Stahlhut Michelle, Downs Jenny, Leonard Helen, Bisgaard Anne-Marie, Nordmark Eva
a Department of Health Sciences, Faculty of Medicine , Lund University , Lund, Sweden.
b Department of Clinical Genetics, Center for Rett Syndrome, Kennedy Center , Rigshospitalet , Glostrup , Denmark.
Disabil Rehabil. 2017 Sep;39(19):1926-1931. doi: 10.1080/09638288.2016.1212280. Epub 2016 Aug 24.
The repertoire of measures of walking in Rett syndrome is limited. This study aimed to determine measurement properties of a modified two-minute walk test (2MWT) and a modified Rett syndrome-specific functional mobility scale (FMS-RS) in Rett syndrome.
Forty-two girls and women with Rett syndrome (median 18.4 years, range 2.4-60.9 years) were assessed for clinical severity, gross motor skills, and mobility. To measure walking capacity, 27 of this group completed a 2MWT twice on two different assessment days. To assess walking performance, the FMS-RS was administered to the total sample of parents (n = 42) on two occasions approximately one week apart.
There were negative correlations between clinical severity and 2MWT (r = -0.48) and FMS-RS (r = -0.60-0.66). There were positive correlations between gross motor skills and mobility and 2MWT (r = 0.51, 0.43) and FMS-RS (r = 0.71-0.93, 0.74-0.94), respectively. Test-retest reliability for the 2MWT was good with high intraday and interday correlations (ICC = 0.86-0.98). For the 2MWT, the standard error of measurement was 13.8 m and we would be 95% confident that changes greater than 38 m would be greater than within subject error. There was good test-retest reliability for all three distances on the FMS-RS (ICC = 0.94-0.99).
Walking capacity as measured by the 2MWT showed expected but limited relationships with measures of different constructs, providing some support for concurrent validity. Walking performance as measured with the FMS-RS was more strongly consistent with other clinical measures supporting its concurrent validity. Test-retest reliability was good for both the FMS-RS and the 2MWT. Therefore, these measures have the potential to be used in clinical practice and research. Implications for Rehabilitation Walking is one of the commonest daily physical activities in ambulant girls and women with RTT. Comprehensive knowledge about the walking abilities in this population is limited. Evidence of validity and test-retest reliability have been demonstrated for the modified two-minute walk test (2MWT) and the Rett syndrome-specific functional mobility scale (FMS-RS). The 2MWT and FMS-RS offer detailed information of the capacity and performance of walking, respectively, in girls and women with RTT.
雷特综合征中步行测量方法的种类有限。本研究旨在确定改良的两分钟步行测试（2MWT）和改良的雷特综合征特异性功能移动量表（FMS-RS）在雷特综合征中的测量特性。
对42名患有雷特综合征的女孩和女性（中位年龄18.4岁，范围2.4 - 60.9岁）进行临床严重程度、粗大运动技能和移动能力评估。为测量步行能力，该组中的27人在两个不同的评估日各完成两次2MWT。为评估步行表现，在大约相隔一周的两个时间点，对所有42名家长进行FMS-RS评估。
临床严重程度与2MWT（r = -0.48）和FMS-RS（r = -0.60 - 0.66）之间存在负相关。粗大运动技能和移动能力与2MWT（r = 0.51，0.43）和FMS-RS（r = 0.71 - 0.93，0.74 - 0.94）之间分别存在正相关。2MWT的重测信度良好，日内和日间相关性较高（ICC = 0.86 - 0.98）。对于2MWT，测量标准误为13.8米，我们有95%的把握认为变化大于38米将大于个体内误差。FMS-RS上所有三个距离的重测信度都很好（ICC = 0.94 - 0.99）。
2MWT测量的步行能力与不同结构的测量指标之间显示出预期但有限的关系，为同时效度提供了一些支持。FMS-RS测量的步行表现与其他临床指标更强烈地一致，支持其同时效度。FMS-RS和2MWT的重测信度都很好。因此，这些测量方法有潜力用于临床实践和研究。康复启示步行是能够行走的雷特综合征女孩和女性最常见的日常身体活动之一。关于该人群步行能力的全面知识有限。改良的两分钟步行测试（2MWT）和雷特综合征特异性功能移动量表（FMS-RS）已证明了效度和重测信度的证据。2MWT和FMS-RS分别提供了雷特综合征女孩和女性步行能力和表现的详细信息。
