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Article

[Does the ataxo-choreic form of DRPLA exist in Europe? Search of mutation in 120 families].

Revue neurologique - 1 Nov 1995

Dubourg O, Dürr A, Chneiweiss H, Stevanin G, Cancel G, Penet C, Agid Y, Brice A

Abstract excerpt

Dentatorubral-pallidoluysian atrophy (DRPLA) is an autosomal dominant neurodegenerative disorder characterized by a degeneration of cerebellar and pallidal efferents, more frequent in Japan. Isolated cases are also encountered. Patients present with variable combination of signs including myoclonus, ataxia, epilepsy, choreoathetosis and dementia, with onset from childhood to the seventh decade. Clinically, DRPLA...

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Identifiers

PMID: 8745629

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PubMed metadata and Literature Corpus relationships. Corpus snapshot updated 7 Sept 2026. Manifest publication-graph-cohort-2026-09-08.1.

Source receipt: pubmed, retrieved 7 Sept 2026.

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[Does the ataxo-choreic form of DRPLA exist in Europe? Search of mutation in 120 families].PMID 8745629Read ↗

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

Late onset ataxia phenotype in dentatorubro-pallidoluysian atrophy (DRPLA).Semantic proximity Read ↗

Dentatorubro-Pallidoluysian Atrophy (DRPLA) among 700 Families with Ataxia in Brazil.Semantic proximity Read ↗

Dentatorubral and pallidoluysian atrophy (DRPLA). Clinical and neuropathological findings in genetically confirmed North American and European pedigrees.Semantic proximity Read ↗

Dentatorubral-pallidoluysian atrophy (DRPLA). Molecular basis for wide clinical features of DRPLA.Semantic proximity Read ↗

Portuguese families with dentatorubropallidoluysian atrophy (DRPLA) share a common haplotype of Asian origin.Semantic proximity Read ↗

Clinical and genetic characteristics of non-Asian dentatorubral-pallidoluysian atrophy: A systematic review.Semantic proximity Read ↗

Unstable expansion of CAG repeat in hereditary dentatorubral–pallidoluysian atrophy (DRPLA)Semantic proximity Read ↗

Genetic analysis of a dentatorubral-pallidoluysian atrophy family: relevance to apparent sporadic cases.Semantic proximity Read ↗

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The first identified French family with dentatorubral-pallidoluysian atrophy.Semantic proximity Read ↗

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