Respiratory muscle weakness due to neuromuscular disease: Management
- Scott K Epstein, MD
Scott K Epstein, MD
- Professor of Medicine
- Tufts University School of Medicine
- Section Editors
- Polly E Parsons, MD
Polly E Parsons, MD
- Editor-in-Chief — Pulmonary and Critical Care Medicine
- Section Editor — Critical Care
- Professor of Medicine
- University of Vermont College of Medicine
- Jeremy M Shefner, MD, PhD
Jeremy M Shefner, MD, PhD
- Section Editor — Neuromuscular Disease
- Professor and Chair of Neurology, Barrow Neurological Institute
- Professor of Neurology, University of Arizona, Phoenix
- Clinical Professor of Neurology, Creighton University
- R Sean Morrison, MD
R Sean Morrison, MD
- Section Editor — Selected End Stage Conditions
- Hermann Merkin Professor of Palliative Care
- Mount Sinai School of Medicine
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 ventilation — Once it is determined that mechanical ventilation is necessary, options include noninvasive positive pressure ventilation (NIV) or invasive positive pressure ventilation. NIV 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)
- Perrin C, Unterborn JN, Ambrosio CD, Hill NS. Pulmonary complications of chronic neuromuscular diseases and their management. Muscle Nerve 2004; 29:5.
- Epstein SK. An overview of respiratory muscle function. Clin Chest Med 1994; 15:619.
- Vianello A, Bevilacqua M, Arcaro G, et al. Non-invasive ventilatory approach to treatment of acute respiratory failure in neuromuscular disorders. A comparison with endotracheal intubation. Intensive Care Med 2000; 26:384.
- Servera E, Sancho J, Zafra MJ, et al. Alternatives to endotracheal intubation for patients with neuromuscular diseases. Am J Phys Med Rehabil 2005; 84:851.
- Rabinstein A, Wijdicks EF. BiPAP in acute respiratory failure due to myasthenic crisis may prevent intubation. Neurology 2002; 59:1647.
- Sancho J, Servera E, Bañuls P, Marin J. Predictors of need for noninvasive ventilation during respiratory tract infections in medically stable, non-ventilated subjects with amyotrophic lateral sclerosis. Respir Care 2015; 60:492.
- Ward S, Chatwin M, Heather S, Simonds AK. Randomised controlled trial of non-invasive ventilation (NIV) for nocturnal hypoventilation in neuromuscular and chest wall disease patients with daytime normocapnia. Thorax 2005; 60:1019.
- Annane D, Chevrolet JC, Chevret S, Raphael JC. Nocturnal mechanical ventilation for chronic hypoventilation in patients with neuromuscular and chest wall disorders. Cochrane Database Syst Rev 2000:CD001941.
- Annane D, Orlikowski D, Chevret S. Nocturnal mechanical ventilation for chronic hypoventilation in patients with neuromuscular and chest wall disorders. Cochrane Database Syst Rev 2014; :CD001941.
- Aboussouan LS, Khan SU, Banerjee M, et al. Objective measures of the efficacy of noninvasive positive-pressure ventilation in amyotrophic lateral sclerosis. Muscle Nerve 2001; 24:403.
- Aboussouan LS, Khan SU, Meeker DP, et al. Effect of noninvasive positive-pressure ventilation on survival in amyotrophic lateral sclerosis. Ann Intern Med 1997; 127:450.
- Farrero E, Prats E, Povedano M, et al. Survival in amyotrophic lateral sclerosis with home mechanical ventilation: the impact of systematic respiratory assessment and bulbar involvement. Chest 2005; 127:2132.
- Kleopa KA, Sherman M, Neal B, et al. Bipap improves survival and rate of pulmonary function decline in patients with ALS. J Neurol Sci 1999; 164:82.
- Pinto AC, Evangelista T, Carvalho M, et al. Respiratory assistance with a non-invasive ventilator (Bipap) in MND/ALS patients: survival rates in a controlled trial. J Neurol Sci 1995; 129 Suppl:19.
