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早期亨廷顿舞蹈病中的冷漠与功能状态

Apathy and Functional Status in Early-Stage Huntington's Disease.

作者信息

Gibson Jessie S, Hay Kaitlyn R, Claassen Daniel O, McDonell Katherine E, Brown Amy E, Wynn Amy, Jiang Jessica, Isaacs David A

机构信息

University of Virginia School of Nursing, Charlottesville (Gibson, Jiang); Department of Neurology, Vanderbilt University Medical Center, Nashville (Hay, Claassen, McDonell, Brown, Wynn, Isaacs).

出版信息

J Neuropsychiatry Clin Neurosci. 2025 Spring;37(2):125-130. doi: 10.1176/appi.neuropsych.20230225. Epub 2024 Nov 19.

DOI:10.1176/appi.neuropsych.20230225
PMID:39558706
原文链接:https://pmc.ncbi.nlm.nih.gov/articles/PMC11996602/
Abstract

OBJECTIVE

Apathy is common in Huntington's disease (HD) and difficult to treat. Multiple recent calls have been made to increase understanding of apathy across the spectrum of HD severity. Functional status is an important outcome in HD trials; however, no consensus currently exists regarding the impact of apathy on functional status in HD. The authors aimed to identify correlates of apathy and effects on functional status in a primarily early-stage HD sample.

METHODS

This study included secondary analyses of data from a study of neuropsychiatric symptoms in a clinical HD sample. Spearman correlation analyses were used to assess the relationships between apathy (with the Frontal Systems Behavior Scale-Apathy [FrSBe-Apathy] subscore), clinical variables, and patient-reported outcomes. To assess the association of apathy with functional status, two multiple regression analyses were performed, with a different functional status measure (Adult Functional Adaptive Behavior [AFAB] scale or Total Functional Capacity [TFC] scale) as the dependent variable in each analysis.

RESULTS

Statistically significant correlates of apathy included the Quality of Life in Neurological Disorders (Neuro-QoL) Satisfaction With Social Roles and Activities and Neuro-QoL Positive Affect and Well-Being scores (N=70 patients). Univariate correlation analyses also revealed statistically significant associations of FrSBe-Apathy scores with both functional status measures. In the multiple regression analyses, apathy significantly contributed to variability in functional status as measured by both the AFAB (N=49 patients) and TFC (N=56 patients) scales.

CONCLUSIONS

These results underscore the need to address apathy as a target for improving functional status, social satisfaction, and well-being in HD, even for individuals with early-stage HD.

摘要

目的

冷漠在亨廷顿舞蹈症(HD)中很常见且难以治疗。最近多次呼吁要加深对HD严重程度范围内冷漠症状的理解。功能状态是HD试验中的一项重要结果;然而,目前对于冷漠对HD患者功能状态的影响尚无共识。作者旨在确定在主要为早期HD样本中冷漠的相关因素及其对功能状态的影响。

方法

本研究纳入了对一个临床HD样本中神经精神症状研究数据的二次分析。采用斯皮尔曼相关性分析来评估冷漠(采用额叶系统行为量表 - 冷漠[FrSBe - 冷漠]子评分)、临床变量与患者报告结局之间的关系。为评估冷漠与功能状态的关联,进行了两项多元回归分析,每项分析中分别以不同的功能状态测量指标(成人功能适应性行为[AFAB]量表或总功能能力[TFC]量表)作为因变量。

结果

冷漠的统计学显著相关因素包括神经疾病生活质量(Neuro - QoL)对社会角色和活动的满意度以及Neuro - QoL积极情感和幸福感评分(N = 70例患者)。单变量相关性分析还显示FrSBe - 冷漠评分与两种功能状态测量指标均存在统计学显著关联。在多元回归分析中,冷漠对AFAB量表(N = 49例患者)和TFC量表(N = 56例患者)所测量的功能状态变异性均有显著贡献。

结论

这些结果强调了将冷漠作为改善HD患者功能状态、社会满意度和幸福感的目标来加以应对的必要性,即使是对于早期HD患者。

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本文引用的文献

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Frequency and Pathophysiology of Apathy in Huntington Disease: A Systematic Review and Meta-Analysis.亨廷顿舞蹈病中淡漠的频率及病理生理学:一项系统评价与荟萃分析
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Standardizing the CAP Score in Huntington's Disease by Predicting Age-at-Onset.通过预测发病年龄使亨廷顿病的 CAP 评分标准化。
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Anosognosia and Memory Encoding in Huntington Disease.亨廷顿病中的认知失认症和记忆编码。
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Apathy and Huntington's Disease: A Literature Review Based on PRISMA.冷漠与亨廷顿舞蹈症:一项基于系统评价与Meta分析的首选报告项目(PRISMA)的文献综述
J Neuropsychiatry Clin Neurosci. 2022 Spring;34(2):100-112. doi: 10.1176/appi.neuropsych.21060154. Epub 2021 Dec 28.
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Timing and Impact of Psychiatric, Cognitive, and Motor Abnormalities in Huntington Disease.亨廷顿舞蹈症患者精神、认知及运动异常的发生时间与影响
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Measuring Functional Status in Huntington's Disease.测量亨廷顿病患者的功能状态。
Mov Disord. 2021 Mar;36(3):757-761. doi: 10.1002/mds.28363. Epub 2020 Oct 27.
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The Impact of Anosognosia on Clinical and Patient-Reported Assessments of Psychiatric Symptoms in Huntington's Disease.认知障碍对亨廷顿病患者精神症状的临床和患者报告评估的影响。
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A Systematic Review of Neuropsychiatric Symptoms and Functional Capacity in Huntington's Disease.亨廷顿舞蹈病神经精神症状与功能能力的系统评价
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