[Reprinted from The Journal of Nervous and Mental Disease, Vol. 
XIV., August, 1887 j 
HEMIPLEGIA IN CHILDHOOD. 
PHILIP COOMBS KNAPP, A.M., M.D. (Harvard), 
PHYSICIAN TO OUT-PATIENTS WITH DISEASES OF THE NERVOUS SYSTEM, BOSTON CITY HOSPI- 
TAL, AND PHYSICIAN TO THE DEPARTMENT FOR NERVOUS DISEASES, BOSTON DISPENSARY. 
THE recent contributions of Strumpell (70, 71) on the 
subject of cerebral infantile paralysis have 
aroused a new interest in a symptom-complex 
which, although long recognized by foreign writers, has 
been much neglected by Americans and Englishmen. Al- 
though hemiplegia in childhood is often attended by cer- 
tain peculiarities which entitle it to special consideration, 
none of the works on children's diseases, with the single 
exception of Money's (49) treatise which has just appeared, 
describes it or discusses the vexed question as to its pa- 
thology. Most of them speak of ordinary cerebral 
hemorrhage and embolism ; but, with the exception of 
Money, none of them so much as mention the atrophy of 
the cortex so often met with, or give any full account of 
the special features of hemiplegia in childhood. This 
omission is the more singular, since many of the recent 
text-books on nervous diseases describe the affection with 
more or less fulness. 
We owe to Cazauvieilh (14) the earliest description of 
that form of cerebral infantile paralysis which is now re- 
garded as typical-the form due to atrophy of the convo- 
lutions-although he states that cases had been met with 
previously by his instructors in Paris. In 1853 Little (40), 
in England, in writing of " spastic rigidity of the limbs of 
the new-born " described cases of cerebral infantile para- 
lysis, but he failed to distinguish these cases clearly from 
'Candidate's paper read by title at the meeting of the Am. Neurol. Assoc., 
July, 1887. 2 
PHILIP COOMBS KNAPP. 
cases of spastic paraplegia from spinal disease, or to recog- 
nize the pathological changes underlying the affection. 
During the past year, I have had the good fortune to 
see no less than ten cases of paralysis due to cerebral dis- 
ease in childhood. None of these cases have as yet come 
to autopsy, so that I can add nothing to the study of the 
morbid processes involved, but clinical study certainly 
gives some hints as to a possible pathology in the cases 
which I shall report. 
Observation I.-Convulsions in infancy. Right hemiplegia with 
contracture. Convulsions, now often limited to right arm. Arrest 
of growth on paralyzed side} 
Frederick K., n years old, the second child of German parents. 
His mother had weak, faint spells before he was born, and is said 
to have uterine disease. With this exception the family history is 
good. One child died of heart disease, five others are living and 
healthy. There is no history of syphilis or hereditary nervous 
taint. He was born at term, the labor being twelve hours in 
length and rather difficult, but instruments were not used. The 
head presented. After birth no abnormality was noted except 
1 The illustrations are reproduced from photographs taken by Dr. R. A, 
Kingman, of Boston. HEMIPLEGIA IN CHILDHOOD. 
3 
blueness of the fingers. At the age of three months, he began to 
have convulsions, which gradually increased in frequency. When 
one year old, he began to get his teeth, dentition being difficult, 
and each tooth requiring to be lanced several times. The con- 
vulsions at this time grew worse, he had at times fifty or sixty a 
day, and sometimes ten or a dozen while his father was taking 
him to a doctor. Cutting the gums seemed to give relief. I 
could learn little as to the character of these convulsions except 
that they were general-he became rigid and all his limbs worked. 
Since dentition the convulsions have continued with more or less 
frequency. He has gone without any for two months at a time, 
and then he has had them quite frequently. March 26th, 1887, 
two days before he was brought to the hospital he had twelve, and 
the next day ten. The present convulsions begin with a feeling 
of something going down the right arm, which comes on long 
enough before the attack to enable him to go and lie down. In 
the severe attacks, everything looks dark to him; he can neither see 
nor speak; he cries out and works all over. Only in the severe 
attacks does he lose consciousness. Most of his attacks are con- 
fined to the right arm, and many of them to the right hand, and 
in these he is conscious and can speak. On May 23d, I saw him 
in a mild attack. There was no change of color in the face, and 
the right hand was the only part affected. He was perfectly con- 
scious throughout and could answer questions. The pupils were 
not observed. The fingeis of the right hand were strongly ex- 
tended and abducted-a position which he could not putthem into 
of his own will-and they twitched a little in adduction and flexion. 
The whole attack lasted forty or fifty seconds. 
The boy could not walk until he was three years old, and then 
the parents noticed that the right side was much weaker than the 
left, a condition which probably had come on some time before. 
He now walks with a limp, but the arm remains partly para- 
lyzed, and there is a decided arrest of growth on that side. He 
could not talk until he was three years old, and now his speech is 
rather indistinct. The trouble is purely one of co-ordination, for he 
never uses the wrong word or is at a loss for a word; he reads and 
writes as well as could be expected, and his speech is always in- 
telligent. In spite of his hemiplegia and convulsions, his general 
health is very good ; he never has the headache, eats and sleeps 
well, and has no trouble of any sort. His parents say that his 
teacher considers him good scholar, and they regard him 
as very bright, as bright if not brighter than their other children. 
