The Stolen Hours Karen Swan

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The discovery of ataxic phenomena as a symptom of peripheral
neuritis has added another to the long list of pseudo-tabic affections.
Indeed, Dejerine, who greatly advanced our knowledge of this
affection, undertook on the strength of his discovery to place tabes
among the peripheral affections complicated by secondary affection
of the cord. In a large number of cases of peripheral neuritis,
particularly the alcoholic form described by Fischer, the static ataxia,
belt sensation, bladder trouble, and reflex iridoplegia are absent. In
tabes the severe pains, if influenced by deep pressure at all, are
affected favorably, but in peripheral neuritis pressure on the affected
nerve-trunk greatly aggravates the trouble.

In addition, those severe forms of neuritis that lead to ataxia,

abolition of deep and other reflexes, are accompanied by qualitative
electrical changes and atrophic paralysis—features not found in
tabes. Sometimes a disseminated neuritis will become so
generalized as to cause diplopia and other evidences of ocular
paralysis through the affection of the peripheral nerves, but, so far as
my observations extend, not with the characteristic pupillary
phenomena of tabes.

Cerebellar disease, alcoholic and hysterical neuroses, sometimes

produce ataxia, and this may be associated with one or more of the
other characteristic symptoms of tabes. Cerebellar ataxia is usually
very different from that of tabes, as far as the locomotor element is
concerned. In tabes it is the movements the patient makes which
cause him to stagger; in cerebellar disease those movements made
to prevent staggering are usually co-ordinated. The gait properly
called cerebellar is therefore very different from that of tabes. There
is, however, static ataxia in both cerebellar and posterior-column
disease. In addition, the knee-jerk may be abolished in the former,
heightening the resemblance: the history of the case is, however,
decisive where these latter symptoms might lead to doubt. Bladder
trouble in cerebellar disease is not an early feature, nor are
paræsthesias and delayed pain-conduction intrinsic features, of
cerebellar disease. Their presence is in favor of tabes.
Alcoholic ataxia is very rapid in its course, while tabic ataxia is
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
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.
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
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
recognized by the American neurologist Seguin86 thirteen years ago;
two years later Erb87 described it under the title at the head of this
section, and about the same time Charcot88 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
Charcot90 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.
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).
 87 “Ueber einem wenig bekannten spinalen Symptomencomplex,” Berliner klinische
Wochenschrift, 1875, No. 26.

88 “Sclérose primitive de la partie postérieure des cordons antero-lateraux,” Gazette

médicale de Paris, 1874, pp. 38, 39.

89 “Ueber primäre degeneration einzelner Rückenmarkstränge,” Sitzungsberichte der

Kaiserlichen Akademie der Wissenschaften zu Wien, Mathematisch-
Naturwissenschäftliche Klasse, Band xxi. Jahrgang 1856, p. 112.

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, 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
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).

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
 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 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
94 “Bericht über die Thätigkeit des Jennerischen Kinderspitals,” Wiener medizinischer
Blätter, 1884, No. 23.

95 The contracture in the lower extremities, differing from the rule, was a flexion
contracture.
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 Dreschfeld98 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, Zacher101 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
disuse102 cannot be excluded. On the other hand, the symptoms of
most paretic dements presenting lateral-column 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.
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.

97 Schulz (Deutsches Archiv für klinische Medizin, Band xxiii.) and Strümpell (Archiv
für Psychiatrie, x.).

98 Journal of Anatomy and Physiology, xv. p. 510.

99 Archiv für Psychiatrie, xv. p. 246.

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.

101 Archiv für Psychiatrie, xiii. p. 155.

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 Burdach103
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.
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 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 Charcot104 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.
104 In 1869 and 1874 (Leçons sur les Maladies de la Système nerveux) Charcot
termed this, and one other form of disease associated with muscular atrophy,
amyotrophic, to distinguish them from the myopathic forms. He considers amyotrophic
 lateral sclerosis as deuteropathic, the nuclear atrophy being secondary to the lesion
of the white substance, and the progressive muscular atrophy of the type described
by Duchenne and Aran as a protopathic form of the amyotrophies. Among the true
myopathies he enumerates pseudo-hypertrophic paralysis, Erb's juvenile form,
Duchenne's infantile form, certain mixed forms, and, without justification, Leyden's
hereditary forms (report by Marie et Guivron, Progrès médicale, 1885, No. 10).

