Neuroimaging Laboratory, University of Campinas-UNICAMP, Brazil.
J Neuroimaging. 2012 Jul;22(3):285-91. doi: 10.1111/j.1552-6569.2011.00614.x. Epub 2011 Jun 23.
BACKGROUND/PURPOSE: Previous imaging studies in the Machado-Joseph disease (MJD/SCA3) have mostly concentrated on the cerebellum and brainstem. Our goal was to perform a whole brain longitudinal evaluation.
We included 45 patients and 51 controls, who underwent two brain magnetic resonance imaging and magnetic resonance spectroscopy (mean interval of 12.5 ± 1.5 months). We used voxel-based morphometry (VBM) and the MarsBar analysis toolbox to extract grey matter density (GMD) values from regions of interest. We used a linear regression model and a general linear model to correlate GMD with clinical markers, and paired t-test for the longitudinal evaluation.
We observed decreased GMD (P < .01) at frontal, parietal, temporal and occipital lobes, subcortical grey matter, cerebellum, and brainstem. White matter atrophy was restricted to the cerebellum. Age, CAG, and disease duration predicted GMD in different areas, but age and CAG were the most important predictors. The longitudinal analysis failed to demonstrate changes. Changes in regions other than the cerebellum appeared to contribute significantly to the final International Cooperative Ataxia Rating Scale score.
We confirmed cortical involvement in MJD/SCA3. The most important factors in predicting GMD were age and CAG. The lack of progression of atrophy may indicate floor effect and/or short duration of follow-up.
背景/目的：既往关于 Machado-Joseph 病（MJD/SCA3）的影像学研究大多集中于小脑和脑干。我们的目标是进行全脑纵向评估。
我们纳入了 45 例患者和 51 例对照者，他们接受了两次脑部磁共振成像和磁共振波谱检查（平均间隔 12.5±1.5 个月）。我们使用基于体素的形态测量学（VBM）和 MarsBar 分析工具箱，从感兴趣区提取灰质密度（GMD）值。我们使用线性回归模型和广义线性模型将 GMD 与临床标志物相关联，并使用配对 t 检验进行纵向评估。
我们观察到额叶、顶叶、颞叶和枕叶、皮质下灰质、小脑和脑干的 GMD 降低（P＜0.01）。白质萎缩仅限于小脑。年龄、CAG 和疾病持续时间可以预测不同区域的 GMD，但年龄和 CAG 是最重要的预测因素。纵向分析未能显示出变化。除小脑以外的区域的变化似乎对最终的国际合作共济失调评分有重要贡献。
我们证实了 Machado-Joseph 病（MJD/SCA3）的皮质受累。预测 GMD 的最重要因素是年龄和 CAG。萎缩进展的缺乏可能表明存在下限效应和/或随访时间较短。
