Spinocerebellar ataxia type 10: Frequency of epilepsy in a large sample of Brazilian patients

Hélio A G Teive, Renato P. Munhoz, Salmo Raskin, Walter O. Arruda, Luciano De Paola, Lineu C. Werneck, Tetsuo Ashizawa

Research output: Contribution to journalArticle

32 Scopus citations

Abstract

Spinocerebellar ataxia type 10 (SCA10) is an autosomal dominant disorder caused by an ATTCT repeat intronic expansion in the SCA10 gene. SCA 10 has been reported in Mexican, Brazilian, Argentinean and Venezuelan families. Its phenotype is overall characterized by cerebellar ataxia and epilepsy. Interestingly, Brazilian patients reported so far showed pure cerebellar ataxia, without epilepsy. Here, authors provide a systematic analysis of the presence, frequency and electroencephalographic presentation of epilepsy among 80 SCA10 patients from 10 Brazilian families. Overall, the frequency of epilepsy was considered rare, been found in 3.75 % of the cases while this finding in populations from other geographic areas reaches 60% of SCA10 cases.

Original languageEnglish (US)
Pages (from-to)2875-2878
Number of pages4
JournalMovement Disorders
Volume25
Issue number16
DOIs
StatePublished - Dec 15 2010

Keywords

  • Autosomal dominant cerebellar ataxia
  • Epilepsy
  • SCA
  • Spinocerebellar ataxia type 10

ASJC Scopus subject areas

  • Neurology
  • Clinical Neurology

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