The Southern Medical Journal (SMJ) is the official, peer-reviewed journal of the Southern Medical Association. It has a multidisciplinary and inter-professional focus that covers a broad range of topics relevant to physicians and other healthcare specialists.

SMJ // Article

Expired CME Article

Myasthenia Gravis Crisis

Authors: Eric M. Bershad, MD, Eliahu S. Feen, MD, Jose I. Suarez, MD

Abstract

Myasthenia gravis (MG) is an autoimmune disorder resulting from the production of antibodies against acetylcholine receptors leading to the destruction of the postsynaptic membrane at the neuromuscular junction. In the US there are about 18,000 people with MG. Myasthenia gravis crisis (MGC) is defined as any MG exacerbation necessitating mechanical ventilation. Most patients presenting with MGC have an identifiable risk factor. The diagnosis of MGC should be suspected in all patients with respiratory failure, particularly those with unclear etiology. Acute management of MGC requires supportive general and ventilatory therapy and institution of measures to improve the neuromuscular blockade. The latter includes plasma exchange or IV immunoglobulin, and removal of the offending trigger. The outcome of patients with MGC has improved significantly and the current mortality rate is about 4 to 8%.


Key Points


* Myasthenia gravis crisis (MGC) is defined as any myasthenia gravis (MG) exacerbation necessitating mechanical ventilation.


* About 15–20% of patients with myasthenia gravis will experience MGC, typically within the first 2 years of MG diagnosis.


* Most patients with myasthenia gravis crisis have an identifiable precipitating event; however in 30–40% of patients, no triggering factor is found.


* Plasma exchange or intravenous immunoglobulins are indicated to help facilitate recovery of neuromuscular blockade.


* The overall outcome for patients with myasthenia gravis crisis is excellent if therapeutic and supportive measures are instituted promptly.

This content is limited to qualifying members.

Existing members, please login first

If you have an existing account please login now to access this article or view purchase options.

Purchase only this article ($25)

Create a free account, then purchase this article to download or access it online for 24 hours.

Purchase an SMJ online subscription ($75)

Create a free account, then purchase a subscription to get complete access to all articles for a full year.

Purchase a membership plan (fees vary)

Premium members can access all articles plus recieve many more benefits. View all membership plans and benefit packages.

References

1. Kuks J, Oosterhuis H. Clinical presentation and epidemiology of myasthenia gravis. In: Kaminski H, ed. Myasthenia Gravis and Related Disorders. Totowa, Humana Press, 2003, pp 107–113.
 
2. Filho J, Suarez J. Neurocritical care of myasthenia gravis crisis. In: Kaminski H, ed. Myasthenia Gravis and Related Disorders. Totowa, Humana Press, 2003, pp223–234.
 
3. Bedlack RS, Sanders DB. How to handle myasthenic crisis: essential steps in patient care. Postgrad Med 2000;107:211–212.
 
4. 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.
 
5. Borodic G. Myasthenic crisis after botulinum toxin. Lancet 1998;352:1832.
 
6. Mayer SA. Intensive care of the myasthenic patient. Neurology 1997;48(Suppl 5):S70–S75.
 
7. Wittbrodt ET. Drugs and myasthenia gravis: an update. Arch Intern Med 1997;157:399–408.
 
8. Johns TR. Long-term corticosteroid treatment of myasthenia gravis. Ann N Y Acad Sci 1987;505:568–583.
 
9. Drachman DB. Myasthenia gravis. N Engl J Med 1994;330:1797–1810.
 
10. Ruff R. Neuromuscular junction physiology and pathophysiology. In: Kaminski H, ed. Myasthenia Gravis and Related Disorders. Totowa, Humana Press, 2003, pp 1–13.
 
