Language

English

Publication Date

3-1-2024

Journal

Current Neurology and Neuroscience Reports

DOI

10.1007/s11910-024-01331-4

PMID

38270820

PMCID

PMC10922758

PubMedCentral® Posted Date

3-8-2024

PubMedCentral® Full Text Version

Author MSS

Abstract

Purpose of review: Spinocerebellar ataxias (SCAs) are autosomal dominant degenerative syndromes that present with ataxia and brain stem abnormalities. This review describes the cognitive and behavioral symptoms of SCAs in the context of recent knowledge of the role of the cerebellum in higher intellectual function.

Recent findings: Recent studies suggest that patients with spinocerebellar ataxia can display cognitive deficits even early in the disease. These have been given the term cerebellar cognitive affective syndrome (CCAS). CCAS can be tracked using newly developed rating scales. In addition, patients with spinocerebellar ataxia also display impulsive and compulsive behavior, depression, anxiety, fatigue, and sleep disturbances. This review stresses the importance of recognizing non-motor symptoms in SCAs. There is a pressing need for novel therapeutic interventions to address these symptoms given their deleterious impact on patients' quality of life.

Keywords

Humans, Quality of Life, Spinocerebellar Ataxias, Cerebellum, Emotions, Cognition, cerebellar ataxia, non-motor symptoms, depression, impulsivity

Published Open-Access

yes

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