LE JOURNAL CANADIEN DES SCIENCES NEUROLOGIQUES

Pure Spastic Paralysis of Corticospinal Origin
C. M. FISHER

SUMMARY: This presentation includes
six cases of chronic bilateral pure motor
hemiplegia, one of these with pathological findings; one clinical case of chronic
pure motor quadriplegia
and one
pathologically-studied
case of chronic
pure motor paraplegia. These cases may
illustrate a spectrum of pure corticospinal disorders that heretofore has not
been fully recognized.

RESUME: Cet article presente six cas
d'hemiplegie
mot rice pure chronique
bilaterale,
dont un avec
anomalies
pathologiques;
un cas clinique
de
quadriplegic motrice pure chronique et
un cas autopsie de paraplegie motrice
pure chronique. II est possible qu'il
s'agisse ici d'illustrations du spectre non
prealablement
reconnu de desordres
cortico-spinaux purs.

From the Neurological Service of the Massachusetts General Hospital, Boston, Massachusetts
02114, U.S.A.
Presented at the Scientific Session honoring Dr.
R. D. Adams, The Ether Dome, Massachusetts
General Hospital, May 19-20, 1977.
This study was supported in part by NINCDS,
Grant NS05152.
Reprint requests to Dr. C. Miller Fisher, Department of Neurology, Massachusetts General Hospital, Fruit St. Boston 02114, U.S.A.

The nosology of slowly progressive nonfamilial pure corticospinal
disorders remains a matter of uncertainty. The best known of these is
so-called primary lateral sclerosis
with spastic paraplegia. In the literature, references are made to similar
states at higher levels of the neuraxis
giving rise respectively to spastic
quadriplegia, pseudobulbar palsy
and spastic bilateral hemiplegia, but
well delineated clinical examples are
rare and pathological confirmation
still rarer.
In recent years we have encountered 6 patients with an unusual
neurologic illness in which there
slowly developed an extensive spastic paralysis involving legs, arms,
face, and tongue without obvious
involvement of any system other
than the corticospinal tracts and in
the absence of a family history of a
similar condition. In only one case
has a neuropathological study been
made and unfortunately the clinical
course was complicated by a stroke.
These 6 cases which may represent a
syndrome not fully recognized are
the main subject of the present report. In addition, however, in order
to round out the picture, 2 further
cases will be described, the first a
case of spastic paraplegia with
pathological findings and the second
an unusual example of prolonged
spastic quadriparesis.
HISTORICAL SUMMARY
Shortly after Charcot and Joffroy
(1869) delineated amyotrophic lateral
sclerosis, Erb (1875) reported the
occurrence of cases with only a corticospinal deficit and no muscular
atrophy, referring to the condition as
primary lateral sclerosis. Later, Erb
(1902) summarized the literature on
the subject which consisted of 10
pathologically.studied cases showing severe sclerosis of the lateral

Vol. 4, No. 4
https://doi.org/10.1017/S0317167100025075 Published online by Cambridge University Press

columns of the spinal cord from the
lumbar to the cervical region and
often in addition slight lesions of the
tracts of Goll and the direct cerebellar tracts. At least 4 of these cases
were examples of the familial variety. Clinically, the patients were described as slowly developing a spastic paraparesis with increased tendon reflexes and Babinski signs
without bladder dysfunction or sensory changes. In some cases the
tendon reflexes of the arms were
enhanced. The age of onset (and the
duration of the illness) in the autopsied cases were as follows: 43 years
(20 years), 37 (25), 42 (20), 52 (4), 8
(20), 10 (20) and 1 of unstated age
with symptoms for 35 years. Spiller
(1904) reported a case (case 6) in
which there was isolated spastic
paralysis of the legs for 4V2 years.
The crossed and uncrossed pyramidal tracts of the spinal cord were
distinctly pale. Stark and Moersch
(1945) recorded 43 cases of nonfamilial primary lateral sclerosis, none
with pathological study. In addition
to a spastic paraparesis, 28 had
hyperactive reflexes in the arms, 8
had increased tendon reflexes and
functional impairment in the arms
and 7 showed no involvement of the
arms. Wechsler and Brody (1946)
described 15 cases of slowly progressive primary lateral sclerosis in
which the disease remained in the
lower extremities without involvement of the arms or brain stem after
follow-up periods of up to 12 years.
Only two had sphincter disturbances. Through the years, primary
lateral sclerosis has generally been
regarded as a variant of amyotrophic
lateral sclerosis or motor neuron
disease in which anterior horn cell
disease appeared later or not at all,
the condition lying at the opposite
end of the spectrum from progressive muscular atrophy in which cor-

