Journal
ARQUIVOS DE NEURO-PSIQUIATRIA
Volume 71, Issue 9A, Pages 627-639Publisher
ASSOC ARQUIVOS NEURO- PSIQUIATRIA
DOI: 10.1590/0004-282X20130108
Keywords
myasthenic crisis; myasthenia gravis; respiratory failure; immunosupressive therapy; thymectomy
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Myasthenia gravis (MG) is an autoimmune disorder affecting neuromuscular transmission leading to generalized or localized muscle weakness due most frequently to the presence of autoantibodies against acetylcholine receptors in the postsynaptic motor end-plate. Myasthenic crisis (MC) is a complication of MG characterized by worsening muscle weakness, resulting in respiratory failure that requires intubation and mechanical ventilation. It also includes postsurgical patients, in whom exacerbation of muscle weakness from MG causes a delay in extubation. MC is a very important, serious, and reversible neurological emergency that affects 20-30% of the myasthenic patients, usually within the first year of illness and maybe the debut form of the disease. Most patients have a predisposing factor that triggers the crisis, generally an infection of the respiratory tract. Immunogtobulins, plasma exchange, and steroids are the cornerstones of immunotherapy. Today with the modern neurocritical care, mortality rate of MC is less than 5%.
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