Our Present Knowledge re- 
garding Muscular Atrophies 
and Hypertrophies. 
BY 
LANDON CARTER GRAY, M.D., 
PROFESSOR OF NERVOUS AND MENTAL DI8EA8B IN THE 
NEW YORK POLYCLINIC. 
REPRINTED FROM 
®I)e Neto $XeIiicaI journal 
for May 19, 1888. Reprinted from the New York Medical Journal 
for May 19, 188S. 
OUR PRESENT KNOWLEDGE REGARDING 
MUSCULAR ATROPHIES AND HYPERTROPHIES. 
By LANDON CARTER GRAY, M. D., 
PROFESSOR OF NERVOUS AND MENTAL DISEASE IN THE NEW YORK POLYCLINIC. 
The muscles, the motor nerves, and the anterior column 
of gray matter, whence the motor nerves originate, consti- 
tute, anatomically and pathologically, a system apart, which 
may be termed the neuro-muscular apparatus. Disease of 
any one of the three component parts of this neuro-muscu- 
lar apparatus-muscles, motor nerves, anterior column of 
gray matter-is so apt to extend into the two others, or be 
associated with them, that the close anatomical connection 
between the three is evidently the main material reason for 
as intimate a pathological relationship. It is, therefore, 
necessary to have a clear conception of the anatomy of these 
structures. 
The anterior column of gray matter extends throughout 
the spinal cord and the upper enlarged intracranial end of 
the spinal cord, which is known as the oblong cord or me- 
dulla (medulla oblongata), and into the third ventricle. It 
is placed in different positions in these portions of the nerv- 
ous system. In the spinal cord it is in the center, in the 
shape of so-called "horns," or "cornua." In the intra- 
cranial portion of the spinal cord-the medulla oblongata, 2 
OUR PRESENT KNOWLEDGE REGARDING 
the floor of the third ventricle, and the aqueduct of Sylvius 
-it is diffused into separate masses, so-called " nuclei." 
Fig. 1.-CC. the corpus callosum ; P, the pons Varolii; 1, the infundibulum, or funnel-shaped prolongation of 
the third ventricle ; 3, the third ventricle ; 4, the fourth ventricle, the floor of which is formed by the upper 
surface of the medulla oblongata ; CE, the cerebellum. From the fourth to the third ventricle is seen a 
channel, which is the aqueduct of Sylvius. The three diverging lines MO point to the surface whence arise 
the fibers of the motor oculi, the anterior line indicating the origin of the fibers to the iris, and the tensor 
of the chorioid; and in the floor of the third ventricle, the aqueduct of Sylvius, the fourth ventricle, and 
below, are the nuclei of the other cranial nerves. 
This anterior column of gray matter extends, therefore, 
from the cauda equina up into the anterior and lowermost MUSCULAR ATROPHIES AND HYPERTROPHIES. 
3 
portion of the third ventricle. It is always, without a 
known fact to the contrary, motor in its function, and gives 
origin only to the motor nerves. At the present day our 
knowledge is very exact concerning the levels in which the 
different motor nerves thus have their origin, commencing 
above with the third pair, or motor oculi, and passing down 
to the nerves of the lower extremities. Fig. 1 will show the 
intracranial portion of this gray matter, and should be care- 
fully studied. It represents a longitudinal section through 
the center of the intracranial mass, showing the ventricular 
cavities and their relations. The nerve cells within this an- 
terior column of gray matter are known as the ganglion cells. 
They are of considerable size and endowed with a distinct 
nucleus and nucleolus and with a number of protoplasmic 
prolongations running off from the body of the cell, known 
as processes. These processes arc of two kinds. One branches 
and rebranches as it passes away from the cellular body, 
growing smaller as it goes, and is called the " protoplasmic 
process." The other does not branch as it passes away 
from the cell, but grows slightly larger, and has been seen 
by a great number of competent observers to take on a 
covering of myelin and pass to the periphery ; hence, it is 
extremely probable that it represents the axis-cylinder of a 
peripheral nerve, and it is called the " axis-cylinder pro- 
cess." The termination in the muscles of the nerve-fibers 
thus originating has been carefully investigated by many of 
the best-known histologists of the day ; most recently by 
Gessler in the green Italian lizard and the guinea-pig' 
Gessler states that the motor end-plate is essentially the 
same in the Mammalia and reptiles. This motor end-plate 
consists of a fine nerve-plexus, between or under which-for 
authors differ on this very immaterial point-lies a finely 
granulated mass, containing two varieties of nuclei-the one 
kind, the smaller, being granulated and of irregular shape; 4 
OUR PRESENT KNOWLEDGE REGARDING 
the other kind being larger, oval in shape, with double con- 
tours and a nucleolus. The former are known as the Schei- 
den- or Gedstkerne, or, as Ranvier calls them, the noyaux 
vaginaux or de Varboration, while the latter are the Grund- 
or Sohlenkerne, or noyaux fondamentaux of Ranvier. To 
this motor end-plate, lying upon the muscle, come the me- 
dullated nerve-fibers at a right angle, losing their medullary 
sheath as they pass into the nerve-plexus just described. 
There are reasons for supposing that these ganglion 
cells in the anterior column of gray matter are of two kinds 
in regard to their function; for these anterior columns are 
subject, on the one hand, to disease in which motor paraly- 
sis is the first and predominant symptom and, on the other 
hand, to other disease in which wasting of muscular fiber is 
the first and predominant symptom. It would, therefore, 
seem as if in the first case there were a motor cell, whose 
disease gave rise to paralysis, while in the latter case it 
seems impossible to deny that it is disease of a nutritive or 
trophic cell which has produced the primary wasting of 
muscular fiber. To be sure, in every case of disease of 
the cells of the anterior gray column there is sooner or 
later certain to be both motor paralysis and muscular 
atrophy, without regard to which came first; and the two 
kinds of cells are thus always affected, although not simul- 
taneously. 
