Use of Noninvasive Ventilation with Volume-Assured Pressure Support in Neuralgic Amyotrophy with Bilateral Diaphragmatic Paralysis

Montserrat Diaz-Abad1, *, Neil Porter2, Lindsay Zilliox2, Nevins Todd1
1 Department of Medicine, University of Maryland School of Medicine, 100 South Paca Street 2nd Fl, Baltimore, Maryland, 21201, USA
2 Department of Neurology, University of Maryland School of Medicine, 100 South Paca Street 3rd Fl, Baltimore, Maryland, 21201, USA

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Creative Commons License
© 2019 Diaz-Abad et al.

open-access license: This is an open access article distributed under the terms of the Creative Commons Attribution 4.0 International Public License (CC-BY 4.0), a copy of which is available at: This license permits unrestricted use, distribution, and reproduction in any medium, provided the original author and source are credited.

* Address correspondence to this author at the Department of Medicine, University of Maryland School of Medicine. Sleep Disorders Center, 100 North Greene Street Room 214, Baltimore, Maryland 21201, USA; Tel: 410-706-4771; Fax: 410-706-0345;


Neuralgic Amyotrophy (NA) is a rare, acute onset inflammatory brachial plexopathy that frequently presents with acute pain followed by shoulder girdle muscle weakness. Phrenic nerve involvement affecting the diaphragms occurs in 7-10% of cases. We present the case of a 52-year-old man with neuralgic amyotrophy with phrenic nerve involvement and bilateral diaphragmatic paralysis with marked respiratory symptoms and sleep hypoventilation, who was treated with non-invasive ventilation with volume assured pressure support mode. By 21 months post disease onset, the patient had experienced marked improvement in orthopnea, sleep quality and functional status. This is the first reported case of the use of this mode of noninvasive ventilation in neuralgic amyotrophy.

Keywords: Neuralgic amyotrophy, Phrenic nerve dysfunction, Diaphragmatic paralysis, Noninvasive ventilation, Sleep hypoventilation, EMG.