Diagnosis. The diagnosis of a case of family ataxia is almost selfevident if numerous members of the same or succeeding generations are affected, but in certain instances no such collateral cases exist. The differential diagnosis must then be made from tabes, chorea, and insular sclerosis. In tabes the gait lacks the cerebellar quality. Posterior sclerosis also presents visceral crises, lightning pains, vesical weakness, sensory disturbances, and pupillary symptoms. It is a disease of full adult life and has commonly a syphilitic history, and nystagmus is extremely uncommon in it. The cases of family ataxia occurring late in life usually present increased reflexes.

From chorea, for which it is frequently mistaken, especially in children, family ataxia may be distinguished by the major affection of the upper extremities in Sydenham's disease, its rather abrupt onset, and the absence of nystagmus, scoliosis, club-foot, and persistent abnormalities of the tendon reflexes. Huntingdon's family chorea of adult life has its mental features and forced attitudes and movements, which are greatly in excess of anything seen in the family ataxias.

Insular sclerosis presents the closest resemblance to family ataxia, especially Marie's form; and as it also may affect several members of the same family, the differentiation may be most difficult, if not impossible. We must recall its distinctive intention tremor, its lack of static instability, its scanning staccato speech, its spastic features and gait.

Prognosis. The outlook in these family ataxias is always gloomy. Aside from the helplessness there is no suffering, and, as a rule, they bear their lot with an apathetic good nature.

Treatment. The management of these cases does not promise much improvement. Some have thought that spinal stretching and electricity improved the incoördination. Carefully planned exercises may, perhaps, assist in the same direction.

HEREDITARY SPASTIC PARAPLEGIA.

Commencing with Strümpell, groups of cases have been recorded by Bernhardt, Philips, South, Newmark,i Achard and Fresson,2 Bayley, 3 and others, which present pure spasticity and familial traits. Bayley traced the disease through five generations, the individuals affected showing marked similarity in all respects. In his series it seemed that the disease once escaped did not reappear in the descendants.

There

The spastic condition may appear at any age from the first (Achard and Fresson) to the fifty-sixth year (Strümpell) and very commonly is preceded by some infectious disease, as typhoid, measles, etc. is paresis only in the terminal stages and no ataxia. The muscular hypertonus and increased reflexes are alone noted. Sensory and sphincter difficulties are absent and cerebral features are insignificant, or only such as are attributable to defects in the intracerebral portion of the

1" Medical News," Feb. 16, 1897.

2" Gaz. hebdom. de Med. et de Chir.," Dec., 1896.
3" Jour. Nerv. and Ment. Dis.," Nov., 1897.

motor pathway. The leg rigidity may, however, render the gait markedly spastic and walking finally impossible.

The symptoms are referable to the pyramidal tracts which, with the columns of Goll and the direct cerebellar tracts, were found degenerated by Strümpell. Jendrassek 1 believes that the process may begin in the brain, bulb, or cord, and is retrogressive in nature. From the familial feature of these cases it is evident that the basis of the paraplegia is a teratological defect and its sometimes late appearance is no bar to such belief.

Treatment promises little, though massage and carefully selected exercises may accomplish something.

The prognosis is not hopeful, but the course of the disease is usually very slow and for a given family nearly the same in all the affected members.

SCLEROSIS OF THE CORD DUE TO VEGETABLE INTOXICANTS— ERGOTISM, LATHYRISM, PELLAGRA.

Ergotism.-Epidemics of ergotism have arisen in various localities of the Old World, usually in times of bad harvest, when the people were reduced to eating ergoted grain. Brain symptoms in the form of mania are often associated, and epileptoid attacks are common. The disease respects neither age nor sex, and sometimes animals present similar cord symptoms. These, in man, consist of paresthesias, lightning pains, girdle sensations, analgesia, static instability, and an ataxic gait with obliteration of the patellar reflexes. Neither optic atrophy nor pupillary stasis occurs. In all recent autopsies a sclerosis confined to the posterior columns, and conforming closely to the changes in tabes, has been found. In many instances the symptoms develop after the poisonous food has been discontinued for some months. The tendency of the disease is toward recovery by progressive amelioration during several years. In some instances even the knee-jerk is restored. The mortality, however, in acute cases, is rather great. (For fuller particulars the reader is referred to the article of Tuczek, "Archiv f. Psychiatrie," Bd. xiii, S. 99.)

Lathyrism.2-Epidemics of this disease in Europe, Africa, and Asia have been noted for centuries. The cases are marked by rigidity of the lower extremities coming on within a few days, and paraplegic symptoms, with weakness of the bladder, and generally end in slow recovery. They coincide with famine conditions, when the people eke out their subsistence with the various vetches, such as Lathyrus sativus, or cicera, or clymenum. The morbific constituent appears to be an alkaloid not destroyed by ordinary cooking. The symptoms come on gradually or sometimes rather abruptly, and a spasmodic paraplegia is developed, producing the paraplegic state, so far as motion and locomotion is concerned. Sensation is not materially disturbed, though paresthesias and some blunting of cutaneous sensation are usual. Mental symptoms and

1"Deutsch. Arch. f. klin. Med.," Bd. lviii.

