北京协和中枢神经系统脱髓鞘疾病课件

北京协和中枢神经系统脱髓鞘疾病北京协和中枢神经系统脱髓鞘疾病MyelininCNSisformedbytheoligodendrocytesChemicalcomposition:proteolipid,myelinbasicprotein,2’-3’cyclicnucleotidephosphohydrolase,myelin-associatedglyco-protein,myelin-oligodendrocyteglyco-protein.MyelininCNSisformedbytheIntactmyelinisrequiredforactionpotentialconduction

IntactmyelinisrequiredforMyelinednervefiberarerichinwhitematterofcerebral、cerebella、brainstem、spinalcord，opticnerveMyelinednervefiberarerichDemyelinating——

myelinisbroken,

axonremainsintactDemyelinating——

DemelinateddisordersinCNSCongenitalobtainedleukodystrophyinflammatoryothersprimarysecondaryMSNMOADEMBalo’sDemelinateddisordersinCNSCoInflammatorydemyelinatingdisordersincentralnervesystemMultipleSclerosis(MS)1Neuromyelitisoptica(NMO)2AcuteDisseminatedEncephalomyelitis(ADEM)3Concentricsclerosis(Balo’sdisease)4InflammatorydemyelinatingdisInflammatorydemyelinatingdisordersInCNSMultipleSclerosis(MS)InflammatorydemyelinatingdisWhatisMultipleSclerosis?MultipleSclerosis(MS)“sclerosis”comesfromtheGreekword“skleros”,meaninghard.Inmultiplesclerosis,hardareascalled“plaques”.“Multiple”referstothemanydifferentareasofthenervoussystemthatmayhavedamagedmyelin.WhatisMultipleSclerosis?MulWhatisMultipleSclerosis?chronicinflammatorydiseaseofCNSmalfunctionoftheimmunesystemwhichleadstoattacksagainstmyelinsheathinsulatingmyelinisdamaged.Thelossofmyelininsulationdegradesthenervetransmissionability.Thusamultitudeofvariousneurologicaldisabilitiescanbeobservedinpatientsaffectedbythisdiseasedependingonwhichnervesaredamaged.WhatisMultipleSclerosis?chEpidemiologyapproximately1.1millionpeopleareaffectedinUSinallpartsoftheworldandinallraces,butwhitesofnorthernEuropeandescenthavethehighestincidence.occurinanyage.usuallydiagnosedinaged15-45years;averageageatdiagnosisis29yearsinwomenand31yearsinmen.femaletomaleratiois2:1.

EpidemiologysymptomsMSwasfirstdescribedbyCruveilhierin1835.AgenerallyvaliddescriptionofMSsymptomswasmadebyCharcotintheyear1868.In1904thedescriptionwassupplementedbyMüller.symptomsMSwasfirstdescribedMultifocallesionsMultifocallesionsMultifocallesionsMultifocallesionssymptomsCommonsymptoms:SensorydisturbanceWeaknessProblemsinwalking/balance/coordinationVisualproblems：opticnerveOtherpossiblesymptoms:BladderproblemSpasticityFatigueFacialweaknessTrigeminalneuralgiaslurredspeechtroubleswallowingDeafnesstemporaryblindnessCognitiveproblemsEpilepsyDepressionsymptomsCommonsymptoms:OthersignsLocalweaknessLocalsensorydisturbancespoorcoordinationofupperandlowerextremitymovements,wide-basedgaitwithinabilitytotandemwalk.