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ticospinal tract sclerosis failed to
develop. Mackay (1963) is representative of this view in his claim that
primary lateral sclerosis is simply
amyotrophic lateral sclerosis without, as yet, its complement of atrophic change which is always added
unless (rarely) death supervenes. He
followed 11 cases of primary lateral
sclerosis for years and found that 10
eventually
showed
atrophic
changes, only one remaining purely
spastic. Not included in his main
study were 3 patients whose spastic
paresis persisted uncomplicated for
21, 14 and 8 years respectively.
An illness closely resembling
primary lateral sclerosis and which
ran in families was initially described
by Strumpell (1886). Apparently it is
more frequent, for Paskind and
Stone (1933) in 1933 found references to 151 families with more than
one
member
with
spastic
paraparesis. In the familial illness
the predominant clinical findings are
spasticity of the legs but there also
are instances of involvement of the
arms, pes equino-varus, cerebellar
ataxia, tremor, dementia, optic atrophy, muscle wasting and scoliosis,
indications
of a link
with
Friedreich's ataxia or other hereditary ataxias. Roe (1963) found 176
reports of 250 families with the
hereditary form. Schwarz (1952)
summarized from the literature 24
cases of familial spastic paralysis in
which there were pathological
studies. Of the 24 cases, only 7 were
typical examples of a pure corticospinal deficit. Pathologically, in these
typical cases there were no changes
in the internal capsule, cerebral
peduncles, pons or medulla. The lateral corticospinal tracts were symmetrically involved in all cases,
chiefly at the thoracic level, less at
the upper cervical level. The uncrossed pyramidal tract was involved in one half the cases. The
fasciculus gracilis was symmetrically demyelinated in the cervical
region. It is possible that sporadic
primary lateral sclerosis is an isolated manifestation of a familial trait,
rather than part of motor neuron
disease.
It should be emphasized that in
the non-familial group pathological

studies are available in only a few
cases and in none since 1905. Most
cases are from the period before the
discovery of the myelopathy of cervical spondylosis and before the
routine use of myelography. Also
chronic multiple sclerosis, spinal angioma, vascular disease, slowly
growing tumor, atlanto-occipital
anomalies, and the Arnold-Chiari
malformation may not have always
been satisfactorily excluded. On the
other hand, in response to the claim
that all pure corticospinal disease is
but amyotrophic lateral sclerosis in
evolution, one might ask for more
clinical details of the patients who
were followed for years and finally
developed atrophic changes. How
long did the purely spastic state persist before atrophy occurred? Which
parts developed atrophy? What was
the pace of evolution? Was a
pathological study obtained? Such a
presentation, which is not available
in the literature, would assist the
clinician in classifying his cases.
Compared with that of primary
lateral sclerosis, the history of the
other pure corticospinal disorders is
brief. Chronic progressive
spastic
quadriplegia is vaguely alluded to in
discussions of primary lateral
sclerosis in which the arms become
involved, but no specific case has
been recorded. Chronic progressive
spastic bulbar paralysis has been
described by Aird (1962) as an illness
occurring in early middle life, frequently in association with slight
spasticity of the limbs and hyperactive deep reflexes and is classified as
a variant of amyotrophic lateral
sclerosis. The entity of pure spastic
dysphonia
or dysarthria
is not
clearly set forth in the literature.
Chronic
progressive
double
hemiphegia
is represented by a
single but striking case report. Buzzard and Barnes (1906) reported the
case of a woman, aged 53, who for
10 years had a slowly progressive illness beginning with uncontrollable
laughter and progressing to dysarthria and finally weakness of the
legs. The patient walked with a stiff
gait scraping the floor, rapid movements of the fingers and tongue were
slowed, there was no muscular atrophy, the reflexes were brisk and

252 - NOVEMBER 1977
https://doi.org/10.1017/S0317167100025075 Published online by Cambridge University Press

