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Abstract #0095

Longitudinal evaluation of the muscles of patients with Myotonic Dystrophy type 1 treated by metformin, using quantitative 1H and 23Na MRI

Benjamin Marty1, Pierre-Yves Baudin1, Aurélie Canal2, Jean-Yves Hogrel2, Melinda Gyenge3, Nuria Jebrouni3, Teresa Gerhalter4, Armin M Nagel4, Harmen Reyngoudt1, and Guillaume Bassez3
1NMR Laboratory, Neuromuscular Investigation Center, Institute of Myology, Paris, France, 2Neuromuscular Physiology and Evaluation Laboratory, Neuromuscular Investigation Center, Institute of Myology, Paris, France, 3Institute of Myology, Paris, France, 4Institute of Radiology, University Hospital Erlangen, FAU, Erlangen, Germany

Synopsis

Keywords: Muscle, Tissue CharacterizationMyotonic dystrophy type 1 (DM1) is a neuromuscular disorder resulting in progressive muscle wasting and dysfunction. We aimed at determining the relationship between several 1H and 23Na MRI indices of disease severity and disease activity in the muscles of DM1 patients, and evaluating their response to a 12-month metformin treatment. We showed that these indices could differentiate DM1 patients from healthy controls. Variables related to disease severity correlated with functional tests and indices related to disease activity were increased and mutually correlated. Over the 12-months treatment interval, MRI variables were more sensitive than functional outcomes to detect disease progression.

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