nystagmus,internuclearophthalmoplegia,visualdisturbances,palloroftheopticdisc,Lhermittesign,traversespinalmyelopathy，Brown-sequardsyndromeindifferentlevelsofspinalcordsignsLocalweaknessCourses(multiplephases)Courses(multiplephases)LaboratoryfindingsMagneticResonanceImaging(MRI)willshowpatchesoftissueCSF：WBC，protein，MBP，OB，specificAbsEvokedPotentials：visualevokedpotentials（VEP）auditoryevokedpotentials（BAEP）somatosensoryevokedpotentials（SEP）LaboratoryfindingsMagneticReHowismultiplesclerosisdiagnosed?Time——mutiplephasesSpace——mutifocallesionsExcludeothersHowismultiplesclerosisdiagTheDiagnosticCriteria

ofMS（Poser,1983)NumberofAttackEvidenceofMoreThanOneLesionClinicalLab.CSFOCBorIgGA.ClinicallyDefiniteB.Lab-SupportedDefiniteC.ClinicallyProbableD.Lab-SupportedProbableA122A221and1B121or1+B212+B311and1+C121C212C311and1D1200+TheDiagnosticCriteriaofMSDiagnosticCriteriaforMultipleSclerosis

(McDonaldCriteria，2001）（1）ClinicalPresentationAdditionalDataNeeded2ormoreattacks(relapses)2ormoreobjectiveclinicallesions

None;clinicalevidencewillsuffice

(additionalevidencedesirablebutmustbeconsistentwithMS)2ormoreattacks1objectiveclinicallesionDisseminationinspace,demonstratedby:

MRI

orapositiveCSFand2ormoreMRIlesionsconsistentwithMSorfurtherclinicalattackinvolvingdifferentsite1attack2ormoreobjectiveclinicallesionsDisseminationintime,demonstratedby:MRIorsecondclinicalattack1attack1objectiveclinicallesion(monosymptomaticpresentation)Disseminationinspacebydemonstratedby:MRIorpositiveCSFand2ormoreMRIlesionsconsistentwithMSandDisseminationintimedemonstratedby:MRIorsecondclinicalattackDiagnosticCriteriaforMultipDiagnosticCriteriaforMultipleSclerosis

(McDonaldCriteria，2001）(2)Insidiousneurologicalprogression

suggestiveofMS

(primaryprogressiveMS)

PositiveCSFand

Disseminationinspacedemonstratedby:MRIevidenceof9ormoreT2brainlesionsor2ormorespinalcordlesionsor4-8brainand1spinalcordlesionorpositiveVEPwith4-8MRIlesionsorpositiveVEPwith<4brainlesionsplus1spinalcordlesionand

Disseminationintimedemonstratedby:MRIorcontinuedprogressionfor1year

DiagnosticCriteriaforMultip北京协和中枢神经系统脱髓鞘疾病课件北京协和中枢神经系统脱髓鞘疾病课件北京协和中枢神经系统脱髓鞘疾病课件DifferentialdiagnosisFirstattack:singlelesionormultiplelesions:infection,tumor,infarction,Relapsetype:infarction,embolism,vasculitis,Progressive:

inherited,degenerating,DifferentialdiagnosisFirstatPathologyofMSGrossneuropathologygreyplaques(acuteplaquemaybepink)mayoccuranywhereMicroscopicneuropathologyperivascularlymphocytesinfiltrate;lossofoligodendrocytes;myelinstripping;macrophageinfiltrate;astrogliosis;relativesparingofaxonsPathologyofMSGrossneuropath北京协和中枢神经系统脱髓鞘疾病课件北京协和中枢神经系统脱髓鞘疾病课件北京协和中枢神经系统脱髓鞘疾病课件WhatCausesMS?TheexactcauseofMSisnotknownEnvironmentalFactors：

Geographyisclearlyanimportantfactortherateisapproximatelytwiceasbelowthe37thparallel.GeneticFactors：

MSisnotstrictlyaninheriteddisorder.SusceptibilitytoMSprobablyhasageneticcomponent.

WhatCausesMS?TheexactcausePathogenesisofMS:incompletelyunderstood.Immune-mediateddisorder

withan

initialtrigger

Molecularmimicry

OligodendrocytesusceptibleCNSinfectionsmayalsoleadtothetransmigrationofactivatedTlymphocytesintotheCNS,whichinitiatetheprocessPathogenesisofMS:incompleteHowisMSTreated?ImmunotherapyCorticosteroidscorticosteroidscanreducethedurationofsymptoms,theydonotcureMS.UsedinAcuteattacks

intravenousmethylprednisolone1000mgdailyforthreedays.

