School of Psychological Sciences and Turner Institute for Brain and Mental Health, Monash University, Melbourne, Victoria, Australia.
Neuroscience Research Australia, Sydney, NSW, Australia.
Psychol Med. 2021 Jun;51(8):1338-1344. doi: 10.1017/S0033291720000094. Epub 2020 Feb 17.
Cognitive impairment is a core feature of Huntington's disease (HD), however, the onset and rate of cognitive decline is highly variable. Apathy is the most common neuropsychiatric symptom of HD, and is associated with cognitive impairment. The aim of this study was to investigate apathy as a predictor of subsequent cognitive decline over 2 years in premanifest and early HD, using a prospective, longitudinal design.
A total of 118 premanifest HD gene carriers, 111 early HD and 118 healthy control participants from the multi-centre TRACK-HD study were included. Apathy symptoms were assessed at baseline using the apathy severity rating from the Short Problem Behaviours Assessment. A composite of 12 outcome measures from nine cognitive tasks was used to assess cognitive function at baseline and after 24 months.
In the premanifest group, after controlling for age, depression and motor signs, more apathy symptoms predicted faster cognitive decline over 2 years. In contrast, in the early HD group, more motor signs, but not apathy, predicted faster subsequent cognitive decline. In the control group, only older age predicted cognitive decline.
Our findings indicate that in premanifest HD, apathy is a harbinger for cognitive decline. In contrast, after motor onset, in early diagnosed HD, motor symptom severity more strongly predicts the rate of cognitive decline.
认知障碍是亨廷顿病（HD）的核心特征，然而，认知障碍的发病和进展速度具有高度可变性。淡漠是 HD 最常见的神经精神症状，与认知障碍有关。本研究旨在采用前瞻性、纵向设计，探讨淡漠是否可预测无明显症状和早期 HD 患者 2 年内的认知衰退。
共有 118 名来自多中心 TRACK-HD 研究的无明显症状的 HD 基因携带者、111 名早期 HD 患者和 118 名健康对照者纳入研究。在基线时使用简短问题行为评估中的淡漠严重程度评分评估淡漠症状。使用 9 项认知任务的 12 项结果测量的组合在基线和 24 个月时评估认知功能。
在无明显症状组中，在校正年龄、抑郁和运动症状后，更多的淡漠症状预示着 2 年内认知衰退更快。相比之下，在早期 HD 组中，更多的运动症状，而不是淡漠，预示着随后的认知衰退更快。在对照组中，只有年龄较大与认知衰退相关。
我们的研究结果表明，在无明显症状的 HD 中，淡漠是认知衰退的先兆。相比之下，在运动症状出现后，在早期诊断的 HD 中，运动症状的严重程度更能预测认知衰退的速度。
