tabes is developed include a large proportion of instances in which
the secondary manifestations were slight and unlike that florid
syphilis with well-marked cutaneous and visceral lesions which is more
apt to be followed by transitory or severe vascular affections of the
cord and brain.
Excesses in alcohol, tobacco, and abuse of the sexual function are
among the factors which frequently aggravate the tendency to tabes,
and one or more of them will usually be found associated with the
constitutional factor in syphilitic tabes. Both alcohol and nicotine
have a deleterious effect on nervous nutrition and on the spinal
functions, as is illustrated in the effect of the former in producing
general neuritis, and of both in provoking optic-nerve atrophy and
general paralysis of the insane, not to speak of the pupillary states
which often follow their abuse, and the undeniable existence of a true
alcoholic ataxia. Sexual excesses were, as stated, at one time
regarded as the chief cause: the reaction that set in against this
belief went to the extreme of questioning its influence altogether. It
is to-day regarded as an important aggravating cause in a large number
of cases, and this irrespective of whether it be the result of a
satyriacal irritation of the initial period or a precedent factor. In
a large number of my patients (18 out of 23 in whom this subject was
inquired into) the habit of withdrawing had been indulged in,[67] and,
as the patients admitted, with distinct deleterious effects, such as
fulness and throbbing in the lumbo-sacral region, tremor {855} and
rigidity, with tingling or numbness, in the limbs, blurred vision, and
sometimes severe occipital headache; in one case lightning-like pains
in the region of the anus ensued.[68]
[Footnote 67: Coitus reservatus, the real crime of the Onan of
Scripture.]
[Footnote 68: Leyden states that coitus in the upright position has
been accused of producing tabes, without mentioning his authority. I
have no observation on this subject touching tabes, but am prepared to
credit its bad effect from the account of a masturbator, who during
the orgasm produced while standing felt a distinct shock, like that
from a battery, shooting from the lumbar region into his lower limbs,
and causing him to fall as if knocked down. He consulted me in great
alarm—was scarcely able to walk from motor weakness, and had no
knee-phenomenon; in a few weeks it returned, and no further morbid
sign appeared. Masturbators of the worst type occasionally manifest
ataxia, and in three cases I have been able to establish the return of
the knee-jerk, together with other improvements in the spinal
exhaustion of these subjects. The loss and diminution of the patellar
jerk, and the frequently associated urinary incontinence, as well as
certain of the peripheral pains found in masturbators, certainly prove
that undue repetition of the sexual act (be it natural or artificial)
is competent to affect the cord in a way that cannot but be injurious
in case of a predisposition to tabes, if not without the latter.]
Of single causes, none exerts so direct and indisputable an influence
on the production of tabes as the action of cold and wet upon the
lower segment of the body. It is usually the case that such exposure
is frequently repeated and combined with over-exertion before the
disease is produced, but it is occasionally possible to trace the very
first symptom of the disease directly to a single exposure. A soldier
who stands up to his knees in a rifle-pit half full of water finds his
limbs numb or tingling; develops slight motor weakness, then
lightning-like pains, and ultimately a typical tabes. In the case of a
peddler who presented an advanced form of the disease, the first
symptoms had developed after a single wetting of his feet: while
walking along one of our watering-places with his wares the swell of a
steamer inundated the beach. He had been subject to perspiring feet
before that, and the perspiration remained checked from that time
on.[69] The influence of surface chilling was remarkably manifest in
all three of my female cases. In one of them it was due to frequent
wetting of the feet; in the second, a midwife, the first symptoms
began immediately after standing on a cold hearthstone while preparing
some article needed in a lying-in case. In the third case, a lady who
contracted and safely passed through a scarlatina in her twenty-eighth
year was taken out driving while desquamation was going on. She became
thoroughly chilled, experienced numbness in the fingers and toes, and
from that day on developed a slowly progressing tabes involving all
extremities alike.[70]
[Footnote 69: Checking of habitual perspiration by violent measures is
mentioned by the German textbook writers as a frequent cause, but
occurs quite rarely in the modern tables.]
[Footnote 70: In view of the absence of spinal—or, in fact, any
nervous—symptoms prior to the exposure referred to, it does not seem
necessary to insist that this was not an instance of a true
post-scarlatinal tabes; and possibly the case thus designated by
Tuczek (_Archiv für Psychiatrie_, xiii. p. 147) may have been really
due to chilling of the delicate body-surface after desquamation or
during that process. The typical form of myelitis and sclerosis after
exanthematous fevers is rather of the disseminated type.]
