Skip to main content
SpringerLink
Log in

Non-invasive Ventilation in NeuroMuscular Diseases

  • Chapter
  • First Online:
Sleep Issues in Neuromuscular Disorders

Abstract

Neuromuscular diseases result in respiratory failure due to insufficient ventilation from respiratory muscle weakness. In patients without bulbar affection, non-invasive ventilation (NIV) can be used as an alternative to ventilation, usually via tracheostomy. When used in conjunction with cough assist methods and techniques to sustain and improve clearance of respiratory secretions, NIV can be used both day and night. Mouthpiece ventilation (MPV), also known as sips ventilation, first reported at a post-poliomyelitis respiratory equipment conference in 1953 has also enabled better quality of life. When initiated prior to development of hypercapnia in early myasthenia crisis, NIV decreases the rate of intubation and shortens the duration of hospitalization. Choice of NIV for chronic progressive NMDs must be based on the knowledge and assessment of the ventilatory abnormality for that specific diagnosis.

This is a preview of subscription content, log in via an institution to check access.

Access this chapter

Log in via an institution
Chapter
USD 29.95
Price excludes VAT (USA)
  • Available as PDF
  • Read on any device
  • Instant download
  • Own it forever
eBook
USD 54.99
Price excludes VAT (USA)
  • Available as EPUB and PDF
  • Read on any device
  • Instant download
  • Own it forever
Softcover Book
USD 69.99
Price excludes VAT (USA)
  • Compact, lightweight edition
  • Dispatched in 3 to 5 business days
  • Free shipping worldwide - see info
Hardcover Book
USD 99.99
Price excludes VAT (USA)
  • Durable hardcover edition
  • Dispatched in 3 to 5 business days
  • Free shipping worldwide - see info

Tax calculation will be finalised at checkout

Purchases are for personal use only

Institutional subscriptions

References

  1. Miller RG, Jackson CE, Kasarskis EJ, England JD, Forshew D, Johnston W, et al. Practice parameter update: the care of the patient with amyotrophic lateral sclerosis: drug, nutritional, and respiratory therapies (an evidence-based review): report of the Quality Standards Subcommittee of the American Academy of Neurology. Neurology. 2009;73(15):1218–26.

    Article  CAS  Google Scholar 

  2. Benditt JO, Boitano LJ. Pulmonary issues in patients with chronic neuromuscular disease. Am J Respir Crit Care Med. 2013;187(10):1046–55.

    Article  Google Scholar 

  3. Perrin C, Unterborn JN, Ambrosio CD, Hill NS. Pulmonary complications of chronic neuromuscular diseases and their management. Muscle Nerve. 2004;29(1):5–27.

    Article  Google Scholar 

  4. Bach JR, Goncalves MR, Hon A, Ishikawa Y, De Vito EL, Prado F, et al. Changing trends in the management of end-stage neuromuscular respiratory muscle failure: recommendations of an international consensus. Am J Phys Med Rehabil. 2013;92(3):267–77.

    Article  Google Scholar 

  5. Aboussouan LS. Sleep-disordered breathing in neuromuscular disease. Am J Respir Crit Care Med. 2015;191(9):979–89.

    Article  Google Scholar 

  6. Rabinstein AA. Noninvasive ventilation for neuromuscular respiratory failure: when to use and when to avoid. Curr Opin Crit Care. 2016;22(2):94–9.

    PubMed  Google Scholar 

  7. Gregoretti C, Pisani L, Cortegiani A, Ranieri VM. Noninvasive ventilation in critically ill patients. Crit Care Clin. 2015;31(3):435–57.

    Article  Google Scholar 

  8. Barrett KE, Barman SM, Boitano S, Brooks HL. Regulation of respiration. Ganong’s review of medical physiology. 25th ed. McGraw-Hill Education: New York; 2016.

    Google Scholar 

  9. Shneerson J, Simonds A. Noninvasive ventilation for chest wall and neuromuscular disorders. Eur Respir J. 2002;20(2):480–7.

    Article  CAS  Google Scholar 

  10. Ambrosino N, Carpene N, Gherardi M. Chronic respiratory care for neuromuscular diseases in adults. Eur Respir J. 2009;34(2):444–51.

    Article  CAS  Google Scholar 

  11. Papastamelos C, Panitch HB, Allen JL. Chest wall compliance in infants and children with neuromuscular disease. Am J Respir Crit Care Med. 1996;154(4 Pt 1):1045–8.

