Isokinetic strength and degeneration of lower extremity muscles in patients with myotonic dystrophy; an MRI study

Abstract

Our aim was to determine isokinetic strength and degeneration of lower extremity muscles in patients with Myotonic Dystrophy (DM1). In 19 patients with DM1 and 19 matched controls, strength measured by isokinetic dynamometry was expressed as percentage of expected strength (ePct), adjusted for age, height, weight and gender. MRI of the hip, thigh and calf muscles were obtained. Fat fraction (FF), mean contractile cross-sectional area (cCSA) and specific strength (Nm/cm²) were calculated. Patients' ankle plantar flexors, knee flexors and extensors had higher FF (Δ: 0.08 - 0.42) and lower cCSA (Δ: 3.2 -17.1 cm²) compared to controls (p ≤ 0.005). EPct (Δ: 19.5 - 41.6%) and specific strength (Δ: 0.27 - 0.96 Nm/cm²) were lower in the majority of patients muscle groups (p˂0.05). Close correlations were found for patients when relating ePct to; FF for plantar flexors (R²=0.742, p<0.001) and knee extensors (R²=0.732, p<0.001), cCSA for plantar flexors (R²=0.696, p<0.001) and knee extensors (R²=0.633, p<0.001), and specific strength for dorsal flexors (ρ=0.855, p = 0.008). In conclusion, patients had weaker lower extremity muscles with higher FF, lower cCSA and specific strength compared to controls. Muscle degeneration determined by quantitative MRI strongly correlated to strength supporting its feasibility to quantify muscle dysfunction in DM1.

Keywords: Isokinetic muscle strength; Muscular dystrophy in lower extremities; Myotonic dystrophy; Quantitative magnetic resonance imaging; Specific strength.