Akazawa Sayaka, Otsuka Yoshihisa, Hashimoto Rei, Matsumoto Minori, Yoneda Yukihiro, Kageyama Yasufumi
Division of Neurology, Hyogo Prefectural Amagasaki General Medical Center.
Division of Urology, Hyogo Prefectural Amagasaki General Medical Center.
Rinsho Shinkeigaku. 2022 May 31;62(5):395-398. doi: 10.5692/clinicalneurol.cn-001725. Epub 2022 Apr 26.
A 72-year-old man, who had received pembrolizumab of immune checkpoint inhibitor (ICI) over 6 months for ureter cancer, developed progressive skeletal muscle weakness, dysarthria, dyspnea, and consciousness disturbance over the past two weeks. The systemic work-up tests documented an encephalitis, myopathy, and myocarditis. Multiple autoimmune antibodies of anti-Tr, anti-titin, anti-kv1.4, anti-GM1 and anti-GD1a were positive in the serum. Although myopathy and myocarditis responded to high-dose steroid pulse therapy, encephalopathy deteriorated. Electroencephalogram showed a fluctuated pattern of rhythmic delta activity with fast waves, and a rapid response to intravenous diazepam revealed a condition of nonconvulsive status epileptics (NCSE). The patient had an uneventful course after anti-epileptic medication. The ICIs therapy may trigger a broader activation of multiple autoimmune mechanisms. When an encephalitis by immune-related adverse events does not respond to standard immunotherapy, NCSE may be a main pathophysiological mechanism, thereby anti-epileptics being an alternative treatment option.
一名72岁男性,因输尿管癌接受免疫检查点抑制剂(ICI)派姆单抗治疗6个月以上,在过去两周内出现进行性骨骼肌无力、构音障碍、呼吸困难和意识障碍。全身检查发现有脑炎、肌病和心肌炎。血清中多种自身免疫抗体抗-Tr、抗肌联蛋白、抗-kv1.4、抗-GM1和抗-GD1a呈阳性。尽管肌病和心肌炎对大剂量类固醇脉冲疗法有反应,但脑病仍恶化。脑电图显示有节律的δ活动与快波的波动模式,静脉注射地西泮后的快速反应显示为非惊厥性癫痫持续状态(NCSE)。患者在接受抗癫痫药物治疗后病情平稳。ICI治疗可能引发多种自身免疫机制的更广泛激活。当免疫相关不良事件引起的脑炎对标准免疫治疗无反应时,NCSE可能是主要的病理生理机制,则抗癫痫药物可能是一种替代治疗选择。