神經(jīng)病學(xué)課件：Multiple-Sclerosis_第1頁(yè)
神經(jīng)病學(xué)課件：Multiple-Sclerosis_第2頁(yè)
神經(jīng)病學(xué)課件：Multiple-Sclerosis_第3頁(yè)
神經(jīng)病學(xué)課件：Multiple-Sclerosis_第4頁(yè)
神經(jīng)病學(xué)課件：Multiple-Sclerosis_第5頁(yè)
已閱讀5頁(yè)，還剩63頁(yè)未讀， 繼續(xù)免費(fèi)閱讀

下載本文檔

版權(quán)說(shuō)明：本文檔由用戶提供并上傳，收益歸屬內(nèi)容提供方，若內(nèi)容存在侵權(quán)，請(qǐng)進(jìn)行舉報(bào)或認(rèn)領(lǐng)

文檔簡(jiǎn)介

MultipleSclerosisMultipleSclerosisDefinitionofMultipleSclerosisAninflammatorydemyelinatingdiseaseoftheCNSwherethereis:Disseminationinspace(DIS)Disseminationintime(DIT)NoalternativeneurologicdiseaseMSisaclinicaldiagnosisDefinitionofMultipleSclerosEpidemiologyofMSAgeonset20–50yearsoldWomenare2timesmorelikelytodevelopMS500,000casesinUSOver2.5millionpeoplearoundtheworldMoreprevalentinwhitesofnorthernEuropeanancestryVitaminD3GeneticInfluencesEpidemiologyofMSAgeonset20ProdromalandpotentialcausalpathwayforMSRamagopalanSetal.LancetNeurol2010;9:727–39ProdromalandpotentialcausalPATHOPHYSIOLOGYOFMSDestructionofMyelin,oligodendrocytes,nerveaxonsHypothesisofmolecularmimicry:infectedvirussharewithmyelinmoleculescommonantigensTargetedantigensmimickedbyvirusMyelinbasicprotein(MBP)Myelinoligodendrocyteglycoprotein(MOG)Proteolipidprotein(PLP)Myelinassociatedglycoprotein(MAG)PATHOPHYSIOLOGYOFMSDestructiPATHOPHYSIOLOGYOFMSCellsCD4TH1,CD4TH2andCD8TcellsMacrophagesandmicroglialcellsMastcellsBcellsCytokines,chemokinesandadhesionmolecules

Inerleukinfamily(IL-4,10,12)IFN-gammaALCAM(Activatedleukocytecelladhesionmolecule)TumournecrosisfactorreceptorsuperfamilyPATHOPHYSIOLOGYOFMSCellsTheimmunopathogenesisofMSGoleRetal.ActaNeurolScand2011:124:75–84TheimmunopathogenesisofMSGo

MultipleSclerosisSubtypes

(LublinF,etalNeurology1996)AsymptomaticSymptomaticRelapsing-remitting(85%atonset)Primaryprogressive(10%)SecondaryProgressive(transitionalform)ProgressiveRelapsing(5%)

MultipleSclerosisSubtypes

(MultipleSclerosisSubtypesMultipleSclerosisSubtypesSYMPTOMSOFMSVisualdisturbancesBlurredvision,diplopia,intranuclearophthalmoplegiaMarcusGunnpupilFatigueMotorSpasticity,paresis,dysarthria,spasms,muscleweaknessSensorychangesHeatintoleranceParaesthesia,neuralgiaLhermitte’ssign(dorsalcolumnsign)inducedbyheadflexionCerebellumAtaxia,nystagmus-tremor-dysarthria(Charcottriads)Bladder/bowelurgencyandincontinenceCognitivedeficitsSYMPTOMSOFMSVisualdisturbanBlurredvisionBlurredvisionDiagnosticapproachesCSF:nonspecificincellularprofileCSFIgGIndexOligoclonalbandsElectrophysiologyVEP(visualevokedpotentials):75%abnormalBAEP(brainstemauditoryevokedresponse)—30%abnormalSSER(somatosensoryevokedresponse)Imaging:MRIisusedroutinely,mosteffectiveDiagnosticapproachesCSF:nonCSFIgGINDEXCSFIgGtoCSFalbuminratiocomparedtoserumIgGtoserumalbuminratio

