
pressure point, also present in men, inguinal pressure point, just above 
Poupart's ligament, epigastric point, mammary point, jugular point, 
the supra-orbital and infra-orbital points, and the vertebral points. 
There are extended discussions of the location of these points and their 
relation to the so-called hysterogenic zones. Suggestion is the important 
element, but there are certain areas where pressure with suggestion 
seems to have more influence than other zones; the same phenomena 
may be brought about, however, by other suggestive means. After all, 
the point has no real relation to the disease. The most classical points 
are usually those upon pressure of which physiological stimuli are more 
readily set up in the normal individual. Pressure upon other zones, 
or even the same point, may bring about the cessation of an attack. 
Pressure on these points may also determine localized pains, paralyses, 
contractures, ataxias, etc., and vice versa, it can be used to cause these 
symptoms to disappear. HYSTERIA 
683 
Spontaneous Pains, found in perhaps 80 per cent, of all hysterics, 
are exceedingly variable, but may be roughly classed as follows: 
(a) Topalgias or circumscribed painful areas having no anatomical 
relation to any known nerve distribution. The study of these has been 
much confused by lack of careful consideration of Head's zones of reflex 
visceral disturbance and vice versa, hysterical topalgias have been often 
considered as evidence of visceral disease. The occurrence of topalgias 
as isolated phenomena should be interpreted more as evidence of reflex 
visceral disturbance than as indicative of hysteria, and the modern 
tendency is to recognize more and more the strength of Head's position, 
that a great mass of symptoms which have been regarded as hysterical 
are in reality but the peripheral reflex disturbances due to visceral disorder 
or disease. Further, many topalgias are found in the neurasthenic. 
(&) Neuralgias.-In the earlier studies these were described as occurring 
frequently, but more careful inquiry has revealed faulty diagnosis, so 
that, at present, a neuralgia, even when apparently not conforming to 
the known anatomical distribution of a nerve trunk, should be diagnosed 
as hysterical only after careful elimination of organic features. Examina- 
tion may reveal a cervical rib to account for a persistent brachial neuralgia 
or sugar in the urine may reveal the diabetic origin of another. 
(c) Arthralgia.-Pains in the joints offer particularly difficult problems. 
Hysterical coxalgia should never be so diagnosed in the absence of a 
radiographic examination. Since the advent of such methods they have 
been much less frequently diagnosed, even when the arthralgias are 
accompanied by hypesthesia or hyperesthesia, contractures and disturb- 
ances of gait, apparently hysterical. Certain cases are so quickly relieved 
by suggestive therapeutics that the occurrence of true hysterical arthral- 
gias seems certain. 
(d) Enteralgia.-Many striking cases of visceral pains of undoubted 
psychical origin are in the literature. Mention has been made of hysteri- 
cal appendicitis, concerning which A. Dubois has written an extensive 
monograph. Hysterical ileus, with pain and fecal vomiting, is held by 
Babinski to belong to the group of "simulation," in which opinion 
Strumpell shares, but Bergman has collected the older cases, Voss the 
newer ones, and Naunyn believes it a possible hysterical condition. 
Studies on antiperistalsis by Muhsam1 seems to point to the possible 
psychogenic origin of such reversed peristalsis. Vomiting is a frequent 
hysterical symptom, and even the hyperemesis gravidarum is held by 
E. H. Muller to be of possibly psychical origin. 
(e) Cardialgias.-Binswanger described cardialgia as a frequent 
hysterical symptom. In its severest forms-angina pectoris hysterica- 
it is undoubtedly very rare, but as palpitation, sense of oppression, 
irregular pains, quick pulse, dropped beats, etc., it is frequent. Hysterical 
bradycardia has been described by Triboulet. 
In addition to the false cardiopathies of both hysterical and neuras- 
thenic origin, there are the false gastropathies, mental anorexias, false 
enteropathies, false genitopathies, etc., on which Dejerine has cast such 
1 Mitt. a. d. Grenzgebiete d. Med. u. Chir., 1900, vi. 684 
DISEASES OF THE NERVOUS SYSTEM 
a wholesome illumination. They have often been indiscriminately 
herded with the hysterical symptomatology, or with hysteroneurasthenia; 
many Are found in Janet's psychasthenic group. In their purest expres- 
sion they should be kept in a group by themselves as the false pathies. 
They frequently are complications of hysteria, and are too largely the 
product of bad medical suggestion. 
Disturbance of Vestibular Apparatus.-Dizziness of vestibular origin 
as a pure hysterical reaction is undescribed, although Frankl-Hoch wart's 
and Bouyer's cases of pseudo-Meniere's disease have been referred to 
this category, and hysterical aural vertigo has been described. Voss 
has also given an apparently clear case of aural vertigo of hysterical 
origin, operated upon without finding any organic cause. A close reading 
of these cases shows many deficiencies of examination sufficient to exclude 
them from the category of well-known but transient or even mild organic 
vestibular affections. It is better perhaps to acknowledge them as due 
to somatic causes yet unknown than to shut ones eyes to careful methods 
of examination by calling such hysterical. The possibility of the occur- 
rence of the hysterical reaction due to cerebellar (vestibular) disturbance 
is not to be overlooked. The same is true for a large number of the dizzy 
spells frequently met with. Binswanger has correctly estimated these as 
common complaints arising from a variety of transitory causes, weakness, 
tire, fatigue from eye movements, gastro-enteric disturbance, etc. 
Smell.-The percentage of normal variation in smell is not yet definitely 
established, hence the work of Lichwitz, who found complete and half- 
sided anosmias and other anomalies of smell, is not conclusive. Irregu- 
larities of smell in the non-hysteric are very commonly found by Ziehen, 
who makes a systematic investigation of smell in every case in his clinic. 
Gilles de la Tourette is inclined to regard such disturbances as of diagnostic 
value. Babinski fails to find any marked anomalies unless the methods 
of investigation are palpably suggestive. Voss finds 30 per cent, of his 
cases with anomalies. This is about the average run of variations found 
in the psychiatric clinic at Berlin for all cases. Voss finds the left side 
more often involved. 
Taste.-Variations in taste for sweet, sour, bitter are not frequent, 
but are recorded by numerous authors. Hyperagusia is especially 
pronounced. Agusia is a frequent accompaniment of the facial anes- 
thesia. Hemilateral agusia and hyperagusia are described. 
Hearing - Hysterical deafness is rare, yet recorded by Binswanger 
Habermann, Magnus, Barth, Walton, and others, yet changes in modula- 
tion of the voice, so characteristic of organic affections of the ear, are 
practically absent. Weber makes some observations to the contrary 
which are striking. Bone conduction is also modified. Voss has described 
double hysterical deafness in combination with hysterical paraplegia. 
Sight.-Double-sided blindness is' rare, and few reported cases are 
free from scepticism. Unilateral blindness is frequent. Normal eye 
grounds, unmodified pupillary reactions, acute onset under psychical 
shock are the usual diagnostic features, taken in connection with the other 
features of the hysterical personality. The test with colored glasses and 
colored word stimuli, with later association tests, reveals the fact that the HYSTERIA 
685 
patients see, and the image is not in the subconscious. It is voluntarily 
repressed. Freud's studies on psychogenic blindness are valuable.1 
The lid reflex to sudden illumination is a valuable diagnostic sign. 
The consensual light reaction has not been carefully observed. Voss 
reports a case with its diminution in a unilateral amaurosis. 
Diminution of vision as a subjective complaint is extremely common; 
it usually involves the left eye, while restriction of the visual fields has 
become a classic since the work of Fere. Babinski denies its presence 
absolutely, and claims it to be the product of maladroit suggestion. 
Such has been largely the writer's experience. Such limitations are 
described as concentric and bilateral, and are claimed for a third of all 
the cases; Voss gives 25 per cent. Increased fatigue, inability to concen- 
trate the attention, etc., are among the etiological factors. It is subject 
to great variation, and simulation and exaggerations are frequent. 
Central scotomata, sector and ring-shaped defects, are among the 
rarer anomalies on record. Disturbances in color perception are also 
on record. Achromatopsia for single colors is the most frequent, although 
apparent true color blindness is described. Seeing double with one eye 
(polyopia monocularis) was first described by Parinaud. Within recent 
years Voss reports four cases and negatives the ciliary accommodation 
cramp hypothesis in favor of its being dependent on an amaurosis. 
Ziehen suggests a hallucinatory basis, especially for Ulrich's patient, 
who saw things six times with one eye. 
Pupillary disturbances may originate from psychical influences. 
Pupillary immobility is claimed as a hysterical symptom, but most 
authors maintain that even in the severest hysterical attacks absolute 
rigidity is absent. Voss believes he has seen slowed reactions during an 
attack-attention has also been directed to this author's observation 
regarding unequal pupillary reactions in one-sided amaurosis. Spiller 
has reported irregular pupillary phenomena in a case of hysterical chorea. 
Micropsia and macropsia are rare phenomena (Janet, O. Fischer). 
Vasomotor and Trophic Disturbances.-Here may be grouped a number 
of phenomena which have excited great discussion; hysterical fever, 
changes in the skin, oedema, urticaria, with modifications of the secretions, 
and metabolic disturbances serving as the chief examples. 
Hysterical Fever.-That hysteria may, without other complicating 
factors, give rise to hyperthermia is the conclusion of Manahiloff, who, 
in a Montpellier Thesis of 1903, has gone over the entire field critically 
and thoroughly. The fever is due to a disturbance of the heat regulatory 
centres. Kausch,2 in a collected criticism, comes to the same conclusion. 
There are not wanting, however, those who contest the validity of a 
hysterical fever. Oppenheim believes in its possibility, and has recently 
reported personal observations, and in Voss' latest monograph the 
evidence is confirmatory. 
The temperature curve is not characteristic, and the fever is found 
usually only in the severest forms, especially those with major convulsive 
phenomena. The grade of rise is not great, as a rule, yet temperatures 
1 Sammi. klin. Schrifkn., iii; see also Ames, Psychoanalytic Rev., 1913, 
2 Milt. a. d. Grenz, der Med. u. Chir., 1906. 686 
DISEASES OF THE NERVOUS SYSTEM 
of 104° to 106° F. have been recorded. In certain cases with fever the 
accompanying pulse and respiratory changes have not taken place, 
while in others, dyspnoea and tachypnoea occur. Curious anomalies 
are reported by several observers, such as an axillary temperature 2.3° 
F. higher than the rectal; differences on the two sides of the body, etc. 
Such variations are known to exist in organic lesions of the nervous 
system. A diagnosis of hysterical fever should be made only after every 
possible cause of hyperthermia had been excluded. A diagnosis of 
hysteria founded on fever alone is untenable. 
Skin Changes.-Notwithstanding the excellent review of Cassirer1 
the question of the occurrence of hysterical lesions of the skin has not 
reached a unanimous conclusion. Babinski classes the usual skin erup- 
tions, bullae, gangrene, ulcers, etc., among the deceits or artefacts. There 
is no doubt that many are such, and there is further evidence to support 
the contention that hysteria may be solely a complicating factor in an 
individual with peculiar trophic skin phenomena, but there seems no 
reason for doubting the psychogenic origin of many skin lesions. 
Among the contributions to the literature of multiple gangrene of 
the skin thought to be of hysterical origin are those of Kreibich and 
Matzenauer, who observed markedly increased vasomotor irritability 
in hystericals, in some of whom the slightest skin irritation would lead 
to gangrenous necrotic ulceration. Bettmann has reported a case of 
gangrene in a hysterical individual, due to the application of an extremely 
weak lysol solution, and in many hystericals the skin may be excessively 
delicate in its reaction to external irritants. It is not a far cry to interpret 
an acceleration of a reparative process to the same hysterical type. At 
any rate, in spite of the practically unscientific aspect of the Lourdes 
" marvels," certain interesting rapid cures of ulcerous processes are worthy 
of more careful consideration than has hitherto been given them. 
Bullous dermatitis is not infrequently observed as a hysterical phenom- 
enon, while among the rarer types of so-called hysterical skin disorders 
are reckoned erythema, urticaria, dermographia, ecchymoses, chromid- 
rosis, vicarious bleedings, etc. They are to be accepted with considerable 
reserve for the vast majority of these skin changes have been artefacts. 
Dupre has suggested the term mythomania to cover a number of these 
chronic self-mutilators. Yet there remain a few cases in which fraud 
cannot be proved. Certain cases of circumaural acne, of psoriasis and of 
urticaria offer examples of auto-erotic hysterical conversions. 
(Edema.-The instructive discussion of the Paris Neurological Society 
did not settle the debated point of hysterical oedema, which was first 
noted by Sydenham, nor could any unanimity be reached concerning 
the relation of suggestion to oedema. The cases reported have been 
many, and a general classification into white and blue oedema is made, 
the former being soft, the latter hard. These come on rapidly, usually 
following an affect shock, and disappear as quickly. Pitres has made 
them disappear apparently under the influence of a magnet. They may 
be associated with sensory or motor symptoms and be distributed as a 
1 Die vasomotorisch-tropischen Neurosen, 2d edition, Berlin, 1913. HYSTERIA 
687 
hemiplegia, a paraplegia, or a monoplegia. In some instances of blue 
oedema the skin is infiltrated and pits on pressure. In other cases there 
seems to be a local vasomotor paralysis, with no infiltration and no pitting. 
Andemach1 has reported an unusually well-authenticated case, also with 
loss of the knee-jerks. The exclusively hysterical nature of these oedemas 
is not yet conclusively established; but the preponderance of evidence 
is in favor of the possibility of their psychogenic origin. 
Bleeding.-Hysterical phthisis has been, for the most part, relegated 
to the mistakes in diagnosis, as well as practically all of the gastric and 
kidney hemorrhages, but a number of observations of bleeding from the 
skin, mucous membranes, or viscera, often termed vicarious menstruation, 
etc., remain, after the deceits are exhausted, as bona fide hysterical 
phenomena. Many of these patients show a number of related vasomotor 
disturbances. The bleeding may be scanty or profuse, in which latter 
case, however, the general health does not seem affected. 
Secretory Disturbances.-The influence of hysterical emotional states 
on the secretory activities is evident, and a number of anomalies are 
recorded. The sweat glands usually functionate normally, but lack of 
perspiration, which may be unilateral, has been observed. Local hyper- 
idrosis has also been described. Under artificial conditions, such as 
medication with pilocarpine, the sweat secretion has been made symmet- 
rical, even in a hysterical individual who was evidencing unilateral 
secretory anomalies. The salivary secretion is more often diminished 
than increased. Yet a striking case of sialorrhoea has been described 
by Matthieu and Roux, in which there was a secretion of two-thirds of a 
liter daily. This case was complicated by excessive vomiting. Increased 
flow of tears or absolute dryness may be met with. Increased bronchial 
secretion, accompanied by coughing, or excessive swallowing of laryngeal 
or pharyngeal mucus, may also be noted, as well as increased nasal 
secretion with sneezing attacks. From the diagnostic side it may be 
borne in mind that unilateral and even bilateral disturbances of these 
secretions diagnosed as hysteria have in some cases developed multiple 
sclerosis, or syringomyelia. 
The urinary secretion is frequently very variable; at the present time 
one meets with fewer cases of absolute anuria than in the Charcot days, 
especially since ureteral catheterization has been made possible, yet in a 
few instances one finds relative anuria in association with hyperidrosis 
or excessive stools. Simulation is the usual explanation, for Babinski 
the sole explanation, but there are definite cases of relative anuria. 
Polyuria is more frequent. Matthieu has reported a case in which 30 
to 40 liters of urine a day were excreted. During major attacks an 
increase of urine may be expected. Incontinence is rare, but occasionally 
occurs either as a paroxysmal or interparoxysmal phenomena. In 
hysterical trance or ecstatic states attention must be directed to the 
bladder in order to avoid overdistension. 
The intestinal secretions may be profoundly modified by the mental 
state. Cannon has shown for the intestines what his predecessors have 
1 Munch, med. Woch., October 26, 1909, p. 2222. 688 
DISEASES OF THE NERVOUS SYSTEM 
shown for gastric motility and secretory activity. Diminished or increased 
gastric and intestinal secretions are among the most frequent anomalies 
in the hysterical. Hysterical diarrhoea is not infrequent, while hysterical 
constipation is very common, especially in anal erotic conversions. 
Metabolic Disturbances.-The loss of flesh in some hysterics is classic. 
They may be true hysterics or the mental anorexias of Dejerine. A 
notable example of the "human skeleton" cured in recent years at 
Lourdes belongs to this group. The loss of flesh is usually the result of 
not eating, but some patients grow thin in spite of fairly normal feeding. 
A few cases of hysterical adiposis are recorded, but their exact significance 
is doubtful. The researches of Gilles de la Tourette have shown that 
in the interparoxysmal period there are no marked deviations from the 
normal metabolism. During the attacks, however, a modification of the 
usual relationship of calcium and magnesium to the phosphates of potas- 
sium and sodium may take place, i. e., there may be an inversion of the 
formula of the phosphates. The most recent studies carried on in Bins- 
wanger's clinic have yielded no striking conclusions. 
Blood.-No generalizations of value have yet come from blood studies. 
A recent work of Schultz shows that the oft-noted anemia may be asso- 
ciated with even an increased hemoglobin content; that the red blood 
cells are usually normal, and further, that as a result of the vasomotor 
disturbances in hysteria it is necessary to take blood for examination 
from different parts of the body. The conflicting results of previous 
studies are partly explicable because of lack of attention to this detail. 
The relationship of eosinophilia to the vagotonic constitution should be 
borne in mind in the study of certain patients. 
Reflexes.-The corneal reflex, conjunctival and palpebral reflexes are 
frequently diminished. The corneal reflex is rarely absent, and the 
secretion of tears is not modified, as a rule, even in the presence of dimin- 
ished corneal and palpebral reflexes. Irregular distribution in the 
sneezing reflex is occasional. It has little diagnostic significance, and 
loss of the faucial and pharyngeal reflexes, on which much stress has 
been laid, is of minor value. They are frequently very sluggish in the 
non-hysterical, especially those who have had much treatment of the 
throat. In the hysterical, irregularities in the faucial reflex are more 
apt to be present than in the non-hysterical. Little is known of the 
jaw-jerk. 
The triceps and radial periosteal reflexes are usually increased. In 
the presence of contractures they may not be obtainable. At times 
they are lost, but their diagnostic value is minimal. Marked irregularity 
would point to organic lesions. The abdominal, epigastric, and cre- 
masteric reflexes are subject to considerable normal variation, but when 
unequal, organic affection should be searched for; mistakes occur by the 
contraction of the abdominal muscles. Even in the presence of anesthetic 
skin areas the cremasteric reflex is rarely modified. The anal reflex may 
be absent; occasionally it shows hyperesthetic sensibility in much the 
same manner as one finds in vaginismus-also a rare hysterical reflex. 
Anal hypesthesia is not infrequent, especially in women, but what relation 
it has to the anal erotics of Freud is not vet determined. HYSTERIA 
689 
The knee-jerks are usually increased, but, as a rule, symmetrically. 
Loss of the knee-jerk is undoubtedly present as a hysterical sign, although 
contested by Babinski. Many observations leave little doubt as to the 
actual occurrence of loss of knee-jerk during and after hysterical attacks. 
They are extremely rare, and may later permit of other interpretations. 
A similar position may be taken for the Achilles jerk. There is a known 
variability here. However, if tested by the kneeling method its absence 
is strongly indicative of organic lesion. In the observations of Nonne 
and Willroth, the interesting finding was a periodic loss of the tendon 
reflexes. In the interparoxysmal periods they were normal or in- 
creased. 
Psychoses.-The use of the term is to a certain extent tautological, 
since from one point of view hysteria itself is primarily a mental disorder, 
but putting aside a too strict interpretation of the word psychosis, under 
this head can be grouped a series of phenomena that bear at times a 
close relation to the somatic disturbances. 
The most striking of these profound mental alterations, and one which 
has been understood only within comparatively recent years, is the 
so-called hysterical dream state (Dammerzustand). For years it has 
been known that following an epileptic attack or during an alcoholic 
debauch a patient may show a condition of dreamy delirium, which may 
not seriously interfere with his general orientation or conduct, but during 
which ordinary normal consciousness is not operative. Similar attacks 
are known to follow major hysterical seizures, or may originate without 
any convulsive antecedents. These are of shorter or longer duration, 
persisting from a few moments to perhaps a few weeks. Occasionally 
such a dream state may be terminated by convulsive seizures. The 
most striking of these states may be observed during major hysterical 
attacks, but there are other types during which the patients seem perfectly 
quiet and contained, or they are only dazed. If consciousness is deeply 
clouded, one finds these patients in a continuous slumber, with closed 
eyelids, sunken head, and relaxed muscles. They are the "living dead" 
frequently commented on by the lay press. 
In another type one finds the patients about, even attending to their 
daily affairs, but doing them in a mechanical automatic manner, engaged, 
as it were, in a deep revery all of the time. Patients in this condition 
have taken long voyages, and on coming to themselves are at a loss to 
account for their actions. Many fugues, or flights, are due to these 
hysterical attacks. Occasionally such people commit criminal acts, 
and important and difficult medicolegal problems arise. Somnambulistic 
performances belong to this general type of phenomena, although here 
the clouding of consciousness is rarely as profound as in the dream states 
following convulsive attacks. In certain of these dream states the patient 
rehearses in his delirium occurrences that produced the initial affect 
shock. Whether the dream state be accompanied by motor restlessness, 
agitation, or by stupor and lethargy, there is usually after the attack 
complete or partial retrograde amnesia. It is rarely a complete amnesia, 
but it may be. 
A further feature of considerable importance is the occurrence of a 690 
DISEASES OF THE NERVOUS SYSTEM 
peculiar type of conduct which was first clearly set forth by Ganser, of 
1 )resden, as " Vorbeireden." Many of the patients, especially the younger 
ones, show a peculiar foolish behavior quite like young boys of from 
eight to ten years of age. They are very short and snappy in their 
answers, burst out into uncontrollable laughter, imitate the cries of 
animals, and are extremely difficult to manage. In answer to questions 
they give indirect or inverse answers, half answers, contradict themselves 
-very obviously at times, apparently not caring just what they say. 
At first sight their answers seem like deliberate attempts at falsehood 
or simulation, but the great similarity in this type of case is so striking 
as to permit the recognition of the Ganser symptom. This type of answer 
is not peculiar to hysteria; it is found in alcoholism, in epileptic dream 
states, and is very common in dementia prsecox. 
Hysterical dream states may be confused with similar dammerzustande 
of alcoholic, epileptic, or of traumatic origin. The diagnosis of a hysteri- 
cal dream state is founded on the grounds of a dreamy disturbance of 
consciousness, suggestibility, an extreme changeability of the conduct, 
on foolish, childish conduct, and the presence of somatic hysterical signs. 
Diagnosis may be almost impossible in the presence of alcoholism. 
It is of extreme medicolegal importance to recall that the motives of 
waking consciousness may be carried over into the dreamy state, and 
that criminal acts which seem to have a perfectly well-recognized motive 
may be performed in a state of consciousness in which the sick indi- 
vidual does not know the difference between right and wrong. The 
question of legal responsibility in hysteria is extremely knotty. It has 
rarely entered into the courts in the United States or England, but is 
well-recognized by Continental jurists. 
Closely related to the Ganser syndrome is that of pseudologia phantas- 
tica, or pathological liars. This condition is well studied in the mono- 
graphs of Binswanger and Voss, and particularly in the communication 
of Delbruch. Pathological hysterical swindlers have been made the 
subject of an important monographic treatment by Kraepelin.1 
Course and Prognosis.-Ziehen has coined the word "delective" to 
express the multiplex combinations of the hysterical manifestations. 
By this is meant that any symptom in the broad group may be chosen 
by the individual, and that in successive attacks the picture may be quite 
dissimilar. This renders it almost impossible to describe the course of 
this psychical reaction. • In general, it is a chronic affair, no consideration 
being given at this time to the purely symptomatic hysterical outbreaks 
which accompany other disorders. 
It has been only recently that the suspicion has become a conviction 
that many of the symptoms which mark the course of hysteria are of 
medical manufacture, and the varied groupings of the picture come into 
existence in response to suggestions on the part of the attending physician, 
nurses, or anxious friends. Psychical contagion is one of the most 
difficult features to combat in limiting the symptomatology. Too great 
solicitude that the patient should not tire herself, or exercise too much, 
1 Allg. Zeitsch.f. Psych., 1906. HYSTERIA 
691 
may be the point of departure of a paresis or paralysis. This plays a 
great part in modifying the course and in varying its manifestations. 
Although the course in ordinary hysteria may be regarded as chronic, 
much will depend upon the element of causation, the subsequent affect 
shocks, and naturally the factor of most importance, the mental founda- 
tion of the individual. In a young individual, subjected to a single 
affect shock, other things being equal, one expects a less protracted 
course with fewer variations than in an older individual subjected to the 
vicissitudes of constantly recurring causes for emotional disturbance. 
The prognosis in general, so far as the somatic signs are concerned, 
is good, but here a general rule will not apply to the individual case. 
There are certain hysterical individuals who never recover. The hysteri- 
cal constitution is so definitely established that, although the individual 
paralysis, analgesia, or anesthesia is recovered from, the patients go on 
to other manifestations. They become chronic invalids, the despair 
of all those connected with them. The severe degenerative hysterias- 
usually superior feeble-minded deviates, with hysterical complications, 
rarely ever recover. In the hysterical complications of organic disease 
one does not look for recovery. The neurasthenic hysterias present a 
hopeful prognosis if the affective incidents can be regulated and the 
struggle for existence better adjusted. 
The traumatic hysterias, barring the other factors, i. e., mental inferi- 
ority, psychopathic constitution, alcoholism, neurasthenia, etc., have a 
fairly good prognosis. The continuance of litigation, with its emotionally 
disturbing factors of expectancy, the desire to get even, and the constant 
suggestion to make one's self out as sick as possible in view of the effect 
on the jury, are the most pernicious features of the traumatic hystero- 
neurasthenias. It is not true, however, that these patients get well 
as soon as they are paid. In the long period of waiting they often 
develop a true psychotic state that takes many years to efface. 
In the type of hysteria met with in the well-bred and intelligent, the 
prognosis is fairly good in the young; with advancing years it becomes 
less hopeful. The most favorable types are those seen in childhood. 
A few cases of fatal hysteria have been reported. The exact significance 
of these cases is far from being settled. The longer a hysterical symptom 
has persisted, the more sinister the prognosis, but paraplegias, hemi- 
plegias, of three or even five years' duration, have been known to 
recover. 
Diagnosis.-If hysteria may simulate almost every known combination 
of symptoms, its diagnosis manifestly presents features of more than 
usual difficulty. Year by year, however, the analysis becomes more and 
more searching, and it is only with reference to certain conditions that a 
differential diagnosis becomes complicated. 
The Epilepsies.-In estimating the character of the major convulsive 
attacks, the epilepsies come into review, although the analogies are but 
superficial. Much has been written of the borderland states between 
these two conditions, but the more they are studied, and the more known 
of the hysterias, for both are not entities, the less real resemblance is 
found. There is really little doubt that the two conditions may be present 692 
DISEASES OF THE NERVOUS SYSTEM 
in the same individual, and further it may occur that the same pathological 
process may be the determining moment for both conditions. 
Sommer suggests a fourfold division of the complex cases: (1) Epilepsy, 
which symptomatically resembles the severe forms of hysteria; (2) 
hysteria, which symptomatically resembles genuine epilepsy; (3) epilepsies 
to which hysteria is added, as a result of the organic cause of the epilepsy; 
and (4) hysteria in which epilepsy occurs as a pure complication. The 
term hystero-epilepsy only renders the concept hazy, and is best avoided 
entirely, even in the Richer sense as a name for the major hysterics, 
although both Binswanger and Apelt believe in a hystero-epilepsy, in 
the sense of the co-existence of the two diseases. Even though it be 
known that the positive signs fail in the differential diagnosis of particu- 
larly difficult cases, yet with continued observation a diagnosis is possible 
in the sense of the Sommer divisions just quoted. 
In epilepsy the signs usually relied upon to make a diagnosis in the 
ordinary cases are loss of consciousness, complete amnesia, injury to the 
tongue or body, involuntary loss of urine or feces (the former more 
frequent), immobility of the pupils (not invariable), clonus or Babinski 
phenomena at the end of the attack (also not always), exhaustion, and 
sleep. In the hysterical attack the loss of consciousness is nearly com- 
plete, the amnesia is island-like, or may be cleared up entirely by asso- 
ciation experiments, or in hypnosis, the biting of the tongue or injury to 
the body in falling is apt to be slight, if present at all, the urine and feces 
are rarely discharged involuntarily, only in rare cases are the pupils 
immobile, and ankle clonus and the Babinski phenomena are not seen. 
After a hysterical convulsion the patient is rarely sleepy, even if fatigued. 
Meningitis Cerebrospinalis Acuta.-The occurrence of lymphocytosis 
and bacteria in the spinal fluid and exact observation of the temperature 
have disposed of most of the former pseudo-meningitis attacks. 
Organic Brain Disease.-A number of organic brain affections may be 
the exciting causes of the outbreak of typical hysterical attacks. Hemor- 
rhagic meningitis, brain tumors, cerebrospinal carcinosis, septic encephal- 
itis, syphilitic meningitis, gummata, trauma with microscopic lesions, 
are among the causes known to have occasioned true hysterical con- 
vulsive outbreaks. It is of moment to realize that a number of such 
patients with organic affections have in their early stages masqueraded 
as hysterical-and under the eyes of clinicians of high standing. 
Multiple Sclerosis.-Of all organic neurological affections this offers 
the most difficulty in differentiation from hysteria, especially in its early 
stages, and the problem is further complicated since the two disorders 
may coexist in the same patient. The regressive character of many of 
the symptoms of multiple sclerosis further complicates the picture. 
Nystagmus, ankle clonus, Babinski's sign, and temporal disk pallor when 
present speak almost indubitably for multiple sclerosis. Confusion has 
been introduced by Van Gehuchten in his reported cases of hysteria 
with the Babinski phenomena. Most patients with multiple sclerosis 
can be markedly helped by suggestive treatment. This constitutes 
another occasion for question in the diagnosis. The disturbances in 
sensibility in the two affections may be identical. The newer studies on HYSTERIA 
693 
the knee-jerks by Weiler have shown important differences, and the use 
of the association test after the methods of Jung and Ricklin throw more 
light on the psychical mechanisms and give further aid in the differentia- 
tion. When the two conditions seem to co-exist, the greatest care is 
necessary not to attach too little importance to the organic signs men- 
tioned as being extremely rare in hysteria. 
Hemi-anesthesia.-The differential diagnosis of organic lesion of the 
sensory cortex, or the posterior third of the internal capsule, is often not 
simple when the picture is one of a pure hemi-anesthesia. Here the 
search for astereognosis and for disturbances of deep sensibility must 
be made with great caution, avoiding the slightest intimation of suggestion 
in the examination. In the hemi-anesthesia of the thalamic syndrome, 
the accompanying pain, slight paresis, disturbance of deep sensibility, 
of postural sense, slight choreo-athetoid movements, increased reflexes, 
no Babinski sign, all on the same side, are the chief signs pointing to an 
organic lesion. Thalamic lesions, when small, have been diagnosed as 
hysterical hemiplegias with hemi-anesthesia, largely because of the 
absence of the Babinski phenomenon. 
Syringomyelia.-Schlesinger, in his monograph on syringomyelia, has 
called particular attention to the vasomotor disturbances occurring in 
syringomyelia when limited to the posterior horn, which may resemble 
very closely those of hysteria. Such a localization is rare but difficulties 
arise in the initial stages. 
Chorea.-Hysterical choreas offer many points of confusion. In the 
more frankly organic choreas, with spinal fluid findings and slight hypo- 
tonus, tendency to adiadokokinesis and altered plantar response, the 
diagnosis is not difficult; nor in those patients with acute infectious 
disease, notably streptococcus infections. There remain a number of 
choreiform affections that recover rapidly on suggestive treatment, 
but even they should not necessarily be classed as hysterical. Choreas 
to be considered hysterical should be accompanied by other signs of that 
psychoneurosis. 
Neurasthenia.-Ziehen has given the following rather didactic, yet 
practically useful differentiation: 
Hysteria. 
Mostly unilateral, patch-like or regional 
anesthesia, hypesthesia, and hyper- 
esthesia, and analgesia, hypalgesia, 
and hyperalgesia. 
Pressure points with hysterogenic and 
eventually also hysterophrenic charac- 
ters more marked mostly in one side, 
usually combined with hyperesthesia. 
Paralyses not unusual. Headache local- 
ized in spots, rarely a sense of pressure. 
Visual fields diminished. 
Neurasthenia. 
Sensibility intact or generally increased. 
Pressure points without such charac- 
ters, usually symmetrical and with 
intact skin sensibility. 
Nearly always abnormal tire. Head- 
ache often band-like and sense of 
pressure frequent. 
Visual fields only diminished under the 
influence of fatigue. 694 
DISEASES OF THE NERVOUS SYSTEM 
Hysteria. 
Smell, taste, and hearing often involved. 
Skin reflexes often unequally modified. 
Often typical attacks. 
Mood excessively variable. 
Intellectual activity disturbed, espe- 
cially the attention. 
Marked suggestibility. 
Sleep often excellent. 
Course polymorphous. 
Neurasthenia. 
Smell, taste, and hearing involved sym- 
metrically and usually in the sense 
of a hyperesthesia. 
Skin reflexes rarely different on two 
sides of body. 
No attacks-or rarely attack-like emo- 
tional movements set up by affect 
action, mostly depressive ideas. 
Mood irritable or hypochondriacal. 
Intellectual activity disturbed by reason 
of early fatigue. 
Suggestion influences slight. 
Sleep mostly bad. 
Course rarely polymorphous. 
Neuralgias.-The frequent attacks of pain of the hysterical are often 
confused with neuralgic pains of reflex or direct origin. The lay ideas of 
nerve distribution offer a clue to the diagnosis. 
Psychoses.-Since hysteria is accompanied by, or founded upon, a 
modification of the entire personality, it is not surprising that hysterical 
psychical symptoms should be found in the non-hysterical psychoses. 
Nissl has stated that in from 10 to 12 per cent, of the psychoses in women 
this is so. It is practically true for the manic-depressive group and in 
the initial stages of dementia prsecox, particularly those with preponder- 
ating catatonic signs. Occasionally one finds typical hysterical pictures 
in general paresis, and the hysteria of senile and presenile depressions 
is classical. The hysterical dream states are diagnosed with great 
difficulty from similar conditions in epilepsy, alcoholism, manic stupor, 
catatonia, and traumatic dream states. 
So far as manic-depressive insanity is concerned, the accompanying 
signs of divertibility, flight of ideas, of psychomotor activity and the 
press of activity are usually sufficient to make a diagnosis. Mistakes 
are common, and the injudicious advice of marriage (the panacea for 
hysteria) has been a hideous mistake in view of the subsequent develop- 
ment of a marked psychosis. 
Paresis.-Paresis should offer little difficulty. Irregular pupils, char- 
acteristic memory defect, speech disturbances, tremors, spinal puncture 
findings and the Wassermann reaction give definite signs. 
Dementia Proecox.-The entire hysteria problem centres about this 
psychosis, and diagnostic problems arise, which at the present time are 
almost impossible of solution. Thus the differentiation of a catatonic 
stupor from a hysterical dream state may be impossible. Often the 
only help lies in the history. The sudden onset, after a psychical shock, 
speaks for a hysterical dream state, while a longer and slower course is 
indicative of catatonia. The foolish conduct, negativisms, catalepsy, 
amnesia, grimaces, analgesias, etc., are common to both conditions. 695 
HYSTERIA 
A negativism that lasts for days and weeks is evidence against hysteria, 
and energetic refusal of nourishment points in the same direction. Hys- 
terics even in a dream state are less liable to soil themselves than cata- 
tonics. Unilateral pressure points and disturbances of sensibility are 
more indicative of hysteria, but not definitely so. 
A stuporous state preceded by hypochondriacal ideas, by ideas of 
influence, and changes in character in the direction of emotional stupidity, 
or disinterestedness and menstrual disturbances, points to catatonia. 
The remarkable analogy drawn by Jung1 between hysteria and dementia 
praecox explains in large part why such a difficulty arises, and the present 
author2 has attempted to show that the symptoms of the two conditions, 
although identical, are, nevertheless, representative of quite different 
stages in the synthesis and analysis of personality. As one in going up a 
mountain obtains the same view at any one spot as when coming down, 
so in the peculiar dissociations of personality in its still loose synthesis 
(hysteria) and in the dissociation of a disintegrating personality (demen- 
tia praecox) one may obtain quite similar symptom pictures. The emo- 
tional apathy of the dementia praecox patient shows a striking resemblance 
to the indifference of the hysterical. Jung has shown that in both there 
are emotional complexes, which are covered up and hidden, or repressed. 
In the hysterical the mood does not last long, but is suddenly interrupted 
by an explosion, a crying spell, muscular contortions, and the like. The 
impulsive acts of the apathetic dementia patient are similar, although 
here the protective mechanisms are harder to penetrate and the painful 
buried complex more difficult to reach by reason of the severe blocking. 
The tendency to repress the unpleasant and to bury it as deeply as 
possible, which is a healthy psychological means of adjustment, is seen 
in its diseased phases in both disorders. In the hysterical, following 
Freud's interpretation, one finds the conversions, the displacements, 
whereas in the dementia praecox cases one finds the peculiar transpositions 
and blocking, which prevents adequate reactions to reality. Although 
Jung deprecates the use of the phrases "hysterical character" and 
"dementia praecox character," yet they are useful concepts, if one does 
not push the words too far. All types of temperament may be found 
among hystericals, yet it is characteristic of these personalities that a 
powerful emotional complex is present which is incompatible with the 
ego-complex. One encounters certain embellishments on the part of 
the dementia patients which show the influence of such emotional com- 
plexes, which are also present in hystericals. It is frequently seen in 
forms of studied and pretentious behavior, aristocratic gaits, philosophical 
enthusiasms, religious originalities, etc. These are frequent expression 
of either the hysterical or dementia praecox reactions. Delusions of social 
elevation in dementia praecox frequently manifest themselves in exagger- 
ated manners, studied speech, bombastic expressions, affected eloquence, 
and high-sounding phrases. One sees in the quasi-religious schemes 
such as Christian Science, both hysterical and dementia praecox features. 
1 Physiology of Dementia Proecox, translated by Peterson and Brill, New York, 
1909. Nervous and Mental Disease Monograph Series, No. 3. 
2 Predementia Proecox, Am. Jour. Med. Sc., 1907, cxxxiv 157. 696 
DISEASES OF THE NERVOUS SYSTEM 
Treatment.-No pathological manifestation is in such urgent need of 
therapeutic individualization as hysteria, and to lay down general laws 
of treatment is to invite failure. The vast majority of patients suffering 
from the hysterical reaction can be benefited, but it requires a great nicety 
of adjustment of means to ends, and a refined tact and at times almost 
superhuman ability to choose from the various resources at hand in order 
to accomplish permanent results. Without a fairly complete insight 
into the chief etiological factors in the individual case little can be 
accomplished beyond the mere removal of some of the more striking 
symptoms. 
In no nervous disorder is prophylaxis more important and so universally 
neglected. For this latter reason it should occupy the first place in 
therapeutic considerations. It should include a knowledge of and a 
contest against all the various influences that conduce to the development 
of the hysterical character or that encourage its further development 
in a susceptible individual. 
The facts concerning heredity teach that syphilis, tuberculosis, and 
above all alcoholism form the soil in which the best hysterical products are 
raised. Accidents during childbirth are also fruitful sources for the 
development of the nervous constitution that falls a prey to the hysterical 
reactions. Thus, enlightened eugenics would avoid the marriage of 
blood relations in whom nervous affections are present, and the marriage 
of syphilitics and the tuberculous should be most rigidly scrutinized. 
Only under the best of circumstances can one minimize the real danger 
in the probable progeny of such marriages. 
The hygiene of the pregnant mother, so essential from all other points 
of view, is doubly important from that of nervous inheritance. The 
general laws concerning hygiene of the nursling and child cannot be 
entered into here. Statistics would seem to show that children nursed 
by their own mothers run less risk of developing hysterical accidents, 
yet with improved pediatric methods the application of this must not be 
too rigidly insisted on, especially when the mother's health-not her 
comfort alone-suffers from the strain of continued nursing. 
An unwise and unreasonable desire to stimulate the very young child 
is a prolific source of the nervous disposition. The nursing child should 
be left very much alone, and not made the plaything of every passing 
stranger, much less the centre of admiration of doting relatives and 
friends. Much has been written concerning the nourishment of the 
growing child, with the unfortunate result that there is a great deal of 
rampant faddism, and the boy or girl often gains that little knowledge 
which is a dangerous thing. Discussion of what the children should or 
should not eat, if at all necessary, should be carried on behind closed 
doors, and not made the tri-daily subject of admonitive conversation 
with the family at the table. The exaggerated fancies concerning what 
is digestible and what is indigestible gained by the children from such 
discussions at meal-time form the basis of a multiform hysterical dyspepsia 
in after-coming years. There is probably no single means whereby 
pernicious suggestions play havoc with the mental health of children, 
and adults as well, than the continued discussion of digestive physiology HYSTERIA 
697 
at meals. Hot biscuits and other items of diet play a minor role in 
comparison with half-baked dietetic ideas set forth by anxious parents. 
The merest common-sense notions of eating rationally and slowly are 
all that it is necessary for a child to know about its diet. If children 
do not care to eat certain things, it is extremely unwise to make a fuss 
about it. It only concentrates their minds on the implied importance 
of it. If left alone they grow out of the notions sooner than when a 
scene is made over every childish whim. Such commands fix the child's 
distastes, and prevent the free play of the important law of forgetting. 
Certain articles of food should, however, be tabooed. These are 
alcohol and all alcoholic containing liquids. Tea and coffee are not good 
for growing children, and an excessive meat diet as well as an excessive 
fat diet is harmful. A mixed diet with well-cooked carbohydrates and 
abundant fresh vegetables is desirable. Avoidance of monotony in the 
meals will give appetite and zest to eating as well as a wider experience 
that tends to correct one-sided notions concerning food. 
The mode of life of the child is in need of careful regulation, yet the 
machinery of such control must not always be on view. An orderly 
day without interruption in its specified tasks and an abundance of sleep 
are imperative in the ages up to about eight, or the ordinary beginning 
of school period. The afternoon nap for the child up to six is desirable. 
Naturally, children's parties, with rich food, candy, and the excitement of 
imitating the social whirl of their elders, are bad. Anything that over- 
stimulates the child is disadvantageous. It has been found that single 
children are much more apt to be hysterical than those growing up in a 
large family, probably because the hotbed culture due to constant contact 
with older minds often develops immature fruit, expecially if the younger 
mind is overstimulated or is spoiled by excessive indulgence. It is also 
a striking fact that a single brother in a large family of sisters comes out 
a good candidate for the hysterical reaction. 
The young child of nervous organization will need special physical 
and mental training. Gymnastics and games in the open are advantage- 
ous; hydrotherapy is useful if not overdone. The daily cold plunge is a 
useful tonic, but such methods may be very readily carried to extremes. 
The child's enjoyment of such procedures is a better guide to their useful- 
ness than a rigid scheme of "hardening" the children. Summer sea baths 
or lake baths in the open give the ideal hydrotherapy. 
Play is much vaunted, but it is in need of careful scrutiny. As a rule, 
the childish mind needs little stimulation of the imaginative faculties. 
The child lives in a sort of disconnected dream most of the time, and 
what is needed is not forcing in this quality, but direction of it. Just 
how this direction shall be carried out in the individual case must be left 
to the tactful mother. If she be hysterical, the most difficult problems 
arise, for then one has both heredity and constant suggestion to battle 
against. Under such conditions most children do better away from home, 
unless the mother has gained by hard experience and careful study the 
lessons that help her to remedy her own anomalies of inheritance or 
defects of bad training. 
The factor of suggestion is practically identical with that of imitation. 698 
DISEASES OF THE NERVOUS SYSTEM 
Here enter both the advantages and the dangers in the bringing up of 
the children. The child imitates its surroundings and adapts its imita- 
tions in accord more or less with its primary inherited likes and dislikes. 
These are, however, not immutable, and are made so only by the constant 
imitation of the parents. The children profit or suffer from the daily 
examples set before them, and by influencing the parents to the soundest 
methods of training one arrives at a scientific pedagogy that can eliminate 
the most pernicious hysterical weeds that a bad heredity can sow. The 
ability on the part of parents to be cheerful under all circumstances; to 
bear petty annoyances of life with equanimity; to keep their worries 
and angers to themselves, is a constant stimulus in the right direction 
for developing children. 
The school question soon obtrudes itself, and here a host of difficulties 
arise, concerning which unanimity of opinion is obviously lacking. Too 
often one regulates the going to school by chronology, but experience 
teaches that no two minds are alike, and that there is a physiological 
age that may be different from a chronological age. In the mental 
sphere this is very striking, and it is sound pedagogy to attempt to teach 
a child this or that not according to his years or months or days, but 
according to his individual development. It is for this reason, if no 
other, that parents should know the teachers in a school, if outside 
schooling seems to offer the best resources, and a constant interchange 
of thought should take place between the school and the home. 
In connection with the education of the hysterically susceptible child, 
particular attention should be directed to the emotional life. Here is 
the weak point in the hysterical character-the lack of emotional control; 
and especial emphasis should be laid upon the training of this pathological 
lability of the affective life. Children should not be overindulged; their 
minds should be directed from their little aches and pains; they must 
understand what danger is without fearing it. They should know what 
hunger and cold are, and learn to disregard both. The observance of 
the wishes of others, and the need for accommodating their desires to 
the interests of others should be tactfully taught. Simply telling children 
these things is not real education. The opposing affective states must 
be called up, and thus self-control taught. The process of physical 
hardening is of no value if the emotional self-control is not acquired at 
the same time; fortunately the necessity for action and precision required 
in outdoor games, or indoor athletics, is a most helpful aid in acquiring 
control over the activities of the sympathetic nervous system, which is 
the most important link in the emotional chain. A proper sympathetic 
nervous system hygiene does not overlook the importance of regularity 
in defecation and urination. 
A close control of imaginative literature is essential. Highly exciting 
tales are to be excluded, but they are rendered obnoxious, as a rule, by an 
early cultivation of the young boy's or girl's ideas of style in language and 
good writing, as the defects soon become so obvious that such kind of 
writing fails to attract by reason of its vulgarity or poor style. Hysterical 
adults would do well to avoid reading works that stimulate day dreaming, 
castle building, and especially the erotic. HYSTERIA 
699 
The young boy or girl should be encouraged to build, to dig, to carpen- 
ter, to swim, to skate, to ride horseback, and works of phantasy should 
be kept in the background. Much of the handiwork done by girls is 
bad, especially that which can be done mechanically, as it encourages 
day dreaming. If the stream of consciousness be directed in productive 
lines there can be no objection to such work. The cultivation of efficiency 
and productivity are in direct antithesis to the dreamy, hazy, inefficient, 
slipshod features of the hysterical character.1 
Isolation and solitary pre-occupation are bad features for the predis- 
posed individual, hence the question of the child's companions should be 
made a matter of special moment by the parents. Leaving these matters 
to adjust themselves by chance is usually a lazy method, yet it may be 
less harmful than the setting up of hypothetical, overdrawn, and impos- 
sible ideals. The real rub with companions, whereby the child must 
make the necessary adjustments and find itself, is more helpful if the 
wise parent sees the faults in adjustment and will take the trouble to 
work on them, than when it tries to seek an impossible degree of perfec- 
tion in the environment. Young children who are wrapped in cotton 
wool invariably suffer from this fancied protection. Hence good public 
schools and the large private schools are often much preferable to the 
smaller schools, where, unfortunately, much snobbery is rampant and 
special privileges are granted. These schools may not harm the normal 
child, many of them are highly desirable for special training, but they 
should not be selected for the child with hysterical, syphilitic or alcoholic 
antecedents, or for those who show the development of great emotional 
instability. The so-called "tender" or "sensitive" child must not be 
handled too tenderly, nor yet be left absolutely to its own resources. 
The avoidance of sexual factors even for the young child is a sine qua 
non. In practice, however, it is much more difficult than in theory. 
For this reason nature study has an added claim for recognition. It not 
only brings the young bodies into the open air, but also brings the mind 
into contact with real things and actual forces. Their sexual education 
thus becomes natural and gradual, and sexual ideas are less liable to be 
distorted as the period of puberty comes on. 
Special difficulties arise in the training of children who show a marked 
tendency to morbid anxiety, fears, and self-consciousness. Those who 
are afraid of being left with strangers, of sleeping in the dark, of going 
into the woods alone, of doing anything alone for the first time, are rarely 
helped by harsh measures. Such methods only tend to suppress their 
expression and force the children into expedients of lying and even 
hysterical conversions. Supposed headaches or other physical ailments 
are complained of in order to avoid exposure to disagreeable experi- 
ences, and thus the patient is forced into a hysterical defence re- 
action. 
The principle of prophylaxis continues just as operative in the years 
after puberty as before, although the personality becomes more fixed and 
is modified with greater difficulty, but at no time can it be said that one's 
1 See Payot, Education of the Will, Funk-Wagnall, 1909. 700 
DISEASES OF THE NERVOUS SYSTEM 
ideas become so firmly settled as to be incapable of some change, at least 
if only the correct method of approach be found. 
Etiological Therapy.-Emphasis has been laid upon the fact that in 
many individuals possessed of varying degrees of the hysterical constitu- 
tion physical factors may play an important part in determining a break- 
down in resistance, and the development of hysterical symptoms. Many 
of these individuals are not really hysterical in the narrow sense of the 
word. They possess only that common fund of hysterical reactions with 
which all are born. With them, however, the stress is too much by 
reason of an added physical or social burden. To ascertain just what 
this is, and to relieve it is the problem of the attending physician. The 
diagnosis is often not simple, since considerable doubt may arise as to 
whether the physical disorder is primary or secondary. 
The organs of generation in women call for special scrutiny, and 
although disturbances here rarely have any relation to the hysterical 
condition, nevertheless, in those with such a predisposition gynecological 
disorders may break down an acquired resistance to hysterical manifes- 
tations. Even in the average woman pelvic disease may bring about 
hysterical outbreaks. Under any condition grave gynecological disorders 
require proper treatment. 
It should not be overlooked that unnecessary gynecological interference 
in the typical hysterical approaches the criminal, or that major gyneco- 
logical operations are a frequent and direct cause of hysterical break- 
downs. The gynecologist has a stony path to travel in the hysteria 
domain. Attention might be directed here to the clinical group that 
Dupre had dubbed "mythomanias," since so many of them have close 
hysterical relationships. Many of these patients are obsessed with the 
idea of operation, and invent symptoms in order to gain their point. 
In hysterical virgins gynecological interference, barring definite and 
precise indications, is to be avoided. It almost invariably aggravates 
the condition, and by suggestion may become the nucleus of a "mytho- 
manic" obsession. Too much stress cannot be laid upon this subject, 
for there is a great amount of harm done by gynecological tinkering. 
Another consideration with even less to recommend it is that of gastric 
disturbance. Of all hysterical manifestations, those of the stomach 
are among the most common, and numerous are the attempts made to 
influence the mental state by way of the gastro-intestinal canal; and, 
strictly speaking, without results. The good results are obtained through 
suggestion alone, and the bad results, also due to suggestion, are legion 
in number. Gastrotherapy, with its faddy dietetics-speaking only 
from the hysteria standpoint, be it understood-has contributed more to 
the actual production of hystero-neurasthenic stomach disorders than 
almost any other form of bad medical suggestion. This may seem an 
extreme position, but a clarified judgment forces one into it. 
One special etiological factor demands an emphatic expression. It 
concerns the sexual life, and more particularly marriage. The hoary 
age of that etiological factor known as the unsatisfied uterine longing, 
and its supposed remedy, marriage, both of which are still popular 
serve to show how extremely subtle the gradations are in the type of 701 
HYSTERIA 
disorder under discussion. Its justification seems to rest on the universal 
fact that flighty young people settle down after marriage. But there is a 
great difference, from the standpoint of psychiatry, between the hysterical 
constitution and the irregular or often so-called hysterical conduct of 
young people, women particularly. Marriage does not cure hysteria, 
and whatever judgment may be reached with reference to Freud's ideas 
of juvenile sexual traumata as a cause of hysteria, it is certain that 
marriage on general grounds is dangerous therapeutic counsel for the 
hysteric. The solution of the problem of a contrary position, should 
marriage be denied the hysteric, is also difficult. Certain writers take 
the position that the physician should not advise under these conditions; 
he should simply state the situation objectively and let the family 
decide for themselves. As an actual matter of fact, the specialist is 
called upon to advise, but his advice should be given in the form of a 
free discussion after all the facts of the case are available-the personality 
of the contracting parties, their heredity, social opportunities, and attitude 
toward, and capacity for, meeting their responsibilities. 
Treatment of Developed Hysteria.-Psychotherapy expresses in a word 
the most profitable mode of treatment. But the word conveys but a 
very inadequate idea of what is meant by the general notion. It here 
means all those mental methods, with or without physical adjuncts, 
whereby the personality of the individual may be influenced to a healthier 
degree of self-control. Psychotherapy includes all mental influences, 
from the use of the cheapest charlatanism to that of the highest pragmatic 
philosophies. That which may be accomplished by a cheap trick for 
the ignorant coal heaver may have to be worked out with much labor 
by means of the most tactful dialetics with an educated college professor. 
A word, a command, may relieve a child of a hysterical paralysis, but it 
may require years of careful psycho-analysis to eradicate the same in a 
woman of intellectual and refined cultivation. The use of the command 
and "harsh method" for the latter would be as ridiculous as the psycho- 
analytical method in the former. 
Psychotherapeutics is so wide that it is no wonder that it finds its 
practitioners in all spheres of life. Such have come into existence as a 
more or less direct outgrowth of the social millieu, and their teachings 
are more or less adapted to individual needs and ignorance. General 
laws are inapplicable, but there must be a fundamental principle of 
moral reeducation in order to obtain permanent results. One may 
have to run the gamut of bullying, and cajoling, of hypnotic hocus pocus, 
and appeal to social or religious prejudices; one must learn to play upon 
conceit and vanity, on love of family pride and desire for social prestige; 
the entire armamentarium of suggestive influences will be found necessary 
if one would conquer all cases, and the man is rare who can command 
them all. Hence in practice one falls back upon his or her natural bent, 
and invariably develops a one-sided or many-sided psychotherapy 
according to his natural endowment and acquired method. 
Such positive psychotherapeutic treatment falls naturally into a few 
large groups, for the exclusive use of which one finds many special pleaders. 
The most important of these may be conveniently grouped as hypnosis, 702 
DISEASES OF TH FI NERVOUS SYSTEM 
suggestion, and reeducation. In using these categories it is evident that 
they are not to be understood as different things or as mutually exclusive, 
nor as exhausting the subdivisions of the subject. 
Hypnosis.-The concept of hypnosis has changed considerably since 
the early days of Liebault, Charcot, and Bernheim, yet the word is still 
frequently understood in the sense of an appeal to the miraculous, the 
superhuman, or the subconscious. Under its modern definition as only 
a form of suggestion it loses much of its significance. As used here it is 
understood as a mode of impressing certain ideas on an individual's 
mind after having, by trick methods, induced a condition of modified 
consciousness, known as hypnotic sleep, the hypnoidal state, etc., not to 
mention the numberless variants insisted upon by various students. 
First, as to its applicability in hysteria, it is generally known that some 
are refractory. They cannot be hypnotized; they usually represent a 
higher level of intelligence than those who may be hypnotized. In such 
its efficacy is nil. Among those who are most readily hypnotized one 
finds the weak-minded, the mental inferiors of modern German classifica- 
tion. The appeal to the marvellous and the apparently supernatural 
has a great hold upon these, but since their hysteria is due to their real 
constitutional mental inferiority, the hypnotic suggestions are of very 
little permanent value; they simply reinforce and repeat the type of 
suggestibility that is an essential feature of the disorder. 
Another type, how numerous they are it is difficult to decide, responds 
to hypnotic suggestions, not on a basis of weak-mindedness, at least not 
in the sense of general averages, but rather as a pure expression of the 
hysterical personality. To obtain a foothold with these patients it 
may be necessary to start them with hypnosis, but with hypnosis alone 
one rarely cures a severe hysteria. It may be of advantage as an entering 
wedge, but if continued it only perpetuates the type of reaction we are 
trying to eradicate. Hypnosis seems to make some startling cures, but 
alone it does not modify; in fact, it renders the hysterical personality 
more susceptible. It therefore does harm as an exclusive mode of 
treatment. One is making real progress in the treatment of a hysteric 
when the patient has learned to be uninfluenced by hypnotic passes. 
In the hands of the unscrupulous or the unpractised a great deal of 
damage can be done. The fancied influence for absolute evil that 
hypnotists are believed to exert on their subjects can in reality be accom- 
plished only in the weak-minded. Such individuals do not need any 
pressing of hands or looking into mirrors to produce their lack of will. 
It is a striking commentary on the value of hypnosis in the treatment 
of hysteria to find it practically rejected by its warmest advocates of 
twenty years ago. With the limitation thus outlined, however, it will 
always persist as a useful adjunct in the beginning stages of treatment for 
some patients. 
Suggestion.-A definition of suggestion as used in psychotherapy is as 
unsatisfactory as that of hypnosis; and the difficulties have become 
more manifest since Babinski uttered his terse definition of hysteria 
as "that series of phenomena that can be produced by suggestion and 
cured by persuasion." In 1891 Bernheim claimed that suggestion could HYSTERIA 
703 
do away with the hemi-anesthesia of a hemorrhage in the optic thalamus; 
it could cure a multiple sclerosis, or overcome the palsy of a lead neuritis. 
Such a suggestive faith could move mountains, but it has no such import 
in the present section. On the other hand, there is the idea of suggestion 
which says that it is "some kind of an idea that enters into the mind 
in some sort of a manner," as Janet puts it. Such a use reduces it to 
a level with credulity, or belief, or unreasoning faith. Babinski has 
defined his own use of the word as " suggestion should express the action 
by which one endeavors to make another accept or realize an idea which 
is manifestly unreasonable, and that persuasion ought to be applied to 
ideas that are reasonable or which, at least, are not in opposition to good 
sense." Suggestion thus becomes a one-sided mechanism; it can bring 
about hysterical phenomena, but can do nothing to relieve them. 
For our restricted purposes of psychotherapy, suggestion consists in 
bringing about an affect state by influences, the import of which are not 
apparent to the individual. It consists in inducing mental associations 
leading to the modifications of the patient's emotional and, therefore, 
psychical state that will cause actions which make for a better adjustment. 
Suggestions enter consciousness, either perceived on the threshold of 
consciousness, or on its margin, awaken ideas, associations, and in a 
manner similar to an endless chain bring about what Cajal has well 
termed "avalanche" action, which has a compelling force on the individ- 
ual, who may be, and usually is, unmindful of the origin of the influences. 
The wise suggestor knows how, by little hints and side remarks, by appeal 
to fear, to jealousy, or to praise, to cause a summation of impulses which 
have an impelling force far exceeding that of a command. Such sugges- 
tions may thus have a very extended action, they may govern the activity 
of the glands of the heart or vasomotors, they may split definite idea 
complexes, and may so affect the senses that illusions and positive and 
negative hallucinations may take place. 
Suggestion used in this sense, then, is different from command or 
direct action, and it also differs from persuasion. A loose application 
of the word suggestion confuses these three procedures. Perhaps, after 
all, the distinctions are superfluous in practice, and the resourceful 
therapeutist makes use of all three. One may command a monobrachial 
hemiplegic to raise his arm; if uttered suddenly and in emphatic tones, 
success may crown the effort. One may urge and urge a patient day by 
day to walk, telling him he is getting stronger and stronger and will 
soon be well; this is persuasion or mediate suggestion. The hysterical 
bedridden paraplegic may suddenly get up and run in response to the 
cry of a child who is in danger; this is suggestion (indirect suggestion). 
The child's cry, its helplessness, the need for relief, make a continual 
emotional appeal of such power that the forces of inhibition are stam- 
peded, as it were, and effective action results. By casually commenting 
on the erect carriage, and graceful, easy walk of a fellow patient, an 
astasic-abasic may be immensely helped, provided the praise and com- 
mendation be wisely apportioned and carefully administered. The 
vanity, the desire for praise, the egotism, the ideals of a patient must 
be correctly estimated in order to bring about corrective suggestions. 704 
DISEASES OF THE NERVOUS SYSTEM 
Such suggestive treatment is usually combined with a purposeful neglect 
of the chief appearance in the disease, i. e., so far as direct attention 
or questioning is concerned, and later, persuasion or complete discussion 
of the disorder can be added to reinforce the advance started by the 
suggestive ideas. 
Command, suggestion, or persuasion are only symptomatic remedies; 
they do not change the chief factor which is responsible, namely, the 
hysterical character. Following the opening which they afford, a more 
fundamental method is necessary in order to permanently modify the 
personality and to make a recurrence of the manifestation less probable 
or impossible. 
Reeducation.-This is the ideal psychotherapeutic goal. It aims to 
reconstruct the individual on a firm basis of reasonable and helpfid, 
philosophy that permits him to understand his weakness and his strength, 
his limitations and his powers. Mills1 has well summarized a part of its 
aims in saying: "This method contemplates teaching the patient what 
he has, what he has not, what he seems to have, what he can do, what 
he cannot do, and what he simply believes he cannot do." But it is 
more than this. It concerns itself less with the patient's illness than it 
does with the steady cultivation within him of that individual mental 
and moral stability that makes true men and women, and not molluscs. 
The exposition of the aims to be accomplished needs a short summary 
of the methods to supplement it. In a quick review of the individual 
patients, one is struck at once with the fact that they are very dissimilar 
and require quite different treatment. One recognizes that for the 
imbecile and weak-minded types, for whom perhaps only a cure of 
symptoms can be hoped for, hypnotism may be justifiable. An appeal 
to the miraculous relieves for a time, but the patients usually go on their 
way with ever-changing symptoms. Reeducation for such is a waste 
of effort, of time, and may squander a pittance which might better be 
utilized to keep the natient isolated and away from those particular 
parasites who feed upon the credulity of this type. The manifold fads, 
cults, and quasi-religions derive their followers and endowments largely 
from this class. 
Granted a normal mental endowment in a comparatively young 
individual, one has a number of expedients. If neurasthenic factors 
appear large in the history, an isolation technique is advisable. Known 
under various names, it matters little who first invented it, but certainly 
Weir Mitchell did much to give it definiteness and point, nor did he 
neglect the reeducation features. The patient should be isolated, 
separated from home and family, preferably in a suitable hospital room 
or ward, or private house. The choice of a suitable nurse is of paramount 
importance. One's chances of success, in the vast majority of cases, 
depend more on the cooperation of the physician and nurse than on the 
particular methods pursued. 
The daily regime must be rigid or pliable according to the case. The 
majority need a rigid schedule, the very strictness is a part of their 
1 Monthly Cyclopedia and Medical Bulletin, July, 1908. HYSTERIA 
705 
moral rehabilitation. For food one commences with milk, three or four 
quarts daily-keeping close watch on the weekly body weight, then 
rapidly rising to five, six, or more quarts, as indicated by the character 
of the stools and the digestion. The patient is not permitted to see 
anyone; she cannot write or read, but must lie in bed. The physician's 
visits must be frequent, and must be made an event in the day. One 
must bring to the sick room each and every time some new mental 
pabulum which can be worked over by the patient. It may be a chapter 
from Job, a fragment of natural history, a bit of vital human experience, 
a thought from Epictetus, perhaps a problem of conduct to be solved- 
something that remotely or directly bears on the subject in view, i. e., 
the gaining of a new and healthy point of view of life, and the stimulation 
of moral fibre. The nurse throughout must have the same point of 
attack-not obtrusively so-and above all a cheap and boisterous opti- 
mism is to be suppressed. 
Little by little, as the weight increases, reading may be introduced, 
but the books read need care of selection. One makes a great mistake 
in advising a book one does not know well. It may contain ideas that 
have a special affinity for the intense emotional complexes which are 
closely related to the patient's hysterical reactions. After four, five, 
or six weeks the patient may be permitted more latitude, both with 
reference to meals and associations with the outside world. This tech- 
nique is to be found in many special manuals as worked out by hours, 
minutes, and seconds. To follow such too rigidly for all cases would be 
an absurdity. A regular schedule should be devised for each patient. 
In addition to the general psychotherapeutic talks, which should not 
leave out of sight the emotional training as well as the more narrow 
intellectual, special days should be set aside for the discussion of some of 
the more intensely colored emotional features of the patient's history. 
In certain types of patients this may have to take on the character of a 
prolonged psycho-analysis in the sense elaborated by Breur and Freud. 
The tactics of these conversations require individual adjustment, and 
are a purely personal acquirement. Its principles may be reduced to 
writing, but its practice is acquired only by force of natural endowment 
and studied skill. The refined dialecticism of a Dubois, the charm of 
personality and resource of a Weir Mitchell, the hearty emotional appeal 
and camaraderie of a Dejerine, are individual expressions of great masters 
in their particular line of therapeutic resource. 
The use of religious ideas and ideals in psychotherapy calls for special 
mention. Since religion is an expression of the emotional response of the 
individual to the most ancient of all suggestive factors-namely, the 
riddle of existence and the hereafter-it is not surprising that it should 
play an immense role in the affect of the individual. The evolution of 
the customs and usages of the priestly class symbolizes the expression 
of this emotionalism and thus sways it. Religious belief and its founda- 
tions should never be lost sight of in an enlightened psychotherapy, and 
the use of religious motives, of clericals themselves, is often of inestimable 
service. To the medical psychotherapeutist, however, trained as he 
alone is trained to properl v estimate the etiological factors, physical, 706 
DISEASES OF THE NERVOUS SYSTEM 
mental, and moral, which cannot be understood without a firm grasp of 
biological medicine, it is a sad sight to see the pitiful reversion in modern 
times to the pre-Hippocratic priestly mode of treatment of hysteria and 
kindred psychoneuroses. Emmanuelism, Christian Science, Mental 
Healing, and the like are but faint and futile grasps at the large truths 
that medicine has made its own in the progress of twenty-five centuries. 
The successes of such practitioners are unfortunately offset by the 
manufactured hysterical products which such systems produce. But the 
practitioner of medicine is not free from responsibility in this respect. 
Hydrotherapy, electrotherapy, pharmacotherapy, physicotherapy are 
necessary adjuncts, according to etiological requirements, but must be 
considered in the light of more or less temporary assistance. As exclusive 
mediums for suggestive influences they fall short of the ultimate aims 
of a complete psychotherapy; but as rational means to give physical 
tone, muscular activity, to correct disturbances of visceral adjustments, 
etc., they are necessary. The opium and hyoscyamus narcotics, analgesic 
antipyretics, and alcohol hypnotics are to be given with great caution, 
and never over any extended period of time. Iron, arsenic, and strych- 
nine are not specifics, but are useful tonics. The salts of the alkaline 
earths, usually in combination with the phosphates, are indicated as 
quieting an excessive muscular irritability. 
Valerian and specified drugs have no specific value, but it is not impos- 
sible to find combinations of the volatile oil class of drugs that have very 
definite tonic action on a dilapidated vegetative nervous system, and 
as such are symptomatically valuable. The search for remedies having 
definite and selective action on the sympathetic nervous centres, on the 
cord, or their representatives in the cortex has only just begun. The 
treatment of hysterical delirium may require the bromide hypnotics- 
it is only under rare circumstances that hyoscine is necessary. Paral- 
dehyde is, by reason of its extremely unpleasant taste, one of the most 
useful of the alcohol hypnotics. 
Analytical or Cathartic Method.1-No therapeutic consideration of 
hysteria can claim completeness without definite mention of the method 
originally termed the cathartic method by Breur, and later amplified by 
Freud as the analytical method. In its rough form it may be termed the 
"talking it out" procedure in psychotherapy, and the usefulness of the 
confesssional in church practice is due, in large part, to the same principles. 
Freud's claim that the analytical method is one which acts most pene- 
tratingly and carries farthest is undoubtedly well justified, but unquestion- 
ably its application has its limitations. Although at first practised with 
partial hypnotic addenda, Freud now practically abandons such methods. 
He quotes Leonardo da Vinci as saying " that the art of painting consists 
in placing little heaps of paint on uncolored canvas where before there 
have been none, while sculpturing, on the other hand, takes away from 
the stone as much as covers the surface of the statue contained therein." 
The method of suggestion acts like the former, analysis like the latter. 
Freud's technique has slowly evolved, and a presentation that is 
1 See Jones, Article on Psychoneuroses in Modern Treatment of Nervous and Mental 
Diseases, Ed. by White and Jolliffe, vol. i. HYSTERIA 
707 
valid for the present may change in the near future. His early procedure 
was to place the patient in the recumbent position, the physician sitting 
behind the patient's head at the end of the lounge. The physician thus 
remains practically out of sight of the patient, who is then asked to give 
a detailed account of his troubles, and to say everything that comes 
to the mind irrespective of its seeming logic or sense and apart from 
disturbing, mortifying, or unnice suggestions. In all such histories 
gaps are inevitable. These the patient is urged to fill in by thinking 
closely of the attendant circumstances, speaking aloud all of the flitting 
thoughts that pass during this search ("free association"). All the 
thoughts are requested to be uttered, notwithstanding their disagreeable 
nature. The patient must exercise no critique and remain passive. 
It will be found that the disagreeable thoughts are pushed back with 
the greatest resistance. This is made all the more striking since the 
hysterical reaction, i. e., the symptom, is the symbolic expression of the 
realization of a repressed wish and gives the patient some gratification. 
A great effort is made to retain the symptom, especially as its origin 
is not really perceived, and since it represents, in symbol, the individual's 
former conscious strivings. In psycho-analysis one attempts to overcome 
all of these resistances and by a series of judicious and tactful probings 
reconduct into the patient's consciousness the hidden thoughts which 
underlie these symptoms. Evtry symptom has some meaning; behind 
it there lies some associated mechanism, the origin of which the patient 
unconsciously or partly consciously represses. In the psychoneurotic 
symbol may be read the cryptic expression of the original thought driven 
back and hidden. 
To slowly analyze and pick apart the mechanism is the object of the 
analytical method. One needs not only special tact for such excursions 
into the subtleties of the mental life of some individuals, but also a 
developed method of interpretation. Every act, every symbolic expres- 
sion or action, lapse in speech, mannerism, needs to be carefully noted 
and its bearing coordinated. Freud lays particular emphasis on the 
analysis of dreams, since he believes that in the dream the unconscious, 
or the "repressed conscious," is more apt to reveal itself. Hence a 
careful reading of Freud's Interpretation of Dreams1 is of the greatest 
value in this study, also his Psychopathology of Every-day Life as well 
as the Three Contributions to the Sexual Theory.2 Hitchmann- 
Freud's theories of the neuroses is invaluable.3 In his work on dreams 
he has developed to the full the chief directions along which his mind 
has travelled in the psycho-analytical method. 
It is of the utmost importance to trace back into the earliest years the 
striking emotional influences that have come into experience, for, for 
Freud, the hysterical reaction consists in a perverted type of reaction 
to these experiences. As is known, the blurring, or loss of an emotional 
influence-an affect, in short-is due to a number of factors. In normal 
life forgetting is the commonest type of a corrective adaptation, and 
1 Macmillan Company, New York, 1913; tr. by Brill. 
2 Tr. by Brill, Nervous and Menial Disease Monograph Series, No. 7. 
3 Tr. by Payne, same series, No. 7. 708 
DISEASES OF THE NERVOUS SYSTEM 
forgetting is carried out with special ease if the emotional stress has not 
been excessive. Forgetting, however, is only a secondary phenomena, 
and usually is more successful if the immediate reaction has been an 
adequate one. Such immediate reactions express themselves as tears, 
as anger, as impulsive acts, etc., and in such reaction the affect is dis- 
charged. In every-day life one calls it giving vent to one's feelings. 
If, however, the reaction is suppressed, the affect becomes united to the 
memory of the experience, and an emotional complex, or, to use a rather 
broad simile, a psychic boil results, which must heal by absorption, 
by discharge, or by other means. Freud uses the term ab-react (abre- 
agieren) to signify the adequate reaction, or discharge, of such affects 
or their resulting complexes. Talking the whole thing over, giving 
vent to one's secrets and confessions, are well-known abreactions. 
In hysteria certain of these complexes remain prominent; they are 
neither reacted too promptly, nor is their unpleasant feeling tone dimin- 
ished by the blurring process of forgetting, although it is characteristic 
of the Freud point of view that the actual experience which gives rise 
to them becomes forgotten and the cause of the affect disturbance which 
becomes later converted, it may be into physical signs, remains apparently 
unknown to the patient. It must be dug out by psycho-analysis, and 
when discovered catharsis takes place and the patient is cured. 
It is further of importance to realize that abreaction is impossible for 
certain types of psychical shock or trauma. The inevitable, in the loss 
of a beloved person, is not overcome by frank discussions; social relations 
may make it impossible to mention the shock; or, again, it may be that 
it may concern itself with things which the person wished to forget, and 
which were intentionally inhibited and repressed from conscious memory. 
In the limited space the full value of Freud's cathartic method cannot 
be presented. His original works must be consulted, as most of his 
critics apparently have not taken the trouble to read them. If truth be 
often stranger than fiction, certainly the charge of their being romances 
does not preclude their value. Experience is accumulating rapidly to 
show that in well conducted psycho-analysis one has the most valuable 
means of treating hysteria.1 
1 Jung, Theory of Psychoanalysis, Psychoanalytic Review, vol. i, No. 1. Jelliffe, 
Technique of Psychoanalysis, Psychoanalytic Review, vol. i, No. 1 et seq. CHAPTER XVIII. 
MIGRAINES. NEURALGIA. PROFESSIONAL SPASMS. OCCU- 
PATION NEUROSES. TETANY. 
SMITH ELY JELLIFFE, M.D. 
THE MIGRAINES. 
Under this head will be discussed a series of characteristic phenomena, 
which are among the oldest affections known in literature, and which are 
here brought together arbitrarily, with the full recognition that divergent 
opinions are held as to their essential relationships. 
OPHTHALMIC MIGRAINE. 
Synonyms.-Sick headache; megrims; hemicrania; bilious headache; 
Hemikranie, Migrane. Latin, cephalagia biliosa; suffusio dimidians; 
migraine; emicrania. 
Definition.-The difficulty of limiting this protean affection is great. 
It is practicable only to attempt a definition of the more classical types 
of the affection, neglecting numerous related forms bound up with other 
disorders. Taking these limitations into consideration, migraine may 
be defined as a periodical abnormal state in which the patient suffers 
from a peculiar oppressive pain in the head, unilateral or bilateral, local- 
ized or general, which develops very gradually from heaviness to dulness, 
to pain that is splitting, and is accompanied or more often preceded by 
characteristic visual signs, such as scotomata, flying specks, or partial 
blindness. Chilliness, depression and sensory visceral disturbances, 
particularly in the stomach, are also usually present. An attack may 
be terminated, after a few minutes, by vomiting, or it may persist hours 
or even days. After a variable length of time, usually following a heavy 
sleep, the patient regains his previous condition of well-being. 
Tissot is credited with having given the first classic on migraine and 
his remained authoritative up to the appearance of Liveing's monograph, 
"On Megrim, Sick Headache, and Some Allied Disorders" (1873), 
although in the interim the symptomatology was becoming richer and 
the case analyses more exhaustive. Thus, Vater, Hennicke, and Heberden 
made observations upon the scotomata. Plenck, Parry, and Wollaston 
drew from personal experiences the picture of half-sided blindness. 
Schbnlein and Romberg introduced the neuralgic theories, while DuBois- 
Reymond, influenced by the newer work of Claude Bernard, devel- 
oped the hypothesis of arterial spasm which Mollendorf controverted, 
and postulated a sympathetic paralysis, both of which views were con- 
ciliated by Jaccoud and by Eulenberg (1867), who described angiotonic 
709 710 
DISEASES OF THE NERVOUS SYSTEM 
and angioparalytic conditions. Recent monographs are by Auerbach 
and Jelliffe.1 
Etiology. -A great number of hypotheses have been advanced. That 
which maintains that the disorder arises autochthonously, affecting that 
portion of the brain which is the seat of a constitutional anomaly and acts 
here locally has been termed by Spitzner the central theory. Another 
group of hypotheses may be termed the centripetal theories. These 
assume that some cause, toxic, reflex, etc., originates in a peripheral 
organ, which, in turn, acting through the circulation or the sensory 
nerves, or both, affects the brain cortex and gives rise to irritative pro- 
cesses, and this causes the attack. The vasomotor hypothesis assumes 
that the essential feature is a vasomotor spasm followed by vasomotor 
dilatation, thus causing anemia and hyperemia, which act on the nervous 
tissues and cause the headache. 
The toxic hypotheses have many advocates, but all suggest different 
poisons, and these do not seem able ever to cause the condition when 
given under non-vital conditions. The followers of the reflex hypotheses 
successively call on the uterus, ovaries, foreskin, adherent clitoris, gas- 
troptosis, enteroptosis, the eyes, the ears, the nose, the throat, etc. 
Each specialist here advocates his own particular organ as the essential 
factor, just as each chemist claims his own chemical, uric acid, xanthin, 
paraxanthin, indican, etc., to be the real cause. 
The combined hypotheses next come into review, the toxic-reflex, the 
vasotoxic, the vasotoxic central theories, etc. The organs which have 
been declared at fault have been the vasomotor centres, the sympathetic, 
the gastro-intestinal canal, secretory, excretory, ductless glands, organs 
of generation, male and female, the various organs of sense, the eye, ear, 
nose, tongue, the muscles of the body and the general metabolism of the 
entire body, the brain in general, the cortex, the pons, the thalamus. 
Many authors pick out several factors; others confine themselves to one. 
Some faddists say it is eye strain; others ear strain; others masturbation; 
while others blame corsets, tight collars, or other folderol. 
Spitzner2 has subjected these ideas to a thorough criticism, and has 
advocated the hypothesis that migraine is due to a constitutional anatom- 
ical defect, namely, an absolute or relative stenosis of the foramen of 
Monro. This is the fundamental substratum of the migraine constitu- 
tion. The occasional causing of a passive or active hyperemia of the 
brain leads to a hyperemia of the choroid plexus. This causes a more or 
less complete plugging of the foramen of Munro, with the production of 
an increase of pressure in one or both of the ventricles. The increased 
pressure on the vessels causes more distension and more pressure on the 
walls of the ventricles; a vicious cycle is established, and the migraine 
mounts to its height, until the pressure is relieved either by a spontaneous 
reduction or by the sudden let down in tension due to a shock reaction- 
such as occurs in vomiting, from the use of various vasodilators, etc. 
The general hypothesis of disturbance of the vegetative nervous system 
accounts for most of the facts, and the position taken is that such dis- 
1 Modern Treatment of Nervous and Mental Diseases, edited by White and Jelliffe. 
2 Ueber Migraine, 1907. THE MIGRAINES 
711 
turbance may be conditioned by a host of causes. The view advocated 
then admits that a certain amount of truth exists in many of the explana- 
tions, but maintains that a one-sided mode of interpretation is inad- 
missible. Unconscious psychical factors, particularly repression, probably 
play a very large part. 
Heredity explains very little in the consideration of migraine. In a 
recent inquiry of some 3000 individuals it was found that less than 2 
per cent, had never had any headaches which could not be allied to 
migraine. Typical attacks in any great number were lacking in perhaps 
50 per cent., but close questioning revealed a few classical attacks inter- 
spersed with many abortive attacks. With such a wide prevalence then 
heredity is a negligable factor. It is like the doctrine of original sin. 
Relation of Epilepsy to Migraine.-This has been widely discussed. To 
those who look upon a wTord diagnosis of many dissimilar conditions as 
symbols of entities, such a discussion is, perhaps, pertinent, even if on an 
illogical basis, but wThen the standpoint is maintained that there are many 
epilepsies and many migraines, and that both words may stand for things 
at times very much related and at other times not related at all, discussion 
is more or less fruitless. It is not impossible to understand that a pro- 
found change in the character of the cerebral circulation, which may be 
due to transitory causes, and which may give rise to an attack of migraine, 
may also, in rare instances, be an exciting cause of a motor disturbance 
sufficient to set free an epileptiform convulsion. In such a sense the 
subject may be discussed. An increasingly greater number of atypical 
and borderland conditions are coming into review, and in the more recent 
analyses convulsive attacks indistinguishable from the hysterical and 
schizophrenic motor reactions are being described. Until a clearer 
conception is gained of the mutual relations of cerebral circulation and 
motor discharge it is folly to attempt to review the interesting, though 
fallacious, suggestions of Fere, Mobius, and others, who claim a necessary 
relation between migraine and epilepsy. From the standpoint of organic 
brain disease as a factor in the causation of a migraine the discussion is 
of much service. From the psychogenetic viewpoint there are close 
relations between some migraines and some epilepsies. 
Abortive Attacks.-It is difficult to be certain of the character of 
many conditions in patients who suffer from migraine, as there exists an 
involuntary tendency to drag every symptom within the net of this one 
great malady. Experience shows, however, that sufferers from migraine 
possesses a keen insight into their undeveloped or abortive attacks, and 
after years of observation are able to recognize them with precision. 
Incomplete or abortive attacks may be said to be the rule rather than the 
exception, and all attempts to classify the disorder according to the 
number of symptoms present lead to confusion. 
The commonest abortive attacks are those that begin in the classical 
manner, with chilliness, perhaps with pinched face and cold extremities. 
The patient then has the scotomata and wretchedness, depression and 
apprehension, and then while waiting for the headache the patient 
notices that it does not come, and, although many may still have heaviness 
and discomfort, the feeling of relief is sufficient to make them feel well. 712 
DISEASES OF THE NERVOUS SYSTEM 
Others have added the sensation of pricking in the fingers, numbness 
in the hand or arm, or other sensory disturbances without the headaches. 
In some the entire attack will consist of a disturbed painful sense of dis- 
comfort, without sensory symptoms, scotomata, or headache, but they 
feel sick at the stomach, and have an attack of what they term " bilious- 
ness," which clears up after vomiting. This feeling will recur with 
sufficient frequency, and at times be combined with such other symptoms 
of a migraine attack, in its varying aspects, as to stamp the whole process 
as a variant of a true attack. Isolated attacks of vomiting, as the sole 
expression of a migraine, are known. 
Attacks of scotomata occur alone, without antecedent distress, and no 
after-effects are noted. These are most uncommon, and historically it 
may be noted that Parry and Airy had such attacks. It is highly probable 
that the majority of patients who have had many migraine attacks will 
have had some of this nature. Attacks of scotomata and vomiting occur 
without headache. In many, headache is the only symptom. 
Some patients have attacks of hemiparesthesia, without any other 
symptoms of migraine. Such generally occur at night, and usually follow 
severe mental exertion, in one patient under observation a severe ordeal 
in playing a difficult piece of music will bring on such an attack without 
other signs. This patient's severe attacks are very extreme. 
Under the heading of equivalents, Liveing speaks of stomach attacks 
associated with some of the vascular phenomena of migraine; glossal 
spasms are also mentioned by him. Attacks of giddiness, vertigo, 
intestinal colic, mental anxiety and depression which occur at periodic 
times in partial association with migraine symptoms, are also noted as 
equivalents. There is need of further study of these isolated phenomena 
associated with disturbances of the autonomic nervous system. 
Attempts have been made to determine the relative frequency of 
migraine attacks with and without the visual signs. These are not 
over reliable, because of the vast preponderance of abortive attacks over 
those of the complete classical type. It may be recalled that many 
authors have attempted to create a special type of eye migraines which 
are autonomous, but the gradations are so numerous when different 
individuals and differing attacks in the same individual are considered 
that such a view receives little support. 
Mobius expresses the opinion that the percentage of visual accom- 
paniments with the attacks is usually overstated. His statistics show 
130 cases, with 14 visual aura. In Liveing's 60 patients, 37 suffered from 
scotamata. Gowers said that the cases are about half and half, with 
and without eye signs. It is difficult to state an individual position, the 
results of personal inquiries having been so diverse. Close questioning 
has revealed the fact that at some time or other in the course the majority 
have had visual symptoms, and it is not improbable that the usual 
statistics are largely derived from studies of too few attacks, i. e., largely 
from the severer attacks only. Some notes on individual histories are 
of interest. Several patients have kept fairly accurate records of their 
migraine attacks for several years. One shows 168 attacks in a period 
of about ten years; of these, about 100 were abortive attacks, the vast THE MIGRAINES 
713 
majority of which, 60 per cent., consisted of scotomata alone. Of the 
68 remaining attacks, about 50 per cent, were ordinary hemicrania, 
lateral or bilateral, without scotomata, the others ophthalmic migraine, 
usually unilateral and with scotomata. Not one of the attacks was 
ever accompanied by vomiting. Two were associated with aphasia, 
fifteen with sensory tactile associations; there were five or six attacks 
of hemiparesthesia, one in the day-time, the rest at night. Spasms 
of the orbicularis were a common accompaniment. 
Similar results in about a dozen other cases show records of from 50 
to 200 attacks. It is rare that a patient suffering from migraine of any 
severity goes through life without some one or more of the sensory 
phenomena, and notably the scotomata. But a great many individuals 
have their attacks, both mild and severe, while asleep, and may never 
know of the occurrence of scotomata. Many of these patients, who 
have given no history of scotomata, have had frequent night attacks 
with sore eyeballs and sore spots on the scalp, two symptoms of frequent, 
although not necessary, relationship with other sensory symptoms. 
Classical Migraine.-Early Symptoms.-These may be termed pre- 
cursors to a full attack of migraine, or they may constitute the symptoms 
of an abortive attack. The most striking are a sense of heaviness, with 
yawning, chilliness, dizziness, or depression, motor twitching, even sharp 
spasms, closure of the eyelids, sensory phenomena, chiefly paresthesia, 
occasionally anesthesia, and affections of the eyes or other sensory organs, 
ringing in the ears, blowing, modifications of taste, smell, touch, etc. 
The temporal arteries are often smaller, and the saliva diminished. 
The premonitory signs, which show a great degree of variability in 
different individuals, and also in different attacks in the same individual, 
may be felt several minutes before the attack, in some rare instances 
even hours. This is frequently the case in women in whom the onset 
of the menstrual function seems to bear some relation to the attack. 
At times such attacks of depression and anxiety, combined with a sense 
of chilliness and dizziness in the head, will constitute the entire picture 
of an abortive attack. Many attacks come unheralded. Many patients 
having attacks at night find themselves tired, and heavy, with sore 
spots on the scalp in the morning. 
In all probability premonitory symptoms of some type are invariably 
present; when thought to be absent it is because the patient has overlooked 
them, either by reason of the mild character, or because of naturally 
poor powers of observation. Many patients, who have had migraine for 
years, have never noticed their one-sided localization, or the well-known 
fortification spectra, until their attention has been directed specifically 
to them. Many patients will deny ever having had zig-zags of light, 
etc., until shown Airy's pictures, when they remember having seen such 
phenomena. It is because of such poor observation that many cases 
are overlooked, which fact lends further support to the personal belief 
that this disorder is much more prevalent than is usually supposed. 
Sensory Symptoms.-In the more classical attacks the patients have 
preliminary sensory symptoms. These are spoken of by Mobius in the 
sense of an aura, but the term is objectionable-even misleading. A 714 
DISEASES OF THE NERVOUS SYSTEM 
sense of eoldness and chilliness is one of the commonest sensations. 
This is usually general, and is associated with a pale countenance, goose 
flesh, perhaps clammy hands, and a sense of misery. Cases are known, 
and are by no means uncommon, in which the chilliness has been one- 
sided, and is accompanied by other phenomena involving the entire half 
of the body of the same side. Yawning is a common early sign. 
Unilateral paresthesia is not an uncommon early sensory sign. Many 
patients note a tingling or numbness in the fingers of one hand; this may 
spread up the arm, and in rare instances general unilateral paresthesia 
of a very uncomfortable nature may be present. In some instances such 
unilateral paresthesia has constituted the sole symptoms of an attack, 
save for the heaviness and usual discomfort. Occurring at night, such 
attacks are often extremely wearing, keeping the patient awake. Photo- 
phobia, flow of tears, strange sounds, tinnitus, peculiar odors, queer 
tastes, peppery or flat, may be noted. 
Anesthesia is less often observed, largely because of the negative 
character of the symptom. When involving the face or mouth it is 
complained of. Anesthesia frequently follows the tingling of the early 
paresthetic disturbances. Franz1 has shown that there is a very evident 
decrease in the pain threshold, especially after the headache has set in. 
The visual phenomena are the most striking, and hence held to be of 
the most frequent occurrence. The ease of observation in part accounts 
for the usually accepted opinion that they are the commonest of the early 
symptoms. Very few individuals have been subjected to a careful 
sensory analysis. If more were investigated, it is probable that other 
slight sensory signs would be found to be equally prevalent and as evan- 
escent. The visual signs have been described by many writers, and many 
illustrations have been made showing their chief characteristics. The 
extreme uniformity of their general character is striking, as well as the 
variations of the same pattern. 
As a rule, the patient notices a slight blurring of his vision if reading, or 
a slight flicker of light located in one eye, to one side of the centre. Closer 
observation reveals either a slight cloudy spot, which seems to follow 
the eye in reading, cutting out the after-images a letter or so from the 
point of the centre of vision. The slight subjective sense of difficulty 
in reading may precede the discovery of a scintillating spot, which becomes 
visible on closing the eyes. Little by little this spot spreads out, usually 
in a crescent-like fashion. General statistics are thus far unavailable, 
but personal experience has shown that the majority of these scotomata 
have begun in the left eye, are situated to the left of the middle line, with 
the convexity of the crescentic border to the left. As the crescent 
gradually grows larger, the difficulty in seeing clearly becomes more 
marked, especially on the periphery of the visual field. For most, the 
scotomata is in constant motion, flashing in its spectral zig-zag fashion, 
thus giving origin to the classical name "fortification spectrum," from 
the play of colors, and the fortress-like "ins and outs" of the outline. 
After a variable time, from five to twenty minutes, the scotomata 
1 Am. Jour, of Physiology, 1905. PLATE X 
From Dr. Hubert Airy's Paper, On a Distinct Form of Transient 
Hemiopsia, Philosophical Transactions, 1870, p. 247. 
Figs. 1 to 4.-Early stages of Fortification Spectra as seen in dark. O=sight point. 
Figs. 5 to 8.-Stmilar series, beginning lower. 
Fig. 9.-Fully developed. i?=secondary attack within. THE MIGRAINES 
715 
gradually subside, or suddenly disappear, to be followed by the headache. 
Not infrequently the headache never comes, and the preliminary sensory 
phenomena of chilliness, heaviness, and scotomata constitute an abortive 
attack. The classic of Liveing reproduces the excellent illustration of 
Airy's which is here shown. 
Occasionally the right half of the field is involved. Sometimes it is 
the upper half, one of Mobius' patients saying that everybody seemed 
headless; occasionally, it is the lower. In rare instances the patient 
complains of total blindness, i. e., central scotomata. Berbez reports 
an interesting case of ring-like scotomata-the patient, on looking at his 
watch, could see only the central pin where the hands were united; the 
figures on the dial were all obscured by the scintillating scotomata. 
These scotomata are usually bilateral phenomena, they may begin 
in one eye before appearing in the other, and be somewhat different in the 
two eyes, and may disappear in one eye sooner than the other. Scoto- 
mata limited to one eye are probably rare. 
The retinal changes during the time of the occurrence of these scotomata 
are uncertain. Blanching of the papillae has been observed by some 
(Galezowski); pulsation of the retinal arteries with dilatation by others. 
Personal experience has shown similar dilatation in a few cases, but, as 
a rule, a normal fundus is found. The picture seen will depend upon 
the stage of the attack and upon its severity. 
Pupillary dilatation occasionally occurs. Slight irregularity of the 
pupils during a severe attack of an ophthalmic migraine, dilatation being 
usual on the affected side, is not unusual. Bilateral pupillary contraction 
is the rule in the acute stage of the headache. 
During the onset of the fortification spectra, it not infrequently happens 
that mild motor phenomena occur in the eyelid of the side to be affected. 
The eyelid droops a particle, and Gowers and others report double 
vision, interpretable as a sign of paresis in an ocular muscle. As these 
phenomena are not rare in the severe types of migraine, called by some 
ophthalmoplegic migraine, their consideration will be deferred. 
Motor Disturbances.-Speech.-This may be considered as both a motor 
and sensory phenomena, for the most frequent type of change is sensory 
aphasia in most instances. Anarthrias are known, especially in the 
ophthalmoplegic variety, but for ophthalmic migraine the type of aphasia 
observed is very characteristic. As described by Charcot, it is an inter- 
mittent, halting aphasia. At one moment the patient can get the right 
word, at the next he cannot. He stumbles on a word; uses madame for 
monsieur, etc. In Liveing's cases, 15 out of 60 had speech disturbances; 
one, on hearing clock bells, was unable to inquire what they were. Fere 
cites the case of a coachman who forgot where he was going to drive 
his passengers; Berbez, a like case in which a pedestrian lost his way, as 
he could not read the street signs understandingly. Gowers speaks of a 
case of word deafness. Cases of agraphia are also known. Mobius 
reports a case with typical scintillating scotomata at one time on the right 
side, and at another on the left. When the patient suffered from a right- 
sided scotomata he had sensory aphasic signs, but they were not present 
when the scotomata was on the left side. The disturbance sometimes 716 
DISEASES OF THE NERVOUS SYSTEM 
resembles a paraphasia, the patient using a jumble of words. In a 
personal observation the patient could not sing a well-known tune 
correctly, his sense of muscial values having been interfered with. 
The onset of the aphasic disturbance may vary greatly. It is usually 
temporary, persisting at times not over a few minutes, again persisting 
a few hours. It frequently antedates the headache, or is coincident with 
it. In a reported case of Meige the aphasia persisted as long as the 
headache, and disappeared, as a rule, when that disappeared. The 
patient showed a loss of ability to say certain wTords and a tendency to 
the employment of incorrect words. There was no anarthria. .The 
symptoms in one of Meige's patients were closely similar to those of a 
case reported by the writer.1 
Paralytic Phenomena.-Attention has already been called to the rare 
occurrence of hemiparesis, which may even involve the facial muscles. 
Up to the present time no instances of crossed hemiplegic type have been 
found in the literature. This of interest in connection with the hypothesis 
of the bulbar origin of migraine, especially of the ophthalmoplegic variety. 
Cerebellar Symptoms.-Oppenheim has called attention to a cerebellar 
hemicrania in a patient in whom every attack of migraine was accom- 
panied by typical cerebellar symptoms. The patient was uncertain in 
his gait, walked like a drunken man, was dizzy, and had the sensation 
that the body or individual parts of it were doubled. The sense of 
equilibrium was disturbed in each attack. Dizziness and loss of the 
sense of equilibrium are not infrequent, but such a complete syndrome 
has been described only by Oppenheim. 
Headache.-This is the most common feature, and exhibits a great 
amount of variability as to location, quality, intensity, and duration. 
In the more classical attacks the headache begins on the average about 
fifteen to thirty minutes after the appearance of the scotomata or other 
sensory phenomena. It frequently begins on one side, and may remain 
so or become bilateral. As a rule, it is frontal, or occupies the vertex, 
but may involve the temporal regions or the occiput, sometimes as low 
down as the neck.' Gowers' experience points to the parietal region as 
being oftenest affected, and usually over a small area. Henschen, in 
123 patients, shows the pain to have located 110 times in the forehead, 
100 times in the parietal region, and 54 times in the occiput. There is 
usually pain over the eyes, and the eyeballs are usually painful to pressure. 
In a few instances pressure over the malar bones is painful, and occasion- 
ally there is a well-marked jaw ache. 
Statistics of the percentage of location are difficult to present, since the 
individual will have all the different varieties. Thus, in a case already 
cited, in which the abortive attacks were so frequent, the headaches 
comparatively rare, the strictly unilateral headaches were only 5 per cent, 
of the entire number. In others the hemicranic type runs much higher. 
In Henschen's records of 123 cases, 56 had one-sided attacks, in 67 both 
sides were involved. In Liveing's 61 patients, 17 had one-sided attacks, 
in 7 the attacks were variable, while in 34 both sides were involved. 
1 New York Med. Jour., 1906, Ixxxiii, 33. THE MIGRAINES 
717 
Mobius and others note that the headache often begins on the side opposite 
to the beginning of the sensory aura. Personal studies do not confirm 
Mobius' statement. Averages one way or another do not occur if a sum- 
mary of all attacks is attempted instead of picking out those which are 
most vividly present in the memory of the patient. It does seem, 
however, as first noted by Liveing, that one-sided sensory symptoms are 
oftener accompanied by one-sided than by bilateral headaches. With 
bilateral sensory phenomena, scotomata, etc., bilateral pains are the 
commonest. In many attacks the pains are limited to the eyes, the 
soreness of the eyeballs being so marked that it is painful to move them. 
Pain in the neck may also cause the desire to hold the neck rigid. 
The character of the pain defies analysis, since descriptive phrases are 
used in such various ways by different observers. In some attacks the 
head simply feels slightly sore, or heavy, or dull, or thick; "like a block 
of wood," is a frequent expression. " Filled with sawdust," one patient 
says. Again, the pain is agonizing, impossible to describe. Some 
patients shriek with the pain, become hysterical, and roll about the 
floor, grasping the head between the hands, wishing to beat their brains 
out. Between these extremes numberless gradients are found among 
different individuals, and in different attacks in the same individual. 
Nearly all patients will say that the severe pains are throbbing or thump- 
ing, usually indicating great pressure from within or without; as Mobius 
has said, " some patients think the head will burst, others that it is being 
squeezed in a vise." Descriptions of bursting are more common. The 
pain is an all-prevading one, gradually mounting to a maximum, then 
running along continuously without any break, with, at all times, sudden 
accessions, especially on movement, if one leans over, or is forced to 
sudden exertion. In but the rarest instances is it described as lancinating 
in quality. It is the type of pain apparently seen in cerebral tumor, in 
acute hydrocephalus, in cerebrospinal meningitis, and allied to the pain 
of opium poisoning, or of sea-sick headache; all pointing in the direction 
of a modification of intracerebral pressure, at times an increase, or it 
may be a decrease, either of which may cause severe pain. Occasionally 
the phenomena of a bilateral headache with marked predominance of 
one-sided pain will be observed. 
The severity of the pain may be conditioned by a number of factors. 
Movement uniformly increases it. Bending over becomes impossible. 
The first movement on lying down is usually accompanied by a sudden 
rise in severity, but this gradually subsides. The taking of alcohol 
usually increases the severity of the pain, as does also the use of tobacco. 
Eating, if possible, may help somewhat, but usually augments the pain, 
and is avoided. Strong sensory impressions invariably increase the 
pain. Noises of various kinds often aggravate the pain tremendously 
and cause certain patients marked distress. Mobius notes that the rage 
of migrainous parents directed toward their noisy children often resem- 
bles a pathological hatred. Strong light is invariably avoided, because 
of its tendency to increase the pain. The movements of the eyeball and 
attempts at visual accommodation cause an increase in the pain. 
Psychical effort is often impossible; in milder attacks the awakening 718 
DISEASES OF THE NERVOUS SYSTEM 
of a strong mental stimulus may make one forget the pain. Mobius 
says that his attacks, usually light ones, are frequently forgotten during 
an interesting visit to the hospital, to be once more prominent afterward. 
The writer has frequently begun a lecture with a severe migraine to find 
it almost forgotten until the close, when it reappeared, usually with 
renewed vigor. 
The movements of straining at stool, and vomiting, coughing, etc., 
invariably cause a rapid and sharp rise in the severity of the pain. 
Sensory stimuli may have an unpleasant effect. Thus, certain odors 
cause distress; the smell of cooking accelerates vomiting. Certain skin 
phenomena, such as painful spots, are frequent after the headaches. 
In certain personal experiments with drugs the following have 
invariably increased the headache within a few moments: A few whiffs 
of chloroform or of ether, adrenalin by mouth, digitalis, strophanthin, 
and ergot. Drugs that raise the blood pressure, in general, increase the 
pain especially when taken at the beginning of the headache. 
The headache may clear away very suddenly after an attack of vomit- 
ing, or it may pass without vomiting; in some it fades away gradually. 
It may last a few minutes, a few hours, or a few days. Some cases of 
what Mobius chooses to call status hemicranicus are recorded. 
Vasomotor Disturbances.-Practically all attacks of migraine are accom- 
panied by visible vasomotor disturbances. The battle of the adherents 
of the vasoconstrictor vs. the vasodilator hypothesis lends point to this 
observation. In most cases vasoconstrictor phenomena (coldness, pale- 
ness, goose-flesh, etc.) precede, to be followed later by vasodilator changes. 
Thomas contributes a statistical study of 107 cases in support of the 
early pallor, small pulse, and coldness, which pass over into the pheno- 
mena of warmth, red, flushed face and skin, and full pulse. The period 
of initial constriction may be unnoticed by reason of its transitory char- 
acter. In some instances this initial vasoconstriction may be very marked 
and give rise to the phenomena of localized cyanosis, even advancing 
to the picture of the constriction phase of the Raynaud disease type. 
In the same manner the secondary vasomotor dilatation may pass the 
bounds ordinarily observed and lead to localized oedema, to the erythro- 
melalgia type, or, exceptionally, to hemorrhagic phenomena in the con- 
junctiva, ocular tissues, or even in the walls of the stomach. 
Secretions.-Alteration in secretory functions are frequently observed 
early or late in the attack. Reference has been made to the excess of 
secretion of tears as a frequent precursor. Vomiting of frothy mucus, 
liquid diarrhoea, increase of sweat, coryza (Calmeil), or incessant saliva- 
tion (Liveing, Tissot) are common phenomena. The changes in urinary 
secretion have attracted careful attention and given rise to many hypo- 
theses. The early vasoconstriction of the periphery, coldness, lack of 
secretion, of perspiration, etc., account in a purely mechanical way for 
the increase of urinary secretion in the early stages, and also for most 
of the phenomena interpreted so one-sidedly by Haig and others. 
Trophic Disturbances.-These have been reported by several observers. 
Cornu says that nearly all of his cases of migraine show facial assymetry, 
and facial atrophy is recorded. These instances are largely coincidences, THE MIGRAINES 
719 
and are not necessary attributes of the migraine. A facial atrophy which 
could be interpretable only on the basis of a migrainous disturbance of 
the vasomotor apparatus is very problematical, and certainly Cornu's 
results are not confirmed by others. Loss of weight in the severe rapidly 
recurrent cases is due to disturbances in general nutrition due to gastric, 
rather than to other causes. Herpes zoster is a not infrequent accom- 
paniment of some cases, but the recognition of its infectious nature 
successfully disposes of its essential relationship to migraine. 
Psychical Disturbances.-These have been noted by many observers, 
Liveing being one of the first to point out the relationship of disturbed 
psychical states to the attacks of migraine. Mobius takes the position 
that such psychical disturbances are largely due to the pain, and are 
epiphenomena, as it were, in many instances being related to hysteria. 
This position is not tenable in view of the many observations pointing 
in other directions. 
In the vast majority of migraine attacks there are no mental changes, 
either before, during, or after the attacks. ZXIild depression, hopelessness, 
despondency with clear consciousness, are frequent mental states. With 
very severe pains Mobius admits the clouding of consciousness, and is 
not sure that the severe stuporous states are not due to pain as well. 
Mingazzini, on the other hand, believes there is justification in erecting 
a special group, which he has termed the hemicranic dysphrenias (disfrenie 
emicraniche), and he distinguishes a transitory and a more permanent 
variety. Recent observers are practically in accord in showing that severe 
mental disturbances varying in character and intensity may be part of 
a migraine attack. 
Guidi has amplified these observations by reporting the history of a 
number of cases in which the patients suffered in the day or hours before 
the onset, in a much more decided manner than the feelings of anxiety 
or of depression described by Liveing. Thus Guidi calls attention to 
grave alterations in the psychical state of a number of his patients. In 
one the entire character of the personality would change preceding the 
attack. A patient who had always been calm, reserved, quiet, and 
modest, suddenly became much agitated, was forward, noisy, and loqua- 
cious, and told salacious stories, which was far from his usual behavior. 
While in health a spare eater, preceding an attack he suddenly became 
very hungry, and hankered especially for starchy foods. During the 
attack the patient had glycosuria, which disappeared later. 
With the onset of pain the picture is less clear, yet there is little doubt 
that many patients suffer from profound psychical disturbances, which 
arise independently of the pain. One such case which is typical, under 
personal observation, would be interpreted by Mobius, and rightly so, 
as one in which the pain is the first link in a hysterical reaction. But 
there are other cases which do not belong to this group. Mingazzini's 
hemicranic dysphrenias may be cited as examples, in part, at least. 
In others severe disturbances have occurred, such as states of anxiety, 
rising to actual anguish (Charcot); phobias of inability to perform acts 
(Cornu-Charcot); terror (Liveing, Fere, Kraft-Ebing); hallucinations 
of sight (phosphenes, colored lights, animals) and hearing, with mental 720 
DISEASES OF THE NERVOUS SYSTEM 
confusion (Forli, Mingazzini); maniacal excitement (Mingazzini, Jelliffe); 
and stupor, unconsciousness (many authors). Liveing reports that 25 
per cent, of his cases showed psychical symptoms. The Italian observers 
record fewer, but it appears that at least from 10 to 15 per cent, of 
the cases of grave hemicrania show some distinct mental disturbance 
in some one or more of their attacks which is more significant than the 
usual universal depression. 
Symptomatic Migraines.-The occurrence of migraine-like attacks 
accompanying, or due to, definite disease condition, notably organic 
disease of the brain, is well known. The association of migraine with 
gout and malarial affections has been noted. So far as gout as an etiologi- 
cal factor is concerned, Mobius is inclined to see nothing more than a 
coincidence; while, as for malaria, he holds it to cause an orbital neuralgia, 
not a migraine. As for the latter, it seems clear that the well-known 
effects of malarial infection on bloodvessel, tonus are entirely suffi- 
cient to cause a typical migraine attack. It is known that attacks of 
migraine may be very frequent during the continuance of a malarial 
infection. 
Migraine-like attacks are not infrequent in cerebral tumor; they may 
appear periodically, as in cases fully reported by Abercrombie and Mobius, 
or they may be continuous and distinguishable with great difficulty from 
the pain of tumor, as in cases reported by Wernicke, who has said that 
such attacks may be quite readily confused with those more typical of 
tumor. In tumors, however, vomiting brings little or no relief; quiet 
gives less relief, and the fluctuation in the intensity of pain is less promi- 
nent. A primary onset of migraine-like attacks in adult life should always 
awaken the suspicion of an organic brain lesion. 
Oppenheim lias called particular attention to the occurrence of 
migraine-like attacks in the onset of tabes; Mobius is inclined to think 
it a rare combination, and regards such either as a pure coincidence or a 
migraine-like neuralgia. In general paresis, migraine-like attacks may 
be an initial symptom. 
Diagnosis.-The difficulties appear in the consideration of ordinary 
headaches and in neurasthenic headaches; in distinguishing between the 
scotomata of migraine and other scotomata; the paresthesia of migraine 
and other paresthesias; the aphasia, the vomiting, etc., as seen in migraine, 
and the same as due to other causes. In most individuals abortive and 
incomplete attacks are the rule, and it is often extremely difficult to 
determine the precise significance of these attacks. 
Mobius has suggested that the problem is not only whether the case 
is one of migraine or not, but whether it is migraine alone, and not some- 
thing additional. This author's contention that migraine is hereditary 
and begins in youth, would seem to make it a simple matter, but clinical 
experience shows that real migraines do appear in later years, apart 
from other affections, and as for the heredity factor, the extreme preva- 
lence of the affection makes it difficult to accurately weigh this factor. 
If to heredity we strictly apply the Mendelian criterion, that the absence 
of the disorder in either progenitor negatives its appearance in the 
descendant, then the Mobius doctrine falls; if, however, by going into the THE MIGRAINES 
721 
collaterals, as is so frequently done, then almost every disease having a 
large incidence may be proved to be hereditary. 
The periodic recurrence is a difficult criterion. There is usually no 
difficulty in diagnosing the classical attacks from simple headache, but 
at times such a differentiation is impossible. Many chronic sufferers 
from migraine know well their real attacks, are able to distinguish abortive 
attacks, and also have headache of an entirely different nature. The 
simplest test separating abortive migraines from simple headache is the 
occurrence of sensory phenomena, other than pain, which have their 
main seat in vasomotor disturbances. It is on this account we would 
ally the severe headaches following the use of alcohol, ether, chloroform, 
opium, or related drugs to the migraines, rather than to simple headaches. 
The headaches of neurasthenia, anemia, syphilis, lead poisoning, nasal 
sinus involvement, supra-orbital neuralgia, nephritis, eye strain, glau- 
coma, etc., should present little difficulty. 
Treatment.-The treatment of the migraine attack is, for the most 
part, fairly satisfactory. There are few patients for whom some relief 
cannot be obtained, both with reference to the diminution in the number 
of attacks, and to the mitigation of the severity of the attacks themselves. 
The migraine habit, constitution, or liability-call it what one will- 
exists in very varying degress; in some a very slight disturbance is suffi- 
cient, to set free those forces which culminate in an attack; for others 
it requires a very much greater mal-adjustment. If the general reflex 
vascular hypothesis be taken as a tentative explanation, it is very readily 
understood why the taking away of various forms of peripheral irritation 
may result in eliminating one or more, and in certain instances all, of the 
causes which set the migraine reaction in operation. 
It is folly to shut one's eyes to the very evident clinical fact that certain 
migraines are relieved, if not entirely wiped away, by the correction of 
some peripheral disorder, sometimes more than one, which has had 
definite action on the nervous system. Just what the differential relation 
may have been between the severity of the irritant and the mildness of 
the attack is impossible to judge, but certainly the relief from eye-strain, 
from diseased turbinates, from adenoids, from constipation, from dysmen- 
orrhoea, from a number of minor yet definite peripheral irritations, will 
relieve a certain number of patients. One should, therefore, eliminate 
at the outset such of these structural defects as are shown to have some 
influence on the nervous system. In denying any possibility to these 
influences in the causation of migraine one errs almost as badly as when 
maintaining some one of them to be the only element. 
Gastro-intestinal factors are closely analogous to those just mentioned. 
In the minds of most clinicians, and certainly as generalized in the feelings 
of most of those afflicted, it is in the stomach, liver, or intestines that the 
main seat of the trouble is to be sought. The gastro-intestinal factor is 
undoubted in many cases; it may be exclusively gastric or colonic. As 
to the significance of chemical features, resulting from altered gastric 
secretions or from toxic bacterial products, we are entirely in the dark. 
It is certain that none of the products which have been held responsible 
as auto-intoxicants are universal causes. At any rate, the general features 722 
DISEASES OF THE NERVOUS SYSTEM 
of gastro-intestinal hygiene should be carried out. Constipation is to 
be avoided and such diet taken as experience has shown is individually 
applicable. Excesses in certain articles of diet are held by many as 
exciting causes; in many, such empirical feelings should be respected; the 
patient often knows himself better than does the physician. 
In some, excessive carbohydrate intake acts disastrously; in others, 
wine, whisky, or gin. The history of inability to eat fatty food, particu- 
larly sausages, is not infrequent. In rarer instances, one notes that 
certain auditory stimuli may bring on a migraine. To attend certain 
fatiguing and thrilling operas is followed in some by migraine attacks. 
For attacks conditioned by outside factors, such as malaria, gout, 
lead poisoning, etc., it is evident that these should receive attention. 
If the varying elements mentioned have any real relation, it is evident 
why such a variety of measures will be of help to a few, and why so many 
more will be worthless for many but useful for some. Medication 
between attacks is largely useless, save naturally in the symptomatic 
migraines. General medication, for no definite purpose but just in hope 
that it may do some good, as iodides, bromides, strychnine, etc., is 
senseless. If definite factors are found that need correction, and can be 
so modified by drugs in the desired direction, then they will prove useful. 
Thus iodides will undoubtedly help many presenile arteriosclerotic 
migraines; bromides are useful for sleepless and irritable conditions which 
provide a good foundation for the nervous instability; laxatives are 
called for if persistent constipation bears any causal relationship. 
Complicated systems of diet have been devised. Usually such are 
more prolific in engendering seini-invalidism than useful for migraine. 
Here and there a patient derives benefit from a strict dietary regime, 
but unless there are real reasons why a patient should not eat red meat, 
or tomatoes, or other sundry articles, as determined by actual experience 
and under repeated experimental trials, in order to eliminate faddist's 
errors, the patient is better off without a diet card. The reasons sought 
for are not those contained in many treatises on dietetics, in which 
medieval notions concerning differences in red meat and white meat, 
vegetables growing under the ground and those above the ground, are 
foolishly perpetuated. The only satisfactory manner to attack the 
metabolic problem is to carry out a complete metabolism analysis. 
The avoidance of alcohol and tobacco, while advisable, is so only 
relatively. The individual's reaction to all influences should be rigidly 
estimated before those usually self-evident restrictions are imposed 
in the name of health. 
Emotional factors are at times the most important. Very frequently 
migraine is a pure psychoneurosis and needs psycho-analysis. 
For the treatment of the attack itself one finds that a like fitting of 
remedies to the individual is called for. In the initial phase of vasocon- 
striction a number of vasodilators are of service, although their action 
is extremely unequal. The nitrites and nitrates have been employed 
for years, and usually with a fair degree of success if the dosage and 
individual member of the group be correctly chosen with reference to 
the severity of the attack. A mixture is of greatest value; nitroglycerin THE MIGRAINES 
723 
and erythrol tetranitrate give the best combination, for following the 
very evanescent and powerful action of the former, the more prolonged 
and steady action of the latter maintains the effect. The slowly acting 
nitrites are practically useless. Nature's readjustment, vasodilatation 
by vomiting, etc., has already reduced the cerebral pressure, and the 
stage has passed when the dilating remedies might be useful. It is 
practically only in the vasoconstriction stage that the nitrites are worth 
much; and in many they are inefficient. Given too late, they overdo 
the dilatation and increase the difficulty. 
The analgesic vasodilators have come to occupy the front rank. The 
precise significance of each must be appreciated in order to obtain the 
best results. Solubility, time of absorption, slight differences in the 
chemical formula and in action, continuance of effect with minimum 
by-effects, are all to be studied. The list is a long one and is constantly 
on the increase. Antipyrine, acetanilide, phenacetin, and the related 
salicylic acid (aspirin, etc.) compounds are the chief members. It is 
to be remembered that while their general action is closely related, there 
are specific differences in the working of each, and the measure of success 
that one has in mastering the majority of migraines depends upon a 
knowledge of these factors. Antipyrine, by reason of its rapid solubility 
and quick action, occupies an important place, but is not always applic- 
able. Acetanilide, alone or in combination with other analgesics of 
related type (salicylic acid derivatives), bromides, and caffeine are also 
valuable. The dosage should be graded according to the usual severity 
of the attacks. Tolerance is established in the quickly recurring attacks, 
and changes must be made. It is not yet certain what part is played 
by the respective analgesic and vasodilatation actions of this group. 
They have robbed migraine of most of its terrors, and tended to diminish 
the use of morphine and its derivatives very markedly. 
Caffeine is a much overrated drug. In the abortive attacks and in 
the morning remains of a migraine it is useful; but for a full-fledged 
attack it is not efficient. Similarly, bromides alone, chloral, and other 
widely used drugs are valuable only in mild attacks. They should be 
used in preference to other more potent remedies, which should be reserved 
for the severer attacks. 
The use of aconite and cannabis indica is more restricted now that 
really efficient analgesics are known. Aconite is rarely called for, while 
cannabis indica or cannabis americana has a limited, although no less 
definite, place. In attacks associated with much mental depression the 
addition of cannabis is often useful. The often experienced inefficacy 
of this latter remedy is largely due to its extreme variability. Tablet 
preparations are usually worthless. This is equally true of the volatile 
nitrite preparations. Opium, or its main derivative morphine, should 
be used only as a last resort. It is rarely really needed. 
Lying down in a quiet, darkened room-a brisk saline laxative taken 
as early as possible, the patient being undressed and well covered-these 
are essential in the severe exhausting attacks. A very hot bath often 
aids very materially. Cold is to be avoided. 
The greatest folly of all is to treat all patients and every attack alike. 724 
DISEASES OF THE NERVOUS SYSTEM 
Ophthalmoplegic Migraine.-Attention has been called to the fact 
that in the ordinary attack of ophthalmic migraine there may occur 
various sensory or motor phenomena, among which anesthesias or 
paralyses are the most marked. These sensory and motor changes are 
extremely diverse but there is one symptom grouping which, by reason 
of its comparative frequency and close similarity, was set apart from 
others occurring in this affection and named by Charcot ophthalmoplegic 
migraine, in order to distinguish it from its more classical relation. It 
consists in a paresis or a paralysis of one or more muscles of the eye, 
innervated chiefly by the oculomotorius, which comes on either following 
or during a migraine attack. Inasmuch as oculomotor pareses or par- 
alyses may occur from a great diversity of causes, apart from a migraine, 
and may appear periodically, it has been held by many that the term 
ophthalmoplegic migraine has no particular right to exist, but the evidence 
is too great to eliminate migraine as a competent producing cause for 
these periodic oculomotor paralyses. 
Etiology.-Whether heredity plays any greater part here than in migraine 
in general is difficult to decide. The present attitude is to restrict the 
influence of heredity in migraine; certainly there is very little evidence 
of direct transmission of ophthalmoplegic migraine as such. Yet if 
there is any truth in the Spitzer hypothesis, heredity should be much 
in evidence in the ophthalmoplegic variety. Perhaps it is, but the 
cases are few and far between. 
There has been much speculation concerning the central or peripheral 
nature of this third nerve palsy. The present view taken for migraine 
in general, that it is due to a disturbance in cerebral pressure, secondary 
to vascular modifications, is sufficient to account for the oculomotor 
palsies as well, in view of the location of the peripheral branches of the 
third nerve in regard to the cerebral vascular plexuses. In fact, the 
occurrence of the ophthalmoplegic type is one of the strong arguments 
for the general pressure hypothesis, as Spitzer has argued. If, as has 
been shown by several autopsies, to these considerations additional local 
causes be added, which increase or permanently maintain such pressure 
effects, the interpretation is comparatively simple. Thus, exudates, 
fibrous processes, swelling in the cavernous sinuses, swelling of the 
hypophysis, tumor formation, gummata, etc., have been found in 
patients suffering from periodic oculomotor paralyses associated with 
migraine. 
It is true that some of these are to be interpreted as symptomatic 
migraines, in which the foreign body acts primarily as an irritant to cause 
the vascular disturbance, which sets free the migraine reaction, and 
secondarily serves as an additional cause of pressure to bring about the 
palsy. In a personally observed case, with basal gummata, the periodic 
oculomotor palsy and migraine attacks have occurred for a period extend- 
ing over four or five years, usually with every menstrual period. Here 
were three interplaying factors, and the exact part played by each can 
only be inferred. The slight disturbance of menstruation, usually 
adjusted, in this case, by reason of the exudate, was not. A migraine 
was set up, the acute pressure of which, added to that of the exudate, NEURALGIA 
725 
caused the ophthalmoplegia which had become fairly persistent in the 
intermigrainous interval in recent years. 
Symptoms.-Leaving aside for the moment the atypical and sympto- 
matic periodic oculomotor paralyses due to other causes than migraine, 
one finds in these patients, usually during or after a severe attack of 
unilateral migraine, with headache, nausea, vomiting, etc., a ptosis of 
the eyelid on the same side, and a loss, partial or complete, of the upward, 
downward, and inward movements of the eye of the same side. This 
eye is usually directed outward and downward, and the patient sees 
double. This may or may not be accompanied by sensory disturbances 
in the superior branch of the trigeminus. 
After a variable length of time, a few days, a week or more, the paralysis 
disappears, usually gradually, and the patient suffers no inconvenience 
from the ocular palsies or the ptosis. In some individuals such palsies 
accompanying a migraine have come on comparatively young in life, 
almost with the beginning of the migraine attacks; for the majority 
however, they follow several years after the establishment of a migraine, 
in some instances as late as thirty years. In some only a very severe 
attack will be accompanied by the oculomotor signs, or only slight palsies; 
transitory ptosis may occur frequently. But in others the palsies develop 
with each attack of migraine and often in increasing severity. The effects 
may persist longer and longer between the attacks, until in a few they 
become permanent palsies. This type, however, often permits of other 
interpretations. 
A double lesion can be understood, although it rarely occurs. Isolated 
abducens palsy has been described, also isolated trochlearis; and complete 
ophthalmoplegia is reported in a single case, but in view of the many 
contributory factors it perhaps is preferable to view such a case from 
another standpoint. 
Diagnosis.-Every patient should be regarded as one suffering from 
something more than the migraine, until all accessory causes are excluded. 
Treatment.-Little needs to be added to the therapy outlined under 
migraine. Syphilis as a cause for both a migraine and an exudate should 
be treated, and the Wassermann reaction utilized to clear up the diagnosis. 
NEURALGIA. 
Definition.-A painful affection of the nerve trunk or its branches, 
characterized by remittent or intermittent rapid flashes of pain, with 
free intervals, not usually accompanied by trophic disturbances of the 
muscles, unless its severity limits the activities of an organ, occasionally 
associated with painful nerve trunks and with disturbances in the skin 
structures. Neuralgias are but the expression of many diverse lesions 
which may involve the body in general, as toxic and infectious states, 
a nerve trunk itself, the sensory ganglia, contiguous structures, or they 
may be the reflex expression of a disorder in a viscus, apparently unrelated 
to the nerve fibres, which are the site of the pains. Neuralgia is, there- 
fore, to be considered solely as a symptom, a syndrome, or a purely 
reflex condition. Pure idiopathic neuralgia may occur solely as a psycho- 726 
DISEASES OF THE NERVOUS SYSTEM 
neurosis-a real hysterical conversion. They are not rare, and may 
involve any nerve distribution. 
Etiology.-An extraordinary wide range of causative factors may 
determine mild or severe neuralgias. The most frequent are: 
(a) Anemia. This is a potent factor in reducing resistance to pain 
and in determining the outbreak of a painful reflex disturbance. 
(b) Toxins of exogenous origin, inorganic and organic, or purely 
endogenous toxins. Thus, poisoning by lead, mercury, arsenic, copper, 
especially mild cases, often causes severe neuralgias. Alcohol and tobacco 
are frequent organic poisons, while the toxins of many infectious disorders 
are especially prone to bring about neuralgias. The morphinist or 
cocainist suffers severely from neuralgia as an abstinence symptom. 
Influenza, tonsillitis, and smallpox are familiar illustrations, while the 
malarial organism causes a toxemia with a pronounced aptitude to 
induce neuralgia. Typhoid fever, measles, gonorrhoea, possibly syphilis, 
and streptococcus infections are frequently accompanied by neuralgias. 
The toxemias of diabetes and nephritis frequently cause severe neuralgia, 
while those of hyper- and hypo-thyroidism are not infrequent. 
(c) Inflammation of the sensory ganglia, which may be either of infec- 
tious or non-infectious nature, gives rise to some of the severest forms, 
as seen in herpes zoster. According to the ganglion involved, there may 
result an ear neuralgia-otitic neuralgia, a zoster of the seventh nerve 
(Hunt), in one or more of its branches-zoster of intercostal ganglia, 
or even down to the lower extremities. Ganglion involvements of non- 
infectious types give rise to neuralgias such as tic douloureux, while 
tumors of the sensory ganglia may condition persistent and obstinate 
neuralgias in the distribution of the affected sensory nerves. 
(d) Involvement of the nerve trunks themselves, either by mild 
neurotic processes, perineuritis, pressure from anatomical structures, 
pressure from lesions, cuts, bullet wounds, tears, tumors, aneurism, 
exostoses, fractures, or displacements may cause severe neuralgias, which 
by the advance of inflammation, the persistence of pressure or the con- 
tinuance of the lesion finally show the neuritic reaction. 
(e) Reflex neuralgias are numerous and puzzling. Pulmonary, cardiac, 
gastric, hepatic, renal, ureteric, intestinal, vesical, uterine, ovarian, 
prostatic, testicular, and affections of other viscera give rise to herpetic 
eruptions, with painful, sensitive skin areas and neuralgias; in many 
instances the neuralgia is not accompanied by herpes. Head's complete 
analysis of this class of cases is of paramount importance.1 Thus, a 
persistent sciatica may be the reflex of a prostatic disturbance. An 
anemic woman may not suffer from pain, but on menstruation her referred 
neuralgic pains may become very severe over the tenth dorsal nerve, 
and pain and tenderness are frequent over the areas of the sixth dorsal 
(heart), seventh dorsal (stomach), and there may be occipital and mid- 
orbital neuralgia (Head). 
(/) Psychical neuralgias are frequent. They are usually hysterical 
conversions, but may be found in a number of psychoneurotics and in 
some psychoses, mild depressed manics, and schizophrenics. 
1 Brain, xvi, 1; xvii, 339; xix, 153. Neuralgia 
727 
(g) Organic disease of the nervous system. General paresis, tabes 
dorsalis, spinal or cerebral disease, thalamus involvement, syphilitic 
meningomyelitis, etc., are often accompanied by neuralgic pains. 
(A) Hereditary predisposition. The neuropathic constitution, hysteri- 
cal personality, physical and mental overwork may be added to the 
conditions mentioned as responsible. Some individuals have neuralgic 
pains on the slightest provocation. The arthritic, gouty, rheumatic, 
and scrofulous may be said to be predisposed. Unknown factors thought 
to be related to atmospheric pressure, humidity, high electrical tension, 
etc., play a role in many of these cases. 
(i) Chronic vascular disease, and especially arteriosclerosis, is a 
frequent cause, particularly in the aged, the senile, and the presenile. 
Syphilitic neuralgias are frequently conditioned by vascular disease. 
(j) Last, but not least, exposure to cold is an important factor. It is 
not certain that all neuralgias caused by cold are not really mild types 
of neuritis or perineuritis, but since a neuralgia may be the expression of a 
mild neuritis or perineuritis, discussion of the distinction is fruitless. 
Symptoms.-Pain is the main factor in neuralgia. For the most part, 
it is the only expression of the nerve disturbance. The character of the 
pain varies considerably, but in general it may be described as unilateral 
and paroxysmal. It is characteristic of most neuralgias that they are 
not primarily localized in the periphery. The pain seems to begin 
beneath the surface, and may then shoot out to the periphery. It may 
be described as biting, boring, tearing, darting, cutting, like an electric 
shock, like a hot iron, etc., each patient having his own pet expression. 
It may come and go in lightning-like flashes or throbbing pulsations, 
persisting for a shorter or longer time, then stopping for minutes, hours, 
or days, then recurring. When continuous, the pain varies considerably 
in its intensity. At times it is agonizing; then again it is growling and 
grumbling beneath the surface. 
The painful area in most neuralgias conforms to the peripheral distribu- 
tion of the sensory nerves. In the herpetic and referred neuralgias 
the root zone area is involved; this supplies an important differential 
in determining these types. At times strictly localized to a more or 
less definite spot, again the pain radiates through an entire limb, over 
one-half of the face, or the trunk. The radiating character of the pain 
in some neuralgias is very striking. It spreads out along the peripheral 
branches of a nerve stem. As in the trigeminal neuralgias, one may 
find the upper, middle, or lower branches involved; alone, in combination, 
or all three. A dental neuralgia may suddenly jump to other branches 
of the fifth nerve and the pain become general. Individual peculiarities 
are constantly met, in which case one theoretically assumes differences 
in irritability of the nerve substances, in the central gray matter. 
Certain points seem to be foci from which the pains start. These are 
usually situated along the nerve trunks, and pressure upon them is 
often sufficient to cause an exacerbation in a mild attack, or to provoke 
an attack in a period of intermission. Valleix attached considerable 
importance to these points. They are found, according to him: (1) At 
the point of emergence of the nerve trunks; (2) at such situations where a 728 
DISEASES OF THE NERVOUS SYSTEM 
nerve trunk transverses a muscle to reach the skin; (3) at points where 
the nerve fibre breaks up-into branches; (4) at points where the nerve 
becomes very superficial; and (5) at Trousseau's apophyseal points. 
Valleix's points play a secondary role in present day interpretations of 
neuralgias, and Romberg has given a severe critique of Valleix's claims. 
They are of interest particularly in the neuritic types, but are absent 
in other forms of neuralgic affections. 
Accompanying phenomena are frequent. In some patients a sense of 
apprehension may precede the coming on of an attack; vague sensations 
of discomfort often antedate the neuralgic outbreak. Ripples of pain, 
like pin pricks, short twinges, etc., announce the advent of a more 
serious attack, or may be the sole evidence of an abortive one. Such 
mild phenomena are extremely frequent in certain of the so-called pre- 
disposed or neuralgic individuals. Some feel that they cannot live at 
high altitudes; others fear rain, or an east wind; a thunder storm cause 
others to have twinges; while, again, certain dietary indiscretions make 
others complain of painful twinges for days. Just what conditions are 
at the basis of these features is unknown, yet they are none the less real. 
One must always bear in mind the indubitable influence of purely mental 
influences; this does not make the pains any the less real but opens a 
view as to possible therapeutic resources. 
Skin hypersensitiveness is frequent. It may precede or accompany 
an attack, and persist after the pain has ceased. Epicritic sensibility 
is mostly implicated. Light touch, a pin prick, or slight degrees of heat 
and cold are magnified. Deep pressure and extremes of heat and cold 
are usually palliative. Anesthesia is not infrequent following an attack 
of pain, and the exact topographical distribution of the sensory modifica- 
tions on the skin throw considerable light on the possible causation of a 
neuralgia (Head). Trousseau's painful spinal point should be recalled in 
this connection. Paresthesia is very frequent, and the femoral distribu- 
tions seem to show it more than others, "meralgia paresthetica." 
Motor disturbances, either as cramp-like contractions or as paralyses, 
are not infrequent in accompanying conditions. 
Vasomotor and secretory symptoms are frequent. The bloodvessels 
are frequently contracted in the early stages of a neuralgic attack, with 
resulting blanching and cooling of the skin. Following this a period of 
warmth, of redness, of free perspiration may result due to the secondary 
dilatation of the vessels. In many cases of trigeminal neuralgia other 
secretions may be modified. Crying, coryza, or salivation are not infre- 
quent, while in general neuralgic attacks an increase in the amount of 
urine and of milk secreted is frequently found. 
Premature graying of the hair, loss of hair, thickening of the skin, 
erythemata, eczema, pemphigus, herpes, thickening of the bones, and, 
occasionally, muscle atrophy are among the rarer trophic by-products. 
During an attack irregularities of the pulse are not unusual; slowing is 
the rule. The pupils are frequently dilated, at times unequally. 
The general physical and psychical reactions are extremely important. 
Loss of sleep and anorexia cause the patient to lose strength and flesh; 
and anxiety, irritability, and petulance are almost inevitable. Mental NEURALGIA 
729 
depression is not infrequent, especially in severe cases of trigeminal and 
sciatic neuralgia. The contracting of a drug habit is not unusual. 
Course.-This depends naturally upon the underlying condition. 
Many are acute and transitory, persist for three or four days, and never 
reappear. Such are the herpetic types. On the contrary, certain 
neuralgias are persistent, chronic, and show a tendency to grow worse. 
Tic douloureux is an example. Many reflex neuralgias run an acute 
recoverable course, but show a marked tendency to recurrence. The 
neuralgias which accompany the chronic cachexias of nephritis, carcinoma, 
brain or spinal cord disease usually progress in a markedly chronic manner. 
In those hereditarily disposed individuals the tendency to chronicity 
with longer and shorter periods is proverbial. Most neuralgias in which 
the causative factor is undiscoverable run a benign course, while the 
neuritic types are less amenable to treatment. 
The subdivisions of neuralgic neuroses, subacute neuritic neuralgia, 
and chronic neuritic neuralgia offer a grouping referable to the course 
which has only clinical convenience to warrant it. 
In the first type one finds the disorder limited more or less to the 
neuropath. The attacks come on without appreciable cause, or follow 
a nervous shock, exposure to cold, or dietary indiscretions. The pain 
comes on with great suddenness and usually without great violence; it 
comes and goes apparently without rhyme or reason, and is not accom- 
panied by painful nerve trunks or trophic disturbances. It recovers 
at times, to recur at intervals of a year or years. 
In the subacute neuritic neuralgic type, exposure to cold or to pressure, 
especially in arthritic patients, determines an attack. The attack 
develops gradually; the pain, at first mild and intermittent, gets worse 
and worse and more continuous. Finally, after a day or more the 
paroxysms become extreme, the intervals between marked by dull pain; 
Valleix's points are characteristic findings. When involving a mixed 
nerve, muscular atrophy or other trophic signs appear, signalizing the 
occurrence of a neuritic process. Local oedema and herpes zoster are 
frequent accompaniments. This type usually commences to recover in 
from two to three weeks, and an ultimate recovery is to be expected. 
Recurrences occur, however, and leap to the third type of chronic neuritic 
neuralgia. This form is frequent in the aged. The history is usually 
that of several subacute attacks with increasing tendency to chronicity. 
Here the trophic disturbances in muscle and in skin are more marked. 
The paroxysms run a remittent course. 
Diagnosis.-Enough has been said to emphasize the need for a searching 
analysis of the causative factors in every neuralgia. They are many, 
and presumably the most widespread diagnostic error is the overlooking 
of an early tabes dorsalis in young to middle aged adults. Children are 
not prone to neuralgias-in the narrower sense-and a neuralgic affection 
in childhood calls for close scrutiny. 
Since unilateral pain, of special localized type, occurring in irregular 
attacks, is almost the sole criterion of neuralgia, it is very frequent that 
organic disease of a viscus will show precisely similar accompanying 
features. In the majority of cases the underlying organic lesion may be 730 
DISEASES OF THE NERVOUS SYSTEM 
detected-occasionally it remains difficult to locate. Not infrequently 
the diagnosis of a persistent neuralgia may be cleared up by the finding 
of malarial organisms in the blood, or more rarely the presence of a marked 
eosinophilia will call attention to trichina as the cause of an obstinate 
neuralgia. Syphilitic neuralgias are by no means infrequent. 
The diagnosis of myalgia from true neuralgia is not often difficult, 
but occasionally, especially in the intercostal and lumbar regions, the 
diagnosis becomes uncertain. These neuralgic-like myalgias are usually 
isolated in their location, are not, as a rule, accompanied by acute exacer- 
bations, nor are the regions usually painful on pressure. Motion, on 
the contrary, usually aggravates myalgia. Motion of the jaw-it may 
be recalled-is enough to start a severe tic douloureux. 
Neuralgic pains about the jaw are at times the precursors or accom- 
paniments of new-growths of the jaw or parotid (Gowers). Neuralgic 
affections of the cranial nerves are not infrequently due to intracranial 
growths. 
Neuritis of a mild grade offers a specially difficult problem. As already 
stated, mild neuritis is a neuralgia. The question to be solved concerns 
the likelihood of a more severe degree of neuritis. In this case the usual 
signs of neuritis appear-painful swollen nerve trunks, trophic disturb- 
ances, more continuous pain, Lasegue's phenomenon, weak, flabby muscle 
fibres and electrical changes. New-growths pressing upon or involving 
the nerve trunks within or without the spinal canal, in the early stages 
particularly, begin their disturbance by a pure neuralgia. Minute 
analysis of the sensory phenomena will usually clear up the diagnosis 
early, although in the earliest phases at times it may be impossible. 
A neuralgic affection may be one of the earliest signs of multiple 
sclerosis. Oppenheim has found a severe tic douloureux to have been 
the earliest sign of this disorder. Syringomyelia may begin as a localized 
neuralgia. Minute hemorrhagic lesions of the spinal cord of traumatic 
origin give rise to neuralgias. 
In the diagnosis of hysterical neuralgia great caution should be exercised. 
Hysterical neuralgias partaking of the nature of a pseudo-neuralgia are 
extremely diffuse, and may react very rapidly and markedly to suggestive 
influences. Hysterical neuralgias are almost invariably accompanied by 
other hysterical conversions. 
Neurasthenic pains need to be differentiated. The many mixed forms 
of neurasthenic, hypochondriacal, and hysterical origin bear their char- 
acteristic sidelights. The diagnosis of these types of neuralgia should 
not be lightly made, for it is not to be forgotten that these syndromes of 
themselves may be the reaction on the part of the nervous system to 
some more fundamental organic lesion. Thus, patients suffering from 
severe neurasthenia, with cachexia, and severe intercostal neuralgic pains 
may have an undiscovered carcinoma of the stomach, mediastinum, etc. 
In tabes dorsalis the pains resemble those of neuralgia very closely, 
but, as a rule, have a wider range, are not localized in a peripheral nerve 
distribution, and are more apt to be radicular in their distribution. Pain 
on pressure of the nerve trunk is usually absent. 
The pains, cramps, and muscular weakness of intermittent claudication NEURALGIA 
731 
sometimes bear some resemblance to a severe neuralgia. Aortic aneurism 
gives rise to reflex neuralgic pains, usually very severe. Aneurisms in 
other regions are to be carefully excluded. 
In reflex neuralgias the use of cocaine or other local anesthetic may 
determine by exclusion, the site of the original lesion. An orthoform 
suppository pressed well against the prostrate has been known to relieve 
a severe sciatic neuralgia. Tumors of the pelvis frequently give rise to 
sciatic and crural neuralgias, and persistent neuralgic pains of the knee 
are often a reflex from hip-joint disorder. 
Prognosis.-This is conditioned by the pathological process that is 
responsible. The more chronic of the neuralgias, which in years gone by 
tended to bring about chronic invalidism or inveterate drug habits, have 
ceased to have such a sinister import by means of a better understanding 
of the underlying conditions, and by a much more resourceful therapy. 
The younger and stronger the individual, and the less the tendency to 
hereditary disposition, the better the prognosis in those neuralgias which 
are idiopathic, as well as those due to alcohol, lead, etc. In the more 
chronic forms, not due to removable conditions, the prognosis is bad. 
Treatment.1-The chief indications are to quiet the pain and root out 
the cause. A painstaking study of the history and an exhaustive physical 
examination are necessary in all cases. The therapy will vary, therefore, 
widely if the cause be ascertained; a course of quinine will cure one patient, 
a surgical operation may be called for in another. 
Taking up the general therapeutic indications, the analgesics which 
have proved serviceable may be discussed first. From the true thera- 
peutic standpoint they are solely palliative. Many cases of severe 
neuralgic pain may be temporarily subdued by the synthetic analgesics 
and the dangers of a morphine habit averted. Phenacetin, acetanilide, 
antipyrine, aspirin, pyramidon, lactophenin, and phenocoll are among 
those that have proved valuable. New ones are being added, and 
among them some are certain to be of value. The salicylic acid group 
combinations are at times useful, especially in the milder cases and in 
patients with arthritic tendencies. In influenza and tonsillitis neuralgias 
the salicylates are useful. Combinations of these with hypnotics, such 
as chloral, paralydehyde, sulphonal, trional, or veronal, are useful in 
procuring sleep, and thus prevent the reduction of the patient's resistance. 
If any of the opium group be necessary, it is better to give such in 
sufficient doses. Usually smaller doses may be given when combined 
with the analgesics mentioned. Aspirin, gr. vii (0.5 gram), codeine, 
gr. | (0.02 gram), and trional, gr. vii (0.5 gram), for instance, is a useful 
combination in mild cases. In chronic neuralgic pains morphine is to be 
avoided as long as possible. This does not apply to a very old patient, 
or one in whom the neuralgia is simply the expression of some chronic 
incurable disorder-carcinoma, for example. The gradual immunity 
acquired, with the need for larger doses, and the pernicious effects of a 
habit apply to all the members of the opium group. 
1 Schmidt, in Modern Treatment of Nervous and Mental Disease, edited by White 
and Jelliffe. 732 
DISEASES OF THE NERVOUS SYSTEM 
It is doubtful if chloroform or ether is to be recommended by inhalation 
in severe neuralgias. They are useful temporarily in great crises. 
Other drugs are quinine, which in combination with the salicylates is 
specially valuable; arsenic, which is serviceable in the neuralgias due to 
anemia, especially in combination with iron. Atropine and aconitine 
were used widely before the days of the antipyritic analgesics. Their 
definitely poisonous qualities have driven them into the background. 
The unreliability of Cannabis indica has done the same for this otherwise 
useful analgesic. The iodides are called for in the syphilitic neuralgias, 
and are useful in many neuritic neuralgias. 
Counterirritation is of great service in most cases of severe neuralgia, 
especially after the acute onset is over. The Paquelin cautery is the best 
means; mustard paste, cantharides, turpentine, chloroform, ether, and 
acupuncture all have their place. Local freezing may be carried out by 
ethyl chloride, methyl chloride, ether, or other volatile substances. 
Menthol, or other similar derivatives may be used for mild neuralgic 
pains to advantage. Local applications of heat are grateful and valuable. 
Hot water bags, hot sand, electrical pads, etc., may be utilized. General 
or local hot water baths, or hot air baths (baking), are at times desirable. 
Direct applications of local analgesics, either to the nerve trunk or 
within the spinal canal, are valuable in many obstinate deep-seated 
neuralgias, especially of medullary origin. Cocaine, tropacocaine, 
eucaine are all useful given by the Corning or Quincke method. 
General hygienic treatment is imperative. A generous diet, full 
sleep, healthful occupation, and freedom from mental worry are essential. 
Cod-liver oil, nitrogenous diet, with iron, arsenic, strychnine, calcium 
salts, are indicated. Faddy dietaries should be avoided. Even in 
arthritic neuralgias it is doubtful if meat does any particular harm when 
not taken to excess. Alcoholic beverages are to be denied. 
Hydrotherapy may be added to other hygienic means, if it proves 
invigorating and causes an increase in appetite and sleep. 
Climatic changes are often advisable. Low-lying, damp, and humid 
atmospheric conditions seem least desirable. The general stimulus 
that comes from a drier, higher atmosphere, even if colder, works to the 
general advantage, even if not directly valuable for the relief of pain. 
Electrotherapy, when well managed and properly selected, is of great 
value in some neuralgias. In general, Leduc's modifications of the 
d'Arsonval's rapidly interrupted current offer the readiest and most 
widely applicable form of electrical current for the relief of neuralgic 
pain. Such treatment is purely palliative and is pernicious if it leads 
one to overlook causal factors. Most of the cures due to galvanism are 
psychical. Some intractable neuralgias need a psychoanalytic cure. 
Surgical intervention is called for in all cases in which pressure is 
demonstrable and the cause removable. Tumors and new-growths, 
involving or pressing upon nerve structures, if removable, should be 
taken away. Surgical interference may be of radical service in many 
of the reflex neuralgias of obscure-origin probably related to visceral 
ptoses. Nerve stretching needs mention mostly to be condemned. NEURALGIA 
733 
SPECIAL LOCALIZED FORMS OF NEURALGIA. 
While any nerve fibre in the body may become painful, there are certain 
regions which show a greater tendency to involvement than others. 
Bernhardt has collected the statistics of localized distribution in some 
685 cases, with the following results: Trigeminal, 124; occipital, 42; 
brachial, 108; intercostal, 45; lumbo-abdominal, 12; crural, 25; obturator, 
2; sciatic, 303; anterior femoral, 11; Achilles, 3; tarsalgia, 4; metatarsalgia, 
4; and coccygeal, 2. In 613 cases collected in the clinic of Dr. M. Allen 
Starr during four years (1902 to 1906) the distribution was as follows: 
Trigeminal, 315; occipital, 28; brachial, 31; ulnar, 1; intercostal, 19; 
lumbo-abdominal, 19; crural, 2; sciatic, 194; coccygeal, 1; peroneal, 2; and 
plantar, 1. 
Thus, with the New York population the affections of the three branches 
of the fifth stand first, the sciatic next, while the brachial, occipital, 
intercostal, and lumbo-abdominal follow. Bernhardt, Lachnit, and 
Conrad have found sciatica to be the commonest type, with trigeminal 
neuralgias second. Eulenberg has found, with the author, that the 
trigeminal affections have been more frequent. In the Vanderbilt 
Clinic (New York) the trigeminal neuralgias have been more common 
in women, while the sciaticas have predominated very markedly in men. 
Trigeminal Neuralgia.-Neuralgias of the branches of the fifth nerve 
are among the commonest of all the neuralgias. The inferior and superior 
branches preponderate in frequency of involvement. Most frequently 
these neuralgic pains are due to some affection of one of the branches. 
Inflamed teeth play a minor role. Affections of the ears, the eyes, 
iritis, cyclitis, iridocyclitis, the skin of the face or head, inflammation 
within the accessory sinuses of the nose, forehead, antrum, mastoid, all 
of these may produce diffuse neuralgic pains, at times clearly separable 
from a simple neuralgia of the fifth, at other times not. The character- 
istic type of trigeminal neuralgia is tic douloureux; trigeminal herpetic 
neuralgia, involving one or more branches, is not uncommon. 
Cold and wet are very important agents in facial neuralgia. In certain 
countries, notably England and the north of Germany, facial neuralgias 
from this cause are extremely common; they seem to be much less frequent 
in the United States, and notably so in southern countries. 
Tabes dorsalis, or multiple sclerosis, may make its first appearance 
as a trigeminal neuralgia. 
Neuralgia of the superior branch is seen more commonly by physicians, 
although the dental branches are involved much more frequently. 'These 
patients go to dentists, and hence do not enter into medical statistics. 
This is a reason why it is incorrectly stated, by most writers, that the 
superior branches of the fifth are most often involved. For the most 
part the milder types of facial neuralgia are induced by irritation of some 
of the terminal filaments, while in the severe form, tic douloureux, which 
is the more classic, a lesion of the Gasserian ganglion is uniformly 
present. Mild cases of tic douloureux may be indistinguishable clinically 
from other types of facial neuralgic pain. 
It would be desirable to restrict this term to a definite and, if possible, 734 
DISEASES OF THE NERVOUS SYSTEM 
limited type of neuralgia of the fifth nerve, particularly to the form due 
to changes of a chronic degenerative nature occurring in the Gasserian 
ganglion. This is not yet possible, and clinically the neurotic and per- 
ipheral cases are either not at all separable from the ganglion cases, or 
with considerable difficulty. 
It affects one side of the face only, as a general rule. In certain gen- 
eralized neuralgias, such as those due to diabetes or nephritis, both fifth 
nerves may become involved. In the majority of cases selection occurs 
among the branches, one or two being involved, rarely all three; the 
ophthalmic branch the oftenest, the inferior maxillary the least. 
The more classical tic douloureux neuralgias are characterized by the 
extreme severity of the pain, usually preceded by paresthetic prodromata, 
and widely accompanied by sympathetic or irradiating pains in other 
branches than the one chiefly involved, or in other nerves. The pain 
may be paroxysmal or continuous, with marked exacerbations. They 
have been compared to the piercing pains of a sharp knife or the burning 
of a red-hot wire. The patient remains for a shorter or longer period, a 
few minutes to several hours, under the grip of the pain, unable to move a 
muscle of the face or fearful of stirring, lest a spasm more fearful than the 
others should occur. The slightest touch is avoided, even the air pressure 
of a suddenly closed door may bring about an exacerbation. The longer 
attacks are rarely as vicious as the shorter ones. 
Valleix's points are relatively constant. In the ophthalmic involve- 
ment the sore parts are found above the supra-orbital notch, at the 
external angle of the upper lid, the upper outer aspect of the nose, and the 
globe of the eye; in the superior maxillary branch the inferior orbital 
notch is the chief point of pain; the malar bone, and opposite the last 
upper molar are other less frequently found points, while the outer 
angle of the mouth and the roof of the mouth are rarely their site. In 
the inferior maxillary distribution the points are chiefly just in front of 
the auditory canal, the site of the tongue, the border of the chin, and 
Trousseau's points over the first and second cervical vertebrae. 
Vasomotor and secretory disturbances are usual. The skin is, as a rule, 
hot and swollen, occasionally pale and frigid; tears, nasal secretions, and 
saliva flow in abundance. The eyelids may be swollen, the conjunctiva 
reddened, to the point of ulceration at times; within the nose and mouth 
extravasations occur, and ulcers are not uncommon. Herpetic attacks 
are also not infrequent, and in some of these attacks grave injury to the 
eye structures may take place. Glaucoma is one of the severe complica- 
tions. Other trophic disturbances have been noted, such as skin erup- 
tions, acne, erysipelatous reddening, graying of hair, and blackening of 
the tongue. In continued cases hemiatrophy is known to occur. Changes 
in the sense of taste, of touch, of hearing, are at times present. Photo- 
phobia is frequent, while diminution in the visual fields and accommoda- 
tion cramps are noted. Gelle has described a neuralgic deafness, due 
possibly to labyrinthine effusion in protracted cases. 
Severe mental disturbances, amounting at times to hallucinatory 
confusion, may be present, and profound states of depression, which have 
resulted in suicide, in isolated cases, are to be guarded against. NEURALGIA 
735 
The motor disturbances consist in convulsive movements of the facial 
muscles (convulsive tics, spasmodic epileptiform neuralgia, Trousseau), 
sudden forced closing of the eyelids, drawing of the mouth to one side, 
or sudden turning of the head. At times the convulsive movements 
extend to the arms. Paralytic phenomena in the third nerve are noted. 
The general physical disturbances noted are prone to occur in this type. 
Course.-In the majority of cases the attacks appear in series and attain 
a periodicity which comes to be dreaded by the sufferer. The free 
intervals usually become shorter and shorter; but many patients may 
have only one attack a year, especially in cold weather, or even at longer 
intervals. A single attack may last a few days, or in the severe forms 
several weeks, the patient not being free from pain day or night, save 
under the influence of morphine. Some patients have a few attacks in a 
lifetime, others are not free from the disease for years. The severer 
convulsive forms are prone to occur late in life, when resistance is low. 
Diagnosis.-Ordinarily the classical form of tic douloureux is recognized 
without difficulty. Patients have had all their teeth extracted, however, 
under the mistaken diagnosis of dental disease, while some intractable 
facial neuralgias have been cured by proper attention to diseased teeth. 
Aneurism of the carotid, tumors pressing upon the nerve or upon the 
Gasserian ganglion, may be difficult to determine as the exciting cause. 
These, however, are usually accompanied by accessory symptoms, 
palsies, eye-ground changes, aneurismal murmurs, pain within the head, 
cerebellar syndromes, ear pains, etc. 
Treatment.-It is as essential to endeavor to find and treat the cause 
of a facial neuralgia as for neuralgia in general. The various remedies 
given under the heading of neuralgia may be tried, and as malarial 
neuralgias are very frequently facial, energetic quinine therapy should 
be given; the absence of blood findings is not contra-indicative, especially 
in malarious regions. Arsenic is a useful adjuvant in these patients, 
and also valuable in non-malarial neuralgias. Gelsemium, the tincture 
in 10 minim doses, gradually ascending, aconitine in doses of -5-^ grain, 
Cannabis indica (fresh) in doses of f to | grain, are reputed as specially 
valuable in the facial cases. Any of the analgesic antipyretics may suit 
individual cases, and avoid the use of morphine, which alone is reliable 
in many severe cases. 
Injection methods have been tried for years. Osmic acid was used 
which set up a degenerative process, and in many instances relief was 
obtained, but regeneration took place even when the injections were 
practised within the main trunks. Schlosser has perfected a method of 
injecting alcohol within the substance of the Gasserian ganglion, which 
has given excellent results. The chief feature in the deep alcohol injec- 
tion method is the introduction of a long, dull, cutting needle into the 
foramen ovale and there injecting in situ the branches of the trigeminus. 
Narcosis is not necessary; 80 per cent, alcohol is used. In three to four 
hours following the injection pain is relieved, and two or three more 
injections are given within a week to complete the treatment. Imme- 
diately following the injections, there is a marked anesthesia on one side 
of the anterior part of the head, including the nostril, palate, and one- 736 
DISEASES OF THE NERVOUS SYSTEM 
half the tongue; a slight paralysis of the muscles of mastication, which 
usually disappears in a few hours; a degenerative process is set up in the 
nerve trunk, which is recoverable, and general sensibility usually returns, 
but the pain is absent. Relief extending over a year in a number of cases 
is reported by numerous observers. Some patients have been relieved 
for four or five years. (Edema of the posterior eye structures and hemor- 
rhage are among the discomforts and even dangers of the operation, 
especially in the use of the intra-orbital methods devised by French 
operators. Repeated injections delay the recurrences more and more, 
and thus the patients may be held immune to the pain. Numerous 
modifications and later methods have been devised. 
As regards surgical means, three procedures have been seriously 
advocated. The first and earliest consisted of peripheral section. Sec- 
tion of the fifth may be employed to advantage in those cases in which 
the disease is undoubtedly peripheral. As modified by more recent 
procedures, the older objection that regeneration takes place is partly 
done away with, and in selected cases peripheral section may be preferred 
to the more severe and serious operations. 
Rose,1 in 1890, first successfully extirpated the ganglion for tic dou- 
loureux. Horsley, Hartley, and Krause further perfected the operation, 
and the modified Hartley-Krause operation by the temporal route has 
been largely the method of choice. Cushing's modifications are of lasting 
value. The operation still remains one of much difficulty and serious- 
ness. Recurrences are known even with this method. 
Van Gehuchten,2 in 1903, suggested what he termed the physiological 
section of the roots of the Gasserian ganglion by tearing. His extensive 
studies on regeneration in nerve fibres showed him that the central side 
of the ganglion must be attacked if the problem of recurrence was to be 
solved, and, furthermore, his method took into account the small number 
of intracranial cases not reached by the more peripheral methods. Spiller, 
as early as 1898, had suggested the surgical expedient of cutting the 
sensory root, which he claims is safer than the operation of tearing. 
The method of division of the sensory root promises to be one of the 
most valuable surgical procedures thus far devised. 
Cervico-occipital Neuralgia.-This occurs in the distribution of the 
sensory nerves of the cervical plexus, consisting chiefly of the occipitalis 
major, the occipitalis minor, auricularis magnus, cervicalis superior, 
supra-clavicularis and phrenic. Neuralgia in this region seems rare. 
Etiology.-The several causes of neuralgia are operative here, and 
need not be repeated. Special determining features seem to be the 
carrying of heavy weights on the shoulders (a more frequent cause seen 
in brachial neuralgias), arthritis deformans of the upper cervical vertebrae, 
caries, syphilis, tuberculosis, tumors, pachymeningitis, falls and blows 
wrenching the cervical vertebrae, enlargement of the cervical lymphatics, 
and aneurisms of the vertebral artery. Psychical neuralgias in this 
general region are infrequent. 
The pains occupy the regions mentioned, being particularly localized 
1 Lancet, 1892, i, 295. 
2 La Nevraxe, 1903, v, 201. NEURALGIA 
737 
in the neck, below the occiput, and running up to the vertex, occasionally 
behind the ears. The Valleix point found most frequently is the occipital 
point between the mastoid apophysis and the first cervical vertebra; 
points between the sternomastoid and trapezius (cervical), the anterior 
border of the mastoid, and the middle of the ear are of less frequent 
occurrence. The pain is very frequently bilateral. Dull pain on press- 
ure, with tender skin, is usual as an interparoxysmal occurrence. This 
tends to make the sufferer hold his head in a stiff position, which in time 
may cause a characteristic attitude. The tenderness may be so acute 
that ruffling of the hair will start a paroxysm. Graying of the hair, 
loss of hair, with other trophic signs, may be present. Sudden pulling 
back of the head is an occasional symptom. 
Diaphragmatic Neuralgia.-This form of neuralgia, also known as 
phrenic neuralgia is of rare occurrence. The pain is usually present 
near the free border of the ribs, or within the chest, between the ribs, 
occasionally as high as the chin and in the neck, beneath the clavicle, 
and in the scalenus anticus muscle. Trousseau's points are located over 
the second to the fifth cervical vertebra. The pain frequently runs 
down the arm, especially in certain complex cases of mixed brachial 
neuralgia. Breathing may be seriously interfered with, the breath 
coming fast and short; longer excursions of the diaphragm are impossible. 
It is a common experience to have a sharp, short stitch in the side, with 
inability to breathe for fear of pain. In the majority the pain is in the 
left side. Anemia, affections of mediastinum, heart, and pericardium, 
and aneurism of the aorta are the most frequent attending features. An 
intractable phrenic neuralgia may complicate an exophthalmic goitre, 
or be present in carcinoma of the neck region. 
Brachial Neuralgia.-In this general form the components of the 
brachial plexus, from the four lower cervical, or some of its filaments, and 
first dorsal roots, are those involved. The chief nerves carrying sensations 
from the skin area of the arms and shoulders are the circumflex, radial, 
internal cutaneous, and musculocutaneous. These enter, for the most 
part, the upper and middle cords of the plexus. In the majority of cases 
the pains of brachial neuralgia are located in the upper arm and about 
the shoulder, i. e., in the area of the circumflex, radial, musculocutaneous, 
and internal cutaneous nerves. 
Bernhardt's statistics show that men are more frequently affected 
than women, but the reverse shows true in the figures of other observers 
(Romberg, Erb). More women come to the Vanderbilt Clinic for 
brachial neuralgia than men, and in most instances it seems that excessive 
sweeping is the attributed cause. In piano players neuralgias in this 
area are frequent. Perhaps these should be relegated to the occupation 
neuroses with the pains of hair dressing, skirt carrying, telegraphy, 
writing, etc. At any rate, arm and shoulder pains are frequent in their 
mild grades at least, and very variable. The usual causative factors 
all come into play here. The neuropathic constitution is put in the 
foreground by Oppenheim; Bernhardt lays considerable stress upon the 
importance of bone injuries with callus formation in the formation of 
many arm neuralgias. Small punctured wounds about the wrist, forearm, 
vol. v-47 738 
DISEASES OF THE NERVOUS SYSTEM 
and arm are responsible for many symptomatic neuralgias. Vertebral 
disease, tumors, aneurisms, syringomyelia, multiple sclerosis, tabes, and 
syphilis are causes. Cervical rib should not be overlooked. 
Symptoms.-Cervicobrachial neuralgias are extremely variable in 
distribution, extent, and severity. The onset is usually sudden, especially 
in those patients in whom an antecedent history of exposure to cold and 
wet is obtainable (motormen, policemen, etc.); at times the beginning 
is preceded by twinges and slight distress. The pains in brachial neu- 
ralgia are less apt to be the sharp shooting variety so dreaded in tic 
douloureux, but sudden accession of sharp pains, varying in their intensity, 
are frequent. As with most neuralgic pains, movement increases them. 
Toward evening the pains are apt to increase and the patient, although 
obtaining relief by lying down, rarely sleeps well. Soreness of the skin, 
slight swelling, and general reduction in tone are the usual accompani- 
ments. With increasing disuse slight atrophy is common, and swelling 
is usual. The tendon reflexes are usually more irritable and active. 
More marked atrophy paresis with vasomotor trophic symptoms and 
altered tendon reflexes indicate a definite neuritic process. Herpetic 
eruptions occur with non-infectious, as well as infectious involvement 
of the sensory ganglia. 
Tender points are very variable. They are most frequently in the 
middle of the back; about the level of the second or third dorsal there is 
usually a sore Trousseau point. Gowers noted that the inferior ulnar 
point in front of the wrist is the commonest sore point. Babinski has 
called particular attention to a radial neuralgia due to a mild or severe 
neuritis of the radial. The pains occupy the posterior portion of the arm, 
and are unusually severe. Neuritic changes are not infrequent. 
Diagnosis.-Particular care is needed in excluding affections of the 
spinal cord, meninges, and vertebrae, as well as angina pectoris. Disease 
of the joints and bones should be excluded at the outset, although it 
may be impossible in some cases of periostitis. In tumors and other 
organic affections of the cord the painful points are usually absent, but 
the earliest and only symptom of spinal cord tumor, intramedullary or 
extramedullary, may be a brachial neuralgia. In tabes the pains are 
apt to be bilateral. 
The occupation neuroses involving the arm and shoulder are many. 
The history of the protracted exercise of certain groups of muscles is 
usually sufficient to identify the proper cause for the neuralgic pains. 
Occupation neuralgias, like neuritic neuralgias, are neuralgias none 
the less, the sole diagnostic question arising as to the cause, and through 
this the proper mode of therapeutic attack and the probable outcome. 
Alcoholic neuritis in its mild grades offers particular embarrassments. 
Lead poisoning is to be borne especially in mind, while diabetes is of 
importance. Brachial psychalgia is a possibility, but the diagnosis must 
be made with extreme caution after exclusion of organic factors. 
Treatment.-Rest is a necessity, and is primarily insured by the use 
of a sling. The diagnosis of the cause being assured, treatment should 
be begun to remove it, either by medical or surgical means. Nerve 
suturing for injury has come to occupy an important part, and is usually NEURALGIA 
739 
attended with good results, even after long periods of loss of function. 
In the early stages active mechanical treatment is to be avoided. Hot 
applications are useful in most acute neuralgias. In later phases massage, 
particularly the Nageli movements, are valuable. The Leduc rapidly 
alternating currents are frequently palliative. Salicylates (especially 
in analgesic combinations), iodides, quinine, arsenic, and large doses of 
strychnine are of value. 
Intercostal Neuralgia.-The twelve dorsal nerves constitute the plexus 
involved, although the upper series, especially of the left side, are most 
frequently concerned. Bernhardt says that the site of election is mostly 
from the fifth to the ninth. Since the dorsal nerves divide into internal 
and external branches the site of the neuralgia may be on the surface or 
within (pleurodynia, etc.). The two upper nerves send branches to the 
internal surface of the arm, and pain is occasionally felt there. The 
abdominal involvements are rarer, and may extend down to the 
genitals. 
The pains are usually less severe than in other regions, although their 
sharp, sticking character distresses the chest movements, especially 
since all motion tends to aggravate them. Tender points are found at 
the site of perforations near the spine. Skin hyperesthesia is extreme 
at times. Herpetic neuritic neuralgias are relatively common in this 
distribution. Mammary neuralgia or mastodynia, which is frequent 
in the later phases of nursing, and in some women at the menstrual 
epoch, is a special form. The pain is usually deep within the gland, 
and may be accompanied by a slightly increased secretion. The whole 
skin may be sensitive, especially the nipple, when the superficial nerves 
are mostly involved. 
Among the causes to be diagnosed may be costal caries, affections of 
the spinal cord and meninges, disorders of the pleura, particularly carcin- 
oma and tuberculosis, tabes, aortic aneurism, dilatation of the stomach, 
carcinoma of the liver, angina pectoris, pericarditis, trauma, etc. 
Treatment.-Local applications are useful, especially the ethyl chloride 
spray. Blisters are efficacious. Bandaging affords marked relief. 
Lumbar Plexus Neuralgias.-These are most conveniently arranged 
as (1) lumbo-abdominal, (2) ilioscrotal or testicular, (3) crural, (4) 
femoral, and (5) obturator, involving in each case certain of the branches 
of this plexus. Mixed and indeterminate forms are not infrequent. 
1. Lumbo-Abdominal.-These occupy the lower half of the trunk, and 
are extremely variable. The chief nerves involved are the iliohypogastric 
and its branches, the inguinal, and genitocrural. Strict localization to 
one trunk is rare, and men are more frequently affected than women. 
