1、脱髓鞘疾病脱髓鞘疾病英文课件英文课件Myelin in CNS is formed by the oligodendrocytesChemical composition:proteolipid,myelin basic protein,2-3 cyclic nucleotide phosphohydrolase,myelin-associated glyco-protein,myelin-oligodendrocyte glyco-protein.Intact myelin is required for action potential conductionMyelined nerve f
2、iber are rich in white matter of cerebral、cerebella、brain stem、spinal cord，optic nerveDemyelinating myelin is broken,axon remains intactDemelinated disorders in CNSCongenitalobtainedleukodystrophyinflammatoryothersprimarysecondaryMSNMONMOADEMBalosInflammatory demyelinating disorders in central nerve
3、 systemMultiple Sclerosis(MS)Multiple Sclerosis(MS)1234 Multiple Sclerosis (MS)Multifocal lesionsMultifocal lesionsThe Diagnostic of MS （Poser,1983)Number of AttackEvidence of More Than One LesionClinicalLab.CSF OCB or IgGA.Clinically DefiniteB.Lab-Supported DefiniteC.Clinically ProbableD.Lab-Suppor
4、ted ProbableA1 2 2A2 2 1 and 1B1 2 1 or 1 +B2 1 2 +B3 1 1 and 1 +C1 2 1C2 1 2C3 1 1 and 1D1 2 0 0 +Optic nerve atrophy lProgressive mutifocal leukoencephalopathylCentral pontine myelinolysislMarchiafava-Bignami diseaselToxic demyelination:chemical agents,COlIrradiation encephalopathylCerebral vascular diseaseX-linked recessive demyelination of cerebral white matter adrenal insufficiency(unresponsive to ACTH)CT:white-matter dz:occipital regions-frontal lprogression-generalized atrophy MRI:hypointense T1/hyperintense T2,atrophic splenium of corpus callosum
