Reprinted from The Journal of Nervous and Mental Disease, October, 1890. 
DIFFUSE CORTICAL SCLEROSIS OF THE 
BRAIN IN CHILDREN.1 
By WILLIAM N. BULLARD, M.D., of Boston. 
1 DESIRE under this title to place certain cases of diffuse 
sclerosis in which the cortical layers of the cerebrum 
are the parts primarily or principally affected, and 
where the microscope shows the presence in these layers of 
a homogeneous ground substance, or of a finely fibrous net- 
work with a few spider-cells in the meshes, occupying the 
position of the neuroglia. In children but few cases in which 
this form of disease exists have been accurately described, 
and in our text-books the whole subject of diffuse sclerosis 
in children is still obscure. 
That the form of cortical sclerosis or degeneration which 
I am ab:ut to describe, can be, in many cases at least, clin- 
ically differentiated from other forms of cerebral sclerosis, I 
feel confident. From ordinary lobar sclerosis and from the 
different forms of tuberous and multiple sclerosis, this affec- 
tion can usually be distinguished. It is, on the other hand, 
closely allied to the conditions found in adults in general 
paralysis (paralytic dementia) and in chronic alcoholism. 
The cases of this affection of which careful descriptions 
exist are very few. 
1 Read in abstract before the American Neurological Association in Phila- 
delphia, June 5th, 1890. In the St. Bartholomew Hospital Reports for 1879. 
Moore gives the case of a girl, five years' old. Previously 
in perfect health. Six weeks before her entrance into the 
hospital she fell twice. This was followed by twitching of 
the left side of the face and hemiparesis of the extremities 
on the left side. Ten days later occurred sudden right hemi- 
plegia and aphasia. On admission to the hospital the pu- 
pils were widely dilated, insensible to light, no strabismus. 
She could not see, but could hear a little. She resented 
being touched, frowned and screamed ; ground her teeth 
frequently. Did not control sphincters. Had slight clonic 
movements of all the extremities, stronger on the left than 
on the right. No loss of sensation. Shortly after entrance 
had two epileptic attacks, and later opisthotonos. Died 
about six weeks after entrance. The autopsy showed scle- 
rosis of the cortex to a large extent ; the white matter was 
unaffected. Microscopically, increase in neuroglia, diminu- 
tion in number and possibly in size of nerve cells. 
Schmaus' case is somewhat analagous, but we are 
inclined to think should rather be classed among the lobar 
sclerosis, in which the process may be very diffuse. It oc- 
curred in a girl three years old. The pathological condition 
was " Diffuse sclerosis with enormous atrophy of the cere- 
brum, especially of the convolutions. Hydrocephalus inter- 
mus et externus ex vacuo. Pachymeningitis interna. 
Hydrocephalus externus. Atrophy and sclerosis of the an- 
terior columns of the cord, microscopically. All cortical 
layers diminished, but easily distinguished. Principal change 
is in the existence of a very thick net-work of fine fibres, 
lying between the ganglion cells. The ground substance in 
the white matter is likewise a fine net-work." 
My own case, which was quite remarkable in some 
respects, is as follows : 
The patient, when first seen, April, 1887, was a boy 13 
years old, a native of Boston. The family history, which 
was carefully enquired into, showed nothing remarkable. 
No neurotic tendency. 
The patient was born at full term, the labor was natural, 
and no instruments were used. His general health has 
2 
WILLIAM N. BULLARD. CORTICAL SCLEROSIS OF THE BRAIN IN CHILDREN. 
always been excellent. He learned to walk when 19 months 
old. Has always been bright and intelligent, wrote a good 
hand, and at the time of his accident was in the 5th class of 
the Grammar school. 
In August, 1886, he had a fall (was said to have been 
knocked down) and struck his head against the curbstone, 
making a wound over the R. parietal bone, the scar of which 
still remains. He did not, however, complain of any pain 
in it, though it was somewhat tender, and his whole head 
has been sensitive all winter. At the time of the accident, 
he was perfectly healthy, and he was promoted in school 
after the injury, but his mother believes that after it, he was 
stupid at school, and did no work. 
