Amyotrophic Lateral Sclerosis With Mental Symptoms

Amyotrophic lateral sclerosis is generally regarded as a disease limited to involvement of the motor system, mainly the pyramidal tracts, the bulbar nuclei and the anterior horn cells. Degeneration of the upper motor neuron begins in the giant pyramidal cells of Betz and extends throughout the neuraxis. The occurrence of mental symptoms was not mentioned by Charcot, but subsequent observers called attention to their existence in isolated cases. The question has always arisen whether the psychotic manifestations were part of the syndrome of amyotrophic lateral sclerosis and could be attributed to the same degenerative process or were the result of cerebral changes which by chance happened to be associated with the upper and lower motor neuron disease. While the cases we shall report do not entirely answer the question, the three that came to necropsy, we believe, throw some light on the problem. REPORT OF CASESCase 1.—History. —H.