The chief causes in addition to those of general moment are local inflam- 
matory conditions or new-growths involving the plexus or some of its 
branches. The pains are usually unilateral, occasionally bilateral, 
involve the region of the back below the ribs, the gluteal region, the 
abdominal and inguinal areas, the scrotum, or the labia. The chief 
Valleix's points are over the lumbar vertebrae, the hip or iliac point, 
hypogastric point, and the scrotal point. Lumbo-abdominal pains are 
usually accompanied by intercostal pains above or thigh pains below. 740 
DISEASES OF THE NERVOUS SYSTEM 
2. Testicular Neuralgia.-Astley Cooper termed this neuralgia the 
"irritable testicle." The pains are usually unilateral, pass into the 
testicle, which may be swollen and tender to the touch. The pain not 
infrequently passes into the leg and back, and the patient may have an 
attack of vomiting. Bernhardt notes that the pain may be so intense 
as to cause the patient to seek castration. The affection is an obstinate 
one, and is not helped, as a rule, by removal of the testicle. Diagnosis 
involves a rigid exclusion of organic disorder of the testicle, although 
many affections (gonorrhoea, tuberculosis, chronic prostatitis, etc.) are 
not infrequently accompanied by persistent neuralgic pains. 
3. Crural Neuralgia.-The crural or femoral nerve is here implicated. 
The pain extends in the upper front and inner side of the thigh, to the 
knee, and further through the saphenous distribution to the ankle and 
inner aspect of foot, extending as far as the big toe. It is almost entirely 
confined to men, and shows considerable variability as to the branch 
involved. It not infrequently accompanies a sciatica. Special etiological 
features are found in fecal impaction, or even chronic constipation, 
disease of the hip or knee bones, enlargement of the inguinal glands, 
aneurism of the iliac artery. Charcot called attention to the frequent 
association of crural neuralgia and diabetes. Spinal arthritis and tabes 
are obscure causes. 
Movements of the thigh usually are painful, and the patient comes to 
bend his body forward in a strained position. The painful points of 
greatest frequency are just below Poupart's ligament, just within the 
inner condyle, over the malleolus, inner side of the instep, and one over 
the great toe. Neuro-atrophic changes usually occur in the quadriceps, 
but the patellar reflex is rarely affected, save when a definite neuritis 
is present. Herpes, reddening, and hyperesthesia are not infrequent. 
In the diagnosis, disease of the inguinal vessels is to be looked for, as 
well as intrapelvic disorders, new-growths, etc. Crural neuralgias have 
a fairly good prognosis. 
4. Femoral Neuralgia.-Here the cutaneous femoris lateralis, arising 
higher up in the pelvis, is involved. The pain is felt in the upper and 
outer aspects of the thigh, extending to the knee. A painful point over 
the anterior spinous process of the ilium is usual. Paresthesia in the 
distribution of this nerve has been extensively studied (Meralgia par- 
esthetica.) The relation of the pressure of corsets has been pointed out, 
and much sitting is frequently associated with this neuralgia. The 
prognosis is favorable. 
5. Obturator Neuralgia.-Lesions of this nerve are fairly constant as 
a result of the pressure of the intestinal loops of a hernia. The pain is 
located in the inner side of the thigh, and is accompanied by a feeling 
of stiffness, creepy, crawly feelings of the skin, and inability to bring the 
thigh toward the middle line of the body. 
Neuralgias of the Pudendal Plexus.-A large number of neuralgias 
of the general plexus are recorded. The median hemorrhoidal branches, 
distributed to the rectum, bladder, and vagina, the inferior branches to 
the anus, ami the pudendal nerve supplying the testicular sac, the labia, 
penis, urethra, and clitoris, are the chief nerves involved. The general OCCUPATION NEUROSES 
741 
terms, spermatic neuralgia, anal neuralgia, perineal neuralgia, rectal 
neuralgia, vesical neuralgia or cystalgia, urethralgia, prostalgia, penis 
neuralgia, irritable uterus, ovarian neuralgia, are utilized to describe 
these different affections. These neuralgias are very rare, but often 
very obstinate. Spermatic neuralgias are among the most frequent, 
and not infrequently accompanied by priapism, perhaps ejaculation. 
Since the advent of bicycle riding neuralgias of this general region 
have been on the increase. The ovarian neuralgias are complex, and 
more often come within the domain of the gynecologist, as structural 
defects are usually the underlying causes. Localized herpetic eruptions 
accompany neuralgias of this plexus. Lesions of the cauda equina are 
to be carefully excluded in neuralgias of this region. 
Neuralgias of Coccygeal Plexus.--Coccygodynia, painful coccyx, is 
a not infrequent disorder in women, especially in multiparae and in the 
badly constipated. Trauma and caries are frequent causes. The 
hysterical coccyx is not infrequent, and referred coccygeal pains are 
common. The pain is so intense at times that defecation is rendered 
impossible; the patient cannot sit, and a grave neurasthenic condition 
supervenes. The medicolegal significance of coccygodynia is real, 
appearing frequently as a local symptom of a general traumatic neurosis. 
Surgeons frequently lay considerable stress on a freely movable coccyx 
in accident litigation. A just estimate of the true bearing of an injury 
to the coccyx can only be arrived at by a careful survey of all of the 
factors of the particular case. 
Local treatment is seldom efficacious save in the truly neuralgic types. 
Resection is rarely a justifiable procedure. Nageli's massage movements 
are particularly efficacious in remedying the mechanical obstructions 
often conducive to persistent constipation. 
OCCUPATION NEUROSES. 
Synonyms.-Professional neuroses; professional cramps; crampes 
fonctionelles et professionelles; spasmes professionelles;. impotence 
professionelles; Beschaf tigungsneurosen; Beschaf tigungskriimpfe. 
Definition.-These are abnormal nervous modifications of voluntary 
muscular coordinations involving certain muscle groups, which are 
subjected at frequent intervals, by reason of a definite occupation, to 
fine, complicated, and coordinated movements. They are known under 
a great variety of names, such as writer's cramp, pianist's cramp, 
violinist's wrist, telegrapher's cramp, milker's cramp, tennis elbow, 
baseball pitcher's glass arm, dancer's cramp, etc. For the most part, 
they consist of tonic contractions, with or without pain, intermingled 
at times with clonic shocks or tremors. The associated movements 
become more and more difficult to carry out, until a motor paresis ter- 
minates the attempt, the muscles all being tied up, as it were, into a 
painful tonic contraction. Muscular atrophy may appear later. A 
great diversity of objective manifestations is to be observed. In general, 
two features are characteristic. The spasms occur in muscle groups 
which carry out some habitual or professional synergistic act, and the 742 
DISEASES OF THE NERVOUS SYSTEM 
cramp occurs only when the act itself, i. e., the bringing together of the 
entire willed mechanism, is performed. When the individual muscles 
are acting independently in some other less frequently performed move- 
ment no such cramp occurs. The implication of mental impulses is 
highly probable. 
The recent monographs of most value are those by Hemak,1 1894, 
Bernhardt,2 1898, and the article on Crampes Professionelles by JMeige.3 
Chapters by Gowers, Oppenheim, and Gasset may also be cited. Thomas 
has written an important monograph.4 
Etiology.-The chief factor is the frequent and continuous use of 
certain muscular movements in an occupation beyond the natural capacity 
of the individual to stand the strain. Added to this are usually certain 
minor or major features which determine why that particular individual 
should develop the neurosis. In many instances an antecedent pressure 
neuritis reduces the capacity of certain groups of muscles involved in a 
coordination, and this throws increased stress on others. In others, 
faulty methods of adjustment, as in writing in a cramped position, or 
with improper light, may prove a determining factor for one individual. 
Mention may also be made of definite physical ills which contribute to 
reduce nervous tone. These are not unimportant; anemia, lead poisoning, 
alcoholism, excessive use of tobacco, venery, diabetes, tuberculosis, the 
beginnings of serious nervous troubles, paresis, tabes, etc., may be distal 
causes. Accident, with strain to muscle, tendon, or joint, may be the 
straw which breaks down the adjustment. Mental causes may show in 
others; anxiety, worry, financial stress may cause persistent insomnia, 
which may contribute to an occupation neurosis. The influence of 
heredity is difficult to trace, for no family is free from some disorder or 
disease, and the evidence is too flimsy to build upon. 
In well-developed cases, more particularly since there is such a mental 
element, it is not unnatural that psychical causation factors should be 
postulated. Duchenne advocated the view that mental factors were 
important. Meige follows more closely the original psychic germ sown 
by Duchenne, and places the mental factors in the foreground. He 
believes that the occupation neuroses are essentially allied to the tics, 
resembling them not only in their objective features, but also in their 
mode of appearance and evolution in the disposed. In the vast majority 
of these cases, he holds that the motor trouble appears very distinctly 
as a consequence of the mental trouble. 
The multiplicity of forms described-contracted, spasmodic, ataxic, 
tremulous, paralytic, etc.-all arise on this common mental basis; they 
may be found isolated in one patient, or may exist in various combi- 
nations. All distinctions between professional and functional spasms, 
cramps, etc., Meige holds, are arbitrary. Psycho-analysis has shown 
the mental factors even more clearly. 
1 Eulenberg's Real-Encyclopadie der gesammten Heilkunde, new edition, 1909. 
2 Die Erkrankungen der peripherischen Nerven, Tiel, ii. 
3 Bouchardt's and. Brissaud's Traits de Medecine, second edition, 1905. 
4 Modern Treatment of Nervous and Mental Diseases, White and Jolliffe, vol. i. OCCUPATION NEUROSES 
743 
Occurrence.-Those coordinated actions of most frequent performance 
will naturally present themselves the more often as disordered. The 
practitioner in the large city will practically see more writer's cramp 
than other types. The practitioner of rural districts will see milker's 
palsies. The medical man in miners' camps will be called more often 
to treat the pick cramp and miner's nystagmus. In every large city there 
are workers in certain occupations in which fine manual work is carried 
on for long hours, under adverse circumstances. Many of these workers 
develop very severe grades of occupation neuroses, but their study has 
not been at all systematic. 
Many writers say that the disorder is more common in men. This 
statement is made largely on the evidence afforded by the sufferers from 
writer's cramp, but since women have taken up telegraphy, stenography, 
and typewriting, and are engaged so w'idely in the manufacture of small 
articles for the trimming of clothing, hats, etc., it would seem that they 
now show more forms of occupation cramps than men do. Gowers' 
statement regarding stenographers not developing writer's cramp is 
erroneous. A limited experience has revealed at least a dozen stenog- 
raphers, male and female, who have had to give up their work because 
of occupation cramps. 
The age of onset is usually after twenty years. Making a combination 
of the reported cases (194) of Berger, Poore, Gowers, Remak, and Bern- 
hardt, the distribution is as follows: Ten to twenty years, 4; twenty 
to thirty years, 67; thirty to forty years, 63; forty to fifty years, 45; 
fifty to sixty years, 10; and sixty to seventy years, 5. To which may be 
added 46 personal cases: Ten to twenty years, 4; twenty to thirty years, 
12; thirty to forty years, 26; and forty to fifty years, 4. 
Occupation.-Practically every known occupation has its neurosis. 
To describe them in detail is impossible. Even new sports introduce new 
occupation neuroses, such as the golfer's "wrist," the tennis "elbow," 
basball "glass arm," etc. Blacksmiths, coopers, carpenters, masons, 
ironers, tailors, and washwomen show the peripheral neuralgic types, 
usually implying pressure. Among cigar rollers, those who crochet, 
knit, sew, or cut, rinsers, cabinetmakers, planers, machinists, locksmiths, 
weavers, chemists (holding beakers and test-tubes), players of musical 
instruments, occupation neuralgias, which may never advance to occu- 
pation palsies, are known. 
Symptoms.-Irrespective of the occupation, the development of a 
professional neurosis seems to proceed along fairly definite lines. Indi- 
vidual variations are found, however, and certain persons, by reason of 
unknown factors, do not travel as far as others in their journey to the 
completed stages. It is for this reason that the etiological features upon 
which Meige lays so much stress may enter into the mastery of the 
situation. In the majority mild or severe occupation neuralgias are 
first observed; many patients never have anything else, and a few never 
have these. Occupation palsies, or impotence, as the French call it, 
present another phase; these may represent the results of a general 
or local asthenia and bear no relation to the fully developed neurosis 
such as is seen in a typical writer's cramp. 744 
DISEASES OF THE NERVOUS SYSTEM 
In those forms in which a general asthenia is at the basis of the motor 
loss, anemia, cachexia, alcoholism, diabetes, tuberculosis, etc., are often 
present. Thus, in many occupations, which call for localized, restricted, 
muscular adaptations involving considerable muscular power, as in cigar 
rollers, engravers, and glassblowers, localized atrophies develop, some- 
times as a result of pressure on an exposed nerve, sometimes from other 
causes. These pressure palsies and pressure atrophies should receive 
very careful scrutiny. Some of the more characteristic are seen in waiters, 
in butchers who carry heavy pieces of meat over the shoulder, in others 
who carry packs, in steel workers, in steersmen who use the arm to push 
the rudder, in polemen (as seen in the European canals) who push the 
boat by their long poles against an elbow. Potato peelers, vegetable 
preparers, drummers, polishers, planers, etc., all show such muscle 
pareses, accompanied usually by neuralgic manifestations. 
For many of those patients in whom atrophies develop, a local cause 
may be found. Some nerve stem is caught between the structures of 
the hand and a surface pressed upon; in rare cases perhaps a nerve may 
suffer from pressure of tendons when such tendons are constantly on the 
stretch. These local atrophies, when seen apart from the more definite 
development of muscular incoordinations in a complicated motor act, 
play a minor role in the true occupation neuroses, and are mentioned 
solely because such atrophies are sometimes contributory factors in the 
production of the sense of.fatigue, and are the cause of paretic phenomena 
in others. In the sense that these show pure peripheral origins they 
differ from the typical cases of occupation neuroses (writer's cramp), 
although it seems that transitional stages are observed between the 
peripheral cramp-like states and the centrally induced phenomena. 
Remak has called attention to the cramps of sewing girls, which, after 
preliminary neuralgic-like pains, come on at night. These patients 
relate that they are awakened out of their sleep by the severity of the 
painful cramps. He has also laid stress upon certain cramps of milkers 
which originate in an apparently different manner from the most classical 
milker's cramp. Perhaps they are of the same nature, seen either at a 
different stage of development or in an individual of different neural 
tendencies. Cases of atrophy of the thenar, hypothenar, and interossei 
muscles are known in milkers, and it is certain that peripheral influences 
cannot be rejected in the interpretation of certain milker's cramps. 
By reason of these and other factors it is not possible always to draw 
sharp lines between the stages in the development of the professional 
cramp. Each case seems to travel a slightly different path, and one sees 
the patients all tending in the same general direction, but at different 
stages and with varying syndromes. The accidental occurrence of 
pressure neuritides constitutes one of the commonest accessory factors. 
Inasmuch as writer's cramp is the most frequent of these, it may 
serve as an example. In the complicated muscular coordination of 
writing every individual follows his own methods, and no two are ever 
alike, but, although the actual writing may be different in a million 
individuals, the general mechanism is largely the same. In general 
three trends are manifest in writing. There are those who make great OCC UP A TION NE UROSES 
745 
use of the smaller movements of the fingers, those who write largely from 
the wrist, and those whose movements are still wider, employing the 
whole arm, freehand, as it were. The general grasp of the pen is about 
the same with all. Those who write a great deal rapidly, such as news- 
paper men, usually acquire a very great flexibility in their writing, and 
use all mechanisms in turn. Thus, they can rest certain groups of 
muscles, and still carry on the writing movements. 
Most observers are in accord in ascribing a greater tendency to the 
development of writer's cramp in those who make greater use of the finer 
finger movements. Those who write from the wrist and forearm are 
less liable, while the large freehand writer is usually immune. Gowers 
is especially emphatic on the harmfulness of resting the hand on the 
fingers. While these factors of finger position do play a part, they are 
not the most important, and the large arm writer is not necessarily 
immune. It is by reason of other factors that the training to large arm 
movements in a "cramper" is often futile. 
Writer's cramp is no recent disease; above all, it is not a creation of the 
steel pen, as has been assumed by some good observers, nor of any special 
type of penmanship. That its clearer description should have coincided 
more or less with the introduction of the steel pen is accidental, for in the 
early days the disease was described as occurring among those who still 
adhered to the use of the old-fashioned quill, and it is known among 
the Chinese, who still use a brush. The mere fact of the widespread 
occurrence of so many different kinds of occupation neuroses is enough 
to negative any such type of inference. Nor can full adherence be given 
to the view that the disorder originates entirely from a faulty manner of 
holding a pen or of the use of certain muscles in a definite way in writing. 
The trouble lies farther back, although it is readily conceded that certain 
faulty methods of writing are more arduous than others, involve more 
muscular action, and are likely to cause greater fatigue. 
Clinicians have described four chief types-the spasmodic, the tremu- 
lous, the neuralgic, and the paretic forms-but these signify little, since 
nearly all cases show more or less of all of these symptoms. Writer's 
cramp, in its strictest sense, shows the spastic type in its purest 
forms. 
In a well-developed case the patient, after perhaps years of gradually 
increasing difficulty, after writing for some minutes, or perhaps hours, 
feels a sense of stiffness in his writing. He has neuralgic pains in certain 
muscles, at the shoulder-joint, the wrist, or in the thumb. A certain 
rigidity seems to pervade the strokes, and he notes a marked increase in 
tension in the thumb which grasps the pen. The index finger becomes 
unruly, the pen is liable to slip, and firmer grasping is resorted to to hold 
the pen in place. After a short time of rest he may be able to resume 
his writing for some time before this grade of difficulty again becomes 
manifest. With increasing severity the troubles become more marked; 
the firmer grasping of the pen becomes a spasm, at times one of flexion, 
but more often an extension (Canstatt's two types), the index finger 
straightens, the pen becomes more upright, the thumb is strongly con- 
tracted to hold the pen against the hand, the spasm extends, the thumb 746 
DISEASES OF THE NERVOUS SYSTEM 
straightens out, and the pen can no longer be held. The writing becomes 
more and more irregular, scratchy, and scrawly until it becomes illegible. 
Other fingers may be involved; in some cases all the fingers are extended, 
the wrist flexed, the arm brought down to the side of the body, while 
severe pains radiate throughout the entire arm. Brusque pronation or 
supination of the arm may then follow. The whole writing apparatus 
becomes fixed in a tonic (sometimes clonic) battle between agonist and 
antagonist, all acting out of concert, and mutually disrupting the har- 
monious relations of a well-planned coordination. Many patients write 
for years, with but the sense of stiffness and some pain. Others, on the 
contrary, develop acute spasms, and no sooner commence to write than 
the cramp develops. In most of the fully developed cases considerable 
mental excitement is present. Vascular disturbances may be distinct. 
The character of the spasms is usually tonic, and they develop slowly. 
Some patients have jerky movements somewhat resembling clonic con- 
tractions, but these seem less common. Tremor is an almost invariable 
accompaniment, and there are writers for whom only tremors exist. 
It is usually conceded that of the predominant types, the spastic form 
is most frequent; the feeling of great weakness and soreness in the muscles, 
which is antecedent to all forms of writer's cramp, is not considered here. 
The spastic form alone, or in combination with tremors makes up about 
40 per cent, of the cases. Berger,1 in his analysis of 04 cases, found that 
in 24 there was a pure spastic type; 10 showed the paralytic type, 8 had 
tremors, while the remainder, 22, had combinations; over 50 per cent, 
had cramps. In Remak's 42 cases there were 9 cases of true writer's 
tremor; 32 showed more or less spastic symptoms. He observed only one 
pure paralytic case. Gowers noted the rarity of paralytic forms. 
It is highly significant that the moment the pen is laid aside the cramp 
ceases, the patient can move his hand or arm in any desired direction, 
and apart from, at times, a mild sense of tire in the affected muscles, all 
signs of previous difficulty vanish. The patient can carry on coordinated 
movements involving the very muscles which were in such a painful 
state of contraction while performing the single act of writing. Benedikt 
is stated to be the first observer to record the tendency that exists for 
the disorder to spread to other frequently repeated intricate coordinations. 
In the tremulous type, which in its pure state is comparatively rare, the 
patient's hand begins to shake with the act of writing, the writing is 
tremulous and shaky, like that of an old person, the whole arm may be 
involved in the tremor, and patients are known who, without pain or 
other disturbance, have written such a tremulous hand for thirty or forty 
years. They have never lost the power of writing, but must always 
write with a distinct tremor. 
In the paralytic type, which is rare in the pure form, the patient loses 
the power to grasp the pen. There is a stage of rapidly advancing 
fatigue and weakness, up to a point when paresis without spasm or tremor, 
and often without severe pain, save that of tire, is reached. The fingers 
become stiff and inactive, as though nailed to the table. After a few 
1 Beschdftigungsneurosen, Eulenberg's Encyclopadie, 1885, vol. ii. OCCUPATION NEUROSES 
747 
moments' rest the patient can resume his writing, to be again interrupted 
by the rapid advance of paresis. The localization of the paretic muscle 
groups may vary. Berger reports a case of isolated shoulder and upper 
arm type. Simple abducens pollicis paralysis is known. Duchenne's 
case of infraspinatus palsy is classical; here the patient could write if 
the paper was pulled along by the left hand, but the carriage of the arm 
across the page was impossible. 
Sensory Disturbances.-Such are usually present if only as a distressed 
sense of tire, of pressure, of tension. Enough has been said of the neu- 
ralgic pains and the pains of tonic contractions. Anesthesia and pares- 
thesia are present, although infrequent; and hyperesthesia with painful 
nerve trunks, may be present, especially in the neuralgic types. Such 
painful nerve trunks with the occurrence of Lasegue's symptom in the 
lower extremities may clear up the diagnosis in mild alcoholic cases. 
Vascular disturbances, either local, as angiospastic phenomena with 
cold hands, or as paralytic phenomena with localized sweating, are rare. 
General anxiety with vascular disturbances is not as infrequent. Electri- 
cal changes are not of frequent occasion; when present they indicate 
the neuritic nature of an occupation palsy, and thus aid in the diagnosis. 
Course and Prognosis.-The outlook is bad if one has in mind only 
those cases which are construed to be occupation palsies in the narrow 
sense. When one takes into consideration, however, all of the forms, 
especially those of widely varying etiology, the prognosis is good. This 
is especially true for the alcoholic and pressure types, while in the purely 
mental types the reverse is true. Most patients with fairly well-developed 
professional neuroses run an up-and-down course with fairly well-marked 
chronicity. With improvement in general strength and rest, the pro- 
fessional movements are better carried out, and vice versa. Some 
patients rest for a year or more, and develop their neurosis within a week 
after resuming their occupation. In such cases the mental factors are 
dominant. These patients need psycho-analysis, not rest. In some patients 
cure seems impossible, either due to defect on the part of the patient to 
grasp the rationale of a course of therapeutic effort, or a lack of proper 
analysis on the part of the attending physician. In severe cases of 
writer's cramp the course is especially obstinate, but not always hopeless. 
Treatment.-Personal factors enter so largely into this that the indi- 
vidual must always be borne in mind. Occupation neuroses develop on 
the one hand on a basis of overwork of certain muscular coordinations, 
without any other complicating factors; at the other extreme, patients 
will be seen in whom the psychical factors are predominent or have 
become so, and the fatigue element is practically nil; occupying a partial 
middle ground are those in whom constant, minute maladjustments are 
to be borne in mind. Few individuals will present examples of the 
extremes; the vast majority will present combinations in varying propor- 
tions of overwork and mental attitudes with certain intermediary asthenia. 
There is little doubt that most patients-save those in whom the 
psychical features are markedly developed-will receive benefit from the 
general methods of increasing physical vigor. A sea trip, change, etc., 
are here indicated. Alcohol and tobacco are to be reduced to a minimum. 748 
DISEASES OF THE NERVOUS SYSTEM 
Abstinence from writing, in the writer's cramp form, is the first requi- 
site. Many clinicians, Gowers among them, recommend the advisability 
of teaching their patients to write with the left hand, thus affording the 
right hand an opportunity to rest. This may effect a comparative 
cure, but not infrequently such patients develop double writer's cramp. 
Such an evolution is presumptive evidence of a large psychic element, 
and for such the methods of Meige are particularly advisable. Methods 
of reeducation of the writing are also desirable in other cases; changes 
in the form of pen, using a large penholder, stub pens, changing the 
slant of the writing, learning to rest one's entire arm on the writing table, 
and to utilize the larger muscles in the writing act-these, with general 
hygienic tonic procedures, are at times sufficient to afford sufficient 
variation for an affected individual to just steer clear of an actual break- 
down. Great care must be exercised in this direction. With definite 
mental features in the case, such schemes are a waste of time, and may 
be bad psychotherapy. 
In the large class in which accessory elements play a part, i. e., congeni- 
tally weak muscles, pressure and toxic neuritides, prophylactic measures 
combined with direct methods of nerve and muscle treatment are needed. 
Strychnine, calcium salts in the form of hypophosphites, iron, atropine, 
galvanization, mechanotherapy, massage, etc., are useful. Bier's hyper- 
emic procedures are helpful. 
Special electrical methods are futile. Many patients are apparently 
benefited by the Leduc oscillatory currents. The role played by electrical 
stimulation is difficult to estimate. In those patients for whom such 
electrical stimulation acts as a general tonic, its action is evident; in 
others the suggestive feature should not be overlooked; in others it is 
useless or worse. All the writers who find electricity beneficial report 
that it must be used for months, which is largely indicative that it is 
solely a general measure and really has little direct value. Psycho- 
analysis is of great service for the strictly psychogenic cases, and a great 
many are such. 
TETANY.1 
Definition.-It cannot be said at the present time just what tetany is, 
or rather, perhaps, it might better be expressed, that so many different 
tetanies are known that it is evident that, like epilepsy, it is not a single 
affection but many; a symptom grouping, very variable as to complete- 
ness, which is best regarded as a more or less localized manifestation of a 
convulsive nature, due to a large number of exciting causes, possibly 
with some single and unique underlying factor. Whether such a position 
can be maintained for the so-called idiopathic epidemic form remains to 
be seen. If the present view be maintained, however, the term tetany 
must be rejected as a disease conception in the vast majority of the 
conditions which now go by that name, or perhaps preferably a binominal 
nomenclature might be adopted which would specify the type under 
consideration. Thus, it might be advisable to advocate the use of the 
1 Falta, Die Erkrankungen der Blut Drusen, 1913; Phleps, Die Tetanic, Lewan- 
doswky, Handbuch, 1913; Biedl, The Internal Secretions, second edition, 1913. TETANY 
749 
terms tetany idiopathica, tetany gastro-enterica, tetany thyroidea, 
tetany toxica, including bacterial and chemical toxins, tetany gravida, 
etc., until it may be shown just what underlying factor is responsible. 
It is highly probable that tetany must be regarded as an example of 
hyperfunctioning of certain parts of the motor mechanism in which 
altered muscular excitability is primary, while special neurological 
features play a secondary role, and yet these latter give to the muscular 
manifestations their special trend. Here particular attention is paid to 
the etiological factor, since the clinical manifestations alone are not always 
sufficient to distinguish the form of toxin present. 
Frankl-Hoch wart,1 speaking more particularly of the adult forms, is 
more didactic in his presentation. Tetany, he considers, has as its main 
symptoms tonic, intermittent, bilateral, often painful cramps, which, 
without, for the most part, any loss of consciousness, involve the muscles 
of the upper extremities, particularly the hand, which is held in the 
obstetrical position. The muscles of the lower extremities may also 
be involved, those of the larynx, of the face, and of the jaw, seldom those 
of the chest, abdomen, neck, diaphragm, or tongue. In rare cases the 
eyeball muscles are implicated, as is also the bladder. In the sensory 
sphere paresthesia and pains are present, while hyperesthesia occurs 
now and then. Pressure upon the brachial plexus may give rise to an 
attack (Trousseau); hyperexcitability to electrical currents is present 
(Erb); mechanical hyperexcitability of the muscles and motor nerves is 
observed (Chvostek), while the sensory hyperactivity to mechanical and 
electrical stimuli is also present (Hoffmann). The psyche is usually 
uninvolved, mental disturbance being found only now and then. In 
the chronic and repeating forms secretory and trophic disturbances occur, 
such as increased perspiration, reddening of the skin, swellings of the 
joints, mild oedema, falling out of the hair and nails, discoloration of 
the skin, urticaria, and herpes. Dyspnoea may supervene; polyuria and 
glycosuria are rare accompanying symptoms. Abortive and incomplete 
forms this author designates as tetanoid. 
For didactic purposes Frankl-Hoch wart divides tetany into simple 
acute forms and chronic recurring forms. A further division of form 
occurring in children and in adults is made. Tetany of the adult he 
groups into eight classes: (1) Tetany idiopathica-tetany of otherwise 
healthy individuals-workman's tetany. This is the form which seems 
to occur epidemically as an acute, or acutely, recurring affection in certain 
cities, notably Vienna, Heidelberg, etc., principally in the early spring 
months, and among certain handworkers-tailors, shoemakers, etc. 
(2) The tetany of gastric and intestinal affections. (3) The' tetanies 
of acute infectious diseases, typhoid fever, cholera, measles, scarlet 
fever, etc. (4) The tetanies of acute poisoning, chloroform, morphine, 
ergot, phosphorus, lead, renal and genital substances. (5) The tetanies 
of maternity (pregnancy, parturition, and nursing). (6) The tetanies 
of parathyroid involvement. (7) The tetanies of other nervous dis- 
eases, exophthalmic goitre, brain tumors, cysticerci, syringomyelia, etc. 
1 Die Tetanie der Erwachsenen, 1907. 750 
DISEASES OF THE NERVOUS SYSTEM 
Whether all of these have a common etiological factor is a problem 
that has not yet been definitely solved. It would seem that from the 
work of MacCallum a deduction might be made allying the various 
forms-namely, that a disturbance of calcium metabolism of the body 
is responsible for the hyperactivation of the neuromuscular responses 
which results in the tetany spasms. 
Incidence.-Tetany in its different manifestations is undoubtedly 
rarely seen and is even less frequently reported. In undeveloped phases 
the tetanoid reaction is comparatively frequent in children. The instances 
of gastric tetany are probably the most frequent, while the pure epidemic 
form has not been encountered in the United States. In Griffith's 
study only 77 cases were found recorded, while Howard's later collection 
brings the American cases to 154 in 1907. Thus, so far as clinicians in 
English-speaking countries are concerned, tetany may be considered as 
being infrequently seen, but even in countries in which it is thought to 
be epidemic it is rarely observed. It is undoubtedly often overlooked 
and at times confused with hysteria. 
Etiology.-At least two factors are concerned in the general hyper- 
excitability of the neuromuscular apparatus and the neural trend that 
determines the manifestations of this hyperexcitability in certain groups 
of muscles and for certain combinations. So far as the first factor is 
concerned, it would seem that a fairly clear concept has been gained, 
particularly through recent work, while for the second no reasonable 
explanation is forthcoming. 
As regards the epidemic tetanies, Schulz and Hoffmann advocated 
the thesis that they were due to disturbances in the thyroid gland, this 
was in the time when the parathyroids were unknown. The fact of the 
close anatomical relationship of the two glands in man and many carnivora 
served to hide the deeper truth of the separation of their physiological 
functions first brought out by Gley in herbivorous animals, in which 
the glands are anatomically separable and thus capable of exact experi- 
mentation. It is unnecessary to go through all the steps which have 
led to the knowledge that the parathyroids play an important role in 
the functions of the body,, and that the peculiar muscular hyperexcitability 
seen more particularly in tetany is in some manner related to these glands 
especially in regard to their insufficient action. It is possible that other 
anomalies of neuromuscular activity are correlated with these glands. 
Lundborg has advocated wide hypothetical possibilities; even the peculiar 
motor manifestations of a group of mental disorders, catatonias and their 
allies, may have some light thrown on them through this avenue. More 
recent workers have not rested on the apparently certain foundation 
that tetany is essentially a manifestation of perverted parathyroid 
activity, but have sought to bring the anomalous forms into conformity 
with this conception, and, further, to obtain a more fundamental insight 
into the essential features of the disturbed neuromuscular reaction. 
Whether the work of MacCallum and Voegtlin1 has definitely solved 
this problem is to be determined, but it would appear that the essential 
1 See Jour, of Exper. Med., 1909, vol. xi, for summaries along the lines of chemical 
research. 751 
TETANY 
factor has been found in the relation of the parathyroid to the calcium 
metabolism of the body. The hyperexcitability of the neuromuscular 
apparatus is primarily due to a lack of calcium in the blood, and this is 
due to a relative or absolute insufficiency of the parathyroid glands. 
Just how the. calcium exchange of the body is controlled by the para- 
thyroids, and what role in such control is played by other glands, is not 
yet known. The conclusions of MacCallum and Voegtlin are so impor- 
tant that they are worthy quoting freely: 
" Tetany occurs spontaneously in many forms, and may also be pro- 
duced by destruction of the parathyroid glands. These glands are 
independent organs with a definite specific function: whether this function 
is intimately related to that of other organs of internal secretion is not 
proven. Failure to produce tetany experimentally is probably due to 
the fact that some parathyroid tissue remains after an apparently com- 
plete extirpation. A small amount of parathyroid tissue is sufficient 
to prevent the development of tetany. 
"In tetany there is apparently some disturbance of the composition 
of the circulating fluids ordinarily prevented by the secretion of the 
parathyroids, which disarranges the balance of the mineral constituents 
of the tissues. Possibly this consists in the appearance of an injurious 
substance of an acid nature, for such tetanies may be relieved by extensive 
bleeding, with replacing of the blood by salt solution. No actual poison- 
ous material has, however, been demonstrated by the transference of the 
blood of a tetanic animal to the veins of a normal one. 
"Numerous researches have shown the important relation of the 
calcium salts to the excitability of the nervous tissues. Their withdrawal 
leaves the nerve cells in a state of hyperexcitability which can be made 
to disappear by supplying them with a solution of a calcium salt. 
"Tetany may be regarded as an expression of the hyperexcitability 
of the nerve cells from such cause. The injection of a solution of a salt 
of calcium into the circulation of an animal in tetany promptly checks 
all the symptoms and restores the animal to an apparently normal 
condition. 
"Studies on the metabolism in parathyroidectomized animals show: 
(a) A marked reduction in the calcium content of the tissues of the 
blood and brain during tetany; (6) an increased output of calcium in 
the urine and feces on the development of tetany; (c) an increased output 
of nitrogen in the urine; (d) an increased output of ammonia in the urine, 
with an increased ammonia ratio in the urine; (e) an increased amount 
of ammonia in the blood. Much of this affords evidence of the existence 
of some type of acid intoxication. Its effects are, however, not neutralized 
by the introduction of alkaline sodium salts, and may perhaps be regarded 
as especially important in producing a drainage of calcium salts from the 
tissues which can be remedied by the re-introduction of calcium salts. 
"In general, the role of the calcium salts in connection with tetany 
may be conceived as follows: These salts have a moderating influence 
on the nerve cells. The parathyroid secretion in some way controls the 
calcium exchange of the body. It may be supposed that in the absence 
of the parathyroid secretion, substances arise which can combine with 752 
DISEASES OF THE NERVOUS SYSTEM 
calcium, abstract it from the tissues, and cause its secretion, and that 
the parathyroid secretion prevents the appearance of such bodies. The 
mechanism of the parathyroid action is not determined, but the result, 
Fig. 41 
Tetanic spasm, showing flexion of wrist 
Fig. 42 
Tetanic spasm, showing plantar flexion of foot and toes. 
the impoverishment of the tissues with respect to calcium, and the 
consequent development of hyperexcitability of the nerve cells, and 
tetany is proven. Only the restoration of calcium to the tissues can TETANY 
753 
prevent this. This explanation is readily applicable to spontaneous 
forms of tetany in which there is a drain of calcium for physiological 
purposes, or in which some other condition causes a drain of calcium. 
In such cases the parathyroids may be relatively insufficient." 
It has been shown that salts of barium and of strontium are capable 
of controlling the spasms of experimental tetany. While this does 
not constitute a real difficulty in the interpretation of the results just 
cited, it remains to be shown just what pharmacological relationships 
these salts have on nerve cells. In view of their close relation to calcium, 
it may be that they temporarily take on the function of calcium. 
The second series of factors still remains to be solved. Why is it, if 
the facts just quoted bear an essential relation to tetany the disease 
reaction seems to be strictly localized? Is this simply bad observation 
on the part of the clinicians, or are there local conditions of diet, water 
supply, etc., that bring about changes in the intake of calcium? Such 
conditions are known to prevail for certain infants fed on cow's milk.1 
Why do certain workers, tailors, shoemakers, etc., suffer and not 
others? Is Frankl-Hoch wart's suggestion regarding the peculiar position 
adopted by these workers of value in relation to the blood supply of the 
organ? What have gastric dilatation, gastro-intestinal disorders, etc., 
to do with the calcium intake ? Are there perversions of gastric chemism 
that favor calcium exclusion? This must be determined in order to 
explain these cases, and why should certain gravid women develop a 
parathyroid insufficiency? The observations of Lange, Jeandelize, and 
others on thyroid activity at this period are only suggestive. Still further, 
why should the hyperexcitability not be more generalized, and bring 
about general epileptiform conditions instead of very localized spasms, 
and further only in certain groups of muscles. These must be more 
thoroughly explained before the general hypothesis can meet with unquali- 
fied recognition, for the symptoms seem to call for a specific rather than a 
general type of poisoning. 
Pathology.-Concerning the histological changes the present view 
excludes a specific pathology. The insufficiency of the parathyroids, 
be it relative or complete, may be brought about by a great variety of 
lesions. Such have been described by various observers, and each, in 
turn, has been considered of specific significance. These in reality offer 
evidence in favor of the parathyroid insufficiency hypothesis, but go no 
further. In the minor grades of tetany in children, particularly in 
so-called spasmophiles which Frankl-Hochwart regards as tetany, the 
findings of Yanase in Escherich's clinic are illuminating. Here hemor- 
rhages in the parathyroids seemed fairly constant findings, and offer 
an explanation of the galvanic hyperexcitability. At the other extreme 
one finds the absolute insufficiency tetanies in experimental parathy- 
reopriva and in the rarer cases, such as Pool's in man. Here the para- 
thyroid insufficiency is established, and the calcium treatment restorative. 
In acute epidemic forms thyroid (and probable parathyroid) involvements 
are known. Tumors, tuberculosis, and a host of other changes in the 
1 See Escherich's Monograph, Tetanic der Kinder, 1909, for summaries. 754 
DISEASES OF THE NERVOUS SYSTEM 
thyroids have been described. It will probably be found that in most 
of these the parathyroids are likewise implicated. Thus, in exophthalmic 
goitre a combination of thyroid and parathyroid symptoms is often 
present. In many tetanies pure thyroid symptoms appear. 
The relations of the ganglion cells in the spinal cord which stand in 
direct connection with the sympathetic system are in need of analysis. 
Only a few of these groups have been localized, for instance, Jacobson's 
localization for the Klumpke syndrome. 
Symptoms.-Considerable variation is to be found, but in general 
four types of symptoms are observable in the fully developed attack. 
These are the muscular spasms, which may go on to an exhaustion 
paralysis, or paresis; the Trousseau phenomenon; increased electrical 
excitability, or the Erb's symptom; and mechanical hyperexcitability 
of the muscles-Chvostek's sign. In some patients one or more of these 
may be missing. Abortive forms, so-called, may present even fewer 
signs. On the other hand, a richer combination of symptoms, apparently 
closely related to the general disorder, may be encountered. Sensory 
disturbances, anomalies of circulation with oedema, of respiration with 
cyanosis, and of temperature are sometimes found. True psychoses, 
perhaps indistinguishable from the hysterical confusions, are found. 
Trophic disorders of the skin, hair, and nails occur. In some rare instances 
widely confused phenomena resembling epileptic seizures occur. 
Muscle Cramps.-The most persistent feature consists in a characteristic 
form of muscular spasm. It occurs chiefly in the extremities, mostly 
involving the flexors. The spasm is tonic, generally bilateral, and is 
usually induced by some irritation, mechanical or electrical. Over- 
exertion, exhaustion, changes in temperature, acute diarrhoea, or emo- 
tional excitement may precipitate an attack. Consciousness is involved 
only in certain forms (parathyroid tetany); pain may or may not be 
present. The small muscles of the hand are usually first implicated, 
perhaps after tingling-like prodromata. There is marked adduction 
in the interossei and the thumb. The hand usually takes a very signifi- 
cant position-that of the "obstetrical hand." It is also described as a 
"penholding" position. In many mild attacks only the thumb may 
be involved, and in others the hand alone. Sometimes the hands are 
closed, making a fist. Flexion at the wrist may follow, the arms then 
being folded across the chest, or they may be held up in the air or down 
at the side. Such wider movements occur in the more severe attacks 
only. A somewhat similar series of flexor cramp-like movements may 
occur alone or in combination in the lower extremities. Talipes equino- 
varus, inversion of the foot, and plantar flexion may be present. The 
legs may be flexed on the thigh, and the thigh on the pelvis in the most 
severe instances. The contractions in the lower extremities are rarely 
as severe as in the upper, and frequently are lacking altogether. Esch- 
erich considers the classical carpopedal spasms to be rare in children. 
In Frankl-Hochwart's series of 122 cases, only 70 showed cramps in 
the lower extremities. Occasionally a patient has cramps in the upper 
and paresthesia in the lower extremities. Frankl-Hoch wart makes the 
assertion that there are no cases on record of tetany confined to the lower TETANY 
755 
extremities. In Howard's series,1 however, and these are from the 
United States, all four extremities were involved in 61 out of 77 cases; 
Fig. 43 
Method of producing tetanic spasm of hand by stretching the brachial plexus by forcible abduction 
of the arm. Note "obstetrical" hand. 
Fig. 44 
Method of producing a tetanic spasm in the feet by stretching the sciatic nerve by forcible flexion of 
the trunk on the thighs. 
1 Am. Jour. Med. Sc., 1906, cxxxi, 301. 756 
DISEASES OF THE NERVOUS SYSTEM 
in 14 the arms alone were involved, while in 2 the spasms were confined 
to the legs, in which latter respect Frankl-Hochwart's claim is negatived. 
Other spasms may occur. Thus, risus sardonicus, 11 in 122 in Frankl- 
Hochwart's series, and trismus, in 10 of Howard's, may both be found, 
although, as a rule, these rarely occur spontaneously, but are invoked 
by tapping over the facial nerve region in bringing out the Chvostek 
or Hoffman phenomena. The back muscles were involved in 24 of 
Howard's series; at times to such an extent as to cause opisthotonos. 
Other muscular spasmodic contractions are rare, although it may be 
said that any muscular contraction may be expected. It is highly 
probable that sharper delimination of the tetanoid from the hysterical 
and epileptoid spasms is needed, and that closer observations will negative 
the broader statements now usually accepted. Some of these rare 
contractions occur in the bladder, the rectum, the diaphragm (which 
has caused asthmatic attacks), and the muscles of the tongue. Laryngo- 
spasm is one of the commonest forms, especially in children, where it 
has been held as specific for the rachitic. Whether it is to be regarded 
as a specialized symptom in tetany, or as an individual expression of 
other conditions, is not at present definitely determined. Spasms of 
the eye muscles, sufficient to cause diplopia, occur. In 7 of Frankl- 
Hochwart's series these eye muscle cramps were present. They occur 
spontaneously. Generalized convulsions are reported in 13 of Howard's 
cases; 9 of these, however, were in children. 
Considerable emphasis may be laid on the bilateral character of these 
muscular movements. It is extremely rare in the true tetany reaction 
to find one side alone involved; Frankl-Hochwart reports two cases only. 
An incidental feature of some of the hand cramps is the occurrence of 
an intervention cramp in the same sense as it occurs in Thomsen's disease. 
The co-existence of the two conditions has been reported. 
These muscular cramps persist for a very variable length of time. 
Not only do they vary in different individuals, but the same patient in 
different attacks, or at different times in the same attack may show 
marked variation. In the majority of cases reported the spasms persist 
for from fifteen minutes to an hour, and two or three hours is not an 
excessive period. Hoffmann has reported a persistent cramp which 
lasted for ten days. In fatal cases the contractions pass over into a 
lethal continuous spasm. 
Clonic spasms are rare, but are known as blepharospasm, spasms of the 
tongue, etc. Postconvulsive paralysis or paresis is an uncommon out- 
come. Excessive muscular tire, however, is not rare. 
Trousseau's Phenomenon.-To be able in a free interval to induce an 
attack, or to increase the attacks in force and frequency during their 
continuance by pressure upon a nerve trunk, or upon a bloodvessel, 
is the essential feature discovered by Trousseau. Frankl-Hochwart 
has almost annihilated the specificity of the conception by stating that 
pressure anywhere on the body is capable of inducing the attack. This 
is true in a relative sense only, since sites of election are of primary 
importance. These are the bicipital sulcus and over the crural artery. 
The pressure must be distinct, and not fleeting. It may be necessary TETANY 
757 
to press for at least five minutes. In Howard's American series this 
phenomenon was present in about 80 per cent, of 45 cases; in Frankl- 
Hochwart's, in 62 per cent, of 122 cases. The significance of this 
symptom is unknown. Is it a result of the combined pressure of artery 
and nerve, as claimed by Trousseau, or is it a sign of anemia, as claimed 
by Kussmaul and others? Inasmuch as a bilateral spasm is set up by 
a unilateral anemia, it seems to call for a wfider interpretation, and 
Schlesinger1 arrived at the hypothesis that it is a reflex. 
Ferenczi2 upholds the anemia etiology, since he has demonstrated that 
by simply holding the arms high extended over the head this reduction 
of blood supply will result in spasmodic contractions. If Frankl-Hoch- 
wart is correct in his broadening out of Trousseau's concept, since pressure 
on a bone may bring about the spasm, the anemia hypothesis seems to 
lack support. Furthermore, his studies on animals would seem to require 
in explanation a direct nerve stimulus, rather than an alteration in the 
blood supply. The real difficulty lies behind in the hyperexcitability 
of the nerve impulse. Trousseau's phenomenon is often well marked in 
hysterical patients, and in the more classic types of hysteria. 
Electrical Hyperexcitability.-This is present in (a) motor nerves, 
(&) sensory nerves, and (c) nerves of special sense. 
(a) Motor Nenes.-Erb first showed that minimal electrical stimula- 
tion (0.5 to 2 milliamperes) brought out the tetany spasm reaction. 
Later studies of Weiss and Frankl-Hochwart showed the constant hyper- 
excitability to the galvanic current, while the reactions to the faradic 
stream were inconstant. A large number of anomalous electrical reactions 
occur in the motor nerves of these patients. Thomas3 has described a 
paradoxical "catelectrotonus tetanus." With cathodal stimulation to 
the nerve, with currents too weak to be recorded by the galvanometer, 
one could notice fibrillary contractions in the muscles, which, with the 
increase in current, became more marked until tetanus developed. This 
spread to all the muscles supplied by the nerve and ceased on the closure 
of the current. Anodal stimulation was negative. 
Peters4 has described a "jumping jack" sign brought about by galvan- 
ism of the spine, the anode being applied to the sternum, the cathode 
over the cervical or thoracic vertebrae; minimal currents (3 to 4 milliam- 
peres, even at times 0.5 to 1 milliampere) may cause the arms and legs 
to jump at each closure of the current. He found that the contractions 
may be induced on both sides if the cathode and anode are placed directly 
on the middle line, whereas, if they are placed on one side of the spinous 
processes the contractions will be limited to that side. In the mild cases 
the phenomenon is not induced. In such cases the use of an Esmarch 
bandage will aid in bringing it out. After lumbar puncture it is impossible 
to bring out the sign. 
(6) Hoffmann's researches showed a marked hyperexcitability of the 
sensory nerves; mild currents causing paresthesia or pain; while (c) 
Chvostek, Jr., showed that for relatively mild currents distinct auditory 
1 Neurol. Centralbl., 1892, p. 66. 
3 Johns Hopkins Hosp. Bull., 1895, vi. 
4 Deutsch. Arch. f. klin. Med., Ixxvii, 69. 
2 Ibid., 1904, p. 294. 758 
DISEASES OF THE NERVOUS SYSTEM 
sensations were elicited by opening and closing the current. Taste 
perceptions react in a similar manner, while the optic apparatus does 
not show any reactions. 
Mechanical Irritability of Nerves and Muscles.-Chvostek first showed 
that simple mechanical stimulation of a nerve trunk is sufficient to induce 
a spasm. Sharp tapping over a nerve trunk, or even over a muscle, is 
capable of inducing a typical flexor spasm. The favorite site for bringing 
out Chvostek's sign is over the facial nerve in front of the ear. It is not 
always present, and in 40 of Howard's patients tested, only 50 per cent, 
responded positively, but the figures of Chvostek and of Frankl-Hochwart 
are much more definite. The latter author classes it as one of the most 
important signs of the tetany reaction, although it is not always present. 
He further distinguishes three grades in the Chvostek phenomenon. In 
the more severe reactions (1) a tap in front of the ear will cause contract- 
ures in the entire facial innervated musculature. Even light stroking 
may set free the muscular contractures (Schultze's phenomenon). In 
the middle grade (2) a stroke of the hammer causes contraction of the 
nasolabial folds, while in the lightest grades (3) the corner of the mouth 
alone contracts after percussion in the classical spot. Chvostek's phe- 
nomenon has been found in other conditions, and its frequent occurrence 
in enteroptosis is of particular significance in reviewing the relation of 
gastric disturbances to the tetany reaction. 
Schlesinger's observations of its occurrence in tuberculosis, chlorosis, 
neurasthenia, hysteria, and severe gastric disturbances are of interest, 
since Frankl-Hochwart agrees with him largely in his findings, and even 
goes further and states that he has found the phenomenon in a number 
of normal individuals. In these, however, it exists in his grades Nos. 
2 and 3. He raises the suggestion, however, that possibly many of 
Schlesinger's patients really were mild or abortive cases of tetany. Its 
occurrence in myxoedema (Kraepelin), cretinism (Eiselsberg), and 
related trophic disturbances is recorded. 
A sensory hyperexcitability has been described by Hoffmann and 
confirmed by many. It manifests itself in a manner analogous to that 
of the Chvostek mechanical excitability of motor nerves. Percussion 
over a sensory nerve trunk gives rise to painful sensations or to widespread 
paresthesia in the entire distribution of the nerve percussed. 
Mental Phenomena.-Although it is considered characteristic that little 
or no involvement of the mental functions should be present, certain 
patients with well-developed tetany show mental symptoms. Frankl- 
Hochwart has called attention to this, and Kraepelin has observed it. 
Such mental symptoms have varied from the marked hyperexcitability 
of the chronic tetanoid patient to acute transitory confusion and delirium 
-quite in the sense of a toxic delirium-to a fairly advanced degree of 
deterioration in the sense of secondary dementia, as seen in myxoedema, 
epilepsy, and related types. The cases reported are still too few and 
imperfectly studied to permit any generalizations. It cannot yet be 
decided whether the tetany is to be regarded as primary or secondary 
in many of these cases, and hence the relationship of the psychosis to 
the tetany or to an accompanying condition is far from being clear. TETANY 
759 
Special Senses.-Modification of sight is a rare complication. Changes 
in the general eye apparatus seen as a whole are of not infrequent occur- 
rence. Thus, ocular palsies, nystagmus, anomalies of light and accommo- 
dation reactions are recorded. Retinal changes are occasional, hyperemia 
and neuritis having been seen. These are to be regarded as largely 
incidental in the development of the disorder, and not an intrinsic part. 
The trophic anomalies known to cause cataract are probably more 
fundamental; they are an impress of the general trophic disturbance 
which also sets up the modifications in the excitability of the neuro- 
muscular apparatus, setting free the tetany spasms. Hearing and taste 
are rarely involved. Buzzing in the ears is to be regarded as a general 
widespread disorder, and has nothing to do with tetany itself. 
Sensory Phenomena.-In addition to the purely motor and electrical 
phenomena, certain sensory changes are often present. These are more 
fleeting and less constant, as a rule. Pains during the contraction are 
usually present. They may be severe, or consist of a slight feeling of 
tension and drawing. Paresthesia is irregularly distributed and ex- 
tremely common. Such sensations Hoffmann claims may be induced 
by tapping on the sensory nerves or by electrically stimulating them. 
Such paresthesia is especially prevalent in the incomplete tetany cases. 
Anesthesia is occasionally found. In such instances one should bear 
pseudo-tetany or hysterical tetany in mind. 
Temperature changes have been noted for many years, but are infre- 
quent, and probably the result of some infectious process, which is a 
possible primary cause for the metabolic disturbance in the individual 
case. Respiratory and circulatory changes are to be regarded from a 
similar standpoint. Involvement of the muscles of respiration naturally 
cause primary disturbances. The genito-urinary findings have been 
inconstant. Blood changes are of considerable interest from the experi- 
mental side. MacCallum and Voegtlin have shown a marked reduction 
in the calcium content of the blood in experimental tetany. 
Trophic Phenomena.-These are very variable, and one suspects that 
some bear very little relation to the disorder. Certain of them are 
probably fundamental. Attempts have been made to explain all these 
changes on the general hypothesis of a vasomotor disturbance. The 
development of a cataract, which is not uncommon, is thus explained 
by Peters; but the general disturbance of parathyroid activity is more 
important. Falling out of the hair, loss of the finger-nails, dental anom- 
alies, irregular sweating, oedema, joint and tendon effusions, cyanosis, 
redness, various eruptions of an erythematous, scarlatiniform, measly, 
herpetic, or urticarial nature, are all seen. Many of the skin eruptions 
occur in the gastric cases. The trophic disturbances in tetany strumipriva 
(parathyroidectomy) are to be seen from the same standpoint. 
Tendon Reflexes.-These are known to be modified in a certain number 
of patients, particularly in the direction of hyperexcitability. Ankle 
clonus has been recorded. 
Course.-Clinicians have recognized arbitrarily three groups of cases 
in adults, and most modern authors are inclined to follow Trousseau 
in his classical description. In the benign form the sensory phenomena, 760 
DISEASES OF THE NERVOUS SYSTEM 
such as formication or a simple sensation of heat, may precede the spasm. 
These are confined for the most part to the hands or occasionally to 
the feet. The contractures may be fleeting, persisting for from five to 
fifteen minutes, or they may persist for an hour or more. Often the 
attack terminates by a recurrence of the sensory symptoms. A period 
of repose lasting for a quarter of an hour to two or three hours supervenes, 
and the spasms recur. 1 n some instances two or three attacks a day may 
persist for several months. These mild attacks may recur at yearly 
intervals, or, as in some of Frankl-Hochwart's cases, several years may 
elapse, and then they will recur. These benign cases are usually unat- 
tended with much pain. Consciousness is not disturbed, and there are 
usually no sensory, trophic, or temperature disturbances. Again, more 
severe attacks are observed. These occur more often in young adults, 
the benign forms having been mostly observed among children. Here 
the contractions are more violent and more painful. The preceding 
sensory phenomena are usually more pronounced, and with the increase 
in the severity of the condition other symptoms may be noted. Head- 
ache, malaise, and a rise in temperature of 1° to 3° may be noted. The 
affected muscles may show signs of congestion, and localized oedema of 
the hands and feet may be observed. Other muscles than those of the 
extremities may be involved. The muscles of the abdomen may be 
frequently contracted or the sternocleidomastoid and the .pectorals; 
strabismus may be noted, either the external or the internal rectus, 
particularly the latter, being involved. Trousseau first noted the spasms 
of the pharynx, the larynx, the bladder, and the muscles of respiration, 
with severe dyspnoea in the event of the last beng affected. 
These severe attacks are rarer than the benign ones. Frankl-Hoch- 
wart has shown that there is a distinct tendency for the well-marked 
lighter cases in many instances to become graver, and the good prognosis 
which most writers have given is now seriously doubted by this observer. 
In the grave form there is no addition of symptoms. The attacks occur 
with greater and greater frequency and become more and more intense, 
and the patients die as a direct result. 
Diagnosis.-The diagnosis of a classical case offers few difficulties. 
The presence of cramps in the upper extremities, alone or in conjunction 
with the lower limbs, with the classical obstetrical hand and the additional 
evidence supplied by the Chvostek, Trousseau, and Erb signs, is usually 
sufficient to determine a diagnosis. Frankl-Hoch wart would separate 
the different forms, saying that gastric tetany often offers particular 
difficulties, especially as a group of individuals exists in whom with gastro- 
intestinal disturbances there is a tendency to cramp-like contractures with 
the typical hand position. He groups them with the hysterias, rather 
than with the tetanies, however. 
Hysterical pseudo-tetanies offer perhaps the most difficult diagnostic 
problems, especially as mixed and combined forms are undoubtedly 
known. It is not to be forgotten that some authors enlarge the hysterical 
group and limit the tetany group, and when it is considered how clumsily 
at times physicians conduct their examinations for the Chvostek, Trous- 
seau, and Erb signs, especially overlooking the opportunities for sugges- TETANY 
761 
tion in their examinations, it is perfectly understandable why such 
variances exist. Apart from tactless suggestive influences, however, 
Curschmann, as well as many another, has shown that the so-called 
cardinal symptoms are in reality not absolutely trustworthy. It is at 
times almost impossible to make a diagnosis from pseudo-tetany on a 
hysterical basis. The mental influence of accident insurance, litigation, 
simulation, aggravation, etc., should receive careful consideration. 
Especially is one liable to cast too much discredit upon the patient's 
reliability if these factors appear. The extensive literature quoted by 
Frankl-Hoch wart should be consulted in this connection. 
Tetany strumipriva, or, better, parathyreopriva, as suggested by Erd- 
heim,1 offers the most classical manifestations of the disorder, throwing, 
as well, considerable light upon some of the possible underlying and 
fundamental features of this peculiar reaction type. Insufficiency of 
the parathyroids results in convulsive phenomena of the tetany type. 
Pineles2 has collected all the cases of tetany parathyreopriva, the study 
of which is one of the most important of the steps which led to the concep- 
tion of tetany as a metabolic disorder, in which parathyroid activities 
are chiefly concerned. Since it is more clearly realized that the para- 
thyroids are embedded in the thyroid tissue, and that the disorders of 
both may be present, the real difficulties in diagnosis consist in the separa- 
tion of the "symptoms due to disturbances in function of each gland. 
Prognosis.-The point of view here maintained precludes the possi- 
bility of the statement of a general prognosis. Very little is known 
definitely of the prognosis in infants and children. Most authors agree 
in giving a fairly good prognosis, although Frankl-Hochwart says that 
healthy children rarely acquire convulsions, and that the prognosis is 
not good. In many of these children only one tetany-like spasm has 
been noted. In others the cramps may persist for weeks and even 
months. In simple cases the prognosis' is much better than in those 
complicated especially with gastric or intestinal affections. Bronchitis, 
pneumonia, and occasionally an ascaris infection also determine a less 
favorable prognosis. Dangerous signs appear with glossal cramps, 
which may cause death. Recurrences are frequent in those who recover. 
Tetany coming on during pregnancy and childbirth usually has a good 
prognosis. The hyperexcitability of the nervous system may persist 
for weeks after delivery. In succeeding pregnancies the recurrence of 
the phenomenon may be looked for. Frankl-Hochwart notes that in 
succeeding pregnancies the attacks are apt to be milder. It should be 
borne in mind, however, that sudden death may occur in these cases. 
In the cases apparently due to disturbances of the stomach surgical 
interference has brought about distinct amelioration. Sudden death 
may occur, and apart from surgical intervention the prognosis is admit- 
tedly bad (70 to 80 per cent.). The cases are comparatively rare, however, 
In severe cases associated with marked gastric dilatation, operation, 
1 Mitt. a. d. Grenzgebiet. d. Med. u. Chir., 1906, vol. xvi. 
2 Sitz. b. d. Akad. d. Wiss. in Wien, 1904, 113, Abt. 3, p. 190; and Mitt. a. d. Grenzg. 
d. Med. u. Chir., 1904, xiv, 120. Also consult Pool, Annals of Surgery, 1907, xli, for 
a complete discussion. 762 
DISEASES OF THE NERVOUS SYSTEM 
if only exploratory, is advisable. The mortality after operation in 
some dozen or more cases now reported is as low as 30 per cent. The 
subsequent history of these patients remains to be reported. 
Tetany following infectious diseases and acute or chronic poisoning 
seems to present a favorable prognosis. 
The prognosis of tetany thyreopriva depends upon the amount of 
thyroid gland removed and whether the parathyroids are included. 
Total extirpation of the entire thyroid tissue and parthyroid is recognized 
to have a fatal outcome. The entire after-history of thyroid operations, 
so far as tetany and its prognosis as consequent on the operative pro- 
cedures are concerned, must be entirely rewritten, since it has been in 
recent years only that the parathyroids have been reckoned with as an 
etiological factor. Tetany appears after total removal of the para- 
thyroids, not only in man, but in lower animals. What the outcome of 
complete parathyroidectomy in man may be with the retention of thyroid 
tissue is not yet satisfactorily determined. In Pool's case, in which it is 
possible that the entire parathyroid was removed at the second operation, 
the patient recovered from the operation and was able to carry on her 
work for a time. What the ultimate history may be is undecided. We 
are as yet not in a position to decide as to the definite fate of these para- 
thyroid removals or insufficiencies under calcium or surgical therapy. 
The most important class numerically are the so-called epidemic 
tetanies. Frankl-Hochwart, in his earlier papers, gave a fairly good 
prognosis in this form, but in later contributions he presents further 
histories of these patients, and finds that the prognosis is far from being 
good. Many of them have died, directly or indirectly as a result of their 
tetany or its causative factor, in from four to eleven years after the 
onset. Intercurrent disease caused death in many, so that this author 
only holds that the tetany has perhaps reduced the resistance. Of his 
many cases, 264 in all, he was able to trace 55 some ten or more years 
after. Of these, 11 had died early; of the 44, only 9 were healthy; 37 
came under personal investigation; 7 had chronic tetany, 19 suffered from 
tetanoid symptoms; in fact, two-thirds had persistent tetany reactions. 
This leads him to the conclusion that the prognosis without treatment is 
in reality very bad, which would be more in consonance with the prevail- 
ing point of view that regards the tetany reaction as a result of diminished 
parathyroid activity. 
Treatment.-From the standpoint here outlined it may be readily 
deduced that a general treatment is not a rational mode of approach. 
The essential feature in the general treatment is the search for the irritant, 
and this cannot always be successful. 
In approaching any specific instance the first question is as to the 
validity of the phenomenon. This is placed first, because it should be 
the simplest factor to exclude. If no reason, remote or apparent, can 
be found which would account for simulation, aggravation, or suggestion, 
it may be assumed that the excitation of the nervous system has other 
than a purely psychogenic origin. In rare instances simulation has been 
a factor; thus, patients have simulated tetanoid contractures in order to 
obtain the pleasure of chloroform seminarcosis. TETANY 
763 
With a positive diagnosis established, the organ involved should eome 
into review. Inasmuch as parathyroid insufficiency is the most general 
cause, it is rational to treat those cases, many in children, the whole 
group of so-called idiopathic tetanies, many tetanies of pregnancy and 
of thyroid disease, by thyroid and parathyroid preparations. Para- 
thyroid preparations seem to fulfil most of the conditions, yet occasionally 
the combined thyroid and parathyroid involvement renders the giving 
of the combined products of more service. 
The use of foodstuffs rich in calcium and of calcium salts follows as 
a natural corollary from the studies enumerated. For the most part, 
it would seem that such medication might entirely replace the use of the 
glandular substances themselves. In experimental tetanies the successful 
effect of the calcum salts has been very striking, and in tetanies in children 
calcium therapy has given almost uniformly good results. Up to the 
present time therapeutic experience is not sufficient to definitely prove 
the durability of calcium medication in the cases in which it seems needed. 
The surgical expedient of transplanting parathyroid tissue has proved 
successful in animal work; its successful application in persistent chronic 
tetanies in man is clearly foreshadowed by the experimental work on 
dogs. The technical difficulties do not seem insuperable in view of the 
ready transplantation of these structures to indifferent parts of the 
body. Parathyroid transplantation should not be delayed too long. 
It should be carried out with the onset of severe symptoms and in latent 
cases that have shown grave signs. In pregnancy, when suspicion of 
a latent tetany is present, parathyroid transplantation is advisable. 
Inasmuch as comparatively definite light has been thrown on the 
whole subject of the tetany reaction by the researches of MacCallum 
and Voegtlin in particular, the stomach tetanies, heretofore a particularly 
obstinate group, offer opportunity for a combined treatment, radical as 
well as conservative. Useless gastric operations need not be performed, 
yet at the same time the lines are laid down that point to the time when 
such operations may be of life-saving service. When obvious gastro- 
intestinal conditions point not only to such irritants in the cycle, it were 
folly to persist in a line of medication which, notwithstanding its theo- 
retical possibilities, does not give results. Gastrostomy, gastrectomy, 
gastroduodenostomy, etc., according to the gastric lesion, may be 
demanded, since the gastric and intestinal mucosa seems to be an 
important factor in the possibility of calcium intake. 
The best methods of administration of calcium salts are matters for 
individual experience. Foodstuffs rich in calcium, milk, eggs, whey, 
etc., are clearly useful forms. Injections of calcium chloride, which 
have been useful in animal work, may be adopted in man. Calcium 
hypophosphites or other salts to be taken by mouth are available, and 
intestinal enemata are indicated if other avenues of medication are 
contra-indicated. The exact dosage remains to be determined. CHAPTER XIX. 
PARALYSIS AGITANS. CHOREA. CHOREIFORM AFFEC- 
TIONS. INFANTILE CONVULSIONS. 
DANIEL J. McCARTHY, M.D. 
PARALYSIS AGITANS. 
Synonyms.-Shaking palsy; Parkinson's disease. 
Definition.-Paralysis agitans is a disease of the nervous system 
characterized by muscular rigidity and muscular weakness, with which 
symptoms is usually associated a definite kind of tremor, and on which 
are more or less dependent a deliberation in initiating and a difficulty 
in carrying out active movements, a characteristic facial expression, a 
peculiar attitude, and a distinctive gait. 
Etiology.-Frequency.-Age.-The disease does not seem to be at all 
rare in America. According to German statistics1 there were 37 typical 
cases among 6000 nervous patients. Although cases of paralysis agitans, 
most of them doubtful, have been reported before the age of thirty years, 
and even before twenty years, in the majority of cases the disease does not 
make its appearance in an individual under forty years of age. Willige2 
found only twelve such cases reported. Gowers found the average 
of onset to be fifty-three years in males and fifty-one years in females. 
Cases beginning as late as the seventy-fourth year have been reported. 
Sex.-The disease is about twice as frequent in men as it is in women 
(73 males to 42 females, Gowers). 
Heredity.-The effect of heredity can best be judged by the figures of 
Gowers and of Berger, who, in 15 per cent, of their respective series, found 
a history of the disease in more than one member of the family. Five of 
19 of Wollenberg's patients gave a family history of nervous or mental 
disease. 
Occupation and station in life seem to have little or no influence in 
determining the onset, and in the majority of cases the disease does not 
seem related to any definite determining factor. A direct exciting cause 
can be traced only in about one-third of all cases (Gowers). The influence 
of the emotions is certainly an important etiological factor in a group of 
cases. Gowers emphasizes the importance of fear or fright and reports 
cases in which such emotion was apparently causative. Anxiety and 
worry may be mentioned as other predisposing emotional states. 
Trauma.-The physical influence of trauma, apparent from the attend- 
ant psychic concomitants, is well recognized as a determining cause. 
1 Eulenberg's Real-Encyclopadia, first edition, Berger. 
2 Ztsch. f. d. g. Neur. u. Psych., iv, H. 4. 
764 PARALYSIS AGITANS 
765 
Walz's analysis1 showed in 26 eases, general concussion in 6, wounds 
(stabs and cuts) in 7, burning and freezing in 1, sprains, twists, and 
fractures in 4, and contusions in 8. Some connection between the part 
injured and the locality of the earliest symptoms is almost unexcep- 
tional. Trauma may determine the spread of a paralysis agitans already 
begun. Walz believes that the disease can appear only in a person whose 
nervous system is deteriorated. 
In a group of cases there is a history of overexertion of the muscles, 
especially of those first affected by the disease. Exposure to cold seems to 
be a determining cause in another group. The influence of the infectious 
fevers is probably small. 
Pathology.-"Chaotic" is the term that adequately expresses our 
knowledge of the pathology of paralysis agitans. Pathological findings 
have been manifold, but a definite lesion occurs so inconstantly that 
at the present time it may be said that the disease has no definite path- 
ology. An exception to this statement seems to exist in the changes in the 
muscles found by Camp,2 to whose excellent article the reader is referred. 
A peculiar difficulty at the outset of any investigation to determine the 
pathology lies in the fact that the changes described are not specific, and 
occur in a variety of conditions. It is difficult, for instance, to differentiate 
the various overgrowths of connective tissue and neuroglia in the spinal 
cord from similar changes found in old persons wTho never have suffered 
from the disease. Moreover, there is a group of cases which at autopsy 
shows in the cerebral nervous system no pathological changes whatever. 
Even the experienced eye of Oppenheim could detect in one case only 
a "questionable" staining of the column of Goll. Most of the microscopic 
changes in the brain and spinal cord which have been recorded were 
reported when the condition was not well recognized, and when the 
tremor was confused with that of other diseases, especially multiple 
sclerosis; for instance, one of the cases reported by Parkinson himself 
was probably a case of multiple sclerosis. Tumors of the brain, sclerosis, 
cysts, and ependymitis have been reported in connection with the con- 
dition, but in these cases either the occurrence of paralysis agitans was 
a coincidence, or the tremor was not typical. A " symptomatic" paralysis 
agitans has been written about, but the use of the term " symptomatic" 
and the reason of occurrence of the tremor have not been made clear. 
The fact that macroscopic findings occur must be taken into account if 
we should regard it simply as a symptom complex. 
The microscopic changes described affect chiefly the spinal cord; they 
are many and difficult to separate from those of senility. Many authors 
agree as to an excess of neurogliar tissue in the cord in these conditions, 
but differ as to its distribution and its significance. Redlich,3 for instance, 
found the chief seat of collections of glial tissue to be in the anterolateral 
columns, especially around the bloodvessels, while Sanders4 finds that 
the neurogliar proliferation is more marked in the gray matter. He 
emphasizes this as being a different condition than that found in senility. 
1 Vierteljahrschrift f. gerichtliche Med., 1896, xii, 323. 
2 Jour. Am. Med. Assn., 1909, xlviii, 1230. 3 Jahrb. f. Psych., xii, 385. 
4 Monatsschr. f. Psych, und Neurol., 1898. 766 
DISEASES OF THE NERVOUS SYSTEM 
He found not only a comparatively greater increase in the amount of 
neuroglial tissue in the anterior horn, but also numerous spindle cells in 
paralysis agitans and cases of senility with a marked tremor. 
Dana describes a very moderate increase in the connective tissue within 
the cord. Tract degenerations have been reported, but they were absent 
by the Marchi method of staining in all of 14 cases examined by Camp. 
Changes in the central canal of the cord, for instance, occlusion and 
widening, and the presence within it of leukocytes, have been reported; 
likewise the presence within the spinal cord of amyloid bodies. 
Vascular changes reported include arteriosclerosis of the vessels of the 
cord; miliary hemorrhages into the spinal cord; proliferation of the 
bloodvessels, which may be surrounded by a zone of leukocytes; miliary 
aneurisms of the spinal cord; varicosities of the veins in the medulla, 
with small areas of softening in the oculomotor roots and pyramidal 
tracts. Numerous authors have emphasized the fact that in the arterio- 
sclerosis found the finer capillaries were more affected than the larger. 
Changes have been described in the cells of the spinal cord, in those of 
the anterior horn, but also those of Clarke's columns. Those in the former 
include pigmentation, chromatolysis, loss or displacement of the nucleus 
of the cell, atrophy of the cell, vacuolation of the cell, tumefaction of the 
nucleolus, etc. Dana describes especially pathological changes in the 
dendritic process of the anterior horn cells, with consequent diminution in 
number. This phenomenon was not observed by Camp in any of fourteen 
cases. Pigmentation of the cells of Clarke's columns has also been 
described. Lewandowsky has described in the dorsal vagus nucleus 
pathological changes to account for the disease. Changes have been 
reported in the Betz cells in the cerebral cortex in a few cases, in con- 
nection with changes in the cells of the anterior horns of the spinal cord. 
Various degenerations and scleroses have been noted in the peripheral 
nerves, but these are by no means constant. 
In view of the striking phenomenon referred to the muscles in paralysis 
agitans, it is not strange that a pathological basis of the disease should 
be sought in the muscles themselves. Certain changes were reported 
as early as the days of Skoda (1862), who found a fatty degeneration. 
Numerous observers have since reported atrophy of the muscle fibres, 
hypertrophy of the same, hyaloid and fatty degeneration and prolifera- 
tions of the nuclei of the sarcolemma, with smallness of these nuclei. 
One would expect that some of these might be dependent solely on old 
age, yet, according to recent observers, the findings in the muscles are 
distinctive and can be distinguished from changes due to senility. 
The findings of Camp, present fairly regularly in a series of nine cases, 
are quoted: "Many of the muscle fibres are swollen, and in cross-section 
were round instead of having the normal polygonal shape. There was 
a marked increase in the number of the nuclei within the fibres, many of 
which were smaller, denser, and rounder than the normal. These nuclei 
were frequently in pairs or in long chains. In several places the fibres 
showed atrophic changes, and in these cases, usually of long-standing 
disease, there was an overgrowth of the connective tissue. In many 
changes the fibres had a hyaloid appearance and the longitudinal stria- PARALYSIS AGITANS 
767 
tions were unusually well marked, and in many of the fibres there was a 
tendency to longitudinal cleavage. In one case trichina spiralis was 
found in the muscles, and in this case, in addition to the above described 
changes, there was an intense interstitial myositis and also a discoidal 
degeneration of the muscle fibres. Muscle spindles were hard to find 
in the sections from any of the cases, but in those examined the muscle 
fibres within them showed similar changes to those surrounding them. 
The nerve fibres within the spindle were normal, and the intramuscular 
nerves showed no degeneration when stained with the Weigert hema- 
toxylin stain. These pathological changes were not all of the same 
intensity in all the muscles nor in different parts of the same muscle." 
On account of the statement expressed in the last sentence, little stress 
can be laid on the fact that in some cases of paralysis agitans pieces of 
muscles have been examined with negative results. 
Ductless Glands.-The thyroid was formerly the ductless gland most 
viewed with suspicion as to a possible role in the causation, and of late 
there has been a tendency to hold lesions of the parathyroid gland 
responsible. It is difficult to separate the changes found in the ductless 
glands from those due to old age, and from changes due to diseases other 
than paralysis agitans. Here, again, we are confronted with the fact 
that in certain cases of paralysis agitans the gland assumed to be the 
seat of the disease may show nothing pathological. Thus, Alquier1 
examined the thyroid and adrenal glands from two cases of paralysis 
agitans, and found them negative. Camp, working on an hypothesis of 
Lundborg and of Berkley, examined parathyroids from two cases and 
found them pathological, the change consisting essentially in a peculiar 
infiltration with fat, especially in relation with the bloodvessels. That 
this is not specific is proved by the observation of R. L. Thomson,2 
who, after a comparison of the parathyroid glands in 9 cases of paralysis 
agitans with those of 39 cases of individuals dead of other diseases, 
found that "the parathyroid glands in individuals dying with this 
disease (paralysis agitans) present no change either in number, size, 
position, or histological structure that would serve to distinguish them 
from the parathyroid glands in individuals dying from other diseases." 
The parathyroid has been the subject during the last three years of 
many papers, but no more definite result has been arrived at. 
Parhon and Goldstein3 noted a marked preponderance of hematoxy- 
linophilic cells in the hypophysis of a woman who had suffered with 
Parkinson's disease. Recently, following the work of Kinnier Wilson 
on lenticular disease, the lenticular nucleus has been carefully studied 
with positive results in a few cases but with negative findings in most 
of the cases studied. 
Pathogenesis.-Many theories have been advanced, but in the absence 
of a definite anatomical or demonstrated physiological basis, these are 
little more than speculation. Gowers offers a somewhat hazy explanation 
by referring the tremor to a derangement of the cortical centres concerned 
in movements that are naturally brought about by fear. Numerous 
1 These de Paris, 1903; Revue Neurolog., 1904, p. 438. 
2 Jour. Med. Research, 1906, xv, 399. 3 Revue Neurolog., 1907, No. 22, p. 1230. 768 
DISEASES OF THE NERVOUS SYSTEM 
authors hold that the disease is due to an aging of the nervous system 
before the rest of the body, and point to the fact that the lesions found 
resemble those of senility. While the theory has some evidence to sustain 
it, it does not explain the rigidity which is characteristic of the disease 
or the changes in the muscles. Dana's theory that the dendrites of the 
anterior horn cells are at fault has some degree of plausibility, although 
the lesion is not always demonstrable. 
The theory that the disease depends on changes in the muscles has much 
to commend it, and it is possible that the fundamental lesion is some 
change in the muscle spindles. This latter assumption is rendered the 
more plausible by the fact that the muscles most severely affected by 
the tremor are those which contain the greatest abundance of muscle 
spindles, and that the tremor does not develop in muscles not supplied 
with the spindles. The subject is, however, rendered complex by our 
lack of knowledge as to the function of the muscle spindles. Even if we 
regard them as sensory organs concerned in muscle tone or in muscle 
sense, it is difficult for us to understand how lesions involving them 
account for the rigidity and tremor of the muscles, unless we at the same 
time ascribe some inhibiting influence over these organs by the cells of 
the anterior horn of the spinal cord. The whole subject is rendered still 
more complex by the fact that myotonia congenita, a disease character- 
ized by tonic cramp of the muscles on attempt at voluntary movement, 
presents the pathological changes in the muscles found in paralysis 
agitans, except that the muscle spindles remain uninvolved. That the 
disease is an abiotrophy of the muscles is held by some authors. 
Symptoms.-As a rule, the disease is ushered in insidiously. There 
is first a prodromal period characterized by various sensations of heat, 
weakness, numbness, rheumatoid pains, headache, local oedema, and 
sensations simulating girdle pains. The symptoms usually affect the 
part of the body first to be affected by the disease. This period is variable 
in duration and is sometimes absent, the disease appearing acutely. 
Paiz1 reports a unique, but not typical case, in which vitiligo was the 
first symptom. 
Tremor is the initial symptom in about two-thirds of the cases. At 
times it is entirely absent (paralysis agitans sine agitatione vel tremore). 
At first it occurs only intermittently, and usually affects the small muscles 
of the hand. The fingers, especially the index finger, are alternately 
flexed and extended at the metacarpophalangeal joints, while the thumb 
moves to and fro laterally at the same time that there is flexion and 
extension, the whole resultant movement being somewhat of a circum- 
duction. This movement, in conjunction with the movement of the 
index finger, produces a "pill-rolling" type of tremor, which is very 
characteristic. This is usually associated with a flexion and extension 
of the wrist-joint and a pronation and supination of the forearm. The 
upper arm is affected less frequently than the forearm. In the legs the 
calf muscles are most frequently affected. The muscles of the neck 
may or may not be affected, in which case nodding or rotary movements 
1 Schmidt's Jahrbuch, 1905, cclxxxviii, 228. PARALYSIS AGITANS 
769 
of the head are produced. The tongue and the muscles of the head and 
face sometimes share in the tremor; rarely, if ever, the orbicularis 
palpebrarum. At times the muscles of expression and of mastication 
are involved. The vocal cords and the muscles of the chest and of the 
abdomen are affected but very rarely. 
The movement varies much as to range, and has a rate of abour four 
and eight-tenths to seven oscillations per second (Gowers); it lessens in 
frequency as it increases in range; the finer tremor of the early stages 
is often distinctly quicker than the coarser period of the late period 
(Gowers). The rate of tremor in the arm and in the leg may vary much. 
The tremor continues during rest, is somewhat diminished by voluntary 
movement, and, as a rule, ceases during sleep and narcosis. It is increased 
by mental excitement, and may be somewhat controlled by passive move- 
ment, or momentarily by the concentration of the patient's attention, as, 
for instance, when he fixes an object with his eyes. When hemiplegia 
occurs in an individual with paralysis agitans, as a rule, the tremor 
ceases in the paralyzed members. This cessation may be transitory. 
The one symptom of the disease, which is cardinal, and which, with its 
associated symptoms, constitutes the disease even in the absence of 
tremor, is permanent muscular rigidity. This rigidity is at once apparent 
to the examining hand, and is responsible for the attitude and gait of the 
patient, the delay in executing voluntary movements, the facial expres- 
sion, and the contractures. It affects especially the muscles of the neck 
and the vertebral column; but is also seen in the extremities and face. 
The rigidity usually is marked only when the disease has advanced 
rather far, but, as Wollenberg remarks, "it may be present subjectively 
to the patient very early, manifesting itself by a slowness and a difficulty 
in executing active movements." Associated with the rigidity is a more 
or less well-marked muscular weakness, which, however, never reaches 
the stage of total paralysis. This weakness, with or without a sense of 
stiffness, precedes tremor in about one-fifth of the cases, and occasionally 
is conspicuous with tremor from the first. This weakness may be manifest 
subjectively at a very early stage; in fact, it may be considered as one 
of the prodromal symptoms. Objective weakness, which is manifested, 
for instance, by the inability of the patient to make much of an impression 
on the scale of a dynamometer, must not be mistaken for the apparent 
weakness associated with the slowness and difficulty in executing active 
movements. The latter seems more or less dependent on muscular 
rigidity, and may consist in the slowness in the movement itself, or in a 
delay in the transmission of the voluntary impulse to the muscle. The 
delay may manifest itself even when the movement is executed with 
fair rapidity (Gowers). 
In addition, in consequence of the muscular rigidity, there is a resistance 
to passive movement. This becomes greater as the disease advances, and 
may amount to nearly absolute fixation of a part, as, for instance, the 
head. This rigidity differs from that seen in hemiplegic and paraplegic 
contractures, in that it is permanently present and is not increased or 
called forth by the movement that tests it. Owing to the rigidity, the 
upper part of the body is bent forward, and there is in consequence a for- 770 
DISEASES OF THE NERVOUS SYSTEM 
ward, in rare cases a lateral or backward, position of the head on the 
trunk. J. A. Sicard and L. Alquier1 report deviations of the spinal column 
in 12 out of 17 cases of paralysis agitans. These they believe to be due 
to the muscular rigidity; they may consist of kyphosis, lordosis, scoliosis, 
or a combination of these deformities. The arms are slightly abducted 
and the elbows somewhat flexed. The wrists are usually somewhat 
extended. There may be an ulnar position of the hand resembling that 
seen in arthritis deformans. The position of the fingers varies; in many 
cases they are slightly flexed at all joints in the position that they naturally 
assume during rest; often they are flexed at the metacarphophalangeal 
joints and extended at the others, from the preponderant contraction 
in the interossei. There may even be overextension of the last phalanx, 
most marked in the thumb, perhaps because the tip of the thumb is 
pressed against the first finger. The rest posture is especially frequent 
when rigidity preponderates over tremor. When the interossei contract 
in the tremor the "interoseal posture" is generally very marked. Usually 
the rigidity can be readily overcome, but in extreme cases the contracture 
of the interossei may go on to the degree of permanent shortening, so 
that the metacarpophalangeal joints cannot be passively extended beyond 
a right angle, just as in contraction of the palmar fascia. Occasionally 
only one finger (as the index) is thus affected. In the legs the rigidity 
involves chiefly the hip-joints and knee-joints, causing slight flexion of 
each and adduction of the thighs. It may extend to the feet and even 
cause talipes equinovarus and distortion of the toes. Permanent con- 
traction of the muscles is very rare, but the writer has known each foot 
to be fixed in inversion. 
In consequence of the peculiar stooping posture of the patient and the 
lack of spontaneity in the movements, the term "statuesque" has been 
applied to the attitude. Tilney2 describes a characteristic position as 
follows: (1) Rise of one shoulder and falling of the other; (2) when the 
patient is sitting the long axis of the head makes an angle with the long 
axis of the body; (3) one eye is less prominent and the opening between 
the lids and the pupil smaller on one side than on the other. 
The muscles of the face share in the rigidity, and show a lack of ex- 
pression which has been compared to a mask. The muscles of the eyes 
are but little if at all affected by the slowness of movement, while nystag- 
mus rarely, if ever, occurs as a symptom. The patient may, however, 
show a slowness in initiating a movement of the eyes from one point to 
another. A staring expression in many cases is caused by partial or 
almost complete absence of winking, due to rigidity of the orbicularis. 
The gait is characteristic. The patient, if seated, arises with difficulty; 
the first few steps are slow and shuffling, then the steps become pro- 
gressively shorter and quicker, until the patient has to come to a stop 
to prevent his falling forward. This characteristic gait (festination or 
propulsion) was described by Parkinson. At times the patient, in 
attempting to walk, makes short steps toward one or the other side 
(lateropulsion), or he may make the short steps backward instead of 
1 Nouvelle Iconographie de la Salpetriere, 1902, xv, 377. 
2 Neurographs, 1911, No. 3. PARALYSIS AGITANS 
771 
forward (retropulsion). An individual patient may show both pro- 
pulsion and retropulsion, which may be demonstrated by gently pushing 
the patient forward or backward, or by making slight traction on his 
clothing. The gait was formerly thought to be due to the fact that 
in consequence of the forward position of the upper part of the patient's 
body he was "running after his centre of gravity." However, many 
patients whose heads are turned backward show propulsion, and vice 
versa. The explanation of the phenomenon is probably to be found in 
the difficulty in overcoming the rigidity of opposing muscle groups and 
in the slowness of transmission of controlling impulses to the muscles. 
The patient's voice is commonly high-pitched and piping, and lacks 
proper modulation. The patient has difficulty in shaping the first word 
of what he wants to say, but the succeeding words are enunciated more 
and more quickly. Muller and also Rosenberg reported cases associated 
with tremors of the vocal cords. Cisler, quoted by Pelz, describes the 
vocal cords of 75 per cent, of his patients as approximating the 
cadaveric position. In very advanced cases the speech is a low 
"mumble," difficult, if not impossible, to understand. 
The tendon reflexes may remain normal, or may be increased. Oppen- 
heim and Frank describe a false ankle clonus due to tremor. Oppenheim 
asserts that a true foot clonus is sometimes present, but that the presence 
of Babinski's sign is to be regarded as an indication either that the 
paralysis agitans is symptomatic, or that some other disease exists. 
The paradoxical phenomenon of Westphal is often present; this consists 
in a long-continued contraction of a muscle on approximation of its ends; 
and is seen typically in the contraction of the tibialis anticus muscle on 
flexion of the foot. 
There is a delay in the latent period of muscular contraction, and a 
lessening of the irritability of the muscles and nerves to the electric 
current (Borgherini). As a rule, the function of the sphincters is well 
retained; occasionally incontinence of urine is present. Nystagmus is 
exceedingly rare. Oppenheim reports a paralysis of convergence of the 
eyes, which he ascribes to a probable tonic rigidity of both abductors. 
The same author also reports the presence of von Graefe's sign. Moczut- 
kowsky1 lays stress on the fact that when the patient's forehead is folded, 
the return to smoothness is very gradual. 
Objective sensory disturbances do not belong to the symptomatology 
although they may occasionally be present. An exception is the fact 
that in certain cases the surface temperature of the body may be increased. 
Beyond a tendency on the part of some patients to adopt a whining and 
complaining manner, the mind remains very clear; in fact, the good 
nature and complaisance of most of the patients, in spite of the severity 
of the symptoms, is a matter of common observation. Dementia may, 
however, complicate a case of the disease. Subjective sensations include 
vague pains, vertigo occasionally, and sensations of heat or cold. 
Muscular atrophy does not form a part of the clinical picture; a slight 
degree may be present in long-standing cases. Trophic and vasomotor 
1 Reference in Neurolog. Centralbl., 1897. 772 
DISEASES OF THE NERVOUS SYSTEM 
disturbances may be present; and the skin of the hand may be smooth, 
shiny, and swollen. The excretion of sweat may be increased, and sali- 
vation and drooling are present. The salivation is probably due, at least 
in part, to delayed deglutition. 
The urine may or may not present an excess of phosphates. The sul- 
phates have been found diminished. The total quantity of urea, as a 
rule, does not vary much from the normal, but it varies in proportion 
with the total amount of nitrogen. The urinary toxicity is normal (Vires). 
Forms.-The modifications of the tremors and distribution of the 
rigidity have given rise to various clinical types. Hemiplegic, mono- 
plegic, paraplegic, and crossed forms have been described. Charcot 
writes of a "forme fruste," in which the tremor is very slight and may be 
entirely absent. Entire absence of the tremor is not such a very rare 
occurrence. The rate of fibre tremor may very much exceed the usual 
number of produced movements. The character of the tremor may be 
altered by the influence of active movements, or it may even be elicited by 
these movements. Rigidity may be long delayed, in which case the tremor 
may be difficult to diagnose from that of hysteria or neurasthenia. Hyper- 
extension of the limbs may replace the usual flexion, while a backward 
position of the head is not unusual. Various diseases (Graves' disease, 
myxcedema, tabes dorsalis, hemiplegia) may complicate the disease and 
make diagnosis difficult. A "forme rheumatismale" or "forme doulou- 
reuse" is spoken of by French authors. At times vasomotor or trophic 
phenomena (muscular atrophy, oedema, purpura) help to make up the 
clinical picture. Arterial hypertension has been reported. 
Diagnosis.-In the advanced stages, as a rule, there is no other affection 
more readily diagnosed; occasionally, however, a case presents difficulty, 
as in one reported by Spiller, which had been considered arthritis de- 
formans until a tremor appeared in one of the arms. Other tremors 
dependent on brain disease simulate those of paralysis agitans so closely 
that a group of symptomatic shaking palsies has been recognized. At 
times the tremor resembles that of Graves' disease, a circumstance all 
the more puzzling because in certain cases paralysis agitans and exoph- 
thalmic goitre co-exist. In this case the former affection may be recog- 
nized by the presence of the muscular rigidity and associated symptoms. 
During the prodromal stages it is difficult or impossible to distinguish 
paralysis agitans from hysteria or neurasthenia. The mental make-up 
of the individual should be taken into account, and the possibility of 
paralysis agitans developing in neurasthenic or hysterical individuals 
should not be ignored. From the character of the pains in early cases of 
paralysis agitans, even tabes may be considered. The early loss of the 
tendon reflexes, especially the knee-jerks in the latter affection, together 
with the other signs of the disease (loss of pupillary reaction to light, 
etc.), will serve to make the diagnosis clear. 
Less easy in some cases is the distinction between paralysis agitans and 
multiple sclerosis. It is especially difficult in those cases of paralysis 
agitans in which the tremor is increased by active movements. The 
permanent character of the rigidity in the former affection and the 
nystagmus, sphincter troubles, scanning speech, etc., of the latter con- PARALYSIS AGITANS 
773 
dition will, however, be apparent. Senile tremor may simulate that of 
paralysis agitans closely; it is, however, not accompanied by rigidity, 
and is more apt to involve the head than is paralysis agitans. It develops, 
as a rule, in true old age, while the onset of paralysis agitans is in the 
presenile degenerative period. Oppenheim lays stress on the differentia- 
tion of paralysis agitans from a condition of paraplegia senilis dependent 
on arteriosclerosis of the brain and spinal cord, in which the body is 
held as in paralysis agitans, but in which true paralysis exists. In some 
cases the latter condition resembles a pseudobulbar palsy, and the 
diagnosis becomes difficult, as pseudobulbar palsy may complicate 
paralysis agitans. Hereditary tremor and toxic tremors can be readily 
differentiated by the absence of rigidity. 
At times it becomes difficult to determine whether a paralysis agitans 
following an injury is a true condition or simply a neurosis. Each case 
should be judged on its merits, but collateral symptoms of hysteria speaks 
against the genuineness of a complicating Parkinson's disease. Oppen- 
heim points out that this neurosis is a persistent affection in itself, but 
lacks the progressive character of paralysis agitans. Finally, we must 
distinguish paralysis agitans from the choreas, and especially from 
posthemiplegic choreas, especially as Parkinson's disease may develop 
on a hemiplegia. The jerky, irregular character of the choreas is absent. 
Prognosis.-The disease in itself does not seem to shorten life. In 
certain cases the symptoms tend to progress, both as to extent and 
intensity, while in others they remain relatively slight. 
Treatment.-Little or nothing can be done to effect a permanent cure. 
All efforts should be concentrated to making the life of the patient as 
comfortable as possible. Suitable diet, bathing, exercise, and rest should 
be provided. The patient should be kept as quiet as possible, and should 
avoid all the health resorts likely to cause mental or physical excitement. 
Electricity should be given as electric baths, especially the dipolar 
faradic (Oppenheim), otherwise it is of no service. 
Friedlander1 recommends active movements of the extensors, and also 
passive movements to relax the rigid muscles and to improve the power 
of their antagonists. This relaxation is well gained by allowing the various 
members of the body to fall against gravity. At first some difficulty in 
inducing the patient to relax sufficiently to let a certain part fall may be 
experienced. After a little experience in letting it fall has been gained 
it should be raised against slight resistance. The movements should be 
practised several times during the day, but not for many minutes at a 
time. This treatment should extend over a long period of time. 
Arsenic in the form of Fowler's solution is probably the best agent for 
maintaining the general tone of the patient. 
Hyoscine hydrobromate (gr. to three times a day) and duboi- 
sine (gr. to T|-o three times a day), preferably hypodermically, 
have been recommended for the control of the symptoms, and in certain 
cases do exert temporary influence. Their use is attended with some 
danger, as they may give rise to symptoms of intoxication, which is 
1 Zeit. f. physikalische und diatatische Therapie, November, 1907, p. 468. 774 
DISEASES OF THE NERVOUS SYSTEM 
a signal for their temporary withdrawal. Neither of the drugs is a 
curative agent. The bromides do not seem to be of value. 
Berkley recommends extract of parathyroid gland. All preparations of 
the gland should be kept on ice. "The initial dose of the powdered gland 
was gr. two to four times per day, preferably in capsules; larger doses 
appear to produce weakness, constipation, nervousness, and even an 
exaggeration of the symptoms of the disease. The first good effects in 
the cases treated were noted, as a rule, after fifty to seventy-five capsules 
had been taken." Berkley1 now gives parathyroid nucleoproteid, gr. 
yo, in capsules twice daily for at least three months to a year. Roussy 
and Clunet2 report good results from radiotherapy of the throat by 
destruction of the hyperplastic elements of the parathyroid. 
ACUTE CHOREA. 
Synonyms.-Sydenham's chorea; chorea minor; St. Vitus' dance. 
Definition.-A disease occurring chiefly in children, due to the effect 
of an infectious agent, or its toxin, on the central nervous system, 
characterized by irregular, involuntary muscular contractions, resulting 
in movements of a purposeless nature, and associated with psychic 
manifestations. 
Etiology.-Age.-Chorea may develop at any time of life, but it is 
essentially a disease of childhood and adolescence, and is found most 
frequently in the later years of childhood. In Osler's analysis of 535 
cases, 33 occurred in the first hemidecade, 228 in the second, 212 in the 
third, and 62 in the fourth. 
Sex.-The disease is much more frequent in the female sex; according 
to Gowers the ratio is 3 to 1. In Osler's statistics females were affected 
in the proportion of 2 to 1. 
Race.-Mitchell called attention to the absence of chorea in the 
negro race, and Osler found only 5 out of 175 cases at the Johns Hopkins 
Hospital. The same author has noted the absence of chorea in the full- 
blooded Indian. It, however, may occur in the half-breed. 
Climate.-An attempt has been made by Morris J. Lewis, in the study 
of cases at the Orthopaedic Hospital in Philadelphia, to establish a 
relationship with climatic conditions (barometric and storm influences), 
and with apparent success. Other observers, however, working along 
the same lines, failed in establishing any such relationship. The fre- 
quency of attacks prevailing in the spring months can be attributed to 
other causes. Children are usually of poorer nerve tone and general 
nutrition after a winter of confinement and school work than at any 
other period of the year. The strain of school examinations also has 
an influence in children of a nervous, excitable temperament. 
Emotional Influence.-One cannot fail to be impressed by the large 
number of patients with chorea who present a nervous, excitable tem- 
perament. There is, indeed, a certain type of child who might be referred 
1 Med. Record, xxviii, 1146. 
2 Arch. d. med. Experimentalet Anat. Path., 1910, No. 3. ACUTE CHOREA 
775 
to as of a choreic temperament. Such children, usually of the female 
sex, show an extreme condition of motor unrest. All of the movements 
are quick and extremely jerky. Under excitement the child is so fidgety, 
the movements of the arms and legs so jerky and apparently purposeless, 
as to constitute a condition of normal chorea. When such children have 
Sydenham's chorea, it is extremely difficult to see where the normal 
movements cease and where the manifestations of the disease begin. 
This is particularly true during convalescence. On such a soil it takes 
but little intoxication of the nervous system, from a relatively mild 
infection, rheumatic or otherwise, to produce a well-developed case of 
chorea. In nervous children a decided emotion, such as fright, or excess- 
ive mental strain, may be the determining factor in the precipitation of 
an attack. It would be a mistake to consider emotional disturbances as 
a cause of a clinical syndrome running such a definite course as Syden- 
ham's chorea. In highly emotional children hysterical manifestations or 
even fully developed major hysteria may complicate the clinical picture 
of chorea. While the association of the two diseases has nothing in 
common, there is a possibility of a hysterical child simulating Sydenham's 
chorea when there has been sufficient opportunity for observation of 
another case. Epidemics of chorea in schools may be so explained. 
Heredity.-The influence of a direct similar heredity is very rarely 
seen. An unstable nervous system, inherited from ancestors suffering 
from epilepsy, hysteria, insanity, etc., affords a good soil for the develop- 
ment of various functional neuroses. Such an inheritance is, however, 
not sufficiently frequently met with as to be considered as an essential 
etiological factor in the development of chorea. 
Pathogenesis.-This has been the subject of much discussion, and has 
led to extensive statistical investigation. Like so many organic and 
functional nervous diseases of unknown origin, it was for a long time, and 
is still in many text-books, attributed to fright, rheumatism, exposure 
to cold and wet, etc. Even the early incidence of the disease does not 
save it from being attributed to syphilis. As a result of careful study 
and observation, Sydenham's chorea, as differentiated from habit chorea, 
organic chorea, and electrical chorea, is now well recognized as a syndrome, 
the result of the actions of an infectious toxic agent upon the central 
nervous system. Wollenberg designated the disease as infectious chorea 
as differentiated from degenerative chorea and other choreiform con- 
ditions. It must, however, be admitted that a satisfactory conclusion 
has not yet been reached concerning the specific infecting agent or toxin. 
Nearly all the investigations so far recorded are either controversial or 
disputatious in character, and for that reason lack scientific value. 
In nearly all of these studies, both clinical and pathological, the authors 
have assumed a position (necessarily one of theory), and have then 
written or investigated in support of their position. 
The evidence is far from conclusive as establishing a distinct causal 
relationship between rheumatic fever and chorea. From a clinical stand- 
point, the statistics vary between 1.6 and 85.5 per cent. In the articles, 
however, in which the subject has been studied from an unbiassed stand- 
point, in which there is every evidence of careful investigation and 776 
DISEASES OF THE NERVOUS SYSTEM 
record by competent observers, there is a fair uniformity in the statistics. 
Thayer, Osler, Wollenberg, etc., give statistics varying from 20 to 25 
per cent., and this even with a wide latitude as to what constitutes 
rheumatic fever. These statistics, as presented by Thayer, are of 
especial interest on account of the relationship between rheumatic fever 
and organic heart disease. Thus, 21.6 per cent, of these cases show an 
association with rheumatic fever, and 25.4 per cent, show evidence of 
organic heart disease. 
When an analysis is made with the autopsy records, another difficulty 
presents itself. Uncomplicated chorea is looked upon as having a very 
favorable progress. The mortality as given by the British collective 
committee is 2 per cent. Sinkler found sixty-four deaths in Philadelphia 
in seventy-four years. Taking into consideration, therefore, the fre- 
quency of the disease, it necessarily follows that the mortality is very 
low. The mortality of chorea gravidarum is given as only 20 to 25 per 
cent., and this is a very rare manifestation of the disease. Admitting 
therefore, the complication of rheumatic fever in 25 per cent, of cases, 
it would not be at all surprising if an analysis of the deaths should show 
a large percentage of cases in which this complication was present. 
Other septic processes, which may have endocarditis as a complication, 
may also find a place in these statistics. When, therefore, Poynton and 
Holmes take the position that chorea, to use the expression of Duckworth, 
is a " cerebral rheumatism," and support this position by finding a micro- 
organism in six cases, this position can only be considered as scientifically 
correct for a single group of cases. In at least one of these cases, however, 
the colon bacillus was found in the blood at autopsy. Little stress was 
laid upon this fact, and he used this case as an argument in favor of his 
position. A more reasonable conclusion from the evidence of these 
six cases would be that when a severe type of chorea complicates a 
rheumatic infection, it is very likely to prove fatal. Various statistics 
suggest that we are justified in considering the close association of rheu- 
matic fever and chorea in the maximum of 25 per cent, of cases. 
It should be remembered that in chorea, like epilepsy, the disturbance 
of the function of the cerebral motor apparatus may occur as the result 
of various causes. It would be unscientific to consider that large group 
of cases in which convulsions begin in children of unstable motor mechan- 
ism, and which are the result of any infection, as cases of measles and 
scarlet fever, because these happen to be the most frequent of the infec- 
tions of childhood. . It seems equally unscientific to consider that chorea 
or similar derangements of the motor mechanism symptomatic of an 
acute infectious toxic process are in all cases due to rheumatic fever, 
because a rheumatic infection occurs relatively frequently as a late 
infection in childhood. Macalister,1 using Ross' method for the study 
of the life of the leukocyte, has shown that while the toxin in the blood 
plasma of chorea is toxic to the leukocytes of healthy persons, the blood 
plasma in cases of rheumatism is scarcely at all toxic, and that the plasma 
from chorea cases was toxic to the leukocytes of rheumatic cases; from 
1 Proceedings of Royal Society of Medicine, July, 1909. ACUTE CHOREA 
777 
which it would appear that the poisons in the two diseases are dissimilar. 
The writer is fully aware of the slight or negative articular manifestations 
of rheumatic fever in childhood, but does not agree with some of the 
observers that every case of endocarditis, pericarditis, and myocarditis 
in childhood, in the absence of joint manifestations, is to be considered 
rheumatic. We must consider, on the one hand, that an infection in 
childhood is likely to be associated with vague pains, and that, on the 
other hand, gonorrhoea, beri-beri, syphilis, influenza, tuberculosis, 
erysipelas, pneumonia, and meningitis are associated with arthritic 
manifestations which are described as "rheumatism," etc. 
Other Infectious Diseases.-Measles, scarlet fever, diphtheria, typhoid 
fever, whooping cough, pneumonia, cholera, and smallpox have occasion- 
ally been reported as causative factors of chorea. Syphilis, and septic 
infections, followed by chorea, are also on record. None of these diseases, 
however, with the possible exception of scarlet fever, occurs with any 
degree of frequency. The rarity of chorea as a complication of this 
condition is seen from the statistics of Carslaw, who found only 3 in 
533 cases of scarlet fever. The statistics of Osler are somew'hat mislead- 
ing unless properly interpreted. Of the 554 cases reported, the history 
of scarlet fever was present in 141 cases, without, however, any causal 
relationship between the two diseases. In none of his cases did the chorea 
follow immediately upon the scarlet fever. In this connection it should 
be noted that any condition, w'hether of an infectious nature or not, 
if it lowers the vitality and thereby lessens the nerve tone, renders the 
child more susceptible not only to chorea, but to other functional nervous 
disturbances. 
Pregnancy.-This is an important factor in the production of chorea, 
more particularly in the chorea of adult life. The statistics recorded 
show' the incidence of chorea to be relatively slight after the sixteenth 
year. Only 16 per cent, of the cases, according to the collective investiga- 
tion committee's report, occurred between the ages of fifteen and tw'enty. 
When, however, chorea occurs during adult life, pregnancy must be 
considered one of the most important, if not the most important, factor 
in its production. Chorea may develop at any time during pregnancy, 
but is most commonly seen in the earlier months. It usually occurs 
with the first pregnancy, but may not appear until the second. After 
its development, however, with the first pregnancy, it may, as in a case 
reported by Fere, occur with each successive pregnancy for as many as 
five times. 
Reflex Causes.-Reflex irritation due to various causes, such as intestinal 
parasites, intestinal indigestion, phimosis, adenoids, and eye-strain, has 
been given as a cause of chorea. There is no question that irregular 
movements simulating true chorea are sometimes produced by such 
causes in nervous children. It would, however, be a mistake to consider 
irregular and bizarre movements as identical with a true chorea. A 
study by de Schwreinitz has shown a condition of hypermetropic astig- 
matism in 77 per cent, of choreic children, and he concludes that a 
refraction error may be a determining cause of a choreic attack in a 
susceptible child. 778 
DISEASES OF THE NERVOUS SYSTEM 
Pathology.-Lesions either gross or microscopic have not been found 
in a sufficient number of cases to constitute a definite pathological basis. 
Minor vascular and perivascular conditions have been described in a 
small number of cases, and have constituted the basis for the embolic 
theory. Terminal emboli have been found with more frequency in the 
central artery of the retina than in the cerebral capillaries; Thomas 
collected 7 cases. It is not surprising, in view of the frequency of chorea 
and the large number of cases showing an endocarditis as a complication, 
that emboli should occasionally be found. The absence of extensive 
capillary emboli in the cerebral tissues and a more careful study of the 
bacterial infections, have led most observers to a position adverse to the 
embolic theory. The other vascular changes, microscopic perivascular 
hemorrhages, perivascular leukocytic infiltration, hyaline changes of 
the vessel walls, hyaline spherical bodies, have occasionally been noted 
in isolated cases. Inasmuch, however, as these same changes are more 
frequently noted in other conditions without choreic manifestations, they 
cannot be regarded as constituting a basic pathology. 
Bacteriology.-The reaction of the tissues of the newborn child to 
bacterial invasion has been found to be somewhat different from that of 
the fully developed individual. The central nervous system is at birth 
in process of development. Relatively few of the fibre tracts have reached 
their full myelinization. The functions of the nervous system are also 
relatively incomplete. In its reaction to varying stimuli, the nervous 
system cannot be regarded as complete in its development and of a 
stable nature until after puberty. An infectious agent or a toxin will 
give entirely different reactions at birth, during childhood, and in adult 
life. In children with a finely balanced nervous system and with a 
sensitive reaction to infectious agents or their toxins, a condition of 
disturbed function of the nervous system is more easily produced than 
in the more rugged type. Poynton and Paine considered the diplococcus 
found by them in rheumatic fever as the causative agent in chorea. 
Previous investigations have shown other organisms. In view of these 
findings we are forced to conclude that the exact bacterial cause of the 
disease has not been conclusively proved. 
Symptoms.-Cases of chorea divide themselves naturally into three 
groups according to the severity of the infection: (1) Mild chorea; 
(2) severe chorea; and (3) malignant chorea, or chorea insaniens. 
Mild Chorea.-A prodromal period of a week or more is usually pre- 
sented. The child is listless, somewhat depressed, nervous, with some 
anorexia and sometimes anemia. The nervous irritability becomes 
more marked, and with it slight, irregular, purposeless, jerky movements 
of one or both upper extremities. This is associated with a pseudo-loss 
of power in the extremities affected. The pseudo-loss of power, which 
is dependent on lack of control, is often noticed by parents as the first 
symptom. A careful testing of muscular power will show usually a 
normal condition. The tendency to drop articles which the patient may 
be carrying is not due to a distinct paralytic condition, but to a relaxation 
of the grasp simultaneously with the development of the choreic jerk. 
When one arm alone is affected, the disease may progress to the lower ACUTE CHOREA 
779 
extremities on the same side. Tn some cases the opposite side does not 
become affected, and a condition of hemichorea is presented. Usually, 
however, the opposite side becomes affected, following which the head 
and face share in the movements. Irregular bizarre forms are sometimes 
presented in which, for example, the arm on one side and the leg on the 
opposite side may be involved. Speech is usually not markedly disturbed 
in the mild form, although those accustomed to the conversation in the 
child will often notice some slight change-a slowness of the speech, with 
some hesitancy and irregularity. The movements in the mild form of 
the disease cease during sleep. An actual loss of power may develop 
in mild cases. The mentality is also affected, even in mild cases. 
Severe Chorea -This form of chorea, which may develop in all its 
intensity from the onset, or may, on the other hand, be of a gradual 
development from the mild form, presents a condition of constant, 
irregular movements of practically all the voluntary muscles. The 
mild form is often disregarded by parents and not considered as a serious 
condition, but in this form the severity of the movements and the inability 
of the patients to help themselves immediately demand attention. 
Not only are the movements more constant, but they are increased 
in severity and very often persist to a minor degree during sleep, and in 
some instances actually awaken the patient. The speech is affected in 
almost all cases. This varies from a hesitancy and explosive type of 
speech to a mumbling, and often inability to speak at all. While choreic 
movements of the tongue are sometimes observed, this symptom is, 
in the writer's experience, more frequently seen in hysterical and habit 
chorea than in Sydenham's chorea. Disturbance of speech is often 
due to irregularity of the respiratory rhythm. When the respiration 
becomes jerky and irregular it is not uncommon to find irregularity of 
the heart action with it. Motor weakness is the rule in this form, but 
whether it is a real loss of power or an interference with purposive action 
by the choreic movements is often difficult to determine. The mental 
symptoms are much more accentuated, the child lacks power of concen- 
tration and attention, is often irascible, and presents some failure of 
memory. In this form a slight elevation of temperature may be noted 
varying from 0.5° to 1°. A decided elevation of temperature should 
always suggest the presence of some complication. 
Malignant Chorea, or Chorea Insaniens.-This may be the terminal 
complication of a severe form of chorea, or may develop as a distinct 
type from the beginning. It occurs more frequently as we approach 
adult life. There is often some source of intense worry or anxiety as a 
complicating factor in the etiology. In some cases it is a manifestation 
of an infectious process on a poorly balanced mentality. Pregnancy 
is an important etiological factor. The motor manifestations become 
intense, universal, and constant, may interfere with the sleep of the 
patient, and rapid exhaustion occurs. A confused delirium, with hallucin- 
ations, delusions, and maniacal outbreaks, ensues; the temperature rises 
as high as 104°, and a fatal termination is the usual result. This condition 
is not to be confused with simple chorea or mild types of chorea com- 
plicated by simple delusional states. 780 
DISEASES OF THE NERVOUS SYSTEM 
Psychic Manifestations.-Burr states that "although there are no 
sharp lines dividing the cases, they may be separated, so far as the 
mental symptoms are concerned, into the following groups: First (and 
this includes the vast majority), patients in whom there is peevishness, 
fretfulness, some loss of the power of fixing the attention, and a slight 
loss of the moral sense shown by disobedience and selfishness. Second, 
those showing, in addition to the above symptoms, night terrors, and 
transitory, visual, auditory, or other hallucinations. Third, those with 
distinct delirium, wild or mild, accompanied with fever. Fourth (and 
this group is very small when we remember how common chorea is), 
those showing stupor, or rather stupidity, and an acute dementia, which 
may follow the condition described under three, or appear without any 
preceding mental symptoms at all severe, and which is usually accom- 
panied with trouble on articulation not caused by choreic movements 
of the lips and tongue, but the result of mental hebetude. Fever is 
usually present for a time at least. Patients of the first and second 
groups almost always recover mentally and physically; those of the 
third group frequently die, and those of the fourth usually either die or, 
recovering from the chorea, remain demented." 
This states the subject of the mental condition in chorea, practically 
in its entirety. While the maniacal condition has been considered by 
some to be in the nature of a "cerebral rheumatism," it can be more 
accurately stated that when this condition is associated with a high 
terminal fever, it represents the effect of the infection producing the 
febrile condition. States of mental alienation with excitement, as 
indicative of a grave form of chorea with an unfavorable prognosis, 
should be differentiated from hysteria complicating chorea. Minor 
hysterical stigmata, as might be expected from the neurotic nature of a 
large number of these children, are sometimes met with. Areas of 
anesthesia, hyperesthesia, points of acute tenderness along the nerves 
and vertebrae, and suggestive pains in the extremities have been described. 
Triboulet suggests a correlation of the symptoms to the metameric 
zones of Head. Rarely, hysterical contractures and convulsions, with 
mental excitement, may occur. The prognosis in these cases, however, 
is not altered by this complication. 
Paralytic Phenomena.-A pseudo-loss of power due to the intensity 
of the choreic movements has been described above. In addition to this, 
an actual loss of power, which approaches but never arrives at a com- 
plete paralysis, has been noted by various observers since Sydenham. 
West gave to this condition the name "chorea mollis." The loss of 
power may precede the development of the choreic movements, may be 
coincidental with them, may remain after the choreic movements have 
to a large extent subsided, and may in some cases inhibit the choreic 
movements to such an extent as to appear to supplant them. The motor 
weakness may be partial in nature, a monoplegia, hemiplegia, paraplegia, 
or in very rare instances may be general throughout the voluntary muscles. 
While this condition is usually associated with very severe forms of 
chorea, it may,, on the other hand, complicate very mild cases. Apart 
from the paralytic phenomena, the etiology, symptomatology, and pro- 
gression remain the same as in the usual type of Sydenham's chorea. ACUTE CHOREA 
781 
Rondeau has noted muscular atrophy as a rare complication. This 
exists in cases in which the chorea complicates, or is associated with, a 
rheumatic affection. He considers it as a reflex joint atrophy. It dis- 
appears with the cure of the chorea and the rheumatic fever. 
Reflexes.- Much conflicting testimony has been presented as to the 
nature of the reflex activity in this disease. The writer's experience has 
shown the reflexes to be uniformly active and often quickened when 
properly elicited. The child, however, must be relaxed, and its attention 
thoroughly diverted, in order to determine the exact nature of the reflexes. 
In chorea mollis, when the loss of power is marked, the reflexes may be 
diminished in the affected part. Triboulet notes a delayed reflex activity. 
Other irregularities of reflex action are sometimes noted. The more 
constant of these is a delayed relaxation of the part to normal after the 
motor explosion (Gordon). The writer has never seen complete abolition 
of the reflexes as described by Joffroy. This author described the reflexes 
as diminished, as a rule, and the normal or excited reflex as an exception. 
Oddo also notes, in a study of 147 choreics, normal reflex activity on both 
sides in 8, and on one side in 28 cases, and arrives at the conclusion that 
normal reflex activity only occurs in mild cases, and that usually the 
reflexes are diminished or suppressed (116 out of 147 cases). He also 
notes that an exaggeration and suppression of the reflexes may occur at 
different times in the same case. 
Urine.-An excess of urine and uric acid has been found in proportion 
to the severity of the muscular activity. Garrod found urohemato- 
porphyrin in 14 out of 20 cases. Herter reported the presence of hemato- 
porphyrin in the urine not only of chorea, but likewise rheumatism. 
Glycosuria has also been noted. 
Complications.-The most important complication, which should 
not only be kept in mind but carefully sought for during the course of 
the disease, is endocarditis. The most comprehensive statement of 
the relationship will be found in a study by Thayer, who found that 
25.4 per cent, of his cases showed evidence of organic heart disease. Osler 
found evidence of organic heart disease in 72 out of 140 patients examined 
more than two years after the attack; and in a collection of 73 autopsies, 
endocarditis in 62. Other complications of a rheumatic nature are 
erythema nodosum, subcutaneous nodules, purpura, etc. Herpes zoster 
occasionally occurs, and has been attributed to the use of arsenic. 
Course and Prognosis.-The usual case of chorea runs a course of 
from six to ten weeks. A mild case sometimes recovers in a shorter 
period. Not infrequently the course is prolonged for three or four months. 
Choreic movements may persist for many months-a condition termed 
residual chorea by Guthrie. This can be explained to a certain extent 
by the nervous condition of the child, the movements remaining as a 
habit chorea, and in rare cases may be due to the presence of some per- 
sistent peripheral irritation. Recurrent attacks of chorea are so frequent 
that the physician must be on the watch for them and take measures to 
prevent them. A boy recently under observation has been affected with 
choreic movements in the last five years for a longer period of time than 
he has been without them. The attacks have occurred each spring and 
fall, lasting three of four months. 782 
DISEASES OF THE NERVOUS SYSTEM 
The outlook, as a rule, is favorable. Under proper treatment the 
patients may be assured of a complete recovery, but the parents should 
be told of the possibility of cardiac complications and the tendency of 
the disease to return during a period of several years. 
Treatment.-Chorea at all times, even the milder forms, is sufficiently 
serious to demand careful attention and supervision. All patients do 
better, the course of the disease is shortened, and the danger of complica- 
tions lessened by confining the child to bed during the period of active 
symptoms. Anything that tends to produce mental excitement should 
be rigidly excluded. A nurse trained to handle nervous children is a 
very helpful adjunct to the treatment. The diet should be simple and 
nutritious, with tea and coffee excluded. In the severe cases isolation 
is necessary. Care should always be used in permitting visitors; strangers 
should be excluded from the sick room, and even members of the family 
when their visits or presence produce undue excitement. School duties 
and intellectual efforts should be avoided. Gentle massage, with bathing 
and a warm or cold wet pack, often have a quieting influence when 
properly administered. It is quite necessary in the poorly nourished, 
in whom overfeeding is necessary, to keep the muscles in good condition 
by routine massage, followed during convalescence by passive and 
restricted movements. These, however, should be carefully watched, 
and if any tendency to accentuation in the motor phenomena is mani- 
fested, they should be decreased or stopped altogether. Electricity is 
sometimes of value both as a body stimulant and in keeping the muscles 
in good condition, but often produces too much excitement to be used. 
Galvanism should be applied in preference to faradism. 
The medicinal treatment is confined to alterative tonics and nerve 
sedatives. In the former, arsenic, in the form of Fowler's solution, is of 
distinct value. It is questionable, however, whether its value is more 
than that of an alterative tonic. There is a possibility of doing harm 
if the administration of the drug is not carefully watched and the cases 
for its use properly selected. It is a mistake to give the drug in large 
doses, and it should never be given until the physiological limit is reached. 
Its full value may be obtained by the administration of small doses; 
two to three drops gradually increased until ten drops three times a day 
are taken. It ought never to be administered except when the patient 
is under the personal observation of a physician. Indiscriminate and 
persistent use of the drug may result in arsenical neuritis and serious 
disturbance of metabolism. Donovan's solution may be administered in 
doses of two to five drops, in older children, three times daily. Strychnine, 
cimicifuga, and belladonna are sometimes used. 
Sedative drugs have a decided value in the more severe and active 
cases. Chloral is the most valuable of these remedies, and is usually 
combined with one of the bromides in doses of 5 grains each, three or four 
times a day. In some cases it may be necessary to increase the bromides 
slowly, to as high as 15 grains, in older children. In other severe cases 
hyoscine may be used, either by mouth or hypodermically. Antipyrine, 
extract of cannabis indica, and chloretone have been found to be of value. 
Chloretone, like hyoscine, has a decidedly quieting influence in some ACUTE CHOREA 
783 
cases, but the administration of both drugs must be carefully guarded. 
Such remedies as aspirin, the salicylates, and alkalies are of value in the 
control of rheumatic symptoms, but have relatively little influence on 
the course of the disease. Potts found apomorphine to be of distinct 
value in controlling the symptoms of very severe cases. 
Salvarsan by intravenous, intramuscular and infraspinous injection 
has given uniformly good results, more particularly in severe and resistant 
cases. This has led Milian1 to the conclusion that chorea is in all cases 
due to syphilis. 
Lumbar puncture,2 the subcutaneous injection of cholesterin,3 thy- 
roidin,4 and trional have been reported as giving successful results. 
In chronic chorea, when the movements persist after the general 
condition is improved, and in some of the milder forms, treatment by 
means of a combination of suggestion, at first passive movements and 
later by means of exercises, may be of distinct value. This treatment 
has been systematized by Guthrie as follows: 1. Suggestions that the 
child should lie quietly should be repeated in the soothing manner used 
by the hypnotist. At the same time the flourishes and wriggles should 
be gently restrained, and the child will soon learn to lie completely relaxed 
and flaccid under observation. 
2. Passive movements combined with suggestion. It will be found 
that choreic movements occur directly the child's limbs are manipulated. 
To correct this condition the patient's hand should be placed between the 
observer's hands, and raised and moved in various directions, suggestion 
being made meanwhile that the child should keep his own hand quite 
still. At first it will be snatched away and flourished as usual, but soon 
the patient learns to control the involuntary movement by an effort of 
will which makes the whole limb rigid. This is an indication of improve- 
ment, although by no means of a cure; but by degrees the child discovers 
that the efforts to control involuntary movements need not be so strenuous 
whilst his limbs are guided and restrained, and accordingly the rigidity 
becomes relaxed. Until the limbs can be passively moved freely in all 
directions without exciting either rigidity or spasm no other than passive 
exercises should be employed. 
3. Voluntary movements under guidance. The patient is then directed 
to perform the same movements as have been passively executed while 
his hand is still controlled, guided, and helped by the observer. 
4. When this can be done without exciting spasms or rigidity, but 
not before, voluntary movements without control, in imitation of the 
observer's should be practised. 
The movements should be of the simplest character at first, and 
gradually made more elaborate. They should be executed slowly and 
steadily, without jerks and flourishes. The chief difficulty is, at this 
stage, incoordination rather than spasm. 
1 Bull, et Mem. de I. Soc. des Hdpit. de Paris, 1912, No. 23. 
2 Richardson, Bull, de I. Soc. de Pediatric de Paris, 1911, p. 111. 
3 Margarot, Montpel. Med., 1912, xxxv, 621-627. 
4 Roden, Lancet, 1911, ii. 784 
DISEASES OF THE NERVOUS SYSTEM 
Elaborate apparatus is unnecessary. Simply bringing the fingers 
together from a distance and touching various parts of the body with 
them are admirable exercises. Precision of movement can be gained and 
the child kept amused by various toys and games. Colored balls hung 
by strings on a frame or strung on wires can be used. Then solid squares 
or cubes can be built in various shapes and forms. Such games as 
draughts, dominoes, or solitaire can be used for older children. A child 
may usually be considered cured when he can build a two-story house 
of cards. 
The aim of exercises is to encourage freedom as well as precision in 
movement. For this reason it is inadvisable to allow the patient to 
write or to thread needles or to sew. All these require much mental 
concentration, fixation, and tension of the various muscles employed. 
There is no objection to freehand drawing on a slate or blackboard, and 
paper plaiting or weaving may be encouraged. It is important that all 
exercises should be carried out under supervision, otherwise the child 
becomes careless and ceases to take any trouble, provided that he can 
amuse himself, and if neglected often drifts into what may be called 
"residual chorea." The treatment in this event is by a course of drilling, 
marching, wheeling, and standing at attention, at word of command 
in particular, for the child's-chief faults are inattention and carelessness, 
which have to be overcome. Ordinary calisthenic exercises are also 
useful in these cases. The important feature of "residual chorea" is 
that the movements are unconsciously performed, and can be immediately 
controlled by effort of will. 
The treatment of the lower extremities is on the same principles as 
that of the upper. The patient should not be allowed to stand, or try 
to walk until all movements of the legs can be executed with fair precision 
while lying down. Such ataxia as still remains should be treated bv 
making the child stand supported by the back of a chair while he places 
each foot separately in various positions. It is necessary finally to observe 
that exercises of any description are inadvisable in all acute and recent 
cases of sthenic chorea. They are only of use when heart, pulse, tem- 
perature, appetite, and digestion are normal, all pains and aches have 
disappeared, and, although general health has seemed fully restored for 
some weeks, choreic movements still continue. 
The principles in this are: (1) That suggestion is of service where 
there is lack of inhibitory control over choreic movements. (2) Inhibi- 
tion when acquired is often exaggerated, and has to be regulated by 
assistance before voluntary movements can be executed. (3) Inco- 
ordination has to be treated by exercises carefully graduated in the order 
of their difficulty of accomplishment. 
HABIT CHOREA, HABIT SPASM, CONVULSIVE TIC. 
Definition.-A tic is a sudden, quick, involuntary coordinated muscular 
action, usually of a purposive type, and partly or entirely under the 
control of the patient. HABIT CHOREA, HABIT SPASM, CONVULSIVE TIC 
785 
Much time and thought have been devoted to the elucidation of the 
subject of tics. Sinkler, in using the term habit chorea, placed them in 
the larger group of the choreas. Objection has been made to this on 
the ground that, being purposive and intentional, they are of an entirely 
different nature from the movements of Sydenham's chorea. The term 
habit spasm is equally objectionable, because a large number of them are 
not in the strict sense of the term of a habit nature; i. e., they do not 
develop by repetition from a volitional act. While Guthrie separates 
these two conditions, it is often difficult in the fully developed complex 
tic to decide whether it began in the form of a habit spasm or not. All 
investigators agree in separating from the group of what might be termed 
harmless tics those cases presenting a similar clinical picture, but develop- 
ing on a foundation of a serious neurosis. In Tourette's disease, which 
represents the most marked type of this latter group, all the voluntary 
muscles may be affected. The cases in this group are classed as incurable. 
A study of a large number of cases of tic will show a neurotic basis not 
only for the psychic tics (Tourette's disease), but in the simpler forms. 
It is likewise true that in many of the simple habit spasms and tics a 
psychic element plays an important role. Patrick has called attention 
to the fact that the tic movement represents a distinct psychic want- 
i. e., a sense of relief as opposed to a very decided sense of psychic distress 
when the movement is inhibited. Each case of tic represents in its 
etiology and its essential nature some definite disturbance of the nervous 
balance. As such, each case needs special and individual study. 
Etiology.-An inherited or acquired neurotic temperament is the 
important etiological factor in most cases. In children of a nervous 
temperament, self-control is an essential factor in maintaining a nervous 
balance and proper functioning of the nervous system. Habit spasms 
and tics in early life and other functional neuroses in adolescence and 
adult life are frequently the result of a lack of discipline in the training 
of a child. The more vicious the heredity, the more need there is for 
careful guarding of the education and the self-control of the child. 
Anything which reduces the nerve tone of a child may determine the 
development of a tic. In some children tic may develop as a result of a 
loss of weight, vigor, and nerve tone, below a certain point. In these 
children such manifestations may be looked upon as an indication for 
medical supervision and treatment. In finely balanced children a nerve 
drain from some irritation, such.as carious teeth, adenoids, phimosis, 
intestinal parasites, etc., may be the determining factor. These causes 
are usually referred to as reflex. Excessive worry, emotional excitement, 
or other forms of mental overstrain may in some cases be the cause. 
Symptoms.-The movements differ from those of Sydenham's chorea 
in- their evident purposive character and their localization to a single 
muscle or group of muscles. The muscles of the face are most frequently 
affected. A sudden, quick blinking of the eyes, which may be repeated 
very frequently at short intervals, or occur only a few times during the 
day, is the form most frequently seen. The eyebrows in other cases 
are suddenly elevated; the face may be drawn to one side, or the facial 
muscles of both sides may be affected, producing a sudden involuntary 786 
DISEASES OF THE NERVOUS SYSTEM 
grin. The tongue may be affected, and a sudden movement as if moisten- 
ing the lower lip may be so frequently repeated as to produce an inflamed 
condition of the skin of this area. In one child, who had been able 
voluntarily to produce a movement of the ears, a sudden jerky movement 
of both ears developed independently of the volition of the patient. 
Spasm of the muscles of the neck results in a jerking of the head to one 
side; shrugging of one or both shoulders is not infrequent. There are 
usually no other symptoms apart from the motor phenomena. Hysterical 
outbreaks are usually met with in the type subject to this affection. 
Diagnosis.-In rare cases several groups of muscles may be affected 
at the same time, but this can be easily differentiated from Sydenham's 
chorea by the purposive character of the movements. 
There is a condition of the muscles of the face, most frequently localized 
to the orbicularis palpebrarum and due to the toxic influence of tea and 
coffee on the nervous mechanism, which should be distinctly differentiated 
from the habit spasm. The manifestations are lightning-like contractions 
of the individual fibrillae, affecting all of them in rapid succession, pro- 
ducing at the most a slight quivering motion of the lids, but never leading 
to the distinct blinking of habit chorea. This is as frequently seen in 
adult life as in children, and yields rapidly when tea and coffee are 
excluded from the diet. In some of the cases eye-strain is a factor. 
Habit spasms should also be differentiated from what has been described 
as impulsive tic (Gilles de la Tourette's disease). Some of the forms of 
this condition appear to be a more serious and wide-spread affection of 
the toxic condition above described, affecting the orbicularis palpebrarum, 
and due to some intoxication. It is not infrequently fatal. It begins, 
as a rule, in very early life, although it may occur as late as early adult 
life. The muscular movements may affect all the voluntary muscles, 
and are lightning-like in character, with marked fibrillary movements. 
Another group of cases described under this condition presents the same 
quick action of the muscles, with mental disturbance and the use of foul 
language. The explosive, quick character of the movements, the mental 
disturbance, and the coprolalia should differentiate it. 
Treatment.-In both habit spasm and impulsive tic an underlying 
cause should be carefully searched for and removed. In the habit spasm 
about the eyes, errors of refraction and loss of muscle balance should be 
first corrected. The mucous membrane of the nose and the condition 
of the turbinate bodies should be examined to determine any cause for 
irritation. The ears and teeth should in the same way receive attention 
in all cases where the symptoms are referable to any part of the face. 
Irritative reflex disturbances in the genito-urinary tract, such as phimosis, 
etc., should be relieved. 
The general nutrition should be brought to a normal standard and a 
proper discipline infused in a routine way into the child's life. While 
punitive disciplinary measures sometimes succeed in early imitative 
cases, they frequently do harm. The child should be sent to bed at a 
definite time early in the evening and compelled to remain in bed an hour 
after the usual time of rising, both as a disciplinary measure and to secure 
an added amount of rest for the weakened nervous system. A period 11 UN TING TON'S CHOREA 
787 
of rest in the middle of the day is also advisable. A cold sponge bath or 
needle bath is valuable as a tonic stimulant if the child reacts well. A 
simple diet without tea or coffee and with little meat, but with plenty 
of milk, eggs, and vegetables, is indicated. Alterative tonics, such as 
Fowler's solution, quinine, and strychnine, are sometimes of value; 
more frequently better results are secured by the use of bromides and 
other nerve sedatives. The child should be encouraged to inhibit the 
movements as far as possible. 
Electric Chorea.-This is a rare disease, first described by Dubini, and 
is manifested as intensely rapid rhythmical movements in the extremities, 
rarely in the head and face. The movements may be very violent, and 
have the appearance of being produced by an electric shock. In the 
severer form described by Dubini as occurring in Italy, paralytic symp- 
toms supervene, and may be associated with epileptiform convulsions. 
Pain in the head and neck may be present early, and toward the end of 
the attack atrophy and wasting of the muscles may occur. Fever may 
be present. The cases terminate in a few weeks or months from heart 
failure or coma. Jaccoud considers the affection to be due to a sympto- 
matic chorea due to a low grade of cerebrospinal meningitis. A form of 
electric chorea, probably due to hysteria, has been described by Bergeron. 
Henoch has also described a form of electric chorea differing from both 
the above, and manifested by spasmodic attacks of lightning-like contrac- 
tions confined to the muscles about the shoulder blade; it is probably 
a form of myoclonus. 
Treatment.-This should be directed to the removal of any underlying 
intoxication. Free purgation, chloral, and bromides have been used, 
but to little effect. 
HUNTINGTON'S CHOREA. 
Synonyms.-Chronic progressive chorea; chronic hereditary chorea; 
degenerative chorea. 
Etiology.-Age.-The disease is preeminently, if not exclusively, a 
disease of adult life. It usually begins between thirty and forty years of 
age. Premonitory symptoms, in the nature of mild choreic movements, 
or more frequently in the nature of extreme clumsiness, or sometimes in an 
extra precision of movement, occur as early as the twentieth year. Cases 
have been reported in childhood, i. e., in the second decennial period, by 
Hoffmann, Peretti, and others. Stevens reports a case in which the 
chorea developed in infancy. The accuracy of this case as one of Hunt- 
ington's chorea has been questioned. Jolly reported a case in which 
chorea and epilepsy developed unquestionably at nine years of age. 
Hemak reports a case in which epilepsy was present from the twenty- 
third to the thirty-first year, followed by chorea at forty. Diefendorf 
reports a case in which epilepsy developed at seventeen and choreic 
manifestations appeared at fifty-three. 
Distribution.-The cases occur practically over all the European 
countries-with the exception of Turkey-the West Indies, and South 
America. Several cases have been reported in negro families, associated 
with other nervous maladies, the most important of which is epilepsy. 788 
DISEASES OF THE NERVOUS SYSTEM 
Heredity.-Direct heredity has been found in practically all of the 
cases studied. The exact percentage, however, of those affected who 
developed chorea has not been determined. Huntington states that 
" if one or both of the parents have shown manifestations of the disease, 
and more especially when these manifestations have been of a serious 
nature, one or more of the offspring almost invariably suffer from the 
disease if they live to adult life; and if by any chance these children get 
through life without it, the thread is broken and the grandchildren or 
great-grandchildren may rest assured that they are free from the disease. 
Unstable and whimsical as the disease may be in other respects, in this 
it is firm; it never skips a generation to manifest itself in another; as spon 
as it has yielded its claims, it never regains them." Huntington further 
speaks of the presence of the nervous temperament in all or nearly all of 
the families in which the choreic taint exists, and that " nervous excite- 
ment to a marked degree almost invariably attends upon every disease 
these people may suffer from, although they may not in health be over- 
nervous." Heilbroner states that there is a tendency in successive 
generations for the onset of the disease to be delayed. 
Sex.-Huntington states that it is more common among men than 
women. He was not aware that season or complexion had any influence 
in the matter. Wollenberg has collected statistics of 128 cases in twenty- 
two families; of which 74 were in men and 54 in women. 
Pathology.-The picture is that of a chronic diffuse cortical encephal- 
itis. Three of the writer's cases in both the gross and microscopic picture 
resembled very closely that seen in paresis. There was a marked atrophy 
of both the convolutions of the frontal lobes and the motor area. The 
arachnoid was thickened relatively (due to cortical atrophy) and actually, 
due to a chronic proliferative process. There was a proliferation of the 
neurogliar network, with an increase of the neurogliar cells both in 
the cortical and subcortical tissue. The atrophic process was shown by 
the absence of the tangential fibres and by the marked perivascular and 
pericellular space dilatations with both perivascular and pericellular 
neurogliar nuclear proliferation. The ganglion cells of the cortex appeared 
to be fewer in number, and with atrophic and pigmentary changes. 
There was an accumulation of subarachnoidal fluid (hydrocephalus 
externus), which was in all probability secondary to the atrophic processes 
in the brain. Throughout the basal ganglia extensive areas of perivascu- 
lar proliferative gliosis were present. In other cases simple hemorrhagic 
pachymeningitis was found. The pathological changes in the basal 
ganglia, especially the lenticular nucleus, represent the basis for the 
choreic movements, the cortical cellular changes that for the dementia. 
Lannois and Paviot explain the development of the disease upon the 
hereditary malformation of the neuroglia of the cortex. 
Symptoms.-The heredity, the chorea, the tendency to insanity, and 
its development in adult life are the important features. Of the chorea, 
it may be stated that the movements resemble very closely, yet are 
distinctly different from those seen in Sydenham's chorea. They are 
essentially of the same purposeless, jerky nature. There is a gradual 
development, at first an apparently extra precision of voluntary motion, HUNTINGTON'S CHOREA 
789 
followed by a distinct clumsy, irregular action of the muscles. They 
are in the beginning of minor intensity and may be confined to the upper 
extremities or the face. Later, the legs, and afterward the voluntary 
muscles of the entire body, with the exception of the eye muscles, become 
affected. In its final development there is a tendency to more symmetry 
of movement than is seen in Sydenham's chorea. This results, when 
the lower extremities become affected, in a shuffling, dancing type of gait, 
associated with irregular movements of the trunk and arms. Choreic 
movements of the facial muscles cause the production of grimaces some- 
what allied to those seen in some cerebral types of chorea. Speech, 
sooner or later, becomes affected on account of the involvement of the 
tongue, the lips, and the respiratory muscles, and assumes a hesitating, 
stuttering, explosive type of articulation. The movements, as a rule, 
but not necessarily, disappear during sleep. The movements are under 
the voluntary control of the patient, even when moderately severe. 
A patient, for years under personal observation, although suffering 
severely from choreic movements, was nevertheless able to attend to his 
occupation as a carpenter, and to drive nails with fair precision. The 
motor power is, as a rule, not involved. In terminal cases, a general or 
hemiplegic loss of power is sometimes noted. The reflexes are exagger- 
ated, as a rule. The electrical excitability remains normal. 
Psychic Symptoms.-Huntington stated that "the tendency to insanity, 
and sometimes that form of insanity which leads to suicide, is marked. 
I know of several instances of suicide of people suffering from this form 
of chorea, or who belonged to families in which the disease existed. As 
the disease progresses, the mind becomes more or less impaired, in many 
amounting to insanity, while in others mind and body both gradually 
fail until death relieves them of their sufferings. At present I know7 of 
two married men, whose wives are living, and who are constantly making 
love to some young lady, not seeming to be aware that there is any 
impropriety in it. They are suffering from chorea to such an extent that 
they can hardly w'alk, and would be thought by a stranger to be intoxi- 
cated. They are men of about fifty years of age, but never let an oppor- 
tunity to flirt with a girl go past unimproved. The effect is ridiculous 
in the extreme." Other observers have also noted the mental condition 
to be a severe and gradually progressive deterioration, ultimately ending 
in absolute dementia. In some .cases the mental defect is noted from 
the onset of the symptoms; in others, the mentality may remain unim- 
paired for years. Mental deterioration is the rule, and it is associated 
with a loss of memory and a tendency to self-destruction which gradually 
develops. When the mental degeneration is well marked, outbreaks 
of violence are sometimes noted. In one of the writer's patients, as the 
disease progressed, the clinical picture of paresis was presented. The 
chronic delusional state is more often noted than would be inferred from 
Huntington's description. 
Diagnosis.-The other forms of adult chorea apart from the infectious 
toxic variety of Sydenham may occur, but this is the only type which 
presents any difficulty in diagnosis. The senile chorea associated with 
arteriosclerosis, and the degenerative processes of the cortex which go 790 
DISEASES OF THE NERVOUS SYSTEM 
with it, are sometimes mistaken for Huntington's chorea. This con- 
dition, however, only appears long after middle life, and lacks the most 
essential feature in Huntington's chorea, the heredity. In investigating 
the family history it should be borne in mind that the disease is usually 
looked upon as a family affliction, and its presence in other members 
of the family is at first denied by the patient. It requires tact and 
careful questioning to elicit this important factor in the diagnosis. 
Prognosis and Treatment.-The prognosis is absolutely hopeless. 
Nothing has been found to retard or inhibit the course of the disease. 
CONVULSIONS OF INFANCY AND CHILDHOOD. 
Convulsions occurring in infancy and childhood vary greatly in nature, 
in causation, in the clinical picture, and in the resultant effect upon the 
nervous system. They may present the symptomatic representation 
of some general disease or some toxic condition or, on the other hand, they 
may present, as an epilepsy, the disease itself. This article will consider 
the subject of infantile convulsions as distinct from epilepsy. 
Etiology.-Age.-In most cases fits occur before the completion of 
the first dentition. Of 300 cases studied by Cautley,1 44 began in the 
first six months of life, 52 in the second, 76 in the second year, and 44 in the 
third year. Thus, no less than 216 were infants under three years of age, 
27 were in their fourth year, and the remainder under twelve. 
There is no question of the different reaction of the nervous system 
in infancy, childhood, and adult life. Even when there is a definite 
pathological factor, as disease of the thymus, this variation is shown, for 
at different periods of the development of the nervous system, thymic 
asthma, convulsions, and myasthenia gravis are presented. At the 
onset of the acute infectious fevers the rigor of the adult is sometimes 
represented by a distinct convulsive seizure. This variation is more 
one of degree than of essential difference. Too much stress is often laid 
upon the term "convulsion." While loss of consciousness is the rule 
in convulsive seizures, it need not necessarily be absolute. The 
motor intermittent muscular reaction, or a decided chill, which has 
been described by a sensation of cold, is essentially a convulsive seizure 
of nervous origin, and not under the control of the patient. The same 
grade of intoxication in infancy and early childhood produces a more 
acute motor reaction with disturbance of consciousness. 
The work of Soltmann explains to a certain extent this variation. 
He found in experimental work on animals that the irritability of the 
motor nerves in the newborn was negative, while the irritability of the 
sensory nerves was low. Both varieties of nerves after birth, however, 
not only reach the normal irritability, but actually develop an electrical 
hyper-irritability above that seen in the adult. The cerebral motor 
area is undeveloped and does not react at birth to electrical irritation. 
The reflex inhibitory centres and paths were also found entirely wanting. 
An important factor is the lack of synchronous development of the 
1 The Clinical Journal. 1905. CONVULSIONS OF INFANCY AND CHILDHOOD 
791 
inhibitory with the motor function and the peripheral irritability. As a 
result of this the reflex irritability in the newborn is somewhat diminished, 
and thereafter shows an increase to a point above normal. Soltmann 
also determined an increased irritability from the fifth to the eleventh 
month. While later investigations have shown that in certain respects 
it has been necessary to modify these observations in all cases, they remain 
for the most part confirmed. All the observers are agreed upon the 
absence of the inhibitory function at birth, its delayed development, and 
the incomplete function of the psychomotor centres. 
Apart from these factors, the absence in infancy of volitional motor 
control, that lack of what might be termed subconscious "willing," a 
part of the psychic function, appears to be an important element in the 
production of convulsive states. The well-trained and well-disciplined 
nervous system is less likely to show motor unbalance under stress and 
insult than when the opposite is the case. In infancy, lack of such 
training and discipline, together with the failure of synchronous action 
of the different functional areas of the brain, combined with the varying 
progress in the development of the elemental functions, renders the 
nervous system susceptible to disturbances of motor and psychic correla- 
tion by toxic, traumatic, or organic influences. In other words, there 
is wanting not only the automatic inhibition of the nervous system, 
but also its reinforcement by volitional inhibition and control. The 
production of convulsive seizures can best be understood in the simple 
reflex group. Convulsions may be produced in guinea-pigs by irritation 
of the sciatic nerve. In a patient under personal observation convulsions 
were produced by the dressing of an irritated surface on the leg. 
The patient, a man, aged forty-two years, had convulsions during child- 
hood up to the sixth year, and was entirely free until the necessity arose 
for active treatment of the local condition. On every occasion on which 
the ulcerated surface was dressed without the use of cocaine, a general 
convulsion resulted, which could be entirely prevented by proper local 
anesthesia, and ceased to occur after improvement in the part. In toxic 
cases the irritation is probably direct upon the central nervous mechanism. 
In convulsions of gastro-intestinal origin there is in all probability a com- 
bination of factors-a reflex exciting factor, a complete toxic factor, and 
a possible disturbance of the cardiorespiratory mechanism. 
Predisposition.-The individuality in children, as regards motor balance, 
coordination of the psychic, motor, and inhibitory functions, and the 
defence of the body in resisting invading toxins is as varied as the physical 
character. This, in some cases, is a matter of heredity; in others, and 
this is more particularly true in infancy and early childhood, hereditary 
influences do not play an important part. In a family of six children 
of good heredity one of the children had convulsions at the onset 
of measles, scarlet fever, and influenza, whereas the other children 
suffering from the same affections of approximately the same intensity 
were free from convulsive seizures. We may, therefore, assume even 
in infancy a predisposition to the convulsive habit. This is true both 
of the convulsions of a reflex nature as well as those to which the term 
"idiopathic" has been given. Moon, who made a careful study of 792 
DISEASES OF THE NERVOUS SYSTEM 
200 cases, found that convulsions in infancy associated with reflex causes 
were quite as serious as regards frequency and the mental and moral 
deterioration as those of an essential nature. In all the varieties of the 
convulsions of childhood a certain intrinsic condition of the brain in the 
nature of an instability of function is the most important etiological 
factor. 
Convulsions in childhood almost always have a serious aspect. With 
proper care and attention at the time of the convulsions, and with careful 
attention to the life, education, and after-treatment, they may mean 
nothing more than an accidental complication of some underlying con- 
dition. On the other hand, they may be the starting point of a serious 
disturbance in the function of the growing nervous system, with mental 
and moral deterioration, and neuroses and epilepsy as the end-result. 
Reflex Causes.-Reflex disturbances, in the writer's opinion, have 
been much exaggerated as a causative factor. That they are an important 
determining factor the writer is not prepared to deny. Except, however, 
for those which produce sufficient sensory irritation to amount to pain, 
they act, for the most part, in lowering the general health and nutrition, 
and causing a loss of nerve tone sufficient to bring the nervous system 
to a convulsive reaction. We should divide reflex factors into those which 
are purely reflex in their action and those associated with the disturbance 
of metabolism. One of the most important factors, namely rickets, 
which has been placed by some authors in the reflex group, probably 
acts in both ways. Irritation and subcutaneous lesions of the skin, 
such as that resulting from excessively hot baths, scalding and burning 
of the skin, sudden and violent variations in the surrounding temperature, 
painful wounds, and continued irritation from foreign bodies, often act 
as the determining factor. Other painful conditions, such as herpes 
vaginalis, phimosis, pressure on the testicles, erosion of the genitals 
from irritation of urine, etc., may act in a like manner. Diseased con- 
ditions from the presence of foreign bodies in the nasal or aural cavities 
are sometimes responsible. Carious conditions of the mastoid and 
disease of the middle ear may be associated with convulsions without 
evidence of sinus or meningeal trouble. Reflex conditions in the gastro- 
intestinal tract have been given as a cause in a large class of cases. The 
eruption of the teeth is given as an important factor by many authorities. 
Henke, however, as early as 1818, called attention to the fact that teething 
was a normal process, and that it usually occurred at about the same 
time as a rather rapid development both in the brain and in the general 
economy, and that the resultant increased sensibility was more to blame 
than the local process. Most observers have supported this position, 
which also received confirmation from the work of Soltmann. These 
statements refer to simple teething processes, and not to those cases in 
which local inflammatory and other complications exist. Ulcerative 
conditions of the aural mucous membrane, enlarged tonsils, and naso- 
pharyngeal adenoids may act in a purely reflex manner or assist in lowering 
the general nutrition and nerve tone. 
Pathological factors in the stomach and intestinal tract present com- 
plicating factors besides the simple reflex action. Overloading the CONVULSIONS OF INFANCY AND CHILDHOOD 
793 
stomach of a person with a finely balanced nervous system with indi- 
gestible food may be the determining factor in a convulsive seizure not 
only in childhood, but at any period of life. The ingestion of irritating 
substances, such as alcohol, reacts in a like manner. Vogel describes 
convulsive conditions as the result of a gastritis from the ingestion of 
sulphuric acid and caustic potash; Bouchat, as the result of the various 
catarrhs of the gastro-intestinal tract; Romberg, from the presence 
of foreign bodies. Intestinal parasites of various kinds have been blamed 
for the production of convulsive conditions. The number of children 
with convulsions is relatively small in proportion to the large number 
who suffer from intestinal parasites. Reflex conditions in the genito- 
urinary tract have been mentioned. Catarrhal conditions, congenital 
anomalies, bladder and kidney stones are factors. 
Infectious Diseases.-Of the infectious fevers, pneumonia, scarlet 
fever, whooping cough, and smallpox are most frequently associated with 
convulsions. In the acute exanthemata the convulsion is most frequently 
seen as an initial symptom, whereas in pleurisy and more particularly 
whooping cough, it occurs in the course of, or more toward the end of, 
the disease. Even in the exanthemata, however, it may occur with the 
eruption, or more often as the terminal phenomenon. The initial con- 
vulsion may be quite independent of the intensity of the infection or the 
degree of fever. In some epidemics convulsions are much more frequent 
than in others. The initial convulsion occurring during the course of 
whooping cough is of much more serious import than the initial convul- 
sions of other infections. In addition to the toxic factor, the mechanical 
obstruction to the return of blood from the brain often leads to capillary 
oozing or even gross hemorrhage. In influenza a non-suppurating 
encephalitis has been found. 
As to other diseases, convulsions are sometimes present in typhoid 
fever, malaria, diphtheria, bronchitis, dysentery, cholera infantum, 
pyemia, and septicemia. In miliary tuberculosis with a meningeal 
complication, epidemic cerebrospinal meningitis, and typhoid meningitis 
the convulsions of a toxic nature should be differentiated from those of 
organic cerebral lesions. 
Drugs.-Alcohol may act directly when given in overdosage or by 
ingestion from the milk of the mother. It is unnecessary here to call 
attention to the overdosage of atropine, santonin, strychnine, etc. 
Emotional.-Fright or other strong emotional disturbance may be the 
determining factor. In infancy and early childhood convulsions are 
sometimes precipitated by«a strong emotional disturbance. That we 
are here dealing with a disturbance of metabolism as well as a sudden 
shock to the nervous system is evidenced by the production of convul- 
sions in suckling children after strong emotional disturbance in the 
mother. 
Constitutional Conditions-.-Rickets.-That this is an important factor 
in the production of convulsions in middle childhood cannot be denied. 
Too much importance has, however, been attributed to it in the con- 
vulsions of infancy. It does not, as a rule, appear until the latter half 
of the first year of life, whereas the most serious form of infantile convul-- 794 
DISEASES OF THE NERVOUS SYSTEM 
sions appears much earlier. The effect of rickets upon the general 
development and more particularly in retarding the development of the 
nervous system renders the latter susceptible to peripheral irritation and 
other complicating factors, owing to lowered nutrition. It is much 
more common in the form of laryngeal spasm than as a general con- 
vulsion. 
Congenital Heart Disease.-Convulsions not infrequently complicate 
marked cardiac weakness. These may be due to an anemic condition 
of the brain, or, more frequently, to long-continued, passive congestion 
in the cerebral veins. Toxic factors, the result of general disturbed 
metabolism, may play an important part as in kidney and liver disease. 
Thymus- Local or laryngeal spasm and generalized convulsions with 
a fatal termination have been found at autopsy to be associated with 
enlargement of the thymus. Some of the cases were associated with 
rickets. Two theories have been adduced to explain the condition; 
the toxic theory-the most probable-and the mechanical theory, which 
explains the condition as due to pressure; of the enlarged thymus upon 
the trachea, lungs, the large vessels, or the pneumogastric nerve. 
Symptoms.-The clinical picture varies greatly, dependent upon the 
intensity of the convulsion and the time of life. In all cases we may 
assume a complete loss of consciousness, although this is difficult to prove 
in early infancy. The earlier the age at which the convulsion occurs 
the more of the toxic and the less of the clonic element is present. The 
relative or complete absence of tonic movements has given rise to the 
term "inward spasm," so much in vogue by mothers, although this 
term is sometimes used to designate the minor laryngeal spasms with 
slight rigidity. The convulsions vary in intensity from a temporary 
loss of consciousness with a fleeting spasm, which may be general or 
local, to a prolonged convulsion with both tonic and clonic movements. 
In the more severe forms the onset may be sudden, without previous 
warning, or more rarely there may be prodromal symptoms, which 
consist of extreme restlessness, irritability, and minor motor phenomena, 
such as twitching of the hands, feet, eyelids, and face. In the mild 
attacks a marked pallor of the face is first noticed, the eyes become 
fixed, at times roll up in the orbit, the breathing is shallow, the body 
becomes rigid, a fewT twitchings of the face and the extremities occur, 
and the attack is terminated by a sudden deep inspiration. 
In the more severe forms the motor phenomena become more marked. 
The head is retracted, the body arched into a position of opisthotonos, 
the hands are clenched, the thumbs are buried in the palms, the elbows 
are flexed, the legs are rigidly extended, and quick spasmodic movements 
of the extremities occur. The respiration is shallow, but may be inter- 
rupted and spasmodic. The pulse is weak, hardly palpable, varies in 
frequency, and may be irregular. The contraction of the facial muscles 
causes a succession of grimaces, and may lead to violent contortions of 
the features with contractions of the eye muscles. There is some frothing 
at the mouth, the skin is clammy, but later the face reddens and the 
collection of mucus in the throat produces a coarse, rattling sound. 
The bladder and the rectum may be emptied; the urine during and CONVULSIONS OF INFANCY AND CHILDHOOD 
795 
succeeding the attack is scanty and may contain albumin. Consciousness 
is lost during the attack, which usually passes over into a stuporous 
condition, although this latter is sometimes absent. A condition of 
general relaxation with marked prostration may follow the attack or 
may be absent. In most cases the convulsions become general, even 
when they start as local manifestations. It is not infrequent, however, 
to see cases in which the convulsive movements are localized to one 
extremity, the face, or the side of the body. These local convulsions 
apparently have as deleterious an effect upon the nervous system as the 
generalized forms. Transient paralyses are sometimes seen, and have 
been ascribed to exhaustion of the cortical cells. Permanent paralysis 
in one or more extremities is sometimes observed, and is due either to 
actual hemorrhage into the cortex, or in some cases to areas of encephalitis. 
Isolated convulsions, as a rule, last for a very short time; clonic move- 
ments may, however, persist, and in some cases may be prolonged for 
half an hour or longer. In exceptionally rare cases, more particularly 
in later childhood, twitchings of certain muscles may persist as a chronic 
affection. Successive convulsions may follow each other in rapid succes- 
sion, and eighty convulsions have been noted in a single day (Holt). 
One matter ought to be kept in mind with reference to partial convul- 
sions, namely, that isolated or Jacksonian convulsions are not to be 
considered as evidence of a focal lesion in infancy and childhood. 
Death may result from a single attack; this, however, is a rare occur- 
rence, and only occurs in very young infants or in those in whom the 
vitality is very much lowered from rickets or other diseases. Death 
may, ensue from exhaustion after long or repeated convulsions. 
The paralytic phenomenon above mentioned is the most common 
sequel of a convulsive attack. Posteclamptic blindness has been reported 
in a small number of cases; Cautley found 8 under eighteen months of 
age, and 3 between two and a half and three years of age. Temporary 
aphasia was present in one of these. Ten of the eleven collected cases 
recovered their sight. In none of the cases examined was there any 
evidence of disease of the optic nerve. 
Diagnosis.-The diagnosis of a convulsion in infancy and childhood 
is not a matter for serious consideration. The differentiation into types 
is also one of relatively little importance. The problem before the 
physician is as to the cause of the condition and the possibility of its 
removal. In the consideration of this subject we have purposely omitted 
convulsions due to gross cerebral disease, but these ought to be kept 
in mind, and in the event of recurrent isolated convulsions careful exami- 
nation of the eyegrounds, of muscle balance, of reflexes, and muscular 
power ought to be made. Convulsions complicating brain diseases 
without an acute meningitis are not, as a rule, accompanied by a marked 
rise in temperature. Convulsions immediately after birth, and more 
particularly after a prolonged and difficult labor, suggest, as a rule, the 
presence of cerebral hemorrhage. Convulsions usually indicate the onset 
of some acute disease when they occur in a child over two years of age 
in association with a high temperature and clinical evidence of some acute 
infection. A reflex and toxic factor is sometimes here intermingled on 796 
DISEASES OF THE NERVOUS SYSTEM 
account of the associated conditions of the gastro-intestinal tract, and 
may to a certain extent cloud the diagnosis. 
The diagnosis of reflex convulsions from worms, phimosis, etc., becomes 
a matter of simple observation. Rickets is frequently overlooked. 
These conditions should always be kept in mind. In an investigation 
of what was thought to be epidemic cerebrospinal meningitis in a home 
for children, those affected were found to be suffering from rickets. 
One of the most difficult problems is the differentiation between 
eclampsia and epilepsy. This is a most difficult matter. While it is 
impossible to diagnose the fits of infancy as definitely epileptic, it is 
equally impossible to state that repeated eclamptic attacks in childhood 
will not persist in later life as epileptic attacks. The physician is per- 
mitted to state that in a single convulsion or group of convulsions in 
infants associated with a definite toxic or reflex factor, the presumption 
is in favor of normal nervous health in the future. If the convulsions 
are continued in later childhood, even with a reflex toxic or febrile factor, 
while the probability under proper conditions of schooling, hygiene, etc., 
may be in favor of freedom from convulsive seizures, serious stress or 
insult to the nervous system may develop and thus lead to the convulsive 
habit. The so-called idiopathic convulsions are more likely to be of a 
true epileptic nature. Convulsions during the first year are more likely to 
be of an eclamptic than of an epileptic nature. As childhood advances, 
the probability is more likely to be in favor of epilepsy; this is more 
particularly true when heredity is bad and there is evidence of a degenerate 
nervous system, a distinct aura preceding the attack, with an initial 
cry and a recovery to a relatively normal condition of health immediately 
after the attack. It is hardly necessary to call attention to the need of a 
careful examination of the urine to exclude renal disease, or of the blood 
to exclude anemia or syphilis. 
Prognosis.-As far as the individual attack is concerned, this must 
depend upon a study of the pulse and respiration, the length of the 
attack, the grade of exhaustion, and the degree of cyanosis and stupor. 
Cyanosis is an important symptom in the convulsions complicating 
disease of the respiratory tract. In nephritis the prognosis will depend 
on the severity of the renal disease. 
Treatment.-This depends upon a study of the causative factors, their 
removal, and a study of the isolated attack. 
Convulsions in infancy and childhood must always be considered as a 
dangerous emergency condition. There should be no delay in attend- 
ance, and no procrastination in active treatment. If the convulsion is 
of the simple variety, and not dependent upon passive cerebral congestion, 
the child should be immediately placed in a quiet, darkened room, and 
the body placed in a mustard bath if easily accessible, or, what is more 
usually convenient, a mustard pack. The combination of a mustard 
pack and a hot foot bath, continued until there is distinct red skin reflex, 
will often be found to be all that is necessary in the mild convulsion. 
If the convulsion does not rapidly subside, the inhalation of chloroform 
should be immediately begun. If there is a history of constipation or 
overfeeding, the lower bowel should be emptied by a simple enema, CONVULSIONS OF INFANCY AND CHILDHOOD 
797 
or in older children by sodium or magnesium sulphate. Immediately 
following the emptying of the bowel, chloral and bromide should be 
injected. Three or four grains (gm. 0.25) of chloral and from 5 to 10 
grains (gm. 0.3 to 0.6) of potassium bromide should be given to a child 
aged six months, and repeated in an hour if necessary; in older children 
a larger quantity in proportion may be administered. The injection 
should not exceed one to two fluidounces in bulk (30 to 60 cc.), should be 
injected high, and prevented from escaping by tight pressure on the 
buttocks. This can be repeated within an hour if necessary. If the 
injection is not retained, or if the convulsion should continue in spite 
of it when the chloroform is withdrawn, morphine should be given hypo- 
dermically (gr. -fa at six months, repeated in an hour if necessary, gr. 
at one year, gr. at two years, with an increase in this dosage if the 
occasion requires it). If the attack has subsided, the child should be 
kept in a quiet room for a few days, the bowels should be thoroughly 
evacuated, a light diet should be administered, and small dosage of 
bromides given for at least a week. If the convulsions show any tendency 
to recur, antipyrine may be combined with the bromides. A hot bath 
or a hot pack is contra-indicated in convulsions complicating advanced 
pulmonary disease, with extensive disease or collapse, or when the fit 
is due to syncope. Careful search should be made of all the possible 
sources of reflex irritation, and these should be immediately corrected. 
The child should be carefully watched, and its health kept up to a normal 
standard. CHAPTER XX. 
MYASTHENIA GRAVIS. PARAMYOCLONUS MULTIPLEX. 
PERIODIC PARALYSIS. 
DANIEL J. McCARTHY, M.D. 
MYASTHENIA GRAVIS. 
Synonyms.-Myasthenia gravis; pseudoparalytica; ascending bulbar 
paralysis; asthenic paralysis; pseudobulbar paralysis without an ana- 
tomical pathological basis. 
Definition.-A disease with fatigue symptoms referable to the muscular 
system, due to an exhausted condition of nervous enervation, without 
definite pathology in the nervous system, and with minor changes 
(lymphocytic infiltration) in the muscles. 
Etiology.-In the preparation of this article 180 cases were extracted, 
and two unrecorded cases added from the author's case histories. 
Sex.-There were 83 males and 96 females. The preponderance of the 
latter can be accounted for by the influence of pregnancy, the menopause, 
and the puerperal state. 
Age.-The disease is most frequent in the third decade. 
Age. Males. Females. 
Under 10 2 2 
10 to 20 8 18 
20 to 30 21 42 
30 to 40 18 20 
40 to 50 24 5 
Over 50 9 3 
The average at which women are affected is decidedly below that for 
men. As will be seen, males are more frequently affected during the fifth 
decade, women during the third. 
Occupation.-The station in life and occupation have no decided 
influence on the disease. 
Infectious Diseases.-Acute inflammatory processes occur with sufficient 
frequency immediately before the disease as to constitute, if not an impor- 
tant etiological, at least an important determining factor. If we consider 
all the possible sources of infection, including the nasopharyngeal infec- 
tions, we find in our tables 39 cases. Influenza was present ten times, 
and among the other infections, scarlet fever, typhoid fever, diphtheria, 
syphilis, herpes, puerperal and postpuerperal infections were noted. 
Nervous Diseases.-The consideration of this disease by neurologists 
as a nervous disease has led to a very careful study of possible nervous 
influences. A distinctly neurotic history, however, is present in only a 
relatively small number of cases. The history of nervous disease in some 
798 MYASTHENIA GRAVIS 
799 
member of the family was present in 35 cases. In most of these the types 
of nervous disease in parents or other members of the family were of such a 
nature that they could easily be excluded as having a distinct bearing upon 
the development of the disease. In 12 of these cases, for example, it 
was stated that there was a family history of "nervousness" without 
having any distinct statement as to the meaning of this term. The mother 
of one patient suffered with unilateral ptosis, the mother of another 
showed a defect of the ocular muscles, and migra ne was present in still 
another case. Oppenheim has paid particular attention to evidence of a 
defectively constituted nervous system. Syphilis and alcoholism may be 
considered as negligible factors, the former occurring in 2 cases, the latter 
in 1. Pregnancy and puerperal conditions were present in 11 cases. In 
2 of the writer's cases a miscarriage was regarded as the causative factor. 
In most of the recorded cases pregnancy has caused a distinctly deleterious 
influence on the course of the disease. In one recorded case, however, 
the patient felt very much improved during the pregnant state, but she 
was much worse during lactation. The menstrual period has a similar 
effect; patients are decidedly worse at this time. 
Pathology.-The changes are mainly confined to the muscles. The 
nervous system in the vast majority of cases is entirely normal. Weigert, 
in a case of myasthenia gravis, which presented lymphosarcoma of the 
thymus gland, found at autopsy an accumulation of small round cells in 
the endomysium and perimysium, and considered them as metastasis 
from the thymus tumor. In subsequent reports these accumulations 
of cells have been termed lymphorrhages, and inasmuch as they may 
occur without primary malignant disease, are looked upon as lymphoid 
structures. They may be present when the thymus rest is normal. 
Examination of a muscle of an affected case will show the muscle fibres 
to be perfectly normal. Within the area of lymphoid infiltration, however, 
a few fibres may be atrophied and lack striation. A lymphorrhage or 
lymphoid infiltration occurs as microscopic accumulations of an irregular 
shape between the fibres, and only rarely infiltrating them. They consist 
for the most part of small lymphoid cells from one to one and a half 
times the diameter of a red blood cell. This smaller type of cell resembles 
the cells of the thymus, but is usually larger than the small cells of the 
fetal thymus. A few cells of a larger type, containing a relatively larger 
amount of protoplasm and a small nucleus, are occasionally seen. Poly- 
morphonuclear leukocytes are only exceptionally present. A loose 
reticulum is present between the cells. The composite structure closely 
resembles adenoid tissue, and the term "lymphorrhage" is an appropriate 
one. Capillary hemorrhages are not infrequent in the neighborhood of 
the lymphoid accumulation. 
