Winder Jessica Y, Roos Raymund A C, Burgunder Jean-Marc, Marinus Johan, Reilmann Ralf
Department of Neurology Leiden University Medical Center Leiden The Netherlands.
Department of Neurology University of Bern Bern Switzerland.
Mov Disord Clin Pract. 2018 Apr 15;5(3):290-295. doi: 10.1002/mdc3.12618. eCollection 2018 May-Jun.
The clinical assessment of motor symptoms in Huntington's disease is usually performed with the Unified Huntington's Disease Rating Scale-Total Motor Score (UHDRS-TMS). A high interrater reliability is desirable to monitor symptom progression. Therefore, a teaching video and a system for annual online certification has been developed and implemented.
The aim of this study is to investigate the interrater reliability of the UHDRS-TMS and of its subitems, and to examine the performance of raters in consecutive years.
Data from the online UHDRS-TMS certification were used. The interrater reliability was assessed for all first-time participants (n = 944) between 2009 and 2016. Intraclass correlation coefficients (ICC) were calculated for each year separately and the mean was taken as the total ICC.
The UHDRS-TMS (ICC = 0.847), tandem walking (0.824), pronate/supinate hands left (0.713), and retropulsion pull test (0.706) showed good interrater reliability. Poor interrater reliability was found for maximal dystonia of the left and right upper extremity (0.187 and 0.322, respectively), maximal dystonia of the left and right lower extremity (0.200 and 0.256, respectively), and maximal dystonia of the trunk (0.389), tongue protrusion (0.266), and rigidity arms left (0.390). Raters performed significantly worse on follow-up certification compared to their first certification.
Our results suggest that the rating of dystonia (absent, slight, mild, moderate, or marked) is subjective and difficult to interpret, especially on video. Therefore, changing the dystonia items of the UHDRS-TMS should be explored. We also recommend that raters should watch the UHDRS-TMS teaching video before each certification.
亨廷顿舞蹈症运动症状的临床评估通常采用统一亨廷顿舞蹈症评定量表 - 总运动评分（UHDRS - TMS）。为监测症状进展，需要较高的评分者间信度。因此，已开发并实施了教学视频和年度在线认证系统。
本研究旨在调查UHDRS - TMS及其子项目的评分者间信度，并检验评分者连续多年的表现。
使用在线UHDRS - TMS认证的数据。对2009年至2016年间所有首次参与者（n = 944）的评分者间信度进行评估。分别计算每年的组内相关系数（ICC），并取平均值作为总ICC。
UHDRS - TMS（ICC = 0.847）、串联行走（0.824）、左手旋前/旋后（0.713）和后推拉力试验（0.706）显示出良好的评分者间信度。左右上肢最大肌张力障碍（分别为0.187和0.322）、左右下肢最大肌张力障碍（分别为0.200和0.256）、躯干最大肌张力障碍（0.389）、伸舌（0.266）和左臂强直（0.390）的评分者间信度较差。与首次认证相比，评分者在后续认证中的表现明显更差。
我们的结果表明，肌张力障碍（无、轻度、中度、重度或显著）的评分具有主观性且难以解读，尤其是在视频上。因此，应探索改变UHDRS - TMS中的肌张力障碍项目。我们还建议评分者在每次认证前观看UHDRS - TMS教学视频。
