AMYOTROPHIC LATERAL SCLEROSIS

Abstract

Amyotrophic lateral sclerosis is regarded generally as a disease of the upper and lower motor neurons. The clinicoanatomic description of the disorder, which was first made by Charcot 1 in 1865, was later confirmed by other observers. The gross atrophy of the motor convolutions was observed by Kahler and Pick 2 in 1879. Kojewnikoff 3 was the first to trace the degeneration of the pyramidal fibers from the motor cortex into the internal capsule, peduncles, pons, medulla oblongata and spinal cord. Charcot and Marie, 4 who confirmed Kojewnikoff's 3 observations, also demonstrated the disappearance of the giant pyramidal cells from the motor cortex. These changes were not always constant, and in 1 instance Marie 5 was unable to detect them. Sarbo, 6 Rossi and Roussy, 7 Probst, 8 Campbell 9 and Spiller, 10 in further contributions, stressed the involvement of area 4; Rossi and Roussy, 7 Probst 8 and Campbell