Disease in any one of the three component parts of this 
neuro-muscular apparatus - ganglion cells, motor nerves, 
muscles-will produce a motor paralysis and atrophy. 
This should be very easily understood. If the ganglion cells 
are diseased, the motor nerve will degenerate and the mus- 
cle will be paralyzed and will atrophy. If the motor nerve 
is diseased, the portion of nerve below the site of disease 
will also degenerate and the muscle will be paralyzed and 
will atrophy as before. If the muscle itself is diseased, it MUSCULAR ATROPHIES AND HYPERTROPHIES. 
5 
may be paralyzed, of course, and may atrophy. The three 
symptoms, therefore, of disease of the neuro-muscular ap- 
paratus are: 1, motor paralysis; 2, atrophy of muscles; 3, 
electrical changes brought about by degeneration of nerve 
and muscle. 
We can now readily understand why there has been 
such dispute, when these three symptoms were present, as 
to which one was diseased of the three component parts of 
the neuro-muscular apparatus-ganglion cells, motor nerves, 
or muscles. The real objective point is the muscle. It is 
very dependent on the 'nervous system, and the nervous 
system is very dependent on it. Neither can do with- 
out the other. The muscle can not contract intelligently 
except by means of the nervous system. The nervous 
system can not express itself except by means of certain 
muscles, and can not exist except by means of certain 
others. The brain, sending down its mandates to the 
cells in the anterior column of gray matter, and thence 
through the motor nerves, can only communicate its vari- 
ous thoughts and emotions to the outside world through 
the muscles, and can only act through them; and the ner- 
vous centers that, in their mysterious way, supply nervous 
force to the muscles of the heart, respiratory structures, and 
arteries, can not maintain life without muscular action, as 
of the vascular apparatus. On the other hand, of what use 
would a leg or a heart be unless each w'as in intimate con- 
nection with the nervous system ? So, after all, it is the 
muscle that is to be studied in disease of this neuro-muscu- 
lar apparatus. Muscular-i. e., motor-paralysis and muscu- 
lar atrophy, then, with or without electrical changes, are the 
signs of disease in the neuro-muscular apparatus, i. e., of 
disease either in the anterior gray column, in the motor 
nerves, or in the muscles. 
A priori it is difficult to understand why disease attack- 6 
OUR PRESENT KNOWLEDGE REGARDING 
ing one portion of this anterior gray column, say that of the 
lumbar enlargement, should not travel up throughout the 
whole column, implicating all the centers of origin of the 
muscles of the trunk, neck, heart, respiratory structures, 
face, and head ; or why disease should not always extend 
inward through the muscles as well as outward to them. 
Laws of disease, however, as yet unknown to us are at play 
here, as in other structures of the body; and we can only 
study the workings of these laws without attempting an ex- 
planation of them. We can certainly say that, during the 
first three years of life, the anterior column of gray matter 
in the dorsal cord is specially prone to one-sided disease. 
We can certainly say that that portion of the anterior gray 
column containing the nuclei of the facial, hypoglossal, and 
spinal accessory nerves is specially prone to disease that 
does not extend upward or downward. We can certainly 
say the same thing of the nuclei of the different nerves of 
the eyeball, the third, fourth, and sixth. We can certainly 
say that in certain individuals the muscles alone are dis- 
eased. But certain as we are in saying all these things, we 
can not attempt to explain why it should be so. It must, 
moreover, be remembered that these different results were 
seen before the pathology was known; so that the nomen- 
clature, being representative of the purely clinical or locally 
pathological standpoint, is by no means consistent. 
The diseases of the neuro-muscular apparatus are known 
as- 
1. Myelitis of the anterior horns, or cornua. 
2. Glosso-labio-laryngeal paralysis, or bulbar * paralysis. 
3. Progressive ophthalmoplegia. 
4. Muscular pseudo-hypertrophy. 
5. Progressive muscular atrophy. 
* An adjective bestowed upon the medulla oblongata because of its 
bulbous shape. MUSCULAR ATROPHIES AND HYPERTROPHIES. 
7 
Of these, the first three-myelitis of the anterior horn, 
bulbar paralysis, and ophthalmoplegia-are due to lesions 
in the anterior gray column; the fourth, pseudo-hyper- 
trophy, is indubitably of muscular origin ; while the fifth, 
progressive muscular atrophy, is sometimes due to lesions 
in the anterior gray matter, sometimes to muscular lesion, 
sometimes to both neural and muscular lesions. Each of 
the three due to central lesion has an individual localization 
in the anterior gray matter. The first, myelitis of the an- 
terior horn, arises from implication of the anterior gray 
matter of the spinal cord ; the second, glosso-labio-laryngeal 
paralysis, arises from implication of the nuclei in the me- 
dulla oblongata of the hypoglossal, facial, and spinal ac- 
cessory nerves; while the third, progressive ophthalmo- 
plegia, is due to affection of the nuclei of the ocular 
nerves in the floor of the aqueduct of Sylvius and the third 
ventricle. 
Myelitis * of the anterior horns, or cornua, is a 
motor paralysis affecting muscles of the extremities that 
are associated in function, and followed by atrophy and 
electrical alterations in the paralyzed muscles. Although 
it may occur at any time of life, cases having been ob- 
served at the extremes of three months and sixty-seven 
years, there are yet two periods of life which seem espe- 
cially predisposed to it-viz., the three first years of in- 
fancy and the period between the eighteenth and fortieth 
years. It may be acute, subacute, or chronic. In all cases 
the paralysis is the first symptom, and is followed by atro- 
phy in the course of a few weeks in the acute cases, or a 
longer time in the chronic ones. It is usually confined to 
one extremity in children, generally to a lower limb, while 
in adults paraplegia is the most usual form of paralysis. 
* Synonyms are anterior polio-myelitis, essential paralysis of chil- 
dren, infantile spinal paralysis, atrophic paralysis of children. 8 
OUR PRESENT KNOWLEDGE REGARDING 
Glosso-labio-laryngeal paralysis * is a motor paraly- 
sis with muscular atrophy, affecting the motor cranial nerves 
and their muscles, especially the hypoglossal, certain filaments 
of the facial, and certain filaments of the spinal accessory. 
