MOG抗体病精编PPT课件.pptxVIP

MOG抗体病南京市江宁医院神经内科 韦存胜Email:neurowei@126.com背景既往研究证实MOG抗体与MS、NMOSD以及ADEM等相关MOG 抗体病是一组独立疾病，其在免疫病理上不同于经典多发性硬化( MS )和AQP4- IgG阳性视神经脊髓炎谱系疾病( NMOSD )Mader S, Gredler V, Schanda K,, et al. Complement activating antibodies to myelin oligodendrocyte glycoprotein in neuromyelitis optica and related disorders. J Neuroinflammation. 2011;8:184.Jarius S, Ruprecht K, Kleiter I, et al. MOG-IgG in NMO and related disorders: a multicenter study of 50 patients. Part 2: epidemiology, clinical presentation, radiological and laboratory features, treatment responses, and long-term outcome. J Neuroinflammation. 2016;13:280.背景专家共识：MOG抗体相关性脑脊髓炎（MOG-EM）诊断标准和检测推荐脱髓鞘病MOG抗体病MOG抗体病概念髓鞘少突胶质细胞糖蛋白（myelin?oligodendrocyte glycoprotein，MOG）抗体介导的的特发性炎性脱髓鞘疾病（idiopathic inflammatory demyelinating disease，IIDDs），称之为MOG抗体介导的IIDDs（简称为“MOG抗体病”）。Chin J Neuroimmunol ＆ Neurol 2017MOG抗体病MOG分子基础髓鞘表面的糖蛋白由位于/wiki/Chromosome_6_(human)6号染色体 p21.3-p22 MOG基因编码参与髓鞘黏附，维持髓鞘结构完整性可作为病原体潜在结合位点MOG sits on the outer layer of the myelin sheath, making it an easy target for antibody attack.Roth MP, Malfroy L, Offer C,etal (July 1995). The human myelin oligodendrocyte glycoprotein (MOG) gene: complete nucleotide sequence and structural characterization. Genomics. 28 (2): 241–50. /wiki/Digital_object_identifierdoi:/10.1006/geno.1995.113710.1006/geno.1995.1137MOG抗体病组织分布MOG myelin oligodendrocyte glycoprotein [ Homo sapiens (human) ] Gene ID: 4340, updated on 8-Jul-2018MOG抗体病流行病学欧美国家报道中MOG抗体病多见于白种人，少见于非洲加勒比人女性多见，男∶女约1∶2.8发病年龄从4-60岁不等，中位年龄在31岁在亚洲人群中，目前尚缺乏该病流行病学的报道Jarius S, Ruprecht K, Kleiter I, et al. MOG-IgG in NMO and related disorders: a multicenter study of 50 patients. Part 2: epidemiology, clinical presentation, radiological and laboratory features, treatment responses, and long-term outcome. J Neuroinflammation. 2016;13:280.MOG抗体病发病机制确切发病机制不明，目前主流观点认为，当嗜神经病毒感染机体时，血-脑屏障被破坏，MOG抗原漏入外周，激活CD4+T细胞，对MOG特异性B细胞募集和激活增加，产生大量MOG-IgG，引起髓鞘损伤。Burrer R, Buchmeier M J, Wolfe T, et al. Exacerbated Pathology of Viral Encephalitis in Mice with