When these changes occur they are constant in affected muscles. Of 
the 29 autopsies reported since they were first described, there is a record 
of examination of the muscles in 16 cases, and lymphorrhages were 
present in 12 of these. Abnormalities of the thymus cannot be considered 
as a constant feature in this disease. Lymphoid deposits occur when the 
thymus is normal; they cannot, therefore, be considered, at least in all 
cases, as secondary to thymus disease. The presence of disease of the 800 
DISEASES OE THE NERVOUS SYSTEM 
thymus gland in 10 cases of the 180 reported, while not a constant factor, 
yet occurs sufficiently frequent to be considered in a causal relation to 
at least one group of these cases. Of the 10 cases recorded, simple per- 
sistent enlargement of the thymus was found in 4 cases (in 2 of these 
associated with hemorrhages and an absence of eosinophiles in the 
thymus). Lymphoidal enlargement of the thymus was found in 4 cases 
and abscess of the thymus in 2 cases. Malignant disease elsewhere in 
the body was found in 4 cases. 
Nervous System.-The examination of the central and peripheral 
nervous system has given, as a rule, negative results. The presence in a 
small number of cases of slight degenerative changes in the ganglion 
cells and capillary hemorrhages in the cerebral gray matter have been 
considered as agonal. Lymphorrhages in the posterior root ganglia in 
one case may be considered as accidental as regards the location in the 
nervous system, and in no sense concerned in the production of the 
disease. From both a pathological and clinical standpoint the presence 
of lymphorrhages in otherwise normal muscles presents a valuable means 
of diagnosis in a suspected case. After extensive control work they 
have been found in other conditions only on one occasion, in a case of 
amyotrophic lateral sclerosis. In this disease there is, however, an asso- 
ciated degeneration of the muscle fibres, with a degeneration of the 
lateral tracts and gray matter of the spinal cord. The more recent work 
supports the statement of Buzzard, that his "experience affords reasonable 
grounds that lymphorrhages are constantly present in the muscles and 
other organs in cases of myasthenia gravis." Their detection, however, 
entails a most thorough and diligent search of large numbers of sections. 
Much speculation and relatively little accurate knowledge as to the 
cause of the disease is to be found in the literature. The consensus of 
opinion appears to be that the disease is due to some toxic agent. Weich- 
ardt has shown that physical fatigue in the normal individual is caused 
by the production of a toxin in the muscles. This toxin, however, cannot 
be demonstrated in the circulatory blood. If the toxin which he has 
isolated be injected into animals, an antitoxin is produced which is 
found in the circulating blood and acts in vivo and in vitro. He has 
also shown that the toxin of excessive fatigue may cause death. It would 
appear that the preservation of muscle excitability is dependent upon a 
period of rest either for the removal of the toxin from the muscle, or the 
production of an antitoxin in the blood which counteracts the fatigue 
toxin. Inasmuch as this toxin cannot be demonstrated in the circulating 
blood, the method of its removal from the muscle is not altogether clear. 
Link believes that the lymphorrhages in the muscles interfere with the 
lymphatic circulation, and thus prevent the removal of the fatigue 
products. This theory assumes the presence of lymphorrhages in the 
muscles early in the disease. Another view is that the toxin is not due to 
a disturbance of metabolism, but is of an exogenous nature, the result 
of some bacterial infection. The supporters of this view point to the 
presence of an infectious process in the history of a large percentage of 
cases, and to the fact that the infections are such, scarlet fever, typhoid 
fever, influenza, etc., as are not infrequently followed by a toxic neuritis. 3/ YASTHENIA GRA VIS 
801 
The presence of the myasthenic reaction is the best evidence that the 
toxic action affecting the nervous system is not restricted to the upper 
motor neuron. The fatigue exhaustion produced by the faradic current 
and its similarity to that produced by voluntary muscle exercise would 
point to the muscular or intramuscular neuromechanism as the seat of 
derangement. It would appear from the experiments of Buzzard that the 
myasthenia is due to an exhaustion of the nerve end organs. In a well- 
marked case of myasthenia gravis, a moderate galvanic current was 
applied to the biceps muscles and a contraction obtained. The muscle 
was then faradized until it gave no response to a strong stimulus. Then 
it was tired out by making the patient flex the elbow against resistance 
exerted until all power of flexion was lost. On applying the same strength 
of galvanic current as used at first, an excellent contraction was obtained. 
Finally, on again applying the faradic current the muscle was found to be 
still unresponsive. The peculiar muscular phenomena in this disease, 
such as are demonstrated by this test, can perhaps be best explained by 
the work of Botazzi, confirmed later by lotezko. They hold that in the 
muscles there are two contractible substances, a fibrillar and protoplasmic, 
giving different electrical reactions. The fibrillar substance gives a 
quick, lightning-like response, such as is produced on the normal muscle 
by the make or break of the galvanic current, whereas the reaction of the 
protoplasmic substance is slow and such as is seen in a degenerated muscle. 
Both substances react to the galvanic current, but the protoplasmic 
substance requires a much stronger stimulus than the fibrillar. A selective 
poison acting more on the protoplasmic and reducing its excitability, 
would explain the exhaustion after a faradic or voluntary fatigue, whereas 
the muscle would still react to the galvanic current by the stimulation 
of the fibrillar substance. This theory relieves the peripheral nervous 
system of much of the responsibility that has been placed upon it in the 
production of myasthenia gravis. 
Assuming the disease to be of toxic origin, a very natural query is as to 
its nature. The view above stated as to the exogenous origin as the result 
of bacterial invasion has little to support it other than the presence of an 
infectious process at the onset in thirty-nine cases, and an analogous 
condition as the result of a toxin in diphtheria and influenza. 
The theory of an endogenous toxin, originally proposed by Weigert, 
has been supported by investigations in the pathology of the disease. 
He held that in Graves' disease and Addison's disease a definite internal 
secretion was necessary to the maintenance of bodily health; persistence 
of a secretion from a gland (the thymus) whose function in a normal 
individual should be in abeyance likewise gives rise to metabolic intoxica- 
tion manifested by myasthenic symptoms. This theory, of course, 
applies to the group of cases in which the thymus was found diseased. 
It would not apply to the cases in which the thymus rest is found in a 
normal condition. A large number of cases in which disease of the 
thymus is not present may be explained (assuming that the thymus is a 
lymphatic structure) by possible lesions of the lymphatic system else- 
where than in the thymus gland. This is supported to a certain extent 
by the presence of lymphoid collections (lymphorrhages) in the heart, 802 
DISEASES OF THE NERVOUS SYSTEM 
liver, and kidney. It is of interest to note that an identical syndrome to 
that of myasthenia gravis is seen in cases of myeloma in which the bone 
marrow is extensively involved (Senator). 
As the lymphorrhages are not present in the affected muscles in a 
sufficient number of cases to account for the disturbance of function, 
we are forced to regard them as the physical manifestations of some under- 
lying intoxication of unknown origin. 
Symptoms.-These are almost entirely confined to the muscular 
system. After a single muscular effort the muscles show decided fatigue, 
and, if the effort is continued, paresis, or even complete paralysis, results. 
After a period of rest the muscle returns to its normal condition. After 
a night's sleep the affected muscles may present a relatively normal 
condition; but, with the fatigue incidental to a day's routine, a moderate 
or even a marked degree of weakness is presented. This condition is 
most marked in muscles such as the elevators of the eyelids and the 
muscles of the jaws, which are maintained in more or less constant action 
during waking hours. The voluntary muscles throughout the entire 
body may be affected. The muscles supplied by the bulb, however, are 
those which are most frequently and earliest involved. The symptoms 
may be restricted to this group of muscles throughout the course. 
Bulbar Muscles.-The disease, as a rule, is of slow onset, and begins with 
an involvement of the levator palpebne superioris. The lids show a 
tendency to droop as the day progresses, until at nightfall the eye may be 
entirely closed. While ptosis, as a rule, is bilateral, it is not unusual to 
find it more marked on one side. Until the disease is well advanced, the 
drooping of the lids is entirely or almost entirely absent after a period of 
prolonged rest. In the advanced cases a well-developed or even complete 
ptosis may be present even after sleep. The other eye muscles, both 
intrinsic and extrinsic, the orbicularis palpebrarum, and the occipito- 
frontalis may also be affected. 
Diplopia due to weakness of the external ocular muscles is a frequent 
symptom. It was noted as an initial symptom in thirty-one cases. The 
type of diplopia presented is fairly characteristic. In successive examina- 
tions, even at short intervals, there is a marked variation in the position of 
the images. This is due to the varying type of palsy as a result of the 
exhaustion of the different eye muscles during the progress of the tests. 
The weakness of the eye muscles and the relative loss of muscular balance 
give rise to irregular, jerky movements when the eyes are deliberately 
moved in one or another direction during examination. This condition has 
been mistaken for a true nystagmus. The convergent movement of the 
eyes, although at first fairly good, is easily exhausted. As a result of this, 
reading becomes impossible on account of the blurring of the print. The 
inequality of the pupils and a loss of reaction of the pupils to light has been 
noted in a few cases. A sluggish reaction of the pupils to light is of more 
frequent occurrence. The sphincter iridis does not show the fatigue 
reaction to repeated light stimuli. Hippus was recorded in one case but 
no significance can be attached to it. The involvement of the upper 
muscles of the face, the occipitofrontalis and orbicularis palpebrarum, in 
association with the ptosis gives rise to a fairly characteristic position of the MYASTHENIA GRAVIS 
803 
head. An isolated bilateral ptosis is partially or completely compensated 
for by contraction of the occipitofrontalis muscles. When this latter 
group of muscles is also affected, the advancing ptosis can only be com- 
pensated for by throwing back the head in an attempt to see under the 
lids. When the muscles of the neck become involved, it is impossible to 
maintain this backward position of the head, and it then not infrequently 
takes a forward paralytic position, resting on the chest. 
Other ocular symptoms have occasionally been noted. Gunn, in one of 
Buzzard's cases, noted an associated dilatation of the pupils with elevation 
of the lids under emotional stress, although the patient was not able to 
Fig. 45 
Fig. 46 
Myasthenia gravis. (Case of Spiller and 
Buckman.) The ptosis of the right eye is not 
so great as that of the left. The photograph was 
taken one or two minutes after the glasses were 
removed. At times he is able to open both eyes 
fully. 
The eyeballs are almost completely covered. 
The photograph was taken after the glasses had 
been removed three or four minutes. 
deliberately elevate the lids by a voluntary effort, von Graefe's symptom 
has been reported in several cases, and Stelwag's in an isolated case. Both 
these symptoms are not infrequent in tuberculosis and in exacerbations 
of chronic alcoholism. They may be considered as significant of an under- 
lying intoxication. 
Involvement of the muscles of the lower facial distribution results in a 
loss of expression, an inability to pucker up the lips or whistle, a drooping 
of the angles of the mouth, drooling, and the so-called "nasal smile." 
The muscles of mastication and deglutition, including the tongue, may 
be affected relatively early, or the involvement may follow in order of 
regular progression after the eye and face muscles. It was present at the 804 
DISEASES OF THE NERVOUS SYSTEM 
onset of the disease, in association with eye symptoms, in four cases. The 
power of mastication may be slowly lost, as during the progress of a meal 
or it may survive only the first attempt at chewing the food. The loss of 
power of the tongue may interfere with the transmission of the bolus of 
food to the pharynx. When the muscles of the palate are involved, 
liquid food may regurgitate through the nose. In advanced cases of 
myasthenia of this group, weakness becomes continuous throughout the 
entire day. When the power of deglutition is retained, the power of 
mastication can be assisted by the use of the hand. Clonic movements 
of the muscles of the jaws are occasionally noted. The difficulty in deg- 
lutition may become so serious as to necessitate the use of the stomach 
tube or alimentation by rectum. Choking attacks are not infrequent, 
and may be a cause of death either from interference with the respiration 
or from exhaustion. 
The laryngeal muscles show fatigue manifestations similar to those 
noted in the extra-ocular muscles. The laryngoscopic picture is not at all 
constant, and varies from time to time. The pharyngeal reflexes are 
usually absent. Anesthesia of the pharynx and larynx has been noted 
in two cases. Fatigue manifestations in the tongue are associated with 
fibrillary tremors. The speech is usually indistinct, and when fatigued 
has the nasal, mumbled character of true bulbar paralysis. In moderately 
advanced cases there is a paretic element which is not seen in true bulbar 
cases. The speech fatigue is accentuated in cases with involvement of 
the muscles of respiration. 
Involvement of the chest muscles becomes a very serious symptom. 
The respiratory excursion is very markedly diminished. Sudden attacks 
of dyspnoea often terminate the life of the patient. They occur usually 
toward the end of the day, or after exertion. It is not, however, always a 
fatigue dyspnoea, and may occur suddenly without any apparent cause. 
Strumpell considered a paralytic condition of the tongue to be an import- 
ant factor in one of his own cases, and relieved the condition by pulling 
the tongue forward. Pneumonic infections are not infrequently a cause 
of death. Cardiac distress may be present in association with or inde- 
pendent of dyspnoea. 
The other muscles of the trunk may become so weakened as the disease 
progresses as to render the patient unable to sit up in bed, or the 
terminal paralysis may render the patient completely helpless. 
Extremities.-These may become involved early, may remain free 
throughout the course of the disease, or be involved in the general 
paralytic phenomenon. The muscles nearest the trunk are the earliest 
affected, although the reverse may be true. When the hand muscles are 
involved, the handwriting shows the characteristic fatigue and a rapid 
loss of regularity in letters as the writing progresses. In the lower 
extremities the fatigue manifestations are usually noticed first in the 
iliopsoas and quadriceps muscles. Giving way of the legs may be a 
relatively early symptom. While the patient at first is able to walk 
fairly long distances, this power is rapidly impaired, until finally there is 
inability to walk at all. In relatively early cases the fatigue manifesta- 
tions disappear after a short period of rest. MYASTHENIA GRAVIS 
805 
Myasthenic Reaction.-The electrical examination of the muscles gives 
important data. If a rapidly intermittent current be applied to the 
affected muscles, there is at first a good, strong contraction; this con- 
traction, however, is not maintained as in a normal muscle, but rapidly 
decreases until there is no response. If the current is intermitted for a 
short period of time (from one to two minutes), a strong response is 
again obtained, which disappears, however, more rapidly than the first. 
This test is called the 'myasthenic reaction. The rapidity with which 
the muscle becomes exhausted depends to a degree on the myasthenia 
present in the affected muscle. In advanced cases, the exhaustion may be 
complete within twenty to thirty seconds. In early or mild cases, only a 
relative degree of exhaustion may be obtained, even with strong currents. 
The reaction is not necessarily constant, and may be different from time 
to time. It is influenced by the degree of fatigue in the muscles examined. 
It may be present in muscles supplied by one branch of a nerve and absent 
in other muscles supplied by the same nerve (Goldflam). After exhaustion 
of the muscles by the faradic current, a fairly good contraction may 
still be maintained by voluntary effort. The return of irritability after 
exhaustion is much more rapid than in normal muscle exhausted by 
fatigue (Murri). 
The reaction to the galvanic current shows only a relatively slight 
degree of diminution of contraction. The exhaustion of the muscle is 
not here produced as with the use of the faradic current. Atrophy of the 
tongue was found in five cases. In ten cases a relatively slight degree 
of atrophy was noted in the affected muscles. 
Reflexes.-The reflexes of the extremities, as a rule, are exaggerated. 
Exhaustion of the quadriceps muscle either by fatigue or by faradization 
has no effect on the knee-jerk. Ankle clonus is never present, and the 
plantar reflex was uniformly of a flexor nature. In advanced cases, 
the jaw-jerk is usually absent. The skin reflexes are normal. 
Negative Symptoms.-There is no disturbance of sensation. After 
exercise a sensation of stiffness or aching in the limbs may be present. 
This sensation is also present in advanced cases due to inability to move 
the affected parts. In rare instances the muscles may be sensitive to deep 
pressure. Buzzard reports a case of anesthesia of the trunk, which he 
ascribes to lymphorrhages in the posterior spinal ganglia. 
There is, as a rule, no atrophy or fibrillary tremors of the affected 
muscles (exceptions noted above). There are no trophic changes in 
the bones or skin. The visceral and rectal sphincters are never 
involved. 
Mental depressive states have been noted in two cases. 
Urine.-Spriggs found that in a case of myasthenia gravis the creatinin 
output, both absolutely and relatively to the total nitrogen, was definitely 
diminished, while that of uric acid remained normal. 
Diagnosis.-This should never be a difficult matter if the regular 
symptoms are kept in mind. A combination of motor symptoms, pro- 
gressive weakness and rapid fatigue of the voluntary muscles after 
exertion, and particularly the muscles supplied by the bulb, the presence 
of the myasthenic reaction and the absence of wasting, fibrillary tremor 806 
DISEASES OF THE NERVOUS SYSTEM 
or sensory disturbance, with a retention of bladder and rectal control; 
of slow, more or less interrupted onset, running a variable course with a 
tendency to improvement and relapse, constitutes a characteristic 
symptom complex which cannot well be mistaken. The conditions from 
which it must be distinguished are true bulbar palsy, pseudobulbar palsy, 
diphtheritic paralysis, poliencephalitis superior, the muscular dystrophies, 
hysteria, and general asthenia. 
True bulbar paralysis affects the lower distribution of the face, the 
tongue and the muscles of deglutition, by preference; the upper facial 
distribution and the ocular muscle are rarely affected, and then only in 
the advanced stages of the disease. There is marked atrophy, fibrillary 
tremors, and the absence of the myasthenic reaction. In pseudobulbar 
paralysis, the upper facial distribution is not affected, and the history 
of repeated apoplectic attacks will make the diagnosis clear. 
Diphtheritic paralysis is of more rapid onset, and there is a history of 
the throat'infection. The electrical reactions in the extremities will 
easily differentiate this from myasthenia gravis. 
In poliencephalitis superior the onset is sudden. The headache, fulness 
in the head, and other symptoms of an inflammatory type are associated 
with constant paralytic phenomena of the ocular muscles, which bear no 
relation to fatigue. 
The facio-scapulo-humeral type of muscular dystrophy may bear a 
striking superficial resemblance, but the presence of muscular dystrophy 
in other members of the same family, the rapid wasting, the relative 
freedom of the muscles of deglutition, and absence of the myasthenic 
reaction should make the diagnosis easy. 
In hysteria associated with neurasthenia the fatigue symptoms may be 
so marked as to bear a striking resemblance to myasthenia gravis. Sooner 
or later transient sensory disturbances, reversal of the color fields, head- 
aches, and other painful phenomena, all of which may be influenced by 
suggestion, will lead to the diagnosis. 
Prognosis.-This is unfavorable in the majority of cases. The disease 
must always be considered as a serious affection, with a possible fatal 
termination. Of the 180 collected cases 72 proved fatal. These statistics 
are, to a certain extent, misleading on account of the tendency in case 
reports to consider only fatal cases. Of the 5 cases which have come* 
under the writer's observation, only the 2 which resulted fatally were 
reported. On the other hand, there is a disposition to report cases 
after a short observation without waiting a reasonable time to follow the 
course of the disease. In the fatal cases, the duration varied from one 
and one-half to two years. The disease may run an acute course, and in 
one case terminated in fourteen days. In another recorded case the 
patient lived fifteen years. It is impossible to state the percentage of 
cases that go on to permanent recovery. It is probable, however, that a 
permanent cure often takes place. Respiratory failure with attacks 
of dyspnoea should be considered a grave symptom. Sudden death in 
such cases is not of infrequent occurrence. The disease varies markedly 
in its course and in its symptoms. Even in apparently favorable cases 
unaccountable relapses are likely to occur. PARAMYOCLONUS MULTIPLEX 
807 
Treatment.-All possible infectious or toxic causes should be, as far as 
possible, removed. In puerperal cases, septic or toxic processes in the 
genito-urinary apparatus should receive particular attention. The 
advisability of the removal of tumors must depend upon the particular 
case and the location of the tumor. 
A full or modified form of rest treatment is indicated. The whole 
therapeutic regime should be so directed as to eliminate all possible 
muscular fatigue. The diet should be so arranged as to secure the best 
nutrition with the least effort in mastication, and in severe cases a full 
milk diet is indicated. In the earlier cases food should be so prepared 
as to avoid the necessity of mastication fatigue. If the use of the stomach 
tube should at any time be deemed necessary, either for purposes of 
diagnosis or alimentation, the greatest care should be used. Death 
may result from the fatigue and excitement incidental to the use of the 
tube, or as the result of attacks of choking. Unless the tube is taken 
easily and without excitement or gagging, its use should be avoided. 
Care should be taken to properly protect the patient from cold. In 
those cases in which the symptoms are aggravated or induced by cold 
weather, a warm, equable climate should be recommended. Electricity 
should only be used for purposes of diagnosis, and even then only with 
the greatest care. Farad ism as a therapeutic agent does harm, and 
galvanism does no good. When massage is used, it should be given only 
for a very short period of time and the effects carefully noted. It is 
likely to do more harm than good. The glandular extracts (suprarenal, 
thyroid, and thymus) have been tried without appreciable results. 
General tonics would appear to be indicated, and in conjunction with 
hygienic measures have given good results. Arsenic in the form of 
Fowler's solution, the iron preparations, and strychnine in small doses 
have been recommended. Strychnine has been used in massive doses 
without distinct benefit. 
PARAMYOCLONUS MULTIPLEX. 
This is an affection first described by Friedreich in 1891, and char- 
acterized by clonic, lightning-like contractions occurring either constantly 
or in paroxysms, affecting at times all the muscles of the body with the 
exception of the eye muscles, but more frequently the muscles of the 
lower extremities. 
Etiology.-The cases reported have been chiefly in males. Some 
emotional disturbance, such as fright, is often the determining factor. In 
pure cases hyperirritability of the nervous system is often present. 
Tuberculosis has been reported as present in certain cases. 
Pathology.-Of the three cases which have come to autopsy, the case 
of Hunt showed hypertrophy of the muscle elements. Friedreich stated 
that the condition was due to an increased irritability of the gray matter 
of the spinal cord with an irregular discharge of motor impulses. That the 
disease is one of the lower motor neurons or of the muscles is accepted. 
Irritation of the motor cortex does not give isolated muscular movements. 
Localized contractions of muscles or individual fibres of muscles have 808 
DISEASES OF THE NERVOUS SYSTEM 
always been considered as having a localization in the gray horns of the 
spinal cord. We would, therefore, conclude, in view of negative findings 
in the nervous system, that the disease is the result of some disturbance 
of function of the inferior neuromuscular mechanism. 
Symptoms.-All of the muscles of the body may be affected. The 
muscles of the extremities, and especially of the more proximal portion, are 
those most usually affected, in the upper extremities the biceps, triceps, 
and supinator longus, and in the lower extremities the internal vastus, 
external vastus, the rectus, and the adductors. The muscles affected 
display the greatest activity, the individual muscles in use springing 
forward in contraction with the greatest rapidity, as if suddenly irritated 
by galvanic shock. These contractures are not associated, as a rule, 
with fibrillary tremor. They do not usually produce any motor response 
in the extremities. Occasionally a barely perceptible involvement of 
the arm may be the result of the more violent contractions. The intensity 
and rapidity of the contractions may be increased by mechanical irritation 
of the skin, mechanical irritation of the muscles, exposure to cold, and by 
excitement. They are usually decreased by voluntary motion, although 
the contrary may be true; they usually disappear during sleep. Occasion- 
ally, as in Friedreich's case, the patient may be frightened out of sleep 
by a sudden very painful crampy movement of both legs, resulting in a 
sudden jerking of these members against the abdomen. The electrical 
irritation of the muscles is normal and the reaction of the muscles to 
mechanical irritation is not increased. There is no disturbance of 
sensation, no atrophy, and no cerebral, spinal, bladder, or rectal 
symptoms. 
Diagnosis.-The symptomatic picture of a well-defined case of para- 
myoclonus multiplex is distinctive. This term has been used so frequently, 
however, to designate other forms of myospasm, that considerable 
difficulty has been experienced in limiting the disease as a clinical entity. 
There is no question that most of the cases reported in the literature 
should not be considered as such in the strict sense of the term. It should 
be remembered that a myokymia or contraction of individual groups of 
muscular fibrillae is not uncommonly present in neurasthenia and other 
functional nervous disorders. The fibrillary tremor of wasting muscles 
need only be mentioned in this connection. 
Dana, in 1903, differentiated the cases recorded as paramyoclonus 
multiplex into the following groups: (1) Paramyoclonus multiplex of 
Friedreich; (2) myoclonus of the functional or hysterical type; (3) 
myoclonia of the convulsive tic type; (4) myoclonia of the degenerative 
chorea or familial, or myoclonia epilepsy type; and (5) myoclonia of the 
infectious and symptomatic choreas. 
There should be no difficulty from a careful study of the case in ex- 
cluding cases of the second group. The contractures are not strictly 
fibrillar, but are of the coarse, irregular, clonic type, associated with 
minor or more extensive movement of the extremities. There are other 
evidences of hysteria usually associated, and this is more particularly true 
in the early stage. The muscular phenomena are not constant, and the 
frequency is influenced by excitement, excessive attention, etc. PERIODIC PARALYSIS 
809 
The cases belonging to the third group (convulsive tic type) are easily 
differentiated as representing a disturbance of cortical origin as differen- 
tiated from the spinal type seen in paramyoclonus multiplex. All move- 
ments belong to the purposive group of gross movements. There are no 
fine, fibrillar tremors of the muscles, but a quick, jerky contraction of the 
muscle groups, such as blinking the eyes, screwing up the face, twisting 
the head, shrugging the shoulders, etc. In Tourette's disease (maladie 
de tic convulsif) this purposive type of muscular spasm may be wide- 
spread and affect the muscles over the entire body. 
The myoclonus epilepsy of the fourth group is easily differentiated 
from paramyoclonus multiplex by the presence of epileptic attacks. It 
is a manifestation of excessive functional disturbance of the cortex. The 
movements do not resemble paramyoclonus multiplex so much as they 
do those seen in Sydenham's chorea. They are, however, prone to be 
rhythmical and associated with some fibrillary tremors. From the infec- 
tious chorea group, this disease is easily differentiated by the gross 
purposeless character of the movements in the latter disease. From the 
electrical chorea of Dubini it is distinguished by the presence of the 
severity and fatal character of the latter affection. In Dubini's disease 
the rapid rhythmic movements are very violent, as if produced by an 
electric shock, and may be associated with fever, paralytic symptoms, 
epileptic convulsions, and atrophy and wasting of the muscles. 
Prognosis.-This must be considered unfavorable in true cases of 
paramyoclonus multiplex. The cases reported in which such startling 
curative results were obtained by the use of electricity are usually looked 
upon as cases of hysteria. The disease usually lasts a long period of 
time, unless, as not infrequently happens, the life of the patient is termin- 
ated by some intercurrent affection such as pulmonary tuberculosis. 
Treatment.-Careful attention should be paid to associated visceral 
disease, more particularly of the lungs. If the patient is under weight, 
a modified rest treatment with overfeeding is indicated. Careful attention 
should be paid to keeping the gastro-intestinal tract in good condition. 
The urine and stools should be carefully watched in order to insure proper 
elimination through these channels. Flushing of the system by the use of 
large quantities of water in the dietary and by the occasional use of 
Epsom salts will be found in some cases to be of assistance. The bromides, 
chloral, and the valerianates have been used for their calming influence. 
Arsenic is of value in some cases. Thyroid extract has been used in this 
disease, but its value has not been established. Various forms of electrical 
treatment have been lauded as curative agents. The galvanic current, 
the static spark and breeze have been reported as producing valuable 
results. They at least offer a helpful means of eliminating the excess of 
functional symptoms present in most of these patients. 
PERIODIC PARALYSIS. FAMILY PERIODIC PARALYSIS. 
Definition.-A flaccid paralysis affecting the muscles of the trunk 
and the extremities, temporary in character and recurring at more or less 
regular intervals. 810 
DISEASES OF THE NERVOUS SYSTEM 
Etiology.-Judging from the list of cases reported in the literature, 
this is a rare disease. Most of the cases reported have been in family 
groups; 5 cases have been reported by Cousot, 22 by Goldflam, 2 by 
Hirsch,1 and 11 by Taylor. Mitchell2 reports one group in which 4 and 
another in which 5 were affected. Holtzapple3 reports 17 cases. 
That heredity is an important etiological factor in these family groups, 
there is no question. In Holtzapple's series, cases in four generations in 
the same family were studied. While a neurotic basis for the disease is 
generally denied, migraine was present in 10 of the 17 cases reported. 
Five had attacks of paralysis and headache. Thirteen other members 
of the family suffered from headache. In some of the cases the headaches 
preceded the attack, developed in early childhood, and disappeared with 
the development of the paralytic phenomena. In other cases the head- 
aches continued, alternating with attacks of paralysis. This, however, 
is the only group of cases in which migraine is of etiological importance. 
The disease may develop as early as the fifth year, but, on the other 
hand, may not appear until after thirty. 
Transmission may occur either through the male or female. Individual 
members of the family may be entirely free. These may, however, transmit 
the disease, the affection in this manner skipping a generation. In Holtz- 
apple's cases, migrainous headaches were present in members of the family 
who did not have the disease. In this very interesting family the disease 
seems to have started from the father of the first generation, who suffered 
from periodic sick headaches. The statement of Taylor that these cases 
have occurred in families of unusual nervous stability and that in no case 
do we find evidence of a degenerative family is not borne out. The 
attacks in some of the cases were most frequent during the cold months. 
This is of especial interest in connection with the article of Rich,4 who 
described a condition of tonic spasm of the face muscles in five members 
of his own family, due to exposure to cold or dampness, in one case there 
being a complete general paralysis with the exception of the tongue, as 
the result of sleeping in moist underclothing. While the paralysis, being 
of the nature of a spasm, is different from the flaccid palsy of periodic 
paralysis, the influence of cold in its production is of interest. Sinkler5 
also reported a sporadic case of recurrent facial paralysis extending 
over a period of ten years, without such etiological factor. Muscular 
exertion, lack of exercise, indiscretions in diet, exposure to a draft, 
menstruation, constipation, nervous or mental fatigue, worry, and 
excitement have been given as conditions which precipitate an attack. 
Pathology.-Two of the patients in Holtzapple's series have come to 
autopsy but no lesion which could be associated with the disease was 
found (Winternitz). Small portions of muscles have been excised and 
studied by Goldflam and Oppenheim. The former found a waxy degenera- 
tion to which he attributes no importance. The latter found an increase 
of the diameter of the individual muscle fibres, hypertrophy of the fibres, 
rarefaction of the primitive fibrilire with vacuole formation. To these 
1 Deut. med. Woch., 1894, No. 32, p. 646. 2 Brain, 1902, xxv, No. 1, p. 109. 
3 Jour. Am. Med. Assn., 1905, xlv, 1224. 4 Medical News, Phila., 1894, xv, 210. 
5 Jour. Nerv. and Mcnt. Dis., 1898, p. 744. PERIODIC PA RA L YSIS 
811 
changes Goldflam attributes qualitative, electrical, reactive changes which 
he alone found in the free intervals between the attacks. These changes 
have a special significance when studied with the cases of Bernhardt, 
which showed from early childhood a persistent and constant loss of 
power in certain muscle groups, separate and apart from the periodic 
attacks which develop later. Both of these authors would place the 
disease on the basis of the muscular dystrophies. 
In the absence of any such condition in the other cases reported, the 
prevailing idea is that we are here dealing with an auto-infection of 
unknown origin, associated with a lowered condition of metabolism and 
induced by excessive muscular activity, followed by a period of rest. 
Extensive investigation of the urine and the blood has not given much 
information as to the nature of this intoxication. Goldflam and Taylor 
reported a lymphocytosis with a moderate eosinophilia. In Mitchell's case 
a study of the toxic effect of the blood serum by intravenous, infraspi- 
nous, intrameningeal, and intraneural injection, gave inconclusive results. 
The same was true as to the alkalinity of the blood, and examination of 
the urine for acetone and diacetic and lactic acids. A low ammonia con- 
tent of the urine was found with an extremely low creatin elimination, 
especially accentuated immediately preceding and during the paralytic 
attack, with a sudden rise to the normal immediately following the attack. 
From this the authors conclude that "the symptoms in this case are not 
solely due to the retention of creatinin or creatin, but that the attacks 
are due to metabolic disturbance, and that this disturbance may be 
situated chiefly or primarily, perhaps entirely, in the muscles themselves." 
Holtzapple found a diminished excretion of urea, and that the worst 
sufferers were those who showed the most marked diminution in the 
average daily output of urea. One of his patients showed a very marked 
increase in the elimination of urinary solids and urea during the attack. 
Schlesinger in an isolated case found acetonuria in the majority of the 
attacks, and occasionally albuminuria and hyaline casts. 
A study of the reported cases suggests a close analogy of the muscular 
phenomena of the disease and those of myasthenia gravis. In some cases 
of myasthenia gravis, attacks of paralysis lasting a few days, resembling 
those of periodic paralysis, are seen.1 
The rapid exhaustion of the muscular power and the failure of the 
muscles to respond to the faradic current are suggestive. A careful study 
of the thymus gland, which is found so frequently diseased in myas- 
thenia gravis, has not been made. 
A study of the series reported by Holtzapple bring this whole group of 
cases into close analogy with periodical ocular paralysis associated with 
migraine. This series of cases, in which migraine plays such a prominent 
part and which is not found in any of the other family groups, may be 
considered to be a connecting link between the periodic family palsies 
and the periodic oculomotor palsies. In the latter group, a partial or 
complete oculomotor paralysis of one side, lasting a few days, a week, or 
longer, is associated with attacks of migraine with a tendency to recur- 
1 Collins. Vide Jour. Nerv. and Men. Dis., 1898, p. 745. 812 
DISEASES OF THE NERVOUS SYSTEM 
rence at fairly regular intervals. There is a partial or complete oculo- 
motor paralysis affecting one or all the muscles supplied by the third 
nerve. In cases of this condition which have come to autopsy, a disease 
process of the third nerve has been found, i. e., in one case a plastic exu- 
date, and in three others tumor formation. Stryzeminski differentiates 
a functional and an organic form. Oppenheim agrees with Charcot upon 
a vasomotor basis for the oculomotor palsy, and considers it to be due 
either to a vascular cramp giving rise to an ischemic form of paralysis, 
or a paralysis of the vessel nerves leading to compression by dilatation 
of the vessels. This would explain the temporary paralysis without 
damage to the nerve and a terminal degeneration when the condition 
is frequently repeated. 
Holtzapple, following Westphal, explains his cases on the ground of a 
"vasomotor neurosis affecting the blood supply to the anterior horns 
which are supplied almost wholly by the anterior spinal artery," and the 
terminal paralyses "to a slow degeneration of the anterior horns due to 
frequent disturbance of nutrition and the atrophy of the muscles due to 
involvement of the trophic cells." 
While the association of this one extensive group of cases with migraine 
points strongly to a central vascular causation, the muscular phenomena 
noted during the period of paralysis do not support this view. This 
would bring the disease within the grouping of poliomyelitis, and to a 
certain extent confirm the opinion of Dana of a recurrent poliomyelitis. 
Isolated cases in childhood of a flaccid paralysis, with loss of reflexes which 
last only a few days and then disappear, are not infrequently seen and 
are usually considered as an abortive or evanescent type of poliomyelitis. 
The objection to this view and to the other theories of a central nature 
of the affection is the complete loss of reaction of the muscles during the 
attack of paralysis to galvanic and faradic stimulation. This would 
indicate an involvement of the terminal distribution of the nerves in the 
muscles and the muscle fibres themselves. As a matter of fact, the loss 
of the myotactic as differentiated from the nerve irritability, together 
with the changes in the metabolism, point to the muscles rather than to 
the nervous mechanism. Oppenheim would explain this on the grounds 
of a peripheral disturbance in the circulation. 
Symptoms.-The clinical picture is so striking as to be almost dramatic. 
The patient, as a rule, retires to bed feeling perfectly well, or with slight 
prodromal symptoms, and awakens during the night with a flaccid motor 
paralysis which may involve all the voluntary muscles except those of 
the face, eyes, tongue, the organs of speech and of deglutition. This 
paralysis may last from a few hours to a few days and disappears gradu- 
ally or rapidly. In the most severe cases the paralysis is complete and 
involves the muscles of the trunk and of all four extremities. The muscles 
of mastication and the tongue muscles may be partially involved. While 
there is no paralysis of respiration, and w'hile the respiratory rate, as a 
rule, remains within the normal range, there is often an inability to take a 
deep breath, and the breathing may be somewhat embarrassed, due to 
an accumulation of mucus in the throat and bronchial tubes. Bladder 
and rectal functions are retained. PERIODIC PARALYSIS 
813 
The paralysis is flaccid; the deep and superficial reflexes of the par- 
alyzed muscles are lost. In one of Holtzapple's patients prodromal 
increase of reflexes was recorded. There are no disturbances of objective 
sensation. Subjective sensory phenomena are sometimes present; 
formication, numbness, a sensation of heaviness may precede and con- 
tinue during the attack. A feeling of soreness of the muscles is not 
infrequently complained of after the attack passes off. 
In Holtzapple's patients, a bulimia commonly preceded the attack, 
and if the appetite was satisfied, and more particularly by an indulgence 
in rich or heavy food, an attack was almost certain to follow during 
the night. Other persons in the same family were not so affected. While 
prodromal symptoms are not uncommon during the day, the paralytic 
phenomenon develops, as a rule, during sleep. The affected individual 
awakens during the night to find himself absolutely helpless as a result 
of paralysis affecting all four extremities, the trunk, and the neck. Sleep 
may not, however, be interfered with, the parlysis first manifesting itself 
on awakening in the morning. In other cases the patient awakens before 
the attack is fully developed, and many hours may elapse before 
the paralysis is complete. In some cases the paralysis may develop 
during the waking hours. In these cases incoordination of the hands and 
even unconsciousness may usher in an attack. After a period of paralysis 
varying from a few hours to a week, motor power gradually returns 
and with it a return of the reflexes and of myotactic irritability. 
While there is no paralysis of the bladder or rectum in the strict sense of 
the term, it is the exception to have urine voided or the bowels moved 
during the attack. This is probably due to a diminution of secretion and 
excretion. This is borne out by the examination of the stomach contents 
in a case of Edsall's. "Even starch digestion was not proceeding and 
there was a total anacidity, showing the whole digestive process to be 
absolutely at a standstill and the gastric motor power diminished, or 
abolished for a time." This also explains the lack of desire for food 
during the attack. During convalescence one or two loose bowel move- 
ments or vomiting may occur. 
The temperature remains normal throughout the paralytic phenomenon. 
The pulse may be normal in frequency and volume or may be weak and 
irregular, with evidence of cardiac dilatation. The electrical reactions 
have been carefully studied during the attacks and in the intervals, 
by Oppenheim, Goldflam, and others. In severe attacks there is complete 
loss of reaction to the faradic and galvanic currents. In the interval 
between the attacks there may, in some cases, be a diminution in the 
reaction, a slow vermicular reaction of the muscles, and other evidence 
of degenerative change. Continued over a long period of time, these 
muscles may show degenerative atrophy. In most cases in the interval 
between the attacks the electrical reaction returns to normal. 
Besides the severe form of the disease, many cases present a less 
intense grade both in distribution and the extent of the paralysis. When 
the paralysis is localized it is most frequently confined to the lower 
extremities. Paralysis of the upper extremities, the neck, the face, and a 
hemiplegic form have been described. In some of the cases, and most 814 
DISEASES OF THE NERVOUS SYSTEM 
frequently the localized forms, there may be only a partial paralysis 
with slight or marked loss of power. Such attacks may be evanescent, 
lasting one-half to one hour, or may be prolonged as in the major attacks. 
In these cases the deep reflexes may be diminished or lost with retention 
of the superficial skin reflexes. There is likewise a diminution and not 
a complete loss of reaction to the electrical current. 
Abortive and equivalent attacks may occur. In the abortive attack 
there may be a heavy, sleepy, tired feeling, with slight weakness in the 
extremities, not sufficient, however, to incapacitate, extending over a 
period of several days. In some of Holtzapple's series, migraine attacks 
alternated with the paralytic phenomena and were sometimes associated 
with it. Consciousness was preserved in all the cases reported, with one 
exception, in which an attack of unconsciousness ushered in the paralysis. 
Between the attacks the patient feels, as a rule, perfectly well. In some 
cases the patient may awake in the morning completely paralyzed, and 
be occupied with laborious work by the afternoon of the same day. In 
other cases the attack passes off more slowly, the full power not being 
restored in from twenty-four to forty-eight hours. In Burr's case it lasted 
an entire week. 
Course.-While cases have been reported as early as the fourth year 
and as late as the thirty-first year, the majority of the cases develop 
about the age of puberty. There is a tendency to a diminution in the 
frequency and intensity of the attacks after middle life. A degenerative 
type of paralysis affecting both upper and lower extremities and confining 
the patient to a wheel-chair developed in some of Holtzapple's cases. 
While this affection is considered by all the other observers as not danger- 
ous to life, sudden death in an attack occurred in six of Holtzapple's 
cases, giving a mortality of 35 per cent, in his individual group. 
Treatment.-A study of the family groups above mentioned suggests 
the idea that the underlying factors vary somewhat in the different 
groups, and this is borne out by the results of treatment. In two of 
Mitchell's cases the bromides give no results; in one it had some slight 
influence. In Holtzapple's cases, 0.5 dram of. potassium bromide with 
1 or 2 grains of caffeine citrate, repeated in one or two hours, had a 
decidedly abortive influence when taken in the early paroxysms. In 
not a single instance did he note the development of an attack when the 
bromide had been taken during the onset. In Mitchell's cases citrate 
of potash in doses of 45 to 60 grains a day had " some small but uncertain 
effect." Administered in the beginning of a seizure in repeated large doses 
it shortened and mitigated the paralytic period. 
Colon lavage, intestinal antiseptics, venesection, hypodermoclysis, 
and various forms of electrical treatment have been tried with negative 
results. A study of the factors which determine the attacks and their 
elimination as far as possible from the life of the individual, together 
with the maintenance of the physical and nervous tone at the normal 
level, would appear to be the most rational treatment of a disease which 
remains with us as it began with Westphal, an unsolved riddle. CHAPTER XXL 
ASTASIA-ABASIA. ADIPOSIS DOLOROSA. 
DANIEL J. MCCARTHY, M.D. 
ASTASIA-ABASIA. 
Definition.-A functional disturbance of the neuromuscular mechanism 
first described byJHocq.in 1888 as a morbid state "in which the impossi- 
bility of standing erect and walking n^pfhally is in contrast with the 
integrity of sensation, of muscular strength, and of coordination of the 
movements of the lower extremities." 
Etiology.-The disease is not an uncommon complication, and in 
reality a symptom-group of the functional neuroses-hysteria, neuras- 
thenia, epilepsy, chorea, etc. While it may occur aT any age, it is more 
frequent in youth and early adult life/yKnapp's statistics of 50 col- 
lected cases show an equal number in men and women. Of these cases, 
21 showed hysteria, 3 chorea, 2 epilepsy, and 4 intention psychoses. 
It may develop spontaneously or during the course of one of the func- 
tional neuroses; emotional excitement, traumatism, or exhaustion may 
be determining factors. It has been noted as a symptom in one case of 
cerebral tumor localized to the prefrontal area. It is, however, not a 
symptom of cerebral tumor, and must be considered in this isolated 
case as an accidental complication. 
The absence of any causative pathology even in cases which have 
existed over a long period of time, the rapid disappearance of the symp- 
toms by suggestion and other methods of treatment, the frequency of 
its occurrence as the complication of a functional neurosis, place it as 
a symptom group of purely functional nature. There is a tendency to 
consider the condition as one of pure hysteria. It may, however, exist 
without other hysterical stigmata, and in some cases at least, is the 
manifestation of the extreme fatigue of neurasthenia and negativism 
of some of the psychoses. 
Symptoms.-In the simplest form the patient is unable either to stand 
or to walk and in some cases even to sit (Akathisia), and yet in the 
recumbent posture retains full power and coordination of all muscles 
necessary for locomotion. A careful examination in such cases will 
not show any loss of power, the sensation may be normal, the reflexes 
show no evidence of organic disease, the bladder and rectal functions are 
retained, and an examination of the cranial nerves reveals nothing 
abnormal. It is not at all surprising, however, that in such cases evidence 
of hysteria in the field of sensation and the special senses should be 
present. When the condition develops upon a basis of neurasthenia, 
marked fatigue and quickened reflexes with paresthesia are to be expected. 
815 816 
DISEASES OF THE NERVOUS SYSTEM 
Apart from the typical and complete case, there is a large group of 
cases in which the ability to stand and to walk is not entirely lost, but 
interfered with to a greater or less degree, as is shown by a sudden or 
general loss of power with incoordination. This is of an irregular type, 
and can be easily distinguished from the ataxic gait of the tabetic or 
that seen in cerebellar disease. Even in cases which show a complete 
loss of the ability to stand or walk the patients are still able to walk on 
all-fours, to walk backward, and in some cases to swim (Oppenheim). 
The fatigue consequent upon muscular exertion, seen in neurasthenia, 
may, in some cases, be so intense as to constitute the basis for the loss of 
power, accentuated and to a certain extent determined by the nervous 
excitement and lowered will-power. In other cases the disease has its 
origin in a psychasthenia. In an individual who has a dominating fear 
of paralysis, after prolonged introspection an astasia-abasia may develop 
upon a moderate or marked grade of fatigue. Excessive fatigue, more 
particularly when associated with mental stress, may be the starting 
point in one who is neither a psychasthenic nor an hysterical patient. 
In the nervous or timid child, fear of pain after a fracture or other injury 
to the foot may cause the condition. 
Ziehen gives the following classification: 
1. Hysterical astasia-abasia dependent upon the subconscious idea 
implying an inability of the patient to stand or walk. He places this 
in the group of hysterical palsies. 
2. Hypochondriacal astasia-abasia dependent upon the conscious but 
false idea of the inability of the patient to stand or walk. This may 
arise as a primary delusion or be dependent upon paresthesia, visceral 
disturbance, diplopia, vertigo, cardiac palpitation, etc. 
3. Affective or emotional astasia-abasia caused by a subemotional 
shock. An attempt to walk is associated with a sudden overwhelming 
fear, inhibiting the motor function. 
4. The psychasthenic form of astasia-abasia dependent upon a morbid 
impulse or concept that develops on a false idea of inability to walk or 
stand, or paralysis, or a fear of falling. This form is dependent on 
defective will power. 
Diagnosis.-Astasia-abasia must be differentiated from paralysis due 
to organic disease of the spinal cord, cerebellar disease, labyrinthine 
vertigo, and myasthenia gravis. The absence of ankle clonus or Babinski 
reflex, or bladder and rectal disturbance, and of sensory disturbance of 
organic distribution, will easily exclude organic disease of the cord. 
In^ cerebellar tumors, optic neuritis and other evidence of intracranial 
presTlir^ together with the typical ataxia, will be present. The rapid 
muscular fatigue seen in myasthenia gratis after exertion may be easily 
mistaken for a partial form of astasia-abasia. This is particularly true 
when the symptoms of the onset are most marked in the lower extremi- 
ties. The loss of power in these cases is as marked, after repeated mus- 
cular exertion, when the patient is recumbent as when he is standing. 
The myasthenic reaction will establish the diagnosis. 
Akinesia algpr^. a condition based upon the same/neurotic basis as 
astasja-abasia7 should be differentiated from it! In this, all the voluntary ADIPOSIS DOLOROSA 
817 
movements are associated with intense pain, which increases in severity 
if the movements are continued. The pain may become so intense as 
to affect the entire body upon the slightest motion, and in this way 
produce a pseudoparalysis. The absence ofpain in simple and uncom-i 
plicated astasia-abasia should differentiate it from the above affection. ' 
Prognosis.-This is, as a rule, favorable. In most cases the condition 
yields easily to treatment. Some cases are very resistent and prolonged. 
The prognosis may be said to be dependent upon the underlying cause 
and the ease with which it may be controlled or removed. In cases 
which are permitted to run for a long period of time, contractures may 
develop which may necessitate vigorous and even surgical treatment. 
This complication is likely to develop when the astasia-abasia is asso- 
ciated with a hypertension of the muscles. 
Treatment.-This does not differ essentially from that detailed .under 
the functional neuroses. Removal of any causative factor which may 
be present, the reestablishment of confidence, the training of the will 
power, and reeducation as to the method of walking, standing, etc. 
(following the general plan outlined by Frenkel in tabes), are indicated. 
In conditions of lowered nerve tone, partial or complete rest treatment, 
with or without hydrotherapy, is advisable. Suggestion by means of 
static electricity, and in stubborn cases under hypnotism, may be_ 
necessary. It should be remembered that the cure of astasia-abasia in 
a patient with other symptoms of hysteria or neurasthenia is simply 
the relief of a symptom-group, and not the cure of the disease. 
ADIPOSIS DOLOROSA (DERCUM'S DISEASE). 
This was first described by Dercum1 in 1888, from a case studied in 
the wards of the Philadelphia Hospital (Blockley). 
Etiology.-It is a disease preeminently of middle life. Cases, however, 
have been described as early as the eleventh year and as late as the 
seventy-eighth year, but these are exceptions. The vast majority of 
cases are found in the female sex. A few cases have been reported in 
men, one of these by Ewald,2 in the forty-seventh year. A history of 
alcoholic excess is not infrequent. Traumatism and emotional excite- 
ment have also been given as causative factors. Syphilis as a factor 
has not been given the consideration which its frequency deserves. The 
disease develops, as a rule, upon a neuropathic basis, and several patients 
have terminated their existence in insane asylums. While heredity is 
not an important factor, in Cheever's3 case the father and sister were 
also affected. Hammond reports two cases occurring in sisters. The 
majority of cases in women have developed at the time of the menopause 
or shortly afterward. One case followed an abortion, and in another 
pregnancy was given as the starting point of the disease. 
1 University Medical Magazine, December, 1888. See also Am. Jour. Med. Sc., 
November, 1892, and Jour. Nerv. and Ment. Dis., August, 1900. 
2 Berl. klin. Woch., 1895, Nos. 2 and 3. 
3 Brit. Med. Jour., 1904, i, 781. 
vol. v-52 818 
DISEASES OF THE NERVOUS SYSTEM 
Pathology.-To understand the position of adiposis dolorosa as a sepa- 
rate affection, it will be necessary to make a rapid survey of the other 
affections of a fatty nature. Apart from simple obesity, the following 
forms have been described: 1. Adiposis tuberosa simplex of Anders.1 
2. Adiposis cerebralis.2 3. Adenolipomatosis.3 4. Multiple lipomatosis. 
1. Adiposis Tuberosa Simplex.-This affection, at least in some of the 
cases, resembles adiposis dolorosa very closely. In patients who present 
for the most part the condition of simple obesity, Anders found localized 
fat tumors, sometimes painful to pressure, scattered through the abdomi- 
nal fat. These differ, however, both in their formation and in their 
disappearance under simple dietetic and hygienic measures, from the 
nodules seen in adiposis dolorosa. 
2. Adiposis Cerebralis.-This condition, described by Frohlich as 
dystrophia adiposo-genitalis, has a very close analogy to adiposis dolorosa. 
In its simplest form it consists of an excessive general adiposis developing 
during the course of a brain tumor, which in most cases has been found 
at autopsy to involve the pituitary body, but which in one case was 
found to involve the pineal gland (Marburg). A close association has 
been found in this group with defective genital development. 
3. Symmetrical Adenolipomatosis.-This condition, first described by 
MacCormac, consists of large fat masses localized to the region of the 
neck, the axilla, and the trunk, and associated with asthenia, mental 
irritability, apathy, hypochondria, enlargement of the spleen, accelera- 
tion of the pulse, and decrease of small mononuclear cells in the blood. 
These fat masses, even when of great size, may be associated with general 
emaciation. Microscopic examinations of excised portions have shown 
the presence of large and sclerosed lymphatic glands in the fatty tissue. 
An increase and diminution in the size of the fat during its development 
led Launois and Bensaude4 to consider the disease to be of lymphatic 
origin. 
4. Multiple Lipomatosis.-Multiple isolated lipomatous tumors have 
occurred symmetrically placed in different portions of the body. On 
account of the symmetrical arrangement and the association of the 
tumors with tabes, general paralysis, sciatica, etc., it has been assumed 
that the nervous system and more particularly the trophic centres in 
the spinal cord were the cause of the affection. There is, however, prac- 
tically no evidence to support such a theory. As many as two thousand 
of these lipomas have occurred in the same individual. 
Careful study of these various forms of diseases of the fat tissue will 
show a certain similarity. A study of the records of seven cases of adiposis 
dolorosa that have come to autopsy has shown lesions in the thyroid 
gland in the nature of atrophy with compensatory hypertrophy in all 
cases. Tumors of the pituitary have been found in three cases, an 
interstitial neuritis in the fat tissue in two cases, and a slight sclerosis 
of the posterior columns of the cord in one case. In one of these cases 
1 Am. Jour. Med. Sc., 1908, cxxxv, 325. 
2 Marburg, Wien, med. Woch., 1908, No. 49. 
3 MacCormac, St. Thomas' Hospital Reports, 1883, xiii, 287. 
4 Nouvelle Iconographie de la Salpetriere, 1900, xiii, 41, 184, 243. ADIPOSIS DOLOROSA 
819 
(Dercum and McCarthy), presenting a typical clinical picture of adiposis 
dolorosa, a tumor of the pituitary was associated with a hypoplasia of 
the genital organs, extensive lymphoid infiltration of the fat and hemo- 
lymph glands. This case forms a connecting link between adiposis 
dolorosa, adiposis cerebralis, and symmetrical adenolipomatosis. A 
case of adiposis, presenting clinically the picture of adenolipomatosis, 
in the writer's ward at the Philadelphia Hospital was found at autopsy 
to be a case of psammocarcinoma of the thyroid, with extensive metas- 
tasis in the fat tissue in the neck, destruction of the posterior portion of 
the pituitary body by an extensive multilocular cyst, sclerosis and old 
hemorrhagic infiltration of the adrenals, and extensive lymphadenitis 
with lymphoid infiltration in the fat masses, thyroid, pituitary, and 
adrenals. 
The compensatory action of the thyroid and pituitary bodies has been 
demonstrated experimentally. It would appear from the frequency of 
the lesions that the thyroid was mainly at fault in the production of the 
disease of the fat tissue. In the case of adiposis cerebralis, the lesions 
of the pituitary and the improvement after the removal of the pituitary 
tumors would point to the pituitary as the source of the trouble. 
The influence of the lymphatic system must be of importance. It has 
been found diseased in the cases above described, and in the tumor masses 
excised from cases of adenolipomatosis. Chipault and others have shown 
that localized lymph stasis may be transformed into fat tissue by a con- 
version of the connective tissue into fat cells. Whether the thyroid and 
the pituitary produce the changes above described, by disturbance of 
metabolism through some perturbation of the internal secretion, or 
whether the changes noted are secondary to changes in the lymphatic 
system, is a subject for investigation. 
The cause of the pain and tenderness in the fat masses in adiposis 
dolorosa is an interesting subject for speculation. Quite a number of 
the cases of adiposis dolorosa have shown some organic change in the 
central or peripheral nervous system. Tenderness over the nerve trunks, 
sensory changes, atrophy and degeneration of the muscles have been 
noted. Traumatism and syphilis have been noted as causative factors. 
Such factors acting upon the peripheral nervous system concerned with 
the nutritive changes in excessive adipose formation would easily produce 
low-grade interstitial changes in the nerves of the adipose tissue, and there- 
by cause the pain and tenderness. The pathological lesions of urticaria 
factitia described by Gilchrist present a pathological picture of transient 
acute inflammation. A similar lesion continued over a longer period 
of time and frequently repeated could easily cause the nerve changes and 
symptoms above noted. Such changes could more easily be produced 
in nerves whose function and nutritive tone were lowered by some 
metabolic or exogenous intoxication. 
Symptoms.-The disease begins, as a rule, in a slow and insidious 
manner, in the formation of either excessive deposits of fat scattered 
diffusely over the trunk and extremities, or in the form of irregular 
deposits of fatty tumors. It may develop as an independent affection 
in which the adiposis constitutes the disease, or may, on the other hand, 820 
DISEASES OF THE NERVOUS SYSTEM 
develop in one who is already of a fatty habit. The development of the 
disease is best studied in the latter group. A woman in middle life, of 
moderate obesity, develops, with or without prodromal symptoms, 
areas of painful swelling, irregular in size and distribution, somewhat 
elevated above the surrounding fat, the overlying skin of a normal 
appearance, slightly reddened, more often congested, and rarely with 
distended veins. These areas vary from one to two or four inches in 
diameter, are soft and somewhat oedematous to touch, very painful to 
palpation, and very often associated with a burning or lancinating sub- 
jective sensation of pain. After several days this condition disappears, 
leaving an indurated area in the fat tissue. This area from time to 
time is subject to a recurrence of the same condition less intensified, 
until finally a distinct nodular tumor formation of considerable resis- 
tance and consistence is left. This nodule is sensitive to pressure, at 
times even to light palpation, and constitutes the basis of the disease, 
adiposis dolorosa. Similar areas develop elsewhere through the fat 
tissue of the trunk and extremities, never affecting the face, the hands, 
or the feet. With further development there may or may not be an 
excessive deposit of fat. In the latter event the nodules may be seen 
distinctly elevated above the surrounding tissue, having somewhat the 
appearance of a multiple lipomatosis. In diffuse cases the fat is deposited 
in large masses, localized to certain areas, such as the abdomen, chest, 
thighs, etc., or massed in a general diffuse way over the entire trunk. 
Examination shows indurated areas, sensitive to palpation and pressure, 
scattered here and there throughout the fat masses. In the acute stage 
the swollen mass often reminds one of "caking breast." As the disease 
progresses to a full development, the areas of acute swelling and tender- 
ness become less frequent, and finally disappear altogether. Nodules in 
the fat remain as circumscribed masses, sensitive to pressure, and asso- 
ciated with diffuse pain of varying character described as neuralgic, 
rheumatic, etc. In some cases there is no history of the acute swollen 
areas, but, on examination, fully developed, painful, tender masses are 
present. In some of the diffuse cases, folds of fat give a sensation to 
the touch as of a mass of Worms. In some ill-defined cases, not properly 
classed with this disease, the whole fat tissue is sensitive without the 
formation of nodular areas and without spontaneous pain. 
Pain is a very prominent symptom present in all well-marked cases at 
some time or other, and may precede the development of the fatty nodules. 
It is often the only symptom complained of, and may be a sharp lancinat- 
ing pain or a dull ache, and is often described by the patient as a burning 
pain. It does not follow the course of the nerves, and is often associated 
with tenderness along the nerve trunks. While the painful and tender 
fatty areas constitute the main and the essential symptoms, a rather 
wide group of associated symptoms are frequently present. The most 
common of these is asthenia. It is exceptional to find a well-developed 
case of adiposis dolorosa in which this symptom, in some grade, is miss- 
ing. It may be nothing more than the nervous apathy and sluggishness 
of very fat people, but in the majority of cases is so marked as to be 
considered of pathological origin. Psychic symptoms are not infrequent. PLATE XI 
Adiposis Dolorosa. (Case of Dercum and McCarthy.) 
Tumor of the pituitary body; thyroidal changes; defective development 
of the testicles. 
From the American Journal of the Medical Sciences, December, 1902. Adenolipomatosis. (Case of Dr. C. K. Mills.) 
plate xrr ADIPOSIS DOLOROSA 
821 
The most common of these is a querulous irritability. Mental apathy 
and psychic depression are most common in the asthenic cases. Delu- 
sional states and dementia sometimes necessitate the incarceration of 
the patient in an asylum. The reflexes are diminished and sometimes 
lost, but may be increased. 
In Dercum's first case there were areas of anesthesia and hyperesthesia, 
and several cases showing sensory changes have since been reported. 
Disturbance of the vasomotor system has been frequently noted. Varia- 
tions in sweat secretion, cyanosis of the extremities, dermographia, 
trophic ulcers, and subcutaneous ecchymoses have been described. 
Fig. 47 
Multiple symmetrical lipomatosis. Hemiplegia. 
Prognosis and Treatment.-The prognosis as to life is good, but as 
to cure is bad. In the majority of cases the disease is very refractory. 
Thyroid gland in ascending doses has been of benefit, in a small number of 
cases, in reducing the pain and the fat tumors. Aspirin and salicylates 
have been used with , some benefit to relieve the pain and tenderness. 
Massage, baths, diet, etc., have been of only temporary benefit. The 
tenderness over the nerve trunks has a tendency to disappear as the disease 
advances. CHAPTER XXII. 
SYPHILITIC DISEASES OF THE CENTRAL NERVOUS SYSTEM. 
B. SACHS, M.D. 
The discovery of the Spirochaeta pallida (Schaudinn) and the sero- 
diagnostic investigations of Wassermann and his followers have brought 
about a great advance in the recognition of syphilitic diseases of the 
nervous system. It is impossible to over-estimate the support which 
these methods give to the accuracy of diagnosis. On the other hand, 
laboratory methods are an addition to, and not a substitute for, clinical 
observations. A positive reaction of the cerebrospinal fluid gives certain 
evidence that the central nervous system is the seat of specific disturb- 
ance. The cerebrospinal content, the increase in the number of lympho- 
cytes, the excess of globulin, make the syphilitic nature of cerebrospinal 
disease still more positive. While the absence of all these changes in 
the cerebrospinal fluid may not militate altogether against the diagnosis 
of syphilis of the nervous system, our present-day understanding of the 
subject makes it at least improbable that there is any very characteristic 
specific disease present. 
At the time of the publication of the first edition of this treatise neurol- 
ogists were still in the habit of making a very marked distinction between 
syphilitic and parasyphilitic diseases of the central nervous system. 
Parasyphilis was regarded as a form of degeneration following upon 
syphilis but not actually due to the virus of the disease. The discovery 
by Noguchi of the presence of the spiroctueta pallida in the cortical tissue 
of patients dying of dementia paralytica has proved that parasyphilitic 
diseases, so-called, are essentially syphilitic, though there may be certain 
differences in kind and degree in the case of late syphilitic manifestations. 
Macintosh and Fildes1 hold that these are not " Syphilitic lesions of an 
unusual or particular form, but that they resemble in all essentials such 
lesions found elsewhere in the body." We shall do well, therefore, for the 
purpose of this article, to maintain the clinical forms formerly established, 
but the student is to remember that, however different the clinical mani- 
festations may be, all the forms herein discussed are etiologically of 
equal value. They are all the expression of a luetic infection which may 
differ in intensity or distribution, but in every instance the disease is 
considered to be due to an infection caused by the Spirochaeta pallida and 
the changes wrought in the central nervous system are a result. 
The British authors quoted above have been at special pains to prove 
that the primary sores, the cutaneous lesions of the secondary period and 
the tertiary lesion, the gumma, revealed the same histological character- 
1 Brain, 1914, xxxvii, 141. 
822 SYPHILITIC DISEASES OF CENTRAL NERVOUS SYSTEM 
823 
istics, viz., perivascular collections of lymphocytes and plasma cells 
associated with a proliferation of the cells of the affected vessels. All 
these lesions contained the Spirochaeta pallida and this organism represents 
the irritant about which the cell reaction is found. There is a qualitative 
difference, however, and in the tertiary process, for instance, the spiro- 
chsetes are present in a very much smaller number than they are in the 
primary or secondary form. 
According to the more recent view, it is well to regard the entire 
syphilitic process as passing through an acute and chronic stage. In the 
acute stage the spirocheete may wander into any of the internal organs and 
a varying degree of degeneration of the parenchymatous tissue may 
follow. As the acute stage of syphilis subsides there is a general destruc- 
tion of the spirochsetes, but some may be retained somewhere in the 
system or in some organs, so that isolated foci may be maintained for 
some considerable period of time, possibly years. The organisms lie 
dormant but are capable of revivification and may thus give rise to the 
very late tertiary lesions. If the lesion is an interstitial one there may 
be very few symptoms developed, whereas if the parenchymatous tissue 
is involved the cellular tissue will degenerate and the special function of 
the organ is certain to be affected. It follows from this that in inter- 
stitial lesions the effect of treatment will be much more apparent than in 
purely parenchymatous lesions. 
It has been well known for many years that, like other poisons, the 
syphilitic virus has distinct organs and sites of predilection. Thus, the 
liver and the testicles have been known to be more often affected than the 
spleen or the lungs. It was not appreciated, however, until lumbar 
puncture became a routine practice in many of our hospital services that 
the central nervous system is involved in the disease process with astonish- 
ing frequency. Ravaut has shown that meningeal irritation was evi- 
denced in 71 out of 118 cerebrospinal fluids examined even when there 
were no obvious nervous manifestations of central syphilis. The head- 
ache, the drowsiness, and the mental derangement which have occasion- 
ally been observed in the early stages of syphilitic infection must now 
be attributed to the early involvement of the central nervous system. 
Within my own experience distinct increase of lymphocytes in cerebro- 
spinal fluid has been found within three weeks of the initial lesion. 
Bearing in mind the fact that all these forms have a common etiology, 
with variations as to intensity and distribution, it is, nevertheless, prac- 
tical to differentiate between the following types of syphilitic disease: 
(1) Cerebrospinal syphilis; (2) acute and chronic specific myelitis; (3) 
syphilitic spinal paralysis; (4) tabes dorsalis; (5) general paresis. 
Cerebrospinal Syphilis.-Almost every manifestation of brain and 
spinal cord disease might be enumerated under this heading. At least, 
there are very few conditions pointing to focal disease in the brain or in 
the medulla spinalis which might not at one time or another be taken to 
be due to preceding syphilitic infection. It is not the intention to enum- 
erate all such diseases, but to select for especial mention a few of the 
clinical conditions which are so distinctly specific that they can by clinical 
examination alone be recognized as a form of syphilis of the nervous 824 
DISEASES OF THE NERVOUS SYSTEM 
system. More than twenty-four years ago the writer1 attempted to 
impress this fact upon the medical public by helping to establish a con- 
dition known as multiple, cerebrospinal syphilis. The resemblance in 
name to multiple cerebrospinal sclerosis was favored because, as a matter 
of fact, the differential diagnosis between these two conditions must 
frequently be made. 
By multiple cerebrospinal syphilis we mean an affection which involves 
distant parts of the cerebrospinal axis and gives rise to a multiplicity 
of symptoms, some of them due to a lesion of parts as widely separated 
as are the brain axis and the sacral segments of the cord. 
Etiology.-Under this heading nothing need be said except that this 
form is frequently due to a more or less recent infection and may appear 
at any time within a few months to a few years after the initial lesion. 
Nowadays the strongest possible corroboration of a suspected diagnosis 
may be had from the examination of the cerebrospinal fluid. Nonne2 
claimed at first that in these very cases the Wassermann reaction of the 
cerebrospinal fluid was often negative; but the more improved methods 
of recent years have shown that the Wassermann reaction of the cerebro- 
spinal fluid is positive in almost all the cases (Kaplan claims 100 per cent.), 
and that the globulin content is excessive in a very large percentage of 
the cases. At the time of a first examination, the amount and character 
of preceding treatment must be taken into account. But a negative 
reaction cannot be taken to prove the absence of syphilitic poison. 
This form of specific disease may safely be said to be due in a consider- 
able number of the cases to a chronic syphilitic involvement of the 
meninges of the brain and spinal cord. According to the predominance 
of the symptoms we may, therefore, have a pachymeningitis or lepto- 
meningitis cerebri specifica, or a pachymeningitis or leptomeningitis 
specifica spinalis. That from this meningitis the process may extend 
into the brain or spinal cord need not be insisted upon, and if some 
writers speak of meningo-encephalitis specifica or of meningomyelitis 
specifica, the morbid process itself unquestionably remains the same. 
The early and persistent headaches are the clinical expression of a chronic 
convexity meningitis of luetic origin. With this widespread involvement 
of the membranes of the brain and spinal cord there may be special 
gummatous deposits, and this accounts for the fact that in so many 
instances we have symptoms pointing with special force to involvement 
of remote parts. A gumma in the vicinity of the crus may be associated 
with a general leptomeningitis, and we can well understand, therefore, 
that in such a caSe there may be complete ophthalmoplegia interna and 
externa, without other cranial nerve symptoms, associated with a spastic 
paraplegia of the lower extremities and involvement of vesical and rectal 
functions, since the dorsal and lumbar portions of the cord are sites of 
predilection for the specific spinal pachymeningitis and leptomeningitis. 
Aside from involvement of the brain and spinal cord substance and the 
meninges, we may have a specific endarteritis as the sole expression of a 
1 New York Med. Jour., 1891, liv, 309. 
2 Syphilis u. Nervensystem, second edition, Berlin, 1909 (contains full literature to 
date). SYPHILITIC DISEASES OF CENTRAL NERVOUS SYSTEM 
825 
more or less chronic syphilitic process. In such conditions attacks of 
hemiplegia due to thrombosis are very apt to occur, and there is no doubt 
that specific disease of the spinal cord bloodvessels with subsequent 
softening may give rise to various clinical groupings pointing to involve- 
ment of the spinal cord itself. 
It is characteristic of syphilitic disease of the nervous system that the 
granulation tissue (granular proliferation) starts from the smallest 
capillaries, either from the capillaries of the connective tissue or from 
the capillaries (vasa nutrientia) of the larger bloodvessels. Oppenheim 
was one of the first to lay special stress upon the gelatinous character of 
the specific thickening of the spinal and cerebral meninges. Mention 
must also be made of the fact that a primary degeneration of the cranial 
nerve nuclei may be an expression of a general syphilitic infection. 
Symptoms.-The disease may come on in acute or subacute fashion. 
Headaches and vomiting are among the earliest symptoms. These are 
sufficient, in many instances, to arouse the suspicion of brain tumor; 
if, as happens often enough, optic neuritis is also present and rapidly 
progressive, the diagnosis of brain tumor may appear to be sufficiently 
well founded to justify cerebral decompression. It will be well, therefore, 
to make the specific tests of blood and cerebrospinal fluid before suggesting 
decompression.1 The onset of the localizing symptoms may be preceded 
by general symptoms such as are known to be due to a general specific 
disease, particularly of the brain, and it may be noted that in a very large 
majority of the cases cerebral symptoms precede those of a spinal char- 
acter. The earliest symptoms of this condition are apt to point to 
involvement of the base of the brain and particularly to involvement of 
the various cranial nerves, and among these the third nerve and the sixth 
are most frequently affected, while in others the fifth, seventh, eighth and 
twelfth nerves may be involved, according to the special distribution of 
the lesion. An early ptosis, an ophthalmoplegia externa and interna, 
unilateral deafness, abnormalities of sensation and even dissociation of 
sensation within the distribution of the fifth nerve, paralysis of one or 
both vocal cords, paralysis and atrophy of the tongue with accompanying 
disturbance of deglutition and of speech, and, of course, unilateral facial 
paralysis-all these symptoms may appear early in the course of a multiple 
cerebrospinal lues. Moreover, cranial nerve symptoms may be associated 
with paralysis of the upper and lower extremities. Not infrequently the 
upper extremities escape, so that we have the peculiar association of 
cranial nerve palsies with a paraplegia of the lower extremities. In the 
vast majority of the cases this paraplegia, whether of the upper or lower 
extremities, is of the spasmodic order, due, no doubt, to the more frequent 
involvement of the lateral portions of the cord, or to the fact that when 
there is a meningeal infiltration the invasion from the meninges affect 
first the tracts running through and near the lateral columns. That the 
spasmodic paraplegia would be associated with increase of the deep 
reflexes and with a disappearance of the superficial reflexes in the regions 
affected goes without saying. The vesical and rectal functions may or 
1 During the past years I have had decompression done in well established forms of 
luetic optic neuritis to prevent further injury to the optic nerves. 826 
DISEASES OF THE NERVOUS SYSTEM 
may not be disturbed. The paraplegia may at times be flaccid, and 
Hoffman1 has reported cases on the order of poliomyelitis anterior due to 
syphilis, while Spiller2 has reported a syphilitic acute anterior poliomyel- 
itis due to thrombosis of the cervical anterior median spinal artery. 
The clinical picture may, therefore, involve one or more parts of the 
central nervous system, and these parts may be remote from one another, 
so that the mere fact that a single lesion cannot explain all the symptoms 
in a given case, and that there must be a multiplicity of lesions, points 
with strong probability to a general luetic process. 
In the article on tabes reference is made to the pseudotabes syphilitica. 
We need not, therefore, at present refer to the possible involvement of 
the posterior columns of the cord giving rise to symptoms resembling 
tabes, and to their association with symptoms pointing to distinct involve- 
ment of the brain axis. But cases of this sort can be differentiated from 
true tabes by the very multiplicity of symptoms, by the association of 
tabic symptoms with a number of ocular nerve palsies, and not merely 
by the association with ptosis or with rectus externus palsy, as happens 
in some cases of tabes dorsalis. Whatever the localization may be that 
is indicated by the clinical symptoms, there are other peculiarities which 
lend support to the diagnosis of a multiple cerebrospinal lues. The 
writer summarized these so long ago as 1893.3 They are: (1) The 
unusual distribution of the disease over the greater portion of the brain 
and spinal cord axis; (2) the relatively slight intensity of the morbid 
process as compared with the extensive area involved, evidenced by the 
preservation of some of the functions with complete loss of others; (3) 
a rapid disappearance of some of the symptoms and a very chronic 
persistence of others; and (4) the frequent history of other cerebrospinal 
accidents. Thus, some of the patients give a history of a preceding 
hemiplegic attack with more or less complete recovery. Others give 
a history of temporary paralyses, of temporary aphasia; still others, of a 
preceding attack of paraplegia which may have lasted a few weeks or a 
few months, followed by satisfactory recovery. It is the remissions and 
exacerbations, the recoveries and relapses, that are more characteristic 
of a cerebrospinal syphilitic process than of any other condition. 
No investigation is complete without a thorough examination of the 
cerebrospinal fluid. In hospital practice it may be feasible to do lumbar 
puncture in every form of brain or spinal syphilis; in private practice 
it may be well to limit the practice strictly to those cases in which the 
diagnosis is somewhat in doubt, or in which it is desirable to gauge or 
determine the effects of treatment by repeated examination of the fluid. 
In several of my own patients, the temporary relief of symptoms, effected 
by the withdrawal of fluid has been so marked, that the patients have 
requested repeated lumbar punctures. 
The facts to be noted are as follows: A positive Wassermann reaction 
of the blood indicates constitutional lues (acquired or hereditary). It 
does not, however, prove that the disease itself is luetic. A negative 
1 Neurol. Centralbl., October 16, 1909. 
2 Jour. Nerv. and Ment. Dis., 1909, xxxvi, 601. 
3 Syphilis of the Spinal Cord, Brain, 1893, xvi, 405. SYPHILITIC DISEASES OF CENTRAL NERVOUS SYSTEM 
827 
reaction of the blood does not exclude syphilis, particularly if the blood 
has been taken from a patient who has been subjected to much anti- 
syphilitic treatment. In general paresis the Wassermann reaction is 
so rarely negative, that negative findings may militate against the 
diagnosis of that disease. 
Diagnosis.-The chief task is to differentiate multiple cerebrospinal 
syphilis from multiple cerebrospinal sclerosis, which can be distinguished 
from the latter by the absence of intention tremor, of nystagmus, and of 
scanning speech, and, above all, by the frequent remissions in the course. 
And yet remissions are characteristic also of disseminated sclerosis. 
However, in disseminated sclerosis there is rarely such complete recovery 
as one not infrequently sees in multiple cerebrospinal syphilis. At the 
present day the examination of the cerebrospinal fluid and of the blood 
will give valuable assistance, and if the findings in the blood and in the 
spinal fluid are positive, disseminated sclerosis may be excluded, since it is 
tolerably certain that it is the rarest thing in the world to have dis- 
seminated sclerosis develop on the basis of a preceding syphilitic disease. 
The only other widespread affections of the central nervous axis 
which might resemble multiple cerebrospinal syphilis are a cerebrospinal 
tuberculosis and a general carcinomatosis or sarcomatosis. From tuber- 
culous disease the syphilitic affection may be distinguished by the more 
rapid course of the former and by the fact that the tuberculous trouble 
very often leads to a strictly limited basilar or spinal meningitis with 
formation of one or more solitary tubercles. Moreover, the tuberculous 
process affects the lower portion of the brain axis and the upper portion 
of the spinal cord much more frequently than it does the dorsolumbar 
region. A general sarcomatosis or carcinomatosis may give rise to 
symptoms somewhat resembling those of multiple cerebrospinal syphilis, 
but, as a matter of fact, a difficulty in diagnosis rarely arises, for the 
preceding history of primary neoplasm in some other organ, particularly 
in the breast or in some of the glands or bones, puts the physician on his 
guard. The distribution of the symptoms may be very similar, but the 
greater gravity of the disease is easily recognized. As a rule, the symp- 
toms are steadily progressive, although, as in a patient recently seen, the 
symptoms may be stationary over a period covering six to eight months. 
The autopsy showed a general carcinomatosis extending all the way up 
and down from the cervical to the sacral region, involving the vertebrae 
and pressing upon the spinal coverings, but the spinal cord itself had only 
been affected by compression. In other cases, however, the carcinomat- 
ous process may cause complete destruction of the cord. To quote from 
a previous article of the present writer, we may state "that there is a 
frequent combination of symptoms that can be recognized as a special 
form of disease-a multiple cerebrospinal syphilis due chiefly to a specific 
meningitis of the brain and cord, which is apt to form special deposits 
in different parts of the central nervous system, or by proliferation to 
make inroads into the brain or cord, and that the association of cranial 
nerve affections with special symptoms, most frequently of a spastic 
type, in the presence of a distinct history of syphilitic contagion and of 
repeated remissions, gives strong evidence of this special form of disease." 828 
DISEASES OF THE NERVOUS SYSTEM 
Acute and Chronic Syphilitic Myelitis.-The frequent localization of 
the syphilitic process in the dorsal and lumbar regions of the spinal cord 
gives rise to a clinical picture pointing to an acute, subacute, or chronic 
myelitis. The absence of root symptoms (pains, paresthesia, etc.) indi- 
cates that the spinal meninges have escaped, but the association with 
distinct cerebral luetic symptoms, such as pupillary immobility or strabis- 
mus, goes to show that the specific myelitis is an especially restricted 
form of the cerebrospinal syphilitic disease. In the variability and 
incompleteness of the symptoms we have the same earmarks of the luetic 
process that were dilated upon in the section on cerebrospinal syphilis. 
The features of syphilitic myelitis are discussed elsewhere (page 237), 
and require no extended discussion here. According to the special areas 
of the cross-section of the cord which may be involved in the luetic process 
the symptoms will naturally vary. The clinical pictures of acute or 
chronic poliomyelitis anterior or of amyotrophic lateral sclerosis may be 
presented when the disease is, in reality, an acute or subacute incomplete 
luetic myelitis. Almost any. form of myelitis may occur due to syphilis. 
In making the diagnosis, it is of the utmost importance to consider other 
evidence of constitutional syphilitic disease which the patient may have 
presented at the time of examination or in preceding years. The writer 
has seen patients treated at one time for a transitory ocular palsy, at 
other times for an attack of hemiplegia, and then again for a spastic 
paraplegia of the lower extremities. One patient, who has passed through 
these three phases, in addition has developed a condition of specific 
dementia bearing a close resemblance to general paresis. The previous 
occurrence of such accidents, the frequent recoveries from more or less 
serious paralytic conditions, are features common only to syphilitic 
spinal disease. It should also be remembered that a specific meningitis 
or meningomyelitis may go hand in hand with the formation of one or 
more gummata, and the difficulties of diagnosis may therefore be increased 
by the fact that symptoms pointing to tumor of the cord may either 
precede those of myelitis or be superadded to them. 
Under normal conditions, the cerebrospinal fluid is under pressure of 
90 to 130 mm. (Nonne); it contains few, if any, cells; surely not more 
than 5 or 6 per mm. (Fuchs, Rosenthal, Chamber). The Wassermann 
reaction is negative (whether 0.2 cm. or larger quantities up to 1 cm. of 
the fluid be used). The globulin reaction is negative.1 
Under pathological conditions, the cerebrospinal fluid flows out under 
increased pressure. The globulin content is markedly increased; there 
is a marked lymphocytosis; all these symptoms have corroborative value, 
but may occur in any organic infectious, irritative, luetic or non-luetic 
disease. A positive Wassermann reaction of the fluid clinches the 
argument in favor of syphilis of the central nervous system (to avoid 
possible error quantities of fluid from 0.2 to 1 cm. should be employed). 
Specific Spinal Paralysis (Erb's Type).-This is discussed elsewhere 
(page 246) and is included here for completeness. Erb called attention 
to a series of symptoms which he considered pointed indubitably to 
1 For all serological details consult Kaplan's Serology of Mental and Nervous Diseases, 
Philadelphia, 1914. SYPHILITIC DISEASES OF CENTRAL NERVOUS SYSTEM 
829 
syphilitic spinal paralysis, but Oppenheim and the writer, while recogniz- 
ing the frequent occurrence of Erb's type, were more inclined to consider 
it a syphilitic spinal paralysis and not the syphilitic spinal paralysis. 
The experience of later years has strengthened this view. Erb's type 
was characterized (1) by the usual symptoms of spastic paraplegia with 
its peculiar gait, carriage, and movements; (2) by marked exaggeration 
of the deep reflexes; then by muscular contractures which are slight as 
compared with the exaggeration of the reflexes. Involvement of the 
bladder, a slight, yet distinct, disturbance of sensation, gradual onset 
of the disease, and a decided tendency to improvement, were further 
characteristics. Erb inclined to attribute this special form to a diffuse 
meningomyelitis, but Nonne has shown very conclusively in several cases 
which came to autopsy that Erb's type of syphilitic spinal paralysis may 
be the expression of a combined systemic degeneration involving the 
pyramidal and cerebellar tracts and the tracts of Goll. 
Before concluding, a quotation is taken from the chapter in the writer's 
book on the Nervous Diseases of Children: "One may suspect specific 
disease of the cord if the patient presents symptoms of paralysis, whether 
they be of the spastic or flaccid character and whether the contractures 
be slight or not, provided the patient furnishes evidence of a morbid 
process affecting a very large part of the cord and yet showing a relatively 
slight intensity at any given level of the cord. He may, for instance, 
exhibit the symptoms of extreme paralysis, spastic or atrophic, with 
partial or slight anesthesia of the parts paralyzed, with little or no involve- 
ment of the bladder, or, as often happens, he may present traces of specific 
disease in other parts of the central nervous system. The chief difference, 
according to this, between the ordinary forms of myelitis and the specific 
diseases of the cord can be understood if we remember that the symptoms 
of an acute or subacute myelitis prove that the entire cross-section of the 
cord is affected almost simultaneously and to an equal degree, whence it 
follows that in such cases severe paralysis is likely to be associated with 
severe anesthesia, with marked contractures, with absolute loss of vesical 
and rectal control, with serious trophic disturbances, and so on; whereas, 
in the cases of spinal syphilis the morbid process invades the cross-section 
of the spinal cord partially and slowly. We may, therefore, find symp- 
toms which point to a very marked affection of one or more of the systems 
of the spinal cord and to relative immunity from disease of the gray matter 
or other portions of the cord. Eurthermore, in the ordinary cases of 
myelitis the symptoms point to a certain portion of the spinal cord at 
which the disease is most intense, while in cases of syphilis of the spinal 
cord the clinical symptoms show that the disease involves a very large 
portion, if not the entire spinal cord, yet affects each single segment 
relatively little. A still further aid to differential diagnosis is the very 
frequent involvement of the brain, at the same time that the majority 
of the symptoms point to disease of the spinal cord, or if these two sets 
of symptoms do not set in simultaneously, we frequently have in a patient 
who presents symptoms of a chronic specific myelitis the history of a 
preceding illness in which the symptoms were of a cerebral, rather than of 
a spinal, character. . . . The rapid and often unexpected recovery, 830 
DISEASES OF THE NEHVOUS SYSTEM 
as well as the relapses, help also to distinguish these cases from the usual 
forms of myelitis." 
Treatment.-All of the preceding forms of syphilitic disease (with the 
possible exception of Erb's type) are amenable to vigorous antisyphilitic 
treatment. Let us suppose that in a given case the blood or the cere- 
brospinal fluid or both have given the well-known indications of syphilis. 
Unless there are serious contra-indications, treatment by salvarsan should 
be instituted promptly. Among the contra-indications are: (1) Very 
advanced age (with its brittle arteries and tendency to hemorrhage); 
early infancy; (2) acute or chronic nephritis; (3) diabetes; (4) myocarditis, 
and advanced arteriosclerosis; (5) advanced optic atrophy (in progressing 
optic neuritis great caution should be exercised). We cannot agree 
with Osler and Churchman1 that nervous lesions are a contra-indication. 
After apoplectic seizures and in basilar or cerebellar lesions, great caution 
should be exercised; in the late stages of general paresis and of tabes the 
treatment may be fruitless, but in all other nervous conditions of specific 
origin salvarsan treatment is amply justified. 
Mention should be made of the neuro-recidives as a possible contra- 
indication for repeated salvarsan injections. Curiously enough, we 
have seen far less of it in this country than was reported abroad. It is 
fair to suppose that the salvarsan injections may have acted as a provoca- 
tive agent, and these recurrent nerve lesions are the expression of a latent 
syphilis. The experienced physician or neurologist will not desist 
because of these nerve lesions, unless the symptoms point to the res- 
piratory or cardiac centres, in which case great caution must be exercised. 
Plan of Treatment.-Salvarsan should be administered by intravenous 
injection; by common consent the intramuscular injections have been 
abandoned. In all adults, especially in women, 0.3 gm. is the initial 
dose, to be repeated after a week or ten days. If the first injection has 
been well tolerated, 0.4 or 0.5 gm. salvarsan may be given at the second 
treatment. Allow an interval of four weeks to elapse, during which 
period one intramuscular injection of one grain of the salicylate of mercury 
may be given each week. Unless the symptoms are very urgent, it is 
well to give the patients a complete rest from all treatment for another 
fortnight, and then repeat the course of salvarsan and mercurial injections. 
At the end of the second course of injections, it will be well to examine 
the blood and spinal fluid again, or, at least, the former, to determine 
whether or not the treatment has had any effect upon the constitu- 
tional symptoms. Dreyfuss has shown that there is more danger in too 
little, than in an excess of, anti-syphilitic treatment. 
In cases of subacute or chronic syphilis of the central nervous system 
(specific headaches, recurrent attacks of specific meningomyelitis, recur- 
rent apoplectiform seizures) it is well to advise the patient to submit to 
repeated courses of treatment every six months. 
The technique of salvarsan injections should be within the grasp of every 
physician. After several years of innumerable modifications, it has 
become relatively simple. A very large personal experience has impressed 
The article on Syphilis (in Vol. Il) has many excellent hints on the subject. SYPHILITIC DISEASES OF CENTRAL NERVOUS SYSTEM 
831 
upon the writer the importance of preparing the salvarsan solution with 
the utmost care.1 Freshly distilled water is the first essential. Next in 
importance comes the accurate neutralization of the solution which should 
be introduced at a few degrees above body temperature. More important 
than all else is the careful introduction of the needle, the point of which 
must lie safely within the lumen of the vein. If the needle is correctly 
placed, the patient does not experience the slightest pain. It is well to go 
very slowly at first. If the injection is in the least painful, withdraw the 
needle at once and completely. Try another vein, or, if necessary, 
the other arm. The smallest amount of the solution poured into the 
perivascular tissue will give rise to disagreeable induration. With 
increasing experience, the use of normal solution before introducing the 
salvarsan has become superfluous; we have also learnt to use more con- 
centrated solutions (0.3 gm. to 40 cc.). If the physician is certain of 
the position of the needle, the solution may be injected with an ordinary 
Luer syringe, without preceding or subsequent saline solution. 
Neosalvarsan has been widely used. It is more easily dissolved; cold 
(sterile) water must be used; the dose is twice as large as that of sal- 
varsan. Excepting the ease of preparation, it has no advantages over 
the older drug. Its equal efficiency is a matter of doubt; those who are 
familiar with the technique should use the original drug. 
Salvarsan injections may now be given in a physician's office or operat- 
ing room. The patient and the physician must have ample time, and the 
patient should be required to rest at least one hour after treatment. A 
light diet and plenty of rest and quiet for the first twenty-four hours 
should be urged. 
In this era of the dominance of laboratory methods, it has been urged 
that salvarsan treatment be continued until the Wassermann reaction 
of the blood and of the spinal fluid becomes negative, and until the cell 
count of the spinal fluid is much diminished. The reduction of the cell 
count may be brought about, in many instances, often without the 
slightest change in the clinical manifestations; the Wassermann reaction, 
particularly of the blood, is often difficult to change; these are "Wasser- 
mann fast" reactions; and in such patients it is safer to be guided by the 
change in clinical conditions, than by a change, or absence of change, in 
biological conditions. 
Intraspinous Treatment.-With the knowledge that the central nervous 
system is frequently the seat of an active luetic process, came the desire 
to introduce some spirocheticidal substance directly into the spinal canal. 
Swift and Ellis introduced the use of salvarsanized serum by injection 
into the spinal canal. They were able to bring about marked changes 
in the cerebrospinal content and reported considerable improvement, 
particularly in cases of tabes dorsalis, and some improvement in general 
paresis. The Swift-Ellis method consists in giving a patient an intraven- 
ous injection of 0.5 gm. of salvarsan. One hour later sufficient blood is 
withdrawn to yield about 15 cc. of serum. The next day this serum, 
carefully prepared, is injected at body temperature into the spinal canal. 
1 The details of the technique are well given in Kaplan's book, p. 213 el seq. 832 
DISEASES OF THE NERVOUS SYSTEM 
The quantity of spinal fluid removed is to equal the quantity of serum- 
salt mixture introduced. 
For a short period the salvarsanized serum method met with con- 
siderable favor. If the patient's own blood-serum was surely "salvar- 
sanized" it was natural to consider the Swift-Ellis method full of hope. 
It would make it possible to bring the arsenic preparation into very close 
contact with the specific tissues within the spinal canal. The discomfort 
occasioned by the infraspinous procedure is considerable; the method is 
also not entirely devoid of danger. It was necessary, therefore, to inquire 
whether this method accomplished much more than did the much simpler 
and less dangerous intravenous method. In association with Strauss 
and Kaliski,1 the present writer gave considerable study to this problem. 
" It seemed . . . that blood withdrawn three-quarters of an hour after 
an intravenous injection would have to depend for whatever efficiency 
might be attributed to it upon the presence of the salvarsan itself, since 
in the short interval . . . the organism could not elaborate any form of 
protective bodies." As a matter of fact, Prof. Benedict-a chemist of 
unquestionable authority,-helped us to determine how little salvarsan 
was actually circulating in the blood. A man weighing about one 
hundred and fifty pounds and having a total blood volume of about eight 
pounds, if injected with 0.4 gm. of salvarsan would have in every cubic 
centimeter of his blood immediately after the injection, before elimination 
or fixation of the body cells began, about 0.0001 gm. of salvarsan. This 
was the maximum amount that Benedict found in 20 cc. of whole blood. 
It was shown that the arsenic is not free in the blood stream but is 
probably bound by the receptors of the body cells. 
Benedict also showed from an analysis of four specimens of spinal 
fluid withdrawn twenty-four hours after an intravenous injection of 
salvarsan (0.4 gm.) that the spinal fluid contains free arsenic in about 
one-sixth to one-tenth the concentration in the whole blood. This very 
striking fact is opposed to the usual belief that none of the drug adminis- 
tered intravenously finds its way into the spinal canal. If the reduction 
of the fluid cells is the one desideratum, Mitchell2 has shown that this 
can be accomplished by lumbar puncture, frequently repeated. 
Marinesco3 and Wechselman developed a method (modified by Ravaut) 
of infraspinous administration of neosalvarsan. Ravaut uses a 6 
per cent, solution of neosalvarsan in water. Each minim would contain 
approximately 0.003 gm. of neosalvarsan. The needle is introduced as 
for lumbar puncture; the spinal fluid allowed to run into the funnel of the 
apparatus, from one to four drops of the solution are added as desired, 
and the mixture then allowed to run by gravity into the spinal canal. 
Kaliski has modified this method so that each 0.1 cc. of a salt solution 
contains 0.001 gm. of neosalvarsan. 
The proper administration of salvarsan will require further study, 
and the elaboration of newer methods. Meanwhile many cases will call 
for the occasional use of mercury and even the iodides. Very recently 
Joseph Byrnes published a method by which mercury was injected intra- 
1 Am. Jour. Med. Sc., 1914, cxlviii, 693. 2 Jour. Nerv. and Ment. Dis., 1914. 
3 Ztschr. f. diatet. und phys. Therapie, 1913, xvii, 194. SYPHILITIC DISEASES OF CENTRAL NERVOUS SYSTEM 
833 
spinously. His results were surely as satisfactory as those obtained by 
other infraspinous methods. 
Mercury should be given in the form of inunctions, or intramuscular 
injections of the salicylate of mercury (| to 1 gr. per week), or in the form 
of the old-fashioned "mixed treatment." The iodides,-the mainstay 
of former decades-may be given in saturated solution, fifteen to fifty 
drops, well diluted, three times daily 
TABES DORSALIS. 
Synonyms.-Tabes dorsalis is also known as locomotor ataxia, and 
posterior spinal sclerosis, " Ruckenmarksschwindsucht" of the Germans. 
Definition.-Tabes dorsalis is a chronic, more or less progressive 
disease of the central nervous system, exhibiting its chief morbid changes 
in the spinal ganglia, in the posterior roots and posterior columns of the 
spinal cord; it is characterized clinically by a very definite series of 
symptoms, among which the Argyll-Robertson pupil, the lightning pains, 
the girdle sensations, the loss of the deep reflexes, the hypotonia, and the 
ataxic gait are the most prominent. 
Etiology.-During the last third of the nineteenth century the neurol- 
ogists were divided into two camps with reference to the etiology of 
tabes dorsalis. The one faction, led by Fournier and Erb, claimed that 
tabes dorsalis was a true syphilitic affection of the spinal cord; the other 
faction, led by Leyden, believed that syphilis was only one of a number of 
different causes that might produce this disease. The latter accused the 
adherents of the opposing theory of attaching undue importance to 
syphilis. As a matter of fact, the statistical data gathered by Erb and 
his pupils proved that in about 95 per cent, of the cases of tabes dorsalis 
there had been preceding syphilitic infection, and we may add to this that 
when the disease afflicted women, as it does much more rarely than men, 
the percentage of preceding syphilitic infection is even higher. The 
writer has for years paid the closest attention to this point, and in his 
own private practice, he has rarely seen a case of tabes in which the 
etiological factor of syphilis could be excluded. In only one case was he 
certain of this, and that was in a patient who had been entirely well until 
the age of thirty-five, and had never exhibited the slightest sign of 
syphilis, nor had he contracted any infection. He was struck with a 
piece of shell after a dynamite explosion, and within six weeks of that 
time developed the first symptoms of what proved to be a rapidly pro- 
gressive form of locomotor ataxia. More recently the Wassermann 
reaction and the study of the cerebrospinal fluid have revealed the 
tremendous importance of syphilis in the etiology, the percentages of 
positive blood findings varying from 90 per cent. (Nonne, Wassermann and 
Plaut) to 76.6 per cent. (Sachs, Castelli). The lower percentages recorded 
by the latter may, perhaps, be due to the established practice in this 
country of subjecting tabic patients to prolonged antisyphilitic treatment. 
Erb's dictum of years ago, that a person who has not had syphilis was 
not likely to have tabes dorsalis, has been fully substantiated by later 834 
DISEASES OF THE NERVOUS SYSTEM 
experience, and while trauma and possibly extreme exertion1 may be 
occasional factors, we are safe in stating that syphilitic infection is the 
almost universal cause of tabes dorsalis. 
Locomotor ataxia appears, as a rule, five to twenty years after the 
initial infection. If syphilis is acquired late in life, syphilitic forms of 
spinal disease may appear within a very few years. The writer has seen 
the first symptoms of tabes dorsalis, including optic atrophy, in a man, 
aged fifty-five years, appear within one year of the initial infection. 
Pathology.-The chief anatomical change is a degeneration of the 
posterior columns of the cord. Even the gross specimen reveals a distinct 
grayish discoloration of these parts. Under the microscope it is seen that 
the degeneration begins in the columns of Burdach at the level of the upper 
lumbar segment. It must be noted that this is the area through which 
the posterior roots pass. At higher levels the columns of Goll are also 
involved at a very early period of the disease. At a later stage the entire 
posterior columns of the lumbar and dorsal segments are completely 
degenerated. In the cervical segments the degeneration may be limited 
to the columns of Goll; later on here, as elsewhere, the greater part of the 
posterior columns is degenerated. In the morbid process the nerve 
trunks are degenerated, while the glia may remain either normal or may 
proliferate. The disease is not strictly limited to the posterior columns. 
The columns of Clarke and the posterior horns (particularly Lissauer's 
root zone) are frequently involved. The posterior roots are diseased so 
often that many investigators (Leyden, Redlich, Oppenheim) believe 
the changes in the spinal cord to be due to early involvement of the 
posterior roots. Oppenheim,2 and Thomas and Hauser3 have traced this 
degeneration to the spinal ganglia, the trophic centres of the posterior 
roots. The morbid process may extend into the medulla oblongata, 
involving the spinal root of the trigeminal nerve (thus accounting for 
the rare and interesting forms in which the trigeminal symptoms appear 
early in the disease). The solitary bundle, the sensory motor nuclei 
of the fifth nerve, the auditory nucleus (rarely), the hypoglossal nucleus, 
and the nucleus of the vagus nerve may become involved. In addition 
to all of these and much more frequently, we may find a degeneration of 
the optic nerve (simple white atrophy). Other cranial nerves may 
undergo simple atrophy. Oppenheim found a degeneration of the vagus 
and recurrent laryngeal and the glossopharyngeal nerves. Obersteiner 
and Schiff accounted for the hemiatrophy of the tongue by a degeneration 
of the hypoglossal. The auditory nerve is, of all cranial nerves, the one 
most rarely affected. Oppenheim's finding of a degeneration of the 
Gasserian ganglion is related to the involvement of the sensory root of 
the fifth nerve. Atrophy of the sensory nerves of the skin has been 
shown to occur. 
All the changes enumerated refer to the sensory fibres of the periphery 
of the spinal cord. French writers (Charcot, Pierret, Raymond, and 
others) have shown that in some cases the anterior gray horns are also 
1 Poisoning by lead and ergot produces clinical and anatomical conditions similar 
to tabes dorsalis. 
2 Archiv. f. Psychiatric, vol. xviii, vol. vii, 44. 3 Now. Icon., etc., 1904. SYPHILITIC DISEASES OF CENTRAL NERVOUS SYSTEM 
835 
involved. This will account for some of the cases of tabes with muscular 
atrophy, although it is doubtful whether the peripheral nerves may not 
be responsible for some of these muscular wastings. In conclusion, we 
may state that while the morbid process is essentially a posterior spinal 
sclerosis, it may involve other parts of the cord and of the central nervous 
system, as well as the peripheral nerves. The toxic agent may exert its 
effects upon almost any or every part of the nervous system, although it 
has certain definite sites of predilection; but to make tabes dorsalis a 
cortical disease because cortical fibres undergo atrophy (according to 
Jendrassik) is carrying the argument a little too far. All these changes 
may be secondary to changes in the central sensory neurones (Strumpell). 
The opponents of the Fournier-Erb doctrine of the specific origin of tabes 
dorsalis have urged that there is nothing characteristically syphilitic about 
the morbid process; there is some truth in this, but the force of this objec- 
tion is lessened by the fact that tabes dorsalis does occur in association 
with a typical specific meningitis.1 McIntosh and Fildes in their recent 
article (1914) express their surprise that so little has been made of this 
specific meningitis. It is generally accepted that Redlich and Obersteiner 
were correct in stating that the degeneration of the columns begins at the 
"ring of Obersteiner"-an apparent constriction in the posterior root at 
its point of insertion into the cord. Some have suspected early involve- 
ment of the cells in the posterior root ganglia, but this has not been proved. 
Nor is it unlikely that the specific leptomeningitis, found in some cases, 
is the invariable starting point of the tabic process. The early involve- 
ment of the meninges and of the roots would account for the earliest 
clinical symptoms. Noguchi2 has proved the presence of the Spirochaeta 
pallida in the posterior columns. 
Symptoms.-With the possible exception of general paresis, there is 
no disease presenting such a multiplicity of symptoms, and it is doubtful 
whether any one patient ever presents even a bare majority of the symp- 
toms. The clinical picture will be described as it is outlined in the 
writer's mind from a large individual experience, even if there be some 
departure from the common description. First of all, there are two 
distinct groups, one the ataxic and the other the ophthalmic. The ataxic 
group represents the classical type. In this group of cases the optic 
nerve symptoms are developed very late or not at all, while all the ataxic 
and sensory symptoms attain to fullest development. In the ophthalmic 
group optic nerve atrophy is one of the earliest and most pronounced 
symptoms of the disease, leading to early blindness, while the ataxic 
symptoms in this group are very imperfectly exhibited. While the 
latter group presents fewer symptoms, the early development of amaurosis 
makes it the form most to be dreaded. Both groups have, however, a 
majority of symptoms in common. It has been customary to divide the 
symptoms into (1) those of the prodromal or pre-ataxic stage, and (2) 
those of the ataxic stage. The prodromal stage may last for months or 
years, and yet the final diagnosis cannot be safely established until 
1 See Nonne's monograph and the writer's article in the New York Med. Jour., 
1894, lix. 
2 Munch, med. Wchnschr., 1913, 737. 836 
DISEASES OF THE NERVOUS SYSTEM 
several of the cardinal symptoms have appeared. The presence of three 
of the cardinal symptoms is sufficient for this purpose, and among those 
cardinal symptoms we must rank subjective as well as objective signs. 
The cardinal symptoms of tabes dorsalis (given approximately in their 
order of importance) are: (1) Lancinating pains, (2) Argyll-Robertson 
pupil, (3) loss of deep reflexes, particularly of the knee-jerks and of the 
Achilles tendon reflex, (4) Romberg symptom (swaying of the body with 
the eyes closed). (5) Girdle sensation in various forms; hyperesthesia 
as well as delayed sensation. (6) Hypotonia of the muscles. (7) 
Bladder disturbances (rectal insufficiency is rare). (8) Ataxic move- 
ments of the lower extremities. (Ataxia of the upper extremities is 
relatively rare.) (9) Sexual weakness. (10) Cranial (more particularly 
ocular) nerve palsies, strabismus, double vision, etc. (11) Optic nerve 
atrophy, single white or primary white atrophy. (12) Visceral crises. 
(13) Trophic disorders. 
Any combination of these symptoms is possible, but the majority of 
cases are characterized for a long period of time by lancinating pains, 
some forms of hypesthesia, pupillary symptoms, and by the loss of the 
deep reflexes. After the lapse of some time the ataxia and the bladder 
symptoms appear. 
The lancinating pains occur, according to Erb, in 90 per cent, of the 
cases, and in 70 per cent, of all cases of tabes they constitute the earliest 
symptom. They are lightning-like, severe, darting, or boring pains 
occurring in any part of the body, most frequently down one or both legs 
within the distribution of the sciatic, around the calves, and into the heel 
or toes. Sometimes they are particularly annoying within the distribu- 
tion of the crural nerves. These lancinating pains occur less frequently 
in the upper extremities, are localized often enough in the thorax and 
sometimes even in the face (tabes superior). The lancinating pains are 
so commonly sciatic in distribution that persistent sciatica, particularly 
if bilateral, is suggestive of tabes. These lancinating pains come on 
without reason when the patient least expects them. Any slight change 
of temperature may be sufficient to produce them. 
Hyperesthesia may be noted within the special areas of pain, so that 
the pressure of the clothing or of the bedclothes becomes intolerable. 
Closely allied to the subjective sensory disturbances just recorded are 
various forms of paresthesia, such as formication, a feeling of "pins and 
needles," a feeling as if the patient were walking on velvet or cotton, 
peculiar feelings of tension about the perineum and the genitals. Formi- 
cation in the distribution of the ulnar nerve is the commonest among those 
in the upper extremities. Disturbances of sensation around the thorax 
give rise to the well-known girdle sensation-a tolerably early and very 
characteristic symptom. These sensory disturbances are often the initial 
symptoms, and can be accounted for by the early involvement of the 
posterior root fibres and their prolongations into the cord. Tabic pains 
are, to a degree, early'rootjsymptoms. 
The Argyll-Robertson pupil occurs in at [least 80 to 90 per cent, of 
all cases. It implies a failure of reaction to light, whereas the pupils 
react promptly during convergence and accommodation. Simple as the SYPHILITIC DISEASES OF CENTRAL NERVOUS SYSTEM 
837 
phenomenon is, experience is necessary to determine the exact manner 
of pupillary response. It is well to examine the patient in a darkened 
room with artificial light (many dark pupils cannot be examined by 
ordinary daylight). Unless every attempt at convergence is excluded, 
a false inference may be drawn. Also remember that the phenomenon 
applies only to eyes with normal or nearly normal vision. 
The immobility of the pupil to light is generally bilateral. Differences 
in the reaction of the two pupils may be observed in the earliest stages. 
At other times it is strongly suspicious of an active luetic process. Tabic 
pupils are generally miotic ("pin-hole pupils"). Even the smallest pupil 
may contract still further during convergence, but will not do so under the 
stimulation of light. The contours of the pupil are apt to be irregular. 
Oculists lay less stress upon this, but neurologists are generally agreed 
with the writer in holding this sign to be one of importance in tabes, 
and particularly in those cases in which the luetic virus is still active. 
Mydriasis occurs in those cases in which there is complete immobility 
of the pupil, and may be unilateral. In the writer's experience the 
Argyll-Robertson pupil is the earliest objective, and by far the most 
constant, symptom. A close second is the loss of the deep reflexes. 
The loss of the patellar tendon reflex (Westphal's phenomenon) is one 
of the earliest and most constant symptoms. It is present in fully 95 
per cent, of the cases, although in a few instances, particularly in the 
earlier stages, the knee-jerk may be either present in one or both legs, 
or may still be elicited by reinforcement (Jendrassik's method). It is 
not unusual to observe a gradual disappearance of the knee-jerks with 
the development of other symptoms. For this examination the patient 
should be tested when the knees are bare, else mistakes are bound to 
occur; and if there is any doubt it is wise to put the patient in a recumbent 
position with the knees semiflexed, or else to lay the patient flat on a 
couch or bed with the legs hanging down over the edge. At times a 
diminished knee-jerk can only be discovered by placing one hand on the 
quadriceps while tapping the tendon with the other. A hypotonic con- 
dition of the quadriceps is a more or less constant accompaniment of 
the loss of the knee-jerk. It is a well-known fact that many persons 
who know the value of this symptom inhibit the reflex more or less 
unconsciously. 
Of late years we have come to regard the loss of the Achilles tendon 
reflex as being of equal importance with the loss of the knee-jerk. Some 
writers even claim that it often precedes the loss of the patellar tendon 
reflex. This is surely not the rule. The loss of the Achilles reflex is of 
especial value if it is present on one side and absent on the other. 
Loss of the deep reflexes of the upper extremities is not as valuable a 
symptom, for the simple reason that these reflexes are not invariably 
present in normal individuals. The triceps tendon reflex is the most 
constant, but failure to elicit it cannot be considered a diagnostic aid 
unless other deep reflexes are also wanting, except possibly in the few 
(and rare) cases of cervical tabes. The unilateral loss of the triceps or 
wrist reflex would be significant. 
By contrast the superficial reflexes remain unaltered, disappearing in 838 
DISEASES OF THE NERVOUS SYSTEM 
those cases only in which there qre very grave disturbances of cutaneous 
sensation. The plantar, the cremasteric, the gluteal, and the abdominal 
reflexes can be elicited in tabic patients easily enough. The Babinski 
phenomenon is never present, as it is absent also in all normal persons. 
The next most important and very constant sign is the Romberg 
symptom-the swaying of the body with the eyes closed. This is due 
chiefly to the loss or alteration of superficial and deep sensibility. To 
elicit this symptom the patient is asked to stand with eyes closed and with 
the feet touching one another at the toes. Erb has shown that this 
necessitates careful balancing of the body, and can be carried out only 
if the patient appreciates exactly his position in space and his contact 
with the floor. The sensation of the skin of the feet must be normal and 
the patient must also have full knowledge of the tension of his muscles 
and ligaments, or else he cannot maintain his proper static equilibrium. 
So long as the patient is able to control by vision his whereabouts in space, 
he may be able to stand perfectly; with the removal of visual control 
the sensory defects become evident. This test should be repeated a 
number of times; even normal individuals on a first examination sway 
considerably when asked to close their eyes. It is rare to find the Rom- 
berg symptom in tabic patients with optic nerve atrophy and blindness. 
Some years ago the writer reported the case of a blind tabic individual 
who swayed when he closed his blind eyes. This was evidently a psychic 
reminiscence of his early tabic period. 
In connection with the Romberg symptom brief reference may be made 
to the sensory disturbances. In the vast majority of tabic patients there 
is, at least in the earlier stages, a very slight change of tactile sensation. 
At all events, nothing more than a moderate tactile hypesthesia exists. 
Such changes as do occur are most common in the soles of the feet, on 
the inner or outer margin of the feet, and within the distribution of the 
ulnar nerve. Starr1 has given excellent illustrations, taken from Bonar, 
of areas of anesthesia in cases of locomotor ataxia. They bear all the 
earmarks of true segmental anesthesia, but few cases present this symptom 
in such marked form. 
The pain sense is more often affected. Hypalgesia and delayed pain 
sensation are the rule. The latter is a very striking phenomenon, the 
patient perceiving it as only a mere'touch (although pricked with a pin), 
and after a second or more perceiving (at times) intense pain. Erb 
has shown that faradic sensibility is much diminished. On the whole, 
this is proportionate to the loss or diminution of the general pain 
sense. 
The temperature sense is apt to be altered. The perception of heat may 
be diminished and cold felt more acutely than by normal individuals. 
Exceptions to this rule are not infrequent. Allochiria, reference of a 
sensory stimulus to the opposite side of the body, the writer has observed 
a number of times; polyesthesia, the perception of several sensory impacts 
when touched but once, has also been recorded, but the writer has not 
seen it except as a species of self-deception. Astereognosis occurs in the 
1 Organic and Functional Nervous Diseases, third edition, 1909, p. 352 el seq. SYPHILITIC DISEASES OF CENTRAL NERVOUS SYSTEM 
839 
advanced stages, also analgesia of the genitals in both sexes; both are, 
however, rare phenomena. 
Sensory disturbances of the trunk of the body are present in a very 
large number of tabic patients. These hypesthetic and anesthetic zones 
are developed early, giving rise to various forms of girdle sensation, 
showing a distinct root and segmental distribution. These forms of 
disturbed sensation are symmetrically developed, and if they are asym- 
metrical they correspond to the anatomical distribution of the earliest 
tabic (root) changes in the dorsal segments. If the lower part of the 
spinal axis is involved, we are apt to find similar paresthesia and hypes- 
thesia around the perineum and genitals. 
Disturbances of deep muscular sensibility are largely responsible for the 
Romberg symptom. They may be revealed by other tests. The patient 
has lost all power to appreciate the exact position of his limbs. If he is 
in the recumbent position and is asked to elevate his leg to the exact 
level at which the examiner has placed the other leg (the patient's eyes 
being closed), he is unable to do this. lie may also fail to appreciate 
simple passive movements of the joints. All these tests can be carried 
out more readily with the lower than with the upper extremities. 
Tabic patients are supposed to have an impaired sense of fatigue (a very 
inconstant symptom). It is certain that they have a diminished osseous 
sense, a diminution or loss of vibratory sensation. 
A most valuable, reliable, and early symptom of tabes is a distinct 
muscular hypotonia. Erb, Leyden, and Frenkel have studied the phenom- 
enon carefully (Frenkel introduced the term). It may and often does 
precede the ataxia, and is, therefore, of great diagnostic importance. 
Not infrequently it helps to establish the diagnosis. It consists in an 
abnormal diminution of the tension of the muscles and ligaments, so that 
excessive passive movements of the joints are possible. The normal 
individual can flex the thigh upon the trunk with the leg extended 50 or 
00 degrees; the hypotonia of tabes permits this flexion to 90 degrees or 
more. Hyperextension of the knee in the recumbent position and genu- 
recurvatum in the erect position are constant phenomena. The same 
freedom of joint movement (according to Frenkel) is to be observed in 
the spinal column also in the joints of the hands and fingers. The 
abdominal and spinal muscles also are in a condition of hypotonia. The 
condition is independent of the degree of ataxia and of the loss of deep 
sensibility. It has a considerable influence on locomotion. 
We pass now to the consideration of the ataxia which is so prominent a 
symptom of the disease. We have hinted above that there are some cases 
not ataxic from the beginning to the end of the disease. Ataxia implies 
no loss of gross muscular power, but an inability properly to coordinate 
muscular movements. In more than nine-tenths of the cases ataxia 
appears first in the lower extremities, and very insidiously at that. 
The patient notices at first that he has become a little unsteady on the 
legs, that he walks as though he were intoxicated, that his legs "give way" 
under him. He experiences great difficulty in going up or down stairs; 
he stumbles in jumping off or on a car; cannot cross the street in safety; 
has to watch his movements; must look at his feet or else he does not 840 
DISEASES OF THE NERVOUS SYSTEM 
know where they are; if he is to walk at all, he must walk with a broad 
base and can walk with a stick only; finds much more difficulty in walking 
at night than during the day, and stumbles or falls if he tries to get out 
of bed in the dark. By degrees he has developed the typical tabic 
gait. 
These earlier stages of tabic ataxia are much more manifest on examina- 
tion. If the patient is asked to stand on one foot, he topples or falls; 
if asked to turn about on his feet quickly, he sways badly; if asked to 
walk a straight line, placing one foot in front of the other, he is utterly 
incapable of doing it; in all movements requiring coordination of the 
muscles he goes wide of the mark. In the recumbent posture he cannot 
elevate the leg when extended without performing zigzag movements; 
he has difficulty in touching one knee with the opposite heel, and with 
the eyes closed all these defects are much exaggerated, particularly if 
there is considerable impairment of deep muscular sensibility. 
Many patients learn to accommodate themselves to a certain degree of 
ataxia, and for years (particularly under the influence of modern thera- 
peutic methods) get about with considerable ease. As the disease 
progresses the increasing ataxia renders the limbs useless and the patient 
becomes chair or bedridden. In extreme cases the patient, even if 
supported, has lost control over his legs so completely that, on attempting 
to walk, the legs are thrown about in the wildest, often grotesque, fashion. 
Ataxic movements of the upper extremities are, on the whole, very rare, 
and there are few who have lost the ability to write or feed themselves. 
In that rare form known as tabes superior the ataxic movements of the 
hands are more pronounced. The ordinary movements of daily routine 
become impossible. Ataxic movements of the facial muscles, of the lips, 
of the tongue, of the laryngeal muscles, are rare. 
These are the most typical and constant symptoms, but there are a 
number of others, the student remembering that their absence need not 
militate against the diagnosis of tabes. 
Vesical Disturbances.-These are important and often most trouble- 
some. They are so common, even in the earliest period, that they not 
infrequently constitute the first symptoms that arouse a suspicion of 
serious organic nervous disease. In the genito-urinary service of a large 
hospital the neurologist is often consulted for the purpose of determining 
whether or not vesical symptoms, which appear to be independent of 
disease of the bladder itself, are the expression of a latent (?) tabes. At 
first dysuria (neuralgic pain in the region of the bladder), hyperesthesia 
of the neck of the bladder, dribbling, frequent micturition or moderate 
retention, are the usual forms of disturbance. As the disease progresses 
the amount of residual urine increases; catheterization may be necessary 
and cystitis is only too apt to be superadded. This increases the discom- 
fort, and if it leads to pyelonephritis there is distinct danger to life. 
Rectal insufficiency is rare. In some patients with hypesthesia of the 
anal region inconvenience is experienced after taking purgatives, but 
otherwise the rectal sphincter continues to perform its function satis- 
factorily. The patient is more depressed over the loss of sexual desire 
and of sexual power, the latter often preceding the former. Female SYPHILITIC DISEASES OF CENTRAL NERVOUS SYSTEM 
841 
tabic patients are reported to experience a loss of sexual desire, but the 
point is not easily established. 
Cranial nerve disease is an important factor. Oculomotor palsies, 
partial or complete, abducens paralysis, rarely trochlearis palsy, each and 
all are known to occur. The palsies of the earlier period-ptosis and 
strabismus-are particularly significant, because they occur even more 
frequently as the symptoms of cerebral lues. The diagnosis between 
tabes dorsalis and cerebral syphilis is established by the association of 
other characteristic symptoms of locomotor ataxia. In the more 
advanced stages we may find paralysis of associated movements of one 
or both eyes, pointing to involvement of the various nuclei. Complete 
ophthalmoplegia externa and interna are observed in rare instances. 
The facial nerve is so rarely involved that mention of this fact would 
not be necessary if facial nerve symptoms and those due to involvement 
of the motor branch of the fifth nerve did not occasionally occur in rare 
forms of tabes complicated by bulbar disease. The sensory branch of the 
fifth nerve is not as exempt as is the motor division of this nerve. It has 
been my peculiar experience to see a number of patients in whom a 
dissociation of sensation, at first unilateral and later on bilateral, within 
the distribution of the trigeminal nerve, has been the earliest symptom 
of tabes. In two of these patients this fifth nerve dissociation preceded 
all other symptoms by many years. During this period the diagnosis 
was much in doubt. The suspicion of an unusual form of syringomyelia 
was entertained until the cardinal symptoms of tabes left no doubt. 
Palsies due to involvement of the tenth and eleventh nerves also occur, 
but rarely, if at all. The most frequent form among these is characterized 
by dyspnoea and inspiratory stridor. One of Erb's patients had to have 
tracheotomy done, and wore a cannula for six years. Hoarseness and 
aphonia, due to paralysis of the recurrent laryngeal nerve, have been 
recorded. Atrophy or paralysis of the trapezius and sternocleidomastoid 
muscles, due to involvement of the eleventh nerve, is excessively rare. A 
little more frequent is the atrophy of the tongue due to disease of the 
hypoglossal nerve, but this condition is associated, as a rule, with other 
symptoms of bulbar paralysis. Progressive cranial nerve palsies, if 
not a typical progressive bulbar paralysis, may occur in association 
with the other symptoms of tabes. The involvement of one or more, 
if not of all of the nuclei of the cranial motor nerves, may cause an endless 
variety of symptoms. The olfactory and auditory nerves escape, as a 
rule, although cases have been described with loss of the sense of smell, 
with delusions of smell, and still others in which there has been continuous 
tinnitus with vertigo. Meniere's symptom group has also been recorded. 
But by far the most important cranial nerve symptom is the optic nerve 
atrophy. This has been referred to above, where it was said that it gives 
rise to a distinct group of cases known as amaurotic tabes. 
Among the orthodox symptoms, although often wanting, are the visceral 
crises and the trophic disorders. We owe our knowledge of the visceral 
crises largely to the accounts given by Charcot. In his inimitable way 
he described gastric crises, and nowadays every neurologist recognizes 
the condition. But they still seem to be a terra incognita for the general 842 
DISEASES OF THE NERVOUS SYSTEM 
practitioner and for the specialist in gastric and intestinal disorders. 
The gastric crises consist of severe neuralgic pains in the epigastrium 
or in some other part of the abdomen, appearing suddenly, lasting for 
hours or days and then disappearing quite as suddenly. The intense 
pain is accompanied by uncontrollable vomiting. It will be readily 
understood why this condition leads to errors in diagnosis. 
Entirely similar to the gastric are the intestinal crises. One patient 
with trigeminal dissociation also suffered from frequent inexplicable 
diarrhoea with intense abdominal pain, until the proper interpretation 
of both was made manifest by the appearance of a series of tabic 
symptoms. 
Among the curiosities of tabetic symptomatology we may class the 
vesical, the renal, the testicular, and even the clitoris crises. Special 
mention must be made of the laryngeal crises. These begin with a 
tingling sensation in the throat, a feeling of constriction leading to a 
condition of temporary asphyxia with stridor, cyanosis, and occasionally 
to a convulsive seizure. The spasm lasts but a few minutes, when the 
patient is out of imminent danger. Oppenheim has described pharyngeal 
crises with pain in the pharynx and frequent movements of swallowing. 
Cardiac crises are referred to because the writer has seen them in several 
cases, characterized by intense pains, like those of angina pectoris; but 
as these patients were at an age when angina pectoris could not be 
excluded, their exact value as a symptom of tabes is a matter of dispute. 
Lastly, a few words about trophic disorders. The best known among 
them is the perforating ulcer, which is generally situated below the big 
toe at the level of the metatarsophalangeal joint, but it may occur in 
other parts of the sole of the foot. It is a small, circular ulcer, extending 
through the soft parts to the bone. It does not yield readily to treat- 
ment. Erb claims that it is cured more often by mercurial salve than by 
anything else, and for this reason he believes it to be specific in character. 
Herpes zoster has been observed often enough to make it worthy of 
special mention. 
Charcot and his pupils were the first to dilate upon the arthropathies. 
The knee-joint is the one most frequently affected; next in frequency come 
the hip- and shoulder-joint, but marked and similar trophic changes 
occur in the elbow- and finger-joints. The chief characteristic is an 
enormous swelling of the joint with little or no increase of synovial 
fluid. The joint is, however, much enlarged, remains normal in color, 
and absolutely painless. The arthropathies may appear early, and if they 
become chronic lead to grotesque deformities, to luxations and subluxa- 
tions, to the formation of osteophytes; the bones become unusually 
porous and the slightest accident may cause fracture in and around the 
joint. Painless fracture of the long bones on slight exertion or slight 
injury is tolerably frequent. In the ankle-joints and in the smaller 
joints of the foot similar structural changes occur that Charcot described 
as the tabic foot-a grossly misshapen foot. A limb or joint so affected 
becomes thoroughly useless, and yet it is astonishing how long patients 
with these arthropathies manage to get about. Not unlike these arthrop- 
athies are the alterations of the alveolar processes leading to a loss of SYPHILITIC DISEASES OF CENTRAL NERVOUS SYSTEM 
843 
teeth. All the teeth may drop out in this way. It is so rare that in 
hundreds of cases of tabes the writer has not seen a single instance. 
Much has been made of the occurrence of cardiac disease in tabes. 
When it does occur it is an accidental complication, and it is due to the 
preceding syphilitic infection. In some investigations of cardiac condi- 
tions, by Collins and the writer,1 with the Wassermann method, the specific 
basis was well established, as was done before by Citron and others. 
Lastly, we must take note of the psychic manifestations. Conditions 
of depression, of neurasthenia, of morbid irritability, are not unusual 
or difficult to account for in view of the distressing condition. Sufferers 
from locomotor ataxia may develop paranoia or paranoid states. Some 
of them may pass through states of acute maniacal excitement or through 
states of transitory dementia with and without aphasia, probably an 
expression of brain syphilis. But all these psychic manifestations are 
relatively less frequent in tabes than the occurrence of symptoms char- 
acteristic of general paresis. Both diseases have a common etiology- 
syphilis. It is not astonishing, therefore, that some patients should 
develop both conditions. As a matter of fact, tabic patients do develop 
often enough the symptoms of general paresis (delusions of grandeur, 
loss of memory, paretic speech, etc.). Still more frequently do patients 
with general paresis develop a series of tabic symptoms (lightning pains, 
ataxia, loss of knee-jerks, etc.). Some symptoms, such as the pupillary 
reflex, immobility, loss of vesical control and of sexual power, both 
diseases have in common. In view of this community of symptoms, 
we need not hesitate to declare that tabes and general paresis are 
due to one and the same process, that both are metasyphilitic diseases, 
and that it is only the difference in localization that determines whether 
the morbid process gives rise to the clinical picture of tabes or of general 
paresis. That there is an intimate etiological relation between the two 
diseases cannot be denied, yet their simultaneous occurrence is, after all, 
relatively rare. Tabes followed by general paresis is distinctly rarer than 
general paresis followed by tabes. The association is rare enough to 
caution the physician not to make the diagnosis of general paresis when- 
ever a tabic patient presents slight psychic abnormalities. 
Before concluding, it may be well to recall the fact that the disease may 
last fifteen to twenty or even thirty years, unless life is terminated by 
some intercurrent disease. Of all the symptoms, blindness, lancinating 
pains, cystitis and the visceral crises are the most distressing. If the 
disease drags on, the ataxic limbs may become paralyzed; in this helpless 
condition the occurrence of bedsores, the incontinence of urine and 
feces, the increasing marasmus, make a truly pitiable picture. For- 
tunately patients are usually relieved of their misery by some intercurrent 
disease before these extreme conditions appear. 
Bearing in mind the great multiplicity of symptoms, one can readily 
see that there may be a vast difference in the grouping of symptoms of 
various cases of tabes, and also of the general course in one or the other 
individual. In some instances the course is unusually slow, so that the 
1 Am. Jour. Med. Sc., 1909, cxxxviii, 344. 844 
DISEASES OE THE NERVOUS SYSTEM 
disease appears to be stationary for ten to fifteen or twenty years; in 
others the disease may come on insidiously and suddenly take a turn for 
the worse, developing within the course of a few months a number of the 
most distressing symptoms. As a rule, the prodromal stage covers a 
period of months or years. By slow stages a slight difficulty in walking 
progresses into a marked ataxia. Only now and then the ataxia is devel- 
oped with startling rapidity. 
It has been customary to designate various types according to the 
predominance of some one set of symptoms or according to the localization 
of the disease as indicated by the chief symptoms. The terms explain 
themselves; thus, various writers choose to speak of tabes dolorosa, 
of tabes paresthetica, of tabes atactica, of tabes paralytica, and tabes 
amaurotica; other designations are tabes visceralis, and tabes superior 
(meaning cases in which the upper extremities are chiefly involved). 
Tabes juvenilis applies to those rare forms in which all the symptoms 
appear early in life. 
Erb has made special studies of the cases to which he applies the term 
tabes incompleta. These are practically identical with the formes frustes 
of the French writers. In this special group only a few of the cardinal 
and characteristic symptoms are present. While this may be the rule 
in the earlier stages of many cases, the term should be applied only 
to those in which this paucity of symptoms has been noted for a number 
of years. There are individuals, who, for years, have had the Argyll- 
Robertson pupil, others who have had visceral crises, still others who 
have had slight vesical disturbances, and every one of them suggestive 
of tabes, and yet in the absence of corroborative symptoms the physician 
may well hesitate to make a final diagnosis of tabes. 
Diagnosis.-It is evident that the fully developed forms can hardly 
be mistaken for any other affection of the nervous system. If two or 
three of the cardinal symptoms are present the diagnosis can be made 
without any hesitation, but it is well also to bear in mind that the disease 
may begin with one or the other of the more unusual symptoms, with 
visceral crises, with an arthropathy, with vesical and rectal disturbance. 
These rather unusual symptoms may precede the cardinal symptoms for 
months or years, and in such cases the exact nature of the disorder cannot 
be diagnosed until one or more of the cardinal symptoms appear. The 
early recognition is so important that the physician may be pardoned 
in venturing the diagnosis of tabes on mere suspicion and of subjecting 
the patient to such treatment as may possibly help to inhibit the progress 
of the disease. In the recognition of the earlier stages, evidence furnished 
by the Wassermann reaction and by examination of the cerebrospinal 
fluid will be of the greatest importance. 
In spite of all precautions, mistakes will happen, and the question will 
often arise whether the condition is one of tabes dorsalis or one of those 
now to be mentioned. The other chronic systemic affections of the spinal 
cord, such as spastic spinal paralysis, amyotrophic lateral sclerosis, and 
the spinal amyotrophies, need hardly be considered. In the latter the 
pupillary phenomenon are wanting, the deep reflexes may not be absent, 
the knee reflex may be increased, all sensory disturbances are wanting, SYPHILITIC DISEASES OF CENTRAL NERVOUS SYSTEM 
845 
and the entire clinical picture is wholly different from that of tabes. 
Some of the symptoms of tabes dorsalis may occur now and then in tumors 
of the cord, invading first the posterior half of the cord, and in multiple 
sclerosis, if the sclerotic areas happen to involve the posterior columns 
and the posterior gray matter rather than the pyramidal tracts, in which 
they generally occur. But in such cases in which there is a doubt, 
the occurrence of nystagmus, of altered speech, and the ataxic tremor 
will help to indicate the disease. Much more difficulty will be encount- 
ered in differentiating between a syphilitic meningomyelitis invading the 
posterior half of the cord and genuine tabes dorsalis. Many years 
ago the writer dilated upon the subject, to show that some cases which 
were considered to be genuine instances of tabes dorsalis were actually 
cases of specific infiltration of the meninges, starting in the meninges but 
invading the cord. The presence of such symptoms which point strongly 
to the true syphilitic nature of the process, such as complete immobility 
of the pupils, paralysis associated with the ataxia at an early stage of the 
disease, marked remissions and exacerbations of the symptoms, would 
help to indicate an active luetic process rather than a typical posterior 
spinal sclerosis. Syringomyelia would hardly come into question. In 
rare instances this disease, beginning in and affecting chiefly the lumbar 
portion of the cord, may give rise to symptoms suggestive of tabes dorsalis, 
but the dissociation of sensation and the absence of typical tabic pupillary 
symptoms will help to establish the differential diagnosis. 
Much more difficulty will be experienced in differentiating between 
tabes dorsalis and the combined sclerosis of the spinal cord. In this 
condition we are apt to have an ataxic paraplegia, and at one or the other 
stage of the disease the ataxia may preponderate to such an extent that 
the patient may present so few of the spastic and paralytic symptoms that 
there will be little reason to suspect anything else than tabes dorsalis. 
If the ataxic symptoms are associated with increase of reflexes, and if the 
pupillary phenomena are not typical of tabes dorsalis, the diagnosis of 
ataxic paraplegia or of combined sclerosis of the cord may safely be made. 
Friedreich's ataxia is recognized by its occurrence early in life, by its 
family or hereditary disposition, by the absence of the characteristic 
pains and sensory disturbances of tabes, by the absence of vesical and 
rectal disturbances, and by the existence of normal pupillary reflexes. 
The ataxia of Friedreich's disease is also coarser and of a more awkward 
type, and it involves the upper extremities much more frequently than 
is the case with true tabes. The " heredo-ataxie cerebelleuse" of Marie 
is recognized still more readily by the exaggeration instead of the absence 
of the deep reflexes; by the presence of club foot, and a number of other 
signs which do not occur in genuine tabes. 
From cerebellar tumors tabes dorsalis will be differentiated quite easily, 
for in the former the form of the ataxia, the vertigo, vomiting, double 
optic neuritis and the absence of pupillary immobility, of the Kornberg 
symptom, and of the lancinating pains, and the much more rapid develop- 
ment of all the symptoms, will lead to the correct diagnosis. The greatest 
difficulty of all will be experienced on attempting to differentiate between 
tabes dorsalis and alcoholic multiple neuritis, or, as it was formerly called, 846 
DISEASES OF THE NERVOUS SYSTEM 
alcoholic pseudo-tabes. The resemblance to tabes lies in the ataxic 
movements of the lower extremities and in the absence of the deep reflexes, 
also in the occurrence every now and then of marked sensory disturb- 
ances. On closer examination the difference is much more marked, for 
in alcoholic pseudo-tabes the pupillary phenomena are not so constant, 
although there is often a sluggish reaction to light. There is almost 
invariably a considerable degree of paralysis with the ataxia, often 
also some muscular wasting. Lancinating pains and girdle sensation 
are not the rule in multiple neuritis due to alcoholism, and the entire 
development of the symptoms is more rapid than is the rule in tabes 
dorsalis. Alcoholic pseudo-tabes leads more commonly to a paralytic 
condition of the lower extremities, more often also than is the case in 
tabes to an involvement of the upper extremities, while the physical symp- 
toms are less pronounced, and the inquiry into the habits of the patient 
will give assistance. In not a few instances the writer has been con- 
vinced that a moderate alcoholic excess was sufficient to produce a 
pseudo-tabes alcoholica which would not have been developed if the 
patient had not previously been infected with syphilis. 
Prognosis.-This is either favorable or unfavorable, not so much 
according to the gravity of the symptoms, as it is proportionate to the 
general optimism or pessimism of the physician. It is true that it is 
practically an incurable disease. The pupillary phenomena and the 
loss of the deep reflexes are rarely if ever recovered from, and in this sense, 
after the symptoms of tabes have appeared, the patient is afflicted with 
the disease for all time. On the other hand, the disease is often very 
slowly progressive; it may at times be said to come to a standstill, and 
many a patient is able to attend to his routine duties and to enjoy a tol- 
erably comfortable life for many years. There is much to be done for 
the tabic patient, and the latter may be given the assurance that there 
are many ways of making him fairly comfortable. The newer methods 
of treatment enable us to stay the progress of the symptoms for a con- 
siderable period of time; and the patients can be taught to use their 
ataxic limbs far better than was possible in former years. 
Some years ago some French neurologists claimed that tabes dorsalis 
seemed to have taken on a milder form; they were inclined to attribute 
this to the more thorough specific treatment which the patients received. 
There is some doubt as to the truth of this, but the fact remains that we 
do not see as many of the distressing forms of extreme ataxia as in former 
years, and tabic amaurosis does not seem to be as common as it was some 
twenty-odd years ago. On the whole, cases of tabes in which lancinating 
pains are the earliest and severest of the symptoms, are not the most 
unfavorable. While these lancinating pains predominate, the other 
symptoms are slow to appear. The cases in which a marked ataxia 
appears at a very early period represent a group of cases which are apt to 
advance rapidly and to lead to a large number of complications, but even 
these can be favorably influenced by various forms of treatment. The 
writer has been particularly struck by the fact that the amaurotic type 
of tabes, while it is perhaps the most unfortunate one, is often more 
slowly progressive and many of these patients would have been tolerably SYPHILITIC DISEASES OF CENTRAL NERVOUS SYSTEM 
847 
comfortable for fifteen or twenty years if it had not been for the loss of 
vision. The prognosis is most unfavorable in those cases with a cystitis 
or a pyelonephritis, in which other syphilitic phenomena, such as con- 
vulsions, have occurred as an early complication. The combination of 
tabes and general paresis is bound to lead, sooner or later, to a fatal 
termination. 
Treatment.-The question of prophylaxis looms up prominently. By 
the time the diagnosis is safely established the spinal cord changes are so 
considerable that one cannot expect to do much more than to arrest the 
disease. No one has yet been able to claim that he can in any way 
influence by treatment sclerotic tissue in the spinal cord after it has been 
once fully developed. As regards prophylactic measures, all that can be 
expected is possibly to lessen the syphilitic contagion. Such a Utopian 
state of affairs is not within our reach, but we can at least demand of 
everyone who has contracted syphilis that he subject himself to prompt 
and sufficient treatment. While it would be difficult to prove that prompt 
and proper antisyphilitic treatment following upon the initial lesion 
prevents the development of syphilitic and parasyphilitic diseases of the 
central nervous system, it is at least probable that these serious manifesta- 
tions could be to a degree lessened if energetic measures were resorted to 
in every instance at the outset of the disease. There is at least no doubt 
that when the first signs of any syphilitic or parasyphilitic affection of the 
central nervous system appear, energetic measures should be used. 
It is safe to establish the general principle that in every case of tabes 
dorsalis in which thorough antisyphilitic treatment has not been adminis- 
tered, such treatment should be given before anything else is attempted. 
At the present date, and in view of recent experiences, this statement 
may be modified to mean that if a positive Wassermann reaction has been 
obtained, the patient should receive prompt antisyphilitic treatment. If 
the reaction is negative, the same treatment may be given if there are 
clinical signs pointing to an active luetic process. 
By appropriate antisyphilitic treatment the writer means salvarsan 
injections, mercury salicylate (gr. j by injection) once a week, repeated 
during ten or twelve weeks, or else sublimate injections of | of a grain (0.01 
gram), repeated every other day until ten, twelve, fifteen, or twenty have 
been given, according to the severity of the case. A tablet (suggested by 
the late Dr. Jones) containing | of a grain (0.01 gram) of bichloride of 
mercury, | of a grain (0.016 gram) of sodium chloride, and of a grain 
(0.004 gram) of muriate of cocaine, has been found particularly useful. 
In spite of the well-known germicidal properties of sublimate, it has been 
the writer's custom to dissolve this tablet in a sufficient quantity of water 
and to boil it in a test-tube before making a deep intramuscular injection. 
It has been the writer's practice never to give iodides and mercurials at 
the same time, in order to avoid the formation of insoluble salts. The 
writer has reason to be thoroughly satisfied with the use of the sodium 
iodide in saturated solution, beginning with ten drops three times a day, 
well diluted, and increasing the daily dose by one drop until thirty to 
forty or fifty drops three times a day are reached. Beyond this it is 
hardly necessary to go, and the writer does not feel warranted any longer 848 
DISEASES OF THE NERVOUS SYSTEM 
in supporting the claim that satisfactory results in many cases cannot be 
obtained unless very large doses, such as 150 grains three times a day, 
are administered. In the treatment of syphilis of the nervous system 
the writer has abandoned entirely the so-called mixed treatment. 
The question has often been raised whether antisyphilitic treatment is 
of any distinct benefit in tabes dorsalis. One cannot claim that the 
disease has ever been cured. The pupillary reflexes do not return to 
normal, nor is an absent knee-jerk restored, but there is no doubt that a 
condition which seemed to be rapidly progressive has been arrested, 
that patients who have been tormented by severe shooting pains have 
suffered less, that vesical and rectal insufficiency has been lessened, and 
in not a few instances impending mental trouble has been averted. 
The writer has no hesitation, therefore, in favoring the energetic use of 
salvarsan, combined with mercurials in the initial treatment of tabes 
dorsalis, and of urging that these rather than iodides be given. 
In addition to this specific treatment much else can be done. The 
patient should be induced to lead a life free of all excitement and excesses. 
Alcohol and tobacco and every other form of indulgence should be 
restricted to very narrow limits. Physical exercise should be maintained 
within sensible limits so long as it is at all possible for the tabic patient 
to get about unaided. In view of Edinger's theories, fatigue should be 
avoided, but the ordinary tabic patient must be encouraged to keep his 
muscles in good condition and to use them in the best possible way so long 
as it is convenient for him to do so. The tabic patient who lies down, as it 
were, under his disease, seems to succumb much more rapidly than he who 
endeavors to make the best of it. Among general measures, hydrothera- 
peutic procedures have met with great favor. While hot baths should 
be avoided, the tepid full bath with slightly cooler ablutions can be safely 
recommended. Cold bathing, particularly sea bathing, is not tolerated 
well, although among our better classes there are many tabic patients 
who feel thoroughly comfortable while continuing the cold water habit, 
and so long as they do not complain of increasing neuralgic pains it need 
not be discouraged. This is a point which may well be determined accord- 
ing to the needs of the individual. 
Galvanism of the spine and general faradization are still employed. 
Many a patient feels comforted if not distinctly improved by such treat- 
ment, and the improvement that is attributed to mild faradization can 
be explained by the effect which it has upon the peripheral sensory neu- 
rones. General massage, particularly in connection with hydriatic 
procedures and with electrical treatment, is of distinct benefit. But there 
is no doubt that more has been accomplished in the successful treatment 
of tabic patients during the last decades by systematic exercise than in 
any other way. To Frenkel,1 of Heiden, belongs the credit of having 
developed a system of exercises so designed as to reeducate the ataxic 
muscles. It has been clearly shown that with the exception, perhaps, 
of the most advanced cases, patients who are so ataxic as not to be able 
to stand or walk unassisted can regain the use of their muscles and can be 
Die Behandlung der Tabischen Ataxie, etc., Leipzig, 1900. SYPHILITIC DISEASES OF CENTRAL NERVOUS SYSTEM 
849 
improved to such an extent that they can follow their ordinary occupa- 
tions. Those who are specially interested in the details of these exercises 
can find full particulars in the monographs of Frenkel and Goldscheider,1 
also in the writings of O. Foerster.2 Maloney3 has studied the relation of 
fear to ataxia and has elaborated a series of educational exercises based 
upon this doctrine tending to eliminate the purely psychologic factors of 
ataxia. 
In addition to the general measures, purely symptomatic treatment 
must often be used. So far as the pains are concerned, nitrate of silver 
has been endorsed by many, but its good effects are questionable. The 
use of pyramidon or aspirin, in combination with codeine, is sometimes 
of value. A distinct caution should be given regarding the use of opiates, 
particularly the use of hypodermic injections of morphine. Many a 
tabic patient has also become a marked morphine habitue and has thus 
made conditions worse than they would otherwise have been. 
The treatment of gastric crises calls for good judgment. It is best 
to give antineuralgic remedies, but during the crises the stomach and 
bowels should have complete rest, hot applications should be applied 
over the abdomen, the patient kept in a recumbent posture, and, if drugs 
must be given, a combination of cerium oxalate and codeine may be used. 
Rodari has advised the use of pantopon with atropine. In not a few 
cases special attention has to be directed to the condition of the bladder. 
Cystitis, with all its attendant dangers, is a common occurrence. This 
is to be treated according to well-received principles, and particularly 
in those cases with considerable residual urine, the bladder should be 
emptied at regular intervals and washed with mild antiseptic solutions. 
There is danger in allowing the patient himself or even a nurse, unless he 
be specially trained, to carry out these procedures. It means much for 
the comfort of the average tabic patient if his cystitis is held in check. 
In spite of all precautions there is greater danger to the patient from his 
cystitis than from almost any other condition. The constant use, for a 
prolonged period of time, of hexamethylenamine need only be men- 
tioned. 
If one were to enumerate every possible form of symptomatic treat- 
ment, it would be necessary to run the gamut of almost every drug in the 
Pharmacopoeia. A word should be said regarding the attempt to influence 
a rapidly progressive optic nerve atrophy by specific treatment. From 
a large experience it is difficult to claim that amaurosis has been com- 
pletely averted in any one instance. At the same time the claim can be 
put forth that the development of total blindness seems to have been 
postponed for a considerable period by the discrete use of antisyphilitic 
measures. It is, however, in these very few cases that one should be most 
careful not to push the measures to the extreme, although the writer 
is not able to endorse the opinions of some that the progress of optic 
nerve atrophy is made more rapid by the use of these measures. It is 
wise, however, to have the vision tested carefully during the treatment in 
1 Anleitung zur Hebungs Behandlung, etc., second edition, Leipzig, 1904. 
2 Physiologic u. Pathologic der Coordination, Jena, 1902. 
3 Jour. Nerv. and Ment. Dis., 1913, xl, 681. 850 
DISEASES OE THE NERVOUS SYSTEM 
any cases of amaurotic tabes, and if the vision is growing rapidly worse 
to desist from this therapeutic plan. 
Quite as important as, if not more important than, drug treatment, is 
the psychic treatment. No patients are more liable to general despond- 
ency, to marked hypochondriasis, than those suffering from locomotor 
ataxia. They should be given every possible consideration, be encour- 
aged in every way, and be told that in spite of having an incurable dis- 
ease, the symptoms may remain stationary for a long period, and that 
life can be made tolerably comfortable in spite of the disease. 
GENERAL PARESIS. 
General paresis of the insane, or dementia paralytica, represents one of 
the severest forms of nervous disease, and is characterized by a long series 
of physical and mental symptoms. From insidious beginnings the 
disease progresses, during a period of several years, to a condition of 
complete dementia. The majority of cases occur in persons between the 
ages of thirty and fifty, although its occurrence is not unknown in children 
(juvenile general paresis) and also in persons entering upon the senile 
period. In the former there is generally a history of hereditary lues, 
while in the latter, syphilitic infection has been acquired late in life. 
Etiology.-General paresis is a disease of civilization, although civil- 
ization alone can hardly be held responsible. Krafft-Ebing's dictum 
that it is due to "civilization and syphilization" has hit the nail on the 
head. The Chinese, Turks, and the Irish were supposed to be tolerably 
free of the disease. The same was at one time supposed to be true of 
the negro race, but in this country we have had ample opportunity 
to discover that neither the Irish nor the negroes enjoy immunity. It 
is well established that in only 40 or 50 per cent, of the patients is there 
any family history of mental or nervous disturbances, of dipsomania, 
or of any other serious form of degenerative nervous disturbance. 
The disease affects men very much more frequently than women, the 
proportion, according to some, being nearly 7 to 1, according to others, 
2 to 1. Among 70 cases in my private practice, 5 were women (7.1 per 
cent.). In all large cities general paresis, and particularly general paresis 
in the female, is far more frequent than in the country. Among men the 
disease is supposed to be commoner among the well-to-do and among 
those who have indulged in all sorts of excesses. The clergy are known 
to be relatively free from the disease, while the German statistics show 
that army officers yield an unusually large percentage. Women of the 
lower classes are distinctly more frequently the victims than are those of 
the better classes. These facts point to the well-established conviction 
that the chief etiological factor of general paresis is syphilitic infection. 
For many years we have had to be content with statistical proof, and those 
of us who have had a large experience have regarded it as almost axiomatic 
that the man who presents symptoms of general paresis must also present 
some symptoms of constitutional syphilis. In the writer's series in private 
practice there have not been more than two or three in whom there was SYPHILITIC DISEASES OF CENTRAL NERVOUS SYSTEM 
851 
any doubt as to the preceding specific infection. Of late years we have 
been made independent of statistical proof. The examination of the 
cerebrospinal fluid has shown a pleocytosis which occurs only in syphilis 
or in a disease of syphilitic origin. Moreover, the Wassermann reaction 
has shown an unusually high percentage of positive reactions in the blood 
of general paretics. Some of the figures have been above 85 and none 
much below 70 per cent. In 31 cases of the writer's the reaction was 
positive in only 67.7 per cent, (previous treatment may have influenced 
this). The discovery of the presence of the Spirochaeta pallida in the 
cortical tissue of paretics has removed every possible doubt. The find- 
ings of Noguchi and Moore have been corroborated by many others. 
There may be special exciting causes, but without syphilis general 
paresis does not occur. The frequent occurrence of general paresis in 
husband and wife teaches the same lesson. 
A period varying from five to twenty years or more may elapse between 
the initial infection and the first development of the disease. It is doubt- 
ful whether thorough or negligent treatment shortly after the initial 
infection has much influence upon the development of general paresis, 
but the mere fact that this is still a matter of doubt makes it incumbent 
upon every physician not only to treat the initial symptoms of syphilis, 
but to subject every person who has been infected with syphilis to repeated 
courses of treatment, whether such person presents symptoms or not. 
With the Wassermann reaction and the spinal cell count as guides there 
is no sufficient reason why any patient should be allowed to take his 
chances. The least the physician can do is to demand that the patient 
have an examination of the blood made at least twice a year, and if there 
be any positive reaction, then surely a course of treatment is necessary, 
whether that patient present any specific symptoms or not. 
Given a syphilitic infection, any excesses, particularly alcoholic and 
venereal, may play the part of further exciting causes. Overwork and 
worry have been assigned as additional etiological factors. Worry and 
emotional excitement play an important part in the development of many 
psychic conditions, and may have a bearing upon the development of 
general paresis, but overwork alone, and more particularly overwork 
without excesses, is rarely followed by any serious results. Excesses are 
harmful; work, and even overwork, as a rule, beneficial. Of the many 
additional exciting causes, the author feels that trauma of the skull is the 
one which deserves greatest consideration. Among the laboring classes 
several cases have been seen, in which the first symptoms of general 
paresis appeared within a few weeks following serious head injury, but 
in not one of these cases could the additional factor of a syphilitic infection 
be excluded. Ziehen is of the opinion that the syphilitic virus is apt to 
exhibit itself in the brain after its resistance to disease has been diminished 
by severe trauma. Possibly the shoe fits the other foot, and it would be 
safer to say that syphilis reduces the resistance of the brain and makes the 
effect of an injury more evident than it otherwise would have been. 
There seems little doubt that alcoholism is an etiological factor of some 
importance. When it is able to exhibit its effects upon the system pre- 
viously weakened by syphilis, the damage it does is doubly great. 852 
DISEASES OF THE NERVOUS SYSTEM 
Pathology.-The morbid changes prove it to be a disease not merely 
of the brain, but of the spinal cord as well. The popular designation of 
the disease as "softening of the brain" is wrong in every particular. 
Macroscopically we may note thickening of the skull, with loss of the 
diploe, adhesions between the dura and the skull, a pachymeningitis, a 
condition of external and internal hydrocephalus, a cloudy and thickened 
appearance of the pia, particularly over the frontal portion of the brain; 
adhesions between the pia and cortex, a granular ependymitis, and, above 
all, a very marked atrophy of the cortex. The fissures appear unusually 
deep and the gyri small. Arteriosclerotic changes are noticeable, more 
especially in the basilar arteries. The weight of the brain is considerably 
reduced, atrophy of the frontal and parietal lobes being responsible for 
this reduction, whereas the temporal and occipital portions of the brain 
are more nearly normal. On section ofthe cortex the gray matter appears 
very considerably reduced in volume. 
There has been considerable discussion as to whether or not one could 
recognize general paresis on the postmortem table. From macroscopic 
appearances alone this would hardly be safe, although we could make 
a fortunate guess in perhaps 50 per cent, of the cases. The researches 
of Cramer, Binswanger, and Nissl and Alzheimer, have thrown a flood of 
light on the histological changes. The pia is invariably diseased, with 
distinct infiltration with plasma cells and lymphocytes. All the blood- 
vessels show either proliferating or degenerative processes. The cortical 
substance itself contains diseased bloodvessels. There is a proliferation 
of the endothelium, the elastic tissue is increased, the adventitia prolifer- 
ates, and the adventitial lymph spaces are dilated and infiltrated. As the 
disease progresses the bloodvessels undergo regenerative changes. The 
ganglion cells suffer the greatest change. Many of them undergo com- 
plete atrophy, and those that are retained and at all recognizable exhibit 
every variety in change of the cell architecture. The changes in the 
interstitial tissue and in the bloodvessels are so great that the question 
has arisen whether the cells are primarily or secondarily affected. At all 
events, it is certain that in general paresis the nuclei and the neuroglia 
fibres are increased, that there is a proliferation of spindle cells, and 
that these are associated with marked changes in the bloodvessels, some 
claiming that the entire process may be conceived as a neuroparalytic 
hyperemia ending in a secondary lymph stasis, and that this lymph stasis 
is responsible for the degeneration of the nerve elements. 
Macintosh and Fildes1 state that the lesion of dementia paralytica is 
as typically syphilitic as a lesion of the liver. It occurs as an inflam- 
matory reaction about the Spirochaeta pallida situated among the nerve 
cells. A reaction of the parenchyma rapidly leads to degeneration, etc. 
According to these authors dementia paralytica is a tertiary syphilitic 
parenchymatous encephalitis. 
In spite of recent advances, it is well to hark back to the important fact 
that Tuczek first insisted upon-that the tangential fibres are destroyed 
in general paresis. It was supposed by some that these fibres disappeared, 
1 Brain, 1914, xxxvii, 178. SYPHILITIC DISEASES OF CENTRAL NERVOUS SYSTEM 
853 
particularly in the association areas of Flechsig. Others maintain that 
the atrophy is diffuse over the entire cortex. While each one of the patho- 
logical features of general paresis may occur in other conditions, it would 
seem fair to conclude that the disappearance of the tangential fibres, 
proliferation of the glia, marked vascular infiltration, together with 
the atrophy of the gyri, particularly in the frontal and parietal lobes, 
would enable one to recognize general paresis. It should also be stated 
that other portions of the brain do not escape; degenerative changes are 
to be found in the basal ganglia, particularly in the optic thalamus. 
Westphal, Siemerling, and others have found degenerative changes in the 
nuclei of the ocular muscles, and such changes have also been noticed 
in the nuclei of the pons and the medulla oblongata. Westphal was 
among the first to note a degeneration of the various systems in the 
spinal cord. The pyramidal tracts and the posterior columns are those 
most frequently affected. The degeneration of the posterior columns 
has been held by many to be identical with the changes in the tabes 
dorsalis; others have thought it to be distinct therefrom. The frequent 
association of tabes with general paresis may be more readily explained 
if we presume these changes to be identical in both diseases. Degen- 
erative changes have also been found in the cerebellum and even in the 
peripheral nerves. The student who wishes to investigate this special 
part of the subject is referred to the articles of Nissl and Alzheimer,1 
Cramer,2 Joffroy et Leri,3 Macintosh and Fildes and Nonne.4 
Symptoms.-There are few diseases so easily recognized as general 
paresis when the symptoms are well developed; there is, on the other 
hand, no disease of the central nervous system which begins so insidiously 
and which is diagnosed so often on mere suspicion. If once suspected, 
in nine cases out of ten the suspicion is corroborated by the further pro- 
gress. The disease is far reaching, not only for the effect that it has upon 
the patient himself, but often enough it has important bearings upon 
business and family ties. Nine times out of ten the patient who is brought 
to the physician's office with a blank countenance, who enters the room 
with an air approaching indifference, or at times in a state of excitement, 
who by the first word he utters reveals the tremor of speech, whose 
attitude shows that he fails to appreciate the importance of a medical 
examination, may be recognized as a sufferer from paretic dementia. 
The blank stare, the paretic speech, and the indifferent manner are the 
first conspicuous signs. The disease is characterized chiefly by a pro- 
gressive form of dementia, by a tremulous speech, by epileptiform and 
apoplectiform attacks, and by disturbances in the pupillary reaction. 
The symptoms may be divided into psychical and physical. The 
mental symptoms are much more important than the physical. Many a 
patient is aware of a change coming over him; others do not appreciate 
that they are in any wise different from their former selves, and resent the 
suggestion of illness. In the earliest stage the patient may have what 
appear to be ordinary neurasthenic symptoms, such as mild depression, 
1 Histol. und histopathologische Arbeiten von Nissl, Band i, 1904. 
2 Handb. der path. Anat, des N ervensystem, Band ii, chap. 41. 
3 L' Encephale, 1908, No. 4, p. 322. 4 Deutsch. Zeitschr. Nervenh., 1913, xliv. 854 
DISEASES OF THE NERVOUS SYSTEM 
a feeling of pressure on the top of the head, irritability, sleeplessness, 
vertigo, and easy fatigue. In association with these a change is noticed 
in the patient's emotions and general behavior. The man who has been 
extremely punctilious about business engagements neglects them and 
can give no special reason why he has neglected them; in fact, he fails 
often to see the importance of having done so. The patient who has 
practised a rational economy becomes wasteful; the man who has been 
sober in his dress takes to the wearing of garish colors; the family man 
who has been respectful toward his wife and children, toward his 
employees and his servants, shows an entire lack of respect, and instead of 
the decent treatment which he was accustomed to accord them, behaves 
tyrannically, if not brutally. Such changes denote a marked defect of 
judgment as to the relations between the patient and his fellow-beings. 
The same patient will squander money, sign checks recklessly, grow 
irritable over small trifles in business, neglecting larger and more impor- 
tant duties, thus proving that his judgment has become defective. 
Impaired judgment may lead the patient into criminal acts. Petty 
thefts, indecent exposures, improper relations toward subordinates, are 
some of the acts committed in the earlier stages of general paresis. 
With this defective judgment defective memory is associated at an early 
date, so that the patient forgets the facts that everyone is expected to 
know-the day of the week, the month, the year, the name of the Presi- 
dent, even the names of his family. The paretic at an early stage may 
be unable, offhand, to give the names of his children, or of his partner; 
his memory for recent events is often more impaired than his memory for 
things that have happened long since. It is curious to see how easily the 
physician may get at the evidence of impaired memory. As a rule, it is 
best to begin by questioning the patient as to his age. If he states, for 
instance, that he is fifty-tw'O years of age, and you ask him the year of 
his birth, he is unable to give it; ask him how long he is married, he fails 
to state it correctly; if the wife in his presence supplies the answer, he is 
unable to state the year of his marriage. A simple sum of subtraction is 
quite beyond his mental powers. If asked to write his name and address, 
he will be able to do it correctly in the majority of instances, unless he 
is in a very advanced stage. Ask him to write the names of some cities 
which have not come within his daily correspondence, and you will soon 
detect his inability to do so. The things which he has known most 
thoroughly he may be able to do; those which require a little reflection 
he fails in. In the early stage the patient may write New York, Boston, 
and Buffalo correctly, he is apt to fail on Philadelphia, Rochester, and 
Constantinople, which last is the stumbling block for many. In every 
instance it is well to assure one's self that the patient spelled correctly 
before the disease came on. His memory may also be tested by putting 
before him the simplest sums in arithmetic. The ordinary additions 
he may be able to do, but ask him to multiply 8 by 13 or 7 by 8, or ask 
him to divide 120 by 15, and one quickly discovers the limitations of his 
knowledge. Far from being annoyed at his mistakes, the average 
paretic shows either an indifference to the test or considers it a huge joke. 
The difference in the response on the part of the neurasthenic and the SYPHILITIC DISEASES OF CENTRAL NERVOUS SYSTEM 
855 
paretic is so marked in his answer to these simple tests, that whatever 
suspicions one may have entertained as to a mere neurasthenia are soon 
set aside by the results of this short examination. The outcome of these 
tests is that the first signs of dementia are quickly discovered, and as the 
disease progresses the dementia becomes more marked, so that finally the 
patient has forgotten the most elementary facts and his memory will 
grow so feeble that in the course of time he is nothing but a vegetating 
organism. So much for the rapidly progressive dementia. 
Associated with this dementia are other psychic symptoms. We have 
alluded to unusual irritability out of all proportion to the provoking 
cause. Of great import are the delusions of grandeur, which were by 
many considered almost pathognomonic, but which seem to many of us 
to play a less important part in the diagnosis than they did fifteen or 
twenty years ago. What the cause of these changes in the general clinical 
picture may be cannot be discussed at this point, but every now and then 
we still hear patients give expression to their exalted state of feelings, and 
the point that is characteristic of the delusions of grandeur of general 
paresis as compared with the delusions of grandeur of paranoia, is that in 
the former the delusions are extravagant, beyond all reason, and wholly 
unsystematized. They are the transitory and fleeting expressions of the 
moment, or mere extravagant utterances, and do not constitute the basis 
for purposive actions as they do in paranoia. 
The delusions of grandeur of the paretic are in general coincident with 
the euphoria, and vary with the station in life which he occupies. The 
paretic laborer may exhibit his exalted state of mind by travelling about, 
as one poor fellow did, all day long on trolley cars to prove that he owned 
the company. The merchant in the earlier stages of paresis may imagine 
himself doing millions instead of thousands of business per year. The 
stock broker imagines himself commissioned to corner steel or some other 
stocks. Unlike the paranoiac, he does not exact from others the respect 
and the homage due to his position. 
In relatively few cases the delusions are of a hypochondriacal and even 
of a melancholy tinge. The belief of an entire absence of stomach or 
bladder, the idea that the food instead of being digested passes into the 
head and not into the stomach, or the notion of a change in the genital 
organs, these and other equally absurd delusions occur in general paresis. 
Maniacal and deeply melancholic conditions, states of extreme anxiety 
and fear, also constitute a peculiar feature of the psychical condition. 
The true value of these is easily recognized, first of all by their absurdity 
and by the association of these delusions with some one of the other 
physical symptoms characteristic of general paresis or with the distinct 
evidence of a rapidly developing dementia. 
It is worth noting that in the early stages many patients realize the 
impending trouble. They may be conscious of the lapses of memory 
or of the difficulties in transacting ordinary business, and some notice the 
difficulty of speech. The writer has had a number of patients who made 
the diagnosis of general paresis on themselves, and in whom the suspicion 
has unfortunately come true. This was particularly striking in the case 
of a young lawyer, who had lost a brother from general paresis some years 856 
DISEASES OF THE NERVOUS SYSTEM 
previously, and who had a distinct recollection of the symptoms as the 
brother exhibited them; at the time he consulted me he recognized that 
he himself was exhibiting the symptoms of the paretic brother. After 
the initial stages have passed the patient soon lapses into a condition 
of apathy, and many of them become entirely oblivious to everything 
going on around them and are bedridden until released by death. 
The physical signs are quite as helpful in establishing the early diagnosis 
as are the mental symptoms. Reflex immobility of the pupils is one of the 
earliest symptoms, and often precedes the onset of other signs by many 
years. In some patients we have the typical Argyll-Robertson pupil, 
in others there is an immobility both to light and during accommodation. 
Irregularity in the contour of the pupil, either when the pupil is dilated 
and particularly when contracted, together with complete immobility, 
suggest an active specific virus. Inequality of the pupils and paralyses 
of the external ocular muscles also occur, the latter more frequently 
in the cases that are associated with tabic symptoms. 
Speech disturbances have an unusual significance and the suspicion 
of general paresis rests more often upon this one symptom than upon any 
other. The disturbance consists in a tremor of speech and in the inability 
to pronounce words with labials and consonants, particularly with I and 
r, and in the inability to repeat test sentences consisting of words joined 
together rather by sound than by their inherent meaning. Ask the 
paretic to say "truly rural," "artillery cavalry brigade;" ask him to. 
repeat such sentences as "Around a rugged rock a ragged rascal ran;" 
and you will find not only difficulty in articulation, but also a memory 
which is so defective that the patient forgets some of the words or some 
of the syllables and makes a jumble of the entire sentence. Neurasthenic 
and other non-paretic patients may hesitate and make some few mistakes 
when asked to repeat these test sentences, but on the second or third 
trial they correct their mistakes and pronounce the sentences well; above 
all, they show no such defective memory as the paretic patient does. 
In connection with the speech disturbance, tremors of the facial muscles, 
of the tongue, and of the hand are particularly noteworthy. The tremor 
of the fascial muscles is, perhaps, the most significant. When the paretic 
is asked to show his teeth, the entire face, including the lips, quivers; 
when asked to protrude the tongue, he exaggerates the movement, the 
tongue is jerked to and fro; and in most characteristic fashion the paretic, 
instead of pushing out the tongue once or twice, as the ordinary patient 
would do, keeps on doing it until he is told to stop. The facial tremor has 
a further significance, inasmuch as it is seen practically in no other 
condition except in acute and chronic alcoholism. The existence of this 
tremor alone necessitates the differential diagnosis between general 
paresis and alcoholism. The tremor of the hands is of a coarse kind and 
is more an awkwardness of movement than anything else. This suggests 
another disturbance so common in the general paretic, viz., a general 
awkwardness of movement associated with the tremor. In tying the 
cravat, in buttoning or unbuttoning a vest, in fastening suspenders; in 
the case of women, in the attempt to sew on a button, to hem a handker- 
chief, even to fasten a hairpin or a breastpin; in short, in attempting to 857 
SYPHILITIC DISEASES OF CENTRAL NERVOUS SYSTEM 
do the most accustomed acts of every-day life, the patient fails to do 
these successfully, and spends an unusual amount of time in accomplishing 
them. He fumbles and grows excited over the most trifling actions. 
The same tremor and awkwardness are shown in the attempt to write. 
The paretic may retain the power to write his name and the words 
which he has been accustomed to write again and again. Oftener his 
writing is tremulous, his memory is so defective that he omits letters and 
syllables in the most ordinary words, and as the disease advances he 
forgets even to write his name. The difficulties in writing may occur so 
early that the patient is startled at his inability, and worries about this 
more than about any other symptom. 
