早期亨廷顿舞蹈症中更广泛脑病理改变的证据：基于MRI的形态测量分析
Evidence for more widespread cerebral pathology in early HD: an MRI-based morphometric analysis.
作者信息
Rosas H D, Koroshetz W J, Chen Y I, Skeuse C, Vangel M, Cudkowicz M E, Caplan K, Marek K, Seidman L J, Makris N, Jenkins B G, Goldstein J M
机构信息
Department of Neurology, Massachusetts General Hospital and Harvard Medical School, Boston, USA.
出版信息
Neurology. 2003 May 27;60(10):1615-20. doi: 10.1212/01.wnl.0000065888.88988.6e.
BACKGROUND
Most clinical symptoms of Huntington disease (HD) have been attributed to striatal degeneration, but extrastriatal degeneration may play an important role in the clinical symptoms because postmortem studies demonstrate that almost all brain structures atrophy.
OBJECTIVE
To fully characterize the morphometric changes that occur in vivo in HD.
METHODS
High-resolution 1.5 mm T1-weighted coronal scans were acquired from 18 individuals in early to mid-stages of HD and 18 healthy age-matched controls. Cortical and subcortical gray and white matter were segmented using a semiautomated intensity contour-mapping algorithm. General linear models for correlated data of the volumes of brain regions were used to compare groups, controlling for age, education, handedness, sex, and total brain volumes.
RESULTS
Subjects with HD had significant volume reductions in almost all brain structures, including total cerebrum, total white matter, cerebral cortex, caudate, putamen, globus pallidus, amygdala, hippocampus, brainstem, and cerebellum.
CONCLUSIONS
Widespread degeneration occurs in early to mid-stages of HD, may explain some of the clinical heterogeneity, and may impact future clinical trials.
背景
亨廷顿舞蹈症（HD）的大多数临床症状归因于纹状体变性，但纹状体外变性可能在临床症状中起重要作用，因为尸检研究表明几乎所有脑结构均萎缩。
目的
全面描述HD患者体内发生的形态学变化。
方法
对18例处于HD早期至中期的个体和18例年龄匹配的健康对照者进行1.5毫米高分辨率T1加权冠状扫描。使用半自动强度轮廓映射算法对皮质和皮质下灰质及白质进行分割。采用脑区体积相关数据的一般线性模型对两组进行比较，并对年龄、教育程度、利手、性别和全脑体积进行控制。
结果
HD患者几乎所有脑结构的体积均显著减小，包括全脑、全白质、大脑皮质、尾状核、壳核、苍白球、杏仁核、海马体、脑干和小脑。
结论
HD早期至中期会出现广泛变性，这可能解释了部分临床异质性，并可能影响未来的临床试验。
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