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

• DOI: 10.1093/braincomms/fcaf118
• Publication Date: 2025-03-30T21:20:04Z
• Authors: Yonghua Huang; Zhiming Zhen; Lihua Deng; Peiling Ou; Linfeng Shi; Feng Shi; Rui Hua; Jiaojiao Wu; Wei Chen; Ru Wen; Jian Wang; Chen Liu
• Keywords: Machado–Joseph disease; Montreal Cognitive Assessment; Cerebellar ataxia

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 investigate relationship between clinical characteristics individuals with SCA3. enrolled 43 patients age- sex-matched healthy controls (HCs). cross-sectional assessed severity using Scale for Assessment Rating Ataxia (SARA) International Cooperative (ICARS). Various cognitive functions were evaluated all subjects Montreal Cognitive (MoCA), Rapid Verbal Retrieval (RAR) Digital Span Test (DST) scales. MRI was used automatically segment 15 brain regions. Compared HCs, showed a significantly higher basal ganglia, temporal lobe, right parietal lobe cerebellum. There positive correlation motor dysfunction volume left cerebellum number SARA ICARS scores. that have increased many regions, leading impairment. could be new imaging biomarker disease monitoring therapeutic target