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[在扩大经胸骨胸腺切除术之前使用齐卢可普兰治疗重症肌无力以预防肌无力危象:一例报告]

[Myasthenia Gravis Treated with Zilucoplan Prior to Extended Transsternal Thymectomy for the Prevention of Myasthenic Crisis: A Case Report].

作者信息

Yamanaka Nanami, Honda Masaya, Kanda So, Tanaka Toshiki, Shimizu Fumitaka, Koga Michiaki, Nakamori Masayuki

机构信息

Department of Neurology and Clinical Neuroscience, Yamaguchi University Graduate School of Medicine.

出版信息

Brain Nerve. 2025 Aug;77(8):921-925. doi: 10.11477/mf.188160960770080921.

Abstract

A 50year-old female was diagnosed with myasthenia gravis (MG) following aspiration pneumonia. Despite treatment with prednisolone (5mg/day) and intravenous immunoglobulin (IVIg), the bulbar palsy persisted. Additionally, chest CT revealed findings suggestive of invasive thymoma or thymic carcinoma, leading to a planned thymectomy with median sternotomy. This case presents a high-risk of myasthenic crisis due to thymoma-associated MG, persistent bulbar palsy, and the need for highly invasive surgery. Therefore, enhanced immunotherapy was required to prevent this crisis, and zilucoplan was chosen because of its rapid onset of action and compatibility with IVIg. Following the initiation of zilucoplan, there was prompt improvement in the symptoms of MG. Effective preoperative control of MG led to a good clinical course, with no significant postoperative myasthenic crisis or exacerbation of symptoms. This is the first report on the use of zilucoplan for the prevention of perioperative myasthenic crisis. (Received April 14, 2025; Accepted June 3, 2025, Published August 1, 2025).

摘要

一名50岁女性在患吸入性肺炎后被诊断为重症肌无力(MG)。尽管接受了泼尼松龙(5mg/天)和静脉注射免疫球蛋白(IVIg)治疗,但延髓麻痹仍持续存在。此外,胸部CT显示有提示侵袭性胸腺瘤或胸腺癌的表现,因此计划行正中开胸胸腺切除术。由于胸腺瘤相关的MG、持续的延髓麻痹以及需要进行高侵袭性手术,该病例存在重症肌无力危象的高风险。因此,需要强化免疫治疗以预防这种危象,选择齐卢可普兰是因为其起效迅速且与IVIg相容。开始使用齐卢可普兰后,MG症状迅速改善。MG的有效术前控制导致了良好的临床过程,术后无明显的重症肌无力危象或症状加重。这是关于使用齐卢可普兰预防围手术期重症肌无力危象的首例报告。(2025年4月14日收到;2025年6月3日接受,2025年8月1日发表)

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