Myasthenic crisis Muscle & Nerve by Benjamin Claytor, Sung-Min Cho, Yuebing Li ISBN 10.1002/MUS.27832 instant download

Myasthenic crisis Muscle & Nerve by Benjamin Claytor, Sung-Min Cho, Yuebing Li ISBN 10.1002/MUS.27832 instant download

Myasthenic crisis (MC) is a life-threatening manifestation of myasthenia gravis (MG) defined by respiratory insufficiency that requires the use of invasive or non- invasive ventilation. This is often the result of respiratory muscle weakness but can also be due to bulbar weakness with upper airway collapse. MC occurs in approximately 15%–20% of patients with MG usually within the first 2 to 3 y of the disease course. Many crises have a specific trigger with respiratory infections being most common; how- ever, no specific trigger is found in 30%–40% of patients. MG patients with a history of MC, severe disease, oropharyngeal weakness, muscle-specific kinase (MuSK) antibodies and thymoma appear to be at higher risk. Most episodes of MC do not occur suddenly, providing a window of opportunity for prevention. Immediate treatment is directed toward airway management and removing any identified triggers. Plasmapheresis is pre- ferred over intravenous immune globulin as the treatments of choice for MC. The major- ity of patients are able to be weaned from mechanical ventilation within 1 mo and the outcomes of MC are generally favorable. The mortality rate in United States cohorts is less than 5% and mortality in MC seems to be driven by age and other medical co-mor- bidities. MC does not appear to affect long-term prognosis as many patients are able to eventually achieve good MG control.