CSFIgG/CSFalbumin serumIgG/serumalbuminReferencevalue<0.85~70%patientsshowhighervalueCSFIgGINDEXCSFIgOligoclonalBandsBaker'sClinicalNeurology,CDROM-2003Lhermitte’ssignOligoclonalBandsBaker'sCliniVisualEvokedPotentials

(Baker’sClinNeurol2003)VisualEvokedPotentials

(BakeMRI:

FLAIR&T1withGadolinium

(NoseworthyJ,etalNEJM,2000)MRI:

FLAIR&T1withGadoliniMRI:T1“BlackHoles”MRI:T1“BlackHoles”MRI:SagittalViewsMRI:SagittalViewsMRI:SpinalImagingMRI:SpinalImagingDIAGNOSTICCRITERIA-12attackswithclinicalevidenceofatleast2lesions2attackswithclinicalevidenceof1lesion

+DISIattack+clinicalevidenceof2lesions+DITNotes:1.Anattack:durationofsymptomshould>24hwithoutfeverorinfection.Demonstratedby(1)examination.(2)historicaleventscharacteristicofMSbutwithnoobjectiveneurologicalfindings.2.Atleastoneattackmustbesupportedbyobjectivefindingsbeforediagnosiscanbemade3.“Fourpoints”diagnosisprocess:attack+clinicalevidence3.DIS=disseminationinspace,DIT=disseminationintimeDIAGNOSTICCRITERIA-12attacksDIAGNOSTICCRITERIA-2Clinicallyisolatedsyndromes1attack+1objectiveclinicalevidence+DIS+DITPrimaryprogressive:Oneyearofdiseaseprogression2of3ofthefollowingDISof>=1T2lesionsofMScharateristicDISof>=2T2lesionsinspinalcordCSFologobandofIgGDIAGNOSTICCRITERIA-2ClinicallPolmanGH,etal.ANNNEUROL2011;69:292–302PolmanGH,etal.McDonaldCriteria(2010Revision)McDonaldCriteria(2010RevisiMcDonaldCriteria(2010Revision)DIT=disseminationintime,DIS=disseminationinspaceMcDonaldCriteria(2010RevisiNotesforMcDonaldCriteria(2010revision)NotesforMcDonaldCriteria(2DifferentialdiagnosisLacunarinfarctionAcutedisseminatedencephalomyelitis(ADEM)Cerebralautosomaldominantateriopathy,subcorticalinfacrts,andleukoencepholopathies(CADASIL)Mitochodiralencephalopathywithlacticacidosisandstroke(MELAS)VasculitisVitaminB12deficiency(spinalcordlesions)DifferentialdiagnosisLacunarDifferentialdiagnosisCaution:Age<15or>60Patientsneverexperiencedvisual,sensory,orbladdersymptomsRoutinetestofthefollowingishelpfulESRSerumVitB12Antinuclearantibody(ANA)DifferentialdiagnosisCaution:TreatmentPrinciplesAcuteattacks-corticosteroidsDiseasemodifyingagentsSymptommanagementagentsTreatmentPrinciplesAcuteattAcuteattacks-corticosteroidsMethylprednisolone,1g/dfor3days,reducedosagein4wPrednisone,80mg/dfor1w,reducedosagein4~6wSideeffects:gastriculcer,fluidretention,potassiumloss,osteoporosisAcuteattacks-corticosteroidsMDiseasemodifyingagentsBeta-interferon(2yrs)Betainterferon1a,22-44ug,sc.twiceaweekBetainterferon1b,250ugsc,q.o.dMoleculardecoy(MBPanalog)Glatirameracetate,20mg,sc,qdfor1yrImmunodepressantMitoxantrone15mgivgttevery3monthForPrimaryprogressiveMSMonoclonalantibody(againstintegrin)Natalizamab300ivgtt/monthUnderevaluationDiseasemodifyingagentsBeta-SideeffectsofMSmedicationLocalinjectionsiteirritation/reactionsFlulikesymptomsRiseinliverenzymesDecreasedwhitecellcountandplateletsOpportunisticinfectionsProgressivemultifocalleukoencephalopathy(PML)SideeffectsofMSmedicationLSymptommanagementSpasm(alospasm)Baclofen5mgtid;tizanidine2mgtid;diazepam10mgqn;dantrolene10mgtidFatigueAmantadine0.1bid;phenytoin0.2qdatmorning;RitalindrugsBladderproblemsUrinaryretention:cholinergicdrugsIncontinence:imipramine10mgqidorcatheterizationTremor:artane2mgtid;propranolol10mgtidSymptommanagementSpasm(alospQuestionsinthissectionWhatismultiplesclerosis(definition)?Clinicalfeaturesofmultiplesclerosis?WhatisdiagnosticcriteriaforgeneralMS?Treatmentprinciplesofmultiplesclerosis?QuestionsinthissectionWhatThankyou

Anyquestions?Thankyou

Anyquestions?

