exceedingly slow. In addition, the former is accompanied by atrophic
paralyses as early features; if such occur in tabes, they occur late
in the disease, and are not marked by degenerative electrical
reactions as alcoholic ataxia is. This disorder is usually, too,
accompanied by fever, which is an exceptional, and certainly never an
intrinsic, feature in tabes. Discontinuing the alcoholic poison is
usually followed by rapid amelioration of the ataxia. Hysterical
ataxia exceptionally apes the ataxia of tabes; the presence of other
hysterical phenomena, the rapid and bizarre change of the symptoms,
are distinguishing features. There are cases of hysterical {861}
ataxia accompanied by concentric limitation of the field of
vision.[84] The outline of the limitation is strikingly like that
accompanying atrophy, but repeated examination shows a variation of a
kind not found in the latter. The blind field advances in one
direction to recede in another on one day, and reoccupies the latter
and recedes from the former on the next.
[Footnote 84: Landesberg, _Journal of Nervous and Mental Diseases_,
vol. xiii. 2.]
Ataxia after most acute diseases, such as scarlatina and typhoid, is
not usually accompanied by loss of the knee-jerk, and never by reflex
iridoplegia. The ataxia after diphtheria is complicated by the former,
but, as already stated, the very opposite state of the pupil serves to
distinguish it.
The hereditary form of tabes is frequently confounded to this day with
true tabes dorsalis. The family history and age of the patient are
collateral evidences of the nature of the case. In the hereditary form
the sphincters, optic nerves, and the eye-muscles are not affected; in
the acquired form the first and last are always involved; and this
involvement occurs so early in the disease as to constitute a valuable
discriminating feature. Although the locomotor ataxia is very similar
in the two affections, static ataxia, the symptom manifested on
closing the eyes, is not found in the hereditary form, while it is
found more constantly than even the locomotor ataxia in the acquired
form.
The electrical reactions in tabes are either normal or quantitatively
slightly increased. This serves to distinguish it from all spinal
affections accompanied by marked paralysis. In the later stages, when
some atrophy results, there may be a quantitative decrease, but these
are without qualitative changes. A change may occur in those
exceptional cases where the anterior gray horns become involved, but
we then have to deal with a true complication; and complications can
be recognized only in their development and by the application of the
diagnostic criteria characterizing the complicating disorder when of
an independent occurrence.[85]
[Footnote 85: It is not impossible that many of the symptoms described
as occurring in advanced tabes are due to independent focal disease.
In the case of a female aged seventy years mentioned by Hirt
(_Berliner klinische Wochenschrift_, 1885, No. 26), who developed
hemiatrophy of the tongue, probably from a hemorrhage in the brain
isthmus, there was no tabes, although this symptom when found with
tabes is supposed to be part of the tabic symptom-group.]
The TREATMENT of tabes dorsalis will be considered at the end of this
article, together with that of the sclerotic processes generally. The
duration and prospects of the disorder have been considered with the
clinical history. In the vast majority of cases the course of the
illness is progressive. A few cases have been reported, but in the
only instance where an autopsy was made to test the matter it was
found that the sclerosis had not been removed. It may be therefore
stated that an arrest of the disease is possible, but that restitution
of the nerve-elements, once destroyed, is impossible.
Spastic Spinal Paralysis.
SYNONYMS.—Primary lateral sclerosis. Tetanoid pseudo-paraplegia,
(Seguin), Tabes dorsal spasmodique. This symptom-group was first {862}
recognized by the American neurologist Seguin[86] thirteen years ago;
two years later Erb[87] described it under the title at the head of
this section, and about the same time Charcot[88] attributed the
well-marked clinical signs thus discovered on both sides of the
Atlantic to a primary sclerosis of the lateral columns of the cord in
that portion which is known as the crossed-pyramid tract, and whose
isolated disease had been known to Türck,[89] but not clearly brought
into relation with what is now regarded as one of the most remarkable
and recognizable of spinal symptom-groups. Our earlier knowledge of
the disease has been much confused through the discovery by
Charcot[90] of the same lesion to which spastic paralysis is
attributed by him, in a female who had been suffering from permanent
contractures of all extremities, and which he persisted in regarding
as hysterical. The only solution of the difficulty is to accept one of
two alternatives—either believing that the sclerosis was in this case
primary, in which case contracture cannot be regarded as hysterical,
or that it was secondary to protracted disuse of the limbs, in which
case it proves nothing. An additional source of confusion has been the
discovery of an analogous affection, now regarded as clinically and
pathologically distinct, known as amyotrophic lateral sclerosis, and
of a corresponding affection of the lateral columns in a large number
of cases of paretic dementia.
