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Article

Prevalence of dentatorubral-pallidoluysian atrophy in a large series of white patients with cerebellar ataxia.

Archives of neurology - 1 Aug 2003

Le Ber Isabelle, Camuzat Agnès, Castelnovo Giovanni, Azulay Jean-Philippe, Genton Pierre, Gastaut Jean-Louis, Broglin Dominique, Labauge Pierre, Brice Alexis, Durr Alexandra

Abstract excerpt

BACKGROUND: Dentatorubral-pallidoluysian atrophy (DRPLA) is a rare neurodegenerative disorder mainly diagnosed in Japan. Its prevalence is low in other countries. Three phenotypes are described: choreoathetoid movements, cerebellar ataxia, and progressive myoclonic epilepsy. OBJECTIVE: To evaluate the frequency of DRPLA in European patients with sporadic or autosomal dominant cerebellar ataxia. METHODS: We...

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Identifiers

PMID: 12925365
DOI: 10.1001/archneur.60.8.1097

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