Skip to main content
SpringerLink
Log in

Myasthenic Crisis

  • Chapter
Critical Care Neurology and Neurosurgery

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

Abstract

Myasthenia gravis (MG) is an autoimmune disorder caused by antibodies directed against the acetylcholine receptor (AchR) of skeletal muscle (1,2). The disease carries a 2:1 female to male predominance and follows a bimodal age distribution, peaking before age 50 among women and again in late midlife among men (3,4). Older cases of newly diagnosed myasthenia are more commonly associated with thymoma. Myasthenic crisis, defined as respiratory failure requiring mechanical ventilation, is a potentially life-threatening complication that occurs in approx 15–20% of patients (2,5). An even greater percentage of all patients affected with the disease experience some degree of respiratory muscle weakness (2,5,6).

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 169.00
Price excludes VAT (USA)
  • Available as EPUB and PDF
  • Read on any device
  • Instant download
  • Own it forever
Softcover Book
USD 219.99
Price excludes VAT (USA)
  • Compact, lightweight edition
  • Dispatched in 3 to 5 business days
  • Free shipping worldwide - see info
Hardcover Book
USD 299.99
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

Preview

Unable to display preview. Download preview PDF.

Unable to display preview. Download preview PDF.

References

  1. Drachman D. Myasthenia gravis. N. Engl. J. Med. 1994; 330: 1797–1810.

    Article  PubMed  CAS  Google Scholar 

  2. Thomas CE, Mayer SA, Gungor Y, et al. Myasthenic crisis: clinical features, mortality, complications, and risk factors for prolonged intubation. Neurology 1997; 48: 1253–1260.

    Article  PubMed  CAS  Google Scholar 

  3. Pascuzzi R. Pearls and pitfalls in the diagnosis and management of neuromuscular junction disorders. Semin. Neurol. 2001; 21: 425–440.

    Article  PubMed  CAS  Google Scholar 

  4. Yavagal DR, Mayer SA. Respiratory complications of rapidly progressive neuromuscular syndromes: Guillain-Barre syndrome and myasthenia gravis. Semin. Resp. Crit. Care Med. 2002; 23: 221–228.

    Article  Google Scholar 

  5. Fink ME. Treatment of the critically ill patient with myasthenia gravis. In: Ropper AH (ed). Neurological and Neurosurgical Intensive Care, 3rd ed. New York: Raven Press, 1993: 351–362.

    Google Scholar 

  6. Mayer SA. Intensive care of the myasthenic patient. Neurology 1997; 48 (Supp 5): S70–S75.

    Article  Google Scholar 

  7. Saltis LM, Martin BR, Traeger SM, Bonfiglio MF. Continuous infusion of pyridogstigmine in the management of myasthenic crisis. Crit. Care Med. 1993; 21: 938–940.

    Article  PubMed  CAS  Google Scholar 

  8. Stricker RB, Kwiatkowska BJ, Habis JA, Kiprov DD. Myasthenic crisis: response to plasmapheresis following failure of intravenous gamma-globulin. Arch. Neurol. 1993; 50: 837–840.

    Article  PubMed  CAS  Google Scholar 

  9. Thomas CE, Mayer SA, Gungor Y, et al. Effect of age on the course and outcome of myasthenic crisis [abstract]. Crit. Care Med. 1996; 24 (suppl): A70.

    Google Scholar 

  10. Donaldson DH, Ansher M, Horan S, Rutherford RB, Ringel SP. The relationship of age to outcome in myasthenia gravis. Neurology 1990; 40: 786–790.

    Article  PubMed  CAS  Google Scholar 

  11. Lewis, JE, Wick MR, Scheithauer BW, Bernatz PE, Taylor WF. Thymoma. Cancer 1987; 60: 2727–2743.

    Article  PubMed  CAS  Google Scholar 

  12. Abramsky O, Aharanov A, Webb C, et al. Cellular immune response to acetylcholine receptor-rich fraction in patients with myasthenia gravis. Clin. Exp. Immunol. 1975; 19; 11–16.

    PubMed  CAS  Google Scholar 

  13. Aharanov A, Tarrah-Hazdai E, Abramsky O, et al. Humoral antibodies to acetylcholine receptors in patients with myasthenia gravis. Lancet 1975; 2: 340–342.

    Article  Google Scholar 

  14. Almon RR, Andrew CG, Appel SH. Serum globulin in myasthenia gravis: Inhibitor of bungarotoxin binding to acetylcholine receptors. Science 1974; 186: 55–57.