the plantar responses were extensor.
Inappropriate spasms of laughter
were prominent. There was no history of a stroke. The patient died
suddenly after being observed in the
hospital for about 4 months. On
neuropathological examination the
pyramidal tracts showed in the
myelin stain sclerosis and pallor
throughout their length — the anterior % of the internal capsule, the
fronto-pontine fibers of the cerebral
peduncles, medullary pyramids and
posterior portions of the lateral columns of the spinal cord from the
cervical to the sacral levels. The
ganglion cells of the cortex may not
have been as large as usual. No
other part of the brain was involved
and the anterior horn cells of the
spinal cord were normal. There was
severe arteriosclerosis of the penetrating arteries of the brain and the
authors concluded that their case
was one of chronic progressive double hemiplegia secondary to the ischemia of vascular disease rather
than of a primary nature. The illustrations are particularly lucid. The
crossed corticospinal tracts were not
involved. Swank and Putnam (1943)
stated that in one of their 21 cases of
primary lateral sclerosis pseudobulbar palsy with emotional lability developed later. Chronic progressive
single or unilateral hemiplegia has
not been described as an entity.
CASE MATERIAL
A. Chronic progressive spastic double hemiplegia — chronic bilateral
pure motor hemiplegia — Chronic
progressive
bilateral
spinobulbar
spasticity.
The six cases described below resemble one another sufficiently to
suggest they represent a recognizable clinical syndrome. A somewhat
serious weakness lies in the fact that
only one case has been studied
pathologically and in that case the
occurrence during the course of the
illness of a pontine hemiplegic stroke
complicated both the clinical and the
pathological aspects. In the remaining five cases, common neurological
disease has been largely excluded,
but the possibility that atypical processes, for example, unremitting
multiple sclerosis, were the cause of
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LE JOURNAL CANADIEN DES SCIENCES NEUROLOGIQUES
the syndrome in some cases is a
concern. Only further pathological
studies will allow more accurate
conclusions. In no case was the
course of the illness marked by exacerbations and remissions.
Case I
The patient began to have stiffness of the
legs, poor balance and difficulty walking at
the age of 67. Within 10 months he began to
use crutches. After 17 months the arms became progressively weaker. The thighs and
legs ached anteriorly and posteriorly. A
myelogram after 15 months was negative. At
the time of onset, the patient had developed
an acute low backache while lifting. There
was a past history of migraine headaches and
of a suprapubic prostatectomy at the age of
62. Examination at the age of 68. 20 months
from onset, showed an exceedingly severe
spastic paraplegia with the legs in rigid extension. The arms were slow and weak. The
tendon reflexes were abnormally brisk and
the plantar responses were extensor. The
cranial nerves and sensation were normal.
There was no dysarthria. The blood pressure
was 175 systolic. The patient gradually got
worse and at the age of 69. 2xk years from the
onset, suddenly developed a severe left hemiplegia associated with dysarthria and dysphagia. Within 1 week he began to walk with
assistance but he never quite recovered to the
prehemiplegic level. After a period of further
improvement, the quadriparesis grew gradually and steadily worse. The low backache
continued. One episode occurred in which the
weakness of the arms and legs markedly increased, with the result that the patient could
scarcely move. The exacerbation had subsided by the next morning. Examination at the
age of 70 (9 months after the hemiplegic
episode) showed spastic dysphonia and severe dysarthria and speech was described as a
barely articulated monotone. There were
spasms of inappropriate rapidly reversing
laughter and crying. Movements of the face,
tongue and all limbs were slow. The limbs
were severely spastic. Using a metal-framed
walker, the patient advanced each foot a few
inches at a time. The tendon reflexes were
brisk and the plantar responses extensor.
Sensation was normal. The ocular movements were normal without nystagmus. The
mind was well preserved. The optic discs
were sharply outlined and of normal color.
There was no cerebellar ataxia. There were
no grasping or sucking reflexes. The Romberg
test was negative.
The hemoglobin was 15.0 gms. and the
WBC 8,100. A urinalysis was normal. There
was gastric free acid. The CSF was under a
pressure of 123 mm.and contained 38 mg.% of
protein and no WBC. The colloidal gold test
was 0012210000. A 64Cu scan was negative.
An EEG was normal. The patient lived in a
nursing home for 2 more years, dying at the
age of 72, 5 years from onset, of a myocardial
infarction. There were no further recognizable strokes. The arms and legs remained
spastic but the patient was able to feed himself and move a wheel chair despite the

CM.

marked spasticity of the arms. His intelligence was preserved. He could not speak
because of articulatory difficulty and communicated by writing notes.

Neuropathological Findings: Grossly there was an old infarct in the
right basis pontis at the junction
of the upper MJ and lower % of
the pons. The infarct consisted of
4 small cavities lying in an anteroposterior line in the paramedian
area a few mm. from the midline.
The right medullary pyramid was
reduced in size. These were the only
gross abnormalities. Sections were
prepared and stained by several
methods including Nissl, myelin,
Scharlach R, Holzer and Bodian. On
microscopic examination the internal capsules and midbrain peduncles
appeared normal. There were no
scattered lesions to suggest multiple
sclerosis. The giant pyramidal cells
of the motor cortex were probably
reduced in number. In the pons the
series of old small cavitating infarcts
in the right paramedian area were
obvious. Both medullary pyramids
were pale in the myelin stain, the
right more than the left. (Figure 1) In
Cajal and Bodian preparations large
fibers were greatly reduced in the
pyramids but gliofibrillar material
was not increased. In the Scharlach
R stain there were no fatty macrophages but myelin was less prominent than in control cases. The cerebral cortex, putamen, globus pallidus, thalami, substantia nigra and
brain stem tegmentum were normal.
In the spinal cord there was pallor
in the region of the lateral corticospinal tracts of the entire cord from
the upper cervical region to the lumbosacral level. (Figures 2 and 3) The
right anterior uncrossed corticospinal tract was pale but the left stained
well. The anterior horn cells at all
levels were preserved in number and
structure. The anterior rootlets in
the cervical and lumbosacral regions
were not demyelinated.
Comment: The pontine paramedian
infarct accounted satisfactorily for
the patient's acute stroke three years
before death. It probably was also
responsible for the greater pallor of
the right medullary pyramid. It
would not account for the striking
pallor of the lateral corticospinal

Fisher

https://doi.org/10.1017/S0317167100025075 Published online by Cambridge University Press

tract bilaterally. Furthermore, the
clinical course was a slow gradual
bilateral progression punctuated
only once by a sudden acute onset
from which good recovery occurred
in keeping with a vascular insult.
The preservation of power in the
limbs late in the illness and the
maintenance of the intellect were
noted in the other cases. It was
therefore concluded that vascular
disease was only a complicating factor and the true nature of the illness
was of an entirely different type.

Figure I—Case I. Section of medulla
oblongata (Cajal with myelin counter
stain) showing marked pallor of right
pyramid, lesser pallor of the left.

Figure 2—Case I. Section of cervical
spinal cord (myelin stain) showing pallor of corticospinal tracts lateral and
anterior on the right side, lateral only
on the left side.

Figure 3—Case 1. Section of thoracic
spinal cord (myelin stain) showing pallor of corticospinal tracts.

