艾萨克斯综合征患者术后出现疼痛性肌束震颤：一例报告
Painful myokymia after surgery in a patient with Isaacs' syndrome: a case report.
作者信息
Okutani Hiroai, Okano Yukari, Hirose Munetaka
机构信息
Department of Anesthesiology and Pain Medicine, Hyogo College of Medicine, 1-1, Mukogawa-cho, Nishinomiya, Hyogo, Japan.
出版信息
JA Clin Rep. 2020 Feb 15;6(1):14. doi: 10.1186/s40981-020-00321-y.
BACKGROUND
Isaacs' syndrome is a peripheral nerve hyperexcitability syndrome and rare acquired channel disease. The symptoms (myokymia, neuromyotonia, and muscle spasm) emerge even during sleep. This report describes the anesthetic management, especially neuromuscular blocking drugs and postoperative pain, in a case of Isaacs' syndrome.
CASE PRESENTATION
A 63-year-old woman with Isaacs' syndrome underwent elective laparoscopic distal gastrectomy under general anesthesia without epidural anesthesia. She received double filtration plasmapheresis four times to alleviate symptoms before surgery. To avoid a prolonged neuromuscular blockade, we performed total intravenous anesthesia and titrated muscle relaxant with neuromuscular monitoring. Anesthetic management was performed without any problems. However, pain management after surgery proved difficult as she experienced severe pain due to myokymia.
CONCLUSIONS
Despite attempts to minimize symptoms, severe postoperative pain associated with myokymia occurred. Continuous regional anesthesia should be considered to treat pain from abnormal discharge in the central nervous system in Isaacs' syndrome.
背景
艾萨克斯综合征是一种周围神经兴奋性过高综合征，是一种罕见的获得性通道病。症状（肌束震颤、神经性肌强直和肌肉痉挛）甚至在睡眠期间也会出现。本报告描述了一例艾萨克斯综合征患者的麻醉管理，尤其是神经肌肉阻滞药物和术后疼痛情况。
病例介绍
一名63岁患有艾萨克斯综合征的女性在未行硬膜外麻醉的情况下接受了全身麻醉下的择期腹腔镜远端胃切除术。术前她接受了4次双重过滤血浆置换以缓解症状。为避免神经肌肉阻滞时间延长，我们采用了全静脉麻醉，并通过神经肌肉监测滴定肌肉松弛剂。麻醉管理过程顺利。然而，术后疼痛管理困难，因为她因肌束震颤而经历了严重疼痛。
结论
尽管试图将症状降至最低，但仍出现了与肌束震颤相关的严重术后疼痛。对于艾萨克斯综合征中因中枢神经系统异常放电引起的疼痛，应考虑采用连续区域麻醉进行治疗。
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