The deep reflexes may be either lost or exaggerated. In keeping with 
the changes which are known to occur in the spinal cord, it is not astonish- 
ing that this difference should occur. In some we have, together with the 
immobility of the pupils, a loss of the deep reflexes, of the knee-jerks, and 
of the Achilles tendon reflexes-the tabic series of symptoms. In others 
the reflexes are exaggerated, the knee-jerks are very much increased, and 
ankle clonus may be present. The exaggeration of the reflexes must be 
considered, particularly in those cases in which there is a question as to 
the condition being one of neurasthenia or of general paresis, although in 
neurasthenia it is most unusual to have an ankle clonus. 
A general muscular weakness is also frequent, particularly in the second 
half of the disease, and offers excuse for the term dementia paralytica, 
which, in perhaps more than one-half of the cases, would seem to be 
unwarranted. With this muscular weakness, particularly if the patients 
are kept in bed, a general flaccid, atrophic condition of the muscles is 
apt to be associated, due more to disuse than to anything else. 
Among the physical symptoms we must also include convulsive and 
apoplectiform seizures, which occur during the progress of the average 
case. While the rule holds good that these seizures occur in the second 
half of the disease, they sometimes constitute one of the earliest signs. 
The writer has always insisted that if a patient past middle life, who has 
neither cardiac nor renal disease, develops epilepsy or has an apoplectic 
attack, the suspicion is justified either of active syphilis or of general 
paresis, if not of both. 
Not infrequently the neurologist is asked to see a patient who is sup- 
posed to be in a uremic convulsion. Examination of the urine, however, 
fails to show any trace of kidney affection, and the suggestion that the 
convulsion may be the first symptom or an early symptom of general 
paresis comes as a surprise to the general practitioner. In the writer's 
experience, patients who exhibit apoplectiform or convulsive seizures at 
an early period run a very rapid course, the disease often ending fatally 
within six months or a year after such convulsions or apoplectic attack. 
The convulsive attack is of the ordinary epileptic character, and can in no 
wise be distinguished from the typical seizure, unless it be that the patient 
recovers very rapidly and appears, possibly for a week or more thereafter, 
to be in unusually good health. A little later, however, the first signs 
of the dementia set in, and the patient is found to present other character- 
istic signs of general paresis. The apoplectic seizure may result in a 858 
DISEASES OF THE NERVOUS SYSTEM 
temporary hemiplegia, from which the patient also may recover with 
unusual rapidity, and in other patients there may be, with or without 
hemiplegia, a transitory aphasia. The symptoms of these apoplectic 
or apoplectiform seizures disappear with unusual rapidity, but they 
leave the patient, as a rule, in a disturbed state, and with every recurrent 
attack-some paretics have six or even more such seizures-the mental 
condition becomes more and more apathetic and there is a steady deterio- 
ration. The rapid disappearance of the apoplectic symptoms leads to the 
suspicion that these attacks are not due to the ordinary vascular accidents, 
but that they must be due to some temporary interference with the circu- 
lation. In patients who have died of general paresis after apoplectic 
seizures no sufficient cause has been found for the apoplexy. 
Vasomotor and trophic disturbances are not unknown. Among these 
we need merely enumerate bedsores, perforating ulcer of the foot, arthrop- 
athies, gangrene. There is also at times an unusual fragility of the 
bones, so that fractures occur most easily and not infrequently lead to the 
suspicion or to the accusation that the patient has been severely handled 
by an attendant. ' In asylums hematoma of the ear is looked upon with 
especial suspicion as a symptom of unusually grave import. It occurs not 
only in general paresis, but also in many other psychic disorders. While 
it may be due to slight trauma, it surely would not occur if it were not 
for the marked tendency to trophic disorder. Pruritus, salivation, and 
universal cyanosis occur in rare instances, but have no pathognomonic 
value. 
General Course.-Enough has been said to indicate that the disease 
begins in insidious fashion, that there is often a suspicion of serious disease 
impending before the definite diagnosis can be made, and that in the 
majority of instances, if the suspicion has once been entertained, the worst 
fears, unfortunately, prove to be well founded. After the initial period 
of excitement or depression, accompanied either by hypochondriacal 
delusions or delusions of grandeur, after the development of the typical 
mental and physical symptoms, the disease passes into the stage of 
progressive dementia. After the lapse of months or of one or two years, 
during which time the patient has been in a state of euphoria, and, as a 
rule, is the only one in the entire circle ignorant of the severity of the 
symptoms, he passes into the terminal stage, in which he is bedridden, a 
mere vegetating organism, soiling himself, developing bedsores, becoming 
more and more emaciated; he dies from exhaustion, an apoplectic seizure, 
or intercurrent diseases. 
Duration.-It is commonly supposed that the disease runs its fatal 
course in two, three, or five years, but every alienist has seen cases that 
have run a course of ten, fifteen, or even twenty years. Many of us have 
obtained the impression that general paresis does not run as rapid a 
course as seemed to have been the rule years ago. It is difficult to decide 
whether this is due, on the one hand, to earlier recognition, so that the 
period of observation is longer, or to the more careful and more persistent 
antisyphilitic treatment which these patients now receive. But that 
careful treatment has much to do with the prolongation of life in general 
paresis is unquestionably true. Some of the longest-lived paretic patients SYPHILITIC DISEASES OF CENTRAL NERVOUS SYSTEM 
859 
are among those who have been nursed at home, where greater care 
can be given them than in the average asylum. There is also a further 
possibility that some of these long-lived cases of general paresis are, after 
all, cases of syphilitic pseudo-paresis, and in this connection it is necessary 
to call attention to the marked remissions occurring in the disease. 
Two and three such periods of remissions amounting to months and even 
years are not unknown. The most troublesome remission, however, 
is the one which frequently sets in after the disease was first suspected 
and after a proper course of treatment has been instituted; the improve- 
ment may be so great that doubt arises as to the diagnosis. This doubt 
is soon dispelled by the development or continuance of the typical 
symptoms. 
Prognosis.-This is most unfavorable if we allow for the few question- 
able cases which are supposed to have recovered. It is safe to predict 
an early fatal termination in almost every case of general paresis, and 
yet the experience of each alienist will undoubtedly bear out that of the 
present writer, who, in spite of his knowledge of the grave prognosis, 
has insisted that recovery is not altogether impossible, or at least that 
such pronounced remissions may occur that the patient may be able to 
resume his duties and be a useful member of his family and of society for 
a number of years. This is supported by the fact that several patients 
in whom, more than twenty years ago, at least three and four prominent 
alienists made the diagnosis of general paresis are at this day mentally 
well, although they still present some of the physical signs of the disease. 
There are disorders in which the diagnosis of general paresis is made for 
the want of better knowledge, and until we learn to discriminate better 
between the various forms of general paresis, it is well to know that some 
cases may behave contrary to the ordinary rule. 
Diagnosis.-A disease presenting as manifold a series of symptoms 
will naturally give rise to great difficulties in diagnosis. When the 
mental condition is in doubt the presence of the physical signs is of the 
greatest possible import. Thus, if a patient presents what would ordi- 
narily be supposed to be symptoms of neurasthenia, and if he, in addition, 
has small pupils and either diminished or exaggerated reflexes and a 
slight peculiarity of speech, the probability of his having more than a 
mere neurasthenia becomes very great. In attempting to differentiate 
between neurasthenia and general paresis stress must be laid on the 
fact that while the neurasthenic may complain of lack of concentration, 
of his inability to recall things, of pains in the head resulting from the effort 
of thinking, etc., he presents no signs of actual mental deterioration. 
Yet the distinction cannot always easily be drawn. In the writer's 
experience a patient was suspected of suffering from neurasthenia until 
the occurrence of an epileptic attack helped to clinch the diagnosis of 
general paresis. Paretic speech can hardly be mistaken for any other 
speech disturbance, but the hypochondriac and the neurasthenic are 
apt to notice slight hesitancy of speech which may occur within normal 
bounds, and noticing these may accentuate them; but even if there is 
slight hesitancy in articulation, it is very rare to have any such evidence 
of defective memory associated with defective speech as occurs in general 860 
DISEASES OF THE NERVOUS SYSTEM 
paresis. Moreover, the neurasthenic is much more conscious of his 
disturbed condition, and is much more likely to attach importance to it 
than the paretic does. Marked as the difference between the two diseases 
is, every now and then cases will occur, particularly in those who have been 
known to have had syphilis, in which for a period of a few weeks or even 
longer there may be a doubt as to whether neurasthenia or general paresis 
is in process of development. The examination of the cerebrospinal 
fluid and the Wassermann reaction of the blood will give further help. 
The Wassermann reaction of the cerebrospinal fluid is positive in almost 
every case of general paresis. 
The occurrence of epileptiform and apoplectic attacks is of great 
importance in doubtful cases; also when the question arises whether the 
case is one of general paresis or of multiple cerebrospinal sclerosis. The 
writer has seen few cases in which there was any reason to hesitate with 
reference to disseminated sclerosis. The tremor, the scanning speech, 
the marked exaggeration of all the deep reflexes, the nystagmus, are 
symptoms which are never or rarely as marked in general paresis as in 
disseminated sclerosis, nor does the mental condition accompanying 
multiple sclerosis at all suggest dementia paralytica. A difficulty may 
occasionally arise if one sees a patient with disseminated sclerosis in or 
near the terminal stage. In that case, without previous history, a con- 
dition of dementia paralytica might be suspected, but the protracted 
course, the existence of physical signs before the mental symptoms 
developed, and the entire aspect will give the diagnosis. 
Far more difficulty is experienced in attempting to differentiate between 
general paresis and syphilitic pseudo-paresis, or lues cerebri. The difficul- 
ties are increased by the fact that the two conditions are very similar, 
and that even the pathologist cannot easily establish the line of demarca- 
tion. Clinically it may be said that in lues cerebri there is always com- 
plete immobility of the pupils both to light and during accommodation. 
The onset is often preceded by other specific accidents, as previous 
paraplegias or hemiplegias that have ended in more or less recovery. 
The dementia is not as rapidly progressive as in the genuine form of 
dementia paralytica. Above all, we may argue the presence of a lues 
cerebri from the effect of antisyphilitic treatment. This is never, or 
rarely, as prompt in general paresis as it is in this special class of cases. 
After all has been said with regard to this differential diagnosis, the fact 
remains that cerebral syphilis often gives rise to a combination of symp- 
toms which it is impossible to differentiate from genuine dementia 
paralytica. Furthermore, everyone has seen cases which have been diag- 
nosed as syphilitic pseudo-paresis, pass into genuine forms of the disease, 
so that it has been the writer's habit, whenever he has been compelled 
to make the diagnosis of a pseudo-paresis syphilitica or of brain syphilis 
with mental symptoms, to state that the case is more hopeful regarding 
the possible effects of treatment than a typical general paresis would be, 
but that these specific forms often terminate in typical genuine paresis. 
There is also an alcoholic pseudo-paresis which may lead to some 
confusion but the difficulties are not nearly as great as in the case of 
syphilitic disease simulating general paresis. The psychic symptoms SYPHILITIC DISEASES OF CENTRAL NERVOUS SYSTEM 
861 
due to alcoholism may result in a demented condition, but, as a rule, there 
has been a history of delirium tremens, there are very marked delusions 
and hallucinations, and, more than this, there are the distinct physical 
symptoms of alcoholism, and the pupillary reflexes are rarely as much 
involved as they are in syphilis or in general paresis. 
There is also an arteriosclerotic dementia which we see in older persons, 
which may have some resemblance to general paresis, but, first of all, 
the age of the patient argues rather in favor of an arteriosclerotic process 
than general paresis; the dementia is not as rapid and rarely as complete; 
and, as a rule, before any suspicious mental condition is reached there 
has been a history of hemiplegia, transitory attacks of aphasia, vertigo, 
and of numerous other conditions associated with arteriosclerosis. 
After giving due consideration to all these points the fact remains 
that when general paresis is suspected it is present in fully 90 per cent, 
of the cases, and that in the remaining 10 per cent, it will be most difficult 
to state whether or not the diagnosis of general paresis or of some forms 
simulating general paresis must be maintained. 
Treatment.-Granted that in the vast majority of instances general 
paresis is an incurable disease, there is, nevertheless, much to do if the 
patient is to be properly cared for. In the initial stages the proper 
treatment may bring about a cessation of the symptoms or a very marked 
remission, and the patient may be enabled to return to his family and to 
his work for an indefinite period. The first essential is that the patient 
be kept free of all worry and excitement, and that he be subjected to as 
few new mental impressions as possible. To send a paretic travelling 
with an attendant for a change of environment is a grievous error. The 
paretic brain needs rest above all things. Many writers insist that all 
paretics shall be committed at once to institutions for the insane. It is 
well to do this, in view of the fact that the paretic is wholly irresponsible 
and may suffer bodily harm and inflict injury to others. In the crowded 
streets of our cities a man in the intitial stages of general paresis is 
scarcely able to take care of himself properly. The paretic surely needs 
supervision at a very early stage. On the other hand, among the well- 
to-do classes, if a reliable attendant can be secured, it is often feasible 
to retain the paretic in his own home very much longer and with far 
less difficulty than in the case of patients suffering from other forms 
of mental derangement. Other things being equal, the writer cannot 
support the contention that every patient suffering from general paresis 
must at once be confined in an asylum. Each case should be judged 
upon its individual merits. 
The close relationship between syphilitic disease of the brain and 
general paresis, the rapid development of pseudo-paresis in syphilitic 
subjects, make it incumbent in every instance to give the patient the 
benefit of salvarsan treatment with mercurial injections (see page 830). 
If the Wassermann reaction of the blood is positive and if the study 
of the cerebrospinal fluid shows a positive Wassermann reaction and 
a marked increase of the cells, there is every reason to persist in an 
intensive course of antisyphilitic treatment. Every neurologist has 
seen cases in which remarkable remissions have occurred after early 862 
DISEASES OF THE NERVOUS SYSTEM 
salvarsan treatment. The very fact that with the refined methods in 
diagnosis the doubtful cases of general paresis seem to be increasing 
makes it more and more important that active treatment should be 
administered in every case but the physician must exercise judgment 
lest the treatment make further inroads upon a constitution that is 
already wasting away with the disease. To persist in often repeated 
courses of salvarsan injections simply because the Wassermann reaction 
remains positive and the cell-count is high is not the wisest procedure, 
because experience proves that in a very large majority of cases of general 
paresis salvarsan treatment will not be able to change a positive Wasser- 
mann of the blood and of the cerebrospinal fluid to a negative phase, and 
the cell count may not always be diminished. If salvarsan treatment 
cannot be given, mercurial treatment with iodides may be attempted. 
The infraspinous treatment of general paresis with salvarsanized 
serum, whether according to the method of Swift and Ellis or that of 
Ogilvie1 has not yielded satisfactory results so far as the experience of 
the writer goes. If we remember that the spirochetes in general paresis 
are imbedded in the tissue of the cortex and away from bloodvessels or 
lymph spaces, we can understand why this direct application of salvarsan 
can not be as effective as might be expected on theoretical grounds. 
The suggestion has been made to produce artificially an increased pleo- 
cytosis in general paresis and thus to counteract the toxin of the disease, 
basing this on the old experience that remissions in dementia paralytica 
were very apt to occur after febrile and infectious processes. With this 
end in view, injections of tuberculin and of neucleinate of soda have 
been recommended, and while a few writers think they have seen very 
remarkable remissions, the author's experience after repeated trials has 
led him to believe that these methods do not accomplish much more than 
can be effected by judicious intravenous salvarsan treatment. Donath 
insists that there was a marked improvement in 70 per cent, of the cases 
treated by injections of nuclein according to the following formula: 
Nucleinate of soda and chloride of sodium, 2 grammes each, in 100 
grammes of sterile distilled water. This quantity to be injected at one 
or two sittings and to be repeated at intervals of five to seven days. 
In every case there is need of purely symptomatic treatment. The 
ordinary states of excitement may be influenced by the rational use of 
bromides, codeine, and the hydrobromate of hyoscine (gr. by mouth 
or hypodermically). The insomnia, which is very troublesome in many 
cases, is to be combated by the use of bromide, chloral, veronal, trional 
and paraldehyde in the ordinary doses. Mild hydriatic procedures are 
in order, but extreme forms, such as cold spinal douches and the like, 
should be avoided. In the chronic and bedridden cases the function 
of the bladder has to be carefully watched; the tendency to bedsores must 
be carefully considered. If the patient cannot feed himself, the food must 
be administered carefully. In the advanced stages treatment is purely 
symptomatic. The rational medicinal treatment and careful nursing in 
the earlier stages are often followed by encouraging improvement. 
1 Jour. Am. Med. Assn., 1914, Ixiii, 1936. CHAPTER XXIII. 
AMAUROTIC FAMILY IDIOCY (TAY-SACHS DISEASE). 
B. SACHS, M.D. 
The name, amaurotic family idiocy, was applied by the writer to a 
family disease of infancy, characterized by an entire lack of mental 
development, a progressive weakness of all the muscles of the body, 
and rapidly developing blindness, with typical changes in the macula 
lutea. The disease is generally fatal, the patients dying, as a rule, in a 
condition of marasmus before the end of the second year of life. 
In 1881 Waren Tay1 published an article describing "symmetrical 
changes in the region of the yellow spot in each eye of an infant." The 
child was twelve months old. It could not hold up its head or move 
its limbs; there was weakness but not absolute paralysis of any part. 
Its entire brain development was slow and poor. At the first examina- 
tion, the optic disks were apparently healthy, but "in the region of the 
yellow spot of each eye there was a conspicuous, tolerably diffuse, large, 
white spot, more or less circular in outline, and showing at its centre 
a brownish-red, fairly circular spot contrasting strongly with the white 
spot, and this central spot did not look like a hemorrhage, nor as if due to 
a pigment, but seemed a gap in the white patch through which one saw 
healthy structures." Five months later examination showed that the 
disks had become atrophied, but that the changes in the macula lutea 
were the same as before. In the same family, according to Tay's reports, 
three similar cases have occurred, each one of the children presenting 
ocular symptoms and exhibiting physical conditions that were similar 
in all respects, and all three dying before the age of two years. 
The writer,2 in 1887, published the history and the postmortem record 
of a patient who had suffered from a peculiar form of idiocy associated with 
blindness. The family character of the affection was not evident until a 
sister of the first patient, born several years later, had become similarly 
affected. In the course of the next six or seven years the author had 
occasion to see several instances of the same disease and to obtain the 
history of a family in which four children had been afflicted with this 
disease and all had died of it. In 18963 the writer was able to give a list 
of 19 cases, of which 8 had come under personal observation, and by 
that time the chief symptoms of the disease had impressed themselves so 
clearly upon his mind that he felt warranted in giving the disease the name 
Amaurotic Family Idiocy. Since that publication numerous cases have 
1 Tr. Ophth. Soc. of the United Kingdom, 1881, i, 55: also 1884, iv. 
2 Jour. Nerv. and Ment. Dis., 1887, xiv, 541; 1892, xvii, 603; and 1896, xxi, 475 
3 New York Med. Jour., 1896, Ixiii, 697. 
863 864 
DISEASES OF THE NERVOUS SYSTEM 
been described by American and by some European writers. An excellent 
review of the entire subject, with the complete literature1 to 1909, and 
with a careful analytical study of 58 cases, is contained in the work of 
Wilbrandt and Sanger (Die Neurologic des Auges). 
Etiology.-Up to the present time it has been impossible to assign any 
definite reason for the development of this disease in special families. 
In some of those examined by the writer there has been blood relationship 
between the parents, and in one of the families there had been innumerable 
psychoses among the relatives of both parents. Injury to the mother 
during pregnancy has been noted in at least one of the writer's cases. 
The family predisposition is evident from the fact that 28 cases occurred, 
to the writer's knowledge, in fifteen families. Carter was the first to 
call attention to the fact that all of the cases reported had occurred among 
Hebrews,2 and even up to the present day not a single indubitable case 
has occurred among any other people. The racial feature is all the more 
astonishing because other diseases to which it is closely allied, and above 
all the juvenile form of amaurotic family idiocy, have been observed and 
recorded among all races and all nationalities. 
It is important to note that syphilis is not an etiological factor. This 
is of interest not only because other family affections have been attributed 
to syphilitic infection, but also because the disease bears a distinct 
resemblance to specific disorders characterized by various forms of 
dementia and by ocular disturbances (Batten). Moreover, there is a 
hereditary optic nerve atrophy occurring late in life which is supposed to 
be due to hereditary syphilis, and with this optic nerve affection the 
present disease might possibly be confounded. 
Pathology.-In 1887, before I had fully recognized all the character- 
istics of this striking affection, I described the condition as one of arrested 
cortical development and supposed that this was due to what I termed 
an "agenesis corticalis." I was much impressed at the examination of 
the brain of the first patient with the confluence of the central and Sylvian 
fissures and a complete exposure of the island of Reil.3 The brain was 
unusually hard, even in the fresh state, and the knife actually grated on 
removing a small section of the cortex. On microscopic examination 
the most important changes were found in the cortex, in sections taken 
from the frontal lobes, the motor areas, from the base of the third con- 
volution, from the first temporal, and from other parts of the cortex. 
The same changes were found in all the cells. I was struck by the fact 
that hardly a single pyramidal cell presented anything like a normal 
appearance. The contour of the cells was either round or elongated, and 
the cell protoplasm presented every possible change from slight to com- 
plete degeneration. In some cells the nucleus and nucleolus were entirely 
1 Robert Bing, in the Ergebnisse der innere Medizin u. Kinderheilkunde, 1909, iv, 
82, has made a careful study of this disease in connection with other family affections; 
Apert (Sem. med., 1908, p. 25) collected 106 cases; of these, 73 cases occurred in twenty- 
five families. 
2 Bing is in error in restricting the disease to Polish Hebrews. My first two cases 
were of German Hebrew extraction. 
3 I do not attach much importance to the confluence of fissures which I described 
in my first cases. AMAUROTIC EAM1LY IDIOCY 
865 
wanting or were relegated to the margin of the cell. A few years later 
Hirsch found that the same cellular changes occurred not only in the gray 
matter of the cortex, but in the gray matter of the entire central nervous 
system; not only in the cortex of the brain, in the cell ganglia, and in the 
gray matter of the spinal cord, but even in the spinal ganglia. This was 
corroborated by a later examination of one of my own cases,1 and in 1903 
I felt warranted in declaring amaurotic family idiocy to be a disease 
chiefly of the cortex and of the gray matter of the entire central nervous 
system. These findings have been corroborated by all recent investi- 
gators, among whom I wish particularly to mention Spiller, Kingdon, and 
Schaffer.2 The last-named writer has devoted himself to the study of the 
Fig. 48 
Brain cortex cell. Showing degenerating ganglion cells. 
pathology of this disease, and has given a most thorough anatomical 
study of 8 cases. All of his findings have been splendidly corroborated by 
a recent study of my own.3 The findings are so characteristic that the 
diagnosis of amaurotic family idiocy can be made without hesitation 
from an inspection of the microscopic specimens. 
Schaffer pointed out that the chief characteristics are: (1) A widespread 
cytopathological process; an unusual swelling of the cell protoplasm 
and of the dendrites; a swelling of the hyaloplasm, which causes a mechan- 
ical destruction of the cell fibrils; ultimately the cell body is a mass of 
1 Jour. Nerv. and Ment. Dis., 1903, xxx, p. 1. 
2 Zeit. f. die Erforschung. u. Behandlung. d. Jugendl. Schwachsinns, 1909. 
3 Sachs and I. Strauss, Jour. Exper. Med., 1910, xii, 685. 
vol. v-55 866 
DISEASES OF THE NERVOUS SYSTEM 
detritus. Curiously enough, the axis-cylinder is not involved in this 
general swelling. (2) Every cell of the central gray matter, both of the 
brain, the spinal cord, and the spinal ganglia, is similarly affected. 
Histologically there is an unusual swelling of the cell body and a more 
or less complete disintegration of the cell protoplasm. The fibrillae dis- 
appear, so that in many of the cells there are only slight traces left of the 
endocellular network. The fibrils near the periphery of the cell body 
are preserved somewhat longer than are those in the central part of the 
cell mass. The nucleus and nucleolus, if at all preserved, are, as a rule, 
pushed nearer to the periphery of the cell body, and as the disintegration 
Fig. 49 
Ganglion cells of the anterior horn of the cord. Showing the excentric nucleus surrounded by the 
remains of the chromatophilic substance and the marked increase in size due to degeneration of the 
cytoplasm. 
of the mass goes on the nucleus and nucleolus also disappear. A pro- 
liferation of the glia, particularly in the cortex, with the development of 
giant glia cells, has been noted. There is no evidence of any inflammatory 
process. Changes in the white fibres are unimportant and unessential. 
It is Schaffer's merit to have laid stress upon the swelling of the dendrites, 
which appears to be so constant as to be almost pathognomonic. The 
deterioration of the cell body and the swelling of these cell processes, 
exclusive of the axone, have been noted in all of Schaffer's and in my own 
recent case. 
Holden, as well as Schumway and Mary Buchanan, made careful 
histological examinations of the eyes. All these writers agree that the AMAUROTIC FAMILY IDIOCY 
867 
essential changes are a degeneration of the ganglion cells of the retina and 
of the nerve fibres of the optic nerves and tracts. They also believe that 
the white area in the fundus is the result of the swollen and degenerated 
ganglion cells, which are present in much greater numbers in the macular 
region than elsewhere, and do not consider this white area to be due to 
oedema of the tissue as was first supposed. 
Pathogenesis.-Writers on this disease have been divided into two 
groups. Some, among whom the writer wishes to class himself, have 
maintained that amaurotic family idiocy is, in a sense, a congenital disease, 
even if it is not manifest at the time of birth. The tendency to the 
Fig. 50 
Purkinje cell. Showing the swollen dendrites. 
disease is unquestionably born with the child. In the writer's opinion 
children afflicted with amaurotic family idiocy are possessed of a nervous 
system so inadequate to the demands imposed upon it that its cells, 
after having performed their function for a few weeks or months, undergo 
complete disintegration. In other words, these children have a very 
limited capacity for normal development. Their central nervous system 
is not equal to the functions that they are expected to perform for more 
than the first three or four months of life. 
Even Schaffer, who was inclined for a time to class amaurotic family 
idiocy among acquired diseases, concedes that the entire nervous system 
of such a child is so delicately organized that it is not able to repair the 
ordinary physiological waste of its cells. The nerve cells are so easily 868 
DISEASES OF THE NERVOUS SYSTEM 
exhausted that they undergo a progressive deterioration, which leads to 
the death of the ganglion cell with a consecutive hyperplasia of the glia 
substance. These views are so thoroughly in accord with those of the 
writer that there seems no radical difference between them. Only a 
few authors now adhere to the view which was held by Hirsch, that the 
disease is altogether an acquired condition and that it is possibly due to 
some toxic agent. Hirsch believes that it might be due to some toxic 
substance in the mother's milk. From the very outset I w'as loath to 
accept the explanation of a toxic origin for any profound family disease, 
and in the case of these amaurotic children Hirsch's view falls to the 
ground because several of those seen by the writer were not nursed by 
Fig. 51 
Large anterior horn cell. Showing very well-preserved endocellular network and swelling of dendrites. 
The fibrils have commenced to disappear in the dendrites. (Bielschowsky.) 
their own mothers, but by wet-nurses of a different race and different 
nationality. However, a child born with a defective nervous system 
may be more subject to toxic influences than normal children. 
Symptoms.-The children with this disease have been born at full 
term and apparently in perfect health. Difficult labor or instrumental 
delivery has no bearing upon its development. The children do well 
during the first three to six months of life, when they become listless and 
apathetic, move their limbs very little, show no interest in their surround- 
ings and show the first signs of visual disturbance which ultimately 
leads to blindness. As time goes on, the child is unable to hold up its 
head or to sit up. Its muscles are generally flaccid, rarely spastic. The 
reflexes may be normal, a trifle subnormal, or somewhat exaggerated, but AMAUROTIC FAMILY IDIOCY 
869 
marked spasticity, such as we find in the spastic diplegias of childhood, 
is not common. In some instances there is an unusual sensitiveness to 
touch and to sound (hyperacusis), the child being startled by the slightest 
noise. Convulsions occur in some cases, but are not a frequent or 
integral symptom. All the functions of the body are in a low state of 
activity. The children are subject to frequent bronchial attacks, and 
often show gastro-intestinal disturbances. An examination of the fundus 
reveals the peculiar condition described by Tay. The "cherry-red spot 
in the region of the macula lutea" has become a familiar phenomenon to 
those who are on the lookout for this disease. There is a gradual increase 
of all the symptoms, the mental defect becomes more and more noticeable, 
the palsy more extreme, complete blindness follows, and the child lapses 
into a condition of marasmus in which it dies. 
To sum up briefly, the chief symptoms are: (1) A mental impairment 
during the first months of life leading to absolute idiocy. (2) Paresis or 
paralysis of the greater part of the body, which may be either flaccid or 
spastic. (3) The reflexes may be normal, deficient, or increased. (4) 
A diminution of vision terminating in absolute blindness (the cherry-red 
spot in the region of the macula lutea and later a simple optic nerve 
atrophy). (5) Marasmus and a fatal termination, as a rule, before the 
age of two years. (6) The occurrence of the affection in several members 
of the same family. 
In some, but not in all of the cases, nystagmus, strabismus, hyperacusis, 
and convulsions may be added to the above cardinal symptoms. A loss 
of the sense of hearing was noted in a few cases. Falkenheim referred to 
"explosive laughter," but the writer has not been able to corroborate this 
finding. If we allow for slight variations, it must be said that there are 
few diseases as uniform in their clinical manifestations as this. In a 
few cases, such as the one reported by Koller, the optic nerve changes 
preceded the characteristic changes in the macula lutea, and in one 
of Higier's cases the optic nerve atrophy was much more marked than the 
changes in the macula lutea. 
Diagnosis.-The clinical symptoms are so distinct that a physician 
who is acquainted with the disease can hardly fail to make the diagnosis. 
The recognition must depend upon the concurrence of the various symp- 
toms. Thus, there are innumerable forms of brain defect that do not 
belong to this category. There are also disturbances of vision, often 
syphilitic and of congenital origin, that have nothing to do with amaurotic 
family idiocy. But if a child that appears to have been well at birth 
loses its vision in the course of the first six months of life, if the child 
becomes listless, and if, on examination, the "cherry-red spot" can 
be found, there can be no doubt whatever about the disease. Some 
difficulty will occasionally be experienced in differentiating between 
amaurotic family idiocy and the congenital diplegias and paraplegias, 
but the preservation of considerable intelligence and the presence of 
normal vision ought to be sufficient to prevent mistakes. Children 
suffering from amaurotic family idiocy may have either flaccid or spastic 
limbs. On the whole, the flaccid limbs are fully as common. It is 
necessary to insist upon this since the writer has found that others seem to 870 
DISEASES OF THE NERVOUS SYSTEM 
consider spasticity a regular feature of the disease, which it surely is not. 
It is important in every suspected case to inquire into the character of the 
mother's labor at the time the child was born. Difficulties in labor have 
nothing to do with amaurotic family idiocy, but are commonly associated 
with congenital spastic palsies and with various forms of congenital 
idiocy and epilepsy. Convulsions may occur in amaurotic family idiocy, 
but do not constitute an integral symptom of the disease. 
It is not always easy to distinguish syphilitic cerebral disease from 
amaurotic family idiocy, and if several members of the same family 
are afflicted, as they well might be in the case of an hereditary syphilitic 
disease, the difficulties become still greater. 
Amaurotic family idiocy as described by the present writer is to be 
differentiated' from the juvenile form to which Spielmeyer1 and Vogt2 
called special attention. This bears a close resemblance in many respects 
to the Tay-Sachs (infantile) type, but it occurs much later in life, often 
not appearing until the eighth, tenth, or twelfth year, is associated with 
blindness (without the changes in the macula), and leads to a fatal 
termination after a period of a few years. The writer has been struck 
by the resemblance between these two forms, and will not deny that they 
are akin to one another, without being identical. This kinship must also 
be upheld in view of Schaffer's investigations, which reveal a great 
resemblance between the histological findings of the juvenile and the 
infantile forms of amaurotic family idiocy. Such differences in histo- 
logical structure as do occur might be due to the fact that the juvenile 
form represents a less violent process, attacking a brain farther advanced 
in development than the infantile brain of typical amaurotic family 
idiocy. While I would not deny that these two forms are somewhat 
related to one another, I cannot consider them identical. 
Treatment.-Under this heading there is little to be said. As in the 
case of other family affections, we have no means of staying the disease. 
I have been asked by the parents of these "amaurotic" children, whether 
the birth of normal children can be expected. In several of the families 
that I have seen, normal children have grown up side by side with the 
unfortunate victims of the disease. In such families it has not been 
restricted to one sex or the other. From my own experience I can only 
advise that the dangers of blood relationship be seriously considered; 
that marriage between persons closely related be discouraged; and 
marriages between persons in whose families there are marked neurotic 
or psychical taints are also undesirable. If the disease has made its 
appearance in the child, purely symptomatic treatment is the only 
kind that can be adopted. 
1 Neurol. Centralbl., 1906, xxv, 51; also Monograph Gotha, 1907 
2 Monalsschr. f. Psych, u. Neurol., 1906, xviii, 161. PART II. 
DISEASES OF THE LOCOMOTOR SYSTEM. 
CHAPTER XXIV. 
MYOSITIS-MYOTONIA CONGENITA: THOMSEN'S DISEASE. 
WALTER R. STEINER, M.D. 
MYOSITIS. 
Definition.-Inflammation of the muscles. The voluntary muscles 
are, generally, alone involved, although the heart muscle may become 
affected, especially in the variety known as polymyositis hemorrhagica. 
Myositis has long been recognized as occurring independently or in 
connection with certain diseases. Its classification presents considerable 
difficulty, for our knowledge of this subject is in a somewhat chaotic state. 
Dujardin-Beaumetz and others have endeavored to divide myositis into 
the acute and chronic varieties, which would greatly simplify matters. 
But unfortunately, as Lorenz pointed out, each variety is subdivided into 
acute, subacute, and chronic types, although the syphilitic and tubercu- 
lous forms have an especially chronic character. At present. Lorenz's 
division seems the most preferable, but we should remember that this 
classification may be only applicable for clinical convenience. 
We shall limit ourselves to the consideration of the primary myositides. 
The examples of myositis found in pyemia, ulcerative endocarditis, 
actinomycosis, erysipelas, gonorrhoea, and other infectious diseases result 
from secondary processes. In tuberculosis, the so-called primary or 
hematogenous form is seen when the infection is carried to the muscles 
by the blood stream from a distant focus, and is not due to an extension 
from contiguous tissues. The secondary form is much the more frequent 
here, as Zeller,1 after a careful search, could only collect thirteen typical 
cases of the former from the literature on this subject, and add two 
personal observations. Later, Kaiser2 added six to this number and 
recently other investigators have increased the list to twenty-one cases.3 
1 Beitr. z. klin. Chir., 1903, xxxix, 633. 2 Arch. f. klin. Chir., 1907, Ixxvii, 1033. 
3 For a further consideration of this form see Dausel Ernst, Ueber primare Muskel- 
tuberkulose, Berlin, 1912. 
871 872 
DISEASES OF THE LOCOMOTOR SYSTEM 
PRIMARY SUPPURATIVE MYOSITIS. 
Definition.-A single or multiple muscle inflammation, mostly acute, 
of bacterial origin, presenting clinically the picture of an acute infectious 
disease and generally ending in suppuration. 
Etiology.-Suard was the first to conjecture that the real cause of 
this disease was of infectious origin, which Scriba1 and Brunon2 later 
proved by finding micro-organisms in the pus from the muscle abscesses. 
The results of the bacteriological labors of these observers were described 
by them in subsequent communications, but Scriba's work, appearing 
only in Japanese medical literature, has been pretty generally.overlooked. 
The latter observed the Staphylococcus pyogenes aureus, while Brunon 
found streptococci, staphylococci, and an undifferentiated bacillus, the 
streptococci predominating. Brunon consequently thought that the 
disease was not due to a fixed variety of bacteria, but to any kind of pus- 
producing micro-organism. Other investigators, as Lorenz,3 Honsell, 
Kojima, and Araki,4 and Ito and Sinnaka,4 have sought for bacteria in 
their cases, and have found, in every instance, the Staphylococcus pyogenes 
aureus. Miyake in a study of 33 cases obtained a pure culture of this 
organism in 27. Sato5 twice obtained a mixed culture of staphylococci, 
streptococci, and an encapsulated diplococcus, resembling the pneumo- 
coccus; in another case, he cultivated staphylococci and streptococci. 
Various portals of entry for the bacteria may be seen, such as small 
infected wounds, furuncles, or acne pustules, but, as Miyake states, they 
cannot be considered as the original foci of infection unless the bacteria 
they contain are similar to those found in the muscle abscesses. Occasion- 
ally the mucous membranes, apparently, permit the infectious organisms 
to gain extrance. From these different foci they are carried to the muscles 
by the blood stream, so the disease should be considered as a septico- 
pyemic infection, with the abscess formation limited to the muscles, and 
the only reported blood culture confirms this theory. As muscles are 
very resistant to inflammation, the presence of a locus minoris resistentice 
is assumed, and various predisposing factors have been assigned. 
Age appears to have no relation to its occurrence, although it was 
formerly thought that the disease was more frequent in children. In 
Miyake's series, 18 were under and 14 were over twenty-five years of age. 
The sex bears no direct influence but it is seen more commonly in men, 
as in them the predisposing causes of trauma and overexertion are more 
frequently observed. In Miyake's series, 21 were men and 12 were 
women. In Ito and Sinnaka's list of 10 cases, 7 were men and 3 were 
women. It is more common in the laboring classes. 
Season.-Some writers have thought the time of year, which has been 
variously given, had something to do with the onset. Fujiy claims it is 
most frequent when the laboring classes work in the fields and are sub- 
jected to the predisposing causes of cold and fatigue. 
1 Miyake, Mitth. a.d. Grenzgeb. d. Med. u. Chir., 1904, xiii, 153 (Bibliography). 
2 Contribution a I'Etude de la Myosite infectieuse primitive, Paris, 1887. 
3 Die Muskelerkrankungen, Vienna, 1898. 156. 
4 See Miyake, op. cit. 5 Deut. Zeitschr. f. Chir., 1903, Ixix, 302. MYOSITIS 
873 
Pathology.-Three varieties of suppurative myositis have been recog- 
nized: (1) With large solitary abscesses; (2) with disseminated abscesses, 
and (3) with diffuse purulent infiltration. 
In primary suppurative myositis, most of the reported cases can be 
classed under the first heading. The skin and underlying connective 
tissue over the affected muscle or muscles are generally normal in appear- 
ance. The muscle itself is of a dark red to grayish red color, densely 
infiltrated with serum and very friable. The abscess, situated in the 
muscle, contains thick, greenish-yellow pus, mixed with blood and tissue 
particles. Its walls are lined with gray or yellowish necrotic fibres. In 
the more severe cases, where the abscess has invaded the whole muscle, 
only a few shreads of muscle tissue may be seen, hanging from the sar- 
colemma sheath (Chassaignac's case). Occasionally the muscle affected 
is described as pale, like the muscles of a fish, and exhibiting yellowish 
lines or streaks. It may also be bluish red in color, depending upon the 
degree and duration of the inflammation. 
Microscopically the findings have varied somewhat. They are of a 
serous, seropurulent, and purulent character. Scriba declared that the 
interstitial tissue was not altered, but Miyake examined the muscle 
tissue in 14 of his 33 cases and observed the interstitial tissue markedly 
increased in the purulent cases and infiltrated with mononuclear and 
polymorphonuclear cells. Occasionally this tissue had completely 
replaced the muscle fibres. 
The different investigators agree in the main on the muscle changes, 
but not upon their extent. Miyake found most of the fibres in his cases 
unaltered, while Scriba described the majority as swollen and showing 
a partial or entire loss of cross-striation. At times irregular cross- 
striations were noted. The sarcolemma sheath was much thickened and 
contained cells of varying sizes and shapes, while the perimysium was 
slightly infiltrated with small round cells. Hyaline, granular, and fatty 
degeneration was not noted by Miyake. Lorenz has described vacuclic 
degeneration. About the abscess walls there is a marked round-cell 
infiltration, and the muscle fibres are here almost completely destroyed. 
Farther out they are readily seen, but they are shrivelled and without 
cross-striation. The muscle nuclei are generally somewhat increased. 
By the Gram stain, bacteria are found in clumps in the vicinity of the 
abscess wall, but are not seen in localities where the inflammatory process 
is less pronounced. In advanced cases there is an extensive amount 
of granulation tissue and a new formation of muscle fibres. 
Symptoms.-The onset is usually sudden, and most of those attacked 
have previously been in good health. A chill, followed by high fever, is 
generally the first symptom noted, but the patient may also complain 
of anorexia, general malaise, headache, profuse perspiration, and pains 
in the extremities. The pain is at first ill-defined but soon becomes 
localized in the muscles, which are very painful. On examination, one 
finds a tender, indurated swelling, which conforms to the shape of the 
muscle and is freely movable. Scriba describes the swelling as of a hard, 
board-like character. If it involves the long muscles, it is spindle-shaped 
in appearance, but if the broad muscles are attacked, it is hemispherical. 874 
DISEASES OF THE LOCOMOTOR SYSTEM 
The muscle itself is contracted, and active and passive movements are 
much limited on account of pain. The skin over the affected muscle is 
hot but generally not reddened. There may be some oedema of the 
underlying tissues. At times an extensive erythema or ecchymosis is 
noted. The induration may be limited to only a portion of the muscle, 
or even its whole extent may be involved. Subsequently, within four 
to ten days, the swelling becomes softer and gives signs of evident fluctua- 
tion. In one case, however, this was delayed for thirty-one days. On 
incision, a muscle abscess is found and a thick, greenish-yellow pus, 
slightly mixed with blood, is evacuated. If the incision is long delayed 
or neglected, the pus may penetrate the muscle sheath and infiltrate the 
surrounding tissues, leading to the formation of multiple abscesses in 
different parts of the body, and finally result in death (Scriba). Rarely 
the stage of induration ends in resolution. 
One or many muscles may be attacked. If multiple, it appears in 
two forms: (1) The so-called typical form, which is multiple from the 
onset; (2) the atypical form, which begins as an isolated focus of infection 
and later becomes multiple (Miyake). 
Recurrences may take place. Two cases have been reported in which 
this happened after an interval of three and four months. The similarity 
between this disease and osteomyelitis, both clinically and bacterio- 
logically, has been especially considered by Walther and Miyake. Brunon 
divides the cases into three types, viz.: (1) Malignant, resulting in death 
in a few days; (2) acute, in which recovery may ensue; and (3) subacute, 
a very light form. Some of his cases belong to other diseases, and a 
consideration of the typical instances of this affection, thus far reported, 
leads us to place them under two headings, the acute and subacute. 
Diagnosis.-If the disease is seen from its outset, this is generally 
relatively simple, provided the following two points be considered: (1) 
The dense infiltration and swelling of the muscle; (2) the painful condition 
of the affected muscle, which is kept firmly contracted, and causes the 
extremity, or part of the body implicated, to assume various positions. 
If, for instance, the biceps brachii is attacked the arm will be flexed, or, 
if the rectus abdominis muscle be involved the body will be inclined 
forward. In each case the insertions of the muscles are more closely 
approximated, to lessen the pain which tension would increase. Occa- 
sionally osteomyelitis is simulated, especially in those cases in which the 
muscle sheaths of the implicated muscles have ruptured. In the latter, 
the disease rarely involves the surrounding tissues, but is seen extending 
to other parts of the body by metastatic foci. In doubtful cases, a wide 
surgical incision, combined with an inspection and palpation of the 
abscess cavity will definitely differentiate the two conditions. Metastatic 
abscesses in pyemia will at times need to be distinguished from this 
malady. In the former there is usually no dense infiltration of the 
muscle or muscles, or, if present, it is extremely slight. The local 
disturbances are absent or so trivial that the patient does not detect them. 
Prognosis.-All uncomplicated cases have a favorable prognosis 
if seen early enough for the requisite surgical treatment. In Miyake's 
series of 33 cases there was only one death, and this was from a complica- MYOSITIS 
875 
tion. After the pus is emptied from the muscle abscesses, the acute 
inflammatory symptoms subside very promptly, and complete healing 
takes place in the affected muscles, after an interval of from one and a 
half to three months. Rarely, the loss of muscle substance is replaced 
by scar tissue, which may cause severe contractures of the muscles. In 
cases in which early surgical treatment has been neglected, if the pus 
penetrates the muscle sheath and forms metastatic foci in other parts 
of the body, the prognosis becomes very grave. Muscle atrophy may 
follow the healing of the abscesses, but there is generally no subsequent 
functional disturbance. 
The complications to be dreaded are pyemia and pneumonia. Sato, 
Kurosawa, and Suzoki have reported cases in which a metastatic lung 
abscess was the cause of death. In Walther's case death ensued in the 
ninth week from an intercurrent endomyocarditis. 
Treatment.-By the use of cold packs and other antiphlogistic means, 
the muscle induration may not undergo suppuration, and absorption may 
result. If fluctuation does occur, an early broad incision should be made 
and the pus thoroughly evacuated. It should be remembered that a 
previous negative exploratory puncture does not exclude the presence of 
pus, which may have been too thick to flow through the needle. Fujiy 
and Honsell oppose surgical procedures unless pus is surely present, but 
in case of doubt it is better to err on the side of caution and open the 
indurated areas widely. If, after the myositis has subsided, contractures 
ensue, massage and various orthopedic measures should be employed. 
NON-SUPPURATIVE MYOSITIS. 
Dermatomyositis.-Definition.-An acute, subacute, or chronic disease, 
of unknown origin, characterized generally by a gradual onset, with 
vague and indefinite prodromes, followed by oedema, dermatitis, and a 
multiple muscle inflammation. 
Etiology.-We are much in the dark as to the cause, although recent 
findings point more strongly to its infectious origin. Hepp and others 
have previously emphasized this view, and the presence of a splenic 
tumor, fever, and angina have seemed to support it. In most of the recent 
cases bacteria have been found, organisms similar to staphylococci 
being described but these cases appear generally atypical. Senator 
advanced a toxic theory, which has, at present, no supporters. 
Distribution.-Cases have been reported in the United States and in a 
number of European countries. 
Race.-The Anglo-Germanic has furnished most of the instances, 
followed by the Latin and Scandinavian races. The case reported by 
the writer is the only one yet observed in the negro.1 
Season.-The time of year seems to have no connection with the disease. 
In the writer's collected series, 10 were attacked in the winter, 5 in the 
spring, 8 in the summer, and 5 in the autumn. 
1 Jour. Exper. Med., 1903, vi, 407 (Bibliography). 876 
DISEASES OF THE LOCOMOTOR SYSTEM 
Sex.-The affection has been observed, according to this series, in 17 
males and 11 females. 
Pathology.--Of the 17 fatal cases in this series, autopsies have been 
performed in all but 7, and in 4 of these the excision of a piece of muscle 
was allowed. We have, consequently, a fair amount of data on which 
to base conclusions. With the exception of an enlarged and soft spleen, 
the pathological findings are limited to the muscles. Any or all may be 
attacked. It was early stated that the muscles of the eye, tongue, heart, 
and diaphragm were exempt, but later investigation has shown they may 
become implicated. Lorenz1 declares the implication of the heart has a 
deeper significance than was at first imagined. The cases presenting 
this complication show a special tendency to relapses, although mild and 
abortive at first. 
The skin covering the muscles is firm and hard and generally does 
not pit on pressure, although oedema is usually present, but may be slight. 
On section the subcutaneous tissues present a firm, tense oedema, and are, 
as a rule, infiltrated with a yellowish, serous fluid. The muscles exhibit 
extensive changes; they are swollen, pale red or pale yellow in color, or 
may reveal, occasionally, yellowish-gray or diffuse reddish streaks. Hepp 
considers the muscles to resemble those of a dog. They are often strongly 
infiltrated with serum and quite moist. In consistency they vary, 
being hard and firm, or soft and boggy. They may be quite friable, as 
in Hepp's case, in which the left rectus muscle was found ruptured at 
autopsy. They are without lustre and of a dull, opaque appearance. 
Hemorrhages may occasionally be seen in them. Microscopically the 
changes are those of a parenchymatous and interstitial inflammation, 
and may vary in extent, being either focal or diffuse. Again, the different 
muscle changes may be seen, occurring in one and the same fibre. These 
are frequently separated from one another by the existing oedema or by 
mononuclear and polymorphonuclear leukocytes. Small hemorrhages may 
also be seen between them. The fibres are found in all stages of degenera- 
tion; they may be normal in size, oedematous or atrophied, coarsely or 
finely granular, hyaline or waxy, occasionally fatty. The striae are 
normal, indistinct or invisible. Longitudinal or cross-cleavage of the 
fibres has been found, and vacuoles have been described in four instances. 
In many cases there is an increase in the number of muscle nuclei. Typi- 
cal interstitial foci of small round cells are found in the perivascular 
connective tissue and, to a lesser extent, between the muscles. In the 
subacute and chronic cases the increase in connective tissue may be 
marked in both the perimysium externum and internum, and solitary 
muscle fibres in the process of degeneration may be surrounded on all 
sides by connective tissue. In five cases the bloodvessels were somewhat 
dilated and filled with blood. In seven of the autopsies, broncho- 
pneumonia was found as the terminal infection. 
Symptoms.-The disease generally attacks persons in the prime of 
life and in the best of health. Fuckel's case, however, was noted in a 
girl after an attack of measles, and both of Wagner's cases had pulmonary 
1 Berl. klin. Woch., 1906, xliii, 727, and Deut. med. Woch., 1906, xxxii, 777. MYOSITIS 
877 
tuberculosis, the second patient having tuberculous ulcers of the intestines. 
Senator's second case was a diabetic. The onset is almost always 
gradual, but may be sudden, with the prodromal symptoms of malaise, 
weakness, anorexia, pains in the extremities, or headache. These symp- 
toms may be of several days' to three weeks' duration, or even longer, 
as in one case. Occasionally they are absent. 
Pain.-Vague pains are next complained of, as well as a stiffness or 
rigidity in the extremities and back. These pains quickly take on a more 
definite character, become more or less circumscribed, and are localized 
in the muscles. Different muscle groups are then successively attacked, 
and eventually the whole skeleton musculature may be implicated. 
Later in the disease the pains become more severe, and are not only 
spontaneous in origin, but also are caused by active and passive move- 
ments. They are described as drawing, tearing, or boring in nature. 
In the most severe cases the patients lie utterly helpless in bed, as if 
completely paralyzed. The muscles involved are generally very painful 
on palpation, although the oedema does not cause them to be well defined. 
Muscle contractures have been observed in a few cases. 
Fever is soon noted. It is usually of moderate intensity and inter- 
mittent or remittent in type. It rarely exceeds 104°, but just before 
death may rise several degrees above this height. 
CEdema.-This appears with the fever and may implicate the whole 
body and extremities, the latter presenting at times a most ungainly 
appearance. It is generally first seen on the face, especially above the 
eyelids, and may cause the countenance to assume an immobile aspect, 
likened by Oppenheim to alabaster. The swelling is, as a rule, always 
noted in the extremities, being apt to involve the proximal parts, e. g., 
the shoulder, upper arm, elbow, thigh, or inguinal region. It is not a 
symmetrical oedema, as the flexor or extensor surfaces are generally 
alone involved. It also varies greatly in character, as at one time it 
may pit on pressure, while at another a dense, hard infiltration of the 
skin is observed, the latter being the more usual. After the skin inflam- 
mation is noticed, it becomes more intense, and may remain localized 
over the affected muscles or spread to surrounding parts. Usually its 
duration is limited to the acute stage of the disease. The wrist- and 
ankle-joints are generally spared. 
Dermatitis.-This is an early symptom, and varies greatly in character, 
being in different cases an erythema, a pseudo-erysipelas, an urticaria, a 
roseola, an eczema, or an inflammation resembling erythema nodosum. 
It may spread continuously or remain limited to the parts where it was 
first observed. At times it occurs only later in the disease. Its location 
is apt to be over the diseased muscles. It may disappear without leaving 
a trace behind, or a pigmentation may indicate its former presence. 
In two instances, a dermatitis of a different type followed the disappear- 
ance of the first eruption. 
Profuse perspiration and an enlarged spleen usually accompany the 
other signs and symptoms. 
Nervous System.-No disturbances of sensation are, as a rule, met 
with, and the nerves are not tender on palpation. Hepp refers to peculiar 878 
DISEASES OF THE LOCOMOTOR SYSTEM 
cramp-like pains in the later stages of the disease, of variable duration, 
and Wagner, Lewy, Lorenz, and Leube have noticed paresthesia during 
the course. This, with the cramp-like pains, was especially marked in 
the writer's case, in which the patient complained of a sensation as if 
something was crawling down from the elbows of each arm to the fingers; 
it was particularly noticeable at night. The knee-jerks and the electrical 
reactions are usually either normal or diminished. In the mild cases the 
knee-jerks may be slightly increased, and, in the most severe instances, 
they may be totally abolished. Oppenheim states that there is, generally, 
a quantitative decrease in the electrical excitability of the muscles, and 
Lewy got a partial reaction of degeneration in his case. 
Stomatitis and angina are at times seen, either early or late in the 
disease. In Lewy's case and in four of Oppenheim's, ulceration of the 
mucous membrane was observed. On this account the latter investigator 
has coined the word dermato-mucoso-myositis, to name this disease. 
The extension of the affection to the muscles of respiration and degluti- 
tion was formerly considered the rule, and Lowenfeld names this as one 
of the three cardinal symptoms. It well accounts for the number of 
fatal cases reported, due to suffocation or bronchopneumonia. The 
urine is usually normal, but may contain albumin, and hyaline and 
granular casts may be found in the urinary sediment. The course may 
be characterized by improvement and relapses in the subacute and 
chronic cases, in which atrophy of the muscles may be observed. The 
acute cases last from one or two weeks to two months; the subacute, two 
to eight months, and the chronic forms from a year and a half to two 
years (Martini, Lorenz). 
Diagnosis.-As a rule, no difficulty will be experienced in typical cases. 
Diseases presenting somewhat similar symptoms are: (1) Trichinosis, 
(2) neuromyositis, (3) primary suppurative myositis, and (4) syphilitic 
myositis. In the first, the initial gastro-intestinal disturbances and the 
discovery of trichime in the stools and excised muscles; in the second, 
the chain of nervous phenomena and the absence of a dermatitis; in the 
third, the presence of a focus of infection with the bacteriological report 
on the muscle examination; and in the fourth, the history of the patient 
and the objective findings, will generally sufficiently differentiate the 
affection. It is also well to bear in mind that one of Oppenheim's cases 
turned out, four years later, to be a typical example of scleroderma, 
while another in his series was so diagnosed by the physician who first 
attended him. . 
Prognosis.-As all the muscles of the body may be implicated, including 
those of respiration and deglutition, death may result from suffocation 
or bronchopneumonia. The early cases of this disease were almost 
invariably fatal, but some of those reported later have resulted in recovery. 
Of the 28 cases collected by the writer, 17 terminated fatally, so the prog- 
nosis is always grave. The outcome was fatal for the two patients in 
both extremes of life. 
Treatment.-This should be chiefly directed to the relief of pain and to 
the keeping up of the patient's nutrition. Various analgesics have been 
tried for the former object, and among them we may mention aspirin 879 
MYOSITIS 
and the salicylates, but no particular drug has as yet been found to offer 
any especial advantage. In severe cases, morphine has sometimes been 
necessary, to quiet the pain. Oppenheim employs hot air, the use of 
hot drinks and aspirin, while every second day he endeavors to cause the 
patient to perspire profusely. At the onset, or later, he uses thermo- 
massage, and subsequently massage, exercises, and electrotherapy. 
Polymyositis Hemorrhagica. - Definition. - An acute, subacute, or 
chronic disease, of unknown origin, strongly resembling dermatomyositis, 
but differing from it chiefly in the presence of a greater or less amount of 
interstitial hemorrhage between the muscles and the occurrence of 
circulatory symptoms, caused by the implication of the cardiac muscle. 
Etiology.-This is quite obscure, but staphylococci were found in two 
instances. In two cases, cold and fatigue seemed to bear some relation 
to the onset; in another case, angina was the first symptom, and the disease 
was thought to be a general infection following it. In still another, there 
was some preceding inflammation of the tissues of the neck. 
Pathology.-The findings vary from simple hemorrhages between 
unchanged muscle fibres to extensive muscle degenerations and new 
connective-tissue formation. Lorenz has divided the changes into the 
acute and chronic forms. The first is characterized by intramus.cular 
hemorrhages, which cause destruction of the fibres, as well as the many 
muscle degenerations described under dermatomyositis. The second 
form is distinguished by the presence of connective tissue containing 
blood pigment and markedly atrophic fibres. For a fuller discussion 
of the pathology, one is referred to the article on Dermatomyositis. 
Symptoms.-These described under dermatomyositis may be well used 
here, although in polymyositis hemorrhagica, the usual absence of 
prodromal symptoms, a more sudden onset, and the occurrence of less 
fever are seen. Pain is, generally, the first symptom noted; it is definitely 
located in a small circumscribed area in the muscles, where, later, a 
nodular tumor, tender on palpation, develops. The extremities are 
usually first attacked. The condition is accompanied by more or less 
oedema, which varies greatly in its characteristics, for it may be as 
extensive as in dermatomyositis, but frequently it is more circumscribed 
and of softer consistency. Different muscle groups are gradually attacked, 
and the whole skeleton musculature may be finally involved. The skin 
may show a hemorrhagic or a measly eruption. The former is revealed 
by the presence of violaceous and purpuric spots, which, when fading, 
leave behind a yellowish-green discoloration. Pigmentation may eventu- 
ally be noted. Prinzing's1 case exhibited well-marked muscle atrophy. 
Circulatory symptoms have been described in every case save one, due to 
the implication of the cardiac muscle. They are cardiac palpitation, 
tachycardia, arhythmia, and, more rarely, murmurs; all of which account 
for the cardiac weakness and collapse so frequently observed. An enlarged 
spleen is an inconstant symptom as well as hemorrhage, apart from that 
into the muscles and skin. Bleeding from the intestines has been 
described, and attacks of epistaxis, hematuria, and profuse menstrual 
1 Munch, med. Woch., 1890, xxxvii, 846. 880 
DISEASES OF THE LOCOMOTOR SYSTEM 
flow. Nephritis is a frequent complication. The disease is generally 
of several months' duration. Fenoglio's1 patient lived on for a year and 
a half, finally dying of pulmonary tuberculosis. 
Diagnosis.-The sudden onset, the moderate fever, the character of the 
skin eruption, the cardiac involvement, and the muscle findings will 
readily cause it to be distinguished from dermatomyositis or other 
seemingly similar affections. 
Prognosis.-Four out of the ten cases collected by Thayer recovered,2 
so that the prognosis is about as grave as in dermatomyositis. 
Treatment.-See Dermatomyositis. 
Neuromyositis.-This term has been applied in about ten instances 
to cases which present the signs and symptoms of a myositis associated 
with those of neuritis. There seems to be some uncertainty as to whether 
the disease exists per se or whether the co-existence of two diseases is 
here observed. It is usually seen in chronic alcoholics and accompanied 
by ataxia. 
MYOSITIS WITH SPECIAL TERMINAL LESIONS. 
Primary Myositis Fibrosa.-Definition.-A single or multiple inflamma- 
tion of the muscles, mostly subacute or chronic, which generally begins 
in the lower extremities, and presents but slight constitutional symptoms. 
Eventually the muscle tissue concerned is largely or wholly replaced 
by connective tissue, and quite pronounced muscle atrophy may be then 
observed. 
Etiology.-There has been considerable discussion whether this disease 
in its primary form is a definite entity. Some have claimed that it is 
only seen secondary to infectious or rheumatic conditions, but there seem 
to be good grounds for considering its independent existence as established. 
Cultures from the tissues and search for micro-organisms in sections have 
been uniformly negative. 
Pathology.-The changes in the muscles are seen in two forms. In the 
first the implicated muscles are swollen and present to view a tumor-like 
mass. On palpation, they are firm and hard. The skin and underlying 
tissues may be involved also, and then a dense oedema appears which 
does not pit on pressure. A brown pigmentation of the skin has been at 
times observed. In the other form, the muscle is changed into a dense, 
tendinous band. On section the affected muscles grate under the knife 
and reveal a hard white surface in the most involved areas, but. in the 
less affected portions show reddish-yellow spots, which represent the 
remains of some of the muscle fibres. Microscopically there is a great 
increase in the interstitial tissue, which, in places, entirely replaces the 
muscle fibres. In other portions the fibres are atrophied and show a 
granular or fatty degeneration. Their cross-striations have disappeared, 
but their longitudinal striations are more evident. A relative increase 
of tendinous tissue is at times seen. 
1 Rivisla clin. Arch. ital. di klin. med., 1890, p. 497. 
2 Boston Med. and Surg. Jour., 1902, cxlvii, 313. MYOSITIS 
881 
Symptoms.-The onset is usually slow and with slight symptoms. 
Indeed, the disease may take from several months to ten years to develop. 
Sharp pain is first complained of in the affected muscles, those of the 
lower extremity being usually the first implicated. Only one muscle 
or a single group of muscles are generally first attacked, although the 
beginning of the malady may be in several muscles. The pain in the 
concerned muscles soon forces the patient to go to bed, and, subsequently, 
a rigidity of the affected limbs results and contractures ensue, the flexor 
muscles being especially prone to involvement. There is no fever, and 
the pain usually present is only of slight duration. The general condition 
is but little altered. Finally the affection may implicate most of the 
voluntary muscles of the body. The electrical excitability of the muscles 
is reduced, or may, in extreme cases, be wholly wanting. Disturbances 
of sensation have only rarely been reported. 
Diagnosis.-As Lorenz has well said, a positive diagnosis is impossible 
without histological examination. The slight or total absence of pain, 
when the affected muscle is palpated, is said to be characteristic as well 
as the gradual hardening of the muscle implicated and the cessation of the 
spontaneous pains, which are first well marked. 
Prognosis.-The duration is long, but eventually a decided improve- 
ment is usually noted. However, it is ever well to remember that the 
affection may remain stationary. 
Treatment.-Drugs are of no service, but massage and electricity are 
of benefit in ameliorating the symptoms and hastening the subsidence of 
the disease. By their use recovery has been noted. 
Progressive Myositis Ossificans.-Definition.-A progressive inflam- 
matory affection of the locomotor system, of unknown origin, character- 
ized by the gradual formation of bony masses in the fascia, muscles, 
aponeuroses, tendons, ligaments, and bones, with resulting ankylosis of 
most of the articulations. 
Etiology.-In spite of careful study the cause is as yet unknown. 
It is frequently described as of congenital origin, in the sense that it 
depends on some aberration of growth, congenitally acquired. Fanning1 
reported a hereditary case but it is unique in the literature. There are, 
at present, two main theories. The first one considers the process of bone 
formation to be of an inflammatory nature, while the second view regards 
the bony growth as representing a tumor formation. Goto,2 accepting 
neither theory, considers the initial process to begin with a connective 
tissue hyperplasia which leads frequently to the final stage of bone forma- 
tion, through one of fibrous induration. 
Age.-The disease is generally first noted in young persons, as Lorenz's 
records show, for out of 45 cases in which the age at onset is given, 38 
were affected before the fifteenth year; 11 of these showed the disease 
during the first year of life, 16 between the first and fifth years, and 11 
between the ages of five and fifteen. Occasionally the patient does not 
fall a victim until later in life. Roger's patient was thirty-five years old 
when stricken. It appears to begin earlier in women than in men. 
1 Lancet, 1901, ii, 849. 
2 Arch. f. klin. Chir., 1912-13, c, 730. 882 
DISEASES OE THE LOCOMOTOR SYSTEM 
Sex.-Males are more frequently attacked than females. Gerber 
gives the ratio as 4 to 1, while Rolleston says it is 5 to 1. In Weill and 
Nissim's series of 50 cases, 38 were men and 12 were women. To account 
for this, Pincus lays particular stress on obstetrical traumatism, and says 
the more frequent injuries of boys at birth explains the preponderance 
of the male cases. 
Race.-The Anglo-Germanic race has furnished most of the instances. 
According to Weill and Nissim's series, 20 cases came from Germany 
and 19 from England. The Latin and Scandinavian races contribute 
a few instances, and Russian, Swedish, and French cases have been 
reported. 
Pathology.-In 1869 Miinchmeyer described the process of bone 
formation in the muscles. He recognized three periods or stages which 
have been followed by other writers: (1) Stage of embryonic infiltration; 
(2) stage of connective-tissue induration; and (3) stage of ossification. 
In the first stage the skin and subcutaneous tissue may appear 
normal, but the underlying affected muscle is much swollen and oedemat- 
ous. Microscopically an extensive infiltration of the intramuscular 
and intermuscular connective tissue is seen. This infiltration consists 
in the formation and proliferation of an embryonic connective tissue, and 
is especially marked about the blood vessels. In the second stage the 
embryonic connective tissue becomes organized and forms adult connec- 
tive tissue, which, at first, proliferates more and more, but finally retracts 
and becomes a hard, fibrous swelling. On section it has the appearance 
of a fibroma, presenting a hard, white mass, in which the remaining 
fibres resemble red striations. These fibres are more numerous at the 
periphery of the tumor where, microscopically, many of them appear 
normal. On passing inward tow'ard the centre of the hard, white mass, 
the fibres, when present, seem atrophied or swollen, and frequently their 
cross-striations are lost. Fatty, waxy, and granular degenerations have 
been described, and many fibres are completely destroyed. Their 
sarcolemma nuclei may be markedly increased and some may resemble 
giant cells. The increased intermuscular connective tissue is very rich 
in cells from the fusiform up to the large polymorphous type, which 
contains numerous karyokinetic figures. Other cells are also seen 
which resemble cartilage cells, without capsules, and later produce 
hyaline cartilage. Toward the middle of these fibrous swellings, small 
angular spaces are observed, in which formative cells lie, which later 
represent osteoblasts and bone corpuscles. The spaces are the osteoid 
trabeculse. When these become calcified, the parts enclosed by the 
trabeculae become bone-marrow, and true compact and spongy bone is 
formed, when ossification, seen in the third stage ensues. New bone 
is also formed in the fasciae, ligaments, tendons, and preexisting bones, 
in a manner similar to that just outlined. It is always of the fibrous, 
cartilaginous, or periosteal type, and histologically and chemically 
resembles true bone. By the fusion of several bony foci a larger mass is 
produced, and eventually a bony tumor, situated originally in a muscle, 
may extend beyond the confines of the affected muscle and give no 
indication of its point of origin. z MYOSITIS 
883 
Friedberg, and Salmann1 are opposed to this sequence of histological 
changes. They regard the disease as a primary parenchymatous muscle 
inflammation, with a secondary participation of the intramuscular and 
intermuscular connective tissue. It is proper to state that but one muscle 
was affected in the latter's case, and the sections were differently inter- 
preted by Lexer when he had the opportunity to examine them, for he 
considered the connective tissue changes to be primary. Goto considers 
the process to begin in the fascia and would name the affection hyper- 
plasia fascialis ossificans progressiva. 
Symptoms.-The disease may exist for years before it is recognized. 
Many cases, indeed, have been diagnosed as "rheumatism," and their 
true nature only revealed at the period of bone formation. At times the 
onset may be so insidious that inflammatory appearances in the affected 
muscle, as well as pain, may be completely lacking. In typical cases the 
signs of a myositis are present; without any known exciting cause or 
after trauma, which may be very insignificant, a swelling is found in 
the muscle involved. The swelling presents a firm, doughy consistency. 
Pain may be present, confined to the muscle attacked or radiating in 
character. There is oedema of the overlying tissues, with some redness 
of the skin. The temperature is usually slightly elevated. In the course 
of a few days, or longer, depending upon the intensity of the process and 
the extent of the lesion, these symptoms vanish, but the muscle does not 
return to its normal state. It remains indurated, and finally a hard, tumor- 
like mass results. The disease may then become quite stationary for a 
variable period of time. Eventually, many of these masses form bony 
deposits, but some may remain as fibrous tumors, while others disappear, 
causing the muscles to undergo fibrous degeneration. In such muscles, 
Munchmeyer and others have described the occasional presence of fibril- 
lary contractions. More rarely, the muscle may be entirely restored to 
its former state by the disappearance of the tumor. The process of bone 
formation occupies no fixed time, generally it is from two to eight months 
(the latter duration being seen in Koht's case). The bony change may 
be observed in the whole muscle or only in its fleshy portions, or, excep- 
tionally, the tendons alone may be concerned. According to Munch- 
meyer, those muscles are spared whose two extremities are not inserted 
in the skeleton, but subsequent investigations have not borne out this 
statement. The condition is usually painless, although, occasionally, 
spontaneous pains are complained of, especially at night. 
The muscles of the back and neck are generally the first involved. 
They wrere the first implicated in 21 out of 38 cases, while the muscles of 
the upper and lower extremities and of the face were the first attacked 
in the other instances (Lorenz). The ossification of the cervical ligaments 
and the muscles of the neck causes the head to be bent forward and the 
neck to be fixed. In extreme cases the flexion is so marked that the chin 
touches the sternum. The ossification of the ligaments, fasciae, and 
muscles of the back varies very greatly. The bony deposits, if involving 
only a muscle, may conform to its shape and feel, on palpation, like 
1 Ueber die Myositis ossificans progressiva, Berlin, 1893. 884 
DISEASES OF THE LOCOMOTOR SYSTEM 
freely movable, irregular plates. Very often the ossification has extended 
farther and the muscles are firmly attached to the skeleton. Generally, 
bony foci may be recognized in many muscles of the back, with no regular 
arrangement, like the ramifications of coral (Copping) or a geographical 
map of a mountainous region (Brennsohn). Minkewitsch describes the 
bony tumors of the back as resembling a stag's antlers. The vertebral 
column eventually becomes fixed, by the formation of bridges of bone, 
and scoliosis may be observed. A kyphosis, either cervical or lumbar, 
is much more rarely seen. The scapulae early become attached to each 
other and to the thorax, while occasionally they are connected with the 
ileum. The movements of both arms consequently are much limited. 
If the pectorals and latissimus dorsi are also involved, the arms are 
rigidly fixed on the thorax, which in some cases is described as a bony 
coat of mail. 
Subsequently, the disease progresses, more muscles become implicated, 
and the corresponding joints fixed. The above outlined train of symptoms 
then ensues, except that fever may not be present. The upper extremities 
are usually earlier attacked than the lower, and a cicatricial shortening 
precedes the ossification, so that the limbs are held in a contracted posi- 
tion. The arms are generally fixed at the shoulder and, by the ossification 
of the biceps and anterior brachial, flexed at the elbow. The fixation 
of the ilio-femoral articulations, as well as the ossification of the gluteal 
muscles, cause the hips also to be slightly flexed at the pelvis. This 
posture, with the forward projection of the head, displaces the patient's 
centre of gravity and gives them a precipitous gait,- their knees being 
generally fixed in extension or slightly flexed. The forearms, hands, 
legs, and feet are only rarely involved. If the lower intercostal or 
abdominal muscles are implicated, the respiration may be upper costal 
in type. 
Finally, the temporals, masseters, and pterygoids are attacked, and the 
patient is unable to move his lower jaw, which may be somewhat atrophied 
at this period. To overcome the difficulty in feeding thus induced, 
teeth and a portion of the alveolar process of the lower jaw have been 
removed. The mimicry facial muscles, the muscles of the eye, tongue, 
diaphragm, heart, perineum, genital apparatus, and sphincters are 
unaffected. The electrical excitability of the muscles varies. With the 
galvanic the anodal closing contraction appeared before the cathodal 
closing contraction in two cases. The response to the faradic has 
generally been described as normal or decreased. Pinter got a reaction 
of degeneration. In women who contract the disease, the menses 
generally cease, or, if they have not yet appeared, they are frequently 
long delayed. 
The presence of exostoses has been frequently described. They are 
usually seen on the internal surface of the arm, the anterior aspect of 
the tibia, the upper part of the fibula, the ribs, or where the long bones 
approach the skin. The frontal bones $nd the phalanges of the fingers 
are also other sites where they have been observed. They may subse- 
quently disappear. In about 75 per cent, of the cases reported, a peculiar 
deformity of the great toes and thumbs has been noted, and occasionally MYOSITIS 
885 
the little fingers also have been involved. This was first described by 
Gerber, but Helferich1 directed more particular attention to it. The 
toes and thumbs are much shorter than normal, due to the dwarfing of 
the metatarsal and metacarpal bones, and subsequent ankylosis of the 
interphalangeal joints. This gave the impression, before the introduction 
of x-rays, that one phalanx was absent. The great toes are, generally, 
directed outward and frequently lie under the second toes, giving rise to 
the deformity of hallux valgus. The condition of the toes and thumbs 
is known as microdactylia. It is interesting to note that it was observed 
in the father of Sympson's patient, although he was not subject to this 
disease. In Michelsohn's patient the deformity was seen in the toes at 
birth, but on the thumbs it was not detected until the seventh year. 
Other malformations, such as lack of some muscles, atrophy of the testicles 
and scrotum, have been noted in individual instances. 
Diagnosis.-No difficulty should be experienced in recognizing this 
condition in advanced cases. The age of the patient, the first localiza- 
tions of the bony deposits, the ever progressing character of the disease, 
and the presence of exostoses and microdactylia are important factors in 
establishing a diagnosis. Multiple exostoses may sometimes be mistaken 
for it, but the location of these tumors at the diaphyses and epiphyses of 
the extremities, the arrest of their development past the age of twenty, 
their bony character from the onset, and the fact that the muscles are 
never involved, except secondarily, should suffice for a proper differentia- 
tion. The joint involvements seen in locomotor ataxia should never be 
confused with this malady, although some muscles near the affected 
joints occasionally undergo extensive ossification. In the disease we are 
discussing, the joints are never implicated. Again, spondylitis deformans 
and the muscle dystrophies bear some relation to progressive myositis 
ossificans, but their proper differentiation should present no difficulty. 
Prognosis.-The disease is essentially of chronic character, and relapses 
alternate with pauses in its progress. At times there appears to be a 
cessation of the symptoms for six, ten, or even twenty-three years (as in 
Koht's case), but the malady is ever a progressive one, and the slightest 
cause may induce the formation of bony masses, such as the mere palpa- 
tion of a muscle. In one patient a relapse occurred in April for five 
successive years without any known cause. Eventually the patients 
become practically helpless and bedridden. Bedsores and abscesses 
about the bony deposits may result, and death may ensue from pyemia 
or some intercurrent infection, such as tuberculosis, to which the patients 
seem susceptible, or pneumonia. Suffocation may intervene, due to the 
limitation of the costal breathing and the onset of oedema of the glottis. 
Treatment.-Medical treatment avails nothing. Hawkins, Gibney 
and others had recourse to surgical measures, and some of the bony 
tumors were removed. The result has been most unsatisfactory, as there 
has been a recurrence at the site of removal in almost every instance. 
Still, in necessary cases, as when the lower jaw is fixed, operative measures 
1 Aerztl. Intelligenzbl., 1879, xxvi, 845. For an excellent consideration of this deform- 
ity see Jungling, Beitr. z. klin. Chir., 1912, Ixxviii, 306. He has collected 29 cases 
and adds one from personal observation. 886 
DISEASES OF THE LOCOMOTOR SYSTEM 
are justifiable. The most important point is prophylaxis. By exercising 
precautions against trauma and cold, the affection can frequently be 
prevented from spreading. Occasionally, even these measures will not 
check the progress. 
MYOTONIA CONGENITA: THOMSEN'S DISEASE. 
Definition.-An affection characterized by tonic muscular cramps at 
the beginning of voluntary movements. 
The disease was named after a Danish physician, J. Thomsen, of 
Kappeln, in Silesia, who was a sufferer from it, and in whose family it 
had existed for five generations.1 
Etiology.-Heredity is an important factor, for the disease may be 
directly transferred from the patient to the offspring, or merely the 
disposition to the malady may be transmitted. It may also be seen in 
the collateral branches of the family, or occur in several members of the 
same family, without direct heredity. In 1890 Hershill2 stated that 
there were fifteen families reported in which the disease thus existed. 
Bernhardt thinks that the marriage of blood relations may be of some 
etiological moment, as two members of the same family, whose parents 
and grandparents were cousins, came to him with the disease. 
Occasionally, without heredity or family taint, the disease comes on 
in childhood, and has been found associated with a fright, a fall, or a 
dog-bite. These factors are probably not the cause, however, and we 
may conceive of the malady as being overlooked, on account of the mild- 
ness of the symptoms, until an accident first attracts particular attention 
to the child. Apart from these, other cases exist in which the symptoms 
have not appeared until later in life. In some instances, as in Thomsen's 
family, there is a marked neurotic family history, but Hale White3 
does not consider this to have much bearing on the etiology and adds 
that the patient himself is rarely of neurotic habit. 
Age.-The disease generally comes under observation when the patients 
are about twenty years of age, but cases are not rare at puberty. If the 
symptoms develop in young children, they usually pass unnoted until 
puberty is reached. Then, for the first time, an awkwardness in different 
movements and an inability to take part in games is observed. Some- 
times they find they cannot get up quickly when called upon to recite, 
or a soldier's career may first reveal the condition by the clumsiness and 
awkwardness with which the different movements are executed. When 
a diagnosis has been made in infancy, as in Friis' case, a difficulty in 
sucking, an immobility of the countenance after weeping, an awkwardness 
in bodily movements, or a panting respiration may be observed. 
Sex.-Males are much more frequently attacked; in Niedendarp's 
list of 58 cases, 52 were men, while only 6 were women. In Hans Koch's 
list of 102 instances, this inequality was likewise very apparent, as 91 
were men, but only 11 women. Of the 91 men, 11 had been soldiers. 
1 Arch. f. Psychiat., 1875, vi, 706. 
2 British Med. Jour., 1890, i, 242 (Bibliography). 
3 Albutt's System of Medicine, 1899, vi, 467. MYOTONIA CONGENITA 
887 
Race.-Cases have been reported in Germany, France, Italy, Russia, 
Sweden, England, and in the United States. It appears to be more 
common in Germany and Scandinavia than elsewhere. 
Pathology.-The material for the study of this disease has come 
mostly from muscles excised during life, as but one autopsy has been 
performed (Dejerine and Sotta1). The patient succumbed to an attack 
of acute nephritis with uremic symptoms. During the year prior to 
his death he wras attacked by a chronic, exfoliative dermatitis, but other- 
wise had been in good health. The central and peripheral nervous 
systems are said to have been normal, but the cerebrum and cerebellum 
were not microscopically examined. There was a marked serous infiltra- 
tion of the entire body, hydro-thorax, hydro-pericardium, and oedema 
of the lungs, besides the special muscle changes. In the earlier cases, 
before Erb's monograph was published, the examinations of the muscles 
were negative, although they were investigated in five instances. In 
1886 Erb's work appeared, in which he detailed the complete histories of 
two patients, with protocols on the examinations of the portions of muscles 
taken from each of them. He also gave a report on some muscle sections 
from a man who had died soon after an operation. These sections showed 
pictures so similar to those seen in his two patients that he investigated 
the man's past history, and found him to have had Thomsen's disease. 
In Erb's cases the marked hypertrophy of the muscles affected was very 
noticeable w'hile the skin over them seemed normal. On palpation the 
muscles possessed a certain elasticity and appeared to be in a somewhat 
contracted state. Some of them even revealed a board-like hardness. 
When called into action they felt much firmer than a normal muscle 
would feel under the same circumstances. Their color on section offered 
nothing distinctive. Erb claimed he could distinguish with the naked 
eye the solitary muscle fibres. Microscopically, the stained specimens 
exhibited changes most marked in the muscle fibres, although the con- 
nective tissue was also somewhat implicated. The fibres, individually, 
showed considerable hypertrophy, some of them being twice and even 
four times the diameter of normal fibres. In fact, 70 to 75 per cent, of all 
the muscles measured from 60 to 140/z, in comparison with the normal 
measurements of from 20 to 65/z. The largest fibre had a diameter of 
195/z. On cross-section the bundles of fibrillae did not retain their normal 
polygonal form, but were much rounder than usual, with blunt edges, 
some being even almost circular in shape. The fibres were also more 
homogeneous than normal, as there was an evident loss of cross-stria- 
tion. Longitudinal sections showed a lack of the normal parallelism; 
they were curved and bent in all directions and provided with swollen, 
irregular borders. The cross-striations appeared finer and more delicate 
than usual, while occasionally they were wanting and pronounced longi- 
tudinal striations were noted, or the fibres presented a very homogeneous 
aspect. In one of his cases, vacuoles w'ere present but they were not 
very frequent. They were of varying sizes, sharply marked off from the 
muscle fibre containing them, and generally somewhat eccentrically 
Rev. de Med., 1895, xv, 241 888 
DISEASES OF THE LOCOMOTOR SYSTEM 
placed. In shape, they were inclined to be ovoid. They contained a 
finely granular, homogeneous mass as their contents, or a mass appearing 
as a membrane or coagulum provided with smaller vacuoles. Apart from 
these abnormalities, the increase in the number of nuclei was even more 
striking. On cross-section they were apt to be located next the sarco- 
lemma sheath, and averaged 6.5 nuclei to each fibre, while calculations 
for normal fibres gave only 1.8 for one specimen and 1.67 for another. 
At times the nuclei were found in the centre of the fibre, singly or in 
clumps. They were not so regular or sharply outlined as normal, and 
seemed larger and plumper. In longitudinal sections they presented 
long rows of six to twelve to twenty in number, and were generally 
arranged along the border of the fibre, in lines parallel to the fibre's 
direction. The connective tissue also showed a moderate increase, but 
was not to be likened to any inflammatory connective-tissue growth. 
This picture was subsequently confirmed by various observers, although 
certain variations and new peculiarities were noted by many of them. 
The muscle hypertrophy was seen by all, but no measurements of the 
enlarged fibres have exceeded those given by Erb for the widest he 
observed. In addition to the hypertrophy, Dejerine and Sotta, Jacoby,1 
Joseph Koch,2 and others describe pronounced degenerative changes in 
the muscles. In Dejerine and Sotta's case, two kinds of muscle changes 
were noticed, the one affecting the specific tissue of the fibre and the other 
the indifferent protoplasm, causing the fibres to be more homogeneous 
and to show the Cohnheim fields more distinctly. In later stages there 
was a splitting of the fibres and a dissociation of the sarcous elements, 
which lay in a cloudy, amorphous heap in dilated sarcolemma sheaths. 
Finally, the resulting loss of muscle tissue was evidenced by vacuoles 
and empty sarcolemma sheaths. A few examples of solitary atrophy 
of the fibres were found, and some striated muscle masses were detected 
without their encircling sheaths. The nuclei were frequently very 
irregularly situated. Their case is also of interest, as the findings varied 
in the different muscles examined, being most markedly expressed in the 
diaphragm muscles and least so in the tongue. They claimed they 
could trace the progress of the disease in the muscles, and described the 
first stage as mainly affecting the nuclei, which were greatly increased, 
and were almost as numerous as they were in other implicated muscles 
of the body, although the fibres here were not markedly hypertrophied. 
These variations these authors consider not as the cause, but rather as 
the result of the disease. They also regard the degree of alterations as 
somewhat proportional to the severity of the symptoms. 
In Jacoby's second paper, published nine years after the first, his views 
are considerably modified. The hypertrophy is there regarded as due to 
the abnormal contractility of the muscle after its excision. If this is 
prevented by proper fixing, immediately after excision, the sections, 
subsequently cut, approximate closely to normal. One of his muscle 
sections slipped from its fastenings and confirmed his theory by showing 
pictures similar to those previously noticed. Consequently, he regards 
1 Jour. Nerv. and Mental Dis., 1887, xiv, 129. 2 Virchows Archiv., 1901, clxiii, 380. MYOTONIA CONGENITA 
889 
the alterations he saw as due to "serous infiltration of the connective 
tissue and to secondary changes in the parenchyma, thereby nutritionally 
produced."1 This accords with Bottiger's criticism of Dejerine and 
Sotta's interpretation of their muscle findings, for Bbttiger claimed the 
oedema might entirely account for the muscle changes. Koch observed 
degenerative processes, associated with those of a regenerative nature. 
Into the interfibrillary spaces, where long rows of nuclei were situated, 
he noted an ingrowth of capillaries from the perimysium internum. 
Finally, a sheath would be formed to enclose a new fibre thus cut off. 
In this way two, three, or even four fibres could be formed from a single 
one. The degenerative changes resulted in the formation of vacuoles 
and empty sarcolemma sheaths, and, for the production of the latter, 
nuclear proliferation was an important factor. In the sarcolemma 
nuclei he found evidences of an active proliferative process by direct 
division. A marked increase in the number of nuclei has generally 
been noted, and some have detected hypertrophy of the individual nuclei, 
the increase in size being in breadth and not in length. A moderate 
increase in the connective tissue has not been as constantly found. 
The work of Schiefferdecker and Schultze2 is of special interest, for, 
in addition to the muscle hypertrophy previously mentioned, they found 
granulations in the sarcoplasm, which they interpreted as an indication 
of a specific disease there. They also noted an increase in the number of 
nuclei, which sometimes appeared in very extensive rows, but no degen- 
erative processes were described or any increase in the number of fibres, 
such as Koch reported. The granulations were only noticed in those 
sections of muscle which had been hardened in a solution containing 
magnesium and sodium sulphate, with sodium chloride and 5 per cent, 
formalin (Jore's solution). They consisted of small granules, generally 
circular in shape, and dark black, dark brown, or blackish brown in 
color, with at times a very glistening appearance. The nuclear changes to 
Schultze seemed to be primary, and thus to confirm Dejerine and Sotta's 
theory. They also seemed proportional to the muscle hypertrophy, 
which did not appear to be functional in origin. 
Concerning the pathogenesis of the disease much has been written, but 
it is nevertheless still wrapt in obscurity. (1) The psychopathic theory 
was first advanced by Thomsen, but it has little clinically to support it, 
and is now generally abandoned. (2) The neuropathic view attributes 
the disease to some disturbance of the central nervous system, possibly 
some functional change in the cord. The theories of Sieligmiiller, Peters, 
Rieder, Westphal, Marie and others can be grouped here. Recently 
Gregor and Schilder3 have shown that the myogram obtained by the 
string galvanometer in this disease proves the muscle contraction is not 
of purely myogenic origin, but arises from central innervation. (3) The 
myopathic idea proclaims the muscles to be the primary cause of the 
malady. Dejerine and Sotta incline strongly to this theory. 
1 Jour. Nerv. and Mental Dis., 1898, xxv, 508. 
2 Deut. Zeitschr. f. Nervenh., 1903, xxxv, 1. 
3 Ztschr. f. d. ges. Neurol, u. Psychiat., 1913, xvii, 206. 890 
DISEASES OF THE LOCOMOTOR SYSTEM 
Symptoms.-The characteristic symptom is the so-called myotonic 
disorder, which is revealed when voluntary movements of any sort are 
attempted. This consists in tonic muscular cramps which forthwith 
ensue, inhibiting all movements for a short space of time and causing a 
certain awkwardness and clumsiness of movement, which is very mortify- 
ing to the patient. Moreover, increased exertion to conquer this opposi- 
tion leads only to renewed tonic, muscular contractions. After the same 
movements have been several times repeated, the muscles, with their 
antagonists, relax from their state of prolonged contraction, so that the 
awkwardness disappears, and subsequent similar movements can be 
executed normally. Occasionally the muscular rigidity is more marked 
during the second attempted movements. If a few moments of rest 
intervene, or some sudden obstacle is seen, causing mental disquietude 
and bringing new muscles into play, the same awkwardness again appears. 
These tonic spasms or contractions of the muscles are never accompanied 
by pain, but are sometimes more marked if the patient be fatigued. 
Cold, heat, depression of mind, and hunger are factors, which may not 
only bring on the spasms, but make them more severe. At times alcohol 
and warmth may cause them to lessen. Danillo thought the latter so 
acted when he gave his patient warm tea, and later proved it by then 
getting a normal myogram. The spasms may be seen not only in volun- 
tary movements, but also in those of a reflex nature, as in coughing and 
sneezing, and after mechanical or electrical excitations. Passive move- 
ments are unaffected. Erb states the contractions are noteworthy for 
(1) their slowness, (2) their tonic character, and (3) their continuance 
after the cessation of the voluntary impulses. 
The light forms may cause but slight inconvenience to the patients, 
who can easily conceal this muscular rigidity by cleverly executed move- 
ments. In the severe cases, the patients on attempting to walk appear as 
if chained to the floor, and the stiffness or rigidity which accompanies 
each forward movement of the leg must wear off before another step can 
be taken. With each successive step the stiffness is of shorter duration 
until it entirely disappears, and the patient may then walk without any 
further mishap. If, however, he should trip against a stone, turn 
suddenly around, or bring any muscle, not then in use, into play, rigidity 
of those muscles will follow, which may result in the patient's falling 
down. Similarly, in mounting stairs, the first ones seem to offer insur- 
mountable difficulty, but when these are overcome, the rest are easily 
ascended; in handshaking, the grasp is slow and its relaxation is delayed, 
while the dynamometer shows the grasp is much below the normal 
standard; in eating, there may be an inability to close the mouth, when 
opened, until the spasm of the depressor muscle of the lower jaw relaxes 
and allows the masseters and internal pterygoids to act; in reading a 
book, the patient may be unable to raise his eyes for some minutes 
from the page, although earnestly desiring to do so, and in some cases 
strabismus is seen, due to spasm of the muscles of the eyeball. Similar 
difficulties are noted in speaking, dancing, drilling, or any movement 
requiring voluntary effort. 
The muscles of the lower extremity are apt to be most frequently MYOTONIA CONGENITA 
891 
involved, although those in the upper extremity, as well as the tongue, 
masseter, eye, and diaphragm muscles, may be affected also. Occasion- 
ally the laryngeal muscles are concerned, in which case the speech is 
altered. Often the same patient shows an implication of the muscles of 
the extremities and the trunk, but the disease is not equally expressed 
in all of them, the chief difficulty being in the lower extremities. If the 
trunk muscles are attacked, all forward movements, such as stooping, 
throw these muscles into a spasm, but they are unaffected in the move- 
ments required in parturition or defecation. Neither are the acts of 
swallowing, respiration, or micturition interfered with. More rarely, 
only a few groups of muscles exhibit this malady, as in Oppenheim's 
patient, in whom the orbicularis palpebrarum alone suffered. The 
muscles are generally markedly hypertrophied to the naked eye and the 
patient appears to be of a very athletic build; his strength is below normal 
and not proportionate to his muscular development. In one of Erb's 
cases the myotonic disturbance seemed to be more pronounced in the 
more markedly developed muscles. Later observers have found an 
atrophy, associated with the hypertrophy, which, in some cases, appeared 
to be of the progressive muscular type. Bramwell and Addis1 have 
found 65 such cases, upon only one of these, however, has an autopsy 
been performed (Steiner's case)2. The muscles of this patient showed a 
widespread cirrhosis and a tabetic-like degeneration of the posterior 
columns of the spinal cord. These cases generally present a weakness 
of the fascial muscles (myopathic face), an atrophy especially of the 
sterno-cleido-mastoids, the vasti muscles of the thighs and dorsi flexors 
of the feet, in addition to the myotonic condition. They are of a distinct 
familial type; Bramwell and Addis, Steinert, Pelz3 and others believe this 
is a form of Thomsen's disease, while Batten4 is the leading exponent 
of those who would give it a distinct clinical entity. Greenfield,5 Lange,6 
and Mann7 have reported instances of myotonia with atrophy associated 
with early cataract formation in several members of a family. 
The presence of von Graefe's sign in exophthalmic goitre was first 
noted in myotonia congenita by Oppenheim. This association has later 
been carefully studied by Sedgwick8 in a family of twenty-nine, in whom 
it was found in thirteen instances. 
The electrical reactions have been found as characteristic as the myotonic 
disorder, and have been collectively designated by Erb "the myotonic 
reaction." They are as follows: (1) The motor nerves show no increase 
of irritability to mechanical stimuli; (2) to the faradic current the motor 
nerves are quantitatively normal, but if the current.be strong, the con- 
traction produced on closing the current lasts much longer than it does 
in health; (3) to the galvanic current, the motor nerves are quantitatively 
normal; but here, also, if the current be strong, the contraction lasts 
longer than in health; (4) mechanical stimuli applied to the muscles, as 
1 Edinburgh Med. Jour., 1913, N. S., xi, 21. 
2 Deutsch. Ztschr. f. Nervenheilk., 1909, xxxvii, 58. 
3 Archiv. f. Psychiat., 1907, xlii, 704. 4 Lancet, 1909, ii, 1486. 
5 Rev. Neurol, and Psychiat., 1911, ix, 169. 
6 Deutsch. Zeit.f. Nervenh., 1910, xl, 65. 
7 Berl. klin. Wchnschr., 1913, 1, 846. 8 Am. Jour. Med. Sc., 1910, cxl, 80. 892 
DISEASES OF THE LOCOMOTOR SYSTEM 
by hitting them, induce contractions more early than in health; (5) 
the faradic current applied directly to the muscles, if strong, sets up a 
contraction which lasts from five to thirty seconds; (6) when the galvanic 
current is applied directly to the muscle, K. C. C. and A. C. C. are 
equally easy to obtain; while in health, as is well known, K. C. C. is more 
readily elicited than A. C. C. In Thomsen's disease, even with weak cur- 
rents, the contraction lasts longer than in health; with strong currents it 
lasts some seconds and relaxes very slowly. With the stabile application, 
well-formed, wave-like contractions are seen to proceed slowly from the 
cathode to the anode (Hale White). All of the reported cases have not 
shown an exact resemblance to Erb's cases, which gave the myotonic 
reaction as Hale White thus described it. They have in almost every 
instance, though, revealed a normal mechanical, faradic and galvanic 
excitability of the motor nerves, but an increased mechanical, faradic, 
and galvanic excitability of the muscles. The peculiar rhythmical, ver- 
micular contractions have only been obtained in occasional cases by the 
application of a strong and steady galvanic current. Erb, subsequently, 
did not attach much importance to them, but thought they could be 
obtained, in every case, by proper manipulations. Babonneix1 has 
studied anew the myotonic reaction, and believes it is due to a func- 
tional exaltation of the sarcoplasm. 
The tendon reflexes vary, being increased, normal, or absent. In two 
of Jacoby's patients the knee-jerks seemed at first exaggerated, but 
became weaker and weaker, after successive elicitation, until no response 
was obtainable. The knee-jerks later reappeared after a short interval 
of rest. There are generally no sensory disturbances, with the exception 
of rare paresthesia. The quantitative urine examinations have yielded 
inconstant results. 
Diagnosis.-The recognition of Thomsen's disease should, as a rule, 
present no difficulty, as the myotonic reaction and the myotonic disorder 
are observed in no other malady. Its general hereditary nature will 
also be of assistance in a diagnosis. Conditions are found, however, 
which present many forms of resemblance to Thomsen's disease and which 
may be confused with it. Talma, Furstner, Jolly, and others have 
described instances showing an abnormal hyper-excitability to mechanical 
and electrical irritants. Although named myotonia acquisita, they have 
little in common with the disease we are now discussing, as Jacoby well 
states, and would be better termed intention spasm. He would apply 
the term myotonia acquisita to cases closely resembling Thomsen's 
disease, which come on after injury or overstrain. Another class of 
cases, presenting temporarily the myotonic disorder and myotonic 
reaction, he calls myotonia transitoria. Eulenberg's paramyotonia 
congenita is, also, a closely allied affection, and was noted in one family 
to the sixth generation. But here the myotonic reaction is absent and 
the muscular rigidity occurs only after the influence of cold. Delprat 
found two typical cases of Thomsen's disease, with temporary rigidity 
of the muscles, in consequence of the effect of cold, in a family with 
1 Gaz. d. hop. Par., 1913, Ixxxvi, 2205. AMYOTONIA CONGENITA 
893 
paramyotonia congenita, and Hlawaczek found the two diseases asso- 
ciated in one individual. Myotonia combined with progressive muscular 
atrophy or hypertrophy has been described, and Schultze, Bettmann, 
Mill and Kaiser, and others have noted its association with tetany and 
athetosis. Hoffmann, in one case, and Nalbandoff, in two cases 
(brothers), have found tabes as an accompanying affection. The former 
also observed a case with multiple neuritis as an additional complica- 
tion.1 In 1907 Pelz considered the atypical cases of Thomsen's disease, 
and placed paramyotonia congenita in this category. He thinks, in the 
atypical forms, there are no absolutely pathognomonic symptoms, but 
considers the disturbances of the mechanical and electrical excitability 
as the most constant. If the disease develops late in life, he does not 
regard it as acquired, but as due to an exacerbation or a long latency 
of a disease which, although present, was slow in revealing itself. 
Prognosis.-The intensity of the disease varies greatly; in the lightest 
cases the patients may hide the rigidity of their muscles by cleverly 
executed movements and experience no great inconvenience; the more 
severe forms unfit those afflicted for many occupations and cause them 
to undergo many hardships. It is an essentially chronic disease, which 
appears to progressively increase up to puberty or even later. In some 
cases relapses and remissions mark the progress. It is never fatal by 
itself, and no recovery from it has ever been reported. 
Treatment.-Nothing which has been hitherto tried in the way of 
treatment has been of any avail. Hydrotherapy, gymnastic exercises, 
and massage have given favorable results in the hands of von Bechterew, 
Rosenbach, and Wersiloff, but nothing permanent has been accomplished. 
Thomsen thought he, personally, felt better the more active his life was. 
It appears that something may be done in the way of prophylaxis by 
cautioning the patient to lead a life as free as possible from mental 
excitement and injurious influences, such as cold. 
AMYOTONIA CONGENITA: OPPENHEIM'S DISEASE. 
Definition.-A general or localized flaccidity of the muscles in child- 
hood, which is associated with a weakness or loss of the tendon reflexes. 
Attention was first called to this condition by Oppenheim in 1900, by 
whom it was named myatonia congenita. This designation, however, 
is so similar to myotonia congenita (Thomsen's Disease), that we prefer 
the above term, to avoid confusion. 
Etiology.-The disease is observed in children in the first months of 
life, and is essentially congenital in origin, without any hereditary ten- 
dency. Trauma at birth has not been noted in any instance. Seventy 
cases have now been reported, upon thirteen of which autopsies have 
been performed. The cause of the disease is unknown. 
Pathology.-Oppenheim2 considered the lesions were in the muscles, 
although he did not entirely exclude an affection of the anterior horn cells 
1 For a full list of the various diseases which have been found associated with Thom- 
sen's Disease, see Ascanzi, Monatsschr. f. Psychiat. u. Neurol., 1912, xxxi, 201. 
2 Monatsschr. f. Psych, u. Neurol., 1900, viii, 232. 894 
DISEASES OF THE LOCOMOTOR SYSTEM 
as the cause. Since then the same findings have been generally noted, 
which have been observed by other investigators in the different forms 
of myopathy. The muscle fibres are mostly reduced in size and atrophied, 
the muscle nuclei are markedly proliferated and the connective-tissue is 
much increased. Fat also is observed in the muscles, all of which points 
either to the defective development of the muscles or to retrogressive 
changes in previously normal fibres. There are also changes in the nerve 
tissue, as seen especially in the diminution in number and the vacuolar 
degeneration of the cells of the anterior horn. Acute swelling has also 
been described in these cells. The changes in the thymus gland are 
considered merely coincident. Marburg considered the malady to be due 
to a fetal poliomyelitis, while Rothmann calls it Werdnig-Hoffman 
disease, but Oppenheim1 considers both of these views absolutely unten- 
able. Councilman2 attributes the disease to a toxin produced in the 
mother. 
Symptoms.-The parents of the child so affected are usually the ones 
to note that the whole body of the child or only the limbs, especially the 
lower, are flaccid and motionless. This flaccidity may be so marked 
as to render the joints abnormally movable. (Edema is a trophic dis- 
order, which may be present to a very marked degree. There is also a 
weakness or a loss of the tendon reflexes associated with this condition. 
Active movements may be impossible or only slightly impaired. The 
cerebral function is undisturbed. The muscles of the eye are not affected, 
but the facial and pharyngeal muscles, with those of respiration, may 
become involved. The implicated muscles are flaccid, soft, and thin, 
but not atrophic. Collier and Wilson3 have observed the presence of 
secondary contractures in some of the affected muscles. The electrical 
reactions show a quantitative alteration or a complete absence, but in 
mild cases the reaction is normal. Collier and Wilson described a reduc- 
tion of the faradic with a retention of the galvanic excitability (amyotonic 
reaction) but Oppenheim could not corroborate it. No disturbances of 
sensation are met with and the sensorium is clear. The tendency to 
relapses should be noted. 
Diagnosis.-The absence of family tendency as a factor will differen- 
tiate this affection from progressive muscular atrophy, while poliomyelitis 
occurs acutely in a previously healthy child. Amaurotic family idiocy 
shows a history of a family tendency, different microscopic and ophthal- 
moscopic findings, and an entirely different clinical history. 
Prognosis.-The children attacked may show in time some improve- 
ment, but the outlook does not appear very encouraging. If the condi- 
tion is generalized, the respiratory muscles are always attacked. In six 
instances bronchopneumonia caused the fatal termination. 
Treatment.-There is no known special treatment. The use of mas- 
sage, electricity, and nerve tonics is indicated.4 
1 Lehrbuch der N ervenkrankheilen, Berlin, 1913, 6 ed., 280. 
2 Am. Jour. Dis. Chil., 1911, ii, 340. 3 Brain, 1908, xxxii, 1. 
4 For a further consideration one is referred to the following articles: Archangelsky 
und Abrikosoff, Arch. f. Kinderh., 1911, Ivi, 101-129 (.Bibliography); and Foot, Am, 
Jour. Dis. Child., 1913, v, 359. CHAPTER XXV. 
ARTHRITIS DEFORMANS. 
THOMAS McCRAE, M.D. 
Introduction.-It is necessary for anyone writing on the subject of 
chronic arthritis to state definitely, so far as possible, what conditions 
are included under various terms and what is meant by the designations 
employed. It is well to confess that our knowledge of chronic diseases 
of the joints is in an unsatisfactory condition, and that great difference 
of opinion exists on many points. The best that one can do at present 
is to put forward his views in such a form that others can at any rate 
understand what is meant, and if they disagree, have no difficulty in 
recognizing where the difference lies. Much of the pathological and 
clinical material is a matter of record; the conclusions drawn are more or 
less matters of opinion. 
In this article the designation arthritis deformans is used to include 
the group of cases of arthritis which have as prominent features a tendency 
to chronicity and to more or less permanent change in the joints or struct- 
ures about the joints, those forms of arthritis with a definite etiology 
(e. g., gonorrhoeal arthritis) being excluded. That this is a satisfactory 
classification cannot be held, but it seems the best we can have at present. 
In gonorrhoeal arthritis there may be chronicity with more or less per- 
manent change in the joints, yet we have a definite etiology by which 
it can be classified. Determine a definite etiology for any case or group 
of cases and they can be separated from the general class. Taking 
arthritis generally we can divide the various forms into: 
1. Traumatic arthritis. 
2. Arthritis due to infection with definite bacteria, such as the gono- 
coccus, streptococcus, tubercle bacillus, pneumococcus, etc., which may 
be obtained in the joint. It is to this group that the term infectious 
arthritis properly belongs. It is probable that rheumatic fever belongs 
here, as the evidence for it being due to infection with a specific organism 
is very strong. 
3. Arthritis occurring in certain diseases, such as scarlet fever and 
syphilis, and apparently dependent on the cause of the disease, although 
in some cases it may be due to a secondary infection. 
4. Gout. 
5. Arthritis due to the injection of various sera. 
6. Arthritis secondary to disease of the nervous system, as locomotor 
ataxia and syringomyelia. In this trauma may play a part. 
7. Arthritis in hemophilia, purpura, etc. 
8. A large group remains, the cases of which for the present are put 
together. In this group there is almost an endless variety, many of 
895 896 
DISEASES OE THE LOCOMOTOR SYSTEM 
them showing acute features at times, others being chronic throughout; 
the degree of involvement varies greatly, but in all there is the pre- 
dominant feature of a tendency toward some permanent change in the 
joint or structures about the joint. In this article these cases are dis- 
cussed under the heading of arthritis deformans. The designation 
chronic polyarthritis might be used almost as well as the one chosen. 
That there can be any confusion as to the essential nature of the 
disease as compared with, for example, gonorrhoeal or tuberculous arthritis 
does not seem likely. Suppose we have regarded a patient as having 
arthritis deformans and subsequently find gonococci in a joint. That 
case is removed at once to a definite class, just as if we found streptococci 
or tubercle bacilli. One may say that this group simply consists of 
those cases with certain forms of chronic arthritic change in which no 
definite etiological factor can be found. This objection, if it be such, 
is perfectly correct. When we have a proved etiological factor for all 
forms of arthritis one kind of classification will be perfectly easy. For 
the present we are doing what we can to make the best of a bad job. 
It is important to remember that arthritis is always secondary to a 
process elsewhere; the joint condition is not primary. 
Have we in this group one disease or two or more? Many take the 
latter view. Thus, A. E. Garrod1 considers that we are dealing with 
several maladies (e. g., rheumatoid arthritis and osteo-arthritis) and not 
with a single disease. The question is a difficult one to settle. There is 
no doubt that certain cases of arthritis show features which seem to 
suggest distinct entities, but others seem to belong to both groups or to 
lie between them, showing evidences of both. In all chronic joint changes 
it is well to keep in mind that the same cause may produce varied results, 
and the same result may come from different causes. The great variation 
in the picture presented by arthritis due to a particular organism (e. g., 
the gonococcus) might easily lead to the severe and mild cases being 
regarded as different, did not the common cause group them together 
etiologically. So in arthritis deformans the writer feels that there are 
so many different grades of change, which merge the one into another, 
that a classification into distinct diseases is not justified. 
"Infectious Arthritis."-This term has been used in different ways. 
Some apply it to certain forms of arthritis which come under the designa- 
tion of arthritis deformans as given here. These are the cases of arthritis, 
which occur secondary to some focus of infection, such as tonsillitis, 
but in which no organisms are found in the joint. Is this designation 
a good one? The writer thinks not, for various reasons. The term 
is with propriety applied to such forms as gonococcus or tuberculous 
arthritis, in which the etiological factor is definitely proved. To use it 
for cases in which the cause is not established must lead to confusion. 
Then by some an "infectious" group is separated, that is, on supposed 
etiological grounds, while other groups, such as atrophic and hypertrophic, 
are separated on anatomical grounds. This is not meant to imply that 
those forms occurring secondary to tonsillitis, for example, are not 
1 A System of Medicine, Allbutt and Rolleston, revised edition, vol. iii. ARTHRITIS DEFORMANS 
897 
due essentially to an infection in some way or other, but the evidence 
seems about as strong for the atrophic and hypertrophic groups. There 
is no uniformity in the use of the term, for some use it only for arthritis 
which is due to a definite infecting organism, while others use it both for 
this and for the group of cases here described under the heading of the 
peri-articular form and by some writers termed rheumatoid arthritis. 
For the cases secondary to a focus of infection, without the presence of 
organisms in the joints, the terms toxic or toxemic might be used. 
By no means all the cases of arthritis occurring with various local 
infections (e. g., tonsillitis) are to be regarded as belonging to the arthritis 
deformans group. Many of them are temporary and recovery follows 
without there being any evident permanent change. It is a question, 
however, whether repeated attacks of this kind do not always leave 
some damage. To decide where the line is to be drawn is difficult. 
The writer feels that if any change is left in a joint the condition probably 
belongs under the heading of arthritis deformans; the milder cases are 
better considered as secondary complications of the original condition. 
" Chronic Rheumatism "-In the view of the writer there is no advan- 
tage and every disadvantage in the use of this term. We have suffered 
and do still suffer from the use of the word "rheumatism." Some 
employ it as synonymous with arthritis of any form, others for rheumatic 
fever. If it is used as a synonym for arthritis, then its use for a special 
form of arthritis should be stopped. It seems much better to use the 
term for rheumatic fever only. If this be done there is no reason for 
the term "chronic rheumatism," for there is no evidence known to 
the writer that rheumatic fever passes into a chronic condition.1 To 
apply the term chronic rheumatism to certain ill-defined joint changes 
is only misleading; call them chronic arthritis and then there is no sugges- 
tion that they have anything to do with rheumatic fever. The use of 
the word "rheumatism" to describe any form of ill-understood pain- 
muscular, arthritic, synovial, or neural-is a diagnostic sin for which no 
good word can be said. 
Terminology.-The designations of a whole or part of this group which 
have been employed are various, and sometimes it is difficult to know 
exactly how much is included by any particular one. Among them are: 
Rheumatic gout (Fuller); chronic rheumatism of the joints (Todd); 
rheumatic arthritis (Adams); nodosity of the joints (Haygarth); rheu- 
matisme chronique primitive • (Charcot); goute asthenique primitive 
(Beauvais); rheumatoid arthritis (Garrod); osteo-arthritis; arthritis 
seche; arthritis deformans (Virchow). There is much to be said for the 
use of the designation "chronic arthritis," as it does not commit one to 
any view as to the nature of the disease. Against this the criticism may 
be passed that the process is often acute at its onset or at times during 
the course. The fact remains, however, that essentially the tendency 
is toward some degree of chronic change. 
1 Certain cases of rheumatic fever in children may drag on for months, showing one 
manifestation after another, especially cardiac, and to these the term "chronic" might 
be applied, but this is not its common use. 
vol. v-57 898 
DISEASES OF THE LOCOMOTOR SYSTEM 
Nature of the Disease.-To recount the various theories is not neces- 
sary. A relationship to rheumatism and gout, an inherited arthritic 
diathesis, a special kind of degeneration, disturbed nutrition, an inflam- 
matory process, of essential nervous origin, due to injury, reflex irritation, 
or disease of a supposed joint centre and some form of infection, have all 
been suggested at some time or other. At present, the view which seems 
most reasonable is that the disease is dependent on an infectious process 
of some kind. This is not to be interpreted as meaning a definite infection 
of the joint with organisms. This may occur, but in many cases the 
arthritis seems to be dependent on local infection in some part of the 
body, with perhaps absorption of toxins and a secondary arthritis. How 
far possible absorption of toxins from the intestines may be a factor we 
are not in a position to state. It seems that in many instances the 
factors of trauma, wear and tear, and the tendency to degenerative 
changes in the joints of those advanced in life are additional factors. 
Changes due to an infectious process, subacute or chronic, may be 
associated with any of these. 
The view of the disease suggested here is that the arthritis is due to an 
infection of some kind-probably this may be with many different 
organisms-as a result of which very varied changes may result. The 
changes involve especially now one and again another part of the joint, 
with a great variety of results, individual reaction and resistance probably 
play a large part in determining the changes. As to how far inherited 
tendencies may affect the process it is difficult to say. Some families seem 
to have joints which are more susceptible to infection and degeneration. 
Classification.-Some writers emphasize the distinction between the 
form in which the bony changes predominate and that in which the 
changes are more in the cartilage and structures about the joint. That 
in many cases this is true there can be no doubt, but it does not seem to 
be true so invariably that it can be used to prove the existence of two 
diseases. Thus, when one finds a patient showing changes of one form 
in one joint and of another form in a second joint, the inference is that 
he has the one disease manifesting itself in different ways. It is possible 
that two diseases may co-exist, but this is too uncommon to explain 
the frequency of these cases. Thus, the patient may have marked 
ostero-arthritic changes in the spine, with distinct hypertrophic changes, 
and in the peripheral joints, well-marked changes affecting the structures 
about the joint. Again, in a patient whose predominant changes are 
peri-articular it may be possible to find distinct hypertrophic changes in 
some situations. The writer feels that there are so many examples of 
these mixed cases that they offer a great objection to the adoption of the 
view that two diseases can be distinguished. That there are striking 
general differences between typical cases in each group is very true, but 
are there not just as marked differences between cases in the same group? 
For clinical study, it is convenient to make a classification without 
regarding the forms as distinct diseases. The character of involvement 
varies, and, as a rule, certain features predominate. This is not always 
true, and it may be difficult to decide in which group to place a given case. 
In some of this series it was difficult to decide whether bony changes or ARTHRITIS DEFORMANS 
899 
those in the peri-articular tissues predominated. In other cases a single 
joint showed various changes. In this article certain groups are dis- 
tinguished for convenience of study, it being understood that the lines 
between these are not definite, but, as a rule, enable one to put a given 
case in one of them. Some cases (66) seem to belong to two of them and 
cannot be definitely classified. 
1. A form in which the changes predominate in the structures apart 
from the bony parts of the joint, although the cartilage is frequently 
involved to some extent. This group is termed "peri-articular," and 
in this series 319 cases were regarded as belonging to it. 
2. A form in which the chief change consists in marked atrophy both 
of the bones and cartilages. With this there are usually marked trophic 
changes in the muscles. This is termed the "atrophic" form, and com- 
prised 9 cases. 
3. A form in which hypertrophic changes are the most prominent. 
There may be marked bony overgrowth, which may occur at the edges of 
articulating surfaces especially, or in the spine, involving the cartilages 
and ligaments. This is termed the "hypertrophic" form, and 106 
cases belonged to it. 
The question arises as to whether one of these forms may pass into 
another. As a rule, this does not seem to occur, although in some cases 
the course suggests that cases showing the changes of the peri-articular 
form may later have those of the atrophic type. Many factors have a 
possible bearing: atrophy from disuse (which, however, gives a different 
picture), secondary trophic disturbances, marked general toxemia, etc. 
Etiology.-The various general factors are as follows: 
1. Sex.-The figures given by various writers vary greatly, but in the 
majority of statistics the larger number is said to belong to the female 
sex. One factor which makes a material difference is whether the cases 
of spondylitis are included or not. They are usually in males, and if 
included make the numbers in the two sexes more nearly equal. There 
is some difference in the relative occurrence of the various forms in the 
sexes. In the present series of 500 cases,1244 were males and 256 females, 
cases of spondylitis being included. In a series of 1228 reported cases 
collected by the Cambridge Committee,2 it was found that 76.5 per 
cent, were females and 23.5 per cent, males. 
2. Age.-It is important to remember that the age of patients when 
seen may give a false idea of the time of greatest prevalence. The 
disease is so chronic that many patients come with advanced changes 
years after the onset. It may be said that the disease can occur at any 
age except in early infancy. The Cambridge report mentions one case 
beginning at eighteen months. The more carefully the histories of those 
in whom apparently the disease begins at an advanced age are studied, 
1 The series used as the basis of this article is composed of the records of patients 
in the Johns Hopkins Hospital, the Jefferson Hospital, and a number personally ob- 
served elsewhere. The report of 110 of them has been published (Jour. Amer. Med, 
Assoc., 1904, xlii, 1, 94, 161). 
2 Bulletins of the Committee for the Study of Special Diseases, Cambridge. This is 
the report of a special investigation of this disease, and should be studied by all those 
interested in the subject. It will be referred to as the Cambridge report. 900 
DISEASES OF THE LOCOMOTOR SYSTEM 
the more often it will be found that there has been arthritis before. 
Still, the first manifestations may be after the age of eighty years. The 
records in this series as to the age when the patient came under observa- 
tion are as follows: 
Number. 
Percentage. 
1 
to 
10 
years 
4 
0.8 
11 
tt 
20 
tt 
24 
4.8 
21 
Ct 
30 
tt 
84 
16.8 
31 
tt 
40 
tt 
114 
22.8 
41 
tt 
50 
tt 
101 
20.2 
51 
tt 
60 
tt 
98 
19.6 
61 
70 
u 
60 
12.0 
71 
tt 
80 
tt 
13 
2.6 
81 
tt 
90 
tt 
2 
0.4 
The age of onset shows that in 40 per cent, this was below the age of 
thirty years, while only 22 per cent, were below the age of thirty on 
admission. The figures as to the time of onset are: 
N umber. 
Percentage. 
1 to 10 years 
. . . . 21 
4.2 
11 " 20 " 
. . . . 72 
14.4 
21 " 30 " 
. . . . 108 
21.6 
31 " 40 " 
. . . . 107 
21.4 
41 " 50 " 
. . . . 83 
16.6 
51 " 60 " 
. . . . 66 
13.2 
61 " 70 " 
. . . . 27 
5.4 
71 " 80 " 
. . 4 
0.8 
Doubtful 
. ... 12 
2.4 
The type of disease has some influence as the peri-articular and atrophic 
forms usually come on earlier than the hypertrophic, which more often 
occurs in older patients. The changes of this type in the larger joints 
are especially common in elderly people. 
3. Race.-This is of special interest in regions where the colored race is 
a factor. In this series there were 474 whites and 26 colored, which shows 
a relatively low proportion of colored patients. This is of importance 
in regard to the influence of certain diseases in the etiology. Thus, the 
great majority of members of the colored race have had a gonorrhoeal 
infection, and tuberculosis is very common among them. It is interesting 
to note that they show a higher relative incidence of rheumatic fever 
than the whites and a much lower relative incidence of arthritis deformans. 
4. Station in Life and Occupation.-There was no evidence in this series 
to suggest that any station in life showed a special influence. The 
patients came from the wealthy and the poor classes in about an equal 
relative proportion. Nor does occupation seem to have any great 
influence. Certainly unusual exposure to wet or weather was not marked 
in this series, and the frequent occurrence among washerwomen, so often 
commented on by English writers, was not evident. The largest number 
of the patients lived indoor lives, which raises the question as to whether 
this may not lower their resistance and predispose to infection, or, at 
any rate, render them less resistant to a chronic infection once acquired. 
5. Infections.-With the view now generally held that the changes 
are due to some form of infection somewhere, the possible causal relation- ARTHRITIS DEFORMANS 
901 
ship of previous infections has always to be remembered. Infection 
occurring shortly before or about the time of onset is important. Of 
these, some are striking in the close relationship. Six cases of infected 
wounds are especially remarkable; in none of these was the arthritis 
suppurative. Boils were associated with the onset in 4 cases, cystitis 
in 6, dysentery in 8, pelvic infection in 18, otitis media in 10, a pyo- 
nephrosis in 1, a miscarriage in 4, and bronchitis in 4. 
Of particular diseases, influenza and tonsillitis are of special interest. 
Influenza had occurred in 28 patients and tonsillitis in 91. The figures 
for influenza (5.6 per cent.) are lower than in the Cambridge report, 
in which 9 per cent, was found. In 26 cases there had been frequent 
attacks of severe pharyngitis, and nasal infection was not infrequent. 
The tonsillar conditions are usually due to infection with some form 
of streptococcus, and it is well to recognize that tonsils which are not 
especially enlarged may contain foci of suppuration in their deeper 
parts, so that infection from this source may be more frequent than these 
figures indicate. To gonorrhoea some writers have ascribed importance 
as an etiological factor. In this series a history was given in 74 cases. 
If it was an important factor one would expect to find more arthritis 
deformans among the negroes, few of whom escape gonorrhoea. There 
was a history of syphilis in 29 cases and of chancroid in one. Scarlet 
fever, erysipelas, typhoid fever, pleurisy, osteomyelitis, all occurred in a 
few cases shortly before the onset. 
6. Mouth Infection.-Pyorrhoea alveolaris, carious teeth, infection about 
the roots of teeth and under capped teeth are all possible sources of infec- 
tion and must always be considered. The number of cases in which 
this source of infection has been proved is steadily increasing. 
7. Trauma.-In some cases this seems to have an influence, but prob- 
ably more often in determining the joint in which the symptoms are first 
evident than the onset of the disease. There is no doubt that in some 
cases the first manifestations of the disease are wrongly diagnosed as 
due to trauma. It is often easy for a patient to remember some strain 
or fall about the time of onset. IIowrever, in one form, spondylitis, 
the influence of trauma seems well established. In other cases the effect 
of an occupation which throws special stress on particular joints seems 
to determine the onset or greater severity there. Frequently there is 
some existing arthritis which is aggravated by the traumatism. 
8. Anxiety and Mental Strain.-The history of prolonged worry, severe 
strain and anxiety, troubles of various kinds, etc., is frequently given by 
the patients (26 per cent, in the Cambridge report). It is often evident 
that these factors have resulted in lowered health, and this may render 
them more susceptible to infection, especially if associated with increased 
work and loss of sleep. In patients who have the disease the influence 
of these factors in determining recurrence is only too evident. 
9. Family History.-This is a difficult matter to speak of with certainty. 
Is there such a thing as an arthritic diathesis? There are many authori- 
ties who firmly believe that there is, and yet the matter is difficult of 
proof. In this series there was a definite family history of arthritis in 
114 cases; of these, 30 were apparently arthritis deformans; 30 were 902 
DISEASES OF THE LOCOMOTOR SYSTEM 
termed "rheumatism," and of these a number were arthritis deformans; 
3 were rheumatic fever, and 6 gout, the others being doubtful. Some 
patients gave a remarkable family history of arthritis deformans. Thus, 
in one, the father, three brothers, and three sisters all had the disease 
in a chronic form; one sister escaped. The patient's mother did not 
have the disease, but her mother and all her brothers and sisters had. 
The writer knows of one family of six in which every individual had a 
deforming arthritis of the slow progressive type, some being almost 
entirely crippled, others having only inconvenience. One patient gave 
a history of the disease in four generations. 
10. Predisposing Causes.-While it is often difficult to decide with 
certainty how much influence these may have, yet in some instances the 
association is so close that they must be given a place. This is seen not 
only in regard to the original attack, but also in recurrences. With a 
patient who has had attacks of the disease, one may be able to predict 
that with certain events, such as an intercurrent attack of illness, the 
arthritis is likely to light up. While this is perhaps most marked in the 
group with peri-articular changes predominating, yet it is also true of 
those with more marked bony changes. Among these predisposing 
causes come first those which affect the general health, such as poor 
hygiene, unsanitary surroundings, hardships, etc. All appear to have an 
effect. In women the menopause is often of importance, and in 27 of 
the female patients the disease began about the menopause. This 
association is seen both in attacks beginning at the menopause and also 
in women who have had the disease in earlier life, and perhaps almost 
entirely recovered, in whom the symptoms again become active at this 
time. Frequent pregnancies seem to have an influence in some patients. 
The onset may come after frequent childbearing, or, more commonly, 
pregnancy after the disease is once established may apparently cause a 
lighting up of the process. The infections also are important. These 
may be of any kind. Influenza and acute bronchitis, an acute dysentery, 
or pelvic disease may be quoted as examples. How often exposure, 
wetting, etc., are factors it is hard to say, but the impression gained from 
the study of this series is that these are not important. How much 
importance gastro-intestinal disease may have is difficult to say. It may 
influence the general health, and so predispose to infection. It must not 
be thought that predisposing causes are always present. The disease 
may begin in those who are apparently in the best of health. 
11. Specific Cause.-The points which support the view that the disease 
is due to infection are as follows: 
(a) The Character of the Attacks.-1These often suggest an acute infec- 
tion. The sudden onset, the inflammatory local changes, the fever, 
the presence of complications, such as endocarditis, pericarditis, and 
pleurisy, and the tendency to run a certain course are all suggestive. 
These features are not constant or invariable, neither is the usual picture 
of any infectious disease. 
(b) The Similarity of the Lesions to those Proved to be Due to Infectious 
Processes.-This is seen in the likeness of the lesions in certain joints to 
those caused by the gonococcus, for example. This is also true of the ARTHRITIS DEFORMANS 
903 
bony changes; thus, the picture presented by spondylitis is exactly like 
that of the spondylitis in typhoid fever. 
(c) The Definite Association between a Focus of Infection and Arthritis 
Deformans.-The examples of this are sometimes very striking. Thus, 
in one patient seen by the writer the deforming arthritis followed a 
suppurating joint. When this was properly treated the general arthritis 
improved rapidly, but some deformity was left. Some would say that 
this was more properly termed "infectious arthritis," but the writer 
considers all of this group as due to some infection, and this arthritis 
certainly was deforming. The same association is seen in cases with 
marked bony changes. In some cases of chronic arthritis associated 
with bronchiectasis, the joint condition varied with that of the bronchiec- 
tasis. The rapid improvement in the arthritis following the removal 
of a source of infection is often remarkable. 
(d) The Definite Cultural Results.-In an increasing number of cases 
organisms are obtained in cultures from various parts, blood, joints, 
glands, etc. The work of Rosenow is particularly suggestive in this 
connection. 
12. Nervous System.-The view was, and is still held, that the cause 
must be in some change in the nervous system. There is much against 
this other than the evidence which speaks for an infection. The similarity 
to the joint lesions in chronic disease of the nervous system is only true 
of the later stages of some forms of arthritis deformans. Examination 
of the central nervous system has not shown any changes to account for 
the arthritis. 
13. Intestinal Infection.-It seems possible that in some patients the 
intestine may be the source of a long-continued infection either by the 
entrance of organisms or by the absorption of toxins. It is possible that 
the total of repeated slight changes may be due to this. The effect of 
the continued swallowing of large numbers of streptococci from an 
infected mouth seems worthy of consideration. As to the possibility 
of the absorption of toxins from the intestine, one hesitates to attach 
much importance. Our knowledge of "auto-intoxication" is not satis- 
factory. One therapeutic point is of interest in this connection: some 
patients are markedly improved by very free continued purgation. In 
others, a sharp attack of diarrhoea may result in an immediate gain 
in the arthritic condition. 
14. Metabolism.-In the minds of many of the laity and of the pro- 
fession the cause is supposed to be in some disturbance of metabolism. 
This is shown by the frequently given opinion that uric acid is the causal 
agent and by the common reduction in the nitrogenous diet. There 
does not seem to be any definite evidence in support of this. Is there 
any evidence that metabolic disturbances are at all responsible? Such 
disturbances do occur, but are they a cause or a result? There is no 
evidence to show that nitrogenous metabolism is at fault. It has been 
suggested that some derangement in carbohydrate digestion may be 
a factor in some cases. Some patients are undoubtedly made worse by 
large amounts of carbohydrates, but this seems due to intestinal dis- 
turbances. That this may be associated with the absorption of toxins 904 
DISEASES OF THE LOCOMOTOR SYSTEM 
is a tempting suggestion, but we have no evidence to support it. The 
most reasonable view seems to be that any metabolic changes are a 
result and not a cause. 
15. Experimental Arthritis.-A remarkable instance of the production 
of " osteo-arthritic" lesions in a rabbit was reported by Poynton and 
Paine,1 who obtained in cultures, from the knee-joint of a man with 
chronic arthritis who died by accident, a diplococcus which injected 
into rabbits produced an arthritis characterized by destruction and 
formation of bone and cartilage. This differed from the arthritis pro- 
duced by the diplococcus of rheumatic fever. 
Pathology.-In a disease with so many manifestations it is difficult to 
give any description which will apply generally, and it is well to recog- 
nize that there may be all grades of change, from the slightest alteration 
to complete disorganization of the joint. Nor are the lesions always 
progressive; there is evidence that slight damage may be done which 
does not advance. There is no definite relationship between the etio- 
logical factor and the resulting change in arthritis; nor is there any 
regular association between the clinical picture and the pathological 
condition. Many causes may produce the same result, and one cause 
may produce various results. We may group the changes as follows: 
1. Effusion.-This is not constant and shows no peculiar features. 
The fluid is generally somewhat turbid and contains cells which have no 
special significance. Early in the course it may be hemorrhagic. 
2. Changes in the synovial membrane, which are of an inflammatory 
nature and often hemorrhagic early in the attack. 
3. Changes in the capsule of the joint and surrounding tissue. 
4. Cartilage.-This may show erosion, atrophy, and ulceration. In 
certain cases there may be marked proliferation. 
5. Bone.-This may show atrophy of slight grade, or in some cases 
this may be extreme. In other cases there is marked proliferation, which 
may lead to extreme deformity. The formation of new bone may 
sometimes occur in the structures outside the joint, as in the ligaments. 
Atrophy and hypertrophy of bone may occur in the same joint. 
Nichols2 divides the lesions into (1) serous, (2) ulceratve, (3) ankylo- 
sing, (4) formative, and (5) fungus or villous. In the Cambridge report 
the principal changes as made out in the x-ray plates were: (1) Those 
limited to the soft tissues, (2) dislocation, (3) atrophy or destruction of 
cartilage, (4) atrophy and destruction of bone, (5) transparent areas, 
which look like punched-out holes in the plates and contain a gelatinous 
material; their significance is doubtful, (6) Bruce's nodes, which are 
small bony deposits, usually on the sides of the phalanges; they are 
probably not typical of any one condition, (7) deposits of new bone, 
(8) fibrous ankylosis, and (9) bony ankylosis. 
It is convenient to discuss the alteration in the various forms of the 
disease, although the association of changes may be very marked. Two 
great forms of change may be recognized: first, those in the soft parts, 
and secondly, those in the cartilage and bones. In some cases one or 
1 Tr. Path. Soc. London, 1902, liii, 221. 
2 Keen's System of Surgery, vol. ii. ARTHRITIS DEFORMANS 
905 
other of these may predominate, but in many both will be found. It 
is frequently only by the z-ray plates that the second group may be 
recognized without opening the joint. Certain points may be noted. 
One is that suppuration never occurs. Another is that the descriptions 
of changes found postmortem, years after the acute features have sub- 
sided, do not necessarily give any indication of what occurred early in 
the course. They are the remains of processes long since over. Again, 
secondary features are common, especially contractures and atrophy 
of the muscles. This latter is sometimes due largely to disuse, but 
again must be due to changes associated with the disease, as it comes 
on too rapidly to be due to disuse, and suggests some trophic disturbance. 
Again, there may be mechanical results, such as dislocation, of varying 
degree, and with or -without marked changes in the cartilage or bone. 
In the peri-articular form there is often a considerable amount of 
effusion, especially in the more acute attacks, which may persist for 
sometime. The fluid shows no peculiar features, although early in the 
course it may be distinctly hemorrhagic. Cultures from the fluid are 
usually negative. The findings in an instance in which the knee-joint 
was opened may be quoted as characteristic. There were some slight 
hemorrhages in the neighborhood of the joint, which on being opened 
was found to contain 100 cc. of fluid. The synovial membrane was 
injected and showed papillary outgrowths of somewhat translucent 
connective tissue. The outer condyle of the femur showed erosion 
of the cartilage, the articular surface consisting of enamel-like bone; the 
corresponding surface of the tibia was the same. Certain areas were 
elevated, apparently due to overgrowth of cartilage. Sections of the 
synovial membrance showed on the surface an exudate of granular 
material and leukocytes. Below this were many bloodvessels and many 
leukocytes. Then came a layer of endothelial cells, which merged into 
the underlying fibroblasts. 
Synovial Membrane.-This usually shows some thickening, which is 
more marked in the peri-articular form. If the joint is opened early in 
the course, the synovial membrane is usually swollen, injected, and 
redder than normal, the very vascular appearance giving a striking 
picture. The surface sometimes shows a velvet-like appearance, or is 
covered by a material not unlike granulation tissue. It may be covered 
with small processes of varying size or shape. The likeness of these to 
tubercles has led to that diagnosis being made. The synovial membrane 
may be thrown into folds. In some cases it becomes adherent to the 
adjoining cartilage, and where this occurs the cartilage is affected and 
usually destroyed. Histologically in the early stages there may be a 
marked hemorrhagic condition. The capillaries are markedly distended, 
and there may be considerable extravasation into the tissues. Nicholson 
in the Cambridge report describes two layers, the outer of which is more 
fibrous than the inner, and in it many fat cells may be seen. There is 
no marked collection of leukocytes, as is often seen in the inner layer. 
This is thinner, composed of delicate areolar tissue, and in some places 
resembles myxomatous tissue. Sometimes it contains large numbers 
of fat cells. One interesting feature is the presence of areas containing 906 
DISEASES OF THE LOCOMOTOR SYSTEM 
large numbers of mononuclear leukocytes, usually around the blood- 
vessels which show an obliterating endarteritis. There may be marked 
proliferation of this layer with the formation of villi. In some cases 
there may be an entire absence of the endothelial lining. 
As the process advances there is usually thickening of the synovial 
membrane and an increase in the fibrous tissue. In some cases a certain 
amount of necrosis may occur. With proliferation of the synovial mem- 
brane the so-called villous arthritis may result, which in some cases is 
most marked. The villous processes are deep red in color and of very 
varying shape and size. They sometimes reach such a size that they 
distend the joint. As a rule, there is comparatively little effusion in these 
villous cases. Secondary changes may result; there may be calcareous 
deposits in the membrane itself or portions of the villi may become 
detached. These subsequently may undergo calcareous change. In 
some cases the synovial membrane becomes closely adherent to the 
surface of the cartilage, the destruction of which is most marked at 
this point. This is well seen in the case reported by Hale White.1 
In the peri-articular form there is usually a good deal of thickening 
in the structures about the joints, and the ligaments may be involved. 
This is especially well brought out by the x-ray plates. These may 
show the changes to be almost entirely outside the joint proper. 
Cartilage.-The changes are very varied in extent, and the slighter 
degrees may only be evident on opening the joint or in the x-ray plate. 
In the early stages the cartilage may be injected. The earliest change 
appears to be erosion, which in some cases seems to be determined by 
the proximity of the . affected synovial membrane. The extent of this 
varies greatly; it may be very slight, or go on until the whole cartilage 
has disappeared and the bone is exposed. Histologically there is fibril- 
lation of the matrix, with disappearance of the cells. This may involve 
only the superficial layers and the structures below are normal. The 
process may advance until no normal cartilage remains. Apparently 
this is not always general, and there may be marked loss of cartilage in 
local areas; in other cases the whole surface shows thinning. The gross 
appearance of the cartilage is often striking. The surface is injected in 
parts, and often irregular, owing to the areas of erosion. In other cases 
the cartilage becomes liquefied and the corpuscles disappear with the 
formation of cysts filled with mucoid fluid. Nicholson describes the 
occurrence of necrosis in the cartilage with the formation of non-staining 
areas. This, however, he regards as being possibly due to gout. 
In some cases there is apparently some formation of new tissue, in 
others there is considerable replacement of cartilage by fibrous tissue. 
The replacement of cartilage by bone is especially marked in the spine. 
In the peripheral joints this rarely occurs. The result in any case is 
likely to be bony ankylosis. 
Bones.-Here the changes vary with the form of the disease. In the 
periarticular type there may be comparatively little change, although 
in severe cases there is considerable atrophy. This, however, is not 
1 Guy's Hospital Reports, 1902, Ivii, 25. PLATE XIII 
Skiagram showing some thinning of the bones, marked 
destruction of the carpus, and alterations in the metacarpo- 
phalangeal and finger-joints. PLATE XIV 
Skiagram of Knee-joint, showing atrophy of the cartilage. ARTHRITIS DEFORMANS 
907 
distinctive and is similar to that seen sometimes with a fracture. If 
the changes in the cartilage result in its complete destruction the under- 
lying bone becomes exposed and eburnated. If the process is very 
acute the bone is more vascular than normal and necrosis may result. 
In some cases the bone becomes rarefied and less dense. This can be 
seen in the z-ray plates and in pathological specimens. 
In the so-called atrophic form the changes are of a different character. 
The most striking thing is the marked rarefaction of the bones through- 
out. With this usually goes destruction of the cartilage, so that as an 
end result (e. g., in the wrist) there may be entire loss of the divisions 
between the carpal bones. 
In the hypertrophic forms there is new bone formation, which may 
vary greatly in extent. The proliferation is most common at the edges 
of the articular cartilages, and the ossification may include parts of the 
adjoining fibrous or ligamentous tissue. This may result in bony out- 
growths of varying size -and shape. In some joints these projections 
may cause locking of the joint. In the knee-joint, bony outgrowths 
may be seen on the patella or at the edges of the femur or tibia. In the 
hip-joint these may be marked and surround the edge of the acetab- 
ulum with an irregular ring or bone. The so-called Heberden's nodes 
on the terminal phalangeal joints are formed of bone. The same changes 
are sometimes marked about the metacarpophalangeal joints. In the 
spine the new formation of bone may replace the cartilages, but more 
frequently occurs in the spinal ligaments, and the anterior and lateral 
ligaments may be entirely replaced. In all of these irregularity of the 
deposits is the rule, and it is rare to find the deposits the same on both 
sides of the vertebrae. The deposit of bone has been compared to lava 
which has flowed down and solidified. Wright has well compared the 
appearance as sometimes being much like a "guttering" candle. It may 
be noted that spondylitis with deposit of new bone is common in 
animals, especially in horses. 
Muscles.-Atrophy is the striking feature, and this is generally found 
to be spread throughout the muscle. In some cases degeneration has 
been found, and there is proliferation of the nuclei with increase of the 
interstitial tissue. At times the changes are probably due to direct 
extension from the joint, in other cases it is suggested that they are due 
to trophic influences. 
Nervous System.-No changes have been found which can be regarded 
as at all characteristic. In a few instances changes in the anterior horn 
of the cord have been noted. Vacuolation of the ganglion cells has been 
associated with altered staining reactions. In the peripheral nerves, 
neuritis has been found, but this is probably secondary. It is easy to 
appreciate that in arthritis of the spinal joints the inflammation may 
involve the nerve roots, or in the hip the sciatic nerve. These changes 
are apparently always secondary. 
Metabolism.-No very striking changes have been found. King,1 in 
a study of the metabolism, found a mild acidosis, with an increase in 
1 Johns Hopkins Hospital Bulletin, 1907, xviii, 274. 908 
DISEASES OF THE LOCOMOTOR SYSTEM 
the organic phosphates and a retention of calcium, magnesium, and 
phosphorus. The study of the urine did not suggest intestinal putrefac- 
tion. In some cases of the atrophic form the calcium output is much 
increased. 
Symptoms.-While an exact classification is difficult, it seems best to 
discuss the clinical features under the various forms, always having in 
mind that there are not invariably clear-cut distinctions between them, 
and that it may be difficult to say to which class a patient belongs 
1. Peri-articular Form.-This is the most frequent form, and com- 
prises the cases described as rheumatoid arthritis. The changes are most 
marked in the synovial membrane and peri-articular tissues. The 
cartilage is also affected in many cases, but the damage is rarely extreme; 
whatever change occurs in the bone or cartilage is secondary. In this 
group we find every grade of change, from that in the patient who has 
a light attack, resulting in very little thickening about the joint, to the 
patient who has changes so marked that he is a miserable cripple, per- 
haps unable to feed himself. There were 319 cases regarded as this form; 
there were others which were predominantly of this group, but showed 
features of the hypertrophic form as well. The females outnumbered 
the males in about the proportion of 3 to 2. 
Onset.-This is usually in one of two ways, acute in one or many joints 
or gradual in one joint, others being slowly involved subsequently. 
A point deserving of emphasis is the suddenness of onset in some cases. 
It is not unusual for patients to be able to state the exact date of onset; 
some can even give almost the hour. Sudden disability is sometimes 
a feature. Thus, in one patient the onset was with severe pain while 
he was walking on the street. The pain was so intense that he had to 
be carried home. The onset was acute in about 40 per cent of the cases 
in this series. The sudden onset is usually with an acute arthritis of 
many joints, which are red and swollen. Occasionally one joint is 
suddenly involved acutely for a day or two and then others follow. 
There is rarely any marked constitutional disturbance or chill. 
The gradual onset may be in one or several joints. It is often found 
that one joint becomes swollen or painful, perhaps never red, and after 
some weeks, or possibly months, another follows. As a rule, the gradual 
onset is of more serious omen than the acute one. 
Joint First Involved.-Leaving out the cases in which the onset is with 
the simultaneous involvement of many joints, many more have the first 
symptoms in one of the joints of the leg than in the arm. It has been 
said that the disease is prone to attack first the joints which are most 
used. However this may be, the statement so often repeated that the 
disease usually begins in the joints of the hands is certainly not correct. 
In this series the figures of the first joint affected, when this was definitely 
known, are as follows: Many joints, 22 per cent.; neck, 3 per cent.; 
shoulder, 12 per cent.; wrist, 8 per cent.; hand, including the thumb, 
10 per cent.; hip, 7 per cent.; knee, 19 per cent.; ankle, 3 per cent., 
foot, 13 per cent. Certain combinations comprised the remainder. 
Character of the Attacks-As in the mode of onset, so in the course, two 
principal forms stand out, the one with acute attacks of polyarthritis, the EXPLANATION OF PLATE XV1. 
Fig. 1. Metacarpo-phalangeal joint. 
Fig. 1 a. Photograph of the same joint after maceration. 
Fig. 2. Skiagram of a metacarpo-phalangeal joint, showing atrophy of carti- 
lage and formation of new bone at the base of the first phalanx and head of meta- 
carpal; a bony spur is also seen 
Fig. 2a. Photograph of the same joint after maceration; the atrophy of 
cartilage, eburnation at head of bone, formation of new bone around joint, and 
a typical bony spur are well shown. 
Fig. 3. Skiagram of first phalangeal joint, showing destruction of cartilage 
and fibrous ankylosis. 
Fig. 3 a. Photograph of same joint after maceration. 
Fig. 4. Skiagram of the wrist, showing bony ankylosis and considerable 
atrophy of the bones forming the joint. 
Fig. 4 a. Photograph of the same specimen after maceration. This specimen 
on dissection showed complete bony ankylosis of the carpi at their articulation, 
and fibrous ankylosis of the ulnar, radius, and metacarpi. 
Fig. 5. Skiagram from metacarpo-phalangeal joints, showing atrophy of 
cartilage and bone with fibrous ankylosis. 
Fig. 5 a. Photograph of macerated specimen of the same joint. 
Fig. 6. Skiagram of the thumb, showing atrophy of bone and destruction of 
the cartilage of the metacarpo-phalangeal and phalangeal joints. Well-marked 
fibrous ankylosis is present. 
Fig. 6 a. Photograph of the same specimen after maceration. 
EXPLANATION OF PLATE XVI. 
Fig. 1. Skiagram of a terminal phalangeal joint, showing atrophy of carti- 
lage, early deposit of new bone in the ligament of joint, and a light area. 
Fig. 1 a. Photograph of the same joint after maceration. A cavity contain- 
ing a deposit of urates is shown. 
Fig. 2. Skiagram of the first phalangeal joint, showing atrophy of cartilage, 
formation of new bone around joint, and a well-marked projection from the shaft 
of the first phalanx. 
Fig. 2a. Photograph of the same joint after maceration, showing a deposit 
of bone around joint and on the shaft of the first phalanx. 
Fig. 3. Skiagram of first phalangeal joint, showing atrophy of cartilage and 
formation of new bone. 
Fig. 3 a. Photograph of the same joint after maceration. 
Fig. 4. Skiagram of wrist, showing ankylosis. 
Fig. 4 a. Photograph of the same specimen after maceration. A longitudinal 
section has been made through the carpi and wrist-joint. The carpi are fused into 
a mass of light cancellous bone. The articulations of the carpi with the ulnar, 
radius, and metacarpi show fibrous ankylosis. 
Fig. 5. Skiagram of metacarpo-phalangeal joint, showing atrophy of cartilage 
and the formation of bony spurs at the head of the metacarpal bone. 
Fig. 5 a. Photograph of the same joint after maceration. The bony spurs 
are well seen. 
Fig. 6. Skiagram of a metacarpo-phalangeal joint from a case of arthritis 
deformans, showing atrophy of cartilage. 
Fig. 6a. Skiagram of a corresponding joint from a case of chronic gout, 
showing similar changes. 
1 The writer wishes to acknowledge the kindness of the Cambridge Committee, especially 
T. S. P. Strangeways, and the Cambridge Press, in being given permission to reproduce these 
plates, which are taken from the Bulletin of the Committee for the Study of Special Diseases, 
1907, vol. i, Nos. 3 to 9, Cambridge. PLATE XV 
FIG. 2 
FIG. 2 a 
FIG. 1 
FIG. 1 a 
FIG 3 
FIG- 4 
FIG. 4 a 
FIG. 3 a 
FIG. 6 
FIG. 6 a 
FIG. 5 
FIG. 5 a PLATE XVI 
FIG. 1 
FIG. 1 a 
FIG. 4 
FIG. 2 
FIG. 2 a 
I 
FIG. 4 a 
FIG. 3 
FIG. 3 a 
FIG. 6 
FIG. 5 
FIG. 5 a 
FIG. 6 a ARTHRITIS DEFORMANS 
909 
other with a slow, chronic, more or less progressive course. There are 
many combinations and varieties. Thus, there may be repeated acute 
attacks at intervals, the patient being comparatively well in the mean- 
time, or after the acute attack there may be a slow, gradual progress, 
or during the course of the chronic form there may be intercurrent 
acute attacks. However, it aids our clinical knowledge to keep these 
two classes in mind, recognizing that there are some which cannot be 
placed in either. In this series about one-third might be classed as 
acute, one-half as chronic, and the remainder with features of both. 
The Acute Form.-In many ways this group is much like rheumatic 
fever, the onset is often sudden, with the involvement of many joints. 
These are red, swollen, painful, and tender to the touch. There is often 
effusion in the larger joints. Certain joints not often involved in rheu- 
matic fever may be affected, such as those of the cervical vertebrae, 
the temporomaxillary and sternoclavicular joints. One point is impor- 
tant-when a joint is once attacked it rarely clears immediately and 
the involvement is usually for the duration of the attack. Again, as the 
acute features subside, the joint usually shows some permanent change 
which may be slight, but it is there. Thus, the finger-joints show slight 
increase in size or the capsule of the knee-joint is thickened. 
Certain general features may be noted. The temperature, as a rule, 
is not very high, being generally about 101° or 102° F. Occasionally 
higher figures are seen, sometimes 103° or 104°, but this is rarely for 
longer than a few days. After a time the temperature often drops to 
100° or 101°, and may run along about these figures for some time. 
Chills and sweating are both uncommon. The pulse generally shows 
a rate which is comparatively high, and this persists after the tem- 
perature has fallen. In a number of cases there is definite enlargement 
of the spleen, which may be made out by palpation or the area of dulness 
is increased. Glandular enlargement is almost always present. This 
is usually general, with the glands in relation to the affected joints show- 
ing the most marked increase in size. As a rule, the administration of 
the salicylate preparations has little result. The persistence of the 
arthritis is one of the most striking features. 
The Chronic Form.-In this the onset is usually gradual, frequently 
in one joint which swells gradually and is painful. There is rarely any 
redness, and effusion in the larger joints is not common. In some cases 
there may be complaint of pain in one joint for weeks before any change 
can be made out on examination. Other joints are progressively 
attacked, and it may be a year before there is general arthritis. In other 
cases one or two joints may be affected for a time, and then with a 
somewhat acute attack several joints are involved simultaneously. 
Again, the process may be rather subacute and gradually assume the 
slow, progressive type. The temperature, as a rule, is not high, often 
running for weeks from 99.5° to 101°. It is fairly constant and may 
never touch normal. The pulse is high, often 110 to 120 or more, and 
remains persistently so. Enlargement of the spleen is not so often found 
as in the more acute forms. The lymph glands in relation to the affected 
joints usually show enlargement. 910 
DISEASES OF THE LOCOMOTOR SYSTEM 
In seeking for an explanation of these various manifestations it is 
tempting, in the line of a probable infective cause, to consider that 
the acute type shows a more marked response and more active resist- 
ance to the infective agent and its toxins. There is little question but 
that the outlook, especially for the end, results in the joints, is much 
better in the acute forms. 
Pain.-This shows much variation, as in some patients there is com- 
paratively little so long as the joint is at rest; after the subsidence of 
acute features, pain usually occurs only on movement. In other patients 
severe pain is constant, and may continue long after all acute features 
have subsided. The amount of pain and degree of arthritis bear no 
relationship to each other-in fact, some of the patients who have the 
most severe pain have very slight joint changes. There are certain 
situations in which the complaint of persistent pain is common. One of 
these is about the heel; there may be no special tenderness on pressure, 
yet every attempt at walking causes so much pain that the patients 
have to remain at rest. The a>ray plates may show no changes to 
account for it. Pain in the wrist-joint and hand is sometimes very 
severe and constant. There may be severe pain from an associated 
neuritis, as occurs especially with involvement of the shoulder- or hip- 
joint. The pain may follow the distribution of a nerve, and be markedly 
increased by pressure over it. In the arthritis of the spinal joints the 
pain may be severe. 
Arthritis.-In considering this it is convenient to discuss the joints 
separately, as the features differ considerably. Vertebrae: Quite apart 
from the condition termed spondylitis deformans in this article, we find 
the joints of the spine not infrequently involved with the peripheral 
joints. In addition, the manifestations of spondylitis deformans may 
be found with a general polyarthritis, but in this there are definite 
changes in the spine. It is important to realize that the joints of the 
vertebrae may be involved in a general polyarthritis, and apparently 
no permanent change of any degree be left. There is an acute arthritis 
which subsides without causing structural change. The most frequent 
seat of involvement is the joints of the cervical vertebrae. There is 
complaint of pain, especially on movement; the head may be held more 
or less fixed, sometimes to one side, as the involvement is not necessarily 
symmetrical. Pain on movement may be very marked, sometimes more 
on movement in one direction than in another, and it may be marked 
on pressure; sometimes distinct grating on movement may be heard 
by the patient, and it is often audible. There is rarely any permanent 
fixation, as after a variable period the process subsides and movements 
are restored. Tenderness on deep pressure may persist for some 
time. 
In addition there may be a similar process elsewhere in the spine, 
with or without the cervical vertebrae being involved. With an arthritis 
of the peripheral joints there may be the complaint of severe pain in one 
part of the spine. This is tender on pressure, the spinal muscles are 
rigid, and movements cause severe suffering. The duration is often for 
several weeks. It is rare in these attacks to find signs suggestive of ARTHRITIS DEFORMANS 
911 
pressure on the nerve roots or marked muscular atrophy. It is impor- 
tant to realize that this may clear completely without any evidence 
of permanent change. If the point is looked into it is not rare to get 
a history of these attacks, and a number of patients with definite bony 
changes in the spine, give a history of previous attacks of this kind, 
after which there was apparently complete recovery before the per- 
manent condition appeared. 
Temporomaxillary Joint.-This is involved much more often in this 
disease than in any other form of arthritis. The patient complains 
of pain and difficulty in moving the jaw. The extent of involvement 
varies greatly; there may be little more than the complaint of pain, 
or the patient may be unable to open the mouth. Not infrequently 
the joint on one side only is involved, or one may be much more affected 
than the other. The process is often temporary. Fortunately the 
amount of permanent change is usually slight. For a time the patient 
may be able to open the jaws very slightly, and occasionally teeth have 
to be removed in order that the patient may be fed. 
Sternoclavicular Joint-There is pain, aggravated by any movement 
of the clavicle, and slight swelling. In rare cases the swelling may be 
quite marked. As a rule, the arthritis clears completely. 
Shoulder-joint.-This is frequently involved. In the acute stages there 
is pain and limitation of motion, especially in elevating the arm above 
the head and in abduction. There is often tenderness on pressure over 
the joint, and on anything more than the slightest movement. There 
is rarely marked effusion; the thickened capsule can often be felt. More 
or less associated neuritis is not uncommon, the pain being described 
as running down the arm or up the neck toward the head. As the acute 
features subside there is often considerable stiffness, crepitus, and 
thickening of the capsule, with diminished motion. If the process be 
long continued there is generally marked atrophy of the muscles of the 
shoulder girdle. After any attack of moderate severity there is almost 
sure to be some disability left behind. 
Elbow-joint.-In the acute attacks the elbow is generally held in a 
somewhat flexed position, movement is often considerably restricted, 
and the swelling may extend for some distance above and below the 
joint. This is often more marked about the elbow than in other joints. 
Movements other than those of flexion and extension are often dimin- 
ished. Pain is rarely extreme, but there may be considerable tenderness 
on pressure. In later stages extension is often limited, but there is 
rarely a marked degree of thickening left about the joint. 
Wrist-joints.-The changes here are generally characteristic. As a 
rule, there is conderable swelling, which may extend above and below 
the joint, so that the ordinary contour is entirely altered. The swelling 
frequently has a soft boggy feeling, and there is commonly decidedly 
less pain than in other joints in proportion to the amount of change. 
Crepitus is often obtained fairly early. In the subsequent stages the 
swelling usually subsides, but there is often very definite thickening 
about the joint, with some restriction of motion. In the early stages, 
there may be complaint of pain in the wrist-joint, especially on the ulnar 912 
DISEASES OF THE LOCOMOTOR SYSTEM 
side, sometimes associated with weakness, so that there is a tendency to 
drop objects, before there are any signs of acute arthritis. 
Hands.-The changes in the metacarpophalangeal joints are often 
very striking. There is generally some swelling, redness, and tender- 
ness, especially on motion. This leaves behind some thickening and 
disability. The degree of involvement in these joints is very variable, 
and only one knuckle may be involved or all of them on both hands. 
The condition after the acute features have subsided is generally very 
characteristic. In some cases there may be subluxation of the joints; 
one form of change, ulnar deflection, being quite common. This may 
come on very rapidly in acute cases-thus, in one patient it was marked 
within one month of an acute onset. A striking feature is atrophy of 
the interossei muscles. In occasional instances this may be present 
without any involvement of the joints of the wrist or hand. It is worthy 
of mention that the hands arc not always involved. 
Fingers.-1These are involved in a considerable number of the cases 
and the changes, as a rule, are very characteristic. In the earlier stages 
Fio. 52 
Hands showing marked late deformity. 
there is usually some swelling, which is often most marked at the first 
interphalangeal joint. The fingers are often held slightly flexed and 
full extension may be difficult. With involvement of the fingers and the 
knuckles it may be impossible for the patient to make a fist. As the 
acute features subside a very characteristic picture is usually left behind, 
the thickening about the joint giving the so-called pod-shaped or fusi- 
form finger. Subsequently there may be quite marked changes in the 
joints, especially in the way of partial dislocations. A common picture 
is flexion of the first phalanx, with hyperextension of the terminal ones; 
but all the joints may be partially flexed, or only the terminal one show 
hyperextension. Lateral deflection is quite common, especially in the 
terminal joint. Hyperextension of the terminal phalanges may come 
on very rapidly, as in one patient in whom this was marked within three 
weeks of the onset. The thumb joints are often involved. 
Sacro-iliac Joint.-Apparently this is not often involved in this form 
of the disease, and it may be difficult to decide whether the arthritis 
is of this form or hypertrophic. ARTHRITIS DEFORMANS 
913 
Hip-joints.-As a rule, the changes in this form of the disease are not 
marked as in the hypertrophic form. During an acute attack of poly- 
arthritis there is complaint of pain in the hip-joint and some pain on 
movement. On examination little may be made out except slight restric- 
tion of movement, especially on abduction and internal rotation. If 
only one joint be involved the patient may walk so as to favor that side. 
As a rule, permanent damage of any extent does not result, although 
at times there may be some fixation with the thigh flexed. 
Knee-joints.-This is the joint most frequently affected and usually 
on both sides. In the acute stage the joint is often markedly swollen, 
red, and tender. Effusion often occurs, and the patella may "float." 
On palpation there may be considerable tenderness, especially on the 
inner side, and sometimes over the inner part of the head of the tibia. 
Tenderness may also be marked in the popliteal space. The joint is 
commonly held somewhat flexed. As the effusion is absorbed the 
thickened capsule can usually be felt best on the inner aspect of the 
joint. If there is much change about the joint, some flexion usually 
occurs with contraction of the tendons, which later may go on to sub- 
luxation. In some very acute cases, and in some chronic ones, in which 
there is a good deal of muscular atrophy, marked enlargement of the 
bones may be suggested. This, however, is apparent and not real, being 
suggested by the atrophy above and below the joint. 
Ankle-joints.-They often show much the same features as the wrists, 
considerable puffy sw'elling extending above and below the joints. There 
may be considerable stiffness and limitation of motion, which, however, 
is usually not permanent. The bones of the feet may be involved with 
the ankle-joint or separately. 
Toes.-The changes are much like those seen in the fingers, but are 
usually less marked. In some cases there is the complaint of persistent 
pain in one or more toe-joints without any evidence of active arthritis. 
Ankylosis may occur in the great toe-joint. 
General features of the arthritis, (a) Symmetry of the Involvement.- 
Much stress was laid on this by the earlier writers, but it is by no means 
the rule when the figures as to the frequency of the involvement are 
studied. Thus, in 35 among 126 cases of arthritis of the shoulder the 
process was unilateral, in 22 of 76 cases of the elbow, and in 24 of 89 cases 
of the hip. In certain instances the disease may involve one side of the 
body only, or all the joints of one extremity. It is rare for a single pair 
of joints, as the knees, to be involved alone. 
(&) Distributions of the Lesions.-This is very variable, and every 
combination occurs. In the total number of joints involved the arms 
exceed the legs, but only by a small amount. If the larger joints are 
considered, the contrary is the case, and the greater number is in the legs. 
The frequency with which the hands are involved accounts for the larger 
total for the upper extremity. 
(c) Variability in the Changes.-This is seen especially in the acute 
attacks, which involve many joints. The majority may recover without 
much change, the brunt of the attack falling on one joint. Both hands 
may seem to be equally involved at first, and yet one regains almost 
vol. v-58 914 
DISEASES OF THE LOCOMOTOR SYSTEM 
complete function, the other showing marked change. Sometimes 
there seems to be some influence from occupation and special strain on 
particular joints. 
Muscular Atrophy.-This is commonly present to some extent, but 
varies greatly both in its extent and rapidity of development. In some 
patients, especially in acute attacks, atrophy advances so quickly that 
some trophic influence seems necessary to account for it. This is usually 
most marked in the hands, the interossei muscles showing very rapid 
change. In other cases the progress is slow and disuse may seem to be 
quite a sufficient explanation. In some patients with repeated acute 
attacks there may be little change with the first one or two, but each 
subsequent attack shows more change. About the shoulder-joint the 
atrophy is sometimes well shown. An interesting point is the occurrence 
of muscular twitching, sometimes seen as a fine fibrillary tremor. 
Temperature.-Two points stand out in the consideration of this: first, 
that the fever often shows an elevation much less than might be expected 
from the degree of arthritis; and second, that it may persist for a long 
period very slightly above normal. The figures for patients who were 
under observation long enough to obtain satisfactory records (the patients 
in whom all acute features had subsided are not considered) showed that 
in the largest number the temperature averaged from 99° to 100° or 101°. 
Although at times it was higher, yet this rarely persisted, and many 
patients go for weeks with a temperature between 99° and 100°. This 
suggests a chronic low-grade infection. In some cases a rise to higher 
figures occurs with a complication, such as pleurisy or pericarditis. In a 
few patients no such explanation could be found. The persistence of 
slight fever with a relatively high pulse rate is often an important point 
in diagnosis. It must also be noted that with an acute polyarthritis the 
temperature sometimes remains practically normal. 
Circulatory System.-Among 500 cases there was evidence of definite 
valvular diseases in 40 instances. The lesion was mitral insufficiency 
in 15 cases, mitral stenosis in 5, combined mitral stenosis and insufficiency 
in 5, aortic insufficiency in 6, aortic and mitral insufficiency in 6, and 
aortic insufficiency with mitral stenosis and insufficiency in 3. In about 
one-quarter of these there was arteriosclerosis, so that the lesions might 
be regarded as sclerotic; in the remainder they suggested an endocarditis. 
In 26 cases there was an apical systolic murmur which was not definitely 
proved to be due to organic valvular disease. In some others there were 
signs of myocarditis. 
Pericarditis occurred in 6 cases under observation and during the 
course of the acute arthritis. Effusion followed in 3; in one of these 
tapping was done repeatedly and the fluid injected into guinea-pigs, 
but with negative results. In 3 cases an acute pleurisy occurred with 
the pericarditis. 
Pulse.-This shows an increase in the rate in the majority of cases. 
In about two-thirds of the cases the rate was over 90. The most frequent 
range is between 90 and 110, only a few patients having a persistent rate 
above 120. This increase in rate is true not only of the acute periods, 
but frequently persists after these have passed, and is also seen in the ARTHRITIS DEFORMANS 
915 
slowly advancing forms. When fever is present the pulse rate is usually 
elevated quite out of proportion to the temperature. In a number of 
cases there were definite evidences of myocarditis, occasionally with 
dilatation. The blood-pressure shows no special abnormality. 
Respiratory Tract.-Bronchitis was present in a few instances, and in 
some it was suggested that the infection which caused it might stand in 
some relationship to the arthritis. Lobar pneumonia occurred in several 
patients during an acute arthritis, in one being associated with a marked 
exacerbation of the joint condition, which rapidly subsided after the 
pneumonia was over. The greatest interest, however, is attached to the 
occurrence of acute pleurisy with the arthritis. This occurred in 7 cases, 
one of which was tuberculous and associated with tuberculous disease of 
the lung. The others were not tuberculous, as was shown by the nega- 
tive reaction to tuberculin given after the acute features had subsided. 
In 3 patients the pleurisy was associated with an acute pericarditis. The 
importance of this as suggesting some infectious process is evident. 
Glandular Enlargement.--General enlargement is common and the 
glands in relation to affected joints are usually enlarged. The degree 
of enlargement varies greatly, those glands which are in relation to the 
more acutely affected joints showing the greatest involvement. Thus, 
the joints of one arm may be involved and those of the other be normal, 
the glandular enlargement being on one side only, or general glandular 
enlargement is present, more marked on the affected side. Occasionally 
the enlarged glands are slightly tender. Histological examination shows 
only a hyperplasia. In some cases the enlargement is persistent. 
Spleen.-Among 166 cases with notes as to the size of the spleen, it was 
found that there was enlargement in 38, in 30 the spleen was palpable, 
and in 8 the area of dulness was definitely increased. In practically all 
the cases with splenic enlargement, there was general glandular enlarge- 
ment. The cases of the so-called Still's disease are included here and 
will be referred to later. It may be said in a general way that enlarge- 
ment of the spleen was relatively more common among the younger 
patients, and in acute than in chronic attacks. 
Subcutaneous Nodules.1-Long regarded as diagnostic of rheumatism, 
this view can no longer be held, as they may occur without evidence 
of arthritis of any kind. However, it can be said in the majority of 
cases they are associated with arthritis, and in the experience of the 
writer this has been rheumatic fever in children and arthritis deformans 
in adults. In this series they were found in 17 patients (3.4 per cent.), 
all being adults. They are generally found about the elbow or wrist, 
sometimes on a finger, are rather hard, round bodies, between the size 
of a grain of wheat and a pea, quite frequently movable, and sometimes 
tender. Their duration is very variable and they may disappear in a 
few days or persist for weeks. Occasionally patients volunteer the state- 
ment that they have been noted to appear, disappear, and reappear. 
They do not seem to be associated with any severe type of the disease 
or with any special joint features. 
1 See Futcher, Johns Hopkins Hosp. Bull., 1895, vi, 133, and Hawthorne, Rheumatism, 
Rheumatoid Arthritis, and Subcutaneous Nodules, London, 1900, J. & A. Churchill. 916 
DISEASES OF THE LOCOMOTOR SYSTEM 
Urine.-This showed no features of moment. In about 20 per cent, 
of the cases albumin was found, and in rather more than half of these 
casts were present. Diabetes co-existed in four patients. Estimations 
of the amount of uric acid were made in a number of cases, but showed 
no constant departure from normal. 
Blood.-It may be said that the examination of the blood yields little 
of importance. The majority of patients have a pale, sallow look, 
and often suggest a degree of anemia which is not confirmed by the blood 
count. The average percentage of hemoglobin in 230 cases was about 
80; the average red count in 220 cases was 4,800,000, and the average 
leukocyte count in 220 cases was 8500 per cmm. Leukocyte counts 
taken during the occurrence of a complication, such as pneumonia, are 
not included. The leukocyte count in 65 cases was over 10,000 and in 
13 cases below 5000 per cmm. It is surprising to find that frequently 
during an acute polyarthritis the white corpuscles are not increased. 