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Case 2
The patient, a male engineer, at the age of
61 first noted loss of coordination of the legs
and inability to run as he ordinarily would. He
was no longer able to balance on his bicycle.
Soon he began to drop things from his hands
and finger movements became slowed. The
disability in the arms and legs slowly and
gradually progressed. He tended to tire easily
and fell frequently. At the age of 65, slurred
speech was first noted. He said his speech
was as if he had to force it out. At the age of
66 he could walk on a treadmill for 40 minutes
at 1xh miles per hour and at the age of 67 only
21 minutes at 2 miles per hour. He thought
that at the beginning of his illness the left leg
and arm were more affected than the right.
When seen at the age of 67, 6 years from the
onset, he had recently noted the appearance
of slight dysphagia, a lowered pitch of the
voice and nocturnal cramps in the legs. Bladder and bowel function were normal. A definite lability of laughter and crying had appeared.
On examination (age 67) there was a definite dysarthria with an element of spastic
dysphonia. He could easily be understood.
There was no nystagmus. La, la, la was
greatly slowed. There was a bilateral
corneal-pterygoid reflex. All limbs were moderately spastic, left more than right. Motor
power was approximately normal when the
patient was given time to make a full exertion.
He could step up on an 8" stool. He could
support himself on the heels or toes. There
was no atrophy or fasciculation. The tendon
reflexes were all brisk and a Babinski response was present bilaterally. The jaw jerk
was absent. Sensation was intact to pinprick,
vibration, touch and joint position sense.
There was no tremor or cerebellar ataxia. He
swayed 2" on the Romberg test. The patient
walked with a slow, spastic gait, scraping the
toes, giving the appearance of wading in water
up to the mid-riff. There was lability of laughter and lacrimation.
An extensive laboratory investigation including myelography, arteriography and CSF
examination was normal.
When reexamined 2 years later (age 69) all
symptoms had progressed. People had difficulty understanding his speech. Swallowing
was more impaired. He could walk on a
treadmill for only 2 minutes at IVi miles per
hour. At night the legs prickled as if a
thousand needles were being jabbed into
them. Nocturnal cramps had continued. The
neurological signs had changed only in degree. Speech was more dysarthric and slowed
(3 words per second), the face moved symmetrically. Motor power was still approximately normal. Maximum rapidity of movements was greatly decreased. Intelligence was
preserved. In the next 3 years there was a
further slow decline, but the patient was still
able to indulge in his hobbies 11 years from
the onset of the illness.

Case 3
The patient, a male sales-manager, first
noted the onset of a progressive clumsiness of
the legs and arms at the age of 61. For the first

2 years, the left arm and leg were predominantly affected, while in the 3rd and 4th years,
the right side became involved. In the 5th year
speech became slurred to an extent, it made
the patient "hang his head in shame". The
legs were stiff and balance so poor, the patient
often fell. On going down stairs, his knees
were on the verge of bucklipg. Cramps occurred in the leg muscles at night. A definite
lability of laughing and crying had appeared.
At the onset of the illness, the left index finger
was numb and this persisted. Bladder and
bowel function were preserved. The patient
had become stooped and feeble. Intelligence
was preserved.
On examination at the age of 66 (in the 5th
year) there was only a slight dysarthria. There
was no nystagmus. The optic discs were
normal. The patient gave the general appearance of rigidity of the trunk and neck. La, la,
la was performed slowly. The jaw jerk and
facial reflexes were very brisk. Motor power
in the arms and legs was approximately normal when the patient was given time to make
a full effort. There was no atrophy or fasciculation. There was moderate spasticity in all
limbs with increased tendon reflexes and a
Babinski sign bilaterally. Sensation was intact
to pinprick, touch, joint position sense and
vibration. The gait was slow, jerky and spastic. The Romberg test was normal.
Extensive laboratory investigation including myelography, cerebral arteriography,
CSF, EEG and EMG were normal.
There was slow progression in the next 5
years without new neurological findings.

Case 4
The patient, a woman, suddenly at the age
of 68 developed a left hemiplegia involving
face, arm and leg from which recovery was
excellent in about 3 weeks. At the age of 69
she complained of slurred speech, hoarseness
and a gradual loss of voice with talking.
Examination showed slight dysarthria, slowness on the la, la, la performance and weakness in placing the tongue in each cheek.
Power in the right hand grip was 7/10, in the
left 8/10. In the next 3 years speech became
gradually and slowly worse and walking more
difficult without further recognizable episodes
of cerebral ischemia. On examination at the
age of 72 speech was slurred almost to the
point of being unintelligible and the tongue
could not be placed in either cheek, but there
was no atrophy. The mouth hung open. There
was no buccal-Ungual dyspraxia. There was
no nystagmus. Swallowing was a little difficult. The jaw jerk was 4-K There was a
tendency to inappropriate grinning. The facial
jerks were 3+. Power in the arms and legs
was approximately normal. The limbs were
spastic, the tendon reflexes all very brisk and
Babinski and Hoffman signs were present
bilaterally. Finger tapping was greatly
slowed. There was no tremor or cerebellar
ataxia. There was no definite sensory loss
except for decreased vibration below the
mid-shin. The patient walked slowly on a
wide base, using a metal-framed walker. She
could not stand by herself. Mind and judgment were unimpaired. The blood pressure
was 200/90.

