Respiratory muscle weakness due to neuromuscular disease: Clinical manifestations and evaluation
- Scott K Epstein, MD
Scott K Epstein, MD
- Professor of Medicine
- Tufts University School of Medicine
- Section Editors
- 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
- 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
- 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]. It can be acute (eg, Guillain-Barré syndrome), chronic and relapsing (eg, multiple sclerosis, myasthenia gravis), or relentlessly progressive (eg, amyotrophic lateral sclerosis [ALS]).
Regardless of its clinical course, respiratory muscle weakness is a serious problem among patients with neuromuscular disease. It is estimated that 15 to 28 percent of patients with myasthenia gravis and 20 to 30 percent of patients with Guillain-Barré syndrome will require invasive mechanical ventilation [3-5]. Many patients with ALS will die from progressive chronic respiratory failure.
The clinical manifestations and evaluation of respiratory muscle weakness due to neuromuscular disease will be reviewed here. The management and outcome of such patients are discussed separately. (See "Respiratory muscle weakness due to neuromuscular disease: Management".)
Respiratory muscle (inspiratory, expiratory, upper airway) weakness due to neuromuscular disease can cause insufficient ventilation, nocturnal hypoventilation, or ineffective cough . It can also be associated with bulbar dysfunction. Each of these abnormalities has its own related symptoms and signs (table 2).
●Insufficient ventilation may induce dyspnea, orthopnea, rapid shallow breathing (tachypnea plus decreased tidal volume), accessory respiratory muscle use, thoracoabdominal paradox (inward motion of the abdomen during inspiration), hypercapnia, or hypoxemia.
- 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.
- Sharshar T, Chevret S, Bourdain F, et al. Early predictors of mechanical ventilation in Guillain-Barré syndrome. Crit Care Med 2003; 31:278.
- Mehta S. Neuromuscular disease causing acute respiratory failure. Respir Care 2006; 51:1016.
- Durand MC, Porcher R, Orlikowski D, et al. Clinical and electrophysiological predictors of respiratory failure in Guillain-Barré syndrome: a prospective study. Lancet Neurol 2006; 5:1021.
- Ambrosino N, Carpenè N, Gherardi M. Chronic respiratory care for neuromuscular diseases in adults. Eur Respir J 2009; 34:444.
- Sancho J, Servera E, Díaz J, Marín J. Predictors of ineffective cough during a chest infection in patients with stable amyotrophic lateral sclerosis. Am J Respir Crit Care Med 2007; 175:1266.
- Servera E, Sancho J. Non-invasive ventilation in amyotrophic lateral sclerosis. Lancet Neurol 2006; 5:291.
- McCool FD, Leith DE. Pathophysiology of cough. Clin Chest Med 1987; 8:189.
- Burakgazi AZ, Höke A. Respiratory muscle weakness in peripheral neuropathies. J Peripher Nerv Syst 2010; 15:307.
- Steier J, Kaul S, Seymour J, et al. The value of multiple tests of respiratory muscle strength. Thorax 2007; 62:975.
- Kreitzer SM, Saunders NA, Tyler HR, Ingram RH Jr. Respiratory muscle function in amyotrophic lateral sclerosis. Am Rev Respir Dis 1978; 117:437.
- Kaminska M, Noel F, Petrof BJ. Optimal method for assessment of respiratory muscle strength in neuromuscular disorders using sniff nasal inspiratory pressure (SNIP). PLoS One 2017; 12:e0177723.
- Santos DB, Desmarais G, Falaize L, et al. Twitch mouth pressure for detecting respiratory muscle weakness in suspicion of neuromuscular disorder. Neuromuscul Disord 2017; 27:518.
- Pinto S, Alves P, Pimentel B, et al. Ultrasound for assessment of diaphragm in ALS. Clin Neurophysiol 2016; 127:892.
- 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.
- Bach JR, Saporito LR. Criteria for extubation and tracheostomy tube removal for patients with ventilatory failure. A different approach to weaning. Chest 1996; 110:1566.
- Bach JR, Ishikawa Y, Kim H. Prevention of pulmonary morbidity for patients with Duchenne muscular dystrophy. Chest 1997; 112:1024.
- Sancho J, Servera E, Díaz J, Marín J. Comparison of peak cough flows measured by pneumotachograph and a portable peak flow meter. Am J Phys Med Rehabil 2004; 83:608.
- Szeinberg A, Tabachnik E, Rashed N, et al. Cough capacity in patients with muscular dystrophy. Chest 1988; 94:1232.
- Chaudri MB, Liu C, Hubbard R, et al. Relationship between supramaximal flow during cough and mortality in motor neurone disease. Eur Respir J 2002; 19:434.
- Miller RG, Rosenberg JA, Gelinas DF, et al. Practice parameter: the care of the patient with amyotrophic lateral sclerosis (an evidence-based review): report of the Quality Standards Subcommittee of the American Academy of Neurology: ALS Practice Parameters Task Force. Neurology 1999; 52:1311.
- Lawn ND, Fletcher DD, Henderson RD, et al. Anticipating mechanical ventilation in Guillain-Barré syndrome. Arch Neurol 2001; 58:893.
- Chevrolet JC, Deléamont P. Repeated vital capacity measurements as predictive parameters for mechanical ventilation need and weaning success in the Guillain-Barré syndrome. Am Rev Respir Dis 1991; 144:814.
- Ropper AH. The Guillain-Barré syndrome. N Engl J Med 1992; 326:1130.
- Rieder P, Louis M, Jolliet P, Chevrolet JC. The repeated measurement of vital capacity is a poor predictor of the need for mechanical ventilation in myasthenia gravis. Intensive Care Med 1995; 21:663.