Two years ago, he was sent to Dr. Baker's home at Baldwinsville 
for his convulsions, but he grew very homesick and stayed but six 
months. Since that time he has taken bromide-how much or 
how constantly I could not learn-without much benefit. Borax- 
fifteen grains three times a day-was given, and since then the 
convulsions have been fewer and less severe. An attempt was 
made to control the individual attack by a ligature at the wrist, 
but it had no effect. 4 
PHILIP COOMBS KNAPP. 
The boy is a little below the average height, but he is remark- 
ably well developed, except on his paralyzed side, and well 
nourished. He appears very bright and quick-witted. The sense 
of smell is alike on the two sides. Vision is good ; the eyes and 
pupils move naturally, and the field of vision and the fundus oculi 
are normal. The tongue is protruded straight. The right side of 
the face is smoother than the left, but he can draw his mouth 
better to the right ; on whistling or laughing, the mouth is drawn 
a little more to the left than to the right. There is no difficulty in 
swallowing. The right side of the thorax is smaller than the left, 
but physical examination reveals nothing abnormal. The right 
arm is much atrophied, the hand is cold and cyanotic, and the 
arm is held against the chest, flexed at the elbow and wrist, 
strongly pronated, with the fingers flexed on the palm. The ac- 
companying photograph, kindly taken for me by Dr. R. A. King- 
man, of Boston, will show his ordinary attitude. Voluntary move- 
ments at the shoulder and elbow are possible, but he can neither 
supinate nor extend the wrist. The wrist can be flexed a little 
more, and there is a little movement of the fingers, chiefly in 
flexion. There is marked rigidity of the wrist on passive motion, 
and some of the elbow. The foot is in equino-varus, and the toes 
can be moved but little voluntarily ; there is also marked resis- 
tance to passive motion at the ankle. The heel, however, reaches 
the floor on standing. There is no power of voluntary motion at 
the ankle, but motion at the hip and knee is fairly good. He 
walks fairly well, swinging his leg in the characteristic manner of 
a hemiplegic patient. The muscles of the arm reacted about alike 
on the two sides to a weak faradic current ; except that on testing 
the extensors of the wrist and the supinators on the right the cur- 
rent had to be stronger, so that it was diffused, causing contrac- 
ture of the antagonists, and the muscles tested did not respond as 
well. Sensation was everywhere normal. The epigastric, ab- 
dominal, and cremaster reflexes were normal; the plantar was nor- 
mal on the left, but absent on the right. There was no triceps, 
radial, or ulnar reflex, or ankle clonus. The patellar reflex was 
exaggerated on the right, but very weak on the left, except by Jen- 
drassik's method. 
The following measurements were taken : 
Semicircumference of chest at nipples,. .... 
RIGHT. 
11% in. 
LEFT. 
I2%in. 
•Circumference of arm 4 inches above olecranon , 
6 " 
" " " 5 " *' styloid process of radius 6V 
7%" 
" " " 2 " *• " " " " 
" 
" " hand at metacarpals, just above thumb, 
5'/2 " 
6'4* 
" " thigh 5 inches above upper edge of patella 
" 
I4%* 
" " calf in largest part, .... 
9% " 
10 " 
Anterior superior spine to internal malleolus, 
23% " 
23%" 
Length of ulna. ........ 
" 
7%u 
Clavicles about the same in length, 
The right hand and fingers are decidedly smaller. .HEMIPLEGIA IN CHILDHOOD. 
5 
Observation II.-Injury to head Convulsions. Paralysis of 
left arm and fc.ce without marked contracture. Arrest of growth in 
arm. 
James E., 14, born in Italy. He was in good health until he 
was six years and a half old when he fell from a window, striking 
his head. He was unconscious for three hours, and then made a 
complete recovery. Three months later he had a convulsion. 
Since then he has had convulsions at various intervals, averaging 
about one a week. It was not easy to get a clear account of these 
attacks from either the boy or his family. He is said to cry out, 
bite his tongue, and lose consciousness, but he does not fall. I 
could not learn whether the attacks were preceded by an aura or 
not. In former attacks, the face was drawn to the left and the 
left arm worked, but now neither the face nor the limbs are con- 
vulsed. Since having the convulsions, he has had loss of power 
in the left arm, which is now much smaller than the other. He 
has had no difficulty in walking, or paralysis of the leg, but lately 
he has complained that his legs ache, and that his left knee is sore. 
He has occasional frontal headache, but no vertigo. Lately he 
has had a little cough and palpitation and some pain in the chest. 
His appetite is good, but he has had a little diarrhoea for two or 
three months before coming to the hospital. He was given bro- 
mide of sodium, fifteen grains three times a day, which stopped his 
convulsions completely during the whole time that he was under 
observation-some five or six weeks. 
