亨廷顿舞蹈症的适应性功能评估。

Assessment of adaptive functioning in Huntington's disease.

作者信息

Bylsma F W, Rothlind J, Hall M R, Folstein S E, Brandt J

机构信息

Department of Psychiatry and Behavioral Sciences, Johns Hopkins University School of Medicine, Baltimore, Maryland 21287-7218.

出版信息

Mov Disord. 1993 Apr;8(2):183-90. doi: 10.1002/mds.870080212.

DOI:10.1002/mds.870080212

PMID:8474487

Abstract

The Huntington's Disease Activities of Daily Living (HD-ADL) scale is a 17-item informant-completed instrument for rating adaptive functioning in Huntington's disease (HD) patients. To assess the reliability and clinical correlates of the HD-ADL, it was administered along with the Mini-Mental State Exam (MMSE) and Quantified Neurological Exam (QNE) to 93 HD patients. The HD-ADL has high internal consistency (coefficient alpha = 0.91). Principal components factor analysis of the HD-ADL revealed four factors (General Functioning, Domestic Activities, Home Upkeep, and Family Relationships) accounting for 72% of the total variance. MMSE score, motor impairment score, and duration of disease were the best predictors of HD-ADL score. Similar results were obtained in an independent replication sample of patients. The HD-ADL scores correlate with Shoulson and Fahn's total functional capacity (TFC) index (r = -0.89). The HD-ADL scale is a reliable and valid instrument for assessing adaptive function in HD patients.

摘要

亨廷顿舞蹈病日常生活活动（HD - ADL）量表是一种由信息提供者完成的包含17个条目的工具，用于评定亨廷顿舞蹈病（HD）患者的适应性功能。为评估HD - ADL的信度及临床相关性，研究者将其与简易精神状态检查表（MMSE）和量化神经学检查（QNE）一起施测于93例HD患者。HD - ADL具有较高的内部一致性（α系数 = 0.91）。对HD - ADL进行主成分因子分析，发现四个因子（一般功能、家务活动、家庭维护和家庭关系），它们解释了总方差的72%。MMSE得分、运动障碍得分和病程是HD - ADL得分的最佳预测指标。在一个独立的患者重复样本中也得到了类似结果。HD - ADL得分与舒尔森和法恩的总功能能力（TFC）指数相关（r = -0.89）。HD - ADL量表是评估HD患者适应性功能的一种可靠且有效的工具。