MORBID ANATOMY.—In advanced cases of amyotrophic lateral

sclerosis there is found marked sclerotic degeneration of both the
crossed and the uncrossed pyramid tracts; atrophy of the cells of the
anterior horns; atrophy of some of the nuclei of the motor and mixed
cranial nerve, particularly the hypoglossal and spinal accessory;
atrophy of the anterior roots of the spinal and the roots of certain
motor cranial nerves; and, finally, atrophy of the voluntary muscles.
The greater part of the tract through which the voluntary impulse
travels after leaving the voluntary motor-fields of the cortex is
therefore continuously involved; and it would seem that there are
cases (Kahler-Pick's) where the entire motor system is affected, the
morbid process demarcating the course taken by the motor impulse
through the cerebro-spinal fibre-labyrinth from the cortical motor-field
down to the muscles.

The morbid process in the muscles consists of a narrowing of the

fibres, which subsequently lose their transverse striation and
undergo a granular disintegration. Sometimes a muscle disappears
entirely; usually the connective-tissue elements, including the
interstitial cellular and adipose tissue, undergo proliferation, so as to
mask the wasting of the muscles to some extent.

The morbid process in the nervous system is also a simple

degenerative process. The nerve-fibres and cells atrophy first, and
the connective-tissue proliferation which marks the sclerotic change
of the diseased area is a secondary process. There is still
considerable dispute among authorities as to which segment of the
motor-conduits the degeneration begins in. But from the great
difference found in the individual cases which have become the
subject of patho-anatomical studies there can be little doubt that
there is no uniformity in this respect. In some cases the lesion is far
advanced in the lateral columns, while the anterior horn is but slightly
involved; in others the reverse is found. Sometimes the nuclei of the
motor cranial nerves are the chief foci of disease; at others they are
the least affected parts of the motor apparatus. As we shall see,
there are differences in the clinical picture corresponding to the
variations of the anatomical findings.

CLINICAL HISTORY.—In typical cases the first symptom is a tired

feeling in one arm or leg, usually the former; in dextral persons the
right arm, the one which is subject to the severest strain, is most
frequently the first to be affected. With the increase in this tired
feeling there develops actual loss of power; the muscles become
wasted, and the other arm becomes involved. Often it is observed
that the right leg suffers with the corresponding arm, but as a rule the
lower extremities do not become involved to any marked degree
within the first six months of the illness. It is then noted that a gait not
unlike that of spastic paralysis is noted, but with more loss of motor
power and less stiffness. There is, however, this noteworthy
difference between the affection of the lower and that of the upper
extremities: that atrophy and loss of power are more marked in the
latter, and spastic phenomena in the former. The knee-jerk and other
deep reflexes are greatly increased, and ankle-clonus is usually very
well marked. As with spastic paralysis, there are no visceral or
sensory disturbances. Unlike that affection, there are qualitative
changes in the electrical reaction of the muscles105 in amyotrophic
lateral sclerosis. The degeneration reaction is found in the atrophied
divisions, and particularly in such groups as those of the thenar and
hypothenar eminence, which undergo complete atrophy at a
comparatively early period of the disease.
105 Moeli, Strümpell, Pick, and Mierzejewski describe cases in which only quantitative
changes were found. They were such in which spastic phenomena preponderated at
the time of the examination.

Soon after the spastic and atrophic involvements of the lower

extremities, symptoms indicating the involvement of the cranial
nerve-nuclei are developed, usually after the disease has lasted a
year or so. Deglutition becomes difficult and speech indistinct, the
general picture of a glosso-labio-laryngeal paralysis being imitated.
The patient cannot pucker his lips, his lingual muscles undergo
atrophy, and fibrillary and fascicular twitches are noted in the tongue
and lips. But just as the atrophic affection of the muscles of the arms
and legs differs from that of progressive muscular atrophy in the fact
that the deep reflexes are exaggerated with amyotrophic sclerosis,
so in the bulbar symptoms of the latter it is found, unlike the typical
form of bulbar paralysis, that the jaw reflex is increased.

