Muscle Mri of Facioscapulohumeral Dystrophy (Fshd): A Growing Demand and a Promising Approach Publisher Pubmed



Fatehi F^{1, 4} ; Salortcampana E^{1, 2} ; Le Troter A³ ; Bendahan D³ ; Attarian S^{1, 2} Show Affiliations
Source: Revue Neurologique Published:2016

Abstract

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. © 2016 Elsevier Masson SAS