Article

Facioscapulohumeral muscular dystrophy (FSHD), an inherited and progressive
muscle disorder, is among the most common hereditary muscle disorders. From a
clinical vantage point, FSHD is characterized by weakness of the facial, shoulder
(often with scapular winging), arm (including biceps and triceps) and abdominal
muscles. Forearm muscles are usually spared and weakness is usually asymmetrical.
Over the past few decades, muscle magnetic resonance imaging (MRI) has become
established as a reliable and accurate noninvasive tool for the diagnosis and
assessment of progression in neuromuscular diseases, showing specific patterns of
muscle involvement for a number of myopathies. More recently, MRI has been used
to noninvasively identify quantitative biomarkers, allowing evaluation of the
natural progression of disease and assessment of therapeutic interventions. In
the present review, the intention was to present the most significant MRI
developments related to diagnosis and pattern recognition in FSHD and to discuss
its capacity to provide outcome measures.
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Langue : ANGLAIS