The average differential count of the leukocytes in 36 cases showed: 
Polymorphonuclears 76.3 per cent., small mononuclears 15 per cent., large 
mononuclears and transitionals 6.3 per cent., eosinophiles 1.6 per cent., 
and other cells 0.8 per cent. In the case of complications, such as 
pleurisy, there was a marked polynuclear leukocytosis, which showed no 
unusual features. It may be said that the blood condition was practically 
the same in all the forms of the disease. The patient with acute arthritis 
involving many of the peripheral joints showed practically the same blood 
condition as the one with the spine alone involved. 
Skin.-One of the most striking phenomena is the presence of pigmen- 
tation, which is especially apt to occur on the face, neck, arms, and hands. 
It may occur almost at any age, the youngest in this series being nineteen 
years and the oldest seventy-eight years. There is nothing distinctive 
about this pigmentation. It may be fairly general over the face and neck 
or occur in irregular areas; on the hands and arms it more often is seen 
in smaller, more irregular areas. In some patients there are many small 
pigmented areas, much like freckles. As a rule, the pigmentation is 
associated with acute general features, and as these subside the discolora- 
tion lessens, sometimes, however, leaving some "staining." 
In some cases, especially in those with marked and rapid muscular 
atrophy, the skin has a curious glossy appearance. This is usually in the 
hands or feet, but may extend to the arms or legs. In some patients 
the hands and feet may be very blue. Profuse sweating of the hands and 
feet is common, and may be a source of great discomfort. This may 
persist long after acute features have disappeared. In the more chronic 
forms the skin is often harsh and dry. There may be disturbance of 
sensation, especially as regards pain, and this may be found to differ 
on the two sides if the arthritis is asymmetrical. IL L. Jones1 notes that 
there may be areas showing sensory changes and sometimes local sweating. 
Complaint of numbness or tingling, sometimes of severe burning sensa- 
tion, is not uncommon. Erythema, urticaria or purpura, may occur. 
Herpes of the lips has been seen occasionally. 
1 The Lancet, 1902, ii, 1746. 917 
ARTHRITIS DEFORMANS 
Reflexes.-These very commonly show some alteration, but the 
findings are by no means constant. The deep reflexes are, as a rule, 
markedly increased, but may be normal or decreased, although they are 
rarely absent. In many instances they vary in relation to the joint 
involvement, and may be different on the two sides of the body or in one 
extremity. They are usually increased on the diseased side. If there 
is involvement of the temporomaxillary joint, the jaw-jerk is generally 
increased. The superficial reflexes are very variable, and may be much 
increased or greatly diminished. The cremasteric reflex is very variable, 
sometimes being markedly increased, in others normal, diminished, or 
absent. Ankle clonus was present in a few cases on both sides and in 
one case on one side only. Patellar clonus was obtained rarely. In 
many cases the reflexes were especially exaggerated when there was 
marked muscular atrophy. R. L. Jones pointed out that there is often a 
correlation between the affected joints and certain reflexes. Thus if 
the middle and ring fingers alone are involved, the flexor tendons give a 
more marked response to tapping than the extensors. The reverse is 
the case if the thumb and index finger be involved. He points out that 
there is often marked agreement in the gluteal and plantar reactions. 
The increase in myotatic variability he regards as of some aid in prognosis 
as it appears early, and if persistent suggests that the process is still 
active. Its disappearance is of good omen. Jones lays stress on the 
association of conditions (e. g., of the plantar and gluteal reflexes), as 
suggesting some affection of certain spinal segments. In some cases 
there may be considerable uncertainty of movement, which usually does 
not amount to tremor. 
(Edema of the feet and legs sometimes occurs, apart from any renal or 
circulatory lesion. It may be seen in acute attacks or more often in 
patients with marked lesions in the joints of the legs, especially if there 
is flexion of the knees with immobility. It is also seen in the patients 
who are unable to get about and sit all day in a chair. 
Features of the Disease in Children.-There is much uncertainty as 
to the nature of chronic arthritis in early life. As Garrod points out, 
chronic arthritis in childhood is of various kinds. Some rare cases suggest 
the hypertrophic or osteo-arthritic type which may be the same as that 
seen in adults. Again, bony changes may occur after hemophilic arthritis, 
as shown by Bowlby. Garrod refers to rare cases in which chronic 
arthritis follows acute rheumatic fever, a condition which the writer 
must confess he has never been able to recognize. Then certain cases 
of tuberculous polyarthritis in children present curious features, and 
syphilitic arthritis in childhood may be chronic. However, all these 
are rare conditions and not so common as the form of polyarthritis in 
childhood, which has been termed "Still's disease." Special attention 
was drawn to this by G. F. Still,1 of London. There are three prominent 
features: a chronic arthritis characterized by enlargement of the joints, 
marked general glandular enlargement, and enlargement of the spleen. 
While different diseases may be included under the term, as has been 
1 Medico-Chirurgical Transactions, London, 1897, Ixxx, 47. A System of Medicine 
(Allbutt), London, 1897, iii, 102. 918 
DISEASES OF THE LOCOMOTOR SYSTEM 
suggested, yet the writer is of the opinion, that it is not a distinct entity, 
but arthritis deformans occurring in childhood. All the cases in this 
series have been of the peri-articular type; none have been recognized 
as of the hypertrophic or osteo-arthritic type, although one of the hyper- 
trophic form in this series began at the age of fourteen years. There 
seems no reason why the hypertrophic form might not occur in earlier 
years. On the view that these cases are manifestations of arthritis 
deformans in childhood, they are included in the general statistics given 
before, but their special features will be considered here. 
Age.-At what age should we draw the line? From the experience of 
this series it is impossible to say arbitrarily what patients should be put 
in the class of "Still's disease." There is a series from four years of age 
up to eighteen with a gradual lessening of the typical p'cture in the older 
patients. In this series one boy, aged eleven years, showed a perfectly 
typical picture of it. Practically the same condition may be seen in 
still older children, and enlargement of the spleen and lymph glands is 
by no means uncommon in later years. In childhood these are undoubt- 
edly likely to be more marked and persistent. 
In these cases in childhood the arthritis comes on insidiously and 
advances slowly. It is of the peri-articular type, the thickening is usually 
marked, and there may be some limitation of motion. There may be 
marked muscular atrophy and wasting. Enlargement of the glands is 
marked; they are discrete and rather hard. Their size often stands 
in direct relation to the degree of arthritis in the neighboring joints. 
The spleen is usually felt below the costal margin and is hard, its size 
varying with the arthritis. 
The condition is chronic and in the majority of reported cases has 
advanced until the patient is completely crippled. This has not been 
the experience of this series. One patient left somewhat improved, 
a second left the hospital with the disease apparently arrested and the 
condition of the joints improving. When seen a year later there had 
been no return of acute symptoms, the child had grown, was able to get 
about well, and showed only some slight thickening about the joints. 
A third patient showed considerable improvement and was able to get 
about. A fourth, aged eleven years, remained in much the same state. 
The Peri-articular Form in Advanced Life.-While the view is sometimes 
expressed that in elderly patients the disease is likely to be more chronic, 
yet this is not by any means always the case. The first attack may be 
a very acute one, as in a patient, aged seventy years, who had never 
suffered from any arthritis previously. Her attack began suddenly, 
involved many joints, and resulted in considerable permanent change 
within three months, after which the process subsided. In other instances 
there may have been a rather chronic progress, or perhaps slight attacks 
years before, and later in life the first acute attack appears. There is 
one form occurring usually in stout elderly women which is worthy of 
note. In such patients the process may be rather chronic, and perhaps 
has caused slight changes in the small joints, but the brunt of the attack 
falls on the knee-jerks. These are painful, rather stiff, and apt to "give 
way" without warning, so that the patients are afraid to walk without ARTHRITIS DEFORMANS 
919 
some support. On palpation there may be only slight tenderness and the 
thickened capsule may be felt. Painter aptly applies the term "knee- 
sprung" to them. He regards them as being of the hypertrophic type, 
but some are undoubtedly peri-articular. 
The So-called "Monarticular" Forms.-This is not a good term, but 
has been used to describe a form of arthritis usually seen in advanced 
life, in which one large joint is especially involved. It is a misnomer 
in the majority of cases, for, if careful examination be made, it is generally 
found that there is slight involvement elsewhere. The joints most 
frequently involved are the shoulder and hip. The changes in the 
Fig. 53 
An example of the so-called monarticular form involving the right shoulder-joint. This patient 
had slight changes in two fingers, the result of an attack some years before. 
shoulder-joint are usually of the peri-articular form, while those in the 
hip-joint are of the hypertrophic form. The process sometimes attacks 
the arm which is especially used (0. g., the right arm in a carpenter). 
The principal symptoms are severe pain, marked disability, especially 
for certain movements, such as raising the arm and putting it behind 
the back, and muscular atrophy, often involving the whole shoulder 
girdle (Fig. 53). Two conditions have to be kept in mind, one is associated 
with it, and the arthritis is often unrecognized, the other it resembles 
and may not be suspected. The first is neuritis, which true enough is 
present, but secondary to the arthritis, and the other is subdeltoid bursitis 920 
DISEASES OF THE LOCOMOTOR SYSTEM 
As will be pointed out later, one large joint (shoulder or hip) may be 
involved with the whole spine (spondylitis deformans) but in these cases 
no special peculiarity is manifested in the peripheral joint, except perhaps 
marked muscular wasting. 
2. Atrophic Form.-This is much the least frequent in occurrence, which 
is fortunate, as it is in many ways the most serious as regards outlook. 
The most marked anatomical change consists in the atrophy of the bone, 
which may be very advanced. There is marked loss of the calcium 
salts. With this there may be extreme disorganization of the joint, which, 
as regards function, is practically destroyed. The progress in some cases 
is extremely rapid, and they might be described as fulminating. 
This form usually occurs in young adults, and more often in females 
than in males. It usually begins about the wrist and hand. The symp- 
toms do not differ very greatly from those in the peri-articular form, 
except that muscular atrophy and disorganization of the joints are more 
marked. The deformity is often greatly due to dislocation, which is 
favored by the marked loss of cartilage. Ankylosis, which is usually 
fibrous, may follow. The hand and wrist have a characteristic appear- 
ance. The patient may have difficulty in even lifting the hand or in 
performing any of the ordinary movements. There may be subluxation 
of the wrist-joint and marked atrophy of the interossei muscles. The 
metacarpophalangeal joints may be much relaxed, and there may be 
great relaxation of the joints of the fingers. The general condition is 
greatly altered. The patients are emaciated, sallow and anemic, and 
are very weak. With the progress of the disease, deformities are common. 
The z-ray plates show atrophy of the bones, quite different from that 
seen from disuse. With this the cartilage disappears, and the wrist-joint 
may be represented by little more than a structureless mass. The 
progress is usually downward, and the outlook in this form is grave. 
3. Hypertrophic Form (Osteo-arthritis).-In this the changes are more 
especially in the cartilage and bone, with varying degrees of involvement, 
but the other structures do not escape and the synovial membrane 
may be involved. The form is usually polyarticular and apparently 
may come on at any age, but the majority of patients are older at the 
time of onset than those belonging to the peri-articular form. In 
advanced life the lesions are often more suggestive of degenerative than 
of inflammatory changes, and yet it has to be remembered that the 
joints are often seen long after the acute changes are over. There were 
106 cases belonging to this form in the series. There are two varieties 
which require special mention, the so-called Heberden's nodes and the 
spinal form, usually termed spondylitis deformans. The term spondy- 
litis includes many varying conditions. As used here the arthritis of the 
joints of the spine which occurs without any evidence of permanent 
bony change-already referred to under the discussion of the peri-articular 
form-has not been termed spondylitis, but arthritis of the spine. In 
this section the word spondylitis will be used for the cases with definite 
changes, although really the term includes any inflammatory condition. 
Some would doubt the propriety, certainly in many cases, of including 
spondylitis deformans under arthritis deformans. It is one of the dis- ARTHRITIS DEFORMANS 
921 
puted points. The character of the lesions and the frequent association 
with the undoubted lesions of arthritis deformans in the peripheral 
joints seem important evidence to the writer. Changes in the vertebrae 
frequently occur with arthritis elsewhere, and this may be either of the 
peri-articular or hypertrophic form. 
General Features-As a rule, the arthritis is polyarticular, the most 
common exception being the involvement of one hip-joint, the usually 
termed morbus coxae senilis. As a rule, the age of the patients is greater 
than in the peri-articular form. Thus, A. G. Garrod found that among 
100 cases, 5 were from thirty to forty years of age, 26 from forty to fifty, 
42 from fifty to sixty, 21 from sixty to seventy, and 6 over seventy years. 
Had the cases of malum coxoe senile been included the average would 
have been higher. Yet the disease may come on earlier than shown by 
these figures. Thus, in one patient of this series, with the onset at the 
age of fourteen years, the progress was rapid, and in two years he showed 
well-marked hypertrophic changes in many joints. 
As a rule, the general features are not so severe as in the other form. 
The arthritis is not so acute, and the temperature and pulse are lower. 
The patient's general condition is much less apt to suffer, and the changes 
in the joints are much less serious on the whole. The principal effects 
are more or less mechanical, and muscular atrophy and contractures 
occur to a much less degree. 
Heberden's Nodes.-"What are those little hard knobs, about the 
size of a small pea, which are frequently seen upon the fingers, particu- 
larly a little below the top near the joint? They have no connection with 
the gout, being found in persons W'ho never had it; they continue for 
life; and being hardly ever attended with pain, or disposed to become 
sores, are rather unsightly than inconvenient, although they must be 
some little hindrance to the free use of the fingers." So wrote Heberden 
under the heading "Digitorum Nodi." 
These nodes are small bony outgrowths at the terminal phalangeal 
joints, sometimes covered by a projection of the synovial membrane. 
They are more common in advanced years, and are found in many 
people who have not had any evidence of arthritis elsewhere. They 
may appear in early life, as in one patient, aged sixteen years, in whom 
the arthritis began two years before, it wras of the peri-articular form 
in the other joints, but he had definite Heberden's nodes of quite large 
size. They may be the only manifestation of arthritis or occur with 
a polyarthritis of the peri-articular form or with a spondylitis deformans 
as the only other manifestation. In some cases they are the first 
manifestation of a general arthritis. In one patient, a woman, aged 
thirty-two years, they appeared very rapidly on all the fingers. 
They only rarely give rise to symptoms, but some patients complain 
greatly of the pain, which is usually aggravated by much use or by any 
injury such as caused by knocking the fingers against some hard object. 
Numbness, a "dead" feeling or tingling, is sometimes complained of, 
especially by elderly patients. They are unsightly, and frequently 
cause disturbance on this account. Their distribution is not necessarily 
regular on the two hands, from one to eight fingers may be involved, and 922 
DISEASES OF THE LOCOMOTOR SYSTEM 
in practically any combination. If one finger is especially used in any 
occupation, as sewing, the nodes on it are likely to be larger than on 
the other fingers. Occasionally there may be an active arthritis, and 
the joints are red, swollen, and painful. The onset is sometimes quite 
acute, and the bony outgrowths appear rapidly. The nodes can be 
readily felt, and stand out markedly in the z-ray pictures. Sometimes 
deflection of the end joint of the finger may be caused by them, although 
this is not common. They often cause some flexion of the end phalanx, 
and may interfere considerably with the finer movements of the fingers. 
Garrod points out that small transparent cystic swellings, which he 
regards as probably being herniae of the synovial membranes, are some- 
times seen on the posterolateral aspects of the joints. 
The question often arises as to the significance of these nodes. Many 
patients who have them carry the opinion that they are a manifestation 
of the effects of "uric acid." In rare cases they occur in patients with 
gout, but as a rule, they may be regarded as almost invariably a mani- 
festation of arthritis deformans. 
Thumb.-The carpometacarpal joint is not infrequently attacked. 
It may be said that involvement of the thumb-joint is more common 
in arthritis deformans than in any other form of arthritis. There may 
be a good deal of bony enlargement, with well-marked crepitus, and more 
complaint of pain in this than in any other joint. The swelling may be 
quite marked. 
Metacarpophalangeal Joints.-These may show marked bony out- 
growths, which sometimes are very prominent and project. If with this 
the joints are much relaxed, great deformity may result. 
Hip-joints.-While the involvement of this joint is especially fre- 
quent and may be the only manifestation of the disease, yet it may be 
part of a general arthritis. The more carefully the histories are studied 
in the cases regarded as monarticular, the more often it will be found 
that there were previous attacks of acute polyarthritis, which possibly 
have left but little damage, and the more thoroughly the patients are 
examined, the more often will slight changes be found in other joints. 
This may be in the form of Heberden's nodes, or perhaps something in 
a shoulder- or finger-joint. Still, in a great number the principal change 
is in the hip-joint. As a rule, only one hip-joint is involved. 
This form occurs usually in advanced life, although examples in early 
life are seen. It is much more common in males, and a history of trauma 
is frequently obtained, although there is often the suspicion that this is 
secondary to the onset and determined the first marked manifestation. 
The most marked features are pain and disability. Pain is not constant, 
but may be very severe. It may be referred to the joint itself or to the 
groin, or be especially felt in the leg, sometimes along the course of the 
sciatic nerve and sometimes being referred to the knee or ankle. This 
sometimes leads to the diagnosis of sciatica, and it has to be remem- 
bered that a neuritis may be present secondary to the arthritis, exactly 
as in the shoulder-joint. Disability may be marked, with definite limi- 
tation of motion. The patients often have a characteristic gait, saving 
the affected leg, perhaps with tilting of the pelvis. Sometimes walking ARTHRITIS DEFORMANS 
923 
is almost impossible, and going up steps is especially likely to cause 
pain. The patients get up from a sitting posture slowly, and may have 
difficulty in crossing the affected leg over the other, perhaps having to 
use the hands to accomplish this. On movement of the leg there may 
be severe pain and marked grating. Limitation of motion is especially 
marked on abduction and inward rotation. Extreme flexion may be 
impossible. There may be shortening of the leg in advanced stages, 
but in the early stages it is usually only apparent. 
There is commonly some muscular atrophy, which may be marked, 
and is found principally in the gluteal region, which may show distinct 
flattening. The knee-jerk of the affected leg is usually increased. 
Knee-joint.-This may be involved as part of a more or less general 
arthritis, but not infrequently this joint suffers more and may be the 
most severely attacked. There is usually the complaint of pain and 
stiffness, with difficulty in going up stairs, kneeling or rising from a 
sitting to a standing position; a feeling of insecurity with a giving way 
of the knee is not uncommon. The joint rarely shows much swelling, 
and in the early stages there is not much restriction of motion. On 
movements of the joint a loud grating noise may be produced, which 
(iarrod well terms a "scrunch." In the early stages, and some patients 
do not progress beyond this, it may not be possible to determine that 
hypertrophic changes are present without an x-ray examination. Quite 
often bony outgrowths from the patella are the first to be recognized 
by palpation. With progress of the malady, the bony outgrowths become 
more prominent and may be felt about the femur and tibia. With 
this there is greater disability and restriction of motion. 
The results of the x-ray examination suggest that a number of instances 
of moderate involvement of the knee-joints, seen especially in elderly 
patients, are of the hypertrophic type. There is but slight arthritis 
elsewhere, and the process may never advance very far. 
Sacro-iliac Joint.-This may be involved, although it may be difficult 
to determine exactly how much change there is in the joint. There is the 
complaint of pain, which may be referred away from the articulation. 
Spondylitis Deformans.-This is sometimes termed "poker-spine, 
poker-back, or rigidity of the spine," and deserves special attention on 
account of its frequency and importance. As already noted under the 
discussion of the polyarticular form, involvement of the joints of the 
spine may occur without, so far as can be determined, any permanent 
change remaining. These may be termed spondylitis, but hardly spon- 
dylitis deformans, so that here the designation is applied to those cases 
in which there is evidence of definite structural change. 
This condition in the spine may occur as the only manifestation, or 
may be associated with arthritis of the peripheral joints. Of the 120 
cases of spondylitis deformans in this series, in 60 the spine alone was 
involved, and in 60 this was associated with arthritis elsewhere. While 
the view is taken here that these changes in the spine are manifestations 
of arthritis deformans, yet this is by no means the universal opinion, 
and there has been much controversy regarding both the place of the 
condition in a general classification and as to whether there are really 924 
DISEASES OF THE LOCOMOTOR SYSTEM 
distinct entities included. Thus, Bechterew has described under the 
name "Steifigkeit der Wirbelsaule" what he regards as a distinct entity. 
Strumpell, under the name of "Chronisch ankylosierende Entziindung 
der Wirbelsaule," and Marie, under the term "Spondylose Rhizome- 
lique," have designated forms with somewhat different manifestations. 
An article by Rhein1 gives a summary of much of the literature. The 
question as to the identity of the various forms is difficult to decide, 
but when we remember how diverse the manifestations of the disease 
are in other joints, it seems reasonable to expect that the same should 
be true of the spine. The extent of spinal involvement is very variable; 
in the lower spine the process is frequently most marked on one side, 
in the cervical region it is much more often on both sides. One 
patient has the spine alone involved, and then the spine and one hip; 
another the spine and both shoulders and both hips; one patient has 
marked signs of pressure over the nerve roots, another has practically 
none; one patient has kyphosis, another has none; all these variations 
are found in such a varying degree that to distinguish various forms 
from their presence or absence seems a mistake. The writer feels that 
these descriptions are of differing pictures of the manifestations of 
arthritis deformans. 
In the study of the symptoms it is well to have in mind the lesions 
which are present. These involve an arthritis of the spinal joints, changes 
in them which are frequently associated with proliferation of bone, 
atrophy of cartilage with its replacement by bone, and osseous changes 
in the ligaments. These last are involved frequently, perhaps the 
anterior lateral ligament most often of all. The extent varies greatly, 
and the process may extend along several vertebrae, making practically 
a solid column for some distance, or a process of one vertebra may be 
joined to that of the next one. The transverse processes are not infre- 
quently involved. On account of the anatomical relations, there is 
frequently some affection of the nerve roots either through extension 
of the inflammation by contiguity or by pressure. Following this there 
are symptoms in the distribution of these nerves. Clinically the results 
of these changes are seen in many ways. Thus, the arthritis causes 
pain and muscle spasm; if the articulations of the ribs with the verte- 
bra: are involved there is severe pain on breathing, and perhaps almost 
entire absence of thoracic respiration. The formation of new bone may 
interfere with the spinal movements, and we have more or less rigidity 
as a result. If there is rapid destruction of the intervertebral cartilages 
without their being replaced by bone we have marked bowing of the 
spine; if there is rapid ankylosis and replacement of the cartilage by 
bone, the spine is fixed in a straight position, the so-called "ppker- 
spine." The osseous changes in the ligaments give rigidity and limita- 
tion of motion. Pressure on or involvement of the nerve roots may 
give marked sensory disturbances; thus there may be severe abdominal 
pain or pain in the distribution of the sciatic nerve. When it is also 
remembered that the process is usually not symmetrical and may involve 
1 Jour. Am. Med. Assn., 1908, li, 462. ARTHRITIS DEFORMANS 
925 
all or only a part of the spine, the possibilities for great variation are 
very evident. This should be kept in mind in the study of the symptoms. 
As to the etiology, the most reasonable view is that it is due in some 
way to an infectious process. The changes are exactly like those which 
have been found in some cases of the so-called typhoid spine. It has 
long been known that there may be a spondylitis in association with 
gonorrhoea. Then in some cases the association with some focus of 
infection may be very definite. Thus, a tonsillitis or an infection of one 
of the nasal fossae apparently stands in direct etiological relationship, 
and with proper treatment of this the spondylitis promptly subsides. 
The importance of trauma must be noted; sometimes this accentuates 
the symptoms, but in other cases undoubtedly determines their onset. 
Occurrence.-This is probably much more frequent than has generally 
been supposed. In this series there were 120 cases, of which 60 were 
spondylitis alone and 60 spondylitis with involvement of the peripheral 
joints. As regards sex, the figures are striking, for it occurs in the great 
majority of instances in males. As already noted, it makes a great 
difference in the relative figures for the sexes in arthritis deformans 
generally as to whether or not these cases of spondylitis are included. 
Of the 120 cases here, 97 were males and 23 females. Of the cases in 
females, the great majority were spondylitis with involvement of the 
peripheral joints. As to color 113 were white and 7 colored. This is 
a very striking difference, and very difficult to explain. The colored 
race are just as susceptible to infection, perhaps more so, and trauma 
must be as common in them as in the whites. 
Age.-There was no special difference in the pure spondylitis cases and 
in those with other joints involved. The age at onset in the cases in 
which this could be determined was: 
11 to 20 years 9 
21 " 30 " 29 
31 " 40 " 34 
41 " 50 " 17 
51 " 60 " 16 
61 " 70 " 8 
The time of onset does not differ greatly from that seen in arthritis 
deformans generally, although the average is at a slightly earlier age. 
It is worthy of note that the patients with the onset after the age of 
sixty years were all examples of spondylitis associated with changes in 
the peripheral joints, and in all of these the onset was fairly acute. As 
a rule, the onset was gradual. Trauma, usually described as "strain" 
while lifting, was associated with the onset in 14 cases. 
The complaint made by the patient is of interest. The largest number 
complained of pain in the back, but in many there was complaint of 
pain in the legs, abdomen, or thorax. Several made the complaint of 
"sciatica." It is well to keep this fact in mind, as there may be no 
mention whatever of symptoms referred to the back, and attention is 
easily directed elsewhere. The first symptom varies greatly. In the 
majority it was pain, referred to the back, hips, or legs, while in others 926 
DISEASES OF THE LOCOMOTOR SYSTEM 
it was referred to the thorax or abdomen. Several complained of stiff- 
ness or weakness in the back. Cramps in the legs were the first manifes- 
tation in some patients. All of these may be on one or both sides of the 
body, but in the majority they are more marked on one side than on 
the other, especially when pain referred to the legs. The variability in 
the symptoms is understood if one keeps in mind the anatomical condition. 
Throughout the course pain is the most marked symptom. This may 
practically be situated anywhere in the trunk or extremities, but the much 
greater frequency with which the lower spine is involved explains the 
corresponding frequency in the pain being referred to the legs. It may 
be in the spine, in the course of certain nerves, or in both situations. In 
some cases the pain may come on in severe attacks, which make the 
patient cry out. One feature is the onset of pain in the night after the 
patient has been asleep. This is most frequent when the disease is 
in the lower spine. It is exceedingly common for the nervous condition 
of the patient to suffer, for as a result of the wearing pain and disturbed 
nights the general health is much affected and the patient becomes very 
neurotic and upset. As a result the nutrition is affected and the patient 
may lose a great deal of weight. The symptoms may vary a good deal 
at different times. Some patients are fairly comfortable as long as they 
are quiet, but any movement stirs up the pain. Others make a special 
complaint of pain during the night, which may be more or less continuous, 
or wakens them from sleep. This is probably due to the muscles being 
relaxed, so that sudden movement causes pain. 
Examination.-In discussing conditions which are found one must 
always remember the great variability, as there are all grades between the 
patient who has rigidity of the whole spine and the one who has involve- 
ment of only two or three vertebrae. The general appearance is some- 
times striking. The patient often looks worn, haggard, and sometimes 
emaciated. The attitude either on standing or walking may be char- 
acteristic. If there is general involvement with flexion of the spine the 
upper part of the body is bent forward, the head projecting, and the eyes 
fixed on the ground so that the patient has difficulty in looking up. 
In cases in which fixation has occurred with the spine perfectly straight, 
the so-called poker back, the patient walks unusually erect. In the cases 
with limited involvement of the lower spine the patient may limp, and 
when he stands hold the leg of the affected side with the knee bent. 
With this there may be distinct tilting of the pelvis. The attitude 
when the patient is sitting down or getting up is often suggestive. They 
do these actions slowly, perhaps resting the hands on the thighs, making 
every effort to avoid bending the spine. If they are asked to stoop and 
pick up an object from the floor the position is generally very character- 
istic. They often lower the body by bending the legs and reaching down 
with the hand, every effort being made to avoid bending the spine. 
On examination of the back much or little may be found. The cases 
of general involvement are so characteristic that they require little 
description. It may be found that the natural curves of the spine have 
disappeared, and especially that the lumbar curve is obliterated. In 
acute cases the condition of the muscles on either side of the spine is ARTHRITIS DEFORMANS 
927 
sometimes striking. They are markedly contracted and may stand ont 
prominently. This is most striking when the process is especially on one 
side, the difference in the state of the muscles being generally very 
pronounced. The important point is the observation of the movements 
of the spine, for some or all of these movements may cause pain, and 
limitation of motion may be very evident. In the early stages this is 
rarely equal in all directions; with general fixation it may be. Some 
patients on attempting to bend forward move somewhat to one side. In 
bending to the sides it may be found that the freest motion is in different 
parts of the spine on going to the right and left. Sometimes contraction 
of the muscles becomes much more evident with the movements. In 
some instances there may be prominence of the spines of one or two 
vertebrae. Occasionally there is distinct tenderness on palpation over 
particular spines. In cases in which the vertebrae of the neck are 
involved it may be possible to feel the bony thickening. If this has been 
especially on one side the head is rigidly fixed in a bent position. There 
may be involvement of the sacro-iliac joints, and this usually renders 
the diagnosis difficult from local involvement of the lower spine. 
Associated Features.-The frequent complaint of pain in parts other 
than the back has been noted. This is usually referred to the area of 
distribution of the nerves, although in some cases there is pain referred to 
the course of a nerve associated with tenderness on pressure over it. 
This may especially concern the sciatic nerve, and the ordinary features 
of sciatica are found. Muscular wasting is quite frequent. This 
sometimes involves the muscles of the back, but more often those of the 
leg; thus, there may be marked flattening of one gluteal region, and also 
wasting of the thigh muscles and these of the leg. As a rule, the reflexes, 
both the knee-jerks and the tendo Achillis reflex, are markedly increased 
but this is not invariable. Ankle clonus was obtained in a few instances. 
With the process in the spine largely on one side, muscular wasting and 
increase in the reflexes may be confined to the leg of the affected side. 
Disturbances of sensation are common, and there may be paresthesia or 
anesthesia. The sensory changes may vary from time to time. Irregular 
jerking movements of the muscles are sometimes seen. 
Features in the thorax are often present if the dorsal vertebrae are 
involved. Thus, in acute stages with involvement of the articulations 
between the ribs and vertebrae, pain on breathing may be so severe that 
the patient makes every effort to spare the thorax and the respiration 
may be entirely abdominal. It may be noted that the same picture is 
presented in the cases of general arthritis, with involvement of the joints 
of the spine. In all these forms as the arthritis subsides, and if there is 
no fixation, the thoracic movements may be entirely restored. In other 
cases, however, there may be fixation, which is permanent. If this be 
associated with bending of the spine the change in the shape of the 
thorax may be marked, and the costal margin may be near the iliac 
crests. 
X-rays.-The study of the x-ray plates is of great importance; per- 
haps the most striking point is the detection of deposits of new bone. 
These in the majority are extremely irregular, and may be largely on 928 
DISEASES OF THE LOCOMOTOR SYSTEM 
one side, or especially in the anterior ligaments. This irregularity 
is really the rule; the symptoms suggest, the x-ray plates confirm it, 
as does also the study of museum specimens. 
Diagnosis.-The cases of general involvement are so characteristic that 
a glance is usually enough to give the diagnosis, but with the local forms 
the story is different. To J. E. Goldthwait,1 of Boston, much of the 
credit of drawing attention to these conditions is due. 
The symptoms complained of most commonly are stiffness, interference 
with motion, pain, and muscular weakness. It may be that many of the 
indefinite pains complained of in the back, especially in the morning on 
waking, are due to this condition. "Lumbago" is at times due to this 
and many of the cases of so-called "sciatica" are caused by arthritis 
of the spine, and also instances of obscure pains in the legs and about the 
body. There is no intention of suggesting that every case of pain in the 
back or of sciatica is due to spondylitis, but in every such case it is most 
important to examine the back. Some cases otherwise regarded as 
neurasthenia with complaint of pain in the back will be found to have 
local arthritis of the spine. 
Of the methods of diagnosis first comes the examination of the spine. 
The patient should be completely stripped, or down to the hips at least. 
In the majority of cases little is to be made out on inspection. There 
may be some curvature, or a projecting spine, or more commonly a 
obliteration of the lumbar curve. Some wasting of the dorsal muscles, 
or in the gluteal region, or of one or both legs, may be found. In some 
the attitude is suggestive. They stand with one leg a little flexed and 
"favor" that side. Next comes the investigation of the mobility of the 
spine. Any limitation of motion is very readily recognized. This may 
be equally marked in all directions, or only in one, although usually at 
least two are combined. He may bend forward but a very short distance, 
and to one side much less than the other, or lateral movement to one side 
only may be much restricted. These movements may cause pain, either 
local or referred. An attempt to bend to the side may cause the same 
pain in the leg of which the patient complains. The attitude on 
attempting to pick up an object on the floor is often very characteristic. 
With this the sensation should be tested; areas of altered sensation are 
important. The reflexes are usually increased. 
The diagnosis may be fairly clear from the examination, but there are 
two important aids-namely, tuberculin and the x-ray picture. The 
use of tuberculin excludes the most common source of difficulty, although 
tuberculous disease of the spine rarely gives the same picture as spondy- 
litis. In some instances there is marked involvement of the hip-joints 
with the spondylitis, and this may have led to a suspicion of tuberculous 
hip-joint disease. The radiograph, if it shows anything, is usually 
characteristic. Should the diagnosis not be certain with any of these 
methods, one help remains-the therapeutic test. Put a light plaster 
jacket on the patient, which should extend from the axillae to the level 
of the trochanters, and if there is spondylitis there should be marked 
1 Boston Med. and Surg. Jour., 1899, vol. cxii, and 1902, vol. cxlvi. ARTHRITIS DEFORMANS 
929 
improvement in the symptoms in a few days. This is especially useful in 
the cases with sciatica. The possibility of sacro-iliac joint disease should 
be kept in mind in obscure cases. 
Villous Arthritis.-This is not a distinct entity, but the term is used to 
describe a condition which may occur from many causes: Trauma, 
either direct or indirect from flat foot, etc., or from changes in the joint 
itself, as detachment of a semilunar cartilage; loose bodies in the joint; 
tuberculosis; syphilis; various forms of infectious arthritis (e. g., gonor- 
rhoeal), and not rarely from arthritis deformans. When it occurs as one 
of the manifestations of arthritis deformans, it has to be regarded as 
only one part of the general picture. 
The degree of proliferation of the fringes varies from a delicate fringe 
of membrane to large, irregular masses which may occupy the greater 
part of the joint cavity. They are usually very vascular, and show both 
fibrous and fatty tissue. The knees, shoulder, ankle, and hip are most 
often involved, the knee being by far the most frequent. In cases in 
which the condition followed trauma, it is usually monarticular, but when 
with general disease it may be polyarticular. The symptoms vary with 
the degree of change. When the fringes are soft they may cause but 
few symptoms. In the average case there is discomfort in the joint 
or pain which is rendered worse by use. There is usually considerable 
general swelling, which suggests marked effusion, and may cause error 
unless a careful examination is made. The patella may seem to float and 
fluctuation may be simulated. Tenderness on pressure may be present. 
Until one is familiar with the condition, the diagnosis of effusion seems 
the most likely one; but there is a curious feeling on palpation, very 
suggestive of a villous arthritis. 
Association of Arthritis Deformans with Other Diseases.-The relationship 
between infective processes of various kinds and these joint conditions 
has been noted. An interesting association is with Raynaud's disease 
and scleroderma, or both. In this series there were eleven patients in 
whom scleroderma co-existed with the arthritis, and in five of these there 
was also Raynaud's disease. It is rather striking that in no instance 
did Raynaud's disease occur alone, but always with scleroderma. In 
some instances the scleroderma was present first, but in some instances 
it and the arthritis appeared about the same time and in others the 
arthritis antedated the scleroderma. Of the etiology of these diseases we 
have little accurate information, although it is strongly suggested that 
they are trophic disorders. It is quite possible that the infection which 
determined the arthritis may also influence the nervous system. In 
these cases there is also evidence of trophic disturbances in the glossy 
condition of the skin, and sometimes in the state of the nails. It was 
rather striking that the examples of these combinations occurred in 
women, and nearly all of them were patients of a marked neurotic char- 
acter, it being necessary to keep in mind, however, that the state of the 
nervous system is largely secondary to the disturbance of the arthritis. 
As a rule, the outlook does not seem to be good in this group. The 
scleroderma seems to have a marked tendency to progress. 
In the Cambridge report attention is drawn to a group of cases which 
vol. v-59 930 
DISEASES OF THE LOCOMOTOR SYSTEM 
show lesions suggesting both gout and arthritis deformans. These are 
puzzling and their exact nature is not decided. 
Diagnosis.-It may be thought that the question of diagnosis would 
be a difficult one in a disease about which there is so much confusion and 
which may really include various diseases. Yet actually when the prob- 
lem is faced the difficulties are not so great. They may be regarded as 
generally belonging to one of two classes: (a) in which the condition is 
acute and the deforming changes are not greatly in evidence, and (6) 
in which chronic changes have appeared. In the first group it may be 
difficult to form a definite opinion for some time; in the second we know 
that the number of diseases causing deformity of the joints is not so 
large that differentiation is often difficult. 
Certain points may be mentioned. That a thorough examination is 
important seems unnecessary to say, and yet the diagnosis of an arthritis 
in the larger joints may be cleared up by finding slight changes from a 
previous attack, perhaps in one finger-joint. An arthritis which recurs 
and which has left permanent damage in a previous attack is usually 
arthritis deformans, gout being excluded. Then it is important to learn 
that this disease may begin suddenly as an acute polyarthritis, which 
too often is regarded as excluding it. The character of the fever curve, 
the ra^id pulse, the special joint changes, are all aids. In the more chronic 
forms the diagnosis is rarely in doubt, but unfortunately at this time the 
benefit of early diagnosis is gone. 
Certain characteristics of the arthritis are important. Perhaps the 
most marked is that when a joint is attacked the condition rarely clears up 
except very slowly, and there is always a tendency to some change. 
If it be kept in mind that rheumatic fever does not leave any permanent 
change in a joint, one frequent source of error will be avoided. Location 
in certain joints is often suggestive. Thus, to have involvement of the 
joints of the cervical vertebrae, the temporomaxillary-joint or the thumb- 
joint, in association with arthritis elsewhere, is often suggestive of 
arthritis deformans. Involvement of one or two finger-joints is likewise 
suspicious. Permanent changes in the joints may be found early; for 
example, the capsule of the knee-joint may show definite thickening, 
or there may be distinct thickening about a finger-joint soon after the 
onset. The greatest aid is given by the z-ray examination, which should 
always be made in a doubtful case. 
In distinguishing this disease from others, it is convenient to consider, 
first, the more acute forms, remembering that what is to be a chronic 
arthritis may have acute features at onset, and, secondly, the more 
chronic forms. Among the acute diseases are the following: 
1. Rheumatic Fever.-It may be quite impossible at first to distinguish 
this. The usual mistake is to regard an acute form of arthritis deformans 
as rheumatic fever, rather than the reverse. Knowledge of how often 
arthritis deformans begins suddenly as a polyarthritis with acute features 
is an aid. There is rarely any shifting from joint to joint in arthritis 
deformans, and a joint once attacked stays affected. The character of 
the arthritis is different, as a rule. The joints are usually not so tender 
to the touch as in rheumatic fever, there is rarely the same degree of ARTHRITIS DEFORMANS 
931 
redness, and it can sometimes be made out that the swelling is more in 
the surrounding tissue than in the joint itself. Certain joints, especially 
those of the neck, the temporomaxillary joint, and those of the thumb, 
are rarely attacked in rheumatic fever. The involvement of the finger- 
joints is often very characteristic. The temperature in arthritis defor- 
mans is usually not as high as in rheumatic fever, the degree of arthritis 
being considered, while the pulse rate may be much higher relatively 
than the fever. The enlargement of the associated lymph glands is 
usually greater than in rheumatic fever. Rapid muscular atrophy, 
especially of the interossei muscles, is suggestive, as it is more likely 
to occur with arthritis deformans. The lack of response to treatment with 
the salicylates may excite suspicion. In a doubtful case the first clue is 
usually given by the persistence of changes in the joints. This should 
always suggest a reconsideration if the diagnosis of rheumatic fever has 
been made. In all cases of acute polyarthritis in which there is some 
question as to the nature, the diagnosis of rheumatic fever should be the 
last to be made and not the first, as is usually the case. 
2. Gonorrhoeal Arthritis.-This undoubtedly is the most difficult disease 
to distinguish. Keeping in mind the possibility and a thorough search 
for gonococci are the great safeguards. The clinical features are much 
the same as in arthritis deformans, but there is often more synovitis 
associated. There is rarely the involvement of the joints of the hands as 
in arthritis deformans. The brunt of the attack in gonorrhoeal arthritis 
often falls especially on one joint, although several joints are attacked. 
The complement fixation test may be of value. 
3. Gout.-This may appear as an acute polyarthritis and cause difficulty 
for a few days, but usually not for Jong, unless it be one of the cases of 
gout which has a prolonged course of acute features. The absence of 
marked changes in the joints and the results of treatment are suggestive. 
The finding of tophi and sometimes a study of the uric acid excretion aids 
its recognition. In some cases of chronic gout without any special 
distinguishing features the diagnosis may be difficult. The relative 
frequency of the diseases should be kept in mind; arthritis deformans is 
much the more common. 
4. Some of the Forms of the So-called "Infectious" Arthritis.-This 
group may pass over into arthritis deformans, and it is difficult to know 
where to draw the line. The frequent occurrence of arthritis with or after 
many acute infections has to be always remembered. It is especially 
important for prognosis to distinguish them, but this may be difficult until 
time gives the answer. The knowledge of a source of infection, the results 
of treating this, the progress of the arthritis, and time are our greatest 
aids. 
Certain forms of arthritis deformans require special mention. 
(a) The So-called Still's Disease.-This is not likely to give much 
difficulty, as the picture it presents is usually characteristic in the asso- 
ciation of a multiple arthritis, especially with peri-articular changes, 
marked general glandular enlargement, and an enlarged spleen. In 
some cases a general tuberculous arthritis may give difficulty, but the 
x-ray plates and the use of tuberculin should settle the question. 932 
DISEASES OF THE LOCOMOTOR SYSTEM 
(b) The "Monarticular" Form.-This term is only used for clinical 
convenience. On careful examination slight changes in other joints can 
usually be found. The greatest difficulty arises in the shoulder-joint 
where two conditions especially cause error. One is neuritis, arthritis 
often being regarded as this, and the other is subdeltoid bursitis, which is 
usually regarded as arthritis. The diagnosis of neuritis may be correct 
so far as it goes, but it is secondary to arthritis. Careful examination 
will usually show signs of arthritis. The features of subdeltoid bursitis 
should be looked for, and it will usually be found that the joint itself is 
free. In subdeltoid bursitis there is pain when any attempt is made to 
abduct the arm, or it may be elicited by pressure over the bursa. Occa- 
sionally the pain is referred along the bicipital groove or down the arm. 
There is marked restriction of motion, especially abduction, and internal 
rotation is usually markedly limited. Sometimes the swollen bursa may 
be felt. In long-continued cases there may be atrophy of the muscles 
of the shoulder girdle. Sometimes, however, the exact diagnosis may 
be difficult, and then the x-ray plate is useful. 
In the hip-joint there is usually less difficulty, although sometimes the 
condition is regarded as tuberculous. The tuberculin test is useful. 
If there be communicated inflammation to the sciatic nerve the diagnosis 
of sciatica may be made. Knowledge of the possibility of this error 
is a great aid in avoiding it. Disease of the sacro-iliac joint may cause 
error. Search for the signs of disease there will usually prevent this. 
(c) Atrophic Form.-It is often possible to recognize this form before 
making the x-ray examination, which makes the diagnosis certain. 
The joints show marked degeneration, partial dislocation occurs early, 
muscular atrophy is often rapid in its appearance and progress, and there 
may be pronounced grating in the affected joints. In interpreting the 
x-ray plates, it must be remembered that there may be a certain amount 
of bony atrophy secondary to any continued arthritis. 
(d) Hypertrophic Form.-While the examination of the joints may 
serve to distinguish this at a glance, as in the Heberden's nodes, yet in 
the larger joints the chief dependence must be placed on the x-ray plates. 
Ina joint-such as the knee-with marked muscular atrophy above and 
below, there is often the suggestion that there is enlargement of the bones; 
the x-ray plate frequently shows that this is not the case. Of special 
forms, the Heberden's nodes rarely give any difficulty, especially if 
several joints are involved. Injury, however, may have results which 
closely resemble them, but this rarely affects more than one or two joints. 
(e) Spondylitis.-This has been discussed on page 928. 
There are certain ailments which may cause confusion, these usually 
being regarded as arthritis deformans. Sprains, pain due to muscular 
strain, pain due to certain occupations, relaxation of ligaments, flat 
foot, neuritis, lumbago, and such like, can generally be excluded or 
recognized by careful examination. 
The x-ray examination is always of the greatest help not only in decid- 
ing as to whether or not arthritis is present and its nature, but also in 
giving information as to the degree of change in the joint. To estimate 
this last should always be our aim. To know the form we are dealing ARTHRITIS DEFORMANS 
933 
with is important both for prognosis and treatment. To know how much 
change there has been in the joint is essential for proper and intelligent 
handling, and should be as much a part of the diagnosis as the determina- 
tion of the disease itself. 
Prognosis.-In forecasting the outlook in a disease with so many 
varying features, it is evident that much has to be left to the judgment 
of the physician and the conditions in a particular patient. There is no 
general rule which we can apply. The type of disease makes a great 
difference as well as the general character of the patient. Speaking 
generally, the writer is inclined to be more hopeful about the outlook than 
the majority of those who have discussed the disease. There are certain 
exceptions which will be noted. Let it be said that there is no intention 
of minimizing in any way the gravity of chronic arthritis. To fight a 
winning battle with one of the most intractable of chronic maladies, 
not as regards life but for the comfort and usefulness of the patient, it is 
necessary to know the power of the enemy and to have a wholesome 
respect for the dangers. Perhaps nothing has given more personal 
encouragement than the results which have been obtained in dispensary 
practice. To get hold of patients early in the course, teach them what 
to do, have them work enthusiastically despite discouragements, and 
finally get through without much damage is cheering. To see a patient 
grow steadily worse despite all treatment dampens our enthusiasm. 
Yet it is important both for the physician and patient that the most 
cheerful view be taken, for both will work harder. The character of the 
patient must always be taken into account. Some will work hard, others 
expect everything to be done for them. The condition of the nervous 
system is often important. High-strung, nervous patients often do 
badly, and any such condition is usually aggravated by the arthritis. 
1. Peri-articular Form.-Here there are many factors to be considered, 
(a) Early Diagnosis: This is most essential, in order that proper treat- 
ment may be begun early and that no treatment will be carried out 
which will do harm. (6) Recognition of the Cause: If the local focus 
of infection can be found and properly treated before great arthritic 
damage is done, the outlook is much more encouraging than when the 
contrary is the case, (c) Age: Generally the older the patient the better 
the outlook, although this is not always true. In older patients there is 
sometimes a tendency to slowly progressive degenerative changes, 
(d) Station in Life: While in some cases the possession of wealth and the 
ability to have expensive treatment, such as massage, is of benefit, yet 
often the poor, who have the stimulus of necessity, seem to do better. 
Too often the rich are able to lie back and be waited on, to their ultimate 
sorrow, while the exertion demanded of the poor may be their greatest 
aid. (e) Character of the Patient: This means a great deal. Those who 
fight and "never say die," always working, as a rule, do well; but those 
who lie back and demand that the physician do everything, who are 
willing to take medicine but not to exercise, generally do badly. (J) 
Character of the Attack: The severity of the initial attack is no guide to 
the ultimate result. As a rule, in the patients with acute attacks the 
outlook is much better than in those with a slow progressive process which 934 
DISEASES OF THE LOCOMOTOR SYSTEM 
advances steadily from joint to joint. The attacks which begin in women 
about the menopause always demand a very guarded prognosis. The 
outlook in them is likely to be bad. The acute progressive cases can 
usually be recognized, and in them a very guarded prognosis should 
always be given, (g) General Nutrition: This is important, and in 
patients whose nutrition is much affected and who do not respond to 
good hygiene, diet, etc., the outlook is always serious. 
There are some local features in the joints which are of aid. (a) 
Pain: The degree of this is no indication of the severity of the arthritis, 
and yet its influence on the general health of the patient cannot be dis- 
regarded. Its presence may hinder use of the joints and favor ankylosis. 
It may be practically impossible for the patients who have severe pains 
in the feet to walk and obtain sufficient exercise. (6) Changes in the 
Joints: With joints in which the changes are entirely peri-articular and 
cartilage and bone show no change, the outlook is better than when signs 
of erosion and thinning of the cartilage are evident. Here, however, 
caution is needful, for the cartilage which shows no change today may 
do so in six months or a year, (c) Joints Involved: This is sometimes a 
help. The elbows if attacked often show marked permanent changes, 
the wrists and shoulders much less. Marked changes about the knuckles 
and finger-joints are often suggestive of much future deformity. The 
hip-joints have a tendency to permanent fixation. The knees easily 
become flexed, and arthritis of the joints of the feet may be very crippling 
without much actual change. The neck and jaws usually do well. 
In the "monarticular form" the outlook, as a rule, is good for fair, 
ultimate recovery, but generally one cannot prevent the rather long 
duration. Some of the patients ■with marked atrophy of the muscles 
may be two years before reaching a fair degree of recovery. In the 
so-called Still's disease, the majority of writers regard the condition as 
practically hopeless. My experience has been more encouraging, several 
patients have done extremely well. 
In this group certain features are of importance in estimating the 
outlook. If there be marked muscular atrophy which has come on 
rapidly, the outlook, as a rule, is more serious. If there be marked 
early tendency to contracture, it is always well to be cautious. Then the 
patients with marked general nervous disturbance, as a rule, do not do 
very well. With this seems to come an intolerance of pain, so that it is 
impossible to carry out the usual measures to prevent ankylosis and 
contracture. Naturally in a disease which is so often accompanied by 
general disturbance of nutrition the condition of digestion is important. 
In patients who are unable to take enough nourishment or whose digestion 
is easily upset, one should always give a guarded prognosis. 
2. Atrophic Form.-In this it may be said in a general way the outlook 
is always serious. We do not seem to have any means of hindering the 
advance of the disease, which, as a rule, progresses to more and more 
disintegration and destruction of the joints. No matter how slight the 
early changes may seem to be, or how good the patient's general con- 
dition, the prognosis should always be guarded. The general condition 
is often the best guide as to the outlook. ARTHRITIS DEFORMANS 
935 
3. Hypertrophic Form.-In this the outlook depends on many different 
features and no general statement can be made, (a) Heberden's Nodes: 
As regards any possibility of absorption of these there is only one answer 
to give: Once present, they are permanent. However, beyond the 
appearance and slight discomfort, they rarely give any severe trouble. 
If they are painful, one can usually assure the patient that this will not 
last for a great time. Much depends on the patient's avoidance of injury, 
and this influences the outlook to a considerable extent. (&) Conditions 
in the Larger Joints of Elderly Patients: Although the general tendency 
is toward slow progression, yet the outlook is in many instances better 
than one might have expected. It is never safe to be too positive about 
the outlook in either direction, and it is not unusual to find them doing 
much better or much worse than one expected. However, as a rule, 
the possibility of complete recovery is almost out of the question, (c) 
Smaller Joints: Although the condition may not advance very materially 
and a fairly good prognosis is often justified, it must always be remem- 
bered that bony outgrowths having once formed cannot be removed 
unless by surgical measures, which are sometimes indicated. The hope 
is that further progress will be prevented, and the patient's condition not 
become worse than it is. (d) Spondylitis: In this the outlook depends 
very largely on how much of the spine is involved. If the patients are 
seen in the acute stages a good prognosis can usually be given as regards 
the freedom from pain, but the amount of disability cannot be estimated. 
If the process is at all general there may be rigidity of the whole spine, 
with more or less deformity, although it is always encouraging that the 
extent of the fixation rarely corresponds with the extent of acute arthritis. 
In the cases of local involvement, as, for example, in the lower spine, the 
outlook is generally good if treatment has been begun early. The process 
is usually confined to a few vertebrae, and the patient recovers with very 
little disability. The possibility of subsequent attacks involving more 
vertebrae can never be excluded. 
In all forms the general condition is important and the possibilities 
of good surroundings and proper nutrition must always be considered. 
As has already been said, the outlook depends a great deal on the patience 
and perseverance of the patient and physician. The possible effect of 
some intercurrent acute infection is difficult to forecast. While, as 
a rule, any such tends to aggravate the arthritis, as, for example, an 
attack of influenza, yet in some patients the state of the joints is much 
better after an acute attack of another disease. 
In all acute cases two questions arise-the outlook in the immediate 
attack, and the ultimate result in the joints. As noted, the acute attacks 
are usually over sooner and often leave but little damage. The general 
features, such as fever, and the arthritic conditions are both important, 
but it is never wise to prophesy too much. In the slowly advancing 
form the prognosis should always be very guarded. As to the ultimate 
result in the joints, the z-ray examination is always of great value. If 
this shows peri-articular change only, the outlook is usually good; but if 
early erosion and destruction of the cartilage is found, one must be very 
cautious. In cases with marked changes, the question as to possible 936 
DISEASES OF THE LOCOMOTOR SYSTEM 
restoration of function is also greatly aided by the radiograph. With 
damaged articular surfaces, displacement, or marked atrophy the outlook 
is never good. If the joint surfaces are not damaged, even with a good 
deal of contraction, it may be possible to get back function in a wonderful 
way. The possibility of aid from surgical intervention should always 
be taken into account. 
Treatment.-It is evident that in a disease which has so many different 
manifestations and methods of progress no scheme of treatment can be 
laid down which will apply to all patients. Again, it is a disease at times 
characterized by acute attacks which may end as such or pass over into a 
chronic stage, or it may be chronic from the onset. Then, too, in its 
treatment both the general condition of the patient and the state of the 
joints have to be considered. It is well to emphasize the fact that 
treatment to be of avail must extend over a long period of time. Dogged 
perseverance must be shown both by the physician and by the patient. 
Too often both lose patience and part company, the patient to see if 
some other practitioner (too often some quack) cannot help him more, 
the physician perhaps with a feeling of relief that this particular knotty 
problem has gone to someone else. Perhaps more than of any other 
disease is it true of this one that the patients rarely go through a very 
long siege of the malady without seeing many physicians. 
The subject has its dark and its bright side. When one sees a patient 
in whom the disease progressively advances, crippling one joint after 
another, more or less steadily advancing in many joints despite all treat- 
ment, the feeling of hopelessness and helplessness cannot be escaped. 
At the opposite extreme, when a dispensary patient steadily improves, 
even under adverse circumstances, one feels that there is some hope for 
the majority. It may be said that this is no disease to be treated by 
medicines alone. The physician must be prepared to work hard and 
keep his patient working hard-often a much more difficult task. The 
whole mode of life must be supervised and constant attention paid to 
every detail. There must be much the same attention given to every 
point as in the case of a patient who has pulmonary tuberculosis. 
At the risk of tiresome repetition, the need of early diagnosis must be 
emphasized again. It seems absurd to insist on this so often did not 
the history of many patients prove the contrary. The great hope for 
many of them lies in early recognition and proper handling. Too often 
the error is made of regarding the disease as "rheumatism," after which 
almost everything done for the patient is more likely to harm than to 
help him. To keep the patient indoors, to cut off protein food, to give 
prolonged hot baths, to administer large doses of salicylates, are injurious 
as a rule. After the disease has run its course, the effort to restore 
function to greatly damaged joints must often be fruitless. Early 
recognition and persistent treatment are all important. 
The treatment of a given patient generally has to be considered from 
two points: (a) that of the general condition, and (6) that of the joints. 
On the whole, the former is the more important; the whole is greater 
than the part, and if the individual is doing badly, his joints are not 
likely to do well. Every disturbance of the general health should be ARTHRITIS DEFORMANS 
937 
regarded seriously, and these patients should take unusual precautions 
with any slight infection, gastro-intestinal trouble, etc. 
Source of Infection.-In line with the view that the disease is dependent 
on some infection, every effort should be made to discover a primary 
focus, if such exist. This demands very thorough investigation-there 
is no use in half-way measures. If definite change has occurred in the 
joints, the removal of the original source of infection will not result 
in complete recovery, but it will probably stop any advance of the process. 
The condition of the mouth must be first looked to, carious teeth and 
areas of infection should receive attention, and pyorrhoea alveolaris be 
treated. The tonsils should always be carefully examined. If they show 
obvious infection, there is no doubt as to the advisability of removal. If 
there be no direct evidence of involvement, the decision is more difficult. 
Frequently a tonsil which show's no surface evidence of disease, after 
removal shows deep pockets which contain streptococci. If there is 
any doubt as to the condition of the tonsils, it is better to give the patient 
the benefit and advise removal. This should be thorough; nothing short 
of complete removal by dissection is sufficient. Careful examination 
should always be made for any focus of infection in the sinuses. Otitis 
media should be treated if present. 
The respiratory tract does not offer many examples of the source of 
infection being there, yet a persistent bronchitis may have an influence 
and should be treated. In the same wray any gastro-intestinal disturb- 
ance should receive attention. The effort should be made to correct 
any digestive trouble. How much intestinal disturbances may be 
casual factors we do not know; but, at any rate, for the sake of the general 
nutrition, if for nothing else, we should try to prevent them. Dysentery 
especially should be thoroughly treated. Some patients in whom there 
is no definite intestinal disturbance are helped by free purgation, and 
others by daily washing out of the colon. Visceroptosis should be 
corrected by the usual measures. 
Next to the mouth and pharynx, perhaps the genito-urinary organs 
are the most important causes of infection. In males, it is especially 
necessary to investigate carefully the prostate gland and seminal vesicles. 
The contents should be massaged out and thoroughly examined. In 
women, the possibility of any infection of the urinary tract and the con- 
dition of the pelvic organs should be determined. Leucorrhoea should 
be treated. Any source of infection should receive attention. 
If local trouble is found anywhere, the measures which are demanded 
for its treatment should be carried out. The question sometimes arises 
as to whether any severe local treatment-such as removal of the tonsils- 
should be carried out while the patient is having acute arthritic symptoms 
and perhaps fever. If we are correct in attributing the condition to the 
local focus, there is every reason why this should be treated at once. An 
analogous case is seen in gonorrhoeal arthritis, in w'hich it is equally 
important to treat the urethritis as the condition in the joint. 
General Considerations.-This is a debilitating disease, and the impor- 
tance of this should be kept in mind. No measure should be adopted 
which can affect the general condition unfavorably or in any way reduce 
the patient's resistance. 938 
DISEASES OF THE LOCOMOTOR SYSTEM 
Hygiene.-The patients should have all the fresh air and sunlight 
possible. If able to walk they should be outdoors whenever possible. 
If unable to do this, perhaps owing to general involvement or acute 
remissions, they should be kept outdoors as much as can be managed, 
exactly as the patient with pulmonary tuberculosis. They are often 
benefited by sleeping outdoors. In cold weather it is necessary to see 
that they are properly clothed, and the extremities especially should be 
kept warm. It is important to protect them against sudden changes 
of temperature. In patients with marked involvement of the feet 
or hands comfort is often given by having them wear bed socks or 
woolen gloves at night. Some patients are helped by periods of rest 
each day. • 
Diet-In general, it may be said that as nourishing a diet as possible 
should be given. Individual peculiarities and special conditions have to 
be considered-that is common-sense. In some patients who have been 
heavy eaters it is well to diminish the amount. The diet should be 
chosen which agrees best with the patient. In addition to the regular 
meals, it is well for a time to give extra nourishment between meals 
and at bedtime. This may be in the form of milk, eggs, cocoa, etc. One 
very common error is to diminish or cut off as far as possible all the 
proteins from the diet, it being often suggested that the disease has 
something to do with uric acid. This almost always results in harm, 
and sometimes very serious harm. The writer would lay special stress 
on this, and emphasize the injury done by restricting the diet for no other 
reason than this. If anything is to be cut off from the diet it is usually 
better to reduce the carbohydrates, as many of the patients are subject 
to some digestive disturbances which are aggravated by too much of this 
form of food. A full protein diet is usually indicated, and, as a rule, 
the fats are well taken. Some are helped by the use of the fermented 
milk preparations. If the patients are accustomed to alcohol there is no 
reason why small amounts should not be given. As a rule, large amounts 
of water should be taken to favor elimination. There is no special 
virtue in any particular water, except that some patients can be induced 
to drink more when some name is attached. 
Bathing.-Apart from some of the measures of local hydrotherapy, such 
as douches, compresses, etc., bathing is not of value, as a rule, in the 
acute stages; in fact, it is often positively harmful. Prolonged hot baths 
are often debilitating and should not be given. A sojourn at various 
springs is usually of more value from the change than from any special 
virtue in the baths. 
Massage.-The majority of patients are helped by general massage; 
that of the joints will be discussed later. It aids the general condition 
and helps to lessen the amount of muscle atrophy. Care should always 
be taken to see that it is not given too vigorously, a mistake often made. 
Many of these patients are exhausted after any but the lightest rubbing, 
so that this, certainly at first, should be as light as possible, and increase 
in the vigor with which the massage is given must be very gradual. 
Two or three times a week are usually enough for general massage. The 
use of some form of vibratory movements is useful in some cases. ARTHRITIS DEFORMANS 
939 
Electricity.-This is sometimes used, and some favorable reports have 
been given, especially from the use of electric light baths, which probably 
owe their effect to their influence on nutrition. 
Radium may be useful in some cases but the results thus far are not 
very encouraging. 
Climate.--There is no doubt of the benefit of a change of climate, 
especially from the North to the South in winter. Whether this is due to 
anything more than the possibility of being more outdoors and leading a 
life in the open air is difficult to say. Certain patients are more apt 
to have recurrences in cold weather, and improve immediately with a 
change to a warmer climate. As a rule, a dry, equable climate suits the 
patients best. 
Vaccines.-If we are correct in supposing that an infectious process of 
some kind is the important etiological factor and the organism can be 
obtained, the use of an autogenous vaccine may be helpful. Stock 
vaccines and serums are not of much value. 
Medicinal.-That we possess any drug which essentially influences the 
disease there is no evidence. Naturally, in a debilitating disease, it is 
important to keep the blood in as good condition as possible, and for 
this iron and arsenic are perhaps best, the iron being given as Blaud's 
pills and the arsenic as arsenous acid combined with Blaud's pill or in 
the form of Fowler's solution. Many patients do very well on the syrup 
of the iodide of iron, which may be given in full doses over a consider- 
able time. Some few patients are benefited by potassium iodide given 
persistently. That any of the salicylate preparations are of value is 
doubtful. They may be used for pain, but their long-continued use is 
not advisable. Guaiacol carbonate in doses of gr. v (gm. 0.3) is strongly 
advised by many of the English writers. Some have obtained good 
results from iodine in the form of the tincture in doses of Thj to ij, 
especially in acute attacks. Thyroid, thymus and pituitary extracts 
given persistently seems to have helped some patients. 
Pain often requires general treatment. Few patients escape this 
entirely, but in many the various local measures are sufficient to give 
relief. Some patients have such severe general pains that drugs must 
be given. The salicylates, acetylsalicylic acid in doses of gr. vij (gm. 
0.5) or more, guaiacol carbonate, gr. v (gm. 0.3), antipyrine, gr. iij (gm. 
0.2), sometimes combined with codeia, gr. | (gm. 0.016), may be tried. 
Morphine should not be given, as there is danger of forming a habit. 
Marshall has drawn attention to the effect of ether, administered as a 
general anesthetic, on the arthritis especially when very acute. The 
ether is given in the usual way for about fifteen minutes. The pain is 
sometimes markedly diminished and the condition of the joints improves. 
The period of improvement varies from a few days to two weeks. This 
is worthy of a trial in severe attacks. 
Treatment of the Joints.-Here only general indications can be given 
and every patient must be carefully studied. It is always important to 
be sure that we are not doing any harm. The various forms demand 
different handling, and a degree of exercise which is of great help to a 
patient with marked peri-articular changes may be harmful to one 940 
DISEASES OF THE LOCOMOTOR SYSTEM 
with hypertrophic changes predominating. As a guide to treatment, 
the study of the a'-ray plates is of great aid. Thus, if there is marked 
destruction of the cartilage or hypertrophic bony changes it is evident 
in a general way that rest is important and exercise harmful, which if 
the changes are peri-articular we are usually safe in advising use. 
Peri-articular Form.-In general terms it may be said that exercise and 
use of the joints are beneficial. This is probably for several reasons, 
the nutrition of the structures is better, the risk of ankylosis and mus- 
cular atrophy is diminished, and the chance of contractures lessened. 
This is well seen in the temporomaxillary joint, which is relatively often 
affected, but which rarely shows ankylosis. Talking and chewing are 
two exercises which keep it from fixation. One caution is important- 
use should not be begun too soon. Rest should be the rule so long as 
the condition is acute. Yet even here there are exceptions, and some- 
times it is well to begin gentle massage and movements before the acute 
features have entirely disappeared. Care should be exercised in the use 
of fixation as a therapeutic measure. Atrophy, adhesions, and con- 
tractures may appear with great rapidity. 
Passive Movements.-These may often be begun early. They should 
be carried out gently at first and increased as the condition warrants. 
The production of pain is not necessarily a contra-indication, and 
especially when there is a tendency to contracture. Some patients 
have pain at first on the gentlest movement, but this usually lessens. 
The early use of passive motion is of great value in preventing adhesions. 
Massage.-This is often most helpful, but must be begun gradually 
and increased slowly. It is important to oversee this point, as many 
of those giving massage are likely to be too energetic. Gentle kneading 
and circular movements about the joint are the most useful. For the 
muscles above or below the joint more vigorous measures may be used. 
If there is severe pain it is often advisable to have the rubbing done 
after the use of measures, such as hyperemia, which ease the pain. The 
patients can often be taught to do a certain amount themselves. They 
can work on the hands and wrists very well after being shown how to 
perform a kneading motion with the thumb on one surface of the hand 
and the fingers on the other. One advantage of this is that they are 
doing good to both hands at the same time. They can sometimes work 
at the elbows, knees, or feet in the same way. 
Exercises.-These are useful. The patient should be started at very 
simple ones in the beginning. Thus, with the hand they may begin by 
making a fist and alternately spreading the fingers and bringing them 
together. A rubber ball with a small hole in it which they can squeeze 
in the hand is of help to many. Various movements at the wrists, elbows, 
and shoulders may be done. Lifting the shoulders, rotating the arm, 
raising it up, etc., may all be tried. In the same way the knees, ankles, 
and toes may be exercised. It is sometimes an advantage to have them 
keep a record of the exercises and the number of times each was done. 
Certain forms of mechanical apparatus may be used in the giving of 
exercises. In all of these it is important to go slowly at first and always 
stop short of fatigue. The patient's strength should be estimated, and ARTHRITIS DEFORMANS 
941 
the number of exercises and the number of times each is to be done care- 
fully ordered. Increase should be very gradual. One great advantage 
of the exercises done by the patient is that it gives them a feeling of 
doing something for themselves and of making a personal fight. 
Counterirritation.-This is often of great help, both for the pain and 
to aid the local nutrition. The Paquelin cautery lightly applied, 
blisters, mustard, or iodine may all be used. Repeated small blisters 
are usually better than one large one. Baking may be considered here. 
This may aid the blood supply, but it is chiefly of help in easing pain. 
Care should be taken not to give the treatment for too long a time; 
twenty to thirty minutes is usually enough. The temperature in the 
oven may be raised to 350° F., but there is usually no advantage in 
reaching the highest temperature which the patient can stand. It is 
well not to cover the joint with too much material. Care should be 
taken not to allow the patient to become chilled afterward, especially 
if there has been sweating. The frequency with which baking is done 
depends on the severity of the condition and how the patient reacts. 
Once a day is generally sufficient for severe cases; in others two or three 
times a week is sufficient. 
Hyperemia.-This is sometimes of great aid both in influencing the 
process in the joint and relieving pain. No rule can be made as to whether 
active or passive hyperemia will afford the greatest relief in a given case. 
In using the Bier method the bandage should be put on above the 
joint with sufficient pressure to interfere with the venous return, but 
not with the arterial flow. The necessary pressure can usually soon 
be determined for each patient. At first the bandage is left on for an 
hour at a time, but this can be increased rapidly until it is on for eight, 
twelve, or even twenty hours. Some patients prefer to have it put on 
at bedtime and left on during the night. It is not uncommon to have 
some transient oedema as a result, but this need not cause any uneasi- 
ness. Another method is to empty the limb of blood as much as possible 
by elevation and stroking toward the body, after which a bandage is 
applied, with pressure sufficient to stop the arterial flow. This may be 
left on from thirty seconds to one minute, and is then removed. This 
should not be done more frequently than once a day. The use of the 
suction methods for producing hyperemia is not of much value. 
Prevention of Deformity.-This is most important, and prevention is 
much easier to carry out than cure after deformity has occurred. Use 
of the joints and local exercise and massage are perhaps the greatest 
aids. Every effort should be made to prevent contractures, which 
are usually most apt to occur in the elbow and knee. By perseverance 
much can be done, but in some patients it seems almost impossible 
to prevent contractures, owing to the pain caused by any manipulation 
or the marked constant contraction of the muscles. In such it may be 
well to put the joint in a splint for part of the day or use light extension 
for some hours each day. The danger of putting joints up in plaster 
casts for several weeks should be recognized, and this is rarely advis- 
able. In some patients the injection of oil into the cavity of the knee- 
joint may be of value. Persistent exercises and passive motion are 
helpful for the shoulders. 942 
DISEASES OF THE LOCOMOTOR SYSTEM 
For the hands a certain amount can be done by the use of light splints, 
well padded, which may be worn at night. The tendency to flexion at 
the first interphalangeal joint and to hyperextension at the proximal 
joint can be considerably lessened by this. In some patients it seems that 
the tendency to ulnar deflection can be diminished by the use of a light 
splint, although one must not hope for too much from this. 
Treatment of Deformity When Present.-At first sight the hope of doing 
much for already established deformity often seems slight, and yet it is 
surprising what can be done by persistent effort. The employment of 
various forms of machines for giving the exercises is of great service in 
these cases. The patients should be encouraged to work themselves 
in every possible way. Sometimes putting them in a rocking-chair and 
letting them rock gently, for gradually increasing periods, is of help for 
contracture of the muscles of the leg. Massage may be steadily used. 
Extension is sometimes useful for flexion of the knees, and gentle forcible 
straightening of the fingers, with the use of splints, often helps deformity 
of the hands. In some cases surgical measures are of value. In deciding 
as to these it is important to study the z-ray plates and determine how 
much damage has been done to the articular surfaces. Contracted 
tendons may be divided and the limb straightened. If this is done the 
joint should not be left in a plaster cast for some time, but passive 
movements should be begun early. In the knee-joint the injection 
of oil into the cavity is sometimes of assistance in aiding an increase 
of movement. Excision of a joint may be advisable, as when one knee 
is markedly contracted and the other in fair condition. It is well to try 
and have the patients go about as much as possible-at first with assist- 
ance and crutches, then with the crutches themselves, and later with 
one crutch and a cane. 
Villous Arthritis.-This may demand special treatment by bandages or 
apparatus, but if these do not aid it may be well to advise surgical 
measures. As a rule, removal of the hypertrophied fringes gives great 
relief and improved function of the joint. 
Atrophic Form-So far as regards treatment this is the most unsatis- 
factory form, as it is too often progressive despite all treatment. Every 
effort should be made to keep up the general health by diet, hygiene, 
etc. With the destruction of the cartilages and marked thinning of the 
bones, use should be somewhat restricted for a time, but some of them 
seem to be better with moderate use. Every patient must be carefully 
studied and while it is difficult to lay down any definite rules, generally 
if there is any doubt it is better to incline to rest. Hyperemia, very 
gentle massage, and baking help the patient's comfort, and should be 
tried. Very often a change to a dry, equable climate gives good results. 
Hypertrophic Form.-In this the matter of rest is important during the 
acute stages, when, as a rule, exercise is harmful and rest is useful. The 
affected joint is usually helped by some protection. Sometimes one has 
to take a middle course, as in the elderly patients with hypertrophic 
changes especially in the hip-joint. If they walk too much, there is 
danger of stirring up the process and making it more acute; if they keep 
too much at rest, there may be danger of fixation. Going up and down ARTHRITIS DEFORMANS 
943 
stairs is often especially harmful to them. With overgrowth of bone it 
is evident that harm may be done by active or passive motion. Rest may 
often be secured by mechanical support, which may be useful even when 
it does not secure complete fixation, although the latter is usually 
advisable in very acute conditions. 
(a) Heberden's Nodes.-These sometimes require treatment, although 
nothing can be done to prevent their appearance. Increase in size is 
often due to trauma or irritation by use, rubbing, etc., so that the patients 
should be especially warned against injury, and if acute features are 
present, such as pain and redness, they should be carefully guarded 
against any trauma and kept as undisturbed as possible. If there be 
pain and tenderness, the most efficient treatment is usually hydrotherapy 
in the form of wet compresses covered with some form of protective, 
put on at night and left until morning. The patients should be cau- 
tioned not to rub the joints, and to limit their movements. 
(b) Spondylitis with Hypertrophic Changes.-Rest is usually the 
greatest aid in treatment. To secure this, a plaster jacket or some form 
of apparatus should be worn. Not infrequently this causes increased 
pain for a few days, and may have to be changed, but relief usually 
comes in a short time. The jacket should come up to the shoulders 
and well down over the hips. Some patients are relieved by a form of 
corset, carefully adjusted, or by a belt made of webbing. These are 
generally advisable after the acute stage is over. Caution should always 
be given as regards the danger of trauma or strain. Lifting of weights 
or severe exertion, especially in strained positions, in fact anything which 
may throw a sudden strain on the spine and its muscles, should be care- 
fully avoided. In the patients who suffer especially when in bed, care 
in the kind of mattress, adjustment of small pillows, etc., may be of use. 
In the patients who have a general process which is recognized early, 
an attempt may be made to have the spine in the most favorable posi- 
tion before fixation occurs. 
In the more generalized forms it is well to save the joints during the 
acute stages. This is not necessarily always complete fixation, but 
certainly excessive or even normal use should be avoided. After the 
acute features are over, such a joint as the knee is often helped by some 
support, such as an elastic covering. If bony exostoses are causing 
mechanical difficulty, surgical measures may give some relief; bony 
spurs, or the osteophytes about the joint, may be removed. 
In all forms attention should be paid to associated conditions, such 
as flat foot, knock-knee, etc. The use of weights may relieve deformities 
greatly. The orthopedic surgeons have done much to aid these patients. 
In conclusion, emphasis should be laid on the need of perseverance 
and patience in treating patients with these chronic joint conditions. 
Early recognition, persistent and intelligent fighting, and hard work often 
accomplish wonders. 
FIBROSITIS. 
Under the term myalgia or the unsuitable one of "muscular rheuma- 
tism" are grouped a variety of conditions but little understood and 944 
DISEASES OF THE LOCOMOTOR SYSTEM 
which have perhaps in common only the symptom of pain. These are 
comparatively frequent in occurrence, vary greatly in severity and 
acuteness, and for the present are often difficult of explanation. The 
acute forms we see especially as torticollis or lumbago. The more chronic 
forms are associated with more or less pain and at times with varying 
degrees of disability and stiffness. The essential nature of the con- 
dition is in doubt. By many it is regarded as associated with rheu- 
matic fever, or being a manifestation of the rheumatic state. Others 
regard it more in the nature of a neuralgia, some as connected with a 
diathesis or gout. The changes are apparently very largely in the 
white fibrous tissue, consequently the tendons, muscle and nerve sheaths, 
periosteum, the various fascia, and the fibrous portions of the liga- 
ments are involved. Consequently, for many of this group the designa- 
tion fibrositis is suitable. 
The etiology probably differs in the acute and chronic forms. The 
acute form occurs more often in men and in adult age. It may follow 
exposure to cold, but here, as in other forms, trauma often plays a part. 
Thus, in the production of torticollis, if a patient is lying with the head 
in a bent position the condition is apparently more likely to develop. 
It is, however, especially in the production of lumbago that trauma 
seems to be important. Some strain, as in lifting or working in a bent 
position, is a common contributing factor. This, with exposure, is a 
common cause in laboring men. The more chronic forms are commoner 
later on in life and occupations involving exposure are apparently impor- 
tant factors. In both the acute and chronic forms one attack seems to 
predispose to another. As a result of the thickening of the muscle sheaths 
a considerable degree of disability may be produced, following which 
there may be muscular atrophy and a certain amount of contraction. 
The essential pathological lesions are apparently in the white fibrous 
tissue, and the condition is probably of an inflammatory nature. There 
is a serous exudation in the affected parts, which later may be followed 
by proliferation of the fibrous tissue. This may be absorbed entirely, 
but if long continued will be more or less permanent and may extend 
over a considerable area. As a result of these, various nodules have 
been described, the histological character of which has been described 
by Stockman. Thus, in one patient a small nodular swelling in the 
buttock was excised, and found to consist of a portion of the perimys- 
ium of the gluteus medius muscle. Sections showed it to consist of the 
fibrous sheath of the muscle hypertrophied and oedematous. In place 
of the fibre, tissue penetrated between the muscle fibres, some of which 
were degenerated. The small vessels showed periarteritis and endar- 
teritis. There was no evidence of any very acute inflammatory reaction 
and no organisms were found in the tissues. 
It is probable that a number of factors enter into the production of 
this condition. The changes described by Stockman suggest either 
chronic low-grade infection or the influence of toxins. Many cases are 
probably due to absorption from a focus of infection. 
As to the symptoms, in the acute form the main features are pain and 
disability often coming on suddenly. The pain may be sharp and fairly ARTHRITIS DEFORMANS 
945 
constant or dull, and becoming severe on movement, or when the 
muscles are placed in certain positions. Special features depend on the 
locality affected and certain forms are fairly well recognized. 
1. Torticollis affects the muscles of the neck laterally or posteriorly. 
The pain is generally very severe, and the patient holds the head to one 
side. The condition is usually unilateral. It may persist for a few 
hours only or for several days. 
2. Lumbago affects the muscles of the back. The onset is usually 
sudden, and often after some muscular effort which involved bending 
or lifting. The pain may only be present on movement, but is some- 
times so severe as to incapacitate the patient and prevent any move- 
ment. The duration is very variable, but is usually a few days. Some 
individuals have regularly recurring attacks. As with torticollis, a 
striking feature is the frequent prompt response-although the effect 
may be only temporary-to local counter-irritation. 
3. Pleurodynia involves the intercostal muscles and sometimes the 
pectoralis and serratus muscles. The pain is severe and greatly aggra- 
vated by any movements. The respiratory movements are restricted 
on the affected side. Pressure may cause severe pain. 
4. Various other forms are described as scapulodynia, dorsodynia, etc. 
Their general features are much the same. A certain set of muscles may 
apparently show a tendency to repeated attacks. 
Constitutional symptoms are not common. Fever is rare and the 
pulse rate is not increased. 
The chronic forms are distinguished especially by aching, soreness, or 
pain, and varying disability which consists chiefly in stiffness and 
inability to perform certain movements. These may be more or less 
constant, or come and go for various periods. In severe cases they are 
present constantly, and exacerbations occur, as, for instance, after 
exposure. Sudden muscular movements are especially apt to cause 
pain. In addition there may be severe pains which are apparently 
neuralgic in character. 
The muscles may be tender on pressure, and in some cases definite 
areas of induration may be felt. Stockman lays considerable stress on 
these, and describes some as being soft and ill-defined, while others are 
firmer. They are usually tender on pressure. He describes the com- 
monest seats as the lumbar aponeurosis, the calves, fascia lata, trapezia, 
insertion of the deltoid, the biceps, the intercostal and pectoral muscles, 
the glutei and soles of the feet. With time, considerable disability and 
marked loss of function may result. 
The recognition of this condition, as a rule, does not offer much diffi- 
culty, with the exception of lumbago. Here the possibility of arthritis 
of the spine, sacro-iliac-joint diseases, tuberculous disease of the spine, 
or secondary malignant growth in the spine must be kept in mind. 
Careful examination with the possibility of these kept in view and the 
need of excluding them before a diagnosis of lumbago is made, are the 
most important points. 
^Concerning treatment, as regards the general management, it is usually 
well to open the bowels thoroughly and to see that the patients take large 
vol. v-60 946 
DISEASES OE THE LOCOMOTOR SYSTEM 
amounts of water. Tor the pain some of the salicylate preparations 
are generally most efficacious. Sodium salicylate, in doses of gr. x to xv 
(gm. 0.6 to 1), or acetylsalicylic acid, in gr. x (gm. 0.6) doses, may be 
given. For some patients small doses of colchicum are most useful. 
Guaiacum and potassium iodide give considerable relief in some instances. 
As to local measures, the most important is rest to the affected muscles. 
This may be secured by mechanical means, especially strapping. It 
is probable that the often used plaster, especially to the back, acts in 
this way as much as any other. Local counterirritation and the appli- 
cation of heat are particularly helpful. The Paquelin cautery may be 
applied or blisters used. Acupuncture is very helpful in lumbago; either 
sterilized needles or hatpins are inserted in the lumbar muscles, after 
cleaning the skin, and left there for five minutes. In some cases the use 
of the constant current is very helpful. In those subject to repeated 
attacks, every care should be taken to avoid the exciting cause, which 
in many patients is exposure to cold. Some of those who arc susceptible 
find that a Turkish bath taken at the very onset frequently has a marked 
effect in limiting the progress. CHAPTER XXVI. 
OSTEOMALACIA. 
GEORGE DOCK, M.D. 
Synonyms.-Mollifies ossium; fragilitas ossium; ostitis malacissans; 
malacosteon; halisteresis ossium; Osteomalacie (German); ramolisse- 
ment des os, osteomalacie (French); rammolimento delle osse (Italian). 
Definition.-A chronic disease characterized by pain and muscular 
weakness, and by decalcification and absorption of bones already formed, 
with bending or fracture, and consequent deformity. 
Osteomalacia has been considered rare everywhere, but with the more 
accurate recognition of its symptoms this has been changed, although it 
is too early to speak positively of the existence or absence of the disease, 
which should be more carefully looked for in all parts of the world. It 
will probably be found that mild cases occur in many places, but the 
existence of certain severe foci will probably not be less striking than it 
now appears. 
Etiology.-The explanation of the endemic occurrence of osteomalacia 
was naturally sought in the soil and water, but has been abandoned. 
Unfavorable hygienic surroundings in the dwelling house or work place, 
dampness, insufficient clothing, trauma, psychic shocks, and poor food, as 
sour rye, have all been looked upon as causes, but they obviously can be 
concerned only in an indirect way. The majority of patients belong to the 
poorer classes, but there are exceptions. 
Sex.-Women show a remarkable predisposition to osteomalacia. 
Among older statistics a proportion of ten to one was general. Latzko 
more recently (1897) found 5 men among 120 cases. The difference 
cannot be explained by the influence of pregnancy, as women who have 
not borne children are also more disposed to the disease than men. 
A relation between osteomalacia and pregnancy, the puerperal state 
and lactation can be seen not only in the human species, but also among 
lower animals (cattle, sheep). Multipara and especially those with 
rapidly following pregnancies are more disposed than others. The 
tendency to dental caries, to slow callus formation after fracture, and the 
craving for earthy substances, so often noted in pregnancy, may be looked 
upon as having some relation to the greater incidence of osteomalacia. 
The belief that osteomalacia occurs chiefly in women of unusual fertility 
is based upon a statistical error. Heredity plays no part; a family 
tendency has rarely been observed. The true relation of osteomalacia to 
rickets is not known. They occur together in certain localities, but 
considering the frequency of rickets this is not remarkable. 
Age.-The majority of cases begin between the twentieth and thirtieth 
years. Cases before twenty are rare, but Drake reports the case of a 
947 948 
DISEASES OF THE LOCOMOTOR SYSTEM 
Brahmin, married at eleven, pregnant at fourteen, and with severe 
osteomalacia at eighteen. The observations of von Recklinghausen, 
Rehn, and Juergens make it probable that osteomalacia can occur in 
childhood, distinct from rachitis, or even in utero. It may be combined 
with rickets. Ziegler denied the non-rachitic nature of the changes in 
one of von Recklinghausen's cases. Juvenile and virile rickets sometimes 
follow trauma and infectious diseases. Langendorff and Mommsen1 
report an interesting case. Senile osteomalacia is rare. Pierart, out of 
247 cases, found only 22 above the age of forty-five. The symptoms may 
be misleading and diagnosis impossible, as in virile osteomalacia. In 
the case of Davis,2 a man, aged thirty-three years, had complained of 
weakness and pain in the upper extremities for a year, and fractured the 
left arm by a fall. There was no union. Exploration led to a diagnosis 
of sarcoma by examination of tissue from the seat of fracture. Careful 
differential diagnosis seemed to confirm this,, and the arm was removed 
at the shoulder. Skiagrams had been made, but were unsatisfactory. 
The later examination showed the process was osteomalacia. 
Pathology.-There is no unanimity regarding the pathology of osteo- 
malacia, and many facts point to a variety of causes and modes. 
An important suggestion grows out of the observation of Hanau on 
"physiological osteomalacia," a porosity of bone not infrequently found, 
especially in the pelvic bones, in pregnant women. From this it would 
seem an easy step to the pathological osteomalacia of pregnancy and 
lactation, and especially when we consider the need on the part of the 
growing infant for lime and phosphorus and its supply in the milk. The 
hyperemia of the bones of the pelvis during pregnancy, the changes that 
occur in the bone marrow, the remissions of the disease during menstrua- 
tion and relapses in new pregnancies, all go far to indicate a close relation 
of osteomalacia to the genital functions of the female. Fehling's brilliant 
hypothesis, that the disease is due to a morbid activity of the ovaries, 
leading to passive hyperemia and absorption of lime, has in so many 
cases been confirmed by the effects of oophorectomy, that it must be 
considered as partly, at least, applicable. On the other hand, it is nega- 
tived by many cases of failure to recover after operation, by relapses, and 
by improvement following Caesarean section, or even anesthesia. Latzko 
saw chloroform anesthesia followed by temporary improvement in ten 
cases. On the other hand, improvement has followed in cases of oopho- 
rectomy under ether and under local anesthesia. Cases of infantile and 
senile osteomalacia, as well as cases in men, are not to be explained on the 
ovarian theory, which, of course, still leaves to be explained the remark- 
able geographical conditions. 
The nervous-metabolic theory of Pommer, though somewhat strength- 
ened by more recent observations on the trophic disturbances of bones, 
and the theory of Comby, of nutritive disturbances of bone by fermenta- 
tion products from a dilated stomach, cannot be generally applied. 
E. Hoennicke3 has advanced a thyroid theory, based on many facts 
relating to the thyroid gland and its functions. He saw a case of osteo- 
1 Virchows Archiv, 1877, Band Ixix. 2 Annals of Surgery, 1904, xi, 225. 
3 Ueber das Wesen der Osteomalacic, Halle, 1905. OSTEOMALACIA 
949 
malacia in a man, aged forty-seven years, who also had exophthalmic 
goitre. Similar cases have been reported by others. Hoennicke shows a 
close relation geographically between osteomalacia and goitre; he found 
an association of goitre in a considerable number of cases of osteomalacia, 
and he points out the well-known trophic and metabolic relations of the 
thyroid to the bones, and the mutual relations of the thyroid and ovaries. 
Erdheim1 calls attention to the relation between lime metabolism 
and the parathyroid bodies and its possible bearing on osteomalacia. On 
examining the parathyroids from eight cases of puerperal osteomalacia 
he found evidences of hyperplasia, followed by atrophy and small-cell 
infiltration. The occurrence of tetany in osteomalacia adds interest to 
the observation, and, as Erdheim points out, suggests that the para- 
thyroid hyperplasia in osteomalacia may be due to an increased demand 
upon the glandular function on account of toxic substances produced in 
the ovaries or some other organ. 
Cristofoletti suggests that the morbid ovarian function causes adrenal 
hypofunction. Falta suggests there may be absence of the physiological 
hyperplasia of the anterior lobe of the pituitary body. In osteomalacia 
adrenalin does not produce glycosuria as readily, or act upon the heart 
and circulation as in health. 
Infection has been asserted to be the cause by many authors, and 
seems highly probable, but positive evidence is not yet available. The 
occurrence in the previous histories of severe infectious diseases, and the 
frequent relation of such infections to the bone marrow, are suggestive. 
Laufer,2 in a valuable study of the subject, suggests the following as a 
working theory: Osteomalacia can be looked upon as a degenerative 
disturbance of nutrition in the bones, in the general sense that all degen- 
erative nutritional disturbances are characterized frequently by diminu- 
tion or disappearance of tissue and by diminution of function. Etiologic- 
ally, it may be supposed that various obscure factors-improper or 
insufficient food, trauma, repeated pregnancies, etc.-cause retrogressive 
changes in the bones. The changes in the bones, decalcification and 
new formation, are very characteristic of bone physiology, as seen after 
fractures, and are at first reparable, but continued action of the noxa or 
other unfavorable conditions permits the characteristic changes of osteo- 
malacia, that may be looked upon as a dystrophy peculiar to the osseous 
system. The possibility of recovery under various conditions leads us 
to suppose the dystrophy is independent of irreparable lesions of bones. 
The bones in osteomalacia, as the name signifies, are soft. At autopsy 
they can be squeezed or twisted, sometimes feel like wet paper, and cut 
without resistance. They look and feel greasy. The difference between 
the brittle and yielding ("waxy") varieties is unimportant. On section 
the bone tissue proper is much reduced, both in the compact and spongy 
part. The bones are often altered in shape, generally or in part. There 
are often cystic cavities of various sizes in the bones, containing clear 
or yellow or red serous fluid, or dark masses of more or less altered blood. 
1 Ueber E pithelkdr perbefunde bei Osteomalacic, Sitzungsbericht der kais. Akad. der 
Wissens. in Wien, Math.-naturw. Klasse, 1907, Band cxvi, Abt. III. 
2 Centralblatt fur die Grenzgebiete der Med. und Chir., 1900. 950 
DISEASES OF THE LOCOMOTOR SYSTEM 
The marrow is yellow and fatty for the most part, sometimes hyperemic 
or lymphoid in appearance. In some places only the periosteum preserves 
the original shape of the bone. The periosteum is often thick, hyperemic, 
and in stripping shows the surface of the bone rough, the Haversian 
canals sometimes wide and filled with serous fluid. In consequence of the 
loss of bone tissue the specific gravity is much reduced, from 1.877 to as 
low as 0.721. The bones permit the passage of Rontgen rays with unusual 
ease, depending on their decreased density. The process of absorption 
begins in the marrow cavity and extends outward. 
Microscopically, besides the increased sponginess or actual loss of bone 
tissue, there is often a border of osteoid tissue along the bone. From the 
fact that this stains red with carmine it has been called the "carmine 
border." This is recognized as decalcified bone, as von Recklinghausen 
set forth. The margin of normal bone is not even or systematic, but very 
often forms a zigzag or irregular line. The decalcified parts at first contain 
canaliculi and lamellae, and show at the margins between the calcified and 
non-calcified parts characteristic lattice-like figures, due to the presence 
of air in the canaliculi and the spaces occupied by the bone corpuscles and 
their processes. Hanau's view, that the lattice figures may correspond 
to defective calcification has not been verified by others. The decalcified 
parts become softened, sometimes fibrous, like asbestos, sometimes 
absorbed, leaving spaces filled with marrow, or with mucoid or granular 
material. Newly formed bone tissue is deposited in various parts, but 
in general in small amounts. The marrow is fatty or lymphoid, and with 
giant cells and osteoblasts in varying proportions. Hemorrhages and 
pigmentation from old hemorrhages cause a great variety of color and of 
microscopic structure. In some areas the marrow is gelatinous or even 
watery. The softening, and especially the irregular and large cystic 
areas of decalcification, explain the more striking deformities. 
The bones most affected are those of the pelvis. The sacrum is pushed 
forward and downward, the posterior parts of the iliac bones with it, 
while the acetabula are pushed up and inward by the heads of the femurs, 
the symphysis being squeezed forward like a beak. Other deformities 
in the shape and position of the pelvis depend upon the position in which 
the patient remains. The most extreme deformity gives the pelvic 
cavity the shape of a clover leaf or a Y. In patients who remain long on 
their backs the pelvis is flattened from before backward. The narrowing 
does not always act as an obstruction to delivery, as the bones are some- 
times elastic (" rubber-pelvis"). Hugenberger thinks that such cases occur 
in 30 per cent, of osteomalacia. Casati, out of 42 cases, was obliged to 
do Caesarean section only twice, and Fehling advised delay in operating 
for similar reasons. 
Vertebra.-The normal curves are exaggerated, especially in the 
cervical part, so that the chin may rest upon the sternum. Lateral 
curvatures are frequent, but vary with the habitual position of the 
patient. Pregnancy often causes the most pronounced deformities. 
The whole vertebral column is shortened, especially in the lumbar 
portion. The body sometimes shows a constriction and duplication at 
the waist, as if the parts had been telescoped. OSTEOMALACIA 
951 
The ribs are often broken from muscular action, in breathing, or from 
pressure. They may in part overlie each other. The sternum is often 
curved or bent. The thorax in general is usually compressed in various 
directions, causing in time displacements of the heart and lungs, with 
dyspnoea, asthmatic attacks, and palpitation. 
The long bones of the extremities are affected late in most cases, but 
may be early, causing spontaneous fractures, with sometimes large but 
usually imperfect callus, or leading to coxa vara, genu valgum, or flat 
foot. The cranium is rarely affected. 
Litzmann has made the following table of the relative frequency of the 
affection of various bones: 
Puerperal. 
Non-puerperal. 
Pelvis 
82 
40 
V ertebrse 
46 
40 
Thorax 
26 
37 
Lower extremities .... 
15 
36 
Upper extremities .... 
10 
30 
Head 
7 
24 
The joints are not affected. The muscles are atrophied and degener- 
ated, sometimes to a remarkable degree. The nerves of the extremities 
were also found degenerated by Schlesinger. 
The ovaries show no constant change. Fehling, who first attracted 
attention to the ovaries, described hyperemia, but Winckel and others 
attributed that to accompanying malpositions. Fehling described hyaline 
degeneration of stroma, follicles, and bloodvessels, but without proving 
any of the changes characteristic of or peculiar to osteomalacia. 
Symptoms.-The beginning of osteomalacia is usually impossible to 
recognize. Sometimes the increase of symptoms during pregnancy or the 
puerperal period permits a relatively easy diagnosis, but in most cases the 
disease is far advanced before any suspicion of its existence is entertained. 
The rarity of the disease has much to do with the present condition of 
diagnosis, and a knowledge of the early symptoms and a keener search 
would result in the more frequent discovery of mild cases. 
The earliest symptom is pain. This is usually called rheumatic, but 
varies in character from dull to neuralgic. In pregnant women it is often 
felt in the pelvis, especially the sacral region; in others in the thorax, back, 
or one or more extremities. Movement, but also remaining quiet for a 
long time, is likely to increase it. The pain is often spontaneous, some- 
times nocturnal, but can usually be provoked by pressure on certain 
bones, especially the pelvis and vertebrae. Tenderness over nerve 
trunks is sometimes present. Girdle pain has been noted. With the 
pain there is usually a feeling of weakness and often a subjective and 
objective stiffness, with contracture or spasm of certain muscles, as the 
iliopsoas (Renz and Koppen) or the adductors of the thighs (Latzko). 
From various causes the gait becomes waddling and uncertain; the body 
sometimes leans forward. Sometimes the gait is spastic, and then the 
patellar reflexes are usually increased, and there may be intention tremor. 
If at the same time there is paresthesia, an erroneous diagnosis can 
easily be made. 952 
DISEASES OF THE LOCOMOTOR SYSTEM 
If the patient is examined at this time, naked, as is necessary in order to 
avoid error in all suspected diseases of the motor apparatus, it may be 
found that besides confirming the statement of the patient or friends that 
she is shorter, some distinct deformity can be detected. There may be 
lordosis, or other curvature of the vertebrae, or change in the shape of the 
thorax, or a furrow in the lumbar region, or a change in the position of the 
hips and the shape of the symphysis pubis. The linea innominata may be 
straightened, the diameters of the pelvis altered, but with the conjugate 
very little or not at all lessened. Examination of the muscles often 
reveals tremor, fibrillary or gross, or spasm or contracture. Paresthesia 
in some cases, especially in the legs, and with other nervous symptoms 
may be due to alterations in nerve trunks from the deformity of the spine 
and pelvis. 
The digestive functions in the early stages are usually not affected. 
Menstruation and conception are often normal. The difficulties of 
confinement need not be described here, but it may be pointed out that 
the fear of conception is not now so great as it has been in the case of 
osteomalacia patients. 
The blood shows no characteristic changes. It becomes chloranemic, 
with reduction of both coloring matter and number of red cells. The 
eosinophile cells are sometimes slightly increased, sometimes diminished 
or absent. Lymphocytes vary; sometimes the large forms are increased. 
Myelocytes sometimes occur. An increase of the resistance of the red 
cells has been asserted. The alkalinity of the blood is not always dimin- 
ished ; it may even be increased. 
The urine often contains excessive amounts of calcium and phosphates, 
but these vary without distinct relation to the clinical features. Lactic 
acid is rarely present, and not characteristic. Bence-Jones's albumose was 
first found in a case of (probable) myeloma, erroneously called osteo- 
malacia. Since then it has often been found in cases of myeloma. In 
Dock's case it was found, without other explanation than the osteomalacia. 
The general and proteid metabolism in osteomalacia is not altered 
except as the result of associated conditions. The bones become poorer 
in calcium and other mineral constituents, especially calcium phosphate, 
and richer in organic matter, especially fat. The earlier statements 
regarding lactic acid in the bones are now known to be erroneous. The 
lime leaves the body through the kidneys and intestines. Phosphate 
calculi are important factors in the urinary excretion. Excretion and 
retention of calcium and phosphates vary, however, even without treat- 
ment, and no conclusion regarding the process in the bones can be drawn 
from the condition of the urine. 
In the further course of the disease, deformities of all the affected bones 
become most pronounced, and the body assumes the most bizarre atti- 
tudes. The skin becomes sallow and flabby. The muscles waste, 
fractures and callus may add to the deformity. (Edema, dyspnoea, 
palpitation of the heart, fever, and sweats may be present in various 
degrees and combinations. Bronchitis and bedsores indicate the extreme 
cachexia. Dysuria is a frequent symptom. Renal calculi may be passed 
in large numbers and cause intense agony. OSTEOMALACIA 
953 
There are many varieties of osteomalacia. In rare cases it runs a rapid 
course, a year or less. In most the course extends over five, ten, or more 
years, and in this time remissions may occur, lasting many months or 
years, or recovery may ensue at any time under treatment, rarely spon- 
taneously. Although pregnancy and even menstruation usually make the 
symptoms worse, improvement or even recovery has been observed in the 
former. Death is usually due to exhaustion, cardiac weakness, broncho- 
pneumonia, or some other infection. Bedsores often influence the 
exhaustion and infection. Exophthalmic goitre and myxoedema have 
been seen with osteomalacia. 
Diagnosis.-In the early stages the differential diagnosis cannot often 
be positive, even in an endemic focus. The rheumatic or hemorrhoidal 
pains of pregnancy must be excluded by careful examination of their 
seat and course, and the presence or absence of bone tenderness. 
Later, lateral sclerosis, transverse myelitis, polyneuritis, tabes, multiple 
myeloma, carcinoma, sarcoma, tuberculosis, osteitis deformans, senile 
osteoporosis, sometimes arthritis deformans, must be excluded. 
It is not necessary to speak in detail of each possibility. Only complete 
and usually repeated examination can prevent error. Multiple myeloma 
can probably not be positively differentiated in all cases. Coxa vara, 
genu valgum, or rupture of pelvic articulations may have to be con- 
sidered. Hysteria sometimes causes a striking simulation of osteomalacia. 
The differentiation of late rickets and osteomalacia depends upon the 
presence or absence of the rachitic epiphyseal changes, the less severe 
pain, and the tendency to cranial changes. Skiagraphic examination will 
often be of value. In some cases the results of treatment may aid. 
Treatment.-Prophylaxis.-Gross errors in hygiene are important to 
correct in all endemic foci of disease, or in an individual case. This is 
especially important in the latter in periods of remission or after recovery. 
Fresh air, good food, exercise, and bathing are all useful. Nursing must 
not be permitted to an osteomalacic mother. 
Diet.-Substances containing considerable lime and phosphorus should 
be preferred, such as milk, eggs, fresh meat, fish, beans, peas, and cereals. 
Drugs.-Lime has been recommended, but is useless theoretically and 
has failed in practice. Calcium phosphate is but little better. The most 
useful drug is phosphorus, which is recommended by all who have used it 
persistently in large doses. It is given most frequently in cod-liver oil, in 
solutions of 0.06, 0.08 or 0.1 to 100. A teaspoonful of this is to be taken 
once daily, or 3 to 5 mg. (grain to Ty) at each dose. If cod-liver oil is 
not well borne, almond oil can be used, as in the Oleum phosphoratum of 
the British Pharmacopoeia. It can also be taken in pills (PiL Phosphori, 
U. S. P.) containing 0.6 milligram (grain each. The treatment must 
be continued at least one or two months. Improvement may not be 
apparent for three or four weeks. Kozminski recommends prolonged 
intermittent treatment based on the bone symptoms. Sternberg has 
given as much as 2.25 grams of phosphorus without toxic symptoms. 
Phosphorus is slower and less certain, but safer than castration, the 
application of which we owe to Porro, who in 1878 reported a case of 
osteomalacia that recovered following the operation named after him. 954 
DISEASES OF THE LOCOMOTOR SYSTEM 
Fochier and Levy thought sterilization was the cause of the recovery. 
Fehling (1886) showed the value of removal of the ovaries, and developed 
the theory already mentioned. While the theory is not accepted, the 
beneficial results of operation cannot be questioned, and although efforts 
have been made to attribute the recovery either to the narcosis or the 
operation per se, they have not been successful. The results of castration 
are sometimes remarkable. Pain sometimes disappears in a few days. 
But the improvement is neither uniform nor permanent, and phosphorus 
treatment may be carried out after it with advantage. 
The indications have been formulated by von Winckel and others as 
follows: (1) Castration is indicated when other methods of treatment have 
failed and softening of the bones has advanced so far that life is endan- 
gered, or if the condition is such that death may occur before medicinal 
treatment can have an effect. (2) It is indicated in patients with pelves 
so narrow that pregnancy would necessitate Caesarean section, and in 
case Caesarean section is necessary. 
Senator has used oophorin with good results, but others (Latzko, 
Schnitzler, Bernstein) who have tried the method have failed. Bossi has 
advocated the use of adrenal preparations subcutaneously. There is an 
error regarding dosage in Bossi's publications, but he apparently used 
the material to the point of tolerance, as shown by chills, tremor, a 
feeling of suffocation, and a sense of constriction behind the sternum, 
coming on soon after the injections and lasting about a half hour. The 
symptoms increase with the number of injections. Besides Bossi, Ruffi, 
Tanturri, and Reinhardt had favorable cases, but others have failed.1 
Hoffmann has used the serum of thyroidectomized sheep and reports 
improvement in two months. Extracts of the pituitary body have been 
used with favorable results. 
In a disease like osteomalacia any treatment based upon probable 
causes seems legitimate, but the chronic and variable course of the disease 
suggests the value of caution in announcing results. 
Symptomatic Treatment.-In all cases various symptoms are certain to 
demand treatment. Comfort, the relief of pain, and the diminished 
danger of fractures should be provided for by a proper bed and care in 
moving or being moved. The skin requires care at all times, and especially 
if bed-sores threaten or occur. The bed pan and urinal must be used. 
Pain in the muscles and cramps should be relieved by hot baths, massage, 
fomentations, or at last morphine. Wine of colchicum seeds has been 
used by many with apparent advantage for the rheumatic pain. Diarrhoea 
and constipation must be treated as in other diseases. 
1 See the review of R. de Bovis, La Semaine Medicate, 1908, 241. CHAPTER XXVII. 
ACHONDROPLASIA. HYPERTROPHIC PULMONARY OSTEO- 
ARTHROPATHY. PAGET'S DISEASE. OSTEOGENESIS 
IMPERFECTA. OSTEOPSATHYROSIS. LEONTIASIS OSSEA. 
MICROCEPHALUS. FACIAL HEMIATROPHY. 
CHARLES P. EMERSON, M.D. 
ACHONDROPLASIA. 
Definition.-Achondroplasia is a disease of fetal life, possibly also of 
early infancy and childhood, which affects especially the long bones 
and the base of the skull. Its most conspicuous clinical features are: 
nanism, due to shortness of the extremities, especially of their proximal 
segments; large head; unusually deep depression of the root of the nose 
and trident-shaped hands. 
Terminology.--Among the titles under which this condition has been 
described are: fetal rickets, intra-uterine rickets, congenital rickets, 
annular rickets (used also for other conditions), and micromelic rickets. 
Achondroplasia1 is, if we regard priority, the correct term, for under 
this title the condition was first described as a clinical entity. It is, 
however, an unfortunate name since cartilaginous bone formation is not, 
as the term implies, absent, but retarded. Nor is all cartilaginous bone 
formation retarded; for instance, the development of the vertebrae and 
short bones is little if any affected. For these reasons many prefer the 
term chondrodystrophia fetalis (Kaufmann2). 
Pathology.-Achondroplasia of the Stillborn.-The great majority of 
achondroplasic fetuses die in utero, and of those which come to term the 
majority die soon after birth. These infants at once attract attention; 
the trunk and head are large but the limbs are ridiculously short. The 
umbilicus may be at the juncture of the middle and lower thirds of the 
body length. The chest is broad, the abdomen prominent. The nose 
is broad and flat; its root is depressed. The fat cheeks almost close the 
eyes. The tongue often projects from the mouth. But what attracts 
attention above all else are the short, often fin-like limbs, which give the 
child the appearance of a "marine mammal," a "cetacean," more than 
of a man. While the shortness of the limbs is seen in both distal and 
proximal segments, yet it is the proximal segments which are especially 
affected. These infants are plump, since there is present usually an 
abnormal amount of subcutaneous fat. The integument hangs in folds, 
1 Parrot, Soc. d'Anthrop., 1878, p. 280. 
2 The best study of the pathology is that of Kaufmann, Untersuch. uber die sogenann. 
fetal Rachitis {chondrodystrophia fetalis}, 1892. 
955 956 
DISEASES OF THE LOCOMOTOR SYSTEM 
giving the child the apperance of a "dwarf in much too large clothes." 
The genitalia are well developed. 
The chief lesions of achondroplasia are in the long bones and seem 
secondary to a disease of their epiphyseal cartilages which inhibits their 
growth in length. The subperiosteal bone formation, on the other 
hand, seems normally or even abnormally active. As a result the long 
bones are thick and strong, but short. Achondroplasia may begin at 
any time before birth. The earlier it begins the shorter are the bones, 
even but one-half of normal length. When it appears late the bones may 
be of normal length. The bones most affected are the humerus and femur, 
next the tibia and ulna, and then the base of the skull. Because the 
fibula and radius seem little affected the disease is assumed to begin as 
a rule at or before the eleventh week of embryonic life. The diaphyses 
of the long bones are about normal in diameter but their walls are often 
thicker and harder than normal, and are sometimes even eburnated. 
They may have no medullary canal, or,.as a result of bone absorption 
an abnormally large one. The smaller and more crooked the bones, the 
harder they are. The bones are very curved but symmetrically so. 
This is due to exaggeration of the normal curves which sometimes describe 
even right angles. In Lecadre's case1 the legs were so bowed that the 
soles of the feet exactly faced each other. There are no bizarre deform- 
ities. The prominences for muscle attachments are hypertrophied. 
The epiphyses are usually of almost normal size, but sometimes are much 
swollen and misshapen. Some are shaped like mushrooms and give the 
bones an hour-glass appearance. In many cases the epiphyses are soft. 
They always are unusually vascular. Ossification in some cases is very 
retarded, in others is abnormally advanced, in which cases the epiphyses 
are firmly united to the diaphyses. 
Those bones which do not pass through a cartilaginous stage in their 
development, for instance the frontal and parietal bones of the skull, 
grow to normal dimensions. This is true also of the clavicles, the pelvis 
and the ribs. The short bones-the vertebrae, metacarpals, etc.- 
develop from cartilage and are said to escape the disease; but it is quite 
certain that they do not always (if ever) entirely escape, although their 
involvement may be slight. 
The skull is voluminous and suggests hydrocephalus. The parietal 
bosses and the forehead are prominent. In some infants true hydro- 
cephalus has been present; in others the unusual thickness of the bones 
explained in part the large size of the head. In still other cases the skull 
is abnormally fragile. Two such cases were reported-one a seven and 
a half months' fetus reported by Champetier de Ribes and Constantin- 
Daniel-whose heads burst during delivery "without violence" by the 
Mauriceau method. In the case reported by Mary A. Smith no bone 
had developed in the vertex of the skull. However, the diagnosis in 
both these cases is doubtful. (Was lues present?) 
The frontal and parietal bones usually develop normally, but not so the 
base of the skull. At birth the foramen magnum is surrounded by a ring 
1 Paris Thesis, 1856. ACHONDROPLASIA 
957 
of four separate bones, which normally are not united to form the occipital 
bone until the sixth year. Provision is thus made for an increase in the 
diameter of this foramen. In the achondroplasic stillborn infant a 
premature synostosis of these bones is usually found, and so the funnel- 
shaped foramen magnum is surrounded by a ring of solid bone, which is 
incapable of much increase in diameter. The pressure which this small 
and solid ring must exert on the growing cord is thought to explain the 
premature death of these infants. 
The portion of the base of the skull formed by the occipital and sphen- 
oid bones, the "os tribasilaris," consists at birth of three pieces, the 
basilar portion of the occipital bone and the presphenoid and postsphenoid 
bones. The synchondrosis intersphenoidalis between the last two bones 
closes at about the time of birth, but the cartilage uniting the sphenoid 
and occipital bones is not solid until about the eighteenth or even the 
twenty-fifth year. In the achondroplasic stillborn infants these bones 
are often firmly united, and the clivus deprived of the possibility of growth 
in length. The clivus is in these cases abnormally vertical (Virchow's 
kyphosis of the skull). This shortening of the base of the skull from the 
normal 7.6 cm. to 4.6 cm., or even less, explains, according to Virchow, 
the depressed root of the nose. Kassowitz found that the root of the 
nose could be depressed also when the tribasal bone was of normal length 
or even wholly cartilaginous, and he thinks that the tissues anterior to 
the sphenoid bone in part at least explain the profile of the face. But 
the fact that this bone is cartilaginous does not indicate that it is capable 
of further growth. 
The ribs show a well-marked rosary, but the prominences are not due, 
as in rickets, to hypertrophy of the cartilage, but to overgrowth of the 
bone in a cup over the end of the cartilage. In some cases (Grawitz) 
the rosary is only apparent, and due to an angular deformity at the 
costochondral articulation. The rosary is usually much more prominent 
on the inner than on the outer aspects of the thorax. 
The vertebrae sometimes show a premature synostosis of the body and 
arch of the upper cervical vertebrae which thus develop in a way similar 
to that modified vertebra which we call the occipital bone. The clavicles 
are usually normal. Shattuck was the first to describe premature 
synostosis of the coracoid process of the scapula. The pelvis is some- 
times distorted, massive, and ill-shaped, and the sacrum is tilted forward. 
Microscopic examination of the epiphyses reveals at once the explana- 
tion of the deformities. The epiphyseal cartilage is abnormal, both its 
ground substance and its cells. The line of ossification, often not visible 
without magnification, is quite straight but very narrow. The zone of 
proliferation of the cartilage cells containing the columns of proliferating 
cells is absent. The cells are irregularly distributed, few and scattered. 
This aplasia of the zone of cartilaginous bone formation is held to be the 
characteristic lesion of achondroplasia. The intercellular substance of 
the epiphyseal cartilages is decidedly abnormal. Near the surface of the 
epiphyses it is transformed to fibrous tissue; the rest, instead of being 
hyaline, is often mucoid and sometimes fibrillar. This fibrillar structure 
may be so marked that its network can be easily seen. The cartilage is 958 
DISEASES OF THE LOCOMOTOR SYSTEM 
sometimes riddled with vacuoles. There is sometimes a broad mucoid 
or gelatinous zone between cartilage and diaphysis. These changes are 
so marked that Trippier said: "There are no epiphyses. In their place 
is another tissue, reddish and completely resembling muscle tissue." 
In other cases the epiphyseal cartilage comes in immediate contact with a 
well-ossified spongiosa, or is separated from it by a thin zone of swollen 
cells. In some cases the completely calcified laminae of spongiosa show 
all the signs of metaplastic ossification of the cartilages. So abnormal 
are the epiphyseal cartilages that the wonder is that the bones grow as 
long as they do (Kassowitz). 
But the most interesting lesion of the epiphyseal cartilages is the 
growth from the periosteum of fibrous tissue which invades the carti- 
lage. The amount of this invading tissue varies from a few fibres to a 
definite membrane which seems to push itself in between diaphysis 
and epiphysis and so by checking enchondrial bone formation inhibits 
the growth in length of the bone. This band of fibrous tissue is vascular 
and encloses in its meshes islands of cartilage and also fat cells, evidence 
of its origin from the periosteum. It is always thinnest in the centre 
and thicker at the margins. If this tissue invades the cartilage from but 
one side, the growth of bone is checked on this side only. Thus arise 
many of the angular deformities which the bones of achondroplasic 
skeletons show and, Kaufmann thinks, many of the so-called fractures. 
One of the best descriptions of these changes is that of Urtel.1 
In nearly every case the epiphyseal cartilages are very vascular. The 
vessels arise in the perichondrium and invade the cartilage, running at 
right angles to the shaft of the bone. Along their course especially does 
the fibrous tissue develop. The marrow spaces are sometimes large 
and the marrow tissue is very cellular and vascular. Giant cells are 
numerous and by their arrangement indicate active bone absorption, 
which probably explains the osteoporosis interna which one often sees. 
This bone absorption may outstrip the active periosteal bone formation 
and so the hard cortex be reduced to so thin a shell that it fractures 
easily. 
Lesions of other organs than the bones are inconstant. In a few 
cases a mild degree of true internal hydrocephalus was described, but 
Durante2 thinks there is no well-marked nervous lesion. The thyroid 
gland of the majority of reported cases was described as normal. In a 
few cases the thyroid was large, "twice the normal size," " hypertrophied" 
(Salvetti), or "absent." The thymus was large in a few cases. The 
liver has several times been reported as enlarged. 
Varieties.-Kaufmann attempted to classify the thirteen infants he 
studied in three groups; the hypoplastic, the malactic, and the hyper- 
plastic. In the first group, " chrondrodystrophia fetalis hypoplastica," 
the five cases had abnormally short but stout trunks, large heads, and 
very short extremities, over which the investment of soft tissues hung 
in tumor-like folds. The thorax was narrow above, wide below, and the 
"rosary" was very marked. The root of the nose was much depressed. 
1 Halle Thesis, 1873. 
2 Bull, de la Soc. Anat., 1900, p. 785. ACHONDROPLASIA 
959 
The eyelids and the lips were swollen, and the cheeks thick. The epiphy- 
seal cartilages were normal in size, firm in consistency and very vascular. 
In the second group, chondrodystrophia fetalis malacica, were three cases. 
The noses of these infants were depressed as a whole, and the epiphyseal 
cartilages were abnormally soft. Of the third variety, chondrodystrophia 
fetalis hyperplastica, Kaufmann reported but one example, which he 
believed to be the only case of the variety up to this time reported. 
This case differed from the others in that the mushroom-shaped epiphyses 
were soft and very vascular and caused a swelling of joints, as in rickets. 
The growth of the cartilage is described as "wild." The bones were 
hard and seemed disposed to fracture; in fact five long bones showed fresh 
breaks. The trunk of this infant was unusually long (42 cm.). Kauf- 
mann admitted that four of the thirteen cases he described could not be 
classified in any of these three groups. Regnault1 denied that the cases 
can be thus classified. He considered all as grades of one and the same 
disease, those children only with the hypoplastic variety being viable. 
Cases of partial achondroplasia have been reported. Pauly and 
Teyssier2 reported the case of a man, 138 cm. in height, who lacked 
the head abnormalities, the trident hand, and the relatively long fibuke. 
Regnault also is confident that partial forms do occur. 
Achondroplasia is often combined with other deformities and abnor- 
malities. Among these are hypospadias, cervical cysts, spina bifida, 
arrested development of the ears, arrested development of the ensiform 
cartilage, umbilical or inguinal hernia, cleft palate, genu valgum, and a 
large variety of tumors. 
Achondroplasia of Children and Adults.-Our knowledge of the pathology 
of achondroplasia of children and adults is obtained chiefly from the 
study of skeletons and of radiographs of living cases. A few cases have 
come to autopsy. The skull is voluminous, its parietal and frontal 
bones are usually prominent and its bones sometimes very thick. The 
foramen magnum is small. Its dimensions in the three adult skeletons 
studied by Breus and Kolisko were: 1.9 by 2.5 cm., 2.2 by 2.8 cm., and 
2.9 by 3.4 cm. These writers suggest that cases of achondroplasia 
be divided into two groups, those with the premature synostosis of the 
bones surrounding this foramen and those without. In some cases the 
skull seems unaffected. The ribs show a bony rosary. The vertebrae 
seem affected in severe cases, but not in the milder ones. The arches 
and bodies of the upper cervical vertebrae in some seems to have united 
prematurely, if we may judge by the stenosis of the spinal canal of certain 
skeletons. The long bones of the extremities are short and thick, their 
normal curves are exaggerated and the protuberances for muscular 
attachment are prominent and strong. 
Studies of the radiographs of adults with achondroplasia suggest two 
distinct groups of cases-those in which the epiphyseal line persists even 
at forty-five years of age, and those in which it disappears abnormally 
early. In cases of the first group the ossification of the epiphysis is much 
retarded (the hypoplastic form of Variot, the true achondroplasia of 
1 Arch. gen. de med., February, 1902. 
2 Prov. med., xiv, p. 489. 960 
DISEASES OF THE LOCOMOTOR SYSTEM 
Parrot). In the second the ossification is abnormally advanced, and 
therefore the epiphyseal cartilages are much reduced in thickness (the 
hyperplastic form of Variot). Variot points out that in both cases the 
result is the same; that is, the long bones do not increase much in length. 
As an illustration of the hypoplastic variety, he reports the case of a girl, 
aged thirteen years, in whom ossification was much retarded. The head 
of the humerus seemed crushed, and the lower end of this bone was carti- 
laginous for 1 or 2 cm. above the joint. The lower ends of the radius 
and ulna were little ossified, while the carpals and especially the metacar- 
pals were almost as cartilaginous as at birth. The same was true, but 
to a less degree, of the bones of the lower extremities. Variot contrasts 
this case with that of Mery and R. Labbe, which he considers, illustrates 
the hyperplastic form. Their patient was a boy aged twelve years, in 
whom ossification was abnormally advanced and the epiphyseal lines 
early obliterated. Since the head of the girl he reported was unaffected, 
while all the other signs of achondroplasia were present, Variot proposes 
to call such cases "achondroplasia without cranial involvement," or 
"epiphyseal dyscrasia." 
The humerus of the adult cases is surprisingly short. In ten skeletons, 
all apparently of adults, the humerus measured in length from 14 to 17 
cm., average 15.3 cm. (normal length about 27 cm.); the ulna, from 10.7 
to 15.8 cm., average 13.2 cm.; the radius, from 8 to 14 cm., average 11.4 
cm.; the femur, from 16.5 to 24 cm., average 20.7 cm.; the tibia, from 11.6 
to 20 cm., average 16.3 cm.; and the fibula, from 16.2 to 21.2 cm., average 
19 cm. The skeleton of the hand measured from 10.3 to 14.5 cm. (normal 
20 cm.), and of the foot, from 14.5 to 18 cm. (normal 25 cm.). The 
scapula is smaller than normal, especially in its vertical diameter, and its 
glenoid cavity is apparently too small for the head of the humerus 
(Cestan). The olecranon seems to be too large for the olecranon fossa. 
The fibula presents an interesting study for it is always much less affected 
than is its companion tibia, but since it is "splinted" to the tibia it must 
find room for its greater length by abnormal curves or by extending as 
high or even higher than the tibia and entering into the formation of the 
knee-joint. 
Achondroplasia in Art and History.-Of all dwarfs only those with 
achondroplasia and rickets could serve as the court buffoons so popular 
at the end of the seventeenth and again in the eighteenth centuries. That 
achondroplasics would have been particularly well adapted for this 
purpose is seen now by the number now found on the vaudeville stage. 
They are quick-witted, very active, and enjoy this occupation. 
The diagnosis of achondroplasia in products of art is not easy, for 
the artist seldom portrays the fine points necessary for a positive diag- 
nosis. In a doubtful case it would be difficult to distinguish in a picture 
between a myxoedematous and an achondroplasic dwarf, for both are 
only exaggerations of the fetal type, which the artists and sculptors of 
antiquity evidently used for symbolic purposes. The normal infant has 
a relatively large trunk and head and short extremities. In various 
museums, we were surprised at the number of crude wood and stone 
images, from a variety of barbaric tribes, which were micromelic. Pictures ACHONDROPLASIA 
961 
on the coffins of certain mummies suggest that these mummies were 
achondroplasic dwarfs. In more recent art, Velasquez'a "Sebastian de 
Morra" and "El Primo" and some of the dwarfs in the pictures of P. 
de Veronese and Julio Romano are almost certainly achondroplasic. 
Etiology.-Heredity.-This is interesting not only for its own sake, 
but also because of the bearing it may have on the origin of certain races 
of dwarfs and of certain varieties of animals (granting of course that 
animals do have this disease). Among the races of dwarfs are the Akkas 
and other African tribes, the Mincopies of the Andaman Islands, the 
Semangs of Malacca, and the J3tas of the Philippines. It is worthy of 
mention that there are no white races of dwarfs, and that all white 
dwarfs are evidently pathological. 
That achondroplasia can be inherited there is now no doubt. Marie 
accepted three cases as certainly inherited (Porak's, Baldwin's, and 
Boeckh's). One of the most interesting cases on record is reported by 
Porter.1 The father, aged eighty years, and his two sons were achon- 
droplasics. Also the father and a brother of this octogenarian were 
said to be achondroplasics. This would mean six cases in three genera- 
tions. The mothers of these patients were all normal women. Comby2 
reports a boy and his maternal grandfather who were achondroplasics, 
and Poncet a brother, sister, father, and grandfather. 
Achondroplasia is also a family disease appearing more than once in 
the same generation. Good illustrations are the family reported by Porter 
and the brother and sister reported by Lannois. Chavigny's patient said 
he was the tallest of four children, all shaped like himself. Chiari3 states 
that a man of normal size (but whose father was a dwarf) was the father 
by two normal sisters of two stillborn achondroplasic infants. Hutchison 
reports that of twins he saw the one was an achondroplasic girl and the 
other a normal boy. 
It is claimed that achondroplasia occurs in animals also. As best 
illustrations are cited the rare "bull-dog calves," which have very short 
snouts, short legs, thick, wrinkled skin, and normal development of the 
short bones. Some claim that the dachshund, the pug dog, guinea-pigs, 
Ancon sheep, the Nato's cow of Chili, Yorkshire pigs, etc., are cases of 
achondroplasic animals, but the better opinion seems to be that the 
resemblance here is accidental. 
Gigantism.-The question of a possible relationship between achondro- 
plasia and gigantism was raised by Lannois who reported the case of an 
achondroplasic man, aged twenty-five years, whose next older brother 
measured 192 cm. in height. The interval between the births of these 
two persons was very short. Two other brothers were very tall. The 
father measured only 160 cm. in height. Lunn4 reported a case of achon- 
droplasia in a patient whose father measured six feet two inches in height, 
his mother five feet ten inches, and whose three brothers and one sister 
were normal. Claudius (Marie's case) came of a tall family. Gigantism 
is in many cases due to pituitary disease. This gland has been carefully 
1 Brit. Med. Jour., 1907, i, 12. 2 Bull, de la Soc. des H6p. de Paris, 1902, 551. 
3 Deut. med. Wchschr., 1913, xxxix, 438. 
4 Trans. Clin. Soc., London, xl, 252. 
vol. v-61 962 
DISEASES OF THE LOCOMOTOR SYSTEM 
studied recently in cases of achondroplasia coming to autopsy, but in 
only one case was it found enlarged or otherwise abnormal. 
The numerous cases of achondroplasia which have occurred in large 
families suggests a possible relationship between numerous pregnancies, 
especially when these occur at short intervals, and this condition. Herr- 
mann's case was one of nine children, Wood and Hewlett's was the 
thirteenth of sixteen children. Mery and Labbe's patient was one of 
thirteen children. 
Congenital dislocation of the hip is sometimes associated with dwarfism 
and in not a few cases it has been associated with achondroplasia. Such 
cases have been reported by Kassowitz, Kirchberg, Simmonds, and 
Kaufmann. Grawitz suggests that congenital dislocation of the hip is 
due to achondroplasia limited to the pelvic bones, the lack of prolifera- 
tion of the cartilage explaining the shallowness of the cotyloid cavities. 
Symptoms.-While the great majority of achondroplasic infants are 
stillborn or die during the first year, those who reach their second year 
seem to have the average expectancy of life. As the child develops the 
disproportion between the amount of loose skin and the length of limb 
disappears. Teething, talking, and walking, the criteria by which an 
infant's progress is usually measured, seem normal in the majority of 
these children, but some, to judge from the records, are backwad. 
The adult achondroplasic presents a striking appearance, the most 
noticeable points of which are the short extremities, the body of almost 
normal size and the large head. Achondroplasics are dwarfs in the sense 
that they are shorter than normal men. Of eighteen achondroplasic 
men all over twenty-one years of age, the heights varied from 93 to 138 
cm., averaging 119 cm. Of eighteen achondroplasic women all over 
twenty-one years of age, the heights varied from 97 to 130 cm., averaging 
116 cm. The trunk of the achondroplasic adult is usually described as 
normal, but in well-marked cases there does seem to be a slight micro- 
somia. The women are usually stout, while the men are very muscular. 
Micromelia is the important mark of achondroplasia; that is, the legs 
are short, even but half the normal length, and the arms are affected 
in about the same degree. This shortening is not symmetrical for the 
root segments, the upper arm and the thigh are relatively more shortened 
than are the distal segments; that is, the condition is one of rhizomelia. 
The upper extremities hang down about to the crest of the ilium, seldom 
below the greater trochanter. The shoulders are attached slightly 
more posteriorly than normal. Owing to the small size of the cotyloid 
cavity the arms cannot hang vertically, but project slightly from the 
sides (Marie, Poncet). The bones of the arms are only slightly curved. 
Since the olecranon cavity is small, the humerus and the ulna are articu- 
lated at an angle and the arm can seldom be extended straighter than 135 
degrees. The articular fold of the elbow is more oblique than normal. 
The radius seems relatively too long and its head is so large that supina- 
tion is limited. 
The hands have a characteristic shape. They are small, short, and 
thick and, since the fingers are all of about equal length, have a cubical 
shape. The ring finger seems to lie in a plane posterior to the others ACHONDROPLASIA 
963 
and is almost covered by them. The first phalanges lie parallel, but 
beyond these some or all of the fingers, as a rule, diverge from one another 
like the prongs of a trident, giving the hand the "trident shape" described 
by Marie. The legs are often bowed or knock-kneed, which is due not 
to curves of bones, but to the joints, for the bones are articulated at an 
angle. The head of the tibia is large. The marked curves and high 
position of the fibula are often quite evident on palpation. The toes 
are of almost equal length. The joints are abnormally lax, especially 
those of the knees, the hands, and the feet. The spine shows an inter- 
esting and quite uniform abnormality in that the sacrum tilts forward 
into the pelvis, with a resultant sharp lumbar lordosis, which explains 
the contraction of the pelvis. The thoracic spine is so straight that the 
back is flat. 
Macrocephaly is the rule. The head is usually globular in shape, with 
well-marked frontal and parietal bosses. Apert reports a patient with 
a head 66 cm. in circumference and Marie a man whose head measured 
67 cm. in circumference. Of eleven men, all over twenty-one years of 
age, the heads measured in circumference from 54 to 67 cm., averaging 
59 cm.; in the case of five women this circumference varied from 52 to 
56 cm., averaging 54 cm. Brachycephalia is an almost constant feature 
and the cephalic index is usually over 0.9; in one of Marie's patients it was 
1. The shape of the head suggests hydrocephalus, but it is as a rule the 
thickness of the skull which explains this shape (Cestan). In most cases 
the closure of the fontanelles and sutures was retarded. 
The face seems small, but actually is large. The features are coarse, 
the forehead prominent, and the eyes deep. The nose is quite character- 
istic. It is short, its base is broad, its root very deep, its tip prominent 
and thick and its nostrils large. The arch of the hard palate is frequently 
high, but the teeth usually show no abnormality. Not all cases show 
these abnormalities of the skull, and Variot proposed to separate them 
into a separate group, "achondroplasia without cranial dystrophy." 
The shape of the head may be asymmetrical (Langenbach). 
The adult achondroplasics are remarkably strong, even when com- 
pared with normal men, and seem to choose, or at least not to avoid, 
strenuous work, even when elderly (Porter). They are excellent athletes, 
and popular circus acrobats, attracting attention not only because of 
their conspicuous build, but because of their strength and skill. 
It is the general belief that these patients are rather limited mentally, 
are mischievous, intemperate, and lascivious. This is by no means the 
rule. We have seen one and several have been reported whose mentality 
was of the highest order, who were students and teachers of excellent 
ability. The child of the lower classes must depend on the public school 
for education, and here the achondroplasic child will learn little that is 
good, since his schoolmates will make his life miserable. It is interesting 
that the majority of the bright patients are women. Of some it is said 
that they were bright when children but mentally rather inferior as adults. 
This would agree with the idea that in some cases there is retarded intel- 
lectual development at puberty. Marie called attention to the probable 
relation between the height of the patient and his intellectual develop- 964 
DISEASES OF THE LOCOMOTOR SYSTEM 
ment, a point well illustrated by three adult men in his clinic. Cladius, 
107.5 cm. tall, aged twenty, was a child in behavior and mind and physic- 
ally was evidently not at puberty. Anatole, aged forty-one, 122 cm. 
tall, was frolicsome, boastful, mischievous, intemperate, and lascivious. 
Surgeus, aged sixty-six, just a little taller than Anatole, was a jeweller 
by trade and a sober, married man. The age at which the disease develops 
possibly may influence the mental development. The children born 
achondroplasic appear to develop mentally as rapidly as do other children, 
but the mental development of those in whom the disease continues, 
or perhaps begins, after birth would seem to be slow. 
Sexually, these patients are well developed both anatomically and 
functionally. Many patients of both sexes seem unusually salacious. 
Some men are notoriously so, while unmarried women in the obstetric 
clinics have given the best opportunities for the study of achondroplasia. 
Apert mentions a woman with contracted pelvis who underwent opera- 
tions for three pregnancies. 
One of the most interesting subjects connected with this disease is 
its postnatal course. And yet one should remember that achondroplasia 
itself is not a disease but a condition, a deformity, the result of a disease 
whose nature is still unknown and the date of whose course is uncertain. 
The first cases described were all stillborn children in whom the disease 
seems to have run its course. In most cases the disease may run its 
entire course and heal in utero; that is, at birth there is no evidence of 
active epiphyseal new-bone formation. These cases are stillborn. In 
other cases the disease seems still florid at birth, while it would seem as 
if some infants apparently normal at birth develop achondroplasia later. 
Kassowitz1 followed the postnatal course of the disease in several 
patients for years after birth, reporting measurements and publishing 
photographs which are very convincing. Both the mother and grand- 
mother of Swoboda's2 patient said that the limbs of this baby girl were 
of normal length at birth. She was stout and so soft and flabby that they 
feared to lift her. She had a congenital left genu valgum with flat foot. 
Before she was ten years old, at which age the diagnosis of achondroplasia 
was made, at least forty medical examinations had been made by almost 
as many physicians. Her head began to enlarge after the first year, 
and she walked at three years. At first there was lumbar kyphosis, which 
changed to a lordosis as soon as she began to walk. The muscular system 
was well developed. The soft, tender flesh and the excessive sweating 
about the head led to the diagnosis of rickets, which was the opinion 
until she was ten years old. Intellectually this girl was normal. 
In many cases these children suffered during infancy from some un- 
certain acute illness, following which in some the achondroplasia was 
first noticed. "Couldn't hold up his head," "hydrocephalus," "lost 
for a while the use of the limbs," "an acute illness," "was weak," are 
among the expressions used to describe these illnesses. The writer 
had excellent opportunities to study one marked case, an adult man, 
whose parents were by no means poor and ignorant, whose mother has 
1 Wien. med. Woch., 1902, No. 28, 1358. 2 Wien. klin. Wchrschr., 1903, No. 23, 669. ACHONDROPLASIA 
965 
repeatedly told the writer the story of his infancy. There seems no 
doubt that the child was born with normal proportions, had, as an infant, 
an acute disease resembling rickets, and that signs of his deformity began 
at about four years of age. 
We may, therefore, believe that achondroplasia is a condition which 
may develop after birth, or rather that the deformities of achondroplasia 
appear after birth, for it may perhaps be unwise to confuse these de- 
formities with the disturbance of which they are the result. The symp- 
toms of the acute disease remarkably resemble rickets. There is retarda- 
tion of development, flabbiness and tenderness of the flesh and sweating 
of the head. The teething, talking, sitting up, and walking are all 
retarded, and the relative micromelia is first noticed after this illness. 
Nature.-All achondroplasic infants were formerly referred to as 
"monstrosities," and the adults as "dwarfs." Virchow termed the 
infant he reported a "fetal cretin," thus attributing the deformity to 
lack of development of the thyroid gland. Fortunately this infant was 
preserved as a museum specimen and so was studied later by Klebs.1 
There is no doubt but that this infant was typically achondroplasic. 
In several other achondroplasic infants lesions of the thyroid were re- 
corded. Many cases have been tested with large doses of thyroid extract, 
but without improvement. Cavazzani reported a case which he thinks 
unique. The mother of an achondroplasic infant had exophthalmic 
goitre. During pregnancy she took large and finally toxic doses of thyroid 
extract. Later the child was given the same drug in large doses. Both 
showed a marked tolerance to this extract. 
Against the thyroid theory it is claimed that in achondroplasia ossifica- 
tion is rapid and in cretins retarded. But this is not always true. Sim- 
monds stated that there certainly are cases with combined achondroplasia 
and myxoedema. In their recent paper Symmers and Wallace2 uphold 
this view. They maintain that there is a cretinoid variety of chon- 
drodystrophia fetalis in which in addition to changes in the osseous 
system there are modifications in the soft tissues (lips, cheeks, eyelids, 
nostrils, ears, tongue, etc.) which are attributable to pathological defects 
in the thyroid gland. In these cases they found extensive chronic pro- 
ductive inflammatory process of the thyroid gland eventuating in replace- 
ment of the colloid vesicles by means of overgrowth of alveolar epithelium 
or by invasion or substitution of the alveoli by connective tissue elements 
derived from the interstitium, aided in both instances by compression 
exerted from the outside by the contracting fibrous trabecula?. It may 
be, however, that such changes in the thyroid gland are not the cause of 
the achondroplasia, and if they are we must then explain the thyroid 
lesions. It is possible that we may find them the result of the same 
disease which disturbed the cartilaginous bone formation. This same 
point of view should be held in discussing the lesions found in other 
ductless glands, for Cestan claimed that the disease certainly originates 
in utero before glandular activity begins, and so cannot primarily be 
due to any defective internal secretion. 
1 Arch. Exp. Path., 1874, Bd. ii, p. 70. 2 Arch. Int. Med., 1913, xii, 37. 966 
DISEASES OF THE LOCOMOTOR SYSTEM 
II. Muller (18G0) indentified this condition with rickets. The ordinary 
rickets, he said, develops after birth; this form runs its whole course 
in utero. Since both rickets and achondroplasia are conditions as yet 
not understood, we are in no position to say whether or not they are 
related. They may indeed be manifestations of one and the same 
disease. They surely are no more different than various forms of tuber- 
culosis, for instance. The three questions are: Do the two diseases 
resemble each other clinically and morphologically? and the almost 
universal answer is that they do not sufficiently to justify the term 
" fetal rickets." The second question: Is there a fetal form of the disease 
we now term rickets? The cases now termed fetal rickets would seem 
to be different from achondroplasia, and yet the more detailed the 
description the more certain we are that they are cases of achondroplasia. 
The third question: Is there a postnatal form of achondroplasia and 
does it resemble common rickets? There surely is and it does not. Fetal 
rickets is described as a disease beginning in the second half of pregnancy 
and in full bloom at birth. Its lesions are isolated and circumscribed, 
consisting of bony softening and tumefaction, and which produce asym- 
metrical curves. The ribs are always affected. The earlier writers 
separated fetal rickets, which runs its entire course in utero, congenital 
rickets, florid at birth, and common rickets, which begins after birth. 
In both rickets and achondroplasia the cartilages are abnormally 
vascular, but the vessels differ in size, structure, and arrangement. In 
rickets there is luxurious cartilaginous formation. The columns of 
swollen cartilage cells are abundant and extend in all directions. In 
achondroplasia they are scanty, and (differing from rickets) the ground 
substance of the cartilage is nowhere normal but fibrillar or glassy. In 
postnatal rickets there is very little real shortening of the bones. The 
limbs are short because the bones are bent, and the bends are pathological, 
asymmetrical and bizarre, while in achondroplasia they are symmetrical, 
and only exaggerations of the physiological curves. In rickets there is 
relatively more involvement of the bones of the thorax, pelvis and spinal 
column than in achondroplasia, although it is now granted that in the 
latter disease these bones may be slightly affected. There is a rosary 
in both cases, but careful examination of the bones shows the marked 
difference that in rickets the swelling is cartilaginous, in achondroplasia 
it is bony. In rickets the shape of the head is quite different from that in 
achondroplasia, although we have seen an achondroplasic person with 
typically rickety head. Even since the first it has been granted that 
typical achondroplasics do show signs of rickets, e. g., the high forehead 
with prominent frontal bosses, Harrison's groove, and some bizarre 
bends of the long bones. But we have contrasted above achondroplasia 
and common rickets, two conditions neither of them a disease, and botli 
the results of unknown diseases. We have brought forward no evidence 
against the possibility that the two conditions are different forms of 
one and the same disease. Marie admits the occurrence of both in the 
same patients, and Macewen1 emphasizes the occurrence of both diseases 
in different children of the same family. 
1 Brit. Med. Jour., 1907, ii, 1646. ACHONDROPLASIA 
967 
Symington and Thomson1 described achondroplasia as an arrest, or 
perversion, of the normal processes of endochondrial ossification of the 
most definite and universal character, involving, during intra-uterine 
life, every element of the skeleton. Durante2 described the process as a 
sclerosis of the zone of endochondrial ossification. 
It is claimed that achondroplasia is the result of a maternal intoxica- 
tion or a fetal auto-intoxication. Durante3 reported one patient whose 
mother was luetic, and another whose mother died of liver and renal 
trouble. He reports also4 a patient whose mother died of tuberculosis 
during the pregnancy. Peloquin believed it due to a maternal infection. 
The opinion is gaining ground that achondroplasia is a luetic or paraluetic 
affection. While the question of the relation to rickets is at present not 
capable of solution, that of lues is, and in all stillborn achondroplasic 
infants search should be made for the Treponema pallidum. 
Syphilis can produce a micromelic dwarf. Birrenbach5 reported the 
case of a stillborn luetic infant which he says was certainly a case of 
achondroplasia. The coincidence of the two conditions may have been 
accidental. 
Diagnosis.-For the diagnosis of achondroplasia of the stillborn child 
Durante believes that the gross appearance is not enough but microscopic 
examination should be made to demonstrate disturbance in the carti- 
laginous ossification. In the living child the radiographs may show 
either too little or too much cartilage at the epiphyses. 
For the diagnosis in the adult it will be necessary to state first just what 
features the case must present to warrant the diagnosis of achondroplasia. 
Most will agree that these are dwarfism due to rhizomelic micromelia in 
an individual whose trunk is of almost normal size. But is this clinical 
picture always due to the same disease or can several diseases cause it? 
There are several types of dwarfs. Some are small in all dimensions 
but have practically normal proportions. Lanceraux gives the upper 
limit of height of these "true dwarfs" as 120 cm. In this strict sense the 
achondroplasic individual is not a dwarf. Other dwarfs owe their con- 
dition to disease of the skeleton, especially rickets and achondroplasia, 
and still a third group to general disturbance of nutrition, as myxoedema 
and congenital lues. 
Infantilism is a term frequently used of dwarfs. By this is meant 
a condition in which the physical proportions of youth persist in adult 
age. The growth in height may not be stunted, although it usually is, 
the limbs are relatively long, the head is small, and the secondary sexual 
characteristics are late in development. It is at once evident that the 
term is a misnomer. Puerile would be a better term. The infant is in 
this sense not "infantile," but fetal, for its limbs are relatively short and 
the head large. The achondroplasic dwarf resembles much more the 
infant than the youth, but the secondary sex characteristics are well 
developed, even abnormally so. There is a rule which will be found useful 
1 Laboratory Reports of Royal College of Physicians, 1892, iv, 238. 
2 Rev. med. de la Suisse romande, 1902, 809. 
3 Bull, de la Soc. Anat., 1900, p. 785. 
4 Rev. med. de la Suisse romande, 1902, p. 809. 5 Thesis, Griefswald, 1901. 968 
DISEASES OF THE LOCOMOTOR SYSTEM 
in studying patients for traces of infantilism or achondroplasia. In 
the normal adult the length of the forearm from the tip of the olecranon 
to the tip of the middle finger is equal to that of the lower leg from the 
lower tip of the patella to the sole of the foot, and this distance is approx- 
imately twice the height of the head from the level of the vertex to the 
point of the chin. The achondroplasic patient should be compared 
always with a person of the same age, not with a child of the same height. 
This is important, since Cestan has shown that many illustrations in art 
may not be cases of achondroplasia, but of infantilism (fetalism). 
Jeffrey Hudson grew over two feet after he was thirty years of age. 
Borwiloski was twenty-eight inches (71 cm.) when twenty-two years old, 
but grew even when in extreme old age. Bebe, the dwarf of King Stanis- 
laus of Poland, died when twenty-two years of age and 90 cm. in height. 
Infantilism is seen in cases of mitral heart disease, but no cardiac dwarf 
has as yet been reported whose height was under 140 cm. Infantilism is 
seen also in idiots and in cases of pancreatic disease. But these cases 
seldom confuse, for their slender trunk, lack of development of the second- 
ary sexual characteristics and especially their small head, will distinguish 
them from achondroplasic dwarfs. The achondroplasic person is a 
deformed dwarf, and this deformity consists in rhizomelic micromelia. 
That is, he is an "ectromelus" ("aborted limbs," a monster with arrested 
development of the limbs). He also suggests a "phocomelus ("seal 
limbs," a monster with shortened arms and thighs, with hands and feet 
attached almost directly to the trunk). The adult rachitic dwarf is 
short chiefly because his legs are bowed. Some micromelia is present 
but not rhizomelia. His arms are relatively long, the thorax is much 
involved, and the hands are not trident-shaped. The myxoedematous 
dwarf or cretin is distinguished by the infantile condition of his body, 
trunk and limbs, his very reduced mental state, his dry and myxoedema- 
tous skin, dull facies, etc. Patel considers that dwarfs with practically 
normal proportions and without signs of myxoedema or achondroplasia 
are cases of acquired athyroidism due to atrophy of the thyroid gland 
at the age of ten or twelve years. 
Prognosis.-The great majority of achondroplasic infants are stillborn, 
having died at the eighth month of intra-uterine life. The reason for the 
death of so many at just this age may be due to pressure on the spinal 
cord by the prematurely synostosed occipital bone. Regnault believed 
that of achondroplasic infants only those with the hypoplastic form 
survive, and by hypoplastic he meant sluggish cartilaginous bone 
formation. 
Treatment.-Any treatment for achondroplasia will, of course, help 
only those patients in whom the disease is florid after birth. For these, 
careful feeding, massage, passive movements, electricity, and all possible 
measures to keep up the general nutrition are indicated. 
Congenital Osteosclerosis.-Schmidt1 reported the case of an infant 
who died thirty hours after birth, with slight thickening of the long 
bones but considerable thickening of the spongiosa, especially of the 
1 Verh. d. Deut. path. Gesellsch., 1907, ii, 288. THE HYPERTROPHIC OSTEOPATHIES 
969 
vertebrae, base of the skull, sternum, and the ends of the long diaphyses. 
The hypophysis of this infant weighed 0.28 gram, or twice the normal 
weight for an infant of this age. There were no signs of lues. Schmidt 
was sure that the process was not due to marrow changes. He quotes 
Assmann1 who reported such a case, and ascribed it to healed intra-uterine 
leukemia. The hypophysis in this case was small but very vascular. 
Pseudo-achondroplasia, Periosteal Dysplasic Type.-Gonnet2 reported 
a case of "pseudo-achondroplasia of the periosteal dysplasic type." 
In body form this child resembled an achondroplasic infant, but crepita- 
tion of the humeri, femora, and tibiae could be produced, and the mem- 
branous pouch of the skull contained only a few islands of bone. Trillat3 
reported a similar case with quite typical achondroplasic body form, but 
with many fractures of the long bones and ossification of the cranium. 
Dyschondroplasia.-This is the name Ollier4 applied to cases with 
irregularity in the ossification of the bones. The seemingly normal 
cartilage ossifies abnormally slowly. Radiographs of these cases show 
transparent areas in the bones, which at first glance might suggest 
sarcoma. This condition is seen in children. They are not dwarfs, and 
if they are micromelic it is not to such a degree as in achondroplasia. 
They have not the skull changes and seldom the trident hand of achon- 
droplasia. There may be slight atrophy of the affected limb. 
Hyperchondroplasia.-This is a condition with abnormal lengthening 
and attenuation of the bones, with increase in width of cartilages of 
conjugation. Mery and Babouneix5 reported such a case. Fournier 
had reported such cases as due to hereditary lues. 
THE HYPERTROPHIC OSTEOPATHIES. 
From the large and very confused group of the hypertrophic osteo- 
pathies certain types have been separated as distinct diseases. Among 
them are: general hyperostosis (Friedreich, 1868); osteitis deformans 
(Paget, 1876); acromegaly (Marie, 1885); chronic pulmonary osteo- 
arthropathy (Marie, 1890). And yet of all the many cases of hypertrophic 
osteopathy but few can definitely be assigned to any one of these sub- 
groups. Some are reported as atypical cases, others as "mixed" cases, 
others as separate diseases, while more were atypical probably only 
before the disease was fully developed and lost their peculiar features 
years after they were reported. 
While most writers consider the above diseases as distinct, several 
French writers insist that they all are illustrations of one or perhaps two 
different diseases, and that their chief differences lie in the localization 
of the lesions. Some think that the causal disease is tuberculosis; 
others, syphilis. Pathologically there is a certain similarity in all these 
cases; one finds bone absorption and new bone formation; callus building 
1 Beit. z. osteoschl. Anamie, Zeigler's Beitrdge, 1907, Band xli. 
2 Bull, de la Soc. d'Obstet. de Paris, 1908, xi, 185. 3 Ibid., 183. 
4 Bull, et mem. de la Soc. de Chir. de Lyon, 1889-1890, iii, 22; Molin, Thesis, Lyon, 
1901. 
5 Soc. Hop. de Paris, July 4, 1902. 970 
DISEASES OF THE LOCOMOTOR SYSTEM 
and condensing osteitis; spontaneous fractures and various deformities, 
either angular, resulting from one area of local softening or fracture, 
or general, from a bending of the bone as a whole. Among the earlier 
terms used for these cases are rickets, osteomalacia, osteoporosis, osteo- 
sclerosis, etc. 
Paget's Disease; Osteitis Deformans; Ostitis Deformans.-The term 
ostitis deformans has long been used of various bone diseases which 
cause deformity of one or several bones. This term included not only 
nearly all the constitutional diseases of bone as we now know them but 
also callus building in the repair of genuine fracture, of spontaneous 
fractures in lues, congenital deformities, etc. The cases now described 
as of Paget's disease were formerly certainly grouped under this title 
(Sternberg). 
The term osteitis deformans was first used by Czerny in 1873 to describe 
a case of spontaneous curvature of the lower limbs. This was probably 
a case of "osteomalacia." In 1877 Paget used this term for that group 
of cases which he described as a separate disease, which disease now 
commonly bears his name. 
Terminology.-The various names which have been applied to this 
disease are: Ostitis deformans; osteitis deformans (Paget); Paget's 
disease; pseudorachitis senilis; osteomalacia chronica deformans hyper- 
trophica; osteite ossifiante diffuse (Lancereaux): osteolysis (Lobstein); 
hypertrophie spongieuse des os; craniosclerose; hyperostose generalisee; 
osteite condensante; osteomalacie hypertrophique benigne; pseudoarach- 
itisme senile; rhumatisme osteohypertrophique des diaphyses et des os 
plats; osteomyelite fibreuse; and sclerose osseuse hypertrophique. 
Occurrence.-Paget's disease is a rather rare condition. Clopton was 
able in 1906 to collect but 75 cases from the literature. There had been 
but two cases at the Johns Hopkins Hospital among over 20,000 medical 
patients. Hurwitz reported the cases from this clinic.1 Sternberg stated 
that the cases were about equally divided between the sexes, but Clopton 
reported that two-thirds of the cases were males. 
Etiology.-Among the factors suggested are lues, cancer, arteriosclero- 
sis, nervous lesions, muscular work, and gout. 
Heredity.-In favor of this the following cases may be cited: Chauffard 
mentions a mother and daughter, and Berger a mother and son similarly 
affected (Lannelongue2). Smith3 mentions a father, seventy-four years 
old, who then had had Paget's disease for thirty-five years, and his 
son forty-two years of age, with Paget's disease then of three years' 
standing. In each of these cases the disease began when the patient 
was thirty-nine years of age. 
Pathology.-This disease affects especially the bones in the long axis of 
the body and in the following order of frequency: skull, tibiae, femora, 
pelvis, spine, clavicles, ribs, and the radii. Often, but not always, the 
long bones are symmetrically affected, but for a long time one or only 
1 Johns Hopkin's Hosp. Bull., 1913, xxiv, 263. 
2 Bull, de l'acad. de med., 1903, xlix, 299, Discussion. 
3 Trans. Med. Soc., London, 1905, xxvii, 324. THE HYPERTROPHIC OSTEOPATHIES 
971 
a few bones may be involved. The bones of the face, hands, and feet 
are least often affected. 
The skull is often from one-half to three-quarters of an inch thick, the 
thickness due for the most part to new bone formation on the outer 
lamella. Early in the disease the new bone is porous, vascular, and so 
soft that it can be cut with a knife, but it hardens progressively until 
in ten or more years it may be like ivory; or it may become loose and 
friable. There is also some increase in the thickness of the inner table of 
the skull. The furrows for the vessels, especially that for the middle 
cerebral artery, seem deepened. There is obliteration of many or all of 
the sutures. The number of nutrient foramina is greatly increased. 
Some emissary veins disappear. The horizontal circumference of the 
skull is increased, e. g., in one of Paget's cases it was 71 cm. and in Stil- 
ling's case 64 cm. The bones of the face are as a rule little changed, with 
the exception of the superciliary ridges which are hypertrophied. In 
some cases, however, the bones of the face are much affected, a condi- 
tion called by some hyperostosis cranii, supposed by Paget to be a 
distinct disease. 
"The shafts of all the long bones are white, nodular and massive, and 
present an appearance of rugged strength and hardness which causes 
them at a distance to look as though hewn out of stone. The nodules are 
in the main smooth and rounded, but in parts, as, for instance, the upper 
end of the humerus, are craggy and irregular. The nutrient foramina are 
increased in size and number" (Waterhouse).1 The deposits of new 
bone follow in general the normal ridges and projections, the deformities, 
therefore, suggesting exaggerations of the normal shape of the 
bones. 
The long bones of the legs are especially affected. The femur is much 
thickened and bent, its convexity outward. The girth of this bone at 
its middle in Waterhouse's patient was five and three-quarter inches. 
The arrangement of the cancellous tissue of the femur is destroyed. The 
tibiae are usually and often markedly affected. They are huge and very 
much bent, their convexities forward, but in some cases and even to an 
extreme degree the convexity is outward. The tibiae are thickened espe- 
cially along their anterior margins. The fibulae also are much thickened 
and bent. The tibiae and fibulae may be connected for some distance by a 
dense bridge of bone, suggesting the presence of a preexisting periostitis. 
The bones of the upper extremities are much less affected, and yet the 
changes in them may be very marked. Indeed, in a few cases the disease 
begins here. The humerus may weigh more than twice as much as a 
healthy bone of the same length and the girth of this bone at its middle 
may be four and one-quarter inches instead of two and one-half inches as 
normal (Waterhouse). 
The spine shows a marked kyphosis and often partial ankylosis. The 
bones of the pelvis if changed at all are only somewhat thickened, though 
the appearance of the patient usually suggests that the pelvis is much 
broadened. A heart-shaped pelvis similar to that in osteomalacia has 
been described. 
1 Lancet, 1907, i, 1215. 972 
DISEASES OF THE LOCOMOTOR SYSTEM 
These bone changes have been carefully studied, and yet little has 
been added to the description of Paget. Stilling describes the disease as 
a chronic inflammation, a rarefying osteitis, which begins beneath the 
periosteum and involves gradually the centre of the bone, with the 
formation of new Ilowship's lacunae, Haversian spaces, and perforating 
canals. In addition there is also new bone formation, both subperiosteal 
and myelogenous. The latter process gradually gains on the former and 
so the bones thicken. The new bone is at first soft and yielding, and bends 
easily on pressure. The calcification of the new bone may proceed 
irregularly, when retarded leaving cysts full of cloudy contents, when 
accelerated producing true bone tumors (Clopton).1 Von Recklinghausen 
thought that the first step was an osteomalacia resulting in a marked 
reduction in the cortex and thus allowing the bones to bend. This was 
followed by an inflammatory process in the malactic areas, characterized 
by the formation of fibrous tissue (Clopton). Steel pictured the process 
as follows: first, the absorption of bone with the enlargement of the 
Haversian canals; then, the formation of new bone which runs diffusely 
through the old portions. This new bone remains uncalcified and is, in 
turn, absorbed. The medullary substance is converted into vascular 
connective tissue. Lunn thought that constitutional conditions caused 
atrophy of much of the skeleton. This led to deformity of those bones 
most used and that to a compensatory hypertrophy. 
The result of these changes is a very variable picture. "The enlarged 
bones are abnormally dense or rarefied. The surface is smooth or rough, 
nodular or protuberant; the cortex thickened or thinned, spongy or 
eburnated; the cancellated structure sclerosed or coarsely trabeculated; 
the narrow spaces obliterated or transformed into cavities of various 
size. The central canal of the long bones is narrowed or dilated even to 
disappearance, and the deformity of these bones is further increased by 
various degrees of abnormal curvature. There is no uniformity in the 
distribution of these alterations, and a single bone may be evidence 
of the decalcification, absorption, the formation of osteoid tissue and 
its calcification, which are the processes concerned in the production of 
the gross changes. Multiplicity of the bones affected is the constant 
characteristic." (Fitz.)2 
It was at first supposed that the abnormal curves in the bones could be 
explained as the result of tension, and that the new bone formation was 
a protective compensatory process. But this can hardly be true. Von 
Recklinghausen pointed out that the hypertrophy was more than com- 
pensation would demand and that the distribution of the new bone did 
not suggest compensation. For instance, the new bone of the tibia is on 
the convexity, not the concavity, of the twisted bone, while the new 
bone of the skull could not furnish any mechanical advantage. He 
suggested that the inflammation which produces the new bone might be 
due to trauma and thermal influences. These enlarged bones are easily 
fractured, which accident may lead to the diagnosis. 
1 Interstate Med. Jour., 1906, xiii, 223. 
2 Trans. Assoc. Am. Phys., 1902, xvii, 398. THE HYPERTROPHIC OSTEOPATHIES 
973 
General arteriosclerosis is a practically constant lesion in cases of 
Paget's disease. This sclerosis while usually extreme need not be uniform. 
Beclert: suggested that the bony changes were due to extreme sclerosis 
of the nutrient arteries of the affected bones. Hypertrophy of the heart 
is an almost constant feature. Valvular lesions, especially of the mitral 
valve are common, and seem the result of atheromatous changes rather 
than of endocarditis. 
Joint changes are rare, but in one case both knees showed the lesions of 
arthritis deformans. 
The central nervous system has been carefully studied in the hope of 
finding the essential lesion of this disease, but while many lesions have 
been described they can best be explained as the result of arteriosclerosis. 
Tumors are not infrequent in cases of Paget's disease. Among these 
are endothelioma (?) of the pleura and radius (Paget); carcinoma of the 
liver (Goodhard); tumor of the dura mater (Wilks); sarcoma of the tibia 
(Wherry); enchondroma of the pelvis (Lunn); cancer of the stomach 
(Moizard and Bourges), etc. A few cases of Paget's disease have later 
developed osteosarcoma, especially of the giant-cell variety. 
The nature of this disease is unknown. Some have considered it merely 
an extreme grade of "arthritis deformans." Most observers, however, 
agree with Paget that it is an inflammation of bone of the nature of which 
little is known. One of the most interesting recent suggestions is that 
Paget's disease is luetic in origin. This idea was first seriously advocated 
by Lannelongue1 in an article entitled "Note on Hereditary Syphilis of 
Bones in the Newborn (Parrot's Disease) in Children and Youths, in 
Adults, and in the Aged (Paget's Disease)." According to this writer 
Paget's disease is a manifestation of hereditary lues in the aged. He based 
this opinion on the similarity of the lesions. Fournier agreed with 
Lannelongue, and mentioned the cases of two brothers, the one of whom 
developed typical hereditary lues and the other Paget's disease. Fourn- 
ier later speaks of Paget's disease as a paraluetic condition, to be classed 
with tabes dorsalis, general paresis, etc. Auffret2 reported a case of 
Paget's disease in a patient who probably had hereditary lues, and 
mentioned other cases. A case reported by Dufour, Bertin and Mourot3 
is that of a woman, aged eighty years, in whom the Wassermann blood 
test was positive and whose severe neuralgic pains were much relieved by 
neosalvarsan. 
Definition and Clinical Picture.-"From these five cases, which, although 
few, are well-marked and in some points uniform, as well as from a recol- 
lection of two more, of which I have no notes, I think we may believe 
that we have to do with a disease of bones of which the following are the 
most frequent characters: It begins in middle age or later, and is very 
slow in progress, may continue for many years without influence on the 
general health, and may give no other trouble than those which are 
due to the changes of shape, size and direction of the diseased bones. 
Even when the skull is hugely thickened, and all its bones exceedingly 
altered in structure, the mind remains unaffected. 
1 Bull, de l'acad. de med., 1903, 3s., xlix, 299. 
2 Rev. d'Orthopedie, November, 1905. 3 Deut. med. Wchnschr., 1913, xxxix, 976. 974 
DISEASES OF THE LOCOMOTOR SYSTEM 
"The disease affects most frequently the long bones of the lower extrem- 
ities and the skull, and is usually symmetrical. The bones enlarge and 
soften, and those bearing weight yield and become unnaturally curved 
and misshapen. The spine, whether by yielding to the weight of the 
overgrown skull or by change in its own structures, may sink and seem to 
shorten with greatly increased dorsal and lumbar curves; the pelvis 
may become wide; the neck of the femur may become nearly horizontal, 
but the limbs, however misshapen, remain strong and fit to support the 
trunk. 
"In its earlier periods, and sometimes through all its course, the disease 
is attended with pains in the affected bones, pains widely various in 
severity, and variously described as rheumatic, gouty, or neuralgic, not 
especially nocturnal or periodical. It is not attended with fever. No 
characteristic conditions of urine or feces have been found in it. It is not 
associated with syphilis or any other known constituent disease, unless it 
be cancer. 
"The bones examined after death show the consequences of an inflam- 
mation, affecting in the skull the whole thickness, in the long bones chiefly 
the compact structure of their walls, and not only the walls of their 
shafts, but in a very characteristic manner, those of their articular 
surfaces. 
"The changes of structure produced in the earliest periods of the disease 
have not yet been observed, but it may certainly be believed that they 
are inflammatory, for the softening is associated with enlargement and 
with excessive production of imperfectly developed structures, and with 
increased blood supply. Whether inflammation in any degree continues 
to the last, or whether after many years of progress any reparative 
changes ensue, after the matter of a so-called consecutive hardening, is 
uncertain. 
"Holding, then, the disease to be an inflammation of bones, I would 
suggest that, for brief reference, and for the present, it may be called 
after its most striking character osteitis deformans. A better name 
may be given when more is known of it" (Paget1). 
Five years later Paget2 reported additional cases and drew the clinical 
picture more graphically as follows: "It usually affects many bones, 
most frequently the long bones of the lower extremities, the clavicles, 
and the vault of the skull. The affected bones become large and heavy, 
but with such weakening of their structure that those which have to 
carry weight or to bear much muscular traction become unnaturally 
curved and misshapen. The disease is very slowly progressive and is 
felt only in pain, like that of rheumatism or neuralgia, in the affected 
limbs and in increased heat at the tibiae. But neither the pain nor the 
heat are constant, nor do they continue during the whole progress of the 
disease; and pain has not been observed in the head even in the cases in 
which the skull was very thickened. There is not any clear evidence of 
general disturbance of health. In all cases traced to the end of life, death 
has ensued through some coincident, not evidently associating, disease, 
1 Trans. Path. Soc., London, 1877. 2 Med. Chir. Trans., London, 1882, Ixv, 225. THE HYPERTROPHIC OSTEOPATHIES 
975 
which has been aggravated by the condition of the bones only in so 
far as they may have diminished the range of breathing and the general 
muscular activity. 
"At present, with the exception of the seventh case, this disease has 
been observed as beginning only in persons over forty years old, and has 
appeared in no usual relation, whether by inheritance or coincidence, 
with any other disease except gout. In all of the cases I have seen the 
general appearance, postures, and the movements of the patients have 
been so alike that these alone might often suffice for the diagnosis of the 
disease. The most characteristic are the loss of height, indicated by the 
low position of the hands when the arms are hanging down; the low 
stooping, with very round shoulders and the head far forward, and with 
the chin raised as if to clear the upper edge of the sternum; the chest 
sunken toward the pelvis, the abdomen pendulous; the curved lower 
limbs, held apart and usually with one advanced in front of the other, and 
both with knees slightly bent; the ankles overhung by the legs, and the 
toes turned out. The enlarged cranium, square looking or bossed, may 
add distinctiveness to these characters, and they are completed in the slow 
and awkward gait of the patients and in the shallow costal breathing 
compensated by wide movements of the diaphragm and abdominal wall, 
and in deep breathing by the uplifted shoulders. I have seen no case in 
which these characters are imitated except in those of ankylosis of the 
vertebrae and ribs, and which have been described by Dr. Allen Sturge 
under the name of spondylitis deformans; but these are easily distin- 
guished by the lower limbs being naturally straight and the clavicles and 
skull unchanged." 
Symptoms-The disease, as a rule, begins late in life, the average age 
of onset being about fifty years. The youngest case reported began at 
twenty-one years, while the oldest began at eighty-two years of age. The 
maximum age, however, is difficult to determine since the disease may 
have existed for years before any objective signs appeared. 
