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Respiratory muscle weakness due to neuromuscular disease: Management

Scott K Epstein, MD
Section Editors
Polly E Parsons, MD
Jeremy M Shefner, MD, PhD
R Sean Morrison, MD
Deputy Editor
Geraldine Finlay, MD


Respiratory muscle weakness is common among patients who have neuromuscular disease (table 1) [1,2]. Some of the diseases respond to specific therapies (eg, Guillain-Barré syndrome, myasthenia gravis, polymyositis), while others are incompletely treated (eg, amyotrophic lateral sclerosis). Regardless of whether the underlying disease is reversible, early identification of respiratory muscle weakness is essential because supportive care can provide symptomatic relief, improve quality of life, and prolong life.

The management of respiratory muscle weakness due to neuromuscular disease will be reviewed here. The clinical manifestations and evaluation of respiratory muscle weakness and treatment of the underlying neuromuscular diseases are discussed separately. (See "Respiratory muscle weakness due to neuromuscular disease: Clinical manifestations and evaluation".)


Subjective clinical findings and objective physiologic tests are used together to determine when mechanical ventilation is indicated. The criteria used to make this decision are described elsewhere. (See "Respiratory muscle weakness due to neuromuscular disease: Clinical manifestations and evaluation", section on 'Assess need for ventilatory support'.)

Noninvasive positive pressure ventilation — Once it is determined that mechanical ventilation is necessary, options include noninvasive positive pressure ventilation (NPPV) or invasive positive pressure ventilation. NPPV may benefit patients who require:

Continuous mechanical ventilation for a short duration (ie, days), such as patients with an acute neuromuscular disease (eg, Guillain-Barré syndrome)


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Literature review current through: Mar 2017. | This topic last updated: Sep 01, 2015.
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