In regard to these very acute cases, it seems to me uncertain whether the disease should be attributed to the syphilis. In my own case twenty years had elapsed since the chancre, alcohol was habitually used in great excess, and the attack was apparently precipitated by great exposure. On the other hand, the man bore well enormous doses of iodide of potassium, and lowly progressed under them.
Finally, there is a class of disease of the spinal cord in which the lesion is undoubtedly the direct outcome of a syphilitic diathesis. In these cases the exudation commences primarily in the membranes of the cord, and may extend into the cord itself. In this class I would include the first two varieties of syphilitic spinal disease of Huebner. The number of recorded autopsies is not great; the only cases with which I am acquainted are those referred to in the note at the bottom of this page.73
73 Winge (Dublin Med. Press, 2d Series, vol. ix., 1863); Moxon (Dublin Quarterly Journ., li., 1870); Charcot and Gombault (Archiv. d. Physiologie, tome v., 143, 1873); Schultze (Archiv. Psychiat., xii. 567); Thos. Buzzard (Diseases of Nervous System, 1882, p. 407); Julliard (Étude Crit. sur les Localis. Spinal de la Syphilis, 1879); Westphal (Arch. Psychi., vol. xi.); Greif (Arch. Psychiat., xii. 579); Homolle (Progrès méd., 1876).
The lesions in these cases are entirely similar to those of brain syphilis. The disease very rarely or never begins in the interior of the cord. I know of no recorded case: Wagner's case, in which a yellow nodule was found within the cervical marrow, was probably not one of syphilis. If a gummatous inflammation does occur inside of the cord, it probably starts from the ependyma. The gummatous exudate may occur in the form of small multiple formations or of an extensive meningitis, with an infiltration of the membranes and their spaces with gummatous material. The membranes are usually agglutinated with one another and with the surface and with the cord. The exudation is usually made up of roundish cells, and in several cases spindle-shaped cells have been noticed, as have also the peculiar Deitres corpuscles already described as they occur in brain syphilis. The changes in the cord itself vary somewhat. In Winge's case the white matter seems to have undergone a rapid myelitis from pressure. It was of a grayish color, with numerous fine granular masses, corpora amylacea, pigment-masses, and fatty globules, the nerve-fibres being broken up. In other cases the change has been a sclerosis. The vessels of the cord have been noticed by various observers in the different stages of the degeneration seen in syphilis of the brain. They are often greatly dilated, their walls thickened, and, together with the lymph-spaces, infiltrated with small cells. Minute hemorrhages have been found.
The so-called syphilitic callus, as described by Heubner, is probably the remnant of a true gummatous inflammation. It consists of a circumscribed induration one to several lines in thickness, originating apparently from the dura mater, and causing sometimes adherence with the vertebræ, in others with the membranes of the spinal cord. In a case described by Virchow of this character the lesion was cervical, and the symptoms were stiffness in the nape of the neck, pains in the neck and arms, and finally paralysis in both arms. A second case is elaborately described by Heubner in his article in Ziemssen's Encyclopædia.
SYMPTOMS.—As the lesion of gummatous spinal syphilis affects primarily membranes of the cord, in the beginning of the attack the symptoms chiefly arise from the implication of the nerve-roots. Of course these symptoms vary with the seat of the lesion, for it must be remembered that the meningeal irritation is at first usually localized in a small region. As in a majority of cases this lesion affects a posterior portion of the cord, and as the posterior nerve-roots seem especially sensitive to irritations of this character, pain is usually a very marked and precocious symptom of spinal syphilis. The seat of the pain varies with the seat of the lesion. At first the pain is slight, but in most cases it soon becomes severe. It is sometimes situated at a fixed spot on the spinal column, where, according to Heubner, it may be increased on pressure. I have seen two or three such cases, but have and still do believe that under these circumstances the patient was suffering not simply from a spinal syphilis, but also from an implication of the vertebral periosteum or of the vertebræ themselves. In one of my cases this diagnosis was confirmed at the autopsy. When the lesion is purely meningeal there is probably no marked local tenderness. The severe pains usually felt in the extremities or in the trunk are often fulgurant; sometimes they are described as resembling the thrust of a knife, and not rarely they closely resemble the pains of locomotor ataxia. In some instances the pains are comparatively slight and are aching in character. Paræsthesiæ are not rare phenomena: such are formications, tingling in the extremities, numbness and feeling as though the limb were asleep, intense sense of coldness on the surface, sensation of water running over the limb. Early in the disorder there is sometimes very marked hyperæsthesia, but later, even though the pain persists, blunting of sensibility is marked, and there may be a complete anæsthesia. This anæsthesia is sometimes localized in certain parts of the limb. Thus, in a case reported by Alfred Mathieu,74 although there was complete anæsthesia of the outer side of the left leg and foot, the inner side retained its normal sensibility. In some cases there is the abdominal cincture of ordinary myelitis. The records show that even in these early stages there may be diplopia, amblyopia, or other disorder of vision, and the pupil may be distinctly affected. In these cases it is probably the upper portion of the cord which is affected.