The first thing peculiar noticed about the patient was 
three weeks after the accident, when he forgot the baby 
whom he had taken out of doors. He was accustomed to 
take the child out, and was very fond of him. Two months 
later, he lost himself one day, and could not be found until 
the day following, having spent the whole night out. He 
could not tell people where his home was, nor find it him- 
self. 
Since the accident, he has gradually lost his memory, 
become forgetful and lost interest in external objects. 
In the beginning of his illness it was noticed that he 
kept his tongue in constant motion in the R. side of his 
mouth, but this ceased some time ago. He has become 
awkward on his feet and cannot use his hands, as he used to 
do. 
Physical strength and general health, excellent. Has 
had apparently slight difficulty in sight. 
Present condition (April, 1887) : Physically well devel- 
oped and in good condition. Is very forgetful and childish, 
weak-minded. Will come across the room to see a watch, 
and smiles at it like a baby. Sits quiet much of the time 
with a vacant expression. Understands fairly well what is 
said to him. At home, likes to sit by the window and watch 
what goes by. Can read the name on a wagon, but does not 
connect any idea with the words, and cannot even do this 
now as well as he could at first. Remembers the boys who 
3 4 
were in his room at school since his injury, but nothing of 
those who were there previous to his injury. Speech slow, 
indistinct. Occasionally omits words. Never uses the 
wrong word. Can use neither hands nor feet well, now. L. 
hand the more helpless. 
Physical Examination : Of average size. Muscular de- 
velopment good. Head of normal size and shape. Scar 
i 1-2 inches long over R. parietal bone. R. abducens seems 
weak. Tongue protruded straight, but seems to move more 
readily to L. Cannot be induced to squeeze with hands, 
but can pull fairly well. L. hemi-paresis. Clonic spasms 
L. arm and face. Incoordination of L. upper extremity and 
probably of L. lower extremity. Stands with legs apart, as 
in hypertrophic paralysis. Waddles in walking, which is 
difficult. Sensation is possibly diminished, as he is said not 
to complain of burns or wounds on hand. He declares, 
however, that he feels the pricking of a pin everywhere on 
the legs readily, and says " prick " when he is touched with 
it. Skin reflexes on trunk and plantar reflexes excellent. 
Knee-jerks alike, exaggerated. 
On the 24th of April, entered the Carney Hospital. His 
eyes were examined by Dr. Standish, who reported R. eye 
veins tortuous, exudation along the arteries and atrophy of 
choroid. L. eye vessels all small. Neuro-retinitis of both 
eyes, most severe on the right. 
Child seems happy. Has a habit of repeating the empha- 
sized words of a sentence spoken to him, and also repeats 
his own words. Temperature morning and evening, nor- 
mal. 
At this time he was seen in consultation with Dr. Bur- 
rell, and it was decided to trephine at the seat of injury in 
the hope of finding some source of irritation, it being evi- 
dent from the child's condition that he was constantly grow- 
ing worse, and the prognosis being distinctly unfavorable, 
unless some change could be induced shortly. 
On the 27th of April, therefore, the patient was ether- 
ized. Slight clonic spasm of L. upper extremity, as patient 
enters under the influence of ether. An oval, somewhat 
excavated cicatrix, 1 1-2 inches long by 3-8 inch, in width, 
WILLIAM N. BULLARD. CORTICAL SCLEROSIS OF THE BRAIN IN CHILDREN. 
5 
is found externally, situated s 3-4 inches to the right of the 
sagittal suture, and somewhat posterior to the line passing 
from one auditory meatus to the other over the vertex. 
The cicatrix was probably over or just posterior to the 
median portion of the ascending parietal convolution. 
The skull was trephined by Dr. Burrell over the cicatrix 
and a large disc removed. The dura-mater appeared nor- 
mal, and there was no bulging. A trocar inserted forwards 
and somewhat inwards, met with no resistance. The bone 
was replaced. The external wound was closed with cat-gut 
sutures. The wound healed readily, and the patient was 
discharged from the hospital. 
