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

Lapresle, J.

Revue Neurologique (Paris) 138(12): 967-978


ISSN/ISBN: 0035-3787
PMID: 6763298
Accession: 044260291

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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.