Spinal concussion has been mentioned by a number of authorities as a
possible cause for tabes, as for other forms of sclerotic spinal
disease. In 1 of 81 cases in my own observation the development of the
disorder could be distinctly traced to a railway injury; in 2 a sudden
aggravation was as distinctly referable to a similar cause.[71] To
what extent railroad {856} travelling, with its attendant continual
jarring of the body, may predispose to the development of tabes or of
other spinal diseases is as yet a matter of mere conjecture. That
railroad travelling exerts a bad influence in some cases of the
established disease is evident; but in others the patients rather like
the motion, and claim to feel benefited by it.
[Footnote 71: A fall from a chair, striking on the back of the latter,
while endeavoring to keep a row of books from coming down in one case,
and the shock of the Ashtabula disaster in the other. The latter
patient, the same one who is referred to as describing the
electric-storm sensation in an earlier part of this article, had his
foot amputated in consequence of that disaster; but, like one of the
characters in _Jacob Faithful_, who felt his toes when the weather
changed, though he left both legs at Aboukir, he felt the terrific
pains of the disease in the absent foot as distinctly as in the other.
Dumenil and Petit (_Archives de Névrologie_, ix. Nos. 25 and 26)
relate cases in which a spinal concussion was the only ascertainable
cause.]
A number of toxic agents have been charged with producing tabes: thus,
Bourdon maintains this of absinthe; Oppenheim attributes one case to
poisoning by illuminating gas, the exposure to its influence being
immediately followed by a gastric crisis, and this by a regulation
tabes.[72] It is supposed that most of the poisons acting on the cord
in this or a similar way, such as arsenic, cyanogen,[73] barium, and
chloral,[74] do not produce a spinal lesion directly, but through the
medium of a secondary cachexia. Of no agent is the effect in producing
tabes so well studied as ergot of rye. It had long been known that
ergot-poisoning provoked certain co-ordinating, motor, and sensory
disturbances, but it was left for Tuczek[75] to show that this
vegetable parasite produces a lesion of the spinal cord which in its
character and distribution apes typical posterior sclerosis so closely
as to justify the designation of a tabes ergotica. Possibly, pellagra,
which is sometimes manifested in a similar way,[76] may yet be shown
to have a like influence.
[Footnote 72: _Archiv für Psychiatrie_, xv. p. 861.]
[Footnote 73: Bunge, _Archiv für experimentelle Pathologie_, xii.]
[Footnote 74: _Transactions of the Clinical Society of London_, xiii.
p. 117, 1880.]
[Footnote 75: _Archiv für Psychiatrie_, xiii. p. 148.]
[Footnote 76: Bouchard, “Étude d'Anatomie pathologique sur un Cas de
Péllagrie,” _Gaz. méd. de Paris_, 1864, No. 39.]
Among the occasional and exceptional causes of tabes, Leyden and Jolly
mention the puerperal state; Bouchut, diphtheria; and several
instances are recorded in which psychical shock was responsible for
the outbreak of the disease. In a small number of cases I found that
mental worry and anxiety coincided with the period of presumable
origin of the disease.
Age seems to have no special determining influence. It is true that
most sufferers from this disease are men in the prime of life or in
the period following it. But it is precisely at these periods that the
exposure to the recognized causes of tabes is greatest. It seems as if
there were very little liability to the development of tabes after the
fiftieth and before the twenty-fifth year; still, some cases of
infantile tabes have been recorded.[77]
[Footnote 77: Excluding the so-called family form of locomotor ataxia:
6 rather imperfectly described cases are cited by Remak (_loc. cit._),
and 3 additional ones related by himself. Of the latter, 2 had
hereditary syphilis, and of 1 the father was both syphilitic and
tabic.]
In the majority of cases tabes is due to a combination of a number of
the above-mentioned factors. The majority of tabic patients in the
middle and wealthy classes have had syphilis, and of these, in turn,
the majority have been guilty of sexual excesses or perverted sexual
acts, while excesses in tobacco and of alcohol are often superadded.
Among {857} the poorer patients we find syphilis less frequently a
factor, but still present, according to various estimates, in from 20
to 60 per cent. of the cases. Excesses in tobacco play a lesser, and
excesses in alcohol a larger, part in the supplemental etiology than
in the other class, while exposure to wet and cold and over-exertion
are noted in the majority; indeed, in a fair proportion they are the
only assignable causes.
DIAGNOSIS.—The recognition of advanced tabes dorsalis is one of the
easiest problems of neurological differentiation. The single symptom
which has given one of its names to the disease—locomotor ataxia—is so
manifest in the gait that even the sufferers from the affection learn
to recognize the disease in their fellow-sufferers by the peculiar
walk.[78]
[Footnote 78: At present I have six tabic patients under treatment,
who are acquainted with each other, and who have made each other's
acquaintance in the singular way of addressing one another on the
strength of mutual suffering at Saratoga, at the Hot Springs of
Arkansas, and in New York City.]
