脱髓鞘疾病进修课件

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1、脱髓鞘疾病中南大学湘雅医院神经内科周文斌神经纤维指轴突而言，分为有髓和无髓神经纤维。Nervefiberoranaxon,MyelinatedNerveFiberUnmyelinatedNerveFiber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。Myelinisalayerthatformsaroundnervesformedbywrappedplasmamembraneofmyelinizationcells.髓鞘：蛋白质22%，脂类78%Myelin：protein22％andlipids78％蛋白质:碱性蛋白、脂

2、蛋白、糖蛋白等。Protein：Basicprotein、lipoprotein、glucoprotein脂类:胆固醇、神经鞘磷脂、脑苷脂与神经节苷脂。Lipide：lipide、sphingomyelin、galactosylceramide、ganglioside生理作用：Physiologicalfunctions神经冲动的快速传导；Speedingthetransmissionofimpulses对神经轴突起绝缘、保护作用。Insulationandprotectionofneuraxon髓鞘系统：髓鞘、轴索与完整的血供Myelinsy

3、stem:myelin、axonandintactbloodsupply脱髓鞘疾病：CNS白质对各种有害因素的反应Demyelinatingdisease脱髓鞘脑病Demyelinatingencephalopathy髓鞘构成缺陷性疾病（白质营养不良症）myelinconstructiondeficiencysyndromeLeukodystrophy周围神经系统脱髓鞘疾病PeripheralnerveousdemyelinationCNS: WhitematterdiseaseDemyelinating:mainlyacquiredMSPML

4、Encephalomyelitis(ADEM)Anti-MAGDiseaseCNS: WhitematterdiseaseDysmyelinating:mainlyinheritedLeukodystrophies:ALDAlexanderCanavandiseaseKrabbeDiseaseMLDPelzaeus-MerzbacherDiseaseRefsumPNS: NeuropathiesAcquired:mainlyautoimmune,demyelinatingpolyneuropathies.Acute:GBSChronic:CID

5、PAnti-MAGsyndromeMultipleMotorNeuropathy(MMN)PNS: NeuropathiesHereditary:mainlydysmyelinating.HMSN/CMTCockayneSyndrome,KrabbeDiseaseMLDRefsumDiseaseConductioninaDemyelinatedNerveFiber-KH多发性硬化MultipleSclerosisCNS白质的慢性，炎性，斑块性脱髓鞘，CD4+T细胞介导的自身免疫性疾病。Chronic,progressiveimmune-medi

6、atedCNSdiseaseCharacterizedbydemyelinationandaxonalloss全世界病人超过200万。Affectingover2millionpersonsworldwide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。whitematterborderingthelateralventricles、opticus、spinalcord、pyramidaltractandCerebellarwhitematter引起年轻成人中神经残疾的最常见疾病（美国）。Themostco

7、mmondisablingneurologicaldiseaseofyoungpeople.概述overview女性多见Womenoutnumbermen2:1缓解复发病程，逐渐加重Progressiverelapsing-remittingcourse可能与早期病毒感染有关nospecificcauses,viralmayberesponsible概述overviewVitD缺乏与MSMS具有显著的地理分布特征:在赤道几内亚区MS的发生率几乎为零，在两半球随着纬度的增加而戏剧性的增加在瑞士高海拔区MS发生率低，低海拔区发生率高低纬度区阳光充足

8、，高海拔区紫外光强度高，能够促使大量VitD合成VitD缺乏与MSVitD能抑制DC分化、Th1细胞增殖，减少IL-12、IFN-γ产生VitD能促进