S.P.43 Physical activity decreases fatty infiltration in FSHD, a quantitative muscle MR study

Abstract

Abstract In facioscapulohumeral dystrophy (FSHD) muscle function is impaired and declines over time. Currently there is no effective treatment available to slow down this decline. The aim of this study was to employ the quantitative MR muscular fat fraction as a biomarker to follow the natural progression of the disease and to assess the effects of two treatment regimes: (1) Aerobic exercise training (AET) on a bicycle ergometer to improve or at least maintain functional capacity and (2) cognitive behavioral therapy (CBT) to stimulate an active lifestyle yet avoiding excessive physical strain. Both aim at improving quality of life and are based on a FSHD-specific model of health status (BMC Neurol 2010;10:56). Twenty-four genetically proven FSHD patients were included. Patients were randomly placed in the control group (n = 8), CBT group (n = 8) or AET group (n = 6) and were examined on a Siemens Trio 3T system. Measurements were performed on the thigh muscles of the patients before and four months after usual care or intervention period. Fat fraction was quantitatively determined with a method earlier described. The change in fat fraction over time was shown to be dependent on the initial fat fraction: the greatest changes occurred in the medium fatty infiltrated muscles compared to the normal muscles in the non-intervention FSHD controls. Unpaired two-tailed t-test revealed significantly smaller increases in fat fraction over time for the CBT and AET group compared to the FSHD control group. No differences were observed between the intervention groups. In FSHD patients the fat fraction quantitatively measured in the thigh muscles by MR increases significantly in a four month period. Remarkably, increasing physical activity by either exercise training or cognitive behavioral therapy slowed down fatty infiltration in the thigh muscles.