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全脑萎缩作为临床前期和早期亨廷顿舞蹈症病情进展的一项指标。

Whole-brain atrophy as a measure of progression in premanifest and early Huntington's disease.

作者信息

Henley Susie M D, Wild Edward J, Hobbs Nicola Z, Frost Chris, MacManus David G, Barker Roger A, Fox Nick C, Tabrizi Sarah J

机构信息

Dementia Research Centre, Institute of Neurology, National Hospital for Neurology and Neurosurgery, University College London, Queen Square, London, United Kingdom.

出版信息

Mov Disord. 2009 Apr 30;24(6):932-6. doi: 10.1002/mds.22485.

Abstract

Therapeutic trials in Huntington's disease (HD) are challenging as clinical progression is slow and variable and reliable biomarkers are lacking. We used magnetic resonance imaging and the brain boundary shift integral to quantify whole-brain atrophy rates over 1 year in early and premanifest HD subjects, and controls. Early HD subjects had statistically significantly (P = 0.007) increased (threefold higher) rates of whole-brain atrophy compared with controls. Higher atrophy rates were associated with longer CAG repeat length. MRI-based measures of whole-brain atrophy may have potential as a measure of progression in HD.

摘要

亨廷顿舞蹈症(HD)的治疗试验颇具挑战性,因为其临床进展缓慢且多变,还缺乏可靠的生物标志物。我们运用磁共振成像和脑边界位移积分来量化早期和症状前HD患者以及对照组在1年时间内的全脑萎缩率。与对照组相比,早期HD患者的全脑萎缩率在统计学上显著升高(高出三倍)(P = 0.007)。更高的萎缩率与更长的CAG重复序列长度相关。基于磁共振成像的全脑萎缩测量指标可能有潜力作为HD病情进展的一项衡量标准。

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