Department of Neurology, Danish Dementia Research Centre, Copenhagen University Hospital - Rigshospitalet, Copenhagen, Denmark.
Department of Psychology, University of Copenhagen, Copenhagen, Denmark.
J Huntingtons Dis. 2022;11(4):373-381. doi: 10.3233/JHD-220540.
Autonomy describes a psychological state of self-regulation of motivation and action, which is a central characteristic of healthy functioning. In neurodegenerative diseases measures of self-perception have been found to be affected by the disease. However, it has never been investigated whether measures of self-perception, like autonomy, is affected in Huntington's disease.
We investigated whether autonomy is affected in Huntington's disease and if the degree of autonomy is associated with motor function, neuropsychiatric symptoms, cognitive impairments, and apathy.
We included 44 premanifest and motor-manifest Huntington's disease gene expansion carriers and 19 controls. Autonomy was examined using two self-report questionnaires, the Autonomy-Connectedness Scale-30 and the Index of Autonomous Functioning. All participants were examined according to motor function, cognitive impairments, and neuropsychiatric symptoms, including apathy.
Statistically significant differences were found between motor-manifest Huntington's disease gene expansion carriers and premanifest Huntington's disease gene expansion carriers or controls on two measures of autonomy. Between 25-38% of motor-manifest Huntington's disease gene expansion carriers scored significantly below the normal level on subscales of autonomy as compared to controls. One autonomy subscale was associated with apathy (r = -0.65), but not with other symptoms of Huntington's disease.
This study provides evidence for impaired autonomy in individuals with Huntington's disease and an association between autonomy and apathy. The results underline the importance of maintaining patient autonomy and involvement in care throughout the disease.
自主性描述了一种自我调节动机和行动的心理状态，是健康功能的核心特征。在神经退行性疾病中，已经发现自我感知的测量受到疾病的影响。然而，尚未研究自主性等自我感知的测量是否在亨廷顿病中受到影响。
我们调查自主性是否在亨廷顿病中受到影响，以及自主性的程度是否与运动功能、神经精神症状、认知障碍和冷漠有关。
我们纳入了 44 名处于前驱期和运动期亨廷顿病基因扩展携带者以及 19 名对照者。使用两个自我报告问卷，即自主性-联系量表-30 和自主功能指数，来评估自主性。所有参与者都根据运动功能、认知障碍和神经精神症状（包括冷漠）进行了检查。
在自主性的两个测量指标上，运动期亨廷顿病基因扩展携带者与前驱期亨廷顿病基因扩展携带者或对照组之间存在统计学显著差异。与对照组相比，25-38%的运动期亨廷顿病基因扩展携带者在自主性的子量表上得分明显低于正常水平。自主性的一个子量表与冷漠（r= -0.65）相关，但与亨廷顿病的其他症状无关。
这项研究提供了亨廷顿病患者自主性受损的证据，以及自主性与冷漠之间的关联。研究结果强调了在整个疾病过程中保持患者自主性和参与护理的重要性。
