- Shawn J Bird, MD
Shawn J Bird, MD
- Professor of Neurology
- University of Pennsylvania School of Medicine
- Joshua M Levine, MD
Joshua M Levine, MD
- Associate Professor of Neurology
- Perelman School of Medicine at the University of Pennsylvania
- Section Editors
- Jeremy M Shefner, MD, PhD
Jeremy M Shefner, MD, PhD
- Section Editor — Neuromuscular Disease
- Professor and Chair of Neurology, Barrow Neurological Institute
- Professor of Neurology, University of Arizona, Phoenix
- Clinical Professor of Neurology, Creighton University
- Ira N Targoff, MD
Ira N Targoff, MD
- Section Editor — Muscle Disease
- Professor of Medicine, Section of Rheumatology
- University of Oklahoma Health Sciences Center
- Polly E Parsons, MD
Polly E Parsons, MD
- Editor-in-Chief — Pulmonary and Critical Care Medicine
- Section Editor — Critical Care
- Professor of Medicine
- University of Vermont College of Medicine
Myasthenic crisis is a life-threatening condition, which is defined as weakness from acquired myasthenia gravis that is severe enough to necessitate intubation or to delay extubation following surgery . The respiratory failure is due to weakness of respiratory muscles.
Severe bulbar (oropharyngeal) muscle weakness often accompanies the respiratory muscle weakness, or may be the predominant feature in some patients. When this results in upper airway obstruction or severe dysphagia with aspiration, intubation and mechanical ventilation are necessary.
This topic will review the evaluation and treatment of myasthenic crisis and rapidly worsening myasthenia gravis. Other aspects of myasthenia gravis are discussed separately. (See "Clinical manifestations of myasthenia gravis" and "Diagnosis of myasthenia gravis" and "Treatment of myasthenia gravis".)
Although data are limited, the proportion of patients with myasthenia gravis who experience at least one myasthenic crisis may be as high as 10 to 20 percent , and the annual risk of myasthenic crisis among patients with myasthenia gravis is approximately 2 to 3 percent . In 13 to 20 percent of patients who present with myasthenic crisis, it is the first manifestation of myasthenia gravis [3-5]. Most myasthenic crises occur in the first few years after the diagnosis of myasthenia gravis, when the disease is often in its most active phase. (See "Clinical manifestations of myasthenia gravis", section on 'Clinical course'.)
The epidemiology of myasthenia gravis is reviewed in detail separately. (See "Clinical manifestations of myasthenia gravis", section on 'Epidemiology'.)
- Bedlack RS, Sanders DB. On the concept of myasthenic crisis. J Clin Neuromuscul Dis 2002; 4:40.
- Wendell LC, Levine JM. Myasthenic crisis. Neurohospitalist 2011; 1:16.
- Berrouschot J, Baumann I, Kalischewski P, et al. Therapy of myasthenic crisis. Crit Care Med 1997; 25:1228.
- Rabinstein AA, Mueller-Kronast N. Risk of extubation failure in patients with myasthenic crisis. Neurocrit Care 2005; 3:213.
- O'Riordan JI, Miller DH, Mottershead JP, et al. The management and outcome of patients with myasthenia gravis treated acutely in a neurological intensive care unit. Eur J Neurol 1998; 5:137.
- Mier A, Laroche C, Green M. Unsuspected myasthenia gravis presenting as respiratory failure. Thorax 1990; 45:422.
- Dushay KM, Zibrak JD, Jensen WA. Myasthenia gravis presenting as isolated respiratory failure. Chest 1990; 97:232.
- Carr AS, Hoeritzauer AI, Kee R, et al. Acute neuromuscular respiratory failure: a population-based study of aetiology and outcome in Northern Ireland. Postgrad Med J 2014; 90:201.
- Cabrera Serrano M, Rabinstein AA. Causes and outcomes of acute neuromuscular respiratory failure. Arch Neurol 2010; 67:1089.
