Cognitive dysfunction in spinocerebellar ataxias.

ABSTRACT: Spinocerebellar ataxias (SCAs) comprise a heterogeneous group of complex neurodegenerative diseases, characterized by the presence of progressive cerebellar ataxia, associated or otherwise with ophthalmoplegia, pyramidal signs, extrapyramidal features, pigmentary retinopathy, peripheral neuropathy, cognitive dysfunction and dementia.

Objective

To verify the presence of cognitive dysfunction among the main types of SCA described in the literature.

Methods

the review was conducted using the search system of the PUBMED and OMIM databases.

Results

Cognitive dysfunction occurs in a considerable proportion of SCA, particularly in SCA 3, which is the most frequent form of SCA worldwide. Dementia has been described in several other types of SCA such as SCA 2, SCA 17 and DRPLA.

SUBMITTER: Teive HAG 

PROVIDER: S-EPMC5618971 | biostudies-literature | 2009 Jul-Sep

REPOSITORIES: biostudies-literature