目的 探讨抗髓鞘少突胶质细胞糖蛋白免疫球蛋白G抗体相关疾病(myelin-oligodendrocytes glycol-protein antibody-associated disease, MOGAD)的临床表现、MRI表现、MOG-IgG检测、诊断标准、治疗及预后。方法 报道成人MOGAD 1例,并进行文献复习。结果 患者女性,38岁,入院3天前出现头疼伴双眼视物模糊,有前驱发热史,头MRI提示丘脑、基底节、大脑脚及脑干病变及强化表现。血清及脑脊液抗体检测提示血清MOG-IgG为1:10弱阳性,脑脊液MOG-IgG为1:10弱阳性。诊断为MOGAD,主要表现为视神经炎及脑干脑炎。应用激素及利妥昔单抗联合治疗,4个月复查时患者症状已完全缓解。结论 MOGAD是近年来提出的一种少见的免疫介导的中枢神经系统炎性脱髓鞘疾病,主要临床表现包括视神经炎、脊髓炎、脑干脑炎、大脑皮质脑炎(通常伴有癫痫发作)以及急性播散性脑脊髓炎。2023年国际专家组提出了MOGAD的全新诊断标准。MOG-IgG检测是诊断MOGAD的关键。目前并没有MOGAD治疗的明确指南。约50%的患者会出现复发。

Objective To discuss the clinical manifestations, magnetic resonance imaging (MRI) features, MOG-IgG testing, diagnostic criteria, treatment and prognosis of myelin oligodendrocyte glycoprotein (MOG)-associated disease (MOGAD). Methods We reported a case of adult MOGAD, and reviewed relevant literatures. Results A 38-year-old female presented with headache and blurred vision in both eyes for three days. She had a history of prodromal fever. The head MRI showed lesions and enhancement in the thalamus, basal ganglia, cerebral peduncle, and brainstem. Serum and cerebrospinal fluid antibody testing showed a weak positive MOG IgG at a 1:10 ratio in both serum and cerebrospinal fluid. She was diagnosed with MOGAD, mainly manifested as optic neuritis and brainstem encephalitis. The combination therapy of corticosteroid and rituximab was given, and the patient's symptoms were completely disappeared at the 4-month follow-up. Conclusion MOGAD is a rare, antibody-mediated infl ammatory demyelinating disorder of the central nervous system (CNS) with cardinal features of optic neuritis, myelitis, brainstem encephalitis, cerebral cortical encephalitis (often with seizures) and acute disseminated encephalomyelitis. New diagnostic criteria for MOGAD have recently been proposed by an international panel of experts in 2023. MOG-IgG detection is the key to diagnosing MOGAD. There is no clear guideline for MOGAD treatment. About 50% of patients will experience a recurrence.