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The Comprehensive Analysis of Motor and Neuropsychiatric Symptoms in Patients with Huntington’s Disease from China: A Cross-Sectional Study
Huntington’s disease (HD) is an autosomal dominant inherited neurodegenerative disorder caused by CAG repeats expansion. There is a paucity of comprehensive clinical analysis in Chinese HD patients due to the low prevalence of HD in Asia. We aimed to comprehensively describe the motor, neuropsychiat...
Autores principales: | , , , , , , , , , , , , , , |
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Formato: | Online Artículo Texto |
Lenguaje: | English |
Publicado: |
MDPI
2022
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Materias: | |
Acceso en línea: | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC9821667/ https://www.ncbi.nlm.nih.gov/pubmed/36615008 http://dx.doi.org/10.3390/jcm12010206 |
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author | Cheng, Yangfan Gu, Xiaojing Liu, Kuncheng Yang, Tianmi Xiao, Yi Jiang, Qirui Huang, Jingxuan Lin, Junyu Wei, Qianqian Ou, Ruwei Hou, Yanbing Zhang, Lingyu Li, Chunyu Burgunder, Jean-Marc Shang, Huifang |
author_facet | Cheng, Yangfan Gu, Xiaojing Liu, Kuncheng Yang, Tianmi Xiao, Yi Jiang, Qirui Huang, Jingxuan Lin, Junyu Wei, Qianqian Ou, Ruwei Hou, Yanbing Zhang, Lingyu Li, Chunyu Burgunder, Jean-Marc Shang, Huifang |
author_sort | Cheng, Yangfan |
collection | PubMed |
description | Huntington’s disease (HD) is an autosomal dominant inherited neurodegenerative disorder caused by CAG repeats expansion. There is a paucity of comprehensive clinical analysis in Chinese HD patients due to the low prevalence of HD in Asia. We aimed to comprehensively describe the motor, neuropsychiatric symptoms, and functional assessment in patients with HD from China. A total of 205 HD patients were assessed by the Unified Huntington’s Disease Rating Scale (UHDRS), the short version of Problem-Behavior Assessment (PBA-s), Hamilton Depression Scale (HAMD) and Beck Depression Inventory (BDI). Multivariate logistic regression analysis was used to explore the independent variables correlated with neuropsychiatric subscales. The mean age of motor symptom onset was 41.8 ± 10.0 years old with a diagnostic delay of 4.3 ± 3.8 years and a median CAG repeats of 44. The patients with a positive family history had a younger onset and larger CAG expansion than the patients without a family history (p < 0.05). There was a significant increase in total motor score across disease stages (p < 0.0001). Depression (51%) was the most common neuropsychiatric symptom at all stages, whereas moderate to severe apathy commonly occurred in advanced HD stages. We found lower functional capacity and higher HAMD were independently correlated with irritability; higher HAMD and higher BDI were independently correlated with affect; male sex and higher HAMD were independently correlated with apathy. In summary, comprehensive clinical profile analysis of Chinese HD patients showed not only chorea-like movement, but psychiatric symptoms were outstanding problems and need to be detected early. Our study provides the basis to guide clinical practice, especially in practical diagnostic and management processes. |
format | Online Article Text |
id | pubmed-9821667 |
institution | National Center for Biotechnology Information |
language | English |
publishDate | 2022 |
publisher | MDPI |
record_format | MEDLINE/PubMed |
spelling | pubmed-98216672023-01-07 The Comprehensive Analysis of Motor and Neuropsychiatric Symptoms in Patients with Huntington’s Disease from China: A Cross-Sectional Study Cheng, Yangfan Gu, Xiaojing Liu, Kuncheng Yang, Tianmi Xiao, Yi Jiang, Qirui Huang, Jingxuan Lin, Junyu Wei, Qianqian Ou, Ruwei Hou, Yanbing Zhang, Lingyu Li, Chunyu Burgunder, Jean-Marc Shang, Huifang J Clin Med Article Huntington’s disease (HD) is an autosomal dominant inherited neurodegenerative disorder caused by CAG repeats expansion. There is a paucity of comprehensive clinical analysis in Chinese HD patients due to the low prevalence of HD in Asia. We aimed to comprehensively describe the motor, neuropsychiatric symptoms, and functional assessment in patients with HD from China. A total of 205 HD patients were assessed by the Unified Huntington’s Disease Rating Scale (UHDRS), the short version of Problem-Behavior Assessment (PBA-s), Hamilton Depression Scale (HAMD) and Beck Depression Inventory (BDI). Multivariate logistic regression analysis was used to explore the independent variables correlated with neuropsychiatric subscales. The mean age of motor symptom onset was 41.8 ± 10.0 years old with a diagnostic delay of 4.3 ± 3.8 years and a median CAG repeats of 44. The patients with a positive family history had a younger onset and larger CAG expansion than the patients without a family history (p < 0.05). There was a significant increase in total motor score across disease stages (p < 0.0001). Depression (51%) was the most common neuropsychiatric symptom at all stages, whereas moderate to severe apathy commonly occurred in advanced HD stages. We found lower functional capacity and higher HAMD were independently correlated with irritability; higher HAMD and higher BDI were independently correlated with affect; male sex and higher HAMD were independently correlated with apathy. In summary, comprehensive clinical profile analysis of Chinese HD patients showed not only chorea-like movement, but psychiatric symptoms were outstanding problems and need to be detected early. Our study provides the basis to guide clinical practice, especially in practical diagnostic and management processes. MDPI 2022-12-27 /pmc/articles/PMC9821667/ /pubmed/36615008 http://dx.doi.org/10.3390/jcm12010206 Text en © 2022 by the authors. https://creativecommons.org/licenses/by/4.0/Licensee MDPI, Basel, Switzerland. This article is an open access article distributed under the terms and conditions of the Creative Commons Attribution (CC BY) license (https://creativecommons.org/licenses/by/4.0/). |
spellingShingle | Article Cheng, Yangfan Gu, Xiaojing Liu, Kuncheng Yang, Tianmi Xiao, Yi Jiang, Qirui Huang, Jingxuan Lin, Junyu Wei, Qianqian Ou, Ruwei Hou, Yanbing Zhang, Lingyu Li, Chunyu Burgunder, Jean-Marc Shang, Huifang The Comprehensive Analysis of Motor and Neuropsychiatric Symptoms in Patients with Huntington’s Disease from China: A Cross-Sectional Study |
title | The Comprehensive Analysis of Motor and Neuropsychiatric Symptoms in Patients with Huntington’s Disease from China: A Cross-Sectional Study |
title_full | The Comprehensive Analysis of Motor and Neuropsychiatric Symptoms in Patients with Huntington’s Disease from China: A Cross-Sectional Study |
title_fullStr | The Comprehensive Analysis of Motor and Neuropsychiatric Symptoms in Patients with Huntington’s Disease from China: A Cross-Sectional Study |
title_full_unstemmed | The Comprehensive Analysis of Motor and Neuropsychiatric Symptoms in Patients with Huntington’s Disease from China: A Cross-Sectional Study |
title_short | The Comprehensive Analysis of Motor and Neuropsychiatric Symptoms in Patients with Huntington’s Disease from China: A Cross-Sectional Study |
title_sort | comprehensive analysis of motor and neuropsychiatric symptoms in patients with huntington’s disease from china: a cross-sectional study |
topic | Article |
url | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC9821667/ https://www.ncbi.nlm.nih.gov/pubmed/36615008 http://dx.doi.org/10.3390/jcm12010206 |
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