The boy is well nourished and developed. The left arm is 
manifestly smaller than the right, and is cold and cyanotic. The 
eyes move naturally, the pupils respond to light, and the fundus 6 
PHILIP COOMBS KNAPP. 
oculi is normal. He can perform all movements with his left 
hand except adduction of the little finger, but all the movements 
are feeble. The dynamometer showed a grasp of 46 with the 
right hand and 22 with the left. There is no contracture or rigidity 
of the arm, but the fingers are slightly flexed and are a little stiff 
and cannot be completely extended. The photograph, taken like 
all the rest by Dr. Kingman, shows this slight contracture, and the 
smaller size of the hand. The boy was directed to hold both 
hands up against a screen as high as he could, and, as will be seen, 
he could not reach as far with the left. He cannot draw his face to 
the left, either voluntarily or on laughing. No cicatrix or depres- 
sion of the skull could be detected. There is no paralysis or 
noticeable weakness of the legs, which are of the same size. The 
fingers of the left hand are smaller and smoother. The muscles 
of the arms react alike to a weak faradic current. The triceps 
and radial reflexes are slightly more marked on the left. The 
patellar reflex is absent, except by Jendrassik's method, when it 
is very weak. Sensation is normal. Examination of the chest 
revealed nothing. 
The following measurements were noted : 
RIGHT. 
Circumference of arm 6 inches above olecranon . . . 75/in. 
LEFT. 
7 in. 
u '/< *<< 3 7. 
" . . . _. 7K 
7% " 
5 
" styloid process of radius " 
6% " 
" " " i " 
" " " " " 5% " 
5% " 
Observation III.-Right hemiplegia with contracture. Some 
mental impairment. Absence of convulsions. Marked arrest of 
growth on paralyzed side. 
Wm. C., ii, of Irish parentage. He was born at term in one 
pain, the head presenting and the labor being natural and easy. 
When three weeks old he had scarlet fever mildly, and when five 
months old he had whooping cough. No history of any syphilitic 
or hereditary nervous taint could he obtained. He learned to 
walk at ten months, and after that walked and ran about like 
other children. About the same time he learned to talk. When 
a year and ten months old he was put to bed in his usual health, 
and nothing was heard from him during the night. The next 
morning it was found that he could not walk or stand alone, and 
later in the day his mouth was drawn to the left. Since that time 
the whole right side has been paralyzed, but there has never been 
any disturbance of speech. For three months he was in bed, but 
after that he got up and began to get about. At present he goes 
to school, and plays with the other boys, and in spite of his par- 
alysis, can get over the ground pretty fast. Except for an attack 
of measles some years ago his health has been good, although he 
is not very vigorous. He began to go to school at the age of five, 
but thus far he has not distinguished himself. He is still in the 
primary school, and spent four years in one room. His teacher 
calls him a good boy but a poor scholar. He can read some and HEMIPLEGIA IN CHILDHOOD. 
7 
write a little with his left hand. His father states that his mem- 
ory is very poor. Until he was seven years of age his mother 
says that both sides of his body grew alike, but after that his right 
side did not grow as fast, so that it is now much smaller than the 
other. He has never had convulsions or petit mal, and is not sub- 
ject to headache. He can talk perfectly well. There are no 
thoracic, abdominal, or urinary symptoms. 
The boy is of the average size, rather poorly nourished, and 
presents a pronounced arrest of development on the right side, 
even the face seeming smaller on that side. He answers ques- 
tions readily and comprehends what is said to him, but he is 
rather stupid. He is said to be able to read his school-books 
quite well, but on trying him with the printed diet list of the hos- 
pital he read the short words well enough, but he had much diffi- 
culty with the longer and more unusual words. The pupils and 
eyes moved naturally, the field of vision was good, and the fundus 
oculi was normal. The tongue was protruded straight. He did 
not wrinkle the right side of his forehead as much as he did the 
left in raising his eye-brows, but he closed his eyes naturally. He 
could draw his face to the right fairly well, but on laughing it was 
drawn to the left, and in repose the left naso-labial fold was more 
marked, and the left angle of the mouth was a little higher. This 
paralysis on mimetic but not on voluntary movement, seen here 
and in Obs. I., is of interest as an indication of the existence of 
separate channels for innervation. Rosenbach,1 who has recently 
called attention to the subject, claims that the movements of ex- 
pression are innervated through the optic thalami. The apparent 
paresis of the upper branch of the facial is remarkable in intra- 
cerebral disease. The accompanying photograph will show 
the atrophy and deformity. His right arm, which is much atro- 
phied, is held to the side, flexed at the elbow and wrist, strongly 
pronated, and the fingers semi-flexed. The skin is cold and 
cyanotic. There is great resistance on attempts at passive 
motion, owing to the great muscular contracture; at the wrist this 
cannot be overcome by any safe expenditure of force. He can 
move the shoulder and elbow a little, but the only movement 
possible of the hand is further flexion of the finger?. The 
thigh is flexed somewhat on the pelvis and rotated inwards, 
and the leg is flexed on the thigh. The gastrocnemius is greatly 
contracted, drawing the heel from the ground, so that he walks on 
the ball and inner edge of his foot-the foot being greatly de- 
formed, and in a position of marked equino-varus. This de- 
formity cannot be overcome by any attempts at replacement. 
There is fairly good movement of the hip, some of the knee, but 
none of the foot or ankle. The pelvis is tilted to the left to coun- 
teract the deformity. There is a compensating lateral curvature 
of the spine which makes the thorax seem smaller on the par- 
1 P. Rosenbach, "Zur Lehre von der Innervation der Ausdrucksbewegungen." 
Neurol. Centralblatt, June ist, 1886. . 8 
PHILIP COOMBS KNAPP. 
alyzed side, although it is not. He gets over the ground pretty 
fast, using the right leg as an occasional point of support, and 
hopping much of the way on the left foot. The muscles of the 
arm reacted alike on the two sides to a weak faradic current. 
