Measuring cognitive impairment and monitoring cognitive decline in Huntington's disease
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. Me...
| Autores: | , , , , , , , , , , , , , , , , , , , , , , , , , , , |
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| Tipo de recurso: | artículo |
| Fecha de publicación: | 2023 |
| País: | España |
| Institución: | Universitat Autònoma de Barcelona |
| Repositorio: | Dipòsit Digital de Documents de la UAB |
| Idioma: | inglés |
| OAI Identifier: | oai:ddd.uab.cat:283155 |
| Acceso en línea: | https://ddd.uab.cat/record/283155 https://dx.doi.org/urn:doi:10.1007/s00415-023-11804-0 |
| Access Level: | acceso abierto |
| Palabra clave: | Huntington's disease Cognition Neuropsychology Disease progression Mild cognitive impairment Dementia |
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Measuring cognitive impairment and monitoring cognitive decline in Huntington's diseasea comparison of assessment instrumentsHorta-Barba, Andrea|||0000-0003-0050-5466Martínez-Horta, Saul|||0000-0003-0125-7249Pérez-Pérez, Jesús|||0000-0002-9835-0484Puig-Davi, Arnau|||0000-0001-8883-4947de Lucia, Natascia|||0000-0002-0818-0494de Michele, GiuseppeSalvatore, Elena|||0000-0003-1493-5133Kehrer, StefaniePriller, Josef|||0000-0001-7596-0979Migliore, Simone|||0000-0002-2665-231XSquitieri, Ferdinando|||0000-0002-7397-1727Castaldo, Ana|||0000-0003-3459-3521Mariotti, Caterina|||0000-0003-2405-3564Mañanes, VerónicaLopez-Sendon, José LuísRodriguez, NoeliaMartinez-Descals, AsunciónJúlio, FilipaJanuario, Cristina|||0000-0002-6978-876XDelussi, marianna|||0000-0002-5888-3024De Tommaso, Marina|||0000-0001-6567-5373Noguera, SandraRuiz-Idiago, JesúsSitek, Emilia J.Wallner, RenataNuzzi, AngelaPagonabarraga Mora, Javier|||0000-0002-3248-704XKulisevsky, Jaime|||0000-0003-4870-1431Huntington's diseaseCognitionNeuropsychologyDisease progressionMild cognitive impairmentDementiaBackground: 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. 22023-01-0120232023-01-01Articlehttp://purl.org/coar/resource_type/c_6501VoRhttp://purl.org/coar/version/c_970fb48d4fbd8a85info:eu-repo/semantics/articleapplication/pdfhttps://ddd.uab.cat/record/283155https://dx.doi.org/urn:doi:10.1007/s00415-023-11804-0reponame:Dipòsit Digital de Documents de la UABinstname:Universitat Autònoma de BarcelonaInglésengopen accesshttp://purl.org/coar/access_right/c_abf2Aquest 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.https://creativecommons.org/licenses/by/4.0/info:eu-repo/semantics/openAccessoai:ddd.uab.cat:2831552026-06-06T12:50:31Z |
| dc.title.none.fl_str_mv |
Measuring cognitive impairment and monitoring cognitive decline in Huntington's disease a comparison of assessment instruments |
| title |
Measuring cognitive impairment and monitoring cognitive decline in Huntington's disease |
| spellingShingle |
Measuring cognitive impairment and monitoring cognitive decline in Huntington's disease Horta-Barba, Andrea|||0000-0003-0050-5466 Huntington's disease Cognition Neuropsychology Disease progression Mild cognitive impairment Dementia |
| title_short |
Measuring cognitive impairment and monitoring cognitive decline in Huntington's disease |
| title_full |
Measuring cognitive impairment and monitoring cognitive decline in Huntington's disease |
| title_fullStr |
Measuring cognitive impairment and monitoring cognitive decline in Huntington's disease |
| title_full_unstemmed |
Measuring cognitive impairment and monitoring cognitive decline in Huntington's disease |
| title_sort |
Measuring cognitive impairment and monitoring cognitive decline in Huntington's disease |
| dc.creator.none.fl_str_mv |
Horta-Barba, Andrea|||0000-0003-0050-5466 Martínez-Horta, Saul|||0000-0003-0125-7249 Pérez-Pérez, Jesús|||0000-0002-9835-0484 Puig-Davi, Arnau|||0000-0001-8883-4947 de Lucia, Natascia|||0000-0002-0818-0494 de Michele, Giuseppe Salvatore, Elena|||0000-0003-1493-5133 Kehrer, Stefanie Priller, Josef|||0000-0001-7596-0979 Migliore, Simone|||0000-0002-2665-231X Squitieri, Ferdinando|||0000-0002-7397-1727 Castaldo, Ana|||0000-0003-3459-3521 Mariotti, Caterina|||0000-0003-2405-3564 Mañanes, Verónica Lopez-Sendon, José Luís Rodriguez, Noelia Martinez-Descals, Asunción Júlio, Filipa Januario, Cristina|||0000-0002-6978-876X Delussi, marianna|||0000-0002-5888-3024 De Tommaso, Marina|||0000-0001-6567-5373 Noguera, Sandra Ruiz-Idiago, Jesús Sitek, Emilia J. Wallner, Renata Nuzzi, Angela Pagonabarraga Mora, Javier|||0000-0002-3248-704X Kulisevsky, Jaime|||0000-0003-4870-1431 |
