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在轻度亨廷顿舞蹈症中,磁共振成像显示壳核体积缩小超过尾状核变化。

Putamen volume reduction on magnetic resonance imaging exceeds caudate changes in mild Huntington's disease.

作者信息

Harris G J, Pearlson G D, Peyser C E, Aylward E H, Roberts J, Barta P E, Chase G A, Folstein S E

机构信息

Department of Psychiatry and Behavioral Sciences, Johns Hopkins Medical Institutions, Baltimore, MD 21205.

出版信息

Ann Neurol. 1992 Jan;31(1):69-75. doi: 10.1002/ana.410310113.

Abstract

The characteristic pathological features of Huntington's disease (HD) are neostriatal atrophy and neuronal loss. Although neuroradiological studies often show caudate atrophy in patients with moderate HD, frequently no caudate atrophy is found early in the illness. There have been no quantitative reports to date on in vivo putamen volume measures in mild HD, although the structure is known to be neuropathologically involved in the illness. We measured volumes of caudate nucleus and putamen and bicaudate ratios (BCR) from magnetic resonance images, blind to diagnosis, in 15 patients with mild HD and 19 age- and sex-matched control subjects using a computerized image analysis system. The region showing greatest atrophy was the putamen, which was reduced 50.1% in mean volume in HD patients compared with control subjects (p less than 0.000001). In contrast, caudate volume was reduced 27.7% (p = 0.004). BCR was increased 28.5% in HD patients (p = 0.0002). Discriminant function analysis was 94% effective in identifying the diagnostic group based on putamen volume alone, whereas caudate measures had considerable overlap. Correction of putamen volume for head size led to 100% separation by group. Putamen measures and BCR correlated with neurological examination scores but caudate volume did not. Volumetric measurement of putamen is a more sensitive indicator of brain abnormalities in mild HD than measures of caudate atrophy.

摘要

亨廷顿舞蹈症(HD)的典型病理特征是新纹状体萎缩和神经元丧失。尽管神经放射学研究常常显示中度HD患者存在尾状核萎缩,但在疾病早期往往未发现尾状核萎缩。虽然已知壳核在该疾病的神经病理学上会受累,但迄今为止尚无关于轻度HD患者壳核体积活体测量的定量报告。我们使用计算机图像分析系统,对15例轻度HD患者和19名年龄及性别匹配的对照者的磁共振图像进行了尾状核和壳核体积以及双侧尾状核比率(BCR)的测量,测量时对诊断结果不知情。萎缩最明显的区域是壳核,与对照者相比,HD患者的壳核平均体积减少了50.1%(p<0.000001)。相比之下,尾状核体积减少了27.7%(p = 0.004)。HD患者的BCR增加了28.5%(p = 0.0002)。仅基于壳核体积的判别函数分析在识别诊断组方面的有效性为94%,而尾状核测量结果有相当大的重叠。将壳核体积校正为头围大小后,两组实现了100%的区分。壳核测量值和BCR与神经学检查评分相关,但尾状核体积与神经学检查评分无关。与尾状核萎缩测量相比,壳核体积测量是轻度HD患者脑异常更敏感的指标。

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