The duration of the disease may be stated at about three years,

death usually occurring in consequence of the involvement of the
cranial nerves. There are cases recorded where the disease was
almost simultaneously developed in all four extremities and the
tongue, reaching a high degree within a year (Mierzejewski). It is
generally agreed that the reason contractures do not develop in
typical cases of this kind, although the lateral column is sclerosed
and spastic phenomena occur early in the disease, is the destruction
of the cell-groups in the anterior horn. The reflex arch through which
a reflex contracture would be mediated is broken, or rather
weakened, in that part of its course which passes through these
cells, and therefore a contracture is as efficiently antagonized as it
would be if the posterior roots were divided. Still, in some cases a
frozen attitude of the lower extremities is very well marked (Vierordt,
Zacher). To reconcile these conflicting observations it has been
suggested that it may be regarded as a question of speed between
the progress of the pyramid-tract and the anterior-horn lesions. If the
former be much in advance of the latter, spastic phenomena will
preponderate and contractures be possible, to disappear with the
subsequent anterior-horn lesion. But if the latter precede and
preponderate, the spastic phenomena will be in the background and
contractures impossible. Indeed, Zacher106 suggests that there may
be an ascending form in which the lesion of the pyramid tract is
secondary to the nuclear atrophy, corresponding to the typical class
of cases on which Charcot based his first description of the disease,
and a descending form in which the pyramid tract is first affected and
the nuclear cell-groups follow. Vierordt107 and Kahler108 express
similar opinions. The latter goes so far as to suggest that progressive
muscular atrophy, progressive glosso-labio-laryngeal paralysis, and
amyotrophic lateral sclerosis are really due to one and the same kind
of degenerative process, merely differing in location. There is
certainly, as he claims, a remarkably complete chain of cases,
beginning with such (1) in which spastic paralysis preponderates,
passing thence to (2) those in which some muscular atrophy
preponderates, then (3) those in which muscular atrophy is in the
foreground and the spastic phenomena are slight, and ending with
(4) the pure atrophies. A similar transition may be established on the
regional principle between pure glosso-labio-laryngeal paralysis and
amyotrophic lateral sclerosis, for there are cases of this affection in
which the oblongata symptoms preponderate throughout, and the
focus of the disease is formed there, just enough lesion being
demonstrable in the pyramid tract and the spinal gray matter to
prove the family relationship of what clinically appears as a spastic
bulbar paralysis.109
106 Archiv für Psychiatrie, xv. p. 416.

107 Ibid., xiv. p. 397.

108 Zeitschrift für Heilkunde, 1884, p. 109.

109 Such a case is described by Freund in Deutsches Archiv für klinische Medizin,
xxxvii. p. 405.

PROGNOSIS.—As far as the typical cases of this disease have been

studied, a fatal termination seems to be invariable. Seeligmüller
reports a few cases in which the progress appeared to become
arrested, but it is not clear that these were not in reality cases of
some juvenile form of muscular atrophy. Not only is the affection in
adults fatal, but it is so in a short period of time as compared with
other spinal disorders, and particularly with the related disorder
spastic paralysis. Few patients survive the third year of their illness;
a number do not live to that length.
 The Combined Forms of Sclerosis.

A number of cases of chronic sclerotic disease of the cord have

been discovered and analyzed during the past decade, which, while
they show the regularity of distribution noted in posterior and lateral
scleroses, differ from them in involving at once more than one
column of the spinal cord. Usually, it is the posterior column and the
posterior part of the lateral column which are affected. The
symptoms constitute a combination of those of tabes dorsalis and of
spastic paralysis. But this combination does not represent a mere
addition of symptoms; where the tendency of the two diseases
conflicts, they neutralize each other. Thus the tendency of lateral
sclerosis uncomplicated by posterior sclerosis is to increase the
patellar jerk; when posterior sclerosis complicates it, the jerk is
annihilated. The degeneration of the lateral column and the ensuing
motor paresis in like manner neutralize the ataxic character of the
gait by limiting its excursiveness.

The upper extremities are usually involved equally with the lower. In
the cases of Kahler-Pick considerable atrophy of the muscles
developed; in those of Prévost and Westphal this was not very
noticeable. In a few cases, where the posterior sclerosis did not
involve the lumbar part of the cord, spastic symptoms were noted in
the lower extremity. In the only case of combined sclerosis now
under my observation this peculiarity, noticed by Prévost, is well
marked. In one of Westphal's cases there was evident mimic ataxia.
The few cases of this affection observed show so many variations
that it would be at present premature to attempt sketching a common
clinical type. The majority of the subjects were affected between the
twenty-fifth and forty-fifth years.

ETIOLOGY.—Little is known of the causes of this disease. Usually

beginning insidiously, no special mode of origin can be determined.
Surface chilling has been assigned in a case by Erlitzky and
Rybalkin, and others are reported to have begun during pregnancy.
One of Westphal's cases developed in a lithographer who had been
subject to epileptoid fits.

DIAGNOSIS.—As yet we have no reliable criteria for distinguishing

between a combined sclerosis and certain forms of diffused sclerosis
during life. Ballet and Minor110 found such a sclerosis diffusely
involving the posterior and lateral columns of the cord in a case
where they were justified from the symptoms in expecting a
combined fascicular sclerosis of these columns, and after a careful
study of all similar cases collated by them in consequence of this
experience, they came to the conclusion that a number of affections
of different origin, but eventually involving both columns, may impose
on the observer as combined fascicular sclerosis during life. Diffuse
meningo-myelitis is one of these affections, and is far from
uncommon, while true system or combined sclerosis is apparently a
very rare disease.
110 Archives de Névrologie, vii. p. 44.

