Neocortical Atrophy in Machado-Joseph Disease: A Longitudinal Neuroimaging Study
Neocortical Atrophy in Machado-Joseph Disease: A Longitudinal Neuroimaging Study
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DOI：
10.1111/j.1552-6569.2011.00614.x
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发表时间：
2012-07-01
影响因子：
2.4
通讯作者：
Cendes, Fernando
中科院分区：
文献类型：
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作者：
D'Abreu, Anelyssa;Franca, Marcondes C., Jr.;Cendes, Fernando
背景/目的以往对Machado-Joseph病（MJD/SCA3）的影像学研究主要集中在小脑和脑干。我们的目标是对整个大脑进行纵向评估。方法我们纳入45例患者和51例对照组，他们接受了两次脑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（VBM）和MarsBar分析工具箱从感兴趣的区域提取灰质密度（GMD）值。我们采用线性回归模型和一般线性模型来分析GMD与临床指标的相关性，并采用配对t检验进行纵向评价。结果我们观察到额叶、顶叶、颞叶、枕叶、皮质下灰质、小脑、脑干的GMD降低（P < 0.01）。白质萎缩仅限于小脑。年龄、CAG和病程可预测不同地区的GMD，但年龄和CAG是最重要的预测因子。纵向分析未能证明变化。小脑以外区域的变化似乎对最终的国际合作共济失调评定量表得分有显著影响。结论我们证实MJD/SCA3受累于皮质。预测GMD最重要的因素是年龄和CAG。缺乏萎缩的进展可能表明地板效应和/或随访时间短。
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. METHODS 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. RESULTS 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. CONCLUSION 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.