Performance of Passive Muscle Stiffness in Diagnosis and Assessment of Disease Progression in Duchenne Muscular Dystrophy

The aim of this study was to evaluate the performance of passive muscle stiffness in diagnosing and assessing disease progression in Duchenne muscular dystrophy (DMD). Boys with DMD and age-matched controls were recruited. Shear wave elastography (SWE) videos were collected by performing dynamic stretching of the gastrocnemius medius (GM). At ankle angles from plantar flexion (PF) 30° to dorsiflexion (DF) 20°, the shear modulus of the GM was measured for each 10° of ankle movement. Shear modulus at each ankle angle was compared between the DMD and control group. Correlation between passive muscle stiffness and motor function grading was also analyzed. A total of 26 patients with DMD and 20 healthy boys were enrolled. At multiple stretch levels, passive muscle stiffness of the GM was significantly higher in patients with DMD than in those in the control group (all p values0.05). The shear modulus of GM at an ankle angle of DF 10° had the largest area under the receiver operating characteristic curve in differentiating DMD patients from normal subjects (AUC = 0.902, 95% confidence interval: 0.814-0.990). Motor function grading was a significant determinant of passive muscle stiffness at an ankle angle of DF 10° (B = 21.409, t = 3.372, p = 0.003). Passive muscle stiffness may potentially serve as a useful non-invasive tool to monitor disease progression in DMD patients.