Neuromuscular diseases and respiratory system
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Department of Thoracic Medicine, “Venizeleion” General Hospital of Heraklion, Crete, Greece
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Emmanouil Ferdoutsis   

Department of Thoracic Medicine, “Venizeleion” General Hospital of Heraklion, Crete, Greece
Pneumon 2017;30(3):176-187
The balance (gas exchange) between the atmosphere and the human body depends not only on the lungs but also in large part on the function of the “ventilator pump”, which consists of the respiratory control center which is located in the brain, the body rib cage, the diaphragm the intercostal, accessory and abdominal muscles. A wide variety of neuromuscular disorders (NMDs) can results in dysfunction of the ventilatory pump that can lead to respiratory failure, pneumonia and even death. Breathing disorders are recognized as the leading cause of mortality in neuromuscular disease and appropriate interventions can prevent complications and prolong life in individuals with neuromuscular disease affecting the respiratory system.1
Bergofsky EH. Respiratory failure in disorders of the thoracic cage. Am Rev Respir Dis 1979; 119:643-69.
Rochester DF, Esau SA. Assessment of ventilatory function in patients with neuromuscular disease. Clin Chest Med 1994; 15:751-63.
Teramoto S. Novel preventive and therapuetic strategy for poststroke pneumonia. Expert Rev Neurother 2009; 9:1187-200.
Hilker R, Poetter C, Findeisen N, et al. Nosocomial pneumonia after acute stroke: implications for neurological intensive care medicine. Stroke 2003; 34:975-81.
Lim SH, Lieu PK, Phua SY, et al. Accuracy of bedside clinical methods compared with fiberoptic endoscopic examination of swallowing (FEES) in determining the risk of aspiration in acute stroke patients. Dysphagia 2001; 16:1-6.
Lakshminarayan K, Tsai AW, Tong X, et al. Utility of dysphagia screening results in predicting poststroke pneumonia. Stroke 2010; 41:2849-54.
Fishburn MJ, Marino RJ, Ditunno JF Jr. Atelectasis and pneumonia in acute spinal cord injury. Arch Phys Med Rehabil 1990; 71:197-200.
Jackson AB, Groomes TE. Incidence of respiratory complications following spinal cord injury. Arch Phys Med Rehabil 1994; 75:270-5.
Hagen EM. Acute complications of spinal cord injuries. World J Orthop 2015; 6:17-23.
Bhaskar KR, Brown R, O’Sullivan DD, Melia S, Duggan M, Reid L. Bronchial mucus hypersecretion in acute quadriplegia. Macromolecular yields and glycoconjugate composition. Am Rev Respir Dis 1991; 143:640-8.
Reines HD, Harris RC. Pulmonary complications of acute spinal cord injuries. Neurosurgery 1987; 21:193-6.
DeVivo MJ, Black KJ, Stover SL. Causes of death during the first 12 years after spinal cord injury. Arch Phys Med Rehabil 1993; 74:248-54.
Almenoff PL, Spungen AM, Lesser M, Bauman WA. Pulmonary function survey in spinal cord injury: influences of smoking and level and completeness of injury. Lung. 1995;173:297-306.
Stocker R, Bürgi U. Respiratory problems after injuries of the cervical spine. Schweiz Med Wochenschr 1998; 128:1462-6.
Berlowitz DJ, Tamplin J. Respiratory muscle training for cervical spinal cord injury. Cochrane Database Syst Rev 2013; CD008507. doi: 10.1002/14651858.CD008507.pub2.
Houttea SV, Vanlandewijcka Y, Gosselinka R. Respiratory muscle training in persons with spinal cord injury: A systematic review. Respiratory Medicine 2006; 100:1886–95.
Monteiro L, Souza-Machado A, Valderramas S, Melo A. The effect of levodopa on pulmonary function in Parkinson's disease: a systematic review and meta-analysis. Clin Ther 2012; 34:1049- 55. doi:10.1016/j.clinthera.2012.03.001. Epub 2012 Mar 31.
Herer B, Arnulf I, Housset B. Effects of levodopa on pulmonary function in Parkinson's disease. Chest 2001; 119:387-93.
