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Respiratory management of patients with neuromuscular disease: current perspectives

Authors Pfeffer G, Povitz M

Received 14 September 2016

Accepted for publication 19 October 2016

Published 18 November 2016 Volume 2016:6 Pages 111—118

DOI https://doi.org/10.2147/DNND.S87323

Checked for plagiarism Yes

Review by Single-blind

Peer reviewers approved by Dr Amy Norman

Peer reviewer comments 2

Editor who approved publication: Dr Thomas Müller


Gerald Pfeffer,1,2 Marcus Povitz3

1Department of Clinical Neurosciences, 2Hotchkiss Brain Institute, University of Calgary, Calgary, AB, 3Department of Medicine, Schulich School of Medicine and Dentistry, Western University, London, ON, Canada

Abstract: Neuromuscular ventilatory weakness can be difficult to recognize because the symptoms can be nocturnal, nonspecific, or attributed to other conditions. The presence of respiratory muscle weakness suggests a number of possible heterogeneous conditions, including neurodegenerative, autoimmune, and genetic neuromuscular diseases. In some conditions, disease-modifying management exists, but in the absence of such intervention, supportive respiratory therapy can improve quality of life and survival. In this review, we discuss the differential diagnosis and diagnostic approach to chronic neuromuscular respiratory weakness. We also review the clinical assessment and management of respiratory failure in these conditions.

Keywords: respiratory failure, hereditary myopathy, amyotrophic lateral sclerosis, myopathy, noninvasive ventilation
 

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