Kierkegaard Marie, Petitclerc Emilie, Hébert Luc J, Gagnon Cynthia
Department of Neurobiology, Care Sciences and Society, Karolinska Institutet, Functional Area Occupational Therapy & Physiotherapy, Allied Health Professionals Function, Karolinska University Hospital, Stockholm SE-171 76, Sweden; Centre de recherche Charles-Le-Moyne, Faculty of Medicine and Health Sciences, Université de Sherbrooke, 2500, Boul. de l'Université, Sherbrooke, Québec J1K 2R1, Canada; Groupe de Recherche Interdisciplinaire sur les Maladies Neuromusculaires (GRIMN), CIUSSS du Saguenay-Lac-St-Jean, Site Jonquière, 2230 rue de l'Hôpital, Saguenay, Québec G7X 7X2, Canada.
Centre de recherche Charles-Le-Moyne, Faculty of Medicine and Health Sciences, Université de Sherbrooke, 2500, Boul. de l'Université, Sherbrooke, Québec J1K 2R1, Canada; Groupe de Recherche Interdisciplinaire sur les Maladies Neuromusculaires (GRIMN), CIUSSS du Saguenay-Lac-St-Jean, Site Jonquière, 2230 rue de l'Hôpital, Saguenay, Québec G7X 7X2, Canada.
Neuromuscul Disord. 2017 Feb;27(2):153-158. doi: 10.1016/j.nmd.2016.11.018. Epub 2016 Dec 5.
Performance-based assessments of physical function are essential in people with myotonic dystrophy type 1 (DM1) to monitor disease progression and evaluate interventions. Commonly used are the six-minute walk test, the 10 m-walk test, the timed up-and-go test, the timed-stands test, grip strength tests and the nine-hole peg test. The number of trials needed on a same-day test occasion and whether the first, best or average of trials should be reported as result is unknown. Thus, the aim was to describe and explore differences between trials in these measures of walking, mobility and fine hand use in 70 adults with DM1. Three trials were performed for each test except for the six-minute walk test where two trials were allowed. There were statistical significant differences over trials in all tests except for the 10 m-walk test and grip strength tests. Pair-wise comparisons showed that the second and third trials were in general better than the first, although effect sizes were small. At which trial the individuals performed their best differed between individuals and tests. People with severe muscular impairment had difficulties to perform repeated trials. Intraclass correlation coefficients were all high in analyses exploring how to report results. The conclusion and clinical implication is that, for a same-day test occasion, one trial is sufficient for the 10 m-walk test and grip strength tests, and that repeated trials should be allowed in the timed up-and-go test, timed-stands test and nine-hole peg tests. We recommend that two trials are performed for these latter tests as such a protocol could accommodate people with various levels of impairments and physical limitations.
基于表现的身体功能评估对于1型强直性肌营养不良（DM1）患者监测疾病进展和评估干预措施至关重要。常用的测试包括六分钟步行测试、10米步行测试、定时起立行走测试、定时站立测试、握力测试和九孔插板测试。同一天测试时所需的试验次数以及应将首次、最佳还是平均试验结果作为报告结果尚不清楚。因此，本研究旨在描述和探索70名成年DM1患者在这些步行、活动能力和精细手部使用测量中的试验差异。除六分钟步行测试允许进行两次试验外，每个测试均进行三次试验。除10米步行测试和握力测试外，所有测试的试验结果均存在统计学显著差异。两两比较表明，第二次和第三次试验总体上优于第一次，尽管效应量较小。个体在哪个试验中表现最佳因个体和测试而异。严重肌肉损伤的患者进行重复试验有困难。在探索如何报告结果的分析中，组内相关系数均较高。结论及临床意义在于，在同一天测试时，10米步行测试和握力测试进行一次试验就足够了，而定时起立行走测试、定时站立测试和九孔插板测试应允许进行重复试验。我们建议对后几种测试进行两次试验，因为这样的方案可以适应不同损伤程度和身体限制的患者。