Prednisone:Cliniciansdifferonwhethertotaperofftreatmentwithoralprednisonefortwoweeks,butthisprobablydoesnotimproveresultsandincreasessideeffects.HowisMSTreated?ImmunotherapInterferons

Betainterferondrugshaveshowntobeeffectiveintreatingtherelapsing-remittingtypeofMS.InterferonsImmunosuppressantsmethotrexate,cyclophosphamide,cyclosporine,mitoxantrone.OthersIvIgplasmaexchangeImmunosuppressantsSymptomaticmanagementBladderfrequencyandurgencywilloftenrespondtooxybutynin.Painandspasmsfromspasticlimbsusuallyrespondtobaclofen.Emotionallabilitywithpathologicallaughingorcryingcanbemanagedwithatricyclicantidepressant.Amantadinereducesfatigueinhalfthepatients.Moredifficulttomanagearepain,sexualdysfunction,weakness,dysesthesiaandothersensorysymptoms,tremor,ataxia,andcognitivechange,buteventhesemayrespondtovarioustherapeuticapproaches.Itisimportanttorecognisethathalfofpatientswithmultiplesclerosiswillbecomedepressedandthattherapyandcounsellingmaybenecessary.SymptomaticmanagementPrognosisMortality/Morbidity:

LifeexpectancyisshortenedonlyslightlywithMS,andthesurvivalrateislinkedtodisability.Usually,deathisduetosecondarycomplications(50-66%),suchaspulmonaryorrenalcauses,suicide,primarycomplications,andcausesotherthanMSseeninthegeneralpopulation.PrognosisMortality/Morbidity:InflammatorydemyelinatingdisordersinCNS

Neuromyelitisoptica(NMO)InflammatorydemyelinatingdisSimilaritieswithMSmutifocallesionsmutiplephasesimmuno-mediatedinflammatorydemyelinationinCNSSimilaritieswithMSmutifocalDifferenceswithMS

----mutifocallesionsMyelitis：longlesion≧3vertebralsegments.Optisneuritis：Brainlesions：atypicalforMS.Multiplephases：morefrequentlyarelapsing-remittingcourse.DifferenceswithMS

----mutif北京协和中枢神经系统脱髓鞘疾病课件OpticnerveatrophyOpticnerveatrophy北京协和中枢神经系统脱髓鞘疾病课件DifferenceswithMS

----PathologicalfeaturesofNMOdemyelinationandnecrosisprominentvascularfibrosisandhyalinizationwithinthelesionsperivasculardepositionofIgGandcomplementperivascularinfiltratesofpolymorphonuclearcells,leucocytes,plasmacells,eosinophilsglialscarsarelessfrequentandusuallyonlypartialincontrasttotypicalMSlesionsDifferenceswithMS

----PathDifferenceswithMS

----pathogenesisofNMOhumoralimmunitytargetantigenisaquaporin-4,thedominantwaterchannelin(CNS)IgG-antibodytoaquaporin-4,namedNMO-IgGDifferenceswithMS

----pathDiagnositiccriteriaforNMODiagnositiccriteriaforNMODifferentialdiagnosisIschemiaofopticnerveAcutemyelitisVasculardiseaseofthespinalcordVasculitisConnectivetissuediseaseDifferentialdiagnosisIschemiaDifferenceswithMS

----Treatmentacutephase：intravenoussteroidsandplasmaexchangetherapy.Topreventrelapse：oralsteroidmedicationandimmunosuppressivedrugs.DifferenceswithMS

----TreatInflammatorydemyelinatingdisordersinCNSAcuteDisseminatedEncephalomyelitis(ADEM)InflammatorydemyelinatingdisAcuteDisseminatedEncephalomyelitisQuiterapidinonset,oftengettingsickoveraperiodofafewdaysMultifocalneurologicaldysfunction,involvingbrain、spinalcord、meningesMRI：multifocallesionsinCNSCSF：WBCcountnormalormildelevated,immuneproteinslevelelevatedGoodresponsetotherapy：corticosteroidtreatmentsPrecipitatingEvents：recentinfectionsandcertainrecentvaccinations，certainmedications,trauma,idiopathic

