Berrouschot J, Baumann I, Kalischewski P, Sterker M, Schneider D
Department of Neurology, University of Leipzig, Germany.
Crit Care Med. 1997 Jul;25(7):1228-35. doi: 10.1097/00003246-199707000-00027.
To investigate the frequency, causes, and characteristic features of the course of isolated myasthenic crisis (defined as acute respiratory failure with the need for mechanical ventilation). To compare the effectiveness of three different therapeutic regimens (i.e., continuous intravenous infusion of pyridostigmine, pyridostigmine plus prednisolone, and plasma exchange) in terms of duration of ventilation and outcome of patients with myasthenic crisis.
Population-based retrospective study covering the period 1970 to 1995.
University hospital, center for myasthenia gravis in Saxony, Germany.
Of 235 patients with myasthenia gravis treated at our institution, 44 patients developed a total of 63 myasthenic crises (average annual incidence 2.5%).
Myasthenic crises were treated with pyridostigmine (n = 24), pyridostigmine plus prednisolone (n = 18), and plasma exchange (n = 21).
There were no significant differences between the three treatment groups with respect to clinical characteristics, duration of mechanical ventilation, complications, and outcome on release from intensive care and after 3 months. Eleven (17%) patients had severe cardiac arrhythmia, which ended fatally for six patients.
None of the therapeutic regimens applied demonstrated any advantage over the others. All three regimens used were found to be effective and should be applied depending on the circumstances prevailing. Patients with myasthenic crisis must undergo careful cardiac monitoring, and temporary pace-making should be provided where clinically indicated.
调查单纯性重症肌无力危象（定义为急性呼吸衰竭且需要机械通气）的发生频率、病因及病程特征。比较三种不同治疗方案（即持续静脉输注吡啶斯的明、吡啶斯的明加泼尼松龙以及血浆置换）在重症肌无力危象患者通气持续时间和预后方面的效果。
基于人群的回顾性研究，涵盖1970年至1995年期间。
德国萨克森州的大学医院，重症肌无力中心。
在我们机构接受治疗的235例重症肌无力患者中，44例共发生了63次重症肌无力危象（年平均发病率2.5%）。
对重症肌无力危象患者采用吡啶斯的明治疗（n = 24）、吡啶斯的明加泼尼松龙治疗（n = 18）以及血浆置换治疗（n = 21）。
三个治疗组在临床特征、机械通气持续时间、并发症以及重症监护病房出院时和3个月后的预后方面无显著差异。11例（17%）患者出现严重心律失常，其中6例死亡。
所应用的治疗方案均未显示出比其他方案有任何优势。发现所有三种方案均有效，应根据具体情况应用。重症肌无力危象患者必须接受仔细的心脏监测，临床指征明确时应进行临时起搏治疗。
