progress of tabes dorsalis are more or less constant, and although
some of them show remarkable remissions and exacerbations, yet may in
their entity be regarded as a continuous condition slowly and surely
increasing in severity, there are others which constitute episodes of
the disease, appearing only to disappear after a brief duration
varying from a few hours to a few days: they have been termed the
crises of tabes dorsalis. These crises consist in disturbances of the
functions of one or several viscera, and are undoubtedly due to an
error in innervation provoked by the progressing affection of the
spinal marrow and oblongata. The most frequent and important are the
gastric crises. In the midst of apparent somatic health, without any
assignable cause, the patient is seized with a terrible distress in
the epigastric region, accompanied by pain which may rival in severity
the fulgurating pains of another phase {836} of the disease, and by
uncontrollable vomiting. Usually, these symptoms are accompanied by
disturbances of some other of the organs under the influence of the
pneumogastric and sympathetic nerves. The heart is agitated by violent
palpitations, a cold sweat breaks out, and a vertigo may accompany it,
which, but for the fact that it is not relieved by the vomiting and
from its other associations, might mislead the physician into
regarding it as a reflex symptom. In other cases the symptoms of
disturbed cardiac innervation or those of respiration are in the
foreground, constituting respectively the cardiac and bronchial
crises. Laryngeal crises are marked by a tickling and strangling
sensation in the throat, and in their severer form, which is
associated with spasm of the glottis, a crowing cough is added.[22]
Enteric crises, which sometimes coexist with gastric crises, at others
follow them, and occasionally occur independently, consist in sudden
diarrhœal movements, with or without pain, and may continue for
several days. Renal or nephritic crises are described[23] as
resembling an attack of renal colic. The sudden disturbances of
bladder function have been described as vesical crises, and recent
French observers have observed so-called _crises clitoridiennes_ in
female tabic patients which were characterized by voluptuous
sensations. All of these symptoms have these in common: that they last
but a short time, that their disappearance is as rapid as their
advent, and that they depend for their distribution on the attitude of
the disease in the cord. The vesical crises are more apt to occur
early than late in the disease and where the belt sensation is in the
hypogastric region. The gastric and enteric crises are usually found
when the belt is in the epigastric level, and the bronchial, cardiac,
and laryngeal crises when it is in the thorax and neck. Some
connection has been observed between the occurrence of the
lightning-like pains and these crises. Thus, a sudden cessation of the
former is sometimes the forerunner of the latter. It is also found
that one variety of these crises disappears to give way to another:
this is particularly noticed with the bronchial crises, which often
cease suddenly, to be followed by vomiting.
[Footnote 22: Krause, in a paper read before the Society of
Neurologists at Berlin (_Neurologisches Centralblatt_, 1885, p. 543),
found either laryngeal crises or other laryngeal symptoms, such as
demonstrable ataxia of movement of the vocal cords, in 13 out of 38
cases. This proportion would be far too high for tabes in general; his
cases were probably advanced ones. He established the interesting fact
that the laryngeal crisis may sometimes be provoked by pressure with a
probe on the superior laryngeal nerve at its laryngeal entry-point.
Jastrowitz claims to have determined the existence of actual paralysis
of the vocal cords with the crises, but Krause concludes from the
experimental inductibility of the crisis that it cannot be due to a
posticus paralysis. In a discussion on the subject Remak affirms that
a unilateral paralysis of the crico-arytenoideus posticus may be an
early or the earliest symptom of tabes. It seems, however, that in all
cases where he determined such paralysis there had been disturbances
of function of other cranial nerves in unusual severity at about the
same time.]
[Footnote 23: They must be extremely rare: they have not occurred in a
single one of my 81 private cases, nor do I recall one in clinic or
dispensary experience.]
