Andres Gil-Salcedo, Marine Lunven, Charlotte Jacquemot, Renaud Massart, Anne-Catherine Bachoud-Levi
{"title":"Specific contribution of cognitive and motor impairments with functional capacity and dependence in Huntington's disease.","authors":"Andres Gil-Salcedo, Marine Lunven, Charlotte Jacquemot, Renaud Massart, Anne-Catherine Bachoud-Levi","doi":"10.1007/s00415-025-12982-9","DOIUrl":null,"url":null,"abstract":"<p><strong>Background: </strong>Huntington's disease (HD) leads to increasing dependence. Unlike psychiatric disorders, motor and cognitive deficits evolve progressively over time. Understanding their specific impact on daily activities is crucial for preserving autonomy. However, because cognitive tasks in HD rely on motor functions, and motor tasks demand cognitive processing, disentangling their specific impact remains a challenge.</p><p><strong>Objective: </strong>To identify the specific contribution of cognitive and motor impairments on global functional capacity, basic and instrumental activities of daily living (ADL/IADL), and work-related activities (WRA) in HD.</p><p><strong>Methods: </strong>158 HD mutation carriers, enrolled in the BioHD (NCT01412125) and RepairHD (NCT03119246) studies, were evaluated with the Unified Huntington's Disease Rating Scale and the SelfCog. The SelfCog assesses motor processing separately from memory, language, executive functions and visuospatial processing. Linear regressions were fitted to assess how functional capacity declined with motor and cognition impairments. Odds of dependence in ADLs, IADLs and WRAs were estimated using logistic regressions.</p><p><strong>Results: </strong>Cognitive and motor performance were independently associated with functional capacities, though motor performance showed a stronger association than cognitive performance. Decline of all SelfCog cognitive domains contributed to functional decline, with stronger association with global and executive scores compared to language, visuospatial, and memory domains. Higher global and executive deficits were associated with an increased risk of dependence in ADLs, IADLs, and WRAs.</p><p><strong>Conclusion: </strong>The independent contributions of motor, followed by cognitive-mainly executive-functions to functional decline suggest targeted interventions to preserve autonomy and quality of life in HD.</p>","PeriodicalId":16558,"journal":{"name":"Journal of Neurology","volume":"272 3","pages":"224"},"PeriodicalIF":4.8000,"publicationDate":"2025-02-22","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11846732/pdf/","citationCount":"0","resultStr":null,"platform":"Semanticscholar","paperid":null,"PeriodicalName":"Journal of Neurology","FirstCategoryId":"3","ListUrlMain":"https://doi.org/10.1007/s00415-025-12982-9","RegionNum":2,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":null,"EPubDate":"","PubModel":"","JCR":"Q1","JCRName":"CLINICAL NEUROLOGY","Score":null,"Total":0}
引用次数: 0
Abstract
Background: Huntington's disease (HD) leads to increasing dependence. Unlike psychiatric disorders, motor and cognitive deficits evolve progressively over time. Understanding their specific impact on daily activities is crucial for preserving autonomy. However, because cognitive tasks in HD rely on motor functions, and motor tasks demand cognitive processing, disentangling their specific impact remains a challenge.
Objective: To identify the specific contribution of cognitive and motor impairments on global functional capacity, basic and instrumental activities of daily living (ADL/IADL), and work-related activities (WRA) in HD.
Methods: 158 HD mutation carriers, enrolled in the BioHD (NCT01412125) and RepairHD (NCT03119246) studies, were evaluated with the Unified Huntington's Disease Rating Scale and the SelfCog. The SelfCog assesses motor processing separately from memory, language, executive functions and visuospatial processing. Linear regressions were fitted to assess how functional capacity declined with motor and cognition impairments. Odds of dependence in ADLs, IADLs and WRAs were estimated using logistic regressions.
Results: Cognitive and motor performance were independently associated with functional capacities, though motor performance showed a stronger association than cognitive performance. Decline of all SelfCog cognitive domains contributed to functional decline, with stronger association with global and executive scores compared to language, visuospatial, and memory domains. Higher global and executive deficits were associated with an increased risk of dependence in ADLs, IADLs, and WRAs.
Conclusion: The independent contributions of motor, followed by cognitive-mainly executive-functions to functional decline suggest targeted interventions to preserve autonomy and quality of life in HD.
期刊介绍：
The Journal of Neurology is an international peer-reviewed journal which provides a source for publishing original communications and reviews on clinical neurology covering the whole field.
In addition, Letters to the Editors serve as a forum for clinical cases and the exchange of ideas which highlight important new findings. A section on Neurological progress serves to summarise the major findings in certain fields of neurology. Commentaries on new developments in clinical neuroscience, which may be commissioned or submitted, are published as editorials.
Every neurologist interested in the current diagnosis and treatment of neurological disorders needs access to the information contained in this valuable journal.
求助内容：
应助结果提醒方式：
京公网安备 11010802042870号
