Beyond the cerebellum: perivascular space burden in spinocerebellar ataxia type 3 extends to multiple brain regions.

ABSTRACT: Spinocerebellar ataxia type 3 (SCA3) is an uncommon inherited (autosomal dominant) neurodegenerative disorder caused by abnormal accumulation of ataxin-3 protein. The perivascular space (PVS) burden reflects protein clearance and may worsen in SCA3 disease. This study aimed to quantify the PVS burden and investigate the relationship between the PVS burden and clinical characteristics in individuals with SCA3. This study enrolled 43 SCA3 patients and 43 age- and sex-matched healthy controls (HCs). The cross-sectional study assessed the severity of ataxia in SCA3 patients using the Scale for the Assessment and Rating of Ataxia (SARA) and the International Cooperative Ataxia Rating Scale (ICARS). Various cognitive functions were evaluated in all subjects using the Montreal Cognitive Assessmen

SUBMITTER: Huang Y 

PROVIDER: S-EPMC11969673 | biostudies-literature | 2025

REPOSITORIES: biostudies-literature