- Bourke SC, Tomlinson M, Williams TL, et al. Effects of non-invasive ventilation on survival and quality of life in patients with amyotrophic lateral sclerosis: a randomised controlled trial. Lancet Neurol 2006; 5:140.
- Ambrosino N, Carpenè N, Gherardi M. Chronic respiratory care for neuromuscular diseases in adults. Eur Respir J 2009; 34:444.
- DiPALS Writing Committee, DiPALS Study Group Collaborators, McDermott CJ, et al. Safety and efficacy of diaphragm pacing in patients with respiratory insufficiency due to amyotrophic lateral sclerosis (DiPALS): a multicentre, open-label, randomised controlled trial. Lancet Neurol 2015; 14:883.
- Gonzalez-Bermejo J, Morélot-Panzini C, Tanguy ML, et al. Early diaphragm pacing in patients with amyotrophic lateral sclerosis (RespiStimALS): a randomised controlled triple-blind trial. Lancet Neurol 2016; 15:1217.
- MacIntyre NR, Cook DJ, Ely EW Jr, et al. Evidence-based guidelines for weaning and discontinuing ventilatory support: a collective task force facilitated by the American College of Chest Physicians; the American Association for Respiratory Care; and the American College of Critical Care Medicine. Chest 2001; 120:375S.
- Thomas CE, Mayer SA, Gungor Y, et al. Myasthenic crisis: clinical features, mortality, complications, and risk factors for prolonged intubation. Neurology 1997; 48:1253.
- Lawn ND, Wijdicks EF. Post-intubation pulmonary function test in Guillain-Barré syndrome. Muscle Nerve 2000; 23:613.
- Bauman KA, Kurili A, Schmidt SL, et al. Home-based overnight transcutaneous capnography/pulse oximetry for diagnosing nocturnal hypoventilation associated with neuromuscular disorders. Arch Phys Med Rehabil 2013; 94:46.
- Aarrestad S, Tollefsen E, Kleiven AL, et al. Validity of transcutaneous PCO2 in monitoring chronic hypoventilation treated with non-invasive ventilation. Respir Med 2016; 112:112.
- Nardi J, Prigent H, Adala A, et al. Nocturnal oximetry and transcutaneous carbon dioxide in home-ventilated neuromuscular patients. Respir Care 2012; 57:1425.
- Mehta S. Neuromuscular disease causing acute respiratory failure. Respir Care 2006; 51:1016.
- Orlikowski D, Sharshar T, Porcher R, et al. Prognosis and risk factors of early onset pneumonia in ventilated patients with Guillain-Barré syndrome. Intensive Care Med 2006; 32:1962.
- Fletcher DD, Lawn ND, Wolter TD, Wijdicks EF. Long-term outcome in patients with Guillain-Barré syndrome requiring mechanical ventilation. Neurology 2000; 54:2311.
- Cheng BC, Chang WN, Chang CS, et al. Predictive factors and long-term outcome of respiratory failure after Guillain-Barré syndrome. Am J Med Sci 2004; 327:336.
- Demoule A, Jung B, Prodanovic H, et al. Diaphragm dysfunction on admission to the intensive care unit. Prevalence, risk factors, and prognostic impact-a prospective study. Am J Respir Crit Care Med 2013; 188:213.
- Orlikowski D, Prigent H, Sharshar T, et al. Respiratory dysfunction in Guillain-Barré Syndrome. Neurocrit Care 2004; 1:415.
- Varelas PN, Chua HC, Natterman J, et al. Ventilatory care in myasthenia gravis crisis: assessing the baseline adverse event rate. Crit Care Med 2002; 30:2663.
- Nguyen TN, Badjatia N, Malhotra A, et al. Factors predicting extubation success in patients with Guillain-Barré syndrome. Neurocrit Care 2006; 5:230.