Sensation was normal. Examination of the chest was negative. 
The abdominal and cremaster reflexes were normal. The plantar 
was normal on the left but absent on the right. The biceps, 
radial, and patellar reflexes were present, and were slightly more 
marked on the right side. The was no wrist, patellar, or ankle 
clonus. 
The following measurements will show the degree of wasting 
on the paralyzed side: 
RIGHT. LEFT. 
Semi-circumference of chest at nipples, .... n|in. 
Circumference of arm 4 inches above olecranon, . . 51 " " 
" " " 3 •* styloid process of ulna, 4I " 6j " 
" " " I " " " " " " 31" 4" 
" " thigh,5 " " upper border of patella, io| " n| " 
" " calf in largest part, . . . . 71 " 9f " HEMIPLEGIA IN CHILDHOOD. 
9 
Anterior superior spine of ileum to internal malleolus, 
RIGHT. LEFT. 
28i in. 29 in, 
Length of ulna, . . . . . . . 
6£ " 7f " 
Fingers of right hand and hand itself much smaller. 
Observation IV.-Right hemiplegia of gradual onset. Tem- 
porary aphasia. Slight arrest of growth. No rigidity, contracture, 
or convulsions. 
Kate H., 12 years old, of Irish parentage. The family history 
shows no syphilitic or hereditary nervous taint. Eleven years ago 
she had diphtheria, three years ago whooping cough, and two 
years ago measles. Except for these illnesses she has been well 
until a year ago. At that time, on her return from school, where 
her work had not been especially hard, she had a " reeling in her 
head," and fell over on the floor. She was picked up and prob- 
ably had some difficulty in walking immediately after. There 
was no loss of consciousness, but her right side gradually became 
paralyzed, and her face was drawn to the left. For six weeks she 
was confined to her bed, then she gradually got able to move 
about and to use her limbs, and since then she has steadily but 
slowly improved. She never had convulsions, but during her con- 
finement to the bed she complained greatly of headache and heat 
in the head, and ice was applied to the head by her physician's 
orders. Six months ago she had some twitching of the right 
hand, which has disappeared. For three months she could not 
talk so as to be understood, but now she talks without any notice- 
able defect. During the first of her illness she was rather slow 
and dull in her mental processes, but now her mental condition is 
natural. At present she has some headache, chiefly frontal or in 
the left temporal region, and a little vertigo. There never have 
been any thoracic symptoms, and before this attack she could 
run and play like other children. The digestive functions are 
performed normally. She sleeps well, and there has been no 
change of disposition. 
The pupils and eyes move naturally; the field of vision and the 
fundus oculi are normal. She cannot draw her mouth to the 
right, but there is no inequality on smiling. She can move her 
arm and leg in all directions, but without much strength. Dorsal 
extension of the right foot is not performed as well as that of the 
left, and extension and abduction of the fingers of the right hand 
is attended with a little tremor. There is no contracture or 
rigidity, but the limbs are a little smaller on the right. She al- 
ways was right-handed, but since her illness she has learned to 
write with the left hand. The heart and lungs were normal on 
examination. The plantar reflex is diminished on the paralyzed 
side ; the abdominal and epigastric reflexes are absent on both 
sides. The triceps reflex was alike on the two sides; the radial 
and ulnar were absent, the patellar was exaggerated, and rather 
more marked on the right side. There was no clonus or front 
tap contraction. Sensation was perfectly good. The muscles of 
the face and arms react alike on the two sides to a weak faradic 10 
1'IllLIP COOMBS KNAPP 
current. Dr. W. N. Bullard, who saw her previously at the Bos- 
ton Dispensary, and kindly permitted me to make use of his 
notes, reports that the galvanic reactions were tested by Dr. J. A. 
Jeffries, who found that the extensor communis digitorum showed 
KaSZ > AnSZ with Ma.; the right peronei muscles, KaSZ > 
AnSZ iV Ma.; the rectus femoris, KaSZ = AnSZ; everywhere else 
KaSZ > KaOZ and AnSZ > AnOZ. The following measure- 
ments show the difference in size. 
RIGHT. 
Circumference of arm, 5 inches above olecranon, . . . 65 in. 
LEFT. 
7i in. 
" " " 5 " 
styloid process of radius, 6| " 
6|" 
66 66 66 J 66 66 
" n n 4f " 
4« " 
" "th'gh, 5 " " 
upper border of patella, I2| " 
I2| " 
" " calf in largest part, 
" 
ioj " 
The right ulna was as long as the left, the semicircumferences of 
the chest were the same, and the legs were of the same length. 
As the paralysis is not of long duration, and as the child is already 
pretty well grown, we should not expect much of an arrest of 
growth in the bones as yet, if at all. 
Observation V.-Cephalalgia, probably of syphilitic origin. 
Right hemiplegia. Arrest of growth on right side. No contracture, 
but exaggeration of tendon reflexes. 
