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Cognitive and behavioral associated changes in manifest Huntington disease: A retrospective cross‐sectional study
INTRODUCTION: Behavioral and cognitive changes can be observed across all Huntington disease (HD) stages. Our multicenter and retrospective study investigated the association between cognitive and behavioral scale scores in manifest HD, at three different yearly timepoints. METHODS: We analyzed cogn...
Autores principales: | , , , , , , , , , |
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Formato: | Online Artículo Texto |
Lenguaje: | English |
Publicado: |
John Wiley and Sons Inc.
2021
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Materias: | |
Acceso en línea: | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC8323039/ https://www.ncbi.nlm.nih.gov/pubmed/34110097 http://dx.doi.org/10.1002/brb3.2151 |
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author | Migliore, Simone D’Aurizio, Giulia Maffi, Sabrina Ceccarelli, Consuelo Ristori, Giovanni Romano, Silvia Castaldo, Anna Mariotti, Caterina Curcio, Giuseppe Squitieri, Ferdinando |
author_facet | Migliore, Simone D’Aurizio, Giulia Maffi, Sabrina Ceccarelli, Consuelo Ristori, Giovanni Romano, Silvia Castaldo, Anna Mariotti, Caterina Curcio, Giuseppe Squitieri, Ferdinando |
author_sort | Migliore, Simone |
collection | PubMed |
description | INTRODUCTION: Behavioral and cognitive changes can be observed across all Huntington disease (HD) stages. Our multicenter and retrospective study investigated the association between cognitive and behavioral scale scores in manifest HD, at three different yearly timepoints. METHODS: We analyzed cognitive and behavioral domains by the Unified Huntington's Disease Rating Scale (UHDRS) and by the Problem Behaviors Assessment Short Form (PBA‐s), at three different yearly times of life (t0 or baseline, t1 after one year, t2 after two years), in 97 patients with manifest HD (mean age 48.62 ± 13.1), from three ENROLL‐HD Centers. In order to test the disease progression, we also examined patients’ motor and functional changes by the UHDRS, overtime. RESULTS: The severity of apathy and of perseveration/obsession was associated with the severity of the cognitive decline (p < .0001), regardless of the yearly timepoint. The score of irritability significantly and positively correlated with perseveration errors in the verbal fluency test at t0 (r = .34; p = .001), while the psychosis significantly and negatively correlated with the information processing speed at t0 (r = −.21; p = .038) and significantly and positively correlated with perseveration errors in the verbal fluency test at t1 (r = .35; p < .0001). The disease progression was confirmed by the significant worsening of the UHDRS‐Total Motor Score (TMS) and of the UHDRS‐Total Functional Capacity (TFC) scale score after two‐year follow‐up (p < .0001). CONCLUSION: Although the progression of abnormal behavioral manifestations cannot be predicted in HD, the severity of apathy and perseveration/obsessions are significantly associated with the severity of the cognitive function impairment, thus contributing, together, to the disease development and to patients’ loss of independence, in addition to the neurological manifestations. This cognitive‐behavior pattern determines a common underlying deficit depending on a dysexecutive syndrome. |
format | Online Article Text |
id | pubmed-8323039 |
institution | National Center for Biotechnology Information |
language | English |
publishDate | 2021 |
publisher | John Wiley and Sons Inc. |
record_format | MEDLINE/PubMed |
spelling | pubmed-83230392021-08-04 Cognitive and behavioral associated changes in manifest Huntington disease: A retrospective cross‐sectional study Migliore, Simone D’Aurizio, Giulia Maffi, Sabrina Ceccarelli, Consuelo Ristori, Giovanni Romano, Silvia Castaldo, Anna Mariotti, Caterina Curcio, Giuseppe Squitieri, Ferdinando Brain Behav Original Research INTRODUCTION: Behavioral and cognitive changes can be observed across all Huntington disease (HD) stages. Our multicenter and retrospective study investigated the association between cognitive and behavioral scale scores in manifest HD, at three different yearly timepoints. METHODS: We analyzed cognitive and behavioral domains by the Unified Huntington's Disease Rating Scale (UHDRS) and by the Problem Behaviors Assessment Short Form (PBA‐s), at three different yearly times of life (t0 or baseline, t1 after one year, t2 after two years), in 97 patients with manifest HD (mean age 48.62 ± 13.1), from three ENROLL‐HD Centers. In order to test the disease progression, we also examined patients’ motor and functional changes by the UHDRS, overtime. RESULTS: The severity of apathy and of perseveration/obsession was associated with the severity of the cognitive decline (p < .0001), regardless of the yearly timepoint. The score of irritability significantly and positively correlated with perseveration errors in the verbal fluency test at t0 (r = .34; p = .001), while the psychosis significantly and negatively correlated with the information processing speed at t0 (r = −.21; p = .038) and significantly and positively correlated with perseveration errors in the verbal fluency test at t1 (r = .35; p < .0001). The disease progression was confirmed by the significant worsening of the UHDRS‐Total Motor Score (TMS) and of the UHDRS‐Total Functional Capacity (TFC) scale score after two‐year follow‐up (p < .0001). CONCLUSION: Although the progression of abnormal behavioral manifestations cannot be predicted in HD, the severity of apathy and perseveration/obsessions are significantly associated with the severity of the cognitive function impairment, thus contributing, together, to the disease development and to patients’ loss of independence, in addition to the neurological manifestations. This cognitive‐behavior pattern determines a common underlying deficit depending on a dysexecutive syndrome. John Wiley and Sons Inc. 2021-06-10 /pmc/articles/PMC8323039/ /pubmed/34110097 http://dx.doi.org/10.1002/brb3.2151 Text en © 2021 The Authors. Brain and Behavior published by Wiley Periodicals LLC https://creativecommons.org/licenses/by/4.0/This is an open access article under the terms of the http://creativecommons.org/licenses/by/4.0/ (https://creativecommons.org/licenses/by/4.0/) License, which permits use, distribution and reproduction in any medium, provided the original work is properly cited. |
spellingShingle | Original Research Migliore, Simone D’Aurizio, Giulia Maffi, Sabrina Ceccarelli, Consuelo Ristori, Giovanni Romano, Silvia Castaldo, Anna Mariotti, Caterina Curcio, Giuseppe Squitieri, Ferdinando Cognitive and behavioral associated changes in manifest Huntington disease: A retrospective cross‐sectional study |
title | Cognitive and behavioral associated changes in manifest Huntington disease: A retrospective cross‐sectional study |
title_full | Cognitive and behavioral associated changes in manifest Huntington disease: A retrospective cross‐sectional study |
title_fullStr | Cognitive and behavioral associated changes in manifest Huntington disease: A retrospective cross‐sectional study |
title_full_unstemmed | Cognitive and behavioral associated changes in manifest Huntington disease: A retrospective cross‐sectional study |
title_short | Cognitive and behavioral associated changes in manifest Huntington disease: A retrospective cross‐sectional study |
title_sort | cognitive and behavioral associated changes in manifest huntington disease: a retrospective cross‐sectional study |
topic | Original Research |
url | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC8323039/ https://www.ncbi.nlm.nih.gov/pubmed/34110097 http://dx.doi.org/10.1002/brb3.2151 |
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