ill] Tin' ( 1 orti'.r hi Aiiii/iitropliif Lateral Sclerosis 39 



Ch;uvi>t anil Marie noted a disappearance of giant cells from the paracentral lobule. 



In addition to these observers, who confined their attention to the nerve cell, others, 

 employing the method of Marchi, have seen degeneration in nerve fibres in the white substance 

 underlying the central convolutions; indeed, Kowjewnikoff, of Moscow, has succeeded in tracing 

 degeneration from cortex to internal capsule. 



I have no doubt that other publications could be referred to bearing on this topic, but 

 1 hope I have cited enough to show that the occurrence of cortical changes in Amyotrophic 

 Lateral Sclerosis, in some cases, at any rate, is well established. 



I can now proceed with the account of the two cases which I have investigated, and 

 to remove doubt concerning the correctness of the diagnosis, I will briefly narrate the 

 clinical and pathological data on which that diagnosis was based. 



CASE No. 1. 

 CLINICAL HISTORY. 



.1. S., male, aet. 24, was admitted into Rainhill Asylum in March, 1889. 



In April, 1898, nine years after admission, it was reported that he was "losing weight and apparently 

 failing in general health," he also complained of vague muscular pains (evidently cramp) and of weakness, 

 but the cause of these was not then discovered. He was kept under observation, however, and, in July, 

 developed the first signs of Amyotrophic Lateral Sclerosis, for in both hands, flattening of the thenar eminence, 

 undue prominence of the metacarpal bones, and hollowing between the same bones on the dorsiun of the 

 hand, all pointing to wasting of the thenar muscles and interossei, were noticed. 



In October, the wasting had spread to all the muscles of the fore-arm, upper arm and shoulder, 

 the trapezius was likewise affected, and in some of these muscles fibrillary switchings were observed. The 

 head of the humerus and spine of the scapula were beginning to stand out prominently and he was barely 

 able to flex his arm at the elbow or elevate his shoulders. At this period, also, the gait was unsteady, 

 but although the legs were thin in general, no muscle, or group of muscles, could be pointed to as specially 

 affected. The knee jerks were active, ankle clonus present, and wrist tap contractions obtainable. There 

 \v;is no impairment of sensation to touch and pain, or to heat and cold. The pupils reacted normally to light 

 and accommodation, and sight, hearing, smell and taste seemed acute. Examination of the thoracic and 

 abdominal organs brought no positive result. 



Six months later March, 1899 the patient had lost the power to walk, and the atrophy to a further 

 degree had invaded the leg muscles, especially the extensors of the knee and foot, and the reflexes were 

 not so active. There were signs also of an involvement of trunk muscles, but those of the neck were int.i. i. 



At this time, the electrical reactions were carefully tested, but unfortunately the details of this examina- 

 tion have been lost, and my notes only contain a brief statement to the effect that there was a moderate 

 diminution nf the excitability of many of the leg muscles, while in the case of some of the arm muscles 

 the change had gone on to extinction. 



The further history of the case is uninteresting; he remained bedridden and, month by month, as the 

 atrophy progressed, grew more powerless. When addressed he could generally give an intelligent reply, and 

 this was .spoken in a clear voice. He wore a drawn, haggard expression, but there was no paralysis of 

 facial or ocular muscles. Not until the later part of his illness did he lose control over his sphincters. 

 He suffered from no trophic lesions at any time and, so far as could be judged, no sensory troubles. Towards 

 the end, atrophy of the intercostal muscles impeded respiration, and on January 29th, 1899, eighteen months 

 after the first muscular atrophy was noticed and the disease positively diagnosed, the supervention of deglutitory 

 troubles brought about a fatal termination. 



