Atkins Kelly J, Andrews Sophie C, Chong Trevor T-J, Stout Julie C
School of Psychological Sciences, Turner Institute for Brain and Mental Health Monash University Melbourne Victoria Australia.
Neuroscience Research Australia Sydney New South Wales Australia.
Mov Disord Clin Pract. 2021 Feb 12;8(3):361-370. doi: 10.1002/mdc3.13147. eCollection 2021 Apr.
Apathy is a disorder of motivation common to Huntington's disease (HD). Recent conceptual frameworks suggest that apathy is not unitary but consists of discrete subtypes ("dimensions"). Which of the proposed dimensions are preferentially affected in HD, and how these dimensions evolve with disease progression is unknown.
The Dimensional Apathy Scale (DAS) separates apathy into Executive, Initiation and Emotional subscales. Using the DAS, we aimed to: 1) Determine the apathy subtypes prevalent in HD; 2) Compare the DAS against a unitary measure of apathy (Apathy Evaluation Scale, AES); 3) Assess the reliability of self- and observer-ratings; and 4) Determine the relationship between the DAS, and disease burden, total functional capacity (TFC) and the AES.
Fifty pre-manifest, 51 manifest-HD, 87 controls, and 50 HD-observers completed the DAS, AES, and TFC.
Manifest-HD participants had the highest levels of apathy across all dimensions (30.4% on Executive subscale, 34.8% on Initiation subscale, and 15.2% on Emotional subscale), relative to pre-manifest and control participants. Self- and observer-ratings on the DAS did not differ. Hierarchical regressions across the entire gene-expanded sample showed that scores on the Initiation subscale correlated with AES scores; higher Executive subscale scores were related to higher disease burden; and Emotional subscale scores with lower total functional capacity.
In this first study of the DAS in HD, manifest-HD participants were more apathetic than pre-manifest and control participants across all apathy subtypes. The DAS may be a useful tool for measuring different aspects of apathy in people with HD.
冷漠是亨廷顿舞蹈症（HD）常见的动机障碍。最近的概念框架表明，冷漠并非单一的，而是由离散的亚型（“维度”）组成。在HD中，哪些提议的维度受到优先影响，以及这些维度如何随疾病进展而演变尚不清楚。
维度冷漠量表（DAS）将冷漠分为执行、启动和情感子量表。使用DAS，我们旨在：1）确定HD中普遍存在的冷漠亚型；2）将DAS与单一的冷漠测量方法（冷漠评估量表，AES）进行比较；3）评估自我评分和观察者评分的可靠性；4）确定DAS与疾病负担、总功能能力（TFC）和AES之间的关系。
50名症状前患者、51名症状期HD患者、87名对照者以及50名HD观察者完成了DAS、AES和TFC评估。
相对于症状前患者和对照者，症状期HD参与者在所有维度上的冷漠程度最高（执行子量表上为30.4%，启动子量表上为34.8%，情感子量表上为15.2%）。DAS的自我评分和观察者评分没有差异。对整个基因扩展样本进行的分层回归显示，启动子量表得分与AES得分相关；执行子量表得分越高与疾病负担越高相关；情感子量表得分与总功能能力越低相关。
在这项关于HD中DAS的首次研究中，症状期HD参与者在所有冷漠亚型上比症状前患者和对照者更冷漠。DAS可能是测量HD患者冷漠不同方面的有用工具。
