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.

译文

这项研究的目的是检查3型和6型脊髓小脑共济失调 (SCA) 的小脑和/或脑干萎缩的不同模式。研究了18名患者 (SCA3 n = 9,SCA6 n = 9) 和15名健康志愿者。将基于体素的形态计量学 (VBM) 应用于每组高分辨率T1-weighted脑体积的分段灰质 (GM) 和白质 (WM)。与对照组相比,我们发现SCA3中的脑桥和ver中的灰质减少。在SCA6中,双侧半球小叶以及ver骨中都发现了明显的灰质损失。白质分析显示SCA3发生了显着变化,尤其是在脑桥,齿状核 (DN) 周围的白质和小脑梗中,而在SCA6患者中未发现明显的白质减少。我们的结果表明,通过磁共振成像 (MRI) 在SCA3和SCA6患者中检测到的灰质和白质的不同模式,证实了SCA6中皮质小脑萎缩的病理概念。相反,SCA3代表一种桥小脑萎缩的形式,主要影响桥脑核和纤维束。

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