Lizarraga Alexis A, Lizarraga Karlo J, Benatar Michael
Department of Neurology, University of Miami School of Medicine, Miami, Florida.
Semin Neurol. 2016 Dec;36(6):615-624. doi: 10.1055/s-0036-1592106. Epub 2016 Dec 1.
After prompt diagnosis, severe myasthenia gravis and Guillain-Barré syndrome (GBS) usually require management in the intensive care unit. In the myasthenic patient, recognition of precipitating factors is paramount, and frequent monitoring of bulbar, upper airway, and/or respiratory muscle strength is needed to identify impending myasthenic crisis. Noninvasive ventilation can be attempted prior to intubation and mechanical ventilation in the setting of respiratory failure. Cholinesterase inhibitors should be discontinued, but resumed prior to extubation, and steroid dosage could be increased once the airway is secured. In GBS, hemodynamic and respiratory monitoring are essential; however, respiratory failure can develop rapidly and intubation with mechanical ventilation is often required and can be prolonged. Guillain-Barré syndrome can also be complicated by dysautonomia necessitating specific therapies. Prompt recognition and initiation of immunotherapy including intravenous immunoglobulin or plasmapheresis, together with supportive care including treatment of underlying infections and physical therapy, can improve outcomes in both myasthenic crisis and GBS.
在明确诊断后，重症肌无力和吉兰 - 巴雷综合征（GBS）通常需要在重症监护病房进行治疗。对于重症肌无力患者，识别诱发因素至关重要，需要频繁监测延髓、上呼吸道和/或呼吸肌力量，以确定即将发生的肌无力危象。在呼吸衰竭的情况下，可在插管和机械通气之前尝试无创通气。应停用胆碱酯酶抑制剂，但在拔管前恢复使用，并且一旦气道安全，可增加类固醇剂量。在GBS中，血流动力学和呼吸监测至关重要；然而，呼吸衰竭可能迅速发展，通常需要插管并进行机械通气，且通气时间可能延长。吉兰 - 巴雷综合征也可能并发自主神经功能障碍，需要进行特定治疗。及时识别并启动包括静脉注射免疫球蛋白或血浆置换在内的免疫治疗，以及包括治疗潜在感染和物理治疗在内的支持性护理，可改善肌无力危象和GBS的治疗效果。
