Júlio Filipa, Ribeiro Maria J, Morgadinho Ana, Sousa Mário, van Asselen Marieke, Simões Mário R, Castelo-Branco Miguel, Januário Cristina
University of Coimbra, Faculty of Psychology and Education Sciences, Coimbra, Portugal.
University of Coimbra, Coimbra Institute for Biomedical Imaging and Translational Research (CIBIT), Coimbra, Portugal.
Disabil Rehabil. 2022 Mar;44(6):921-939. doi: 10.1080/09638288.2020.1783001. Epub 2020 Jul 3.
Patients with Parkinson's and Huntington's Disease (PD and HD) present impairments in cognitively challenging everyday activities. This study contrasts these two basal ganglia disorders on the ability to perform daily life- like tasks and their level of awareness regarding the disease impact on function.
19 controls, 10 early-onset PD, 20 early stage PD, and 15 early manifest HD patients were compared in the "EcoKitchen," a virtual reality task with increasing executive load, the "Behavioural Assessment of Dysexecutive Syndrome battery - BADS," and "The Adults and Older Adults Functional Assessment Inventory - IAFAI," a self-report functional questionnaire. The EcoKitchen clinical correlates were investigated.
All clinical groups presented slower EcoKitchen performance than controls, however, only HD patients showed decreased accuracy. HD and PD patients exhibited reduced BADS scores compared to the other study participants. Importantly, on the IAFAI, PD patients signalled more physically related incapacities and HD patients indicated more cognitively related incapacities. Accordingly, the EcoKitchen performance was significantly associated with PD motor symptom severity.
Our findings suggest differential disease impact on cognition and function across PD and HD patients, with preserved awareness regarding disease- related functional sequelae. These observations have important implications for clinical management, research and rehabilitation.Implications for rehabilitationPatients with early stage Parkinson's and Huntington's disease have diagnosis-specific impairments in the performance of executively demanding everyday activities and, yet, show preserved awareness about the disease impact on their daily life.An active involvement of patients in the rehabilitation process should be encouraged, as their appraisal of the disease effects can help on practical decisions about meaningful targets for intervention, vocational choices, quality-of-life issues and/or specific everyday skills to boost.The EcoKitchen, a non-immersive virtual reality task, can detect and quantify early deficits in everyday-like tasks and is therefore a valuable tool for assessing the effects of rehabilitation strategies on the functional cognition of these patients.Rehabilitation efforts in the mild stages of Parkinson's and Huntington's disease should be aware of greater time needs from the patients in the performance of daily life tasks, target executive skills, and give a more prominent role to patients in symptoms report and management.
帕金森病和亨廷顿舞蹈病（PD和HD）患者在具有认知挑战性的日常活动中存在功能障碍。本研究对比了这两种基底神经节疾病在执行类似日常生活任务的能力以及对疾病对功能影响的认知水平方面的差异。
对19名对照组、10名早发型PD患者、20名早期PD患者和15名早期显性HD患者进行了比较，使用“生态厨房”（一种执行负荷逐渐增加的虚拟现实任务）、“执行功能障碍综合征行为评估量表 - BADS”以及“成人及老年人功能评估量表 - IAFAI”（一份自我报告功能问卷）。对生态厨房的临床相关性进行了研究。
所有临床组在生态厨房任务中的表现均比对照组慢，然而，只有HD患者的准确性降低。与其他研究参与者相比，HD和PD患者的BADS评分降低。重要的是，在IAFAI上，PD患者表示身体相关的能力丧失更多，而HD患者表示认知相关的能力丧失更多。因此，生态厨房的表现与PD运动症状严重程度显著相关。
我们的研究结果表明，PD和HD患者在认知和功能方面受到的疾病影响存在差异，但对疾病相关功能后遗症的认知保持完好。这些观察结果对临床管理、研究和康复具有重要意义。
对康复的启示
早期帕金森病和亨廷顿舞蹈病患者在执行要求较高的日常活动时存在特定于诊断的功能障碍，但对疾病对其日常生活的影响仍有清晰认知。
应鼓励患者积极参与康复过程，因为他们对疾病影响的评估有助于就有意义的干预目标、职业选择、生活质量问题和/或提升特定日常技能等实际决策提供帮助。
生态厨房，一种非沉浸式虚拟现实任务，可以检测和量化类似日常任务中的早期缺陷，因此是评估康复策略对这些患者功能认知影响的有价值工具。
帕金森病和亨廷顿舞蹈病轻度阶段的康复工作应意识到患者在执行日常生活任务时需要更多时间，以执行技能为目标，并在症状报告和管理中给予患者更突出的作用。