Usually the atrophy is the first symptom that is noticed, 
although some authorities contend that the paralysis may 
be primary. The atrophy first shows itself in the muscles 
Fig. 2. 
of the tongue, then in the muscles of the lips and the lower 
face (seldom in the eyelids), and, finally, in the muscles of 
the larynx, pharynx, oesophagus, and heart. The lingual 
difficulty is evidenced, as has been said, by the atrophy of 
the tongue, giving the tongue a fissured and crenulated ap- 
pearance, as if its substance had been chipped out in places 
and trimmed on the edges, as is seen in Fig. 2, in which 
the thinning of the organ causes it to curl over and have a 
fallacious increase in size. Furthermore, the increasing 
paralysis causes difficulty in the pronunciation of the letters 
* The synonym is bulbar paralysis. MUSCULAR ATROPHIES AND [HYPERTROPHIES. 
9 
which are mainly pronounced by the tongue, and some 
difficulty in deglutition. The affection of the lower face 
and lips is denoted by a wasting of their muscles, by diffi- 
culty in the pronunciation of the lip-sounds, and by altera- 
tion in the expression of the face. 
Progressive ophthalmoplegia is manifested by a pa- 
ralysis of the muscles of the eye, which are of two varieties 
-the exterior and the interior. The interior muscles are the 
sphincter of the pupil and the tensor of the chorioid, while 
the exterior muscles are the six well-known ones attached 
to the globe. The third pair, the motor oculi, supplies these 
interior muscles and four of the six external ones, as well 
as the levator palpebrse superioris. The four thus sup- 
plied are the internal rectus, turning the eye inward; the 
superior rectus and the inferior oblique, the two elevators 
of the eye; and the inferior rectus, one of the two depres- 
sors of the eye. Of the two others of these six exterior 
muscles, one, the superior oblique, is supplied by the fourth 
or trochlear nerve, while the other, the external rectus, is 
supplied by the sixth nerve, the abducens. It is the nuclei 
of these different ocular nerves which are affected in the 
so-called ophthalmoplegia, and the symptoms are thus the 
symptoms of paralysis of these exterior and interior mus- 
cles, together with the levator palpcbrse superioris. As 
might be expected by reasoning beforehand, the paralysis 
may sometimes affect the exterior muscles, sometimes the 
interior muscles, and sometimes partially both. 
Muscular pseudo-hypertrophy is a disease of early 
childhood, characterized by a deposit of fat around the mus- 
cular fiber, more especially in the muscles of the lower extremi- 
ties, which come to have a hypertrophied appearance. The 
upper extremities are usually affected simultaneously with 
an atrophy, and the contrast between the seemingly sturdy 
legs and the wasted arms and shoulders is not likely to be 10 
OUR PRESENT KNOWLEDGE REGARDING 
forgotten when once seen. Attention is at first directed to 
the disease by the stumblings and falls and increasing weak- 
ness, which seem the more inexplicable because of the simu- 
lated robustness of the members. 
Progressive muscular atrophy is a disease about 
which there has been much dispute and great confusion. 
The early writers were strongly of the opinion that it was al- 
ways due to disease of the anterior gray column ; Friedreich 
wrote a great tome upon the subject in 1873 to prove that 
it was always of muscular origin; and in the last few years 
facts have accumulated to prove that it has sometimes a 
muscular and sometimes a central origin. The great strife 
waged in regard to the pathology led to an attempt to throw 
light upon this subject by the study of so-called " types," 
or, in other words, to say that, when certain groups of muscles 
were affected, the disease must be of central origin, and that, 
when certain other muscular groups were affected, the dis- 
ease must be of peripheral origin. This type-making busi- 
ness has been carried to such ridiculous extremes that there 
are fully a dozen different groups which the pretended dis- 
coverers would have us know by their own names; never- 
theless, we are warranted, both from a clinical and from a 
pathological standpoint, in accepting four types : 
1. The hand type. 
2. The juvenile type (Erb). 
3. The infantile facial type (Landouzy and Dejerine). 
4. The peroneal type (Charcot, Marie, Tooth). 
1. The Hand Type.-In this the onset is generally in the 
right hand, occasionally in both hands. The short muscles 
of the thumb and the ball of the little finger are generally 
first affected-the so-called thenar and hypothenar emi- 
nences. It is usual for the atrophy to begin in the abductor 
pollicis brevis, and then be observable in the opponens and 
adductor. The complete atrophy of these thumb muscles MUSCULAR ATROPHIES AND HYPERTROPHIES. 
11 
tb 
£ 
produces a condition to which the name has been given of 
"ape-hand" (Affenhand). Fig. 3 shows this beautifully, 
besides the droop of the wrist due to ulnar paralysis. 12 
OUR PRESENT KNOWLEDGE REGARDING 
At about the same time, sometimes a little sooner, sometimes 
a little later, the interossei are implicated, as is to be seen by 
a sinking of the spaces on the back of the hand; when this 
atrophy has attained to a certain grade, it produces, together 
with a contraction of the antagonist muscles, the so-called 
" claw-hand " (main-en-griffe, Klauenhand}. 
Consecutively to the hand muscles, often after a long 
time, sometimes even after years, the forearm or upper-arm 
muscles are usually attacked. In the forearm it is apt to 
be the abductor and extensor pollicis longus, the supinators, 
and the flexors. In the upper arm the deltoid is almost in- 
variably the first to waste, then the biceps, and, finally, the 
triceps last of all. But, sooner or later, the muscles of the 
trunk may be implicated, especially the trapezius, the pec- 
torals, the rhomboids, and the latissimus dorsi. Atrophy is 
infrequent of the cervical or respiratory muscles, or of the 
diaphragm. After many years of duration the nuclei in 
the medulla oblongata may be affected, and then may en- 
sue atrophy of the tongue and difficulty of deglutition, 
and death may result from inanition or respiratory dis- 
turbance. 
