PRIMARY LATERAL SPINAL SCLEROSIS.3 
by j. b. marVTn, m. d. 
Professor Theory and Practice and Clinical Medicine, 
Kentucky School of Medicine. 
The disease designated by different au- 
thors as primary lateral spinal sclerosis, spas- 
tic spinal paralysis, spasmodic tabes dor- 
salis, is rare, and only recently has been 
recognized as a distinct affection, principally 
through the writings of Erb, 1875, and 
Charcot, 1876. Seguin, of New York, in 
1873 recorded five cases of what he termed 
tetanoid paraplegia, and to him belongs the 
credit of priority in describing the symp- 
tom group. The disease comes on very 
gradually, is protopathic in origin, is associ- 
ated with systematic, symmetrical sclerosis 
of the lateral columns of the cord in the 
crossed pyramidal tract; the sclerosis is not 
a secondary degeneration due to a lesion 
higher up in the cord or the brain. The 
disease is an affection of adult life, gen- 
erally appearing between the ages of twenty 
and fifty years. According to most writers, 
males are more liable to the disease than fe- 
males. In forty-nine cases cited by Althaus, 
there were twenty-four males and twenty- 
five females. The real cause of the disease 
is unknown; the neurotic temperament is 
marked in many cases; external injury, 
rarely syphilis, scarlet fever, typhoid fever, 
and other acute diseases may be followed 
by it. Brunelli, of Rome (International 
* Rend before the Kentucky S-tWe Medical Society. June 
26, 1885. / 
V 2 
Medical Congress, 1881), reported eleven 
cases occurring near Rome, caused by eat- 
ing Lathyrus cice?-a. The disease is attended 
by loss of power in the extremities, with 
muscular rigidity, spasmodic twitchings 
and tremor, and an increase in the tendon 
reflexes. The muscles are well nourished, 
and there are no disorders of sensation. 
The positive symptoms, i. e., muscular con- 
traction and increased reflexes, and the 
negative symptoms, i. <?., no sensory, vesical, 
or rectal troubles, show unmistakably that 
the lesion is located in the crossed pyra- 
midal tract. The symptoms are striking, 
yet the disease seems so far to have eluded 
the grasp of the pathologist. Ross, Bas- 
tian, and others, attribute to Drs. Dresch- 
field and Morgan, of Manchester, England, 
the honor of being the first to have proved 
by dissection the connection of the symp- 
toms of the disease with sclerosis of the lat- 
eral columns in a primary and uncompli- 
cated case of the disease. 
The account given by Dr. Dreschfield 
(Transactions of the International Medical 
Congress, London, 1881) states that the 
giant cells of the anterior cornuse were dis- 
eased. Althaus, in his recent work on 
Sclerosis of the Spinal Cord, claims that this 
case was of the amyotrophic variety. The 
only case which he accepts as of undoubted 
and uncomplicated primary lateral sclerosis 
is the one recently recorded by Minkow- 
sky,* occurring at the University Hospital 
of Konigsberg, under the care of Professor 
* Deutsches Archiv fur Klin. Med., vol. xxxiv, page 
433. 1884. 3 
Naunyn. The patient was a woman, aged 
nineteen years, suffering with secondary 
syphilis. Fourteen months after admission 
the patient died. Microscopical examina- 
tion of sections of the cord showed sclerosis 
of the postero-lateral columns, correspond- 
ing to the crossed pyramidal and direct 
cerebellar column. The gray matter of the 
anterior cornua was perfectly normal. Pri- 
mary lateral sclerosis is a very rare disease, 
and there is danger of confounding it with 
the comparatively common condition of 
secondary descending degeneration, which 
may produce the same motor symptoms. 
A diagnosis of primary lateral sclerosis is 
only justified when the history shows a very 
slow and chronic onset, and there is no 
lesion present which could give rise to 
secondary degeneration. Special attention 
should be given to the condition of-sensi- 
bility and to the state of the vertebral col- 
umn. The disease is especially liable to 
be confounded with transverse myelitis. 
Cases of transverse myelitis of the dorsal re- 
gion, in which the sensory disorders which 
were present at first have disappeared, 
while the motor phenomena have remained, 
are extremely liable to be confounded with 
primary lateral sclerosis. In the latter dis- 
ease there are no sensory disorders. Motor 
rigidity and weakness are developed to- 
gether; rigidity is more marked than paral- 
ysis, and there is no muscular atrophy at the 
upper level of the paralysis. Hughes Ben- 
net has recently drawn attention to the fact 
that young women may have all the symp- 
toms of primary lateral sclerosis, and he la- 4 
ments that there is not a single point by 
means of which a diagnosis can be made. 
Though it is generally easy to distinguish 
the functional from the organic lesion, yet 
the fact remains that in some cases compe- 
tent observers have been able to differen- 
tiate sclerosis and pseudo-sclerosis only on 
the post-mortem table. Charcot claims that 
the lesion in primary sclerosis is wedge- 
shaped, and extends exteriorly to the pia 
mater and interiorly as far as the posterior 
cornua. This peculiarity in the localization 
of the disease, he claims, distinguishes it 
from secondary lateral sclerosis consequent 
on cerebral injuries; the lesion in this case 
being rounded, and not extending exteriorly 
as far as the pia mater, and also from scler- 
osis following myelitis, etc., when a small 
layer of healthy white matter remains be- 
tween the posterior cornua and the scler- 
osed tissue. 
