Noda Tomoko, Kageyama Haruka, Miura Miki, Tamura Takuya, Ito Hiroki
Department of Neurology, Ichinomiya Municipal Hospital.
Rinsho Shinkeigaku. 2019 Aug 29;59(8):502-508. doi: 10.5692/clinicalneurol.cn-001251. Epub 2019 Jul 23.
A 77-year-old woman with lung adenocarcinoma noticed bilateral ptosis 7 weeks after a first pembrolizumab infusion. Her symptoms rapidly progressed to generalized manifestations including limb and neck weakness, dyspnea, and dysphasia within the following two weeks. We diagnosed him with pembrolizumab-related myasthenia gravis and myositis based on clinical symptoms, elevation of muscle enzymes and anti-acetylcholine receptor antibodies, repetitive nerve stimulation and muscle biopsy. We commenced combination immunotherapy, including intravenous and oral steroid therapy, immune absorption therapy and plasma exchange therapy with noninvasive positive-pressure ventilation and tracheotomy positive pressure ventilation. She had gradual symptoms improvement and discharged after 209 days in a hospital. In this case, anti-titin antibodies, one of anti-striational antibodies, was positive and correlated with severity of myasthenia gravis. With the development of immune checkpoint inhibitors for various malignancies, clinicians should closely monitor patients for important immune-related adverse events and coordinate on early treatment.
一名77岁的肺腺癌女性在首次输注帕博利珠单抗7周后出现双侧上睑下垂。在接下来的两周内，她的症状迅速发展为全身性表现，包括肢体和颈部无力、呼吸困难和吞咽困难。基于临床症状、肌肉酶升高、抗乙酰胆碱受体抗体、重复神经电刺激和肌肉活检，我们诊断她患有帕博利珠单抗相关的重症肌无力和肌炎。我们开始了联合免疫治疗，包括静脉和口服类固醇治疗、免疫吸附治疗以及无创正压通气和气管切开正压通气的血浆置换治疗。她的症状逐渐改善，住院209天后出院。在这个病例中，抗肌联蛋白抗体（抗横纹肌抗体之一）呈阳性，且与重症肌无力的严重程度相关。随着针对各种恶性肿瘤的免疫检查点抑制剂的发展，临床医生应密切监测患者是否出现重要的免疫相关不良事件，并协调早期治疗。
