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Abstract #0737

Skeletal muscle Mg2+, membrane permeability, and pH are altered in Becker muscular dystrophy: A 31P-MRS and DT-MRI study

Donnie Cameron1, Melissa T. Hooijmans2, Erik H. Niks3, and Hermien E. Kan1
1C.J. Gorter MRI Center, Department of Radiology, Leiden University Medical Center, Leiden, Netherlands, 2Department of Radiology and Nuclear Medicine, Amsterdam UMC, Location AMC, Amsterdam, Netherlands, 3Department of Neurology, Leiden University Medical Center, Leiden, Netherlands

Synopsis

Keywords: Muscle, Permeability, Neuromuscular diseasesBecker muscular dystrophy (BMD) is an X-linked disorder characterised by variable, progressive muscle damage and loss of function. MR-derived fat fraction is used as a disease-progression marker, but early markers are lacking. Here we compared membrane permeability measures between BMD patients and controls to test whether these are altered in BMD. Phosphorus-(31P)-MRS showed reduced ionised magnesium [Mg2+], and increased phosphodiester/adenosine-triphosphate ratios and pH in the tibialis anterior muscle in patients, while diffusion-tensor-(DT)-MRI-derived permeability showed no inter-group differences. Future studies should determine the predictive value of 31P-MRS-measured [Mg2+], pH, and membrane breakdown for disease progression, to establish their potential as biomarkers.

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