迟发型和成人型1型强直性肌营养不良表型中的下肢肌肉力量损害
Lower limb muscle strength impairment in late-onset and adult myotonic dystrophy type 1 phenotypes.
作者信息
Petitclerc Émilie, Hébert Luc J, Mathieu Jean, Desrosiers Johanne, Gagnon Cynthia
机构信息
Faculty of Medicine and Health Sciences, Université de Sherbrooke, Sherbrooke, Québec, Canada.
Faculty of Medicine, Rehabilitation (Physiotherapy) and Radiology Department, Université Laval, Québec City, Québec, Canada.
出版信息
Muscle Nerve. 2017 Jul;56(1):57-63. doi: 10.1002/mus.25451. Epub 2016 Nov 25.
INTRODUCTION
Lower limb strength has never been characterized separately for late-onset and adult myotonic dystrophy type 1 (DM1) phenotypes.
METHODS
The purpose of this study was to: (1) describe and compare lower limb strength between the 2 DM1 phenotypes; and (2) compare the impairment profiles obtained from 2 assessment methods [manual (MMT) and quantitative (QMT) muscle testing] among 107 patients.
RESULTS
Both MMT and QMT showed more pronounced weakness in the adult phenotype. In the late-onset phenotype, although MMT showed normal strength, QMT revealed a loss of 11.7%-20.4%. Participants with grade 1 or 2 on the Muscle Impairment Rating Scale had weakness detected using QMT, which suggests earlier muscle impairment than MMT alone would suggest.
CONCLUSIONS
To avoid muscle wasting, physical activity recommendations should be made for the late-onset phenotype and in the early stages of the disease for the adult phenotype. MMT is not recommended for use in clinical trials. Muscle Nerve 56: 57-63, 2017.
引言
迟发性和成人型1型强直性肌营养不良（DM1）表型的下肢力量从未被单独描述过。
方法
本研究的目的是：（1）描述和比较两种DM1表型之间的下肢力量；（2）比较107例患者通过两种评估方法[手法（MMT）和定量（QMT）肌肉测试]获得的损伤情况。
结果
MMT和QMT均显示成人表型的肌无力更为明显。在迟发性表型中，尽管MMT显示力量正常，但QMT显示力量损失了11.7%-20.4%。肌肉损伤评定量表评分为1级或2级的参与者通过QMT检测到肌无力，这表明肌肉损伤比单独使用MMT所提示的更早。
结论
为避免肌肉萎缩，应针对迟发性表型以及疾病早期的成人表型提出体育活动建议。不建议在临床试验中使用MMT。《肌肉与神经》56: 57-63, 2017年。
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