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In the next 3 years the patient became
progressively more helpless. On examination
at the age of 75 she was unable to speak at all.
There was moderate dysphagia. She communicated by means of laborious writing. The
mouth hung open and the lips were held in a
position of constant smiling. There was no
nystagmus. Eye closure was strong, whereas
power of lip closure was zero. The lips moved
well in laughter. There was a definite inappropriate lability of laughing and crying. The
tongue moved a few mm. from side to side.
Tone was moderately increased in all limbs
and the tendon reflexes were brisk. There was
a bilateral Babinski sign. When the patient
was given time to exert full effort there was
good power in all limbs. Sensation was unchanged from previously. There was no tremor or cerebellar ataxia. The patient knew
the date and the stock market quotations of
the day. The CSF was under a pressure of 170
and contained 32 mgs. of protein and no
WBCs. The gamma globulin was 2.5%. A
pneumoencephalogram showed a ventricular
span of 40 mm. and slight widening of the
quadrigeminal cistern, suggesting mid-brain
atrophy. During the following 1V4 years there
was a further decline and at the age of 76 the
patient sat with her head hanging and chin on
chest. The mouth hung open 1". The patient
smiled broadly. The tongue protruded only to
the line of the teeth. The patient drooled and
made unintelligible laryngeal sounds. There
was no nystagmus and the patient could not
turn her eyes from side to side on spoken
command. She could very slowly bring a
coffee cup or a napkin to her mouth. She
wrote slowly at about one letter every 3
seconds. She chewed and swallowed slowly
but seldom choked. There was a remarkable
clasp-knife spasticity of all limbs and a
Babinski sign bilaterally. There was no tremor or cerebellar ataxia. Sensation in the
fingers and toes was within normal limits for
the patient's age. She was continent without
urgency. The left leg involuntarily extended
at the knee for about 3 seconds at a time. The
patient died at the age of 76 from gastroenteritis and perforation of the bowel.

Case 5
The patient, a woman journalist, noted at
the age of 66 that her speech was slurred and
less rapid whereas normally she spoke "like a
cannonball." The impairment gradually progressed and at the time of examination 3 years
later at the age of 69 she said she spoke "like a
3-year-old child separating the syllables."
Her throat became tired when she talked.
Speaking became an effort and she had to
"push" her voice which seemed to be higher
pitched. At times there was stridor on inspiration. In the previous few months inappropriate lability of laughter and crying had appeared. Six months before examination the
right eyelid drooped slightly. For 6 weeks
there had also been difficulty and pain in
swallowing. Occasionally she saw slightly
double. The arms and legs were reported as
normal but typing had become inaccurate and
slow and whereas the patient had formerly
swum a V6 mile daily, she now could scarcely
swim 6 strokes. Climbing the stairs was dif-

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ficult. Six weeks before, she developed back
pain at the TI2 to L2 level. In general, the
patient thought she had slowed up greatly in
the previous 2 years.
Neurological examination showed slow, indistinct, slurred, precise speech. The extraocular movements were full without nystagmus. The orbicularis oculi was strong bilaterally but there was a right lower facial weakness. La, la, la and me, me, me sounds were
greatly slowed. Serial expiration was difficult.
The tongue was strong and free of atrophy.
The palate elevated poorly. There was no
obvious ptosis. The jaw jerk was 3+ and the
facial reflexes were enhanced. Power in the
arms and legs was approximately normal. The
tendon reflexes were increased, particularly
on the right side, and the plantar responses
were flexor. Sensation to pinprick, vibration,
touch and joint position sense was normal.
Rapid alternating movements were well performed. The Romberg test was negative and
the patient could walk tandem in a straight
line. Her intellect was preserved. The CSF
was under a pressure of 22 mm. of water and
contained 72 mg.% of protein.
In the following year speech became more
impaired and rapid movements of the fingers
and toes became slowed. All tendon reflexes
were brisk but the plantar responses were
flexor. There was slight weakness in the right
upper extremity. There was no tremor or
cogwheeling.
Another year later, at the age of 71. speech
had become almost unintelligible. The right
arm had become weak and of little use. The
left arm could not be used for fine tasks but
served for eating and dressing. Gait was slow
and the patient had fallen many times. Dysphagia was slowly advancing. There had been
no tingling or numbness. Hearing was normal.
Her mind was intact. The extraocular movements were full without nystagmus. There
was great difficulty in moving the tongue from
side to side. There was difficulty in turning
the eyes from side to side on command. There
was a moderate right facial weakness. Jaw
closure was weak. The tongue moved slowly
but was not atrophic. The jaw jerk was increased.
Motor power was good in resisting passive
movements but the left arm and leg were
stronger than the right. All movements were
greatly slowed. There was a clasp-knife spasticity of all extremities. The tendon reflexes
were hyperactive and there was a Babinski
sign bilaterally. Sensation was intact to pinprick, vibration, touch, joint position sense
and stereognosis. There was no tremor, cerebellar ataxia or cogwheeling. The patient
walked with 14" slow shuffling steps. The
Romberg test was negative. An EEG was normal. An EMG showed no evidence of lower
motor neuron disease. Cortical function tests
showed retention of her mental ability. The
blood pressure was 122/76.
The patient committed suicide 3 months
later.
Case 6
The patient, a woman, first noted slurred,
slowed, deliberate speech at the age of 50.
During the next year she began to drag the

