Clinical trials related to dystrophinopathies are increasingly using quantitative MRI to assess the evolution of muscle status in patients with these neuromuscular diseases.
In this prospective study, researchers from the NMR laboratory (NIC, Institute of Myology) and a clinician (Reference Centre for MNM Paris-Est, Institute of Myology) aimed to establish the sensitivity of the quantification of the extracellular volume fraction (ECV) via a magnetic resonance fingerprinting sequence with separation of water and fat as a quantitative imaging biomarker of skeletal muscle tissue alterations in Becker muscular dystrophy (BMD) compared to the quantification of the fat fraction (FF) and water relaxation times.
The results of the tests conducted in 28 BMD patients and 19 healthy volunteers over the period April 2018 to October 2022, show that :
- ECV is higher in participants with BMD than in controls;
- ECV is also higher than in the muscles of healthy controls in BMD patients with normal FF;
- ECV correlates with FF, Walton and Gardner-Medwin scale score and serum cardiac troponin T level.
The authors conclude that magnetic resonance fingerprinting with water and fat separation indicates a significant increase in the ECV fraction of skeletal muscle in study participants with BMD.