74 Ann. de Dermatol. et Syph., vol. iii., 1882.
Disturbances of motility in the majority of cases do not develop until some time after sensation has been affected, but may come on very early. Usually, the first symptoms are those of irritation, such as rigidity of the neck, back, and limbs or even of isolated groups of muscles. Tremors have been described as frequently present. These may be convulsive, and are often plainly reflex in their origin; indeed, I am inclined to believe that they are always reflex tremblings, and never true tremors. Heubner describes a case in which a paralyzed limb was thrown into violent tremblings whenever passive motion was attempted. The patella-reflex is usually grossly exaggerated, although it may be lost in the later stages of the disorder. Not rarely there is the condition which has received the misnomer of spinal epilepsy. This exaggeration of the reflexes may be limited to one leg, when it is almost pathognomonic. In some cases severe cramps are excited by movement. Usually there is no tenderness. These symptoms of the meningitic stage may continue for weeks or months without there being pronounced paralysis, although locomotion is not rarely interfered with by the stiffness of the legs. Finally, if the case progresses the patient notices a weakness in one or both legs, or (if the disease be situated high up in the spinal cord) in one arm, which rapidly increases until there is almost complete loss of power. This rapid increase of palsy following long-continued disturbance of sensation is almost pathognomonic. In most cases one side of the body is more affected than the other. The sphincters are prone to be implicated, and in advanced stages of the disease there is usually complete loss of control over the bladder and rectum. The patient may live for months without very distinct change of this condition, or bed-sores and other trophic disturbances may rapidly develop and death ensue in a short time. I have seen under these circumstances marked elevation of temperature, rapid feeble pulse, mental weakness, and the general symptoms of septicæmia last for many weeks. Ammoniacal cystitis is of course prone to be developed during this stage. When motility fails, sensibility is usually blunted, although the pains may even increase. Heubner affirms that an incompleteness of the anæsthesia is characteristic of the disorder.
The typical course of spinal syphilis, such as has been described, may be variously departed from. Sometimes the power of co-ordination is early affected, and the symptoms may resemble those of locomotor ataxia. I doubt, however, whether under any circumstances there is a loss of the patella-reflex in the early stages of the gummatous disease of the spinal cord. In other cases the paralytic symptoms may be very prominent from the beginning: thus, in the case of R. P——, aged 27, which I believed to be gummatous disease of the spinal cord, the first disorder was a feeling of malaise lasting for about a week, followed by the sudden, rapidly-developed paralysis of the bladder, loss of power in the legs, and to a less extent in the arms, the only pain being a dull, steady ache in the arms. The bowels were obstinately costive. Double vision was soon very pronounced. When I first saw the patient, about three weeks after this, there was decided impairment of sensibility in the legs, but not in the arms; marked muscular weakness of both legs and arms; no loss of co-ordinating power; dropping of the right eyelid, with double vision; and only some slight aching pains in the arms. By the use of large doses of iodide of potassium and other appropriate measures a good recovery was secured.
A case illustrating the occasional difficulty of diagnosing spinal syphilis is reported by C. Eisenlohr.75 The first symptom was obstinate constipation, with very great discomfort after defecation; then appeared incontinence of urine with weakness of the legs: finally, a sudden complete palsy of the right leg occurred, with marked anæsthesia in both legs, partial loss of power in left leg, violent boring abdominal pains, and distress in the bladder. In the last stages there were severe neuralgic pains in both legs, with complete loss of sensation, bed-sores, atrophy of the leg-muscles, with reactions of degeneration, and death from exhaustion. At the autopsy an advanced meningitis was found which had apparently commenced in the regions of the cauda equina, and given rise to complete degeneration of the nerves. The only alteration of the cord was an ascending degeneration of the posterior columns.