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本文引用的文献

1
Patient-reported impact of symptoms in Huntington disease: PRISM-HD.亨廷顿病患者报告的症状影响:PRISM-HD。
Neurology. 2020 May 12;94(19):e2045-e2053. doi: 10.1212/WNL.0000000000008906. Epub 2020 Mar 19.
2
Sex differences in movement disorders.运动障碍的性别差异。
Nat Rev Neurol. 2020 Feb;16(2):84-96. doi: 10.1038/s41582-019-0294-x. Epub 2020 Jan 3.
3
Impact of sex differences and gender specificity on behavioral characteristics and pathophysiology of neurodegenerative disorders.性别差异和性别特异性对神经退行性疾病的行为特征和病理生理学的影响。
Neurosci Biobehav Rev. 2019 Jul;102:95-105. doi: 10.1016/j.neubiorev.2019.04.003. Epub 2019 Apr 5.
4
Huntington's disease in the United States: Variation by demographic and socioeconomic factors.美国亨廷顿病:人口统计学和社会经济因素的差异。
Mov Disord. 2019 Jun;34(6):858-865. doi: 10.1002/mds.27653. Epub 2019 Mar 13.
5
Factors contributing to clinical picture and progression of Huntington's disease.导致亨廷顿舞蹈症临床表现及病情进展的因素。
Neural Regen Res. 2018 Aug;13(8):1364-1365. doi: 10.4103/1673-5374.235241.
6
Age at Death and Causes of Death in Patients with Huntington Disease in Norway in 1986-2015.1986 - 2015年挪威亨廷顿病患者的死亡年龄及死因
J Huntingtons Dis. 2018;7(1):77-86. doi: 10.3233/JHD-170270.
7
The contribution of gender differences in motor, behavioral and cognitive features to functional capacity, independence and quality of life in patients with Huntington's disease.性别差异在运动、行为和认知特征方面对亨廷顿病患者的功能能力、独立性和生活质量的贡献。
Parkinsonism Relat Disord. 2018 Apr;49:42-47. doi: 10.1016/j.parkreldis.2018.01.006. Epub 2018 Jan 5.
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Neuropsychiatric Burden in Huntington's Disease.亨廷顿舞蹈症的神经精神负担
Brain Sci. 2017 Jun 16;7(6):67. doi: 10.3390/brainsci7060067.
9
Modeling Variability in the Progression of Huntington's Disease A Novel Modeling Approach Applied to Structural Imaging Markers from TRACK-HD.亨廷顿舞蹈病进展变异性建模:一种应用于TRACK-HD结构成像标记物的新型建模方法
CPT Pharmacometrics Syst Pharmacol. 2016 Aug;5(8):437-45. doi: 10.1002/psp4.12097. Epub 2016 Aug 2.
10
Disease stage, but not sex, predicts depression and psychological distress in Huntington's disease: A European population study.疾病阶段而非性别可预测亨廷顿舞蹈症患者的抑郁和心理困扰:一项欧洲人群研究。
J Psychosom Res. 2016 Jan;80:17-22. doi: 10.1016/j.jpsychores.2015.11.003. Epub 2015 Nov 12.

亨廷顿舞蹈症的性别差异:评估“亨廷顿舞蹈症注册研究(Enroll-HD)”数据库

Sex Differences in Huntington's Disease: Evaluating the Enroll-HD Database.

作者信息

Hentosh Samantha, Zhu Liang, Patino Jorge, Furr J Weldon, Rocha Natalia P, Furr Stimming Erin

机构信息

McGovern Medical School, The University of Texas Health Science Center (UTHealth) Houston Texas USA.

Department of Internal Medicine McGovern Medical School, The University of Texas Health Science Center (UTHealth) Houston Texas USA.

出版信息

Mov Disord Clin Pract. 2021 Mar 8;8(3):420-426. doi: 10.1002/mdc3.13178. eCollection 2021 Apr.

DOI:10.1002/mdc3.13178
PMID:33816672
原文链接:https://pmc.ncbi.nlm.nih.gov/articles/PMC8015889/
Abstract

BACKGROUND

Identifying sex-related differences is critical for enhancing our understanding of factors that may impact prognosis and advance treatments in Huntington's disease (HD).

OBJECTIVES

To investigate if sex-related differences exist in clinical HD.

METHODS

Longitudinal study of the Enroll-HD database. Manifest HD patients were included in the analysis (N = 8401). Linear mixed models were used to assess motor, behavioral, and cognitive functioning over a series of four annual visits, and compared male and female HD gene carriers.

RESULTS

HD patients showed significant sex-dependent differences in motor, cognitive, and behavioral symptoms. Both sexes had worsened motor symptoms over the course of four visits, but there was a significant disparity between sexes, with females consistently presenting with more symptoms than males. For behavioral symptoms, specifically depressive symptoms, females had significantly more depressive symptoms, although self-reported symptoms in both sexes became less severe throughout time.

CONCLUSIONS

Our analyses suggest that women have worse symptoms than men during the course of HD.

摘要

背景

识别性别相关差异对于增进我们对可能影响亨廷顿舞蹈症(HD)预后和推进治疗的因素的理解至关重要。

目的

调查临床HD中是否存在性别相关差异。

方法

对Enroll-HD数据库进行纵向研究。分析纳入有明显症状的HD患者(N = 8401)。采用线性混合模型评估在连续四次年度访视期间的运动、行为和认知功能,并比较男性和女性HD基因携带者。

结果

HD患者在运动、认知和行为症状方面表现出显著的性别依赖性差异。在四次访视过程中,两性的运动症状均有所恶化,但两性之间存在显著差异,女性始终比男性表现出更多症状。对于行为症状,特别是抑郁症状,女性有明显更多的抑郁症状,尽管随着时间推移两性自我报告的症状都有所减轻。

结论

我们的分析表明,在HD病程中女性的症状比男性更严重。