The myasthenic patient in crisis: an update of the management in Neurointensive Care Unit

• Published in: Arquivos de neuro-psiquiatria 2013-09, Vol.71 (9A), p.627-639
• Main Authors: Godoy, Daniel Agustin, Mello, Leonardo Jardim Vaz de, Masotti, Luca, Di Napoli, Mario
• Format: Article
• Language: English
• Subjects: crise miatenica; Diagnosis, Differential; falencia respiratoria; Humans; immunosupressive therapy; miastenia gravis; myasthenia gravis; Myasthenia Gravis - diagnosis; Myasthenia Gravis - physiopathology; Myasthenia Gravis - therapy; myasthenic crisis; NEUROSCIENCES; PSYCHIATRY; respiratory failure; Risk Factors; Severity of Illness Index; terapia imunosupressiva; thymectomy; timectomia
• ISSN: 0004-282X; 1678-4227; 1678-4227; 0004-282X
• DOI: 10.1590/0004-282X20130108

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. Immunoglobulins, plasma exchange, and steroids are the cornerstones of immunotherapy. Today with the modern neurocritical care, mortality rate of MC is less than 5%.