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.
J Neurol. 2020 May;267(5):1527-1535. doi: 10.1007/s00415-020-09730-6. Epub 2020 Feb 7.
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.
We aimed to explore the utility of the Parkinson's disease-Cognitive Rating Scale (PD-CRS) for the screening of global cognition in HD.
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.
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.
The PD-CRS is a valid and reliable instrument to assess global cognition in HD in routine clinical care and clinical trials.
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。
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。
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。
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。
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。