The disease begins insidiously, usually with rheumatoid pains in the 
legs. The patient seldom notes the developing deformity. He may notice 
that he has to buy larger and larger hats, and he often complains that his 
legs are getting less and less serviceable, are becoming weak and awkward, 
and that he tires easily. But his friends will note that he is growing 
shorter, that his legs are getting more and more bowed, the back bent, 
the arms relatively longer, and his whole appearance more ape-like. The 
changes usually begin in one tibia, then extend to the other, and then 
in irregular sequence the other bones. The bowing of the legs is both 
anterior and lateral. The lateral bowing may change a condition of 
knock-knees to one of bow-legs, and the result suggests poorly treated 
fractures (Waterhouse's case). In some the trouble begins in the skull. 
The disease is usually progressive, affecting the bones in irregular se- 
quence, although it may remain limited to one bone for years as in one of 
the writer's cases in which for years the left femur alone was affected. 
The disease seems to progress until death, which, as a rule, is due to an 
intercurrent infection, to cancer, or to accident, for Paget's disease itself 
does not seem to shorten life. 976 
DISEASES OF THE LOCOMOTOR SYSTEM 
The clinical picture given by Paget and quoted above is so graphic 
that we will merely add the opinion of many that leontiasis ossea is a 
form of this disease. If this is true, then in Paget's disease, in addition 
to the above mentioned symptoms, the changes in the bones of the face 
may be marked, the cranial nerves much affected, the headache severe, 
and there may be mental disturbances. 
The patient's height may shorten one foot, owing to the kyphosis of 
the spine and the bowing of the legs. In one of the Johns Hopkins Hos- 
pital patients whose height on admission was four feet six and one-quarter 
inches the shortening seems to have been more than one foot. Pain in the 
legs, especially below the knees is the commonest symptom of onset. 
These pains may precede by several years the visible changes in the 
skeleton. The pains are sometimes "in the arms and legs and around 
the hips, more in the muscles and sinews than in the bones and joints.'' 
They may be worse at night, and often seem to depend on changes in the 
weather. In some cases these pains are entirely absent, while others, 
especially those with marked spinal curvature, have pain only on motion. 
So much do cases vary as regards pains that Joucheray separated a 
"painful" and a "painless" variety of Paget's disease. The latter cases 
are less marked and develop more slowly. Fractures of the involved 
bones are not infrequent. 
As the disease progresses the thorax becomes narrow and the ribs so 
immovable that respiration finally may be purely diaphragmatic. Other 
symptoms than those mentioned above are unusual. The patients feel 
"well," for Paget's disease seems to have little influence on the general 
health. Some patients complain of bronchitis, which is not remarkable 
considering the rigidity of the chest wall, while cardiac disturbances, 
valvular and myocardial, are not rare, which is easily explained by the 
arteriosclerosis. Melanoderma was an interesting feature of Hudela and 
Heitz's case. 
Emphasis has been laid on the claim that in Paget's disease the mind 
is unaffected, and yet in Fitz's case the "mild stupor, depression and 
delusions" were sufficient to necessitate confinement in an asylum. 
Diagnosis.-A fully developed, well-marked case of Paget's disease can 
usually be recognized at a glance, but an early diagnosis is often difficult. 
These patients may be treated for years for "muscular rheumatism," 
"chronic rheumatism," neuralgia, sciatica, senile marasmus, arterio- 
sclerosis, etc. The early cases with involvement of but one bone are 
particularly difficult of diagnosis, for some have believed that "multiplic- 
ity of the bones affected is the constant characteristic." Nevertheless 
the disease may be for years limited to one bone and since the disease 
may begin at eighty years of age or over, many patients must die before 
the second bone becomes involved. This difficulty of diagnosis should 
be emphasized, since some studied cases may not have been Paget's disease 
(e. g., the value of the findings in the case studied by Apert, Bornait, and 
Legueule1 depends entirely on the correctness of their diagnosis). Many 
pathologists believe that the bone changes are not specific, others that the 
1 Bull, el mem. de la Soc. med. des Hop. de Paris, March 14, 1907, p. 235. THE HYPERTROPHIC OSTEOPATHIES 
977 
diagnosis can be made by the microscopic study of the bone lesions 
if the pathologist has several bones at his disposal. If, then, a careful 
pathological study may not be conclusive, how difficult must be the 
clinical diagnosis. Lues would be a natural diagnosis when one tibia 
only is affected. When the disease is more fully developed one must 
exclude osteomalacia, spondylitis deformans, acromegaly, hypertrophic 
pulmonary osteoarthropathy, and neoplasm. The differential diagnosis 
between Paget's disease and diffuse or tubercular hyperostosis will 
depend on one's definitions. 
The relationship between osteomalacia and Paget's disease may be 
closer than is generally believed. There are two forms of osteomalacia 
which have a fair claim to recognition as clinical entities: the osteomalacia 
of pregnancy especially and senile osteomalacia. The points which 
osteomalacia and Paget's disease have in common are, kyphosis, the 
shortening of stature, the bending of the limbs, and the rheumatoid pains. 
But in osteomalacia there is no hypertrophy of the bones, the involve- 
ment is symmetrical, the deformities begin in the skeleton of the trunk 
and later involve the extremities, the skull is affected only in the severest 
cases and the pains are much less pronounced. In puerperal osteomalacia 
the bone changes begin in the pelvis and lumbar spine. In senile osteo- 
malacia pain is prominent, and these patients are bed-ridden early. 
Hyperostosis cranii is a disease described as involving chiefly the skull. 
The formation of hyperostoses, the increased intercranial pressure, and 
cerebral nerve paralysis are its prominent features. Sternberg admitted 
that there are border-line cases between this and Paget's disease, and now 
it is believed that they are all one disease. 
In hypertrophic pulmonary osteo-arthropathy the long bones are not 
bent, the ends of certain bones only are involved, the fingers are much 
affected, and the cranial bones are not involved. 
Cases of spondylitis deformans with involvement of the spine and 
large joints may resemble early cases of Paget's disease, but the entire 
absence of changes in the long bones is a fundamental difference. 
Acromegaly bears only a superficial resemblance to Paget's disease. 
In both there is enlargement of the head, kyphosis, and thickening of the 
long bones. But in acromegaly the bony lesions are symmetrical. There 
are characteristic changes in the soft tissues which entirely fail in Paget's 
disease, and it is the bones of the face which are enlarged rather than 
those of the cranium. 
Prognosis.-Paget's disease seems to have almost no influence on the 
general health, and these patients are, as a rule, remarkably comfortable. 
If, however, the cases of general hyperostosis are included under this 
heading then the above statement, must be modified, for the prognosis 
will in the latter group depend on.the local pressure (e. g., on the brain) 
exerted by the bony tumors. Cases of Paget's disease possibly are more 
susceptible to acute infections than is the normal man of the same age; 
pulmonary emphysema, chronic bronchitis, etc., would be expected; and 
arteriosclerosis of a rather severe grade is the rule. 
Treatment.-There is as yet no treatment of value. One can give the 
various antineuralgic remedies for the pains, especially the iodides and 
vol. v-62 978 
DISEASES OE THE LOCOMOTOR SYSTEM 
quinine. If it is true that Paget's disease is due to lues one would expect 
improvement under antiluetic treatment, and in some cases this would 
seem to be the case. The failure of other cases to improve under anti- 
luetic treatment is not a strong argument against the luetic origin, for 
late tertiary lesions are little influenced by treatment. 
Tumor-building Osteitis Deformans (v. Recklinghausen). - General 
Hyperostosis of the Skeleton with Cyst-building (Virchow). Osteomalacia 
with Cyst-building (Hirschberg).-This condition seems to be intermediate 
between Paget's disease and multiple primary neoplasm of bone. The 
skeleton is much deformed by multiple hyperostoses, fractures, patho- 
logical curves, definite tumors and cysts. In the medullary canals in 
one case (v. Recklinghausen) were multiple fibrocystomas, in another 
case at exposed points giant-cell sarcomas with cyst formation. In 
Virchow's case there were colossal hyperostoses of the skull and hyper- 
ostoses and curves of some long bones, but osteoporosis of others. In 
Hirschberg's case there were multiple sarcomas, with cysts and multiple 
fractures. 
OSTEOGENESIS IMPERFECTA. 
Terminology.-Osteogenesis imperfecta; Periosteal dyscrasia; Osteo- 
malacia congenita (Jurgens). Rhachitis annularis; Chronic parenchy- 
matous osteitis (Schmidt); Fragilitas ossium congenita (Klebs); 
Periosteal aplasia (Klebs); Osteoporosis congenita and osteosclerosis 
congenita (Paltauf); Osteopsathyrosis; Myeloplastic bone aplasia 
(Kardamatis, 1913). 
These terms have been applied to illustrations of a large group of 
perhaps quite different diseases seen in fetuses, stillborn infants, and 
very seldom in the living child, which have in common one feature, 
imperfect bone formation. Formerly all such cases were diagnosed 
"fetal rickets." Those cases described by some as "annular rickets" 
or "multiple intra-uterine fractures" are considered by others to be the 
fetal form of osteopsathyrosis of adults; those of "osteomalcia con- 
genita" are regarded by Jurgens as the congenital form of osteomalacia 
of adults. In general, newborn infants with osteogenesis imperfecta are, 
in marked contrast to those with achondroplasia, thin, very wrinkled 
and with poor muscular development. Since the body proportions are 
fairly normal the condition may easily be overlooked. 
While in achondroplasia the disease would seem to affect the epiphyseal, 
not the subperiosteal, bone formation, in osteogenesis imperfecta, on the 
other hand, the subperiosteal bone formation is affected, and the epiphy- 
seal little, or none at all. In osteogenesis imperfecta the bones are of 
normal length, but their shafts are either soft or fragile. In some cases 
the long bones present angular deformities, " false joints," "callus masses," 
and other lesions which are explained as "healed or partially healed 
fractures;" in others the bones are so soft and pliable that they can be 
bent easily without crepitation; in others the diaphysis consists of 
alternate segments of cartilage and bone, while in still other cases the 
bones are slender and brittle, some indeed containing only parchment-like 
lamellae of osseous tissue. Each rib may be studded with cartilage OSTEOGENESIS IMPERFECTA 
979 
knots, and the long bones present a succession of thickenings along their 
diaphyses. The pelvis may resemble that of osteomalacia. The skulls 
of these infants are especially interesting. The vault of the cranium is 
in some a membranous pouch in which lie one (in Stilling's case in the 
forehead) or many little islands of bone with jagged stellate margins. 
In other cases the ossification of the cranium is extensive but these bones 
parchment-like. The bones of the base of the skull and of the face are 
normal. Skiagrams show the cortex of the bones to be thin, the spongiosa 
soft and porous, and the presence of cavities and cysts. The cavities 
are full of a gelatinous material. Kardamatis explained the cysts as 
due to either compression of the lymph vessels and bloodvessels of the 
marrow, or to a primary disease of the walls of these vessels, leading 
to widening of the lymph spaces and bloodvessels. 
Etiology.-At first it was thought that these fetuses seldom lived. 
Now we are sure that some cases of osteopsathyrosis in children are 
illustrations of this disease, and Rontgenology shows it to be far more 
common than was supposed. Some cases are one of twins, the other 
normal. Zurhelle1 reported cases of mother and living child both thus 
affected, which cases he claimed prove the inheritance of osteogenesis 
imperfecta and the identity of this condition and osteopsathyrosis. 
Pathology.-Histologically the cartilaginous ossification is normal, 
but the subperiosteal is abnormal. The osteoblasts are few and scattered; 
the osteoclasts are abundant. The result is faulty bone formation and 
the reduction by absorption of the diaphyseal cortex to thin lammellae 
which had been formed. 
Since one meets the term intra-uterine fracture so often, a short dis- 
cussion of this subject may be in place. It sometimes, although rarely, 
happens that one or more bones of an infant are actually broken, usually 
during or recently before birth. These genuine fractures are usually 
single. While genuine fractures of a normal fetus knit rapidly, the so- 
called fractures under discussion must have knit very slowly, since 
the lesions thus interpreted are sometimes false joints, the ends of the 
fragments being atrophied and pointed, or separated by spaces filled 
by cartilage; other cases have fractures of very fragile bones which 
consist of parchment-like lamella? of bony tissue; but in by far the 
majority of cases the lesions described as fractures are enlargements along 
the shaft of the bone, suggesting callus formation, or angular deformities, 
which suggest healed breaks. One may well doubt, however, that many, 
if indeed any, of these lesions are the results of real fractures. Direct 
violence could scarcely produce the multiple "fractures" (even 113 in 
Chaussier's case) which these infants show; these bones thus affected 
are not always more brittle than normal; the callus and angular deformi- 
ties are at positions (e. g., near epiphyses) where a mechanical pull or a 
blow would not be likely to fracture the bone; and, finally, there is a 
suspicious symmetry in the arrangement of these lesions. The cases 
with multiple callus formations have long borne the diagnosis " annular 
rickets," the rings of callus being explained as irregular proliferations of 
1 Deut. med. Wchschr., 1913, xxxix, 1484. 980 
DISEASES OE THE LOCOMOTOR SYSTEM 
the periosteum. The angular deformities are often, and some think 
usually, due to ingrowths of periosteum between diaphysis and epiphysis 
on one side only of the bone, thus inhibiting the growth of the bone on 
this side. In favor of the view that these lesions are developmental 
abnormalities rather than fractures is the partial or total absence of 
lesions of the fibula when the tibia is involved. Some cases of this group 
seem due to lues. 
Osteitis Fibrosa.-As osteitis fibrosa has been described a condition 
characterized by the rarefaction in localized areas of the bone tissue 
and its substitution by fibrous tissue or by a cyst (osteitis fibrosa cystica) 
even the size of a hen's egg. These areas are often mistaken for sar- 
comas. Some have followed trauma. 
OSTEOPSATHYROSIS. 
Osteopsathyrosis (Lobstein, 1833); Fragilitas ossium (Klebs); Lob- 
stein's disease. This rare condition, characterized by abnormal brittle- 
ness of the bones, occurs in infancy, childhood, and adult life. 
Abnormal fragility of the bones occurs in old age (senile osteoporosis), 
in cases with atrophy of disuse, in cases of cachexia (especially that due 
to malignant disease), in various diseases of metabolism as rickets and 
scurvy, in certain nervous diseases, as locomotor ataxia, general paresis 
and anterior poliomyelitis, and in general intoxication, as phosphorus 
poisoning. Among the insane a special form of fragilitas ossium occurs, 
which is due to rarefaction of the bones. The atrophy of the bony 
tissue in the above mentioned groups of cases may be marked. These 
conditions are ascribed to poor nutrition and to the frequent traumas 
which these patients suffer. Localized brittleness of bones may be due 
to the local osseous lesions of lues, osteosarcoma, the metastases of carci- 
noma, multiple myelomas, and to echinococcus cysts. In all the above 
conditions examination will reveal local lesions, gross or microscopic 
changes in the bones, which easily explain their brittleness. 
The term osteopsathyrosis, or Lobstein's disease, etc., is reserved for 
"idiopathic cases," cases "without a demonstrable (as yet) lesion." 
It is true that radiographs of the long bones of these cases would suggest 
that the cortex is rather thin, the shafts slender, and for this reason, 
perhaps, the epiphyses are apparently enlarged, and yet the lesions would 
seem scarcely sufficient to explain the fragility. If osteopsathyrosis is 
osteogenesis imperfecta of viable infants, then the brittleness of the 
bones is due to the inability of the periosteum to develop a sufficiently 
thick cortex; others think that it is due to relatively overactive absorp- 
tion of the cortex. Some think it secondary to changes in the nervous 
system. Lipschutz thinks the lesion lies in the cancellous tissue, since 
in his case the fractures involved the extremities of the bones, and the 
skiagraphs showed that here the trabeculae were abnormally few. Zesos 
suggested that muscular atrophy is a part of the same condition and that 
the disease is related to insufficiency of glands of internal secretion. 
One case is reported to have improved after the transplantation of the 
thyroid. HYPERTROPHIC OSTEO-ARTHROPATHY 
981 
These eases would seem to be rare, since so few are on record. The 
sexes are equally affected. In a rather large proportion the condition 
is inherited and in still more cases it affects several of the same generation. 
The general health of these patients would appear to be excellent. The 
fractures follow very trivial injuries, as a slight blow, muscle pull, a 
fall, and turning over in bed. Simple chewing may fracture the jaw. 
The long bones are the ones most frequently broken. The number of 
fractures which these patients may have during their whole life varies 
from a few to more than one hundred. 
This disease affects especially the period of development. The first 
fracture may occur during birth, during the first bath, or much later, 
even in the fourteenth year and in adult life. In most cases the con- 
dition ceases at thirty years of age, but in one case it continued until the 
seventieth year. In some cases the bones would seem earlier to be soft 
and pliable and later rigid and fragile. 
Symptoms.-Usually the fractures are rather painless and as a rule 
they knit quickly, more rarely slowly The amount of callus thrown 
out varies much. The patients usually are markedly deformed. These 
deformities are due for the most part to the union of the fragments in 
bad positions, and yet some of the deformities develop during a stage 
when the bones are abnormally soft and pliable. This applies especially 
to the pelvis. The tibia is often sabre-shaped, wide, and flattened later- 
ally. In one case there was progressive ankylosis of the joints. 
Prognosis.-Osteopsathyrosis can make a patient wretched for years 
and then in middle life the bones become stronger. In these cases the 
prognosis is one of function rather than life; the deformities resulting 
from union in faulty positions may be a great calamity. 
Treatment.-The only medication suggested which seems to have 
been beneficial is phosphorus and transplantation of the thyroid gland. 
The treatment of the fractures is a surgical problem. 
HYPERTROPHIC PULMONARY OSTEO-ARTHROPATHY. 
Synonyms.-Toxigenic ossifying osteo-periostitis; osteo-arthropathic 
hypertrophiante pneumique; secondary hypertrophic osteo-arthropathy; 
clubbed fingers; akro-elephantiasis ossea et mollis. 
Definition.-The above terms have been applied to conditions char- 
acterized by symmetrical enlargement of the ends of the fingers and toes 
and of the distal ends of certain long bones. These lesions do not con- 
stitute independent diseases, but are secondary to diseases of certain 
organs, especially the lungs, or to a local neuritis. 
Etiology.-Marie considered that this condition was always secondary 
to other diseases, usually of the lungs, because, of the 8 cases he studied, 
4 certainly and 3 possibly had pulmonary trouble. Wynn1 in 100 cases, 
from the literature, found pulmonary trouble in 68; Alexander in 77 of 
103 cases (bronchiectasis in 25, chronic pulmonary tuberculosis in 15, 
empyema in 8, malignant disease of the mediastinum or lung in 8, chronic 
1 Birmingham Med. Review, 1904, Iv, p. 212. 982 
DISEASES OF THE LOCOMOTOR SYSTEM 
bronchitis in 28, pneumonia and pleurisy in 2, and a variety of pul- 
monary diseases in 17. Only two cases, one of which was congenital, did 
not show a definite antecedent disease. 
Alexander, who was very critical in his selection of cases, found that 
osteo-arthropathy occurs usually with pulmonary troubles, especially 
those with a septic focus as bronchiectasis. In other cases the pulmonary 
lesion was chronic pulmonary congestion and chronic passive congestion 
of the whole body. Marie ascribes the changes to toxins produced in a 
focus of infection. This theory may explain the majority of the cases, 
but certainly not those with congenital heart disease and clubbed fingers. 
Some claim that cyanosis causes tissue proliferation, but there are many 
patients with extreme and chronic cyanosis whose fingers are not clubbed. 
Shaw1 claims that his is the only case of hypertrophic osteo-arthropathy 
associated with congenital heart disease without any pulmonary lesion. 
Other diseases claimed as primary are hypertrophic cirrhosis of the 
liver and lues. The neuropathic theory also has many adherents. In 
favor of this are the associated paresthesias, the sensory changes suggest- 
ing syringomyelia, epileptiform attacks, and the marked sweating. 
That clubbing of the fingers may follow a traumatic neuritis is granted, 
as in Mobius' case of neuritis of the ulnar nerve with extreme clubbing 
of the two fingers which that nerve supplies. This patient had tubercu- 
losis also. On the other hand in chronic hypertrophic pulmonary osteo- 
arthropathy the trophic changes in the skin so common in neuritis are 
not mentioned, and as yet no lesion of the nervous system has been 
found. Clubbing of the fingers and swelling of the distal end of the 
forearm and less in the upper arm of the left side has been reported in 
cases of subclavian aneurism, in aneurism of the arch of the aorta, and 
in cases with unreduced dislocation of the shoulder. In these cases 
the clubbing may have been due to pressure on the nerves or to passive 
congestion. Congestion alone, however, could hardly cause this. The 
pressure of aneurism on the nerve trunks probably causes a severe neuritis 
which leads to the hypertrophy. 
That tuberculosis is the chief cause is a theory just now attracting 
attention. Thorburg advocated this in 1893.2 Poncet and his pupils con- 
sider it to be benign tuberculous rheumatism and Alamantine3 proposes 
to rename the disease "subacute tuberculous osteo-arthritis." They, 
it would seem, do not claim that Marie's syndrome is tuberculosis of the 
periosteum or bone, but that it is caused by toxins from a tuberculous 
focus and so they agree with Marie. The specific lesions of tuberculosis 
have never been found in the involved bones. Trauma is said to pre- 
dispose the bone to this condition. Italian writers claim that the germs 
or toxins which cause the bone lesions do so by first causing polyglandular 
disturbances. Others would separate the cases without a discoverable 
primary disease into a separate group with which the congenital cases 
might be grouped. The term akro-elephantiasis ossea et mollis has been 
proposed for cases in which neither the lungs nor the heart are diseased. 
1 Clin. Soc. Trans., 1907, xl, 259. 
3 Rev. de Chir., 1907, xxxv, 992. 
2 Brit. Med. Jour., 1893, i, 1155. HYPERTROPHIC OST EO-ART II RO PA TH V 
983 
Sternberg concludes that, since the etiology is so varied, one must 
suspect that the cases diagnosed as chronic hypertrophic pulmonary 
osteo-arthropathy are not all the same disease. The periosteal changes 
and the clubbing of the fingers may not be related. We do see clubbed 
fingers without periosteal changes in long bones, but not the reverse. 
Race- It is interesting that all but 3 of the 117 cases reviewed were 
of the white races. 
Sex.-Of the 77 carefully selected cases collected by Alexander, 64 
were men and 13 women. Landis states that males are eight times 
more frequently affected than females. 
Age.-While all ages are affected, in the majority of cases the onset 
is in the third and fourth decades. In a few cases the condition seemed 
congenital, even inherited (Marie). 
We know nothing of the conditions immediately determining the 
development of the disease. Bronchiectasis or chronic pulmonary 
tuberculosis may continue for years and then suddenly the hypertrophic 
osteo-arthropathy may appear. 
Pathology.-There are reports of but sixteen complete autopsies. 
These are well reviewed by Alexander.1 The lesions of chronic hyper- 
trophic pulmonary osteo-arthropathy should be carefully separated 
from those of the primary trouble. Its characteristic lesion is a sym- 
metrical deposit of new subperiosteal bone on the shafts of the long bones. 
The bones most frequently affected are the lower ends of the radius and 
ulna, the metacarpals, and the first two rows of phalanges. More rarely 
the lower end of the humerus and the upper ends of the radius and ulna 
are involved. In the forearm the new bone begins abruptly as a thin 
layer, about four or five inches above the wrist-joint, and forms a sheath 
covering the lower ends of the ulna and radius as far as the epiphyseal 
line. The circumference of the shaft is about equally affected. I he 
layer of new bone is generally thickest at the juncture of the shaft with 
the lower extremity of the bone. In Landis' case there was an osteophyte 
growing from the olecranon. The carpal bones are not affected, but the 
shafts of the metacarpals are ensheathed and so appear to be uniformly 
enlarged. The same is true of the first and second rows of the phalanges, 
but the change is less marked than on the metacarpals. The terminal 
phalanges are apparently unaffected. The corresponding bones of the 
lower limb, the lower ends of the tibia and fibula especially, are affected. 
The deposit of new bone on these bones is more diffuse, and may extend 
over the whole shaft, covering also their upper ends. The layer of new 
bone is thicker on the tibia than elsewhere. Here it may be a quarter 
of an inch thick. Sometimes the lower end of the femur and more rarely 
the upper ends of the tibia and fibula also are covered. Ihe tarsal bones 
are not affected. The metatarsal bones show changes similar to those of 
the metacarpals, but the phalanges of the toes are less frequently affected 
than of the fingers. Deposits of new bone have been found on the 
clavicles, iliac crests, and the patellae. The symmetrical arrangement 
of the lesions is remarkable. 
1 St. Bartholomew's Hosp. Rep., 1906, xlii, 41; see also Sternberg, Nothnagel's System, 
1903, Band vii, Part 2. 984 
DISEASES OE THE LOCOMOTOR SYSTEM 
The cortex of the bone may show some sclerosis and thickening, with 
diminution in the size of the medullary cavity. The marrow is said to be 
embryonic in character, with a tendency to fatty degeneration in its 
central part (Wynn). In one case at least the cortex of the shafts was 
softer than normal (Alexander). The periosteum is thick and abnormally 
vascular over the new bone. The main nutrient canals are large, and 
many additional vessels enter the compact layer. The skull is little if 
any affected. Marie reported one case with marked thickening of the 
upper jaw, but no similar case has since been reported. These bone 
changes are due to a chronic inflammation which varies enormously 
in extent and severity. The bone changes are only part, and often a 
small part, of the process. There is much thickening of the connective 
tissue covering the affected bones, especially in the neighborhood of the 
wrists, ankles, and fingers. The clubbing of the fingers is due entirely 
to changes in the soft tissues of the finger tips. These changes are: 
increase of fat, an overgrowth of the papillae (Fowler and Godlee), 
dilatation of the capillary loops in the nail beds (Freytag). The clubbing 
disappeared in the finger preserved by Fowler in alcohol, also in the finger 
which Landis preserved in Kaiserling's fluid. When the x-rays were 
first used the clubbing was supposed to be due to the irregular spicules of 
bone radiating from the terminal phalanx, but these are not more common 
or more prominent in the clubbed than in some normal fingers. 
In many cases there is increase of fluid in the joints, especially the 
wrists, knees, ankles and fingers, due to subacute synovitis. As a rule 
the cartilages show no changes. There is little lipping, little osteophyte 
formation, and little eburnation of the joints. The thyroid gland and the 
pituitary body are normal. 
Symptoms.-The first symptoms may be pain, or clumsiness in fine 
movements, stiffness of the joints, or muscular weakness. In some cases 
the acute stage is rather brief, the condition in a few months passing 
from its acute to its chronic stage. The bone lesions once developed may 
progress no further, although the pulmonary condition which it com- 
plicates may progress without improvement. 
In describing this condition we follow for the most part Marie's original 
description (see also Alexander, Thayer,1 Wynn, and Landis). The 
hands appear enormous, even larger than in acromegaly. Their general 
relative proportions are not, as in acromegaly, fairly normal, but the hands 
are deformed and resemble somewhat an animal's paw. The fingers 
especially are affected, particularly the terminal phalanges, and have the 
shape of a drumstick. Sometimes the greatest swelling is not terminal, 
and the fingers are spindle-shaped. Marie mentions a patient in whom 
the terminal phalanx of the middle finger measured 10 cm. in circumfer- 
ence. The fingers often seem somewhat longer than normal. Hyper- 
extension of the terminal phalanx has been noted. The joints of the 
fingers are sometimes swollen and their movements so limited that the 
hand cannot be completely closed. 
The nails are large, even 2.5 cm. broad, and curved both longitudinally 
1 New York Med. Jour., 1896, Ixiii, 33. HYPERTROPHIC OSTEO-ARTH ROPA THY 
985 
and transversely. They may overlie their beds laterally and reach the 
border of the finger. If the nail was previously long, the end of the 
finger, when viewed in profile, has the shape of a parrot's head. If pre- 
viously short, the nail becomes round and fits on the finger like a watch 
crystal. They often resemble the bowl of a spoon. These nails show 
marked longitudinal striation and are brittle. The root of the nail is 
elevated and almost fluctuating. The root of the nail of a normal finger 
is depressed, rather firmly fixed to the periosteum, and when pressed 
at its root yields very little. The slightest grade of clubbing of the fingers 
is evidenced by the fact that its root can be depressed on pressure. In 
more marked cases the entire dorsum of the nail including its root is 
in a line with the dorsum of the finger. In an extreme case the dorsum 
of the finger is convex from the last knuckle to the tip of the nail, the 
root of the nail forming the summit of the convexity. In these cases 
the root of the nail "floats" when pressed and projects as a ridge when the 
free end of the nail is pressed. This occurs in no other condition (Wynn). 
The nails grow with abnormal rapidity. 
The tips of the fingers are cyanotic, but there is usually a zone of bright 
rose color at the periphery of the nails. As a rule all of the fingers are 
affected, but the thumb, index and middle fingers are often more affected 
than the other two. Sometimes one or two fingers alone are affected 
(possibly those which have received more traumatism). 
The forearm above the wrist is so much enlarged that from elbow to 
wrist it may have a uniform circumference. The ulna and radius at 
the wrist, especially the latter, project prominently. 
Changes similar to those in the hand, but usually less in degree, occur 
in the feet and ankles. The malleoli are quite prominent and the great toe 
especially is affected. Since the lower end of the femur and the whole 
of the tibia and fibula are enlarged, the knee-joint stands out prominently 
and fhe whole leg may have an elephantine appearance. This hyper- 
trophy may affect the other long bones, especially at their extremities, 
the humerus and femur, the clavicle, sternum, ribs, and the iliac 
crests. With improvement in the general condition the swelling may 
diminish. 
The lesions of the long bones are usually painful, both spontaneously 
and on pressure. In some cases the pains are persistent, but more often 
they are severe at certain times, particularly in the evening. They may 
be sharp and stabbing and last from a few minutes to half an hour. These 
pains are increased on motion and on exposure to cold, and relieved by 
rest and warmth. They lessen or disappear when osteosclerosis begins. 
Certain bones which are not swollen may be painful on pressure. 
In some cases the symptoms of arthritis are conspicuous, but the 
joints usually appear more involved than is actually the case. They are 
swollen and painful, but not red, and there is increase in the synovial 
fluid. The joints nearest the affected bones are most involved. The 
wrists and ankles are the ones most affected, less often the elbows, the 
shoulders, and the small joints of the hands and feet. The hips usually 
escape. The temporomaxillary and sternoclavicular joints practically 
never are involved. 986 
DISEASES OF THE NERVOUS SYSTEM 
detritus. Curiously enough, the axis-cylinder is not involved in this 
general swelling. (2) Every cell of the central gray matter, both of the 
brain, the spinal cord, and the spinal ganglia, is similarly affected. 
Histologically there is an unusual swelling of the cell body and a more 
or less complete disintegration of the cell protoplasm. The fibrillae dis- 
appear, so that in many of the cells there are only slight traces left of the 
endocellular network. The fibrils near the periphery of the cell body 
are preserved somewhat longer than are those in the central part of the 
cell mass. The nucleus and nucleolus, if at all preserved, are, as a rule, 
pushed nearer to the periphery of the cell body, and as the disintegration 
Fig. 49 
Ganglion cells of the anterior horn of the cord. Showing the cxcentric nucleus surrounded by the 
remains of the chromatophilic substance and the marked increase in size due to degeneration of the 
cytoplasm. 
of the mass goes on the nucleus and nucleolus also disappear. A pro- 
liferation of the glia, particularly in the cortex, with the development of 
giant glia cells, has been noted. There is no evidence of any inflammatory 
process. Changes in the white fibres are unimportant and unessential. 
It is Schaffer's merit to have laid stress upon the swelling of the dendrites, 
which appears to be so constant as to be almost pathognomonic. The 
deterioration of the cell body and the swelling of these cell processes, 
exclusive of the axone, have been noted in all of Schaffer's and in my own 
recent case. 
Ilolden, as well as Schumway and Mary Buchanan, made careful 
histological examinations of the eyes. All these writers agree that the AMAUROTIC FAMILY IDIOCY 
987 
essential changes are a degeneration of the ganglion cells of the retina and 
of the nerve fibres of the optic nerves and tracts. They also believe that 
the white area in the fundus is the result of the swollen and degenerated 
ganglion cells, which are present in much greater numbers in the macular 
region than elsewhere, and do not consider this white area to be due to 
oedema of the tissue as was first supposed. 
Pathogenesis.-Writers on this disease have been divided into two 
groups. Some, among whom the writer wishes to class himself, have 
maintained that amaurotic family idiocy is, in a sense, a congenital disease, 
even if it is not manifest at the time of birth. The tendency to the 
Fig. 50 
Purkinje cell. Showing the swollen dendrites. 
disease is unquestionably born with the child. In the writer's opinion 
children afflicted with amaurotic family idiocy are possessed of a nervous 
system so inadequate to the demands imposed upon it that its cells, 
after having performed their function for a few weeks or months, undergo 
complete disintegration. In other words, these children have a very 
limited capacity for normal development. Their central nervous system 
is not equal to the functions that they are expected to perform for more 
than the first three or four months of life. 
' Even Schaffer, who was inclined for a time to class amaurotic family 
idiocy among acquired diseases, concedes that the entire nervous system 
of such a child is so delicately organized that it is not able to repair the 
ordinary physiological waste of its cells. The nerve cells are so easily 988 
DISEASES OF THE LOCOMOTOR SYSTEM 
have no observable relation to that of the disease considered primary, 
one must be skeptical as to an immediate relationship between the two 
and suspect that some intermediate condition, as a toxic or traumatic 
neuritis, determines the bone lesions and controls their prognosis. 
Treatment.-It is of prime importance to treat the primary disease, 
and marked improvement in the bony condition has followed successful 
treatment. One can do much to relieve the pains in the bones and 
joints. For this warm applications seem efficacious. Demons and 
Binant have used subcutaneous injection of lung extract with doubtful 
success. 
GENERAL HYPEROSTOSIS. 
Under the term "general hyperostosis" are grouped cases in which 
the disease affects the entire skeleton; "general hyperostosis of the skull," 
those in which the skull only is involved. Some doubt that the lesions 
are ever entirely limited to the skull, but point out that the other bones 
are often so slightly diseased that these lesions would be easily overlooked. 
Many of the reports are descriptions of skulls in museums, the rest of 
the skeletons not preserved; only recently have clinical reports appeared. 
General Hyperostosis of the Skull, Leontiasis Ossea (Virchow), 
Cephalomegalia, Megalocephlia, Ostitis Fibrosa Hyperostotica Cranii 
et Faciei.-These names have been given to a rare condition character- 
ized by diffuse hypertrophy of the bones of the skull. All the bones of the 
face and cranium are affected, but in varying degree. The result is a 
huge skull weighing even 4000 grams, and much deformed. The surface 
of the bones is rough, yet there are no large exostoses. The air sinuses of 
the face may disappear, the orbits are encroached upon, the sutures 
disappear, the foramina, especially those for nerves, are narrowed, 
some of the grooves for the vessels are widened, some narrowed, even 
obliterating the vessels. The thickened bones may be as hard as ivory 
or the cancellous tissue may persist. 
The interesting question is the relation of this condition to general 
hyperostosis and of both to osteitis deformans. Paget considered the 
diseases distinct. Sternberg1 admitted that there are border-line cases. 
Lately the opinion has gained ground that hyperostosis cranii is not a 
disease entity, but a condition common to perhaps several diseases. 
One2 of Sternberg's 12 cases turned out later to be a case of syringomyelia; 
at least one other (Ede's case3), of Paget's disease, and still another of 
lues (Putnam). Prince4 said: "We have no sure ground for differentiating 
hyperostosis cranii from osteitis deformans. Both are probably trophic 
disturbances, perhaps allied, and possibly only different manifestations 
of one and the same disease." Of course, the differences between these 
two diseases cannot be overlooked. The cases described as hyperostosis 
cranii have, for the most part, developed at an earlier age than do the 
majority of cases of Paget's disease. In the former the lesions on the 
skull are much more marked, and the symptoms from them much more 
1 Thesis, Paris, 1997. 
3 Am. Jour. Med. Sc., 1896. 
2 Hale White, British Med. Jour., 1896, p. 1377. 
4 Trans. Assoc. Am. Phys 1902, xvii, 382. GENERAL HYPEROSTOSIS 
989 
pronounced than in typical Paget's disease. But these differences are 
by no means fundamental and may depend on the localization of the 
lesions. According to a later view the condition should be named "ostitis 
fibrosa hyperostotica cranii et faciei'' and regarded as an inflammation 
caused by trauma, bacterial infection or lues. 
Clinical Course and Symptoms.-Of the 21 cases collected by Prince, 
some began in late childhood or puberty, only 10 after thirty years of 
age, and of these only 4 after forty years. The disease begins insidiously 
with headaches and a general increase in the volume of the skull and a 
marked bulging of the forehead. The result is a huge, misshapen head. 
The headaches are sometimes continuous, sometimes paroxysmal, and 
are accompanied by symptoms of compression of the nerves in the fora- 
mina; neuralgia, blindness, deafness, bilateral facial paralysis, and dis- 
turbances in chewing, swallowing, and breathing, with paralyses. The 
circulation of the head changes considerably, owing to stenosis or oblitera- 
tion of the vessels in the foramina. Some subcutaneous veins may be 
very much distended. Stenosis of the canalis caroticus may cause a 
loud roaring in the ears, diminished by pressure on the carotid artery 
(Putnam). Exophthalmos is often present. Insomnia and mental 
apathy develop later; mental disturbances are common; epileptiform 
convulsions and finally paralysis of the extremities "complete the sad 
picture." Death follows paralysis of the muscles involved in mastication 
and swallowing, or, as often happens in cases of chronic intracerebral 
compression, it may come suddenly with convulsions. The duration is 
given as from twenty to thirty years. 
Treatment.-The inflamed areas resulting in hypertrophy should be 
excised early; later cerebral decompression may be necessary. 
Nodular Hyperostosis of the Skull.-This form of leontiasis ossea differs 
from the preceding, or diffuse form, in that the hyperostoses are more 
definitely tumors on a more normal bone. These tumors may be flat 
or mound-like, and are of various sizes and shapes, but are seldom prom- 
inent and warty. The underlying bone is not perfectly normal, but is 
the seat of a diffuse hypertrophy of varying degree. The results are 
hideous deformities. The most common situation for these tumors is 
the cranium. Situated here they may grow inward, exerting pressure 
on the brain. In some cases the tumors at first are limited to the lower 
jaw, but later develop on other bones as well. One form, in which the 
tumors are for a long time at least limited to the jaw bones and are 
symmetrically placed, has been described as an independent disease under 
the name "hyperostosis maxillarum." 
So often is this condition combined with giant growth that Sternberg 
suspects some relation between these two conditions. A somewhat similar 
though much less marked development of hyperostosis is seen in idiots 
and the insane. 
Pathology.-These hyperostoses cannot be sharply differentiated from 
exostoses and osteomas. They cannot always be differentiated from 
sarcomas, even on microscopic examination. Gummas must be excluded. 
Symptoms.-The tumors develop slowly and cause symptoms which 
depend on the organ pressed against. The most important group of symp- 990 
DISEASES OF THE LOCOMOTOR SYSTEM 
toms are those resulting from pressure on the brain, on the eyeball, on 
the respiratory passages, etc. 
Prognosis.-This depends on the situation of the tumors. When their 
pressure does not interfere with the function of any important organ the 
disease may not appreciably influence life. The deformities may be 
hideous, and while the effects of pressure from such slowly-growing tumors 
may be much less marked than from the more rapidly-growing ones, yet 
these patients may suffer severely. 
Treatment.-This must be local, since the cause of the condition is not 
yet known. It is always well to try antiluetic treatment in the hope that 
this may benefit the patient, but, this failing, surgical removal of those 
tumors whose presence is a menace to life or a detriment to important 
functions is indicated. 
Daireoff1 thinks that in addition to the above forms there is another 
group of the systematic osteopathies which he describes as deforming 
hyperostosis. This affects symmetrically the inferior epiphyses of the 
bones of the legs and forearms and the bones of the feet and hands. The 
result is elephantiasis-like extremities. The osseous hypertrophy develops 
slowly, and is preceded by a period of functional trouble. It seems a 
trophic disturbance. 
MICROCEPHALIA. 
The most important features of microcephalia are retardation in the 
development of the cerebral cortex and mental reduction. Marked 
grades of microcephalia are rare. 
The cases may be divided into two groups, those the result of antenatal 
disease, and those due to developmental deficiency. The latter group of 
the "idiopathic" cases are ascribed to "inhibition" of growth of the 
brain due to pathological "feebleness" of the embryonic tissue from which 
the cerebral hemisphere will develop. This is the "atavistic" group or 
the "primary" group of Giacomini. While these cases may be relatively 
numerous, as yet but one case has been carefully studied pathologically 
and every trace of antenatal disease excluded.2 This case was that of a 
woman, aged forty-nine years, whose brain weighed but 370 grams. 
The convolutions were abnormal in their arrangement. The woman had 
also a parenchymatous goitre. He states that this woman's mentality 
was much better than one would expect. 
If we accept as correct the diagnoses of the cases reported as primary, 
the following is their clinical picture: The cranium is small, even but 
twelve inches in circumference (this patient was a child twelve years old, 
Shuttleworth), yet often fairly well-shaped and sutures normal; the 
face always relatively and sometimes absolutely large; the brain small, 
with primitive arrangements of the convolutions. The supposition is 
that the brain does not grow and therefore the skull also does not. There 
are no indications of intracranial pressure and no signs of brain lesions, 
such as motor disturbances, convulsions, etc. The reflexes are usually 
normal. Coordination of all the voluntary movements is perfect, although 
1 Thesis, Paris, 1900. 2 Hilty, Arb. aus. d. Hirnanat. Inst. Zurich, 1907, ii, 205. FACIAL HEMIATROPHY 
991 
the movements sometimes are remarkably quick, being described as ape- 
like.1 The intellectual development varies much. Walking is delayed, 
e. g., to the fourth year. Some of these patients never try to talk, and yet 
they may be able to recognize their friends. 
The microcephalia of the cases of the second group is ascribed to ante- 
natal intracranial disease. What these diseases may be is a disputed 
question. They would seem to resemble in part those producing hydro- 
cephalus, in part those producing porencephalus. The head is small 
and very narrow laterally, hence resembling a tent; the forehead is very 
narrow, pointed above, and receding. The sutures have been described 
as prematurely united, and yet of the 14 skulls studied by Gliih2 in not one 
were all ossified. These cases present motor disturbances, spastic para- 
plegia, anesthesias, and usually exaggerated reflexes. Among these 
cases there may be a few in which premature union of the sutures inhibits 
the growth of the brain. With this idea in view surgeons have attempted 
to relieve the condition by operation, but with very doubtful results. 
FACIAL HEMIATROPHY. 
Synonyms.-Neurotic progressive facial atrophy; Hemiatrophia faciei 
progressiva (Parry, 1825). 
In this disease there is a slow, progressive wasting of the skin first (some 
say of the fat), then of the subcutaneous tissue, later of the bones and last 
and least of the facial muscles. Usually one side alone of the face is 
affected, seldom both. Not over 200 cases are on record. The majority 
of the cases are women. The left side of the face is more often affected 
than is the right. The disease begins, in a majority of cases, during 
childhood and youth, and there is good evidence that in some cases the 
condition has a congenital anlage, i. e., in Harbitz's case the disease on 
the left side of the face was accompanied by acromegaly and congenital 
absence of the left kidney. As immediate causes have been mentioned 
exposure to cold, insignificant traumas, acute infectious diseases and 
infections from the tonsils. Believed formerly by some to be due to 
disease of the local tissues, it is now granted by most to be due chiefly to 
disease of the trigeminus of the affected side, although other nerves in 
some cases are involved, as branches of the facial nerve, the cervical 
sympathetic nerves, and in one case the left radial nerve, causing atrophy 
of the muscles and skin which it supplies. In the only well-reported 
autopsy was found the terminal stage of an interstitial neuritis from 
origin to periphery of all branches of the fifth nerve, especially the 
superior maxillary branch. 
Symptoms.-The onset of the atrophy, usually insidious and without 
subjective symptoms, is sometimes preceded by sensory and motor 
symptoms in the region of the fifth nerve of the side later affected; 
neuralgic pains, hyperesthesias, paresthesias, spasm of the masseter 
muscle and even epileptiform convulsions. The first objective evidence 
1 Jones, Brit. Jour. Child. Dis., 1905, ii, 214. 
2 Deut. med. Wchschr., 1912, xxxviii, 1440. 992 
DISEASES OE TILE LOCOMOTOli SYSTEM 
is thinning of the skin, usually over a wide area but sometimes at one or 
more small patches of leukoderma which later coalesce. The area of 
atrophy increases; the subcutaneous fat gradually disappears, and soon 
the skin seems attached directly to the bone. The bones, especially the 
superior maxillary, are early affected, and the muscles last and least. 
The hair of the scalp, the eyebrows, eyelashes, and the beard over the 
affected area may fall out, or turn white, diffusely or in patches. The 
activity of the sebaceous glands ceases, that of the sweat glands may 
continue almost normally. The skin becomes rough and hard, and 
resembles somewhat that of scleroderma. The patient complains of 
twitching of the skin and a drawn feeling. The skin sensations are little 
affected, the skin reflexes remain normal, although the vasodilator reflex 
on that side may be lost. The eyeball sinks because of the loss of orbital 
fat. The muscles of mastication become weak early but the facial muscles 
are little involved. Their reactions on Faradic and galvanic stimulation 
are prompt. The half of the tongue and soft palate corresponding to the 
facial involvement are sometimes markedly atrophied. The bones of 
the face waste, and so their normal eminences become less prominent. 
The alveolar process especially suffers and the teeth drop out. The 
features are gradually drawn to the affected side. The result is that the 
affected side of the face becomes much smaller than the other, and the 
two sides seem like the faces of different persons. The lesion stops 
abruptly at the mid-line. The course is slow and progressive until the 
atrophy reaches a certain grade, after which time the condition may for 
a long time remain unchanged. Spontaneous improvement may occur. 
Diagnosis.-In this, one must exclude scleroderma, the atrophy follow- 
ing nuclear lesions and paralysis of the cervical sympathetic nerve, 
that following anterior poliomyelitis, and the facial atrophy seen in hemi- 
plegia. The face is asymmetrical in congenital wry-necks. The normal 
side looks atrophied in acquired facial hemi-hypertrophy. 
Prognosis.-This condition does not seem to influence appreciably 
the general health. When once it begins there is nothing which seems to 
stay its course, and the periods of improvement cannot be attributed, 
so far as we know, to any therapeutic measure. 
Treatment.-There is no treatment which affects the course but the 
appearance has been much improved by paraffin injections. INPEX TO VOLUME V. 
A 
Abdominal reflexes, anomalies of, 78 
Abducens nerves, anatomy of, 483 
Abscess of brain, 378 
of spinal cord, 254 
Abscesses, metastatic, diagnosis of, from 
primary suppurative myositis, 874 
Achilles tendon reflex, 77 
in tabes dorsalis, 837 
Achondroplasia, 955 
of adults, 959 
in art, 960 
bones in, 956 
of children, 959 
diagnosis of, 967 
from infantilism, 967 
epiphyseal cartilages in, 958 
etiology of, 961 
gigantism and, 961 
hands in, 962 
heredity and, 961 
macrocephaly in, 963 
mentality in, 963 
nature of, 965 
pathology of, 955 
periosteal dysplasic, 969 
prognosis of, 968 
ribs in, 957 
rickets and, 966 
skull in, 956 
of stillborn, 955 
symptoms of, 962 
syphilis and, 967 
thyroid and, 965 
treatment of, 968 
varieties of, 958 
vertebrae in, 957 
Achromates, 25 
Achromatopsia, 271 
Acromegaly, diagnosis of, from Paget's 
disease, 977 
Acroparesthesia, 32 
Actinomycoma of brain, 321 
Acts, anomalies in duration of, 66 
in intensity of, 66 
Adductor laryngeal paralysis, 521 
Ade^olipomatosis, symmetrical(818\^ 
Ad umokokigesis? 72 
in bramfumor, 339 / 
Adiposis cerebralis, 81$/ 
dolorosa^ 17 
ductless glands in, 818 
etiology of, 817 
pain in, 819, 820 
vol. v-63 
Adiposis dolorosa, pathology of, 818 
prognosis of, 821 
symptoms of, 819 
treatment of, 821 
tuberosa simplex, 818 
Affective states, anomalies of, 59 
Agenesis of cerebellum, 287 
Agifation, affective, 65 
hallucinatory, 65 
motor, 63 
Agnosia, 269 
Agraphia, 75 
in aphasia, 297, 298 
in migraine, 715 
Akinesia algera, 816 
Akinetic motor phenomena in hysteria, 678 
Akro-elephantiasis ossea, 982 
Albuminuria in infantile convulsions, 795 
in polyneuritis, 590 
Albuminuric retinitis, 475 
Alcoholic neuritis, 579 
diagnosis of, from tabes dorsalis, 
845 
pseudo-paresis, diagnosis of, from 
paresis, 860 
Alcoholism, diagnosis of, from apoplexy, 
442 
encephalitis and, 490 
epilepsy and, 593, 596 
syphilitic myelitis and, 238 
Alexia, 54 
'1 mUfSin tumor/ 333 
Allochiria, 268 
in tabes dorsalis, 838 
Allopsyche, 42 
Alveolar sarcoma of brain, 314 
Amaurosis, 34, 480 
Amaurotic family idiocy, 863 
Amblyopia, 34, 480 
Amimia in aphasia, 296 
Amnesia, 53, 270 
anterograde, 53 
circumscribed, 53 
retarded, 53 
retrograde, 53 
retrospective, 53 
Amnesic aphasia, 54, 74, 298 
Amyotonia congenita, 893 / 
diagnosis of, 894 k 
electrical reactions in, 894 
etiology of, 893 
muscles in, 894 
pathology of, 893 
symptoms of, 894 
treatment of, 894 
993 994 
INDEX TO VOLUME V 
Amyotrophic lateral sclerosis, 89. See 
Sclerosis, amyotrophic lateral. 
syringomyelia, 220 
Anacusis- 33 
^^-Alialgesia, 30 
dolorosa, 30 
in hysteria, 682 
in syringomyelia, 223 
Anarthria, 74 
Anemia of brain, 392 
in hysteria, 688 
in multiple neuritis, 585 
neuralgia and, 726 
retinitis in, 475 
of spinal cord, 255 
subacute combined sclerosis and, 
128 
Anesthesia, 28 
in brain tumor, 337 
in compression of spinal cord, 191 
dissociative, in syringomyelia, 223 
in hysteria, 651, 680, 681 
of larynx, 522 
in migraine, 713, 714 
in neuralgia, 728 
in occupation neuroses, 747 
in tabes dorsalis, 838 
tactile, in syringomyelia, 223 
in traumatic neuroses, 651 
Aneurism of cerebral arteries, 398 
diagnosis of, from brain tumor, 344 
Anger, 61 
Angioma of brain, 317 
Annular rickets, 955 
Anomalies of conduct, 63 
in duration of ideas, 52 
in intensity of ideas, 52 
of reflexes, 76 
of sense of pain, 29 
of will, 63 
Anomia, 76 
Anopsia, 34 
Anosmia, 33, 471 
Anterior crural nerve, paralysis of, 557 
Anuria in hysteria, 687 
Apathy, pathological, 61 
Aphasia, 292 
in abscess of brain, 381 
amnesic, 54, 298 
in apoplexy, 457 
of apperception, 292 
in arteriosclerosis of brain, 397 
in brain tumor, 331 
Broca's, 295 
classification of, 294 
compound, 293 
diagnosis of, 303 
extrinsic, 301 
intrinsic, 301 
jargon, 297 
Marie's theories on, 300 
mental defect of, 302 
motor, 75, 292, 295 
cortical, 295 
subcortical, 295 
symptoms of, 296 
optic, 54, 299 
Aphasia in pachymeningitis interna, 165 
sensory, 75, 292, 296 
agraphia in, 297 
apraxia and, 299 
logorrhoea in, 297 
mind blindness in, 299 
subcortical, 299 
symptoms of, 299 
tone deafness in, 297 
Wernicke's, 299 
word blindness in, 298 
deafness in, 297 
subcortical, 75 
summary of, 299 
total, 75 
true, 292 
visual, 298 
Aphemia, 75, 300 
Aplasia, bone, myeloplastic, 978 
Apoplexy, 405 
age and, 407 
aphasia in, 457 
arteriosclerosis and, 396, 427 
aura in, 433 
duration of, 435 
bleeding in, 453 
blood-pressure in, 435 
brain injuries in, 438 
' tumor and, 419 
cerebral aneurism and, 399 
diplegia and, 419 
hemorrhages and, 410, 411, 414, 
415 
palsies, congenital and, 410 
softening in, 431 
Cheyne-Stokes' breathing in, 436 
childbirth and, 423 
chronic nephritis and, 427 
consciousness in, 432, 433 
in decade I, 409 
II, 421 
III, 423 
IV, 425 
V, 426 
VI, 427 
VII, 428 
VIII, 429 
IX, 429 
diabetes mellitus and, 427 
diagnosis of, 441 
coma in, 450 
convulsions in, 450 
from alcoholic coma, 442 
from brain tumor, 343 
from diabetic coma, 442 
from encephalitis, 375 
from epilepsy, 443 
from hysteria, 443, 446 
from malaria, 444 
from opium poisoning, 442 
from sunstroke, 444 
from trauma, 444 
from uremia, 441 
occupation and, 449 
of vascular lesion, 447 
diphtheria and, 417 
embolism and, 418, 421, 424, 425, 427 INDEX TO VOLUME V 
995 
Apoplexy, embolism and, diagnosis of, 448 
epilepsy and, 421 
etiology of, 406 
exciting causes of, 430 
eyes in, 439 
eye-grounds in, 437 
fever in, 437 
hemiplegia and, 412, 457 
congenital and, 409 
etiology of, 413 
hemorrhage and, 422, 424, 427, 428, 
431 
diagnosis of, 448, 450 
hysteria and, 419 
Little's disease and, 419 
measles and, 417 
occurrence of, 406 
onset of, 431 
paralysis in, 438, 440 
paralytic conjugate deviation in, 439 
porencephaly and, 409 
pregnancy and, 423, 425 
prognosis of, 451 
pulse in, 835 
pupils in, 439 
race and, 407 
reflexes in, 439 
respiration in, 436 
rupture of aneurism and, 429 
scarlet fever and, 417 
sequels of, 456 
sex and, 407 
spastic conjugate deviation in, 438 
symptoms of, 429 
syphilis and, 423, 425, 426, 427 
hereditary and, 418 
thrombosis and, 423, 425 
diagnosis of, 448 
toxemia and, 425 
trauma and, 424 
treatment of, 452 
prophylactic, 452 
surgical, 454 
typhoid fever and, 417, 425 
whooping cough and, 417 
Apraxia, 66, 75, 269 
aphasia and, 299 
in diseases of corpus callosum, 276 
Arachnoidal cysts of brain, 318 
Argyll-Robertson pupil, 487 
in general paresis, 856 
in tabes dorsalis, 836 
Arhythmic movements in hysteria, 677 
Arm, lower, paralysis of, 552 
upper, paralysis of, 551 
Arteries, carotid, anatomy of, 388 
cerebral, aneurism of, 398 
embolism of, 400 
obliteration of, 400 
rupture of, 400 
thrombosis of, 400 
vertebral, anatomy of, 387 
Arteriosclerosis, apoplexy and, 427 
of brain, 393 
cerebral aneurism and, 399 
thrombosis and, 400 
neuralgia and, 727 
Arteriosclerosis in Paget's disease, 973 
Arteriosclerotic dementia, diagnosis of, 
from paresis, 861 
Artery, basilar, anatomy of, 387 
Arthralgia in hysteria, 683 
Arthritis deformans, 895 
age and, 899 
atrophic, 920 
bones in, 907 
diagnosis of, 932 
prognosis of, 934 
symptoms of, 920 
treatment of, 942 
bones in, 904, 906 
cartilage in, 904, 906 
classification of, 898 
deformity in, prevention of, 941 
treatment of, 942 
diagnosis of, 930 
from gonorrhoeal arthritis, 
931 
from gout, 931 
from osteo-arthropathy, 987 
from rheumatic fever, 930 
diet in, 938 
effusion in, 904 
endocarditis in, 914 
etiology of, 899 
gout and, 930 
heredity and, 901 
hygiene in, 938 
hypertrophic, 920 
bones in, 907 
diagnosis of, 932 
Heberden's nodes in, 921 
treatment of, 943 
hip-joints in, 922 
knee-joint in, 923 
metacarpophalangeal joints 
in, 922 
pain in, 921, 922 
prognosis of, 935 
sacro-iliac joint in, 923 
symptoms of, 920 
treatment of, 942 
infections and, 900, 902 
influenza and, 901 
intestinal infection and, 903 
joints in, treatment of, 939 
massage in, 938, 940 
menopause and, 902 
metabolism and, 903, 907 
monarticular, 919, 934 
diagnosis of, 932 
muscles in, 907 
nature of, 898 
nervous system in, 903, 907 
pathology of, 904 
peri-articular, 908 
acute, 909 
in advanced life, 918 
arthritis in, 910 
atrophy in, 914 
blood in, 916 
character of attack in, , 908 
in children, 917 
chronic, 909 996 
INDEX TO VOLUME V 
Arthritis deformans, peri-articular, circu- 
latory system in, 914 
counterirritation in, 941 
distribution of lesions in, 913 
exercises in, 940 
fever in, 909, 914 
glandular enlargement in, 
909, 915 
hip-joint in, 913 
leukocytosis in, 916 
oedema in, 917 
onset of, 908 
pain in, 909, 910 
pericarditis in, 914 
pleurisy in, 915 
prognosis of, 933 
pulse in, 909, 914 
reflexes in, 917 
respiratory tract in, 915 
should er-joint in, 911 
skin in, 916 
spleen in, 909, 915 
subcutaneous nodules in, 915 
symmetry in, 913 
symptoms of, 908 
treatment of, 940 
urine in, 916 
pregnancy and, 902 
prognosis of, 933 
pyorrhoea alveolaris and, 901 
race and, 900 
Raynaud's disease and, 929 
scleroderma and, 929 
sex and, 899 
of spinal joints, 910 
spondylitis in, 923 
Still's disease and, 917, 931 
symptoms of, 908 
synovial membrane in, 904, 905 
tonsillitis and, 901 
trauma and, 901 
treatment of, 936 
vaccines in, 939 
villous arthritis in, 929 
in hypertrophic osteo-arthropathy, 
985 
infectious, 896 
rheumatoid, 897, 908 
villous, 929 
treatment of, 942 
Arthropathy in syringomyelia, 225 
in tabes dorsalis, 842 
Ascending multiple neuritis, 576 
Association, anomalies of, 46 
tests, 48 
Astasia-abasia, 815 
affective, 816 
diagnosis of, 816 
from akinesia algesia, 816 
from myasthenia gravis, 816 
emotional, 816 
etiology of, 815 
fatigue in, 816 
hypochondriacal, 816 
hysteria and, 686, 816 
prognosis of, §17 
psychasthenic, 816 
Astasia-abasia, symptoms of, 815 
treatment of, 817 
Astereognosis, 30, 268 
in lesion of optic thalamus, 281 
in tabes dorsalis, 838 
Asthenic paralysis, 798 
Asthenopia, 36 
Astigmatism, 36 
Asyllabia, 298 
Asymbolia, 269 
optic, 54 
Asynergy in brain abscess, 381 
tumor, 339 
in lesion of cerebellum, 286 
Ataxia, 71 
in abscess of brain, 381, 384 
in brain tumor, 336, 339 
cerebellar, 72 
Marie's hereditary, 130, 136 
in disseminated sclerosis, 147, 154 
Friedreich's, 130 
ataxia in, 135 
diagnosis of, 138 
from disseminated sclerosis 
138 
from Huntington's chorea 
139 
from tabes dorsalis, 138 
etiology of, 131 
gait in, 136 
nystagmus in, 135 
pain in, 136 
pathology of, 131 
prognosis of, 139 
symptoms of, 133 
treatment of, 139 
vasomotor paresis in, 136 
in hysteria, 652 
in lesion of cerebellum, 287 
of mid-brain, 284 
of optic thalamus, 280 
in multiple neuritis, 583 
in tabes dorsalis, 839 
Athetoid movements, 70 
Athetosis in cerebral diplegia, 420 
in lesion of optic thalamus, 280 
Athymia, 61 
Atony of muscles, 68 
Atrophic arthritis deformans, 920 
Atrophy of cerebellum, 287 
facial, in migraine, 719 
muscular, 68 
X in arthritis deformans, 914 
in diseases of peripheral nerves 
534 
progressive,Charcot-Marie-Tootl 
type, 109 
diagnosis of, from syringo 
myelia, 226 
neural, 109 
contractures in, 110 
diagnosis of, 111 
pathology of, 109 
reflexes in, 110 
symptoms of, 109 
spinal, 80 
age and, 81 INDEX TO VOLUME V 
997 
Atrophy, muscular, progressive spinal, 
atrophy in, 84 
of childhood, 87 
atrophy in, 88 
course of, 88 
diagnosis of, 88 
familial, 87 
hereditary, 87 
paresis in, 88 
pathology of, 88 
symptoms of, 88 
tremors in, 88 
definition of, 80 
diagnosis of, 86 
from progressive 
neural muscular, 
111 
electrical reactions in, 
85 
etiology of, 81 
heredity and, 81 
paralysis in, 84 
pathology of, 82 
pregnancy and, 81 
prognosis of, 86 
reflexes in, 85 
symptoms of, 83 
treatment of, 87 
tremors in, 85 
weakness in, 84 
type, Duchenne-Aran, 80 
Werdnig-Hoffmann, 87 
in occupation neuroses, 744 
olivopontocerebellar, 287 
optic, 37, 478 
in tabes dorsalis, 841 
in osteo-arthropathy, 986 
in progressive muscular dystrophy, 
119 
of tongue, 531 
Attention, anomalies of, 45 
Attonnity, 65 
Auditory nerve, diseases of, 506 
vertigo, 512 
Aura in epilepsy, 600 
Aural vertigo, 513 
Automatism, 44 
Autopsyche, 43 
B 
Babinski's hypothesis of hysteria, 658 
phenomenon, 78 
sign in apoplexy, 440 
in disseminated sclerosis, 149 
Bacteria, multiple neuritis and, 575, 576 
myelitis and, 229 
Bacteriological examination of cerebro- 
spinal fluid, 177 
Bacteriology of chorea, 778 
of encephalitis, 362 
of infective myelitis, 231 
of Landry's paralysis, 248 
Basal ganglia, tumors of, 334 
Basilar artery, anatomy of, 387 
Bathyanesthesia, 30 
Bathyesthesia, 17 
Benedikt's syndrome, 369 
Biological hypothesis of hysteria, 665 
Birth palsies, brachial, 552 
trauma, epilepsy and, 594 
Bladder in hemiplegia, 467 
in syphilitic myelitis, 240 
in tabes dorsalis, 840 
Bleeding in apoplexy, 453 
in hysteria, 687 
Blind spot, 34 
Blindness, 34 
in amaurotic family idiocy, 869 
cortical, 54 
day, 480 
in hysteria, 684 
mind, 53, 271 
night, 480 
psychic, 299 
word, 54 
Blood in arthritis deformans, 916 
in hysteria, 688 
in osteomalacia, 952 
Bloodvessels of brain, 387 
in syphilitic myelitis, 238 
Bones in achondroplasia, 956 
in arthritis deformans, 904, 906, 907 
in facial hemiatrophy, 992 
in osteogenesis imperfecta, 979 
in osteomalacia, 949 
in Paget's disease, 971 
in progressive myositis ossificans, 883 
in syringomyelia, 225 
Brachial neuralgia, 737 
neuritis, 553 
plexus, paralysis of, 550 
prognosis of, 553 
Bradylalia, 74 
Brain, abscess of, 378 
aphasia in, 381 
asynergy in, 381 
ataxia in, 381, 384 
bronchiectasis and, 379 
convulsions in, 380, 381 
diagnosis of, from acute menin- 
gitis, 172, 386 
from sinus thrombosis, 386 
from tumor, 344, 386 
empyema and, 379 
etiology of, 378 
eye-grounds in, 380 
fever in, 380 
headache in, 379 
hemianopsia in, 381, 382, 384 
hemiataxia in, 381, 384 
hemiparesis in, 384 
idiopathic, 385 
infected wounds and, 379 
metastatic, 384 
nystagmus in, 384 
otitic, 383 
pain in, 381, 384 
paralysis in, 381 
paraphasia in, 381 
pathology of, 378 
prognosis of, 386 
pulse in, 380 
respiration in, 380 998 
INDEX TO VOLUME V 
Brain, abscess of, rhinogenic, 385 
sensation in, 381 
sinus thrombosis and, 379 
symptoms of, 379 
cerebellar, 381 
focal, 380 
motor, 381 
frontal, 381 
occipital, 382 
temporal, 381, 383 
temporal lobe, 383 
traumatic, 382 
acute, 382 
diagnosis of, 382 
latent, 382 
treatment of, 386 
vertigo in, 379, 384 
vomiting in, 379 
word blindness in, 382 
actinomycoma of, 321 
acute red softening of, 359 
in amaurotic family idiocy, 864 
anemia of, 392 
Cheyne-Stokes respiration in, 392 
convulsions in, 392 
headache in, 393 
symptoms of, 392 
angioma of, 317 
arteries of, anastomosis of, 387 
aneurism of, 398 
apoplexy and, 399 
arteriosclerosis and, 399 
choked disk in, 399 
dizziness in, 399 
headache in, 399 
syphilis and, 399 
vomiting in, 399 
embolism of, 400 
age and, 401 
etiology of, 401 
pathology of, 403 
obliteration of, 400 
rupture of, 400 
thrombosis of, 400 
age and, 400 
arteriosclerosis and, 400 
chlorosis and, 400 
etiology of, 400 
pathology of, 403 
syphilis and, 400, 401 
typhoid fever and, 400 
arteriosclerosis of, 393 
age and, 394 
aphasia in, 397 
apoplexy in, 396 
convulsions in, 397 
diagnosis of, 398 
from paresis, 398 
etiology of, 393 
headache in, 396 
neurasthenia in, 395 
pathology of, 394 
psychoses in, 397 
symptoms of, 394 
mental, 397 
syphilis and, 393 
treatment of, 398 
Brain, arteriosclerosis of, vertigo in, 396 
bloodvessels of, 387 
anatomy of, 387 
diseases of, 387 
physiology of, 389 
carcinoma of, 315 
centre of hearing in, 271 
of smell in, 271 
centrum semiovale, lesion of, 275 
cerebellum, lesion of, 284 
chloroma of, 315 
cholesteatoma of, 316 
chordoma of, 317 
convolutions of, central, lesions of, 274 
paralysis in, 274 
spasm in, 275 
corpora striata, lesion of, 277 
corpus callosum, lesion of, 276 
cortex of, motor area of, 265, 273 
diseases of, focal symp- 
toms of, 266 
sensory area of, 265, 273 
diseases of, focal symp- 
toms of, 267 
localization of, 273 
crura cerebri, lesion of, 281 
cysticercus cellulose of, 318 
cystoglioma of, 313 
cystosarcoma of, 315 
cysts of, 318 
arachnoidal, 318 
congenital, 318 
echinococcus, 319 
epilepsy and, 599 
hydatid, 319 
parasitic, 318 
simple, 318 
traumatic, diagnosis of, from 
tumor, 346 
disease of, diagnosis of, from hysteria, 
692 
topical diagnosis of, 265 
enchondroma of, 317 
endothelioma of, 313 
diagnosis of, from neurofibroma, 
314 
dural, 313 
epithelioma of, 315 
fibroma of, 317 
acoustic, 314 
glioma of, 311 
diagnosis of, 312 
diffuse, 312 
ependymal, 311 
granuloma of, infectious, 319 
gumma of, 320 
hemorrhage in, 401 
hyperemia of, 392 
inflammation of, 359 
injuries of, in apoplexy, 438 
internal capsule, lesion of, 276 
lenticular nucleus, lesion of, 278 
zone, lesion of, 278 
lipoma of, 317 
myeloma of, 315 
myxosarcoma of, 315 
neuroblastoma of, 317 INDEX TO VOLUME V 
999 
Brain, neurocystoma of, 317 
neurofibroma of, diagnosis of, from 
endothelioma, 314 
neuroglioma of, 311 
neuroma of, 317 
optic thalamus, lesion of, 279 
osteosarcoma of, 314 
papilloma of, 315 
pons, lesion of, 289 
pontomedullocerebellar space, lesion 
of, 289 
psammoma of, 318 
psammosarcoma of, 315 
quadrigeminal bodies, lesion of, 282 
sarcoma of, 313, 314 
alveolar, 314 
diffuse, 315 
perforating, 314 
sclerosis of, 140 
stem, tumors of, 335 
syphilis of, 823. See Syphilis, cere- 
brospinal. 
syphiloma of, 320, 321 
tegmentum, lesion of, 281 
teratoma of, 316 
tubercle of, solitary, 319 
tuberculoma of, 319 
tuberculous meningo-encephalitis of, 
319 
tumor of, 308 
adiadokokinesis in, 339 
age and,309 
alexia in, 333 
anesthesia in, 337 
aphasia in, 331 
asynergia in, 339 
ataxia in, 336, 339 
in basal ganglia, 334 
in brain stem, 335 
cachexia hypophyseopriva in, 341 
in cavum Meckelii, 340 
in centrum semiovale, 334 
in cerebellum, 338 
cerebrospinal fluid in, 323 
choked disk in, 326, 338 
labyrinth in, 328 
convulsions in, 329, 333 
in corpora quadrigemina, 336 
in corpus callosum, 335 
course of, 346 
in cranial base, 340 
in crura, 335 
decompression in, 349 
dermoid, 316 
diagnosis of, 330, 342 
from abscess, 344, 386 
from aneurism, 344 
from apoplexy, 343 
from blastomycotic menin- 
gitis, 345 
from cerebral thrombosis, 
343 
from disseminated sclerosis, 
156, 343 
from encephalitis, 374 
from ependymitis, 345 
from general paresis, 343 
Brain, tumor of, diagnosis of, from hema- 
toma of dura mater, 345 
from hydrocephalus, 345 
from hysteria, 342 
from lead encephalopathy, 
346 
from nephritis, 344 
from pseudo-tumor, 346 
from serous meningitis, 345 
from tabes dorsalis, 845 
from traumatic cysts, 346 
from vascular lesions, 343 
lumbar puncture in, 330 
regional, 330 
dislocation from pressure in, 323 
dizziness in, 329, 338 
drowsiness in, 329 
dyschromatopsia in, 329 
dysesthesia in, 339 
epilepsy in, 331, 333, 599 
etiology of, 309 
in fourth ventricle, 338 
in frontal lobe, 331 
glycosuria in, 338, 342 
headache in, 324, 338 
hemiachromatopsia in, 334 
hemianopsia in, 332, 334 
hemiplegia and, 419 
hydrocephalus in, 338 
in hypophysis cerebri, 341 
hypopituitarism in, 341 
incidence of, 308 
in medulla oblongata, 337 
Menieres syndrome in, 337 
migraine and, 720 
nystagmus in, 336, 339, 340 
in occipital lobe, 334 
oedema in, 323 
pain in, 338 
in paracentral convolutions, 332 
paralysis in, 337 
in parietal lobe, 333 
pathological physiology of, 321 
pathology of, 310 
in pineal gland, 336 
polydipsia in, 338 
polyuria in, 337, 338, 342 
in pons, 336 
prognosis of, 346 
psychic disturbances in, 329, 
342 
salivation in, 332, 337 
sex and, 309 
symptoms of, 321 
focal, 329 
general, 322 
in tegmentum, 336 
in temporal lobe, 331 
thalamic syndrome in, 334 
trauma and, 309 
treatment of, 347 
tremors in, 340 
vertigo in, 329, 338 
vomiting in, 326, 338 
Broca's aphasia, 75, 295 
Bromides in epilepsy, 620 
I Bronchiectasis, abscess of brain and, 379 1000 
INDEX TO VOLUME V 
Bronchiectasis, hypertrophic osteo-arthro- 
pathy and, 982 
Brown-Sequard paralysis, 185, 204 
Bulbar changes in amyotrophic lateral 
sclerosis, 92, 100 
muscles in myasthenia gravis, 802 
myelitis, acute, 368 
palsy, acute inflammatory, 368 
progressive, 113 
paralysis, ascending, 798 
C 
Cachexia hypophyseopriva in brain tu- 
mor, 341 
multiple neuritis and, 576 
Calcium, tetany and, 751 
Capsule, internal, lesion of, 276 
conjugate deviation of eyes 
in, 277 
hemianesthesia in, 276 
hemianopsia in, 276 
hemicontracture in, 277 
hemiplegia in, 276 
hemithetosis in, 277 
paralysis in, 276 
Carcinoma of brain, 315 
of spinal cord, 196 
Carcinomatosis, diagnosis of, from cerebro- 
spinal syphilis, 827 
Cardiac crises in tabes dorsalis, 842 
epilepsy, 597 
nerves, paralysis of, 522 
Cardialgia in hysteria, 683 
Caries, sicca, 189 
spinal, 188. See Spinal caries. 
Carotid arteries, anatomy of, 388 
Cartilage in arthritis deformans, 904, 906 
Catatonic rigidity, 65 
Cauda equina, diseases of, diagnosis of, 186 
from paralysis of lower 
limbs, 561 
from sciatica, 565 
Causalgia in diseases of peripheral nerves, 
539 
Cavum Meckelii, tumors of, 340 
Centrum semiovale, lesion of, 275 
epileptoid attacks in, 275 
speech in, 275 
vision in, 275 
tumor in, 276, 334 
Cephalocele, hydrocephalus and, 354 
Cephalomegalia, 988 
Cerebellar artery, posterior inferior, occlu- 
sion of, 288 
ataxia in, 288 
paralysis in, 288 
sympathetic in, 288 
vertigo in, 288 
ataxia, 72 
gait in, 74 
Marie's hereditary, 130, 136 
deficit, syndrome of, 287 
localization, 286 
symptoms in migraine, 716 
syndrome, 286 
Cerebellum, agenesis of, 287 
atrophy of, 287 
functions of, 284 
lesion of, 284 
asynergia in, 286 
ataxia in, 287 
gait in, 286 
hypotonia in, 287 
paralysis in, 286 
reflexes in, 286 
symptoms of, 285 
sclerosis of, 287 
tumors of, 338 
Cerebral arteries, aneurism of, 398 
embolism of, 400 
obliteration of, 400 
rupture of, 400 
thrombosis of, 400 
arteriosclerosis, 393. See Brain, 
arteriosclerosis of. 
bloodvessels, diseases of, 387 
diplegia, 419 
disseminated sclerosis, 154 
hemiplegia, congenital, 409 
infantile, 412 
hemisphere, ganglia of, 278 
hemorrhage, 401, 410, 411, 414, 415 
age and, 403 
diagnosis of, from encephalitis, 
375 
etiology of, 401 
pathology of, 405 
localization hypothesis of hysteria, 
655 
nerves, diseases of, 471 
palsies, congenital, 410 
softening in apoplexy, 431 
pathology of, 403 
thrombosis, diagnosis of, from brain 
tumor, 343 
toxemia, diagnosis of, from encepha- 
litis, 375 
Cerebrospinal disseminated sclerosis, 154 
fever, hydrocephalus and, 357 
fluid, 351 
circulation of, 352 
examination of, 174 
bacteriological, 177 
chemical, 176 
cytological, 176 
lymphocytosis of, 177 
pressure of, 175 
in syphilis, 828 
meningitis, acute, 167 
syphilis, 823 
diagnosis of, from disseminated 
sclerosis, 156 
tuberculosis, diagnosis of, from 
syphilis, 827 
Cervical plexus, diseases of, 542 
paralysis of, 543 
ribs, 554 
cyanosis in, 556 
diagnosis of, 556 
gangrene in, 556 
pain in, 555 
pulse in, 556 INDEX TO VOLUME V 
1001 
Cervical ribs, symptoms of, 555 
thrombosis in, 556 
treatment of, 556 
Cervico-occipital neuralgia, 736 
Charcot-Marie-Tooth type of progressive 
muscular atrophy, 109 
Cheirokinesthetic agraphia, 75 
Cheyne-Stokes respiration in acute menin- 
gitis, 170 
in apoplexy, 436 
Chloroma of brain, 315 
Chlorosis, cerebral thrombosis and, 400 
Choked disk, 36 
in brain tumor, 326, 338 
in cerebral aneurism, 399 
mechanism of, 476 
stages of, 327 
Cholesteatoma of brain, 316 
Chondrodystrophia fetalis hypoplastica, 
958 
malacica, 959 
Chordoma of brain, 317 
Chorea, acute, 774 
age and, 774 
bacteriology of, 778 
climate and, 774 
complications of, 781 
course of, 781 
definition of, 774 
diagnosis of, from hysteria, 693 
emotions and, 774 
endocarditis and, 776, 781 
etiology of, 774 
reflex, 777 
fever in, 779 
heredity and, 775 
herpes zoster in, 781 
infections and, 777 
malignant, 779 
mild, 778 
paralytic phenomena in, 780 
pathogenesis of, 775 
pathology of, 778 
pregnancy and, 777, 779 
prognosis of, 781 
psychic manifestations in, 780 
race and, 774 
reflexes in, 781 
rheumatic fever and, 775 
severe, 779 
sex and, 774 
symptoms of, 778 
synonyms of, 774 
syphilis and, 775, 777 
treatment of, 782 
urine in, 781 
chronic hereditary, 787 
degenerative, 787 
electric, 787 
habit, 784 
definition of, 784 
diagnosis of, 786 
etiology of, 785 
symptoms of, 785 
treatment of, 786 
Huntington's, 787 
diagnosis of, 789 
Chorea, Huntington's, etiology of, 787 
heredity and, 788 
pathology of, 788 
prognosis of, 790 
symptoms of, 788 
psychic, 789 
treatment of, 790 
insaniens, 779 
minor, 774 
mollis, 780 
posthemiplegic, 466 
progressive, 787 
Sydenham's, 774 
Choreiform movements, 70 
Choroidoretinitis, syphilitic, 475 
Chvostek's phenomenon, 71 
sign in tetany, 758 
Circulatory disturbances, neurasthenia 
and, 631 
system in arthritis deformans, 914 
Circumflex nerve, paralysis of, 546 
Circumspection, 43 
Clubbed fingers, 981 
Coccygeal plexus, neuralgia of, 741 
Cochlear nerve, diseases of, 506 
symptoms of, 507 
treatment of, 508 
Color blindness, 25 
Coma, diagnosis of, 441 
in lesion of pons, 290 
Compression myelitis, 229 
Confusion, 43 
Congenital cerebral hemiplegia, 409 
cysts of brain, 318 
heart disease, convulsions and, 794 
osteosclerosis, 968 
rickets, 955 
Conjugate deviation in apoplexy, 438, 439 
Consciousness, allopsyche and, 42 
anomalies of, 41 
autopsyche and, 43 
somatopsyche and, 42 
Contractures in amyotrophic lateral 
sclerosis, 99 
in facial paralysis, 502 
in hemiplegia, 464 
in hereditary spastic spinal paralysis, 
106 
in hysteria, 651 
of muscles, 68 
in progressive neural muscular 
atrophy, 110 
in traumatic neuroses, 651 
Convolutions, central, lesions of, 274 
Convulsions in abscess of brain, 380, 381 
in acute meningitis, 170 
in anemia of brain, 392 
in arteriosclerosis of brain, 397 
in brain tumor, 329, 333 
in general paresis, 857 
hemiplegia and, 415 
in hysteria, 673 
infantile, 790 
age and, 790 
albuminuria in, 795 
alcohol and, 793 
congenital heart disease and, 794 1002 
INDEX TO VOLUME V 
Convulsions, infantile, consciousness in 
loss of, 795 
diagnosis of, 795 
from epilepsy, 796 
epilepsy and, 594 
etiology of, 790 
infections and, 793 
motor phenomena in, 794 
onset of, 794 
opisthotonos in, 794 
paralysis in, 795 
predisposition to, 791 
prognosis of, 796 
reflex irritation and, 792 
respiration in, 794 
rickets and, 792, 793 
symptoms of, 794 
thymus and, 794 
treatment of, 796 
whooping cough and, 793 
in lesion of pons, 290 
in pachymeningitis interna, 165 
postnatal infantile hemiplegia and, 
415 
Convulsive tic, 784 
Coordination, disturbances of, 71 
Corpora quadrigemina, tumors of, 336 
striata, lesion of, 278 
Corpus callosum, disease of, 276 
tumor of, 276, 335 
Cortex of brain, motor area of, 265, 273 
sensory area of, 265, 273 
Cortical blindness, 54 
deafness, 33 
Coryza in migraine, 718 
Cramps in occupation neuroses, 744 
professional, 741 
Cranial base, tumors of, 340 
nerves in tabes dorsalis, 841 
Cremaster reflex, anomalies of, 78 
Crura cerebri, lesion of, 281 
tumors of, 335 
Crural neuralgia, 740 
Cutaneous reflexes, anomalies of, 77 
Cyanosis in cervical ribs, 556 
hypertrophic osteo-arthropathy and, 
982 
Cystoglioma of brain, 313 
Cystosarcoma of brain, 315 
Cysts of brain, 318 
Cytological examination of cerebrospinal 
fluid, 176 
D 
Day blindness, 480 
Deafness in cerebrospinal syphilis, 825 
mind, 54 
word, 54 
Deep reflexes, anomalies of, 77 
Deformity in compression of spinal cord, 
190 
Degeneration, progressive lenticular, 278 
reaction of, 72 
Degenerative myelitis, 236 
Delirium in acute meningitis, 170 
acutum, 66 
Delusions, 56 
anomalies of conduct due to, 67 
depressive, 58 
distinction from defects in judgment, 
58 
expansive, 58 
in general paresis, 855 
of grandeur, 57, 855 
of persecution, 58 
primary, 56 
secondary, 56 
Dementia, anomalies of conduct and, 67 
praecox, hysteria and, 694, 695 
Depression, pathological, 60 
Dercum's disease, 817 
Dermatitis, bullous, in hysteria, 686 
in dermatomyositis, 877 
Dermatomyositis, 875 
dermatitis in, 877 
diagnosis of, 878 
distribution of, 875 
etiology of, 875 
fever in, 877 
nervous system in, 877 
oedema in, 877 
pain in, 877 
pathology of, 876 
prognosis of, 878 
symptoms of, 876 
treatment of, 878 
Dermoid tumors of brain, 316 
Development, mental, trauma and, 650 
Diabetes, coma of, diagnosis of, from 
apoplexy, 442 
mellitus, apoplexy and, 427 
Diadokokinesis, 72 
Diaphragmatic neuralgia, 737 
Diarrhoea in hysteria, 688 
in migraine, 718 
Dichromates, 25 
Diffuse sclerosis, 160 
Diphtheria, apoplexy and, 417 
nfultiple neuritis and, 575 
Diplegia, cerebral, 419 
athetosis in, 420 
epilepsy and, 421 
symptoms of, 419 
congenital, diagnosis of, from amau- 
rotic family idiocy, 869 
Diplopia in disseminated sclerosis, 150 
monocular, 480 
in myasthenia gravis, 802 
Dislocation from pressure in brain tumors, 
323 
Disorientation, 44 
Disseminated myelitis, 235 
sclerosis, 140 
Dissociation, 51 
Dizziness in brain tumor, 329, 338 
Dorsodynia, 945 
Dream state, 689 
Drowsiness in brain tumor, 329 
Duchenne-Erb palsy, 551 
Dura mater, diseases of, 163 
hematoma of, diagnosis of, from 
brain tumor, 345 
Dysacusis, 33 INDEX TO VOLUME V 
1003 
Dysarthria, 74 
Dyschiria, 268 
Dyschondroplasia, 969 
Dyschromatopsia in brain tumor, 329 
Dysesthesia, 32 
in brain tumor, 339 
Dysphagia in brain tumor, 337 
Dysphonia, spastic, 522 
Dysplasic periosteal achondroplasia, 969 
Dyspnoea in Landry's paralysis, 251 
in myasthenia gravis, 804 
in paralysis of cervical plexus, 543 
Dyspraxia, 75 
Dysthymia, 60 
Dystrophy, muscular, bulbar, diagnosis of, 
from bulbar palsy, 117 
progressive, 117 
atrophy in, 119 
diagnosis of, 123 
from anterior poliomye- 
litis, 124 
from myasthenia gravis, 
124 
from progressive neural 
muscular atrophy, 111 
etiology of, 117 
facio-scapulo-humeral, 122 
hereditary, 123 
infantile, 123 
pathology of, 118 
prognosis of, 124 
pseudo-hypertrophic, 122 
reflexes in, 121 
symptoms of, 119 
treatment of, 124 
types of, 122 
weakness in, 120 
of Zimmerlin, 123 
E 
Echinococcus cysts of brain, 319 
Echolalia, 65 
Echopraxia, 65 
Elbow-joint in arthritis deformans, 911 
Electric chorea, 787 
Electrical hyperexcitability in tetany, 757 
reactions in diseases of peripheral 
nerves, 535 
in multiple neuritis, 582 
in progressive spinal muscular 
atrophy, 85 
Elephantiasis neuromatosa, 571 
Embolism, apoplexy and, 418, 422, 424, 
427, 428, 431 
of cerebral arteries, 400 
postnatal infantile hemiplegia and, 
418 
Emmetropia, 36 
Emotional states, anomalies of, 59 
Emotions, 59 
Empyema, abscess of brain and, 379 
Encephalic tuberculoma of brain, 319 
Encephalitis, 359 
acute, 360, 361 
hemorrhagic, 367 
Encephalitis, acute hyperplastic, 370 
non-suppurative, 370 
in advanced years, 375 
bacteriology of, 362 
chronic, 361 
classification of, 360 
congenita, 361, 370 
definition of, 359 
diagnosis of, 373 
from apoplexy, 375 
from brain tumor, 374 
from bulbar palsy, 117 
from cerebral hemorrhage, 375 
toxemia, 375 
from hysteria, 375 
from sinus thrombosis, 374 
from syphilis of brain, 375 
epilepsy and, 599 
etiology of, 361 
experimental, 371 
in infancy and childhood, 376 
influenzal, 362 
neonatorum, 370 
postnatal infantile hemiplegia and, 
416 
prognosis of, 377 
progressive, 361 
pyogenic, 363 
age and, 364 
course of, 364 
diagnosis of, 364 
onset of, 364 
pain in, 363 
staphylococcus in, 363 
symptoms of, 364 
subacute, 360, 361 
superior, 490 
alcoholism and, 490 
synonyms of, 359 
treatment of, 377 
Encephalomalacia, pathology of, 403 
Encephalomyelitis, acute, 369 
Benedikt's syndrome in, 369 
diagnosis of, 370 
Millard-Gubler syndrome in, 369 
ophthalmoplegia in, 369 
Weber's syndrome in, 369 
Encephalopathy, lead, diagnosis of, from 
brain tumor, 346 
Enchondroma of brain, 317 
Endarteritis in cerebrospinal syphilis, 824 
Endocarditis in arthritis deformans, 914 
chorea and, 776, 781 
Endothelioma of brain, 313 
Enteralgia in hysteria, 683 
Ependymal glioma of brain, 311 
inflammation, hydrocephalus and, 358 
Ependymitis, diagnosis of, from brain 
tumor, 345 
Epicritic sensibility, 17 
Epigastric reflexes, 78 
Epilepsy, 592 
affect, 610 
alcoholism and, 593, 596 
apoplexy and, 421 
atypical, 606 
aura in, 600 1004 
INDEX TO VOLUME V 
Epilepsy, behavior in, 612 
birth trauma and, 594 
in brain tumor, 331, 333 
bromides in, 620 
cardiac, 597 
cerebral diplegia and, 421 
cysts of brain and, 599 
definition of, 592 
degeneration in, 603 
diagnosis of, 613 
from apoplexy, 443 
from hysteria, 614, 691 
from infantile convulsions, 796 
diet in, 618 
disposition in, 611 
drugs in, 620 
encephalitis in, 599 
epileptoid conditions and, 607 
etiology of, 593 
exhaustion in, 607 
experimental seizures in, 609 
familial, 594 
hydrocephalus and, 599 
hydrotherapy in, 619 
hygiene in, 616 
hysteroid seizures in, 610 
idiopathic, 592 
imbecility and, 594 
incomplete, 605 
infantile convulsions and, 594 
Jacksonian, 592 
latent, 592 
meningitis in, 599 
menstrual, 598 
migraine and, 615, 711 
mortality in, 593 
nocturnal, 598, 610 
pathology of, 598 
petit mal, 604 
porencephaly in, 598 
psychic, 602 
alteration in, 602 
influences and, 597 
psychical anomalies of, 611 
psychosis in, 603 
rationale of seizures in, 608 
reflex, 596 
rudimentary, 605 
seizures in, 601 
simulation of, 615 
spasmophilia and, 604 
speech in, 612 
stages of, 601 
status epilepticus in, 609 
stupor in, 607 
symptoms of, 600 
synonyms of, 592 
syphilis and, 595 
toxins and, 594 
trauma and, 596 
treatment of, 615, 616 
mental, 619 
operative, 623 
true, 592 
tumors of brain and, 599 
vasomotor disturbances in, 606 
will in, 612 
Epileptic attacks in disseminated sclerosis, 
153 
in pseudo-sclerosis, 159 
insanity, traumatism and, 647 
psyche, 598, 612 
Epileptoid attacks in lesion of centrum 
semiovale, 275 
Epiphyseal cartilages in achondroplasia, 
958 
Epistaxis in polymyositis hemorrhagica, 
879 
Epithelioma of brain, 315 
Equilibrium, sense of, 18 
Erb's phenomenon, 71 
sign in tetany, 757 
syphilitic paraplegia, 246, 828 
Erythropsia, 480 
Exaltation, pathological, 60 
Excitability, affective, pathological, 62 
Exophthalmos in ocular paralysis, 485 
Extracerebellar tumors, 338 
Extradural hemorrhage, 257 
Eyes, conjugate deviation of, in lesion of 
internal capsule, 277 
in hysteria, 684 
Eye-grounds in abscess of brain, 380 
in tumor of brain, 328 
F 
Facial atrophy in migraine, 719 
hemiatrophy, 991 
nerve, paralysis of, 495 
in tabes dorsalis, 841 
neuralgia, 733 
paralysis, 495 
course of, 501 
diagnosis of, 501 
etiology of, 495 
fever in, 498 
in hysteria, 679 
meningitis and, 497 
onset of, 498 
otitis and, 497 
pain in, 498 
poliomyelitis and, 497 
polyneuritis and, 497 
prognosis of, 501 
secondary contractures in, 502 
symptoms of, 498 
treatment of, 502 
surgical, 503 
spasm, 504 
etiology of, 504 
treatment of, 505 
Facio-scapulo-humeral progressive mus- 
cular dystrophy, 122 
Falling sickness, 592 
Family periodic paralysis, 809 
Fatigue, sense of, 19, 32 
Feelings, instability of, pathological, 62 
Femoral neuralgia, 740 
Fetal rickets, 955 
Fever in abscess of brain, 380 
in apoplexy, 437 
in arthritis deformans, 909, 914 INDEX TO VOLUME V 
1005 
Fever in caries of spine, 192 
■ in chorea, 779 
in dermatomyositis, 877 
in facial paralysis, 498 
in hysteria, 685 
in sciatica, 563 
in subacute combined sclerosis, 129 
in tetany, 759 
Fibrillary twitchings, 70 
Fibroma of brain, 317 
Fibrositis, 943 
acute, 944 
etiology of, 944 
pathology of, 944 
symptoms of, 944 
treatment of, 945 
Fingers in arthritis deformans, 912 
in hypertrophic osteo-arthropathy, 
984 
Foot-jerk, 77 
Fourth ventricle, tumors of, 338 
Fracture, intra-uterine, 979 
Fragilitas ossium, 947, 980 
congenital, 978 
Freud's hypothesis of hysteria, 657 
Friedreich's ataxia, 130. See Ataxia, 
Friedreich's 
Frohlich's syndrome, 341 
Frontal lobe, tumors of, 331 
G 
Gait, anomalies of, 73 
in Friedreich's ataxia, 136 
in lesion of cerebellum, 286 
in multiple neuritis, 581 
in osteomalacia, 951 
in paralysis agitans, 770 
Ganglia of cerebral hemisphere, 278 
inflammation of, neuralgia and, 726 
Gangrene in cervical ribs, 556 
Gastric crises in tabes dorsalis, 841, 849 
disease, tetany and, 749 
nerves, paralysis of, 523 
Gelle's test, 24 
Geniculate ganglia, inflammation of, 505 
Gerlier's disease, 515 
Gigantism, achondroplasia and, 961 
Glandular enlargement in arthritis de- 
formans, 909, 915 
Glaucoma in trigeminal neuraglia, 734 
Glioma of brain, diffuse, 312 
ependymal, 311 
of mid-brain, 284 
Gliomatosis in syringomyelia, 213 
Gliosis in epilepsy, 599 
Glosso-labio-laryngeal paralysis, 113 
Glossopharyngeal nerve, diseases of, 516 
Gluteal nerves, paralysis of, 558 
Glycosuria in tumors of brain, 338, 342 
Goitre, exophthalmic, diagnosis, of, from 
neurasthenia, 634 
Gout, arthritis deformans and, 930 
migraine and, 720 
Granuloma of brain, infectious, 319 
Gumma of brain, 320 
H 
Habit chorea, 784 
spasm, 784 
Hallucinations, 37, 39 
auditory, 39 
complex, 39 
gustatory, 40 
olfactory, 40 
tactile, 40 
of visceral sense, 40 
visual, 39 
Hands in achondroplasia, 962 
in arthritis deformans, 912 
in hypertrophic osteo-arthropathy, 
984 
Headache in abscess of brain, 379 
in acute bulbar myelitis, 368 
meningitis, 170 
in anemia of brain, 393 
in arteriosclerosis of brain, 396 
in brain tumor, 324, 338 
in cerebral aneurism, 399 
in cerebrospinal syphilis, 824 
in lesion of pons, 290 
in Meniere's disease, 513 
in migraine, 716 
in neurasthenia, 631 
in pachymeningitis interna, 165 
in recurrent oculomotor palsy, 488 
sick, 709 
Hearing, anomalies of, 33 
centre of, 271 
in hemiplegia, 468 
in hysteria, 684 
in lesion of quadrigeminal bodies, 
282 
in neurasthenia, 632, 634 
sense of, 22 
examination of, 23 
Heart disease, congenital, convulsions, 
and, 794 
Heberden's nodes in arthritis deformans, 
92i 
diagnosis of, from hypertrophic 
osteo-arthropathy, 987 
Hematomyelia, 260 
in cervical enlargement, 262 
in conus medullaris, 262 
diagnosis of, 263 
from acute poliomyelitis, 263 
from hematorrachis, 263 
from myelitis, 263 
from syringomyelia, 263 
in dorsal cord, 262 
etiology of, 260 
histology of, 261 
incontinence of urine in, 262 
in lumbosacral enlargement, 262 
onset of, 261 
paralysis in, 261 
pathology of, 261 
prognosis of, 263 
symptoms of, 261 
treatment of, 264 
Hematorrachis, 258 1006 
INDEX TO VOLUME V 
Hematuria in polymyositis hemorrhagica, 
879 
Hemeralopia, 35, 480 
Hemiachromatopsia, 34 
in brain tumor, 334 
Hemiamblyopia, 34 
Hemianesthesia, diagnosis of, from 
hysteria, 693 
in hysteria, 681 
in lesion of internal capsule, 276 
of pons, 290 
Hemianopic pupillary reaction, 482 
Hemianopsia, 34, 270, 481 
in abscess of brain, 381, 382, 384 
in brain tumor, 332, 334 
in hemiplegia, 468 
heteronyma, 34 
homonyma, 34 
in lesion of internal capsule, 276 
quadrantic, 34 
symptoms of, 271 
Hemiataxia in abscess of brain, 381, 384 
Hemiathetosis, 70 
in lesion of internal capsule, 277 
Hemiatrophy, facial, 991 
bones in, 992 
diagnosis of, 992 
hair in, 992 
prognosis of, 992 
skin in, 992 
symptoms of, 991 
treatment of, 992 
Hemicontracture in lesion of internal 
capsule, 277 
Hemicrania, 709 
Hemihyperesthesia in hysteria, 681 
Hemiparesis in abscess of brain, 384 
Hemiparesthesia in migraine, 712 
Hemiplegia, apoplexy and, 412, 457 
associated movements in, 462 
bedsores in, 469 
bladder in, 467 
cerebral, congenital, 409 
contractures in, 464 
gait in, 464 
hearing in, 468 
hemianopsia in, 468 
hysteria and, 424, 678 
infantile, 412 
brain tumor and, 419 
congenital, 409 
convulsions and, 415 
diphtheria and, 417 
embolism and, 418 
encephalitis and, 416 
etiology of, 413 
infections and, 417 
measles and, 417 
postnatal, 412 
scarlet fever and, 417 
syphilis and, 418 
typhoid fever and, 417 
whooping cough and, 417 
larynx in, 459 
in lesion of internal capsule, 276 
of optic thalamus, 279 
of pons, 290 
Hemiplegia in lesion of pontomedullocere- 
bellar space, 290 
mastication in, 459 
mouth in, 460 
muscles in, 457, 461 
muscular rigidity in, 463 
tension in, 463 
palate in, 459 
posthemiplegic chorea in, 466 
ptosis in, 459 
reflexes in, deep, 465 
respiration in, 460 
sensation in, 467 
sympathetic effects in, 468 
tongue in, 460 
treatment of, 469 
tremor in, 465 
vasomotor effects in, 468 
Hemorrhage, apoplexy and, 422, 424, 
427, 428, 431 
cerebral, 401, 410, 411, 414, 415 
consciousness in, 434 
onset of, 431 
extradural, 257 
intradural, 258 
diagnosis of, 259 
from hematomyelia, 263 
pain in, 258 
paralysis in, 258 
prognosis of, 259 
sensation in, 258 
symptoms of, 258 
treatment of, 260 
into spinal cord, 260 
membranes, 257 
Hereditary cerebellar ataxia, Marie's, 130, 
136 
spastic spinal paralysis, 105 
Hernia, foraminal, of medulla, 324 
Herpes zoster, 571 
of auricle, 505 
in chorea, 781 
definition of, 571 
etiology of, 571 
fever in, 572 
hyperalgesia in, 572 
in migraine, 719 
pain in, 572, 573 
pathology of, 572 
skin in, 572 
symptoms of, 572 
treatment of, 573 
Hip disease, diagnosis of, from sciatica, 
565 
Hip-joint in arthritis deformans, 913, 922 
Hoffmann's symptom, 71 
Hunger, sense of, 19, 31 
Huntington's chorea, 787 
diagnosis of, from Friedreich's 
ataxia, 139 
Hydatid cysts of brain, 319 
Hydrocephalus, 351 
acquired, 356 
cerebrospinal fever and, 357 
diagnosis of, 355 
etiology of, 356 
inflammation and, 357, 358 1007 
INDEX TO VOLUME V 
Hydrocephalus, acquired, meningitis, pos- 
terior basic and, 357 
serosa and, 358 
tuberculous and, 357 
tumor and, 356 
iagnosis of, from brain tumor, 
345 
epilepsy and, 599 
essential, 353 
cephalocele and, 354 
course of, 354 
diagnosis of, 355 
from hydrocephalus, 355 
from rickets, 355 
etiology of, 353 
pathology of, 354 
spina bifida and, 354 
symptoms of, 354 
treatment of, 356 
externus, 353 
idiopathic, 353 
internus, 353 
secondary, 356 
in tumor of brain, 338 
Hydromyelia, 210 
Hypacusis, 33 
Hypalgesia in tabes dorsalis, 838 
Hyperalgesia, 30, 33 
in diseases of peripheral nerves, 
539 
in herpes zoster, 572 
hysteria and, 682 
Hyperchondroplasia, 969 
Hyperemia of brain, 392 
of spinal cord, 257 
Hyperesthesia, 28 
in acute meningitis, 170 
in hysteria, 681 
of larynx, 522 
in occupation neuroses, 747 
in tabes dorsalis, 836 
Hypermetropia, 36 
Hypermnesia, 53 
Hyperopia, 36 
Hyperostosis, 969 
cranii, diagnosis of, from Paget's dis- 
ease, 977 
of skeleton with cyst-building, 978 
of skull, general, 988 
nodular, 989 
Hyperthymia, 60 
Hypertonicity in disseminated sclerosis, 
147 
Hypertony of muscles, 68 
Hypertrophic internal pachymeningitis, 
166 
osteopathies, 969 
Hypertrophy of muscles, 68 
Hypesthesia in diseases of spinal roots, 
568 
in tabes dorsalis, 838 
Hypochondria, diagnosis of, from neuras- 
thenia, 633 
Hypogastric reflexes, 78 
Hypoglossal nerve, diseases of, 530 
Hypomnesia, 53 
Hypopituitarism in brain tumor, 342 
Hypothymia, 61 
Hypotonia in lesion of cerebellum, 287 
in tabes dorsalis, 839 
Hypotony of muscles, 68 
Hysteria, 654 
adductor paralysis and, 521 
akinetic motor phenomena in, 678 
analgesia in, 682 
anesthesia in, 680, 681 
anuria in, 687 
arhythmic movements in, 677 
arthralgia in, 683 
astasia-abasia and, 680 
ataxia in, 652 
attacks in, 671 
varieties of, 675 
Babinski's hypothesis of, 658 
biological hypothesis of, 665 
bleeding in, 687 
blindness in, 684 
blood in, 688 
cardialgia in, 683 
character in, 668 
choreiform movements in, 677 
contractures in, 651 
convulsions in, 673 
coordinated movements in, 676 
course of, 675, 690 
deep sensibility in, 682 
dementia prsecox and, 695 
diagnosis of, 691 
from acute meningitis, 172, 692 
from apoplexy, 443 
from brain tumor, 342 
from chorea, 693 
from dementia prsecox, 694 
from diseases of brain, 692 
from disseminated sclerosis, 
155 
from encephalitis, 375 
from epilepsy, 614, 691 
from hemi-anesthesia, 693 
from lateral sclerosis, 104 
from manic-depressive insanity, 
694 
from multiple sclerosis, 692 
from myasthenia gravis, 806 
from neuralgia, 694 
from neurasthenia, 633, 693 
from organic disease, 650 
from paresis, 694 
from syringomyelia, 693 
diarrhoea in, 688 
dissociation in, 656 
dizziness in, 684 
dream states in, 674 
emotional instability in, 669 
emotions and, 698 
enteralgia in, 683 
etiology of, 654 
eyes in, 684 
facial paralysis in, 679 
fever in, 685 
Freud's hypothesis of, 657 
glosso-labial paralysis in, 679 
hearing in, 684 
hemi-anesthesia in, 681 1008 
INDEX TO VOLUME V 
Hysteria, hemihyperesthesia in, 681 
hemiplegia and, 419, 424, 678 
humoralism and, 654 
hyperalgesia in, 682 
hypesthesia in, 681 
hypnotism in, 702 
metabolism in, 688 
monoplegia in, 681 
motor disturbances in, 671 
mutism in, 652 
myasthenia in, 678 
myoclonus in, 677 
mythomania and, 659 
negativism in, 670 
nerve theories of, 655 
neuralgia in, 683 
oedema in, 686 
ophthalmoplegia in, 679 
paralysis in, 650, 678 
of larynx in, 679 
paraplegia in, 679 
physiological theories of, 660 
pithiatism and, 659 
postconvulsive period of, 674 
prognosis of, 690 
pseudo-tabes and, 680 
psychological hypothesis of, 655 
psychopathology of, 654 
psychoses in, 689 
psychotherapy and, 702 
ptosis in, 679 
reeducation in, 704 
reflexes in, 688 
rhythmic movements in, 676 
secretory disturbances in, 687 
sensation in, 681 
sensationalism in, 670 
sight in, 684 
simulation and, 659, 667 
skin in, 686 
smell in, 684 
stigmata of, 667 - 
suggestion in, 669, 697, 702 
sympathetic system in, 663 
symptoms of, 666 
taste in, 684 
temperature sense in, 682 
tetany and, 677 
topalgia in, 683 
trauma and, 657 
traumatic, 644, 691 
treatment of, 696, 701 
analytical or cathartic, 706 
etiological, 700 
tremors in, 676 
trophic disturbances in, 685 
vasomotor disturbances in, 685 
hypothesis of, 662 
vestibular apparatus in, 684 
visceral pains in, 663 
vision in, 652 
Hysterical astasia-abasia, 815 
disseminated sclerosis, 154 
insanity, trauma and, 647 
neuralgia, diagnosis of, 730 
pseudo-tetany, diagnosis of, from 
tetany, 760 
I 
Ideas, associative, anomalies of, 51 
autochthonous, 52 
duration of, anomalies of, 52 
exaggerated, 52 
falsification of content of, 56 
flight of, 46 
hyperdynamic, 52 
imperative, 52 
anomalies of conduct due to, 67 
intensity of, anomalies of, 52 
Ideation, anomalies of, 46 
Idiocy, amaurotic family, 863 
blindness in, 869 
brain in, 864 
diagnosis of, 869 
from congenital diplegia 
869 
paraplegia, 869 
from syphilis of brain, 
870 
etiology of, 864 
fundus in, 863, 869 
histology of, 866 
pathogenesis of, 867 
pathology of, 864 
race and, 864 
reflexes in, 869 
symptoms of, 868 
treatment of, 870 
Idiopathic abscess of brain, 385 
Illusions, 37, 38 
memory, 55 
Imbecility, anomalies of conduct due to, 
67 
epilepsy and, 594 
Incoherence, 43, 51 
Incoordination in syringomyelia, 221 
Infantilism, 967 
diagnosis of, from achondroplasia, 967 
Infectious arthritis, 896 
Infective myelitis, 229 
Inflammation of brain, 359 
of geniculate ganglia, 505 
of optic nerve, 476 
Influenza, arthritis deformans and, 901 
neurasthenia and, 629 
Influenzal encephalitis, 362 
Insanity, diagnosis of, from neurasthenia, 
633 
epileptic, trauma and, 647 
hysterical, trauma and, 647 
manic-depressive, diagnosis of, from 
hysteria, 694 
Insular sclerosis, 140 
Intercostal neuralgia, 739 
Intermittent claudication, diagnosis of, 
from neuralgia, 730 
from sciatica, 565 
disseminated sclerosis, 154 
Interstitial hypertrophic progressive neu- 
ritis of childhood, 112 
Intestinal crises in tabes dorsalis, 842 
disease, tetany and, 749 
infection, arthritis deformans and, 
903 INDEX TO VOLUME V 
1009 
Intracerebellar tumors, 338 
Intracranial pressure, 391 
Intradural hemorrhage, 258 
Intra-ocular muscles, paralysis of, 486 
Intra-uterine fracture, 979 
rickets, 955 
Irritability, pathological, 61 
Irritation, motor, 70 
J 
Jacksonian epilepsy, 592 
Joints in syringomyelia, 225 
K 
Kernig's sign in acute meningitis, 170 
Kinanesthesia, 30 
Klumpke palsy, 552 
Knee-jerk, 77 
in tabes dorsalis, 837 
Knee-joint in arthritis deformans, 913, 
923 
Korsakow's syndrome, 45 
L 
Labyrinth, choked, in brain tumors, 328 
Landry's paralysis, 247. See Paralysis, 
Landry's 
Language, zone of, 293 
Laryngeal muscles, spasm of, 522 
nerves, paralysis of, 518 
adductor, 521 
diagnosis of, 519 
larynx in, 519 
phonation in, 519 
recurrent, bilateral, 519 
total unilateral, 519 
respiration in, 519 
treatment of, 520 
Larynx in disease of vagus nerve, 517 
in hemiplegia, 459 
in myasthenia gravis, 804 
sensory neuroses of, 522 
Lasegue's sign in sciatica, 563 
Lateral sclerosis, 101. See Sclerosis, 
lateral. 
Lathyrism, 139 
Lead poisoning amyotrophic lateral scler- 
osis and, 89 
Lenticular degeneration, 278 
nucleus, lesion of, 278 
zone, lesion of, 278 
Leontiasis ossea, 988 
Leprosy, diagnosis of, from progressive 
spinal muscular atrophy, 86 
Leptomeninges in syphilitic myelitis, 239 
Leptomeningitis, 167 
acute, 167 
chronic, 173 
primary, 167 
secondary, 168 
Leukemia, retinitis in, 475 
vol. v-64 
Leukocytosis in acute meningitis, 171 
in arthritis deformans, 916 
Lid reflex, 78 
Lipoma of brain, 317 
Lipomatosis, multiple, 818 
Little's disease, 419 
Liver, cirrhosis of, hypertrophic osteo- 
arthropathy and, 982 
in lenticular degeneration, 278 
Lobstein's disease, 980 
Locomotor ataxia, 833. See Tabes 
dorsalis, 833 
system, diseases of, 871 
Logorrhoea, 46 
in aphasia, 297 
Lumbago, 945 
traumatic, 644 
Lumbar plexus, neuralgia of, 739 
paralysis of, 560 
puncture, 174 
in acute meningitis, 171 
in diagnosis of brain tumor, 330 
Lumbo-abdominal neuralgia, 739 
Lymphatics in adiposis dolorosa, 819 
Lymphocytosis in spinal fluid, 177 
Lymphorrhagia in myasthenia gravis, 799 
M 
Macrocephaly in achondroplasia, 963 
Malaria, diagnosis of, from apoplexy, 
444 
migraines and, 720 
Malnutrition, multiple neuritis and, 576 
Mammary neuralgia, 739 
Mania, acute, traumatism and, 647 
Marie's hereditary cerebellar ataxia, 130, 
136 
theories in aphasia, 300 
Mastication in hemiplegia, 459 
Mastodynia, 739 
Measles, apoplexy and, 417 
Median nerve, paralysis of, 548 
Medulla, hemorrhage of, diagnosis of, from 
progressive bulbar palsy, 117 
oblongata, foraminal hernia of, 324 
tumors of, 337 
diagnosis of, from progres- 
sive bulbar palsy, 117 
Megalocephalia, 988 
Megalopsia, 480 
Megrims, 709 
Memory, anomalies of, 53 
illusions, 55 
phantasms, 55 
Mendel's sign, 78 
Meniere's disease, 512 
diagnosis of, 513 
pathology of, 513 
prognosis of, 514 
treatment of, 514 
syndrome in brain tumor, 337 
Meninges, anatomy of, 162 
in cerebrospinal syphilis, 824 
diseases of, 162 
physiology of, 162 1010 
INDEX TO VOLUME V 
Meningism, diagnosis of, from acute 
meningitis, 171 
Meningitis, blastomycotic, diagnosis of, 
from brain tumor, 345 
cerebrospinal, acute, 167 
Cheyne-Stokes respiration in, 
170 
convulsions in, 170 
delirium in, 170 
diagnosis of, 171 
from brain abscess, 172 
from hysteria, 172 
from meningitis, 171, 
172 
from otitis, 172 
etiology of, 167 
headache in, 170 
hyperesthesia in, 170 
influenza and, 168 
Kernig's sign in, 170 
leukocytosis in, 171 
lumbar puncture in, 171 
optic neuritis in, 170 
pain in, 170 
pathology of, 169 
photophobia in, 170 
pneumonia and, 168 
prognosis of, 173 
pulse in, 170 
reflexes in, 171 
rigidity of neck in, 170 
scarlet fever and, 168 
symptoms of, 169 
treatment of, 173 
vomiting in, 170 
diagnosis of, from abscess of brain, 
386 
from hysteria, 692 
epilepsy and, 599 
facial paralysis and, 497 
localized serous spinal, 174 
posterior basic, hydrocephalus and, 
357 
serosa, hydrocephalus and, 358 
serous, diagnosis of, from brain tumor, 
345 
tuberculous, diagnosis of, from acute 
otitis, 172 
hydrocephalus and, 357 
Meningo-encephalitis, tuberculous, of 
brain, 319 
Meningomyelitis, syphilitic, diagnosis of, 
from spinal caries, 193 
from tabes dorsalis, 845 
Menopause, arthritis deformans and, 902 
Mental disturbances, traumatic, 646 
failure in amyotrophic lateral scler- 
osis, 99 
fog, 44 
phenomena in tetany, 758 
Meralgia paresthetica, 557 
anterior, 558 
Metabolism in arthritis deformans, 903, 
907 
in hysteria, 688 
multiple neuritis and, 575 
osteomalacia and, 949, 952 
Metacarpophalangeal joints in arthritis 
deformans, 922 
Metastatic abscess of brain, 384 
Microcephalia, 990 
Micromelic rickets, 955 
Micturition in disseminated sclerosis, 150 
Mid-brain, glioma of, 284 
lesion of, 282 
ataxia in, 283 
paralysis in, 283 
Migraine, ophthalmic, 709 
anesthesia in, 713, 714 
abortive, 711 
agraphia in, 715 
cerebral tumor and, 720 
chilliness in, 713 
classical, 713 
combined hypothesis of, 710 
definition of, 709 
diagnosis of, 720 
dizziness in, 716 
epilepsy and, 615, 711 
etiology of, 710 
facial atrophy in, 719 
gout and, 720 
headache in, 716 
hemiparesthesia in, 712 
heredity and, 711 
herpes zoster in, 719 
malarial fever and, 720 
motor disturbances in, 715 
pain in, 717 
paralytic phenomena in, 716 
paresthesia in, 713, 714 
paresis and, 720 
psychical disturbances in, 719 
reflex hypothesis of, 710 
scotomata in, 712, 714 
secretions in, 718 
speech in, 715 
status hemicranius in, 718 
symptomatic, 720 
symptoms of, cerebellar, 716 
early, 713 
sensory, 713 
visual, 712, 714 
tabes and, 720 
theories of, 710 
toxic hypothesis of, 710 
treatment of, 721 
trophic disturbances in, 718 
vasomotor disturbances in, 718 
hypothesis of, 710 
vomiting in, 712 
ophthalmoplegic, 724 
diagnosis of, 725 
etiology of, 724 
symptoms of, 725 
treatment of, 725 
periodic paralysis and, 811 
Miliary sclerosis, 161 
Millard-Gubler paralysis, 290 
in acute encephalomyelitis, 369 
Mind blindness, 53, 271, 483 
in aphasia, 299 
deafness, 54 
Mollifies ossium, 947 INDEX TO VOLUME V 
1011 
Molluscum fibrosum, 571 
Monarticular arthritis deformans, 919, 
934 
Monocular diplopia, 480 
Monoideic somnambulism, 44 
Monoplegia in hysteria, 681 
Moods, 59, 62 
Morvan's type of syringomyelia, 220 
Motility, disturbances of, 68 
Motor actions, acceleration of, 64 
incoherence of, 66 
retardation of, 65 
agitation, 63 
aphasia, 75, 292, 295 
area of cortex of brain, 265 
disturbances in migraine, 715 
in neuralgia, 728 
irritation, 70 . 
nerves in tetany, 757 
perseveration, 67 
phenomena, in infantile convulsions, 
794 
stupor, 63, 65 
Mouth in hemiplegia, 460 
Movement, sensation of, 17 
Mucous membrane in hysteria, 682 
Multiple neuritis, 573 
sclerosis, 140 
Muscles in amyotonia congenita, 894 
in arthritis deformans, 907 
atony of, 68 
atrophy of, 68 
in arthritis deformans, 914 
in diseases of peripheral nerves, 
534 
in paralysis agitans, 771 
progressive spinal, 82 
contractures of, 68 
cramps of, in tetany, 754 
disturbed innervation of, in lesion of 
optic thalamus, 280 
electrical reactions of, 72 
hypertony of, 68 
hypertrophy of, 68 
hypotony of, 68 
inflammation of, 871. See Myositis, 
irritability of, in tetany, 758 
irritation of, 70 
in myasthenia gravis, 799, 802 
in myotonia congenita, 887 
of neck, spasm of, 525 
ocular, paralysis of, 485 
paralyses of, 68 
in paramyoclonus multiplex, 807 
in periodic paralysis, 813 
in progressive myositis ossificans, 883 
rigidity of, in paralysis agitans, 776 
sense, 17 
spasms of, 71 
tension of, in diseases of peripheral 
nerves, 534 
in hemiplegia, 463 
of tongue, spasm of, 532 
tonus of, 68 
Muscular atrophy, 80 
dystrophy, diagnosis of, from myas- 
thenia gravis, 806 
Muscular dystrophy, progressive, 117 
hypotonia in tabes dorsalis, 839 
rigidity in hemiplegia, 463 
in paralysis agitans, 769 
wasting in spondylitis deformans, 927 
weakness in general paresis, 857 
Musculocutaneous nerve, paralysis of, 546 
Musculospiral nerve, paralysis of, 547 
Mutism in hysteria, 652 
Myalgia, diagnosis of, from neuralgia, 730 
Myasthenia gravis, 798 
age and, 798 
bulbar muscles in, 802 
diagnosis of, 805 
from astasia-abasia, 816 
from diphtheritic paralysis, 
806 
from hysteria, 806 
from muscular dystrophy 
806 
from poliencephalitis, 806 
from progressive bulbar 
palsy, 116, 806 
muscular dystrophy, 124 
diplopia in, 802 
dyspnoea in, 804 
etiology of, 798 
extremities in, 804 
in hysteria, 678 
infections and, 798 
larynx in, 804 
lymphorrhages in, 799 
muscles in, 799, 802 
myasthenic reaction in, 801, 805 
nervous diseases and, 798 
pathology of, 799 
pregnancy and, 799 
prognosis of, 806 
reflexes in, 805 
sex and, 798 
symptoms of, 802 
thymus gland and, 799 
toxins and, 800 
treatment of, 807 
urine in, 805 
von Graefe's sign in, 803 
Myasthenic reaction, 801, 805 
Mydriasis, 77 
in tabes dorsalis, 837 
Myelitis, 227 
acute ascending, 236 
bulbar, 368 
bacteria and, 229 
chronic, 245 
compression, 229 
definition of, 227 
degenerative, 236 
diagnosis of, 241 
from disseminated sclerosis, 155 
from hematomyelia, 263 
from Landry's paralysis, 252 
from lateral sclerosis, 104 
from peripheral neuritis, 242 
disseminated, 235 
infective, 229 
anesthesia in, 234 
bacteriology of, 231 1012 
1NDEX TO VOLUME V 
Myelitis infective, cervical, 234 
course of, 235 
dorsal, 234 
etiology of, 229 
histology of, 232 
lumbosacral, 235 
nervous elements in, 233 
neuroglia in, 232 
pathogenesis of, 229 
pathology of, 231 
symptoms of, 233 
transverse, 234 
treatment, 243 
vascular changes in, 232 
prognosis of, 243 
syphilis and, 228 
syphilitic, 237, 828 
age and, 237 
alcoholism and, 238 
bladder in, 240 
bloodvessels in, 238 
course of, 241 
diagnosis of, 242, 828 
etiology of, 237 
histology of, 238 
leptomeninges in, 239 
nervous elements in, 239 
neuroglia in, 239 
onset of, 240 
pain in, 240 
paralysis in, 240 
paraplegia in, 241 
pathology of, 238, 828 
sex and, 237 
symptoms of, 240, 828 
treatment of, 244, 830 
toxic, 236 
differentiation from infective, 236 
etiology of, 236 
pathology of, 237 
pregnancy and, 236 
symptoms of, 237 
treatment of, 243 
treatment of, 244 
Myeloma of brain, 315 
of spinal cord, 198 
Myeloplastic bone aplasia, 978 
Myoclonus in hysteria, 677 
Myopathy, primary, 117 
Myopia, 36 
Myopsia, 480 
Myosis, 77 
Myositis, 871 
acute, diagnosis of, from neuritis, 587 
definition of, 871 
non-suppurative, 875 
ossificans, progressive, 881 
bones in, 883, 884 
diagnosis of, 885 
from locomotor ataxia, 
885 
from multiple exostoses, 
885 
from spondylitis defor- 
mans, 885 
etiology of, 881 
fever in, 883 
Myositis ossificans, progressive, muscles 
in, 883 
oedema in, 883 
pain in, 883 
pathology of, 882 
prognosis of, 885 
• symptoms of, 883 
treatment of, 885 
primary, fibrosa, 880 
suppurative, 872 
chills in, 873 
diagnosis of, 874 
from metastatic ab- 
scesses, 874 
from osteomyelitis, 874 
ecchymosis in, 874 
erythema in, 874 
etiology of, 872 
fever in, 873 
oedema in, 874 
pain in, 873 
pathology of, 873 
prognosis of, 874 
symptoms of, 873 
treatment of, 875 
with special terminal lesions, 880 
Myotonia congenita, 886 
age and, 886 
cramps in, 890 
diagnosis of, 892 
electrical reactions in, 891 
etiology of, 886 
muscles in, 887, 890 
myotonic reaction in, 891 
pathogenesis of, 889 
pathology of, 887 
prognosis of, 893 
reflexes in, 892 
sex and, 886 
symptoms of, 890 
treatment of, 893 
Myotonic reaction, 891 
Mythomania, 659 
Myxosarcoma of brain, 315 
N 
Nails in diseases of peripheral nerves, 541 
in osteo-arthropathy, 984 
Neck, muscles of, spasm of, 525 
rigidity of, in acute meningitis, 170 
Nephritis, chronic, apoplexy and, 427 
diagnosis of, from brain tumor, 344 
in polymyositis hemorrhagica, 880 
Nerve deafness, 509 
Rinne's test in, 510 
Weber's test in, 510 
irritability of, in tetany, 758 
motor, in tetany, 757 
section of, recovery from, 538 
sensation and, 536 
sensory, irritation of, 539 
trunks, neuralgia and, 726 
tumors of, 568. See Neuromata. 
Nervous system, arthritis deformans and, 
903, 907 INDEX TO VOLUME V 
1013 
Nervous system in dermatomyositis, 877 
myasthenia gravis and, 798 
in Paget's disease, 973 
parasyphilis of, 822 
syphilis of, 822 
Spirochaeta pallida in, 823 
tetany and, 749 
Neuralgia, 725 
anemia and, 726 
anesthesia in, 728 
arteriosclerosis and, 727 
brachial, 737 
diagnosis of, 738 
pain in, 738 
symptoms of, 738 
treatment of, 738 
cervico-occipital, 736 
etiology of, 736 
pain in, 736 
Valleix's points in, 737 
of coccygeal plexus, 741 
counterirritation in, 732 
course of, 729 
crural, 740 
definition of, 725 
diagnosis of, 729 
from hysteria, 694 
from intermittent claudication, 
730 
from myalgia, 730 
from neurasthenia, 730 
from neuritis, 730 
from tabes dorsalis, 730 
diaphragmatic, 737 
etiology of, 726 
facial, 733 
femoral, 740 
ganglia and, 726 
in hysteria, 683 
hysterical, diagnosis of, 730 
intercostal, 739 
localized forms of, 733 
of lumbar plexus, 739 
lumbo-abdominal, 739 
mammary, 739 
motor disturbances in, 728 
nerve trunks and, 726 
obturator, 740 
pain in, 727 
paresis and, 727 
paresthesia in, 728 
prognosis of, 731 
psychical, 726 
of pudendal plexus, 740 
reflex, 726, 731 
sciatic, diagnosis of, from simple 
sciatica, 565 
secretory disturbances in, 728 
skin in, 728 
symptoms of, 727 
syphilis and, 727 
tabes dorsalis and, 727 
testicular, 740 
toxins and, 726 
treatment of, 731 
surgical, 732 
trigeminal, 733 
Neuralgia, trigeminal, course of, 735 
diagnosis of, 735 
exposure and, 733 
glaucoma in, 734 
in multiple sclerosis, 733 
pain in, 734 
photophobia in, 734 
skin in, 734 
symptoms of, 733 
in tabes dorsalis, 733 
tic douloureux, 733 
treatment of, 735 
Valleix's points in, 734 
visual disturbances in, 734 
Valleix's points in, 727 
vasomotor disturbances in, 728 
Neurasthenia, 624 
. appetite in, 631 
in arteriosclerosis of brain, 395 
circulation in, 631 
climate and, 628 
diagnosis of, 632 
from Graves' disease, 634 
from hypochondria, 633 
from hysteria, 633, 693 
from insanity, 633 
from neuralgia, 730 
emotional stress and, 627, 630 
etiology of, 624 
headache in, 631, 634 
hearing in, 632, 634 
heredity and, 625 
influenza and, 629 
isolation in, 637 
occupation and, 627 
pain in, 631 
paresthesia in, 631 
prognosis of, 634 
psychotherapy in, 640 
race and, 628 
reflexes in, 632 
secondary, 628 
symptomatic, 628 
symptoms of, 630 
syphilis and, 629 
in tabes dorsalis, 843 
traumatic, 643 
treatment of, 635 
typhoid fever and, 629 
urine in, 632 
vertigo in, 631 
vision in, 632 
Neuritis, brachial, 553 
diagnosis of, from neuralgia, 730 
interstitial hypertrophic progressive, 
! of childhood, 112 
multiple, 573 
alcoholic, 579 
diagnosis of, from tabes 
dorsalis, 845 
anemia in, 585 
ascending, 576 
ataxia in, 583 
bacteria and, 575, 576 
cachexia and, 576 
classification of, 574 
complications of, 586 1014 
INDEX TO VOLUME V 
Neuritis, multiple, course of, 585 
cranial nerves in, 582 
diagnosis of, 586 
from acute myositis, 587 
poliomyelitis, 586 
from Landry's paralysis, 586 
from oedema, 587 
from progressive bulbar 
palsy, 116 
neural, muscular atrophy, 
111 
spinal muscular atrophy 
86 
from tabes dorsalis, 587 
from trichiniasis, 587 
diphtheria and, 575 
electrical reactions in, 582 
etiology of, 574 
gait in, 581 
incidence of, 576 
malnutrition and, 576 
metabolism and, 575 
oedema in, 585 
onset of, 579 
paralysis in, 580 
pathology of, 576 
poisons and, 575 
polyarthritis and, 585 
prognosis of, 588 
reflexes in, 584 
scarlet fever and, 575 
smallpox and, 575 
sphincters in, 584 
symptoms of, 579 
mental, 585 
motor, 580 
sensory, 583 
tenderness in, 583 
treatment of, 588 
trophic changes in, 584 
tuberculosis and, 575, 586 
typhoid fever and, 575 
vasomotor changes in, 584 
optic, 36 
in acute meningitis, 170 
peripheral, diagnosis of, from syphil- 
itic myelitis, 242 
retrobulbar, 37, 479 
sciatic, diagnosis of, from organic 
sciatica, 565 
Neuroblastoma of brain, 317 
Neurocytoma of brain, 317 
Neurofibromatosis, general, 570 
Neuroglia in infective myelitis, 232 
in syphilitic myelitis, 239 
Neuroglioma of brain, 311 
Neuroma of brain, 317 
Neuromata, 568 
false, 569 
multiple, 590 
pathology of, 569 
plexiform, 571 
solitary, 569 
true, 568 
Neuromyositis, 880 
Neuroses of larynx, sensory, 522 
occupation, 741 
Neuroses, occupation, age and, 743 
anesthesia in, 747 
atrophy in, 744 
course of, 747 
cramps in, 744 
definition of, 741 
etiology of, 742 
heredity and, 742 
hyperesthesia in, 747 
occupation and, 743 
occurrence of, 743 
palsies in, 744 
paresthesia in, 747 
prognosis of, 747 
psychical factors in, 742 
sensory disturbances in, 747 
symptoms of, 743 
synonyms of, 741 
treatment of, 747 
writer's cramp, 744 
professional, 741 
traumatic, 642 
anesthesia in, 651 
ataxia in, 652 
contractures in, 651 
diagnosis of, 648 
fraud in, 642 
mutism in, 652 
paralysis in, 651 
prognosis of, 652 
vision in, 652 
Neuro-tabes peripherica, 579, 584 
Neurotic progressive facial atrophy, 991 
Night blindness, 35, 480 
Nodosity of joints, 897 
Nyctalopia, 35, 480 
Nystagmus, 70 
in abscess of brain, 384 
in brain tumor, 336, 339, 340 
in disseminated sclerosis, 151 
in Friedreich's ataxia, 135 
in Meniere's disease, 513 
in ocular paralysis, 486 
in pseudo-sclerosis, 159 
in syringomyelia, 222 
in vestibular disease, 511 
O 
Oblique muscles, paralysis of, 485 
Obsessions, 52 
"Obstetrical hand," 71 
Obturator nerve, paralysis of, 557 
Occupation neuroses, 741 
Ocular muscles in disseminated sclerosis, 
151 
paralysis of, 485 
exophthalmos in, 485 
nystagmus in, 486 
nerves, paralysis of, 483 
in meningitis, 488 
ophthalmoplegia in, 483 
ptosis in, 488 
symptoms of, 483 
in syphilis, 488 
in tabes, 488 INDEX TO VOLUME V 
1015 
Ocular palsy, nuclear, 365 
Oculomotor nerve, anatomy of, 483 
palsy, recurrent, 488 
in tabes dorsalis, 841 
(Edema in amyotonia congenita, 894 
in arthritis deformans, 917 
in brain tumor, 323 
in dermatomyositis, 877 
diagnosis of, from neuritis, 587 
in hysteria, 686 
in multiple neuritis, 585 
in polymyositis hemorrhagica, 879 
(Esophageal nerves, paralysis of, 523 
Olfactory nerve, diseases of, 471 
Olivopontocerebellar atrophy, 287 
Ophthalmoplegia, 483 
in acute encephalomyelitis, 369 
in cerebrospinal syphilis, 825 
chronic, 491 
external, 86 
diplopia in, 484 
externa, 490 
in hysteria, 678 
interna, 490 
in lesion of quadrigeminal bodies, 283 
strabismus in, 484 
in superior hemorrhagic polienceph- 
alitis, 365 
in tabes dorsalis, 841 
Ophthalmoplegic migraine, 724 
Ophthalmoscopic examination in apoplexy, 
437 
Opium poisoning, diagnosis of, from apo- 
plexy, 442 
Oppenheim's disease, 893 
sign, 78 
Optic aphasia, 54, 299 
asymbolia, 54 
atrophy, 478 
diagnosis of, 479 
primary, 479 
secondary, 479 
chiasm, affections of, 481 
disks, in disseminated sclerosis, 152 
in papillitis, 478 
disseminated sclerosis, 154 
nerve, anatomy of, 473 
atrophy of, 37 
in tabes dorsalis, 841 
diseases of, 476 
inflammation of, 476 
neuritis, 36, 326 
thalamus, lesion of, 279 
astereognosis in, 281 
ataxia in, 280 
athetosis in, 280 
disturbed innervation of 
muscles in, 280 
hemiplegia in, 279 
tracts, affections of, 481 
Osteitis, chronic parenchymatous, 978 
deformans, 969, 970 
arteriosclerosis in, 973 
bones in, 971 
deformity in, 974 
diagnosis of, 976 
from acromegaly, 977 
Osteitis deformans, diagnosis of, from 
hyperostosis cranii, 977 
from osteo-arthropathy, 977 
from osteomalacia, 977 
from spondylitis, 977 
etiology of, 970 
heredity and, 970 
occurrence of, 970 
pain in, 974, 975 
pathology of, 970 
prognosis of, 977 
skull in, 971 
spine in, 971 
symptoms of, 973 
syphilis and, 973 
treatment of, 977 
tumor-building, 978 
tumors and, 973 
fibrosa, 980 
Osteo-arthritis, 897, 920 
Osteo-arthropathy, hypertrophic pulmon- 
ary, 981 
age and, 983 
arthritis in, 985 
atrophy in, 986 
bones in, 984 
bronchiectasis and, 982 
cirrhosis of liver and, 982 
cyanosis and, 982 
diagnosis of, 987 
from arthritis defor- 
mans, 987 
from Heberden's nodes, 
987 
from Paget's disease, 
977 
etiology of, 981 
fingers in, 984 
hands in, 984 
nails in, 984 
pain in, 985 
pathology of, 983 
prognosis of, 987 
race and, 983 
sex and, 983 
symptoms of, 984 
syphilis and, 982 
treatment of, 988 
tuberculosis and, 981 
types of, 986 
secondary, 981 
Osteogenesis imperfecta, 978 
bones in, 979 
etiology of, 979 
intra-uterine fracture and, 979 
pathology of, 979 
Osteomalacia, 947 
age and, 947 
bones in, 949 
congenita, 978 
definition of, 947 
diagnosis of, 953 
from Paget's disease, 977 
diet in, 953 
etiology of, 947 
gait in, 951 
metabolism and, 949, 952 1016 
INDEX TO VOLUME V 
Osteomalacia, ovaries and, 948 
pain in, 951 
parathyroid gland and, 949 
pathology of, 948 
pregnancy and, 948 
reflexes in, 951 
sex and, 947 
symptoms of, 951 
tetany and, 949 
thyroid gland in, 948 
treatment of, 953 
urine in, 952 
varieties of, 953 
vertebrae in, 950 
with cyst-building, 978 
Osteomyelitis, diagnosis of, from primary 
suppurative myositis, 874 
Osteopathies, hypertrophic, 969 
Osteoporosis congenita, 978 
Osteopsathyrosis, 978, 980 
etiology of, 980 
prognosis of, 981 
symptoms of, 981 
treatment of, 981 
Osteosarcoma of brain, 314 
Osteosclerosis, congenital, 968, 978 
Ostitis deformans, 970 
fibrosa hyperostotica cranii et faciei, 
988 
Otitic abscess of brain, 383 
Otitis, diagnosis of, from acute meningitis, 
172 
Ovaries in osteomalacia, 948 
P 
Pachymeningitis, 163 
cervicalis hypertrophica, 166 
diagnosis of, 167 
from progressive spinal 
muscular atrophy, 86 
from tumors of spinal 
cord, 208 
etiology of, 166 
symptoms of, 166 
treatment of, 167 
externa, 163 
cerebral, 163 
spinal, 163 
symptoms of, 164 
treatment of, 164 
interna, 164 
aphasia in, 165 
convulsions in, 165 
diagnosis of, 165 
headache in, 165 
hemorrhagica, 164 
optic neuritis in, 165 
pathology of, 164 
symptoms of, 165 
treatment of, 166 
vomiting in, 165 
Paget's disease, 970 
Pain in abscess of brain, 381, 384 
in acute meningitis, 170 
in adiposis dolorosa, 819, 820 
Pain in amyotrophic lateral sclerosis, 99 
in arthritis deformans, 909, 910, 921, 
922 
in brachial neuritis, 553 
in brain tumor, 338 
in cervical ribs, 555 
in cervico-occipital neuralgia, 736 
in compression of spinal cord, 190 
in dermatomyositis, 877 
in diseases of spinal roots, 567 
in disseminated sclerosis, 149 
in facial paralysis, 498 
in Friedreich's ataxia, 136 
in herpes zoster, 572 
in intercostal neuralgia, 739 
in intradural hemorrhage, 258 
in Landry's paralysis, 251 
in lesion of optic thalamus, 280 
of spinal cord, 183 
in neuralgia, 727 
in neurasthenia, 631 
in ophthalmic migraine, 717 
in osteo-arthropathy, 985 
in pachymeningitis cervicalis hyper- 
trophica, 166 
in Paget's disease, 974, 975 
in paralyzing vertigo, 515 
in polymyositis hemorrhagica, 879 
in primary myositis fibrosa, 881 
in recurrent oculomotor palsy, 488 
in sciatica, 562 
sense, 17 
anomalies of, 29 
in solitary neuromata, 569 
in spasmodic torticollis, 528 
in spondylitis deformans, 925, 926 
in syphilitic myelitis, 240 
in tabes dorsalis, 836 
in tetany, 757, 759 
in trigeminal neuralgia, 734 
in tumors of spinal cord, 201, 202 
Palate in hemiplegia, 459 
paralysis of, in hysteria, 679 
Pallanesthesia, 30 
Palmar reflex, 78 
Palsies, cerebral, congenital, 410 
Duchenne-Erb, 551 
Klumpke's, 552 
in occupation neuroses, 744 
oculomotor, recurrent, 488 
in tabes dorsalis, 841 
Palsy, bulbar, acute inflammatory, 368 
of childhood, diagnosis of, from 
progressive bulbar palsy, 117 
diagnosis of, from progressive 
bulbar palsy, 117 
progressive, 113 
diagnosis of, 116 
from bulbar palsy, 117 
of childhood, 
117 
type of muscular 
dystrophy, 117 
from encephalitis, 117 
from hemorrhage, 117 
from multiple neuritis, 
116 INDEX TO VOLUME V 
1017 
Palsy, bulbar, progressive, diagnosis of, 
from myasthenia 
gravis, 116 
from tumor of pons and 
medulla, 117 
etiology of, 113 
pathology of, 114 
reflexes in, 116 
speech in, 115 
symptoms of, 115 
treatment of, 117 
shaking, 764 
Papillitis, 326 
mechanism of, 476 
optic disk in, 478 
Papilloma of brain, 315 
Paracentral convolutions, tumors of, 332 
Paragraphia, 66, 77 
Paralysis in abscess of brain, 381 
in acute infective polyneuritis, 590 
agitans, 764 
age and, 764 
atrophy in, 771 
definition of, 764 
diagnosis of, 772 
from chorea, 773 
from multiple sclerosis, 772 
from senile tremor, 773 
ductless glands and, 767 
etiology of, 764 
face in, 770 
forms of, 772 
gait in, 770 
heredity and, 764 
parathyroid glands and, 767 
pathogenesis of, 767 
pathology of, 765 
prognosis of, 773 
reflexes in, 771 
rigidity in, 769 
symptoms of, 768 
trauma and, 764 
treatment of, 773 
tremor in, 768 
voice in, 771 
in amaurotic family idiocy, 869 
in apoplexy, 438, 440 
ascending bulbar, 798 
asthenic, 798 
atrophic, in syringomyelia, 221 
birth, brachial, 552 
of brachial plexus, 550 
in brain tumor, 337 
of cardiac nerves, 522 
in cerebrospinal syphilis, 825 
of cervical plexus, 543 
of circumflex nerve, 546 
diaphragmatic, 544 
diphtheritic, diagnosis of, from myas- 
thenia gravis, 806 
Duchenne-Erb, 551 
of external rectus muscle, 485 
of facial nerve, 495 
flaccid, 178 
of gastric nerves, 523 
glosso-labio-laryngeal, 113 
of gluteal nerves, 558 
Paralysis in hematomyelia, 260 
in hysteria, 650, 678, 679 
diagnosis of, 650, 678 
from apoplexy, 446 
in infantile convulsions, 795 
of inferior oblique muscle, 485 
rectus muscle, 485 
of internal rectus muscle, 485 
in intradural hemorrhage, 258 
of intra-ocular muscles, 486 
Klumpke's, 552 
Landry's, 247 
bacteriology of, 248 
bladder in, 251 
diagnosis of, 252 
from myelitis, 252 
from neuritis, 586, 587 
from poliomyelitis, 252 
dyspnoea in, 251 
etiology of, 248 
pain in, 251 
paresthesia in, 251 
pathology of, 249 
prognosis of, 251 
symptoms of, 250 
treatment of, 254 
vasomotor disturbances in, 251 
laryngeal, 518, 521 
in lesion of cerebellum, 286 
of crura cerebri, 281 
of internal capsule, 276 
of mid-brain, 283 
of pons, 289 
of tegmentum, 281 
of long thoracic nerve, 544 
of lower arm, 552 
of lumbar plexus, 560 
of median nerve, 548 
Millard-Gubler type, 290 
in multiple neuritis, 580 
of muscles, 68 
of musculocutaneous nerve, 546 
of musculospiral nerve, 547 
of nerves of legs, 556 
diagnosis of, 560 
from disease of cord, 560 
from lesions of cauda 
equina, 561 
of sacral plexus, 561 
of ocular nerves, 483 
sympathetic, in syringomyelia, 224 
of oesophageal nerves, 523 
periodic, 809 
auto-infection and, 811 
blood in, 811 
course of, 814 
definition of, 809 
etiology of, 810 
heredity and, 810 
migraine and, 811 
muscles in, 813 
paralysis in, 813 
pathology of, 810 
pulse in, 813 
reflexes in, 814 
symptoms of, 812 
transmission of, 810 1018 
1NDEX TO VOLUME V 
Paralysis, periodic, treatment of, 814 
urine in, 811 
of pharyngeal nerves, 517 
of phrenic nerve, 543 
of popliteal nerves, 559 
of posterior tibial nerve, 559 
in progressive spinal muscular atrophy, 
84 
pseudo-bulbar, 290, 978 
of pulmonary nerves, 523 
of radial nerve, 547 
of sacral plexus, 560 
of sciatic nerve, 558, 560 
"soul," 268 
spastic, 179 
spinal, 101 
hereditary, 105 
in syringomyelia, 221 
of spinal accessory nerve, 524 
of superior oblique muscle, 486 
rectus muscle, 485 
of suprascapular nerve, 545 
in syphilitic myelitis, 240, 828 
of tongue, 530 
in traumatic neuroses, 651 
of trigeminal nerve, 493, 494 
of ulnar nerve, 549 
unilateral, 108 
of upper arm, 551 
of vagus nerve, 517 
Paralytic conjugate deviation in apoplexy, 
439 
phenomena in chorea, 780 
in migraine, 716 
Paramimia, 66 
Paramnesia, 55 
Paramyoclonus multiplex, 807 
diagnosis of, 808 
etiology of, 807 
muscles in, 808 
pathology of, 807 
prognosis of, 809 
symptoms of, 808 
treatment of, 809 
Paranoia, trauma and, 647 
Paraphasia, 66 
in abscess of brain, 381 
Paraplegia in cerebrospinal syphilis, 825 
congenital, diagnosis of, from amau- 
rotic family idiocy, 869 
Erb's syphilitic, 246, 828 
in hysteria, 679 
spastic, diagnosis of, from dissemin- 
ated sclerosis, 156 
in subacute confined sclerosis, 127, 128 
in syphilitic myelitis, 241 
in tumors of spinal cord, 203 
Parapraxia, 66 
Parasitic cysts of brain, 318 
Parasyphilis of nervous system, 822 
Parathyroid gland, osteomalacia and, 949 
tetany and, 750, 753 
Parenchymatous multiple neuritis, 577 
Paresis, general, 850 
Argyll-Robertson pupil in, 856 
convulsions in, 857 
course of, 857 
Paresis, general, delusions in, 855 
diagnosis of, 859 
from alcoholic pseudo-pare- 
sis, 8(50 
from arteriosclerosis, 398 
from arteriosclerotic de- 
mentia, 861 
from brain tumor, 343 
from disseminated sclerosis, 
156, 860 
from syphilitic pseudo- 
paresis, 860 
duration of, 858 
etiology of, 850 
hematoma in, 857 
memory in, 854 
pathology of, 852 
prognosis of, 859 
pupils in, 856 
reflexes in, 857 
speech in, 856 
Spirochaeta pallida in, 851, 852 
symptoms of, 853 
syphilis and, 850 
tabes dorsalis and, 843 
trauma and, 647 
treatment of, 861 
tremors in, 856 
trophic disturbances in, 858 
vasomotor disturbances in, 858 
Wassermann reaction in, 851 
migraine and, 720 
neuralgia and, 727 
Paresthesia in diseases of peripheral 
nerves, 539 
of spinal roots, 567 
in Landry's paralysis, 251 
of larynx, 522 
in migraine, 713, 714 
in neuralgia, 728 
in neurasthenia, 631 
in occupation neuroses, 747 
in osteomalacia, 951 
in sciatica, 563 
in solitary neuromata, 570 
Paretic gait, 73 
Parietal lobe, tumors of, 333 
Parkinson's disease, 764 
Patellar tendon reflex, 77 
Pellagra, 139 
Pelvis, tumor in, diagnosis of, from 
sciatica, 565 
Perception, anomalies of, 37 
sense, 18 
Peri-articular arthritis deformans, 908 
Pericarditis in arthritis deformans, 914 
Periodic paralysis, 809 
Periosteal dyscrasia, 978 
Peripheral nerves, diseases of, 533 
atrophy in, 534 
causalgia in, 539 
deep reflexes in, 534 
distal reaction in, 535 
electrical reactions in, 535 
hyperalgesia in, 539 
loss of power in, 534 
motor irritation in, 535 INDEX TO VOLUME V 
1019 
Peripheral nerves diseases of, nails in, 
541 
paresthesia in, 539 
prognosis of, 541 
reaction of degeneration in, 
535 
skin in, 540 
symptoms of, motor, 533 
sensory, 536 
trophic, 540 
vasomotor, 540 
tension of muscles in, 534 
treatment of, 541 
neuritis, 573 
diagnosis of, from syphilitic mye- 
litis, 242 
Petit mal, 604 
aura in, 605 
Pharyngeal nerves, paralysis of, 517 
Pharynx in myasthenia gravis, 804 
Phobias, 52 
Phonic spasm, 522 
Photophobia in acute meningitis, 170 
in trigeminal neuralgia, 734 
Photopsia, 36 
Phrenic nerve, paralysis of, 543 
Pineal gland, lesion of, 291 
tumors of, 336 
Pithiatism, 659 
Pituitary glands, lesion of, 291 
Pleurisy in arthritis deformans, 915 
Pleurodynia, 945 
Plexiform neuromata, 571 
Pneumogastric nerve, diseases of, 517 
Pneumonia, acute meningitis and, 168 
Poisoning, opium, diagnosis of, from 
apoplexy, 442 
tetany and, 749 
Poliencephalitis, acute inferior, 368 
diagnosis of, from myasthenia gravis, 
806 
hemorrhagic superior, 365 
alcoholic, 366 
diagnosis of, 367, 373 
gait in, 367 
headache in, 366 
non-alcoholic, 365 
ophthalmoplegia in, 365 
Poliencephalomyelitis, 369 
Poliomyelitis, acute, diagnosis of, from 
hematomyelia, 263 
from neuritis, 586 
from progressive spinal mus- 
cular atrophy, 124 
anterior, chronic, 82 
diagnosis of, from progressive 
muscular dystrophy, 124 
neural muscular atrophy, 
111 
diagnosis of, from Landry's paralysis, 
252 
facial paralysis and, 497 
Polyarthritis, multiple neuritis and, 585 
Polychromates, 25 
Polydipsia in tumor of brain, 338 
Polyideic somnambulism, 44 
Polymyositis hemorrhagica, 879 
Polymyositis hemorrhagica, diagnosis of, 
880 
etiology of, 879 
oedema in, 879 
pathology of, 879 
prognosis of, 880 
skin in, 879 
symptoms of, 879 
treatment of, 880 
Polyneuritic psychosis, 45 
Polyneuritis, 573 
acute infective, 590 
course of, 590 
onset of, 590 
paralysis in, 590 
spleen in, 590 
symptoms of, 590 
facial paralysis and, 497 
recurring, 591 
Polyuria in brain tumor, 337, 338, 342 
Pons, hemorrhage of, diagnosis of, from 
progressive bulbar palsy, 117 
lesion of, 289 
coma in, 290 
convulsions in, 290 
headache in, 290 
hemianesthesia in, 290 
hemiplegia in, 290 
paralysis in, 289 
vertigo in, 290 
tumors of, 336 
diagnosis of, from progressive 
bulbar palsy, 117 
Pontile syndrome, 289 
Pontomedullocerebellar space, lesion in, 
289 
Popliteal nerves, paralysis of, 559 
Porencephaly, apoplexy and, 409 
epilepsy and, 598 
Position, anomalies of, 30 
Posterior spinal sclerosis, 833. See Tabes 
dorsalis. 
Posthemiplegic chorea, 466 
Pott's disease, 188. See Spinal caries. 
Pregnancy, apoplexy and, 423, 425 
arthritis deformans and, 902 
chorea and, 777, 779 
myasthenia gravis and, 799 
osteomalacia and, 948 
progressive spinal muscular atrophy 
and, 81 
Presbyopia, 36 
Pressure sense of, 19, 32 
Professional cramps, 741 
neuroses, 741 
Progressive bulbar palsy, 113 
muscular dystrophy, 117 
diagnosis of, from progressive 
muscular atrophy, 86 
spinal muscular atrophy, 82 
of childhood, 87 
Propulsion, 74 
Protopathic sensibility, 17 
Psammoma of brain, 318 
Psammosarcoma of brain, 315 
Pseudo-achondroplasia, 969 
Pseudo-bulbar paralysis, 290, 798 1020 
INDEX TO VOLUME V 
Pseudo-hallucinations, 37 
Pseudo-hypertrophic progressive muscular 
dystrophy, 122 
Pseudo-paralytica, 798 
Pseudo-paresis, alcoholic, diagnosis of, from 
paresis, 860 
syphilitic, diagnosis of, from paresis, 
860 
Pseudo-sclerosis, 158 
diagnosis of, 159 
epileptic attacks in, 159 
etiology of, 158 
nystagmus in, 159 
pain in, 159 
pathology of, 158 
reflexes in, 159 
speech in, 159 
symptoms of, 159 
tremors in, 159 
weakness in, 159 ' 
Pseudo-tabes, hysteria and, 680 
syphilitica, 826 
Pseudo-tetany, hysterical, diagnosis of, 
from tetany, 760 
Psychic blindness, 299 
disturbances in brain tumor, 329, 342 
in migraine, 719 
epilepsy, 602 
factors in occupation neuroses, 742 
influences, epilepsy and, 597 
manifestations in chorea, 780 
neuralgia, 726 
symptoms of Huntington's chorea, 789 
Psychoses in hysteria, 689 
post-epileptic, 603 
traumatic, 642 
'diagnosis of, 648 
prognosis of, 652 
Ptosis in cerebrospinal syphilis, 825 
in hemiplegia, 459 
in hysteria, 679 
in ocular paralysis, 488 
in paralyzing vertigo, 515 
Pudendal plexus, neuralgia of, 740 
Pulmonary nerves, paralysis of, 523 
Pulse in abscess of brain, 380 
in acute meningitis, 170 
in apoplexy, 435 
in arthritis deformans, 909, 914 
in cervical ribs, 556 
Pupillary reaction, hemianopic, 482 
of Wernicke, 271 
reflexes, anomalies of, 76 
Pupils in apoplexy, 439 
in disseminated sclerosis, 152 
in general paresis, 856 
in tabes dorsalis, 837 
variations of, 487 
Pyorrhoea alveolaris, arthritis deformans 
and, 901 
Pyramidal tracts in amyotrophic lateral 
sclerosis, 90 
Q 
Quadrigeminal bodies, lesion of, 282 
hearing in, 282 
Quadrigeminal bodies, lesion of, ophthal- 
moplegia in, 283 
vision in, 282 
tumor of, 282 
R 
Radial nerve, paralysis of, 547 
Raynaud's disease, arthritis deformans 
and, 929 
Reaction, anomalies of mode of, 48 
of degeneration, 72 
in amyotrophic lateral sclerosis, 
99 
distal, 535 
myasthenic, 73 
myotonic, 73, 891 
Recording faculty, examination of, 45 
Rectal reflex in lesions of spinal cord, 
182 
Rectus muscles, external, paralysis of, 485 
inferior, paralysis of, 485 
internal, paralysis of, 485 
superior, paralysis of, 485 
Reflex hypothesis of migraine, 710 
irritation, convulsions and, 792 
neuralgia, 726 
Reflexes in amaurotic family idiocy, 869 
in amyotrophic lateral sclerosis, 97 
in apoplexy, 439 
in arthritis deformans, 917 
in chorea, 781 
in diseases of peripheral nerves, 534 
in disseminated sclerosis, 149 
in general paresis, 857 
in hemiplegia, 465 
in hysteria, 688 
in lesion of cerebellum, 286 
in multiple neuritis, 584 
in myasthenia gravis, 805 
in myotonia congenita, 892 
in neurasthenia, 632 
in osteomalacia, 951 
in paralysis agitans, 771 
in periodic paralysis, 814 
in progressive bulbar palsy, 116 
neural muscular atrophy, 110 
spinal muscular atrophy, 85 
in pseudo-sclerosis, 159 
in spondylitis deformans, 927 
in tabes dorsalis, 837 
in tetany, 759 
Reproduction, anomalies of, 53 
Resistance, sensation of, 17 
Respiration in abscess of brain, 380 
in apoplexy, 436 
in hemiplegia, 460 
in Paget's disease, 976 
Retina, lesions of, 474 
Retinitis, 474 
albuminuric, 475 
syphilitic, 475 
Retrobulbar neuritis, 37, 479 
Retropulsion, 74 
Rhachitis annularis, 978 
Rheumatic fever, chorea and, 775 INDEX TO VOLUME V 
1021 
Rheumatic fever, diagnosis of, from ar- 
thritis deformans, 930 
Rheumatoid arthritis, 897, 908 
Rhinogenic abscess of brain, 385 
Ribs in achondroplasia, 957 
cervical, 554 
Rickets, achondroplasia and, 966 
annular, 955 
congenital, 955 
convulsions and, 792, 793 
diagnosis of, from hydrocephalus, 355 
fetal, 955 
intra-uterine, 955 
micromelic, 955 
Rigidity in compression of spinal cord, 190 
Rinne's test in nerve deafness, 510 
Romberg symptom in tabes dorsalis, 838 
Rupture of cerebral arteries, 400 
Rythmic movements in hysteria, 676 
S 
Sacral cord, disease of, diagnosis of, from 
paralysis of nerves, 561 
plexus, diseases of, diagnosis of, from 
paralysis of nerves, 561 
paralysis of, 560 
Sacro-iliac disease, diagnosis of, from 
sciatica, 565 i 
joint in arthritis deformans, 912, 923 
Sacrum, disease of, diagnosis of, from 
sciatica, 565 
Sadness, pathological, 60 
St. Vitus' dance, 774 
Salivation in migraine, 718 
Salvarsan in cerebrospinal syphilis, 830 
intraspinous, 831, 862 
Salvarsanized serum, 831, 862 
Sarcoma of brain, 313, 314, 315 
of spinal cord, 197 
Sarcomatosis, diagnosis of, from syphilis, 
827 
Scanning speech, 74 
Scapular reflex, 78 
Scapulodynia, 945 
Scarlet fever, apoplexy and, 417 
multiple neuritis and, 575 
Schwabach's test, 24 
Sciatic nerve, paralysis of, 558, 560 
in spondylitis deformans, 927 
Sciatica, 562 
diagnosis of, 565 
from disease of cauda equina, 565 
of sacrum, 565 
from hip disease, 565 
from intermittent claudication, 
565 
from pelvic tumors, 565 
from sacro-iliac disease, 565 
from tabes dorsalis, 565 
etiology of, 562 
fever in, 563 
Lasdgue's sign in, 563 
organic, diagnosis of, from sciatic neu- 
ritis, 565 
pain in, 562 
Sciatica, paresthesia in, 563 
prognosis of, 565 
simple, diagnosis of, from sciatic neu- 
ralgia, 565 
symptoms of, 562 
treatment of, 566 
Scleroderma, arthritis deformans and, 929 
Sclerosis, amyotrophic lateral, 89 
bulbar changes in, 92, 100 
contractures in, 99 
diagnosis of, 101 
from progressive spinal 
muscular atrophy, 86 
duration of, 94 
etiology of, 89 
frequency of, 90 
lead and, 89 
mental failure in, 99 
muscles in, 100 
pain in, 99 
pathology of, 90 
prognosis of, 101 
pyramidal tracts in, 90 
reflexes in, 98 
reaction of degeneration in, 
99 
symptoms of, 93 
syphilis and, 90 
treatment of, 101 
tremors in, 98 
types of, 95 
amyotrophic, 95 
labio-glosso-laryngeal, 
95 
ordinary, 95 
spastic, 96 
weakness in, 97 
of brain, 140 
of cerebellum, 287 
combined, diagnosis of, from lateral 
sclerosis, 104 
from tabes dorsalis, 845 
diffuse, 160 
disseminated, 140 
age and, 141 
ataxia in, 147, 154 
Babinski's sign in, 149 
cerebral, 154 
cerebrospinal, 154 
classical, 154 
course of, 157 
defecation in, 150 
diagnosis of, 154 
from brain tumor, 343 
from cerebrospinal syphilis, 
156 
from Friedreich's ataxia, 138 
from general paresis, 156, 860 
from hysteria, 155, 692 
from lateral sclerosis, 104 
from myelitis, 155 
from paralysis agitans, 772 
from spastic paraplegia, 156 
from subacute combined de- 
generation, 155 
from syphilis, 827 
from tabes dorsalis, 845 1022 
INDEX TO VOLUME V 
Sclerosis, disseminated, diagnosis of, from 
tumor of brain, 156 
diplopia in, 150 
emotion and, 143 
epileptic attacks in, 153 
etiology of, 141 
frequency of, 140 
gait in, 148 
heredity and, 142 
histology of, 144 
hypertonicity in, 147 
hysterical, 154 
infections and, 141 
intention tremor in, 148 
intermittent, 154 
metallic poisons and, 142 
micturition in, 150 
nystagmus in, 151 
ocular, 154 
muscles in, 151 
onset of, 154 
optic, 154 
disks in, 151 
pain in, 149 
pathogenesis of, 145 
pathology of, 143 
prognosis of, 156 
pupil in, 152 
reflexes in, 149 
sacral, 154 
speech in, 152 
spinal, 154 
symptoms of, 147 
mental, 153 
motor, 147 
sensory, 148 
syphilis and, 142 
-trauma and, 142 
treatment of, 157 
trigeminal neuralgia in, 733 
trophic changes in, 153 
types of, 154 
vasomotor changes in, 153 
vision in, 150 
weakness in, 147, 154 
insular, 140 
lateral, 101 
diagnosis of, 104 
from caries of spine, 104 
from combined sclerosis, 104 
from hysteria, 104 
from multiple sclerosis, 104 
from myelitis, 104 
from secondary degenera- 
tion, 104 
from tumors of spinal cord, 
104 
pathology of, 101 
spasticity in, 102 
symptoms of, 102 
treatment of, 104 
types of, 103 
weakness in, 102 
miliary, 161 
multiple, 140 
posterior spinal, 833. See Tabes 
dorsalis. 
Sclerosis, subacute combined, of cord, 126 
anemia and, 128 
ataxic paraplegia in, 127 
diagnosis of, 129 
from tabes, 129 
duration of, 129 
etiology of, 126 
fever in, 129 
flaccid paraplegia in, 128 
onset of, 128 
pathology of, 126 
prognosis of, 130 
spastic paraplegia in, 
128 
spasticity in, 127 
symptoms of, 127 
treatment of, 130 
tuberous, 160 
Scotoma, 34 
Scotomata in migraine, 712, 714 
Secretions in migraine, 718 
Secretory disturbances in hysteria, 687 
in neuralgia, 728 
in syringomyelia, 224 
functions, anomalies of, 79 
Senile dementia, trauma and, 647 
Sensation, deep, 536 
disturbances of, 28 
epicritic, 537 
protopathic, 537 
Sense of acceleration, 18 
of equilibrium, 18 
exterior, 19 
of fatigue, 19, 32 
of hearing, 22 
of hunger, 19, 31 
interior, 17 
of itching, 20 
of movement, 30 
muscle, 17 
pain, 17 
anomalies of, 29 
perception, 18, 30 
of position, anomalies of, 30 
of pressure, 19, 32 
of resistance, 17, 30 
sexual, 19, 32 
of sight, 24 
of smell, 21, 33 
of taste, 21, 33 
temperature, 20, 32 
of thirst, 19, 32 
of tickling, 20 
of touch, 19, 32 
vestibular, 18, 30 
vibration, 18, 30 
Sensibility, deep, 17, 184 
anomalies of, 30 
disturbances of, in lesions of the 
spinal cord, 183 
epicritic, 17, 184 
mesoblastic, 17 
anomalies of, 30 
muscular, in tabes dorsalis, 839 
osseous, 30 
protopathic, 17, 184 
stereognostic, 18, 30 INDEX TO VOLUME V 
1023 
Sensory aphasia, 74, 292, 296 
area of cortex of brain, 265 
disturbances in occupation neuroses, 
747 
phenomena in tetany, 759 
Sexual reflex in spinal cord lesions, 182 
sense, 19, 32 
Shaking palsy, 764 
Shoulder-joint in arthritis deformans, 911 
Sick headache, 709 
Sight, anomalies of, 34 
sense of, 24 
Simulation, in hysteria, 659, 667 
Sinus thrombosis, brain abscess and, 378 
diagnosis of, from brain abscess, 
386 
from encephalitis, 374 
Skin in arthritis deformans, 916 
in diseases of peripheral nerves, 540 
in facial hemiatrophy, 992 
in herpes zoster, 572 
in hysteria, 686 
in neuralgia, 728 
in polymyositis hemorrhagica, 879 
in syringomyelia, 224 
in trigeminal neuralgia, 734 
Skull in achondroplasia, 956 
hyperostosis of, 988 
in Paget's disease, 971 
Smallpox, multiple, neuritis and, 575 
Smell, anomalies of, 33 
centre of, 271 
disturbances of, 471 
diagnosis of, 472 
etiology of, 471 
pathology of, 472 
prognosis of, 473 
symptoms of, 472 
in hysteria, 684 
sense of, 21 
anomalies of, 33 
Softening, central, onset of, 431 
Somatopsyche, 42 
Somnambulisms, monoideic, 44 , 
polyideic, 44 
"Soul paralysis," 268 
Spasm, habit, 784 
of muscles of neck, 525 
of tongue, 532 
phonic, 522 
Spasmodic torticollis, 526 
Spasmophilia, 604 
Spastic conjugate deviation in apoplexy, 
438 
dysphonia, 522 
spinal paralysis, 101 
hereditary, 105 
syringomyelia, 220 
tabes, 101 
Spastic-paretic gait, 73 
Speech, anomalies of, 74 
in disseminated sclerosis, 152 
in epilepsy, 612 
in general paresis, 856 
in lesion of centrum semiovale, 275 
in migraine, 715 
in progressive bulbar palsy, 115 
Speech in pseudo-sclerosis, 159 
Sphincters in multiple neuritis, 584 
Spina bifida, hydrocephalus and, 354 
Spinal accessory nerve, paralysis of, 524 
ataxic gait, 73 
caries, 188 
diagnosis of, from lateral scler- 
osis, 104 
from tumors, 208 
dry, 189 
etiology of, 188 
pathology of, 188 
cord, abscess of, 254 
anemia of, 255 
carcinoma of, 196 
diagnosis of, from caries, 193 
cavities of, 210 
combined system, diseases of, 125 
compression of, 188 
from caries, anesthesia in, 191 
course of, 192 
deformity in, 190 
diagnosis of, 192 
from congenital ab- 
normalities, 193 
from functional par- 
aplegia, 194 
from syphilitic men- 
ingomyelitis, 193 
from syringomyelia, 
193 
extension of, 195 
fever in, 192 
onset of, 192 
pain in, 190 
prognosis in, 194 
prophylaxis in, 194 
rest in, 195 
rigidity in, 190 
root pains in, 191 
symptoms of, 190 
treatment of, 194 
operative, 195 
weakness in, 191 
congenital abnormalities of, diag- 
nosis of, from caries, 193 
cysts of, diagnosis of, from 
tumors, 208 
diseases of, 178 
focal, differentiation of, 187 
embolism in, 256 
fissures of, 210 
hemorrhage into, 260 
. hyperemia of, 257 
impulses in, 184 
lesions of, 178, 180 
Brown-Sequard paralysis in, 
185 
, extramedullary, 187 
flaccid paralysis in, 178 
intramedullary, 187 
oculopupillary symptoms 
and, 181 
optic neuritis in, 181 
pain in, 183 
paralysis in, 178, 179 
reflexes in, 182 1024 
INDEX TO VOLUME V 
Spinal cord, lesions of, secretory pheno- 
mena in, 181 
sensibility in, 183 
spastic paralysis in, 179 
symptoms of, cardiac, 182 
respiratory, 182 
splanchnic, 182 
vasomotor phenomena in, 
181 
myeloma of, 198 
sacral, diseases of, diagnosis of, 
from paralysis of nerves, 561 
sarcoma of, 197 
sclerosis of, combined, caused by 
vegetable poisons, 139 
segment of, as centre of activity, 
180 
segmental localization of, 178 
spinal root symptoms of, 180 
syphilis of, 823. See Syphilis, 
cerebrospinal. 
thrombosis in, 256 
tumors of, 196 
Brown-Sequard paralysis in, 
204 
cauda equina, 206 
cervical enlargement, 204 
course of, 206 
diagnosis of, 207 
from caries, 208 
from cervical pachymen- 
ingitis, 208 
from cysts, 208 
from lateral sclerosis, 
104 
from tabes dorsalis, 845 
dorsal, 205 
- intravertebral, 198 
extradural, 198 
incidence of, 200 
intradural, 198 
intramedullary, 199 
pathology of, 199 
lumbosacral, 205 
pain in, 201, 202 
paraplegia in, 203 
prognosis of, 209 
result of, 206 
site of, 201 
symptoms of, 201 
sensory, 203 
spinal cord, 203 
root, 202 
vertebral, 201 
treatment of, 209 
of upper cervical region, 204 
vertebral, 196 
benign, 198 
vascular disorders of, 255 
curvature in syringomyelia, 222 
disseminated sclerosis, 154 
membranes, hemorrhage into, 257 
meningitis, localized serous, 174 
muscular atrophy, progressive, 82, 87 
nerves, diseases of, 542 
paralysis, spastic, 101 
hereditary, 105 
Spinal paralysis, syphilitic, 828 
roots, diseases of, 567 
diagnosis of, 568 
onset of, 567 
symptoms of, 567 
treatment of, 568 
sclerosis, posterior, 833. See Tabes 
dorsalis. 