11. Mygland A, Santillan C, Kaminski HJ. Autoantibody testing of autoimmune neuromuscular junction, hyperexcitability, and muscle disorders. In: Katirji B, Kaminski HJ, Preston DC, Ruff RL, Shapiro BE, eds. Neuromuscular Disorders in Clinical Practice. Boston, Butterworth Heinemann, 2002, pp 64–73.
 
12. Agius MA, Richman DP, Vincent A. Specific antibodies in the diagnosis and management of autoimmune disorders of neuromuscular transmission and related diseases. In: Kaminski H, ed. Myasthenia Gravis and Related Disorders. Totowa, Humana Press, 2003, pp 177–196.
 
13. Qureshi AI, Choundry MA, Mohammad Y, et al. Respiratory failure as a first presentation of myasthenia gravis. Med Sci Monit 2004; 10:CR684–CR689.
 
14. Putman MT, Wise RA. Myasthenia gravis and upper airway obstruction. Chest 1996;109:400–404.
 
15. Itoh H, Shibata K, Nitta S. Sensitivity to vecuronium in seropositive and seronegative patients with myasthenia gravis. Anesth Analg 2002;95:109–113.
 
16. 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;342:1301–1308.
 
17. Mayer SA, Thomas CE. Therapy of myasthenic crisis. Crit Care Med 1998;26:1136–1137.
 
18. Qureshi AI, Choudhry MA, Akbar MS, et al. Plasma exchange versus intravenous immunoglobulin treatment in myasthenic crisis. Neurology 1999;52:629–632.
 
19. Gajdos P, Chevret S, Clair B, et al. Clinical trial of plasma exchange and high-dose intravenous immunoglobulin in myasthenia gravis: Myasthenia Gravis Clinical Study Group. Ann Neurol 1997;41:789–796.
 
20. Gajdos P, Tranchant C, Clair B, et al. Treatment of myasthenia gravis exacerbation with intravenous immunoglobulin: a randomized double-blind clinical trial. Arch Neurol 2005;62:1689–1693.
 
21. Eibl MM. Intravenous immunoglobulins in neurological disorders: safety issues. Neurol Sci 2003;24(Suppl 4):S222–S226.
 
22. Dalakas MC, Clark WM. Strokes, thromboembolic events, and IVIg: rare incidents blemish an excellent safety record. Neurology 2003;60:1736–1737.
 
23. Dalakas MC. Intravenous immunoglobulin in the treatment of autoimmune neuromuscular diseases: present status and practical therapeutic guidelines. Muscle Nerve 1999;22:1479–1497.
 
24. Berrouschot J, Baumann I, Kalischewski P, et al. Therapy of myasthenic crisis. Crit Care Med 1997;25:1228–1235.
 
25. Kollef MH. Prevention of hospital-associated pneumonia and ventilator-associated pneumonia. Crit Care Med 2004;32:1396–1405.
 
26. Reardon CC, Christiansen D, Barnett ED, et al. Intrapulmonary percussive ventilation vs incentive spirometry for children with neuromuscular disease. Arch Pediatr Adolesc Med 2005;159:526–531.
 
27. Kamran SI, Downey D, Ruff RL. Pneumatic sequential compression reduces the risk of deep vein thrombosis in stroke patients. Neurology 1998;50:1683–1688.
 
28. Wijdicks F. Management of airway and mechanical ventilation. In: Wijdicks F, ed. The Clinical Practice of Critical Care Neurology. Philadelphia, Lippincott-Raven, 1997, pp 25–45.
 
29. Kaminski H. Treatment of Myasthenia Gravis. Myasthenia Gravis and Related Disorders. Totowa, Humana Press, 2003, pp 197–221.
 
30. Gronseth GS, Barohn RJ. Practice parameter: thymectomy for autoimmune myasthenia gravis (an evidence-based review): report of the Quality Standards Subcommittee of the American Academy of Neurology. Neurology 2000;55:7–15.
 
31. Gronseth GS, Barohn RJ. Thymectomy for myasthenia gravis. Curr Treat Options Neurol 2002;4:203–209.