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如何在妊娠期间管理 MuSK 抗体阳性肌无力危象?

How to manage MuSK antibody-positive myasthenic crisis during pregnancy?

机构信息

Kütahya Health Sciences University Faculty of Medicine, Department of Neurology, Turkey.

出版信息

Ideggyogy Sz. 2022 Mar 31;75(3-04):141-144. doi: 10.18071/isz.75.0141.

Abstract

Myasthenia gravis (MG) is an autoimmune disease that is characterised by the formation of antibodies against acetylcholine receptors in the postsynaptic membrane of the neuromuscular junction. The course of the disease cannot be predicted during pregnancy. A subtype of MG with positive muscle-specific receptor tyrosine kinase (anti-MuSK) antibodies exhibits more localised clinical characteristics and a poor response to treatment compared with the disease subtype that involves positivity for acetylcholine receptor antibodies. Myasthenic crisis is more frequently observed in anti-MuSK-positive myasthenia patients. Anti-MuSK-positive myasthenic crisis management is very difficult and a risky situation during pregnancy. The reported case was 30 years old, female, 9 weeks pregnant and musk antibody positive. She stopped her treatment without asking her doctor because she was planning pregnancy in the 6-month period before her hospitalization. She was intubated for a long time in the intensive care unit due to myasthenic crisis and was very resistant to treatment. During this period, her pregnancy was terminated due to fetal anomaly. Plasmapheresis, IVIg and immunosuppressive treatments were applied. Our patient was discharged after a period of about 10 weeks. We share our treatment management.

摘要

重症肌无力(MG)是一种自身免疫性疾病,其特征是在神经肌肉接头的突触后膜上形成针对乙酰胆碱受体的抗体。在怀孕期间无法预测疾病的进程。与涉及乙酰胆碱受体抗体阳性的疾病亚型相比,具有肌肉特异性受体酪氨酸激酶(抗 MuSK)抗体阳性的 MG 亚型表现出更局限的临床特征和对治疗的反应不佳。抗 MuSK 阳性的肌无力危象在肌无力患者中更为常见。抗 MuSK 阳性的肌无力危象的管理在怀孕期间非常困难且存在风险。本例患者为 30 岁女性,怀孕 9 周,MuSK 抗体阳性。她在住院前的 6 个月内计划怀孕,因此未经医生允许就停止了治疗。由于肌无力危象,她在重症监护病房中长时间插管,并且对治疗非常耐药。在此期间,由于胎儿畸形而终止了妊娠。进行了血浆置换、IVIg 和免疫抑制治疗。大约 10 周后,我们的患者出院了。我们分享了治疗管理。

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