The relationship between regional body composition and quantitative strength in facioscapulohumeral muscular dystrophy (FSHD)

This study determines in facioscapulohumeral muscular dystrophy (FSHD) and able-bodied controls (1) the regional body composition and (2) the correlation between regional lean tissue mass and the corresponding regional strength. This is a cross-sectional, criterion standard, case-control study at a university based neuromuscular disease clinic. A dual-energy X-ray absorptiometry (DEXA) scanner was used to obtain regional body composition measurements in 14 FSHD and anthropometrically matched control pairs. A dynamometer determined peak isometric strength for the elbow and knee. Compared to controls, FSHD subjects showed increased regional fat tissue mass (p < 0.001-0.017), decreased regional lean tissue mass (p < 0.001-0.010), and decreased strength (p < 0.001-0.020). There was a correlation between quantitative strength and lean tissue mass for both FSHD and controls (r = 0.791-0.906: p < 0.001). FSHD subjects have higher regional fat tissue mass and lower regional lean tissue mass despite similar BMI and anthropometrics. Regional lean tissue mass correlates with strength. (c) 2008 Elsevier B.V. All rights reserved.