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Teaching Video NeuroImage: Response to IVIg in a Patient With Steroid-Refractory Immune Checkpoint Inhibitor-Related Ocular Myopathy
Neurology ( IF 9.9 ) Pub Date : 2023-11-21 , DOI: 10.1212/wnl.0000000000207864
Sara J Hooshmand 1 , Catarina Aragon Pinto 1 , Teerin Liewluck 1 , Marcus V Pinto 1
Affiliation  

A 76-year-old woman with metastatic melanoma presented with a 3-day history of progressive diplopia approximately 3 weeks after her first ipilimumab and nivolumab therapy. Neurologic examination demonstrated bilateral nonfatigable ptosis and severe bilateral ophthalmoplegia without evidence of bulbar or neck weakness. Creatine kinase (CK) was 5213 U/L (normal:26-192). AChR and MuSK antibodies were negative. Electrodiagnostic studies revealed early recruiting, small motor unit potentials with fibrillations in several muscles, and no decrement on repetitive nerve stimulation. Brain and orbit MRIs were normal. CK normalized in 1 week. Immune checkpoint inhibitors (ICIs) were discontinued. She was started on 200 mg of prednisone per day tapered slowly over 6 months. At 3-month follow-up, patient had minimal improvement (Video 1). Monthly IVIg was initiated, and ophthalmoplegia completely resolved (Video 1). ICI myopathy (ICIM) is a drug-induced immune-mediated myopathy that has a predilection for the extraocular muscles.1 ICIM usually has an excellent response to IVIg,1 which should be considered in all steroid-refractory cases.



中文翻译:

NeuroImage 教学视频:类固醇难治性免疫检查点抑制剂相关眼肌病患者对 IVIg 的反应

一名患有转移性黑色素瘤的 76 岁女性在首次伊匹单抗和纳武单抗治疗后约 3 周出现 3 天进行性复视病史。神经系统检查显示双侧非疲劳性上睑下垂和严重的双侧眼肌麻痹,但没有延髓或颈部无力的证据。肌酸激酶 (CK) 为 5213 U/L(正常值:26-192)。AChR 和 MuSK 抗体呈阴性。电诊断研究显示早期募集、小运动单位电位并伴有多块肌肉颤动,并且重复性神经刺激没有减弱。脑部和眼眶 MRI 正常。CK 在 1 周内恢复正常。免疫检查点抑制剂(ICIs)已停用。她开始每天服用 200 毫克泼尼松,并在 6 个月内缓慢减量。在 3 个月的随访中,患者的改善甚微(视频 1)。开始每月一次 IVIg,眼肌麻痹完全消失(视频 1)。ICI 肌病 (ICIM) 是一种药物诱导的免疫介导的肌病,好发于眼外肌。1 ICIM 通常对 IVIg 具有良好的反应,1在所有类固醇难治性病例中都应考虑这一点。

更新日期:2023-11-22
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