2

Brunelli, Trans. Seventh Internat. Cong.," vol. ii, p. 45.

cerebral features, including involvement of cranial nerves, seem to be lacking. The exact lesion is not known, but the symptoms all point to disturbance in the lateral tracts, or, perhaps, to a posterolateral sclerosis with preponderating spasticity. Often such cases suggest a focal lesion of the cord, but the absence of serious sensory disturbance and the complete recovery that, as a rule, ensues are not consistent with such a point

of view.

Pellagra is sometimes confounded with ergotism. It has occurred mainly in poverty-stricken Italian districts, and is traceable to the use of diseased and fermenting maize. It produces mental symptoms that usually call for asylum treatment. In an acute form, called pellagrous typhus, it may rapidly cause death, and then myelitis, enteritis, meningitis, and lymphatic adenitis plainly declare the toxemic nature of the malady. Many cases are marked by a rough, intense, rapidly extending erythema, usually commencing about the neck. Belmondo considers the disease to be due to a specific organism. Lombroso attributes it to a fungus in the maize.

The cord-lesions that are found consist of a leptomeningitis, often with much thickening, and even with the formation of osseous plaques. In the cord itself the anterior cornual cells are frequently atrophied and pigmented. There is commonly a posterolateral sclerosis. This affects the colums of Goll and Burdach, mainly in the upper cord-levels, but spares the root-zone of the postero-external column. The crossed pyramidal tract, especially the lower portion, is also sharply sclerosed, the direct cerebellar tract usually escaping.

The spinal symptoms correspond. Ataxia is most marked in the upper extremities; spasticity is pronounced in the lower limbs. The iridian reflexes are spared, and cutaneous sensibility is not much affected. Strangely, in spite of the usual changes in the anterior cornual cells, muscular atrophy is insignificant. The disease, clinically and anatomically, presents much resemblance to paretic dementia.

PART VI.

DISEASES OF THE GENERAL NERVOUS SYSTEM WITH KNOWN ANATOMICAL BASIS.

AMONG the various diseases that have already been described, several have presented symptoms referable to the various portions of the entire nervous apparatus and properly belong to this section. Thus, in multiple neuritis all nervous levels may be invaded. Cerebral, spinal, and nervetrunk lesions are all encountered. The same is true of cerebrospinal meningitis, tabes, paretic dementia, ergotism, lathyrism, and pellagra; but for purposes of comparison as well as contrast it was thought most expedient to take them up in the order chosen, being guided by the preponderance of nerve-trunk or spinal-cord features in each instance. To those named we here add multiple insular sclerosis and syphilis of the nervous system. With some of the syphilitic lesions we are already acquainted.

CHAPTER I.

MULTIPLE CEREBROSPINAL SCLEROSIS.

MULTIPLE cerebrospinal sclerosis-multiple sclerosis, insular sclerosis, sclerose en plaques, or disseminated sclerosis-is a disease secondary usually to some intoxication or infection often of a mixed sort. It is marked by numerous islets or plaques of sclerosis irregularly distributed in the brain, cord, and cranial nerves. These are related to the bloodsupply of the part and probably primarily vascular.

For

Etiology. This sclerotic disease is not very common. instance, Uhthoff in six or seven years could only gather about 100 cases from all the hospitals and clinics of Berlin. It affects both sexes indifferently and presents a large preponderance of cases between the ages of twenty and thirty years. Marie asserts its absolute rarity after forty, but cases do occur. Children are not infrequently affected and it may even be congenital. Considerable stress was formerly laid upon

heredity, but this element is chiefly manifest in a neuropathic tendency. Erb, Oppenheim, and Duchenne have cited examples of direct succession, and it has, in a small number of instances, affected several children of the same family. Hervouet 1 has even seen nine cases in one generation. Overwork, cold, traumatism, and various excesses have been accused as causative. Such relation is subject to every doubt, though all these influences are capable of aggravating the disease when once established and of precipitating additional manifestations of its activity. The most important etiological factors are the infections. Typhoid, pneumonia, malaria, measles, scarlet fever, small-pox, diphtheria, whooping-cough, erysipelas, dysentery, cholera, and influenza have been followed by the development of the symptoms of multiple sclerosis within a few weeks or months.2 Oppenheim 3 found that in eleven

out of twenty-eight cases treated by him the patients had long been exposed to the influence of lead, copper, zinc, etc., and emphasizes the significance of occupations attended by such intoxications. Charcot, in one of his later lectures, reported a case consecutive to cerebral rheumatism. Infection may explain some of the family groups of multiple sclerosis and Oppenheim's observation of the disease in a mother and child. From the numerous and often mixed infections noted, it seems unlikely that we have to deal with a specific organism. It is also a debated question whether the morbific microbes act through their elaborated poisons or by embolic colonization. It is, however, quite definitely settled that the irritative influence reaches the central nervous organs through the blood-channels and there sets up the localized scle

"Gaz. des Hôpitaux de Toulouse," 1893. 2 Williamson, "Brain," 1896.

3"Berlin. klin. Wochens.," March, 1896.