Spinocerebellar ataxia type 10 in the South of Brazil: the Amerindian-Belgian connection

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Spinocerebellar ataxia type 10 in the South of Brazil: the Amerindian-Belgian connection.

Spinocerebellar ataxia type 10 (SCA10) is a rare form of autosomal dominant ataxia found predominantly in patients from Latin America with Amerindian ancestry. The authors report the history of SCA10 families from the south of Brazil (the states of Paraná and Santa Catarina), emphasizing the Belgian-Amerindian connection.

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Spinocerebellar ataxia type 6 in Brazil.

Spinocerebellar ataxia type 6 (SCA 6) is an autosomal dominant cerebellar ataxia caused by CAG repeat expansion in the SCA6 gene, a alpha 1A voltage-dependent calcium channel subunit gene on chromosome 19p13. SCA-6 is characterized predominantly by slowly progressive pure cerebellar ataxia with late onset. We report three index patients, with pure, late onset, cerebellar ataxia, belonging to th...

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Cognitive impairment in Spinocerebellar ataxia type 10

Background: Cognitive and psychiatric dysfunction has been described in several neurodegenerative diseases but has not been systematically evaluated in spinocerebellar ataxia type 10 (SCA10). Objective: The aim of the present study was to investigate the core cognitive features in a large cohort of Brazilian patients with SCA10, comparing the results against a healthy control group. Methods: Tw...

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The history of spinocerebellar ataxia type 10 in Brazil: travels of a gene.

The authors report the history of spinocerebellar ataxia 10 (SCA10), since its first report in a large Portuguese-ancestry family with autosomal dominant pure cerebellar ataxia, till the final identification of further families without Mexican ancestry. These families present a quite different phenotype from those SCA10 families described in Mexico.

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ژورنال

عنوان ژورنال: Arquivos de Neuro-Psiquiatria

سال: 2015

ISSN: 0004-282X

DOI: 10.1590/0004-282x20150086