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未出现明显亨廷顿病的执行功能。

Executive functions in premanifest Huntington's disease.

机构信息

Department of Neurology, Memory and Aging Center, University of California, San Francisco, San Francisco, California, USA.

出版信息

Mov Disord. 2014 Mar;29(3):405-9. doi: 10.1002/mds.25762. Epub 2013 Dec 27.

Abstract

BACKGROUND

We investigated the viability of psychometrically robust executive function measures as markers for premanifest Huntington's disease (HD).

METHODS

Fifteen premanifest HD subjects and 42 controls were compared on the NIH EXAMINER executive function battery. This battery yields an overall executive composite score, plus working memory, cognitive control, and fluency scores that are measured on psychometrically matched scales. The scores were correlated with two disease markers, disease burden and striatal volumes, in the premanifest HD subjects.

RESULTS

The premanifest HD subjects scored significantly lower on the working memory score. The executive composite positively correlated with striatal volumes, and the working memory score negatively correlated with disease burden. The cognitive control and fluency scores did not differ between the groups or correlate significantly with the disease markers.

CONCLUSIONS

The NIH EXAMINER executive composite and working memory scores are sensitive markers of cognitive dysfunction, striatal volume, and disease burden in premanifest HD.

摘要

背景

我们研究了心理测量学上可靠的执行功能测量指标作为前驱亨廷顿病(HD)标志物的可行性。

方法

15 名前驱 HD 受试者和 42 名对照者在 NIH EXAMINER 执行功能测试套件上进行了比较。该测试套件提供了一个总体执行功能综合评分,以及工作记忆、认知控制和流畅性评分,这些评分都是在心理测量匹配的量表上测量的。在前驱 HD 受试者中,这些评分与两种疾病标志物(疾病负担和纹状体体积)相关联。

结果

前驱 HD 受试者在工作记忆评分上显著较低。执行功能综合评分与纹状体体积呈正相关,而工作记忆评分与疾病负担呈负相关。认知控制和流畅性评分在两组之间没有差异,也与疾病标志物没有显著相关。

结论

NIH EXAMINER 执行功能综合评分和工作记忆评分是前驱 HD 认知功能障碍、纹状体体积和疾病负担的敏感标志物。

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