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Veeger TTJ, van Zwet EW, Al Mohamad D, et al. Muscle architecture is associated with muscle fat replacement in Duchenne and Becker muscular dystrophies. Muscle Nerve. 2021;64(5):576-584doi: 10.1002/mus.27399.
Veeger, T. T. J., van Zwet, E. W., Al Mohamad, D., Naarding, K. J., van de Velde, N. M., Hooijmans, M. T., Webb, A. G., Niks, E. H., de Groot, J. H., & Kan, H. E. (2021). Muscle architecture is associated with muscle fat replacement in Duchenne and Becker muscular dystrophies. Muscle & nerve, 64(5), 576-584. https://doi.org/10.1002/mus.27399
Veeger, Thom T J, et al. "Muscle architecture is associated with muscle fat replacement in Duchenne and Becker muscular dystrophies." Muscle & nerve vol. 64,5 (2021): 576-584. doi: https://doi.org/10.1002/mus.27399
Veeger TTJ, van Zwet EW, Al Mohamad D, Naarding KJ, van de Velde NM, Hooijmans MT, Webb AG, Niks EH, de Groot JH, Kan HE. Muscle architecture is associated with muscle fat replacement in Duchenne and Becker muscular dystrophies. Muscle Nerve. 2021 Nov;64(5):576-584. doi: 10.1002/mus.27399. Epub 2021 Aug 25. PMID: 34383334.
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Muscle Nerve. 2021 Nov;64(5):576-584. doi: 10.1002/mus.27399. Epub 2021 Aug 25.

Muscle architecture is associated with muscle fat replacement in Duchenne and Becker muscular dystrophies.

Muscle & nerve

Thom T J Veeger, Erik W van Zwet, Diaa Al Mohamad, Karin J Naarding, Nienke M van de Velde, Melissa T Hooijmans, Andrew G Webb, Erik H Niks, Jurriaan H de Groot, Hermien E Kan

Affiliations

  1. C.J. Gorter Center for High Field MRI, Department of Radiology, Leiden University Medical Center, Leiden, The Netherlands.
  2. Department of Biostatistics, Leiden University Medical Center, Leiden, The Netherlands.
  3. Department of Neurology, Leiden University Medical Center, Leiden, The Netherlands.
  4. Department of Radiology & Nuclear Medicine, Amsterdam University Medical Centers, Location AMC, Amsterdam, The Netherlands.
  5. Department of Rehabilitation Medicine, Leiden University Medical Center, Leiden, The Netherlands.

PMID: 34383334 DOI: 10.1002/mus.27399

Abstract

INTRODUCTION/AIMS: Duchenne and Becker muscular dystrophies (DMD and BMD, respectively) are characterized by fat replacement of different skeletal muscles in a specific temporal order. Given the structural role of dystrophin in skeletal muscle mechanics, muscle architecture could be important in the progressive pathophysiology of muscle degeneration. Therefore, the aim of this study was to assess the role of muscle architecture in the progression of fat replacement in DMD and BMD.

METHODS: We assessed the association between literature-based leg muscle architectural characteristics and muscle fat fraction from 22 DMD and 24 BMD patients. Dixon-based magnetic resonance imaging estimates of fat fractions at baseline and 12 (only DMD) and 24 months were related to fiber length and physiological cross-sectional area (PCSA) using age-controlled linear mixed modeling.

RESULTS: DMD and BMD muscles with long fibers and BMD muscles with large PCSAs were associated with increased fat fraction. The effect of fiber length was stronger in muscles with larger PCSA.

DISCUSSION: Muscle architecture may explain the pathophysiology of muscle degeneration in dystrophinopathies, in which proximal muscles with a larger mass (fiber length × PCSA) are more susceptible, confirming the clinical observation of a temporal proximal-to-distal progression. These results give more insight into the mechanical role in the pathophysiology of muscular dystrophies. Ultimately, this new information can be used to help support the selection of current and the development of future therapies.

© 2021 The Authors. Muscle & Nerve published by Wiley Periodicals LLC.

Keywords: MRI; dystrophin; fat fraction; muscle degeneration; pathophysiology

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