Myasthenic Crisis.
Myasthenic Crisis.
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DOI：
10.1007/s11940-004-0034-3
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发表时间：
2004-01-01
影响因子：
2
通讯作者：
Qureshi, Adnan I.
中科院分区：
文献类型：
--
作者：
Kirmani, Jawad F.;Yahia, Abutaher M.;Qureshi, Adnan I.
重症肌无力是最常见的神经肌肉接头疾病。重症肌无力危象定义为重症肌无力患者需要机械通气的呼吸衰竭，是一种常见的危及生命的并发症，发生在约15%至20%的重症肌无力患者中。有效的机械通气，专门的神经重症监护病房的出现，以及免疫治疗的广泛使用，大大改变了重症肌无力危象的预后。作者建议重症肌无力危象患者更自由地插管;早期插管和机械通气可能是重症肌无力危象管理中最重要的步骤。作者倾向于采用经口气管入路插管，并放置小口径十二指肠管，这可能有助于降低误吸风险，并且可能比常规鼻胃管更舒适。血浆置换治疗重症肌无力合并呼吸衰竭比静脉注射免疫球蛋白更有效。需要进行随机试验以证实血浆置换与静脉注射免疫球蛋白相比的上级疗效。在急性情况下，免疫抑制和静脉或肌肉注射吡啶斯的明的作用仍然有限，有时有争议。治疗应根据个人情况，使用最佳临床判断进行调整。
Myasthenia gravis is the most common disorder of the neuromuscular junction. Myasthenia crisis, defined as respiratory failure requiring mechanical ventilation in myasthenia gravis, is a common life-threatening complication that occurs in approximately 15% to 20% of patients with myasthenia gravis. The advent of effective mechanical ventilation, specialized neurointensive care units, and the widespread use of immunotherapies have substantially altered the prognosis of myasthenic crisis. The authors recommend more liberal intubation of patients with myasthenia gravis crisis; early intubation and mechanical ventilation is perhaps the most important step in the management of myasthenia gravis crisis. The authors favor an orotracheal approach for intubation, and placement of small bore duodenal tubes that may help decrease the risk of aspiration and may be more comfortable than regular nasogastric tubes for the patient. Plasma exchange is more effective than intravenous immunoglobulin in the treatment of myasthenia gravis involving respiratory failure. A randomized trial is required to confirm the superior efficacy of plasma exchange compared with intravenous immunoglobulin. In the acute setting, the role of immunosuppression and intravenous or intramuscular pyridostigmine remains limited and at times controversial. The therapy should be tailored on an individual basis using the best clinical judgment.