Lai Yi-Chun, Chen Jung-Yueh, Wu Huey-Dong, Yang Chih-Chao, Lin Chin-Hsien, Lee Pei-Lin
Division of Pulmonary Medicine, Department of Internal Medicine, National Yang Ming University Hospital, I Lan, Taiwan.
Department of Internal Medicine, E-Da Hospital, Kaohsiung, Taiwan.
J Clin Sleep Med. 2016 May 15;12(5):767-9. doi: 10.5664/jcsm.5816.
Myasthenia gravis (MG) can result in weakness of the respiratory muscles in 30% of patients. A life-threatening exacerbation, MG crisis can cause respiratory insufficiency requiring mechanical ventilation. Sleep disordered breathing (SDB) is seen in 40% to 60% of stable MG patients. Factors associated with SDB include age, male sex, obesity, and steroid use. Continuous positive airway pressure (CPAP) can reverse paradoxical weakness in MG patients with obstructive sleep apnea (OSA), but whether SDB can contribute to respiratory failure in MG and whether CPAP works in such patients remain unclear. This report presents a 54-year-old woman with MG with a history of 7 episodes of respiratory failure requiring mechanical ventilation. For each episode, she was treated for MG crisis using plasmapheresis and high-dose steroids. Later, OSA and obesity hypoventilation syndrome were confirmed by polysomnography with transcutaneous CO2 monitoring. Thereafter, the patient had no further recurrence of MG crisis for 5 years, using pyridostigmine and CPAP only.
重症肌无力(MG)可导致30%的患者呼吸肌无力。重症肌无力危象是一种危及生命的加重情况,可导致呼吸功能不全,需要机械通气。40%至60%病情稳定的MG患者存在睡眠呼吸障碍(SDB)。与SDB相关的因素包括年龄、男性、肥胖和使用类固醇。持续气道正压通气(CPAP)可逆转患有阻塞性睡眠呼吸暂停(OSA)的MG患者的矛盾性肌无力,但SDB是否会导致MG患者呼吸衰竭以及CPAP对此类患者是否有效仍不清楚。本报告介绍了一名54岁患有MG的女性,她有7次呼吸衰竭发作史,均需要机械通气。每次发作时,她都通过血浆置换和大剂量类固醇治疗重症肌无力危象。后来,通过多导睡眠图和经皮二氧化碳监测确诊为OSA和肥胖低通气综合征。此后,该患者仅使用吡啶斯的明和CPAP,5年内重症肌无力危象未再复发。