Kirmani Jawad F., Yahia Abutaher M., Qureshi Adnan I.
Departments of Neurosurgery and Neurology, State University of New York at Buffalo, School of Medicine and Biomedical Sciences, 3 Gates Circle, Buffalo, NY 14209, USA.
Curr Treat Options Neurol. 2004 Jan;6(1):3-15. doi: 10.1007/s11940-004-0034-3.
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.
重症肌无力是神经肌肉接头最常见的疾病。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,约15%至20%的重症肌无力患者会出现。有效的机械通气、专业的神经重症监护病房的出现以及免疫疗法的广泛应用,已极大地改变了重症肌无力危象的预后。作者建议对重症肌无力危象患者进行更宽松的插管;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一步。作者倾向于经口气管插管法,并放置细径十二指肠管,这可能有助于降低误吸风险,且对患者来说可能比常规鼻胃管更舒适。在治疗伴有呼吸衰竭的重症肌无力时,血浆置换比静脉注射免疫球蛋白更有效。需要进行一项随机试验来证实血浆置换相对于静脉注射免疫球蛋白的卓越疗效。在急性情况下,免疫抑制以及静脉或肌肉注射吡啶斯的明的作用仍然有限,且有时存在争议。应根据个体情况,运用最佳临床判断来制定治疗方案。