The Family Form of Tabes Dorsalis.

SYNONYMS.—Friedreich's disease, the Family form of locomotor

ataxia, Hereditary ataxia, Hereditary tabes.

Friedreich discovered a peculiar form of co-ordinating disturbance in

a number of children of the same family, which he brought into
relation with a lesion of the posterior columns, and which has been
since found by him and by subsequent observers to occur in other
cases, always affecting several members of the same family, as in
the first case observed by him. The clinical and pathological
features, though resembling those of the tabes dorsalis of adults in
many respects, are distinct in others, and for this reason it is
generally assigned a separate place in classification.
ETIOLOGY.—The disorder is usually manifested in juvenile life, the
age of the affected subjects varying from the seventh to the twenty-
fifth year. The male sex preponderates in the statistics of the
affection. Some neuropathic vice can always be found in the
patient's immediate ancestry, and the limitation of the disease to
families burdened by such a diathesis is exemplified in the fact that
the ninety individual cases thus far accurately studied occurred in
thirty-six families.111 The disease type of the ancestors of the patients
is usually different from that of the latter. Alcoholism in the father is
one of the commonest forms, but convulsions, hysteria, and insanity
are also frequent features of the family history. In a few cases tabes
dorsalis, properly so called, was present in the father. In others there
was consanguinity of the father and mother. In Musso's group the
parents had been brother and sister, and their mother had been a
melancholic dement. Three other grandchildren and six
grandchildren by the incestuous marriage developed the family form
of tabes. In this family the frequent experience of hereditary
transmission was verified—that the neurotic taint skipped the
intermediate generations.
111 Raffaele Vizzioli, Giornale di Neuropatologia, 1885.

CLINICAL HISTORY.—Usually the first symptom is ataxia of the lower

extremities; occasionally this is preceded by severe frontal headache
or by vague rheumatoid pains. The inco-ordination is very similar to
that of true tabes dorsalis, but swaying on closing the eyes is not
noticed early in the disease, as in the latter affection. The arms soon
become involved in the ataxia, but cutaneous sensibility and the
muscular sense remain either intact or nearly so—a fact utilized with
some success by Erb in polemicizing against the theory of Leyden
that the ataxia of tabes is due to imperfect sensation. Later in the
disease, usually after a few years, a peculiar speech-disturbance is
noticed, which resembles the scanning of disseminated sclerosis. It
depends on ataxia of the tongue and lips. This is usually associated
with nystagmus. About this time the patient develops a different set
of motor symptoms from those characterizing the onset of the
disease; contractures, paralysis, and atrophy are found in the
affected extremities; sometimes the patients cannot ascend a stair,
owing to their inability to lift the feet high enough. Pes equino-varus,
deformity of other joints and of the vertebral column, have been
observed112 to result from the associated effects of paralysis and
contracture. At this stage some sensory disturbance may be
developed, formication having been observed toward the close of the
history in a number of cases. But the distribution of this disturbance
is usually different from that of tabes dorsalis, being more intense in
the trunk than in the extremities or evenly marked in the entire
periphery.
112 H. E. Smith, Boston Medical and Surgical Journal, 1885, vol. cxiii. p. 361.

COURSE AND PROGNOSIS.—The progress of this disease is slow. It has

not yet been known to be arrested by any therapeutical procedure.
Death rarely occurs directly from the disease by exhaustion; more
commonly life is cut short by some intercurrent affection. Unless this
occurs the patients may survive the commencement of the illness
from eight to forty and more years.

MORBID ANATOMY.—The sclerosis which is found to be the constant

lesion underlying this disease corresponds in every character to a
combined sclerosis of the pyramid tracts and the posterior columns.
Usually, the crossed-pyramid tract is degenerated in its spinal
course, and the uncrossed in the cervical and dorsal part, which, in
many subjects at least, is its whole extent. The cerebral part of the
pyramid tract is not affected. The nerve-fibres found normally in the
gray substance are materially reduced, probably in dependence
upon the atrophy of the great nerve-tracts.

The lesion of the posterior columns resembles that of true tabes very
closely, particularly in the lumbar part of the cord. It is, however, not
probable that it commences in precisely the same distribution, and if
cases dying early in the disease be autopsied it will be interesting to
see whether the initial sclerosis occupies identical fields—a
contingency which is unlikely, owing to the profound difference in the
initial symptoms of true tabes and the family form. It is claimed by
Schultze that in addition to the pyramid and posterior tracts the