Siesling S, Zwinderman A H, van Vugt J P, Kieburtz K, Roos R A
Department of Neurology, Leiden University Hospital, The Netherlands.
Mov Disord. 1997 Mar;12(2):229-34. doi: 10.1002/mds.870120214.
Objective assessment of clinical findings of patients with Huntington's disease (HD) is necessary for an evaluation of the individual progression of the disease and the effect of therapy, and it requires specific assessment scales. The Unified Huntington's Disease Rating Scale (UHDRS) is an overall scale to assess clinical performance and functional capacity. In the course of carrying out studies in HD, several items in the motor function section were found to be difficult to score, had high cognitive loading, and appeared to be redundant. The objective of the study was to shorten the motor section of the UHDRS to the smallest number of items, without loss of internal consistency, while still assessing the important clinical features of HD. Shortening the total motor score of the UHDRS was carried out on the UHDRS data set of the Leiden University Hospital using four methods. The first two methods employed principal-component analysis with Varimax rotation. Strongly interrelated test items were uncovered, resulting in a reduction of test items to a smaller set. The third method reduced items so that the internal consistency (Cronbach's alpha) was maximal. The fourth method omitted items subjectively. The results of the Dutch data set were validated on follow-up data and on a data set from the University of Rochester Medical Center, New York. This study determined that the number of items in the motor function section could be reduced from 31 to 15. The reduced set maintains the relationships between the motor and other sections of the UHDRS and still assesses the major clinical features of HD.
对亨廷顿舞蹈症（HD）患者的临床症状进行客观评估，对于评估疾病的个体进展及治疗效果是必要的，这需要特定的评估量表。统一亨廷顿舞蹈症评定量表（UHDRS）是用于评估临床表现和功能能力的综合量表。在对HD进行研究的过程中，发现运动功能部分的几个项目难以评分、认知负荷高且似乎存在冗余。本研究的目的是将UHDRS的运动部分缩短至最少项目数量，同时不损失内部一致性，且仍能评估HD的重要临床特征。使用四种方法对莱顿大学医院UHDRS数据集进行了UHDRS总运动评分的缩短。前两种方法采用主成分分析和方差最大化旋转。发现了高度相关的测试项目，从而将测试项目减少为更小的集合。第三种方法减少项目以使内部一致性（克朗巴哈系数）最大化。第四种方法主观地省略项目。荷兰数据集的结果在随访数据和纽约罗切斯特大学医学中心的数据集上进行了验证。本研究确定运动功能部分的项目数量可从31项减少至15项。精简后的量表保持了UHDRS运动部分与其他部分之间的关系，且仍能评估HD的主要临床特征。
