23Na MRI and myometry to compare eplerenone vs. glucocorticoid treatment in Duchenne dystrophy

Philip A. Glemser 1,2, Heike Jaeger 3, Armin M. Nagel 4,5, Andreas E. Ziegler 6, David Simons 1, Heinz-Peter Schlemmer 1, Frank Lehmann-Horn 3, Karin Jurkat-Rott 7 and Marc-André Weber 8

1 Department of Radiology, German Cancer Research Center (DKFZ), Heidelberg, Germany; 2 Institute of Forensic Medicine, University of Heidelberg, Heidelberg, Germany; 3 Division of Neurophysiology, Ulm University, Ulm, Germany; 4 Division of Medical Physics in Radiology, German Cancer Research Center (DKFZ), Heidelberg, Germany; 5 Institute of Radiology, University Hospital Erlangen, Erlangen, Germany; 6 Center for Child and Adolescent Medicine, University Hospital Heidelberg, Heidelberg, Germany; 7 Department of Neurosurgery, Ulm University, Ulm, Germany; 8 Diagnostic and Interventional Radiology, University Hospital Heidelberg, Heidelberg, German

In this pilot study we tested whether a low dose application of a mild diuretic substance such as eplerenone is beneficial in early stages of Duchenne muscular dystrophy using 23Na und 1H imaging, myometry, and clinical testing versus the glucocorticoid gold standard.

Two 7-years old patients with DMD were examined on a 3T MRI system. 1H MRI and 23Na density-adapted 3-dimensional radial MRI sequences were performed both before and 1, 3 and 6 months after therapy with eplerenone respectively cortisone. We quantified fatty infiltration on T1-weighted images using subcutaneous fat as reference and fat fraction with a two-point DIXON sequence. Muscle oedema was quantified on STIR images and DIXON water maps with background noise as reference. We quantified Na+ by a muscular tissue concentration sequence with a 51.3mM Na+ with 5% agarose reference tube. A Na+ IR-sequence was used for determination of mainly myoplasmic Na+. Correspondingly myometry of muscles and tendons were assessed. Clinical tests (i.e. 4-steps-test) and blood counts (i.e. K+) were done by a pediatrician.

Under eplerenone therapy we detected a reduction of muscular oedema, intracellular-weighted sodium IR signal and muscular sodium concentration. The oedema reduction in the DMD patient receiving eplerenone was more pronounced to the patient with cortisone. Myometric-measured tissue parameters such as muscle stiffness had a more pronounced effect in the child treated with eplerenone after a first increase in muscle stiffness both after eplerenone and cortisone treatment. Clinical abilities during both therapies were mostly constant.

Eplerenone might be a possible new therapy option in DMD patients.

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