Amyotrophic lateral sclerosis is a disease characterized by: (1) muscular atrophy with fibrillary twitchings, (2) spasticity with evidence of lesions of the upper motor neurons and (3) symptoms of disorder in the brain stem, especially its bulbar portion. Although Charcot 1 (1870) was first to differentiate ordinary muscular atrophy and amyotrophic lateral sclerosis, he said little about psychic disturbances not essential to a diagnosis of the disease but sometimes associated with it.
Westphal2 (1885) and Zacher3 (1886), in isolated cases, noted amyotrophic lateral sclerosis associated with dementia paralytica. Oppenheim and Siemerling4 (1886) were the first to consider and separate various types of purely bulbar palsy. They thought that the explosive emotional reactions in such cases were due to lesions of the upper motor neurons that end in the nuclei. Marie5 (1892), thinking psychic disturbances fairly common in amyotrophic lateral sclerosis, was among the first to call