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Sleep Disordered Breathing in Duchenne Muscular Dystrophy

  • Sleep (M Thorpy and M Billiard, Section Editors)
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Abstract

This review aims to explain the inevitable imbalance between respiratory load, drive, and muscular force that occurs in the natural aging of Duchenne muscular dystrophy and that predisposes these patients to sleep disordered breathing (SDB). In DMD, SDB is characterized by oxygen desaturation, apneas, hypercapnia, and hypoventilation during sleep and ultimately develops into respiratory failure during wakefulness. It can be present in all age groups. Young patients risk obstructive apneas because of weight gain, secondary to progressive physical inactivity and prolonged corticosteroid therapy; older patients hypoventilate and desaturate because of respiratory muscle weakness, in particular the diaphragm. These conditions are further exacerbated during REM sleep, the phase of maximal muscle hypotonia during which the diaphragm has to provide most of the ventilation. Evidence is given to the daytime predictors of early symptoms of SDB, important indicators for the proper time to initiate mechanical ventilation.

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Acknowledgements

This study is supported by Fondo DMD “Amici di Emanuele.” A special thanks goes to David Kuller for his corrections and suggestions.

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Authors and Affiliations

  1. Dipartimento di Elettronica, Informazione e Bioingegneria, Politecnico di Milano, 20133, Milan, Italy

    Antonella LoMauro & Andrea Aliverti

  2. Istituto di Ricovero e Cura a Carattere Scientifico “E. Medea”, Bosisio Parini, Italy

    Maria Grazia D’Angelo

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  1. Antonella LoMauro
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  2. Maria Grazia D’Angelo
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  3. Andrea Aliverti
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Correspondence to Antonella LoMauro.

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Antonella Lo Mauro, Maria Grazia D’Angelo, and Andrea Aliverti declare that they have no conflict of interest.

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This article does not contain any studies with human or animal subjects performed by any of the authors.

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This article is part of the Topical Collection on Sleep.

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LoMauro, A., D’Angelo, M.G. & Aliverti, A. Sleep Disordered Breathing in Duchenne Muscular Dystrophy. Curr Neurol Neurosci Rep 17, 44 (2017). https://doi.org/10.1007/s11910-017-0750-1

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