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Dejerine-Sottas neuropathy
Also known as:
Dejerine's disease
Dejerine-Sottas atrophy
Dejerine-Sottas syndrome
Gombault’s degeneration or demyelination
Gombault's neuritis
Gombault’s syndrome
Associated persons:
Joseph Jules Dejerine
François Alexis Albert Gombault
Jules Sottas
Description:
A slowly progressive hereditary form of hypertrophic neuritis characterised by motor and sensory disturbance in the extremities commencing in childhood or adolescence, with numbness, par aesthesia, cramps, and lancinating pain in the extremities, followed by muscle weakness and clawing of the hands and deformities of the feet. Affected muscles show decreased responsiveness to electric stimulation. The nerve roots are thickened and show characteristic «onion bulb» histological changes (Schwann-cell hypertrophy). Ocular complications include myosis, nystagmus, anisocoria, papillotonia, and optic atrophy. Aetiology unknown.
Dejerine and Sottas in 1893 presented autopsy studies of a brother and sister with progressive atrophy of the muscles of the extremities and thus established definite genetic determinants. The disturbance is believed to be transmitted as an autosomal dominant trait.
Bibliography:
- F. A. A. Gombault:
Contribution à l'étude anatomique de la névrite parenchymateuse subaiguë ou chronique. Névrite segmentaire péraxiale.
Archives de neurologie, Paris, 1880, 1: 11-38.
- J. J.Dejerine, J. Sottas:
Sur la névrite interstitielle hypertrophique et progressive de l’enfance; affection souvent familiale et à debut infantile, caractérisée par une atrophie musculaire des extrémities, avec troubles marqués de la sensibilité et ataxie des mouvements et relevant d'une névrite interstitielle hypertrophique a marche ascendante avec lésions médullaires consécutives.
Comptes rendus des séances de la Société de biologie, Paris, 1893, 45: 63-96.
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