MultipleSclerosisMultipleSclerosisDefinitionofMultipleSclerosisAninflammatorydemyelinatingdiseaseoftheCNSwherethereis:Disseminationinspace(DIS)Disseminationintime(DIT)NoalternativeneurologicdiseaseMSisaclinicaldiagnosisDefinitionofMultipleSclerosEpidemiologyofMSAgeonset20–50yearsoldWomenare2timesmorelikelytodevelopMS500,000casesinUSOver2.5millionpeoplearoundtheworldMoreprevalentinwhitesofnorthernEuropeanancestryVitaminD3GeneticInfluencesEpidemiologyofMSAgeonset20ProdromalandpotentialcausalpathwayforMSRamagopalanSetal.LancetNeurol2010;9:727–39ProdromalandpotentialcausalPATHOPHYSIOLOGYOFMSDestructionofMyelin,oligodendrocytes,nerveaxonsHypothesisofmolecularmimicry:infectedvirussharewithmyelinmoleculescommonantigensTargetedantigensmimickedbyvirusMyelinbasicprotein(MBP)Myelinoligodendrocyteglycoprotein(MOG)Proteolipidprotein(PLP)Myelinassociatedglycoprotein(MAG)PATHOPHYSIOLOGYOFMSDestructiPATHOPHYSIOLOGYOFMSCellsCD4TH1,CD4TH2andCD8TcellsMacrophagesandmicroglialcellsMastcellsBcellsCytokines,chemokinesandadhesionmolecules

Inerleukinfamily(IL-4,10,12)IFN-gammaALCAM(Activatedleukocytecelladhesionmolecule)TumournecrosisfactorreceptorsuperfamilyPATHOPHYSIOLOGYOFMSCellsTheimmunopathogenesisofMSGoleRetal.ActaNeurolScand2011:124:75–84TheimmunopathogenesisofMSGo

MultipleSclerosisSubtypes

(LublinF,etalNeurology1996)AsymptomaticSymptomaticRelapsing-remitting(85%atonset)Primaryprogressive(10%)SecondaryProgressive(transitionalform)ProgressiveRelapsing(5%)

MultipleSclerosisSubtypes

(MultipleSclerosisSubtypesMultipleSclerosisSubtypesSYMPTOMSOFMSVisualdisturbancesBlurredvision,diplopia,intranuclearophthalmoplegiaMarcusGunnpupilFatigueMotorSpasticity,paresis,dysarthria,spasms,muscleweaknessSensorychangesHeatintoleranceParaesthesia,neuralgiaLhermitte’ssign(dorsalcolumnsign)inducedbyheadflexionCerebellumAtaxia,nystagmus-tremor-dysarthria(Charcottriads)Bladder/bowelurgencyandincontinenceCognitivedeficitsSYMPTOMSOFMSVisualdisturbanBlurredvisionBlurredvisionDiagnosticapproachesCSF:nonspecificincellularprofileCSFIgGIndexOligoclonalbandsElectrophysiologyVEP(visualevokedpotentials):75%abnormalBAEP(brainstemauditoryevokedresponse)—30%abnormalSSER(somatosensoryevokedresponse)Imaging:MRIisusedroutinely,mosteffectiveDiagnosticapproachesCSF:nonCSFIgGINDEXCSFIgGtoCSFalbuminratiocomparedtoserumIgGtoserumalbuminratio

CSFIgG/CSFalbumin serumIgG/serumalbuminReferencevalue<0.85~70%patientsshowhighervalueCSFIgGINDEXCSFIgOligoclonalBandsBaker'sClinicalNeurology,CDROM-2003Lhermitte’ssignOligoclonalBandsBaker'sCliniVisualEvokedPotentials

(Baker’sClinNeurol2003)VisualEvokedPotentials

(BakeMRI:

FLAIR&T1withGadolinium

(NoseworthyJ,etalNEJM,2000)MRI:

