van der Zwaan Kasper Frederik, Roos Raymund Ac, de Bot Susanne T
Department of Neurology, LUMC, Leiden, The Netherlands.
J Huntingtons Dis. 2025 May;14(2):179-190. doi: 10.1177/18796397251349114. Epub 2025 Jun 11.
BackgroundWork plays a crucial role in life, contributing to financial stability and well-being. Huntington's disease (HD), a genetic neurodegenerative disorder, can significantly affect work capacity. Anosognosia (lack of awareness of impairments) and avoidant coping are common in HD but remain unexplored in relation to work outcomes.ObjectiveThis study investigated the relationships between anosognosia, coping styles, and work capacity in individuals with pre-motor manifest and motor manifest HD.MethodsUtilizing the HD-Work dataset, we analyzed motor and cognitive functioning, coping styles, work capacity, and anosognosia in participants with pre-motor manifest and motor manifest HD (n = 117). Anosognosia was operationalized through expert rating, participant - proxy, and cognitive - performance discrepancies. Work capacity was measured using the occupation item of the Total Functional Capacity scale, and coping styles were assessed with the .ResultsAnosognosia was strongly associated with cognitive decline, while avoidant coping was less prevalent. Both anosognosia and avoidance coping were correlated with frontal behaviors but not with work capacity. A positive association between avoidant coping and anosognosia was found. The most common coping style used was passive coping. Participants did not often seek social comfort.ConclusionsThe best predictor of anosognosia was cognitive decline. The positive association between avoidant coping and anosognosia suggested a potential misattribution of avoidant coping to anosognosia. This study emphasized the importance of recognizing avoidant and passive coping strategies in early-stage HD, as well as anosognosia in relation to cognitive decline, even though these factors do not directly impact work capacity.
背景
工作在生活中起着至关重要的作用，有助于经济稳定和幸福。亨廷顿舞蹈症（HD）是一种遗传性神经退行性疾病，会显著影响工作能力。疾病感缺失（对损伤缺乏意识）和回避应对在HD中很常见，但在工作成果方面仍未得到充分研究。
目的
本研究调查了运动前期表现型和运动表现型HD患者的疾病感缺失、应对方式和工作能力之间的关系。
方法
利用HD - Work数据集，我们分析了运动前期表现型和运动表现型HD患者（n = 117）的运动和认知功能、应对方式、工作能力和疾病感缺失。疾病感缺失通过专家评分、患者 - 代理人及认知 - 表现差异来衡量。工作能力使用总功能能力量表的职业项目进行测量，应对方式通过[未提及具体量表名称]进行评估。
结果
疾病感缺失与认知衰退密切相关，而回避应对则不太普遍。疾病感缺失和回避应对均与额叶行为相关，但与工作能力无关。发现回避应对与疾病感缺失之间存在正相关。最常用的应对方式是被动应对。参与者并不经常寻求社会支持。
结论
疾病感缺失的最佳预测因素是认知衰退。回避应对与疾病感缺失之间的正相关表明，回避应对可能被错误地归因于疾病感缺失。本研究强调了在HD早期阶段识别回避和被动应对策略以及疾病感缺失与认知衰退关系的重要性，尽管这些因素并不直接影响工作能力。
