Department of Neurology, Danish Dementia Research Centre, Copenhagen University Hospital - Rigshospitalet, Copenhagen, Denmark.
Department of Psychology, University of Copenhagen, Copenhagen, Denmark.
Appl Neuropsychol Adult. 2024 Sep-Oct;31(5):777-786. doi: 10.1080/23279095.2022.2073824. Epub 2022 May 13.
This study sought to investigate if there was a significant difference between the Huntington's Disease gene expansion carriers who were impaired on the cognitive domains, and . Also, it was investigated which of the cognitive domains could predict the decrease in total functional capacity over a 6-year follow-up period. Premanifest and motor-manifest Huntington's Disease gene expansion carriers ( = 98), were examined with a neurological and neuropsychological examination at Time 1 (year 2012-2013). Regression-based normative data was used to classify impairments on the two cognitive domains. Follow-up participants ( = 80) had their functional capacity reexamined at Time 2 (year 2018-2020), to examine which cognitive domain could predict the decrease in functional capacity over the 6-year follow-up. More than 50% of the participants were impaired on the domain of . These participants were significantly different from the participants who were impaired on . The motor function and impairments on significantly predicted the decline in functional capacity. The Emotion Hexagon test was the only significant social cognitive task, that predicted the decline in functional capacity. Social cognition includes unique and separate functions in Huntington's Disease, unaffected by executive functions. This study emphasizes the importance of regular assessment of social cognition in Huntington's Disease and the clinical relevance of impaired social cognitive function.
本研究旨在探讨亨廷顿病基因扩展携带者在认知领域受损与 之间是否存在显著差异。此外，还研究了哪些认知领域可以预测在 6 年随访期间总功能能力的下降。在时间 1（2012-2013 年），对前显型和运动显型亨廷顿病基因扩展携带者（ = 98）进行了神经学和神经心理学检查。回归基准规范数据用于对两个认知领域的损伤进行分类。随访参与者（ = 80）在时间 2（2018-2020 年）重新检查了他们的功能能力，以检查哪个认知领域可以预测在 6 年随访期间功能能力的下降。超过 50%的参与者在 领域受损。这些参与者与在 上受损的参与者明显不同。运动功能和 在 上的损伤显著预测了功能能力的下降。情绪六边形测试是唯一显著的社会认知任务，可预测功能能力的下降。社会认知在亨廷顿病中包含独特且独立的功能，不受执行功能的影响。本研究强调了在亨廷顿病中定期评估社会认知的重要性以及受损的社会认知功能的临床相关性。