Although there are other chronic affections of the cord which manifest
ataxia, such as myelitis predominating in the posterior columns,
disseminated sclerosis in a similar distribution, and some partially
recovered cases of acute myelitis, the gait is not exactly like that
of tabes. The uncertainty may be as great, but the peculiarly stamping
and throwing motions are rarely present in these affections. The
clinical picture presented by the ataxic patient, aside from his gait,
is equally characteristic in advanced cases. Absence of the knee-jerk
and other deep reflexes, the bladder paralysis, sensory disturbance,
delayed pain-conduction, trophic disturbances, and reflex iridoplegia
are found in the same combination in no other chronic disorder of the
cord. It is supposable that an imperfect transverse myelitis in the
lumbar part of the cord might produce the reflex, ataxic, sensory,
sexual, and vesical symptoms of ataxia, but the brachial symptoms
found in typical tabes as well as gastric crises would be absent. The
pupillary symptoms would also fail to be developed, in all
probability. It is to be remembered that only fascicular cord
affections can produce a clinical picture exactly like that of tabes
in more than one important respect. In analyzing the individual
symptoms of the early stage the more important differential features
can be most practically surveyed.
The discovery of no single symptom of tabes dorsalis marks so
important an epoch in its study as Westphal's observation that the
knee-phenomenon is usually destroyed in it. Had this symptom not been
detected, so Tuczek admits, ergotin tabes would have eluded
recognition.[79] It was claimed by a majority of neurologists at first
that this jerk is always abolished in tabes, but it is now recognized
that there are exceptions, as is shown by cases of Hirt,[80] Westphal,
and others, not to mention some well-established cases of its return
during the progress of the disease.
[Footnote 79: It is not to be wondered that, like most new
discoveries, that of the pathological changes of the patellar reflex
should have been made the basis of premature generalizations. The
attempt of Shaw (_Archives of Medicine_) to establish a relation
between disturbances of the speech-faculty and an increased knee-jerk
has not met with any encouragement or confirmation, and has been
rebutted by Bettencourt, Rodrigues (_L'Éncephale_, 1885, 2), and
others.]
[Footnote 80: _Berliner klinische Wochenschrift_, 1886, 10.]
The knee-phenomenon is supposed to be a constant attribute of
physiological man. It is difficult to elicit it in children, and
frequently impossible to obtain it in young infants. It also
disappears in old age, {858} without having any special signification,
except that this occurrence seems to be in direct relation to senile
involution. In 2403 boys between the ages of six and thirteen years,
Pelizæus[81] found it absent in one only. It is customarily elicited
by having the patient while sitting in a chair throw one leg over the
other; hereupon the ligamentum patellæ is struck a short, quick blow.
Under physiological circumstances the leg is jerked outward
involuntarily after an interval of about one-fifth of a second—one
that is scarcely appreciated by the eye. But if it be found absent by
this mode of examination, the case is not to be regarded as one of
absence of the jerk without further ado. The patient is made to sit on
a table, his legs dangling down and his body leaning back, while he
clenches his fists. By this means the jerk will often be produced
where it appears to be impossible to evolve it by the ordinary means.
It is also well to try different parts of the ligament, and when
comparing both sides to strike on the corresponding spot and in the
same direction. Many subjects who appear to be irresponsive will
respond very well when a point on the outer edge near the tibial
insertion is percussed. The elbow reflex, which has the same
signification for the upper extremity that the knee-jerk has for the
lower, is elicited in the same manner.
[Footnote 81: _Archiv für Psychiatrie_, xv. p. 206.]
The absence of the knee-jerk is usually regarded as a suspicious
circumstance in persons of middle life; and where it can be
demonstrated that it has been present years previously and
subsequently disappeared, it is looked upon as of grave import. I,
however, published three years ago an authentic case of disappearance
of the knee-jerk in a physician now in active practice in New York
City who to this day enjoys excellent health and has developed no
other sign of spinal disease. The knee-jerk is also abolished in a
number of conditions not belonging to the domain of strictly spinal
diseases, such as diphtheria, diabetes, secondary syphilis, and severe
cases of intermittent fever. Of these, diabetes alone can be possibly
confounded with tabes dorsalis. The difficulty of differentiating
early tabes and diabetes is enhanced by the fact that on the one hand
there are often ataxic symptoms with diabetes, while on the other both
glycosuria and diabetes insipidus may complicate tabes. Senator,
Frerichs, Rosenstein, Leval-Piquechef, Charcot, Raymond, Demange,
Féré, Bernard, and T. A. McBride all recognize the occasional presence
of the ataxic gait, paræsthesia, belt sensation, and even fulgurating
pains, besides the abolition of the jerk, in diabetes mellitus.[82] In
pure cases of diabetes, however, I am not aware that spinal myosis or
the reflex paralytic pupil has been found.
