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Open Access 01-10-2017 | Review

Quantifying fat replacement of muscle by quantitative MRI in muscular dystrophy

Authors: Jedrzej Burakiewicz, Christopher D. J. Sinclair, Dirk Fischer, Glenn A. Walter, Hermien E. Kan, Kieren G. Hollingsworth

Published in: Journal of Neurology | Issue 10/2017

Abstract

The muscular dystrophies are rare orphan diseases, characterized by progressive muscle weakness: the most common and well known is Duchenne muscular dystrophy which affects young boys and progresses quickly during childhood. However, over 70 distinct variants have been identified to date, with different rates of progression, implications for morbidity, mortality, and quality of life. There are presently no curative therapies for these diseases, but a range of potential therapies are presently reaching the stage of multi-centre, multi-national first-in-man clinical trials. There is a need for sensitive, objective end-points to assess the efficacy of the proposed therapies. Present clinical measurements are often too dependent on patient effort or motivation, and lack sensitivity to small changes, or are invasive. Quantitative MRI to measure the fat replacement of skeletal muscle by either chemical shift imaging methods (Dixon or IDEAL) or spectroscopy has been demonstrated to provide such a sensitive, objective end-point in a number of studies. This review considers the importance of the outcome measures, discusses the considerations required to make robust measurements and appropriate quality assurance measures, and draws together the existing literature for cross-sectional and longitudinal cohort studies using these methods in muscular dystrophy.

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Title: Quantifying fat replacement of muscle by quantitative MRI in muscular dystrophy
Authors: Jedrzej Burakiewicz
Christopher D. J. Sinclair
Dirk Fischer
Glenn A. Walter
Hermien E. Kan
Kieren G. Hollingsworth
Publication date: 01-10-2017
Publisher: Springer Berlin Heidelberg
Published in: Journal of Neurology / Issue 10/2017
Print ISSN: 0340-5354
Electronic ISSN: 1432-1459
DOI: https://doi.org/10.1007/s00415-017-8547-3

At a glance: The STEP trials

Springer Medicine

Quantifying fat replacement of muscle by quantitative MRI in muscular dystrophy

Abstract

At a glance: The STEP trials

Springer Medicine

Abstract

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