2. The Juvenile Type (Erb).-This form was first de- 
scribed by Erb, in 1884, and differs essentially from the 
hand type in the location of its onset, which is almost 
always in the muscles of the shoulder and upper arm, far 
less often in the muscles of the pelvis and lower extremi- 
ties, while, as has just been explained, the hand type 
primarily affects the hand muscles; and only secondarily, 
often after a long time, are the shoulder and arm muscles 
involved. The shoulder and arm muscles often atrophied 
are the pectorals, trapezius, rhomboids, serrati, latissimus 
dorsi, longissimus, sacro-lumbalis, forearm flexors, supinator 
longus, and triceps; and, in the lower extremities, the 
glutsei, quadriceps, peronei, and tibialis anticus. MUSCULAR ATROPHIES AND HYPERTROPHIES. 
13 
3. The Infantile Facial Type (Landouzy and Dejerine). 
-It generally begins with an atrophy of the muscles of 
expression, which is thus described by Landouzy and De- 
jerine : " The face is in repose, the lips protruding, the brow 
like ivory, a seeming protrusion of the eyes; in efforts at 
mimicry the smile is sad (tire en traversf the movements 
of the lips are incomplete, and the immobility of the lips is 
in marked contrast to the animation of the eyes." When 
the face is wasted, it is the rule that the muscles of the 
shoulder and arms are involved next; but certain muscles 
usually remain intact-such as the supra-spinalis, the infra- 
spinalis, the infra-scapularis, and the flexors of the hand and 
fingers. 
4. The Peroneal Type (Charcot, Marie, Tooth).-The 
French writers described this form in 1886, while Dr. 
Tooth states that he also called attention to it at the same 
time in his graduation thesis. As is indicated by the 
name I have given it, it first shows itself in the muscles of 
the leg, invading the hand, and then the forearm several 
years afterward. 
The accompanying table shows, side by side, the loca- 
tion of the atrophy in each of these four types: 14 
OUR PRESENT KNOWLEDGE REGARDING 
Hand type. 
Juvenile type. 
Inf. facial type. 
Peroneal type. 
Muscles of the 
Muscles of the 
Facial muscles 
Muscles of the 
thumb and fin- 
shoulder and 
of expression. 
leg, then the 
gers: 
upper arm: 
hand and 
Abductor pollicis 
Pectorals, 
Trapezius, 
In the shoulder 
forearm. 
brevis, 
Opponens, 
and arm mus- 
Rhomboids, 
cles these are 
Adductor, 
Serrati, 
intact: 
Interossei, 
Latissimus, 
Longissimus, 
Supraspinalis, 
Lumbricales. 
Infraspinalis, 
Ape-hand. 
Sacro-lumbalis, 
Flexors of the 
Claw-hand. 
Flexors of the 
hand and fin- 
forearm, 
gers. 
Arm muscles: 
Supinator longus, 
Abductor and ex- 
Triceps. 
tensor longus 
pollicis, 
Muscles of the 
Supinators, 
lower extrem- 
Flexors, 
ity: 
Deltoid, 
Biceps, 
Glutaei, 
Quadriceps, 
Triceps. 
Peronei, 
Tibialis anticus. 
The course of progressive muscular atrophy is gradual, 
and the duration is from five to thirty years. The chronic 
and fibrillary atrophy may not be detected for some time, 
until enough of the muscle has been affected to alter the 
contour and consistence, which latter is then found upon 
palpation to be softer and less resilient than in the healthy 
muscle. The paralysis is usually proportionate to the 
atrophy. It not infrequently happens that the loss of mus- 
cular strength first attracts the attention of the patient, and 
an examination then brings the atrophy to view. So-called 
" fibrillary contractions " are frequently present in all the 
types, and consist of minute movements of the muscular 
fibers, varying in extent from an occasional whipcord-like 
movement under the skin, likened by the patient to a pulse- 
beat, to a wide set of fibrillary movements that may set a 
whole group of muscles in tremulous movement. In many MUSCULAR ATROPHIES AND HYPERTROPHIES. 
15 
cases of progressive muscular atrophy the muscular atrophy 
is curiously conjoined with real or pseudo-hypertrophy-i. e., 
an actual increase in the size of the muscular fiber, or a 
substitution of fat in the place of the muscular fiber or 
around it. This is well seen in Figs. 4 and 5, which are of 
a patient who has been in my hospital wards for some eight 
years, and whose case began with atrophy in the thumb 
muscles, as is shown in Fig. 3. Robust as this man seems, 
he can not raise himself from a sitting posture without great 
aid, and, when erect, can be literally pushed over by a light 
touch with the finger. Especially noteworthy are the con- 
trasts between the size of his shoulders and his upper arms 
and between his forearm and his upper arm ; the great ridge 
in his dorsal and lumbar spine from curvature of the verte- 
bral column anteriorly to compensate for the atrophy and 
paralysis of the anterior abdominal muscles; the pseudo- 
hypertrophic outgrowth on the left calf; and the many 
alternating atrophies and hypertrophies. 
The electrical changes in these different diseases of the 
neuro-muscular apparatus are often very considerable. In 
order to understand them it is necessary to have some com- 
prehension of the mode of reaction of a healthy nerve and 
muscle to the galvanic and faradaic current. For diagnostic 
purposes two currents, the galvanic and the faradaic, are 
used. When the faradaic current is applied to a nerve 
leading to a muscle, or to the muscle itself, a contraction 
of the muscle results, dependent upon the strength of the 
current used, very little difference being observed between 
the effect of the positive and that of the negative pole. 