Michael R., 21, S., Plumber. Came to the out-patient depart- 
ment of the Boston City Hospital complaining of severe headache, 
weakness, and malaise. He seemed so ill that no careful exami- 
nation was made. He stated then that his headache had lasted for 
five or six weeks, that he had used alcohol to excess, and that he had 
never had syphilis. For a month or six weeks he had noticed that 
his face was drawn a little to the left, and that his right side was 
weaker than thele ft. On stripping him there was found to be a 
distinct right hemiparesis, and a marked difference in the size, of 
the limbs on the two sides of the body. Dr. Gleason, who was 
acting as externe, reported that the muscles of the arms reacted 
alike on the two sides to a mild faradic current. I am obliged to 
quote my own observations from memory, but I am indebted to 
Dr. C. F. Folsom, to whose service he was admitted, for kindly 
furnishing me a copy of the records of his case while in the hospi- 
tal, as follows: 
"Admitted June 15th, 1887. Family history good. Left- 
sided pleurisy two years ago. No other sickness. No syphilis. 
Drinks to excess. Not feeling well for two weeks, general 
weakness, frontal headache, loss of appetite. Bowels con- 
stipated. No abdominal or febrile symptoms. Last two days 
vomited three times. About six months ago noticed right hand 
smaller than left. No pain in right arm or leg, but they are not 
as strong as formerly; finds it difficult to use right hand in fine 
work. Eyesight and eyes all right. 
" Well developed and nourished. Tongue moist, with a white coat, 
protruded a little to the right. Pupils normal. Left corner of HEMIPLEGIA IN CHILDHOOD. 
11 
mouth drawn up a little. More contraction of left muscles than 
right when he knits his brows. Right grasp weaker than left. 
Heart's apex in or just outside of mammillary line. Prolonged first 
sound at apex; action a little uneven. Lungs and abdomen 
negative. Both knee-jerks exaggerated, especially right. Con- 
siderable tremor of leg when lightly tapped. Ankle clonus. Wrist 
clonus a little more marked right than left. On walking, right leg 
seems stiff, and foot occasionally drags. Stands well with feet to- 
gether and eyes shut. No tenderness of shins. No neuritis or 
oedema. Patient right-handed. Left upper arm 2.5 cm. larger 
than right; forearm 1 cm. larger; hand at the metacarpo-phalan- 
geal joints 2 cm. larger; right arm to end of middle finger 1 cm. 
shorter. 
" June 23d.-Has had two or three attacks of extremely severe 
headache, relieved by morphine, and diminished by iodide of 
potassium, gr. xl., t. i. d. Now slight headache. Some dizziness 
and nervousness. Facial paralysis seems to be increasing. Up 
and about daily. 
"June 28th.-Discharged at own request, against advice." 
In spite of the patient's statement, it did not seem possible either 
to Dr. Folsom or myself that any recent lesion could cause such 
an atrophy of the right side, involving the bones; and further- 
more, the benefit derived from iodide made us strongly suspect 
some syphilitic taint. It seemed to us not unlikely that he had had 
a hemiplegia in childhood, from which he had recovered, but 
that it had caused an arrest of growth on that side, and possibly 
an exaggeration of the tendon reflexes. This had passed un- 
noticed until recently, when a syphilitic process (endarteritis ?) 
had developed at the site of the old lesion, as it is prone 
to do, causing weakness of the right side, and perhaps facial 
paralysis, and, with its attendant symptoms of headache and 
malaise, had called his attention to his right side, which he discov- 
ered for the first time to be smaller than the left. It is a matter of 
common experience that symptoms that do not cause discomfort 
are often overlooked, and, in a patient whose mental powers were 
as untrained as this man's, such a hypothesis is by no means im- 
probable. 
A glance at the illustrations to this article-notably 
those of Observations I. and III.-or at the patients them- 
selves, would lead one very naturally to make a diagnosis 
of an old anterior poliomyelitis ; but more careful exami- 
nation will show that in all these cases the lesion is without 
doubt cerebral. The hemiplegicdistribution of the paralysis, 
the implication of the facial muscles, the exaggerated re- 
flexes, the spastic contracture seen chiefly in the arm, the nor- 
mal electrical reactions, the presence of convulsions, all 12 
PHILIP COOMBS KNAPP. 
point to a lesion in the brain and not in the anterior cornua 
of the cord. It is a cerebral infantile paralysis and not a 
spinal. In spinal paralysis hemiplegia is rare and the 
face is very rarely if ever involved , the tendon reflexes are 
absent or at least diminished, convulsions, except in the 
initial stage, are never present, and the characteristic 
reaction of degeneration, or the absence of faradic reaction 
or of both faradic and galvanic reactions, is to be expected. 
There is one point of distinction in the cases reported 
which seems to me of decided importance, and that is the 
existence of epileptiform attacks after any symptoms of 
active disease have passed away. This believe to be of 
great value in diagnosis as a means of differentiation be- 
tween a lesion involving the cortex and a lesion farther 
down in the brain, deep down in the centrum ovale or in 
the region of the basal ganglia. In the first two cases 1 
believe that the lesion is cortical; in the third and fourth 
that it is central; the fifth I imagine is central, but the im- 
possibility of getting any definite history from the patient 
renders it useless to attempt to draw any conclusions from 
it, and I shall therefore leave it out of account. 