Sathyaprabha TN, Kapavarapu PK, Pall PK, Thennarasu K, Raju TR. Pulmonary functions in Parkinson’s disease. Indian J Chest Dis Allied Sci 2005; 47:251-7.
Gosselink R, Kovacs L, Decramer M. Respiratory muscle involvement in multiple sclerosis. Eur Respir J 1999; 13:449-54.
Tantucci C, Massucci M, Piperno R, Betti L, Grassi V, Sorbini CA. Control of breathing and respiratory muscle strength in patients with multiple sclerosis. Chest 1994; 105:1163-70.
Westerdahl E , Wittrin A, Kånåhols M, Gunnarsson M, Nilsagård Y. Deep breathing exercises with positive expiratory pressure in patients with multiple sclerosis - a randomized controlled trial. Clin Respir J 2016; 10:698-706.
Ray AD, Udhoji S, Mashtare TL, Fisher NM. A combined inspiratory and expiratory muscle training program improves respiratory muscle strength and fatigue in multiple sclerosis. Arch Phys Med Rehabil 2013; 94:1964-70.
Martín-Valero R, Zamora-Pascual N, Armenta-Peinado JA. Training of respiratory muscles in patients with multiple sclerosis: a systematic review. Respir Care 2014; 59:1764-72.
John TJ. The final stages of the global eradication of polio. N Engl J Med 2000; 343:806–7.
Trojan DA, Cashman NR. Post-poliomyelitis syndrome. Muscle Nerve 2005; 31:6–19.
Tiffreau V, Rapin A, Serafi R, et al. Post-polio syndrome and rehabilitation. Ann Phys Rehabil Med 2010; 53:42–50.
Lira CA, Minozzo FC, Sousa BS, et al. Lung function in postpoliomyelitis syndrome: a cross-sectional study. J Bras Pneumol 2013; 39:455–460.
Trojan DA, Cashman NR. Post-poliomyelitis syndrome. Muscle Nerve 2005; 31:6-19.
Soliman MG, Higgins SE, El-Kabir DR, Davidson AC, Williams AJ, Howard RS. Non-invasive assessment of respiratory muscle strength in patients with previous poliomyelitis. Respir Med 2005; 99:1217-22. Epub 2005 Apr 12.
Paez-Colasante X, Figueroa-Romero C, Sakowski SA, Goutman SA, Feldman EL. Amyotrophic lateral sclerosis: mechanisms and therapeutics in the epigenomic era. Nat Rev Neurol 2015; 11:266-79.
Steljes DG, Kryger MH, Kirk BW, Millar TW. Sleep in postpolio syndrome. Chest 1990; 98:133-40.
Morgan RK, McNally S, Alexander M, Conroy R, Hardiman O, Costello RW. Use of Sniff nasal-inspiratory force to predict survival in amyotrophic lateral sclerosis. Am J Respir Crit Care Med 2005; 171:269-74. Epub 2004 Oct 29.
Peysson S, Vandenberghe N, Philit F, et al. Factors predicting survival following noninvasive ventilation in amyotrophic lateral sclerosis. Eur Neurol 2008; 59:164-71.
Bourke SC, Bullock RE, Williams TL, Shaw PJ, Gibson GJ. Noninvasive ventilation in ALS: indications and effect on quality of life. Neurology 2003; 61:171-7.
Mustfa N, Walsh E, Bryant V, et al. The effect of noninvasive ventilation on ALS patients and their caregivers. Neurology 2006; 66:1211-7.
Farrero E, Prats E, Povedano M, Martinez-Matos JA, Manresa F, Escarrabill J. Survival in amyotrophic lateral sclerosis with home mechanical ventilation: the impact of systematic respiratory assessment and bulbar involvement. Chest 2005; 127:2132-8.
EFNS guidelines on the clinical management of amyotrophic lateral sclerosis (MALS) -- revised report of an EFNS task force. Eur J Neurol 2012; 19:360-75.
Gibson G. Diaphragmatic paresis: Pathophysiology, clinical features, and investigation. Thorax 1989; 44:960-70.
Hart N, Nickol AH, Cramer D, et al. Effect of severe isolated unilateral and bilateral diaphragm weakness on exercise performance. Am J Respir Crit Care Med 2002; 165:1265-70.
Gierada DS, Slone RM, Fleishman MJ. Imaging evaluation of the diaphragm. Chest Surg Clin N Am 1998; 8:237-80.
