脱髓鞘疾病MS本科生修改版.pptx

脫髓鞘成病湘雅医浣周次效H神经纤维指轴突而言，分为有髓和无髓Nerve fiber or an axon,Myelinated Nerve Fiber Unmyelinated Nerve FiberH髓鞘:紧裹在有髓鞘神经轴突外面的脂 质细胞膜，由髓鞘形成细胞膜组成。Myelin is a layer that forms around nerves formed by wrapped plasma membrane of myelinization cells. EH CNS的髓鞘主要有MBP、PLP等成份。Myelin basic protein (MBP), myelin proteolipid protein are the major component of the central nervous system (CNS) myelin, I 丿(*關鞘：蛋白质22%,脂类78%Myelin: protein 22 % and lipids 78%■蛋白质：碱性蛋白、脂蛋白、糖蛋白等。Protein Basic protein, lipoprotein, glucoprotein■脂类：胆固醇、神经鞘磷脂、脑昔脂与神经节昔脂。 Lipide ： lipide. sphingomyelin. galactosyl ceramide, ganglioside二 生理作用:=￡^註丄二《血functionsH-神经冲动的快速传导；Speeding the transmission of impulses■对神经轴突起绝缘、保护作用。Insulation and protection of neuraxon髓鞘系统:V髓鞘、轴索与完整的血供■ Myelin system: myelin、axon and intact blood supply脱髓鞘疾病:■ ?NS白质対在神有害囱素的反应Demyelinating diseaseH 脱髓鞘脑病 Demyelinating encephalopathy■髓鞘构成缺陷性疾病（白质营养不良症）myelin construction C deficiency syndrome Leukodystrophy■周围神经系统脱髓鞘疾病Peripheral nerveous demyelinationCNS: White matter disease■ Demyelinating: mainly acquired3 MSa PMLa Encephalomyelitisa Anti-MAG DiseaseCNS: White matter disease■ Dysmyelinating: mainly inheritedLeukodystrophies:a ALD Alexandera Canavan diseasea Krabbe Diseasea MLDa Pelzaeus-Merzbacher Diseasea RefsumPNS: Neuropathies■ Acquired: mainly autoimmune, demyelinating polyneuropathies, a Acute: GBS3 Chronic:aCIDPa Anti-MAG syndromea Multiple Motor Neuropathy (MMN)PNS: Neuropathies■ Hereditary: mainly dysmyelinating.3 HMSN/CMT3 Cockayne Syndrome,a Krabbe Diseasea MLDa Refsum DiseaseNervenrcomMyelin GRAY MATTIMicrcgllAil811Unmyolln^t 打xooNormal Conduction of Myelinated Nerve FibersNerve impulseAxonAxonMyelin\ MyelinJ Node of RanvierDemyelination of Nerve Fibers in MS多发性硬化概述oue/viewL _ _三上 T■CNS白质的慢性，炎性，斑块性脱髓鞘，CD 4+ T细胞介导的自身免疫性疾病。Chronic, progressive immune-mediated CNS diseaseQ Characterized by demyelination and axonal loss全世界病人超过200万。Affecting over 2 million persons worldwide概述 oue/view■最常损害部位是脑室周围白质、视神经、 脊髓、脑T传导束及小脑白质。white matter border