脱髓鞘疾病ms本科生修改版.ppt

脱髓鞘疾病 湘雅医院 周文斌 神经纤维指轴突而言，分为有髓和无髓神经纤维。 Nerve fiber or an axon, Myelinated Nerve Fiber Unmyelinated Nerve Fiber 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. 髓鞘：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、脑苷脂与神经节苷脂。Lipide：lipide、sphingomyelin、galactosyl ceramide、ganglioside 生理作用： Physiological functions 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： 髓鞘、轴索与完整的血供 Myelin system: myelin、axon and intact blood supply 脱髓鞘疾病： CNS白质对各种有害因素的反应 Demyelinating disease 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 deficiency syndrome Leukodystrophy 周围神经系统脱髓鞘疾病 Peripheral nerveous demyelination CNS:? White matter disease Demyelinating: mainly acquired MS PML Encephalomyelitis Anti-MAG Disease CNS:? White matter disease Dysmyelinating: mainly inherited Leukodystrophies:? ALD Alexander Canavan disease Krabbe Disease MLD Pelzaeus-Merzbacher Disease Refsum PNS:? Neuropathies Acquired: mainly autoimmune, demyelinating polyneuropathies. Acute:? GBS Chronic:? CIDP Anti-MAG syndrome Multiple Motor Neuropathy (MMN) PNS:? Neuropathies Hereditary: mainly dysmyelinating.  HMSN/CMT Cockayne Syndrome, Krabbe Disease MLD Refsum Disease 多发性硬化 Multiple Sclerosis 概述overview 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 disease Characterized by demyelination and axonal loss 全世界病人超过200万。 Affecting over 2 million persons worldwide 概述overview 最常损害部位是脑室周围白质、视神经、脊髓、脑干传导束及小脑白质。 white matter bordering the lateral ventricles、opticus、spinal cord、pyramidal tract and Cerebellar white matter 引起年轻成人中神经残疾的最常见疾病（美国）。 The most common disabling neurological disease of young people . 概述overview 女性多见