- Rabinstein AA, Mueller-Kronast N. Risk of extubation failure in patients with myasthenic crisis. Neurocrit Care 2005; 3:213.
- Finder JD, Birnkrant D, Carl J, et al. Respiratory care of the patient with Duchenne muscular dystrophy: ATS consensus statement. Am J Respir Crit Care Med 2004; 170:456.
- Morrow B, Zampoli M, van Aswegen H, Argent A. Mechanical insufflation-exsufflation for people with neuromuscular disorders. Cochrane Database Syst Rev 2013; :CD010044.
- Vianello A, Corrado A, Arcaro G, et al. Mechanical insufflation-exsufflation improves outcomes for neuromuscular disease patients with respiratory tract infections. Am J Phys Med Rehabil 2005; 84:83.
- Sancho J, Servera E, Vergara P, Marín J. Mechanical insufflation-exsufflation vs. tracheal suctioning via tracheostomy tubes for patients with amyotrophic lateral sclerosis: a pilot study. Am J Phys Med Rehabil 2003; 82:750.
- Chatwin M, Ross E, Hart N, et al. Cough augmentation with mechanical insufflation/exsufflation in patients with neuromuscular weakness. Eur Respir J 2003; 21:502.
- Sivasothy P, Brown L, Smith IE, Shneerson JM. Effect of manually assisted cough and mechanical insufflation on cough flow of normal subjects, patients with chronic obstructive pulmonary disease (COPD), and patients with respiratory muscle weakness. Thorax 2001; 56:438.
- Winck JC, Gonçalves MR, Lourenço C, et al. Effects of mechanical insufflation-exsufflation on respiratory parameters for patients with chronic airway secretion encumbrance. Chest 2004; 126:774.
- Boitano LJ. Management of airway clearance in neuromuscular disease. Respir Care 2006; 51:913.
- Rafiq MK, Bradburn M, Proctor AR, et al. A preliminary randomized trial of the mechanical insufflator-exsufflator versus breath-stacking technique in patients with amyotrophic lateral sclerosis. Amyotroph Lateral Scler Frontotemporal Degener 2015; 16:448.
- Toussaint M, Pernet K, Steens M, et al. Cough Augmentation in Subjects With Duchenne Muscular Dystrophy: Comparison of Air Stacking via a Resuscitator Bag Versus Mechanical Ventilation. Respir Care 2016; 61:61.
- Lacombe M, Del Amo Castrillo L, Boré A, et al. Comparison of three cough-augmentation techniques in neuromuscular patients: mechanical insufflation combined with manually assisted cough, insufflation-exsufflation alone and insufflation-exsufflation combined with manually assisted cough. Respiration 2014; 88:215.
- Heyland DK, Dhaliwal R, Drover JW, et al. Canadian clinical practice guidelines for nutrition support in mechanically ventilated, critically ill adult patients. JPEN J Parenter Enteral Nutr 2003; 27:355.
- Drakulovic MB, Torres A, Bauer TT, et al. Supine body position as a risk factor for nosocomial pneumonia in mechanically ventilated patients: a randomised trial. Lancet 1999; 354:1851.
- MECHANICAL VENTILATION
- Noninvasive ventilation
- - Acute respiratry failure (short-term use)
- - Early chronic respiratory failure (intermittent long-term use)
- - Technical aspects
- Invasive mechanical ventilation
- Discontinuing ventilatory support
- COUGH ASSISTANCE
- Mechanical insufflation-exsufflation
- Manually-assisted cough
- Hyperinflation maneuvers
- Secretion mobilization techniques
- GENERAL CARE
- Fluid and electrolytes
- Venous thromboembolism prophylaxis
- Stress ulcer prophylaxis
- Prevention of ventilator-associated pneumonia
- Physical therapy
- PATIENT VALUES AND PREFERENCES
- SUMMARY AND RECOMMENDATIONS