Beyond ambulation: Measuring physical activity in youth with Duchenne muscular dystrophy

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. (C) 2020 Elsevier B.V. All rights reserved.