AMYOTROPHIC LATERAL SCLEROSIS: ORIGIN AND EXTENT OF THE UPPER MOTOR NEURON LESION

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 Charcot¹ in 1865, was later confirmed by other observers. The gross atrophy of the motor convolutions was observed by Kahler and Pick² in 1879. Kojewnikoff³ 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's³ observations, also demonstrated the disappearance of the giant pyramidal cells from the motor cortex. These changes were not always constant, and in 1 instance Marie⁵ was unable to detect them. Sarbó,⁶ Rossi and Roussy,⁷ Probst,⁸ Campbell⁹ and Spiller,¹⁰ in further contributions, stressed the involvement of area 4; Rossi and Roussy,⁷ Probst⁸ and Campbell