MostADEMaremonophasic，someareMDEM（multiphasicDisseminatedEncephalomyelitis),ordeveloptoMSAcuteDisseminatedEncephalomy北京协和中枢神经系统脱髓鞘疾病课件InflammatorydemyelinatingdisordersinCNS

Concentricsclerosis(Balo’sdisease)InflammatorydemyelinatingdisConcentricsclerosis(Balo’s)Balodisease(concentricsclerosis)isararevariantofinflammatorydemyelinatingdiseaseDiagnosisismadeuponbiopsywhichshowsalternatingbandsofmyelinatinganddemyelinatingfibersinwhitematter.

Monophasic,non-remittingConcentricsclerosis(Balo’s)B北京协和中枢神经系统脱髓鞘疾病课件北京协和中枢神经系统脱髓鞘疾病课件OtherdemyelinatingdisordersProgressivemutifocalleukoencephalopathyCentralpontinemyelinolysisMarchiafava-BignamidiseaseToxicdemyelination:chemicalagents,COIrradiationencephalopathyCerebralvasculardiseaseOtherdemyelinatingdisordersPOtherdemyelinatingdisorders

Centralpontinemyelinolysis，CPMOtherdemyelinatingdisorders

Centralpontinemyelinolysis，CPMCentralpontinemyelinolysis(CPM)isanuncommonconsequence

ofcertainmetabolicderangements.Theseincluderapidcorrection

ofhyponatremia,aswellashyperosmolarconditions,suchas

hyperglycemia.ThemicroscopicappearanceofCPMislossof

myelinwithsparingofaxons,withoutevidenceofinflammation

.Centralpontinemyelinolysis，COtherdemyelinatingdisorders

Progressivemultifocalleukoencephalopathy(PML)Itisademyelinatingdiseasecausesbyapolyomavirus(theJCvirus).TheJCviruspreferentiallyinfectsoligodendrocytes,thusdemyelinationinCNS.immunosuppressedpatientsandAIDSpatients,areatrisk.Neurologicsymptomsdependonthelocationofthelesionsrelentlesslyprogressive.Otherdemyelinatingdisorders北京协和中枢神经系统脱髓鞘疾病课件Otherdemyelinatingdisorders

DelayedEncephalopathyofAcuteCarbonMonoxideIntoxicationOtherdemyelinatingdisordersOtherdemyelinatingdisorders

Marchiafava-BignamidiseaseOtherdemyelinatingdisordersleukodystrophyinheriteddysmyelinatingdiseaseadrenoleukodystrophy

metachromaticleukodystrophy

spongydegeneration(Canavandisease)

globoidcell(Krabbe)leukodystrophy

Alexanderdisease

Pelizaeus-Merzbacherdisease

Cockaynesyndrome

leukodystrophyinheriteddysmyeadrenoleukodystrophyX-linkedrecessivedemyelinationofcerebralwhitematteradrenalinsufficiency(unresponsivetoACTH)CT:white-matterdz:occipitalregions-->frontalprogression-->generalizedatrophyMRI:hypointenseT1/hyperintenseT2,atrophicspleniumofcorpuscallosumadrenoleukodystrophyX-linkedrmetachromaticleukodystrophydymyelinatingdisease

autosomalrecessivearylsulfataseA--absentfromurineandserummostpresentby2yrs,dieat3-4yrsmayariseatanyageMR:hypointenseT1/hyperintenseT2,ofwhitematter,primarilyincentrumsemiovale

metachromaticleukodystrophydmetachromaticleukodystrophymetachromaticleukodystrophy北京协和中枢神经系统脱髓鞘疾病北京协和中枢神经系统脱髓鞘疾病MyelininCNSisformedbytheoligodendrocytesChemicalcomposition:proteolipid,myelinbasicprotein,2’-3’cyclicnucleotidephosphohydrolase,myelin-associatedglyco-protein,myelin-oligodendrocyteglyco-protein.MyelininCNSisformedbytheIntactmyelinisrequiredforactionpotentialconduction