    Article  CAS  Google Scholar 

  12. Poponick JM, Jacobs I, Supinski G, DiMarco AF. Effect of upper respiratory tract infection in patients with neuromuscular disease. Am J Respir Crit Care Med. 1997;156(2):659–64.

    Article  CAS  Google Scholar 

  13. Tzeng AC, Bach JR. Prevention of pulmonary morbidity for patients with neuromuscular disease. Chest. 2000;118(5):1390–6.

    Article  CAS  Google Scholar 

  14. Bach JR, Rajaraman R, Ballanger F, Tzeng AC, Ishikawa Y, Kulessa R, et al. Neuromuscular ventilatory insufficiency: effect of home mechanical ventilator use v oxygen therapy on pneumonia and hospitalization rates. Am J Phys Med Rehabil. 1998;77(1):8–19.

    Article  CAS  Google Scholar 

  15. Lemay J, Sériès F, Sénéchal M, Maranda B, Maltais F. Unusual respiratory manifestations in two young adults with Duchenne muscular dystrophy. Can Respir J. 2012;19(1):37.

    Article  Google Scholar 

  16. Renard D, Humbertclaude V, Labauge P. Macroglossia in adult Duchenne muscular dystrophy. Acta Neurol Belg. 2010;110(3):288.

    CAS  PubMed  Google Scholar 

  17. Barbe F, Quera-Salva M, McCann C, Gajdos P, Raphael J, de Lattre J, et al. Sleep-related respiratory disturbances in patients with Duchenne muscular dystrophy. Eur Respir J. 1994;7(8):1403–8.

    Article  CAS  Google Scholar 

  18. Rabinstein AA, Wijdicks EFM. Warning signs of imminent respiratory failure in neurological patients. Semin Neurol. 2003;23(1):97–104.

    Article  Google Scholar 

  19. Clinical indications for noninvasive positive pressure ventilation in chronic respiratory failure due to restrictive lung disease, COPD, and nocturnal hypoventilation—a consensus conference report. Chest. 1999;116(2):521–34.

    Google Scholar 

  20. Ozsancak A, D’Ambrosio C, Hill NS. Nocturnal noninvasive ventilation. Chest. 2008;133(5):1275–86.

    Article  Google Scholar 

  21. Nava S, Hill N. Non-invasive ventilation in acute respiratory failure. Lancet. 2009;374(9685):250–9.

    Article  Google Scholar 

  22. Bach JR. Continuous noninvasive ventilation for patients with neuromuscular disease and spinal cord injury. Semin Respir Crit Care Med. 2002;23(3):283–92.

    Article  Google Scholar 

  23. Bach JR, Alba AS, Saporito LR. Intermittent positive pressure ventilation via the mouth as an alternative to tracheostomy for 257 ventilator users. Chest. 1993;103(1):174–82.

    Article  CAS  Google Scholar 

  24. Toussaint M, Steens M, Wasteels G, Soudon P. Diurnal ventilation via mouthpiece: survival in end-stage Duchenne patients. Eur Respir J. 2006;28(3):549–55.

    Article  CAS  Google Scholar 

  25. Affeldt J. Roundtable conference on poliomyelitis equipment. New York: National Foundation for Infantile Paralysis-March of Dimes White Plains; 1953.

    Google Scholar 

  26. Servera E, Sancho J, Zafra MJ, Catala A, Vergara P, Marin J. Alternatives to endotracheal intubation for patients with neuromuscular diseases. Am J Phys Med Rehabil. 2005;84(11):851–7.

    Article  Google Scholar 

  27. Toussaint M, Chatwin M, Soudon P. Mechanical ventilation in Duchenne patients with chronic respiratory insufficiency: clinical implications of 20 years published experience. Chron Respir Dis. 2007;4(3):167–77.

    Article  CAS  Google Scholar 

  28. Rabinstein A, Wijdicks EF. BiPAP in acute respiratory failure due to myasthenic crisis may prevent intubation. Neurology. 2002;59(10):1647–9.

    Article  Google Scholar 

  29. Vianello A, Bevilacqua M, Arcaro G, Gallan F, Serra E. Non-invasive ventilatory approach to treatment of acute respiratory failure in neuromuscular disorders. A comparison with endotracheal intubation. Intensive Care Med. 2000;26(4):384–90.