    Article  PubMed  CAS  Google Scholar 

  15. Bender AN, Ringel SP, Engel WH, et al. Myasthenia gravis: a serum fraction blocking acetylcholine receptors of human neuromuscular junction. Lancet 1975; 1: 607–609.

    Article  PubMed  CAS  Google Scholar 

  16. Lindstrom JM, Lennon VA, Seybold MR, et al. Experimental autoimmune myasthenia and myasthenia gravis. Biochemical and immunological aspects. Ann. NY Acad. Sci. 1976; 274: 254–174.

    Article  PubMed  CAS  Google Scholar 

  17. Richman DP, Antel JP, Patrick JW, Anrason BG. Cellular immunity to acetylcholine receptor in myasthenia gravis: Relationship to histocompatibility type and antigenic site. Neurology 1979; 29: 291–296.

    Article  PubMed  CAS  Google Scholar 

  18. Richman DP, Patrick J, Arnason BGW. Cellular immunity in myasthenia gravis: response to purified acetylcholine receptor and autologous thymocytes. N. Engl. J. Med. 1976; 294: 694–698.

    Article  PubMed  CAS  Google Scholar 

  19. Toyka KV, Drachman DB, Pestronk A, et al. Myasthenia gravis: Passive transfer from man to mouse. Science 1975; 1990: 397–399.

    Google Scholar 

  20. Cohen MS, Younger D. Aspects of the natural history of myasthenia gravis: Crisis and death. Ann. NY Acad. Sci. 1981; 377: 670–677.

    Article  PubMed  CAS  Google Scholar 

  21. Ferguson IT, Murphy RP, Lascelles RG. Ventilatory failure in myasthenia gravis. J. Neurol. Neurosurg. Psychiatry 1982; 45: 217–222.

    Article  PubMed  CAS  Google Scholar 

  22. Sellman MS, Mayer RF. Treatment of myasthenic crisis in late life. South Med. J. 1985; 78: 1208–1210.

    Article  PubMed  CAS  Google Scholar 

  23. Burke ME. Myasthenia gravis and pregnancy. J. Perinat. Neonatal Nurs. 1993; 7: 11–21.

    PubMed  CAS  Google Scholar 

  24. Tanwani LK, Lohano V, Ewart R, Broadstone VL, Mokshagundam SP. Myasthenia gravis in conjunction with Graves’ disease: A diagnostic challenge. Endocr. Pract. 2001; 7: 275–278.

    PubMed  CAS  Google Scholar 

  25. Manthous, Hall JB. Liberation from mechanical ventilation, a decade in progress. Chest 1998; 114: 886–901.

    Article  PubMed  CAS  Google Scholar 

  26. Jenkins R, Witorsch P, Smyth NP. Aspects of treatment in myasthenia gravis. South Med. J. 1970; 63: 1127–1130.

    Article  PubMed  CAS  Google Scholar 

  27. Osserman KE, Genkins G. Studies in myasthenia gravis: Reduction in mortality rate after crisis. JAMA 1963; 183: 97–101.

    PubMed  CAS  Google Scholar 

  28. Tether JE. Management of myasthenic and cholinergic crises. Am. J. Med. 1955; 19: 740–742.

    Article  PubMed  CAS  Google Scholar 

  29. Hoch W, McConville J, Helms S, Newsom-Davis J, Melms A, Vincent A. Auto-antibodies to the receptor tyrosine kinase MuSK in patients with myasthenia gravis without acetylcholine receptor antibodies. Nat. Med. 2001; 7: 365–368.

    Article  PubMed  CAS  Google Scholar 

  30. Lubke E, Freiburg A, Skeie GO, et al. Striational autoantibodies in myasthenia gravis patients recognize I-band titin epitopes. J. Neuroimmunol. 1998; 81: 98–108.

    Article  PubMed  CAS  Google Scholar 

  31. Howard FM Jr, Lennon VA, Finley J, Matsumoto J, Elveback LR. Clinical correlations of antibodies that bind, block, or modulate human acetylcholine receptors in myasthenia gravis. Ann. NY Acad. Sci. 1987; 505: 526–538.

    Article  PubMed  Google Scholar 

  32. Somnier FE, Engel PJ. The occurrence of anti-titin antibodies and thymomas: A population survey of MG 1970–1999. Neurology 2002; 59: 92–98.

    Article  PubMed  Google Scholar 

  33. Yamamoto AM, Gajdos P, Eymard B, et al. Anti-titin antibodies in myasthenia gravis; tight association with thymoma and heterogeneity of nonthymoma patients. Arch. Neurol. 2001; 58: 885–890.