There is some analogy between the exceptionally-occurring mental
disturbance of tabic patients and the crises. Like them, it resembles
a disturbance of innervation, in this instance the centres regulating
the cerebral circulation appearing to be at fault. It also seems as if
in cases of this character the direction of the mental disturbance
were determined in some sense by the emotional relations of the
oblongata, for the insane outbreak usually consists in a brief but
extreme outburst either of a {837} depressed and melancholiac or an
expansive maniacal or delirious outburst. It is a very rare
occurrence, and usually limited to the latest stages. Much more common
is the development of paretic dementia, but this is to be regarded
rather as a complication than an integral feature of tabes. Most tabic
patients retain their mental equilibrium to the last moment; some
develop truly heroic resignation to their terrible sufferings and
gloomy prospects; and a few, becoming irritable, petulant, and
abnormally selfish, show the effect of invalidism manifested equally
with other chronic diseases.
In addition to the visceral crises there are other disturbances of
innervation of a nutritive or trophic character which are
characteristic of tabes. There is scarcely one of the eruptions or
other changes which are attributable to central nervous disease which
may not occur at some one or other period of this disease. Thus,
herpetic eruptions may occur independently or in conjunction with
lancinating pains, in one case even corresponding to the area of the
belt sensation. Discoloration of the skin or hair, the latter turning
gray or white in circular[24] or in irregular patches, spontaneous
falling of the nails, spontaneous hemorrhages in the matrix of the
nail, ichthyosis,[25] purpura, diffuse œdemas, localized perspiration
sometimes associated with sudamina and accompanied by ecchymoses,
muscular atrophies, bullæ, and pemphigus-like eruptions, have been
noted by various observers. As yet, they are to be regarded rather as
clinical curiosities than as constant features, as diagnostic
criteria, or as presenting special indications for treatment. The
trophic disturbances affecting the bones and joints and a peculiar
ulcerative process known as the perforating ulcer of the foot are
better studied, and, ranking among the most formidable and striking
manifestations of tabes, merit special consideration. The tabic joint
affection, first described by Charcot as a trophic manifestation of
tabes, is usually located in the hip or knee, but almost any joint,
including the temporo-maxillary articulation, may be its seat. The
earlier or lighter forms consist in serous exudations with or without
active local manifestations resembling those of synovitis; but the
commoner and graver form consists in actual deformity of the articular
surfaces, such as enlargement or atrophy, sometimes supplemented by
the formation of osteophytes. In addition, spontaneous dislocations
occur in a manner which is often quite surprising.[26] As a type of
such an occurrence the following may serve: A tabic patient presented
a synovial swelling of the left knee of truly enormous dimensions; as
soon as this became reduced a distinct crepitus was elicited, and both
the femoral and tibial articular surfaces found enlarged. The whole
process occupied only a few days; the lateral ligaments were then
found so relaxed that the leg could be freely moved in every
direction.
[Footnote 24: Rossolymmo, _Archiv für Psychiatrie_, xv. p. 727. I have
never observed this symptom as beautifully developed as in a case of
spinal irritation, in which the possibility of the existence of any
organic disease could be positively excluded. Bulkley has observed
albinism.]
[Footnote 25: In three cases by Ballet et Dutil, _Progrès médicale_,
May 19, 1882.]
[Footnote 26: Boyer (_Revue de Médecine_, 1884, p. 487) records a case
where the hip became luxated spontaneously as an early symptom.]
The connection between the morbid process in the spinal cord and these
remarkable arthropathies is as yet unexplained; and as Charcot's
original {838} proposition, that they can be always referred to
lesions in the anterior horns of the gray matter, has not been
sustained, some of the German clinicians, notably Strümpell, are
inclined to attribute them, in part at least, to ordinary results and
accidents due to the anæsthesia and its disturbing effect on voluntary
and automatic joint protection. A controversy arose which was
participated in by the Clinical Society of London,[27] and evoked
opinions from nearly all the eminent neurologists of Europe. The
result of this thorough discussion has been to establish the joint
affection as a true tabic symptom due to the same morbid process which
underlies the less enigmatical symptoms of the disease. One of the
best reasons for regarding these joint lesions as of trophic origin is
the fact that they are associated with textural bone-changes by which
they become either unduly soft or brittle, and therefore exposed to
apparently spontaneous fracture. Cases of fracture of almost every
long and some of the short bones are recorded, among the most
remarkable being one by Dutil,[28] where fracture of the radius
occurred without adequate cause in the initial period of tabes; and
one by Krönig,[29] in which the patient, who had been tabic for eight
years, broke a lumbar vertebra while catching himself in the act of
falling down stairs.
