The myasthenic patient in crisis: an update of the management in Neurointensive Care Unit
The myasthenic patient in crisis: an update of the management in Neurointensive Care Unit
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DOI：
10.1590/0004-282x20130108
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发表时间：
2013-09-01
影响因子：
1.4
通讯作者：
Napoli, Mario Di
中科院分区：
文献类型：
--
作者：
Godoy, Daniel Agustin;Mello, Leonardo Jardim Vaz de;Napoli, Mario Di
重症肌无力(MG)是一种自身免疫性疾病，影响神经肌肉传递，导致全身性或局限性肌肉无力，最常见的原因是突触后运动终板存在抗乙酰胆碱受体的自身抗体。肌无力危象(MC)是MG的一种并发症，其特征是肌肉无力加剧，导致呼吸衰竭，需要插管和机械通气。它还包括手术后的患者，在这些患者中，MG肌肉无力的恶化会导致延迟拔管。MC是一种非常重要、严重和可逆的神经急症，影响20%-30%的肌无力患者，通常在疾病的第一年内，可能是疾病的首发形式。大多数患者都有触发危机的诱因，通常是呼吸道感染。免疫球蛋白、血浆置换和类固醇是免疫治疗的基石。在现代神经危重护理的今天，MC的死亡率不到5%。
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%.