Department of Pediatrics, Vanderbilt University Medical Center, 8232 Doctors' Office Tower, 2200 Children's Way, Nashville, TN 37232, United States.
Division of Gastroenterology, Hepatology and Nutrition, Department of Medicine, Vanderbilt University Medical Center, Nashville, TN, United States.
Neuromuscul Disord. 2020 Apr;30(4):277-282. doi: 10.1016/j.nmd.2020.02.007. Epub 2020 Feb 20.
Patients with Duchenne muscular dystrophy (DMD) develop skeletal muscle weakness and cardiomyopathy. Validated skeletal muscle outcome measures are limited to ambulatory patients, but most DMD patients in cardiac trials are non-ambulatory. New objective functional assessments are needed. This study's objective was to assess the correlation and longitudinal change of two measures: quantitative muscle testing (QMT) and accelerometry. Patients with DMD were prospectively enrolled and underwent QMT and wore wrist and ankle accelerometers for seven days at baseline, 1-, and 2-years. QMT measures were indexed to age. Accelerometer recordings were total vector magnitudes and awake vector magnitude. Correlations were assessed using a Spearman correlation, and longitudinal change was evaluated using a paired t-test or a Wilcoxon signed rank test. Forty-eight participants were included. QMT and accelerometry measures had a moderate or strong correlation, particularly indexed arm QMT with total wrist vector magnitude (rho=0.85, p<0.001), total indexed QMT with total wrist vector magnitude (rho=0.8, p<0.001) and indexed leg QMT with total ankle vector magnitude (rho=0.69, p<0.001). QMT and accelerometry measures declined significantly over time. Accelerometry correlates with QMT and indexed QMT in boys with DMD. A combination of QMT and accelerometry may provide a complementary assessment of skeletal muscle function in non-ambulatory boys with DMD.
患有杜氏肌营养不良症(DMD)的患者会出现骨骼肌无力和心肌病。经过验证的骨骼肌结果测量方法仅限于可走动的患者,但大多数参与心脏试验的 DMD 患者都无法走动。因此需要新的客观功能评估方法。本研究的目的是评估两种测量方法(定量肌肉测试(QMT)和加速度计)的相关性和纵向变化。前瞻性招募了患有 DMD 的患者,并在基线、1 年和 2 年时进行了 QMT 和手腕及脚踝加速度计佩戴 7 天。QMT 测量结果以年龄为指标。加速度计记录的是总向量幅度和清醒时的向量幅度。使用 Spearman 相关分析评估相关性,使用配对 t 检验或 Wilcoxon 符号秩检验评估纵向变化。共纳入了 48 名参与者。QMT 和加速度计测量具有中度或高度相关性,尤其是索引臂 QMT 与总腕部向量幅度(rho=0.85,p<0.001)、总索引 QMT 与总腕部向量幅度(rho=0.8,p<0.001)以及索引腿 QMT 与总脚踝向量幅度(rho=0.69,p<0.001)相关性最强。QMT 和加速度计测量结果随时间显著下降。加速度计与 DMD 男孩的 QMT 和索引 QMT 相关。QMT 和加速度计的组合可能为无法走动的 DMD 男孩的骨骼肌功能提供互补评估。