Brain structural damage in spinocerebellar ataxia type 1: A VBM study

• Published in: Journal of neurology 2008-08, Vol.255 (8), p.1153-1158
• Main Authors: Ginestroni, A., Della Nave, R., Tessa, C., Giannelli, M., De Grandis, D., Plasmati, R., Salvi, F., Piacentini, S., Mascalchi, M.
• Format: Article
• Language: English
• Subjects: Adult; Ataxia; Atrophy; Biological and medical sciences; Brain; Brain - pathology; Brain Injuries - etiology; Brain Injuries - pathology; Case-Control Studies; Data processing; Degenerative and inherited degenerative diseases of the nervous system. Leukodystrophies. Prion diseases; Female; Humans; Image Processing, Computer-Assisted; Magnetic Resonance Imaging - methods; Male; Medical sciences; Medicine; Medicine & Public Health; Middle Aged; Neurology; Neuropathology; Neuroradiology; Neurosciences; Original Communication; Retrospective Studies; Severity of Illness Index; Spinocerebellar Ataxias - complications; Spinocerebellar Ataxias - pathology; Statistical analysis
• ISSN: 0340-5354; 1432-1459
• DOI: 10.1007/s00415-008-0860-4

Background and objective Neuropathological description of the brain in spinocerebellar ataxia type 1(SCA1) is limited to a few cases. Voxel-based morphometry (VBM) enables an unbiased in vivo whole-brain quantitative analysis of regional differences in gray matter (GM) and white matter (WM) volume. We assessed with VBM the structural damage in patients with genetically confirmed SCA1. Method Fifteen SCA1 patients and 15 age-matched healthy controls underwent MR examination with acquisition of high-resolution T1-weighted images. The results were correlated with the disease duration and severity of the clinical deficit assessed with the International Cerebellar Ataxia Rating Scale (ICARS) and Inherited Ataxia Clinical Rating Scale (IACRS). Results As compared to controls, patients with SCA1 showed a significant (p < 0.05 corrected for multiple comparison) symmetric loss of volume of the GM in the rostral cerebellar vermis and paramedian portions of the anterior cerebellar lobes. WM was decreased in the peridentate region and middle cerebellar peduncles but not in the pons. No GM or WM volume loss was found in the cerebral hemispheres. The cerebellar and brainstem GM and WM volume loss correlated with disease duration and the ICARS and IACRS scores. Conclusions VBM confirms that atrophy predominantly involves the brainstem and cerebellum in SCA1. The correlation with the clinical features indicates that VBM might be useful to monitor disease progression.