right leg and in the following year the left.
Thereafter, there was a slow gradual progression of gait impairment without remission. At
the age of 56. handwriting became slowed,
buttoning difficult and rapid movements of
the fingers impaired. Dysphagia occurred on
drinking or eating too quickly. On neurological examination at the age of 60, 10 years from
the onset, speech was slow and slurred and
had a quality of spastic dysphonia. It could be
readily understood. La. la. la and me. me. me
were greatly slowed. The patient walked slowly scraping the toes on the floor in typical
spastic fashion. She could take steps by herself
but preferred to hold on. Power in the limbs
was within normal limits, both proximally and
distally when the patient was given sufficient
time to exert full force. She could step up on
an 8" stool with either leg. The arms were
moderately spastic, the legs much more so.
The tendon reflexes were all extremely
hyperactive and Babinski signs were present
bilaterally. Ankle clonus was present bilaterally. There was no atrophy of muscles. The
voice was hoarse but the patient thought this
was normal. The jaw jerk was brisk but the
facial reflexes were not increased. There were
slight mirror movements of the left arm when
the right arm was pronated and supinated.
Sensation was intact to pinprick, vibration,
touch and joint position sense in the fingers
and toes. There was no tremor or cerebellar
ataxia. The most rapid movements of the
limbs were greatly slowed. There was no
nystagmus. The optic discs were of good
color. The intellect was preserved. There was
inappropriate ease of laughter and crying.
There was no urgency or incontinence of
bowel or bladder. There had been no diplopia.
There had been no numbness of the fingers or
toes except occasionally for a few minutes.
Numerous laboratory tests including EEG,
EMG. carotid and vertebral angiography,
CSF and brain scans were all normal.
COMMENT
The cases included 3 males and 3
females, ranging in age at onset from
50 to 69 years, 5 being in the 60s. In
all 3 women the illness began with
dysarthria and advanced to involvement of the limbs, whereas in the
men, the opposite was the case. The
tempo of the gradually progressive
process was variable and led to
severe disability in the following
periods respectively — 5 years, 8
years, 7 years, 6 years, 5 years and
in case 6 the disability was only
moderate after 10 years. The final
picture was stereotyped — spastic
quadriparesis, hyperactive tendon
reflexes, a Babinski sign bilaterally,
severe dysarthria and moderate dysphagia with preservation of power to
resist passive motion. Lability of
laughing and crying occurred in all
cases. There was no sensory loss,

C. M. Fisher
https://doi.org/10.1017/S0317167100025075 Published online by Cambridge University Press

cerebellar incoordination, nystagmus, tremor, impairment of eye
movements, bowel and bladder dysfunction, muscle atrophy or fasciculation. The intellect was preserved.
There was no clinical or laboratory
evidence of anterior horn cell involvement. Intrinsically, the illness
does not appear to be fatal, and the
causes of death in the 3 cases dying
were myocardial infarction, perforation of the bowel and suicide respectively.
Judging from the pathological findings in case 1, there is a loss of fibers
and myelin in the corticospinal system bilaterally, traceable from the
lumbar level upwards through the
spinal cord to the level of the medullary pyramids but not higher. The
fasciculus gracilis may have been a
little pale at the cervical levels. In
the available sections of the cerebral
cortex the giant cells of Betz were
probably reduced. The anterior horn
cells of the spinal cord were intact.
Regarding the nature of the illness
the process corresponds to amyotrophic lateral sclerosis in which anterior horn cell disease is missing.
However, if the disease were a variant of amyotrophic lateral sclerosis,
one might expect to encounter intermediary forms in which amyotrophy is added to spasticity after a
period of several years, but this has
not been our experience. The relative benignity of the process would
indicate that it is lower motor neuron
disease that is the limiting factor in
survival in amyotrophic lateral
sclerosis. There is no advantage in
forcing the illness into the category
of amyotrophic lateral sclerosis and
indeed a good case could be made
for regarding it as a variant of primary lateral sclerosis. The illness
faintly resembles progressive supranuclear palsy but lacks supranuclear ophthalmoplegia, axial dystonia
in extension, rigidity of the limbs
and dementia. It might be added
here that in the 6 cases, urinary and
blood screening tests for amino acid
abnormalities were negative.
B. Chronic progressive
spastic
quadriplegia.
There is only one patient in this
subcategory, an unusual case of