IntactmyelinisrequiredforMyelinednervefiberarerichinwhitematterofcerebral、cerebella、brainstem、spinalcord，opticnerveMyelinednervefiberarerichDemyelinating——

myelinisbroken,

axonremainsintactDemyelinating——

DemelinateddisordersinCNSCongenitalobtainedleukodystrophyinflammatoryothersprimarysecondaryMSNMOADEMBalo’sDemelinateddisordersinCNSCoInflammatorydemyelinatingdisordersincentralnervesystemMultipleSclerosis(MS)1Neuromyelitisoptica(NMO)2AcuteDisseminatedEncephalomyelitis(ADEM)3Concentricsclerosis(Balo’sdisease)4InflammatorydemyelinatingdisInflammatorydemyelinatingdisordersInCNSMultipleSclerosis(MS)InflammatorydemyelinatingdisWhatisMultipleSclerosis?MultipleSclerosis(MS)“sclerosis”comesfromtheGreekword“skleros”,meaninghard.Inmultiplesclerosis,hardareascalled“plaques”.“Multiple”referstothemanydifferentareasofthenervoussystemthatmayhavedamagedmyelin.WhatisMultipleSclerosis?MulWhatisMultipleSclerosis?chronicinflammatorydiseaseofCNSmalfunctionoftheimmunesystemwhichleadstoattacksagainstmyelinsheathinsulatingmyelinisdamaged.Thelossofmyelininsulationdegradesthenervetransmissionability.Thusamultitudeofvariousneurologicaldisabilitiescanbeobservedinpatientsaffectedbythisdiseasedependingonwhichnervesaredamaged.WhatisMultipleSclerosis?chEpidemiologyapproximately1.1millionpeopleareaffectedinUSinallpartsoftheworldandinallraces,butwhitesofnorthernEuropeandescenthavethehighestincidence.occurinanyage.usuallydiagnosedinaged15-45years;averageageatdiagnosisis29yearsinwomenand31yearsinmen.femaletomaleratiois2:1.

EpidemiologysymptomsMSwasfirstdescribedbyCruveilhierin1835.AgenerallyvaliddescriptionofMSsymptomswasmadebyCharcotintheyear1868.In1904thedescriptionwassupplementedbyMüller.symptomsMSwasfirstdescribedMultifocallesionsMultifocallesionsMultifocallesionsMultifocallesionssymptomsCommonsymptoms:SensorydisturbanceWeaknessProblemsinwalking/balance/coordinationVisualproblems：opticnerveOtherpossiblesymptoms:BladderproblemSpasticityFatigueFacialweaknessTrigeminalneuralgiaslurredspeechtroubleswallowingDeafnesstemporaryblindnessCognitiveproblemsEpilepsyDepressionsymptomsCommonsymptoms:OthersignsLocalweaknessLocalsensorydisturbancespoorcoordinationofupperandlowerextremitymovements,wide-basedgaitwithinabilitytotandemwalk.

nystagmus,internuclearophthalmoplegia,visualdisturbances,palloroftheopticdisc,Lhermittesign,traversespinalmyelopathy，Brown-sequardsyndromeindifferentlevelsofspinalcordsignsLocalweaknessCourses(multiplephases)Courses(multiplephases)LaboratoryfindingsMagneticResonanceImaging(MRI)willshowpatchesoftissueCSF：WBC，protein，MBP，OB，specificAbsEvokedPotentials：visualevokedpotentials（VEP）auditoryevokedpotentials（BAEP）somatosensoryevokedpotentials（SEP）LaboratoryfindingsMagneticReHowismultiplesclerosisdiagnosed?Time——mutiplephasesSpace——mutifocallesionsExcludeothersHowismultiplesclerosisdiagTheDiagnosticCriteria

ofMS（Poser,1983)NumberofAttackEvidenceofMoreThanOneLesionClinicalLab.CSFOCBorIgGA.ClinicallyDefiniteB.Lab-SupportedDefiniteC.ClinicallyProbableD.Lab-SupportedProbableA122A221and1B121or1+B212+B311and1+C121C212C311and1D1200+TheDiagnosticCriteriaofMSDiagnosticCriteriaforMultipleSclerosis