| author |
Horta-Barba, Andrea|||0000-0003-0050-5466 |
| author_facet |
Horta-Barba, Andrea|||0000-0003-0050-5466 Martínez-Horta, Saul|||0000-0003-0125-7249 Pérez-Pérez, Jesús|||0000-0002-9835-0484 Puig-Davi, Arnau|||0000-0001-8883-4947 de Lucia, Natascia|||0000-0002-0818-0494 de Michele, Giuseppe Salvatore, Elena|||0000-0003-1493-5133 Kehrer, Stefanie Priller, Josef|||0000-0001-7596-0979 Migliore, Simone|||0000-0002-2665-231X Squitieri, Ferdinando|||0000-0002-7397-1727 Castaldo, Ana|||0000-0003-3459-3521 Mariotti, Caterina|||0000-0003-2405-3564 Mañanes, Verónica Lopez-Sendon, José Luís Rodriguez, Noelia Martinez-Descals, Asunción Júlio, Filipa Januario, Cristina|||0000-0002-6978-876X Delussi, marianna|||0000-0002-5888-3024 De Tommaso, Marina|||0000-0001-6567-5373 Noguera, Sandra Ruiz-Idiago, Jesús Sitek, Emilia J. Wallner, Renata Nuzzi, Angela Pagonabarraga Mora, Javier|||0000-0002-3248-704X Kulisevsky, Jaime|||0000-0003-4870-1431 |
| author_role |
author |
| author2 |
Martínez-Horta, Saul|||0000-0003-0125-7249 Pérez-Pérez, Jesús|||0000-0002-9835-0484 Puig-Davi, Arnau|||0000-0001-8883-4947 de Lucia, Natascia|||0000-0002-0818-0494 de Michele, Giuseppe Salvatore, Elena|||0000-0003-1493-5133 Kehrer, Stefanie Priller, Josef|||0000-0001-7596-0979 Migliore, Simone|||0000-0002-2665-231X Squitieri, Ferdinando|||0000-0002-7397-1727 Castaldo, Ana|||0000-0003-3459-3521 Mariotti, Caterina|||0000-0003-2405-3564 Mañanes, Verónica Lopez-Sendon, José Luís Rodriguez, Noelia Martinez-Descals, Asunción Júlio, Filipa Januario, Cristina|||0000-0002-6978-876X Delussi, marianna|||0000-0002-5888-3024 De Tommaso, Marina|||0000-0001-6567-5373 Noguera, Sandra Ruiz-Idiago, Jesús Sitek, Emilia J. Wallner, Renata Nuzzi, Angela Pagonabarraga Mora, Javier|||0000-0002-3248-704X Kulisevsky, Jaime|||0000-0003-4870-1431 |
| author2_role |
author author author author author author author author author author author author author author author author author author author author author author author author author author author |
| dc.subject.none.fl_str_mv |
Huntington's disease Cognition Neuropsychology Disease progression Mild cognitive impairment Dementia |
| topic |
Huntington's disease Cognition Neuropsychology Disease progression Mild cognitive impairment Dementia |
| description |
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. |
| publishDate |
2023 |
| dc.date.none.fl_str_mv |
2 2023-01-01 2023 2023-01-01 |
| dc.type.none.fl_str_mv |
Article http://purl.org/coar/resource_type/c_6501 VoR http://purl.org/coar/version/c_970fb48d4fbd8a85 |
| dc.type.openaire.fl_str_mv |
info:eu-repo/semantics/article |
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article |
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https://ddd.uab.cat/record/283155 https://dx.doi.org/urn:doi:10.1007/s00415-023-11804-0 |
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https://ddd.uab.cat/record/283155 https://dx.doi.org/urn:doi:10.1007/s00415-023-11804-0 |
| dc.language.none.fl_str_mv |
Inglés eng |
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Inglés |
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eng |
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open access http://purl.org/coar/access_right/c_abf2 https://creativecommons.org/licenses/by/4.0/ |
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info:eu-repo/semantics/openAccess |
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open access http://purl.org/coar/access_right/c_abf2 https://creativecommons.org/licenses/by/4.0/ |
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openAccess |
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application/pdf |
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reponame:Dipòsit Digital de Documents de la UAB instname:Universitat Autònoma de Barcelona |
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Universitat Autònoma de Barcelona |
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