When, however, a galvanic current is applied to a motor 
nerve leading to a muscle, or to the muscle itself, the re- 
sulting contraction of the muscle will be very different ac- 
cordingly as the positive or negative pole is employed. If 
we search for the weakest galvanic current that will cause a Fig. 4. Fig. 5. 18 
OUR PRESENT KNOWLEDGE REGARDING 
muscular contraction, we shall obtain it first with the nega- 
tive pole, before we can obtain any contraction whatsoever 
with the positive. Nor is this all. This contraction thus 
obtained with the negative pole is only seen when we close 
the circuit, and is not at all present when we open the cir- 
cuit. Let the galvanic current now be made stronger, and 
we shall obtain an intensification of the contraction thus 
produced with the negative pole at the closing; and after a 
while we shall obtain a contraction with the positive pole, 
sometimes first at the closing of the circuit, sometimes at 
the opening of the circuit. Then, making the galvanic cur- 
rent still stronger, we shall obtain an intensification of what 
we have already obtained-viz., a contraction with the nega- 
tive pole at the closing, and with the positive both at the 
closing and the opening. It will be noticed that so far we 
have obtained no contraction at all with the negative pole 
at the opening of the circuit. It is very important to re- 
member that this will not be obtained in a healthy nerve 
or muscle until the current becomes so strong as to be 
painful. 
From this it will be observed that the healthy muscle 
reacts first to the negative pole at the closing, and then to the 
positive pole either at the closing or the opening, and final- 
ly that the negative pole at the opening does not produce a 
contraction unless the current is so strong as to be painful. 
Suppose, then, that the negative pole at the opening gives 
us a muscular contraction in a certain case with the same 
strength of current that is sufficient for a contraction with 
the negative pole at the closing, is it not evident that the 
reaction of the healthy nerve and muscle is entirely altered ? 
Or suppose that exactly the same strength of current gives 
us equally strong contractions with the negative closing and 
the positive opening and closing, is it not again evident that 
there is some alteration ? In other words, any marked de- MUSCULAR ATROPHIES AND HYPERTROPHIES. 
19 
parture from this healthy formula of nerve and muscle is 
evidence of disease. At the same time that we get these 
changes with the galvanic current we should also have oth- 
ers with the faradaic, consisting simply of decreasing reac- 
tion to the current, so that larger and larger quantities would 
be required to produce a muscular contraction. But there 
are other electrical symptoms in these cases, which any one 
can understand who will take the trouble to study them in 
any case of neuritis in the human being, or in an artificial 
neuritis in the frog, rabbit, or guinea-pig that can be pro- 
duced by section of the sciatic nerve. It will then be found 
that during the first ten days or two weeks there will be, in 
the motor nerve and in the attached muscle, decreasing re- 
action to both the galvanic and the faradaic currents. 
About the third week the nerve and muscle will begin to 
behave differently to each of the two currents. To the 
faradaic the nerve and muscle will display a constantly de- 
creasing reaction, so that larger and larger quantities of elec- 
tricity will be required, until it may happen that no contrac- 
tion whatsoever can be produced. But, singular to say, at 
this period-i. e., about the third week-the nerve begins 
to show an increasing reaction to galvanism, so that lesser 
and lesser quantities are required to produce a muscular con- 
traction. At the same time a reversal in the healthy polar 
formula, such as has been above described, is apt to ensue, 
so that the negative opening may equal the positive closure, 
or the positive opening and closure may equal a negative 
closure. In the course of time this increased reaction to 
galvanism and the reversal of the polar formula may pass 
away, and the nerve and muscle may again decrease in its 
reaction to this current. During this whole period of altered 
electrical reactions the muscular contraction is more slug- 
gish than in the healthy muscle. All these alterations in 
the reaction of the healthy nerve and muscle, either togeth- 20 
OUR PRESENT KNOWLEDGE REGARDING 
er or separately, are known as the reaction of degeneration. 
They consist, as will have been seen, of- 
1. Decreased reaction to faradaism and galvanism. 
2. Increased reaction to galvanism. 
3. Reversal of the healthy polar formula. 
4. Sluggish muscular contraction. 
These should be studied with the same care and patience 
that are given to auscultation and percussion. 
The pathology of these different diseases of the neuro- 
muscular apparatus is very well understood at the present 
day. In three of the five diseases which have been enumer- 
ated-myelitis of the anterior horn, glosso-labio-laryngeal 
paralysis, and progressive ophthalmoplegia-the lesion con- 
sists of death or injury of the ganglion cells in the anterior 
gray horns of the spinal cord or in the motor nuclei of the 
medulla oblongata, the aqueduct of Sylvius, and the third 
ventricle. The changes in these ganglion cells vary somewhat 
according to the extent and kind of the disease. In some 
cases there is not a vestige left of the cells. In others they 
are more or less disintegrated, some processes being lost, 
others being altered in shape and size, while the nuclei or 
nucleoli may be destroyed, although these latter almost in- 
variably persist when any vestige of the cell is left. In 
myelitis of the anterior horns and in glosso-labio-laryngeal 
paralysis the affection is generally confined to the cells, or 
other changes are evidently consecutive to these, such as in- 
crease in the size of the vessels, increased growth of the 
connective tissue, and shrinking of the cord or medulla ob- 
longata on the affected side. But in progressive ophthal- 
moplegia the affection of the ganglion cells seems to be only 
part of a somewhat diffused myelitic process. When the 
ganglion cells are affected, the motor nerves and the at- 
tached muscles will be found to have undergone serious 
alterations. The nerves are apt to be thinner, grayish look- MUSCULAR ATROPHIES AND HYPERTROPHIES. 