(McDonaldCriteria，2001）（1）ClinicalPresentationAdditionalDataNeeded2ormoreattacks(relapses)2ormoreobjectiveclinicallesions

None;clinicalevidencewillsuffice

(additionalevidencedesirablebutmustbeconsistentwithMS)2ormoreattacks1objectiveclinicallesionDisseminationinspace,demonstratedby:

MRI

orapositiveCSFand2ormoreMRIlesionsconsistentwithMSorfurtherclinicalattackinvolvingdifferentsite1attack2ormoreobjectiveclinicallesionsDisseminationintime,demonstratedby:MRIorsecondclinicalattack1attack1objectiveclinicallesion(monosymptomaticpresentation)Disseminationinspacebydemonstratedby:MRIorpositiveCSFand2ormoreMRIlesionsconsistentwithMSandDisseminationintimedemonstratedby:MRIorsecondclinicalattackDiagnosticCriteriaforMultipDiagnosticCriteriaforMultipleSclerosis

(McDonaldCriteria，2001）(2)Insidiousneurologicalprogression

suggestiveofMS

(primaryprogressiveMS)

PositiveCSFand

Disseminationinspacedemonstratedby:MRIevidenceof9ormoreT2brainlesionsor2ormorespinalcordlesionsor4-8brainand1spinalcordlesionorpositiveVEPwith4-8MRIlesionsorpositiveVEPwith<4brainlesionsplus1spinalcordlesionand

Disseminationintimedemonstratedby:MRIorcontinuedprogressionfor1year

DiagnosticCriteriaforMultip北京协和中枢神经系统脱髓鞘疾病课件北京协和中枢神经系统脱髓鞘疾病课件北京协和中枢神经系统脱髓鞘疾病课件DifferentialdiagnosisFirstattack:singlelesionormultiplelesions:infection,tumor,infarction,Relapsetype:infarction,embolism,vasculitis,Progressive:

inherited,degenerating,DifferentialdiagnosisFirstatPathologyofMSGrossneuropathologygreyplaques(acuteplaquemaybepink)mayoccuranywhereMicroscopicneuropathologyperivascularlymphocytesinfiltrate;lossofoligodendrocytes;myelinstripping;macrophageinfiltrate;astrogliosis;relativesparingofaxonsPathologyofMSGrossneuropath北京协和中枢神经系统脱髓鞘疾病课件北京协和中枢神经系统脱髓鞘疾病课件北京协和中枢神经系统脱髓鞘疾病课件WhatCausesMS?TheexactcauseofMSisnotknownEnvironmentalFactors：

Geographyisclearlyanimportantfactortherateisapproximatelytwiceasbelowthe37thparallel.GeneticFactors：

MSisnotstrictlyaninheriteddisorder.SusceptibilitytoMSprobablyhasageneticcomponent.

WhatCausesMS?TheexactcausePathogenesisofMS:incompletelyunderstood.Immune-mediateddisorder

withan

initialtrigger

Molecularmimicry

OligodendrocytesusceptibleCNSinfectionsmayalsoleadtothetransmigrationofactivatedTlymphocytesintotheCNS,whichinitiatetheprocessPathogenesisofMS:incompleteHowisMSTreated?ImmunotherapyCorticosteroidscorticosteroidscanreducethedurationofsymptoms,theydonotcureMS.UsedinAcuteattacks

intravenousmethylprednisolone1000mgdailyforthreedays.