Mier-Jedrzelowicz A, Brophy C, Moxham J, et al. Assessment of diaphragm weakness. Am Rev Respir Dis 1988; 137:877-83.
Stradling JR, Warley AR: Bilateral diaphragm paralysis and sleep apnoea without diurnal respiratory failure. Thorax. 43:75-77 1988.
Qureshi A. Diaphragm paralysis. Semin Respir Crit Care Med 2009; 30:315-20.
Miller SG, Brook MM, Tacy TA: Reliability of two-dimensional echocardiography in the assessment of clinically significant abnormal hemidiaphragm motion in pediatric cardiothoracic patients: Comparison with fluoroscopy. Pediatr Crit Care Med 2006; 7:441-4.
Kumar N, Folger WN, Bolton CF. Dyspnea as the predominant manifestation of bilateral phrenic neuropathy. Mayo Clin Proc 2004; 79:1563-5.
Laporta D, Grassino A. Assessment of transdiaphragmatic pressure in humans. J Appl Physiol 1985; 58:1469-76.
McCool FD, Tzelepis GE. Dysfunction of the Diaphragm. N Engl J Med 2012; 366:932-42.
Kharma N. Dysfunction of the diaphragm: imaging as a diagnostic tool. Curr Opin Pulm Med 2013; 19:394-8.
Mehta S. Neuromuscular disease causing acute respiratory failure. Respir Care 2006; 51:1016-21 [discussion appears 1021–1023].
Orlikowski D, Prigent H, Sharshar T, et al. Respiratory dysfunction in Guillain-Barré syndrome. Neurocrit Care 2004; 1:415-22.
Ropper AH. The Guillain-Barré syndrome. N Engl J Med 1992; 326:1130-6.
Mehta S. Neuromuscular disease causing acute respiratory failure. Respir Care 2006; 51:1016-21.
Hughes RA, Wijdicks EF, Benson E, et al; Multidisciplinary Consensus Group. Supportive care for patients with Guillain-Barré syndrome. Arch Neurol 2005; 62:1194-8.
Sharshar T, Chevret S, Bourdain F, Raphaël JC; French Cooperative Group on Plasma Exchange in Guillain-Barré Syndrome. Early predictors of mechanical ventilation in Guillain-Barré syndrome. Crit Care Med 2003;31:278-83.
Hermans G, Jonghe BD, Bruyninckx F, Berghe GV. Clinical review: Critical illness polyneuropathy and myopathy. Crit Care 2008; 12: 238.
Thiele RI, Jakob H, Hund E, et al. Sepsis and catecholamine support are the major risk factors for critical illness polyneuropathy after open heart surgery. Thorac Cardiovasc Surg 2000; 48:145-50.
De Jonghe B, Lacherade JC, Durand MC, et al. Critical illness neuromuscular syndromes. Neurol Clin 2008; 26:507-20.
Griffin D, Fairman N, Coursin D, et al. Acute myopathy during treatment of status asthmaticus with corticosteroids and steroidal muscle relaxants. Chest 1992; 102:510-4.
Letter MA, Schmitz PI, Visser LH, et al. Risk factors for the development of polyneuropathy and myopathy in critically ill patients. Crit Care Med 2001; 29:2281-6.
Deem S. Intensive-care-unit-acquired muscle weakness. Respir Care 2006; 51:1042-52.
Bolton CF. Neuromuscular manifestations of critical illness. Muscle Nerve 2005; 32:140.
Sieb JP. Myasthenia gravis: an update for the clinician. Clin Exp Immunol 2014; 175:408-18.
Sivakumar Sathasivam. Current and emerging treatments for the management of myasthenia gravis. Ther Clin Risk Manag 2011; 7:313–23.
Mayer SA. Intensive care of the myasthenic patient. Neurology 1997; 48(Suppl 5):S70–S5.
Bedlack RS, Sanders DB. On the concept of myasthenic crisis. J Clin Neuromuscul Dis 2002; 4:40-2.
Rabinstein A, Wijdicks EF. BiPAP in acute respiratory failure due to myasthenic crisis may prevent intubation. Neurology 2002; 59:1647-9.