Spine in Paget's disease, 971 
Spirochaeta pallida in general paresis, 
851, 852 
in nervous system, 823 
in tabes dorsalis, 835 
Spleen in acute infective polyneuritis, 590 
in arthritis deformans, 909, 915 
Spondylitis deformans, 923 
age and, 925 
atrophy in, 927 
diagnosis of, 928 
from Paget's disease, 977 
etiology of, 925 
occurrence of, 925 
pain in, 925, 926 
reflexes in, 927 
sciatic nerve in, 927 
signs of, 926 
symptoms of, 924 
tendo-Achilles reflex in, 927 
trauma and, 925 
x-rays in, 927. 
Stammering, 74 
Staphylococcus pyogenes aureus in pyo- 
genic encephalitis, 363 
Status epilepticus, 609 
hemicranus in migraine, 718 
Stereognosis, anomalies of, 30 
Stereognostic sense, 18 
Sternoclavicular joint in arthritis defor- 
mans, 911 
Still's disease, 917, 931 
Strabismus in ophthalmoplegia, 484 
Stupor, 47, 63, 65 
Stuttering, 74 
Subacute combined sclerosis of spinal 
cord, 126 
Subcutaneous nodules in arthritis defor- 
mans, 915 
Sunstroke, diagnosis of, from apoplexy, 
444 
Superior pontile syndrome, 291 
Suprascapular nerve, paralysis of, 545 
Sydenham's chorea, 774 
Syndrome, Benedikt's, 369 
cerebellar deficit, 287 
Millard-Gubler, 369 
pontile, 289 
superior, 291 
tegmental, 281 
thalamic, 279, 334, 467 
Weber's, 369 
Synesthesia, 29 
Synovial membrane in arthritis defor- 
mans, 904, 905 
Syphilis, achondroplasia and, 967 
amyotrophic lateral sclerosis and, 90 
apoplexy and, 423, 425, 426, 427 
arteriosclerosis of brain and, 393 INDEX TO VOLUME V 
1025 
Syphilis of brain, 823. See Syphilis, cere- 
brospinal. 
cerebral aneurism and, 399 
thrombosis and, 400, 401 
cerebrospinal, 823, 824 
deafness in, 825 
diagnosis of, 827 
from carcinomatosis, 827 
from disseminated sclerosis, 
156, 827 
from sarcomatosis, 827 
from tabes, 826 
from tuberculosis, 827 
endarteritis in, 824 
etiology of, 824 
headache in, 825 
meninges in, 824 
ophthalmoplegia in, 825 
paralysis in, 825 
paraplegia in, 825 
ptosis in, 825 
salvarsan in, 830 
symptoms of, 825 
treatment of, 830 
infraspinous, 831 
vomiting in, 825 
Wassermann reaction in, 826, 827 
chorea and, 775, 777 
epilepsy and, 595 
general paresis and, 850 
hereditary, hemiplegia in, 418 
myelitis and, 228 
of nervous system, 822 
neuralgia and, 727 
neurasthenia and, 629 
in ocular paralysis, 488 
osteitis deformans and, 973 
osteo-arthropathy and, 982 
postnatal infantile hemiplegia and, 418 
of spinal cord, 823. See Syphilis, 
cerebrospinal. 
syringomyelia and, 212 
tabes dorsalis and, 833 
Syphilitic choroidoretinitis, 475 
meningomyelitis, diagnosis of, from 
tabes dorsalis, 845 
myelitis, 237, 828 
paraplegia, Erb's, 246, 828 
pseudoparesis, diagnosis of, from 
paresis, 860 
retinitis, 475 
spinal paralysis, 828 
Syphiloma of brain, 320, 321 
Syringomyelia, 211 
age and, 211 
amyotrophic, 220 
analgesia in, 223 
anesthesia in, 223 
bones in, 225 
bulbar symptoms in, 222 
cavity in, 212 
central canal in, 214 
congenital anomalies and, 211 
definition of, 211 
diagnosis of, 226 
from caries of spinal cord, 193 
from hematomyelia, 263 
vol. v-65 
Syringomyelia, diagnosis of, from hysteria, 
693 
from progressive muscular 
atrophy, 226 
from spinal muscular atrophy, 86 
from tabes dorsalis, 845 
etiology of, 211 
gliomatosis in, 213 
heredity and, 211 
incoordination in, 221 
joints in, 225 
Morvan's type, 220 
nervous elements in, 214 
nystagmus in, 222 
onset of, 220 
paralysis in, atrophic, 221 
ocular sympathetic, 224 
spastic, 221 
pathogenesis of, 215 
pathology of, 212 
prognosis of, 227 
secretory disturbances in, 224 
sensation in, 222 
skin in, 224 
spastic, 220 
sphincters in, 226 
spinal curvature in, 222 
symptoms of, 218 
syphilis and, 212 
thermo-anesthesia in, 222 
trauma and, 211 
treatment of, 226 
tremors in, 222 
trophic disturbances in, 224 
urticaria in, 224 
vascular lesions in, 214 
vasomotor disturbances in, 224 
T 
Tabes amaurotica, 844 
atactica, 844 
dolorosa, 844 
dorsalis, 833 
Achilles tendon reflex in, 837 
age and, 834 
allochiria in, 838 
■ anesthesia in, 838 
Argyll-Robertson pupil in, 836 
arthropathy in, 842 
astereognosis in, 838 
ataxia in, 839 
atrophy of optic nerve in, 841 
bladder in, 840 
cardiac crises in, 842 
course of, 843 
cranial nerves in, 841 
definition of, 833 
diagnosis of, 844 
from brain tumor, 845 
from cerebrospinal syphilis, 
826 
from combined sclerosis, 845 
from Friedreich's ataxia, 138, 
845 
from multiple neuritis, 845 1026 
INDEX TO VOLUME V 
Tabes dorsalis, diagnosis of, from multiple 
sclerosis, 845 
from neuralgia, 730 
from neuritis, 587 
from sciatica, 565 
from spinal tumor, 845 
from subacute combined 
sclerosis, 129 
from syphilitic meningo- 
myelitis, 845 
from syringomyelia, 845 
duration of, 843 
etiology of, 833 
facial nerves in, 841 
gastric crises in, 841, 849 
hypalgesia in, 838 
hyperesthesia in, 836, 838 
intestinal crises in, 842 
knee-jerks in, 837 
migraine and, 720 
muscular hypotonia in, 839 
sensibility in, 839 
mydriasis in, 837 
neuralgia and, 727 
neurasthenia in, 843 
oculomotor palsies in, 841 
ophthalmoplegia in, 841 
pain in, 836 
paresis and, 843 
pathology of, 834 
prognosis of, 846 
prophylaxis in, 847 
pupils in, 837 
Romberg symptom in, 838 
sensation in, 838 
Spirochaeta pallida in, 835 
superficial reflexes in, 837 
symptoms of, 835 
syphilis and, 833 
treatment of, 847 
trigeminal nerve in, 841 
neuralgia in, 733 
trophic disorders in, 841 
visceral crises in, 841 
Wassermann reaction in, 847 
incompleta, 844 
juvenilis, 844 
paralytica, 844 
paresthetica, 844 
spastic, 101 
superior, 840, 844 
visceralis, 844 
Taste in hysteria, 684 
sense of, 21 
Tay-Sachs disease, 863 
Tegmental syndrome, 281 
Tegmentum, lesion of, 281 
paralysis in, 281 
syndrome of, 281 
tumors of, 336 
Temporal lobe, tumors of, 331 
Temporomaxillary joint in arthritis de- 
formans, 911 
Tendo-Achilles reflex in spondylitis de- 
formans, 927 
Teratoma of brain, 316 
Test., association, 48 
Testicular neuralgia, 740 
Tetany, 748 
benign, 759 
calcium and, 751 
Chvostek's sign in, 758 
course of, 759 
definition of, 748 
diagnosis of, 760 
from hysterical pseudotetany, 760 
electrical hyperexcitability in, 757 
epidemic, 750, 762 
Erb's sign in, 757 
etiology of, 750 
gastric disease and, 749 
grave, 760 
hysteria and, 677 
idiopathic, 749 
incidence of, 750 
infectious diseases and, 749 
intestinal disease and, 749 
laryngospasm in, 756 
maternity and, 749 
mechanical irritability in, 758 
mental phenomena in, 758 
motor nerves in, 757 
muscle cramps in, 754 
nervous disease and, 749 
osteomalacia and, 949 
pain in, 757, 759 
parathyreopriva, 761 
parathyroids and, 749, 750, 753 
pathology of, 753 
poisoning and, 749 
prognosis of, 761 
reflexes in, 759 
sensory phenomena in, 759 
severe, 760 
sight in, 759 
strumipriva, 761 
symptoms of, 754 
treatment of, 762 
trophic phenomena in, 759 
Trousseau's phenomena in, 756 
Thalamic syndrome, 279, 334, 467 
Thermanesthesias, 32 
Thermo-anesthesia in syringomyelia, 222 
Thirst, sense of, 19, 32 
Thomsen's disease, 886 
Thoracic nerve, long, paralysis of, 544 
Thought inhibition, 47 
Thrombosis, apoplexy and, 423, 425 
of cerebral arteries, 400 
diagnosis of, from tumor, 343 
in cervical ribs, 556 
of spinal cord, 256 
Thumb in arthritis deformans, 922 
Thymus gland, convulsions and, 794 
in myasthenia gravis, 799 
Thyroid gland, achondroplasia and, 965 
in osteomalacia, 948 
Tibial nerve, posterior, paralysis of, 559 
Tic convulsif, 504, 784 
etiology of, 504 
treatment of, 505 
douloureux, 493 
Tinnitus aurium, 508 
diagnosis of, 509 INDEX TO VOLUME V 
1027 
Tinnitus aurium, treatment of, 509 
in vestibular disease, 511 
Toes in arthritis deformans, 913 
Tone deafness in aphasia, 297 
Tongue, atrophy of, 531 
in hemiplegia, 460 
muscles of, spasm of, 532 
paralysis of, 530 
in hysteria, 679 
Tonsillitis, arthritis deformans and, 901 
Tonus of muscles, 68 
Topalgia in hysteria, 683 
Topical diagnosis of diseases of brain, 265 
Torticollis, 526, 945 
spasmodic, 526 
etiology of, 526 
pain in, 528 
pathology of, 528 
prognosis of, 528 
symptoms of, 528 
treatment of, 529 
Touch, sense of, 19, 32 
Toxemia, apoplexy and, 425 
Toxic hypothesis of migraine, 710 
myelitis, 236 
Toxins, epilepsy and, 594 
myasthenia gravis and, 800 
neuralgia and, 726 
Trauma, acute mania and, 647 
apoplexy and, 424 
arthritis deformans and, 901 
diagnosis of, from apoplexy, 444 
disseminated sclerosis and, 142 
epileptic insanity and, 647 
general paresis and, 647 
hysteria and, 657 
hysterical insanity and, 647 
paralysis agitans and, 764 
paranoia and, 647 
senile dementia and, 647 
spondylitis deformans and, 925 
syringomyelia and, 211 
villous arthritis and, 929 
Traumatic abscess of brain, 382 
hysteria, 644 
lumbago, 644 
mental disturbances, 646 
neurasthenia, 643 
neuroses, 642 
psychoses, 642 
Tremor, 70 
in amyotrophic lateral sclerosis, 98 
in brain tumor, 340 
in general paresis, 856 
in hemiplegia, 465 
in hysteria, 676 
in paralysis agitans, 768 
in progressive spinal muscular 
atrophy, 85 
senile, diagnosis of, from paralysis 
agitans, 773 
in syringomyelia, 222 
Trichiniasis, diagnosis of, from neuritis,587 
Trigeminal nerve, anatomy of, 491 
diseases of, 491 
pathology of, 492 
symptoms of, 493 
Trigeminal nerve, diseases of, taste and, 
493 
tic douloureux in, 493 
maxillary branches, 491 
motor paralysis of, 494 
ophthalmic branches, 491 
paralysis of, 493, 494 
in tabes dorsalis, 841 
trismus and, 495 
neuralgia, 733 
Trismus, 495 
Trochlear nerve, anatomy of, 483 
Trophic changes in disseminated sclerosis, 
153 
in general paresis, 858 
in hysteria, 685 
in migraine, 718 
in multiple neuritis, 584 
in syringomyelia, 224 
in tabes dorsalis, 841 
functions, anomalies of, 79 
phenomena in tetany, 759 
Trousseau's phenomenon, 71 
in tetany, 756 
Tubercle of brain, solitary, 319 
Tubercula dolorosa, 570 
Tuberculoma of brain, encephalic, 319 
Tuberculosis, cerebrospinal, diagnosis of, 
from syphilis, 827 
multiple neuritis and, 575, 586 
osteo-arthropathy and, 982 
Tuberous sclerosis, 160 
Tumors of basal ganglia, 334 
of brain, 308 
stem, 335 
of cavum Meckelii, 340 
of centrum semiovale, 276, 334 
of cerebellum, 338 
of corpora quadrigemina, 336 
of corpus callosum, 276, 335 
of cranial base, 340 
of crura, 335 
extracerebellar, 338 
of fourth ventricle, 338 
of frontal lobe, 331 
hydrocephalus and, 356 
intracerebellar, 338 
intra vertebral, of spinal cord, 198 
of medulla oblongata, 337 
of nerves, 568 
Paget's disease and, 973 
of paracentral convolution, 332 
of parietal lobe, 333 
of pineal gland, 336 
of pons, 336 
of quadrigeminal bodies, 282 
of spinal cord, 196 
of tegmentum, 336 
of temporal lobe, 331 
vertebral, of spinal cord, 196 
Twilight states, 44 
Twitchings, fibrillary, 70 
Typhoid fever, apoplexy and, 417, 425 
cerebral thrombosis and, 400 
disseminated sclerosis and, 141 
multiple neuritis and, 575 
neurasthenia and, 629 1028 
INDEX TO VOLUME V 
U 
Ulnar nerve, paralysis of, 549 
Uncinate fits, 332 
Unconsciousness, 43 
Unilateral paralysis, 108 
Uremia, diagnosis of, from apoplexy, 441 
Urine in arthritis deformans, 916 
in migraine, 718 
in myasthenia gravis, 805 
in neurasthenia, 632 
in osteomalacia, 952 
in periodic paralysis, 811 
Urticaria in syringomyelia, 224 
Uvular reflex, 78 
V 
Vagal attacks, 523 
Vagus nerve, branches of, cardiac, par- 
alysis of, 522 
gastric, paralysis of, 523 
laryngeal, paralysis of, 518 
oesophageal, paralysis of, 523 
pharyngeal, paralysis of, 517 
pulmonary, paralysis of, 523 
diseases of, 517 
paralysis of, 517 
vagal attacks and, 523 
Valleix's points, 17, 727, 734, 737 
Vascular changes in infective myelitis, 232 
disorders of spinal cord, 255 
lesions, diagnosis of, from brain 
tumor, 343 
Vasomotor changes in disseminated scler- 
osis, 153 
in multiple neuritis, 584 
disturbances in epilepsy, 606 
in general paresis, 858 
in hysteria, 685 
in Landry's paralysis, 251 
in migraine, 718 
in neuralgia, 728 
in syringomyelia, 224 
effects in hemiplegia, 468 
functions, anomalies of, 79 
hypothesis of hysteria, 662 
of migraine, 710 
paresis in Freidreich's ataxia, 136 
Vertebra) in achondroplasia, 957 
Vertebral arteries, anatomy of, 387 
Vertigo, 30, 511 
in abscess of brain, 379, 384 
in anemia of brain, 392 
in arteriosclerosis of brain, 396 
auditory, 512 
aural, 513 
in brain tumor, 329, 338 
in lesion of pons, 290 
in neurasthenia, 631 
paralyzing, 515 
Vesical reflex in lesions of the spinal 
cord, 182 
Vestibular apparatus in hysteria, 684 
nerve, diseases of, 511 
nystagmus in, 511 
tinnitus in, 511 
Vibration sense, 18 
Villous arthritis, 929 
Vision, 480 
acuity of, determination of, 26 
capacity of, determination of, 27 
central, 25 
excentric, 25 
in hysteria, 652 
in lesion of centrum semiovale, 275 
of quadrigeminal bodies, 282 
in migraine, 712, 714 
in neurasthenia, 632 
paracentral, 25 
in paralyzing vertigo, 515 
in tetany, 759 
in traumatic neuroses, 652 
in trigeminal neuralgia, 734 
Visual aphasia, 298 
Voice in paralysis agitans, 771 
Vomiting in abscess of brain, 379 
in acute bulbar myelitis, 368 
meningitis, 170 
in brain tumor, 326, 338 
in cerebral aneurism, 399 
in cerebrospinal syphilis, 825 
in disease of vagus nerve, 517 
in Meniere's disease, 513 
in migraine, 712 
in pachymeningitis interna, 165 
in recurrent oculomotor palsy, 488 
von Graefe's sign in myasthenia gravis, 803 
von Recklinghausen's disease, 571 
W 
Wassermann reaction in cerebrospinal 
syphilis, 826, 827 
in general paresis, 851 
in tabes dorsalis, 847 
Weakness in lateral sclerosis, 102 
in progressive muscular dystrophy, 
120 
spinal muscular atrophy, 84 
Weber's syndrome, 369 
test, 24 
in nerve deafness, 510 
Werdnig-Hoffmann type of spinal mus- 
cular atrophy, 87 
Wernicke's aphasia, 299 
disease, 365 
zone of, 300 
Whooping cough, apoplexy and, 417 
Word amnesia, 76 
blindness, 54, 76, 298 
in abscess of brain, 382 
in aphasia, 298 
deafness, 54, 75, 297 
in aphasia, 297 
Wrist-joint in arthritis deformans, 911 
Writer's cramp, 744 
paralytic, 746 
tremulous, 746 
Writing, anomalies of, 74 
Wryneck, 526 
X 
X-rays in spondylitis deformans, 927 GENERAL INDEX. 
A 
Abdomen in acute enteritis, iii, 330 
peritonitis, iii, 678, 680 
in arteriosclerosis, iv, 464 
in ascites, iii, 662 
in chronic enteritis, iii, 335 
perihepatitis, iii, 491 
peritonitis, iii, 719 
in cirrhosis of liver, iii, 522 
of stomach, iii, 277 
in mesenteric embolism, iii, 436 
thrombosis, iii, 436 
in mucous colitis, iii, 439 
in rickets, ii, 790 
in splanchnoptosis, iii, 753 
in subphrenic pyopneumothorax, iii, 
712 
in tuberculosis of mesenteric glands, 
iv, 744 
of peritoneum, iii, 724 
Abdominal actinomycosis, i, 1039, 1043 
heart, iv, 346 
mediastinal contents, ii, 1109 
pain in amoebic dysentery, ii, 34, 37 
in lobar pneumonia, i, 222 
in typhoid fever, i, 89, 106 
reflexes, anomalies of, v, 78 
Aberrant goitre, iv, 841 
Abortive plague, i, 632 
pneumonia, i, 237 
smallpox, i, 803 
typhoid fever, i, 152 
Abscess in bacillary dysentery, i, 777 
of brain, v, 378 
in amoebic dysentery, ii, 42 
in bronchiectasis, ii, 917 
in pleuritis, ii, 1045 
cervical lymphadenitis and, iv, 733 
of Fallopian tubes, subphrenic abscess 
and, iii, 701 
filarial, ii, 308 
hepatopulmonary, iii, 503 
ischiorectal, in tuberculosis, i, 432 
of kidney, iii, 937 
in bronchiectasis, ii, 917 
of liver, iii, 496 
in amoebic dysentery, ii, 38 
in bronchiectasis, ii, 917 
in typhoid fever, i, 118 
in lobar pneumonia, i, 217 
of lung, ii, 993 
in amoebic dysentery, ii, 43 
in bronchopneumonia, ii, 914 
in influenza, i, 538, 540 
Abscess of lung in lobar pneumonia, i, 250 
in tuberculosis, i, 430 
of mesentery, iii, 696 
metastatic, in gangrene of lung, ii, 991 
of omentum, iii, 696 
of pancreas, iii, 635 
paranephric, iii, 970 
peri-appendicular, iii, 364, 370 
pericolic, iii, 697 
perigastric, iii, 696 
perinephric, iii, 970 
peritoneal, residual, iii, 698 
peritonsillar, i, 705; ii, 849 
retroperitoneal, iii, 698 
retropharyngeal, i, 706; ii, 846; iv, 
734 
of spinal cord, v, 254 
of spleen in amoebic dysentery, ii, 43 
subphrenic abscess and, iii, 700 
subphrenic, iii, 699 
in lobar pneumonia, i, 253 
thoracic, in tuberculosis, i, 435 
in typhoid fever, i, 101, 148 
Absence of adrenal glands, iv, 781 
of ductus arteriosus Botalli, iv, 420 
of pericardium, iv, 77, 344 
of right superior vena cava, iv, 440 
of spleen, iv, 938, 944 
of thoracic duct, iv, 580 
of thymus, iv, 908 
Absinthism, ii, 425 
Acanthocephali, ii, 296 
Acardia, iv, 350 
Acariasis, ii, 317 
Accessory coronary arteries, iv, 439 
pulmonary arteries, iv, 439 
spleen, iv, 938 
thymus, iv, 908 
Acetone bodies in urine, iii, 826 
in diabetes mellitus, ii, 702 
Achilles tendon reflex, v, 77 
in tabes dorsalis, v, 837 
Acholic stools, iii, 294 
Achondroplasia, v, 955 
Achromates, v, 25 
Achromatopsia, v, 271 
Achrodcytosis, iii, 73 
Achylia in chronic gastritis, iii, 242 
gastrica, iii, 29, 153 
in pernicious anemia, iv, 634 
Acidity, gastric, in cholelithiasis, iii, 576 
in duodenal ulcer, iii, 192, 346 
in gastric ulcer, iii, 181 
Acidosis, ii, 526 
in hepatic disease, ii, 665 
1029 1030 
GENERAL INDEX 
Acne in congenital syphilis, ii, 190 
of mouth, iii, 54 
Acoria, iii, 156 
Acrocyanosis, iv, 985 
Acromegaly, iv, 805 
in exophthalmic goitre, iv, 869 
glycosuria and, ii, 677, 684 
Acroparesthesia, v, 32 
Acroteric scleroderma, iv, 1024 
Actinomyces bovis, i, 1033 
kidney infections and, iii, 952 
Actinomycoma of brain, v, 321 
Actinomycosis, i, 1032 
abdominal, i, 1039, 1043 
of appendix, iii, 386 
cutaneous, i, 1040 
of head, i, 1038, 1043 
of liver, iii, 541 
of mouth, iii, 59 
of myocardium, iv, 145 
of neck, i, 1038, 1043 
of oesophagus, iii, 86 
of pleura, ii, 1050 
thoracic, i, 1039, 1043 
of thyroid gland, iv, 830 
Acts, anomalies of, v, 66 
Adams-Stokes' syndrome, iv, 80 
Addison's disease, i, 370; iv, 786 
in tuberculosis, i, 436 
Adductor laryngeal paralysis, v, 521 
Adenitis, acute cervical, i, 1012 
in diphtheria, i, 701 
in scarlet fever, i, 874 
in carcinoma of stomach, iii, 208 
febrile, i, 636 
gonococcal, i, 761 
in lobar pneumonia, i, 233 
in syphilis, ii, 158 
Adenocarcinoma of thyroid gland, iv, 832 
834 
Adenoids, ii, 840 
diphtheria and, i, 691 
thymus and, iv, 913, 916, 924 
Adenolipomatosis, symmetrical, v, 818 
Adenoma of intestines, iii, 433 
of kidney, iii, 1003 
of lung, ii, 997 
of pancreas, iii, 649 
Adherent pericarditis, iv, 44, 51, 58 
Adiadokokinesis, v, 72 
in brain tumor, v, 339 
Adiposis cerebralis, v, 818 
dolorosa, v, 817 
tuberosa simplex, v, 818 
Adiposity, ii, 761 
Adrenal glands, absence of, iv, 781 
in acromegaly, iv, 808 
Addison's disease and, iv, 787 
in alcoholism, ii, 404 
anemia of, iv, 782 
anomalies of, iv, 781 
atrophy of, iv, 788 
carcinoma of, iv, 784 
chromaffin tissue of, iv, 777 
circulatory disturbances of, iv, 
782 
development of, iv, 777 
I Adrenal glands, diabetes mellitus and, ii, 
682 
extract of, iv, 778 
function of, disordered, iv, 779 
hemorrhage of, iv, 782 
hyperemia of, iv, 782 
hypernephroma of, iv, 784 
hyperplasia of, iv, 781 
hypertrophy of, iv, 781 
in hypopituitarism, iv, 817 
hypoplasia of, iv, 781 
infections of, iv, 781 
inflammation of, iv, 783 
intoxications of, iv, 781 
sarcoma of, iv, 784 
softening of, iv, 783 
syphilis of, iv, 786 
tuberculosis of, i, 369; iv, 786 
tumors of, iv, 784 
insufficiency, iv, 779, 793 
Afebrile endocarditis, iv, 163 
typhoid fever, i, 153 
Affective states, anomalies of, v, 59 
Afferent nerves, respiration and, ii, 813 
Agamodistomum ophthalmobium, ii, 245 
Agamofilaria georgiana, ii, 315 
oculi, ii, 314 
Agamomeris restiformis, ii, 316 
Agenesis of cerebellum, v, 287 
Agglutination, i, 59 
in thrombosis, iv, 527 
Agglutinins, i, 59 
in tuberculosis, i, 313 
in typhoid fever, i, 162 
Agitation, affective, v, 65 
hallucinatory, v, 65 
motor, v, 63 
I Aglossia, iii, 60 
Agnosia, v, 269 
Agraphia, v, 75 
in aphasia, v, 297, 298 
in migraine, v, 715 
Agusia, iii, 43 
Air embolism, iv, 559 
passages, upper, obstruction of, ii, 816 
pathological effects of, ii, 343 
Akinesia algera, v, 816 
Akro-elephantiasis ossea, v, 982 
Albumin in pleural fluid, ii, 1014 
Albuminuria, iii, 794 
in acute infective polyneuritis, v, 590 
nephritis, iii, 862 
in angioneurotic oedema, iv, 1008 
in appendicitis, iii, 371 
in arteriosclerosis, iv, 463 
in ascites, iii, 664 
in chlorosis, iv, 651 
in chronic nephritis, iii, 882, 902 
circulatory disturbances and, iii, 795 
in cirrhosis of liver, iii, 526 
in congenital cyanosis, iv, 337 
in diabetes mellitus, ii, 703 
in diphtheria, i, 701 
febrile, iii, 795, 867 
functional, iii, 795 
gastro-intestinal, ii, 513 
gonococcal, i, 762 GENERAL INDEX 
1031 
Albuminuria in gout, ii, 748 
in hay fever, ii, 864 
in infantile convulsions, v, 795 
in infectious jaundice, i, 1016 
jaundice and, iii, 796 
kidney and, iii, 774 
of pregnancy and, iii, 868 
in lead poisoning, ii, 365 
in leukemia, iv, 666, 672 
in lobar pneumonia, i, 233, 258 
in mercury poisoning, ii, 380 
in mitral insufficiency, iv, 236 
in morphinism, ii, 440 
in nephroptosis, iii, 756 
in purpura rheumatica, iv, 703 
in pyelonephritis, iii, 958 
in Raynaud's disease, iv, 989 
in renal infarction, iv, 564 
in rheumatic fever, i, 998 
in smallpox, i, 807 
in syphilis of kidneys, ii, 180 
in tetanus, i, 578 
in tuberculosis, i, 390, 434 
of kidney, iii, 982 
in typhus fever, i, 930 
Albuminuric bronchitis, ii, 904 
retinitis, iii, 911; v, 475 
Albumoses in carcinoma of stomach, iii, 207 
Albumosuria, iii, 820 
Alcohol, aneurism and, iv, 476 
in protein metabolism, ii, 584 
Alcoholic neuritis, v, 579 
trance, ii, 424 
Alcoholism, ii, 396 
acute hepatitis and, iii, 494 
nephritis and, iii, 866 
yellow atrophy of liver and, iii, 
473 
angioneurotic oedema and, iv, 999 
arteriosclerosis and, iv, 455 
carcinoma of stomach and, iii, 201 
chronic, ii, 422 
nephritis and, iii, 880, 891 
pancreatitis and, iii, 627 
peritonitis and, iii, 717 
cirrhosis of liver and, iii, 508, 536 
encephalitis and, v, 490 
epilepsy and, v, 593, 596 
fatty liver and, iii, 467 
idiocy and, ii, 407 
Korsakow's psychosis and, ii, 406,427 
lobar pneumonia and, i, 206 
myocarditis and, iv, 112 
periodic ii, 423 
syphilitic myelitis and, v, 238 
tuberculosis of peritoneum and, iii, 
721 
typhoid fever and, i, 159 
Aleukemic lymphocytoma, iv, 748 
Alexia, v, 54 
in brain tumor, v, 333 
Alimentary glycosuria, ii, 690 
system in tuberculosis, i, 387 
tract in cardiac insufficiency, iv, 204 
in exophthalmic goitre, iv, 867 
syphilis of, ii, 174 
in typhoid fever, i, 82 
Alimentary tract in uremia, iii, 837 
in whooping cough, i, 660 
Alkali-free diet, ii, 526 
Alkapton substances, ii, 655 
Alkaptonuria, ii, 655, 711; iii, 817 
Allergic phenomena in man, i, 62 
Allergy, immunity and, i, 65 
Allochiria, v, 268 
in tabes dorsalis, v, 838 
Alloxuric bodies, ii, 732 
Alopecia in exophthalmic goitre, iv, 867 
in syphilis, ii, 167 
from x-rays, ii, 336 
Alternation of heart-beat, iv, 96 
Alveolar sarcoma of brain, v, 314 
Amaurosis, v, 34, 480 
in anemia, iii, 836 
in nephritis, iii, 865, 910 
Amaurotic family idiocy, v, 863 
Amblyopia, v, 341, 480 
in lobar pneumonia, i, 263 
Amboceptors, i, 54 
in tuberculosis, i, 314 
Ambulatory plague, i, 632 
typhoid fever, i, 92, 153 
Amimia in aphasia, v, 296 
Amino-acids, ii, 506, 507, 581, 587, 644 
in metabolism, ii, 654, 662 
Amnesia, ii, 439; v, 53, 270 
Amnesic aphasia, v, 54, 74, 298 
Amoeba in carcinoma of stomach, iii, 213 
Ehrenberg, ii, 22, 28 
of human intestine, ii, 18 
lobospinosa, ii, 24 
Amoebic colitis, ii, 17 
dysentery, ii, 17 
typhoid fever and, i, 158 
Amphistoma hominis, ii, 238 
Ampulla of Vater, carcinoma of, iii, 594 
Amyl alcohol, ii, 400 
Amyloid casts, iii, 799 
degeneration, intestinal hemorrhage 
and, iii, 307 
of liver, iii, 469 
of lymph nodes, iv, 746 
of myocardium, iv, 116 
syphilis and, ii, 152 
disease, abscess of lung and, ii, 995 
of intestines, iii, 392 
in tuberculosis, i, 435 
kidney, iii, 933 
liver, ascites and, iii, 658 
spleen, iv, 949 
tumors of tongue, iii, 66 
Amylopsin, iii, 289 
Amyotonia congenita, v, 893 
Amyotrophic lateral sclerosis, v, 89 
syringomyelia, v, 220 
Anabolism, ii, 549 
Anacidity of stomach, iii, 153 
Anacusis, v, 33 
Analgesia, v, 30 
dolorosa, v, 30 
in hysteria, v, 682 
in syringomyelia, v, 223 
Anaphylactic shock, i, 730 
Anaphylaxis, i, 61 1032 
GENERAL INDEX 
Anarthria, v, 74 
Anchylostomiasis, ii, 271 
Anemia in Addison's disease, iv, 791, 794 
of adrenal glands, iv, 782 
in amoebic dysentery, ii, 38, 68 
in amyloid kidney, iii, 935 
aplastic, iv, 644 
purpura hemorrhagica and; iv, 
699 
of brain, v, 392 
brick-makers', ii, 271 
in carcinoma of intestines, iii, 430 
of stomach, iii, 208 
cryptogenic, iv, 619 
gastric ulcer and, iii, 171, 176 
in Hodgkin's disease, iv, 768 
in hysteria, v, 688 
of infancy, iv, 658 
intestinal hemorrhage and, iii, 307 
of kidney, iii, 787 
in leukemia, iv, 665, 671 
miners', ii, 271 
in multiple neuritis, v, 585 
myelophthisic, iv, 645 
neuralgia and, v, 726 
pernicious, iv, 619 
pigmentation of liver and, iii, 
471 
posthemorrhagic, acute, iv, 655 
in Raynaud's disease, iv, 983 
in rickets, ii, 790 
secondary, iv, 654 
chronic, iv, 658 
septic, acute, iv, 658 
of spinal cord, v, 255 
splenic, iv, 954, 957 
secondary anemia and, iv, 658 
symptomatic, iv, 654 
in syphilis, ii, 151, 158 
in tuberculosis, i, 433 
of kidney, iii, 985 
tunnel, ii, 271 
Anergy, i, 62 
Anesthesia, v, 28 
in brain tumor, v, 337 
in compression of spinal cord, v, 191 
in hysteria, v, 680, 681 
of larynx, v, 522 
in migraine, v, 713, 714 
in neuralgia, v, 728 
in occupation neuroses, v, 747 
in peripheral embolism, iv, 568 
thrombosis, iv, 568 
in Raynaud's disease, iv, 987 
in syringomyelia, v, 223 
in tabes dorsalis, v, 838 
Aneurism, iv, 472 
of aorta, abdominal, iv, 509 
arch of, iv, 492 
bronchostenosis and, ii, 921 
descending thoracic, iv, 508 
dilatation, iv, 484 
syphilis and, ii, 183 
thoracic, iv, 519 
arteriovenous, iv, 472, 510 
of abdominal aorta and inferior 
vena cava, iv, 513 
Aneurism, arteriovenous, of aorta and 
pulmonary artery, iv, 513 
and superior vena cava, iv, 
512 
internal, iv, 512 
traumatic, iv, 510 
atheroma and, iv, 477 
blood-pressure and, iv, 478 
bones and, iv, 482 
of cerebral arteries, v, 398 
circumscribed saccular, iv, 472 
cirsoid, iv, 486 
of coronaries, iv, 437 
cylindrical, iv, 479 
dilatation, iv, 472, 484 
dissecting, iv, 472, 487, 489 
healed, iv, 490 
of ductus arteriosus Botalli, iv, 419 
embolism and, iv, 476, 557 
erosion, iv, 472 
false, iv, 472 
fusiform, iv, 479 
of heart, iv, 115, 127, 482 
of hepatic artery, iii, 458 
miliary, hematemesis from, iii, 283 
mural, iv, 483 
mycotic, iv, 476 
parasitic, iv, 472 
sacculated, iv, 479, 492 
of sinus of Valsalva, iv, 492 
true, iv, 472 
of undefended space of interventri- 
cular septum, iv, 369 
of valves of heart, iv, 482 
varicose, iv, 510 
without pulsation, iv, 518 
Aneurismal pouching of fossa ovalis, iv, 
359 
varix, iv, 510 
Anger, v, 61 
Angina abdominis, iv, 464 
Ludwig's, ii, 846; iii, 75 
pectoris, iv, 289 
Angioma of brain, v, 317 
cavernosum of spleen, iv, 550 
chyle, iv, 586 
of lips, iii, 45 
of liver, iii, 546 
of peritoneum, iii, 733 
Angioneuroses, purpura and, iv, 707 
Angioneurotic group, iv, 707 
oedema, iv, 998 
of mouth, iii, 55 
of pharynx, ii, 847 
Anguillula aceti, ii, 315 
Anguilluliasis, ii, 285 
Anguillulina putrefaciens, ii, 294 
Animal parasites, ii, 217 
Anisocoria in aneurism of aorta, iv, 494 
Ankylostomiasis, ii, 271 
Annular rickets, v, 955 
Annulus ovalis, iv, 326 
Anomalies of adrenal glands, iv, 781 
of arch of aorta, iv, 433 
of auriculoventricular cusps, iv, 407 
of biliary ducts, iii, 547 
of conduct, v, 63 GENERAL INDEX 
1033 
Anomalies of coronary arteries, iv, 437 
of ductus arteriosus Botalli, iv, 409 
of gall-bladder, iii, 547 
of ideas, v, 52 
of heart as a whole, iv, 350 
of kidney, iii, 785 
of pericardium, iv, 344 
of pulmonary arteries, iv, 439 
veins, iv, 441 
of reflexes, v, 76 
of semilunar cusps, iv, 400 
of sense of pain, v, 29 
of septum of heart, iv, 352 
of systemic veins, iv, 439 
of thoracic duct, iv, 580 
of thymus, iv, 908 
of tongue, iii, 60 
of will, v, 63 
Anomalous chorda) tendinese, iv, 354 
septa in left auricle, iv, 352 
in right auricle, iv, 352 
in ventricles, iv, 354 
Anomia, v, 76 
Anopsia, v, 34 
Anosmia, v, 33, 471 
Anthracemia, i, 639 
Anthracosis, ii, 976 
hepatic, iii, 470 
Anthrax, i, 639 
Anti-anaphylaxis, i, 62 
Antibodies, i, 46, 47, 49, 50, 51, 313 
Antigen-antibody reactions, i, 51 
Antigens, i, 48 
Antiperistalsis of intestines, iii, 287, 288 
Antitoxins, i, 47, 53 
in diphtheria, i, 54, 715 
in tuberculosis, i, 313 
Antituberculins in tuberculosis, i, 313 
Antityphoid inoculation, i, 176 
Anuria, iii, 841 
Aorta, abdominal, aneurism of, iv, 509 
aneurism of, iv, 492, 493, 508 
bronchostenosis and, ii, 921 
syphilis and, ii, 183 
arch of, aneurism of, iv, 492 
anomalies of, iv, 433 
ascending, aneurism of, iv, 493 
double, iv, 434 
right, iv, 434 
transverse, aneurism of, iv, 493 
atresia of, congenital, iv, 399 
coarctation of, iv, 420 
endocarditis and, iv, 429 
descending thoracic, aneurism of, iv, 
508 
right subclavian artery from, 
iv, 436 
dilatation of, iv, 484 
dynamic, iv, 513 
elasticity of, iv, 34 
embolism of, iv, 565 
hypoplasia of, iv, 431 
aneurism and, iv, 478 
defects of interauricular septum 
and, iv, 359 
intima of, fissure of, iv, 487 
rupture of, iv, 487 
Aorta, rupture of, in coarctation, iv, 426 
stenosis of, congenital, iv, 399 
thrombosis of, iv, 565 
Aortic arches, primitive, development of, 
iv, 329 
atresia, congenital, iv, 398 
insufficiency, iv, 217 
septum, defects of, iv, 374 
development of, iv, 327 
deviation of, iv, 379 
stenosis, iv, 228 
Aortitis, acute, iv, 450 
chronic, circulation in, iv, 25 
in pneumonia, iv, 450 
in rheumatic fever, iv, 450, 475 
syphilitic, ii, 182; iv, 474 
tuberculous, iv, 452 
Aphasia, v, 292 
in abscess of brain, v, 381 
in apoplexy, v, 457 
in arteriosclerosis, iv, 461; v, 397 
in brain tumor, v, 331 
Broca's, v, 295 
motor, v, 75, 292, 295 
optic, v, 54, 299 
in pachymeningitis interna, v, 165 
sensory, v, 75, 292, 296 
subcortical, v, 75 
visual, v, 298 
Aphemia, v, 300 
Aphthae, Bednar's, iii, 52 
Aphthous fever, i, 1025 
stomatitis, iii, 46 
Apical pneumonia, i, 236 
Aplasia, myeloplastic, v, 978 
Aplastic anemia, iv, 644 
Apoplexy, v, 405 
arteriosclerosis and, v, 396, 427 
brain tumor and, v, 419 
cerebral aneurism and, v, 399, 429 
diplegia and, v, 419 
hemorrhages and, v, 410, 411, 
414, 415 
palsies and, v, 410 
chronic nephritis and, v, 427 
diabetes mellitus and, v, 427 
diphtheria and, v, 417 
embolism and, v, 418, 421, 424, 425, 
427 
hemiplegia and, v, 409, 412, 457 
hemorrhages and, v, 422, 424, 427, 
428, 431 
hysteria and, v, 419 
measles and, v, 417 
pancreatic, iii, 610 
porencephaly and, v, 409 
pregnancy and, v, 423, 425 
rupture of aneurism and, v, 429 
scarlet fever and, v, 417 
syphilis and, v, 423, 425, 426, 427 
thrombosis and, v, 423, 425 
typhoid fever and, v, 417, 425 
Appendicitis, iii, 355 
abscess of liver and, iii, 497 
in amoebic dysentery, ii, 44 
catarrhal, iii, 372 
cholelithiasis and, iii, 571 1034 
GENERAL INDEX 
Appendicitis, chronic, iii, 365, 375 
gangrenous, iii, 372 
hyperacidity and, iii, 114 
kidney infections and, iii, 952 
lymphoid tissue and, iii, 361 
mesenteric lymphadenitis and, Iv, 736 
mild, iii, 373 
myocarditis and, iv, 113 
nephroptosis and, iii, 757 
parasites and, iii, 360 
rheumatic fever and, i, 998 
simple, iii, 372 
subphrenic abscess and, iii, 700 
thrombosis of portal vein and, iv, 544 
in tuberculosis, i, 432 
typhoid fever and, i, 159 
Appendicular abscess, pneumothorax and, 
iii, 711 
colic, iii, 373 
Appendix, actinomycosis of, iii, 386 
cystic distension of, iii, 386 
gangrene of, iii, 363 
inflammation of, iii, 355 
mesentery of, iii, 357 
necrosis of, iii, 363 
obliteration of, iii, 366 
palpation of, iii, 371 
polyps of, iii, 386 
sarcoma of, iii, 387 
tuberculosis of, iii, 385 
tumors of, iii, 386 
Apraxia, v, 66, 75, 269 
aphasia and, 299 
in diseases of corpus collosum, v, 276 
Aptyalism, iii, 68 
Arachnoidal cysts of brain, v, 318 
Arari-Duchenne muscular atrophy, v, 80 
paralysis in lead poisoning, ii, 366 
Ardent fever, i, 1011 
Argas miniatus, ii, 317 
persicus, ii, 317 
reflexus, ii, 317 
Argopsylla gallinacea, ii, 325 
Argyll-Robertson pupil, v, 487 
in general paresis, v, 856 
in tabes dorsalis, v, 836 
Argyria, ii, 384 
Arhythmia, cardiac, iv, 29 
in exophthalmic goitre, iv, 861 
in lobar pneumonia, i, 256 
in myocarditis, iv, 133 
in syphilis of myocardium, iv, 143 
Arm in aneurism of aorta, iv, 495 
paralysis of, v, 551, 552 
Arrow-headed eelworms, ii, 288 
Arsenic poisoning, ii, 373 
Arterial spasm, gastric ulcer and, iii, 171 
thrombosis, iv, 537 
trunk, common, iv, 375 
trunks, transposition of, iv, 379 
Arteries in aortic insufficiency, iv, 223, 226 
in arteriosclerosis, iv, 466 
carotid, embolism of, iv, 567 
thrombosis of, iv, 567 
cerebral, aneurism of, v, 398 
obliteration of, v, 400 
rupture of, v, 400 
Arteries, cerebral, thrombosis of, v, 400 
in chronic nephritis, iii, 898, 903 
coronary, in arteriosclerosis, iv, 463 
embolism of, iv, 569 
thrombosis of, iv, 569 
gumma of, ii, 181 
hepatic, embolism of, iv, 569 
peripheral, embolism of, iv, 568 
thrombosis of, iv, 568 
pulmonary, dilatation of, iv, 398 
renal, anomalies of, iii, 786 
retinal, embolism of, iv, 570 
thrombosis of, iv, 570 
in rheumatic fever, i, 989 
subclavian, embolism of, iv, 567 
thrombosis of, iv, 567 
syphilis of, ii, 181 
in typhoid fever, i, 81, 127 
Arteriosclerosis, iv, 452 
aortic insufficiency and, iv, 218 
stenosis and, iv, 218 
apoplexy and, v, 427 
of brain, v, 393 
cardiac insufficiency and, iv, 199 
cerebral aneurism and, v, 399 
thrombosis and, v, 400 
coronary thrombosis and, iv, 569 
diabetes mellitus and, ii, 682 
experimental, iv, 458 
in gout, ii, 742 
hyperpietic, iv, 457 
interstitial nephritis and, ii, 893 
of intestines, iii, 437 
lead poisoning and, ii, 365; iv, 455 
mitral insufficiency and, iv, 232 
neuralgia and, v, 727 
in Paget's disease, v, 973 
peritonitis and, iii, 716 
Raynaud's disease and, iv, 978, 990 
Stokes-Adams' syndrome and, iv, 306 
syphilis and, ii, 162; iv, 454 
toxic, iv, 457 
Arteriosclerotic kidney, iv, 463 
Arteriovenous aneurism, iv, 472, 510 
Arteritis, iv, 449 
Arthracotic cirrhosis of liver, iii, 531 
Arthritis, acute endocarditis and, iv, 155 
in cerebrospinal meningitis, i, 610 
in chickenpox, i, 853 
deformans, v, 895 
atrophic, v, 920 
gout and, v, 930 
hypertrophic, v, 920 
infections and, v, 900, 902, 903 
influenza and, v, 901 
metabolism and, v, 903, 907 
monarticular, v, 919, 934 
peri-articular, v, 908 
Raynaud's disease and, v, 929 
scleroderma and, v, 929 
of special joints, v, 910 
Still's disease and, v, 917, 931 
gonorrhoeal, i, 753, 756 ■ 
in hemophilia, iv, 724 
infectious, v, 896 
in lobar pneumonia, i, 263 
in measles, i, 907 GENERAL INDEX 
1035 
Arthritis in osteo-arthropathy, v, 985 
in purpura hemorrhagica, iv, 712 
in rheumatic fever, i, 990 
•in scarlet fever, i, 878 
in scleroderma, iv, 1018, 1021 
in tuberculosis, i, 504 
in typhoid fever, i, 148 
villous, v, 929 
Arthropathy in syringomyelia, v, 225 
in tabes dorsalis, v, 842 
Ascariasis, ii, 287 
Ascaris lumbricoides, ii, 287 
appendicitis and, iii, 360 
maritima, ii, 288 
texana, ii, 288 
Ascending aorta, aneurism of, iv, 493 
multiple neuritis, v, 576 
Ascites, iii, 657 
chyliform, iii, 661 
chylous, iii, 660; iv, 590 
fatty, iii, 661 
lactescent, iii, 662 
milky, iii, 662 
non-fatty, iii, 662 
Ascitic fluid, iii, 659, 665 
Asepsis in genito-urinary diagnosis, iii, 1029 
Ash-free diet, ii, 526 
Asiatic cholera, i, 672 
clonorchiasis, ii, 230 
Aspergillosis, pulmonary, i, 1058 
Aspergillus fumigatus, i, 1058 
Asphyxia, cardiac insufficiency and, iv, 195 
in enlargement of thymus, iv, 916 
mechanics of, ii, 835 
Aspiration pneumonia, i, 206; ii, 958 
Association tests, v, 48 
Astasia-abasia, v, 815 
hysteria and, v, 816 
Astereognosis, v, 30, 268 
in lesion of optic thalamus, v, 281 
in tabes dorsalis, v, 838 
Asthenic paralysis, v, 798 
pneumonia, i, 236 
Asthenopia, v, 36 
Asthma, ii, 931 
cardiac, iv, 207 
gout and, ii, 933 
hay, ii, 940 
Kopp's, iv, 918 
pregnancy and, ii, 933 
renal, ii, 905 
thymic, iv, 918 
in tuberculosis, i, 430 
Astigmatism, v, 36 
Asyllabia, v, 298 
Asymbolia, v, 54, 269 
Asynergy in brain abscess, v, 381 
tumor, v, 339 
in lesion of cerebellum, v, 286 
Asystole, ventricular, iv, 89 
Ataxia, v, 71 
cerebellar, v, 72 
Marie's hereditary, v, 130, 136 
Friedreich's, v, 130 
Atelectasis in aneurism of aorta, iv, 503 
in lobar pneumonia, i, 248 
tumors of lung and, ii, 1001 
Ateleiosis, iv, 817 
Atheroma, iv, 432 
aneurism and, iv, 477 
in coarctation of aorta, iv, 426 
of coronary arteries, cardiac insuffi- 
ciency and, iv, 195 
Athetoid movements, v, 70 
Athetosis in cerebral diplegia, v, 420 
in lesion of optic thalamus, v, 280 
Athymia, v, 61 
Athyrea, iv, 880 
Athyreosis, iv, 880 
Athyroidism, iv, 880 
Atonic constipation, iii, 313 
Atony of muscle, v, 68 
of stomach, iii, 160 
Atresia of aorta, congenital, iv, 398, 399 
mitral, iv, 406 
pulmonary, iv, 388 
tricuspid, iv, 403 
Atrophic arthritis deformans, v, 920 
diffuse scleroderma, iv, 1016 
Atrophy of adrenal glands, iv, 788 
of cerebellum, v, 287 
in erythromelalgia, iv, 1028 
facial, in migraine, v, 719 
gastric, pernicious anemia and, iv, 623 
of liver, iii, 465 
acute yellow, iii, 472 
muscular, v, 68 
in arthritis deformans, v, 914 
in diseases of peripheral nerves, 
v, 534 
progressive,Charcot-Marie-Tooth 
type, v, 109 
neural, v, 109 
spinal, v, 80 
of childhood, v, 87 
type Duchenne-Aran, v, 80 
Werdnig-Hoffmann, v, 87 
olivopontocerebellar, v, 287 
optic, v, 37, 478 
in osteo-arthropathy, v, 986 
of salivary gland, iii, 74 
in scleroderma, iv, 1019 
of thymus, iv, 910 
of tongue, iii, 66; v, 531 
Attention, anomalies of, v, 45 
Attonnity, v, 65 
Auditory nerve, diseases of, v, 506 
vertigo, v, 512 
Aura in epilepsy, v, 600 
Aural vertigo, v, 513 
Auricle, left, double, iv, 352 
hypertrophy of, iv, .185 
right, hypertrophy of, iv, 184 
Auricles in circulation, role of, iv, 24 
Auricular fibrillation, iv, 86 
flutter, iv, 86 
Auriculo-ventricular bundle, iv, 80 
cusps, anomalies of, iv, 407 
development of, iv, 328 
orifice, defects of, iv, 403 
double, iv, 407 
Auscultation in lobar pneumonia, i, 244 
Auto-inoculation in carcinoma of stomach, 
iii, 201 1036 
GENERAL INDEX 
Auto-intoxication, ii, 503 
Autolysis, ii, 663 
Automatic mechanism in intestinal pro- 
cesses, iii, 291 
Automatism, ii, 424; v, 44 
Autonomic system, iii, 24, 35, 106 
Autopsyche, v, 43 
Axillary glands, lymphadenitis of, iv, 735 
tuberculosis of, iv, 744 
Azotorrhoea in calculus of pancreas, iii, 635 
in disease of pancreas, iii, 602 
B 
Babinski's hypothesis of hysteria, v, 658 
sign, v, 78 
in apoplexy, v, 440 
in cerebrospinal meningitis, i, 606 
in disseminated sclerosis, v, 149 
Bacillary dysentery, i, 766 
Bacillen emulsion, i, 305 
Bacilluria in typhoid fever, i, 142 
Bacillus aerogenes capsulatus in acute 
peritonitis, iii, 676 
appendicitis, iii, 361 
in intestines, iii, 296 
anthracis, i, 561, 641 
bifidus communis in intestines, iii, 296 
Boas-Oppler, iii, 212 
botulinus, ii, 459, 460, 469 
coli in appendicitis, iii, 361 
in diarrhoea, iii, 325 
in fat necrosis, iii, 607 
in intestines, iii, 296 
in jaundice, iii, 448 
in peritonitis, iii, 674, 717 
diphtheria?, i, 689 
in mouth, iii, 40 
dysenteria?, i, 766 
enteritidis, ii, 454, 459, 463, 464 
in diarrhoea, iii, 325 
icteroides, i, 937 
influenzae in bronchopneumonia, ii, 959 
lactis aerogenes in appendicitis, iii, 361 
in intestines, iii, 296 
lepra?, i, 523 
in mouth, iii, 41 
mallei, i, 649 
maximus in mouth, iii, 40 
necrodentalis in mouth, iii, 40 
paratyphosus, ii, 459 
pertussis, i, 656 
pestis, i, 623 
piscicidus, ii, 452 
proteus fluorescens, i, 1015 
in jaundice, iii, 448 
pseudodiphtheria? in mouth, iii, 40 
putrificus in intestines, iii, 296 
pyocyaneus, i, 558 
in acute peritonitis, iii, 675 
in appendicitis, iii, 361 
in diarrhoea, iii, 325 
in mouth, iii, 40 
salmonicida, ii, 452 
tetani, i, 575 
tuberculosis, i, 294 
Bacillus tuberculosis in mouth, iii, 41 
typhosus, i, 77 
in peritonitis, iii, 675, 717 
Bacteria in acute enteritis, iii, 329 
in constipation, iii, 312 
in intestines, iii, 295 
multiple neuritis and, v, 575, 576 
myelitis and, v, 229 
of normal air passages, ii, 883 
pathogenic, i, 18 
in urethra, iii, 943 
Bacteriology of acute cholecystitis, iii, 561 
peritonitis, iii, 674 
of appendicitis, iii, 361 
of ascites, iii, 660 
of biliary infections, iii, 549 
of bronchopneumonia, ii, 959 
of cholelithiasis, iii, 570 
of chorea, v, 778 
of cystitis, iii, 944 
of diarrhoea, iii, 324 
of encephalitis, v, 362 
of gangrenous stomatitis, iii, 49 
of infections of kidney, iii, 947, 950 
of infective myelitis, v, 231 
of intestinal tract, iii, 295 
of Landry's paralysis, v, 248 
of mouth, iii, 39 
of mumps, i, 919 
of purpura, iv, 697 
of pyelitis, iii, 947, 950 
of rheumatic fever, i, 975 
of serofibrinous pleuritis, ii, 1032 
of subphrenic abscess, iii, 706 
of suppurative pancreatitis, iii, 623 
of tuberculosis of peritoneum, iii, 721 
of ulcerative stomatitis, iii, 48 
of urethritis iii, 943, 946 
of urine, iii, 943 
Bacteriolysins in tuberculosis, i, 314 
Bacterioproteins, i, 41 
Bacteriotoxins, i, 34 
Bacterium gingivitidis in mouth, iii, 40, 55 
Bacteriuria, iii, 948 
Baelz's disease, iii, 45 
Bands from chronic peritonitis, iii, 715 
Barbadoes leg, iv, 596 
Basal ganglia, tumors of, v, 334 
Basic meningitis, posterior, i, 615 
Bathyanesthesia, v, 30 
Bathyesthesia, v, 17 
Becquerel's rays, ii, 339 
Bed-bugs, ii, 327 
Bednar's aphthae, iii, 52 
Bence-Jones proteins in urine, iii, 819 
Benedikt's syndrome, v, 369 
Beriberi, ii, 489 
Bertiella satyri, ii, 246, 255 
Bifid apex of heart, iv, 350 
Bile, iii, 289 
Bilharziosis, ii, 239 
Biliary cirrhosis of liver, iii, 535 
ducts, abscess of liver and, iii, 498 
anomalies of, iii, 547 
carcinoma of, iii, 593 
common, obstruction of, iii, 581 
congenital obliteration of, iii, 547 GENERAL INDEX 
1037 
Biliary ducts, extrahepatic, carcinoma of, 
iii, 594 
fistula in cholelithiasis, iii, 584 
tract, digestion and, iii, 34 
infections of, iii, 548 
Bilirubin in liver, iii, 470 
Biliverdin in liver, iii, 470 
Biological hypothesis of hysteria, v, 665 
Biot's respiration, ii, 835 
Birth palsies, brachial, v, 552 
traumatism, epilepsy and, v, 594 
Black tongue, iii, 61 
Blackwater fever, ii, 109 
Bladder in hemiplegia, v, 467 
in renal calculus, iii, 1021 
rupture of peri-appendicular abscess 
into, iii, 365 
in syphilitic myelitis, v, 240 
in tabes dorsalis, v, 840 
tuberculosis of, i, 366 
kidney and, iii, 981 
in typhoid fever, i, 86 
Blastomycetes, i, 1054 
Blastomycosis, i, 1054 
of myocardium, iv, 144 
Bleeding in apoplexy, v, 453 
in hysteria, v, 687 
in purpura iv, 696 
Blind spot, v, 34 
Blindness, v, 34 
in amaurotic family idiocy, v, 869 
cortical, v, 54 
day, v, 480 
in hysteria, v, 684 
in lead poisoning, ii, 368 
mind, v, 53, 271 
night, v, 480 
psychic, v, 299 
in retinal embolism, iv, 571 
thrombosis, iv, 571 
smallpox and, i, 812 
in uremia, iii, 836 
word, v, 54 
Blood in abscess of liver, iii, 502 
in acromegaly, iv, 812 
in acute nephritis, iii, 864 
peritonitis, iii, 683 
yellow atrophy of liver, iii, 476 
in Addison's disease, iv, 793 
in aplastic anemia, iv, 645 
in arthritis deformans, v, 916 
in atypical leukemia, iv, 679 
bacteriology of, i, 559 
in blackwater fever, ii, 115 
in carcinoma of stomach, iii, 204 
cells, enumeration of, iv, 606 
in cerebrospinal meningitis, i, 605 
in chloroma, iv, 682 
in chlorosis, iv, 651 
in cirrhosis of liver, iii, 525, 539 
coagulation of, iv, 719 
color index of, iv, 607 
congenital cyanosis and, iv, 335 
cultures, i, 559 
in diabetes mellitus, ii, 695 
distribution of, in circulatory dis- 
eases, iv, 18 
Blood in echinococcus disease of pleura, ii, 
1068 
in enlargement of thymus, iv, 924 
in exophthalmic goitre, iv, 867 
in feces in amoebic dysentery, ii, 36 
formation of, iv, 603 
in gonorrhoeal arthritis, i, 757 
in gout, ii, 742 
in Hodgkin's disease, iv, 768 
in hysteria, v, 688 
in infantile scurvy, ii, 806 
in infectious diseases, i, 42 
in influenza, i, 545 
in jaundice, iii, 452 
in kala-azar, iv, 972 
in lobar pneumonia, i, 218, 234 
in lymphoid leukemia, iv, 672 
in malarial fever, ii, 88 
mass, movement of, iv, 18 
in measles, i, 905 
in myeloid leukemia, iv, 666 
in myxoedema, iv, 894 
in obesity, ii, 765, 767 
"occult"' tests for, iii, 180 
in osteomalacia, v, 952 
in pellagra, ii, 484 
in pernicious anemia, iv, 635, 639 
in plague, i, 631 
platelets, counting of, iv, 694 
in pleuritis, ii, 1009, 1024, 1043 
in polycythemia, iv, 685 
portal, spleen and, iv, 936 
in posthemorrhagic anemia, iv, 656 
in pseudoleukemia, iv, 681 
in purpura, iv, 693, 699 
renal function and, iii, 770 
in rheumatic fever, i, 989 
in rubella, i, 914 
in scarlet fever, i, 876 
in scleroderma, iv, 1021 
in smallpox, i, 808 
in sporadic cretinism, iv, 885 
in syphilis, ii, 151 
in toxemia, i, 568 
in tuberculosis, i, 382 
of peritoneum, iii, 726 
in typhoid fever, i, 130 
in uremia, iii, 831 
in urine, iii, 802 
in whooping cough, i, 659 
Blood-fluke infection, ii, 239 
Blood-making organs, iv, 611 
in infectious diseases, i, 42 
Blood-pressure in Addison's disease, iv, 
793 
in aneurism of aorta, iv, 498 
arteriosclerosis and, iv, 456 
in cardiac insufficiency, iv, 211 
high, aneurism and, iv, 478 
muscular work and, iv, 456 
in hypertrophy of heart, iv, 186 
in lobar pneumonia, i, 226 
in mitral insufficiency, iv, 235 
in myocarditis, iv, 121 
in nephritis, iii, 903, 904, 921, 927 
in patent ductus arteriosus, iv, 411 
in tuberculosis, i, 385 1038 
GENERAL INDEX 
Blood-pressure in typhoid fever, i, 127 
Bloodvessels in aneurism of aorta, iv, 504 
in chronic nephritis, iii, 884, 896 
congenital cyanosis and, iv, 335 
in goitre, iv, 843 
in jaundice, iii, 453 
syphilis of, ii, 180 
in syphilitic myelitis, v, 238 
tuberculosis of, i, 360, 386 
Blue line in lead poisoning, ii, 363 
Boas-Oppler bacillus, iii, 212 
Boils in diabetes mellitus, ii, 704 
Bone lesions, gonococcal, i, 760 
in typhoid fever, i, 146 
Bone-marrow, iv, 611 
in anemia, iv, 612, 645 
in blackwater fever, ii, 113 
in Gaucher's splenomegaly, iv, 963 
in Hodgkin's disease, iv, 764 
hyperplastic, iv, 612 
in kala-azar, iv, 970 
in malarial fever, ii, 72 
in myeloid leukemia, iv, 663 
in pernicious anemia, iv, 623, 625 
in scarlet fever, i, 866 
in smallpox, i, 790 
in splenic anemia, iv, 956 
tuberculosis of, i, 371 
Bones in achondroplasia, v, 956 
in acromegaly, iv, 807 
aneurism and, iv, 482 
in arthritis deformans, v, 904, 906, 907 
in creatinism, iv, 881, 884 
in exophthalmic goitre, iv, 868 
in facial hemiatrophy, v, 992 
in hypopituitarism, iv, 815 
in myositis ossificans, v, 883, 884 
in osteogenesis imperfecta, v, 979 
in osteomalacia, v, 949 
in Paget's disease, v, 971 
in scleroderma, iv, 1020 
in syringomyelia, v, 225 
Boophilus annulatus, ii, 317 
Botryoid liver, ii, 177 
Botulismus, ii, 460 
Bovovaccines, i, 305 
Bowels, ulceration of, iii, 343 
Brachial neuralgia, v, 737 
neuritis, v, 553 
plexus, paralysis of, v, 550 
Brachiocephalic trunk, common, iv, 437 
Bradycardia, iv, 282 
in hysteria, iv, 283 
in intoxications, iv, 285 
in jaundice, iii, 453 
in melancholia, iv, 283 
myocardial, iv, 282 
in neurasthenia, iv, 283 
in pericarditis, iv, 283 
simple, iv, 82 
in Stokes-Adams syndrome, iv, 305 
in tabes dorsalis, iv, 283 
tachycardia and, iv, 302 
vagus and, iv, 282, 284 
Bradylalia, v, 74 
Brain, abscess of v, 378 
in bronchiectasis, ii, 917 
Brain, abscess of, traumatic, v, 382 
in acromegaly, iv, 808 
actinomycoma of, v, 32 
acute red softening of, v, 359 
in alcoholism, ii, 401 
in amaurotic family idiocy, v, 864 
anemia of, v, 392 
angioma of, v, 317 
arteries of, aneurism of, v, 398 
embolism of, v, 400 
rupture of, v, 400 
thrombosis of, v, 400 
arteriosclerosis of, v, 393 
in blackwater fever, ii, 113 
bloodvessels of, diseases of, v, 387 
carcinoma of, v, 315 
centre of hearing in, v, 271 
of smell in, v, 271 
centrum semiovale, lesion of, v, 275 
cerebellum, lesion of, v, 284 
in cerebrospinal meningitis, i, 597 
chloroma of, v, 315 
cholesteatoma of, v, 316 
chordoma of, v, 317 
convolutions of, lesions of, v, 274 
corpora striata, lesion of, v, 277 
corpus callosum, lesion of, v, 276 
cortex of, motor area of, v, 265, 273 
sensory area of, v, 265, 273 
crura cerebri, lesion of, v, 281 
cysticercus cellulosaj of, v, 318 
cystoglioma of, v, 313 
cystosarcoma of, v, 315 
cysts of, v, 318 
in delirium tremens, ii, 405 
enchondroma of, v, 317 
endothelioma of, v, 313 
epithelioma of, v, 315 
fibroma of, v, 317 
glioma of, v, 311 
granuloma of, infectious, v, 319 
hyperemia of, v, 392 
inflammation of, v, 359 
injuries of, in apoplexy, v, 438 
internal capsule, lesion of, v, 276 
lenticular nucleus, lesion of, v, 278 
zone, lesion of, v, 278 
lipoma of, v, 317 
in malarial fevers, ii, 70 
myeloma of, v, 315 
myxosarcoma of, v, 315 
neuroblastoma of, v, 317 
neurocystoma of, v, 317 
neurofibroma of, v, 314 
neuroglioma of, v, 311 
neuroma of, v, 317 
optic thalamus, lesion of, 279 
osteosarcoma of, v, 314 
papilloma of, v, 315 
in pellagra, ii, 477 
pons, lesion of, v, 289 
pontomedullocerebellar space, lesion 
of, v, 289 
psammoma of, v, 318 
psammosarcoina of, v, 315 
quadrigeminal bodies, lesion of, v, 282 
in Raynaud's disease, iv, 988 GENERAL INDEX 
1039 
Brain, sarcoma of, v, 313, 314 
sclerosis of, v, 140 
stem, tumors of, v, 335 
syphilis of, v, 823 
syphiloma of, v, 320, 321 
tegmentum, lesion of, v, 281 
teratoma of, v, 316 
tuberculoma of, v, 319 
tuberculosis of, i, 370 
tuberculous meningo-encephalitis of, 
v, 319 
tumors of, v, 308 
in basal ganglia, v, 334 
in brain stem, v, 335 
in cavum Meckelii, v, 340 
in centrum semiovale, v, 334 
in cerebellum, v, 338 
in corpora quadrigemina, v, 336 
in corpus collosum, v, 335 
in cranial base, v, 340 
in crura, v, 335 
dermoid, v, 316 
in fourth ventricle, v, 338 
in frontal lobe, v, 331 
hemiplegia and, v, 419 
in hypophysis cerebri, v, 341 
in medulla oblongata, v, 337 
migraine and, v, 720 
in occipital lobe, v, 334 
in paracentral convolutions,, v, 
332 
in parietal lobe, v, 333 
in pineal gland, v, 336 
in pons, v, 336 
in tegmentum, v, 336 
in temporal lobe, v, 331 
trauma and, v, 309 
wet, ii, 415 
Brass poisoning, ii, 386 
Braunia jassyensis, ii, 246, 258 
Break-bone fever, i, 950 
Breast, tuberculosis of, i, 368 
Breath, offensive, iii, 42 
Brick-makers' anemia, ii, 271 
Bright's disease, iii, 778, 845 
Broad tapeworm, ii, 255 
Broca's aphasia, v, 75, 295 
Bromides in epilepsy, v, 620 
Bronchi in aneurism of aorta, iv, 502, 507 
foreign bodies in, ii, 926, 928 
leprosy of, ii, 906 
stenosis of, ii, 820 
syphilis of, ii, 170, 906 
tuberculosis of, i, 348; ii, 905 
Bronchial calculi, ii, 925 
glands in lobar pneumonia, i, 253 
lymphadenitis of, iv, 735 
tuberculosis of, iv, 743 
obstruction, ii, 920 
Bronchiectasis, ii, 913 
abscess of brain and, v, 379 
in aneurism of aorta, iv, 503 
in cerebrospinal meningitis, i, 599 
osteo-arthropathy and, v, 982 
pneumokoniosis and, ii, 977 
pneumothorax and, ii, 1074 
in syphilis of bronchi, ii, 907 
Bronchiectasis in syphilis of trachea, ii, 
907 
tricuspid insufficiency and, iv, 256 
in tuberculosis, i, 430 
in whooping cough, i, 660 
Bronchitis, acute, ii, 881 
albuminuric, ii, 904 
bronchopneumonia and, ii, 887 
capillary, ii, 887, 957 
chronic, ii, 894 
fetid, ii, 911 
fibrinous, ii, 907 
gangrenous, ii, 911 
in gout, ii, 749 
in influenza, i, 537, 540; ii, 902 
in lobar pneumonia, i, 248 
in malarial fevers, ii, 86 
in measles, i, 906; ii, 901 
in mitral insufficiency, ii, 903; iv, 236 
stenosis, ii, 903 
in nephritis, iii, 870, 905 
plastic, ii, 907 
pneumokoniosis and, ii, 977 
pseudomembranous, ii, 907 
purulent, ii, 897 
putrid, ii, 911 
in rickets, ii, 790 
secondary, ii, 901 
serous, ii, 896 
tricuspid insufficiency and, iv, 246 
in tuberculosis, i, 322, 419, 429 
in typhoid fever, i, 121 
in whooping cough, i, 660; ii, 902 
Bronchoblennorrhoea, ii, 897 
Bronchocele, iv, 835 
Broncholiths, ii, 925 
Bronchopneumonia, ii, 957 
in bronchiectasis, ii, 917 
in diphtheria, i, 693, 701; ii, 958 
in fetid bronchitis, ii, 912 
in influenza, i, 537, 540 
in measles, i, 906; ii, 958, 963 
pleuritis and, ii, 964, 1012 
in scarlet fever, i, 864; ii, 958 
in tuberculosis, i, 430; ii, 964 
tuberculous, acute, i, 425 
in typhoid fever, i, 122 
in typhus fever, i, 930 
in whooping cough, i, 660; ii, 958, 963 
Bronchopulmonary lithiasis, ii, 925 
Bronchostenosis, ii, 920 
Bronze diabetes, ii, 681; iii, 529 
Brown induration of lungs, ii, 945 
Brown-Sequard paralysis in lesions of 
spinal cord, v, 185, 204 
Brudzinski's reflex, i, 607 
Bubo, iv, 738 
gonorrhceal, iv, 739 
indolent, iv, 740 
in plague, i, 624 
Bubonic plague, i, 632 
Bulbar changes in amyotrophic lateral 
sclerosis, v, 91, 100 
dyspnoea, iv, 40 
measles in myasthenia gravis, v, 802 
palsy, acute inflammatory, v, 368 
progressive, v, 113 1040 
GENERAL INDEX 
Bulbar paralysis, ascending, v, 798 
Bulbus cordis, development of, iv, 325 
Bulimia, iii, 132 
Bundle of His, iv, 80, 203 
development of, iv, 328 
interventricular defects and, iv, 
367 
Stokes-Adams syndrome and, iv, 
308 
Burns from electricity, ii, 342 
from heat, ii, 348 
nephritis and, iii, 853 
Burrowing fleas, ii, 325 
Bursae, tuberculosis of, i, 373 
Butyl alcohol, ii, 400 
Butyric acid in carcinoma of stomach, iii, 
211 
C 
Cachectic splanchnoptosis, iii, 754 
Cachexia hypophyseopriva, v, 341 
of malarial fever, ii, 88 
miners', ii, 271 
multiple sclerosis and, v, 576 
thyreoidea, iv, 888 
in tuberculosis of kidney, iii, 985 
Caisson disease, ii, 345 
Calcareous degeneration of myocardium, 
iv, 116 
Calcification of lymph nodes, iv, 746 
of thymus, iv, 912 
Calcium carbonate calculus, iii, 1009 
metabolism of, ii, 561, 636 
oxalate calculus, iii, 1008, 1025 
* phosphate calculus, iii, 1015 
tetany and, v, 751 
Calculus, biliary, iii, 573 
bronchial, ii, 925 
of pancreas, iii, 632 
renal, iii, 1007 
salivary, iii, 71 
tartar, iii, 71 
tuberculosis of kidney and, iii, 978 
ureteral, iii, 1007 
Calmette's immunization in rabies, i, 970 
Cancer from x-rays, ii, 336 
Cancrum oris, iii, 49 
Capillaries in aortic insufficiency, iv, 223 
Capillary bronchitis, ii, 957 
thrombosis, iv, 537, 547 
Capsular splenitis, iv, 943 
Capsule, internal, lesion of, v, 276 
Carbohydrates, absorption of, in intes- 
tines, iii, 290 
diabetes mellitus and, ii, 677 
fat formation and, ii, 603 
metabolism of, ii, 596, 687 
in diabetes mellitus, ii, 691 
Carbon balance, ii, 560 
bisulphide poisoning, ii, 392 
dioxide in metabolism, ii, 618 
metabolism of, ii, 560 
moiety of protein, ii, 595, 622 
monoxide poisoning, ii, 388 
Carbuncles in diabetes mellitus, ii, 704 
Carcinoma of adrenal glands, iv, 784 
Carcinoma of ampulla of Vater, iii, 597 
of appendix, iii, 386 
of biliary ducts, iii, 593 
of brain, v, 315 
of choledochopancreatic duct, iii, 594 
of colon, iii, 429 
of duodenum, iii, 427 
of gall-bladder, iii, 593 
of ileum, iii, 428 
of intestines, iii, 424 
of jejunum, iii, 428 
of kidney, iii, 1001 
of lips, iii, 45 
of liver, iii, 541 
of lung, ii, 998 
of lymph nodes, iv, 747 
of lymphatic vessels, iv, 601 
of mediastinum, ii, 1100 
of mouth, iii, 52 
of oesophagus, iii, 98 
of pancreas, iii, 648 
of pericardium, iv, 74 
of pituitary body, iv, 804 
of pleura, ii, 1061 
of prostate, iii, 1038 
secondary anemia and, iv, 659 
of spinal cord, v, 196 
of spleen, iv, 951 
of stomach, iii, 199 
of thoracic duct, iv, 588 
of thymus, iv, 932 
of thyroid gland, iv, 832 
of tongue, iii, 66 
in tuberculosis, i, 435 
Cardiac arhythmia, iv, 29, 286 
psychical, iv, 287 
in Stokes-Adams syndrome, iv, 
305 
in tuberculous meningitis, iv, 287 
asthma, iv, 207 
complications in lobar pneumonia, i, 
255 
crises in tabes dorsalis, v, 842 
disease, congenital, iv, 323 
endocarditis, iv, 162 
epilepsy, v, 597 
hypertrophy in myocarditis, iv, 121 
insufficiency, iv, 191 
nerves, paralysis of, v, 552 
neuroses, iv, 296 
nutrition, derangements of, iv, 108 
syncope, iv, 88, 89 
thrombosis, iv, 531, 536, 548 
Cardialgia in hysteria, v, 683 
Cardiolysis, iv, 66 
Cardioptosis, iii, 752 
Cardiopulmonary murmurs, iv, 298 
Cardiospasm, iii, 147 
acute dilatation and, iii, 251 
neurasthenia and, iii, 147 
Cardiovascular disease, general considera- 
tions of, iv, 17 
lesions in gout, ii, 745 
system in lead poisoning, ii, 365 
in rheumatic fever, i, 984 
Caries, sicca, v, 189 
spinal, v, 188 GENERAL INDEX 
1041 
Carious teeth, tuberculosis of cervical 
glands and, iv, 743 
Carotid artery, embolism of, iv, 567 
thrombosis of, iv, 567 
Carriers of bacillary dysentery, i, 771 
of diphtheria, i, 732 
of malarial fevers, ii, 99 
of scarlet fever, i, 862 
of typhoid fever, i, 76, 197 
Caseous pneumonia, i, 352, 424 
Casts in diabetes mellitus, ii, 704 
in fibrinous bronchitis, ii, 907, 909 
kidney and, iii, 774 
of pregnancy and, iii, 868 
in parenchymatous nephritis, iii, 863, 
882, 901 
renal, iii, 798 
Catarrh, in chronic bronchitis, ii, 896 
postnasal, ii, 838 
suffocative, ii, 962 
Catarrhal appendicitis, iii, 372 
cholangitis, iii, 552, 559 
cholecystitis, iii, 562 
jaundice, iii, 448, 552 
laryngitis, ii, 868 
pharyngitis, ii, 844 
pneumonia, ii, 957 
pyelitis, iii, 953 
stomatitis, iii, 45 
ulcers, iii, 351 
Catatonic rigidity, v, 65 
Cavum Meckelii, tumors of, v, 340 
Cecum, rupture of peri-appendicular ab- 
scess into, iii, 365 
Cell embolism, iv, 561 
Cellulitis in scarlet fever, i, 878 
Centrum semiovale, lesion of, v, 275 
tumor in, v, 276, 334 
Cephalocele, hydrocephalus and, v, 354 
Cephalomegalia, v, 988 
Cerebellar artery, posterior inferior, occlu- 
sion of, v, 288 
ataxia, v, 72 
Marie's hereditary, v, 130, 136 
ataxic gait, v, 74 
localization, v, 286 
syndrome, v, 286 
Cerebellum, agnesis of, v, 287 
atrophy of, v, 287 
lesion of, v, 284 
sclerosis of, v, 287 
tumors of, v, 338 
Cerebral abscess, v, 378 
empyema and, ii, 1045 
arteries, aneurism of, v, 398 
embolism of, v, 400 
rupture of, v, 400 
thrombosis of, v, 400 
arteriosclerosis, v, 393 
bloodvessels, diseases of, v, 387 
diplegia, v, 419 
disseminated sclerosis, v, 154 
hemiplegia, congenital, v, 409 
infantile, v, 412 
hemisphere, ganglia of, v, 278 
hemorrhage, v, 401, 410, 411, 414, 415 
in purpura, iv, 697 
vol. v-66 
Cerebral localization hypothesis of hys- 
teria, v, 655 
nerves, diseases of, v, 471 
palsies, congenital, v, 410 
signs of Stokes-Adams syndrome, v, 
307 
softening in apoplexy, v, 431 
symptoms of lead poisoning, ii, 367 
syphilis, diabetes insipidus and, ii, 722 
Cerebrospinal disseminated sclerosis, v, 
154 
fever, i, 594 
hydrocephalus and, v, 357 
fluid, v, 351 
circulation of, v, 352 
examination of, v, 174 
lymphocytosis of, v, 177 
pressure of, v, 175 
in syphilis, v, 828 
meningitis, acute, v, 167 
epidemic, i, 594 
syphilis, v, 823 
vomiting, iii, 143 
Cervical adenitis, acute, i, 1012 
glands, tuberculosis of, iv, 743 
heart, iv, 346 
lymphadenitis, iv, 733, 734 
plexus, diseases of, v, 542 
ribs, v, 554 
Cervico-occipital neuralgia, v, 736 
Cestode infection, ii, 245 
Chalicosis, ii, 976 
Chancre, ii, 154 
Charbon, i, 639 
Charcot-Leyden crystals in asthma, ii, 939 
Charcot-Marie-Tooth type of progressive 
muscular atrophy, v, 109 
Cheinokinesthetic agraphia, v, 75 
Chest, deformities of, hypertrophy of 
heart and, iv, 181 
skin of, in aneurism of aorta, iv, 495 
Cheyne-Stokes respiration, ii, 834 
in acute meningitis, v, 170 
in apoplexy, v, 436 
in chronic nephritis, iii, 905 
in fat embolism, iv, 560 
in Stokes-Adams syndrome, iv, 
307 
Chicken mites, ii, 323 
Chickenpox, i, 849 
Chigger, ii, 324 
Chigoe, ii, 324 
Chloroma, iv, 679, 681 
of brain, v, 315 
Chlorosis, iv, 645 
cerebral thrombosis, and, v, 400 
exophthalmic goitre and, iv, 851 
gastric ulcer and, iii, 171 
hyperacidity and, iii, 114 
hyperthyroidism and, iv, 648 
hypoplasia of aorta and, iv, 432 
tropical, ii, 271 
Choked disk, v, 36 
in brain tumor, v, 326, 338 
in cerebral aneurism, v, 399 
stages of, v, 327 
Cholangitis, catarrhal, acute, iii, 552 1042 
GENERAL INDEX 
Cholangitis, catarrhal, cholelithiasis and, 
iii, 570, 574, 583 
chronic, iii, 559 
suppurative, iii, 556 
Cholecystitis, abscess of liver and, iii, 497 
acute, iii, 561 
chronic, iii, 568 
peritonitis and, iii, 714 
in typhoid fever, i, 119 
Choledochopancreatic duct, carcinoma of, 
iii, 594 
Cholelithiasis, iii, 569 
appendicitis and, iii, 571 
atony of stomach and, iii, 161 
carcinoma of gall-bladder and, iii, 593 
cholangeitis and, iii, 570, 574, 583 
hyperacidity and, iii, 114 
metabolism and, iii, 571 
pancreas and, iii, 613 
pericholecystic adhesions and, iii, 583 
pregnancy and, iii, 571 
Cholemia, iii, 454 
Choleperitoneum, iii, 670 
Cholera, Asiatic, i, 672 
infantum, i, 776; iii, 341 
morbus, iii, 331 
nostras, iii, 331 
vibrio, i, 673 
Cholesteatoma of brain, v, 316 
Cholesterin cysts of spleen, iv, 952 
Choluria, iii, 828 
Chondritis, gonococcal, i, 760 
Chondrodystrophia fetalis hypoplastica, v, 
958, 959 
Chondrosarcoma of lymphatic vessels, iv, 
601 
Chordae tendineae, anomalous, iv, 354 
Chordoma of brain, v, 317 
Chorea, acute, v, 774 
chronic, v, 787 
degenerative, v, 787 
electric, v, 787 
habit, v, 784 
Huntington's, v, 787 
insaniens, v, 779 
minor, v, 774 
mollis, v, 780 
nephritis and, iii, 850 
pericarditis and, iv, 42 
posthemiplegic, v, 466 
rheumatic fever and, i, 993; v, 775 
Sydenham's, v, 774 
Choroidal tubercles in acute miliary tuber- 
culosis, i, 419 
Choroidoretinitis, syphilitic, v, 475 
Chromaffin bodies, iv, 778 
system, diabetes mellitus and, ii, 682 
tissue, iv, 777 
Chvostek's phenomenon v, 71 
in myxoedema, iv, 893 
in sporadic cretinism, iv, 885 
in tetany, v, 758 
Chyle angiomata, iv, 586 
cysts of thoracic duct, iv, 586 
stone in thoracic duct, iv, 582 
Chyliform ascites, iii, 661 
pleural fluids, ii, 1057 
Chylopericardium, iv, 590 
Chylothorax, ii, 1057; iv, 590 
in anomalies of thoracic duct, iv, 580 
in carcinoma of thoracic duct, iv, 589 
in sarcoma of thoracic duct, iv, 588 
in thrombosis of thoracic duct, iv, 581 
traumatic, ii, 1059 
Chylous ascites, iii, 660; iv, 589 
pleural fluids, ii, 1057 
Chyluria, iii, 805; iv, 591 
in carcinoma of thoracic duct, iv, 589 
in filariasis, ii, 309 
in lymphangitis, iv, 595 
in thrombosis of thoracic duct, iv, 581 
Cicatricial stenosis of pylorus, iii, 262 
Cimex lectularius, ii, 327 
Circulatory changes in pituitary body, iv, 
804 
disturbances of adrenal glands, iv, 782 
of kidney, iii, 787 
of lungs, ii, 945 
neurasthenia and, v, 631 
of thymus, iv, 909 
organs, syphilis of, ii, 180 
system in arthritis deformans, v, 914 
in chlorosis, iv, 650 
gastric ulcer and, iii, 171 
in influenza, i, 544 
in pernicious anemia, iv, 632 
in plague, i, 628 
in rickets, ii, 790 
in tuberculosis, i, 359, 384 
in typhoid fever, i, 81, 124, 131 
in typhus fever, i, 926, 930 
in uremia, iii, 838 
Circumflex nerve, paralysis of, v, 546 
Circumscribed pericarditis, iv, 42 
saccular aneurism, iv, 472 
Circumspection, v, 43 
Cirrhosis of liver, iii, 505 
arthracotic, iii, 531 
cardiac, iii, 483 
malarial, iii, 530 
of lungs, ii, 969 
of stomach, iii, 273 
Cirsoid aneurism, iv, 437, 486 
Cladothrix buccalis in mouth, iii, 40 
Clergyman's sore throat, ii, 844 
Clonorchiasis, Asiatic, ii, 230 
Clonorchis, ii, 229 
Clubbed fingers, v, 981 
Coarctation of aorta, iv, 420 
Cocaine habit, ii, 445 
Coccygeal plexus, neuralgia of, v, 741 
Cochlear nerve, diseases of, v, 506 
Coin sound in pneumothorax, ii, 1081 
Colic, appendicular, iii, 373 
biliary, iii, 576 
gall-stone, iii, 576 
intestinal, iii, 303 
lead, iii, 306 
mucous, iii, 437 
Colitis, iii, 336 
amoebic, ii, 17 
in lobar pneumonia, i, 254 
membranous, iii, 437 
mucous, iii, 33, 437, 757 GENERAL INDEX 
1043 
Colitis, simple, iii, 336 
Colles' law in syphilis, ii, 153 
Colloid goitre, iv, 839 
matter of thyroid gland, iv, 823 
Colodysentery, i, 775 
Colon bacillus infections, i, 199 
carcinoma of, iii, 429 
congenital dilatation of, iii, 321 
disease of, peritonitis and, iii, 673 
displaced, iii, 752 
distension of, kidney infections and, 
iii, 952 
Color blindness, v, 25 
Colorless stools, iii, 294 
Combustion products poisoning, ii, 388 
Compensatory emphysema of lungs, ii, 
982 
hypertrophy of heart, iv, 176 
Complement fixation, i, 57 
test in glanders, i, 654 
in gonococcus infections, i, 
744 
Compression of intestines, iii, 394 
myelitis, v, 229 
paralysis in syphilis, ii, 162 
Compsomyia marcellaria, ii, 328 
Condyloma, syphilitic, ii, 165 
Confusion, v, 43 
Congenital anomalies of liver, iii, 455 
aortic atresia, iv, 398, 399 
insufficiency, iv, 407 
stenosis, iv, 398, 399 
cardiac disease, iv, 323 
convulsions and, v, 794 
cerebral hemiplegia, v, 409 
chronic interstitial nephritis, iii, 891 
cyanosis, iv, 333 
cystic kidney, iii, 786 
cysts of brain, v, 318 
diverticula of intestines, iii, 421 
elephantiasis, iv, 597 
hypertrophic stenosis of pylorus, iii, 
269 
hypertrophy of heart, iv, 351 
malformations of oesophagus, iii, 78 
mitral atresia, iv, 406 
obliteration of biliary ducts, iii, 547 
osteosclerosis, v, 968 
pulmonary atresia, iv, 388 
pylorospasm, iii, 269 
rhabdomyoma, iv, 351 
rickets, ii, 795; v, 955 
stenosis of intestine, iii, 408 
of oesophagus, iii, 96 
syphilis, ii, 187 
of liver, ii, 177 
tricuspid atresia, iv, 403 
insufficiency, iv, 470 
stenosis, iv, 403 
Congestion of bowels, constipation and, 
iii, 312 
of liver, iii, 478 
of lungs, ii, 945 
of spleen, iv, 940 
of thymus, iv, 909, 912 
of thyroid gland, iv, 825 
Conjugate deviation in apoplexy, v, 438 
Conjunctivitis in angioneurotic oedema, iv, 
1007 
in cerebrospinal meningitis, i, 604 
gonorrhoeal, i, 746 
in lobar pneumonia, i, 262 
in smallpox, i, 812 
Conorhinus sanguisuga, ii, 327 
Consciousness, v, 41, 42, 43 
Constipation, iii, 309 
atonic, iii, 313 
spastic, iii, 313 
Constriction of intestines, iii, 394 
Continuous gastromyxorrhcea, iii, 131 
Contraction, premature, of heart, iv, 84, 
91, 95, 100 
Contractures in amyotrophic lateral 
sclerosis, v, 99 
in facial paralysis, v, 502 
in hemiplegia, v, 464 
in hereditary spastic spinal paralysis, 
v, 106 
in hysteria, v, 651 
of muscles, v, 68 
in progressive neural muscular atro- 
phy, v, 110 
in traumatic neuroses, v, 151 
Conus stenosis, left-sided, iv, 400 
Convolutions, central, lesions of, v, 274 
Convulsions in abscess of brain, v, 380, 381 
in acute meningitis, v, 170 
miliary tuberculosis, i, 421 
nephritis, iii, 394 
in Addison's disease, iv, 793 
in anemia of brain, v, 392 
in arteriosclerosis, iv, 462 
of brain, v, 397 
in brain tumor, v, 329, 333 
in cardiac insufficiency, iv, 209 
in cerebrospinal meningitis, i, 605 
in chronic interstitial nephritis, iii, 908 
in fat embolism, iv, 560 
in general paresis, v, 857 
hemiplegia and, v, 415 
in hysteria, v, 673 
infantile, v, 790 
in lead poisoning, ii, 368 
in lesion of pons, v, 290 
in lobar pneumonia, i, 223 
in measles, i, 907 
in mercury poisoning, ii, 380 
in pachymeningitis interna, v, 165 
postnatal infantile hemiplegia and, v, 
415 
in rabies, i, 965 
in typhoid fever, i, 138 
in uremia, iii, 835 
Convulsive tic, v, 784 
Coordination, disturbances of, v, 71 
Copper poisoning, ii, 386 
Cor biatriatrum triloculare, iv, 372 
biloculare, iv, 370 
biventriculare triloculare, iv, 374 
Coronary arteries, accessory, iv, 439 
anomalies of, iv, 437 
in arteriosclerosis, iv, 463 
atheroma of, cardiac insufficiency 
and, iv, 195 1044 
GENERAL INDEX 
Coronary arteries, cirsoid aneurism of, iv 
437 
embolism of, iv, 195, 569 
sclerosis of, myocarditis and, iv, 
109 
thrombosis of, iv, 109, 195, 569 
disease, angina pectoris and, iv, 
289 
spasm, angina pectoris and, iv, 293 
Corpora quadrigemina, tumors of, v, 336 
striata, lesion of, v, 278 
Corpus callosum, disease of, v, 276 
tumor of, v, 276, 355 
Corrigan pulse, iv, 220, 224 
Corrosive oesophagitis, iii, 81 
Cortex of brain, v, 265, 273 
Cortical blindness, v, 54 
deafness, v, 33 
Coryza, acute bronchitis and, ii, 881 
in measles, i, 906 
in migraine, v, 718 
in tuberculosis, i, 506 
Coughing, mechanism of, ii, 828 
Courvoisier's law, iii, 579 
Cowpox, i, 822 
Cramps in occupation neuroses, v, 744 
professional, v, 741 
Cranial base, tumors of, v, 340 
nerves in tabes dorsalis, v, 841 
Cranium in rickets, ii, 785 
Creatin in fasting, ii, 614 
in protein metabolism, ii, 589 
Creatinin, excretion of, iii, 813 
Cremaster reflex, anomalies of, v, 78 
Cretinism, iv, 881 
Crisis in lobar pneumonia, i, 240 
Croupous pneumonia, i, 202 
Crura cerebri, lesion of, v, 281 
tumors of, v, 335 
Crural neuralgia, v, 740 
Cryoscopy in lobar pneumonia, i, 233 
of urine, iii, 792 
Cryptogenic anemia, iv, 619 
Crystallization of renal calculi, iii, 1014 
Crystals in urine, iii, 800 
Crytometry in tuberculosis, i, 404 
Ctenocephalus canis, ii, 325 
Curschmann's spirals in asthma, ii, 939 
Cutaneous actinomycosis, i, 1040 
burns, duodenal ulcer and, iii, 349 
diphtheria, i, 699 
lesions in syphilis, ii, 163, 168 
in uremia, iii, 840 
reaction in syphilis, ii, 193 
reflexes, anomalies of, v, 77 
syphilides, ii, 150, 184 
Cyanosis, congenital, iv, 333 
Cylindrical aneurism, iy, 479 
Cylindroids in urine, iii, 800 
Cystic distension of appendix, iii, 386 
duct, cholelithiasis and, iii, 580 
obstruction of, iii, 580 
kidney, congenital, iii, 786 
lymphangiomata, iv, 586 
pyelitis, iii, 354 
softening of thymus, iv, 912 
Cysticercosis, ii, 263 
Cysticercus bovis, ii, 247, 264 
cellulosae, ii, 249, 263 
cyst of peritoneum, iii, 736 
of spleen, iv, 954 
fasciolaris, ii, 252 
tenuicollis, ii, 264 
Cystin calculus, iii, 1009 
Cystinuria, ii, 533, 656; iii, 817 
Cystitis in appendicitis, iii, 371 
gonococcal, i, 762 
kidney infections and, iii, 952 
in smallpox, i, 812 
tuberculosis of kidney and, iii 978 
in typhoid fever, i, 143 
Cystoglioma of brain, v, 313 
Cystosarcoma of brain, v, 315 
Cystoscopy, iii, 938 
in hypernephroma, iii, 998 
Cysts of brain, v, 318 
chyle, of thoracic duct, iv, 586 
of liver, iii, 547 
of lymph nodes, iv, 586, 753 
of mediastinum, ii, 1101 
of mouth, iii, 52 
necrosis of pancreas and, iii, 619 
ovarian, acute peritonitis and, iii, 
673 
of peritoneum, iii, 733-739 
of spleen, iv, 951 
of thymus, iv, 931, 933 
Cytodiagnosis, ii, 1030 
Cytological examination of cerebrospinal 
fluid, v, 176 
Cytology of pleural fluids, ii, 1030 
in tuberculosis, i, 422 
Cytolysis, auto-intoxication and, ii, 504 
Cytoryctes vaccinje, i, 825 
variolae, i, 786 
D 
Dacryoadenitis, gonococcal, i, 764 
Davainea asiatica, ii, 246, 255 
madagascariensis, ii, 246, 255 
Day blindness, v, 480 
Deafness, mind, v, 54 
word, v, 54 
Decubital ulcers, iii, 351 
of oesophagus, iii, 84 
Degeneration, fatty, ii, 604 
of liver, iii, 466 
of lymph nodes, iv, 746 
of myocardium, iv, 108 
of pituitary body, iv, 804 
progressive lenticular, v, 278 
reaction of, v, 72 
Degenerative myelitis, v, 236 
Deglutition pneumonia, ii, 958 
Delirium cordis, iv, 106 
tremens, ii, 408 
in uremia, iii, 837 
Delusions, v, 56 
Demacentor andersonii, ii, 317, 318 
Dementia, anomalies of conduct in, v, 67 
in arteriosclerosis, iv, 462 
paralytica, v, 850 GENERAL INDEX 
1045 
Dementia prsecox, hysteria and, v, 694, 
695 
Demodex foliiculorum, ii, 321 
Dengue, i, 950 
Depression, pathological, v, 60 
Dercum's disease, v, 817 
Dermanyssus, ii, 323 
Dermatitis, bullous, in hysteria, v, 686 
coccidioides, i, 1050 
in dermatomyositis, v, 877 
from rr-rays, ii, 335 
Dermatocentor andersonii, i, 1021 
Dermatomyositis, v, 875 
Dermatoses of mouth, iii, 53 
Dermoid cyst of mediastinum, ii, 1101 
of mouth, iii, 53 
of peritoneum, iii, 739 
of spleen, iv, 953 
of thymus, iv, 933 
tumors of brain, v, 316 
Dextrocardia, iv, 347 
Diabetes, bronze, ii, 681 
insipidus, ii, 721 
calculus of pancreas and, iii, 635 
carcinoma of pancreas and, iii, 
650, 652 
lipogenous, ii, 676 
mellitus, ii, 674 
apoplexy and, v, 427 
cyst of pancreas and, iii, 645 
diabetes insipidus and, ii, 724 
gangrene of lung and, ii, 991 
lobar pneumonia and, i, 207 
pancreas and, iii, 604, 619, 630 
tuberculosis and, i, 320, 435 
Diabetogenous obesity, ii, 676 
Diacetic acid in diabetes mellitus, ii, 702 
Diadokokinesis, v, 72 
Diamino-acids, ii, 656 
Diaminuria, ii, 656 
Diaphragm, immobility of, in adherent 
pericardium, iv, 61 
perforation of, in purulent pleuritis, ii, 
1045 
Diaphragmatic neuralgia, v, 737 
pleuritis, ii, 1049 
Diarrhoea, iii, 322 
in childhood, iii, 337, 340, 341 
Diastole, nature of, iv, 22, 32 
Diastolic collapse of veins, iv, 62 
Diazo reaction in typhoid fever, i, 141 
Dibothriocephalus cordatus, ii, 246, 257 
latus, ii, 245, 255 
Dichromates, v, 25 
Dicrocoelium, ii, 229, 234 
Dicrotism, iv, 35 
in typhoid fever, i, 124 
Dietl's crises in nephroptosis, iii, 756, 759 
Digestive gastrosuccorrhoea, iii, 128 
tract in lead poisoning, ii, 364 
tuberculosis of, i, 360 
Dilatation aneurism, iv, 472, 482 
of aorta, dynamic, iv, 513 
in diseases of valves of heart, iv, 214 
of heart, iv, 30, 189 
defects of interauricular septum 
and, iv, 359 
Dilatation of hepatic artery, iii, 458 
veins, iii, 459 
idiopathic, iii, 320 
of oesophagus, iii, 85, 90, 91 
of pulmonary artery, iv, 398 
defects of interauricular sep- 
tum and, iv, 359 
of stomach in lobar pneumonia, i, 254 
in tuberculosis, i, 502 
tricuspid insufficiency and, iv, 247 
Diphtheria, i, 689 
antitoxin in, i, 54, 715 
apoplexy and, v, 417 
arteriosclerosis and, iv, 454 
bronchopneumonia and, ii, 958 
cardiac insufficiency and, iv, 196 
carriers, i, 732 
cutaneous, i, 699 
enlargement of thymus and, iv, 915 
of eye, i, 699 
laryngeal, i, 698 
of lungs, i, 700 
multiple neuritis and, v, 575 
myocarditis and, iv, 108, 116 
nasal, i, 698 
nephritis and, iii, 849 
pharyngeal, i, 696 
tuberculosis and, i, 321, 436 
of uterus, i, 699 
Diphtheritic gastritis, iii, 236 
pyelitis, iii, 954 
Diplegia, cerebral, v, 419 
Diplococcus catarrhalis, i, 595 
intracellularis meningitidis, i, 594 
rheumaticus, i, 975 
Diplogonoporus, ii, 246, 258 
Diplopia in disseminated sclerosis, v, 150 
in interstitial nephritis, iii, 910 
monocular, v, 480 
in myasthenia gravis, v, 802 
Dipsomania, ii, 423 
Dipterous infection, ii, 328 
Dipylidium caninum, ii, 246, 255 
Dirofilaria, ii, 314 
Dirt eating, ii, 271 
Dislocated spleen, iv, 938 
Disorientation, v, 44 
Dissecting aneurism, iv, 472, 487, 489 
Disseminated gummatous sporotrichosis, i, 
1056 
myelitis, v, 235 
sclerosis, v, 140 
Dissociation, v, 51 
Distension in intestinal indigestion, iii, 301 
of stomach, cardiac arhythmia and, iv, 
287 
Distoma hepaticum, ii, 235 
Distomatosis, ii, 225 
hepatic, ii, 229 
intestinal, ii, 237 
ophthalmic, ii, 235 
pulmonary, ii, 226 
renal, ii, 239 
Distomiasis, ii, 225 
Diver's paralysis, ii, 345 
Diverticula of intestines, iii, 420 
of oesophagus, iii, 92 1046 
GENERAL INDEX 
Diverticulitis, iii, 423 
Diverticulum of heart, iv, 351 
of pericardium, iv, 346 
of Vater, biliary infections through, 
iii, 550 
Dracontiasis, ii, 303 
Dracunculosis, ii, 303 
Dream states, v, 689 
Dropsy, renal, ascites and, iii, 659 
Drunkenness, pathological, ii, 424 
Dry pleurisy, ii, 1051 
Duchenne-Erb palsy, v, 551 
Ductless glands, diseases of, iv, 777 
Ductus arteriosus Botalli, absence of, iv, 
420 
aneurism of, iv, 419 
anomalies of, iv, 409, 420 
endarteritis and, iv, 413 
left subclavian artery from, 
iv, 436 
patency of, iv, 409 
Dum-dum fever, iv, 965 
Duodenal ulcer, iii, 191, 344 
chronic peritonitis and, iii, 
714 
intestinal hemorrhage and, iii, 
307 
subphrenic abscess and, iii, 
701 
pyopneumothorax and, iii, 
710 
Duodenitis, necrosis of pancreas and, iii, 
612 
Duodenum, carcinoma of, iii, 427 
perforation of, in necrosis of pancreas, 
iii, 619 
stenosis of, iii, 262 
Dura mater, diseases of, v, 163 
Dwarf tapeworm, ii, 252 
Dysacusis, v, 33 
Dysarthria, v, 74 
Dyschiria, v, 268 
Dyschondroplasia, v, 969 
Dyschromatopsia in brain tumor, v, 
329 
Dysentery, amoebic, ii, 17, 33, 34 
bacillary, i, 766 
Dysesthesia, v, 32 
in brain tumor, v, 339 
Dyspepsia, nervous, iii, 164 
Dyspeptic cirrhosis of liver, iii, 509 
Dysphonia, spastic, v, 522 
Dysplasic periosteal achondroplasia, v, 
969 
Dyspnoea, bulbar, iv, 40 
nervous, iv, 37 
reflex, iv, 38 
toxic, iv, 37 
Dyspragia, angiosclerotic, iii, 437 
Dyspraxia, v, 75 
Dysthymia, v, 60 
Dystrophy, progressive muscular, v, 117 
facio-scapulo-humeral, v, 122 
hereditary, v, 123 
infantile, v, 123 
pseudo-hypertrophic, v, 122 
of Zimmerlin, v, 123 
E 
Ear in acromegaly, iv, 811 
in cerebrospinal meningitis, i, 604, 609 
in measles, i, 905 
in myxoedema, iv, 891 
in tuberculosis, i, 431 
in typhoid fever, i, 140 
Echinochynchus, ii, 297 
Echinococcosis, ii, 264 
Echinococcus cyst of brain, v, 319 
of mediastinum, ii, 1109 
disease, ii, 264 
of pleura, ii, 1065 
granulosus, ii, 263 
kidney infections and, iii, 952 
inultilocularis, ii, 266 
of myocardium, iv, 147 
of thyroid gland, iv, 831 
Echinostoma ilocanum, ii, 238 
Echolalia, v, 65 
Echopraxia, v, 65 
Eclampsia in kidney of pregnancy, iii, 869 
parathyroid gland and, iv, 903 
Ectasia of thoracic duct, iv, 585 
Ecthyma in congenital syphilis, ii, 190 
Ectopia cordis, iv, 346 
Eczema in gout, ii, 748 
of lips, iii, 45 
of mouth, iii, 54 
in vaccination, i, 843 
Eelworm infection, ii, 287 
Egyptian chlorosis, ii, 271 
hematuria, ii, 239 
Electric chorea, v, 787 
Electrical hyperexcitability in tetany, v, 
757 
reactions in diseases of peripheral 
nerves, v, 535 
in multiple neuritis, v, 582 
in progressive spinal muscular 
atrophy, v, 85 
i Electricity, ii, 339 
Electrocardiograph, iv, 80 
in congenital disease of heart, iv, 444 
Elephantiasis Arabum, iv, 596 
in filariasis, ii, 310 
lymphangiectatica, iv, 596-598 
neuromatosa, v, 571 
in thrombosis of thoracic duct, iv, 581 
Elephantoid fever, ii, 308 
Emboli, endogenous, iv, 553 
exogenous, iv, 553 
infective, iv, 557 
Embolic aneurisms, iv, 557 
ulcers, iii, 350 
Embolism, iv, 552 
air, iv, 559 
aneurism and, iv, 476 
of aorta, iv, 565 
in aortic insufficiency, iv, 228 
apoplexy and, v, 418, 422, 424, 427, 
428, 431 
of carotid artery, iv, 567 
.cell, iv, 561 
of cerebral arteries, v, 400 
of coronary arteries, iv, 569 GENERAL INDEN 
1047 
Embolism, crossed, iv, 553 
fat, iv, 560 
gastric ulcer and, iii, 171 
of hepatic artery, iii, 458; iv, 569 
infarcts and, iv, 556, 946 
infective, iv, 557 
in lobar pneumonia, i, 258 
mercurial, iv, 561 
mesenteric, iii, 307, 434; iv, 565 
in mitral insufficiency, iv, 236 
stenosis, iv, 242 
paradoxical, iv, 363, 553 
paraffin, iv, 562 
parasitic, iv, 558 
of peripheral arteries, iv, 568 
of portal vein, iii, 463 
postnatal infantile hemiplegia and, v, 
418 
of pulmonary artery, iv, 562 
in pyemia, i, 565 
of renal artery, iv, 564 
of retinal arteries, iv, 570 
retrograde, iv, 553 
of splenic artery, iv, 564 
of subclavian artery, iv, 567 
in tuberculosis, i, 433 
tumor cells and, iv, 557 
in typhoid fever, i, 129 
venous, iv, 552 
Embryocardia, iv, 279 
Embryonic tumors of kidney, iii, 1003 
Emmetropia, v, 36 
Emotional states, anomalies of, v, 59 
Emphysema, cardiac insufficiency and, iv, 
199 
in diphtheria, i, 693 
of lungs, ii, 979 
of mediastinum, ii, 1110 
in tuberculosis, i, 430 
in whooping cough, i, 660 
Emphysematous liver, iii, 466 
Empyema, ii, 1040 
abscess of brain and, v, 379 
chronic, ii, 1052 
encysted, ii, 1049 
of gall-bladder, iii, 563, 566 
in influenza, i, 542 
interlobar, ii, 1050 
in lobar pneumonia, i, 248 
necessitatis, ii, 1044 
pulsating, ii, 1043 
of renal pelvis, iii, 937 
subphrenic abscess and, iii, 701 
in tuberculosis, i, 431 
tuberculous, ii, 1048 
in typhoid fever, i, 123 
Encephalic tuberculoma of brain, v, 319 
Encephalitis, v, 359 
acute, v, 360, 361 
hemorrhagic, v, 367 
hyperplastic, v, 370 
non-suppurative, v, 370 
congenita, v, 361, 370 
epilepsy and, v, 599 
experimental, v, 371 
in infancy and childhood, v, 376 
influenzal, i, 543; v, 362 
Encephalitis, neonatorum, v, 370 
postnatal infantile hemiplegia and, v, 
416 
progressive, v, 361 
pyogenic, v, 363 
subacute, v, 360, 361 
superior, v, 490 
Encephalomyelitis, acute, v, 369 
Encephalopathy in lead poisoning, ii, 367 
Enchondroma of brain, v, 317 
of lung, ii, 997 
Encysted empyema, ii, 1049 
pleuritis, ii, 1049 
tuberculosis of peritoneum, iii, 723 
Endarteritis, acute gummatous, ii, 181 
pulmonary, iv, 413 
in cerebral syphilis, v, 824 
deformans, iv, 452 
ductus arteriosus Botalli and, iv, 413 
obliterative, ii, 181; iv, 458 
Endocarditis, acute, iv, 148 
aortic insufficiency and, iv, 218 
stenosis and, iv, 229 
in arthritis deformans, v, 914 
chorea and, v, 776, 781 
chronic, iv, 212 
septic, iv, 148 
coarctation of aorta and, iv, 429 
coronary embolism and, iv, 569 
in erysipelas, i, 587 
gonorrhoeal, i, 750 
in influenza, i, 544 
in lobar pneumonia, i, 255 
in lymphoid leukemia, iv, 675 
malignant, iv, 161-163 
in measles, i, 907 
mesenteric embolism and, iii, 435 
mitral insufficiency and, iv, 232 
stenosis and, iv, 239 
necrotic, iv, 151 
primary, iv, 153 
pulmonary stenosis and, iv, 258 
in purpura, iv, 706 
recurrent, iv, 148 
in rheumatic fever, i, 985 
in scarlet fever, i, 877 
simple, iv, 148, 158 
subaortic stenosis and, iv, 399 
syphilitic, ii, 183 
tricuspid insufficiency and, iv, 244 
in tuberculosis, i, 433 
in typhoid fever, i, 126 
ulcerative, iv, 148, 151 
vegetative, iv, 151 
verrucose, iv, 150 
Endogenous emboli, iv, 553 
Endolymphangitis, iv, 595 
Endophlebitis of portal vein, iii, 460, 465 
Endothelioma of brain, v, 313 
of lymph nodes, iv, 747 
lymphangiomatosum, iv, 600 
of lymphatic vessels, iv, 600, 601 
of mediastinum, ii, 1100 
of thoracic duct, iv, 588 
Endotoxin, i, 33 
Energy metabolism, ii, 564 
muscular, ii, 621, 631 1048 
GENERAL INDEX 
Enteralgia, iii, 305 
in hysteria, v, 683 
Enteritis, iii, 328, 334 
lymphadenitis and, iv, 736 
Enterocolitis, diarrhoea and, iii, 324 
Enterodysentery, i, 775 
Enterokinase, iii, 290 
Enteroptosis, iii, 747 
in atony of stomach, iii, 162 
Enterostenosis, iii, 753 
Entomoebse blattse, ii, 22 
coli, ii, 22, 23, 36, 45 
hartmanni, ii, 19 
histolytica, ii, 22, 23, 26, 46 
hominis, ii, 22 
tetragena, ii, 24, 47 
Enzymes, defensive, ii, 651 
intracellular, ii, 663 
inverting, iii, 290 
protective, ii, 651 
spleen and, iv, 934 
Eosinophiles in myeloid leukemia, iv, 668 
Eosinophilia, iv, 615 
Ependymal glioma of brain, v, 311 
inflammation, hydrocephalus and, v, 
358 
Epicritic sensibility, v, 17 
Epidemic cerebrospinal meningitis, i, 594 
Epididymis, tuberculosis of, i, 366 
Epigastric reflexes, v, 78 
Epilepsy, v, 592 
alcoholism and, ii, 408; v, 593, 596 
apoplexy and, v, 421 
in brain tumor, v, 331, 333 
cerebral diplegia and, v, 421 
in disseminated sclerosis, v, 153 
idiopathic, v, 592 
infantile convulsions and, v, 594 
Jacksonian, v, 592 
migraine and, v, 615, 711 
parathyroid glands and, iv, 903 
petit mal, v, 604 
in pseudo-sclerosis, v, 159 
in Raynaud's disease, iv, 988 
Epileptic insanity, traumatism and, v, 647 
psyche, v, 598, 612 
Epileptoid attacks in lesion of centrum 
semiovale, v, 275 
Epinephritis, iii, 970 
Epiphyseal cartilages in achondroplasia, 
v, 958 
Epiploitis, iii, 695 
Epithelial casts, iii, 799 
cells in tuberculosis of kidney, iii, 984 
Epithelioid tuberculosis of lymph nodes, 
iv, 741 
Epithelioma of brain, v, 315 
Epitrochlear gland, tuberculosis of, iv, 744 
Equilibrium, sense of, v, 18 • 
Erb's phenomenon, v, 71 
sign in tetany, v, 757 
syphilitic paraplegia, v, 246, 828 
Erepsin, iii, 290 
Ergotismus, ii, 468 
Erosion aneurism, iv, 472 
Eructation, nervous, iii, 141 
Erysipelas, i, 585 
Erysipelas, lymphangitis and, iv, 592 
of mouth, iii, 54 
in myeloid leukemia, iv, 670 
nephritis and, iii, 850 
typhoid fever and, i, 158 
Erythema, acute endocarditis and, iv, 
155 
in arsenic poisoning, ii, 375 
in congenital syphilis, ii, 189 
gonococcal, i, 764 
in Henoch's purpura, iv, 709 
infectiosum, i, 916 
nodosum, gonococcal, i, 764 
nephritis and, iii, 850 
in rheumatic fever, i, 997 
in pellagra, ii, 482 
purpura and, iv, 705 
rheumatica and, iv, 703 
in rheumatic fever, i, 996 
in smallpox, i, 799 
in typhoid fever, i, 100 
Erythematous scleroderma, iv, 1017 
Erythrocytosis megalosplenica, iv, 682 
Erythromelalgia, iv, 1025 
in arteriosclerosis, iv, 465 
Erythropsia, v, 480 
Estivo-autumnal parasite, ii, 56, 59 
Eustachian valve, iv, 327 
Eustrongylus gigas, ii, 316 
kidney infections and, iii, 952 
Exaltation, pathological, v, 60 
Excitability, affective, pathological, v, 
62 
Exogenous emboli, iv, 553 
Exophthalmic goitre, iv, 849 
Exophthalmos in congenital cyanosis, iv 
339 
in exophthalmic goitre, iv, 862 
in hypopituitarism, iv, 817 
in ocular paralysis, v, 485 
Extracerebellar tumors, v, 338 
Extradural hemorrhage, v, 257 
Extraperitoneal subphrenic abscess, iii 
705 
Extrasystole, iv, 84 
Exudate in ascites, iii, 659 
in pericarditis, iv, 43 
Eye lesions from electricity, ii, 342 
from x-rays, ii, 336 
Eyes in acromegaly, iv, 810 
in aneurism of arch of aorta, iv, 494 
diphtheria of, i, 699 
in exophthalmic goitre, iv, 862 
in hysteria, v, 684 
in influenza, i, 545 
in lymphoid leukemia, iv, 672 
in measles, i, 905 
in meningitis, cerebrospinal, i, 604, 
609 
posterior basic, i, 617 
in myeloid leukemia, iv, 666 
in myxoedema, iv, 893 
in Raynaud's disease, iv, 987 
in scarlet fever, i, 875 
in typhoid fever, i, 139 
Eye-grounds in abscess of brain, v, 380 
in tumor of brain, v, 326 GENERAL INDEX 
1049 
F 
Facial atrophy in migraine, v, 719 
hemiatrophy, v, 991 
irritability in rickets, ii, 789 
nerve, paralysis of, v, 495 
in tabes dorsalis, v, 841 
neuralgia, v, 733 
paralysis, v, 495 
in hysteria, v, 679 
otitis and, v, 497 
poliomyelitis and, v, 497 
polyneuritis and, v, 497 
spasm, v, 504 
Facies in acute peritonitis, iii, 679 
cholerica, i, 683 
Facio-scapulo-humeral progressive muscu- 
lar dystrophy, v, 122 
Faget's sign in yellow fever, i, 944 
Falling sickness, v, 592 
Fallopian tubes, abscess of, subphrenic 
abscess and, iii, 701 
tuberculosis of, i, 368 
Family periodic paralysis, v, 809 
Farcy, i, 649, 653 
Fasciae, tuberculosis of, i, 373 
Fasciola, ii, 229, 235 
Fasciolopsis, ii, 237, 238 
Fat, carbohydrates and, ii, 603 
embolism, iv, 560 
thoracic duct and, iv, 582 
metabolism, ii, 601 
in obesity, ii, 605 
necrosis, iii, 606 
tapeworm, ii, 247 
Fatigue, sense of, v, 19, 32 
Fatty ascites, iii, 661 
casts, iii, 800 
degeneration of lymph nodes, iv, 746 
in myocarditis, iv, 115 
in pernicious anemia, iv, 625 
feces, iii, 294l 
heart in myocarditis, iv, 115 
infiltration of lymph nodes, iv, 746 
liver, iii, 466 
stools, iii, 294 
Febricula, i, 1009 
Fecal concretions in appendicitis, iii, 360 
masses, intestinal obstruction from, 
iii, 403 
Feces, iii, 292 
acholic, iii, 294 
colorless, iii, 294 
fatty, iii, 294 
Femoral glands, lymphadenitis of, iv, 736 
neuralgia, v, 740 
Ferments in carcinoma of stomach, iii, 211 
Fetal passages in transposition of arterial 
trunks, iv, 385 
rickets, v, 955 
Fetid bronchitis, ii, 911 
Fibrillary twitchings, v, 70 
Fibrillation, auricular, iv, 86, 92, 96, 99 
of heart, insufficiency and, iv, 192 
Fibrinous bronchitis, ii, 907 
pleuritis, ii, 1005 
pyelitis, iii, 954 
Fibroid degeneration of myocardium, iv, 
114 
nodules, endocarditis and, iv, 155 
tumors of pericardium, iv, 76 
Fibroma of lung, ii, 998 
of peritoneum, iii, 731 
of spleen, iv, 950 
of tongue, iii, 66 
Fibromyoma of peritoneum, iii, 730 
Fibrosa obliterans, iv, 595 
Fibrosis in chronic myocarditis, iv, 114 
of lungs in bronchiectasis, ii, 917 
Fibrous stenosis of oesophagus, iii, 96 
Filaria, ii, 296-315 
inflammation of thoracic duct and, 
iv, 584 
Filariasis, ii, 305 
Fingers in arthritis deformans, v, 912 
clubbing of, in bronchiectasis, ii, 
916 
in osteo-arthropathy, v, 984 
Fish poisons, ii, 451 
Fissures of intima of aorta, iv, 487 
Fistula-in-ano in tuberculosis, i, 432 
Fistulae, biliary, in cholelithiasis, iii, 584 
Fleas, ii,. 325 
Fletcherism, ii, 660 
Flint murmur in aortic insufficiency, iv, 
226 
Floating spleen, iv, 938 
Florida fever, i, 1011 
Fluke infections, ii, 225 
Fluoroscope in congenital disease of heart, 
iv, 444 
Fluoroscopic findings in constipation, iii, 
313 
Flutter, auricular, iv, 86, 92, 94, 99 
Folin's theory of protein metabolism, ii, 
594 
Follicular pharyngitis, ii, 845 
ulcers, iii, 331 
Foot, painful, i, 760 
Foot-jerk, v, 77 
Foot-and-mouth disease, i, 1025 
Foramen ovale, patent, iv, 355 
Foreign bodies in appendicitis, iii, 360 
in bronchi, ii, 926 
intestinal obstruction from, iii, 
404 
protein, ii, 651 
Fossa ovalis, aneurismal pouching of, iv, 
359 
Fourth disease, i, 915 
ventricle, tumors of, v, 338 
Fracture, intra-uterine, v, 979 
Fragilitas ossium, v, 947, 980 
congenital, v, 978 
Fragmentation of myocardium, iv, 116 
Frenum of tongue, deformities of, iii, 61 
ulcer of, iii, 63 
Freud's hypothesis of hysteria, v, 657 
Friedreich's ataxia, v, 130 
Frohlich's syndrome, iv, 815; v, 341 
Frontal lobe, tumors of, v, 331 
Furuncles in scarlet fever, i, 878 
in typhoid fever, i, 101 
Fusiform aneurism, iv, 479 1050 
GENERAL INDEX 
G 
Galactosuria, ii, 600 
Gall-bladder, anomalies of, iii, 547 
carcinoma of, iii, 593 
of liver and, iii, 543 
of pancreas and, iii, 652 
in catarrhal cholangitis, iii, 554 
in cholelithiasis, iii, 579 
in chronic peritonitis, iii, 719 
diseases of, peritonitis and, iii, 673 
empyema of, iii, 563, 566 
in suppurative cholangitis, iii, 557 
tuberculosis of, i, 364 
in typhoid fever, i, 86, 119 
wall of, biliary infection through, iii, 
551 
Gall-ducts, tuberculosis of, i, 364 
Gall-stones, iii, 572 
chronic cholangitis and, iii, 559 
cholecystitis and, iii, 568 
colic, iii, 576 
infection and, iii, 578 
in common duct, iii, 58 T 
in cystic duct, iii, 580 
in diverticulum of Vater, iii, ,582 
glycosuria and, ii, 678 
intestinal obstruction from, iii, 403 
Ganglia of cerebral hemisphere, v, 278 
in cerebrospinal meningitis, i, 598 
inflammation of, neuralgia and, v, 726 
Ganglionated neuroma of peritoneum, iii, 
731 
Gangrene, acute arteritis and, iv, 450 
in aortic embolism, iv, 566 
thrombosis, iv, 566 
of appendix, iii, 363 
in cervical ribs, v, 556 
in chickenpox, i, 852 
in diabetes mellitus, ii, 705 
in diseases of cord, iv, 992 
in ergotism, iv, 995 
from x-rays, ii, 336 
in hemiplegia, iv, 992 
in infectious diseases^ iv, 978, 993 
in lobar pneumonia, i, 217 
of lung, ii, 988 
in bronchiectasis, ii, 917 
in bronchopneumonia, ii, 964 
in fetid bronchitis, ii, 912 
in influenza, i, 541 
in lobar pneumonia, i, 251 
pneumothorax and, ii, 1074 
in tuberculosis, ii, 430 
tumors of lung and, ii, 1001 
in obliterative arteritis, iv, 993 
of pancreas, necrosis and, iii, 615 
in pathomimia, iv, 995 
post-vaccinal, i, 842 
in Raynaud's disease, iv, 983, 986 
in scarlet fever, i, 878 
of skin in smallpox, i, 812 
in thrombo-angeitis obliterans, iv, 547 
in typhoid fever, i, 101, 149 
Gangrenous appendicitis, iii, 372 
bronchitis, ii, 911 
cholecystitis, iii, 563 
Gangrenous pyelitis, iii, 954 
stomatitis, iii, 49 
Gas analysis in pneumothorax, ii, 1081 
Gaseous distension of intestines, iii, 298 
liver, iii, 466 
Gastralgia, nervous, iii, 135 
in mycotic gastritis, iii, 236 
Gastralgokenosis, iii, 134 
Gastric anacidity, iii, 153 
analysis in carcinoma of stomach, iii, 
210 
in gastric ulcer, iii, 181 
atrophy, pernicious anemia and, iv. 
