A rare disease of the nervous system with degeneration of the nerves conducting signals to muscles. It is characterized by early asymmetric weakness, usually in one limb, followed by fatigue, cramps, and progressive wasting and fibrillation of the muscles of the upper extremity and weakness and spasticity of the lower extremity. Death occurs within three years from the onset of symptoms in about half of all cases. Sporadic occurrence is the rule, but a high incidence of familial cases has been reported. When occurring in younger persons, the illness appears to be transmitted as an autosomal dominant trait. The gene causing the disease was found in 1993. The disorder usually affects adults over 50 years of age but may occur in persons aged 20 to 76 years. Male-female ratio is 2:1.
In media, the disorder is commonly referred to as Lou Gehrig's disease, after Henry Louis (Lou) Gehrig, an American baseball player who died from it in 1941.
- J. M. Charcot, Alexis Joffroy:
Deux cas d’atrophie musculaire progressive avec lésions de la substance grise et des faiseaux antérolatéraux de la moelle épinière.
Archives de physiologie normale et pathologique, Paris, 1869, 2: 354-367, 629-649, 744-760.
- J. M. Charcot:
Des amyotrophies spinales chroniques. Progrés médical, Paris, 1874, 2: 573-574.
- J. M. Charcot, P.Marie:
Deux nouveaux cas de sclérose latérale amyotrophique suivi d'autopsie.
Archives de Neurologie, 1885.