Mühlau M, Weindl A, Wohlschläger A M, Gaser C, Städtler M, Valet M, Zimmer C, Kassubek J, Peinemann A
Department of Neurology, Technische Universität München, Munich, Germany.
J Neural Transm (Vienna). 2007 Mar;114(3):367-72. doi: 10.1007/s00702-006-0571-x. Epub 2006 Oct 6.
In Huntington disease (HD), both the genetic defect and mutant gene product huntington are known but the exact mechanisms that lead to neuronal loss are poorly understood. Until now, the distribution of tissue loss throughout the brain has been investigated intensively. Here we searched for areas that, antipodal to the striatum, display grey-matter (GM) preservation. We performed high resolution T1-weighted magnetic resonance imaging and voxel-based morphometry in 46 patients in early HD and 46 healthy controls. We applied an analysis of covariance (ANCOVA) model with the total GM volume of each participant as covariate. In accordance with earlier reports, group comparisons revealed GM decrease in the striatum, insula, and thalamus as well as in dorsolateral frontal and occipital areas. In contrast, the limbic prefrontal cortex displayed GM preservation. Our findings support hypotheses that postulate differential involvement of frontosubcortical circuits in the pathophysiology of HD.
在亨廷顿舞蹈症（HD）中，基因缺陷和突变基因产物亨廷顿蛋白均已明确，但导致神经元丧失的确切机制仍知之甚少。到目前为止，人们已对整个大脑中组织损失的分布进行了深入研究。在此，我们寻找与纹状体相反、呈现灰质（GM）保留的区域。我们对46例早期HD患者和46名健康对照者进行了高分辨率T1加权磁共振成像和基于体素的形态测量。我们应用协方差分析（ANCOVA）模型，将每个参与者的总GM体积作为协变量。与早期报告一致，组间比较显示纹状体、岛叶、丘脑以及背外侧额叶和枕叶区域的GM减少。相比之下，边缘前额叶皮质呈现GM保留。我们的研究结果支持如下假说，即额颞叶皮质下环路在HD病理生理学中存在不同程度的受累。
