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亨廷顿舞蹈症中冷漠、健康相关生活质量与功能之间的关系。

Relationships Among Apathy, Health-Related Quality of Life, and Function in Huntington's Disease.

作者信息

Fritz Nora E, Boileau Nicholas R, Stout Julie C, Ready Rebecca, Perlmutter Joel S, Paulsen Jane S, Quaid Kimberly, Barton Stacey, McCormack Michael K, Perlman Susan L, Carlozzi Noelle E

机构信息

From the Departments of Health Care Sciences and Neurology, Wayne State University, Detroit (NEF); the Department of Physical Medicine and Rehabilitation, University of Michigan, Ann Arbor (NRB, NEC); the Institute of Cognitive and Clinical Neurosciences, School of Psychological Sciences, Monash University, Clayton, Victoria, Australia (JCS); the Department of Psychological and Brain Sciences, University of Massachusetts Amherst, Amherst (RR); the Departments of Neurology, Radiology, Neuroscience, Physical Therapy, and Occupational Therapy, Washington University, St. Louis (JSP, SB); the Department of Neurology, Department of Psychiatry, and Department of Psychological and Brain Sciences, University of Iowa, Iowa City (JSP); the Department of Medical and Molecular Genetics, Indiana University, Indianapolis (KQ); the Department of Psychiatry, Rutgers University Robert Wood Johnson Medical School, Piscataway, NJ (MKM); Department of Pathology, Rowan University, School of Medicine, Stratford, NJ (MKM); and the Department of Neurology, University of California Los Angeles, Los Angeles (SLP).

出版信息

J Neuropsychiatry Clin Neurosci. 2018 Summer;30(3):194-201. doi: 10.1176/appi.neuropsych.17080173. Epub 2018 Mar 21.

Abstract

Up to 90% of individuals with Huntington's disease (HD)-a progressive, inherited neurodegenerative disorder-experience apathy. Apathy is particularly debilitating because it is marked by a reduction in goal-directed behaviors, including self-care, social interactions, and mobility. The objective of this study was to examine relationships between variables of apathy, functional status, physical function, cognitive function, behavioral status/emotional function, and health-related quality of life. Clinician-rated measures of physical, cognitive, and behavioral function, including one clinician-rated item on apathy, and self-reported measures of physical function, health-related quality of life, and emotional, cognitive, and social function were collected in a single session from 487 persons with the HD mutation (prodromal, N=193; early-stage manifest, N=186; late-stage manifest, N=108). Multiple linear regression models were used to examine which outcomes best predicted clinician-rated apathy after controlling for disease stage. Greater apathy related to less independence, increased motor impairment, and more clinician-rated behavioral problems (i.e., anger, irritability, depression). Similarly, poorer self-reported health-related quality of life; greater chorea; greater upper- and lower-extremity dysfunction; greater speech and swallowing dysfunction; worse anxiety, depression, and behavioral dyscontrol; worse cognitive function; and less satisfaction with social roles related to greater apathy. In conclusion, apathy related to physical, cognitive, and behavioral dysfunction across disease stages. Future work should explore whether clinical interventions targeting different functional domains may have the potential to reduce apathy in this patient population.

摘要

高达90%的亨廷顿舞蹈症(HD)患者——一种进行性的、遗传性神经退行性疾病——会出现冷漠症状。冷漠尤其使人衰弱,因为它的特点是目标导向行为减少,包括自我护理、社交互动和活动能力。本研究的目的是检验冷漠变量、功能状态、身体功能、认知功能、行为状态/情感功能以及健康相关生活质量之间的关系。在一次就诊过程中,收集了487名携带HD突变者(前驱期,N = 193;早期显性期,N = 186;晚期显性期,N = 108)的临床医生评定的身体、认知和行为功能指标,包括一项关于冷漠的临床医生评定项目,以及自我报告的身体功能、健康相关生活质量和情感、认知及社会功能指标。使用多元线性回归模型来检验在控制疾病阶段后,哪些结果最能预测临床医生评定的冷漠程度。更高程度的冷漠与更低的独立性、增加的运动障碍以及更多临床医生评定的行为问题(即愤怒、易怒、抑郁)相关。同样,自我报告的健康相关生活质量较差;舞蹈症更严重;上肢和下肢功能障碍更严重;言语和吞咽功能障碍更严重;焦虑、抑郁和行为失控更严重;认知功能更差;以及对社会角色的满意度更低均与更高程度的冷漠相关。总之,冷漠在疾病各阶段均与身体、认知和行为功能障碍相关。未来的研究应探索针对不同功能领域的临床干预措施是否有可能降低该患者群体的冷漠程度。

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