[Footnote 86: E. C. Seguin, “Description of a Peculiar Paraplegiform
Affection (tetanoid pseudo-paraplegia),” _Archives of Scientific and
Practical Medicine_, February, 1873. Erb's statement (_Ziemssen's
Cyclopædia_, vol. xi. 2, p. 628) that Seguin's description includes
symptoms which do not properly appertain here can no longer be
sustained, in view of the similar and identical symptoms which have
been since attributed to the same affection and to amyotrophic lateral
sclerosis by later writers. There seems to be no question that the
priority of recognition of the clinical form belongs to this side of
the Atlantic.
That Seguin's title was not as badly chosen as Erb implies in his
first reference to it may be inferred from the fact that Strümpell,
without any reference to Seguin, and evidently independently of him,
uses the following language ten years later: “As also in these cases,
the movements are not immaterially influenced by the ever-occurring
spasms, a motor disturbance may be simulated which we feel inclined to
term spastic pseudo-paralysis, or, more correctly, pseudo-paresis”
(_Pathologie und Therapie_).]
[Footnote 87: “Ueber einem wenig bekannten spinalen Symptomencomplex,”
_Berliner klinische Wochenschrift_, 1875, No. 26.]
[Footnote 88: “Sclérose primitive de la partie postérieure des cordons
antero-lateraux,” _Gazette médicale de Paris_, 1874, pp. 38, 39.]
[Footnote 89: “Ueber primäre degeneration einzelner
Rückenmarkstränge,” _Sitzungsberichte der Kaiserlichen Akademie der
Wissenschaften zu Wien, Mathematisch-Naturwissenschäftliche Klasse_,
Band xxi. Jahrgang 1856, p. 112.]
[Footnote 90: _Gazette hébdomadaire_, 1865, 7.]
CLINICAL HISTORY.—Spastic paralysis manifests itself chiefly in three
symptoms: first and most noticeable, a condition of rigidity of the
limbs; second, an increase of all the muscular reflexes; and third, a
true motor paresis. It manifests itself in the adolescent or middle
period of life, usually without any noticeable cause, beginning in the
lower extremities, and, if progressive, involving the muscles of the
trunk and arms. The invasion of the disease is first marked by an
increase in the excursiveness of the muscular phenomena, such as the
knee-jerk and the ankle-clonus. But while this reflex irritability is
originally only manifested when the diagnostician applies his special
tests, it soon becomes so great that the most trivial influence
suffices to bring about exaggerated muscular reaction. The mere
tension of a tendon in walking, the weight of the limb, the slightest
change in position, suffice to produce reflex muscular tension. The
muscles feel firm and stiff to the touch, as if permanently
contracted, {863} and the lower limbs, as a result of the combined
contraction of special muscular groups, assume a characteristic
position—namely, an extension contracture of the leg on the thigh and
a flexion of the foot on the leg. In advanced cases the contracture is
so extreme that it is almost or actually impossible to flex the leg
and to extend (dorsiflect) the feet. This is particularly noticeable
when it is attempted to overcome the strained position suddenly, while
gradual traction will often succeed in relaxing the contracture. The
latter procedure succeeds because sudden traction of the tendons and
the reflex contraction thereby provoked are avoided by it. Gowers
compares this symptom to the mechanism of a clasp-knife. When the leg
is slowly extended it yields to the manipulator's influence, but as
soon as it reaches full extension it remains like the blade of the
knife fixed by a spring.
