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Myasthenia Gravis and Related Disorders pp 189–197Cite as

Neurocritical Care of Myasthenic Crisis

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Part of the book series: Current Clinical Neurology ((CCNEU))

Abstract

Myasthenic crisis (MC) is the most severe clinical manifestation of myasthenia gravis (MG). MC is defined as worsening MG symptoms which lead to respiratory failure and institutions of mechanical ventilation. MC is most commonly precipitated by infections (30–40% of cases). MC may occur in patients previously diagnosed but also may be the presenting event of MG. Therefore, it should be considered in every patient with respiratory failure of unknown cause particularly those with difficulty weaning off the mechanical ventilator. It is common practice to initiate a trial of noninvasive positive ventilation as a temporizing measure in patients with MG exacerbation who are expected to recover quickly and in whom immunotherapy can be instituted promptly. Once mechanical ventilation is started, patients should be treated with lung-protective ventilation (low tidal volumes, PEEP, and plateau pressures <30 cm H2O). Plasma exchange is the preferred initial treatment in the ICU to improve muscle strength and respiratory status.

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References

  1. Berrouschot J, Baumann I, Kalischewski P, Sterker M, Schneider D. Therapy of myasthenic crisis. Crit Care Med. 1997;25:1228–35.

    Article  CAS  PubMed  Google Scholar 

  2. Vern JC. Myasthenia gravis: management of myasthenic crisis and preoperative care. Semin Neurol. 2004;24:75–81.

    Article  Google Scholar 

  3. Thomas CE, Mayer SA, Gungor Y, Swarup R, Webster EA, Chang I, et al. Myasthenic crisis: clinical features, mortality, complications, and risk factors for prolonged intubation. Neurology. 1997;48:1253–60.

    Article  CAS  PubMed  Google Scholar 

  4. Mayer SA. Intensive care of the myasthenic patient. Neurology. 1997;48(Suppl 5):S70–5.

    Article  Google Scholar 

  5. Bedlack RS, Sanders DB. How to handle myasthenic crisis. Postgrad Med. 2000;107(4):211–4, 220–2.

    Article  CAS  PubMed  Google Scholar 

  6. Weijnen FG, Van Der Bilt A, Wokke JHJ, Wassenberg MWM, Oudenaarde I. Oral functions of patients with myasthenia gravis. Ann N Y Acad Sci. 1998;841:773–6.

    Article  CAS  PubMed  Google Scholar 

  7. Wittbrodt ET. Drugs and myasthenia gravis. Arch Intern Med. 1997;157:399–408.

    Article  CAS  PubMed  Google Scholar 

  8. Pascuzzi RM. Medications and myasthenia gravis: a reference for health care professionals. http://www.myathenia.org/drugs/reference.htm.

  9. Perrot X, Bernard N, Vial C, Antoine JC, Laurent H, Vial T, et al. Myasthenia gravis exacerbation or unmasking associated with telithromycin treatment. Neurology. 2006;67:1–3.

    Article  Google Scholar 

  10. Jennet AM, Bali D, Jasti P, Shah B, Browning LA. Telithromycin and myasthenia crisis. Clin Infect Dis. 2006;43:1621–2.

    Article  Google Scholar 

  11. Grob D. Natural history of myasthenia gravis. In: Engel AG, editor. Myathenia gravis and myasthenic disorders. New York: Oxford University Press; 1999. p. 131–45.

    Google Scholar 

  12. Borodic G. Myasthenic crisis after botulinum toxin. Lancet. 1998;352:1832.

    Article  CAS  PubMed  Google Scholar 

  13. Weng YY, Yang DH, Qian MZ, Wei MM, Yin F, Li J, et al. Low serum albumin concentrations are associated with disease severity in patients with myasthenia gravis. Medicine. 2016;95(39):e5000.

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  14. Li KK, Qian K, Feng YG, Guo W, Tan QY, Deng B. Predictive factors of prolonged mechanical ventilation, overall survival, and quality of life in patients with post-thymectomy myasthenic crisis. World J Surg Oncol. 2017;15:150. https://doi.org/10.1186/s12957-017-1209-1.

    Article  PubMed  PubMed Central  Google Scholar 

  15. Ropper AH. The Guillain-Barré syndrome. N Engl J Med. 1992;326:1130–6.

    Article  CAS  PubMed  Google Scholar 

  16. Putman MT, Wise RA. Myasthenia gravis and upper airway obstruction. Chest. 1996;109:400–4.

    Article  CAS  PubMed  Google Scholar 

  17. Cridge PB, Allegra J, Gerhard H. Myasthenic crisis presenting as isolated vocal cord paralysis. Am J Emerg Med. 2000;18:232–3.

    Article  CAS  PubMed  Google Scholar 

  18. DePippo KL, Holas MA, Reding MJ. Validation of the 3 oz water swallow test for aspiration after stroke. Arch Neurol. 1992;49:1259–61.

    Article  CAS  PubMed  Google Scholar 

  19. McDonagh DL, Borel CO. Ventilatory management in the neurosciences critical care unit. In: Suarez JI, editor. Critical care neurology and neurosurgery. Totowa: Humana Press; 2004. p. 151–66.

    Chapter  Google Scholar 

  20. Suarez JI, Boonyapisit K, Zaidat OO, Kaminski HJ, Ruff RL. Predictors of mechanical ventilation in patients with myasthenia gravis exacerbation [abstract]. J Neurol Sci. 2001;187:S464.

    Google Scholar 

  21. Mayer SA, Thomas CE. Therapy of myasthenic crisis. Crit Care Med. 1998;26:1136.

    Article  CAS  PubMed  Google Scholar 

  22. Maher J, Rutledge F, Remtulla H, Parkes A, Bernardi L, Bolton CF. Neuromuscular disorders associated with failure to wean from the ventilator. Intensive Care Med. 1995;21:737–43.

