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评估一种用于评估亨廷顿病患者认知功能意识的绩效评分方法。

Evaluation of a performance-rating method to assess awareness of cognitive functioning in Huntington's disease.

机构信息

New Mexico VA Health Care System, Albuquerque, NM, USA.

Department of Psychiatry & Behavioral Sciences, University of New Mexico, Albuquerque, NM, USA.

出版信息

Clin Neuropsychol. 2020 Apr;34(3):477-497. doi: 10.1080/13854046.2019.1640286. Epub 2019 Jul 19.

DOI:10.1080/13854046.2019.1640286
PMID:31322031
Abstract

: Anosognosia, or lack of awareness of symptoms, is commonly observed in Huntington's disease (HD) using patient/informant-report discrepancy methods. The purpose of this study was to examine the utility of a performance-rating method for assessing awareness of cognitive performance in HD. Persons with manifest HD ( = 54) rated their performance on the daily living tests from the Neuropsychological Assessment Battery (NAB) using a bell curve. We also examined patient/informant-report discrepancies of executive functioning (FrSBe) in a subset ( = 47) of patients and their informants. Patients were able to reliably use our method of categorizing their performance as evidenced by significant correlations between verbal descriptions of their performance and the percentile ratings they indicated on the bell curve. Patients across the range of observed Unified HD Rating Scale motor scale scores rated their performance on the NAB tasks significantly higher than actual performance for both pretest prediction and posttest evaluation ratings. Only patients with more severe motor impairment underestimated their problems relative to informant report on the FrSBe. This is the first study to use a performance-rating method for examining awareness in HD. Patients could reliably use this performance-based bell curve method to provide accurate estimations of their performance and as a group they tended to overestimate performance as has been demonstrated in previous studies. Unlike previous studies using self-report methodology, we also demonstrated decreased awareness in the persons with HD with less severe motor impairment using the performance-rating method, suggesting the two methods may measure different constructs of awareness.

摘要

失认症,或对症状缺乏意识,在使用患者/知情者报告差异方法时,在亨廷顿病(HD)中很常见。本研究的目的是检验一种用于评估 HD 患者认知表现意识的绩效评定方法的效用。表现明显的 HD 患者( = 54)使用正态曲线对神经心理评估电池(NAB)中的日常生活测试进行自我评分。我们还在患者及其知情者的亚组( = 47)中检查了执行功能(FrSBe)的患者/知情者报告差异。患者能够可靠地使用我们的分类方法来评估自己的表现,这表现在他们对自己表现的口头描述与他们在正态曲线上指示的百分位评分之间存在显著相关性。在观察到的统一 HD 评分量表运动量表的各个评分范围内的患者对 NAB 任务的自我评分明显高于实际表现,无论是在预测试预测还是后测试评估评分中。只有运动障碍更严重的患者相对于 FrSBe 中的知情者报告低估了自己的问题。这是第一项使用绩效评定方法检查 HD 患者意识的研究。患者可以可靠地使用这种基于表现的正态曲线方法来提供对自己表现的准确估计,并且作为一个群体,他们往往会高估自己的表现,这在之前的研究中已经得到了证明。与使用自我报告方法学的先前研究不同,我们还使用绩效评定方法在运动障碍程度较轻的 HD 患者中证明了意识下降,这表明这两种方法可能测量了不同的意识结构。

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Functional and cognitive capacity differ in dystonic motor subtypes when compared to choreatic and hypokinetic-rigid motor subtypes in Huntington's disease.与亨廷顿病的舞蹈样和运动徐缓-强直型运动亚型相比,肌张力障碍型运动亚型的功能和认知能力存在差异。
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