The variable course of FSHD within families and its typical asymmetric weakness had led to the hypothesis that daily exertion and overexertion of muscles might increase the rate of disease progression. However, previous small and uncontrolled studies suggested a positive effect of strength training and did not point towards extra susceptibility for muscle overstrain. In animals and healthy volunteers 2-adrenergic agonists, including albuterol, are known to increase muscle strength and muscle mass. Their anabolic effect is potentially augmented when these agents are administered in combination with resistance exercise. A pilot study and a subsequent randomized controlled trial with albuterol indicated an (temporary) anabolic effect in FSHD patients as well.

In the presented intervention study 65 adult FSHD patients were randomly assigned to one of the following four treatment groups: training plus albuterol, training plus placebo, non-training plus albuterol, or non-training plus placebo. The first intervention was training or non-training, starting just after the baseline visit until after the final visit at 52 weeks. The moderate-intensity strength training program included mainly dynamic exercises of elbow flexors and ankle dorsiflexors. After 26 weeks albuterol was added in a double-blind, placebo-controlled design. Treatment was then continued for another 26 weeks. The primary outcome measure was the maximum voluntary isometric strength (MVIC) at 52 weeks. Secondary outcomes comprised dynamic strength and muscle volume. Both the training program and albuterol were well tolerated. Training of the elbow flexors did not result in a significant effect on MVIC, but dynamic strength improved significantly. Elbow flexor MVIC increased significantly in albuterol versus placebo treated patients. Ankle dorsiflexor strength decreased in all treatment groups. Eleven out of twelve non-trained muscles in the albuterol group showed a positive effect on MVIC compared to the placebo group (p < 0.05 in seven muscle groups). Muscle volume decreased in the placebo-treated, and increased in the albuterol-treated patients. No synergistic or antagonistic effects were observed between training and albuterol. We concluded that in FSHD strength training and albuterol appeared safe interventions with limited positive effect on muscle strength and volume. We also stated that our results were deemed insufficient for general prescription of exercise programs or albuterol in FSHD.