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Abstract #0497

Behavioural intervention in myotonic dystrophy type 1 assessed by  longitudinal MRI of skeletal muscles

Linda Heskamp1, Kees Okkersen2, Marlies van Nimwegen2, Marieke Ploegmakers1, Guillaume Bassez3, Jean-Francois Deux4, Baziel van Engelen2, and Arend Heerschap1

1Radiology and Nuclear Medicine, Radboud university medical center, Nijmegen, Netherlands, 2Neurology, Radboud university medical center, Nijmegen, Netherlands, 3Neuromuscular Reference Center, Henri Mondor university Hospital, Paris, France, 4Radiology, Henri Mondor university Hospital, Paris, France

A behavioural intervention, directed to increase physical activity, had a beneficial effect on lower extremity muscle function in patients with myotonic dystrophy type 1 (DM1). Here, we evaluated potential causes of the effect of this intervention with quantitative MRI in the 20 lower extremity muscles of 27 DM1 patients. We showed that it leads to a ~4% average increase in muscle mass, especially in healthy appearing muscle. Fat infiltration was not decelerated. Therefore, we conclude that some muscles in DM1 patients are trainable, preferably early on in the course of the disease.

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