PURPOSE Individuals with Huntington disease (HD) experience progressive cognitive decline that may appear years before motor manifestations of the disease. These declines have a profound effect on health-related quality of life (HRQOL) over the disease course, and thus it is important that self-report measures of cognitive function are validated for use in longitudinal studies. METHODS 359 individuals with premanifest or manifest HD completed baseline and at least one follow-up (12- and 24-month) assessment. Neuro-QoL™ Cognitive Function was administered at each time-point. Participants completed a self-reported global rating of cognitive change, as well as performance-based cognitive changes (using the Symbol Digit Modalities Test). Standardized response means (SRMs) and general linear models evaluated whether Neuro-QoL™ Cognitive Function was responsive to change over time with respect to self-reported and performance-based anchors. Test-retest reliability and known-group validity were also examined. RESULTS Responsiveness was supported by effect sizes that were small in magnitude, but in the expected direction relative to self-reported and performance-based change. General linear models generally supported 12- and 24-month responsiveness relative to self-reported cognitive change and 12-month responsiveness relative to performance-based change. Test-retest reliability was excellent, and the measure exhibited known-group validity. CONCLUSION Longitudinal analyses generally indicate that the Neuro-QoL™ Cognitive Function measure is sensitive to change over time in individuals with HD. Neuro-QoL Cognitive Function changes reflect self-reported cognitive change at 12 and 24 months and performance-based change at 12 months. This measure may be useful in clinical trials or longitudinal observation studies.

中文翻译：

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亨廷顿病 (HD) 患者神经生活质量认知功能的心理测量特性和反应性。

目的 患有亨廷顿病 (HD) 的人会经历进行性认知能力下降，这可能比该疾病的运动表现早几年出现。这些下降对疾病过程中的健康相关生活质量 (HRQOL) 产生深远影响，因此重要的是，认知功能的自我报告测量值在纵向研究中得到验证。方法 359 名患有前表现或表现 HD 的个体完成了基线和至少一项后续（12 个月和 24 个月）评估。在每个时间点施用 Neuro-QoL™ 认知功能。参与者完成了自我报告的全球认知变化评分，以及基于表现的认知变化（使用符号数字模态测试）。标准化响应均值 (SRM) 和一般线性模型评估 Neuro-QoL™ 认知功能是否对自我报告和基于绩效的锚随时间的变化作出响应。还检查了重测信度和已知组效度。结果 响应性得到了规模较小但相对于自我报告和基于绩效的变化的预期方向的影响。一般线性模型通常支持相对于自我报告的认知变化的 12 个月和 24 个月的响应性以及相对于基于绩效的变化的 12 个月的响应性。重测信度非常好，测量显示已知组效度。结论 纵向分析通常表明 Neuro-QoL™ 认知功能测量对 HD 个体随时间的变化很敏感。Neuro-QoL 认知功能变化反映了 12 个月和 24 个月时自我报告的认知变化以及 12 个月时基于表现的变化。该措施在临床试验或纵向观察研究中可能有用。