Abstract #1433

Natural Progression of Fatty Infiltration and Beneficial Effects of Non-Pharmaceutical Intervention in FSHD Detected by Quantitative MRI

Barbara Janssen¹, Nicoline Voet², Rob Arts¹, Christine Nabuurs¹, George Padberg³, Alexander Geurts², Baziel van Engelen³, Arend Heerschap¹

¹Radiology, Radboud University Nijmegen Medical Centre, Nijmegen, Gelderland, Netherlands; ²Rehabilitation, Radboud University Nijmegen Medical Centre, Nijmegen, Gelderland, Netherlands; ³Neurology, Radboud University Nijmegen Medical Centre, Nijmegen, Gelderland, Netherlands

To assess natural progression towards a diseased state in FSHD-patients, and effects of Cognitive Behavioral Therapy and Aerobic Exercise Training (AET) we used muscle fraction measured by 1H-MRI of the thigh muscles as non-invasive biomarkers. A decrease in muscle fraction, corresponding to an increase in fatty infiltration, could be noticed in controls after a four month period of usual care. This increase was largest in intermediate affected muscles. Interventions appeared to beneficial to patient since no significant increase in fat infiltration could be noticed after four months after intervention, heavily affected muscles even showed an increase in muscle fraction.