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亨廷顿病患者中帕金森病认知评定量表用于筛查整体认知状态的效用。

Utility of the Parkinson's disease-Cognitive Rating Scale for the screening of global cognitive status in Huntington's disease.

机构信息

Movement Disorders Unit, Neurology Department, Hospital de la Santa Creu i Sant Pau, Mas Casanovas 90, 08041, Barcelona, Spain.

Biomedical Research Institute (IIB-Sant Pau), Barcelona, Spain.

出版信息

J Neurol. 2020 May;267(5):1527-1535. doi: 10.1007/s00415-020-09730-6. Epub 2020 Feb 7.

Abstract

BACKGROUND

Cognitive impairment is an essential feature of Huntington's disease (HD) and dementia is a predictable outcome in all patients. However, validated instruments to assess global cognitive performance in the field of HD are lacking.

OBJECTIVES

We aimed to explore the utility of the Parkinson's disease-Cognitive Rating Scale (PD-CRS) for the screening of global cognition in HD.

METHODS

A multicenter cohort of 132 HD patients at different disease stages and 33 matched healthy controls were classified as having preserved cognition, mild cognitive impairment (HD-MCI) or dementia (HD-Dem) according to the Clinical Dementia Rating and Functional Independence Score. The PD-CRS and the Mini-Mental State Examination were administered. Receiver operating characteristic curve analysis was used to determine optimal cutoffs to differentiate patients according to their cognitive status.

RESULTS

A PD-CRS cutoff score ≤ 81/82 was optimal to detect HD-MCI (sensitivity = 93%; specificity = 80%; area under the curve (AUC) = 0.940), and ≤ 63/64 was optimal to detect HD-Dem (sensitivity = 90%; specificity = 87%; AUC = 0.933). MMSE scores failed to show robust psychometric properties in this context.

DISCUSSION

The PD-CRS is a valid and reliable instrument to assess global cognition in HD in routine clinical care and clinical trials.

摘要

背景

认知障碍是亨廷顿病(HD)的一个重要特征,所有患者都可能出现痴呆。然而,目前缺乏用于评估 HD 领域整体认知表现的经过验证的工具。

目的

我们旨在探讨帕金森病认知评定量表(PD-CRS)在 HD 患者中进行整体认知筛查的效用。

方法

一项多中心队列研究纳入了 132 名处于不同疾病阶段的 HD 患者和 33 名匹配的健康对照者,根据临床痴呆评定量表和功能独立性评分将其分为认知正常、轻度认知障碍(HD-MCI)或痴呆(HD-Dem)。对患者进行 PD-CRS 和简易精神状态检查(MMSE)。采用受试者工作特征曲线分析确定最佳截断值,以根据认知状态对患者进行区分。

结果

PD-CRS 截断值≤81/82 时,对 HD-MCI 的检测具有最佳的敏感性(93%)和特异性(80%)(曲线下面积(AUC)为 0.940),而截断值≤63/64 时,对 HD-Dem 的检测具有最佳的敏感性(90%)和特异性(87%)(AUC 为 0.933)。在这种情况下,MMSE 评分未能表现出稳健的心理测量学特性。

讨论

PD-CRS 是一种在常规临床护理和临床试验中评估 HD 患者整体认知的有效且可靠的工具。

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