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. 2024;103(10):601-621.
doi: 10.1159/000539726. Epub 2024 Jun 10.

Future Directions for Respiratory Muscle Training in Neuromuscular Disorders: A Scoping Review

Esmee S B van Kleef  1 , Diego Poddighe  2   3 , Mayra Caleffi Pereira  2   3   4 , Marit L Schuurbiers  5 , Jan T Groothuis  6 , Peter J Wijkstra  7   8 , Nicol C Voermans  5 , Rik Gosselink  2   3 , Daniel Langer  2   3 , Jonne Doorduin  5   9
Affiliations

Future Directions for Respiratory Muscle Training in Neuromuscular Disorders: A Scoping Review

Esmee S B van Kleef et al. Respiration. 2024.

Abstract

Background: Respiratory muscle training (RMT) aims to improve inspiratory and/or expiratory muscle function in neuromuscular disorders (NMDs). A comprehensive overview of the available literature is lacking. This scoping review explores methodological characteristics, (adverse) effects, and adherence of RMT studies in NMDs. Moreover, it identifies limitations and research gaps in the literature and provides future research directions.

Summary: Eligible studies were identified using MEDLINE, Embase, Cochrane Database of Systematic Reviews, and Cochrane Central Register of Controlled Trials databases. Three reviewers independently selected articles. Inclusion criteria were English language, original research articles on RMT using a device, patients with an NMD, and pulmonary function tests or respiratory muscle strength as outcome measures. We included NMDs with slow, intermediate and fast progression. Exclusion criteria were critically ill patients, weaning from mechanical ventilation, other neurological disorders, and RMT combined with non-respiratory interventions. One reviewer extracted the data on patients' characteristics, methodological characteristics, results of outcome measures, adverse events, and patient adherence. Forty-five studies were identified. We found a large diversity in study designs and training protocols. The effects of RMT on respiratory muscle strength and/or endurance are variable. Patient adherence was high and no serious adverse events were reported.

Key messages: The diversity in studies across the available literature precludes definitive conclusions regarding the effects of RMT on respiratory muscle function and clinically relevant outcomes in NMDs. Therefore, well-powered and -designed studies that focus on clinically relevant outcomes and assess whether RMT can improve or offset deterioration of respiratory muscle weakness in NMDs are needed.

Keywords: Neuromuscular disorders; Respiratory muscle training; Scoping review.

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Conflict of interest statement

The authors have no conflicts of interest to declare.

Figures

Fig. 1.
Fig. 1.
Flowchart for the study selection process. NMD, neuromuscular disorder.
See this image and copyright information in PMC

References

    1. Boentert M, Wenninger S, Sansone VA. Respiratory involvement in neuromuscular disorders. Curr Opin Neurol. 2017;30(5):529–37. - PubMed
    1. Ambrosino N, Carpenè N, Gherardi M. Chronic respiratory care for neuromuscular diseases in adults. Eur Respir J. 2009;34(2):444–51. - PubMed
    1. Simonds AK, Muntoni F, Heather S, Fielding S. Impact of nasal ventilation on survival in hypercapnic Duchenne muscular dystrophy. Thorax. 1998;53(11):949–52. - PMC - PubMed
    1. Bourke SC, Tomlinson M, Williams TL, Bullock RE, Shaw PJ, Gibson GJ. Effects of non-invasive ventilation on survival and quality of life in patients with amyotrophic lateral sclerosis: a randomised controlled trial. Lancet Neurol. 2006;5(2):140–7. - PubMed
    1. Sheers N, Howard ME, Berlowitz DJ. Respiratory adjuncts to NIV in neuromuscular disease. Respirology. 2019;24(6):512–20. - PubMed

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