目的:探讨首次发作的成人抗髓鞘少突胶质细胞糖蛋白免疫球蛋白G抗体(anti-myelin oligodendrocyte glycoprotein-lgG ,MOG-IgG )相关疾病(MOG-IgG associated disorders, MOGAD)的临床特点。

方法:回顾性分析2020年6月至2023年6月就诊于北京大望路急诊抢救医院和北京朝阳中西医结合急诊抢救医院神经内科的16例首次发作的成人MOGAD患者的一般资料、临床表现及表型、血清MOG-IgG水平、影像学资料、治疗及预后。

结果:纳入16例患者中男性6例，女性10例，中位年龄37岁。临床表现符合视神经炎(optic neuritis,ON)7例(7/16,43.8% ),脑膜脑炎7例(7/16,43.8% ),脊髓炎2例(2/16, 12.5%),脑干脑炎1例(1/16,6.2%),其中1例(1/16,6.2% )为ON合并脑膜脑炎。血清MOG-IgG效价为1 : 10~1 : 100。治疗前所有患者MRI检查均有病变,累及视神经7例(7/16,43.8%),累及大脑皮质6例(6/16,37.5%),累及软脑膜2例(2/16,12.5%),累及胸髓2例(2/16,12.5%),累及 脑桥1例(1/16,6.2%)。有强化者15例(15/16,93.8%),无强化者1例(1/16, 6.2%),表现为斑片状、点状、线样强化。所有纳入患者急性期均给予静脉注射甲泼尼龙冲击治疗,其中13 例(13/16,81.2%)患者联合应用静脉注射免疫球蛋白。缓解期所有患者均予以泼尼松小剂量 维持,其中4例(4/16,25%)联合应用吗替麦考酚酯。治疗2个月后9例患者恢复良好(9/16, 56.2%),7例恢复不良(7/16,43.8%)。

结论:MOGAD临床表现复杂,对于有ON、脑膜脑炎、脑干脑炎及脊髓炎等表现的患者,应进-步化验血清MOG-IgG抗体排除MOGAD,确诊后要积极进行相关免疫治疗。

Objective:To explore the clinical features of first-episode adult anti-myelin oligodendrocyte glycoprotein-IgG associated disorders (MOGAD).

Methods:A retrospective analysis was performed for the general data, clinical manifestations and phenotype, serum MOG-IgG titers, imaging data, treatment and prognosis of 16 adult patients with first-episode MOGAD who were admitted to the Department of Neurology of Beijing Dawang Road Emergency Hospital and Beijing Chaoyang Integrative Medicine Emergency Hospital from June 2020 to June 2023.

Results:Among the 16 patients, 6 were males and 10 were females, with a median age of 37 years. The clinical manifestations were consistent with optic neuritis (ON) in 7 cases (7/16, 43.8%), meningoencephalitis in 7 cases(7/16, 43.8%), myelitis in 2 cases (2/16, 12.5%), and brainstem encephalitis in 1 case (1/16, 6.2%), of which 1 case (1/16, 6.2%) was ON combined with meningoencephalitis. Serum MOG-IgG titer was 1:10 to 1:100. Before treatment, all patients had lesions on MRI examination, 7 cases (7/16, 43.8%) involved the optic nerve, 6 cases(6/16, 37.5%) involved the cerebral cortex, 2 cases (2/16, 12.5%) involved the leptomeninges, 2 cases (2/16, 12.5%) involved the thoracic spinal cord, and 1 case(1/16, 6.2%) involved the pons. There were 15 cases (15/16, 93.8%) with enhancement and 1 case (1/16, 6.2% ) without enhancement, which showed patchy, punctate and linear enhancement. All included patients were treated with intravenous methylprednisolone pulse in the acute phase, and 13 (13/16, 81.2%) patients were treated with intravenous immunoglobulin. All patients in remission were maintained with low-dose prednisone, and 4 patients (4/16, 25%) were treated with mycophenolate mofetil. After 2 months of treatment, 9 patients recovered well (9/16, 56.2%), and 7 cases recovered poorly (7/16, 43.8%).

Conclusion:The clinical manifestations of MOGAD are complex, and for patients with ON, meningoencephalitis, brainstem encephalitis and myelitis, serum MOG-IgG antibodies should be further tested to rule out MOGAD, and relevant immunotherapy should be actively carried out after diagnosis.