G.P.15

The aim of this study was to investigate the effect of aerobic exercise training (AET) and cognitive behavior therapy (CBT) on chronic fatigue in patients with facioscapulohumeral muscular dystrophy (FSHD). We performed a multi-center, assessor-blinded, randomized clinical trial (RCT). Fifty-seven patients with FSHD type 1 with severe chronic fatigue were randomly allocated to AET, CBT, or usual care (UC). Outcomes (including a 3 Tesla quantitative MR imaging of the upper leg muscles) were assessed at baseline, immediately following 16 weeks of intervention, and after a 12-week follow-up. A linear mixed model for repeated measurements was used to study the estimated group differences. Following treatment, both the AET and CBT intervention groups had significantly less fatigue relative to the UC group, with a difference of −9.1 for AET (95%CI: −12.4 to −5.8) and −13.3 for CBT (95%CI: −16.5 to −10.2). These beneficial effects lasted through the follow-up period, with a difference of −8.2 for AET (95%CI: −12.4 to −5.8) and −10.2 for CBT (95%CI −16.5 to −10.1). The patients who received CBT had an increase in registered and experienced physical activity, sleep quality, and social participation. The patients who received AET had an increase in registered physical activity only. The increase in registered physical activity in both groups and the improvement in social participation following CBT were still present at follow-up. MRI determined fat fractions showed decelerated progression of fatty infiltration, most prominently in the adductor magnus muscle. In this muscle fat percentage increased with 1.56% monthly (UC) with a difference of −1.21 for AET (95%CI: −1.99 to −0.34) and −1.73 for CBT (95%CI: −2.56 to −0.87). This is the first RCT showing that chronic fatigue can be ameliorated in patients with a muscular dystrophy. AET and/or CBT reduce fatigue and slow progression of fatty infiltration in patients with FSHD.