北京協(xié)和中樞神經(jīng)系統(tǒng)脫髓鞘疾病課件

北京協(xié)和中樞神經(jīng)系統(tǒng)脫髓鞘疾病北京協(xié)和中樞神經(jīng)系統(tǒng)脫髓鞘疾病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北京協(xié)和中樞神經(jīng)系統(tǒng)脫髓鞘疾病課件北京協(xié)和中樞神經(jīng)系統(tǒng)脫髓鞘疾病課件北京協(xié)和中樞神經(jīng)系統(tǒng)脫髓鞘疾病課件DifferentialdiagnosisFirstattack:singlelesionormultiplelesions:infection,tumor,infarction,Relapsetype:infarction,embolism,vasculitis,Progressive:

inherited,degenerating,DifferentialdiagnosisFirstatPathologyofMSGrossneuropathologygreyplaques(acuteplaquemaybepink)mayoccuranywhereMicroscopicneuropathologyperivascularlymphocytesinfiltrate;lossofoligodendrocytes;myelinstripping;macrophageinfiltrate;astrogliosis;relativesparingofaxonsPathologyofMSGrossneuropath北京協(xié)和中樞神經(jīng)系統(tǒng)脫髓鞘疾病課件北京協(xié)和中樞神經(jīng)系統(tǒng)脫髓鞘疾病課件北京協(xié)和中樞神經(jīng)系統(tǒng)脫髓鞘疾病課件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北京協(xié)和中樞神經(jīng)系統(tǒng)脫髓鞘疾病課件OpticnerveatrophyOpticnerveatrophy北京協(xié)和中樞神經(jīng)系統(tǒng)脫髓鞘疾病課件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北京協(xié)和中樞神經(jīng)系統(tǒng)脫髓鞘疾病課件InflammatorydemyelinatingdisordersinCNS

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

Monophasic,non-remittingConcentricsclerosis(Balo’s)B北京協(xié)和中樞神經(jīng)系統(tǒng)脫髓鞘疾病課件北京協(xié)和中樞神經(jīng)系統(tǒng)脫髓鞘疾病課件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北京協(xié)和中樞神經(jīng)系統(tǒng)脫髓鞘疾病課件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北京協(xié)和中樞神經(jīng)系統(tǒng)脫髓鞘疾病北京協(xié)和中樞神經(jīng)系統(tǒng)脫髓鞘疾病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北京協(xié)和中樞神經(jīng)系統(tǒng)脫髓鞘疾病課件北京協(xié)和中樞神經(jīng)系統(tǒng)脫髓鞘疾病課件北京協(xié)和中樞神經(jīng)系統(tǒng)脫髓鞘疾病課件DifferentialdiagnosisFirstattack:singlelesionormultiplelesions:infection,tumor,infarction,Relapsetype:infarction,embolism,vasculitis,Progressive:

inherited,degenerating,DifferentialdiagnosisFirstatPathologyofMSGrossneuropathologygreyplaques(acuteplaquemaybepink)mayoccuranywhereMicroscopicneuropathologyperivascularlymphocytesinfiltrate;lossofoligodendrocytes;myelinstripping;macrophageinfiltrate;astrogliosis;relativesparingofaxonsPathologyofMSGrossneuropath北京協(xié)和中樞神經(jīng)系統(tǒng)脫髓鞘疾病課件北京協(xié)和中樞神經(jīng)系統(tǒng)脫髓鞘疾病課件北京協(xié)和中樞神經(jīng)系統(tǒng)脫髓鞘疾病課件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北京協(xié)和中樞神經(jīng)系統(tǒng)脫髓鞘疾病課件OpticnerveatrophyOpticnerveatrophy北京協(xié)和中樞神經(jīng)系統(tǒng)脫髓鞘疾病課件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