[Roussy-Levy hereditary areflexic dysstasia. Its historical relation to Friedreich's disease, Charcot-Marie-Tooth atrophy and Dejerine-Sottas hypertrophic neuritis; the present status of the original family; the nosologic role of this entity].
This survey of Roussy-Lévy disease begins with an historical account of the three neurological conditions from which this entity has been separated: Friedreich disease, described in 1861-1863, which proved with time to be a genuine anatomoclinical disorder: Charcot-Marie-Tooth atrophy, described in 1886, particular because of its morphology and evolution but due to various processes: Dejerine-Sottas hypertrophic neuritis, described in 1893, which was the first variant to be individualized within the heterogenous group of primary and familial hypertrophic neuritis. The initial description of Roussy-Lévy disease--in 1926, 1932, and 1934--and the controversies raised by this concept are recalled as well as the present state of the original family: five out of seven members have been examined since 1956 and it has been demonstrated that they are suffering from a form of hypertrophic neuritis. However it is this author's opinion that the concept of an autonomous Roussy-Lévy disease within hypertrophic neuritis is justified by the following criteria: dominant transmission, very precocious onset, extreme slowness of the evolution, remarkable benignity of the prognosis.
D001259:Ataxia / Q000175:diagnosis / Q000235:genetics / Q000266:history*; D002607:Charcot-Marie-Tooth Disease / Q000266:history*; D005260:Female; D005602:France; D005621:Friedreich Ataxia / Q000266:history*; D049672:History, 19th Century; D049673:History, 20th Century; D006801:Humans; D006984:Hypertrophy; D008297:Male; D009133:Muscular Atrophy / Q000145:classification / Q000266:history*; D009443:Neuritis / Q000235:genetics / Q000266:history*; D010525:Peripheral Nerves / Q000473:pathology; D012021:Reflex, Abnormal / Q000235:genetics / Q000266:history*
La dystasie aréflexique héréditaire de Roussy-Lévy. Ses rapports historiques avec la maladie de Friedreich, l'atrophie Charcot-Marie-Tooth et la névrite hypertrophique de Dejerine-Sottas; l'état actuel de la famille originale; la place nosologique de cette entité.