Although some degree of muscular weakness is experienced in the
beginning of the affection—often more marked in one leg than the
other—it is insufficient to account for the grave and characteristic
disturbance of locomotion. This is due rather to the stiffness of the
limb resulting from the morbid muscular spasm. As the limbs are rigid,
the steps are short; the leg not being flexed, and consequently not
being lifted from the ground, the gait consists in an awkward
shuffle.[91] The feet are in continuous contact with the ground, and
it is observed that there is a tendency to walk on tiptoe, owing to
the contracture of the gastrocnemius, in this respect resembling the
pes-equinus position. In some cases it has been observed that the
reflex excitability was so great that the mere need of urinating
brought on a sudden tonic spasm, and there have been noted others in
which the very first spasmodic phenomena occurring in the history of
the case appeared while the patient emptied his bladder.[92]
[Footnote 91: It was Theodore Simon, I believe, who first suggested
the registration of peculiarities in gait by using sand on the floor
or compelling the patient to walk over large sheets of paper with
black-leaded shoes. Among the varieties of gait noted in paretic
dementia by him he accurately describes that of spastic paralysis
(_Die Gehirnerweichung der Irren_, Hamburg, 1871).]
[Footnote 92: Westphal, _Archiv für Psychiatrie_, xv. p. 224; _ibid._,
p. 229.]
When the upper extremities are involved, the same initial muscular
weakness and exaggerated reflex excitability are noted, but the
contracture at the elbow, unlike that at the knee, is usually in the
flexed position.
If from any cause the spastic phenomena happen to be in the background
temporarily, so as to admit testing the voluntary muscular power, it
is always found to be grossly impaired.[93]
[Footnote 93: This statement is made by Westphal, but as he considers
the only pure cases of pure primary lateral sclerosis to be those
associated with paretic dementia, and it is in the latter form alone
that there are marked exacerbations and remissions in the spastic
symptoms, it probably does not apply to uncomplicated spastic
paralysis.]
As the disease progresses locomotion becomes impossible; the advancing
rigidity of the trunk-muscles renders sitting impossible, and the
patients become bedridden. But even in this advanced stage no sensory
or vegetative disturbances were noted. The cutaneous reflexes
sometimes appear exaggerated, but this is not an evidence of sensory
hyperexcitability, but of the increased ease with which the motor
response is elicited—a feature which is also illustrated by the spasm
resulting from fulness of the bladder or even from the mere act of
micturition. Ataxia has never been noted in pure cases, and the
uncertainty in gait sometimes {864} noted at the onset of the disease
is secondary to the motor weakness and the interference with free
mobility by the, as it were, frozen state of the muscles. Occasionally
the paresis becomes a veritable paralysis, but this occurrence is
limited to one or several muscular groups. Cases are related in which
the spastic symptoms occurred on one side, involving one arm and leg
for years before involving the other. It is not improbable that these
were cases of some obscure cerebral affection. Other exceptional cases
in which the spastic phenomena appeared first in the arms are better
authenticated.
The early occurrence of ankle-clonus in this disease heralds the
appearance of apparently spontaneous clonus when the toes are put on
the ground, and later on of cramps of the gastrocnemius or other
muscles, which produce an exacerbation of the existing stiffness.
Occasionally sudden spasms occur while the patient is at rest, and
which resemble the sudden shocks which healthy persons occasionally
experience when about to fall asleep.
The electrical reactions show little that can be called
characteristic, and there is little unanimity among observers on this
point. The majority agree that there is a slight quantitative decrease
of both faradic and galvanic excitability in parallelism with the
degree of paresis. In the pure form of the disease there are no other
symptoms than those mentioned. Should evidences of involvement of the
gray substance of the cord or the cranial nerves be added, it is a
certain indication that we have to do with the similar but far from
identical affection, amyotrophic lateral sclerosis.
The course of the disease is extremely slow and its development
insidious. It is considered incurable, and although a few cases have
been described as terminating in recovery, the most recent and
reliable annals fail to make mention of any cure in a well-established
case of spastic paralysis. The disease is not in itself fatal, death
usually occurring from intercurrent affections.
