Abstract

In healthy subjects, regular exercise is beneficial for mental and physical health, and post-exercise protein supplementation has been shown to enhance the physiological responses to training. FSHD is a muscular dystrophy characterized by slow progression and asymmetry of muscle weakness, often starting in facial, shoulder, humeral and calf muscles. Moderate, and even high-intensity exercise, has been shown to be well tolerated in these patients. In this double-blinded, placebo-controlled study, we tested the effect of training and protein supplementation in FSHD. Since the study is still ongoing, the blinding cannot be broken, and we therefore here present the preliminary data on exercise effects. The full results will be available before the WMS meeting. Twenty-three patients with FSHD, and a disease severity-score of 1–12 (mean 5.8), were tested before and after 12 weeks (36 sessions of 30 min) of ergometer cycle training at home at an intensity of 70% of maximal oxygen uptake, and compared with a group of FSHD patients (FSH-score 5.3), who did not train. Maximal oxygen uptake and maximal load increased by 6% (14.5 SD; p In conclusion; regular aerobic training improves fitness in patients with FSHD. The effect of protein supplementation will be communicated at the meeting.

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