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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...

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Autores principales: 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
Formato: Online Artículo Texto
Lenguaje:English
Publicado: MDPI 2022
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.
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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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