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progressive quadriparesis in which a
severe disability developed over a
period of 6 years and then remained
stationary for the next 9 years.
Case 7.
A male civil engineer first noted the onset
of progressive difficulty walking at the age of
50. The right leg was involved first and the leftleg about 3 months later. After 9 months he
began to use a cane. After 18 months the arms
became involved, in that writing was difficult.
All limbs became progressively slow, stiff and
weak. At the time of neurological examination
3Vi years from the onset, the patient could
stand but scarcely move the legs. The right
arm was slightly useful, the left arm almost
useless. Flexor spasms of the legs occurred at
night and on standing the legs tended to
scissor. Urinary bladder function varied from
urgency and occasional incontinence on the
one hand to comfort for as long as 20 hours.
Bowel and sexual functions were normal.
Shortness of breath occurred after talking.
There had been no pain, numbness, dysarthria or dysphagia. The patient reported a
striking variation in the disability and in the
earlier stages sometimes he was able to walk
fairly well or climb stairs, for approximately 4
hours at a time. At first the right leg would
become heavy after walking 400 meters with
full recovery after 20 minutes of rest. His
voice had reduced volume.
On examination all limbs were extremely
spastic. There was, however, surprisingly
good strength in resisting passive movements
at the shoulders, elbows, wrists, hips and
knees. The hand grips were approximately
7/10 on the right and 6/10 on the left. The right
hand was useful for gripping a cane. The
tendon reflexes were all greatly increased and
there was a bilateral Babinski response. The
jaw jerk was 1+ and there was no suck or
grasp response. Sensation was intact to pinprick, vibration, touch, joint position sense
and cold in the upper limbs and in the lower
limbs except that vibration was felt for 11/20
seconds at the right great toe and was absent
at the left great toe. The knee jerks were 4+,
the ankle jerks 2+. Shrugging the shoulders
was greatly slowed. Periodically there occurred strong extensor spasms of the legs.
Cranial nerve function was approximately
normal. The pupils were 3 mm. and reacted to
light. The extraocular movements were full
without nystagmus. The fundi and discs were
normal. The right side of the face was questionably weak. The tongue was strong without
atrophy. Rapidity of tongue movements was
not slowed. There was no cerebellar ataxia or
tremor. Intelligence was intact. The blood
pressure was 120/70.
The laboratory tests were as follows:
Hemoglobin 14.2, white blood count 9,100.
Urinalysis normal. Sedimentation rate 12.
Urine and blood tests for abnormal amino
acids were negative. An eiectromyogram
showed no fibrillations or fasciculations in
arms or legs. A cervical myelogram was
normal. The CSF was clear and colorless,
contained no WBCs and the protein was 50
mg.%.

The patient slowly became more disabled in
the following 2 years. Thereafter, however,
although almost completely paralyzed in the
arms and legs, he remained on a plateau
enjoying otherwise good health with preservation of mind, speech and swallowing. At the
age of 65, 15 years from the onset, he was being cared for in a chronic hcspital where he
was occupied reading the works of Sir Winston
Churchill utilizing an electronic page-turner.
(We are indebted to Dr. John Armstrong for
the follow-up information that no muscle attrophy has occurred.)

Comment: Although only the clinical
details are known the illness appears
to be unique and not easy to fit into
the ranks of any known neurological
disease. Is it doubly atypical amyotrophic lateral sclerosis with neither
muscle atrophy nor bulbar involvement? This is conceivable. Or is it
"primary lateral sclerosis" with
higher spinal cord involvement than
in the reported cases. Striimpell
(1901) referred to cases of spastic
paralysis of the arms and legs at an
advanced stage associated with a
slight muscle atrophy and thought
the process merged with amyotrophic lateral sclerosis. However,
cervical spondylosis was not recognized in that era.
If the present syndrome and primary lateral sclerosis are related to
amyotrophic lateral sclerosis, it
might be anticipated that cases of
amyotrophic lateral sclerosis would
be found in which there is uppermotor-neuron involvement of only
the lower extremities, sparing the
arms and tongue, or of the lower and
upper extremities, sparing the tongue.
Yet pathologically, demyelination of the internal capsule occurs in
almost all cases of amyotrophic lateral sclerosis (Lawyer and Netsky,
1953). This case of pure quadriplegia
illustrates again that, as in primary
lateral sclerosis, corticospinal tract
disease may "burn itself out" following which survival is prolonged.
C. Pure spastic paraparesis.
The case to be described resembles primary lateral sclerosis in
many ways but is atypical in the age
of the patient and the short duration
of the process at the time of death. A
cervical myelogram ostensibly excluded compression myelopathy.
The pathological study is the only
one reported in many years.

256 - NOVEMBER 1977
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Case 8
The patient, a retired abstemious
machinist, noted the insidious development of
increasing stiffness of the legs at the age of 77.
At the time of onset, there was a dull aching
pain in the paravertebral region on the right
side between the neck and the mid-thoracic
level and a few weeks later a similar pain on
the left side. Examination 6 months from the
onset showed much difficulty in getting to the
feet and a striking slowness in walking, which
was a great effort. The gait was stiff-legged
and of the circumduction type. The cranial
nerves were normal and there was no nystagmus or dysarthria. The arms were normal
except for brisk tendon reflexes. The legs
were essentially normal in strength despite
great spasticity. There was no cerebellar
ataxia. The sphincters were intact. Sensation
was normal. Using a biothesiometer, vibration was slightly reduced below the knees.
Intelligence was preserved. The blood pressure was 110/70.
The hematocrit was 41 and the WBC 6,850.
The sedimentation rate was 11. Urinalysis
normal. A gastric aspirate contained free acid.
The CSF dynamics with the head in neutral,
extended and flexed positions were normal.
The CSF was clear, contained 63 mg.% of
protein and the colloidal gold was 1122211000.
A myelogram showed moderate cervical
spondylosis without cord compression. An
EMG of both legs and the left arm was
normal. Skull x-rays were normal. An EEG
was slightly abnormal. Hinton tests of the
blood and CSF were normal. The jaw jerk
was 1+. Flexor spasms of the legs were
troublesome. Frequency and urgency of urination began, without incontinence.
During the following year, a slow gradual
progression of the difficulty in walking occurred but the patient was still able to walk with a
metal-framed walker. Flexor spasms continued to occur both day and night. Six
months after discharge, the patient noted a
prickling numbness of the fingers of the right
hand, beginning in the tip of the thumb and
index finger and progressing towards the 5th
finger. The tingling involved only the distal
two phalanges. Buttoning and writing were
difficult because of the sensory impairment.
Because of bladder incontinence an indwelling Foley catheter was inserted. Examination
Wi years from the onset showed increased
stiffness, spasticity and disability. The tendon
reflexes in the arms were 2+, the knee jerks
3 + and there was a bilateral Babinski response. Pinprick and light touch were diminished in the fingers of the right hand but
vibration and position sense were intact. It
was clear that there had been progression in
the previous year.
Two months later the patient died from
chronic bowel obstruction which proved to be
due to a volvulus.

