成人MOG抗体病首次发作的临床和影像学特征分析

Clinical and neuroimaging features of adults with myelin oligodendrocyte glycoprotein antibody-associated disease at first attack

目的探讨成人髓鞘少突胶质细胞糖蛋白(myelin oligodendrocyte glycoprotein,MOG)抗体病首次发作的临床和影像学特征。方法回顾性分析2018年1月-2022年1月在江西省人民医院和新余市人民医院神经内科收治的14例MOG抗体病患者首次发作的临床症状、影像学特点、实验室检查、预后情况。结果纳入的14例患者中男6例,女8例,中位年龄29.5岁。首发症状以发热和头痛(5例)、癫痫(3例)、头晕(3例)多见。幕上病灶包括丘脑(7例)、皮质下白质(6例)、皮质(5例)、胼胝体(2例)、基底节(2例)。幕下多累及脑干,包括桥脑(5例)、小脑中脚(3例)、中脑(2例)、延髓(2例)。脊髓受累3例,长节段横贯性1例。脑脊液细胞数增高13例,7例脑脊液蛋白升高。血清MOG抗体滴度范围在1∶3.2~1∶512。14例患者均接受静脉糖皮质激素冲击治疗,仅1例患者出现视神经炎复发。结论本组成人MOG抗体病女性稍多于男性,以ADEM表型最多见。幕上病灶以丘脑和皮质下白质多见,幕下以脑桥多见。急性期经静脉激素冲击治疗效果显著,临床预后大多良好。

Objective To investigate the clinical and neuroimaging features of adults with myelin oligodendrocyte glycoprotein(MOG)antibody-associated disease at the first attack.Methods We retrospectively analyzed the clinical manifestations,radiological features,laboratory findings,and outcome of 14 adult patients with MOG antibody-associated disease at the first attack who were hospitalized in the departments of neurology of Jiangxi Provincial People's Hospital and Xinyu People's Hospital from January 2018 to January 2022.Results The 14 patients included six males and eight females,with a median age of 29.5 years.The most common initial symptoms were fever and headache(n=5),seizure(n=3),and dizziness(n=3).The supratentorial lesions were located in the thalamus(n=7),subcortical white matter(n=6),cortex(n=5),corpus callosum(n=2),and basal ganglia(n=2).The infratentorial lesions were frequently located in the brainstem:the pons(n=5),middle cerebellar peduncle(n=3),midbrain(n=2),and medulla(n=2).Three patients had spinal cord involvement,with one case of longitudinally extensive transverse myelitis.Thirteen patients had elevated cerebrospinal fluid cell counts,and seven had elevated cerebrospinal fluid protein levels.The serum MOG antibody titer ranged from 1∶3.2 to 1∶512.All the 14 patients received intravenous pulse glucocorticoid therapy.Only one patient had a relapse with optic neuritis.Conclusion In our study,MOG antibody-associated disease showed a slight female predominance and frequently presented as acute disseminated encephalomyelitis.The supratentorial lesions were often located in the thalamus and subcortical white matter,while the infratentorial lesions were frequently in the pons.Intravenous pulse steroid therapy was effecitve in the acute phase.The majority of the patients had a favorable outcome.