Regional specificity of brain atrophy in Huntington's disease
Regional specificity of brain atrophy in Huntington's disease
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DOI：
10.1006/exnr.1998.6919
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发表时间：
1998-12-01
影响因子：
5.3
通讯作者：
McCusker, E
中科院分区：
文献类型：
--
作者：
Halliday, GM;McRitchie, DA;McCusker, E
本研究分析了亨廷顿病患者皮质和皮质下脑体积之间的关系。在征得亲属同意的情况下，尸检时收集了7名临床诊断为亨廷顿病且有阳性家族史的患者和12名对照者的大脑。对所有研究受试者进行了详细的临床评估，确认了亨廷顿病患者的基因型。如前所述，在连续3 mm冠状切片上进行脑体积分析。所有患有亨廷顿病的患者都表现出由于皮质和皮质下灰质体积减少而导致的显著脑萎缩。皮质的萎缩是相对均匀的，虽然内侧颞叶结构幸免。所有病例的尾状核和壳核均显著减少，这种萎缩与皮质萎缩的严重程度相关，提示相关的疾病过程。皮质而非皮质下萎缩率与CAG重复次数相关。额叶白色物质的丢失与皮质和纹状体萎缩相关。舞蹈病的发病年龄与皮质下萎缩的数量相关，而舞蹈病的持续时间与白色物质的萎缩呈负相关。这些结果表明，在亨廷顿病患者中，疾病过程更为广泛和全球性。(C)北京：科学出版社.
The present study analyzes the relationship between cortical and subcortical brain volumes in patients with Huntington's disease. The brains of seven patients with a clinical diagnosis and positive family history of Huntington's disease and 12 controls were collected at autopsy with consent from relatives. Detailed clinical assessments were available for all study subjects with genotype confirmation for patients with Huntington's disease. Volume analysis of the brain on serial 3-mm coronal slices was performed as previously described. All patients with Huntington's disease exhibited significant brain atrophy resulting from volume reductions in both cortical and subcortical grey matter. Atrophy of the cortex was relatively uniform, although the medial temporal lobe structures were spared. The caudate nucleus and putamen were strikingly reduced in all cases and this atrophy correlated with the severity of cortical atrophy, suggesting an associated disease process. The rate of cortical but not subcortical atrophy correlated with CAG repeat numbers. Loss of frontal white matter correlated with both cortical and striatal atrophy. Age of onset of chorea correlated with the amount of subcortical atrophy, while duration of chorea correlated negatively with atrophy of the white matter. These results suggest a more widespread and global disease process in patients with Huntington's disease. (C) 1998 Academic Press.