ETIOLOGY.—Our knowledge of the causes of this disease is practically
nil. It has been, like tabes dorsalis, attributed to a family
tendency. Excessive sexual indulgence, over-exertion, and syphilis
have been recorded as possible causes in the few cases in which an
etiological assignment could be attempted. Tuczek surmises that the
spastic phenomena of lathyrism, a constitutional disease analogous to
pellagra and ergotism which is observed in those who live on bread
prepared from a legumen grown in Italy (lathyrus bean), and which
manifests itself in spastic symptoms, may be due to an affection of
the pyramid tract, just as tabes ergotica is due to an affection of
the posterior columns. One case of spastic paralysis of a severe
nature, associated with scanning speech, in consequence of a
lightning-stroke, is reported by Demme.[94] I have been able to assign
a cause in but two cases of spastic paralysis. Both were in Quakers;
in both there had been a history of almost incredible sexual excesses.
The disease in both involved the muscles of the jaw and face. An
autopsy in the one case revealed no lesion whatever.[95]
[Footnote 94: “Bericht über die Thätigkeit des Jennerischen
Kinderspitals,” _Wiener medizinischer Blätter_, 1884, No. 23.]
[Footnote 95: The contracture in the lower extremities, differing from
the rule, was a flexion contracture.]
{865} MORBID ANATOMY AND PHYSIOLOGY.—Although Charcot's announcement
that spastic paralysis is due to sclerosis of the crossed-pyramid
tract was made with great positiveness, the more careful authorities
have not committed themselves to his view without reservation. Their
reserved position is the result of some observations which certainly
show that there is no constancy between the distribution of the lesion
and the distribution of the spastic paralysis;[96] while, on the other
hand, characteristic spastic symptoms have been noted with purely
cerebral lesions.[97] Morgan and Dreschfeld[98] publish cases in which
the lesion was stated to be characteristic, but as the cell-groups in
the anterior horn were found by them to have been more or less
affected, it is evident they had cases of amyotrophic lateral
sclerosis to deal with. In view of similar revelations in a large
number of the cases that had been considered as spastic paralyses
during life, and in which similar findings were found after death,
Westphal,[99] one of the most critical students of the subject,
concludes that thus far an anatomical basis has not been demonstrated
with any constancy for the cases of spastic paralysis uncomplicated
with paretic dementia. With this disease a sclerosis of the lateral
column, apparently independent of the cerebral affection, is often
found. It has no continuity, as a rule, with the cerebral lesion, and
it may be limited to special districts of the cord. It is not usually
intense enough to produce material destruction of the tract itself,
and for this reason, probably, we do not find any other symptoms than
a paretic weakness and an increase of the patellar and other muscular
phenomena developed in the majority of paretics. In some, however, the
characteristic spastic gait and muscular rigidity do develop. Westphal
conjectures that if paretic dements lived as long as the sufferers
from uncomplicated spastic paralysis, they would ultimately show the
typical symptoms.[100] Numerous observations, however, show that the
presence and intensity of the spastic symptoms in paretic dementia are
not related to the presence and intensity of lateral-column lesion.
Thus, Zacher[101] failed to find such lesion in a case where the
spastic symptoms had been well marked. It must be remembered, in
drawing conclusions regarding the pathogeny of simple spastic
paralysis from the lateral-cord affection and associated symptoms of
paretic dementia, that the possibility of the lesion of the pyramid
tract in this affection being secondary to disuse[102] cannot be
excluded. On the other hand, the symptoms of most paretic dements
presenting lateral-column {866} lesion differ in some respects from
those of a pure spastic paralytic. There is a precedent clumsiness and
helplessness of movement; the patient stumbles and trips more than is
the case with the pure spastic gait; he wavers after suddenly turning
around, and there is considerable tremor with intended movement. There
is also more exacerbation and remission of these symptoms than is the
case with true spastic paralysis, and it is observed that the
exacerbations usually follow apoplectiform and epileptiform attacks,
thus showing that the cerebral condition, after all, may be the
determining factor.
[Footnote 96: Fischer and Schultze (_Archiv für Psychiatrie_, xi. 3)
report an impure case in which, with exquisite spastic symptoms in the
neck and arms, the degeneration of the pyramid tract was limited to
the dorsal part of the cord.]
[Footnote 97: Schulz (_Deutsches Archiv für klinische Medizin_, Band
xxiii.) and Strümpell (_Archiv für Psychiatrie_, x.).]
[Footnote 98: _Journal of Anatomy and Physiology_, xv. p. 510.]
[Footnote 99: _Archiv für Psychiatrie_, xv. p. 246.]
