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Rev Neurol (Paris). 2016 Oct;172(10):566-571. doi: 10.1016/j.neurol.2016.08.002. Epub 2016 Sep 20.

Muscle MRI of facioscapulohumeral dystrophy (FSHD): A growing demand and a promising approach.

Author information

1
Reference center for Neuromuscular disorders and ALS, Timone University Hospital, Aix-Marseille University, 264, rue Saint-Pierre, 13385 Marseille cedex 05, France; Iranian Center of Neurological research and Shariati hospital, Neurology Department, Tehran University of Medical Sciences, Tehran, Iran.
2
Reference center for Neuromuscular disorders and ALS, Timone University Hospital, Aix-Marseille University, 264, rue Saint-Pierre, 13385 Marseille cedex 05, France; Aix-Marseille université, Inserm UMR S 910 Medical Genetics and Functional Genomics, 13385 Marseille, France.
3
Aix-Marseille université, centre de résonance magnétique biologique et médicale, UMR CNRS 7339, 13385 Marseille, France.
4
Reference center for Neuromuscular disorders and ALS, Timone University Hospital, Aix-Marseille University, 264, rue Saint-Pierre, 13385 Marseille cedex 05, France; Aix-Marseille université, Inserm UMR S 910 Medical Genetics and Functional Genomics, 13385 Marseille, France. Electronic address: sattarian@ap-hm.fr.

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.

KEYWORDS:

FSHD; Facioscapulohumeral dystrophy; MRI; Measurement; Pattern recognition

PMID:
27663058
DOI:
10.1016/j.neurol.2016.08.002
[Indexed for MEDLINE]

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