Measuring cognitive impairment and monitoring cognitive decline in Huntington's disease : a comparison of assessment instruments
Horta, Andrea (Universitat Autònoma de Barcelona. Departament de Medicina)
Martinez-Horta, Saul (Universitat Autònoma de Barcelona. Departament de Medicina)
Pérez-Pérez, Jesús (Universitat Autònoma de Barcelona. Departament de Medicina)
Puig-Davi, Arnau (Institut d'Investigació Biomèdica Sant Pau)
de Lucia, Natascia (University of Naples "Federico II", Naples, Italy)
de Michele, Giuseppe (University of Naples "Federico II", Naples, Italy)
Salvatore, Elena (University of Naples "Federico II", Naples, Italy)
Kehrer, Stefanie (Charité - Universitätsmedizin Berlin)
Priller, Josef (Charité - Universitätsmedizin Berlin)
Migliore, Simone (Fondazione IRCCS Casa Sollievo Della Sofferenza Research Hospital, San Giovanni Rotondo, Italy)
Squitieri, Ferdinando (Fondazione IRCCS Casa Sollievo Della Sofferenza Research Hospital, San Giovanni Rotondo, Italy)
Castaldo, Ana (Fondazione IRCCS Istituto Neurologico Carlo Besta, Milan, Italy)
Mariotti, Caterina (Fondazione IRCCS Istituto Neurologico Carlo Besta, Milan, Italy)
Mañanes, Verónica (Hospital Universitario Ramón y Cajal (Madrid))
Lopez-Sendon, José Luís (Hospital Universitario Ramón y Cajal (Madrid))
Rodriguez, Noelia (Fundación Jimenez Diaz)
Martinez-Descals, Asunción (Fundación Jimenez Diaz)
Júlio, Filipa (Coimbra University Hospital)
Januário, Cristina (Coimbra University Hospital)
Delussi, marianna (European Huntington's Disease Network (EHDN), Ulm, Germany)
de Tommaso, Marina (European Huntington's Disease Network (EHDN), Ulm, Germany)
Noguera, Sandra (Hospital Mare de Deu de La Mercè)
Ruiz-Idiago, Jesús (Hospital Mare de Deu de La Mercè)
Sitek, Emilia J. (St. Adalbert Hospital, Copernicus, Gdańsk, Poland)
Wallner, Renata (Medical University of Wroclaw, Wroclaw, Poland)
Nuzzi, Angela (European Huntington's Disease Network (EHDN), Ulm, Germany)
Pagonabarraga Mora, Javier (Universitat Autònoma de Barcelona. Departament de Medicina)
Kulisevsky, Jaime (Universitat Autònoma de Barcelona. Departament de Medicina)

Date:	2023
Abstract:	Background: Progressive cognitive decline is an inevitable feature of Huntington's disease (HD) but specific criteria and instruments are still insufficiently developed to reliably classify patients into categories of cognitive severity and to monitor the progression of cognitive impairment. Methods: We collected data from a cohort of 180 positive gene-carriers: 33 with premanifest HD and 147 with manifest HD. Using a specifically developed gold-standard for cognitive status we classified participants into those with normal cognition, those with mild cognitive impairment, and those with dementia. We administered the Parkinson's Disease-Cognitive Rating Scale (PD-CRS), the MMSE and the UHDRS cogscore at baseline, and at 6-month and 12-month follow-up visits. Cutoff scores discriminating between the three cognitive categories were calculated for each instrument. For each cognitive group and instrument we addressed cognitive progression, sensitivity to change, and the minimally clinical important difference corresponding to conversion from one category to another. Results: The PD-CRS cutoff scores for MCI and dementia showed excellent sensitivity and specificity ratios that were not achieved with the other instruments. Throughout follow-up, in all cognitive groups, PD-CRS captured the rate of conversion from one cognitive category to another and also the different patterns in terms of cognitive trajectories. Conclusion: The PD-CRS is a valid and reliable instrument to capture MCI and dementia syndromes in HD. It captures the different trajectories of cognitive progression as a function of cognitive status and shows sensitivity to change in MCI and dementia.
Note:	Altres ajuts: acords transformatius de la UAB
Rights:	Aquest document està subjecte a una llicència d'ús Creative Commons. Es permet la reproducció total o parcial, la distribució, la comunicació pública de l'obra i la creació d'obres derivades, fins i tot amb finalitats comercials, sempre i quan es reconegui l'autoria de l'obra original.
Language:	Anglès
Document:	Article ; recerca ; Versió publicada
Subject:	Huntington's disease ; Cognition ; Neuropsychology ; Disease progression ; Mild cognitive impairment ; Dementia
Published in:	Journal of Neurology, Vol. 270, Num. 11 (november 2023) , p. 5408-5417, ISSN 1432-1459

DOI: 10.1007/s00415-023-11804-0
PMID: 37462754

10 p, 876.7 KB

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Research literature > UAB research groups literature > Research Centres and Groups (research output) > Health sciences and biosciences > Institut de Recerca Sant Pau
Articles > Research articles
Articles > Published articles