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亨廷顿研究小组的神经心理学工作组关于亨廷顿病患者临床神经心理学评估的最佳实践建议的立场。

Huntington study group's neuropsychology working group position on best practice recommendations for the clinical neuropsychological evaluation of patients with Huntington disease.

机构信息

Department of Neurology, Vanderbilt University School of Medicine, Nashville, TN, USA.

Department of Neurology, University of Virginia School of Medicine, Charlottesville, VA, USA.

出版信息

Clin Neuropsychol. 2024 May;38(4):984-1006. doi: 10.1080/13854046.2023.2267789. Epub 2023 Oct 18.

Abstract

Neuropsychological evaluation is critical to detection and management of cognitive and neuropsychiatric changes associated with Huntington disease (HD). Accurate assessment of non-motor complications of HD is critical given the prominent impact on functional disability, frequently commensurate with or exceeding that of motor symptoms. The increasing emphasis on developing disease-modifying therapies targeting cognitive decline in HD requires consensus on clinical neuropsychological assessment methods. The Neuropsychology Working Group (NPWG) of the Huntington Study Group (HSG) sought to provide evidence and consensus-based, practical guidelines for the evaluation of cognitive and neuropsychiatric symptoms associated with HD. : The NPWG recruited a multi-disciplinary group of neuropsychologists, neurologists, and psychiatrists to inform best practices in assessing, diagnosing, and treating the non-motor symptoms in HD. A review was circulated among the NPWG, and in an iterative process informed by reviewed literature, best practices in neuropsychological evaluation of patients with HD were identified. : A brief review of the available literature and rational for a clinical consensus battery is offered. : Clinical neuropsychologists are uniquely positioned to both detect and characterize the non-motor symptoms in HD, and further, provide neurologists and allied health professions with clinically meaningful information that impacts functional outcomes and quality of life. The NPWG provides guidance on best practices to clinical neuropsychologists in this statement. A companion paper operationalizing clinical application of previous research-based non-motor diagnostic criteria for HD is forthcoming, which also advises on non-motor symptom screening methods for the non-neuropsychologist working with HD.

摘要

神经心理学评估对于检测和管理与亨廷顿病(HD)相关的认知和神经精神变化至关重要。考虑到非运动并发症对功能障碍的显著影响,通常与运动症状相当或超过运动症状,因此准确评估 HD 的非运动并发症至关重要。由于越来越强调开发针对 HD 认知能力下降的疾病修饰疗法,因此需要就临床神经心理学评估方法达成共识。亨廷顿研究小组(HSG)的神经心理学工作组(NPWG)旨在为评估与 HD 相关的认知和神经精神症状提供基于证据和共识的实用指南。NPWG 招募了一组多学科的神经心理学家、神经病学家和精神科医生,以告知评估、诊断和治疗 HD 非运动症状的最佳实践。NPWG 对该审查进行了传阅,并在对已审查文献进行回顾的基础上,确定了对 HD 患者进行神经心理学评估的最佳实践。提供了对现有文献的简要回顾,以及对临床共识电池的基本原理的介绍。临床神经心理学家具有独特的优势,可以发现和描述 HD 中的非运动症状,并且可以为神经病学家和相关健康专业人员提供对功能结果和生活质量有影响的具有临床意义的信息。NPWG 在该声明中为临床神经心理学家提供了最佳实践指南。即将发布一份操作性文件,对以前基于研究的 HD 非运动性诊断标准进行临床应用,并就与 HD 一起工作的非神经心理学家的非运动性症状筛查方法提供建议。

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