623 
contents in atony of stomach, iii, 162 
in chronic gastritis, iii, 242 
crises in tabes dorsalis, v, 841, 846 
disease, carcinoma and, iii, 200 
tetany and, v, 749 
emptiness, painful, iii, 134 
hyperesthesia, iii, 137 
idiosyncrasies, iii, 138 
juice, hypersecretion of, iii, 122 
nerves, paralysis of, v, 523 
neurasthenia, iii, 164 
neuroses, iii, 106 
regurgitation, iii, 140 
tuberculosis, i, 388, 431 
ulcer, iii, 168 
chronic peritonitis and, iii, 714 
intestinal hemorrhage and, iii, 
307 
pyloric stenosis and, iii, 189 
subphrenic abscess and, iii, 70 
pyopneumothorax and, iii, 
710 
tetany and, iii, 189 
in tuberculosis, i, 432; iii, 170 
Gastritis, acute, iii, 226 
chronic, iii, 237, 240 
diphtheritic, iii, 236 
interstitial purulent, iii, 231 
mesenteric lymphadenitis and, iv, 736 
mucous, iii, 238 
mycotic, iii, 235 
phlegmonous, iii, 231 
proliferative, iii, 238 
secondary, iii, 239 
syphilitic, ii, 174 
toxic, iii, 233 
Gastrodiscus hominis, ii, 238 
Gastro-enteritis in lead poisoning, ii, 360 
Gastro-intestinal auto-intoxication, ii, 506, 
511, 524 
cardiac neuroses, iv, 297 
complications in lobar pneumonia, i, 
253 
disease, typhoid fever and, i, 72, 158 
influenza, i, 543 
sepsis, pernicious anemia and, iv, 623 
splanchnoptosis, iii, 754 
symptoms in amoebic dysentery, ii, 35 
tract, in angioneurotic oedema, iv, 1007 
in cirrhosis of liver, iii, 517 
in diabetes mellitus, ii, 705 
in jaundice, iii, 455 
in measles, i, 906 GENERAL INDEX 
1051 
Gastro-intestinal tract in pernicious 
anemia, iv, 634 
Gastromyxorrhcea, iii, 130 
Gastroptosis, iii, 754 
in atony of stomach, iii, 162 
gastrectasis and, iii, 254 
Gastrosuccorrhoea, iii, 122 
continua chronica, iii, 125 
periodica, iii, 125 
digestive, iii, 128 
in pylorospasm, iii, 150 
Gaucher's splenomegaly, iv, 962 
Gelatinous pneumonia, i, 352 
Gelle's test, v, 24 
Geniculate ganglia, inflammation of, v, 505 
Genital organs in hypopituitarism, iv, 816 
Genito-urinary diagnosis, iii, 1026 
system in lead poisoning, ii, 365 
tract, acute endocarditis and, iv, 153 
in influenza, i, 545 
in plague, i, 629 
in tuberculosis, i, 365, 389 
in typhus fever, i, 926, 930 
Geographical tongue, iii, 63 
Gerlier's disease, v, 515 
Giant urticaria, iv, 998 
Gigantism, iv, 806 
achondroplasia and, v, 961 
Gigantorhynchus, ii, 297 
Glanders, i, 649 
Glands, lymphatic, in typhoid fever, i, 146 
mesenteric, in typhoid fever, i, 85 
in smallpox, i, 790 
submaxillary, in typhoid fever, i, 104 
Glandular enlargement in arthritis defor- 
mans, v, 909, 915 
in lymphoid leukemia, iv, 671 
in pernicious anemia, iv, 635 
fever, i, 1012 
swelling in Hodgkin's disease, iv, 765 
system in typhoid fever, i, 145 
tuberculosis, i, 412 
Glaucoma in congenital cyanosis, iv, 339 
in trigeminal neuralgia, v, 734 
Glenard's disease, iii, 747 
Gliadins in nutrition, ii, 646 
Glioma of brain, v, 284, 311, 312 
Gliomatosis in syringomyelia, v, 213 
Gliosis in epilepsy, v, 599 
Glossitis, iii, 62, 63 
in typhoid fever, i, 103 
Glossodynia, iii, 63, 64 
Glosso-labio-laryngeal paralysis, v, 113 
Glossopharyngeal nerve, diseases of, v, 516 
Glossoplegia, iii, 62 
Glucose in urine, iii, 821 
Gluteal nerves, paralysis of, v, 558 
Glyciphagus ii, 323 
Glycocele, ii, 741 
Glycosuria, ii, 543; iii, 821 
in acromegaly, iv, 812 
alimentary, ii, 690 
brain tumor and, ii, 678; v, 338, 342 
in calculus of pancreas, iii, 635 
in carcinoma of pancreas, iii, 652 
cerebral hemorrhage and, ii, 678 
diseases of pancreas and, iii, 604, 606 
Glycosuria in exophthalmic goitre, iv, 868 
gall-stones and, ii, 678 
in gangrenous pancreatitis, iii 619 
in gout, ii, 750 
in interstitial pancreatitis, iii, 630 
in necrosis of pancreas, iii, 617 
in suppurative pancreatitis, iii, 625 
Glycuronic acid in metabolism, ii, 599 
in urine, iii, 823 
in diabetes mellitus, ii, 702 
Gnathostoma siamense, ii, 302 
Goitre, iv, 835 
aberrant, iv, 841 
acute, iv, 840 
cardiac insufficiency and, iv, 199 
chronic, iv, 840 
colloid, iv, 839 
exophthalmic, iv, 849 
abortive, iv, 870 
acute, iv, 870 
angioneurotic oedema and, iv, 
1000 
cardiac insufficiency and, iv, 201 
chronic, iv, 870 
enlargement of thymus and, iv, 
913 
hypertrophy of heart and, iv, 182 
incomplete, iv, 870 
iodine and, iv, 851, 857, 875 
thyroiditis and, iv, 829 
fibrous, iv, 840 
intralaryngeal, iv, 842 
intratracheal, iv, 842 
iodism and, iv, 823, 847 
myxoedema and, iv, 890 
parenchymatous, iv, 839 
radium and, iv, 838 
vascular, iv, 839 
water and, iv, 838 
Gonococcal adenitis, i, 761 
arthritis, i, 753 
bone lesions, i, 760 
chondritis, i, 760 
conjunctivitis, i, 746 
neonatorum, i, 747 
cystitis, i, 762 
endocarditis, i, 750 
erythema, i, 764 
nodosum, i, 764 
hyperkeratosis, i, 765 
infections, i, 743 
meningomyelitis, i, 761 
myocarditis, i, 752 
myositis, i, 760 
neuralgia, i, 761 
neuritis, i, 761 
pericarditis, i, 752 
perichondritis, i, 760 
peritonitis, i, 763; iii, 686 
phlebitis, i, 752 
polyneuritis, i, 761 
proctitis, i, 747 
sciatica, i, 761 
septicemia, i, 749 
stomatitis, i, 748; iii, 51 
tenosynovitis, i, 760 
urticaria, i, 764 1052 
GENERAL INDEX 
Gonococcal wound nfection, i, 748 
Gonococcic infections, chronic peritonitis 
and, iii, 714 
kidney infections and, iii, 952 
tuberculosis of kidney and, iii, 
978 
Gonococcus, i, 743 
chronic nephritis and, iii, 880 
Gonorrhoea, endocarditis and, iv, 153, 157 
lobar pneumonia following, i, 267 
myocarditis and, iv, 108 
pericarditis and, iv, 42 
typhoid fever and, i, 158 
Gonorrhoeal bubo, iv, 739 
heel, i, 760 
Gordius ii, 296 
Gout, ii, 729 
acute, ii, 745 
alcohol and, ii, 730 
arthritis deformans and, v, 930 
asthma and, ii, 933 
chronic, ii, 746 
diabetes mellitus and, ii, 677, 750 
lead poisoning and, ii, 731 
migraine and, v, 720 
phlebitis and, iv, 574 
trauma and, ii, 732 
Graefe's sign in exophthalmic goitre, iv, 
863 
Granular casts, iii, 799 
pharyngitis, ii, 845 
pyelitis, iii, 954 
Granuloma of brain, infectious, v, 319 
of oesophagus, iii, 85 
Gravel, sediment, iii, 1017 
Graves' disease, iv, 849 
Grocers' itch, ii, 323 
Groin glands, varicose, ii, 308 
Guinea-worm infection, ii, 303 
Gumma of arteries, ii, 181 
of brain, v, 320 
of heart, ii, 183 
of kidney, ii, 180 
of liver, ii, 176 
of lung, ii, 172 
of spleen, ii, 176 
of stomach, ii, 174 
in syphilis, ii, 150 
of trachea, ii, 170 
Gummatous syphiloderm, ii, 184 
Gums in infantile scurvy, ii, 806 
in scurvy, ii, 806 
in tuberculosis, i, 387; iii, 57 
in ulcerative stomatitis, iii, 48 
H 
Habit chorea, v, 784 
spasm, v, 784 
Haemonchus contortus, ii, 294 
Hallucinations, v, 37, 39 
Halzoun, ii, 236 
Hamularia equina, ii, 315 
Hands in achondroplasia, v, 962 
in aneurism of aorta, iv, 495 
in arthritis deformans, v, 912 
Hands in myxoedema, iv, 891 
in osteo-arthropathy, v, 984 
Hanot's cirrhosis, iii, 535 
Harelip, iii, 45 
Harris' immunization in rabies, i, 971 
Harvest mites, ii, 321 
Hay asthma, ii, 860 
fever, ii, 855 
American, ii, 859 
Head, actinomycosis of, i, 1038 
sweating in rickets, ii, 782 
tetanus, i, 579 
Headache, sick, v, 709 
Heart, abdominal, iv, 346 
in acute myocarditis, iv, 116 
in Addison's disease, iv, 789, 793 
air in, cardiac insufficiency and, iv, 
194 
in alcoholism, ii, 401, 403 
in amyloid kidney, iii, 935 
aneurism of, iv, 482 
of aorta and, iv, 499 
in myocarditis, iv, 115, 127 
anomalies of, iv, 350 
in aortic insufficiency, iv, 223 
apex of, bifid, iv, 350 
arhythmia of, iv, 29 
in arterial transposition, iv, 386 
in arteriosclerosis, iv, 462 
beat, alternation of, iv, 88 
mechanism of, iv, 78 
pathological, iv, 92 
premature contractions in, iv, 84 
rapid, iv, 81 
respiratory irregularity and, iv, 83 
slow, iv, 82 
biloculate, iv, 370 
cervical, iv, 346 
chambers of, displacement of, iv, 350 
in chlorosis, iv, 651 
in chronic nephritis, iii, 884, 898 
in cirrhosis of liver, iii, 518, 525 
in coarctation of aorta, iv, 427, 431 
in congestion of liver, iii, 481 
development of, iv, 323 
diastole of, iv, 22, 32 
digestive disturbances and, iv, 310 
dilatation of, iv, 30, 187, 189 
in diphtheria, i, 694, 702 
diseases of, congenital, iv, 323 
convulsions and, v, 794 
neuroses and, iv, 296 
displacements of, iv, 346 
diverticulum of, iv, 351 
in exophthalmic goitre, iv, 860 
fatty, in myocarditis, iv, 115 
fibrillation of, insufficiency and, iv, 
192 
functional diseases of, iv, 275 
gastric distension and, iv, 311 
in goitre, iv, 843 
in gout, ii, 749 
gumma of, ii, 183 
hypertrophy of, iv, 30, 166 
congenital, iv, 351 
defects of inter auricular septum 
and, iv, 359 GENERAL INDEX 
1053 
Heart, impulse of, iv, 21 
insufficiency of, iv, 187, 191 
acute, iv, 191 
chronic, iv, 198 
hemopericardium and, iv, 193 
right, iv, 197 
irritable, iv, 319 
in jaundice, iii, 453 
in lobar pneumonia, i, 226 
in lymphoid leukemia, iv, 671 
mechanism of, iv, 27, 278, 314 
muscle, degeneration of, iv, 190 
functions of, iv, 78, 187 
reserve power of, iv, 188, 216 
tonicity of, iv, 190 
in myxoedema, iv, 894 
pectoral, iv, 346 
in pericarditis, iv, 45, 48 
in pernicious anemia, iv, 632 
in plague, i, 628 
in Raynaud's disease, iv, 990 
in rheumatic fever, i, 984 
rupture of, hemopericardium and, iv, 
70 
insufficiency and, iv, 193 
in myocarditis, iv, 115, 127 
in scarlet fever, i, 865, 877 
senile, myocarditis and, iv, 126 
septum of, absence of, iv, 370 
anomalies of, iv, 352 
development of, iv, 370 
in serofibrinous pleuritis, ii, 1022 
sexual organs and, iv, 316 
standstill of, iv, 83 
in Stokes-Adams syndrome, iv, 307 
strain, iv, 82 
myocarditis and, iv, 109 
syphilis of, ii, 183 
of myocardium and, iv, 143 
in toxemia, i, 563 
triloculate, iv, 372 
in tuberculosis, i, 385, 433 
in typhoid fever, i, 125 
valves of, aneurism of, iv, 482 
lesions of, combined, iv, 259 
rupture of, aortic insufficiency 
and, iv, 219, 227 
insufficiency and, iv, 193 
weakness, iv, 319 
work of, iv, 33 
wounds of, insufficiency and, iv, 191 
Heart-block, iv, 83 
in lobar pneumonia, i, 257 
Stokes-Adams syndrome and, iv, 306 
ventricular systole and, iv, 89 
Hearts, multiple, iv, 350 
Heat, burns from, ii, 348 
exhaustion, ii, 330 
Heberden's nodes, v, 921 
Heel, gonorrhceal, i, 760 
Helminthoma elasticum, ii, 308 
Hemangioma of intestines, iii, 434 
Hemarthrosis in hemophilia, iv, 350 
Hematemesis, iii, 279 
Hematochyluria bilharzian, ii, 239 
in filariasis, ii, 309 
Hematogenous jaundice, iii, 447 
Hematology, main data of, iv, 605 
Hematoma in hemophilia, iv, 723 
retroperitoneal, iii, 738 
Hematomyelia, v, 260 
in cervical enlargement, v, 262 
in conus medullar is, v, 262 
in lumbosacral enlargement, v, 262 
in purpura hemorrhagica, iv, 712 
Hematopoiesis in spleen, iv, 934 
Hematoporphyrinuria, iii, 809 
Hematorrachis, v, 258 
in purpura hemorrhagica, iv, 712 
Hematuri , iii, 802 
in carcinoma of prostate, iii, 1038 
in chronic nephritis, iii, 901 
Egyptian, ii, 239 
in embolism, iv, 558 
endemic, ii, 239 
in Henoch's purpura, iv, 710 
in hypernephroma, iii, 996 
in infantile scurvy, ii, 806 
in nephroptosis, iii, 756 
in polymyositis hemorrhagica, v, 879 
in purpura hemorrhagica, iv, 711 
in renal infarction, iv, 564 
, in sarcoma of kidney, iii, 1003 
in tuberculosis of kidney, iii, 984 
in tumors of kidney, 1005 
in typhoid fever, i, 143 
Hemeralopia, v, 35, 480 
Hemiachromatopsia in brain tumors, v, 334 
Hemiamblyopia, v, 34 
Hemianesthesia in hysteria, v, 681 
in lesion of internal capsule, v, 276 
of pons, v, 290 
Hemianopic pupillary reaction, v, 482 
Hemianopsia, v, 34, 270, 480 
in brain abscess, v, 381, 382, 384 
tumor, v, 332, 334 
in hemiplegia, v, 468 
heteronyma, v, 34 
homonyma, v, 34 
in lesion of internal capsule, v, 276 
quadrantic, v, 34 
Hemiataxia in abscess of brain, v, 381, 384 
Hemiathetosis, v, 70 
Hemiatrophy, facial, v, 991 
Hemicardia, iv, 350 
Hemichromatopsia, v, 34 
Hemicontracture in lesion of internal cap- 
sule, v, 277 
Hemicrania, v, 709 
Hemihyperesthesia in hysteria, v, 681 
Hemiparesis in abscess of brain, v, 384 
Hemiparesthesia in migraine, v, 712 
Hemiplegia in acute miliary tuberculosis, 
i, 421 
apoplexy and, v, 412, 457 
cerebral, congenital, v, 409 
in diabetes mellitus, ii, 707 
hysteria and, v, 424, 678 
infantile, v, 412 
in lesion of internal capsule, v, 276 
of optic thalamus, v, 279 
of pons, v, 290 
of pontomedullocerebellar space, 
v, 290 1054 
GENERAL INDEX 
Hemiplegia in lobar pneumonia, i, 262 
Hemochromatosis, ii, 681; iii, 529, 606 
interstitial pancreatitis and, iii, 630 
portal cirrhosis of liver and, iii, 510 
Hemofuscin in liver, iii, 470 
Hemoglobin, estimation of, iv, 607 
Hemoglobinuria, iii, 818 
in angioneurotic oedema, iv, 1008 
in blackwater fever, ii, 113 
in Raynaud's disease, iv, 989 
Hemohydrothorax, ii, 1054 
Hemolymph nodes, iv, 613, 753 
in Banti's disease, iv, 753 
in leukemia, iv, 753 
in malarial fever, iv, 754 
in mercuric chloride poisoning, 
iv, 754 
in pernicious anemia, iv, 753 
in posthemorrhagic anemia, iv, 
753 
in pseudomelanosis, iv, 753 
in trypanosomiasis, iv, 754 
Hemolysis, secondary anemia and, iv, 655 
in spleen, iv, 934 
Hemopericardium, iv, 70 
cardiac insufficiency and, iv, 193 
Hemoperitoneum, iii, 669 
Hemophilia, iv, 717 
Hemopneumopericardium, iv, 71 
Hemopoietic tissues, atypical, sarcoma- 
like growth of, iv, 679 
Hemoptysis, ii, 951 
Hemorrhage, cerebral, v, 401, 410, 411, 
414, 415 
extradural, v, 257 
intestinal, iii, 307 
intradural, v, 258 
mediastinal, ii, 1110 
into spinal cord, v, 260 
membranes, v, 257 
Hemorrhagic chickenpox, i, 852 
cysts of spleen, iv, 951 
diarrhoea in embolism, iv, 558 
diathesis in vaccination, i, 842 
erosions of stomach in uremia, iii, 838 
exanthemata in syphilis, ii, 189 
exudate in pericarditis, iv, 44 
necrosis of pancreas, iii, 610 
pericarditis, iv, 50 
pleural fluids, ii, 1053 
pleurisy, ii, 1053 
pustular smallpox, i, 804 
septicemia, i, 558 
typhoid fever, i, 153 
Hemorrhoids, intestinal hemorrhage and, 
iii, 307 
Hemoserothorax, ii, 1053 
Hemosiderin in liver, iii, 470 
in malarial fever, ii, 68 
Hemothorax, ii, 1054 
Henoch's purpura, iv, 708 
Hepatic artery, abscess of liver and, iii, 497 
aneurism of, iii, 458 
dilatation of, iii, 458 
embolism of, iii, 458; iv, 569 
thrombosis of, iii, 458 
distomatosis, ii, 229 
Hepatic infarction, iv, 569 
intermittent fever, iii, 582 
splanchnoptosis, iii, 754 
toxemia, myocarditis and, iv, 124 
veins, abscess of liver and, iii, 497 
dilatation of, iii, 459 
embolism of, iii, 460 
thrombosis of, iii, 460; iv, 545 
Hepatitis, acute, iii, 494 
chronic interstitial, iii, 505 
suppurative, iii, 496 
Hepatogenous jaundice, iii, 447 
Hepatoptosis, iii, 458, 757 
Hepatopulmonary abscess, ii, 43, 48 
Hereditary cerebellar ataxia, Marie's, v, 
130, 236 
spastic spinal paralysis, v, 105 
Hernia, foraminal, of medulla, v, 324 
internal, intestinal obstruction from, 
iii, 405 
intestinal hemorrhage and, iii, 307 
mediastinal, ii, 1109 
of pericardium, iv, 346 
Herpes in arsenic poisoning, ii, 375 
buccalis, iii, 51 
in influenza, i, 545 
labialis, iii, 45 
lymphangitis and, iv, 592 
of oesophagus, iii, 90 
in pneumonia, i, 232, 424 
in typhoid fever, i, 90, 101 
zoster, v, 571 
of auricle, v, 505 
in chorea, v, 781 
in migraine, v, 719 
in tuberculosis, i, 393, 435 
in tumors of lung, ii, 1000 
Herpetic stomatitis, iii, 51 
Herpetiform scleroderma, iv, 1024 
Heterophytes heterophytes, ii, 238 
Heterotaxy, incomplete, iv, 349 
Hexathyridium venanum, ii, 235 
Hexosephosphate, metabolism of, ii, 562 
Hip-joint in arthritis deformans, v, 913, 
922 
Hippocratic fingers in biliary cirrhosis of 
liver, iii, 539 
in tuberculosis, i, 411 
Hirudinei, ii, 316 
His, bundle of, development of, iv, 328 
Histrogaster spermaticus, ii, 323 
Hodgkin's disease, iv, 751, 755 
Hoffman's symptom, v, 71 
Holothyrus coccinella, ii, 323 
Homogentisic acid, ii, 655 
in urine, iii, 824 
Hookworm disease, ii, 271 
Horner's complex, iii, 103 
Hour-glass stomach, iii, 182 
Human trypanosomiasis, ii, 119, 127 
1 Hunger, sense of, v, 193 
Hunterian chancre, ii, 154 
Huntington's chorea, v, 787 
Hutchinson's teeth, iii, 59 
Hyaline casts, iii, 799 
degeneration of lymph nodes, iv, 746 
of myocardium, iv, 116 GENERAL INDEX 
1055 
Hyaline perihepatitis, iii, 488 
Hyaloserositis, iv, 60, 63, 64 
Hydatid cyst of brain, v, 319 
of liver, subphrenic pyopneumo- 
thorax and, iii, 711 
of pericardium, iv, 76 
of peritoneum, iii, 733 
of spleen, iv, 953 
disease, ii, 264 
Hydrocephalus, v, 351 
acquired, v, 356 
epilepsy and, v, 599 
essential, v, 353 
externus, v, 353 
idiopathic, v, 353 
internus, v, 353 
in meningitis, i, 610, 618 
in rickets, ii, 789 
secondary, v, 356 
in tumor of brain, v, 338 
Hydromyelia, v, 210 
Hydronephrosis, renal calculus and, iii, 
1018 
tuberculosis of kidney and, iii, 978 
Hydropericardium, iv, 69 
Hydrophobia, i, 956 
Hydropneumopericardium, iv, 71 
Hydrothorax, ii, 1052 
in aneurism of aorta, iv, 508 
in mitral insufficiency, iv, 236 
Hymenolepis diminuta, ii, 246, 254 
lanceolata, ii, 246, 254 
nana, ii, 245, 252 
Hypacusis, v, 33 
Hypalgia in tabes dorsalis,'v, 838 
Hyperacidity, iii, 112 
in chronic gastritis, iii, 242 
in duodenal ulcer, iii, 643 
larval, iii, 121 
ulcer of stomach and, iii, 114, 171 
Hyperalgesia, v, 30, 33 
in appendicitis, iii, 367 
in diseases of peripheral nerves, v, 539 
in herpes zoster, v, 572 
hysteria and, v, 682 
Hyperchlorhydria, iii, 112 
in chlorosis, iv, 651 
Hyperchondroplasia, v, 969 
Hyperemia of adrenal glands, iv, 782 
of brain, v, 392 
hypertrophy of heart and, iv, 168 
of kidney, iii, 788 
in Raynaud's disease, iv, 983, 985 
of spinal cord, v, 257 
Hyperepinephy, iv, 779 
Hyperesthesia, v, 28 
in acute meningitis, v, 170 
in beriberi, ii, 495 
in cerebrospinal meningitis, i, 607 
in embolism of arteries, iv, 568 
in erythromelalgia, iv, 1028 
gastrica, iii, 137 
in hysteria, v, 681 
of larynx, v, 522 
in leprosy, i, 526 
in occupation neuroses, v, 747 
in pernicious anemia, iv, 631 
Hyperesthesia in Raynaud's disease, iv, 
987 
in tabes dorsalis, v, 836 
in thrombosis of arteries, iv, 568 
in tuberculosis, i, 501 
Hyperkeratosis, gonococcal, i, 765 
of tongue, iii, 61 
Hypermetropia, v, 36 
Hypermnesia, v, 53 
Hypernephroma, iii, 994 
of adrenal glands, iv, 784 
tuberculosis and, iii, 978 
Hyperopia, v, 36 
Hyperostosis, v, 969 
of skeleton with cyst-building, v, 978 
of skull, v, 988, 989 
Hyperplasia of adrenal glands, iv, 781 
of thymus, iv, 912 
tuberculosis of lymph nodes and, iv, 
741 
Hyperplastic bone-marrow, iv, 612 
intestinal tuberculosis, iii, 390 
Hyperpnoea in cardiac insufficiency, iv, 206 
Hyperptyalism, iii, 68 
Hyperpyrexia in tetanus, i, 578 
Hypersarcosis, iv, 596 
Hypersecretion of gastric juice, iii, 122 
Hyperthymia, v, 60 
Hyperthyroidism, chlorosis and, iv, 648 
myocarditis and, iv, 113 
Hypertony of muscles, v, 68 
Hypertrophic biliary cirrhosis, iii, 535 
internal pachymeningitis, v, 166 
osteopathies, v, 969 
pulmonary osteo-arthropathy, i, 411 
stenosis of pylorus, iii, 263 
Hypertrophy of adrenal glands, iv, 781 
of auricles, iv, 184, 185 
cardiac, in myocarditis, iv, 121 
of heart, iv, 30, 116 
congenital, iv, 351 
defects of interauricular septum 
and, iv, 359 
of muscles, v, 68 
of pharyngeal tonsil, ii, 840 
of prostate, iii, 1035 
kidney infections and, iii, 952 
of thymus, iv, 912 
of ventricles, iv, 185 
Hypesthesia in disease of spinal roots, v, 
561 
in tabes dorsalis, v, 838 
Hyphomycetic stomatitis, iii, 50 
Hypochlorhydria, iii, 152 
Hypochondriasis in constipation, iii, 314 
Hypo-epinephy, iv, 779 
Hypogastric reflexes, v, 78 
Hypoglossal nerve, diseases of, v, 530 
Hypomnesia, v, 53 
Hypophysis cerebri, iv, 803 
in exophthalmic goitre, iv, 857 
in myxoedema, iv, 890 
Hypopituitarism, iv, 815 
in brain tumor, v, 342 
Hypoplasia of adrenal glands, iv, 781 
of aorta, iv, 431 
aneurism and, iv, 478 1056 
GENERAL INDEX 
Hypoplasia of aorta, defects of interauri- 
cular septum and, iv, 359 
Hypothymia, v, 61 
Hypothyroidism, iv, 880 
Hypotonia in lesion of cerebellum, v, 287 
in tabes dorsalis, v, 839 
Hypotony of muscles, v, 68 
Hypotyalism, iii, 68 
Hysteria, v, 654 
adductor paralysis and, v, 521 
angioneurotic oedema and, iv, 1001 
astasia-abasia and, v, 680 
dementia praecox and, v, 695 
electrical shock and, ii, 341 
exophthalmic goitre and, iv, 870 
hemiplegia and, v, 419, 424, 678 
humoralism and, v, 654 
mythomania and, v, 659 
pithiatism and, v, 659 
psychotherapy and, v, 702 
traumatic, v, 644, 691 
in typhoid fever, i, 136 
Hysterical angina pectoris, iv, 295 
astasia-abasia, v, 815 
disseminated sclerosis, v, 154 
insanity, trauma and, v, 647 
I 
Ichthyosis buccalis, iii, 64 
Icterus in blackwater fever, ii, 114 
neonatorum, iii, 450 
in purpura hemorrhagica, iv, 711 
Ideas, associative, anomalies of, v, 51 
autochthonus, v, 52 
exaggerated, v, 52 
flight of, v, 46 
hyperdynamic, v, 52 
imperative, v, 52 
intensity of, anomalies of, v, 52 
Ideation, anomalies of, v, 46 
Idiocy, alcoholism and, ii, 407 
amaurotic family, v, 863 
in endemic cretinism, iv, 882 
in sporadic cretinism, iv, 885 
Idiopathic abscess of brain, v, 385 
dilatation of colon, iii, 320 
phlebitis, iv, 574 
purpura, iv, 691 
recurrent thrombophlebitis, iv, 531 
Ileocolitis in measles, i, 906 
Ileum, carcinoma of, iii, 428 
Ileus, iii, 394 
Illuminating gas poisoning, ii, 388 
Illusions, v, 37, 38, 55 
Imbecility, epilepsy and, v, 594 
Immunity, i, 35, 46, 65 
Impetigo in congenital syphilis, ii, 190 
contagiosa in scarlet fever, i, 878 
in vaccination, i, 843 
varicellosa, i, 852 
Inanition toxemia, myocarditis and, iv, 124 
Incarceration of intestines, iii, 394, 406 
Incoherence, v, 43, 51 
Incompetence, myocardial, of obese, iv, 128 
Incontinence of pylorus, iii, 159 
Incoordination in syringomyelia, v, 221 
Indian liver-fluke disease, ii, 234 
Indicanuria, ii, 509, 667; iii, 811 
Infantilism, v, 967 
Infarction, embolism and, iv, 556 
gastric ulcer and, iii, 172 
hepatic, iv, 569 
intestinal hemorrhage and, iii, 307 
of kidney, iii, 789; iv, 564 
of lung, ii, 954; iv, 563 
pulmonary, iv, 563 
of spleen, iv, 946 
in typhoid fever, i, 122, 129 
Infection, aerial, i, 21 
bronchial, i, 27 
droplet, i, 24 
duct, i, 21 
gastro-intestinal, i, 27 
germinal, i, 28 
mixed, i, 31 
nasopharyngeal, i, 26 
oral, i, 26 
secondary, i, 31 
water-borne, i, 24 
Infectious agents, i, 18 
arthritis, v, 896 
jaundice, i, 1014 
Infective myelitis, v, 229 
oesophagitis, iii, 81 
Inferior vena cava, thrombosis of, iv, 541 
Infiltration, fatty, ii, 604 
Inflammation of adrenal glands, iv, 783 
of brain, v, 359 
of geniculate ganglia, v, 505 
of labial glands, iii, 45 
of lymphatic glands, iv, 730 
of mouth, iii, 45 
of oesophagus, iii, 79 
of optic nerve, v, 476 
of parotid gland, iii, 69 
of spleen, iv, 940 
in syphilis, ii, 151 
of thoracic duct, iv, 582 
of thymus, iv, 928 
of thyroid gland, iv, 825 
of ureter, iii, 974 
Influenza, i, 534 
acute endocarditis and, iv, 158 
arteriosclerosis and, iv, 454 
arthritis deformans and, v, 901 
cardiac arhythmia and, iv, 288 
chronic, i, 541 
gastro-intestinal, i, 543 
genito-urinary, i, 545 
lobar pneumonia and, i, 207, 266, 542 
lymphadenitis and, iv, 737 
myocarditis and, iv, 108, 117 
nephritis and, iii, 851 
nervous, i, 542 
neurasthenia and, v, 629 
respiratory, i, 539 
sporadic, i, 534 
tuberculosis and, i, 321 
typhoid fever and, i, 158 
typical, i, 538 
Influenzal encephalitis, v, 362 
Inguinal glands, lymphadenitis of, iv, 736 GENERAL INDEX 
1057 
Inguinal glands, tuberculosis of, iv, 744 
Inhalation pneumonia, i, 206 
Innervation of lungs, ii, 814 
of respiratory organs, ii, 814 
Insanity, aneurism and, iv, 478 
epileptic, trauma and, v, 647 
hysterical, trauma and, v, 647 
lobar pneumonia and, i, 208 
in pellagra, ii, 485 
Insects, parasitic, ii, 324 
Insolation, ii, 331 
Insufficiency, aortic, iv, 217 
of heart, iv, 191 
mitral, iv, 232 
congenital, iv, 407 
pulmonary, iv, 255 
tricuspid, iv, 244 
congenital, 407 
Insular sclerosis, v, 140 
Interacinar pancreatitis, iii, 629 
Interauricular septum, defects of, iv, 355 
development of, iv, 325 
Intercostal neuralgia, v, 739 
Interlobar empyema, ii, 1050 
Interlobular pancreatitis, iii, 629 
Intermittent claudication in arteriosclero- 
sis, iv, 465 
in thrombo-angeitis obliterans, 
iv, 547 
disseminated sclerosis, v, 15 I 
gastromyxorrhcea, iii, 130 
oedema, iv, 998 
Interstitial emphysema of lungs, ii, 982 
hypertrophic progressive neuritis of 
childhood, v, 112 
myocarditis, iv, 113, 114 
pancreatitis, iii, 626 
Interventricular septum, defects of, iv, 363 
development of, iv, 327 
undefended space of, aneurism of, 
iv, 369 
Intestinal anthrax, i, 645 
colic, iii, 303 
crises in tabes dorsalis, v, 842 
disease, tetany and, v, 749 
distomatosis, ii, 237 
flora, relationships of, ii, 652 
hemorrhage, iii, 307 
in amoebic dysentery, ii, 44 
in lobar pneumonia, i, 254 
in tuberculosis, i, 419 
in typhoid fever, i, 108 
indigestion, iii, 301, 324 
infection, arthritis deformans and, v, 
903 
parasites, anemia and, iv, 623, 641, 659 
in lobar pneumonia, i, 254 
plague, i, 635 
processes, automatic mechanism in, 
iii, 291 
roundworms, ii, 271 
sand, iii, 295, 440 
teniasis, ii, 245 
Intestines, adenoma of, iii, 433 
in amoebic dysentery, ii, 29 
amyloid disease of, iii, 392 
antiperistalsis of, iii, 287 
vol. v-67 
Intestines, arteriosclerosis of, iii, 437 
in Asiatic cholera, i, 681 
in bacillary dysentery, i, 772, 775 
bacillus aerogenes in, iii, 296 
bifidus communis in, iii, 296 
coli in, iii, 296 
lactis aerogenes in, iii, 296 
putrificus in, iii, 296 
bacteria in, iii, 295 
carcinoma of, iii, 424 
peritonitis and,'iii, 426 
compression of, iii, 394 
constriction of, iii, 394 
descent of, iii, 757 
diseases of, peritonitis and, iii, 672 
diverticula of, iii, 420 
gaseous distension of, iii, 298 
hemangioma of, iii, 434 
human, amoeba of, ii, 18 
incarceration of, iii, 394 
lipoma of, iii, 433 
lymphangioma of, iii, 434 
lymphosarcoma of, iii, 431 
motor function of, iii, 286 
movements of, iii, 287 
myxoma of, iii, 433 
neuralgia of, iii, 305 
obstruction of, iii, 394 
acute, iii, 396 
in cholelithiasis, iii, 584 
chronic, iii, 395, 397 
obturation of, iii, 394 
occlusion of, iii, 394 
paralysis of, in peritonitis, iii, 681 
perforation of, in gastric ulcer, iii, 186 
in typhoid fever, i, 111 
peristalsis of, iii, 286 
rhythmic segmentation of, iii, 286 
sarcoma of, iii, 431 
in scarlet fever, i, 865 
strangulation of, iii, 394 
stricture of, iii, 394 
syphilis of, ii, 175; iii, 391 
tuberculosis of, i, 361, 432; iii, 387 
tumors of, iii, 424, 433 
in typhoid fever, i, 82, 105 
ulcer of, iii, 343, 838 
Intoxications, adrenal glands and, iv, 781 
aneurism and, iv, 476 
arteriosclerosis and, iv, 455 
nephritis and, iii, 880 
spleen and, iv, 936 
Intracellular enzymes, ii, 663 
Intracerebellar tumors, v, 338 
Intracranial pressure, v, 391 
Intradural hemorrhage, v, 258 
Intralaryngeal goitre, iv, 842 
Intra-ocular muscles, paralysis of, v, 486 
Intraperitoneal subphrenic abscess, iii, 703 
Intrapleural pressure in pneumothorax, ii, 
1072 
tension in serofibrinous pleuritis, ii, 
1014 
Intratracheal goitre, iv, 842 
Intra-uterine fracture, v, 979 
rickets, v, 955 
Intubation in diphtheria, i, 723 1058 
GENERAL INDEX 
Intussusception, intestinal obstruction 
from, iii, 409 
typhoid fever and, i, 159 
Inverting enzymes, iii, 290 
Iodide, purpura and, iv, 689 
Iodine, exophthalmic goitre and, iv, 851, 
857 
thyroid gland and, iv, 823 
lodiococcus vaginatus in mouth, iii, 40 
lodism, ii, 209 
Irritability, pathological, v, 61 
Irritable heart, iv, 319 
Irritation, motor, v, 70 
Ischemia of kidney, iii, 787 
of myocardium, iv, 290 
in Raynaud's disease, iv, 983 
Ischiorectal abscess in tuberculosis, i, 432 
Ischuria, iii, 791, 806 
Islands of Langerhans, diabetes mellitus 
and, ii, 680, 693 
Itch, grocer's, ii, 323 
sarcoptic, ii, 318 
Itching in uremia, iii, 840 
Ixodiasis, ii, 317 
J 
Jacksonian epilepsv, v, 592 
Jackstone calculus, iii, 1009 
Japanese liver-fluke disease, ii, 230 
Jaundice, iii, 446, 552 
in abscess of liver, iii, 500 
in acute cholangitis, iii, 553 
cholecystitis, iii, 565 
enteritis, iii, 330 
gastritis, iii, 229 
hepatitis, iii, 495 
yellow atrophy of liver, iii, 475 
in aneurism of hepatic artery, iii, 459 
in biliary cirrhosis of liver, iii, 538 
in calculus of pancreas, iii, 635 
in carcinoma of gall-bladder, iii, 596 
of liver, iii, 542 
of pancreas, iii, 650, 652 
catarrhal, iii, 448, 552 
in cholelithiasis, iii, 579 
in chronic cholangitis, iii, 560 
peritonitis, iii, 714 
chronic splenomegalic hemolytic, iii, 
450 
in cirrhosis of liver, iii, 520 
of stomach, iii, 278 
in congestion of liver, iii, 479, 484 
in cyst of pancreas, iii, 645 
in gangrenous pancreatitis, iii, 619 
in gastritis, iii, 234 
in Hodgkin's disease, iv, 766 
hematogenous, iii, 447 
hepatogenous, iii, 447 
in hypernephroma, iii, 997 
infectious, i, 1014 
in lobar pneumonia, i, 259 
in mitral insufficiency, iv, 236 
nephritis and, iii, 854 
in newborn, iii, 450 
obstructive, iii, 447 
in pernicious anemia, iv, 628 
Jaundice in portal thrombosis, iv, 544 
in scarlet fever, i, 879 
simple, iii, 448 
in splenic anemia, iv, 958 
in suppurative cholangitis, iii, 557 
pancreatitis, iii, 624 
in syphilis, ii, 158 
in tuberculosis of peritoneum, iii, 726 
in typhoid fever, i, 118 
in typhus fever, i, 930 
j Jaw, actinomycosis of, iii, 59 
Jejunal peptic ulcer, iii, 349 
Jejunum, carcinoma of, iii, 428 
Jigger flea, ii, 324 
Joints in hemophilia, iv, 724 
in purpura rheumatica, iv, 703 
in Raynaud's disease, iv, 990 
in scleroderma, iv, 1021 
in syringomyelia, v, 225 
tuberculosis of, i, 371 
Jumping fleas, ii, 325 
Juvenile vomiting, iii, 145 
K 
i Kakke, ii, 489 
Kala-azar, iv, 964 
acute hepatitis and, iii, 494 
Keratitis in lobar pneumonia, i, 262 
Keratosis in arsenic poisoning, ii, 375 
buccalis, iii, 64 
Kernig's sign in cerebrospinal meningitis 
i, 599, 605; v, 170 
in tuberculosis, i, 422 
Ketone complex, ii, 546 
Kidney, abscess of, iii, 937 
in bronchiectasis, ii, 917 
absence of, iii, 785, 937 
acute dilatation of stomach and, iii, 
251 
in Addison's disease, iv, 795 
adenoma of, iii, 1003 
malignant papillary cyst, iii, 1002 
in alcoholism, ii, 404 
amyloid, iii, 933 
tuberculosis and, iii, 933 
anemia of, iii, 787 
arteries of, anomalies of, iii, 786 
arteriosclerotic, iii, 847, 897; iv, 463 
in Asiatic cholera, i, 681 
in blackwater fever, ii, 112 
carcinoma of, iii, 1001 
casts and, iii, 774 
circulatory disturbances of, iii, 787 
in cirrhosis of liver, iii, 518, 526 
cystic congenital, iii, 786 
in diabetes insipidus, ii, 722 
mellitus, ii, 696 
in diphtheria, i, 695 
displacements of, iii, 786 
excretion of solids by, iii, 768 
of water by, iii, 767 
in exophthalmic goitre, iv, 868 
functional activity of, iii, 942 
competence of, iii, 781 
test of, iii, 781 GENERAL INDEX 
1059 
Kidney in gout, ii, 744 
gumma of, ii, 180 
in Hodgkin's disease, iv, 764 
hyperemia of, iii, 788 
hypernephroma of, iii, 994 
infarct of, iii, 789 
infections of, iii, 951; iv, 564 
in influenza, i, 545 
internal secretion of, iii, 769, 833 
interstitial tissue of, in nephritis, iii, 
859,897 
ischemia of, iii, 787 
in lead poisoning, ii, 362 
in malarial fevers, ii, 71 
malformations of, iii, 785 
mobility of, iii, 786 
in nephritis, iii, 857, 881, 895 
parathyroid glands and, iv, 904 
pelvis of, tumors of, iii, 1006 
phthalein test of, iii, 781 
position of, anomalies of, iii, 786 
of pregnancy, iii, 867 
in renal calculus, iii, 1017 
sarcoma of, iii, 1003 
in scarlet fever, i, 866 
secondary contracted, iii, 882, 884 
shape of, anomalies of, iii, 786 
in smallpox, i, 792 
surgical, iii, 937 
syphilis of, ii, 179 
toxins and, iii, 773 
tuberculosis of, i, 365; iii, 976, 979, 981 
pyogenic infections and, iii, 952 
tubules of, in acute nephritis, iii, 859 
in chronic nephritis, iii, 856 
disturbances of, iii, 772 
tumors of, iii, 994, 1003, 1005 
in typhoid fever, i, 86 
variegated, iii, 882 
Kinanesthesias, v, 30 
Klumpke palsy, v, 552 
Knee-jerk, v, 77 
in tabes dorsalis, v, 837 
Knee-joint in arthritis deformans, v, 913, 
923 
Koch's bacillus emulsion, i, 305 
tuberculin R., i, 305 
Koplik spots in measles, i, 900 
Kopp's asthma, iv, 918 
Korsakow's psychosis, ii, 427; v, 45 
alcoholism and, ii, 406, 427 
Kussmaul's sign in adherent pericardium, 
iv, 62 
L 
Labial glands, inflammation of, iii, 45 
Labyrinth, choked, in brain tumor, v, 328 
Lacrimal glands, enlargement of, iii, 73 
Lactescent ascites, iii, 662 
Lactosuria, ii, 600; iii, 822 
Lancet fluke infection, ii, 233, 234 
Landry's paralysis, v, 247 
Language, zone of, v, 293 
Larval hyperacidity, iii, 121 
Laryngeal diphtheria, i, 698 
muscles, spasm of, v, 522 
Laryngeal nerve, paralysis of, v, 518 
in patent ductus arteriosus, iv, 
418 
in thoracic aneurism, iv, 502 
tuberculosis, i, 412 
Laryngismus stridulus, ii, 880 
Laryngitis, acute, ii, 868 
chronic, ii, 870 
in influenza, i, 537, 540 
in lobar pneumonia, i, 223, 248 
in measles, i, 906 
oedematous, ii, 878 
in rheumatic fever, i, 996 
syphilitic, ii, 871 
tuberculous, i, 348; ii, 876 
Larynx in acromegaly, iv, 810 
in angioneurotic oedema, iv, 1007 
in disease of vagus nerve, v, 517 
in hemiplegia, v, 459 
in myasthenia gravis, v, 804 
obstruction of, ii, 816 
oedema of, ii, 878 
in angioneurotic oedema, iv, 1005, 
1007 
lobar pneumonia and, i, 248 
paralysis of, ii, 867 
sensory neuroses of, v, 522 
syphilis of, ii, 871 
tuberculosis of, i, 348; ii, 876 
in typhoid fever, i, 121 
Lasegue's sign in sciatica, v, 563 
Lateral sclerosis, v, 101 
Lathyrism, v, 139 
Lead colic, iii, 306 
poisoning, ii, 355 
amyotrophiolateral sclerosis and, 
v, 89 
arteriosclerosis and, iv, 455 
in children, ii, 369 
secondary anemia and, iv, 658 
Lecithins, metabolism of, ii, 562 
Legs, hereditary oedema of, iv, 1011 
Leishmania, iv, 964 
Leishmaniasis, iv, 964 
Lenticular degeneration, v, 278 
nucleus, lesion of, v, 278 
zone, lesion of, v, 278 
Leontiasis ossea, v, 988 
Leprosy, i, 522 
of bronchi, ii, 906 
of lymph nodes, iv, 746 
of mouth, iii, 59 
nerve, i, 527 
tubercle, i, 528 
Leptodera pellio, ii, 316 
Leptomeninges in syphilitic myelitis, v, 
239 
Leptomeningitis, v, 167 
Leptothrix buccalis in mouth, iii, 40 
Leptus americanus, ii, 321 
autumnalis, ii, 321 
irritans, ii, 321 
Leukanemia, iv, 682 
Leukemia, iv, 659 
acute, iv, 674, 677 
atypical, iv, 679, 682 
lymphoid, iv, 670 1060 
GENERAL INDEX 
Leukemia, mixed, iv, 679 
myeloid, iv, 661 
Leukemic blood without leukemic change 
in blood-making organs, iv, 680 
infiltration of liver, iii, 471 
lymphocytoma, iv, 748 
ulcers, iii, 352 
Leukoblastic bone-marrow, iv, 611 
Leukocytes in chlorosis, iv, 652 
in Hodgkin's disease, iv, 769 
in lymphoid leukemia, iv, 673 
in myeloid leukemia, iv, 667 
in pernicious anemia, iv, 638 
in polycythemia, iv, 685 
in splenic anemia, iv, 957 
in tuberculosis, i, 383 
in typhoid fever, i, 130 
Leukocytosis, i, 40; iv, 614 
in abscess of liver, iii, 502 
in acute cholecystitis, iii, 565 
meningitis, v, 171 
in appendicitis, iii, 369 
in arthritis deformans, v, 916 
in cerebrospinal meningitis, i, 605 
in erysipelas, i, 588 
in fat embolism, iv, 560 
in gangrenous pancreatitis, iii, 618 
in Henoch's purpura, iv, 710 
in infections, iv, 614 
in inflammation of thoracic duct, iv, 
583 
in lobar pneumonia, i, 234 
in lymphadenitis simplex, iv, 731 
in necrosis of pancreas, iii, 617 
in purpura, iv, 700 
in rubella, i, 914 
in smallpox, i, 808 
in subphrenic pyopneumothorax, iii, 
712 
in suppurative cholangitis, iii, 558 
pancreatitis, iii, 625 
in tetanus, i, 578 
toxic, iv, 614 
in tuberculosis, i, 419 
of peritoneum, iii, 726 
in venous thrombosis, iv, 539 
in whooping cough, i, 659 
Leukoderma, ii, 186 
in scleroderma, iv, 1019 
Leukolysis in lobar pneumonia, i, 235 
Leukoplakia, iii, 64 
Lcvulosuria, ii, 600; iii, 821 
in hepatic disease, iii, 445 
Lichen planus of mouth, iii, 54 
scrofulosum, i, 375 
Lid reflex, v, 78 
Liebig's theory of protein metabolism, ii, 
591 
Ligaments in rickets, ii, 782 
Limnatis nilotica, ii, 316 
Line® atrophica) in typhoid fever, i, 102 
Lingua nigra, iii, 61 
Linguatula serrata, ii, 323 
Linguopapillitis, iii, 62 
Linitis, plastic, iii, 273 
Lipase, iii, 289 
Lipemia in diabetes mellitus, ii, 695 
Lipins, ii, 605 
Lipogenous diabetes, ii, 676 
Lipoids, ii, 605, 648 
Lipoma of brain, v, 317 
of intestines, iii, 433 
of lung, ii, 998 
of peritoneum, iii, 732 
of thymus, iv, 932 
Lipomatosis multiple, v, 818 
universalis, ii, 761 
Lips, actinomycosis of, iii, 59 
double, iii, 44 
eczema of, iii, 45 
eruptions of, iii, 45 
fissure of, iii, 44 
tuberculosis of, iii, 57 
tumors of, iii, 45 
Litheinia, ii, 747 
Lithemic splanchnoptosis, iii, 754 
Lithiasis, bronchopulmonary, ii, 925 
Lithosis, ii, 976 
Lithuria, iii, 712 
Litten's sign in pleural effusion, ii, 1023 
Little's disease, v, 419 
Liver, abscess of, iii, 496 
in amoebic dysentery, ii, 38 
subphrenic abscess and, iii, 700 
in typhoid fever, i, 118 
actinomycosis of, iii, 541 
in acute hepatitis, iii, 495 
acute yellow atrophy of, iii, 472 
in alcoholism, ii, 403 
amyloid, iii, 469 
ascites and, iii, 658 
angioma of, iii, 546 
anomalies of, iii; 455, 456, 457 
atrophy of, iii, 465 
in blackwater fever, ii, 113 
in bronchiectasis, ii, 917 
carcinoma of, iii, 541 
ascites and, iii, 542, 658 
cardiac, iii, 481 
in catarrhal cholangitis, iii, 554 
in chronic interstitial nephritis, iii, 899 
perihepatitis, iii, 490, 492 
peritonitis, iii, 718 
cirrhosis of, iii, 505 
arthracotic, iii, 531 
ascites and, iii, 658 
biliary, iii, 535 
alcohol and, iii, 536 
Budd's non-alcoholic, iii, 509 
in children, iii, 531 
experimental, iii, 510 
hemochromatosis and, iii, 529 
intestinal hemorrhage and, iii, 307 
in lenticular degeneration, v, 278 
malarial, iii, 530 
mesenteric thrombosis and, iii, 
435 
osteo-arthropathy and, v, 982 
portal, iii, 508 
tuberculosis of peritoneum and, 
iii, 526, 721 
in congenital syphilis, ii, 190 
congestion of, iii, 478, 481 
constricted, iii, 456 GENERAL INDEX 
1061 
Liver, cysts of, iii, 547 
in defects of interauricular septum, 
iv, 362 
deformities of, iii, 456 
diabetes mellitus and, ii, 681, 685, 696 
disorders of, retrogressive, iii, 465 
emphysematous, iii, 466 
enlargement of, in lobar pneumonia, 
i, 260 
fatty, iii, 466 
function of, iii, 31, 444 
disorders of, iii, 442 
gaseous, iii, 466 
in Gaucher's splenomegaly, iv, 963 
gumma of, ii, 176 
in hepatic thrombosis, iv, 546 
in Hodgkin's disease, iv, 763, 767 
in jaundice, iii, 454 
in kala-azar, iv, 970, 972 
leukemic infiltration of, iii, 471 
in lymphoid leukemia, iv, 672 
in malarial fevers, ii, 70, 73 
in mitral insufficiency, iv, 236, 237 
in myeloid leukemia, iv, 663, 666 
necrosis of, iii, 471 
parenchymatous degeneration of, iii, 
466 
pericarditic pseudocirrhosis of, iii, 488, 
716; iv, 60 
in pernicious anemia, iv, 627, 634 
pigmentation of, iii, 470 
in plague, i, 629 
in polycythemia, iv, 685 
sarcoma of, iii, 546 
in splenic anemia, iv, 955 
sugar-iced, iii, 488, 718 
in suppurative cholangitis, iii, 557 
syphilis of, ii, 176; iii, 541 
chronic peritonitis and, iii, 717 
in thrombosis of portal vein, iii, 462 
in tricuspid stenosis, iv, 253 
tropical, iii, 494 
tuberculosis of, i, 363; iii, 541 
tumors of, iii; 541, 546 
in typhoid fever, i, 85, 118 
Liver-fluke disease, ii, 229 
Lobar pneumonia, i, 202 
Lobstein's disease, v, 980 
Lobulated spleen, iv, 938 
Lockjaw, i, 575 
Locomotor ataxia, v, 833 
Loculated tuberculosis of peritoneum, iii, 
723 
Logorrhoea in aphasia, v, 297 
Lordosis in acromegaly, iv, 810 
Lousiness, ii, 326 
Ludwig's angina, iii, 75 
cervical lymphadenitis and, iv, 733 
in scarlet fever, i, 878 
Lues venera, ii, 144 
Luetin, ii, 193 
Lumbago, v, 644, 945 
Lumbar plexus, neuralgia of, v, 739 
paralysis of, v, 560 
puncture, i, 608, 612; v, 171, 174 
Lumbo-abdominal neuralgia, v, 739 
Lump jaw, i, 1033 
Lung, abscess of, ii, 993 
in bronchopneumonia, ii, 964 
in influenza, i, 538, 540 
in lobar pneumonia, i, 250 
pneumothorax and, ii, 1074 
in tuberculosis, i, 430 
adenoma of, ii, 997 
in alcoholism, ii, 403 
in aneurism of aorta, iv, 503, 507 
arteries of, obliteration of, cardiac 
insufficiency and, iv, 195 
brown induration of, ii, 945 
carcinoma of, ii, 998 
of liver and, iii, 543 
circulatory disturbances of, ii, 945 
cirrhosis of, ii, 969 
of liver and, iii, 518 
congestion of, ii, 945 
in defects of interauricular septum, iv, 
362 
diphtheria of, i, 700 
embolism of, ii, 954; iv, 560, 563 
emphysema of, ii, 979 
enchondroma of, ii, 997 
fibroma of, ii, 998 
fibrosis of, in bronchiectasis, ii, 917 
gangrene of, ii, 988 
in bronchiectasis, ii, 917 
in bronchopneumonia, ii, 964 
in fetid bronchitis, ii, 912 
foreign bodies and, ii, 989 
in influenza, 541 
in lobar pneumonia, i, 251 
pneumothorax and, ii, 1074 
in scurvy, ii, 802 
in tuberculosis, i, 430 
gumma of, ii, 172 
in Hodgkin's disease, iv, 764 
infarction of, ii, 954; iv, 563 
innervation of, ii, 814 
lipoma of, ii, 998 
mechanism of, ii, 826 
new growths of, ii, 997 
oedema of, ii, 948 
in serofibrinous pleuritis, ii, 1026 
osteoma of, ii, 997 
perforation of,in pleuritis, ii, 1026,1044 
sarcoma of, ii, 998 
in smallpox, i, 790 
syphilis of, ii, 171 
tricuspid insufficiency and, iv, 246 
tuberculosis of, i, 348 
bronchial glands and, iv, 743 
pericarditis and, iv, 42 
tumors of, ii, 997 
in typhoid fever, i, 80 
ulceration of, in fetid bronchitis, ii, 
912 
Lung-fluke disease, ii, 226 
Lupus, i, 374 
erythematosus of mouth, iii, 53 
Lymph cysts, iv, 586 
of spleen, iv, 951 
fistula, iv, 595, 598 
glands in amoebic dysentery, ii, 32 
in diphtheria, i, 695 
inflammation of, iv, 730 1062 
GENERAL INDEX 
Lymph glands in lymphoid leukemia, iv, 
671 
in myeloid leukemia, iv, 663, 666 
in plague, i, 624 
relation of, to blood, iv, 613 
in rubella, i, 915 
in scarlet fever, i, 866, 877 
in sleeping sickness, ii, 129 
tuberculosis of, i, 257 
in typhoid fever, i, 146 
nodes, amyloid degeneration of, iv, 
746 
calcification of, iv, 746 
carcinoma of, iv, 747 
cysts of, iv, 753 
degeneration of, iv, 746 
endothelioma of, iv, 747 
in enlargement of thymus, iv, 916 
in exophthalmic goitre, iv, 867 
in Gaucher's splenomegaly, iv, 963 
hematogenous affections of, iv, 
730 
in Hodgkin's disease, iv, 756 
infiltration of, iv, 746 
inflammation of, iv, 730 
leprosy of, iv, 746 
in leukemia, iv, 663, 666, 671, 
747, 751 
neoplasms of, iv, 747, 752 
parasites of, iv, 753 
in pernicious anemia, iv, 627 
pigmentation of, iv, 746 
sarcoma of, iv, 747 
scrofula of, iv, 745 
in splenic anemia, iv, 956 
syphilis of, iv, 745 
tuberculosis of, iv, 741 
tumors of, iv, 747, 752 
scrotum in filariasis, ii, 309 
tissue, tuberculosis of, i, 357 
varices in filariasis, ii, 309 
vessels, carcinoma of, iv, 601 
chondrosarcoma of, iv, 601 
endothelioma of, iv, 600, 601 
lymphangioma of, iv, 600 
lymphosarcoma of, iv, 601 
neoplasms of, iv, 600, 601 
sarcoma of, iv, 601 
syphilis of, iv, 596 
tuberculosis of, iv, 595 
Lymphadenitis, iv, 730 
in amoebic dysentery, iv, 738 
in anthrax, iv, 737 
of axillary glands, iv, 735 
of bronchial glands, iv, 735 
in bubonic plague, iv, 740 
in cerebrospinal meningitis, iv, 738 
cervical, iv, 733 
in chancroid, iv, 739 
in chickenpox, iv, 737 
chronic, iv, 732 
in diphtheria, iv, 736 
in erysipelas, iv, 737 
of femoral glands, iv, 736 
in foot and mouth disease, iv, 738 
in glanders, iv, 737 
in glandular fever, iv, 738 
Lymphadenitis in infectious jaundice, iv, 
738 
in influenza, iv, 737 
of inguinal glands, iv, 736 
in malarial fever, iv, 738 
in Malta fever, iv, 738 
in measles, iv, 736 
of mesenteric glands, iv, 736 
in miliary fever, iv, 738 
in mumps, iv, 737 
in pneumococcus septicemia, iv, 738 
purulenta, iv, 731 
in pyemia, iv, 737 
in relapsing fever, iv, 738 
in rheumatic fever, i, 999; iv, 737 
in Rocky Mountain spotted fever, iv, 
738 
in rubella, iv, 736 
in scarlet fever, iv, 736 
in septicemia, iv, 737 
simplex, iv, 730 
in smallpox, iv, 737 
in syphilis, iv, 740 
in trypanosomiasis, iv, 738 
tuberculous, iv, 741 
in typhoid fever, iv, 738 
in typhus fever, iv, 738 
in vaccinia, iv, 737 
Lymphadenoma of thymus, iv, 932 
Lymphangiectasia, iv, 596 
Lymphangio-endothelioma, iv, 600 
Lymphangioma, iv, 596 
of intestines, iii, 434 
of lips, iii, 45 
Lymphangitis, iv, 592 
filarial, ii, 308 
productiva, iv, 595 
Lymphangivitis of thoracic duct, iv, 584 
Lymphatic channels, tuberculosis of, i, 359 
circulation, iv, 752 
biliary infection and, iii, 551 
system in exophthalmic goitre, iv, 856 
Lymphocytes in leukemia, iv, 667, 674 
Lymphocytoma, iv, 748 
Lymphocytosis, iv, 615 
in exophthalmic goitre, iv, 867 
in spinal fluid, v, 177 
Lymphogenous lymphadenitis, iv, 729 
Lymphoid leukemia, iv, 670 
tissue, appendicitis and, iii, 361 
tuberculosis of lymph nodes, iv, 741 
Lymphoma of pericardium, iv, 76 
of thymus, iv, 932 
Lymphomata, iv, 748 
Lymphorrhagia, iv, 595 
in elephantiasis, iv, 598 
in myasthenia gravis, v, 799 
Lymphosarcoma of intestines, iii, 431 
of lymphatic vessels, iv, 601 
of mediastinum, ii, 1094 
of thymus, iv, 932 
Lysins, i, 54 
M 
McBurney's point in appendicitis, iii, 367 
Macewen's sign in cerebrospinal menin- 
gitis, i, 608 GENERAL INDEX 
1063 
Macrocephaly in achondroplasia, v, 963 
Macrogamete, ii, 61 
Macrogametocytes, ii, 61 
Macroglossia, iii, 60 
Madura foot, i, 1049 
Magnesium phosphate calculus, iii, 1015 
Mal des montagnes, ii, 344 
Malarial fevers, ii, 54 
acute endocarditis and, iv, 157 
hepatitis and, iii, 494 
anemia and, iv, 658 
angioneurotic oedema and, iv, 999 
cirrhosis of liver and, iii, 509, 530 
estivo-autumnal, quotidian, ii, 80 
inflammation of spleen and, iv, 
941 
irregular, ii, 84 
latent, ii, 85 
masked, ii, 85 
migraine and, v, 720 
nephritis and, iii, 850 
pernicious, ii, 80 
anemia and, iv, 622 
pigmentation of liver and, iii, 470 
quartan, ii, 77 
remittent, ii, 84 
tertian, ii, 74 
tuberculosis and, i, 322 
typhoid fever and, i, 156; ii, 87 
Malformations of kidney, iii, 785 
of spleen, iv, 937 
Malignant oedema, i, 645 
pustule, i, 639, 644 
Mallein, i, 650 
Malnutrition, ii, 666 
kidney infections and, iii, 952 
multiple neuritis and, v, 576 
Malpighian bodies in nephritis, iii, 858, 896 
Malpositions of spleen, iv, 938 
Malta fever, i, 644 
Mammary glands in exophthalmic goitre, 
iv, 868 
in tuberculosis, i, 435 
neuralgia, v, 739 
Manchurian "typhus," i, 199 
Manganese poisoning, ii, 386 
Mania, acute, traumatism and, v, 647 
in lead poisoning, ii, 368 
in uremia, iii, 837 
Marie's disease, iv, 616 
hereditary cerebellar ataxia, v, 130,136 
theories on aphasia, v, 300 
Mast-cells, iv, 616 
in myeloid leukemia, iv, 668 
Mastitis in rheumatic fever, i, 999 
in typhoid fever, i, 145 
Mastodynia, v, 739 
Mastoiditis in scarlet fever, i, 875 
Measles, i, 897 
apoplexy and, v, 417 
arteriosclerosis and, iv, 454 
chickenpox and, i, 907 
malignant, i, 903 
nephritis and, iii, 849 
pregnancy and, i, 907 
smallpox and, i, 907 
tuberculosis and, i, 321, 907 
Measles, whooping cough and, i, 659 
Meat poisoning, ii, 458 
Meckel's diverticulum, iii, 421 
Mediastinal emphysema, ii, 1110 
hemorrhage, ii, 1110 
hernia, ii, 1109 
Mediastinitis, ii, 1104 
in lobar pneumonia, i, 253 
pericarditis and, iv, 59, 62 
Mediastinum, carcinoma of, ii, 1100 
cysts of, ii, 1101, 1109 
endothelioma of, ii, 1100 
lymph nodes of, hyperplasia of, ii, 1110 
pigmentation of, ii, 1111 
sclerosis of, ii, 1111 
tuberculosis of, ii, 1111 
sarcoma of, ii, 1094 
teratoma of, ii, 1101 
tumors of, ii, 1093 
Mediterranean fever, i, 664 
Medulla oblongata, foraminal hernia of, v, 
324 
tumors of, v, 337 
Megaloblasts in pernicious anemia, iv, 638 
Megalocephalia, v, 988 
Megalopsia, v, 480 
Megastoma entericum in carcinoma of 
stomach, iii, 213 
Megrims, v, 709 
Melancholia in pernicious anemia, iv, 631 
Melanemia in malarial fevers, ii, 68 
Melanoglossia, iii, 61 
Melanuria, iii, 811 
Membranous cholecystitis, iii, 563 
colitis, iii, 437 
Memory, anomalies of, v, 53, 55 
Mendel's sign, v, 78 
Meniere's disease, v, 512 
syndrome in brain tumor, v, 337 
Meninges in cerebrospinal syphilis, v, 824 
diseases of, v, 162 
Meningism in lobar pneumonia, i, 260 
in typhoid fever, i, 137 
Meningitis, acute cerebrospinal, v, 167 
epidemic cerebrospinal, i, 594 
epilepsy and, v, 599 
in influenza, i, 537, 543 
in lobar pneumonia, i, 260 
localized serous spinal, v, 174 
posterior basic, i, 615 
hydrocephalus and, v, 357 
in scarlet fever, i, 878 
serous, ii, 415 
hydrocephalus and, v, 358 
tuberculous, i, 420, 434 
cardiac arhythmia and, iv, 287 
hydrocephalus and, v, 357 
in typhoid fever, i, 137 
Meningo-encephalitis, tuberculous, of 
brain, v, 319 
Meningomyelitis, gonococcal, i, 761 
in syphilis, ii, 162 
Mental disturbances, traumatic, v, 646 
fog, v, 44 
phenomena in tetany, v, 758 
Meralgia paresthetica, v, 557 
Mercurial embolism, iv, 561 1064 
GENERAL INDEX 
Mercurial stomatitis, iii, 48 
Mercury poisoning, ii, 378 
purpura and, iv, 689 
in syphilis, ii, 207 
Mermithidae, ii, 296 
Merozoites, ii, 58, 59, 60 
Merycism, iii, 138 
Mesaortitis, acute, iv, 451 
Mesenteric cysts of peritoneum, iii, 736 
embolism, iii, 434 
glands, acute peritonitis and, iii, 673 
lymphadenitis of, iv, 736 
tuberculosis of, iv, 743 
in typhoid fever, i, 85 
thrombosis, iii, 434; iv, 542 
cirrhosis of liver and, iii, 435 
Metabolism, ii, 549 
in acromegaly, iv, 812 
in arthritis deformans, v, 903, 907 
auto-intoxication and, ii, 505 
of calcium, ii, 561, 636 
carbohydrate, ii, 596, 687 
of carbon, ii, 560 
cholelithiasis and, iii, 571 
in diabetes mellitus, ii, 543, 686, 691 
during fasting, ii, 606 
energy, ii, 564 
in exophthalmic goitre, iv, 869 
fat, ii, 601 
in gout, ii, 732 
of hexosephosphate, ii, 562 
in hypopituitarism, iv, 816 
in hysteria, v, 688 
in infections, i, 44 
of lecithins, ii, 562 
in lobar pneumonia, i, 217 
of magnesium, ii, 561 
multiple neuritis and, v, 575 
in myxcedema, ii, 517 
nephritis and, iii, 854, 892 
of nitrogen, ii, 556 
of nucleic acid, ii, 562 
in obesity, ii, 516, 666 
osteomalacia and, v, 949, 952 
of phosphoproteins, ii, 562 
of phosphorus, ii, 561, 637 
protein, ii, 579, 582 
purpura and, iv, 700 
spleen and, iv, 934 
in sporadic cretinism, iv, 885 
of sulphur, ii, 561 
in tuberculosis, i, 381 
of uric acid, ii, 732 
Metacarpophalangeal joints in arthritis 
deformans, v, 922 
Metallic tinkle in pneumothorax, ii, 1080 
Metapneumonic pleuritis, ii, 1012 
Metastasis in mumps, i, 921 
Metastatic abscess of brain, v, 384 
Metastrongylus apis, ii, 302 
Metasyphilis, ii, 185 
Meteorism, iii, 298 
in angioneurotic oedema, iv, 1008 
in hepatoptosis, iii, 757 
in lobar pneumonia, i, 254 
in typhoid fever, i, 106 
Methyl alcohol poisoning, ii, 398 
Meyer's sensitized bacillus emulsions, i, 
305 
Microcephalia, v, 990 
Micrococcus of endocarditis, i, 559 
gingiva; in mouth, iii, 40 
melitensis, i, 664 
paramelitensis, i, 665 
rheumaticus, i, 976 
Microfilaria, ii, 305, 315 
Microgametes, ii, 61 
Microgametocyte, ii, 61 
Microglossia, iii, 60 
Micromelic rickets, v, 955 
Mid-brain, glioma of, v, 284 
lesion of, v, 282 
Migraine, epilepsy and, v, 615 
ophthalmic, v, 709 
gout and, ii, 748; v, 720 
ophthalmoplegic, v, 724 
periodic paralysis and, v, 811 
Mikulicz's disease, iii, 73 
Miliary aneurisms, hematemesis from, iii, 
283 
eruption in typhoid fever, i, 101 
fever, i, 1017 
sclerosis, v, 161 
tuberculosis, acute, i, 417 
Milk poisoning, ii, 466 
sickness, i, 1028 
Milky ascites, iii, 662 
Millard-Gubler paralysis, v, 290, 369 
Milroy's disease, iv, 1011 
Mind blindness, v, 53, 271, 299, 483 
deafness, v, 54 
Miner's anemia, ii, 271 
cachexia, ii, 271 
Miosis, v, 77 
in exophthalmic goitre, iv, 864 
Mitral atresia, congenital, iv, 406 
insufficiency, iv, 232 
in tuberculosis, i, 433 
stenosis, iv, 239 
in tuberculosis, i, 433 
valve, division of, iv, 359 
Moebius sign in exophthalmic goitre, iv, 
863 
Moeller's glossitis, iii, 63 
Mollifies ossium, v, 947 
Molhiscum fibrosum, v, 571 
Monarticular arthritis deformans, v, 919, 
934 
Monocular diplopia, v, 480 
Monoideic somnambulism, v, 44 
Monoplegia in arteriosclerosis, iv, 461 
in hysteria, v, 681 
in tuberculosis, i, 421 
Monostomulum lentis, ii, 245 
Moods, v, 59, 62 
Morbus ca;ruleus, iv, 333 
gallicus, ii, 144 
herculeus, iv, 596 
Morphinism, ii, 438 
Morvan's type of syringomyelia, v, 220 
Mosquito brigades, ii, 97 
malarial, ii, 63 
Motility, disturbances of, v, 68 
Motor actions, v, 64, 65, 66 GENERAL INDEX 
1065 
Motor agitation, v, 63 
aphasia, v, 75, 292, 295 
area of cortex of brain, v, 265 
disturbances in migraine, v, 715 
in neuralgia, v, 728 
functions of intestines, iii, 286 
insufficiency of stomach, iii, 247 
nerves in tetany, v, 757 
paralysis of intestines, iii, 418 
phenomena in infantile convulsions, 
preservation, v, 67 
v, 794 
stupor, v, 63, 65 
Mountain sickness, ii, 344 
Mouth, acne of, iii, 54 
actinomycosis of, iii, 59 
angioneurotic oedema of, iii, 53 
carcinoma of, iii, 52 
cysts of, iii, 52 
dermatoses of, iii, 53 
in diabetes, iii, 39 
eczema of, iii, 54 
erysipelas of, iii, 54 
in foot-and-mouth disease, i, 1027 
in hemiplegia, v, 460 
inflammation of, iii, 45 
leprosy of, iii, 59 
in leukemia, iii, 55 
lichen planus of, iii, 54 
in locomotor ataxia, iii, 55 
lupus erythematosus of, iii, 53 
pemphigus of, iii, 54 
in pernicious anemia, iii, 55; iv, 630 
in pregnancy, iii, 56 
psoriasis of, iii, 53 
scleroderma of, iii, 54 
scleroma of, iii, 60 
in scurvy, ii, 800; iii, 55 
sepsis of, iii, 41 
in sprue, iii, 393 
syphilis of, iii, 58 
tinea circinata of, iii. 54 
tuberculosis of, i, 360; iii, 57 
tumors of, iii, 52 
urticaria of, iii, 53 
in varicella, iii, 56 
in variola, iii, 56 
Movement, sensation of, v, 17 
Mucocele of appendix, iii, 386 
Mucous colic, iii, 437 
colitis, iii, 437 
diarrhcea, iii, 437 
gastritis, iii, 238 
lesions in syphilis, ii, 163 
membrane in acute laryngitis, ii, 869 
in angioneurotic oedema, iv, 1007 
in asthma, ii, 932 
endocarditis and, iv, 152 
in hay fever, ii, 855, 857, 864 
in hysteria, v, 682 
in jaundice, iii, 451 
in pellagra, ii, 479, 480 
in syphilis, ii, 151, 168 
of larynx, ii, 872 
in tuberculosis of larynx, ii, 876 
patches in congenital syphilis, ii, 189 
Mucus in feces, iii, 293 
Mulberry calculus, iii, 1009 
Multiple neuritis, v, 593 
sclerosis, v, 140 
Mumps, i, 918 
Mural aneurism, iv, 483 
Murmur, cardiopulmonary, iv, 298 
Muscles, abdominal, constipation and, iii, 
312 
in acromegaly, iv, 811 
in acute poliomyelitis, i, 736 
in amyotonia congenita, v, 894 
in arthritis deformans, v, 907 
atony of, v, 68 
atrophy of, v, 68 
in disease of peripheral nerves, v, 
534 
in paralysis agitans, v, 771 
contractures of, v, 68 
cramps of, in arteriosclerosis, iv, 464 
in tetany, v, 754 
electrical reactions of, v, 72 
in exophthalmic goitre, iv, 857, 865 
hypertony of, v, 68 
hypertrophy of, v, 68 
hypotony of, v, 68 
in tabes dorsalis, v, 839 
inflammation of, v, 871 
irritation of, v, 70 
in myositis ossificans, v, 883 
in myotonia congenita, v, 887 
of neck, spasm of, v, 525 
ocular, paralysis of, v, 485 
paralysis of, v, 68 
in paramyoclonus multiplex, v, 807 
in periodic paralysis, v, 813 
in rickets, ii, 780 
in scleroderma, iv, 1020 
sense, v, 17 
spasms of, v, 71 
of tongue, spasm of, v, 532 
tonus of, v, 68 
tuberculosis of, i, 373 
Muscular atrophy, v, 80 
in arthritis deformans, v, 914 
progressive spinal, v, 82 
dystrophy, progressive, v, 117 
rigidity in hemiplegia, v, 463 
in paralysis agitans, v, 769 
tension in hemiplegia, v, 463 
wasting in spondylitis, v, 927 
weakness in chlorosis, iv, 649 
in general paresis, v, 851 
in pernicious anemia, iv, 629 
in rickets, ii, 782 
Musculocutaneous nerve, paralysis of, v, 
546 
Musculospiral nerve, paralysis of, v, 547 
Mutism in hysteria, v, 652 
Myasthenia, dilatation of stomach and, iii 
255 
gravis, v, 798 
in hysteria, v, 678 
parathyroid glands and, iv, 903 
Myasthenic reaction, v, 801, 805 
Mycetoma, i, 1049 
Mycobacterium tuberculosis, i, 294 
Mycoses, i, 1032 1066 
GENERAL INDEX 
Mycosis intestinalis, i, 645 
inucorina, i, 1060 
of tonsils, ii, 852 
Mycotic aneurism, iv, 476 
gastritis, iii, 235 
stomatitis, iii, 235 
Mydriasis v, 77 
in exophthalmic goitre, iv, 864 
in lobar pneumonia, i, 263 
in tabes dorsalis, v, 837 
Myelitis, v, 227 
acute ascending, v, 236 
bulbar, v, 368 
chronic, v, 245 
compression, v, 229 
degenerative, v, 236 
disseminated, v, 235 
infective, v, 229 
syphilitic, v, 237, 828 
toxic, v, 236 
Myelocytes in lobar pneumonia, i, 234 
in myeloid leukemia, iv, 667 
in pernicious anemia, iv, 638 
Myelocytosis, iv, 616 
Myeloid leukemia, iv, 661 
Myeloma of brain, v, 315 
of spinal cord, v, 198 
Myelophthisic anemia, iv, 645 
Myeloplastic bone aplasia,»v, 978 
Myiasis, ii, 328 
Myocardial bradycardia, iv, 282 
incompetence of obese, iv, 128 
Myocarditis, iv, 108 
acute interstitial, iv, 113 
necrotic, iv, 114 
parenchymatous, iv, 113 
angina pectoris and, iv, 291 
in arteriosclerosis, iv, 462 
cardiac arhythmia and, iv, 287 
chronic, iv, 109 
in endocarditis, iv, 161 
gonorrhoeal, i, 752 
in gout, ii, 749 
hypertrophy of heart and, iv, 182 
in measles, i, 907 
in pericarditis, iv, 45 
in scarlet fever, i, 877 
in smallpox, i, 811 
syphilitic, ii, 184 
tricufepid insufficiency and, iv, 247 
in typhoid fever, i, 126 
in typhus fever, i, 930 
Myocardium, actinomycosis of, iv, 145 
blastomycosis of, iv, 144 
degeneration of, iv, 108-116 
in diphtheria, i, 694 
echinococcus of, iv, 147 
fragmentation of, iv, 116 
insufficiency of, iv, 131, 187 
ischemia of, iv, 290 
new growths of, iv, 142, 145 
parasites of, iv, 142 
segmentation of, iv, 116 
syphilis of, iv, 142 
tuberculosis of, i, 359; iv, 144 
Myoclonus in hysteria, v, 677 
parathyroid glands and, iv, 903 
I Myogenic theory of mechanism of heart, 
iv, 27 
Myoidema in tuberculosis, i, 411 
Myoma of appendix, iii, 386 
of peritoneum, iii, 730 
Myopathy, primary, v, 117 
Myopia, v, 36 
I Myositis, v, 871 
gonococcal, i, 760 
non-suppurative, i, 760; v, 875 
ossificans, progressive, v, 881 
primary fibrosa, v, 880 
suppurative, i, 761; v, 872 
with special terminal lesions, v, 880 
Myospasm caused by heat exposure, ii, 351 
Myotonia congenita, v, 886 
parathyroid glands and, iv, 903 
Myotonic reaction, v, 891 
Myriapoda, ii, 324 
i Mythomania, v, 659 
Mytilotoxin, ii, 457 
Myxoedema, iv, 888 
congenital, iv, 883 
in exophthalmic goitre, iv, 869, 890 
goitre and, iv, 890 
infantile, iv, 883 
spontaneous, iv, 888 
thyroiditis and, iv, 829 
Myxoma of appendix, iii, 386 
of intestines, iii, 433 
of peritoneum, iii, 733 
Myxosarcoma of brain, v, 315 
N 
Nagana, ii, 117 
Nails in diseases of peripheral nerves, v, 
541 
in endemic cretinism, iv, 882 
in osteo-arthropathy, v, 984 
in syphilis, ii, 167 
in typhoid fever, i, 102 
Nasal diphtheria, i, 698 
Nasopharyngitis, ii, 837, 838 
Nasopharynx, adenoid growths in, ii, 840 
tuberculosis of, i, 389 
Nauheim baths in myocarditis, iv, 138 
Necator americanus, ii, 272 
Neck, actinomycosis of, i, 1038 
in cerebrospinal meningitis, i, 607 
muscles of, spasm of, v, 525 
Necrophagus sanguinarius, ii, 323 
Necrosis of appendix, iii, 363 
fat, iii, 606 
hemorrhagic, of pancreas, iii, 610 
of jaw in phosphorus poisoning, ii, 383 
of liver, iii, 471 
in Raynaud's disease, iv, 986 
Necrotic endocarditis, iv, 151 
Negri bodies, i, 959, 961 
Nemathelmintes, ii, 270 
Nematodes of urogenital system, ii, 315 
Neoplasms of lymph nodes, iv, 747, 752 
of lymphatic vessels, iv, 600, 601 
of pericardium, iv, 74 
Neosalvarsan in syphilis, ii, 212 GENERAL INDEX 
1067 
Nephritis, iii, 778, 845 
acute, iii, 857 
alcoholism and, iii, 866 
in Asiatic cholera, iii, 867 
in chronic nephritis, iii, 912 
in Henoch's purpura, iv, 710 
primary, iii, 867 
in purpura hemorrhagica, iv, 712 
in typhoid fever, iii, 867 
yellow atrophy of liver and, iii, 
850 
arteriosclerosis and, iv, 455 
burns and, iii, 853 
in chickenpox, i, 853 
cholera and, iii, 849 
chorea and, iii, 850 
chronic, diffuse, iii, 847 
interstitial, iii, 890 
myocarditis and, iv, 123, 125 
in myxoedema, iv, 894 
parenchymatous, iii, 880 
pericarditis and, iv, 42 
pernicious anemia and, iv, 627 
diarrhoea and, iii, 323 
in diphtheria, i, 695; iii, 849 
in glandular fever, i, 1013 
lobar pneumonia and, i, 208, 258 
in malarial fevers, ii, 86; iii, 850 
in mumps, i, 921 
in polymyositis hemorrhagica, v, 880 
in rheumatic fever, i, 998; iii, 850 
in scarlet fever, i, 874; iii, 849 
in serofibrinous pleuritis, ii, 1026 
in smallpox, i, 812; iii, 849 
in syphilis, ii, 180 
tuberculosis and, i, 434 
in typhoid fever, i, 143; iii, 849 
Nephrolysins, iii, 834 
Nephroptosis, iii, 750, 755 
kidney infections and, iii, 952 
tuberculosis of kidney and, iii, 978 
Nerve deafness, v, 509 
trunks, neuralgia and, v, 726 
Nerves in acute poliomyelitis, i, 736 
in beriberi, ii, 493 
in cerebrospinal meningitis, i, 598 
in goitre, iv, 842 
tumors of, v, 568 
Nervous anorexia, iii, 157 
dyspepsia, iii, 164 
dyspncea, iv, 37 
eructation, iii, 141 
gastralgia, iii, 135 
hypertrophy of heart, iv, 167 
influenza, i, 542 
nausea, iii, 146 
purpura, iv, 690 
shock, pernicious anemia and, iv, 624 
system in Addison's disease, iv, 793 
in alcoholism, ii, 405 
angioneurotic oedema and, iv, 999 
in arteriosclerosis, iv, 461 
arthritis deformans and, v, 903, 
907 
in cardiac insufficiency, iv, 208 
in chlorosis, iv, 650 
in dermatomyositis, v, 877 
Nervous system, diabetes mellitus and, ii, 
678, 697 
in diphtheria, i, 695 
in exophthalmic goitre, iv, 857, 
864 
in jaundice, iii, 454 
in lead poisoning, ii, 365 
in lobar pneumonia, i, 260 
in measles, i, 907 
in motor insufficiency of stomach, 
iii, 248 
in myxoedema, iv, 892 
in opium smoking, ii, 436 
in Paget's disease, v, 973 
parasyphilis of, v, 822 
in pellagra, ii, 477, 481 
in pernicious anemia, iv, 628 
in plague, i, 630 
in purpura, iv, 697 
in rheumatic fever, i, 992 
syphilis of, v, 822 
in tuberculosis, i, 390, 421 
in typhoid fever, i, 80, 132 
in uremia, iii, 835 
vomiting, iii, 142 
Neuralgia, v, 725 
brachial, v, 737 
cervico-occipital, v, 736 
in chronic nephritis, iii, 907 
of coccygeal plexus, v, 741 
diaphragmatic, v, 737 
facial, v, 733 
femoral, v, 740 
ganglia and, v, 726 
gonococcal, i, 761 
in gout, ii, 748 
in hemophilia, iv, 725 
in hysteria, v, 683 
intercostal, v, 739 
of intestines, iii, 305 
of lumbar plexus, v, 739 
lumbo-abdominal, v, 739 
mammary, v, 739 
nerve trunks and, v, 740 
obturator, v, 740 
of pudendal plexus, v, 740 
"red painful," iv, 1028 
reflex, v, 726, 731 
testicular, v, 740 
trigeminal, v, 733 
Valleix's points in, v, 727 
Neurasthenia, v, 624 
achylia gastrica and, iii, 153 
in arteriosclerosis of brain, v, 395 
cardiospasm and, iii, 147 
in constipation, iii, 315 
electrical shock and, ii, 341 
gastric, iii, 164 
in mucous colitis, iii, 439 
nervous anorexia and, iii, 157 
dyspepsia and, iii, 165 
in splanchnoptosis, iii, 753, 754 
syphilis and, v, 629 
in tabes dorsalis, v, 843 
traumatic, v, 643 
Neuritic pains in arteriosclerosis, iv, 465 
Neuritis in arsenic poisoning, ii, 375 1068 
GENERAL INDEX 
Neuritis, brachial, v, 553 
in diabetes mellitus, ii, 706, 708 
gonococcal, i, 761 
in hemophilia, iv, 725 
in influenza, i, 543 
interstitial hypertrophic progressive, 
of childhood, v, 112 
in lobar pneumonia, i, 262 
in mercury poisoning, ii, 380 
multiple, v, 573 
alcoholic, v, 579 
ascending, v, 576 
optic, v, 36 
in acute meningitis, v, 170 
retrobulbar, v, 37, 479 
in rheumatic fever, i, 995 
in tuberculosis, i, 392 
in typhoid fever, i, 139 
Neuroblastoma of brain, v, 317 
Neurocytes hydrophobias, i, 959 
Neurocytoma of brain, v, 317 
Neurofibromatosis, general, v, 570 
Neuroglia in infective myelitis, v, 232 
in syphilitic myelitis, v, 239 
Neuroglioma of brain, v, 311 
Neuroma of brain, v, 317 
gangliated, of peritoneum, iii, 731 
Neuromata, v, 568 
Neuromyositis, v, 880 
Neuropathic hematemesis, iii, 282 
Neuroses, cardiovascular disease and, iv, 
296 
gastric, iii, 106 
gonococcal, i, 762 
of larynx, sensory, v, 522 
occupation, v, 741 
of oesophagus, iii, 86 
poly symptomatic, iii, 164 
professional, v, 741 
traumatic, v, 642 
in tuberculosis, i, 391 
Neuro-tabes peripherica, v, 579, 584 
Neurotic progressive facial atrophy, v, 991 
Neutrophiles in myeloid leukemia, iv, 667 
Neutrophilic myelocytes, iv, 609 
Night-blindness, v, 35, 480 
in scurvy, ii, 802 
Night-sweats in tuberculosis, i, 381 
Nitric oxide poisoning, ii, 395 
Nitrogen balance, ii, 556 
equilibrium, ii, 558 
in feces, ii, 640 
metabolism of, ii, 556 
non-protein, in metabolism, ii, 662 
in urine in diabetes mellitus, ii, 703 
Nocardiosis of pleura, ii, 1051 
Nodosity of joints, v, 897 
Nodules in septic endocarditis, iv, 163 
Noma, iii, 49 
Normoblasts in pernicious anemia, iv, 638 
Nose in cerebrospinal meningitis, i, 604 
in glanders, i, 652 
obstruction of, ii, 815 
tuberculosis of, i, 347 
in typhoid fever, i, 120, 140 
Nyctalopia, v, 35, 480 
Nystagmus, v, 70 
Nystagmus in brain abscess, v, 384 
tumor, v, 336, 339, 340 
in disseminated sclerosis, v, 151 
in Friedreich's ataxia, v, 135 
in lead poisoning, ii, 368 
in M^nidre's disease, v, 513 
in mercury poisoning, ii, 379 
in ocular paralysis, v, 486 
in pseudo-sclerosis, v, 159 
in syringomyelia, v, 222 
in vestibular disease, v, 511 
O 
Obesity, ii, 761 
fat metabolism and, ii, 605 
myocardial incompetence of, iv, 128 
Oblique muscles, paralysis of, v, 485 
Obliterative tuberculosis of peritoneum, iii, 
724 
Obsessions, v, 52 
"Obstetrical hand," v, 71 
Obstipation, iii, 309 
Obstruction of intestines, iii, 394 
Obturation of intestines, iii, 394 
Obturator nerve, paralysis of, 557 
Occlusion of intestines, iii, 394 
"Occult blood" in carcinoma of stomach, 
iii, 207, 211 
in gastric ulcer, iii, 179 
tests for, iii, 180 
Occupation neuroses, v, 741 
Ochronosis, iii, 817 
Ocular muscles in disseminated sclerosis, 
v, 151 
paralysis of, v, 485 
nerves, paralysis of, 483 
palsy, nuclear, v, 365 
Oculomotor palsy, recurrent, v, 488 
in tabes dorsalis, v, 841 
O'Dwyer's intubation instruments, i, 726 
(Edema, angioneurotic, iv, 998 
of mouth, iii, 53 
of pharynx, ii, 847 
of larynx, ii, 878 
of legs, hereditary, iv, 1011 
of lungs, ii, 948 
(Esophageal nerves, paralysis of, v, 523 
(Esophagitis, iii, 79 
(Esophagostomum, ii, 294 
(Esophagus, actinomycosis of, iii, 86 
in aneurism of aorta, iv, 502, 509 
carcinoma of, iii, 98 
congenital malformations of, iii, 78 
dilatation of, iii, 90 
diverticula of, iii, 92 
in goitre, iv, 843 
herpes of, iii, 90 
inflammation of, iii, 79 
neuroses of, iii, 86 
non-malignant growths of, iii, 105 
paralysis of, iii, 90 
perforation of, ii, 1045 
rupture of, iii, 85 
sarcoma of, iii, 105 
stenosis of, iii, 96 GENERAL INDEX 
1069 
(Esophagus, stricture of, iii, 96 
syphilis of, ii, 174; iii, 86 
tuberculosis of, i, 361; iii, 85 
in typhoid fever, i, 104 
ulcers of, iii, 84 
Oidiomycosis, i, 1050 
Oidium albicans, i, 1054; iii, 46,50 
Olfactory nerve, diseases of, v, 471 
Oliguria in tuberculosis of kidney, iii, 982 
Olivopontocerebellar atrophy, v, 287 
Omental cysts of peritoneum, iii, 737 
Omentitis, mesenteric lymphadenitis and, 
iv, 736 
Omentum, abscess of, iii, 696 
inflammation of, iii, 695 
volvulus of, iii, 418 
Oocyst, ii, 63 
Ookinete, ii, 62 
Ophthalmic distomatosis, ii, 245 
Ophthalmoplegia, v, 483 
in acute encephalomyelitis, v, 369 
in cerebrospinal syphilis, v, 825 
chronic, v, 491 
externa, v, 490 
in hysteria, v, 678 
interna, v, 490 
in lesion of quadrigeminal bodies, v, 
283 
in superior poliencephalitis, v, 365 
in tabes dorsalis, v, 841 * 
Ophthalmoplegic migraine, 724 
Ophthalmo-reaction in typhoid fever, i, 162 
Opisthorchiasis, ii, 225, 232, 233 
Opisthorchis, ii, 229, 232 
Opium habit, ii, 433 
poisoning, ii, 433 
smoking, ii, 435 
Oppenheim's disease, v, 893 
sign, v, 78 
Opsonic index in septicemia, i, 569, 573 
in tuberculosis, i, 511 
Opsonins, i, 57 
in tuberculosis, i, 313 
Optic aphasia, v, 54, 299 
asymbolia, v, 54 
atrophy, v, 478 
chiasm, affections of, v, 481 
disks in disseminated sclerosis, v, 151 
in papillitis, v, 478 
nerve, atrophy of, v, 37 
in tabes dorsalis, v, 841 
inflammation of, v, 476 
neuritis, v, 36, 326 
in diabetes mellitus, ii, 708 
in tuberculosis, i, 421 
thalamus, lesion of, v, 279 
tracts, affections of, v, 481 
Oral sepsis, iii, 41 
Orchitis in congenital syphilis, ii, 190 
in filariasis, ii, 310 
in lobar pneumonia, i, 264 
in mumps, i, 921 
in typhoid fever, i, 145 
Oriental sore, iv, 964 
Ornithodoros, ii, 317 
Orthopnoea in cardiac insufficiency, iv, 296 
Osteitis, chronic parenchymatous, v, 978 
Osteitis deformans, v, 969 
tumor-building, v, 978 
fibrosa, 980 
in syphilis, ii, 163 
Osteo-arthritis, v, 897, 920 
hypertrophica, v, 987 
Osteo-arthropathy, hypertrophic, v, 981 
in tuberculosis, i, 411 
Osteochondritis in syphilis, ii, 190 
Osteogenesis imperfecta, v, 907 
Osteoma of lung, ii, 997 
Osteomalacia, v, 947 
congenita, v, 978 
in exophthalmic goitre, iv, 868 
parathyroid glands and, iv, 903 
Osteopathies, hypertrophic, v, 969 
Osteoporosis congenita,, v, 978 
Osteopsathyrosis, v, 978, 980 
Osteosarcoma of brain, v, 314 
Osteosclerosis, congenital, v, 968, 978 
Ostitis deformans, v, 970 
fibrosa hypertrophica, v, 988 
Ostium primum, iv, 326 
secundum, iv, 326 
Otitic abscess of brain, v, 383 
Otitis media in chickenpox, i, 853 
in diphtheria, i, 700 
in glandular fever, i, 1013 
in influenza, i, 537, 545 
in lobar pneumonia, i, 263 
in scarlet fever, i, 864, 875 
in smallpox, i, 812 
in typhoid fever, i, 140 
Ovarian cyst, acute peritonitis and, iii, 673 
Ovaries in osteomalacia, v, 948 
tuberculosis of, i, 368 
in typhoid fever, i, 144 
Ovaritis in mumps, i, 921 
Oxalic acid calculus, iii, 1015 
Oxaluria, ii, 540; iii, 815 
in diabetes mellitus, ii, 703 
in gout, ii, 748 
Oxyuriasis, ii, 291 
Oxyuris vermicular is, ii, 291 
appendicitis and, iii, 360 
P 
Pachyderma, iv, 596 
Pachymeningitis, v, 163 
cervicalis hypertrophica, v, 166 
externa, v, 163 
interna, v, 164 
Paget's disease, v, 970 
Pain sense, v, 17 
Painful foot, i, 760 
heel, i, 760 
Palate in hemiplegia, v, 459 
paralysis of, in hysteria, v, 679 
soft, paralysis of, iii, 62 
tuberculosis of, iii, 57 
Pallanesthesia, v, 30 
Palmar reflex, v, 78 
Palsy, bulbar, acute inflammatory, v, 368 
progressive, v, 113 
congenital cerebral, v, 410 1070 
GENERAL INDEX 
Palsy, Duchenne-Erb, v, 551 
Klumpke's, v, 552 
oculomotor, recurrent, v, 488 
in tabes dorsalis, v, 841 
shaking, v, 764 
Panatrophy, local, in scleroderma, iv, 1022 ! 
Pancreas, abscess of, calculus and, iii, 635 
subphrenic abscess and, iii, 701 
adenoma of, iii, 649 
arteriosclerosis of, iii, 605 
calculus of, iii, 632 
carcinoma and, iii, 636 
carcinoma of, iii, 648 
calculus and, iii, 636 
cyst of, iii, 637 
diabetes mellitus and, iii, 645 
necrosis and, iii, 619 
descent of, iii, 758 
dilatation of stomach and, iii, 251 
diseases of, diabetes mellitus and, iii, | 
604 
glycosuria and, iii, 604, 606 
metabolic function and, iii, 601 
pancreatic secretion and, iii, 599 
displaced, iii, 752 
fat necrosis and, iii, 606 
gangrene of, iii, 615 
hemorrhagic necrosis of, iii, 610 
in hypopituitarism, iv, 817 
sarcoma of, iii, 650 
syphilis of, ii, 174; iii, 632 
tuberculosis of, i, 363; iii, 631 
in typhoid fever, i, 86 
Pancreatic apoplexy, iii, 610 
juice, ferments of, iii, 289 
secretion of, iii, 288 
Pancreatitis, chronic, iii, 629 
experimental, iii, 612 
in mumps, i, 921 
suppurative, iii, 622 
traumatic, iii, 612 
in typhoid fever, i, 120 
Papillary cystadenoma of kidney, iii, 1002 ! 
Papillitis, v, 326 
lingualis, iii, 62 
Papilloma of brain, v, 315 
Papular eruption in syphilis, ii, 164 
Paracentesis abdominis, iii, 667 
in cirrhosis of liver, iii, 524, 534 I 
of pericardium, iv, 56 
thoracic, cardiac insufficiency and, iv, j 
196 
Paracentral convolutions, tumors of, v, 332 
Parachordodes, ii, 296 
Paradoxical embolism, iv, 363, 414, 553 
Paraffin embolism, iv, 562 
Paragonimiasis, ii, 226 
Paragonimus westermanii, ii, 226 
Paragordius, ii, 296 
Paragraphia, v, 66, 75 
Paragusia, iii, 43 
Paralactic acid, ii, 599 
Paralysis in abscess of brain, v, 381 
in acute infective polyneuritis, v, 590 
agitans, v, 764 
ductless glands and, v, 767 
exophthalmic goitre and, iv, 870 | 
Paralysis agitans, parathyroid glands and, 
iv, 903; v, 767 
trauma and, v, 764 
in amaurotic family idiocy, v, 869 
in apoplexy, v, 438, 440* 
in arsenic poisoning, ii, 376 
ascending bulbar, v, 798 
asthenic, v, 798 
atrophic, in syringomyelia, v, 221 
in beriberi, ii, 495 
birth, brachial, v, 552 
of brachial plexus, v, 550 
in brain tumor, v, 337 
of cardiac nerves, v, 522 
in cerebrospinal syphilis, v, 825 
of cervical plexus, v, 543 
of circumflex nerve, v, 546 
compression, in syphilis, ii, 162 
diaphragmatic, v, 544 
in diphtheria, i, 701 
diver's, ii, 345 
Duchenne-Erb, v, 551 
Erb's syphilitic, v, 246 
of external rectus muscle, v, 485 
of facial nerve, v, 495 
in hysteria, v, 679 
flaccid, v, 178 
of gastric nerves, v, 523 
glosso-labio-laryngeal, v, 113 
of gluteal nerves, v, 558 
in hematomyelia, v, 260 
in hysteria, v, 650, 678 
in infantile convulsions, v, 795 
of inferior oblique muscle, v, 485 
rectus muscle, v, 485 
of internal rectus muscle, v, 485 
of intestines in acute peritonitis, ii, 681 
obstruction from, iii, 418 
in intradural hemorrhage, v, 258 
of intra-ocular muscles, v, 486 
Klumpke's, v, 552 
Landry's, v, 247 
laryngeal, ii, 867; v, 518, 521 
in lead poisoning, ii, 365 
in lesion of cerebellum, v, 286 
of crura cerebri, v, 281 
of internal capsule, v, 276 
of mid-brain, v, 283 
of pons, v, 289 
of tegmentum, v, 281 
of long thoracic nerve, v, 544 
of lower arm, v, 552 
of lumbar plexus, v, 560 
of median nerve, v, 548 
in mercury poisoning, ii, 379 
Millard-Gubler type, v, 290 
in multiple neuritis,, v, 580 
of muscles, v, 68 
of musculocutaneous nerve, v, 546 
of musculospiral nerve, v, 547 
of nerves of legs, v, 556 
of ocular nerves, v, 483 
of oesophageal nerves, v, 523 
of oesophagus, iii, 90 
periodic, v, 809 
in pernicious anemia, iv, 631 
of pharyngeal nerves, v, 517 GENERAL INDEX 
1071 
Paralysis of phrenic nerve, v, 543 
in polycythemia, iv, 685 
of popliteal nerves, v, 559 
in progressive spinal muscular atrophy, 
v, 84 
pseudo-bulbar, v, 290, 978 
of pulmonary nerves, v, 523 
of radial nerve, v, 547 
of sacral plexus, v, 560 
of sciatic nerve, v, 558, 560 
in smallpox, i, 813 
of soft palate, iii, 62 
"soul," v, 268 
spastic, v, 179 
spinal, v, 101 
hereditary, v, 105 
of spmal accessory nerve, v, 524 
of superior oblique muscle, v, 486 
rectus muscle, v, 485 
of suprascapular nerve, v, 545 
in syphilitic myelitis, v, 240 
of tongue, iii, 62; v, 530 
in traumatic neuroses, v, 651 
of trigeminal nerve, v, 549 
in tumor of thyroid gland, iv, 833 
unilateral, v, 108 
of upper arm, v, 551 
in uremia, iii, 836 
of vagus nerve, v, 517 
Paralytic conjugate deviation in apoplexy, 
v, 439 
phenomena in chorea, v, 780 
in migraine, v, 716 
rabies, i, 965 
Paralyzing vertigo, v, 515 
Paramimia, v, 66 
Paramnesia, v, 65 
Paramyoclonus multiplex, v, 807 
Paranephric abscess, iii, 970 
Paranephritis, iii, 970 
in tuberculosis of kidney, iii, 980 
Paranoia, trauma and, v, 647 
Paraphasia, v, 66 
Paraplegia in abscess of brain, v, 381 
in aortic embolism, iv, 566 
thrombosis, iv, 566 
in arteriosclerosis, iv, 461 
in cerebrospinal syphilis, v, 825 
in combined sclerosis, v, 127 
in diabetes mellitus, ii, 707 
Erb's syphilitic, v, 246 
in hysteria, v, 679 
spastic, in Hodgkin's disease, iv, 767 
in syphilitic myelitis, v, 241 
in tumors of spinal cord, v, 203 
Parapleural echinococcus, ii, 1066 
Parapraxia, v, 66 
Parasites, animal, ii, 277 
appendicitis and, iii, 360 
embolism and, iv, 558 
inflammation of thoracic duct and, iv, 
584. 
intestinal, in lobar pneumonia, i, 254 
pernicious anemia and, iv, 623 
in kala-azar, iv, 965 
kidney infections and, iii, 952 
of lymph nodes, iv, 753 
Parasites, malarial, ii, 56, 59, 78 
of myocardium, iv, 142 
of thoracic duct, iv, 589 
Parasitic aneurism, iv, 472 
cysts of brain, v, 318 
of peritoneum, iii, 733 
of spleen, iv, 953 
Parasyphilis, ii, 185 
of nervous system, v, 822 
Parathyroid glands, iv, 898 
eclampsia and, iv, 903 
epilepsy and, iv, 903 
in exophthalmic goitre, iv, 857 
kidneys and, iv, 904 
myasthenia and, iv, 903 
myoclonus and, iv, 903 
myotonia and, iv, 903 
osteomalacia and, iv, 903; v, 949 
paralysis agitans and, iv, 903 
pellagra and, iv, 903 
rickets and, iv, 903 
tetany and, iv, 901; v, 750, 753 
thyroid gland and, iv, 903 
tumors of, iv, 904 
insufficiency, iv, 901 
Paratyphoid fever, i, 198 
Paravertebral triangle of dulness in sero- 
fibrinous pleuritis, ii, 1023 
Parenchymatous changes in kidney, iii, 774 
degeneration of liver, iii, 466 
goitre, iv, 839 
hepatitis, iii, 494 
multiple neuritis, v, 577 
myocarditis, iv, 113 
Paresis in diabetes inellitus, ii, 707 
general, v, 850 
tabes dorsalis, and, v, 843 
trauma and, v, 647 
in lead poisoning, ii, 368 
migraine and, v, 720 
neuralgia and, v, 727 
Paretic gait, v, 73 
Parietal lobe, tumors of, v, 333 
Parkinson's disease, v, 764 
Parorexia, iii, 134 
Parotid gland in mumps, i, 920 
Parotitis, acute, iii, 69 
in bacillary dysentery, i, 776 
chronic, iii, 69 
epidemic, i, 919 
gastric ulcer and, iii, 189 
in glandular fever, i, 1013 
in lobar pneumonia, i, 263 
in rheumatic fever, i, 999 
in subphrenic pyopneumothorax, iii, 
712 
in typhoid fever, i, 103 
Paroxysmal tachycardia, iv, 300 
Pasteur immunization in rabies, i, 970 
Patellar tendon reflex, v, 77 
Patency of vluctus arteriosus Botalli, iv, 
409 
Patent foramen ovale, iv, 355 
Pectoral heart, iv, 346 
Pediculoides ventricosus, ii, 322 
Pediculosis, ii, 326 
I Pediculus, ii, 326 1072 
GENERAL INDEX 
Peliomata in typhoid fever, i, 101 
Pel iosis rheuinatica, iv, 701 
Pellagra, ii, 472; v, 139 
parathyroid glands and, iv, 903 
Pelvic peritonitis, kidney infections and, 
iii, 952 
Pelvis in endemic cretinism, iv, 882 
of kidney, tumors of, iii, 1006 
Pemphigoid rash in typhoid fever, i, 101 
Pemphigus in congenital syphilis, ii, 189 
of mouth, iii, 54 
pellagrosus, ii, 483 
in vaccination, i, 843 
Pentastomum constriction, ii, 324 
denticulatum cyst of spleen, iv, 954 
Pentosuria, ii, 600, 712; iii, 822 
Peptic ulcer, iii, 168 
jejunal, iii, 349 
of oesophagus, iii, 84 
Peptonuria, iii, 820 
Perception, anomalies of, v, 37 
sense, v, 18 
Periaortitis, acute, iv, 452 
Periarteritis nodosa, iv, 470 
nodular, ii, 181 
Peri-appendicular abscess, iii, 364, 370, 377 
Peri-articular arthritis deformans, v, 908 
Pericardial effusion, iv, 49 
sac, obliteration of, hypertrophy of 
heart and, iv, 181 
Pericarditic pseudocirrhosis of liver, iii, 
488, 716; iv, 60, 63, 64 
Pericarditis, iv, 41 
in abscess of lung, ii, 995 
acute, iv, 43 
adhesive, iv, 44, 58 
in arthritis deformans, v, 914 
in cerebrospinal meningitis, i, 609 
chorea and, iv, 42 
chronic, iv, 51 
perihepatitis and, iii, 492 
circumscribed, iv, 42 
with effusion, iv, 49 
in endocarditis, iv, 161 
gonorrhoeal, i, 752; iv, 42 
in gout, ii, 749 
in lobar pneumonia, i, 257 
in measles, i, 907 
mediastinitis and, iv, 59, 62 
purulent, iv, 50 
in rheumatic fever, i, 988; iv, 41 
in scarlet fever, i, 877 
serofibrinous, iv, 50 
sicca, iv, 50 
tricuspid insufficiency and, iv, 247 
tuberculous, iv, 42, 66 
in typhoid fever, i, 126 
in uremia, iii, 839 
Pericardium, absence of, iv, 77, 344 
adherent, iv, 58 
cardiac insufficiency and, iv, 199 
anomalies of, iv, 344 
carcinoma of, iv, 74 
defects of, iv, 77, 344 
diverticulum of, iv, 346 
effusions into, cardiac insufficiency 
and, iv, 193 
Pericardium, fibroids of, iv, 7G 
hernia of, iv, 346 
hydatids of, iv, 76 
lymphoma of, iv, 76 
neoplasms of, iv, 74 
paracentesis of, iv, 56 
sarcoma of, iv, 74 
syphilis of, iv, 76 
tuberculosis of, i, 359; iv, 66 
unattached, iv, 345 
Perichondritis, gonococcal, i, 760 
in lobar pneumonia, i, 248 
Pericolic abscesses, iii, 697 
Pericolitis, iii, 354 
acute, iii, 694 
Perigastric abscess, iii, 696 
adhesions in stenosis of pylorus, iii, 
262 
Perigastritis in gastric ulcer, iii, 815 
Perihepatitis, iii, 486, 488, 718 
Perimesosigmoiditis, iii, 715 
Perinephric abscess, iii, 970 
Perinephritis, iii, 970 
in tuberculosis of kidney, iii, 980 
Periodic paralysis, v, 809 
Periodontitis, iii, 56 
Periosteal dyscrasia, v, 978 
Periostitis alveolaris dentalis, iii, 56 
in syphilis, ii, 163 
Peripheral arteries, embolism of, iv, 568 
thrombosis of, iv, 568 
nerves, diseases of, v, 533 
neuritis, v, 573 
venous thrombosis, iv, 539 
Peripleuritis, ii, 1051 
Perisigmoiditis, iii, 695 
Perisplenic peritonitis, iv, 943 
Perisplenitis, iv, 943 
in abscess of spleen, iv, 945 
Peristalsis in cirrhosis of stomach, iii, 277 
in intestinal colic, iii, 304 
of intestines, iii, 286 
in stenosis of pylorus, iii, 271 
Peristaltic unrest, iii, 151 
Peritoneal abscesses, iii, 698 
Peritoneum, absorption from, iii, 655 
angioma of, iii, 733 
in cirrhosis of liver, iii, 518 
cysts of, iii, 733 
fibroma of, iii, 730 
fibromyoma of, iii, 730 
gangliated neuroma of, iii, 731 
immunity of, iii, 657 
lipoma of, iii, 732 
myoma of, iii, 730 
myxoma of, iii, 733 
pseudomyxoma of, iii, 740 
retroperitoneal hematoma of, iii, 738 
tuberculosis of, i, 364; iii, 720 
tumors of, iii, 730, 740 
Peritonitis, acute circumscribed, iii, 693 
diffuse, iii, 670 
in amoebic dysentery, ii, 32, 44 
in appendicitis, iii, 364, 372, 374 
in bacillary dysentery, i, 776 
chronic diffuse, iii, 715 
simple, iii, 713 GENERAL INDEX 
1073 
Peritonitis deformans, iii, 718 
fibrosa, iii, 718 
from intussusception, iii, 410 
gonococcal, i, 763; iii, 686 
in lobar pneumonia, i, 254 
lymphadenitis and, iv, 736 
pelvic, kidney infections and, iii, 952 
perisplenic, iv, 943 
pneumococcic, iii, 685 
progressive fibrinopurulent, iii, 676 
puerperal, iii, 686 
in rheumatic fever, i, 997 
in scarlet fever, i, 878 
in suppurative pancreatitis, iii, 625 
tuberculous, i, 364; iii, 720 
in typhoid fever, i, 116 
Peritonsillar abscess, ii, 849 
Perityphlitis, iii, 355 
Peri-ureteritis, iii, 974 
kidney infections and, iii, 952 
in pyelitis, iii, 954 
Pernicious anemia, iv, 619 
malarial fevers, ii, 80 
Persistent truncus arteriosus, iv, 375 
Petechise in smallpox, i, 799 
in toxic gastritis, iii, 234 
in tuberculosis, i, 419 
Petit mal, v, 604 
Pfeiffer's reactions in Asiatic cholera, i, 679 
Pfliiger's theory of protein metabolism, ii, 
592 
Phagocytosis, i, 57 
Phantom tumor, iii, 299 
Pharyngeal diphtheria, i, 696 
nerves, paralysis of, v, 517 
tonsil, hypertrophy of, ii, 840 
tuberculosis, i, 429 
Pharyngitis, ii, 843, 844; 845, 846 
Pharynx, diseases of, ii, 842 
in myasthenia gravis, v, 804 
oedema of, ii, 847 
in scarlet fever, i, 863 
, in typhoid fever, i, 104 
Phimosis, kidney infections and, iii, 952 
Phlebitis, iv, 574 
acute suppurative, iv, 577 
chronic, iv, 575 
in glanders, i, 651 
gonorrhoeal, i, 752 
in gout, ii, 749; iv, 574 
idiopathic, iv, 574 
migrans, iv, 531, 576 
phlebosclerosis and, iv, 576 
in rheumatic fever, i, 990 
in scarlet fever, i, 878 
septic, iv, 574 
suppurative, of hepatic veins, iii, 460 
syphilitic, iv, 576 
traumatic, iv, 574 
tuberculous, iv, 576 
in typhoid fever, i, 128, 132 
Phlebosclerosis, phlebitis and, iv, 576 
Phlegmonous cholecystitis, iii, 563 
oesophagitis, iii, 80 
pharyngitis, ii, 846 
Phloridzin diabetes, ii, 685 
Phobias, v, 52 
vol. v-68 
Phonic spasm, v, 522 
Phosphaturia, iii, 814 
Phosphorus poisoning, ii, 381 
Photopsia, v, 36 
Phrenic nerve in aneurism of aorta, iv, 501 
paralysis of, v, 543 
Phthisis, acute, i, 424 
florida, i, 344, 354 
Phthirius pubis, ii, 326 
Physaloptera, ii, 293 
Phytin, metabolism of, ii, 562 
Phytotoxins, i, 34 
Pick's disease, iii, 488, 716 
Pigmentation in Addison's disease, iv, 789, 
790, 792, 795 
in arsenic poisoning, ii, 375 
in exophthalmic goitre, iv, 866 
in Hodgkin's disease, iv, 767 
of lymph nodes, iv, 746 
in scleroderma, iv, 1019 
in silver poisoning, ii, 385 
in splenic anemia, iv, 958 
of thymus, iv, 912 
in typhoid fever, i, 102 
Pineal body, iv, 819 
in hypopituitarism, iv, 817 
lesion of, v, 291 
tumor of, v, 336 
Pinworm infection, ii, 291 
Pithiatism, v, 659 
Pituitary body, iv, 833 
in acromegaly, iv, 808 
carcinoma of, iv, 804 
circulatory changes in, iv, 804 
degeneration of, iv, 804 
diabetes and, ii, 683, 722 
lesion of, v, 291 
sarcoma of, iv, 804 
tumors of, iv, 804 
hypertrophy of heart and, iv, 
113 
dystrophy, iv, 815 
Plague, i, 620 
bubonic, i, 632 
fulminating, i, 634 
intestinal, i, 635 
pestis minor, i, 632 
septicemic, i, 634 
Plasmodium falciparum, ii, 56, 59, 92, 102 
quotidianum, ii, 57, 92, 102 
malaria), ii, 56, 58, 92, 102 
vivax, ii, 56, 57, 92, 102 
Plastic bronchitis, ii, 907 
pleuritis, ii, 1005 
Pleura, actinomycosis of, ii, 1050 
carcinoma of, ii, 1061 
diseases of, ii, 1004 
echinococcus disease of, ii, 1065 
lipoma of, ii, 1061 
nocardiosis of, ii, 1051 
sarcoma of, ii, 1064 
syphilis of, ii, 1051 
tuberculosis of, i, 356 
pericarditis and, iv, 42 
tumors of, ii, 1060, 1061, 1064 
Pleural echinococcus, ii, 1065 
fluids in carcinoma, ii, 1063 1074 
GENERAL INDEX 
Pleural fluids, chyliform, ii, 1057 
chylous, ii, 1057 
pseudochylous, ii, 1057 
Pleuritic adhesions, hypertrophy of heart 
and, iv, 181 
Pleuritis, ii, 1004 
in abscess of lung, ii, 995 
acute fibrinous, ii, 1005 
purulent, ii, 1040 
serofibrinous, ii, 1010 
in aneurism of, aorta, iv, 508 
in arthritis deformans, v, 915 
bronchiectasis and, ii, 914 
in bronchopneumonia, ii, 964 
chronic, ii, 1051 
perihepatitis and, iii, 492 
peritonitis and, iii, 716 
with effusion, ii, 1052 
in cirrhosis of liver, iii, 526 
defects of pericardium and, iv, 345 
diaphragmatic, ii, 1049 
encysted, ii, 1049 
fibrinous, ii, 1005 
gonococcal, i, 762 
hemorrhagic, ii, 1053 
infectious (non-tuberculous) ii, 1011 
interlobar, ii, 1050 
in lobar pneumonia, i, 248 
in malarial fevers, ii, 86 
metapneumonic, ii, 1012 
pericarditis and, iv, 42 
plastic, ii, 1005 
in rheumatic fever, i, 996 
in scarlet fever, i, 876 
in subphrenic pyopneumothorax, iii, 
712 
in tuberculosis, i, 430; ii, 1010 
tumors of lung and, ii, 1001 
in typhoid fever, i, 123 
in typhus fever, i, 930 
in whooping cough, i, 660 
with effusion, ii, 1010 
Pleurodynia, v, 945 
Pleurogenous interstitial pneumonia, ii, 
970 
Plexiform neuromata, v, 571 
Plumbism, ii, 355 
Pneumatosis, iii, 150 
Pncumaturia, iii, 806 
Pneumococcus, i, 208, 274 
in empyema, ii, 1041 
Pneumogastric nerve, diseases of, v, 517 
Pneumokoniosis, ii, 976 
cirrhosis of liver and, iii, 510 
Pneumonia, abortive, i, 237 
acute endocarditis and, iv, 153 
meningitis and, v, 168 
apical, i, 236 
aspiration, i, 206; ii, 958 
asthenic, i, 236 
bronchiectasis and, ii, 914, 917 
caseous, i, 352, 424 
catarrhal, ii, 957 
central, i, 237 
in cerebrospinal meningitis, i, 599, 609 
in childhood, i, 238 
deglutition, ii, 958 
Pneumonia in diabetes mellitus, ii, 706 
in erysipelas, i, 587 
gangrene of lung and, ii, 989 
gelatinous, i, 352 
in glanders, i, 652 
gonococcal, i, 762 
inhalation, i, 206 
lobar, i, 202 
defect of pericardium and, iv, 345 
in diphtheria, i, 266 
in malarial fevers, i, 266 
in measles, i, 265 
in pregnancy, i, 264 
in scarlet fever, i, 265 
in typhoid fever, i, 122, 207, 267 
in typhus fever, i, 267 
lobular, ii, 957 
in malarial fevers, ii, 86 
pericarditis and, iv, 42 
in plague, i, 628, 634 
pleurogenous interstitial, ii, 970 
postoperative, i, 237 
in rheumatic fever, i, 996 
in scarlet fever, i, 878 
senile, i, 236 
terminal, i, 237 
in tuberculosis, i, 350, 424 
tumors of lung and, ii, 1001 
white, ii, 172 
Pneumonic fever, i, 202 
Pneumonitis, i, 202 
Pneumopericardium, iv, 71 
Pneumothorax, ii, 1071 
abscess of lung and, ii, 1074 
acutissimus, ii, 1084 
artificial, ii, 1084 
bronchiectasis and, ii, 1074 
closed, ii, 1084 
defect of pericardium and, iv, 345 
double, ii, 1084 
gangrene of lung and, ii, 1074 
infarction of lung and, ii, 1074 
in influenza, i, 542 
in lobar pneumonia, i, 250 
open, ii, 1083 
partial, ii, 1084 
thoracentesis and, u, 1038, 1074 
in tuberculosis, i, 430, 512: ii, 1073 
valvular, ii, 1083 
wounds of chest and, ii, 1074 
Pneumotyphoid, i, 90 
Poikilocytosis in pernicious anemia, iv, 637 
Poisoning, alcohol, ii, 396 
arsenic, ii, 373 
brass, ii, 386 
carbon monoxide, ii, 388 
cardiac insufficiency and, iv, 199, 202 
cheese, ii, 467 
cocaine, ii, 445 
copper, ii, 386 
by fish, ii, 454 
food, ii, 450 
illuminating gas, ii, 388 
lead, ii, 355, 369 
arteriosclerosis and, iv, 455 
manganese, ii, 386 
meat, ii, 458, 461, 462 GENERAL INDEX 
1075 
Poisoning, mercury, ii, 378 
methyl alcohol, ii, 398 
milk, ii, 466 
nitric oxide, ii, 395 
opium, ii, 433 
phosphorus, ii, 381 
shell-fish, ii, 455 
silver, ii, 384 
tetany and, v, 749 
tin, ii, 386 
vegetable, ii, 468 
zinc, ii, 386 
Poliencephalitis, acute inferior, v, 368 
hemorrhagic, ii, 429 
superior, v, 365 
Poliencephalomyelitis, v, 369 
Poliomyelitis, acute, i, 733 
chronic anterior, v, 82 
Pollantin, ii, 862, 865 
Pollen antitoxin, ii, 861 
toxin, ii, 859 
Polonium, rays from, ii, 339 
Polyarteritis acuta nodosa, iv, 470 
Polyarthritis, multiple neuritis and, v, 
585 
Polychromates, v, 25 
Polycythemia, iv, 684 
of congenital cyanosis, iv, 337, 340 
in patent ductus arteriosis, iv, 415 
tuberculosis of spleen and, iv, 684 
Polydipsia in diabetes mellitus, ii, 698 
in tumor of brain, v, 338 
Polyglandular syndrome, iv, 818 
Polygraphy, heart-beat and, iv, 80 
Polyideic somnambulism, v, 44 
Polymyositis hemorrhagica, v, 879 
Polyneuritic psychosis, v, 45 
Polyneuritis, v, 573 
acute infective, v, 590 
in arsenic poisoning, ii, 376 
facial paralysis and, v, 497 
gonococcal, i, 761 
recurring, v, 591 
Polynuclear cells in lymphoid leukemia, iv, 
673 
Polyorrhymenitis, iii, 488; iv, 60, 63, 64 
Polyplasmia in chlorosis, iv, 653 
Polyps of appendix, iii, 386 
Polysymptomatic neuroses, iii, 164 
Polyuria, 791, 806 
in acromegaly, iv, 808 ' 
in brain tumor, v, 337, 338, 342 
in chronic interstitial nephritis, iii, 900 
pyelitis, iii, 961 
in diabetes, ii, 698, 725 
in exophthalmic goitre, iv, 868 
in hypopituitarism, iv, 816 
in typhoid fever, i, 141 
Pons, lesion of, v, 289 
tumors of, v, 336 
Pontile syndrome, v, 289 
Pontomedullocerebellar space, lesion of, v, 
289 
Popliteal nerves, paralysis of, v, 559 
i Porencephaly, apoplexy and, v, 409 
epilepsy and, v, 598 
Pork-measly tapeworm, ii, 249 
Porocephaliasis, ii, 323 
Portal cirrhosis of liver, iii, 508 
pyemia, i, 566; iii, 463 
vein, abscess of liver and, iii, 497 
embolism of, iii, 463 
perforation of, in gastric ulcer, 
iii, 186 
in splenic anemia, iv, 955 
thrombosis of, iii, 460; iv, 544 
Position, anomalies of, v, 30 
Posterior basic meningitis, i, 615 
spinal sclerosis, v, 833 
Posthemoplegic chorea, v, 466 
Posthemorrhagic anemia, acute, iv, 655 
Postnasal catarrh, ii, 838 
Postoperative pneumonia, i, 237 
thrombosis, iv, 528 
Pott's disease, v, 188 
Pox, ii, 144 
Precipitin test in glanders, i, 654 
Precipitins, i, 57 
Pregnancy, acute yellow atrophy of liver 
and, iii, 472 
apoplexy and, v, 423, 425 
arthritis deformans and, v, 902 
asthma and, ii, 933 
cholelithiasis and, iii, 571 
chorea and, v, 777, 779 
diabetes mellitus and, ii, 677 
exophthalmic goitre and, iv, 851 
kidney of, iii, 867 
chronic nephritis and, iii, 893 
infections and, iii, 952 
myasthenia gravis and, v, 799 
necrosis of pancreas and, iii, 612 
nephritis and, iii, 856 
osteomalacia and, v, 948 
pernicious anemia and, iv, 621 
progressive spinal muscular atrophy 
and, v, 81 
pyelonephritis of, iii, 961 
serodiagnosis of, ii, 652 
syphilis and, ii, 187 
toxemia of, myocarditis and, iv, 108 
tuberculosis of kidney and, iii, 978 
of peritoneum and, iii, 721 
Presbyopia, v, 36 
Pressure, sense of, v, 19, 32 
ulcers of oesophagus, iii, 84 
Priapism in myeloid leukemia, iv, 665 
Primative aortic arches, development of, 
iv, 329 
Proctitis, gonorrhoeal, i, 747 
Professional neuroses, v, 741 
Profeta's immunity in syphilis, ii, 153 
Progeria, iv, 817 
Progressive bulbar palsy, v, 113 
muscular dystrophy, v, 117 
spinal muscular atrophy, v, 82, 87 
Propulsion, v, 74 
Propyl alcohol, ii, 400 
I Prostate, carcinoma of, iii, 1038 
diseases of, iii, 1026 
hypertrophy of, iii, 1035 
kidney infections and, iii, 952 
tuberculosis of, i, 366 
Prostatitis, chronic, i, 746; iii, 1032 1076 
GENERAL INDEX 
Protein equilibrium, ii, 560 
fat formation and, ii, 604 
foreign, ii, 651 
metabolism, ii, 579 
microbic, i, 33 
sparing, ii, 583 
Protopathic sensibility, v, 17 
Protozoa, diarrhoea and, iii, 325 
pathogenic, i, 19 
Protozoon in scarlet fever, i, 860, 863 
Pruritus in catarrhal cholangitis, iii, 553 
in diabetes mellitus, ii, 704 
in exophthalmic goitre, iv, 866 
in Hodgkin's disease, iv, 768 
in jaundice, iii, 452 
Psammoma of brain, v, 318 
Psammosarcoma of brain, v, 315 
Pseudamphistomum truncatum, ii, 233 
Pseudo-achondroplasia, v, 969 
Pseudo-angina, iv, 293 
Pseudobulbar paralysis, v, 290, 798 
Pseudochylous fluids, ii, 1058 
Pseudocirrhosis, pericardial, iii, 488, 716; 
iv, 60 
Pseudocrisis in lobar pneumonia, i, 240 
Pseudohallucinations, v, 37 
Pseudohypertrophic progressive muscular 
atrophy, v, 122 
Pseudoleukemia, iv, 681, 772 
Pseudomembranous bronchitis, ii, 907 
pyelitis, iii, 954 
Pseudomyxoma of peritoneum, iii, 740 
Pseudoparalytica, v, 798 
Pseudopellagra, ii, 485 
Pseudoperforation of gastric ulcer, iii, 188 
Pseudosclerosis, v, 158 
Pseudotabes, syphilitic, v, 826 
Pseudotrichinse, ii, 302 
Pseudotubercle bacilli, i, 297 
Psilosis, iii, 393; ii, 45 
Psittacosis, i, 1024 
Psoriaform syphilide, ii, 189 
Psoriasis lingua?, iii, 64 
of mouth, iii, 53 
syphilitica, ii, 165 
in vaccination, i, 843 
Psychasthenia, v, 816 
Psychic blindness, v, 299 
epilepsy, v, 602 
vomiting, iii, 144 
Psychical neuralgia, v, 726 
Psychoneuroses, digestion and, iii, 37 
Psychoses in hysteria, v, 689 
traumatic, v, 642 
in tuberculosis, i, 321, 392 
in typhoid fever, i, 133 
Psychosis, Korsakow's, ii, 406, 427 
polyneuritic, v, 45 
post-epileptic, v, 603 
Ptomain poisoning, ii, 510 
nephritis and, iii, 853 
Ptomains, i, 553 
Ptosis in cerebrospinal syphilis, v, 825 
in hemiplegia, v, 459 
in hysteria, v, 679 
in ocular paralysis, v, 488 
in paralyzing vertigo, v, 515 
Ptosis in tuberculosis, i, 423 
Ptyalism, iii, 68 
Pudendal plexus, neuralgia of, v, 740 
Puerperal eclampsia, acute yellow atrophy 
of liver and, iii, 473 
infections, chronic peritonitis and, iii, 
714 
peritonitis, iii, 686 
Pulex irritans, ii, 325 
Pulmonary anthrax, i, 646 
artery, accessory, iv, 439 
anomalies of, iv, 439 
dilatation of, iv, 398 
defects of interauricular sep- 
tum and, iv, 359 
embolism of, iv, 562 
left subclavian artery from, iv, 
436 
aspergillosis, i, 1058 
atresia, congenital, iv, 388 
diphtheria, i, 706 
distomatosis, ii, 226 
endarteritis, acute, iv, 413 
hypertrophic osteo-arthropathy, i, 411; 
v, 981 
insufficiency, iv, 255 
nerves, paralysis of, v, 523 
oedema in rheumatic fever, i, 996 
roundworms, ii, 302 
stasis, iv, 19 
stenosis, iv, 258 
congenital, iv, 388 
in tuberculosis, i, 433 
thrombosis, iv, 547 
cardiac insufficiency and, iv, 197 
tuberculosis, acute, i, 424 
miliary, i, 419 
chronic, i, 426 
latent, i, 427 
subacute, i, 426 
veins, anomalies of, iv, 441 
Pulsus alternans, iv, 187 
paradoxus in adherent pericardium, 
iv, 62 
Pupillary reaction, hemianopic, v, 482 
of Wernicke, v, 271 
reflexes, anomalies of, v, 76 
Pupils in apoplexy, v, 439 
in disseminated sclerosis, v, 152 
in general paresis, v, 856 
in tabes dorsalis, v, 837 
Purins, ii, 733 
in protein metabolism, ii, 589 
Purpura, iv, 687 
abdominalis, iv, 708 
angioneurotic oedema and, iv, 707, 
999, 1002 
in cerebrospinal meningitis, iv, 688 
chronic, iv, 713 
continuous, iv, 713 
erythema and, iv, 705 
fulminans, iv, 712 
hemorrhagica, iv, 711 
aplastic anemia and, iv, 699 
Henoch's, iv, 701 
idiopathic, iv, 691 
intermittent, iv, 713 GENERAL INDEX 
1077 
Purpura, iodine and, iv, 689 
mechanical, iv, 690 
nervous, iv, 690 
orthostatique, iv, 690 
in Raynaud's disease, iv, 990 
rheumatic fever and, iv, 704 
rheumatica, iv, 701 
secondary, iv, 688 
senile, iv, 688 
simplex, iv, 700 
in smallpox, iv, 688 
toxic, iv, 689 
in tuberculosis, iv, 688 
in typhus fever, iv, 688 
Purpuric smallpox, i, 804 
Pustular eruption in syphilis, ii, 166 
Putrid bronchitis, ii, 911 
Pyelitis, iii, 937 
acute catarrhal, iii, 953 
in children, iii, 961 
chronic, iii, 954 
croupous, iii, 954 
cystic, iii, 954 
diphtheritic, iii, 954 
fibrinous, iii, 954 
gangrenous, iii, 954 
granular, iii, 954 
pseudomembranous, iii, 954 
in typhoid fever, i, 143 
Pyelonephritis, iii, 937 
of pregnancy, iii, 961 
Pyelonephrosis, iii, 937 
Pyemia, i, 551 
in bronchiectasis, ii, 917 
endocarditis and, iv, 161 
inflammation of thoracic duct and, iv, 
582 
myocarditis and, iv, 109 
nephritis and, iii, 850 
portal, i, 566; iii, 463 
Pylephlebitis, suppurative, i, 570; iii, 
463 
Pylethrombosis, iv, 544 
Pylorospasm, iii, 149, 262 
acute dilatation and, iii, 25 
congenital, iii, 269 
Pylorus, incontinence of, iii, 159 
stenosis of, iii, 262 
gastric ulcer and, iii, 189 
Pyogenic infections of kidney, iii, 937 
Pyonephrosis, iii, 957 
renal calculus and, iii, 1018 
Pyopneumopericardium, iv, 71 
Pyopneumothorax, subphrenic, iii, 710 
Pyorrhoea alveolaris, iii, 56 
arthritis deformans, and, v, 901 
in tuberculosis, i, 431 
Pyroplasma hominis, i, 1021 
Pyrosis in acute gastritis, iii, 228 
Pyuria, iii, 805 
in typhoid fever, i, 142 
Q 
Quadrigeminal bodies, lesion of, v, 282 
tumor of, v, 282 
Quartan malarial parasite, ii, 56, 58 
Quincke's disease, iv, 998 
Quinsy, ii, 849 
R 
Rabies, i, 956 
Radial nerve, paralysis of, v, 547 
Radium, goitre and, iv, 838 
rays from, ii, 339 
Rales in tuberculosis, i, 408, 441 
Ranula, iii, 52 
pancreatica, iii, 640 
Rat-bite disease, i, 1030 
Rat trypanosome, ii, 116 
Raynaud's disease, iv, 975 
arthritis deformans and, v, 929 
Reaction, anomalies of, v, 48 
of degeneration in amyotrophic lateral 
sclerosis, v, 99 
distal, v, 535 
myasthenic, v, 73 
myotonic, v, 73, 891 
Rectum, injuries of, kidney infections and, 
iii, 952 
syphilis of, ii, 175 
in typhoid fever, i, 117 
ulcers of, in tuberculosis, i, 432 
Rectus muscles, paralysis of, v, 485 
Recurrent endocarditis, iv, 148 
laryngeal nerve in aneurism of aorta, 
iv, 502 
Reflex dyspnoea, iv, 38 
neuralgia, v, 726 
vomiting, iii, 145 
Regurgitation, gastric, iii, 140 
Relapsing fevers, ii, 133 
Remittent fever, ii, 133 
Renal arteries, anomalies of, iii, 786 
embolism of, iv, 564 
calculus, iii, 1007 
casts, iii, 798 
colic, renal calculus and, iii, 1019 
pelvis, empyema of, iii, 937 
system in cardiac insufficiency, iv, 199, 
210 
in typhoid fever, i, 140 
vessels, thrombosis of, iii, 789; iv, 542 
Residual peritoneal abscesses, iii, 698 
Resistance, sensation of, v, 17 
Respiratory centre, ii, 812 
accessory, ii, 815 
influenza, i, 539 
irregularity, heart beat and, iv, 83 
movements in pericarditis, iv, 48 
quotient, ii, 618 
stridor in bronchial lymphadenitis, iv, 
735 
in tuberculosis of bronchial 
glands, iv, 743 
system in acute endocarditis, iv, 153 
in angioneurotic oedema, iv, 1007 
in arthritis deformans, v, 915 
in cardiac insufficiency, iv, 206 
in chlorosis, iv, 651 
in exophthalmic goitre, iv, 868 
in gout, ii, 745 1078 
GENERAL INDEX 
Respiratory system in lead poisoning, ii, 
'361 
in pernicious anemia, iv, 634 
in rheumatic fever, i, 995 
syphilis of, ii, 170 
tuberculosis of, i, 347 
in typhoid fever, i, 80, 120 
tract, obstruction of, ii, 815, 818 
Retina in chronic nephritis, iii, 910 
lesions of, v, 474 
Retinal arteries, embolism of, iv, 570 
thrombosis of, iv, 570 
hemorrhages in chronic interstitial 
nephritis, iii, 911 
in leukemia, iv, 665, 671 
in pernicious anemia, iv, 632 
Retinitis, v, 474 
albuminuric, iii, 911; v, 475 
syphilitic, v, 473 
Retrobulbar neuritis, v, 37, 479 
Retroperitoneal abscesses, iii, 698 
subphrenic abscess and, iii, 701 
cysts of peritoneum, iii, 738 
hematoma, iii, 738 
sarcoma, iii, 742 
Retropharyngeal abscess, ii, 846; iv, 734 
Retropulsion, v, 74 
Revaccination, i, 837 
Rhabditis, ii, 295 
cornwalli, ii, 302 
genitalis, ii, 316 
mellyi, ii, 303 
terrisola, ii, 302 
Rhabdomyoma, congenital, iv, 351 
Rhachitis annularis, v, 978 
Rhagades in congenital syphilis, ii, 189 
Rheumatic fever, i, 974 
acute endocarditis and, iv, 154 
aneurism and, iv, 475 
angioneurotic oedema and, iv, 999 
aortic insufficiency in, iv, 221 
aortitis in, iv, 450, 475 
cerebral, i, 992 
chorea and, v, 775 
dilatation of heart in, iv, 49 
endocarditis and, iv, 156 
exophthalmic goitre and, iv, 851 
lymphadenitis in, iv, 737 
myocarditis and, iv, 108, 118 
nephritis and, iii, 850 
pericarditis and, iv, 41 
purpura and, iv, 704 
thrombosis in, iv, 529 
tricuspid insufficiency and, iv, 244 
Rheumatoid arthritis, v, 897, 908 
Rhinitis in influenza, i, 539 
Rhinogenic abscess of brain, v, 385 
Rhythmic segmentation of intestines, iii, 
286 
Ribs in achondroplasia, v, 957 
cervical, v, 554 
Rickets, ii, 775 
achondroplasia and, v, 966 
annular, v, 955 
congenital, ii, 795; v, 955 
convulsions and, v, 792, 793 
fetal, v, 955 
Rickets, infantile scurvy and, ii, 807 
- intra-uterine, v, 955 
late, ii, 790 
micromelic, v, 955 
parathyroid glands and, iv, 903 
syphilis and, ii, 777 
thymus and, iv, 924 
tuberculosis and, i, 322 
Rickety rosary, ii, 783 
Riga's disease, iii, 66 
Rigg's disease, iii, 56 
Rinne's test in nerve deafness, v, 510 
Rocky Mountain spotted fever, i, 1020 
Romberg symptom in tabes dorsalis, v, 838 
Rose fever, ii, 859 
spots in typhoid fever, i, 100 
Roseola in syphilis, ii, 164 
Rotch's sign in congenital cyanosis, iv, 337 
Round ulcer, iii, 168 
Roundworm infection, ii, 270 
Roundworms, intestinal, ii, 271, 297 
muscular, ii, 297 
pulmonary, ii, 302 
subcutaneous, ii, 302 
Rubella, i, 912 
Rumination, iii, 138 
Rupture of aorta, iv, 487 
of cerebral arteries, v, 400 
of heart, hemopericardium and, iv, 70 
insufficiency and, iv, 193 
of oesophagus, iii, 85 
of spleen, iv, 947 
of thoracic duct, iv, 586 
of valves of heart, aortic insufficiency 
and, 219, 227 
S 
Sacculated aneurism, iv, 479, 492 
Sacral disseminated sclerosis, v, 154 
plexus, paralysis of, v, 560 
Sacro-iliac joint in arthritis deformans, v, 
912 923 
St. Anthony's fire, i, 585 
St. Gothard tunnel disease, ii, 271 
St. Vitus' dance, v, 774 
Saliva, iii, 67 
Salivary calculi, iii, 71 
gland, atrophy of, iii, 74 
enlargement of, iii, 73 
stone in, iii, 71 
swelling of, iii, 70 
syphilis of, ii, 174; iii, 71 
tuberculosis of, i, 363; iii, 70 
tumors of, iii, 72 
secretion, iii, 68 
, Salivation, iii, 48, 68 
Salpingitis, thoracic duct and, iv, 583 
Salvarsan in cerebrospinal syphilis, v, 830 
infraspinous, v, 831, 862 
mercury and, ii, 214 
in relapsing fever, ii, 142 
in syphilis, ii, 211 
Salvarsanized serum, v, 831, 862 
Sand, intestinal, iii, 295 
. Sanguinous cysts of peritoneum, iii, 738 GENERAL IN DEN 
1079 
Sarcime in carcinoma of stomach, iii, 213 
Sarcoma of adrenal glands, iv, 781 
of appendix, iii, 387 
of brain, v, 313, 314 
of intestine, iii, 431 
of kidney, iii, 1003 
of liver, iii, 546 
of lung, ii, 998 
of lymph nodes, iv, 747 
of lymphatic vessels, iv, 601 
of mediastinum, ii, 1093 
of oesophagus, iii, 105 
of pancreas, iii, 650 
of pericardium, iv, 74 
of pituitary body, iv, 804 
of pleura, ii, 1064 
retroperitoneal, iii, 742 
of spinal cord, v, 197 
of spleen, iv, 951 
of thoracic duct, iv, 587 
of thymus, iv, 932 
of thyroid gland, iv, 832 
Sarcoptes, ii, 318, 319 
Sarcoptic ascariasis, ii, 318 
itch, ii, 318 
Saturnism, ii, 355 
Scanning speech, v, 74 
Scapulodynia, v, 945 
Scarlet fever, i, 856 
acute endocarditis in, iv, 156 
in animals, i, 860 
apoplexy and, v, 417 
arteriosclerosis and, iv, 454 
bronchopneumonia and, ii, 958 
diphtheria and, i, 700 
multiple neuritis and, v, 575 
myocarditis and, iv, 108 
nephritis and, iii, 849 
purpura fulminans and, iv, 712 
surgical, i, 884 
tuberculosis and, i, 321 
typhoid fever and, i, 158 
vaccination and, i, 843, 887 
whooping cough and, i, 659 
Schistosoma, ii, 239, 240 
Schizonts, ii, 63 
Schonlein's disease, iv, 701 
Schwabach's test, v, 24 
Sciatic nerve, paralysis of, v, 558, 560 
in spondylitis deformans, v, 927 
Sciatica, v, 562 
gonococcal, i, 761 
Scirrhus of thyroid gland, iv, 833 
Scleroderma, diffuse, iv, 1014 
Raynaud's disease and, iv, 986, 
1018, 1021 
of mouth, iii, 54, 60 
Sclerosis, amyotrophic lateral, v, 89 
of brain, v, 140 
of cerebellum, v, 287 
of coronary arteries, myocarditis and, 
iv, 109 
diffuse, v, 160 
disseminated, v, 140, 154 
insular, v, 140 
lateral, v, 101 
of mediastinal glands, ii, 1111 
Sclerosis, miliary, v, 161 
multiple, v, 140 
posterior spinal, v, 833 
subacute combined, of cord, v, 126 
tuberous, v, 160 
Scoleciasis, ii, 328 
Scotoma, v, 34 
Scotomata in migraine, v, 712, 714 
Scorbutus, ii, 799 
Screw-worm, ii, 328 
Scrofula, i, 357 
of lymph nodes, iv, 745 
Scrofuloderma, i, 374 
Scrofulosis, tuberculosis and, i, 320 
Scurvy, ii, 799 
infantile, ii, 804 
rickets and, ii, 807 
Secretion, iii, 19, 30, 290 
of saliva, iii, 67 
Secretory functions, anomalies of, v, 79 
Segmentation of myocardium, iv, 116 
Semilunar cusps, anomalies of, iv, 400 
defective development of, iv, 403 
Seminal vesicles, tuberculosis of, i, 366 
Semple's immunization in rabies, i, 977 
Senile aortic insufficiency, iv, 219 
dementia, trauma and, v, 647 
emphysema of lungs, ii, 982 
heart, myocarditis and, iv, 126 
pneumonia, i, 236 
purpura, iv, 689 
Sensation, deep, v, 536 
disturbances of, v, 28 
epicritic, v, 537 
protopathic, v, 537 
Sense of acceleration, v, 18 
of equilibrium, v, 11 
of fatigue, v, 19, 32 
of hearing, v, 22 
of hunger, v, 19, 31 
interior, v, 17 
of movement, v, 30 
muscle, v, 17 
pain, v, 17 
perception, v, 18, 30 
of position, v, 30 
of pressure, v, 19, 32 
of resistance, v, 17, 30 
sexual, v, 19, 32 
of sight, v, 24 
of smell, v, 21, 33 
of taste, v, 21, 33 
temperature, v, 20, 32 
of thirst, v, 19, 32 
of touch, v, 19, 32 
vestibular, v, 18, 30 
vibration, v, 18, 30 
Sensibility, deep, v, 17, 184 
anomalies of, v, 30 
epicritic, v, 17, 184 
mesoblastic, v, 17 
anomalies of, v, 30 
osseous, v, 30 
protopathic, v, 17, 184 
stereognostic, v, 18, 30 
Sensory aphasia, v, 74, 292, 296 
area of cortex of brain, v, 265 1080 
GENERAL INDEX 
Sensory disturbances in occupation neu- 
roses, v, 747 
phenomena in tetany, v, 759 
Sepsis, gastro-intestinal, pernicious anemia 
and, iv, 623 
oral, iii, 41 
Septic anemia, acute, iv, 658 
endocarditis, chronic, iv, 148 
phlebitis, iv, 574 
Septicemia, i, 551 
acute endocarditis and, i, 558 
in anthrax, i, 557 
in appendicitis, iii, 372 
in bubonic plague, i, 558 
in diphtheria, i, 555 
in gonorrhoea, i, 556, 749 
inflammation of thoracic duct and, iv, 
582 
in influenza, i, 544, 555 
in pneumonia, i, 555 
in pulmonary insufficiency, iv, 257 
in rheumatic fever, i, 558 
in scarlet fever, i, 864 
septic anemia and, iv, 658 
in typhoid fever, i, 557 
Septicemic plague, i, 634 
Septicopyemia, endocarditis and, iv, 157 
Septicopyemic abscess of liver, iii, 499 
Septum, aortic, defects of, iv, 374, 377 
development of, iv, 327 
of heart, absence of, complete, iv, 370 
anomalies of, iv, 352 
development of, iv, 370 
interauricular, defects of, iv, 355 
interventricular, defects of, iv, 363 
development of, iv, 327 
Sera, nephritis and, iii, 852 
Serofibrinous exudate in pericarditis, iv, 
43 
pericarditis, iv, 50 
pleuritis, ii, 1000 
Serositis, multiple, iii, 488; iv, 60, 63, 64 
Serous cysts of spleen, iv, 951 
meningitis, ii, 415 
"Serum disease," i, 62, 729 
Setaria equina, ii, 315 
Shaking palsy, v, 764 
Shell-fish poisoning, ii, 455 
Sialodochitis fibrinosa, iii, 70 
Sialorrhcea, iii, 35, 68 
Sialosis, iii, 68 
Siberian liver-fluke disease, ii, 233 
Sick headache, v, 709 
Siderosis, ii, 976 
Sight, anomalies of, v, 34 
sense of, v, 24 
Silicosis, hepatic, iii, 470 
Silver poisoning, ii, 384 
Simulation in hysteria, v, 659, 667 
Sino-auricular node, iv, 79 
Sinus irregularities, iv, 83 
thrombosis, abscess of brain and, v, 
378 
of Valsalva, aneurism of, iv, 492 
Siriasis, ii, 333 
Sitophobia, iii, 158, 165 
Skin in acromegaly, iv, 807 
Skin in acute catarrhal cholangitis, iii, 553 
nephritis, iii, 864 
in Addison's disease, iv, 789, 791, 792 
in angioneurotic oedema, iv, 1005 
in arsenic poisoning, ii, 375 
in arthritis deformans, v, 916 
atrophy of, from x-rays, ii, 336 
in cardiac insufficiency, iv, 209 
in chronic interstitial nephritis, iii, 906 
in diabetes mellitus, ii, 704 
in diffuse scleroderma, iv, 1016, 1018 
in diseases of peripheral nerves, v, 
540 
in endemic cretinism, iv, 882 884 
eruptions, gonococcal, i, 764 
in erythromelalgia, iv, 1028 
in exophthalmic goitre, iv, 866 
exudative lesions of, iv, 1003 
in facial hemiatrophy, v, 992 
in herpes zoster, v, 572 
in Hodgkin's disease, iv, 767 
in hypopituitarism, iv, 815 
in hysteria, v, 686 
infections, endocarditis and, iv, 153, 
157 
in influenza, i, 545 
in jaundice, iii, 451 
in kala-azar, iv, 970, 971 
in lead poisoning, ii, 361 
in leprosy, i, 527 
in lobar pneumonia, i, 259 
in lymphoid leukemia, iv, 672, 676 
in measles, i, 907 
in meningitis, i, 599, 603, 609 
in myxcedema, iv, 890, 891 
in neuralgia, v, 728, 734 
in oidiomycosis, i, 1052 
in opium smoking, ii, 436 
in pellagra, ii, 478 
in plague, i, 623 
in polymyositis hemorrhagica, v, 879 
in Raynaud's disease, iv, 990 
reflexes in meningitis, i, 607 
in rheumatic fever, i, 996 
in scarlet fever, i, 863, 872 
in silver poisoning, ii, 385 
in splenic anemia, iv, 958 
in sunstroke, ii, 331 
in syringomyelia, v, 224 
tuberculosis of, i, 373, 386 
in typhoid fever, i, 99 
Skull in achondroplasia, v, 956 
in cerebrospinal meningitis, i, 610, 613 
in endemic cretinism, iv, 882 
hyperostosis of, v, 988 
in Paget's disease, v, 971 
percussion of, in acute miliary tuber- 
culosis, i, 423 
Sleeping sickness, ii, 119, 120, 128 
Smallpox, i, 783 
acute endocarditis and, iv, 156 
arteriosclerosis and, iv, 454 
confluent; i, 803 
discrete, i, 803 
experimental, i, 793 
fetal, i, 810 
hemorrhagic pustular, i, 804 GENERAL INDEX 
1081 
Smallpox inoculation, i, 792 
lymphadenitis in, iv, 737 
measles and, i, 907 
nephritis and, iii, 849 
purpuric, i, 804; iv, 688 
Smell, anomalies of, v, 33 
centre of, v, 271 
disturbances of, v, 471 
in hysteria, v, 684 
sense of, v, 21, 33 
Smoker's patch, iii, 64 
Sneezing, mechanism of, ii, 827 
Snuffles in congenital syphilis, ii, 188 
Softening of adrenal glands, iv, 783 
Somatic teniasis, ii, 263 
Somatopsyche, v, 42 
Somnambulisms, v, 44 
"Soul paralysis," v, 268 
Sparganum, ii, 263, 270 
Spargosis, iv, 596 
Spasm, habit, v, 784 
of muscles of neck, v, 525 
of tongue, v, 532 
phonic, v, 522 
of tongue, iii, 62 
Spasmodic torticollis, v, 526 
Spasmophilia, v, 604 
Spastic constipation, iii, 313 
dysphonia, v, 522 
gait in pernicious anemia, iv, 630 
paraplegia in Hodgkin's disease, iv, 
767 
spinal paralysis, v, 101, 105 
syringomyelia, v, 220 
tabes, v, 101 
Spastic-paretic gait, v, 73 
Speech, anomalies of, v, 74 
Sphincters in multiple neuritis, v, 584 
Spina bifida, hydrocephalus and, v, 354 
Spinal accessory nerve, paialysis of, v, 524 
ataxic gait, v, 73 
caries, v, 188 
cord, abscess of, v, 254 
in Addison's disease, iv, 789 
anemia of, v, 255 
in aneurism of aorta, iv, 505 
carcinoma of, v, 196 
in cerebrospinal meningitis, i, 597 
combined system diseases of, v, 
125 
compression of, v, 188 
diseases of, v, 178, 187 
fissure of, v, 210 
hemorrhage into, v, 260 
hyperemia of, v, 257 
lesions of, v, 178, 180 
myeloma of, v, 198 
in pellagra, ii, 478 
in pernicious anemia, iv, 625 
in Raynaud's disease, iv, 989 
in rheumatic fever, i, 994 
sarcoma of, v, 191 
sclerosis of, combined, v, 139 
segmental localization of, v, 178 
syphilis of, v, 823 
thrombosis of, v, 256 
tumors of, v, 196 
Spinal cord, vascular disorders of, v, 255 
curvature in syringomyelia, v, 222 
disseminated sclerosis, v, 154 
membranes, hemorrhage into, v, 257 
meningitis, localized serous, v, 174 
muscular atrophy, progressive, v, 82, 
87 
nerves, diseases of, v, 542 
paralysis, spastic, v, 101, 105 
syphilitic, v, 828 
roots, diseases of, v, 567 
sclerosis, posterior, v, 833 
Spine, deformities of, acute dilatation of 
stomach and, iii, 251 
in Paget's disease, v, 971 
tuberculosis of, subphrenic abscess 
and, iii, 701 
Spirillum fever, ii, 133 
Spirochaeta duttoni, ii, 136 
pallida, ii, 144 
in general paresis, v, 851, 852 
in syphilis of nervous system, v, 
823 
in tabes dorsalis, v, 835 
recurrentis, ii, 133 
refringens, ii, 146, 148 
Spirochetes in mouth, iii, 41 
Spirometry in tuberculosis, i, 404 
Splanchnoptosis, iii, 747 
in chlorosis, iv, 651 
digestion and, iii, 35 
gastro-intestinal, iii, 754 
hepatic, iii, 754 
lithemic, iii, 754 
pregnancy and, iii, 751 
Spleen, abscess of, iv, 944 
subphrenic abscess and, iii, 700 
absence of, iv, 938 
accessory, iv, 938 
in acute hepatitis, iii, 495 
infective polyneuritis, v, 590 
yellow atrophy of liver, iii, 476 
in Addison's disease, iv, 794 
in alcoholism, ii, 404 
amyloid, iv, 949 
kidney and, iii, 935 
liver and, iii, 469 
angioma cavernosum of, iv, 950 
in aplastic anemia, iv, 645 
in arthritis deformans, v, 909, 915 
carcinoma of, iv, 951 
of liver and, iii, 544 
in catarrhal cholangitis, iii, 554 
in cerebrospinal meningitis, i, 599 
in chlorosis, iv, 651 
in chronic peritonitis, iii, 718 
in cirrhosis of liver, iii, 517, 523, 538 
in congenital syphilis, ii, 190 
congestion of, iv, 940 
cysts of, iv, 951 
descent of, iii, 758 
in diphtheria, i, 694 
dislocated, iv, 938 
in enlargement of thymus, iv, 916 
enzymes and, iv, 935 
in exophthalmic goitre, iv, 857, 867 
fibroma of, iv, 950 1082 
GENERAL INDEX 
Spleen, floating, iv, 938 
in Gaucher's splenomegaly, iv, 962 
in glandular fever, i, 1013 
gumma of, ii, 176 
in Henoch's purpura, iv, 710 
in hepatic thrombosis, iv, 546 
in Hodgkin's disease, iv, 762, 767 
infarction of, iv, 564, 946 
infectious diseases and, iv, 936 
inflammation of, iv, 940 
intoxications and, iv, 936 
in kala-azar, iv, 970, 971 
large, iv, 938 
in lobar pneumonia, i, 233 
tabulated, iv, 938 
lymphangioma of, iv, 950 
in lymphoid leukemia, iv, 672 
in malarial fevers, ii, 71, 78 
malformations of, iv, 937 
malpositions of, iv, 938 
metabolism of, iv, 935 
movable, iv, 938 
movements of, iv, 934 
multiple, iv, 938 
in myeloid leukemia, iv, 662, 665 
nerve supply of, iv, 934 
in pernicious anemia, iv, 626, 634 
in plague, i, 628 
in polycythemia, iv, 685 
portal blood and, iv, 936 
in purpura hemorrhagica, iv, 711 
in rickets, ii, 790 
rupture of, iv, 947 
sarcoma of, iv, 951 
in scarlet fever, i, 865 
in septicemia, i, 562 
small, iv, 938 
in smallpox, i, 790 
in splenic anemia, iv, 955 
syphilis of, ii, 176 
tuberculosis of, i, 369 
polycythemia and, iv, 684 
tumors of, iv, 942, 950 
in typhoid fever, i, 85, 117 
in typhus fever, i, 926 
vessels of, thrombosis of, iv, 546 
wandering, iv, 938 
Splenectomy in pernicious anemia, iv, 644 
Splenic anemia, iv, 954 
artery, embolism of, iv, 554 
fever, i, 639 
vein, thrombosis of, iv, 948 
in necrosis of pancreas, iii, 
619 
Splenitis, acute suppurative, iv, 944 
capsular, iv, 943 
Splenomegaly, Gaucher's, iv, 962 
in splenic anemia, iv, 957 
tropical febrile, iv, 964 
Splenoptosis, iv, 938 
Splits of intima of aorta, iv, 487 
Spondylitis deformans, v, 923 
in typhoid fever, i, 147 
Sporadic cretinism, iv, 883 
Sporoblasts, ii, 63 
Sporont, ii, 62 
Sporotrichosis, i, 1055 
Sporotrichosis, disseminated gummatous 
i, 1056 
localized, i, 1057 
Sporozoites, ii, 63 
Sprue, ii, 45; iii, 393 
Stammering, v, 74 
Staphylococcus in empyema, ii, 1041 
epidermidis albus in peritonitis, iii, 674 
pyogenes albus in peritonitis, iii, 674 
aureus in encephalitis, v, 363 
in peritonitis, iii, 675 
in mouth, iii, 40 
Starvation, metabolism of, ii, 606 
Stasis in irritable heart, iv, 319 
vascular, iv, 20 
venous, in pericarditis, iv, 21, 48 
Status epilepticus, v, 609 
hemicranus in migraine, v, 718 
lymphaticus, iv, 912, 916 
thymic death and, iv, 921 
thymicolymphaticus, iv, 916 
Steapsin, iii, 289 
Steatorrhcea in calculus of pancreas, iii, 635 
in cyst of pancreas, iii, 645 
in diseases of pancreas, iii, 599 
Stenosing tuberculosis of intestines, iii, 390 
Stenosis, aortic, iv, 228 
congenital, iv, 391, 399 
of duodenum, iii, 262 
of intestines, congenital, obstruction 
from, iii, 408 
left-sided conus, iv, 400 
mitral, iv, 239 
congenital, iv, 406 
defects of interauricular septum 
and, iv, 381 
of (esophagus, iii, 96 
pulmonary, iv, 258 
congenital, iv, 388 
of pylorus, iii, 262 
congenital hypertrophic, iii, 269 
subaortic, iv, 398 
tricuspid, iv, 250 
congenital, iv, 403 
Stercoral ulcers, iii, 351 
Stereognosis, v, 18, 30 
Sternoclavicular joint in arthritis defor- 
mans, v, 911 
Still's disease, v, 917, 931 
Stokes-Adams syndrome, iv, 90, 209, 305 
in aortic stenosis, iv, 231 
। arteriosclerosis and, iv, 306 
bulbar origin of, iv, 309 
bundle of His and, iv, 308 
heart-block and, iv, 306 
in myocarditis, iv, 127 
syphilis and, iv, 308 
vagus and, iv, 307 
Stomach in Addison's disease, iv, 789 
in alcoholism, ii, 404 
anacidity of, iii, 153 
atony of, iii, 160 
cholelithiasis and, iii, 161 
carcinoma of, iii, 199 
colloid, iii, 203 
multiple, iii, 202 
pernicious anemia and, iv, 627 GENERAL INDEX 
1083 
Stomach, carcinoma of, scirrhus, iii, 203 
secondary, iii, 201 
simplex, iii, 203 
squamous-celled, iii, 203 
cardia of, carcinoma of, iii, 220 
spasm of, iii, 147 
cirrhosis of, iii, 241, 273 
circumscribed, iii, 276 
dilatation of, acute, iii, 24, 248 
chronic, iii, 253 
gastroptosis and, iii, 254 
idiopathic, iii, 253 
in lobar pneumonia, i, 254 
nephroptosis and, iii, 756 
obstructive, iii, 255 
symptomatic, iii, 255 
in tuberculosis, i, 502 
diseases of, functional, iii, 106 
organic, iii, 168 
distension of, iii, 150 
cardiac arhythmia and, iv, 287 
disturbance of function of, iii, 23 
hemorrhagic erosions of, iii, 838 
hyperacidity of, iii, 112 
hyperchlorhydria of, iii, 112 
hypochlorhydria of, iii, 152 
lesser curvature of, carcinoma of, iii, 
219 
motor insufficiency of, iii, 247 
neuroses of, iii, 106 
perforation of, iii, 619 
pylorus of, incontinence of, iii, 159 
spasm of, iii, 149 
stenosis of, iii, 262 
subacidity of, iii, 152 
syphilis of, ii, 174 
tuberculosis of, i, 361, 431 
in typhoid fever, i, 104 
ulcer of, iii, 168 
arteriosclerosis and, iv, 464 
Stomatitis, aphthous, iii, 46 
catarrhal, iii, 45 
in chronic intestinal nephritis, iii, 904 
gangrenous, iii, 49 
gonorrhoeal, i, 748; iii, 51 
herpetic, iii, 51 
hypomycetic, iii, 50 
in measles, i, 906 
mercurial, iii, 48 
mycotic, iii, 50 
in tuberculosis, i, 431 
ulcerative, iii, 47 
in uremia, iii, 838 
Strabismus in ophthalmoplegia, v, 484 
in tuberculosis, i, 423 
Strangulation of intestines, iii, 394 
hemorrhage and, iii, 307 
Streptococcus brevis in mouth, iii, 40 
in empyema, ii, 1041 
erysipelatis, i, 585 
pyogenes in acute peritonitis, iii, 674 
in appendicitis, iii, 361 
in mouth, iii, 40 
rheumaticus, i, 975 
in scarlet fever, i, 859, 864 
Streptothrichosis, i, 1045 
Stricture of intestines, iii, 394 
Stricture of intestines, obstruction from, 
iii, 407 
of oesophagus, non-malignant, iii, 96 
Strongyloides stercoralis, ii, 285, 286 
Strongyloidosis, ii, 285 
Strongylus gibsoni, ii, 294 
Struma, iv, 835 
Stupor, v, 47, 63, 65 
Stuttering, v, 74 
Subacidity, iii, 152 
Subacute combined sclerosis of spinal 
cord, v, 126 
Subaortic stenosis, iv, 398 
Subclavian artery, embolism of, iv, 567 
left, from pulmonary artery, iv, 
436 
right, from descending thoracic 
aorta, iv, 436 
thrombosis of, iv, 567 
Submaxillary glands in typhoid fever, i, 
104 
Suboxidation, ii, 515, 543, 546 
Subphrenic abscess, iii, 699 
left anterior intraperitoneal, iii 
703 
extraperitoneal, iii, 705 
posterior intraperitoneal, iii, 
705 
in lobar pneumonia, i, 253 
right anterior, iii, 703 
extrahepatic, iii, 705 
without gaseous contents, iii, 699 
pneumothorax, ii, 1089 
pyopneumothorax, iii, 710 
appendicular abscess and, iii, 
711 
duodenal ulcer and, iii, 710 
gastric ulcer and, iii, 710 
hydatid cyst of liver and, iii, 711 
Succus entericus, iii, 290 
Succussion sound in pneumothorax, ii, 
1080 
Suffocative catarrh, ii, 962 
Suffocation, mechanics of, ii, 835 
Sugar in urine, iii, 821 
in diabetes mellitus, ii, 701 
Sugar-content of blood, ii, 597 
Sugar-iced liver, iii, 488 
Sulphur, metabolism of, ii, 561 
Sunstroke, ii, 331 
Sun-traumatism, ii, 333 
Superior vena cava, thrombosis of, iv, 541 
Superoxidation, ii, 518, 543, 546 
Suppurative cholangitis, iii, 556 
nephritis, iii, 937 
pancreatitis, iii, 622 
phlebitis of hepatic veins, iii, 460 
pyelonephritis, iii, 937 
pylephlebitis, i, 570; iii, 463 
Suprarenal gland in chronic interstitial 
nephritis, iii, 899 
diseases of, iv, 777 
Suprascapular nerve, paralysis of, v, 545 
Surgical kidney, iii, 937 
Surra, ii, 117 
Sweating sickness, i, 1017 
Sydenham's chorea, v, 774 1084 
GENERAL INDEX 
Sympathetic nerves in aneurism of aorta, 
iv, 501 
system, iii, 24, 35, 107 
Addison's disease and, iv, 788 
in exophthalmic goitre, iv, 857 
Symptomatic anemia, iv, 654 
Syndrome, Benedikt's, v, 369 
cerebellar deficit, v, 287 
Millard-Gubler, v, 369 
pontile, v, 289 
superior, v, 291 
tegmental, v, 281 
thalamic, v, 279, 334, 467 
Weber's, v, 369 
Synechia cordis, iv, 23 
Synesthesia, v, 29 
Synovial membrane in arthritis deformans, 
v, 904, 905 
Syphilides, cutaneous, ii, 150 
pigmented, ii, 186 
tuberculous, ii, 166 
ulcerated, ii, 166 
Syphilis, ii, 144 
achondroplasia and, v, 967 
acute yellow atrophy of liver and, iii, 
473 
of adrenal glands, iv, 786 
of alimentary canal, ii, 174 
amyloid degeneration and, ii, 152 
kidney and, iii, 933 
spleen and, iv, 949 
amyotrophic lateral sclerosis and, v, 90 
aneurism and, iv, 474 
aortic insufficiency and, iv, 219, 222 
apoplexy and, v, 423, 425, 426, 427 
of arteries, ii, 181 
arteriosclerosis and, iv, 454; v, 393 
of brain, v, 823 
of bronchi, ii, 170, 906 
cerebral, aneurism and, v, 399 
diabetes insipidus and, ii, 722 
thrombosis and, v, 400, 401 
cerebrospinal, v, 823, 824 
chancre in, ii, 154 
chorea and, v, 775, 777 
chronic perihepatitis and, iii, 489 
of circulatory organs, ii, 180 
cirrhosis of liver and, iii, 509 
congenital, ii, 187 
dilatation of aorta and, iv, 485 
epilepsy and, v, 595 
exophthalmic goitre and, iv, 851 
fetal, ii, 187 
general paresis and, v, 850 
gumma in, ii, 150 
of heart, ii, 183 
hereditary hemiplegia in, v, 418 
hypertrophic osteo-arthropathy and, 
v, 982 
inflammation of spleen and, iv, 941 
of thoracic duct and, iv, 584 
insontiuin, ii, 156 
of intestine, ii, 175; iii, 391 
of kidneys, ii, 179 
of larynx, ii, 871 
leukoplakia and, iii, 64 
of liver, ii, 176; iii, 541 
Syphilis of lung, ii, 171 
of lymph nodes, iv, 745 
of lymphatic vessels, iv, 596 
of mouth, iii, 58 
myelitis and, v, 228 
myocarditis and, iv, 112 
of myocardium, iv, 142 
nephritis and, iii, 851 
of nervous system, v, 822 
neurasthenia and, v, 629 
in ocular paralysis, v, 488 
of oesophagus, ii, 174; iii, 86 
osteitis deformans and, v, 973 
of pancreas, ii, 174; iii, 632 
of pericardium, iv, 76 
pernicious anemia and, iv, 622 
of pleura, ii, 1051 
postnatal infantile hemiplegia and, v, 
418 
pregnancy and, ii, 187 
of rectum, ii, 175 
of respiratory system, ii, 170 
of salivary glands, ii, 174; iii, 71 
secondary, ii, 157, 168 
of spinal cord, v, 823 
of spleen, ii, 176 
Stokes-Adams syndrome and, iv, 308 
of stomach, ii, 174 
syringomyelia and, v, 212 
tabes dorsalis and, v, 833 
tertiary, ii, 169, 184 
of thoracic duct, iv, 585 
thrombosis in, iv, 530 
of thymus, iv, 929 
of thyroid gland, iv, 831 
of trachea, ii, 170, 906 
tuberculosis and, i, 320, 436 
typhoid fever and, i, 159 
vaccination and, i, 844 
Syphilitic aortitis, ii, 182; iv, 474 
arteritis, ii, 181 
choroidoretinitis, v, 475 
endocarditis, ii, 183 
myelitis, v, 237, 828 
myocarditis, ii, 184 
paraplegia, Erb's, v, 246 
phlebitis, iv, 576 
retinitis, v, 475 
spinal paralysis, v, 828 
vitiligo, ii, 186 
Syphiloderm, gummatous, ii, 184 
tuberculous, ii, 184 
ulcerative, ii, 185 
Syphiloma, ii, 149 
of brain, v, 320, 321 
Syringomyelia, v, 211 
Systemic veins, anomalies of, iv, 439 
Systolic retraction in adherent pericar- 
dium, iv, 61 
T 
Tabes amaurotica, v, 844 
atactica, v, 844 
dolorosa, v, 844 
dorsalis, v, 833 GENERAL INDEX 
1085 
Tabes dorsalis, exophthalmic goitre and, 
iv, 870 
migraine and, v, 720 
neuralgia and, v, 727 
syphilis and, v, 833 
incompleta, v, 844 
juvenilis, v, 844 
mesenterica, i, 359; iv, 744 
paralytica, v, 844 
paresthetica, v, 844 
spastic, v, 101 
superior, v, 840, 844 
visceralis, v, 844 
Tache c5rebrale in meningitis, i, 601 
Tachycardia, iv, 90, 279 
in defects of interauricular septum, iv, 
362 
in exophthalmic goitre, iv, 860 
gastric distension and, iv, 312 
irregular, iv, 93 
orthostatic, iv, 304 
paroxysmal, iv, 85, 281, 300 
bradycardia and, iv, 302 
in patent ductus arteriosus, iv, 415 
primary, iv, 280 
reflex, iv, 280, 312, 315 
regular, iv, 93 
sexual organs and, iv, 318 
simple, iv, 87 
suspension of inhibition and, iv, 314 
in syphilis of myocardium, iv, 143 
in tabes, iv, 277 
in tuberculosis, i, 500 
vagus and, iv, 276, 280, 304 
Tamia, ii, 246, 249, 252, 264, 265 
Taeniasis, ii, 258 
Talalgia, i, 760 
Tapeworm, broad, ii, 255 
dwarf, ii, 252 
fat, ii, 247 
infection, ii, 245 
pork, ii, 249 
Tartar, iii, 71 
Taste, disturbances of, iii, 43 
sense of, v, 21 
Tauraman, i, 305 
Tay-Sachs disease, v, 863 
Teeth, carious, in tuberculosis, i, 431 
in endemic cretinism, iv, 882 
Hutchinson's, iii, 59 
in lead poisoning, ii, 360 
in myxoedema, iv, 891 
in rickets, ii, 782 
Tegmental syndrome, v, 281 
Tegmentum, lesion of, v, 281 
tumors of, v, 336 
Telangiectasis in cirsoid aneurism, iv, 487 
Temporal lobe, tumors of, v, 331 
Tendons, tuberculosis of, i, 373 
Tenesmus in amoebic dysentery, ii, 37 
in diarrhoea, iii, 326 
in intussusception, iii, 411 
Teniasis, ii, 245, 263 
Tenosynovitis, gonococcal, i, 760 
Teratoma of mediastinum, ii, 1101 
of thymus, iv, 933 
Terminal infections, i, 559 
Terminal pneumonia, i, 237 
Ternidens deminutus, ii, 294 
Tertian malarial parasite, ii, 56, 57 
Testicle in smallpox, i, 791 
tuberculosis of, i, 366 
Testicular neuralgia, v, 740 
Tetanolysin, i, 34 
Tetanospasmin, i, 34 
Tetanus, i, 575, 579, 580 
Tetany, v, 748 
auto-intoxication and, ii, 511 
benign, v, 759 
in dilatation of stomach, iii, 257 
epidemic, v, 750, 752 
gastric ulcer and, iii, 189 
hysteria and, v, 677 
idiopathic, v, 749 
in lobar pneumonia, i, 262 
non-operative, iv, 902 
operative, iv, 901 
osteomalacia and, v, 949 
parathyreopriva, v, 761 
parathyroid glands and, iv, 901; v, 
749, 750, 753 
in pylorospasm, iii, 150 
in rickets, ii, 788 
in typhoid fever, i, 136, 158 
vaccination and, i, 844 
Tetranychus molestissimus, ii, 323 
Thalamic syndrome, v, 279, 334, 467 
Thermanesthesias, v, 32 
Thirst, sense of, v, 19, 32 
Thomsen's disease, v, 886 
Thoracentesis, ii, 1035 
emphysema and, ii, 1039 
pneumothorax and, ii, 1038, 1074 
Thoracic abscess in tuberculosis, i, 435 
actinomycosis, i, 1039 
duct, absence of, iv, 580 
anomalies of, iv, 580 
carcinoma of, iv, 588 
chyle cysts of, iv, 586 
ectasia of, iv, 585 
endothelioma of, iv, 588 
fat embolism and, iv, 582 
hemorrhage into, iv, 581 
inflammation of, iv, 582 
lymphangivitis of, iv, 584 
obstruction of, iv, 586 
parasites of, iv, 589 
rupture of, iv, 586 
sarcoma of, iv, 587 
syphilis of, iv, 585 
thrombosis of, iv, 581 
tuberculosis of, iv, 584 
tumors of, iv, 587 
nerve, long, paralysis of, v, 544 
wall, perforation of, in purulent pleu- 
ritus, ii, 1044 
Thorax in acromegaly, iv, 810 
Threadworm infection, ii, 305 
Throat in cerebrospinal meningitis, i, 604 
Thrombi, agglutination of, iv, 527 
arterial, iv, 537 
cardiac, iv, 536 
venous, iv, 537 
Thrombo-angeitis obliterans, iv, 547 1086 
GENERAL INDEX 
Thrombophlebitis, idiopathic recurrent, iv, 
531 
of umbilical veins, iv, 546 
Thrombosis, iv, 526 
in aneurism, iv, 482 
of aorta, iv, 565 
apoplexy and, v, 423, 425 
arterial, iv, 537 
in cachexia, iv, 530 
capillary, iv, 538, 548 
cardiac, iv, 531, 536, 548 
of carotid artery, iv, 567 
of cerebral arteries, v, 400 
in cervical ribs, v, 556 
in chlorosis, iv, 530 
of coronary arteries, iv, 569 
cardiac insufficiency and, iv, 
195 
myocarditis and, iv, 109 
in dysentery, iv, 530 
embolism from, iv, 548 
in enlargement of thymus, iv, 916 
in gonorrhoea, iv, 529 
in gout, iv, 531 
of hepatic artery, iii, 458 
veins, iv, 545 
infarction of spleen and, iv, 946 
of inferior vena cava, iv, 541 
in influenza, iv, 528 
in lobar pneumonia, i, 257 
of lung, ii, 954 
in malarial fevers, iv, 530 
of mesenteric arteries, iii, 434 
hemorrhage and, iii, 307 
vessels, iv, 542 
in mitral stenosis, iv, 242 
in parasitic diseases, iv, 530 
of peripheral arteries, iv, 568 
in pneumonia, iv, 528 
of portal vein, iii, 460; iv, 544 
ascites and, iii, 659 
postoperative, iv, 528 
primary infective, iv, 532 
pulmonary, iv, 547 
of renal vessels, iii, 789; iv, 542 
of retinal arteries, iv, 570 
in rheumatic fever, iv, 529 
of spinal cord, v, 256 
of splenic vein, iv, 948 
of subclavian artery, iv, 567 
of superior vena cava, iv, 541 
in syphilis, ii, 162; iv, 530 
of thoracic duct, iv, 581 
in tuberculosis, i, 433; iv, 529 
in typhoid fever, i, 127, 132; iv, 527 
in typhus fever, iv, 530 
venous, iv, 537 
of vessels of spleen, iv, 546 
Thrombotic ulcers, iii, 350 
Thrush, iii, 50 
in tuberculosis, i, 431 
Thymic asthma, iv, 918 
death, iv, 918 
stridor, iv, 917 
tracheostenosis, iv, 917 
Thymitis, iv, 928 
Thymus, absence of, iv, 908 
Thymus in acromegaly, iv, 931 
in acute infections, iv, 934 
calcification of, iv, 912 
carcinoma of, iv, 932 
circulatory disturbances of, iv, 909 
congestion of, iv, 909, 912 
convulsions and, v, 794 
cystic softening of, iv, 912 
cysts of, iv, 931, 933 
enlargement of, iv, 912 
in exophthalmic goitre, iv, 857, 867, 
931 
hemorrhage in, iv, 910 
hyperplasia of, iv, 912 
hypertrophy of, iv, 912 
in hypopituitarism, iv, 817 
inflammation of, iv, 928 
in leukemia, iv, 932 
lipoma of, iv, 932 
lympho-adenoma of, iv, 932 
lymphoma of, iv, 932 
lymphosarcoma of, iv, 932 
in myasthenia gravis, v, 799 
cedema of, iv, 909 
pigmentation of, iv, 912 
in pseudoleukemia, iv, 932 
retrograde changes in, iv, 910 
sarcoma of, iv, 932 
in sporadic cretinism, iv, 884 
syphilis of, iv, 930 
teratoma of, iv, 933 
tuberculosis of, iv, 929 
tumors of, iv, 931 
Thyroid gland, achondroplasia and, v, 965 
in acromegaly, iv, 808 
actinomycosis of, iv, 830 
in Addison's disease, iv, 789 
adenocarcinoma of, iv, 832, 834 
carcinoma of, iv, 832 
congestion of, iv, 825 
diabetes mellitus and, ii, 684 
echinococcus of, iv, 831 
in endemic cretinism, iv, 882 
in exophthalmic goitre, iv, 853, 
858 
in hypopituitarism, iv, 817 
inflammation of, iv, 825 
in myxmdema, iv, 890 
in osteomalacia, v, 948 
parathyroid glands and, iv, 903 
sarcoma of, iv, 832 
scirrhus of, iv, 833 
syphilis of, iv, 831 
tuberculosis of, iv, 829 
tumors of, iv, 832 
tumors of tongue, iii, 66 
Thyroiditis, iv, 825 
Graves' disease and, iv, 829 
infectious diseases and, iv, 826 
in lobar pneumonia, i, 264 
myxeedema and, iv, 829 
sporadic cretinism, and, iv, 883 
toxic, iv, 826 
Tibial nerve, posterior, paralysis of, v, 559 
Tic convulsif, v, 504, 784 
douloureux, v, 493 
I Tick fever, i, 1020; ii, 133 GENERAL INDEX 
1087 
Tick infection, ii, 317 
Tin poisoning, ii, 386 
Tinea circinata of mouth, iii, 54 
Tinnitus aurium, v, 508 
Toe-drop, ii, 366 
Tone deafness in aphasia, v, 297 
Tongue, actinomycosis of, iii, 59 
anomalies of, iii, 60 
atrophy of, iii, 66; v, 531 
black, iii, 61 
carcinoma of, iii, 66 
doubling of, iii, 61 
fibroma of, iii, 66 
in foot-and-mouth disease, i, 1027 
frenum of, deformities of, iii, 61 
ulcer of, iii, 63 
geographical, iii, 63 
in hemiplegia, v, 460 
hyperkeratosis of, iii, 61 
muscles of, spasm of, v, 532 
in myxcedema, iv, 891 
paralysis of, v, 530 
Higa's disease of, iii, 66 
in scarlet fever, i, 863, 870 
spasm of, iii, 62 
in sprue, iii, 393 
sucking, iii, 61 
swallowing, iii, 61 
syphilis of, ii, 186 
in tuberculosis, i, 387, 431; iii, 59 
tumors of, iii, 66 
wandering rash of, iii, 63 
worm infection, ii, 323 
Tonicity of heart muscle, iv, 190 
Tonsillitis, acute, ii, 848 
angioneurotic cedema and, iv, 999 
arthritic deformans and, v, 901 
chronic, ii, 851 
enlargement of thymus and, iv, 924 
exophthalmic goitre and, iv, 850 
in influenza, i, 539 
in lobar pneumonia, i, 223 
nephritis and, iii, 850 
in purpura rheumatica, iv, 703 
in rheumatic fever, i, 995 
tuberculosis and, i, 321 
Tonsilloliths, ii, 854 
Tonsils in diphtheria, i, 691, 695 
diseases of, ii, 847 
endocarditis and, iv, 152, 154 
in enlargement of thymus, iv, 916 
mycosis of, ii, 852 
pharyngeal, hypertrophy of, ii, 840 
in scarlet fever, i, 863 
tuberculosis of, i, 357, 387, 429 
Tonus of muscles, v, 68 
Topalgia in hysteria, v, 683 
Tophi in gout, ii, 744, 746, 751 
Torticollis, v, 526, 945 
Touch, sense of, v, 19, 32 
Tourniquet's sign in scarlet fever, i, 880 
Toxemia, i, 550 
apoplexy, and, v, 425 
in chronic nephritis, iii, 907 
in diphtheria, i, 563 
myocarditis and, iv, 124 
of pregnancy, myocarditis and, iv, 108 
Toxic dyspnoea, iv, 37 
myelitis, v, 236 
purpura, iv, 689 
thyroiditis, iv, 826 
Toxins, i, 33, 553 
epilepsy and, v, 594 
kidneys and, iii, 773 
myasthenia gravis and, v, 800 
nephritis and, iii, 852 
neuralgia and, v, 726 
Trachea in aneurism of aorta, iv, 502, 507 
compression of, iv, 914 
gumma of, ii, 170 
obstruction of, ii, 816 
syphilis of, ii, 170, 906 
tuberculosis of, ii, 905 
Tracheal tug in aneurism, iv, 495, 497 
Tracheitis in measles, i, 906 
Tracheostenosis, thymic, iv, 917 
Tracheotomy in diphtheria, i, 722 
Traction aneurism, iv, 472 
Transudates in ascites, iii, 659 
Transverse aorta, aneurism of, iv, 493 
Traumatic abscess of brain, v, 382 
arteriovenous aneurism, iv, 510 
chylothorax, ii, 1059 
cysts of pancreas, iii, 641 
hemothorax, ii, 1055 
hysteria, v, 644 
lumbago, v, 644 
mental disturbances, v, 646 
neurasthenia, v, 643 
neuroses, v, 642 
pancreatitis, iii, 612 
phlebitis, iv, 574 
psychoses, v, 742 
Traumatism, abscess of liver and, iii, 497 
aneurism and, iv, 478 
angioneurotic oedema and, iv, 1000 
axillary lymphadenitis and, iv, 735 
carcinoma of stomach and, iii, 251 
delirium tremens and, ii, 410 
dilatation of stomach and, iii, 249 
erysipelas and, i, 586 
kidney infections and, iii, 952 
lymphadenitis simplex and, iv, 730 
lymphangitis and, iv, 592 
movable spleen and, iv, 939 
pleuritis and, ii, 1012 
rupture of spleen and, iv, 947 
tetanus and, i, 576 
Trematode infections, ii, 225 
Tremor, v, 70 
Treponema pallidum, ii, 144 
Trichina, ii, 295, 297 
Trichinella spiralis, ii, 297 
Trichiniasis, ii, 297 
Trichinosis, ii, 297 
typhoid fever and, i, 158 
Trichiurus-, appendicitis and, iii, 360 
Trichocephaliasis, ii, 295 
Trichocephalus, ii, 295 
Trichomonas in carcinoma of stomach, iii, 
213 
intestinalis in gangrene of lung, ii, 988 
Trichostrongylus, ii, 294 
Trichuris trichiura, ii, 295 1088 
GENERAL INDEX 
Tricuspid atresia, congenital, iv, 403 
insufficiency, iv, 244 
in tuberculosis, i, 433 
stenosis, iv, 250 
in tuberculosis, i, 433 
valve, safety valve action of, iv, 245 
Trigeminal nerve, diseases of, v, 491 
paralysis of, v, 493, 494 
in tabes dorsalis, v, 841 
neuralgia, v, 733 
Trismus, i, 575; v, 495 
Trombidium tlalsahuate, ii, 323 
Trophic changes in disseminated sclerosis, 
v, 153 
in multiple neuritis, v, 584 
disturbances in general paresis, v, 858 
in hysteria, v, 685 
in syringomyelia, v, 224 
in tabes dorsalis, v, 841 
• functions, anomalies of, v, 79 
phenomena in tetany, v, 759 
ulcers, iii, 352 
Tropical abscess of liver, iii, 500 
big leg, iv, 596 
chlorosis, ii, 271 
liver, iii, 494 
Trousseau's phenomena, v, 71, 756 
Truncus arteriosus, persistent, iv, 375 
Trypanosoma brucei, ii, 117 
evansi, ii, 117 
gambiense, ii, 119, 121, 122 
lewisi, ii, 116 
rhodasiense, ii, 119, 121, 122 
Trypanosomes in blood, ii, 121 
in cerebrospinal fluid, ii, 121 
in glands, ii, 121, 127 
Trypanosomiasis, ii, 116 
human, ii, 119, 127 
Trypsin, iii, 289 
Tsetse-fly disease, ii, 117 
Tubercle bacillus in empyema, ii, 1041 
in pleuritis, ii, 1011 
in sputum in tuberculosis, i, 445 
in tuberculosis of kidney, iii, 984 
of brain, solitary, v, 319 
leprosy, i, 528 
Tubercula dolorosa, v, 570 
Tuberculin, i, 304, 307, 505 
Tuberculinic acid, i, 303 
Tuberculoma of brain, encephalic, v, 319 
Tuberculomyces, i, 294 
Tuberculosis, i, 287 
acute bronchopneumonic, i, 425 
endocarditis and, iv, 157 
pneumonic, i, 424 
of adrenal glands, i, 369; iv, 786, 787 
amyloid kidney and, iii, 933 
spleen and, iv, 949 
aneurism and, iv, 476 
in animals, i, 293 
of anus, i, 363 
of appendix, iii, 385 
arteriosclerosis and, iv, 454 
of axillary glands, iv, 744 
of bladder, i, 366 
of bloodvessels, i, 360, 3S6 
of bones, i, 371 
Tuberculosis of brain, i, 370 
of breast, i, 368 
of bronchi, i, 348; ii, 905 
of bronchial glands, iv, 743 
bronchopneumonia and, ii, 964 
of bursae, i, 373 
of cervical glands, iv, 743 
lymphadenitis and, iv, 734 
chronic nephritis and, iii, 880 
peritonitis and, iii, 717 
cirrhosis of liver and, iii, 590, 526 
congenital, i, 28 
defect of pericardium and, iv, 345 
in diabetes mellitus, ii, 706 
diphtheria and, i, 321, 436 
of endocardium, i, 360 
of epididymes, i, 366 
of epitrochlear gland, iv, 744 
exophthalmic goitre and, iv, 851 
of Fallopian tubes, i, 368 
of fasciae, i, 373 
fatty liver and, iii, 467 
of gall-bladder, i, 364 
of gall-ducts, i, 364 
gastric, i, 431 
ulcer and, iii, 170 
of genito-urinary tract, i, 365, 389; 
iii, 1034 
of gums, iii, 57 
Hodgkin's disease and, iv, 156, 773 
influenza and, i, 321 
of inguinal glands, iv, 744 
of intestines, i, 361, 432; iii, 387 
of joints, i, 371 
of kidneys, i, 365 
of larynx, i, 348; ii, 876 
in lead poisoning, ii, 364 
of lips, iii, 57 
of liver, i, 363; iii, 541 
lobar pneumonia and, i, 321, 430 
of lungs; i, 348 
pericarditis and, iv, 42 
of lymph glands, i, 357; iv, 741 
of lymphatic channels, i, 359 
tissue, i, 357 
vessels, iv, 595 
in lymphoid leukemia, iv, 675 
malarial fever and, i, 322; ii, 86 
measles and, i, 907 
of mediastinal glands, ii, 1111 
of mesenteric glands, iv, 743 
miliary, acute, i, 417 
meningeal, i, 420 
pulmonary, i, 419 
of mouth, i, 360; iii, 57 
multiple neuritis and, v, 515, 586 
of muscles, i, 373 
in myeloid leukemia, iv, 670 
of myocardium, i, 359; iv, 144 
of nasopharynx, i, 429 
nephritis and, iii, 850 
of nose, i, 347 
of oesophagus, i, 361; iii, 85 
osteo-arthropathy and, v, 982 
of ovaries, i, 368 
of pancreas, i, 363; iii, 631 
of pericardium, i, 359; iv, 66 GENERAL INDEX 
1089 
Tuberculosis of peritoneum,!, 364; iii, 720 
of pleura, i, 356 
pericarditis and, iv, 42 
pleuritis and, ii, 1006, 1026 
pneumothorax and, ii, 1073 
of prostate, i, 366 
pulmonary, i, 517 , 
of rectum, i, 362 
of salivary glands, i, 363; iii, 70 
of seminal vesicles, i, 366 
of skin, i, 373, 386 
of soft palate, iii, 57 
of spleen, i, 369 
of stomach, i, 361, 431 
syphilis of lung and, ii, 173 
of tendons, i, 373 
of testicles, i, 366 
of thoracic duct, iv, 584 
■ of thymus, iv, 929 
of thyroid glands, i, 369, 435; iv, 829 
of tongue, iii, 57 
of tonsil, i, 357, 429 
tonsillitis and, i, 321 
of trachea, i, 348; ii, 905 
trauma and, i, 322 
typhoid fever and, i, 157, 322 
of uterus, i, 368 
of vagina, i, 368 
whooping cough and, i, 321 
Tuberculous aortitis, iv, 452 
bronchopneumonia, i, 425 
hemoserothorax, ii, 1053 
lymphadenitis, iv, 741 
meningitis, i, 420 
arhythmia and, iv, 287 
phlebitis, iv, 576 
pleuritis, ii, 1010 
in cirrhosis of liver, iii, 526 
pneumonia, i, 424 
syphilide, ii, 166 
syphiloderm, ii, 184 
ulcers of intestines, iii, 388 
Tuberous sclerosis, v, 160 
Tumor of adrenal glands, iv, 784 
in appendicitis, iii, 370 
of appendix, iii, 386 
of basal ganglia, v, 334 
of brain, v, 308 
stem, 335 
of cavum Meckelii, v, 340 
of centrum semiovale, v, 276, 334 
of cerebellum, v, 338 
in cirrhosis of stomach, iii, 277 
of corpora quadrigemina, v, 336 
of corpus callosum, v, 276, 335 
of crura, v, 335 
in cyst of pancreas, iii, 643 
extracerebellar, v, 338 
of fourth ventricle, v, 338 
of frontal lobe, v, 331 
in gangrenous pancreatitis, iii, 618 
hydrocephalus and, v, 356 
in hypertrophic stenosis of pylorus, iii, 
271 
in hypopituitarism, iv, 817 
in inflammation of spleen, iv, 942 
of intestines, iii, 424, 433 
Tumor, intracerebellar, v, 338 
of kidney, iii, 994, 1003, 1005 
of lips, iii, 45 
of liver, iii, 541, 546 
of lung, ii, 997 
of lymph nodes, iv, 747, 752 
of mediastinum, ii, 1093 
of medulla oblongata, v, 337 
of mouth, iii, 52 
of nerves, v, 568 
of paracentral convolutions, v, 332 
of parathyroid glands, iv, 904 
of parietal lobe, v, 333 
of pelvis of kidney, iii, 1006 
of pericardium, iv, 92 
of peritoneum, iii, 730, 740 
phantom, iii, 299 
of pineal gland, v, 336 
of pituitary body, iv, 804 
of pleura, ii, 1060, 1064 
of pons, v, 336 
of quadrigeminal bodies, v, 282 
of salivary glands, iii, 7'2 
of spinal cord, v, 196 
of spleen, iv, 950 
in suppurative pancreatitis, iii, 624 
of tegmentum, v, 336 
of temporal lobe, v, 331 
of thoracic duct, iv, 587 
of thymus, iv, 931 
of thyroid gland, iv, 832 
of tongue, iii, 66 
Tunnel anemia, ii, 271 
Twilight states, v, 44 
Twitchings, fibrillary, v, 70 
Tydeus molestus, ii, 323 
Tympanites, iii, 298 
in acute peritonitis, iii, 681 
Typhilitis, iii, 355 
Typhoid bacillus, acute cholecystitis and, 
iii, 561 
in blood, i, 78 
endocarditis, iv, 161 
fever, i, 67 
alcoholism and, i, 159 
ambulatory, i, 153 
amoebic dysentery and, i, 158 
apoplexy and, v, 417, 425 
appendicitis and, i, 159 
arteriosclerosis and, iv, 454 
Asiatic cholera and, i, 158 
bacillary dysentery and, i, 777 
bacterial infections and, i, 159 
cerebral thrombosis and, v, 400 
cirrhosis of liver and, iii, 509 
diphtheria and, i, 158 
disseminated sclerosis and, v, 
141 
erysipelas and, i, 158 
in fetus, i, 156 
gonorrhoea and, i, 158 
hemorrhagic, i, 153 
in infancy, i, 154 
inflammation of spleen and, iv, 
941 
influenza and, i, 158 
intussusception and, i, 159 1090 
GEN ERAI. INDEX 
Typhoid fever, lobar pneumonia and, i, 
122, 207, 267 
malarial fever and, i, 156 
malignant, i, 152 
mild, i, 152 
multiple neuritis and, v, 575 
myocarditis and, iv, 108, 117 
nephritis and, iii, 849 
neurasthenia and, v, 629 
scarlet fever and, i, 158 
thrombosis in, iv, 527, 530 
tuberculosis and, i, 157, 322 
typhus fever and, i, 158 
form of influenza, i, 544 
pellagra, ii, 482 
spine, i, 147 
Typhus fever, i, 924 
typhoid fever and, i, 158 
Tyroglyphus, ii, 323 
Tyrosin in urine, iii, 816 
Tyrot oxicon, ii, 467 
Tyrotoxismus, ii, 467 
U 
Ulcer, duodenal, iii, 191, 344 
subphrenic abscess and, iii 701 
pyopneumothorax and, iii, 
710 
of frenum of tongue, iii, 63 
gastric, iii, 168 
chronic peritonitis and, iii, 714 
intestinal hemorrhage and, iii, 
307 
subphrenic abscess and, iii, 701 
pyopneumothorax and, iii, 
710 
in tuberculosis, i, 432 
of intestine, iii, 343 
of oesophagus, iii, 84 
peptic, iii, 168 
jejunal, iii, 349 
of rectum in tuberculosis, i, 432 
of stomach, arteriosclerosis and, iv,464 
hematemesis from, iii, 283 
hyperacidity and, iii, 114 
syphilitic, ii, 174 
trophic, iii, 352 
in typhoid fever, i, 101 
uremic, in chronic nephritis, iii, 899 
Ulcerated syphilide, ii, 166 
Ulcerative cholecystitis, iii, 563 
endocarditis, iv, 148, 151 
stomatitis, iii, 49 
syphiloderm, ii, 185 
Ulnar nerve, paralysis of, v, 549 
Umbilical veins, thrombophlebitis of, iv, 
546 
Uncinaria americana, ii, 270 
craniformis, ii, 271 
duodenalis, cirrhosis of liver and, iii, 
510 
vulpis, ii, 271 
Uncinariasis, ii, 271 
Uncinate fits, v, 332 
Undulant fever, i, 664 
Unilateral paralysis, v, IOS 
Urachial cysts of peritoneum, iii, 739 
Uranium, rays from, ii, 339 
Urea, intoxication and, ii, 527, 534 
in protein metabolism, ii, 586 
Uremia, ii, 534; iii, 830 
acute, iii, 841 
auto-intoxication and, ii, 534 
chronic, iii, 842 
in gout, ii, 750 
in mercury poisoning, ii, 380 
Uremic ulcers in chronic nephritis, iii, 899 
of intestines, iii, 350, 838 
of oesophagus, iii, 84 
Ureter, inflammation of, iii, 974 
in pyelitis, iii, 954 
in renal calculus, iii, 1017 
stricture of, kidney infections and, 
iii, 952 
in tuberculosis of kidney, iii, 980 
Ureteral catheterization, iii, 939 
Ureteritis, iii, 974 
kidney infections and, iii, 952 
Urethra, stricture of, kidney infections 
and, iii, 952 
Urethritis, tuberculosis of kidney and, iii, 
978 
in typhoid fever, i, 144 
Uric acid in blood, ii, 539, 736 
calculus, iii, 1014 
in diabetes mellitus, ii, 703 
excretion of, in gout, ii, 737 
metabolism in gout, ii, 732 
purins and, ii, 733 
Urinary excretion, anomalies of, iii, 791 
lithiasis, iii, 1007 
tract in typhoid fever, i, 86 
Urine in abscess of liver, iii, 592 
of kidney, iii, 959 
in acromegaly, iv, 812 
in acute catarrhal cholangitis, iii, 553 
dilatation of stomach, iii, 252 
hepatitis, iii, 495 
nephritis, iii, 861 
peritonitis, iii, 684 
yellow atrophy of liver, iii, 476 
in Addison's disease, iv, 795 
air in, iii, 806 
albumin in, iii, 794 
ammoniacal, iii, 793 
in amyloid kidney, iii, 935 
in appendicitis, iii, 371 
in arteriosclerosis, iv, 463 
in arthritis deformans, v, 916 
bile in, iii, 828 
in blackwater fever, ii, 114 
blood in, iii, 820 
in carcinoma of stomach, iii, 207 
in cardiac insufficiency, iv, 210 
casts in, iii, 798 
in chlorosis, iv, 651 
in chronic nephritis, iii, 882, 900 
perihepatitis, iii, 492 
chyle in, iii, 805 
in cirrhosis of liver, iii, 526, 539 
in congestion of liver, iii, 479, 485 
creatinin in, iii, 813 GENERAL INDEX 
1091 
Urine, cryoscopy of, iii, 792 
crystals in, iii, 800 
cylindroids in, iii, 800 
cystin in, iii, 817 
in diabetes mellitus, ii, 700 
in dilatation of stomach, iii, 252, 257 
in erysipelas, i, 588 
fat in, iii, 827 
in fat necrosis, iii, 609 
fibrin in, iii, 806 
in gastric ulcer, iii, 176 
glucose in, iii, 821 
glycuronic acid in, iii, 823 
in gout, ii, 748, 749 
hemogentisic acid in, iii, 824 
in hyperacidity, iii, 117 
in hypernephroma, iii, 997 
in jaundice, iii, 453 
in kala-azar, iv, 972 
in kidney of pregnancy, iii, 868 
lactose in, iii, 822 
leucin in, iii, 816 
levulose in, iii, 821 
in lobar pneumonia, i, 232 
in lymphoid leukemia, iv, 672 
in malarial fevers, ii, 67 
in measles, i, 905 
in migraine, v, 718 
in mitral insufficiency, iv, 236 
in myasthenia gravis, v, 805 
in myeloid leukemia, iv, 666 
in necrosis of pancreas, iii, 617 
in nephritis, iii, 959 
in nephroptosis, iii, 756 
in neurasthenia, v, 632 
in osteomalacia, v, 952 
in paranephritis, iii, 972 
in pellagra, ii, 484 
pentoses in, iii, 822 
in periodic paralysis, v, 811 
in pernicious anemia, iv, 635 
in plague, i, 629 
proteins in, iii, 794, 818 
pus in, iii, 805 
in pyelitis, iii, 958 
in pyelonephritis, iii, 958 
in pyogenic infections of kidney, iii, 
958 
in pyonephrosis, iii, 959 
in Raynaud's disease, iv, 989 
in serofibrinous pleuritis, ii, 1017 
spermatozoa in, 800 
sugars in, 821 
in tuberculosis, i, 389 
of kidney, iii, 983 
of peritoneum, iii, 726 
in typhoid fever, i, 174 
tyrosin in, iii, 816 
in uremia, iii, 830 
in whooping cough, i, 659 
Urobilinuria, iii, 810 
in jaundice, iii, 454 
Uro-erythrinuria, iii, 810 
Urogenital system, nematodes of, ii, 315 
Urostealiths, iii, 1009 
Urticaria, giant, iv, 998 
in Henoch's purpura, iv, 709 
Urticaria of mouth, iii, 53 
oedematosa, iv, 998 
purpura and, iv, 704 
rheumatica and, iv, 703 
in Raynaud's disease, iv, 990 
in syringomyelia, v, 224 
in typhoid fever, i, 101 
Uterus, diphtheria of, i, 699 
perforation of, acute diffuse peritoni- 
tis and, iii, 673 
tuberculosis of, i, 368 
V 
Vaccination, i, 822 
accidental, i, 841 
in purulent pleuritis, ii, 1048 
in smallpox, i, 843 
in tuberculosis, i, 436, 844 
Vaccine, nephritis and, iii, 849 
treatment of typhoid fever, i, 187 
virus, i, 828 
Vaccines, tuberculosis, i, 305 
Vaccinia gangrsenosa, i, 842 
generalized, i, 841 
Vagal attacks, v, 523 
Vagina, tuberculosis of, i, 368 
Vagus nerve, aneurism and, iv, 501 
arhythmia and, iv, 288 
bradycardia and, iv, 282, 284 
paralysis of, v, 517 
Stokes-Adams syndrome and, iv, 
307 
tachycardia and, iv, 276, 280, 304 
Valleix's points, v, 17, 727, 734, 737 
Valsalva, sinus of, aneurism of, iv, 492 
Valves of heart, aneurism of, iv, 482 
combined lesions of, iv, 259 
rupture of, aortic insufficiency 
and, iv, 219, 227 
Valvula foraminis ovalis, iv, 326 
Valvular lesions, hypertrophy of heart 
from, iv, 178 
myocarditis and, iv, 128 
in septic endocarditis, iv, 163 
pneumothorax, ii, 1083 
Varicella, i, 849 
Varices in stomach, hematemesis from, iii, 
283 
Varicose aneurism, iv, 510 
groin glands, ii, 308 
Variola, i, 783 
myocarditis and, iv, 108 
Varioloid, i, 809 
Vascular changes in disseminated sclerosis, 
v, 153 
crises, iv, 460 
goitre, iv, 839 
lesions in lobar pneumonia, i, 257 
stasis, iv, 20 
Vasomotor and trophic disorders, iv, 975 
Vegetable poisoning, ii, 468 
Vegetative aortitis, acute, iv, 456 
endocarditis, iv, 151 
Veins in aortic insufficiency, iv, 224 
portal, in splenic anemia, iv, 955 1092 
GENERAL INDEX 
Veins in typhoid fever, i, 128 
Vena cava, inferior, thrombosis of, iv, 541 
left superior, persistent, iv, 439 
right superior, absence of, iv, 440 
superior, aneurism and, iv, 504 
thrombosis of, iv, 541 
Venal distomatosis, ii, 239 
Venous embolism, iv, 552 
stasis, congenital cyanosis and, iv, 334 
hematemesis from, iii, 283 
in mitral insufficiency, iv, 236 
in pericarditis, iv, 21, 48 
thrombosis, iv, 537 
peripheral, iv, 539 
Ventricles, fibrillation of, iv, 106 
left, hypertrophy of, iv, 185 
right, hypertrophy of, iv, 185 
standstill of, iv, 89 
Ventricular asystole, iv, 89 
Verrucose endocarditis, iv, 150 
Vertigo, v, 30, 511, 515 
Vesicular emphysema of lungs, ii, 982 
eruption in syphilis, ii, 165 
Vestibular nerve, diseases of, v, 511 
Vibration sense, v, 18 
Villous arthritis, v, 929 
Virulence, bacterial, i, 37 
Virus, filterable, i, 19 
in carlet fever, i, 860 
ultramicroscopic, i, 19 
Visceral lesions in syphilis, ii, 169, 190 
Visceroptosis, iii, 747 
Vision, v, 25, 480 
Visual aphasia, v, 298 
Vitamine, ii, 647 
in scurvy, ii, 799, 805 
Vitiligo in exophthalmic goitre, iv, 866 
Vocal cords, paralysis of, in tuberculosis 
of bronchial glands, iv, 743 
fremitus in lobar pneumonia, i, 246 
in tuberculosis, i, 407 
Voit's theory of protein metabolism, ii, 592 
Volvulus, iii, 412 
of omentum, iii, 418 
Vomiting, juvenile, iii, 145 
nervous, iii, 142 
periodical, iii, 144 
reflex, iii, 145 
W 
Wandering rash of tongue, iii, 63 
spleen, iv, 938 
Wassermann reaction in cerebrospinal 
syphilis, v, 826, 827 
in general paresis, v, 851 
in syphilis, ii, 191 
in tabes dorsalis, v, 847 
Watsonius watsoni, ii, 239 
Waxy casts, iii, 799 
Weber's syndrome, v, 369 
test, v, 24 
Weil's disease, i, 1014 
Weir Mitchell's disease, iv, 1025 
Werdnig-Hoffmann type of spinal muscu- 
lar atrophy, v, 87 
Wernicke's aphasia, v, 299 
disease, v, 365 
zone, v, 300 
Wet brain, li, 415 
Whipworm infection, ii, 295 
"White line" in Addison's disease, iv, 792 
Whooping cough, i, 656 
apoplexy and, v, 417 
diphtheria and, i, 659 
scarlet fever and, i, 659 
tuberculosis and, i, 321 
Widal reaction, i, 162 
Wood alcohol poisoning, ii, 298 
Wool-sorter's disease, i, 639 
Word amnesia, v, 76 
blindness, v, 54, 76, 298 
deafness, v, 54, 75, 297 
Wounds of heart, insufficiency and, iv, 191 
Wright's stain, ii, 91 
Wrist-drop, ii, 366 
Writer's cramp, v, 744 
Writing, anomalies of, v, 74 
Wryneck, v, 526 
X 
Xanthelasma in jaundice, iii, 452 
Xanthin calculus, iii, 1009 
Xanthoma in diabetes mellitus, ii, 705 
Xerostomia, iii, 68 
X-rays, ii, 335 
alopecia from, ii, 336 
atrophy of skin from, ii, 336 
burns, ii, 336 
cancer from, ii, 336 
in carcinoma of stomach, iii, 215 
in congenital diseases of heart, iv, 444 
dermatitis from, ii, 335 
in diagnosis of acromegaly, iv, 814 
of cholelithiasis, iii, 585 
in enlargement of thymus, iv, 924 
eye lesions from, ii, 336 
gangrene from, ii, 336 
in gastric ulcer, iii, 181 
in lobar pneumonia, i, 247 
nephritis and, iii, 853 
in spondylitis deformans, v, 927 
sterility and, ii, 337 
swelling of muscles from, ii, 336 
Y 
Yellow fever, i, 936 
nephritis and, iii, 849 
Z 
Zinc poisoning, ii, 386 
Zooparasitic diseases, ii, 217