Table_1_Case Report: Anti-NMDAR Encephalitis With Anti-MOG CNS Demyelination After Recurrent CNS Demyelination.DOC

Introduction: Anti-N-methyl-d-aspartate receptor (NMDAR) encephalitis, a serious neurological autoimmune disorder caused by autoantibodies with diverse clinical manifestations, may simultaneously onset with antimyelin oligodendrocyte glycoprotein (MOG) demyelination after recurrent central nervous system (CNS) demyelination.
Case Report: We present a case of anti-NMDAR encephalitis combining with anti-MOG CNS demyelination following recurrent CNS demyelination. A 38-year-old man admitted to hospital developed epileptic seizures following recurrent episodes of cross-sensory disturbance and dizziness. Magnetic resonance imaging (MRI) showed a demyelinating lesion in the right brainstem initially. Despite a good response to methylprednisolone pulse therapy at the beginning, the patient still had relapses and progression after corticosteroid reduction or withdrawal. Then brain MRI discovered new serpentine lesions involving extensive cerebral cortex on his second relapse. Repeat autoantibodies test indicated cerebrospinal fluid (CSF) NMDAR antibodies coexisted with MOG-Abs simultaneously, suggesting the diagnosis of anti-NMDAR encephalitis with anti-MOG CNS demyelination.
Results: After a definite diagnosis, the patient was treated with mycophenolate mofetil (MMF) and corticosteroid. He was discharged after his symptoms ameliorated. No neurological sequels remained, and there were no effects on his activities of daily living after 6 months of immunoregulatory therapy of MMF and corticosteroid.
Conclusion: For individuals with recurrent CNS demyelination, especially combining with cortical encephalitis, repeated detection of autoantibodies against AE, and demyelination in CSF/serum can be helpful to enable a definite early diagnosis. For patients who suffer from anti-NMDAR encephalitis combining with anti-MOG CNS demyelination, second-line immunotherapy is recommended when first-line treatment such as steroids, intravenous immunoglobulin G (IVIG) and plasma exchange has been proven ineffective to prevent the relapse of disease.

引言：抗N-甲基-D-天冬氨酸受体（NMDAR）脑炎是一类由自身抗体介导的严重神经系统自身免疫性疾病，临床表现多样，可在复发性中枢神经系统（CNS）脱髓鞘后，同时合并抗髓鞘少突胶质细胞糖蛋白（MOG）脱髓鞘病变。

病例报告：我们报告1例在复发性中枢神经系统脱髓鞘后并发抗NMDAR脑炎与抗MOG中枢神经系统脱髓鞘的病例。患者为38岁男性，因反复出现交叉性感觉障碍与头晕后出现癫痫发作入院。初始磁共振成像（MRI）显示右侧脑干存在脱髓鞘病灶。尽管初始给予甲泼尼龙冲击治疗后病情应答良好，但患者在糖皮质激素减量或停药后仍出现病情复发与进展。第二次复发时的颅脑MRI显示出现累及广泛大脑皮质的新型蛇形病灶。复查自身抗体检测显示脑脊液（CSF）中同时存在NMDAR抗体与MOG抗体（MOG-Abs），据此确诊为抗NMDAR脑炎合并抗MOG中枢神经系统脱髓鞘。

结果：明确诊断后，患者接受了吗替麦考酚酯（MMF）与糖皮质激素治疗。患者症状改善后出院。接受6个月的MMF与糖皮质激素免疫调节治疗后，患者未遗留神经系统后遗症，且日常生活活动未受影响。

结论：对于复发性中枢神经系统脱髓鞘患者，尤其是合并皮质脑炎者，反复检测自身免疫性脑炎（AE）相关自身抗体以及脑脊液/血清中的脱髓鞘相关抗体，有助于实现早期明确诊断。对于抗NMDAR脑炎合并抗MOG中枢神经系统脱髓鞘的患者，若一线治疗（如糖皮质激素、静脉注射免疫球蛋白G（IVIG）及血浆置换）无法有效预防疾病复发，则推荐采用二线免疫治疗。