    Article  PubMed  CAS  Google Scholar 

  34. Phillips LH 2nd, Melnick PA. Diagnosis of myasthenia gravis in the 1990s. Semin. Neurol. 1990; 10: 62–69.

    Article  Google Scholar 

  35. DePippo KL, Holas MA, Reding MJ. Validation of the 3 oz water swallow test for aspiration after stroke. Arch. Neurol. 1992; 49: 1259–1261.

    Article  PubMed  CAS  Google Scholar 

  36. Gracey DR, Divertie MB, Howard FM. Mechanical ventilation for respiratory failure in myasthenia gravis: two-year experience with 22 patients. Mayo Clin. Proc. 1983; 58: 597–602.

    PubMed  CAS  Google Scholar 

  37. Nomori H, Ishihara T. Pressure-controlled ventilation via a mini -tracheostomy tube for patients with neuromuscular diesase. Neurology 2000; 55: 698–702.

    Article  PubMed  CAS  Google Scholar 

  38. Kress JP, Pohlman AS, O’Connor MF, Hall JB. Daily interruption of sedative infusions in critically ill patients undertiong mecha nical ventilation. N. Engl. J. Med. 2000; 342: 1471–1477.

    Article  PubMed  CAS  Google Scholar 

  39. Berrouschot J, Baumann I, Kalichewski P. Therapy of myasthenic crisis. Crit. Care Med. 1997; 25: 1228–1235.

    Article  PubMed  CAS  Google Scholar 

  40. Marshall RS, Mayer SA. On Call Neurology. Philadelphia: WB Saunders; 1997:200.`

    Google Scholar 

  41. Thorlacius S, Mollnes TE, Garred P, et al. Plasma exchange in myasthenia gravis: changes in serum complement and immunoglobulins. Acta. Neurol. Scand. 1988; 78: 221–227.

    Article  PubMed  CAS  Google Scholar 

  42. Grob D, Arsura EL, Brunner NG, Namba T. The course of myasthenia gravis and therapies affecting outcome. Ann. NY Acad. Sci. 1987; 505: 472–499.

    Article  PubMed  CAS  Google Scholar 

  43. Ronager J, Ravngborg M, Vorstrup S. Clinical effects of high dose intravenous immunoglobulin compared to plasma exchange in patients with moderate to severe myasthenia gravis. American Academy of Neurology 51st Annual Meeting: Scientific Program, Scientific Sessions. 1999; 52S: 184–185.

    Google Scholar 

  44. Qureshi AI, Choundry MA, Akbar MS, et al. Plasma exchange versus intravenous immunoglobulin treatment in myasthenic crisis. Neurology 1999; 52: 629–632.

    Article  PubMed  CAS  Google Scholar 

  45. Depierraz B, Essinger A, Morin D, Goy JJ, Buchser E. Isolated phrenic nerve injury after apparently atraumatic puncture of the internal jugular vein. Intensive Care Med. 1989; 15: 132–134.

    Article  PubMed  CAS  Google Scholar 

  46. Hadeed HA, Braun TW. Paralysis of the hemidiaphragm as a complication of internal jugular vein cannulation: A report of a case. J. Oral Maxillofac. Surg. 1988; 46: 409–411.

    Article  PubMed  CAS  Google Scholar 

  47. Topaz O, Sharon M, Rechavia E, Mager A, Chetbuon I. Traumatic internal jugular vein cannulation. Ann. Emerg. Med. 1987; 16: 1394–1395.

    Article  PubMed  CAS  Google Scholar 

Download references

Authors
  1. Nazli Janjua
    View author publications

    You can also search for this author in PubMed Google Scholar

  2. Stephan A. Mayer
    View author publications

    You can also search for this author in PubMed Google Scholar

Editor information

Editors and Affiliations

  1. Neurosciences Critical Care, University Hospitals of Cleveland, Case Western Reserve University, Cleveland, OH, USA

    Jose I. Suarez MD

Rights and permissions

Reprints and permissions

Copyright information

© 2004 Springer Science+Business Media New York

About this chapter

Cite this chapter

Janjua, N., Mayer, S.A. (2004). Myasthenic Crisis. In: Suarez, J.I. (eds) Critical Care Neurology and Neurosurgery. Current Clinical Neurology. Humana Press, Totowa, NJ. https://doi.org/10.1007/978-1-59259-660-7_26

Download citation

  • DOI: https://doi.org/10.1007/978-1-59259-660-7_26

  • Publisher Name: Humana Press, Totowa, NJ

  • Print ISBN: 978-1-61737-350-3

  • Online ISBN: 978-1-59259-660-7

  • eBook Packages: Springer Book Archive

Publish with us

Policies and ethics

Search

Navigation