There was a slight, temporary cessation of the clonic 
spasms, but no permanent improvement. The only change 
noted while in the hospital was, that he lost the power of 
speech almost entirely. Screams considerably. 
On the 22d of June, two months after the operation, I 
saw the patient at his home. He was found lying on a mat- 
tress placed on the floor. He is unable to get up by him- 
self, but when standing can walk from one room to another. 
After first leaving the hospital, he screamed almost con- 
stantly for two days ; now screams at intervals, if at all ex- 
cited, and sometimes without apparent cause. Cannot talk 
at all now. No change in the special senses noticed. There 
has been, since April, an increasing incoordination and 
paresis, involving all the extremities, but more markedly 
those on the L. 
August 8, 1887. On the whole, no worse than at last 
report. Does not scream much, except when changed after 
wetting clothes. There has now been incontinence of urine 
for some time. 
September 28. Cannot speak. Cannot sit upright. Can- 
not use hands or feet much. Incontinence of urine and 
faeces. Laughs and makes inarticulate sounds when spoken 
to. Recognizes mother. Grinds his teeth constantly. 
Inclines to pull things to pieces. Sleeps quietly, very 
lightly. 
November 7. L. side now (completely) paralyzed. 6 
WILLIAM N. BULLARD. 
Shortly after this, being utterly helpless, and his mother 
no longer being strong enough to lift him, was committed 
by Dr. Jelly and myself, and sent to the Boston Lunatic 
Hospital. There he died a fortnight or so after admission 
in a condition of extreme dementia. 
The autopsy was performed by Dr. Gannett, and it is 
published as No. 77 in the Forty-ninth Annual Report of 
the Superintendent of the Boston Lunatic Hospital for the 
year 1887. 
"Male, 14 years. Autopsy twenty-nine hours after 
death. Body well developed and fairly well nourished ; 
slight lividity of dependent portions ; rigor mortis present. 
There was a horse-shoe shaped scar over the right posterior 
parietal region ; a circular disc of bone had been removed 
and replaced, and it had become firmly adherent to the sur- 
rounding bone ; dura not remarkable. The brain did not 
quite fill the cavity of the skull, and weighed 1,260 grms. ; 
sulci wide ; pia showed a slight degree of thickening and 
opacity ; the meshes contained a moderate amount of thin, 
clear fluid ; vessels at the base and fissure of Sylvius showed 
nothing remarkable. Both lateral ventricles were very 
greatly increased in size. The surface of the ventricles was 
quite smooth and quite tough to the sense of touch. Epen- 
dyma of fourth ventricle markedly granular. Brain sub- 
stance in general, pale and very tough. Both nuclei caudati 
were unusually thin ; the other basal ganglia not remarka- 
ble ; pia readily separable from brain substance. Organs 
of thorax and abdomen showed no variation from the nor- 
mal." 
DIAGNOSIS. 
Wound of earlier trephining. 
CEdema of pia. 
Chronic lepto-meningitis. 
Atrophy of Brain. 
Chronic internal hydrocephalus, secondary to atrophy 
of brain. 
Chronic ependymitis of fourth ventricle. 
No evidence of compression or obstruction of vena 
Galeni. CORTICAL SCLEROSIS OF THE BRAIN IN CHILDREN. 
7 
Microscopical examination. Pia showed a slight degree 
of thickening from growth of fibrous tissue. 
Brain.-First layer of cortex showed the finely fibrous 
net-work with a few spider cells in the mesh, due to atro- 
phy of the nerve fibres and an increase in the neuroglia. 
There was a slight degree of nerve cell infiltration of the 
adventitial sheaths of the blood vessels in the cortex. Be- 
yond this, nothing abnormal was observed. 
The interest in this case consists in part in its rarity. 
