脱髓鞘病(7年制).ppt

Key points-Demyelinating Diseases of CNS 掌握MS概念、病因、发病机制、临床表现、辅助检查、治疗、诊断标准及鉴别诊断。 熟悉视神经脊髓炎概念、临床表现、辅助检查、诊断及治疗。 了解MS病理、预后；急性播散性脑脊髓炎概念、临床表现、诊断及治疗。 Chapter 1 Intraduction 1. Concept: A group of disease characterized by demyelinating of the brain and spinal cord. PATHOLOGY: Demyelination 髓 鞘 构 成 　　 2. Pathologic Findings ① Destruction of the myelin sheaths of CNS; often primarily in white matter, either in multiple small disseminated foci or in larger foci ; ② Infiltration of inflammatory cells in a perivenous distribution; ③ A relative integrity of the axis cylinders in the lesions and a lack of wallerian, the secondary degeneration of fiber tracts. 临床常见脱髓鞘疾病 多发性硬化症(MS) 多发性硬化Multiple Sclerosis,MS 1. Concept: Ms is a kind of autoimmune diseases characterized by demyelination of CNS. Due to its high incidence, chronicity and tendency to attack young adults, it has become one of the most important CNS diseases. There are multiple areas of demyelination within the CNS.The episodes of demyelination are separated in time and place,and classically the disease runs a relapsing-remitting course. (brain and spinal cord) 2. Etiology And Pathogenesis 1) 病毒感染及自身免疫反应：Since the exact cause is uncertain. Immunological mechanisms undoubtedly play a role,although the causation is probably multifactorial. 麻疹病毒,人类噬 T 淋巴细胞病毒(HTLV-I) ,分子模拟, 细胞免疫及体液免疫。 2) 遗传因素 (inherited factor) 3) 环境因素 (environment) 3. Epidemiology Incidence of MS associated with latitude. On moving from a high-prevalence area to a low-prevalence area prior to puberty,the risk of developing MS is higher than in the low-prevalence area; However the move is made following puberty, the risk of the high-prevalence is retained. Heredity may be an important factor. MS associated with the HLA-DR locus on the sixth chromosome, HLA-DR2 express strongly and then -DR3 , B7 and A3 . 4. Path