    Article  CAS  Google Scholar 

  30. Vianello A, Corrado A, Arcaro G, Gallan F, Ori C, Minuzzo M, et al. Mechanical insufflation-exsufflation improves outcomes for neuromuscular disease patients with respiratory tract infections. Am J Phys Med Rehabil. 2005;84(2):83–8; discussion 9–91.

    Article  Google Scholar 

  31. Aboussouan LS, Khan SU, Meeker DP, Stelmach K, Mitsumoto H. Effect of noninvasive positive-pressure ventilation on survival in amyotrophic lateral sclerosis. Ann Intern Med. 1997;127(6):450–3.

    Article  CAS  Google Scholar 

  32. McKim DA, Road J, Avendano M, Abdool S, Cote F, Duguid N, et al. Home mechanical ventilation: a Canadian Thoracic Society clinical practice guideline. Can Respir J. 2011;18(4):197–215.

    Article  Google Scholar 

  33. Bourke SC, Tomlinson M, Williams TL, Bullock RE, Shaw PJ, Gibson GJ. 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(2):140–7.

    Article  Google Scholar 

  34. Seneviratne J, Mandrekar J, Wijdicks EF, Rabinstein AA. Noninvasive ventilation in myasthenic crisis. Arch Neurol. 2008;65(1):54–8.

    Article  Google Scholar 

  35. Bach JR, Gonçalves MR, Hamdani I, Winck JC. Extubation of patients with neuromuscular weakness: a new management paradigm. Chest. 2010;137(5):1033–9.

    Article  Google Scholar 

  36. Bourke SC, Bullock RE, Williams TL, Shaw PJ, Gibson GJ. Noninvasive ventilation in ALS: indications and effect on quality of life. Neurology. 2003;61(2):171–7.

    Article  CAS  Google Scholar 

  37. Lechtzin N, Scott Y, Busse AM, Clawson LL, Kimball R, Wiener CM. Early use of non-invasive ventilation prolongs survival in subjects with ALS. Amyotroph Lateral Scler. 2007;8(3):185–8.

    Article  Google Scholar 

Download references

Conflict of Interest

None.

Author information

Authors and Affiliations

  1. Divisions of Pulmonary, Critical Care and Infectious Diseases, Department of Medicine, University of Missouri, Columbia, MO, USA

    Hariharan Regunath M.D.

  2. Respiratory Therapy Services, University of Missouri Hospital and Clinics, Columbia, MO, USA

    Troy Whitacre R.R.T.

  3. Division of Pulmonary, Critical Care and Environmental Medicine, Department of Medicine, University of Missouri, Columbia, MO, USA

    Stevan P. Whitt M.D.

Authors
  1. Hariharan Regunath M.D.
    View author publications

    You can also search for this author in PubMed Google Scholar

  2. Troy Whitacre R.R.T.
    View author publications

    You can also search for this author in PubMed Google Scholar

  3. Stevan P. Whitt M.D.
    View author publications

    You can also search for this author in PubMed Google Scholar

Corresponding author

Correspondence to Hariharan Regunath M.D. .

Editor information

Editors and Affiliations

  1. Department of Neurology, University of Missouri, Columbia, Missouri, USA

    Raghav Govindarajan

  2. Department of Neurology, University of Missouri , Columbia, Missouri, USA

    Pradeep C. Bollu

Rights and permissions

Reprints and permissions

Copyright information

© 2018 Springer International Publishing AG, part of Springer Nature

About this chapter

Check for updates. Verify currency and authenticity via CrossMark

Cite this chapter

Regunath, H., Whitacre, T., Whitt, S.P. (2018). Non-invasive Ventilation in NeuroMuscular Diseases. In: Govindarajan, R., Bollu, P. (eds) Sleep Issues in Neuromuscular Disorders. Springer, Cham. https://doi.org/10.1007/978-3-319-73068-4_9

Download citation

  • DOI: https://doi.org/10.1007/978-3-319-73068-4_9

  • Published:

  • Publisher Name: Springer, Cham

  • Print ISBN: 978-3-319-73067-7

  • Online ISBN: 978-3-319-73068-4

  • eBook Packages: MedicineMedicine (R0)

Publish with us

Policies and ethics

Search

Navigation