The disease is probably the most chronic 
of any spinal trouble; it may extend over 
a period of many years; uncomplicated 
cases do n,ot tend to shorten life. The 
disease is liable to extend into the anterior 
cornua, or the postero-external columns, 
wasting, bedsores, cystitis, etc., superven- 
ing; death being due to exhaustion or some 
intercurrent complication. Cases of recov- 
ery are recorded; I am skeptic enough to 
doubt the correctness of the diagnosis. In 
the line of treatment but little is to be ex- 
pected; arsenic, bromides, nitrate of silver, 
anti syphilitics, galvanism, and water-cure, 
have each their advocates. 
The following brief notes will give you 5 
a good clinical picture of the disease. I 
gave the case the severest scrutiny and feel 
justified in my diagnosis: 
In June, 1883, Mr. B., of Tennessee, 
consulted me on account of a peculiar stiff- 
ness of his legs and great difficulty in walk- 
ing. Mr. B. was twenty-seven years of age, 
unmarried, and of rather slight stature, 
though compactly and well built. He gave 
a good family history on both sides. As a 
child he was always healthy and active, he 
was reared in the country; when old 
enough he had plowed and performed the 
usual farm work. He had never been sick 
prior to the onset of his present trouble. 
In the spring of 1874 he first noticed a tired, 
heavy sensation in his legs. He attached 
no importance to these symptoms, as he 
thought they were due to hard work. 
Shortly his feet began to drag, his legs feel- 
ing very stiff and heavy, his toes turn in 
and he easily stumbles, his knees knock to- 
gether, and his legs tremble violently when 
he attempts to walk. He kept his bed for 
some time, hoping in vain for improvement. 
He could walk only by the aid of a cane. 
His general health was good. Mr. B. now 
left his farm and went to Nashville, where 
he was closely confined for several years as 
a book-keeper. 
Present condition: on examination I fail 
to get any history of syphilis, or injury to 
head or back. The muscles of the legs 
and back are well developed; there is no 
atrophy; no change in the electrical re- 
action of the muscles; he has a marked 
spastic gait, the posterior leg muscles are 6 
very rigid, his heels are drawn up, and in 
walking his toes drag and appear to stick to 
the floor, his knees lock and strike together, 
his body sways; back strongly arched and 
chest thrown forward. He can cross his legs 
only with the greatest difficulty. When he 
is tired or excited, or standing in an awk- 
ward posture, his legs tremble violently. 
When sitting, if he presses his toes on the 
floor, violent reflex trembling is excited in 
the legs; ankle and knee clonus are well 
marked, superficial reflexes not as well 
marked as the deep. He has never had 
any symptoms referable to the bladder or 
rectum. He has never had any head symp- 
toms. He does not complain of pain any 
where, but on close questioning he acknowl- 
edges that when he has walked much or 
overexerted himself, he has a dull, aching 
sensation in the lower dorsal region and in 
the calves of his legs. There are no tender 
spots, and no evidence of any thing ab- 
normal about the spinal column. 
In the discussion following the report of 
this case to the Louisville Medico-chirur- 
gical Society, Prof. J. W. Holland stated 
that several years ago he had seen a patient 
with Prof. W. O. Roberts, whom he thought 
presented all the features of primary lateral 
sclerosis, and he had so diagnosed it. 
Prof. Roberts stated that the patient, a 
woman, aged nineteen years, had been con- 
fined to her bed for several months; re- 
cently bedsores and cystitis had developed. 
The patient dying within a few weeks, 
Prof. Roberts kindly notified Prof. Hol- 
land and myself that we might make an 7 
autopsy. On account of difficulties inter- 
posed by friends of the deceased, the brain 
and cervical portion of the cord were not 
examined ; the dorsal and lumbar portions 
of the cord only were carefully removed. 
The cord externally presented nothing 
abnormal. It was suspended in Muller’s 
fluid for several days, and then, with a 
freezing microtome, I made a large number 
of very thin sections. These sections to the 
naked eye revealed a dull, grayish, waxy 
patch in the lateral columns. Sections 
were stained in carmine, picro-carmine, ani- 
line blue, and osmic acid. Carmine and 
picro-carmine gave the best results. The 
sections I pass around show beautifully the 
deeply-stained diseased patches in the lat- 
eral columns, extending from the posterior 
cornua to the periphery. Sections from the 
dorsal regions, examined under the micro- 
scope, reveal no disease of the gray matter; 
the ganglionic cells are numerous, well- 
formed, and present no evidence of granu- 
lar or other degeneration. The disease is 
limited to the lateral columns proper. The 
nerve-tubes are almost entirely destroyed, 
the neuroglia greatly overgrown, numerous 
spider cells, and in some sections oil glob- 
ules and amyloid bodies are seen. At the 
lumbar enlargement the sclerosed patch in 
the lateral columns is not so extensive. A 
slight patch of sclerosis is seen in the pos- 
tero-external columns. The ganglionic cells 
are apparently healthy. The question arises, 
is this a case of primary sclerosis? The ex- 
istence of bedsores and cystitis point to an 
extension of the disease to the gray matter. 8 
Other than a slight patch of sclerosed tissue 
in the postero-external columns in the lum- 
bar region, I am unable to detect any dis- 
ease in the gray matter.* 
There is one flaw in the case, viz., not 
examining the brain and cervical cord; but 
from the history of the case, and the micro- 
scopic appearances of the sections from the 
dorsal and lumbar cord, the lesion being 
symmetrical, and presenting all the features 
as laid down by Charcot and Bouchard, as 
distinguishing primary from secondary de- 
generation, I am inclined to adhere to 
Professor Holland’s original diagnosis, and 
class this among the few cases of primary 
lateral sclerosis in which a post-mortem ex- 
amination has demonstrated the connection 
between the lesion and the symptoms. 
*A number of sections were shown with the micro- 
scope. and micro-photographs projected on the screen with 
magic lanterns. 
Louisville.