Morphological changes of cerebral gray matter in spinocerebellar ataxia type 3 using fractal dimension analysis.

Abstract

Spinocerebellar ataxia type 3 (SCA3), or Machado-Joseph disease, presents as a cerebellar cognitive affective syndrome (CCAS) and represents the predominant SCA genotype in Taiwan. Beyond cerebellar involvement, SCA3 patients exhibit cerebral atrophy. While prior neurodegenerative disease studies relied on voxel-based morphometry (VBM) for brain atrophy assessment, its qualitative nature limits individual and region-specific evaluations. To address this, we employed fractal dimension (FD) analysis to quantify cortical complexity changes in SCA3 patients. We examined 50 SCA3 patients and 50 age- and sex-matched healthy controls (HC), dividing MRI cerebral gray matter (GM) into 68 auto-anatomical subregions. Using three-dimensional FD analysis, we identified GM atrophy manifestations in SCA3 patients. Results revealed lateral atrophy symptoms in the left frontal, parietal, and occipital lobes, and fewer symptoms in the right hemisphere's parietal and occipital lobes. Focal areas of atrophy included regions previously identified in SCA3 studies, alongside additional regions with decreased FD values. Bilateral postcentral gyrus and inferior parietal gyrus exhibited pronounced atrophy, correlating with Scale for the Assessment and Rating of Ataxia (SARA) scores and disease duration. Notably, the most notable focal areas were the bilateral postcentral gyrus and the left superior temporal gyrus, serving as imaging biomarkers for SCA3. Our study enhances understanding of regional brain atrophy in SCA3, corroborating known clinical features while offering new insights into disease progression.