Considering first the cases where epileptiform attacks 
were absent (Obs. III. and IV.), in Obs. III. the lesion has 
destroyed the left motor tract somewhere between the 
cortex and the pons, probably in the internal capsule, and 
has been followed by a secondary degeneration of the 
right crossed pyramidal tract in the cord. In Obs IV, 
there is little or no secondary degeneration and the lesion 
is probably not one which destroys the tract, but one 
which causes a break in the nerve conduction by pressure 
upon it, and it very likely lies in the lenticular nucleus or 
the corpus striatum. 
The pathology of these cases is not without interest, al- 
though it has been less discussed, owing to the much greater 
interest aroused of late in the study of the pathology of 
cortical lesions in children, of which I shall speak later. 
The lesions in or about the basal ganglia giving rise to 
hemiplegia are the same as those in adults-abscess, 
tumor, embolism, thrombosis, and hemorrhage. That in HEMIPLEGIA IN CHILDHOOD. 
13 
Obs. III. or IV. there is any progressive trouble, such 
as abscess or tumor, seems extremely improbable from 
the course of the cases, and the absence of any special 
symptoms, such as headache, vertigo, or optic neuritis. 
At the last meeting of the American Neurological Asso- 
ciation Dr. Sachs (61), in a paper of which only an abstract 
has yet appeared, called attention to the occurrence of 
intracerebral hemorrhage in the young, and laid especial 
stress on the absence of epileptiform attacks as a diagnostic 
point between intracerebral and meningeal hemorrhage 
That intracerebral hemorrhage does occur in children 
has been repeatedly proven by autopsy, although most 
writers on children's diseases claim that it is extremely 
rare. Dr. Sachs stated that, according to certain obser- 
vations made by Osler, miliary aneurisms occur in chil 
dren ; and furthermore, that Recklinghausen claims that 
fatty degeneration of the cerebral arteries, permitting trans- 
udation of blood, was not infrequent. In the discussion 
that followed Dr. Sachs' paper, Dr. Zenner called atten- 
tion to the fact that there may be an embolism from en- 
docarditis when no adventitious murmur is to be heard 
in the heart. The diagnosis between embolism and 
hemorrhage is so difficult as often to be impossible, and 
although in these two cases I am inclined to believe that 
there has been a hemorrhage, I do not feel that such a 
diagnosis could be confirmed without an autopsy. 
Returning now to Obs. I. and II., in which epileptiform 
attacks were present, and in which the lesion was thought 
to be cortical, we enter upon a question in pathology 
which of late has been widely discussed. The discussion 
of the character of the lesion in Obs. II. need not detain us 
long. Evidently of traumatic origin, there has probably 
been a hemorrhage, either in the meninges or involving 
the cortex itself, yet not severe or extensive enough to de- 
stroy its functions. The absence of severe headache, ver- 
tigo, and other general symptoms makes the diagnosis ol 
any other morbid process, such as meningitis or tumor 
less probable. 
In Obs. I., however, the case is different. Here we have 14 
PHILIP COOMBS KNAPP. 
a morbid process coming- on spontaneously, involving the 
cortical gray matter of the left central convolutions, most 
markedly in the centre for the hand, but involving also the 
centres for the face, arm, and leg, and the motor centre for 
speech. From the cortex there has been a descending degen- 
eration of the fibres of the pyramidal tract down into the 
cord. This is one of the cases to which Strumpell (70, 71) 
has given the name of acute poliencephalitis, regarding 
them as similar in respect to their morbid processes to the 
cases of acute anterior poliomyelitis. 
The disease attacks children in the early years of life, 
especially under six years of age, and comes on either 
during a period of perfect health or especially after some 
acute disease, particularly the exanthemata. It may 
begin with fever, delirium, vomiting, and diarrhoea, or, 
very commonly, with convulsions, and Strumpell suggests 
that poliencephalitis may be the cause of death in those 
cases where a child dies suddenly with acute cerebral 
symptoms, such as convulsions. On recovering from this 
initial stage the child is found to be paralyzed, usually 
hemiplegia, the arm, leg, and lower part of the face being 
generally affected, although strabismus is sometimes 
seen. The arm is generally more helpless than the leg, as 
in the hemiplegia of adults. Contracture develops as in 
adults, and there is also a pronounced arrest of growth in 
the paralyzed limbs, involving the bones as well as the 
muscles. There may be a slight quantitative diminution 
of electrical excitability in the paralyzed muscles, although 
this is rare, but there are never any quantitative changes. 
Sensation is normal. The tendon reflexes are exaggerated 
on the paralyzed side and sometimes on both sides; but 
the cutaneous reflexes are absent or diminished. In ris-ht 
hemiplegia there may be disturbances of speech. Epilep- 
tiform attacks generally persist, and in many cases there 
are other motor disturbances-chorea, athetosis, ataxia, or 
associated movements. The child is very apt to be imbe- 
cile or idiotic. In the cases that have come to autopsy, 
porencephalia-a loss of substance or atrophy of the con- 
volutions-has been observed, and many observers have HEMIPLEGIA IN CHILDHOOD. 
15 
found traces of old inflammatory disturbance, spider and 
fatty granular cells. Striimpell, moreover, is inclined to re- 
gard this disease, as well as acute anterior poliomyelitis, 
as of infectious origin-a hypothesis which has received 
a remarkable support from a case cited by Mobius (48). 
