P.16.10 Muscle MRI of scapular girdle in Facioscapulohumeral muscular dystrophy (FSHD)

To assess the pattern and degree of involvement of scapular girdle muscles in Facioscapulohumeral muscular dystrophy (FSHD). We standardized an MRI protocol that allowed the identification and evaluation of 14 muscle groups of the neck and upper girdle. 108 FSHD patients were included in the study. The involvement in FSHD was compared with that of limb girdle muscular dystrophies and other myopathies with upper girdle weakness. We were able to identify the most affected and spared muscles in FSHD. Asymmetry was a consistent feature, similarly to what is found in lower limb muscles, and T2-STIR hyperintensities could be detected as well. The pattern of involvement significantly differed from that encountered in other myopathies. Upper girdle imaging is a helpful tool in guiding the clinical diagnosis of FSHD. Moreover, the detailed knowledge of single muscle involvement provides information useful for understanding and following the natural history of the disease and developing rehabilitation protocols.