脱髓鞘疾病医疗及研究管理知识分析.pptVIP

急性播散性脑脊髓炎ADEM pathogenesis Used to believe the relationship with virus,now it had been proved there are no direct relation. Alvord（1985）It is a cell-mediated autoimmune disease,the delayed type hypersensitivity between the myelin and the anti-myelin antigens,. Now it is believed this disease is induced by T cells，the antigen is the component of myelin /oligodendrocyte,perhaps MBP 急性播散性脑脊髓炎ADEM 证据如下 ADEM与EAE、AHLE与超急性EAE有极强的相似性，由T细胞介导，如EAE及超急性EAE可以通过淋巴细胞（非血清）转移给动物使之发病。 对ADEM患者的血及CSF淋巴细胞研究发现，T细胞对MBP的反应性增强。但MBP不是唯一的相关抗原，蛋白质蛋白（PLP）和髓磷脂-少突胶质细胞糖蛋白（MOG）也可以是抗原之一。 急性播散性脑脊髓炎ADEM 病理学Pathology of ADEM 主要是静脉周围炎性脱髓鞘改变。 The demyelination inflammation perivessels 肉眼可见脑组织肿胀，白质静脉扩张。 Brain swelling can be seen by naked eye with phlebectasia in white matter. 急性播散性脑脊髓炎ADEM 微观上，静脉周围水肿，单个核细胞浸润。 For microcosmic,perivessels edema, MNC infiltration are showed. 多数为淋巴细胞、巨噬细胞浸润，浆细胞、粒细胞则少见，有内皮细胞增生。Most of MNC are lymphocytes and macrophages.Plasmacytes and granulocytes are rare,with the proliferation of endothelium. 急性播散性脑脊髓炎ADEM 在AHLE，大体上可见大脑肿胀，点状出血，环形出血。 In AHLE， brain swelling ，annular and point shape blooding. 显微镜下，有纤维样坏死，有中性粒细胞、偶有嗜酸性粒细胞在血管旁浸润。 With microscope,there are fibroid necrosis with the infilitration of granulocyte in vessels。 急性播散性脑脊髓炎ADEM 血浆蛋白、红细胞、粒细胞分布于血管周围。 Plasma protein, erythrocyte and granulocytearoud the vessels 环状出血合并静脉血栓形成。 Annular shape blooding with venous thrombosis. 病变一般不累及灰质。 No lesion in gray matter 急性播散性脑脊髓炎ADEM 临床表现Clinical situation ADEM的临床表现多种多样。它可以无明显症状，而由其他原因行MRI检查时发现脑白质多发病灶。 The clinical ADEM is nonspecific。 It can be just happened to find the multifocal lesions in white matter by MRI without symptoms 急性播散性脑脊髓炎ADEM 爆发性、急性进展性的疾病，出现抽搐、昏迷甚至死亡。 It can be the explosively acute progressive disease with twitch,coma ,even death. 神经症状通常在感染后1～3个星期出现。尤其是麻疹感染后ADEM发生更快，约3～7天。Symptoms of nervous system always occurred 1-3weeks after infection. For measles,3-7days. 急性播散性脑脊髓炎ADEM 头痛、恶心、呕吐、昏迷、妄想、愚钝并持续数天。 Symptoms includes: headache, nausea, vomitus, c