Prognosis.—The duration of the dis ease is indefinite. Death may occur from a bedridden asthenia, but is usually due to some intercurrent affection. The dis ease may be complicated with insanity.
Treatment.—There is little to be done for these patients. Suspension has been tried, but has proved about as useless as in true tabes. Arsenic is at times beneficial. The Fraenkel method is in dicated for the symptomatic improve ment of the ataxia. Prevention of the disease by means of careful selection in marriage, or, better still, celibacy among the tainted, is much the more hopeful and legitimate line of action. Should the disease appear in the first child, fur ther pregnancies or births should be pre vented. In the light of the known pathology and the probable pathogenesis, the idea of preventing the development of the disease by withdrawing the infant from the mother's breast, as has been suggested, seems, to say the least, far fetched.
Ataxic Paraplegia.
Synonyms.—Progressive spastic ataxia; combined postero-lateral sclerosis.
Definition.—As described by Gowers, it is a combination clinically of ataxia and spastic paraplegia, haying an an atomical basis in lesion of the dorsal and lateral columns. The disease, while presenting a more or less constant clin ical symptom-picture, is probably not a distinct pathological entity.
Symptoms. — The clinical picture is usually clear cut aLd constant. The first symptom is ordinarily that of con stant fatigue, with more or less un steadiness in standing or walking. This ataxia is especially marked in the dark or with the eyes closed. The sphincters may be affected at the same time and sexual power lost or impaired. There are no sensory symptoms except, perhaps, a subjective aching in the legs and lum bar region. Paretic weakness in the legs, particularly the flexors, gradually and progressively develops. One leg may be more affected than the other at first. More or less rigidity, with exaggerated knee-jerks, clonus, and contractures, de velop. The patient becomes more and more dependent upon assistance in walk ing, spreading the feet wide apart with eyes fixed upon the floor. The feet are dragged along, however, and not lifted to an unnecessary height and brought down with unnecessary force as in true tabes. The cranial nerves are rarely in volved, but the mind undergoes degen erative deterioration in the advanced dis ease, the mental changes being identical in character, often, with those of gen eral paresis. The arms may be affected in the same way as the legs, with spastic paralysis and inco-ordination. Trophic
symptoms are absent.
Diagnosis.—The clinical diagnosis is quite simple. The absence of pupillary changes, of sensory symptoms, and of Westphal's symptom excludes true tabes readily. The spasticity and exaggerated reflexes with clonus may suggest primary lateral sclerosis, but there is no ataxia in the latter ehiection. Ataxia and para pareses, with exaggerated knee-jerks, may be present in disseminated sclerosis, but there will be, in addition, involve ment of the cranial nerves, intention tremor, scanning speech, nystagmus, etc. Tumor affecting the base of the brain and involving the cerebellum may in duce symptoms of inco-ordination and spastic paralysis; but here, again, the addition of cranial-nerve symptoms, especially of the optic nerve, will clear away any temporary confusion.
Etiology.—As with most of the degen erative spinal scleroses,ataxic paraplegia is most common in males during middle life, and the causes, so far as known, are also practically the same. Gowers, Osier, and others deny the relationship of syphilis as a cause except in rare in stances: a statement, however, which is I disputed by the majority of observers.
Lead and other poisons may superinduce the disease. Heredity is a minor factor if. it exists at all.
Pathology.—The pathology is a mat ter of much dispute. As described by Gowers, the lesions consist of sclerosis of the posterior and lateral columns, which is very variable in extent and posi tion and not strictly "systemic" in char acter, the mixed. zone of the lateral and the lateral limiting layer between the pyramidal fibres and the gray matter being involved quite often. In the posterior columns the sclerosis is fre quently more marked in the dorsal than in the lumbar segments. Occasionally a zone of sclerosis has been found in the entire periphery of the cord (annular sclerosis). Tfirck's columns may be af fected. Marie, quoted by Osier, does not consider it a systemic disease. He be lieves the distribution of the sclerosis to be dependent upon the arterial sup ply through the branches of the dorsal spinal artery, which are involved. By many the disease is believed to be a form of chronic middorsal myelitis, by others simply an atypical form of tabes, and by others still an atypical variety of multiple sclerosis. An identity with general pare sis ascending has been maintained. Such wide-spread variations in opinion neces sarily leave the final decision still sub judice.