Brain structural damage in spinocerebellar ataxia type 1 - A VBM study
Brain structural damage in spinocerebellar ataxia type 1 - A VBM study
复制标题
DOI：
10.1007/s00415-008-0860-4
复制
发表时间：
2008-08-01
影响因子：
6
通讯作者：
Mascalchi, Mario
中科院分区：
文献类型：
--
作者：
Ginestroni, Andrea;Della Nave, Riccardo;Mascalchi, Mario
背景与目的脊髓小脑性共济失调1型（SCA 1）的脑神经病理学描述仅限于少数病例。基于体素的形态测量学（VBM）能够对灰质（GM）和白色物质（WM）体积的区域差异进行无偏的体内全脑定量分析。我们用VBM评估了经遗传学证实的SCA1患者的结构损伤。方法对15例SCA I患者和15例年龄匹配的健康对照者进行MRI检查，采用高分辨率T1加权像。结果与疾病的持续时间和临床缺陷的严重程度进行了评估，用国际小脑性共济失调评定量表（ICARS）和遗传性共济失调临床评定量表（IACRS）。结果与对照组相比，SCA1患者小脑蚓部和小脑前叶旁正中区的GM体积对称性减少（多重比较校正后p < 0.05）。WM在齿状突周围区和小脑中脚减少，但在脑桥中没有。大脑半球未见GM或WM体积丢失。小脑和脑干GM和WM体积损失与病程和ICARS和IACRS评分相关。结论VBM证实SCA1的萎缩主要累及脑干和小脑。与临床特征的相关性表明VBM可能有助于监测疾病进展。
Background and objective Neuropathological description of the brain in spinocerebellar ataxia type 1 (SCA 1) 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 SCA I 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.