[Footnote 27: The question was formally raised by Morrant Baker at the
December meeting in 1884, and the discussion participated in by
Charcot himself. Among the opponents of Charcot's theory were Jonathan
Hutchinson and Moxon, the latter of whom administered a sound and
well-merited criticism regarding some of the premature, if not
sensational, announcements of the distinguished French neurologist. He
compared the joint lesions of tabes to decubitus: just as the latter
can be avoided by a proper protection of the exposed parts, so the
former would not occur if the joints could be kept in a, surgically
speaking, normal state. Moxon seems to have forgotten, in suggesting
this comparison, that there is a form of decubitus which will occur
independently of the greatest care and in spite of every measure taken
to arrest it, and which can be attributed only to an obscure but
active perversion of nervous nutritive control. Barwell, Paget,
Herbert Page, Broadbent, and McNamara agreed that the joint lesions
are not of surgical or rheumatic origin, but essentially signs of the
nervous affection. Although Barwell's claim, that the rheumatic and
tabic joint diseases are essentially different, because the former is
hyperplastic and the latter is atrophic, is not borne out by all cases
of tabic joint disease, some of which are certainly hyperplastic, yet
the other reasons advanced for regarding these affections as distinct,
and considering the joint affection of Charcot as a trophic disorder,
far outweighed those advanced by the opponents of this view. In fact,
the only ground the latter had to stand on was the fact that Charcot's
asserted anatomical foundation was found to be chimerical.]
[Footnote 28: _Gazette médicale de Paris_, 1885, No. 25.]
[Footnote 29: _Zeitschrift für klinische Medizin_, 1884, vii., Suppl.
H.]
Another reason for considering the arthropathies as of central origin
is their chronological association with other signs whose trophic
origin cannot be disputed, such as unilateral sweats and œdemas and
visceral crises. They are also found to correspond in many cases to
the lancinating pains both in location and time.
The feet of an advanced tabic patient usually appear deformed; the
extremities of the bones, particularly at the metacarpo-phalangeal
articulation, are thickened, and the axis of the foot as a whole is
bent. It is a question how far this deformity may be due to persistent
faulty innervation of the muscles going to the foot and to ensuing
mechanical influences. A most pronounced deformity found in a few
cases has been designated by Féré as the tabic foot.[30] It consists
in the thickening of the tissues on the inner side of the foot,
obliterating its arch in such a manner that in standing the plantar
surface is everywhere in contact with the floor. {839} With this the
tarso-metatarsal and the metacarpo-phalangeal joints of the great toes
appear greatly swollen.
[Footnote 30: Pied tabétique—_Revue de Médecine_, 1884, p. 473.]
There is less dispute concerning the nature of the so-called
perforating ulcer of the foot. This lesion occurs only in cases where
the central or peripheral nervous apparatus is diseased; thus, it may
occur with neuritis, and it is therefore unanimously regarded as due
to morbid nervous influences. In tabes it may follow an exacerbation
of the fulminating pains. It consists in a swelling of the tissues of
the foot; the skin becomes firm and thickened; deep sinuous ulcers are
then developed, showing but slight if any tendency to granulate, and
which extend down to the bone, the latter usually remaining
unaffected.[31] In a few cases more remarkable accidents due to
disturbed nutrition have been noted. Thus, J. Hoffmann records a
subcutaneous rupture of the Achilles tendon, and in another case the
spontaneous discharge of all the teeth of the upper jaw within about a
week's time.[32]
[Footnote 31: Like most of the manifestations of advanced tabes, the
perforating ulcer may occur exceptionally as a pre-ataxic symptom.
Thus, Suckling (_British Medical Journal_, 1885, April 4, p. 693)
mentions its preceding the development of tabes proper by one year.]