- Juel VC. Myasthenia gravis: management of myasthenic crisis and perioperative care. Semin Neurol 2004; 24:75.
- Jani-Acsadi A, Lisak RP. Myasthenic crisis: guidelines for prevention and treatment. J Neurol Sci 2007; 261:127.
- Chaudhuri A, Behan PO. Myasthenic crisis. QJM 2009; 102:97.
- Rabinstein AA, Wijdicks EF. Warning signs of imminent respiratory failure in neurological patients. Semin Neurol 2003; 23:97.
- Bird SJ, Teener JW. Acute neuromuscular weakness. In: The Intensive Care Unit Manual, Lanken PN, Hanson CW, Manaker S (Eds), WB Saunders, Philadelphia 2001. p.755.
- Seneviratne J, Mandrekar J, Wijdicks EF, Rabinstein AA. Noninvasive ventilation in myasthenic crisis. Arch Neurol 2008; 65:54.
- Wu JY, Kuo PH, Fan PC, et al. The role of non-invasive ventilation and factors predicting extubation outcome in myasthenic crisis. Neurocrit Care 2009; 10:35.
- Varelas PN, Chua HC, Natterman J, et al. Ventilatory care in myasthenia gravis crisis: assessing the baseline adverse event rate. Crit Care Med 2002; 30:2663.
- Clark WF, Rock GA, Buskard N, et al. Therapeutic plasma exchange: an update from the Canadian Apheresis Group. Ann Intern Med 1999; 131:453.
- 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.
- Qureshi AI, Choudhry MA, Akbar MS, et al. Plasma exchange versus intravenous immunoglobulin treatment in myasthenic crisis. Neurology 1999; 52:629.
- 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.
- Gajdos P, Chevret S, Toyka KV. Intravenous immunoglobulin for myasthenia gravis. Cochrane Database Syst Rev 2012; 12:CD002277.
- Mandawat A, Kaminski HJ, Cutter G, et al. Comparative analysis of therapeutic options used for myasthenia gravis. Ann Neurol 2010; 68:797.
- Skeie GO, Apostolski S, Evoli A, et al. Guidelines for treatment of autoimmune neuromuscular transmission disorders. Eur J Neurol 2010; 17:893.
- Gilhus NE. Neuromuscular disease: acute treatment for myasthenia gravis. Nat Rev Neurol 2011; 7:132.
- Sathasivam S. Current and emerging treatments for the management of myasthenia gravis. Ther Clin Risk Manag 2011; 7:313.
- Sathasivam S. Evidence for use of intravenous immunoglobulin and plasma exchange in generalised mysasthenia gravis. Adv Clin Neurosci Rehabil 2009; 9:8.
- Seneviratne J, Mandrekar J, Wijdicks EF, Rabinstein AA. Predictors of extubation failure in myasthenic crisis. Arch Neurol 2008; 65:929.
- Thomas CE, Mayer SA, Gungor Y, et al. Myasthenic crisis: clinical features, mortality, complications, and risk factors for prolonged intubation. Neurology 1997; 48:1253.
- Thanaviratananich S, Katirji B, Alshekhlee A. Broken heart syndrome during myasthenic crisis. J Clin Neuromuscul Dis 2014; 15:90.
- Alshekhlee A, Miles JD, Katirji B, et al. Incidence and mortality rates of myasthenia gravis and myasthenic crisis in US hospitals. Neurology 2009; 72:1548.
- CLINICAL PRESENTATION
- Cholinergic crisis
- EVALUATION AND MANAGEMENT
- Assessment of respiratory function
- - Clinical
- - Measures of respiratory muscle function
- Elective intubation
- Rapid therapies
- - Plasma exchange
- - Intravenous immune globulin
- - Choosing rapid therapy
- Immunomodulating therapy
- Weaning from ventilatory support
- INFORMATION FOR PATIENTS
- SUMMARY AND RECOMMENDATIONS