21 
ing, and more transparent, their axis-cylinder and medullary 
sheaths will be found more or less disintegrated, and the 
connective-tissue sheath thickened and its nuclei 
in size and number. The muscles usually undergo an 
atrophy, which may be of t,vo kinds: the so-called simple 
atrophy, in which the muscular fiber is simply decreased in 
size but its transverse striae are preserved, while in the de- 
generative atrophy the transverse striae are lost. But there 
may also be hypertrophy or pseudo-hypertrophy of muscle, 
the first consisting of actual hypertrophy of the muscle fiber, 
the latter being a conversion of the muscle fiber into fat, as 
well as a surrounding deposit of fat. In the fourth of the 
five diseases which have been enumerated, muscular pseudo- 
hypertrophy, the disease is purely of muscular origin, cases 
having been collected by Middleton and Schultze in demon- 
stration of this fact. In progressive muscular atrophy there 
can be no doubt but that some cases are of central origin, 
others of muscular origin, while it would seem, from the 
history of some few cases, as if a third class were both of 
central and of peripheral origin. I have been able to col- 
lect thirteen cases in which the disease was central, as was 
proved post mortem, while there have been but two autop- 
sies of cases of purely muscular origin. 
The diagnosis of these various affections of the neuro- 
muscular apparatus is made, in the vast majority of cases, 
with ease and certainty. In every instance the symptoms 
are purely motor; sensory, cerebral, vesical, and rectal 
symptoms being either temporary in duration or entirely 
absent. Of course, if the anterior gray column should be 
implicated in diffused disease extending into it from other 
parts of the nervous system, the diagnosis may become a 
very complicated one; but this may be met by bearing 
in mind the usual complications. These purely motor 
symptoms consist, as has been said, of motor paralysis, 22 
OUR PRESENT KNOWLEDGE REGARDING 
muscular atrophy or hypertrophy, and altered electrical 
reactions. 
Whenever these three symptoms are present, disease of the 
neuro-muscular apparatus may be positively diagnosticated. 
It then becomes a question as to whether the lesion is in the 
anterior gray column, in the motor nerve, or in the muscles. 
The symptoms upon which lesion of the anterior gray 
column may be affirmed are a sudden onset of paralysis, fol- 
lowed in the course of a few weeks by atrophy and reaction 
of degeneration ; or the group of symptoms described under 
the head of glosso-labio-laryngeal paralysis; or the gradual 
paralysis of the different interior and exterior muscles of the 
eyeball, described under the heading of progressive oph- 
thalmoplegia. 
The symptoms upon which a diagnosis may be positively 
made of disease of the muscles alone are the group of 
phenomena which have been described under the heading of 
muscular pseudo-hypertrophy, and the infantile facial type 
of progressive muscular atrophy described by Landouzy and 
Dejerine. 
Undoubtedly there are many other symptoms that have 
been vaunted by different authors as of importance in the 
differential diagnosis, but these are, in my opinion, the only 
ones upon which we can place absolute reliance. For in- 
stance, it has been contended that the form of progressive 
muscular atrophy which I have described above as the 
" hand type " always points to a spinal origin, yet cases of 
purely muscular disease have begun in the hands. And 
Erb would maintain a purely muscular origin for his cases 
of the so-called juvenile type, but, as Schultze very clearly 
points out, Erb's cases are of several kinds, some being evi- 
dently pseudo-hypertrophies, others belonging to the Lan- 
douzy and Dejerine type, etc., while not a single autopsy 
has been made in this juvenile form of Erb. MUSCULAR ATROPHIES AND HYPERTROPHIES. 
23 
The altered electrical reactions of the so-called reaction 
of degeneration are only indicative of muscular change# 
This is indicated very clearly, it seems to me, by the fact 
that this reaction of degeneration has been found in cases 
of both peripheral and central origin, as well as by the re- 
searches of Gessler upon guinea-pigs and lizards that dem- 
onstrated the dependence of this reaction of degeneration 
upon muscular atrophy alone. Fibrillary contractions, sup- 
posed to be of so much diagnostic importance in the days 
of old, are found in cases of both central and peripheral 
origin, and are, therefore, of no importance in the differ- 
ential diagnosis. 
Causation.-The most frequent causes of the diseases of 
the neuro-muscular apparatus are infection, heredity, muscu- 
lar strain, trauma, acute diseases, exposure to continuous 
cold, and warm weather. The later German authors are dis- 
playing a tendency to ascribe the acute form of myelitis of 
the anterior horn to infection, because of the simultaneous 
manifestation of the disease in two or three children of one 
family or one locality. There is no definite proof of this, 
however, and it is possible that it may be explained by the 
fact to which Dr. Weir Mitchell called attention several 
years ago-viz., the greater frequency of myelitis of the 
anterior horn during the warmer months of the year. 
Heredity is usually found in the infantile facial type of 
Landouzy and Dejerine, and plays a large part in all the 
types. Some authors would maintain that heredity is a 
diagnostic mark of the purely muscular forms, but the scien- 
tific evidence at our command does not warrant this asser- 
tion. Friedreich points out the relation of muscular strain 
to the resulting atrophy in case after case, and it is a very 
familiar clinical fact to me. 
Progressive muscular atrophy has often been known to 
follow typhus, diphtheria, measles, acute rheumatism, par- 24 
OUR PRESENT KNOWLEDGE REGARDING 
turition, and cholera; so that these acute diseases must have 
«ome unfavorable influence upon the neuro-muscular appa- 
ratus. 
Diagnosis.-In making a diagnosis it must not be for 
gotten that other diseases of the spinal cord and its sur 
rounding membranes or osseous structures may extend into 
the anterior horns, in which case the symptoms of motor 
paralysis and muscular atrophy may be superadded to the 
original symptoms of the invading disease. Such compli- 
cating diseases may be spinal haemorrhage, transverse mye- 
litis, syringo-myelitis, or locomotor ataxia. All of these, be- 
sides the characteristic features of each of them, will have 
marked sensory, vesical, and rectal symptoms. Should a 
spinal haemorrhage take place to such a limited extent as 
only to involve one anterior horn, it might be impossible to 
make the differential diagnosis ; but I know of no such case 
on record. In the early stages of syringo-myelitis-i. e., 
formation of a gliomatous tumor and cavity within the sub- 
stance of the cord-the affection might be limited to one 
anterior horn, and thus cause confusion, but the later his- 
tory of the case will make matters plain. 
