4王佳伟+感染与免疫相关性脑脊髓炎(医师会议2015-6-13).ppt

感染与免疫相关性脑脊髓炎Infection and encephalomyelitis;;;;;;概述;神经病毒病可分三类;1.急性病毒感染，随后被宿主免疫系统清除;;三个概念交集; Memory loss Behavioral or psychiatric disturbance, seizures Bizarre movement disorders Immunotherapy responsive Antibodies to: VGKC,NMDAR and GlyR Abs AMPAR,GABAbr,DPPX and others;自身抗体介导性的相关脑炎; 神经系统副肿瘤综合征 ( Paraneoplastic neurological syndrom,PNS);PNS在肿瘤患者中的发病情况;;边缘性脑炎（Limbic encephalitis）;ENCEPHALITIS;Limbic encephalitis;Myelopathy:TM;Myelopathy;Overlapping syndromes;Overlapping syndromes;Case 1;Bien and Bauer. Nervenarzt 2013 ; 84:466–470;10 例抗LGI1脑炎患者临床特点; Dopamine-2 receptor as a putative autoantigen Radiological phenotype localises to D2R expression in human brain ;1684 年Sydenham首先描述 风湿热在神经系统的特征性表现 风湿热患者中的发病率 10% ~30% 病??：A型β- 溶血性链球菌 （Group A β-hemolytic Streptococci,GABHS）感染引起的扁桃体炎和咽喉炎 ;临床特点;抗N-甲基-D-天冬氨酸（NMDA）受体脑炎;*;研究背景;研究背景;抗NMDAR受体脑炎发病机制;;;;正式命名;;;;;; 术中见肿瘤为左卵巢畸胎瘤，大小12×8×7cm，行左附件切除术 ;结 果;线状双链DNA;一年后随诊血清EBV-VCA-IgM转阴;一年后随诊血清抗NMDAR抗体仍阳性;内侧颞叶癫痫：HSV-6慢性感染;NMDAR脑炎病人部分自身免疫性抗体及病原体检测; 抗NMDAR脑炎的脑脊液细胞学演变：类似病毒性脑炎表现 ;头颅MRI;25岁女性布鲁菌病患者，曾误诊为多发性硬化。A.轴位MRI FLAIR像可见右侧中脑大脑脚病变。B.轴位MRI T1WI增强像显示病变呈环形强化;;*;;Myelopathy;Myelopathy;Infections Myelopathies;58岁女性患者发病12月后MRI脊髓像。A.为T2WI矢状位像，可见脊髓中央部高信号，B.为对应的轴位像；C.为Gd-DTPA增强后矢状位T1WI像；D.为对应的轴位像，可见Th4~9双侧皮质脊髓束增强；E.和F.为治疗后3周的T2WI矢状位像及对应的轴位像；G.和H. 为Gd-DTPA增强后矢状位及对应的轴位像，可见治疗后增强病灶消失 ;Viral Myelitis: HTLV-1;26 岁男性,急性起病，出现截瘫和尿潴留，脊髓MRI的T2WI显示脊髓高信号病变 ; 空泡性脊髓病 （HIV感染引起脊髓病变）; ;CSF VZV PCR (+) Vesicular VZV culture;Inflammatory Myelopathies;Transverse myelitis is any myelopathy due toan inflammatory process;Transverse myelitis may result from damage towhite matter, gray matter, or both;Transverse myelitis may be due to monophasic inflammation;Transverse myelitis may be due to underlyingrecurrent/progressive disease;;Wingerchuk DM, et al. Lancet Neurol. 2007;6:805-815 ;Spinal Cord Lesion Phenotype;The Contribution of Immune and Glial Cell Types in Experimental Autoimmune Encephalomyelitis and Multiple Sclerosis;;;A.MRI轴位FLAIR像可见双侧颞叶高