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Respiratory Management in Acute Neuromuscular Disease

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Emergencies in Neuromuscular Disorders

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Abstract

This chapter deals with the pathophysiology and principles of management of respiratory failure due to acute neuromuscular disorders. Neuromuscular respiratory weakness involves all muscles needed to maintain an open airway and suck in air. The inspiratory force is mostly a function of the diaphragm, but other muscles are at play when the diaphragm fails such as the intercostal, paraspinal, and neck muscles. Equally important for respiration is the bulbar musculature, which maintains a patent airway and allows conduit through the dilator muscles of the palate, pharynx, and larynx. Respiration is also exhalation, which is passive, but may need the abdominal wall and internal intercostal muscles, which enable cough. Neuromuscular respiratory failure should be evaluated along the long neural trajectory from the respiratory pattern generator in the brain stem to cervical spine, peripheral nerves, and, finally, the muscles directly involved in respiration. Both central and peripheral lesions can cause respiratory mechanics to fail acutely. The neurology of breathing involves changes in respiratory drive, rhythm, mechanics, and dynamics. This review focuses on the fundamentals of abnormal respiratory mechanics in acute neurological conditions, bedside judgment, interpretation of additional laboratory tests, and initial stabilization, providing practical solutions with illustrative cases taken from the authors’ clinical practice.

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Authors and Affiliations

  1. Cambridge University Hospitals, Cambridge, UK

    Maxwell Damian

  2. Neurocritical Care Services, Mayo Clinic Hospitals, Rochester, MN, USA

    Eelco F. M. Wijdicks

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  1. Cambridge University Hospitals, Cambridge, UK

    Maxwell Damian

  2. Department of Neurology, Amsterdam University Medical Centres, location Academic Medical Center, Amsterdam, The Netherlands

    Marianne de Visser

Electronic Supplementary Material

Neuromuscular respiratory function testing in a 65-year-old patient with facioscapulohumeral muscular dystrophy admitted for falls and chest infection. Note technical difficulties to overcome weakness of mouth seal. Respiratory function is characterized by weakness of chest wall and abdominal muscles affecting forced expiration more than inspiration (by contrast: predominant diaphragmatic weakness in Pompe disease, Case Vignette 2). (MP4 208287 kb)

Clinical demonstration of short-sentence (staccato) speech in a patient with progressive Guillain–Barré syndrome. The pulmonary function tests had declined with a vital capacity of 1.3 L. PImax of −40 cm H2O and PE max of 80 cm H2O. (From Wijdicks EFM. The neurology of acutely failing respiratory mechanics. Ann Neurol 2017; 81: 485–494; Suppl. Material: ana24908-sup-0001-suppinfomovie.mov) (MP4 41554 kb)

Thoraco-abdominal dyssynergy = “paradoxical breathing” in neuromuscular respiratory failure. (From: Wijdicks EFM. Neurogenic paradoxical breathing. Journal of Neurology, Neurosurgery & Psychiatry Published Online First: 18 July 2013. https://doi.org/10.1136/jnnp-2013-305485) J Neurol Neurosurg Psychiatry. 2013;84(11):1296. (MP4 4954 kb)

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Damian, M., Wijdicks, E.F.M. (2022). Respiratory Management in Acute Neuromuscular Disease. In: Damian, M., de Visser, M. (eds) Emergencies in Neuromuscular Disorders. Springer, Cham. https://doi.org/10.1007/978-3-030-91932-0_3

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