Dynamic magnetic resonance imaging of muscle contraction in facioscapulohumeral muscular dystrophy

This database includes the raw data linked with paper “ Dynamic magnetic resonance imaging of muscle contraction in facioscapulohumeral muscular dystrophy”.
Data are related to FSHD patients, who had a confirmed molecular diagnosis. All subjects were scanned on a 3T MAGNETOM Skyra [Siemens Healthineers]. Dynamic scans were performed for both thighs separately in addition to quantitative sequence T2-mapping and Fat Fraction mapping.
Quantitative muscle MRI (water-T2 and fat mapping) is being increasingly used to assess disease involvement in muscle disorders, while imaging techniques for assessment of the dynamic and elastic muscle properties have not yet been translated into clinics. In this exploratory study, we quantitatively characterized muscle deformation (strain) in patients affected by facioscapulohumeral muscular dystrophy (FSHD), a prevalent muscular dystrophy, by applying dynamic MRI synchronized with neuromuscular electrical stimulation (NMES). We evaluated the quadriceps muscles in 34 ambulatory patients and 13 healthy controls, at 6-to 12-month time intervals. While a subgroup of patients behaved similarly to controls, for another subgroup the median strain decreased over time (approximately 57% over 1.5 years). Dynamic MRI parameters did not correlate with quantitative MRI. Our results suggest that the evaluation of muscle contraction by NMES-MRI is feasible and could potentially be used to explore the elastic properties and monitor muscle involvement in FSHD and other neuromuscular disorders.