title: Dissociation of grey and white matter reduction in spinocerebellar ataxia type 3 and 6: a voxel-based morphometry study.
abstract: The aim of this study was to examine the different patterns of cerebellar and/or brainstem atrophy in spinocerebellar ataxia (SCA) type 3 and 6. Eighteen patients (SCA3 n=9, SCA6 n=9) and 15 healthy volunteers were studied. Voxel-based morphometry (VBM) was applied to segmented grey matter (GM) and white matter (WM) of high-resolution T1-weighted brain volumes of each group. We found reduction of grey matter in the pons as well as in the vermis in SCA3 as compared to control subjects. In SCA6 significant grey matter loss was found in hemispheric lobules bilaterally as well as in the vermis. White matter analysis revealed significant changes in SCA3, especially in the pons, in the white matter surrounding the dentate nucleus (DN) and in the cerebellar peduncles, whereas no significant white matter reduction was found in SCA6 patients. Our results demonstrate different patterns of grey and white matter affection detected by magnetic resonance imaging (MRI) in SCA3 and SCA6 patients, confirming the pathological concept of cortical cerebellar atrophy in SCA6. In contrast, SCA3 represents a form of ponto-cerebellar atrophy with predominant affection of pontine nuclei and fibre tracts.
authors.affiliation: Department of Neurology, St. Josef Hospital, Ruhr University Bochum, Bochum, Germany. Carsten.Lukas@ruhr-uni-bochum.de
authors.forename: Carsten
authors.initials: C
authors.lastname: Lukas
authors.forename: Ludger
authors.initials: L
authors.lastname: Schöls
authors.forename: Barbara
authors.initials: B
authors.lastname: Bellenberg
authors.forename: Udo
authors.initials: U
authors.lastname: Rüb
authors.forename: Horst
authors.initials: H
authors.lastname: Przuntek
authors.forename: Gebhard
authors.initials: G
authors.lastname: Schmid
authors.forename: Odo
authors.initials: O
authors.lastname: Köster
authors.forename: Boris
authors.initials: B
authors.lastname: Suchan
mesh_terms: D000368:Aged; D000704:Analysis of Variance; D001931:Brain Mapping; D001933:Brain Stem / Q000473:pathology*; D002531:Cerebellum / Q000473:pathology*; D005260:Female; D006801:Humans; D021621:Imaging, Three-Dimensional / Q000379:methods; D017827:Machado-Joseph Disease / Q000473:pathology*; D008297:Male; D008875:Middle Aged; D020754:Spinocerebellar Ataxias / Q000473:pathology*