Department of Neurology, The First Affiliated Hospital of Zhengzhou University, Zhengzhou, Henan, China.
Henan Key Laboratory of Cerebrovascular Diseases, The First Affiliated Hospital of Zhengzhou University, Zhengzhou University, Zhengzhou, Henan, China.
Front Immunol. 2023 Aug 15;14:1183488. doi: 10.3389/fimmu.2023.1183488. eCollection 2023.
The phenotypic spectrum of myelin oligodendrocyte glycoprotein (MOG)- IgG-associated disorders (MOGAD) has broadened in the past few years, and atypical phenotypes are increasingly recognized. Isolated seizures and MRI-negative brainstem and cerebellar symptoms or encephalitis have rarely been reported as a feature of MOGAD and represent a diagnostic challenge. Meanwhile, the coexistence of MOG IgG and other CNS autoimmune antibodies is infrequent. We report a patient presented with isolated epileptic onset, relapsed with MRI-negative brainstem symptoms and MRI-negative encephalitis. He was positive for MOG IgG throughout the disease course while concomitant NMDAR IgG was not detected positive until second relapse. He showed decreasing response to conventional first-line therapy. The last relapse was during a COVID-19 epidemic with limited inpatient resources. Fortunately, he was ultimately controlled on subcutaneous ofatumumab, a novel fully humanized anti-CD20 mAb. This is the first report about subcutaneous ofatumumab treatment in MOG and NMDAR IgG double positive encephalitis with 12-month follow-up, depicting its potential as a therapeutic option.
髓鞘少突胶质细胞糖蛋白(MOG)-IgG 相关疾病(MOGAD)的表型谱在过去几年中已经拓宽,并且越来越多地认识到非典型表型。孤立性发作以及 MRI 阴性脑干和小脑症状或脑炎作为 MOGAD 的特征很少见,这构成了诊断上的挑战。同时,MOG IgG 与其他中枢神经系统自身免疫抗体共存的情况并不常见。我们报告了一位患者以孤立性癫痫发作起病,复发时伴有 MRI 阴性脑干症状和 MRI 阴性脑炎。在整个疾病过程中,他的 MOG IgG 均呈阳性,而直到第二次复发时才检测到 NMDAR IgG 阳性。他对常规一线治疗的反应逐渐减弱。最后一次复发发生在 COVID-19 流行期间,住院资源有限。幸运的是,他最终在曲妥珠单抗皮下注射治疗下得到了控制,曲妥珠单抗是一种新型的完全人源化抗 CD20 mAb。这是首例关于 MOG 和 NMDAR IgG 双阳性脑炎的曲妥珠单抗皮下注射治疗的报告,随访 12 个月,描绘了其作为一种治疗选择的潜力。