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Cognitive dysfunction in spinocerebellar ataxias
Helio Afonso Ghizoni Teive1, Walter Oleschko Arruda1
1Movement Disorders Unit, Neurology Service, Internal Medicine Department, Hospital de Clínicas, Federal University of Paraná, Curitiba, PR, Brazil.
Dementia & Neuropsychologia
|December 8, 2017
Summary
Cognitive dysfunction is common in spinocerebellar ataxias (SCAs), especially SCA 3. The cerebellum
Area of Science:
- Neuroscience
- Genetics
- Neurology
Background:
- Spinocerebellar ataxias (SCAs) are a diverse group of neurodegenerative disorders.
- Key features include progressive cerebellar ataxia, potentially with other neurological signs.
Purpose of the Study:
- To investigate the prevalence of cognitive dysfunction across major spinocerebellar ataxia types.
- To understand the cerebellar role in cognitive function within SCAs.
Main Methods:
- Literature review using PubMed and OMIM databases.
- Analysis of studies describing cognitive dysfunction in various SCAs.
Main Results:
- Cognitive dysfunction is prevalent in SCAs, notably in SCA 3 (most common worldwide).
- Dementia is reported in SCA 2, SCA 17, and DRPLA.
- Mental retardation is a distinct feature of SCA 13.
Conclusions:
- The cerebellum's involvement in cognition is evident across SCAs.
- SCAs can present with varied cognitive impairments, including dementia and mental retardation.
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