Neuropathological Findings: Sections were prepared of the sensorimotor cortex, several other regions
of the cerebral cortex, thalamus, internal capsule, midbrain, pons,
medulla and 27 levels of the spinal
Corticospinal Spastic

Paralysis

LE JOURNAL CANADIEN DES SCIENCES NEUROLOG1QUES

Figure 4—Case 8. Section of cervical
spinal cord (myelin stain) showing
faint pallor of lateral corticospinal
tracts.

Figure 5—Case 8. Section of thoracic
spinal cord (myelin stain) showing
marked pallor of corticospinal tracts
and a more generalized slight pallor of
the entire cross-section except the
posterior columns.

Figure 6—Case 8. Section of lumbar spinal cord (myelin stain) showing pallor
of corticospinal tracts.
cord. Staining methods included
Nissl, myelin, Bodian, Holzer and
Scharlach R. There was no abnormality of the cerebrum and brain
stem. At the CI level there was a
small but definite zone of pallor in
the posterior portion of the lateral
column in the region of the corticospinal tract bilaterally. At the C8 level
the posterior part of the lateral compartment was extensively pale. (Fig-

Figure 7—Case 8. Section of medulla
oblongata (myelin stain) showing normal staining of pyramids.
ures 4, 5, 6, 7) At T6 this was even
more pronounced. The uncrossed
corticospinal tracts were intact. At
L2, L4 and SI levels a well demarcated zone of corticospinal pallor
was obvious on myelin stains. The
anterior horn cells and the anterior
rootlets at all levels were well within
normal limits. Scharlach R staining
disclosed a scanty collection of fatty
macrophages at the C8 level and
none higher. At the Tl level bilaterally in the area of the myelin pallor,
there was an abundance of Scharlach R-positive fat-filled macrophages. In addition, there was an
abundance of what appeared to be
free Scharlach R-positive-material
collected aroung small blood vessels. A similar appearance was present throughout the entire thoracic
cord and disappeared at the L2 level.
The great number of fatty macrophages at the Tl level compared
with their scarcity at C8 was most
striking and suggested a focal process acting in this region of the
spinal cord, for example, cord compression. However, there was no
evidence of ascending degeneration
in the spinal cord, the uncrossed
corticospinal tracts were intact and
in life a cervical myelogram had
been negative. There was no evidence of multiple sclerosis.
Comment: The slow gradually progressive development of a severe
spastic paraplegia in the absence of a
sensory deficit makes the case a
good candidate for the diagnosis of
primary lateral sclerosis. Electromyographic studies showed no
evidence of anterior horn cell disease. Numbness of the fingers of the

C. M. Fisher
https://doi.org/10.1017/S0317167100025075 Published online by Cambridge University Press

right hand and increased tendon reflexes were the only signs of involvement of the arms. Involvement
of bladder function was atypical.
The pathological findings of selective demyelination of the lateral corticospinal tracts below the C8 level
and slight demyelination of the fasciculus gracilis have been reported
in two cases of familial primary lateral sclerosis (Striimpell, 1901; Bischoff, 1902) and one case of the
sporadic form (Dejerine and Sottas,
1896). The possibility that the combination represents descending and
ascending degeneration due to a cervical cord lesion cannot be discarded
entirely despite the absence of a
spinal block and cord compression
by myelography. However, if compression of the cervical cord occurred evidence of it should have been
observed in at least one level of the
cervical cord.
We have had a number of similar
cases of pure spastic paraparesis of
undetermined origin, some with
questionably significant spondylosis
in which decompression did not
change the downward course of the
illness and in some with no spondylosis at all. Probably in future
cases, more attention should be directed to the crucial matter of an
obscure mechanical insult in the
cervical region.
The preservation of motor power
in the legs despite great spasticity is
commonly referred to in the literature. However, there are cases with
a more or less flaccid pure motor
paraparesis without fasciculation,
atrophy or electromyographic signs
of anterior horn cell disease, but
pathological study of such a case has
not been reported in the literature.
CLOSING REMARKS
Most of the important points have
been discussed in the introduction or
where appropriate in the three different sections. Chronic bilateral pure
motor hemiplegia does not appear to
have been described previously as a
primary disorder and no clear reference to chronic pure motor quadriplegia has been found. These two
states and the category of primary
lateral sclerosis appear to form a
kind of natural spectrum of pure

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corticospinal disease but further
case reports and more pathological
studies are necessary to test this
proposal. Their possible relationship
to amyotrophic lateral sclerosis is
obviously of theoretical interest.
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Corticospinal Spastic

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