MOGAD临床特征及预后分析

Analysis of clinical features and prognosis of MOGAD

目的探究抗髓鞘少突胶质细胞糖蛋白免疫球蛋白G抗体相关疾病(MOGAD)的临床特征。方法回顾性分析8例诊断为MOGAD的患者11次病程的临床资料,总结其临床特征。结果8例患者中男3例,女5例,年龄7~50岁,18岁以下有4例。8例血清抗髓鞘少突胶质细胞糖蛋白免疫球蛋白G抗体(MOG-IgG)均为阳性,1例为双侧视神经炎合并急性播散性脑脊髓炎(ADEM)样表现,3例表现为脑炎,其中有2例合并抗N-甲基-D-天冬氨酸受体(NMDAR)脑炎,另外4例表现为脊髓炎。头颅磁共振成像(MRI)表现为多灶性,斑片状T2高信号,可累及颞叶、额叶、枕叶、顶叶及丘脑、尾状核等部位。脊髓MRI表现为脊髓长或短节段T2高信号,可累及颈髓、胸髓,轴位多累及脊髓中央灰质。8例患者共11次病程,急性期均应用糖皮质激素治疗,有1例联合应用免疫球蛋白静脉滴注(IVIg),预后良好率占72.7%。2例患者有复发病程。结论MOGAD表型多样,临床表现复杂,影像学特异性不高。大多数患者急性期应用激素治疗预后良好,少数会复发。

Objective To explore the clinical characteristics of anti-myelin oligodendrocyte glycoprotein-IgG antibody associated diseases(MOGAD).Methods The clinical data of 11 episodes in eight patients diagnosed with MOGAD were retrospectively analyzed,and their clinical features were summarized.Results There were five females and three males,all aged seven to 50 years,and four cases under 18 years old.Serum MOG-IgG was positive in all patients.There were one case with bilateral optic neuritis combined with acute disseminated encephalomyelitis manifestations(ADEM),three cases with encephalitis including two cases combined with anti-N-methyl-D-aspartate receptor(NMDAR)positive encephalitis,and other four cases with myelitis.Cranial magnetic resonance imaging(MRI)showed multifocal,patchy T2 hyperintensity,involving the temporal lobe,frontal lobe,occipital lobe,parietal lobe,thalamus and caudate nucleus.MRI of spinal cord showed T2 hyperintensity in the long or short segment of the spinal cord,involving the cervical and thoracic spinal cord,and central gray matter of the spinal cord in axial view.All the eight patients in 11 episodes were treated with glucocorticoid in the acute stage,including one case combined with intravenous immune globulin(IVIg).The rate of good prognosis was 72.7%and two patients relapsed.Conclusion The phenotypes of MOGAD are diverse,the clinical manifestations are complex,and the imaging specificity is not high.Most patients have a good prognosis with hormones and a few will relapse.