A differential diagnosis must also be made from the 
acute exanthemata; meningitis, traumatic, from ear disease, 
or cerebro-spinal; meningo-encephalitis of children ; neu- 
ritis, simple or multiple; lead palsy; joint atrophies; and 
hysteria. 
It is possible to confound the early stage of myelitis of 
the anterior horn with meningitis. But in the latter the 
mental disturbance is much more marked and of longer 
duration; the cranial nerves may be seriously implicated, 
the sensory as well as the motor, which never happens in 
myelitis of the anterior horn ; there is usually also the re- 
traction of the head characteristic of meningitis ; and the 
paralysis is almost invariably hemiplegic in distribution, MUSCULAR ATROPHIES AND HYPERTROPHIES. 
25 
while hemiplegia is extremely infrequent in myelitis of the 
anterior horn, and this hemiplegic paralysis is never suc- 
ceeded by atrophy, except from years of disease. 
The ordinary neuritis of a mixed nerve generally causes 
considerable and continuous pain, oedema, and hot and glossy 
skin ; and the chronic form is distinguishable by the dis- 
proportion between the pain and the paralysis and atrophy, 
as well as often by the fact that it is confined to one nerve- 
trunk, or even one branch of a nerve-trunk. In multiple 
neuritis, differentiation is made easy by the infectious his- 
tory, in some cases the malarious neighborhood, the con- 
tinuous pains in both lower extremities, the steady progress 
within a week or two of the bilateral paralysis and atrophy, 
at the same time that the painful symptoms persist. 
The extensor paralysis of lead palsy, conjoined with 
the lead line on the gums, colic, and history of exposures 
to lead, ought never to permit of confusion in diagnosis. 
Atrophy from joint disease is too easily recognized to 
need more than a mere mention. 
It is only necessary to call attention to the possibility 
of hysteria simulating a myelitis of the anterior horn, be- 
cause the lack of the atrophy and of the electrical alterations 
and the presence of an unimpaired tendon reflex will dem- 
onstrate that the anterior cornua are not affected, even 
though there may be some doubt as to the existence of a 
motor paralysis. 
Prognosis.-The prognosis of these different forms of 
disease of the neuro-muscular apparatus varies. In myelitis 
of the anterior horn the rule is that there is always a cer- 
tain incurable residue of paralysis. In the acute forms the 
amount of this can not be determined for several weeks, 
and not for months in the chronic forms. Glosso-labio- 
laryngeal paralysis is always fatal. Progressive ophthalmo- 
plegia is sometimes recovered from, though the majority of 26 
OUR PRESENT KNOWLEDGE REGARDING 
cases are fatal. Muscular pseudo-hypertrophy is incurable. 
Progressive muscular atrophy usually runs a chronic course, 
and ends in death after years of duration; but there is a 
doubt at the present day as to whether the purely muscular 
forms are not amenable to treatment. Schultze details a 
remarkable cure, and I have seen great improvement in 
several cases in which the muscular pain was so great as to 
seemingly warrant the diagnosis of diffused neuritis. 
Treatment.-The treatment of all the different forms of 
disease of the neuro-muscular apparatus is essentially the 
same in its general principles, except that the acute forms 
of myelitis of the anterior horn may be accompanied by 
reflex disturbances calling for special treatment-such as 
fever, coma, convulsions, etc. In the main, however, the 
therapeutic items are these: Rest, electricity, massage, and 
drugs. 
Rest is of prime importance to a muscle or nerve de- 
generation from any cause whatsoever. I have never yet 
failed to obtain some improvement by rest in any case of 
muscular atrophy if there was any muscular tissue remain- 
ing. The rest should be proportioned to the extent and 
acuteness of the disease. In wide-spread or acute atrophy 
rest should be absolute in bed for weeks, or a month. In 
more localized disease the rest need not be so radical. In 
every case, however, and at every stage, it should be borne 
in mind that fatigue is to be avoided. 
The electrical treatment should be by means of galvanic, 
faradaic, and static currents, and should be addressed to the 
spinal cord, the motor nerves, and the muscles themselves. 
Galvanism of the spine should be by means of large elec- 
trodes, one being placed over the upper cervical region and 
the other in the lower dorsal, and the duration of the appli- 
cation should be from five to ten minutes, and the current 
should vary from five to thirty milliamperes. It will be MUSCULAR ATROPHIES AND HYPERTROPHIES. 
27 
observed that I am no believer in the short applications and 
feeble currents that are so much vaunted by the German 
authorities. Patients become used to electricity as they do 
to any drug, with this difference-that the larger doses of 
electricity, unlike the larger doses of many drugs, will cause 
no ill effects. But the idiosyncrasy of each patient will 
vary in regard to the quantity of electricity that can be 
taken, and this must be ascertained. Usually, however, the 
strength will vary between the figures I have mentioned. 
Galvanism and faradaism should be applied to the affected 
nerves and muscles at what are known as the " motor 
points," a description of which can be found in any text- 
book of electricity. The motor points will indicate the 
nerve-trunks and the motor filaments going into the mus- 
cles, and it is precisely at these spots that galvanic and 
faradaic currents must be applied. Here the quantity of 
galvanism should be much smaller, and should vary from 
two to ten milliamperes, care being taken not to cause any 
painful sensation. A gentle faradaic current will be quite 
sufficient, and the application should be from five to ten 
minutes. In some cases I have derived considerable benefit 
from an application of the gentle faradaic current from 
thirty to sixty minutes, flat sponge electrodes being fastened 
on to the affected muscles by means of elastic bands. Static 
electricity will be found of use in certain cases of greatly 
atrophied muscle, and, as Charcot has pointed out, contrac- 
tions can sometimes be obtained by this form of electricity 
when it is impossible to obtain them by galvanism or fara- 
daism. 
Massage can be used with great advantage in the chronic 
forms, or in the chronic or later stages of the acute forms. 