Two children in the same family were attacked within 
twenty-four hours of each other with fever, loss of appe- 
tite, and restlessness. One child, a girl, in a day or two 
developed a typical anterior poliomyelitis, and her brother 
an equally typical spastic hemiplegia, with post-paralytic 
chorea. 
Striimpell (70) closes his paper by saying that we must 
bear in mind that the hemiplegia of children may be due 
to other causes of which I have already spoken. Bern- 
hardt (5) adds that if, " either suddenly while in apparently 
perfect health, or in the beginning, or during the course 
of convalescence from an acute febrile disease (especially 
the exanthemata), we notice in children in the first years 
of life a sudden outburst of unilateral convulsions, lasting 
for hours and followed by hemiplegia with the peculiar 
symptoms described (contractures, hemichorea, hemiathe- 
tosis, associated movements, etc.) ; and if, beside these 
symptoms, the psychical anomalies and especially epilepsy 
persist, we are justified, in my opinion, in recognizing 
this special form of cerebral infantile paralysis, this spastic 
cerebral infantile hemiplegia, as a more or less distinct 
clinical type." 
So far most observers substantially agree. They also 
admitthat the lesion of this "distinct clinical type " is sit- 
uated in the motor region of the cortex, but the nature of 
the morbid process is still a matter of dispute. In the 
majority of cases the resu It of the process is porencephalia- 
a loss of substance in the brain involving the convolutions, 
or a cicatricial thickening and atrophy of the cortex. 
Whatever the primary process may be, it leads ultimately 
to this. Dr. Sarah J. McNutt (44), in a paper read before 
the American Neurological Association three years ago, 
tabulated thirty-four autopsies, of which thirty-two showed 
wasting or porencephalic defects in the cortex. I have 16 
collected twelve other cases, in nine of which a similar 
condition was found [Bernhardt (5), Binswanger (7), Jen- 
drassik and Marie [two] (33), Kast[two] (36), Mordet [50], 
Sander (62), Warner and Beach (73)]. One case [Seelig- 
muller (63)] revealed meningitis with softening of the cor- 
tex; in another [Beach (3)], the parietal convolutions were 
firmer and " coarser,"?', e., less well developed, and the mi- 
croscope sho wed a great increase in the number of vessels, 
with distention of the vessels, and extensive infiltration 
with leucocytes, especially in the perivascular spaces. In 
the last case [Fraser (21)] there was a hypertrophy of the 
parietal lobe on the side opposite the paralysis, but the 
microscope revealed a defective development of cells. 
The cause of this change in the cortex is still uncertain. 
In some cases where the trouble was congenital, Cazau- 
vieilh (14) claimed that there was a defective development 
of the brain due to some intra-uterine disturbance. Little 
(41) attributed the trouble in his cases to injury of the 
brain received at birth. Strtimpell's theory of an inflam- 
matory process involving the cells of the gray matter of 
the cortex has already been referred to, and it is sup- 
ported by Eichhorst (18). Kundrat 37) believes that por- 
encephalia may be either congenital-a defect of develop- 
ment-or acquired. The acquired form follows the dis- 
tribution of the arteries and is often of vascular origin, 
arising from an anaemia without arterial lesion, depend- 
ing upon vascular weakness. This anaemia may be con- 
secutive to pressure during labor. Porencephalia, he 
states, is a loss of substance from some destructive process 
in the brain, arising from hemorrhage, thrombosis, em- 
bolism, or anaemia without arterial lesion. Wood (76) 
and Bastian (2) assert that spastic infantile hemiplegia is 
due to cortical hemorrhage, but they do not say whether 
the hemorrhage may give rise to porencephalia ; Ross (59), 
however, claims that it does. Jendrassik and Marie (33), 
however, could find no traces of hemorrhage or softening 
as a starting point fcr the sclerosis. In their two cases 
they found the perivascular lymph spaces greatly dilated, 
containing spider and fatty granular cells. This they regard 
PHILIP COOMBS KNAPP. HEMIPLEGIA IN CHILDHOOD. 
17 
as the primary process with a secondary diffuse process 
leading to sclerosis and atrophy following it, the exact 
nature of the change being still in doubt. Henoch (29) 
finds an interstitial growth of the neuroglia-a sclerotic 
process-with fatty granular cells and corpora amylacea. 
In this connection it may be said that Jastrowitz (32) does 
not regard the mere presence of fatty granular cells as 
proof of an inflammatory process,but only their aggregation 
into foci. The development of the medullary sheaths in the 
foetal brain is normally attended with the formation of 
fatty granular cells. Steffen (69) attributes the trouble, 
perhaps, to a meningitis. Gowers (24) maintains that these 
cases of hemiplegia are due to a venous thrombosis. This 
view is corroborated by a case reported by Heubner (30), 
where, in addition to a genuine porencephalia, a thrombus 
was found in the right artery of the fissure of Sylvius. The 
patient also had endocarditis. Lambl (38), who exam- 
ined a case of congenital brain defect, gives as the causes 
of porencephalia congenital delect, aplasia from syphilitic 
endarteritis or embolism, loss of blood-supply, interstitial 
encephalitis, a fatty degenerative process, Kundrat's an- 
aemia without arterial lesion, and perhaps hydrocepha- 
lus. 
