P.13.7 Elevated muscle metabolites in Becker muscular dystrophy detected by MR spectroscopy

Abstract

Becker muscular dystrophy (BMD) is characterized by progressive muscle weakness and a variable disease course. Different muscles become infiltrated with fat at different rates and with varying times of onset, for which the underlying pathophysiology is unknown. Phosphorus (31P) magnetic resonance spectroscopy has shown alterations in muscle metabolism in BMD patients. It is unknown whether muscles that do not show fatty infiltration and are therefore early in the dystrophic process already show metabolic changes. Moreover, these changes have never been studied in individual muscles. Using a 7T MRI scanner we examined the level of phosphorus muscle metabolites and the level of fatty infiltration in five main lower leg muscles. 31P 2D multivoxel data sets and a 3-point DIXON, to quantify the percentage of fat infiltration, were acquired in 24 BMD patients (20–63 years) and seven age-matched healthy controls (22–65 years). Phosphodiester (PDE) levels, which are related to cell proliferation, were significantly increased (p 10%) fat levels. Significant increases (p