[Footnote 100: Another feature which antagonizes the development of
spastic phenomena in paretic dementia is the lesion of the posterior
columns which often ensues. In proportion as this is developed it
neutralizes the exaggerated knee-jerk, stiffness, and spastic gait.]
[Footnote 101: _Archiv für Psychiatrie_, xiii. p. 155.]
[Footnote 102: It has been claimed against this view that if this were
so the degeneration of the pyramid tract should be accompanied by
degeneration of the cells in the anterior horn. This claim assumes
that the cells and the pyramid tract are directly continuous, but the
most modern researches, those of Von Monakow, confirming an older
observation of Homén's, show that a system of small cells in the
lateral reticular processes is interpolated; which I can confirm. It
is certainly compatible with an atrophy from disuse of the voluntary
tract that the cells themselves, presiding over reflex and nutritive
functions not necessarily disturbed in paretic dementia, should remain
nearly intact.]
There is another respect in which the two conditions differ. Spastic
paralysis is usually an ascending affection, the lower extremities
being involved first, the trunk next, and the upper extremities last.
But in paretic dementia the spastic phenomena develop in both upper
and lower extremities simultaneously, and there are universal tremors,
probably of irritative origin. The lower extremities are not commonly
in the extension contracture of spastic paralysis, but in the same
flexed position as the arms, the adductors usually preceding the
flexors in becoming rigid. If it be added to this that the
lateral-column lesion in paretic dementia appears to answer all the
requirements which could be made of the lesion on theoretical grounds
were the case one of pure spastic paralysis—that is, that its area
decreases upward—the inconsistency of the observed anatomical and
clinical facts becomes strongly evident. Thus far, the attributing of
spastic paralysis to a primary lesion of the pyramid tract rests in a
few contested cases, on a number of doubtful analogies, and on the
undoubted fact that sclerosis of this tract in the event of a myelitis
is followed by pronounced spastic symptoms in all those muscles which
derive their voluntary innervation from the part of the tract which
lies below the level of the lesion.
The situation of this tract, which was not discovered by Türck and
Flechsig, as is usually supposed, but accurately known to Burdach[103]
in 1819, may be roughly stated as follows: It lies in the dorsal half
of the lateral column, making up the bulk and core of this part of the
column. It is separated from the pia mater by the direct cerebellar
tract, and from the posterior gray horn by a narrow zone of fibres
differentiated by Lissauer (see Tabes). It is connected with the
lateral reticular processes, and in its cephalo-caudal course becomes
gradually attenuated, giving off its fibres to these processes, thus
to be exhausted in the lower part of the lumbar enlargement of the
cord, where it approaches, if it does not actually reach, the surface.
The fibres controlling the voluntary motions of the lower limbs, and
which have a longer course to run before they reach the brain than
those which mediate the voluntary control of the arms, are situated
nearest the lateral boundary of the cord. Where the spastic phenomena
are mainly marked in the lower limbs the sclerotic process has been
found most marked in the corresponding area.
[Footnote 103: _Vom Bau und Leben des Gehirns_. This gifted author
says that the crossed-pyramid tract lies in the lateral column of the
cord, behind a line corresponding to the attachment of the ligamenta
denticulata and removed from the surface.]
The progress of this affection has not been materially modified in any
case by treatment. The same measures employed in sclerotic processes
generally, particularly galvanism and warm baths, are recommended. It
{867} is difficult to understand what good effect ergotin, which is
mentioned by a number of the German writers, can have in a disease of
this nature.
Amyotrophic Lateral Sclerosis.
Amyotrophic lateral sclerosis, so named by Charcot[104] and Joffroy,
who first described it, consists in a disease affecting both the
conducting tracts and nuclear centres of the motor system of the
spinal cord and medulla oblongata, manifesting itself in a combination
of atrophic and irritative phenomena on the part of the muscles. The
relations between the symptoms and lesions of this disease rival in
constancy and preciseness those noted in typical tabes dorsalis. As
the variations in the mode of invasion and distribution of the lesion
account for the widely-differing clinical types of the disease, and
the advanced state of our anatomical and physiological knowledge of
the cord enables us to interpret the reason of this difference, we
shall invert the usual order and discuss the morbid anatomy first.