    Article  CAS  PubMed  Google Scholar 

  23. Vita G, Girlanda P, Puglisi RM, Marabello L, Messina C. Cardiovascular-reflex testing and single fiber eletromyography in botulism. Arch Neurol. 1987;44:202–6.

    Article  CAS  PubMed  Google Scholar 

  24. Suarez JI, Cohen ML, Larkins J, Kernich CA, Hricick DE, Daroff RB. Acute intermittent porphyria. Clinicopathologic correlation. Report of a case and review of the literature. Neurology. 1997;48:1678–83.

    Article  CAS  PubMed  Google Scholar 

  25. Torbey MT, Suarez JI, Geocadin R. Less common causes of quadriparesis and respiratory failure. In: Suarez JI, editor. Critical care neurology and neurosurgery. Totowa: Humana Press; 2004. p. 493–513.

    Chapter  Google Scholar 

  26. Bosch EP, Smith BE. Disorders of peripheral nerves. In: Bradley WG, Daroff RB, Fenichel GM, Marsden CD, editors. Neurology in clinical practice, vol. 2. 3rd ed. Boston: Butterworth-Heineman; 2000. p. 2045–130.

    Google Scholar 

  27. Flower O, Wainwright MS, Caulfield AS. Emergency neurological life support: acute non-traumatic weakness. Neurocrit Care. 2015;23(Suppl 2):S23–47. https://doi.org/10.1007/s12028-015-0160-7.

    Article  PubMed  Google Scholar 

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

    Article  PubMed  Google Scholar 

  29. Agarwal R, Reddy C, Gupta D. Noninvasive ventilation in acute neuromuscular respiratory failure due to myasthenic crisis: case report and review of literature. Emerg Med J. 2006;23:e6.

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  30. The Acute Respiratory Distress Syndrome Network. Ventilation with lower tidal volumes as compared with traditional tidal volumes for acute lung injury and the acute respiratory distress syndrome. N Engl J Med. 2000;342:1301–8.

    Article  Google Scholar 

  31. Wijdicks FM. Management of airway and mechanical ventilation. In: Wijdicks FM, editor. The clinical practice of critical care neurology. Philadelphia: Lippincott-Raven; 1997. p. 25–45.

    Google Scholar 

  32. Borel CO, Teitelbaum JS, Hanley DF. Ventilatory drive and carbon dioxide response in ventilatory failure due to myasthenia gravis and Guillain-Barré syndrome. Crit Care Med. 1993;21:1717–26.

    Article  CAS  PubMed  Google Scholar 

  33. Kokontis L, Gutmann L. Current treatment of neuromuscular diseases. Arch Neurol. 2000;57:939–43.

    Article  CAS  PubMed  Google Scholar 

  34. Seybold ME. Treatment of myasthenia gravis. In: Engel AG, editor. Myasthenia gravis and myasthenic disorders. New York: Oxford University Press; 1999. p. 167–91.

    Google Scholar 

  35. Batocchi AP, Evoli A, Di Schino C, Tonali P. Therapeutic apheresis in myasthenia gravis. Ther Apher. 2000;4:275–9.

    Article  CAS  PubMed  Google Scholar 

  36. Gajdos P, Chevret S, Clair B, Tranchant C, Chastang C. Clinical trial of plasma exchange and high-dose intravenous immunoglobulin in myasthenia gravis. Ann Neurol. 1997;41:789–96.

    Article  CAS  PubMed  Google Scholar 

  37. Qureshi AI, Choudhry MA, Akbar MS, Mohammad Y, Chua HC, Yahia AM, et al. Plasma exchange versus intravenous immunoglobulin treatment in myasthenic crisis. Neurology. 1999;52:629–32.

    Article  CAS  PubMed  Google Scholar 

  38. Stricker RB, Kwiatkowska BJ, Habis JA, Kiprov DD. Myasthenic crisis. Response to plasmapheresis following failure of intravenous γ-globulin. Arch Neurol. 1993;50:837–40.

    Article  CAS  PubMed  Google Scholar 

  39. Sanders DB, Wolfe GI, Benatar M, Evoli A, Gilhus NE, Illa I, et al. International consensus guidance for management of myasthenia gravis. Neurology. 2016;87:419–25.

    Article  PubMed  PubMed Central  Google Scholar 

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Author information

Authors and Affiliations

  1. Department of Neurological Sciences, University of Nebraska Medical Center, Omaha, NE, USA

    Jose Americo M. Fernandes MD

  2. Department of Anesthesiology and Critical Care Medicine, Neurology, and Neurosurgery, Johns Hopkins University School of Medicine, Baltimore, MD, USA

    Jose I. Suarez MD

Authors
  1. Jose Americo M. Fernandes MD
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  2. Jose I. Suarez MD
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Correspondence to Jose I. Suarez MD .

Editor information

Editors and Affiliations

  1. Department of Neurology, George Washington University, Washington, DC, USA

    Henry J. Kaminski

  2. Department of Pharmacology and Physiology, George Washington University, Washington, DC, USA

    Linda L. Kusner

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Fernandes, J.A.M., Suarez, J.I. (2018). Neurocritical Care of Myasthenic Crisis. In: Kaminski, H., Kusner, L. (eds) Myasthenia Gravis and Related Disorders. Current Clinical Neurology. Humana Press, Cham. https://doi.org/10.1007/978-3-319-73585-6_12

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  • DOI: https://doi.org/10.1007/978-3-319-73585-6_12

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  • Publisher Name: Humana Press, Cham

  • Print ISBN: 978-3-319-73584-9

  • Online ISBN: 978-3-319-73585-6

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