Matsushima Masaaki, Yabe Ichiro, Takahashi Ikuko, Hirotani Makoto, Kano Takahiro, Horiuchi Kazuhiro, Houzen Hideki, Sasaki Hidenao
Department of Neurology, Faculty of Medicine and Graduate School of Medicine, Hokkaido University, Kita-15, Nishi-7, Kita-ku, Sapporo, 060-8638 Japan.
Department of Neurology, Obihiro Kosei Hospital, Nishi-6, Minami-8, Obihiro, 080-0016 Japan.
Cerebellum Ataxias. 2017 Jul 3;4:11. doi: 10.1186/s40673-017-0067-5. eCollection 2017.
Multiple system atrophy (MSA) is a rare progressive neurodegenerative disorder for which brief yet sensitive scale is required in order for use in clinical trials and general screening. We previously compared several scales for the assessment of MSA symptoms and devised an eight-item pilot scale with large standardized response mean [handwriting, finger taps, transfers, standing with feet together, turning trunk, turning 360°, gait, body sway]. The aim of the present study is to investigate the validity and reliability of a simple pilot scale for assessment of multiple system atrophy symptoms.
Thirty-two patients with MSA (15 male/17 female; 20 cerebellar subtype [MSA-C]/12 parkinsonian subtype [MSA-P]) were prospectively registered between January 1, 2014 and February 28, 2015. Patients were evaluated by two independent raters using the Unified MSA Rating Scale (UMSARS), Scale for Assessment and Rating of Ataxia (SARA), and the pilot scale. Correlations between UMSARS, SARA, pilot scale scores, intraclass correlation coefficients (ICCs), and Cronbach's alpha coefficients were calculated.
Pilot scale scores significantly correlated with scores for UMSARS Parts I, II, and IV as well as with SARA scores. Intra-rater and inter-rater ICCs and Cronbach's alpha coefficients remained high (> 0.94) for all measures.
The results of the present study indicate the validity and reliability of the eight-item pilot scale, particularly for the assessment of symptoms in patients with early state multiple system atrophy.
多系统萎缩（MSA）是一种罕见的进行性神经退行性疾病，在临床试验和一般筛查中需要使用简短而敏感的量表。我们之前比较了几种用于评估MSA症状的量表，并设计了一个包含八个条目的初步量表，其标准化反应均值较大[书写、手指轻敲、转移、双脚并拢站立、躯干转动、360°转身、步态、身体摆动]。本研究的目的是调查一个简单的初步量表评估多系统萎缩症状的有效性和可靠性。
2014年1月1日至2015年2月28日期间前瞻性登记了32例MSA患者（15例男性/17例女性；20例小脑亚型[MSA-C]/12例帕金森亚型[MSA-P]）。由两名独立评估者使用统一MSA评定量表（UMSARS）、共济失调评估与评定量表（SARA）和初步量表对患者进行评估。计算UMSARS、SARA、初步量表得分之间的相关性、组内相关系数（ICC）和克朗巴哈α系数。
初步量表得分与UMSARS第一、二、四部分得分以及SARA得分显著相关。所有测量的评估者内和评估者间ICC以及克朗巴哈α系数均保持在较高水平（>0.94）。
本研究结果表明该包含八个条目的初步量表具有有效性和可靠性，尤其适用于评估早期多系统萎缩患者的症状。