[Footnote 82: I have now under observation a case of myelitis with
predominating sclerosis of the posterior columns of five years'
standing in a merchant who has been under antidiabetic treatment for
eleven years.]
Abolition of the knee-jerk is found in all organic diseases of the
spinal cord which destroy any part of the neural arch at the upper
lumbar level, where the translation of the reflex occurs, whether it
be in the posterior root-zones or in the gray matter of the origin of
the crural nerves. Thus, acute or chronic myelitis, disseminated
sclerotic foci of this level, may cause obliteration of the reflex at
any time of the disease; so may acute or chronic anterior
poliomyelitis, neoplasms, and amyotrophic lateral sclerosis of the
anterior cornua type if the destruction of the anterior cornua {859}
be complete enough. It is also found abolished with all diseases of
the peripheral nerves—traumatic and neuritic—which produce absolute
motor paralysis of such nerves.
Among the sources of error possibly incurred in examining for this
important symptom the presence of rheumatism is one. There is
sometimes a tetanic rigidity of the joints which prevents the reflex
from becoming manifest. It is also sometimes found to be absent
immediately after severe epileptic attacks, according to Moeli.[83]
[Footnote 83: In three examinations after severe attacks of epilepsy I
found it normal.]
The condition of the pupil is perhaps a more constant sign of early
tabes than the loss of the knee-jerk; at least it has been found well
marked in cases where the jerk had not yet disappeared. It may be
regarded as a rule in neuro-pathology that wherever reflex iridoplegia
is at any time accompanied by other oculo-motor disturbance, it is
either of spinal origin or in exceptional cases due to disease of the
pons varolii. The peculiar character of the pupillary disturbance of
tabes furnishes us with a criterion for distinguishing it from one
affection which in common with it exhibits loss of the
knee-jerk—diphtheria. In diphtheria there is also a reflex disturbance
of the pupil, but it is the reverse of that of early tabes. In the
latter reaction to light is lost, but the accommodative contraction
power is retained; in diphtheria accommodative contraction power is
lost, but reaction to light is retained.
The bladder disturbance has already been described. It is found as a
marked symptom so prominently in no other systemic affection of the
cord, and in few of the non-systemic forms, of sclerosis. In none of
these is it associated with absence of the patellar jerk, reflex
iridoplegia, and fulminating pains, as in tabes, except there be also
some motor paresis. It is the combination of any two of the important
initial symptoms of tabes without paralysis or atrophy that is
regarded as indicative of the disease by most authorities. Thus the
swaying in closing the eyes, if associated with the Argyll-Robertson
pupil, is considered as sufficient to justify the diagnosis of
incipient tabes, even if the knee-jerk be present and fulminating
pains and bladder trouble absent. Undoubtedly, the tabic symptoms must
begin somewhere. But at what point it is justifiable to give a man the
alarming information that he is tabic is a question. I have a number
of neurasthenic subjects now under treatment who have had reflex
iridoplegia for years; in one the knee-jerk is slowly becoming
extinguished; in two it has been becoming more marked after becoming
less; in all the three mentioned there is slight swaying in closing
the eyes and some difficulty in expelling the last drops of urine
while micturating. I do not believe that such a condition justifies a
positive opinion, although the surmise that they are on the road to
developing tabes may turn out correct for all these and for some of
those who have merely reflex iridoplegia.
Incipient tabes cannot be readily confounded with any other chronic
disease of the spinal cord. Some of the cases produced by sudden
refrigeration resemble a beginning myelitis. But the absence of true
paralysis seems to distinguish it from the latter. In all the cases of
so-called acute locomotor ataxia of myelitic origin that I can find a
record of, paralytic symptoms were marked, if not throughout the
disease, at least in the initial period.
{860} Other forms of sclerosis occasionally limited to the posterior
columns imitate the symptoms of tabes. It is unusual, however, for
such sclerosis to be distributed through so great an extent of the
posterior columns as to produce symptoms consistent with tabes in both
the upper and lower extremities. And even where this condition is
complied with, the typical progress so characteristic of tabes is not
adhered to. As previously stated, the progress is weakened by
variations in certain symptoms. Such variations are found in other
forms of sclerosis, but they are not as great, trophic disturbances
not so common, and visceral crises not so violent, as a rule.
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.