The latest contribution to our knowledge of the pathol- 
ogy of the affection has been made by Kast (36), who bad 
an opportunity to examine two cases at an earlier stage in 
the disease than any that have been reported. He agrees 
with Bernhardt in thinking the lesion is not a pure poli- 
encephalitis, but also a chronic leuko-encephalitis, a diffuse 
sclerosis of the gray and white matter of the cerebrum. He 
failed to see the initial stage of perivascular changes de- 
scribed byJendrassik and Marie,although he considered it 
impossible to decide from his data whether vascular 
changes were or were not the primary trouble ; nor did he 
find Gowers' tnrombosis of the veins. He believes it im- 
possible at present to decide as to the primary change, owing 
to the lack of examinations of cases in the initial stages. 
At present, therefore, it seems safe say that probably 
several processes may give rise to porencephalia-hemor- 18 
PHILIP COOMBS KNAPP. 
rhage, embolism or thrombosis, congenital defect or acute 
encephalitis, which may be analogous to acute anterior 
poliomyelitis and may possibly be infectious. 
The prognosis must always be guarded. In Obs. I. 
and IL, I have thus far been fortunate in holding the con- 
vulsions in check, but they often persist. Mild cases of 
paralysis, as in Obs. IV. and V. may make a tolerable recov- 
ery by the aid of tonics, cod-liver oil, massage, and elec- 
tricity; but when there is pronounced secondary degener- 
ation, as in Obs. I. and III., the prospect of recovery is of 
course hopeless. The patient may live to a fair age, al- 
though Henoch (29) claims that few outlive their twenti- 
the year, but it must be remembered that they never have 
a sound brain, and thus must fall behind in life's race. 
Idiocy and imbecility are common sequelae, although not 
present in the cases reported, and Mercklin (46) thinks 
that in those whose minds are not at first affected there is 
a lessened resistance and an increased vulnerability of the 
brain, so that later in life various psychoses may develop. 
Persistent and patient work, however, in the way of 
massage and gymnastics, may produce some gain even in 
bad cases. 
Concerning two of the symptoms, I wish to speak fur- 
ther. Although four of these five cases were cases of right 
hemiplegia, the disturbances of speech were insignificant. 
In some cases aphasia may persist, so that the patient 
may remain mute for life, although Bernhardt (5) ques- 
tions whether congenital aphasia, unattended with idiocy 
or deafness, ever exists. Clarus (17) believes that where 
there has been a destruction of the speech centres the 
prognosis is bad, but with slight and transitory distur- 
bances of them it is good. Bernhardt thinks that aphasia 
in children is generally temporary, and usually of the 
motor type. In children, the right hemisphere may be- 
come educated as a speech centre, and thus acquire the 
power of speech after the centres in the left hemisphere 
have been destroyed. In support of this theory, he cites 
an observation by Kahler (35), where a woman had right 
hemiplegia from congenital atrophy of the convoluti HEMIPLEGIA IM CHILDHOOD. 
19 
including the left third frontal, with perfect power of 
speech. At the age of thirty-five she had apoplexy, 
causing left hemiplegia, from which she died. After her 
attack she was completely aphasic. At the autopsy a 
fresh lesion was found in the right hemisphere. 
The other symptom to which I wish to call attention is 
the very marked arrest of growth in the paralyzed limbs. 
Seeligmiiller (63) and Henoch (29) regard it as merely an 
atrophy from disuse, and assert that it is never so great 
as in anterior poliomyelitis. Such an assertion is unten- 
able, for in some of these cases the atrophyi s as pro- 
nounced as in poliomyelitis, and, as Forster (20) says, no 
atrophy from disuse could affect the growth of the bones. 
In certain cases of old hemiplegia in adults, Charcot,1 
Pitres,2 and Brissaud3 have found that, when there had 
been atrophy of the paralyzed limbs, the secondary de- 
generation had extended from the lateral columns to the 
motor cells in the anterior cornua, which they found 
atrophied. In some of these cases, however (Obs. II. and 
IV.), the signs of secondary degeneration in the lateral 
tract are very slight. In these cases, as in those of Ranke 
(54) and Seeligmiiller (63), the electrical reactions were 
normal; Steffen (69), however, has noted a quantitative 
diminution, and Henoch (29) says that when the muscle 
is entirely wasted they disappear. I have not found this 
diminution in the hemiplegia of childhood, but I have 
seen it in adults, and in one case I have seen AnSZ > 
KaSZ which is, as far as my knowledge goes, unique. 
In the cases reported, the various disturbances of mo- 
tion-chorea, athetosis, ataxia, and associated move- 
ments-so often noted by other observers, were absent, 
except, perhaps, in the earlier course of Obs. IV. In the 
'Charcot, " Legons sur les maladies du systeme nerveux.'' I., 55, Paris, 
1872. 
2 Pitres, "Note sur un cas d'atrophie musculaire consecutive a une sclerose 
laterale secondaire de la moelle epiniere." Arch, de physiol, norm, et path., 
p. 567, 1876. 
3 Brissaud, " De 1'atrophie musculaire dans 1'hemiplegie." Rev. mensuelle 
de med. et de chir., p. 618, 1879. 20 
PHILIP COOMBS KNAPP . 
other cases of cerebral infantile paralysis which I have 
seen, however, such disturbances held a prominent place 
in the picture of the disease; and at some future time 1 
shall hope to present a study of them. 
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