Prednisone:Cliniciansdifferonwhethertotaperofftreatmentwithoralprednisonefortwoweeks,butthisprobablydoesnotimproveresultsandincreasessideeffects.HowisMSTreated?ImmunotherapInterferons

Betainterferondrugshaveshowntobeeffectiveintreatingtherelapsing-remittingtypeofMS.InterferonsImmunosuppressantsmethotrexate,cyclophosphamide,cyclosporine,mitoxantrone.OthersIvIgplasmaexchangeImmunosuppressantsSymptomaticmanagementBladderfrequencyandurgencywilloftenrespondtooxybutynin.Painandspasmsfromspasticlimbsusuallyrespondtobaclofen.Emotionallabilitywithpathologicallaughingorcryingcanbemanagedwithatricyclicantidepressant.Amantadinereducesfatigueinhalfthepatients.Moredifficulttomanagearepain,sexualdysfunction,weakness,dysesthesiaandothersensorysymptoms,tremor,ataxia,andcognitivechange,buteventhesemayrespondtovarioustherapeuticapproaches.Itisimportanttorecognisethathalfofpatientswithmultiplesclerosiswillbecomedepressedandthattherapyandcounsellingmaybenecessary.SymptomaticmanagementPrognosisMortality/Morbidity:

LifeexpectancyisshortenedonlyslightlywithMS,andthesurvivalrateislinkedtodisability.Usually,deathisduetosecondarycomplications(50-66%),suchaspulmonaryorrenalcauses,suicide,primarycomplications,andcausesotherthanMSseeninthegeneralpopulation.PrognosisMortality/Morbidity:InflammatorydemyelinatingdisordersinCNS

Neuromyelitisoptica(NMO)InflammatorydemyelinatingdisSimilaritieswithMSmutifocallesionsmutiplephasesimmuno-mediatedinflammatorydemyelinationinCNSSimilaritieswithMSmutifocalDifferenceswithMS

----mutifocallesionsMyelitis：longlesion≧3vertebralsegments.Optisneuritis：Brainlesions：atypicalforMS.Multiplephases：morefrequentlyarelapsing-remittingcourse.DifferenceswithMS

----mutif北京协和中枢神经系统脱髓鞘疾病课件OpticnerveatrophyOpticnerveatrophy北京协和中枢神经系统脱髓鞘疾病课件DifferenceswithMS

----PathologicalfeaturesofNMOdemyelinationandnecrosisprominentvascularfibrosisandhyalinizationwithinthelesionsperivasculardepositionofIgGandcomplementperivascularinfiltratesofpolymorphonuclearcells,leucocytes,plasmacells,eosinophilsglialscarsarelessfrequentandusuallyonlypartialincontrasttotypicalMSlesionsDifferenceswithMS

----PathDifferenceswithMS

----pathogenesisofNMOhumoralimmunitytargetantigenisaquaporin-4,thedominantwaterchannelin(CNS)IgG-antibodytoaquaporin-4,namedNMO-IgGDifferenceswithMS

----pathDiagnositiccriteriaforNMODiagnositiccriteriaforNMODifferentialdiagnosisIschemiaofopticnerveAcutemyelitisVasculardiseaseofthespinalcordVasculitisConnectivetissuediseaseDifferentialdiagnosisIschemiaDifferenceswithMS

----Treatmentacutephase：intravenoussteroidsandplasmaexchangetherapy.Topreventrelapse：oralsteroidmedicationandimmunosuppressivedrugs.DifferenceswithMS

----TreatInflammatorydemyelinatingdisordersinCNSAcuteDisseminatedEncephalomyelitis(ADEM)InflammatorydemyelinatingdisAcuteDisseminatedEncephalomyelitisQuiterapidinonset,oftengettingsickoveraperiodofafewdaysMultifocalneurologicaldysfunction,involvingbrain、spinalcord、meningesMRI：multifocallesionsinCNSCSF：WBCcountnormalormildelevated,immuneproteinslevelelevatedGoodresponsetotherapy：corticosteroidtreatmentsPrecipitatingEvents：recentinfectionsandcertainrecentvaccinations，certainmedications,trauma,idiopathic

MostADEMaremonophasic，someare