Very few cases of primary diffuse cerebral sclerosis in chil- 
dren of this age, have been reported. There are numerous 
examples of cerebral sclerosis, either congenital or occur- 
ring in the earlier years of childhood, but they apparently 
differ somewhat in character from mine. Pathologically, 
too, I believe them to be distinct. Secondary cerebral 
sclerosis due to hemorrhage, tumor, chronic hydrocephalus 
or other cause (embolism or thrombosis, loss of extremities) 
is common. 
Taking simply Moore's case and my own as the type of 
this form, the clinical picture presented is as follows : The 
onset of this disease was in both cases ushered in by a fall 
from which the commencement of the symptoms is dated 
by the relatives. In a varying period after this, there ap- 
pears some intellectual impairment, shown at first simply 
as obtuseness, general dulness and loss of memory. At about 
the same time temporary clonic spasms begin to occur, per- 
haps first affecting the face or tongue, but gradually involv- 
ing the extremities, those on one side of the body being less 
affected than those on the other. These convulsive attacks 
are fora time unaccompanied by loss of consciousness, though 
later in the disease true epileptic seizures may occur. 
Nearly simultaneously with the development of the active 
motor symptoms, there occurs a loss of motor power, some- 
times appearing suddenly as a hemiplegia ; sometimes very 
gradually, but usually involving the extremities on one side 
especially, later those on the other side being likewise af- 
fected. In addition to this loss of power in the extremities, 
we sometimes find a very marked diminution in the power 8 
WILLIAM N. BULLARD. 
of co-ordination or control, likewise somewhat hemiplegic 
in distribution, which in the earlier stages may even mask 
or conceal the paralysis, The speech is soon affected, either 
becoming slow, thick and difficult, or aphasia may occur in 
connection with a right hemiplegia. The loss of power in 
the extremities increases as a rule progressively and gradu- 
ally involves the sphincters, causing incontinence of urine 
and feces. It has, however, never become absolute in any 
case yet reported, but has reached such a point that the 
patient was incapable of sitting up alone or of making any 
more than a slight motion with any extremity. As the dis- 
ease progresses the spasmodic manifestations incline to 
increase in frequency and severity, and we may have epilep- 
toid seizures and opisthotonos. 
In spite, however, of the progressive character of both 
the motor and intellectual symptoms,we find sensory symp- 
toms almost absent. There are no anaesthesia? or paraes- 
thesfe apparent. No pain is complained of and no definite 
loss of sensation has in any case been determined. Unfor- 
tunately we know little or nothing in regard to the condi- 
tion of the muscular sense. Neuro-retinitis or optic neuritis 
occurs early. The other special senses seem usually unaf- 
fected, but as the progressive failure of intellect becomes 
more and more marked, no absolute determination of their 
condition can be made. Like the motor, the mental symp- 
toms progress gradually, but steadily.. 
In the patient under my care, the mental condition was 
throughout that of a simple, rapidly progressive deteriora- 
tion of all the mental powers, a pure dementia, uncompli- 
cated by any intervals of excitement or violence. There 
was no evidence of melancholia ; on the contrary, the pa- 
tient seemed in excellent spirits, happy and contented, until 
he reached the more advanced stages of the disease. No 
hallucinations or delusions were at any time found. The 
mechanical grinding of the teeth, which became nearly con- 
stant at times, and the at least partially unconscious scream- 
ing, which in the later stages was sometimes continued for 
hours, were rather evidences of unconscious or semi-con- CORTICAL SCLEROSIS OF THE BRAIN IN CHILDREN. 
9 
scious reflex actions than of any conscious mental or intel- 
lectual condition. 
The symptoms of this affection, in short, are those of a 
gradually progressive mental deterioration, uncomplicated 
by hallucinatory or delusional conditions and a gradually 
progressive loss of power, involving all the extremities, the 
trunk muscles and the sphincters, a loss of speech and loss 
of sight, accompanied by the symptoms of motor explosion, 
clonic spasms, tonic spasms, and eventually epileptic 
seizures ; while, on the other hand, we find an almost total 
absence of all sensory disorders. In my case, we find a 
very marked co-ordination of the extremities. In neither 
of these cases was there either tremor or nystagmus, both of 
which were present in the case of Schmaus. 
