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01-11-2019 | Magnetic Resonance Imaging | Original Communication

Imaging respiratory muscle quality and function in Duchenne muscular dystrophy

Authors: Alison M. Barnard, Donovan J. Lott, Abhinandan Batra, William T. Triplett, Sean C. Forbes, Samuel L. Riehl, Rebecca J. Willcocks, Barbara K. Smith, Krista Vandenborne, Glenn A. Walter

Published in: Journal of Neurology | Issue 11/2019

Abstract

Objective

Duchenne muscular dystrophy (DMD) is characterized by damage to muscles including the muscles involved in respiration. Dystrophic muscles become weak and infiltrated with fatty tissue, resulting in progressive respiratory impairment. The objective of this study was to assess respiratory muscle quality and function in DMD using magnetic resonance imaging and to determine the relationship to clinical respiratory function.

Methods

Individuals with DMD (n = 36) and unaffected controls (n = 12) participated in this cross sectional magnetic resonance imaging study. Participants underwent dynamic imaging of the thorax to assess diaphragm and chest wall mobility and chemical shift-encoded imaging of the chest and abdomen to determine fatty infiltration of the accessory respiratory muscles. Additionally, clinical pulmonary function measures were obtained.

Results

Thoracic cavity area was decreased in individuals with DMD compared to controls during tidal and maximal breathing. Individuals with DMD had reduced chest wall movement in the anterior–posterior direction during maximal inspirations and expirations, but diaphragm descent during maximal inspirations (normalized to height) was only decreased in a subset of individuals with maximal inspiratory pressures less than 60% predicted. Muscle fat fraction was elevated in all three expiratory muscles assessed (p < 0.001), and the degree of fatty infiltration correlated with percent predicted maximal expiratory pressures (r = − 0.70, p < 0.001). The intercostal muscles demonstrated minimal visible fatty infiltration; however, this analysis was qualitative and resolution limited.

Interpretation

This magnetic resonance imaging investigation of diaphragm movement, chest wall movement, and accessory respiratory muscle fatty infiltration provides new insights into the relationship between disease progression and clinical respiratory function.

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Title: Imaging respiratory muscle quality and function in Duchenne muscular dystrophy
Authors: Alison M. Barnard
Donovan J. Lott
Abhinandan Batra
William T. Triplett
Sean C. Forbes
Samuel L. Riehl
Rebecca J. Willcocks
Barbara K. Smith
Krista Vandenborne
Glenn A. Walter
Publication date: 01-11-2019
Publisher: Springer Berlin Heidelberg
Keywords: Magnetic Resonance Imaging
Magnetic Resonance Imaging
Muscular Dystrophy
Muscular Dystrophy
Duchenne Muscular Dystrophy
Published in: Journal of Neurology / Issue 11/2019
Print ISSN: 0340-5354
Electronic ISSN: 1432-1459
DOI: https://doi.org/10.1007/s00415-019-09481-z

Keynote webinar | Spotlight on medication adherence

Springer Medicine

Imaging respiratory muscle quality and function in Duchenne muscular dystrophy

Abstract

Objective

Methods

Results

Interpretation

Keynote webinar | Spotlight on medication adherence

Springer Medicine

Abstract

Objective

Methods

Results

Interpretation

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