当前对髓鞘少突胶质细胞糖蛋白抗体相关疾病的认识和挑战  

Current understanding and challenges of myelin oligodendrocyte glycoprotein antibody-associated diseases

作　　者：应国环 吴冰 王欣 戚洪丹 何明英 钱浩 王尚玉 张刚 Ying Guohuan;Wu Bing;Wang Xin;Qi Hongdan;He Mingying;Qian Hao;Wang Shangyu;Zhang Gang(Department of Neurology,Children's Hospital of Nanjing Medical University,Nanjing 210003,China;Nanjing University School of Medicine,Nanjing 210093,China)

机构地区：[1]南京医科大学附属儿童医院神经内科,南京210003 [2]南京大学医学院,南京210093

出　　处：《中华实用儿科临床杂志》2025年第4期316-320,共5页Chinese Journal of Applied Clinical Pediatrics

基　　金：南京市卫生局重点项目(ZKX21046)。

摘　　要：2023年提出了新的诊断标准用于区分髓鞘少突胶质细胞糖蛋白抗体相关疾病(MOGAD)与其他中枢神经系统炎症性疾病。在典型的临床表现和神经影像学特征下,抗髓鞘少突胶质细胞糖蛋白免疫球蛋白G抗体(MOG-IgG)的血清阳性状态对于诊断MOGAD至关重要。过去几年中,细胞转染免疫荧光检测技术的进展提高了诊断准确性,但在不同MOGAD流行率的情况下,血清MOG-IgG滴度的阳性预测值存在差异。因此,需要进一步寻找可替代的诊断方法,并且仔细权衡低MOG-IgG滴度的临床意义。本综述探讨了MOGAD的主要临床特征,并分析了当前对MOGAD的认识及面临的挑战,这些挑战包括:验证对髓鞘少突胶质细胞糖蛋白自身抗体的特异性和致病性的不确定性,需要明确未来治疗的免疫病理学靶点,验证有助于诊断和检测疾病活动的生物标志物,以及确定哪些MOGAD患者需要长期免疫治疗。In 2023,new diagnostic criteria were proposed to distinguish myelin oligodendrocyte glycoprotein antibody-associated disease(MOGAD)from other inflammatory diseases of the central nervous system.With typical clinical presentation and neuroimaging features,the seropositive status of anti-myelin oligodendrocyte glycoprotein-IgG(MOG-IgG)is essential for the diagnosis of MOGAD.Advances in cell transfection immunofluorescence assays have improved diagnostic accuracy in the last few years,but there are differences in the positive predictive value of serum MOG-IgG titers in the context of different MOGAD prevalence.Therefore,alternative diagnostic methods need to be further explored,and the clinical significance of low MOG-IgG titers should be carefully interpreted.In this review,the main clinical features,current understanding and challenges of MOGAD were discussed,including uncertainties about the specificity and pathogenicity of myelin oligodendrocyte glycoprotein autoantibodies and the need to identify immunopathological targets for future therapies.Moreover,it is necessaryto search for and validate biomarkers that can help diagnose and detect disease activity,and it is important to identify MOGAD patients who need long-term immunotherapy.

关 键 词：神经眼科学 髓鞘少突胶质细胞糖蛋白抗体相关疾病 抗髓鞘少突胶质细胞糖蛋白免疫球蛋白G抗体 炎性脱髓鞘疾病 神经学 

分 类 号：R741[医药卫生—神经病学与精神病学]