title: Brain structural damage in spinocerebellar ataxia type 1 : a VBM study.
abstract: 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.
  
  
  METHODS
  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.
authors.affiliation: Radiodiagnostic Section, Dept. of Clinical Physiopathology, University of Florence, Viale Morgagni 85, 50134, Florence, Italy.
authors.forename: Andrea
authors.initials: A
authors.lastname: Ginestroni
authors.forename: Riccardo
authors.initials: R
authors.lastname: Della Nave
authors.forename: Carlo
authors.initials: C
authors.lastname: Tessa
authors.forename: Marco
authors.initials: M
authors.lastname: Giannelli
authors.forename: Domenico
authors.initials: D
authors.lastname: De Grandis
authors.forename: Rosaria
authors.initials: R
authors.lastname: Plasmati
authors.forename: Fabrizio
authors.initials: F
authors.lastname: Salvi
authors.forename: Silvia
authors.initials: S
authors.lastname: Piacentini
authors.forename: Mario
authors.initials: M
authors.lastname: Mascalchi
mesh_terms: D000328:Adult; D001921:Brain / Q000473:pathology*; D001930:Brain Injuries / Q000209:etiology / Q000473:pathology*; D016022:Case-Control Studies; D005260:Female; D006801:Humans; D007091:Image Processing, Computer-Assisted; D008279:Magnetic Resonance Imaging / Q000379:methods; D008297:Male; D008875:Middle Aged; D012189:Retrospective Studies; D012720:Severity of Illness Index; D020754:Spinocerebellar Ataxias / Q000150:complications / Q000473:pathology*