[Footnote 32: _Berliner klinische Wochenschrift_, 1885, No. 12. In
this unique case the loosening and falling out of the teeth preceded
the tabes by a year. Demange, who observed the same phenomenon in two
cases, found that the ascending root of the fifth pair was involved.
The only analogous observation in my experience relates, like the
bleaching of the hair in circular patches, to a case of spinal
irritation: here the gums and alveolar borders underwent atrophy,
exposing the roots of the teeth to beyond the normal alveolar border:
first the right upper row, then the right lower row, and finally the
teeth on the left side in the same order, showed this condition; only
the most posterior fell out.]
COURSE.—Tabes dorsalis is to be regarded as an extremely chronic
affection. Its development, as previously stated, is insidious,[33]
and the symptoms demonstrating the advent of the disease may be so
slight for many years that the patient does not regard himself as ill
or as likely to become so. In this condition he may remain for ten or
more years; and there are cases recorded where the exact signs of the
incipient stage were well marked, and yet no ascertainable progress
was made in a period of such long duration that the inference
naturally follows of a much longer prospective period in which the
patient would not have become ataxic. There may be distinguished two
forms of invasion: the more frequent is marked by the development of
the terebrating, fulgurating, and fulminating pains, to which, sooner
or later, the abolition of the pupillary and certain other deep
reflexes is added. In the vast majority of cases they are found absent
when the physician's attention is directed to the question of the
existence of spinal disease. In about one-fifth of the cases pain is
at no time a prominent feature of the disease, and may be, as is
established by the cases cited, entirely absent. In these patients the
first symptoms noted are muscle-tire on slight exertion. Usually, the
evidences of disturbed sensation and innervation begin in one
extremity or preponderate in it, but symmetry is soon established. The
same is noticed in the extension of the morbid process: when the ulnar
distribution is involved—which is the first danger-signal on the part
of the cervical enlargement of the cord—usually one side is first
involved, but the other soon follows, and becomes affected in equal or
nearly equal degree. In the {840} overwhelming majority of cases the
disease manifests itself in the lower extremities first, remaining
limited to them for a long period, and if extending to the upper
extremities doing so in much slighter intensity than in the lower.
Gull, Leyden, and others have observed cases where the arms were first
and chiefly involved; but this is extremely rare. Cases of a
simultaneous involvement of the upper and lower extremities are more
frequently noted. The symptoms detailed above are developed in varying
order in different patients, and the line has been already indicated
which separates the progressive from the episodial features of the
disease. Even in the ataxic period the patients may remain in a
comparatively stationary period for many months and years.[34] The
general bodily nutrition is usually good, and the muscular strength,
as such, rarely affected to any noteworthy extent until the extreme
phase of the disease is reached. Even the formidable-appearing crises
and trophic disturbances, though occasionally fatal, are not always
so. The direct danger from the disease proper, so far as life is
concerned, is in the possible development of cystitis and pyelitis, of
the malignant bed-sore, and of delirium and coma, the latter of which
is occasionally found to be due to cerebral hemorrhage—a lesion which
some writers regard as possibly due to some impalpable influence of
the spinal disease on the cerebral circulation.[35]
[Footnote 33: Cases of acute locomotor ataxia have been described. In
no instance are they pure or typical, and the last case of the kind
published in America, beginning, as it is evident from the account,
with signs of general myelitis, illustrates the impropriety of
utilizing one or a few symptoms as determining nomenclature.]
[Footnote 34: Thirteen patients are now under my observation, or I
have been able to obtain reliable medical information concerning their
condition, who have been in the ataxic period since the date of my
first examination, varying from a year to seven years ago. Not one of
these patients is materially much worse than at that time. Two only
died, both being bedridden at the time they came under observation. Of
the first group, three have shown more or less lasting improvement in
respect to special symptoms, as will be detailed in connection with
the treatment.]
[Footnote 35: In a case of sudden death, with an asthma-like seizure,
of a tabic patient under the charge of T. A. McBride, I found an
intense injection of one (the right) vagus nucleus, the color of the
ala cinerea and of the nucleus on section being almost black.]