Berrouschot J, Baumann I, Kalischewski P, Sterker M, Schneider D. Therapy of myasthenic crisis. Crit Care Med 1997; 25:1228-35.
Elrington GM, Murray NM, Spiro SG, et al. Neurological paraneoplastic syndromes in patients with small cell lung cancer. A prospective survey of 150 patients. J Neurol Neurosurg Psychiatry 1991; 54:764-7.
Laroche CM, Mier AK, Spiro SG, et al. Respiratory muscle weakness in the Lambert-Eaton myasthenic syndrome. Thorax 1989; 44:913-8.
Lang B, Vincent A. Autoimmune disorders of the neuromuscular junction. Curr Opin Pharmacol 2009; 9:336-40.
Mason WP, Graus F, Lang B, et al. Small-cell lung cancer, paraneoplastic cerebellar degeneration and the Lambert-Eaton myasthenic syndrome. Brain 1997; 120:1279-300.
Hammer TH, Jespersen S, Kanstrup J, Ballegaard VC, Kjerulf A, Gelvan A. Fatal outbreak of botulism in Greenland. Infect Dis (Lond) 2015;47:190-4.
Sobel J. Botulism. Clin Infect Dis 2005; 41:1167-73.
Zhang JC, Sun L, Nie QH. Botulism, where are we now? Clin Toxicol (Phila) 2010; 48:867-79.
Brook I. Botulism: the challenge of diagnosis and treatment. Rev Neurol Dis 2006;3:182-9.
Cardamone M, Darras BT, Ryan MM. Inherited myopathies and muscular dystrophies. Semin Neurol 2008; 28:250-9.
American Thoracic Society consensus statement: Respiratory care of the patient with Duchenne Muscular Dystrophy. AJRCCM 2004; 170:456-65.
Shneerson JM, Simonds AK. Noninvasive ventilation for chest wall and neuromuscular disorders. Eur Respir J 2002; 20:480-7.
Simonds AK, Muntoni F, Heather S, et al. Impact of nasal ventilation on survival in hypercapnic Duchenne muscular dystrophy. Thorax 1998; 53:949-52.
Poussel M, Kaminsky P, Renaud P, Laroppe J, Pruna L, Chenuel B. Supine changes in lung function correlate with chronic respiratory failure in myotonic dystrophy patients. Respir Physiol Neurobiol 2014; 193:43-51.
Aruj PK, Monteiro SG, De Vito EL. Analysis of factors associated with chronic hypercapnia in patients with myotonic dystrophy. Medicina (B Aires) 2013; 73:529-34.
Hazenberg A, van Alfen N, Voet NB, Kerstjens HA, Wijkstra PJ. Facioscapulohumeral muscular dystrophy and respiratory failure; what about the diaphragm? Respir Med Case Rep 2014; 14:37-9.
Simonds AK. Respiratory complications of the muscular dystrophies. Semin Respir Crit Care Med 2002; 23:231-8.
Barohn RJ, Clanton T, Sahenk Z, Mendell JR. Recurrent respiratory insufficiency and depressed ventilatory drive complicating mitochondrial myopathies. Neurology 1990; 40:103-6.
Braun NMT, Aurora NS, Rochester DF. Respiratory muscle and pulmonary function in poliomyositis and other proximal myopathies. Thorax 1983; 38:316-23.
Teixeira A, Cherin P, Demoule A, et al. Diaphragmatic dysfunction in patients with idiopathic inflammatory myopathies. Neuromuscul Disord 2005; 15:32-9.
Fathi M, Lundberg IE, Tornling G. Pulmonary complications of polymyositis and dermatomyositis. Semin Respir Crit Care Med 2007; 28:451-8.
Warrington KJ, Moder KG, Brutinel WM. The shrinking lungs syndrome in systemic lupus erythematosus. Mayo Clin Proc 2000; 75:467-72.
Polsonetti BW, Joy SD, Laos LF. Steroid-induced myopathy in the ICU. Ann Pharmacother 2002; 36:1741-4.
Rabinstein AA. Noninvasive ventilation for neuromuscular respiratory failure: when to use and when to avoid. Curr Opin Crit Care 2016; 22:94-9.
Simons KA. Noninvasive ventilation handbook. European Respiratory Society Practical. Handbook, 2015.
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