It should always be limited in duration from five to twenty 
minutes, and should be very gentle, special care being taken 
not to gripe the muscle or irritate it mechanically in any 28 
OUR PRESENT KNOWLEDGE REGARDING 
way. Massage is, however, a very uncertain agent, in spite 
of all the praise that it is nowadays the fashion to bestow 
upon it, and it should be carefully determined in each in- 
dividual case as to whether the manipulation of the muscle 
is beneficial or not. In some cases it is positively harmful, 
and in these it should be abandoned. 
Except in the acute or subacute forms of myelitis of the 
anterior horn and progressive ophthalmoplegia, drugs are 
usually of little use, and we can only employ them empiric- 
ally. Iodide of potassium, ergot (either in the form of the 
fluid extract or in that of ergotine), and strychnine may be 
used, but I am bound to say that I have never seen any 
tangible effects from them. 
Bibliography. 
Aran, "Arch. g6n. de m6d.," Sept.-October, 1850. 
Aufrecht, "Arch. f. klin. Med.," 1878. 
Baerwinkel, Schmidt's " Jahrbucher," 1861. 
Barthez et Rilliet, "Traite clin. et prat, des maladies des 
enfants," 1863. 
Bauer, Louis, " St. Louis Med. Jour.," 1870. 
Charcot, " Lemons," i, ii, 2d ed., 1877. 
Charcot, "Progres med.," 1885. 
Cornil et L6pine, "Gaz. m6d.," 1875. 
Cruveilhier, "Arch. gen. de mfed.," 1853 and 1856. 
Duchenne, "Electris. Iocalis6e," 1854, 1872. 
Duchenne, "Arch. g&n. de med.," 1861. 
Duchenne et Joffroy, "Arch, de physiol.," 1870. 
Erb, "Arch. f. klin. Med.," 1884; "Neurol. Centralblatt," 
1886. 
Erb, "Hdb. d. Krkheit. d. Nervensystems," 1878. 
Eisenlohr, "Zeitschr. f. klin. Med.," 1880. 
Friedberg, "Pathol, u. Therap. d. Muskellhmg.," Weimar, 
1858. 
Friedreich, "Ueber progressive Muskelatropbie," 1873. 
Frey, A., "Berlin, klin. Wochenschrift.," 1874. MUSCULAR ATROPHIES AND HYPERTROPHIES. 
29 
Gessler, " Die motorische Endplatte," 1885. 
Gombault, "Arch, de physiol.," 1873. 
Graefe, A. v., "Arch. f. Ophthalmol.," 1868. 
Gayet, " Arch, de physiol.," 1875. 
Heine, J., " Beob. ub. Lahmungszust. d. ant. Extrem. u. d. 
Behldlng.," Stuttgart, 1840; " Ueber spin. Kindlmg.," 1860. 
Hutchinson, " Med.-chir. Trans.," 1879. 
Krishaber, "Arch, de physiol.," 1870; "Gaz. hebdom.," 
1872. 
Kussmaul, Volkmann's "Klin. Vortrage," No. 54, 1873. 
Landouzy et D6jdrine, "Rev. de med.," 1884 and 1886. 
Leyden, "Arch. f. Psych.," civ, ii, S. 423. 
Leyden, " Klinik d. Rckmkrheit.," 1876. 
Maier, Virch. " Arch.," Bd. 61. 
Mauthner, " Nuclearlahmung d. Augenmuskeln," 1885. 
Roger et Damascbino, " Gaz. m6d.," 1871; " Rev. de m6d.," 
1881. 
Schultze, " Ueb. die mit Hypertrophie verbund, progress. 
Muskelschwund," civ, 1886. 
Schultze, F., Virchow's "Arch.," Bd. Ixviii, 1876. 
Seppilli, " Rev. sper. di fren.," 1887. 
Seeligmuller, " Lehrbuch d. Krheiten. d. Rckmks. u. Ge- 
hirns," 1887. 
Seeligmuller, " Lehrbuch," 1887. 
Seguin, E. C., " Myelitis of the Anterior Horns," 1877. 
Tooth, " Brain," 1887. 
Trousseau, " Clinique m6d.," 1868. 
Underwood, "Treatise on Dis. of Children," London, 1784. 
Wachsmuth, "Ueb. progress. Bulbarparalyse u. die Diplegia 
facialis," 1864. 
6 East Forty-ninth Street, New York. © ft REASONS WHY 
Physicians Should 
\ Subscribe 
The New York Medical Journal, 
Edited by FRANK P. FOSTER, M. D.,1 
Published by D. APPLETON & CO., 1, 3, & 5 Bond St. 
1. BECAUSE : It is the LEADING JOURNAL of America, and 
contains more reading-matter than any other journal of its class. 
2. BECAUSE : It is the exponent of the most advanced scientific 
medical thought. 
3. BECAUSE : Its contributors are among the most learned medical 
men of this country. 
4. BECAUSE : Its "Original Articles" are the results of scientific 
observation and research, and are of infinite practical value to the 
general practitioner. 
5. BECAUSE : The "Reports on the Progress of Medicine," which 
are published from time to time, contain the most recent discov- 
eries in the various departments of medicine, and are written by 
practitioners especially qualified for the purpose. 
6. BECAUSE : The column devoted in each number to "Therapeu- 
tical Notes " contains a resume of the practical application of the 
most recent therapeutic novelties. 
7. BECAUSE: The Society Proceedings, of which each number con- 
tains one or more, are reports of the practical experience of 
prominent physicians who thus give to the profession the results 
of certain modes of treatment in given cases. 
8. BECAUSE : The Editorial Columns are controlled only by the 
desire to promote the welfare, honor, and advancement of the sci- 
ence of medicine, as viewed from a standpoint looking to the best 
interests of the profession. 
9. BECAUSE : Nothing is admitted to its columns that has not some 
bearing on medicine, or is not possessed of some practical value. 
IO. BECAUSE: It is published solely in the interests of medicine, 
and for the upholding of the elevated position occupied by the 
profession of America. 
Subscription Price, $5.00 per Annum. Volumes begin in January 
and July.