Dejerine–Sottas disease
| Dejerine–Sottas syndrome | |
|---|---|
| Other names | Charcot–Marie–Tooth disease type 3, Dejerine–Sottas syndrome, hereditary motor and sensory polyneuropathy type III |
| MRI compatible with Dejerine-Sottas type spinal nerve enlargement | |
| Specialty | Neurology |
| Symptoms | Weakness, reduced muscle tone, loss of sensation in extremities |
| Usual onset | Infancy or early childhood[1] |
| Causes | Genetic mutations[2] |
| Diagnostic method | Medical imaging[1] |
Dejerine–Sottas disease (also known as Dejerine–Sottas syndrome,[3] hereditary motor and sensory polyneuropathy type III, and Charcot–Marie–Tooth disease type 3) is a hereditary neurological disorder characterized by damage to the peripheral nerves, demyelination, and resulting progressive muscle wasting and somatosensory loss. The condition is caused by mutations in various genes and currently has no known cure.[2]
The disorder is named for Joseph Jules Dejerine and Jules Sottas, French neurologists who first described it.[4][5]
- ^ a b Cite error: The named reference
Access Medicinewas invoked but never defined (see the help page). - ^ a b Muscular Dystrophy Association (2012). "Dejerine-Sottas Disease". Archived from the original on 4 January 2012. Retrieved 7 May 2012.
- ^ Satran, R. (1980). "Dejerine-Sottas Disease Revisited". Archives of Neurology. 37 (2): 67–68. doi:10.1001/archneur.1980.00500510025002. PMID 7356409.
- ^ synd/1736 at Whonamedit?
- ^ Dejerine, J. J.; Sottas, J. (1893). "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" (PDF). Comptes Rendus des Séances de la Société de Biologie. 45. Paris: 63–96.