MORBID ANATOMY AND PHYSIOLOGY.—Our knowledge of the structural disease
underlying the symptoms which comprise the clinical picture of tabes
dorsalis is perhaps the clearest in the domain of spinal pathology.
Not alone the commencement of the morbid process and every stage of
its subsequent encroachment on the spinal cord, but also its relations
to special symptoms, have been demonstrated in numerous cases by some
of the most careful German and French investigators.
In advanced cases of tabes the disease of the spinal cord is so
evident that it was noted even by earlier observers, the region of the
cord lying between the posterior nerve-roots being altered in color,
consistency, and dimensions. It is gray instead of white, hard instead
of soft, and considerably shrunken. The gray discoloration of the
surface appears to be complete in the lumbar and lower dorsal region;
it is less intense in the upper dorsal and cervical part of the cord;
and often shows a division into three streaks, of which one, in the
middle line, occupies both columns of Goll, while on either side
another extends in the inner side of the posterior root-entry. The
posterior roots themselves are frequently atrophied, and exhibit a
firmer consistency and darker color than in health. On making
cross-sections of the spinal cord it is found that the gray
discoloration extends inward, involving more or less of the posterior
columns in well-defined areas. Microscopic examination shows that the
myelin of the nerve-tubes is either destroyed or atrophied, and the
axis-cylinder is found to be affected in the same way, usually
presenting alterations in its {841} refraction and diameter where it
is not entirely destroyed. In the maximum foci of disease the
conducting elements of the cord are nearly destroyed, and their place
is occupied by a firm connective substance made up of wavy bundles,
enclosing here and there a few atrophied axis-cylinders with wasted
myelin-sheaths. The blood-vessels participate in the morbid process,
at least as far as the larger vessels are concerned: these are
sclerotic, their lumen appears contracted, the adventitial sheath is
hypertrophied and occupied by a nucleated, fibrillar connective
substance. The capillaries are sometimes normal, or participate in the
morbid process to the extent of a thickening of the extra-endothelial
sheaths which is either hyaline or fibrillary, and may show
inflammatory cell-proliferation. It is evident that the increase of
connective tissue in the posterior columns is not merely a relative
one, due to the condensation of the normal neuroglia after the
disappearance of the normal nerve-tubes, but is the result of actual
proliferation. Opinion is somewhat divided as to whether this is to be
considered as an inflammatory interstitial proliferation or as a more
passive development of tissue compensating for the destroyed
conducting elements. My own observations incline me to the belief that
in syphilitic tabes the morbid process is essentially interstitial,
while in non-syphilitic tabes it is parenchymatous, the disease
beginning in the nerve-tubes proper. Perhaps the controversies between
pathologists as eminent as Schultze, Tuczek, Rumpf, Zacher,
Kahler-Pick, Rosenstein, and Adamkiewicz—who are rather evenly divided
into opposing camps, one maintaining the parenchymatous, the other the
interstitial, origin of the disease—may be solved when we learn to
distinguish between the non-syphilitic and syphilitic cases, which is
clinically, as yet, impossible. The difficulty of deciding what is a
parenchymatous and what is an interstitial disease of the cord is
considerably enhanced by the fact that the neuroglia, which by some at
least is regarded as an interstitial connective tissue, is derived,
like the nerve-substance proper, from the upper or neuro-epidermic
germ-layer of the embryo, and not from the mesoblast, which furnishes
all other connective substances in the body.
It is regarded as well established that the sclerosis of the areas of
the spinal cord which are affected is preceded by a stage of granular
degeneration.[36] This is supported by the facts that the sclerotic
areas are surrounded by a zone in which sclerosis has not yet
developed, but which is in a state of granular degeneration, and that
in more advanced tabes this belt is also found sclerosed. In paretic
dementia, a disease whose complicating cord affection closely
resembles that of locomotor ataxia, a granular degeneration of the
posterior columns is very common in earlier periods, while in later
periods a sclerotic tissue is found in the same locality.
[Footnote 36: Westphal, _Archiv für Psychiatrie_, ix. p. 725.]