中枢神经系统脱髓鞘疾病 Demylinating Diseases of the Central ....ppt

中枢神经系统脱髓鞘疾病 Demyelinating Diseases of the Central Nervous System 　哈医大二院神经科 Chapter 1 Intraduction 1. Concept A group of diseases of the brain and spinal cord in which demyelination is a prominent feature. 2. Pathologic Findings ① Destruction of the myelin sheaths of nerves; ② Infiltration of inflammatory cells in a perivascular distribution; ③ A particular distribution of lesion, often perivenous and primarily in white matter, either in multiple small disseminated foci or in larger foci ; ④ A relative integrity of the axis cylinders in the lesions and a lack of wallerian, the secondary degeneration of fiber tracts. 多发性硬化Multiple Sclerosis 1. Concept Multiple Sclerosis 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 diseases of CNS. 2. Etiology And Pathogenesis 1)病毒感染及自身免疫反应：麻疹病毒、 人类噬 T 淋巴细胞病毒( HTLV-I) 分子模拟、细胞免疫、体液免疫(1,2,3,4) 2)遗传因素 (inherited factor) 3)环境因素 (environment)(4) 3. Epidemiology Incidence of MS associated with latitude. Contacting with some kind of environment may play an important role in the incidence of MS. 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. Pathologic Findings Characteristic: Multiple demyelinated plaques in white matter of CNS.Position: White matter around the lateral ventricles and spinal cord, optic nerve, brain stem and cerebellar.Acute stage: hyperemia,ondema,demyelination, infiltration of inflammatory cells in perivascular distribution. Recovery stage : Astrocyte proliferition, form