Myasthenic crisis

Myasthenia gravis is the most common disorder of the neuromuscular junction. Myasthenia crisis, defined as respiratory failure requiring mechanical ventilation in myasthenia gravis, is a common life-threatening complication that occurs in approximately 15% to 20% of patients with myasthenia gravis. The advent of effective mechanical ventilation, specialized neurointensive care units, and the widespread use of immunotherapies have substantially altered the prognosis of myasthenic crisis. The authors recommend more liberal intubation of patients with myasthenia gravis crisis; early intubation and mechanical ventilation is perhaps the most important step in the management of myasthenia gravis crisis. The authors favor an orotracheal approach for intubation, and placement of small bore duodenal tubes that may help decrease the risk of aspiration and may be more comfortable than regular nasogastric tubes for the patient. Plasma exchange is more effective than intravenous immunoglobulin in the treatment of myasthenia gravis involving respiratory failure. A randomized trial is required to confirm the superior efficacy of plasma exchange compared with intravenous immunoglobulin. In the acute setting, the role of immunosuppression and intravenous or intramuscular pyridostigmine remains limited and at times controversial. The therapy should be tailored on an individual basis using the best clinical judgment.