FLAIR&T1withGadoliniMRI:T1“BlackHoles”MRI:T1“BlackHoles”MRI:SagittalViewsMRI:SagittalViewsMRI:SpinalImagingMRI:SpinalImagingDIAGNOSTICCRITERIA-12attackswithclinicalevidenceofatleast2lesions2attackswithclinicalevidenceof1lesion

+DISIattack+clinicalevidenceof2lesions+DITNotes:1.Anattack:durationofsymptomshould>24hwithoutfeverorinfection.Demonstratedby(1)examination.(2)historicaleventscharacteristicofMSbutwithnoobjectiveneurologicalfindings.2.Atleastoneattackmustbesupportedbyobjectivefindingsbeforediagnosiscanbemade3.“Fourpoints”diagnosisprocess:attack+clinicalevidence3.DIS=disseminationinspace,DIT=disseminationintimeDIAGNOSTICCRITERIA-12attacksDIAGNOSTICCRITERIA-2Clinicallyisolatedsyndromes1attack+1objectiveclinicalevidence+DIS+DITPrimaryprogressive:Oneyearofdiseaseprogression2of3ofthefollowingDISof>=1T2lesionsofMScharateristicDISof>=2T2lesionsinspinalcordCSFologobandofIgGDIAGNOSTICCRITERIA-2ClinicallPolmanGH,etal.ANNNEUROL2011;69:292–302PolmanGH,etal.McDonaldCriteria(2010Revision)McDonaldCriteria(2010RevisiMcDonaldCriteria(2010Revision)DIT=disseminationintime,DIS=disseminationinspaceMcDonaldCriteria(2010RevisiNotesforMcDonaldCriteria(2010revision)NotesforMcDonaldCriteria(2DifferentialdiagnosisLacunarinfarctionAcutedisseminatedencephalomyelitis(ADEM)Cerebralautosomaldominantateriopathy,subcorticalinfacrts,andleukoencepholopathies(CADASIL)Mitochodiralencephalopathywithlacticacidosisandstroke(MELAS)VasculitisVitaminB12deficiency(spinalcordlesions)DifferentialdiagnosisLacunarDifferentialdiagnosisCaution:Age<15or>60Patientsneverexperiencedvisual,sensory,orbladdersymptomsRoutinetestofthefollowingishelpfulESRSerumVitB12Antinuclearantibody(ANA)DifferentialdiagnosisCaution:TreatmentPrinciplesAcuteattacks-corticosteroidsDiseasemodifyingagentsSymptommanagementagentsTreatmentPrinciplesAcuteattAcuteattacks-corticosteroidsMethylprednisolone,1g/dfor3days,reducedosagein4wPrednisone,80mg/dfor1w,reducedosagein4~6wSideeffects:gastriculcer,fluidretention,potassium

溫馨提示

  • 1. 本站所有資源如無(wú)特殊說(shuō)明，都需要本地電腦安裝OFFICE2007和PDF閱讀器。圖紙軟件為CAD,CAXA,PROE,UG,SolidWorks等.壓縮文件請(qǐng)下載最新的WinRAR軟件解壓。
  • 2. 本站的文檔不包含任何第三方提供的附件圖紙等，如果需要附件，請(qǐng)聯(lián)系上傳者。文件的所有權(quán)益歸上傳用戶所有。
  • 3. 本站RAR壓縮包中若帶圖紙，網(wǎng)頁(yè)內(nèi)容里面會(huì)有圖紙預(yù)覽，若沒(méi)有圖紙預(yù)覽就沒(méi)有圖紙。
  • 4. 未經(jīng)權(quán)益所有人同意不得將文件中的內(nèi)容挪作商業(yè)或盈利用途。
  • 5. 人人文庫(kù)網(wǎng)僅提供信息存儲(chǔ)空間，僅對(duì)用戶上傳內(nèi)容的表現(xiàn)方式做保護(hù)處理，對(duì)用戶上傳分享的文檔內(nèi)容本身不做任何修改或編輯，并不能對(duì)任何下載內(nèi)容負(fù)責(zé)。
  • 6. 下載文件中如有侵權(quán)或不適當(dāng)內(nèi)容，請(qǐng)與我們聯(lián)系，我們立即糾正。
  • 7. 本站不保證下載資源的準(zhǔn)確性、安全性和完整性, 同時(shí)也不承擔(dān)用戶因使用這些下載資源對(duì)自己和他人造成任何形式的傷害或損失。

最新文檔

評(píng)論

0/150

提交評(píng)論