A case related by Turnbull and by him placed under the 
title of general paralysis, commencing in a boy of eight, 
presents symptoms very similar to those of diffuse cortical 
sclerosis. In this case there was less apparent prominence 
of the motor symptoms, although the patient had an attack 
of hemiplegia, which seemed to act as the starting-point on 
which the later dementia was engrafted. The duration of 
the affection in this case was io years. At the autopsy was 
found chronic pachymeningitis, chronic lepto-meningitis, 
external hydrocephalus, ependymitis and atrophy and flat- 
tening of the cerebral convolutions. 
McDowell's case in a lad of 18 is clinically very similar. 
There is no mention of any meningitis at the autopsy, but 
there was atrophy and induration of the whole of both 
cerebral hemispheres, and patches of softening in three 
places. 
We now come to the question of diagnosis. Can this 
affection be accurately diagnosticated during life and dis- 
tinguished from other cerebral affections, with somewhat 
similar symptoms ? 
The most important practical point is to determine in 
any case whether we have to deal with a diffuse process, or 
whether the symptoms presented may possibly be caused 
by some process more or less localized. In certain cases 
this is by no means an easy matter to determine. Where WILLIAM N. BULLARD. 
10 
the explosive symptoms predominate and the paresis is 
slight or unilateral, and especially where, as in my case, a 
cicatrix exists over the motor region on the side opposite to 
that of the hemiparesis, the possibility of a localized start- 
ing-point cannot be excluded. It is the possibility ofa local- 
ized lesion or new growth, accompanied by a chronic or 
subacute inflammation of the meningeal membranes, which 
sometimes renders an absolute diagnosis impossible. A 
cerebral tumor uncomplicated with meningitis would be 
unlikely to produce this combination of symptoms. 
In regard to multiple sclerosis, the differentiation from 
congenital cases and from those forms occurring in epileptic 
idiots is simple, and may be determined by the history. A 
number of cases have been reported, however, by competent 
observers of multiple sclerosis in children, in which the 
symptoms more or less clearly resembled those of the same 
disease in the adult, but so far as I have been able to dis- 
cover, that of Schiile is the only one in which the diagnosis 
was substantiated by the autopsy. At the present time, 
therefore, we can only say that we should expect in uncom- 
plicated cases of multiple sclerosis in children a greater 
similarity to the symptoms of that disease as shown in 
grown persons than to those of diffuse cortical sclerosis. A 
slow development, beginning with nystagamus, tremor and 
paresis, with a distinct subordination, at least until the later 
stages of the disease, of the mental symptoms. 
From the congenital or early forms of cerebral atrophy 
and from all forms of partial or lobar atrophy (excluding 
multiple sclerosis) the history of the case is usually suf- 
cient to exclude. But, in any case, the non-progressive 
character of the disease in these cases forms a-distinction. 
In cases of primary hydrocephalus, so-called, the symp- 
toms are usually of a very different character from those 
just detailed. The presence of rachitis would of itself sug- 
gest this affection. As a rule the mental symptoms are de- 
veloped late, if at all, and the symptoms of cerebral com- 
pression are prominent at some period of the disease. It is, 
perhaps, scarcely needful, however, to mention that hydro- 
cephalus occurs as a secondary condition in diffuse sclerosis. The conclusions that I would draw provisionally are, 
that there exists a form of diffuse cerebral sclerosis in chil- 
dren in which the cortical layers of the brain are more 
especially affected, and which is clinically distinguished 
from the other forms by its appearance in healthy children, 
either without known cause, or after traumata, by the 
steadily progressive character of its symptoms, and by the 
especial prominence of the gradually increasing dementia, 
which finally reaches an extreme degree without a corre- 
spondent loss of motor power, and while the sensation is 
comparatively unaffected. The pathological condition 
found is pathologically undistinguishable from that found 
in adults in general paralysis. 
CORTICAL SCLEROSIS OF THE BRAIN IN CHILDREN 
11