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Measuring the functional impact of cognitive impairment in Huntington's disease

Authors :
Horta, Andrea
Martinez-Horta, Saul
Pérez-Pérez, Jesús
Sampedro, Frederic
Puig-Davi, Arnau
Pagonabarraga Mora, Javier
Kulisevsky, Jaime
Universitat Autònoma de Barcelona
Horta, Andrea
Martinez-Horta, Saul
Pérez-Pérez, Jesús
Sampedro, Frederic
Puig-Davi, Arnau
Pagonabarraga Mora, Javier
Kulisevsky, Jaime
Universitat Autònoma de Barcelona
Publication Year :
2022

Abstract

Background: Patients with Huntington's disease (HD) exhibit a variable predominance of cognitive, behavioral and motor symptoms. A specific instrument focusing on the impact of cognitive impairment in HD over functional capacity is lacking. Objective: To address the need for a brief and specifically developed HD questionnaire able to capture functional aspects suspected to be sensitive to cognitive impairment. Methods: We developed and validated the "Huntington's Disease-Cognitive Functional Rating Scale" (HD-CFRS) in 78 symptomatic carriers of the Huntington's disease mutation. We also administered the HD-CFRS to a knowledgeable informant to measure the level of agreement. To explore the association between HD-CFRS scores and participants' cognitive status, we administered objective measures of cognition. Participants were classified as cognitively preserved (HD-NC), as having mild cognitive impairment (HD-MCI), or as having dementia (HD-Dem). Results: The HD-CFRS showed concurrent validity and internal consistency in the three groups. HD carriers and informants in the HD-NC group obtained similar HD-CFRS scores. However, in patients with mild cognitive impairment and dementia, informers reported greater functional impairment than HD participants. The HD-CFRS total score showed strong correlations with measures assessing cognition. Conclusions: These findings support the utility of the HD-CFRS as a brief and reliable instrument to measure functional defects associated with cognitive impairment in HD. We believe this questionnaire could be a useful tool both for clinical practice and research.

Details

Database :
OAIster
Notes :
application/pdf, English
Publication Type :
Electronic Resource
Accession number :
edsoai.on1337031188
Document Type :
Electronic Resource