神经病学英文课件：04 Guillain Barre Syndrome

Guillain-BarreSyndrome吉兰-巴雷综合征Guillain-BarreSyndrome吉兰-巴雷综合DefinitionGuillain-Barresyndrome(GBS)isaspectrumofimmune-mediatedneuropathies,characterizedbyacuteonset,rapidlyevolving,monophasicpolyradiculoneuropathy,consistingofdemyelinatingandacuteaxonaldegeneratingforms.GBSisacommoncauseofacuteflaccidparalysisworldwide,withanaverageincidenceofabout0.6-1.9per100,000.DefinitionGuillain-BarresyndrPathophysiologyGBSisapostinfectious,immune-mediateddisease.MostpatientsreportaninfectiousillnessintheweekspriortotheonsetofGBS.MolecularmimicryManyoftheidentifiedinfectiousagentsarethoughttoinduceproductionofantibodiesthatcross-reactwithspecificgangliosidesandglycolipids,suchasGM1andGD1b,thataredistributedthroughoutthemyelinintheperipheralnervoussystem,resultinginimmunologicdamagetotheperipheralnervoussystem.PathophysiologyGBSisapostin神经病学英文课件：04GuillainBarreSyndromePathologicfindingsinGBSlymphocyticinfiltrationofspinalrootsandperipheralnerves(cranialnervesmaybeinvolvedaswell),followedbymacrophage-mediated,multifocalstrippingofmyelin,resultsindefectsinthepropagationofelectricalnerveimpulses,witheventualabsenceorprofounddelayinconduction,causingflaccidparalysis.PathologicfindingsinGBSSubtypesofGuillain-BarrésyndromeClassicGBSPharyngeal–cervical–brachialweakness(3%,5%,1%)ParapareticGBS(2%,6%)Bifacialweaknesswithparaesthesias(1%,5%,)MillerFishersyndrome(MFS)(5%,7%)Bickerstaffbrainstemencephalitis(BBE)(7%)SubtypesofGuillain-BarrésynClinicalManifestationClassicGBSWeaknessandareflexia/hyporeflexiainallfourlimbs,symmetricorasymmetric.WeaknessusuallystartsinthelegsandascendsbutmaystartinthearmsWeaknessmaybemild,moderateorcompleteparalysisCranial-nerve-innervatedmusclesorrespiratorymusclesmaybeinvolvedMusclestretchreflexesmaybenormalorexaggeratedin10%ofcasesElectrophysiologicalevidenceofneuropathy.ClinicalManifestationClassicPharyngeal–cervical–brachialweaknessOropharyngeal,neckandarmweaknessandarmareflexia/hyporeflexiaAbsenceoflegweaknessParapareticGBSLegweaknessandlegareflexia/hyporeflexiaAbsenceofarmweaknessBifacialweaknesswithparaesthesiasFacialweaknessandlimbareflexia/hyporeflexiaAbsenceofophthalmoplegia,ataxiaandlimbweaknessPharyngeal–cervical–brachialwMillerFishersyndrome(MFS)Ophthalmoplegia,ataxiaandareflexia/hyporeflexiaAbsenceoflimbweaknessandhypersomnolencePresenceofanti-GQ1bIgGantibodies(83%)MillerFishersyndrome(MFS)Bickerstaffbrainstemencephalitis(BBE)HypersomnolenceandophthalmoplegiaandataxiaAbsenceoflimbweaknessPresenceofanti-GQ1bIgGantibodies(68%)BickerstaffbrainstemencephalCorefeaturesforGBSspectrumdisordersMostlysymmetricpatternoflimband/ormotorcranial-nerveweaknessMonophasicdiseasecoursewithintervalbetweenonsetandnadirofweaknessof12hto28days,followedbyclinicalplateauAbout73%ofpatientsreachanadirofclinicalfunctionatoneweek,80%within2weeksand98%atfourweeks.Approximately25-30%ofpatientswillrequireventilatoryassistanceatsometimeduringtheillness,withamortalityrateashighas20%occurringprimarilyinpatientswhorequiremechanicalventilation.CorefeaturesforGBSspectrumOtherfeaturesofGBSPresenceofdistalparaesthesiaatorbeforetheonsetofweakness.Two-thirdsofcasesofGBSareassociatedwithanantecedentinfection.GBScanoccuratanyage,butthereappearstobeabimodaldistribution,withpeaksinyoungadulthood(15-35ys)andintheelderly(50-75ys).OtherfeaturesofGBSPresenceAxonalvariantofGBSAcutemotoraxonneuropathy(AMAN)AsummerepidemicsinNorthernChinaoftheaxonalvariantwithCampylobacterJejuniinfectionwerereported,thisvariantismuchmorecommoninNorthernChina,JapanandtherestofAmerica.FeaturesofAMANacuteonset,symptomsdevelopin24-48hrs,flaccidlimbsweakness,rarelyinvolvedwithsensation,severeanddisable.20-30%GM1andGD1bantibodypositiveElectrophysioligydemostratesaxonaldegnerationwithdecreasedCMAPAxonalvariantofGBSAcutemotLaboratoryfeaturesAnalysisoftheCSFAlbuminocytologicdissociation----normalnumberofcellswithanelevatedproteinconcentration(greaterthan45mg/dL,with<3cells/mm3).Thisfindingmaybedelayedtotheendofthefirstweek,50%ofthepatientspuncturedinthefirstweekand80%puncturedinthesecondweek(priortotreatment)hadincreasedtotalprotein.Maximumproteinvaluesmaybeseeninthirdorfourthweek.LaboratoryfeaturesAnalysisofElectrophysiologicstudiesBeessentialtoconfirmthediagnosisandexcludeitsmimics.Theearliestfinding

isprolongationorabsenceoftheF-waveandabsentHreflex,whichindicatesdemyelinationoftheproximalnerveroots.Mostpatientsdemonstratetypicaldemyelinationfeatures2-3weeksaftertheonsetofsymptoms,whichincludeslowedmotornerveconductionvelocities,prolongeddistallatenciesandF-wavelatency,andabnormaltemporaldispersion.ElectrophysiologicstudiesMedianmotorstimulationMedianmotorstimulationLeft:Twocompoundmuscleactionpotentialswererecordedfromstimulationatthewristandthenattheelbow.Thelatenciesoftheresponseswererespectively3.6msand8ms.Thedistancebetweenelbowandwristwasmeasuredat24cm.Theconductionvelocityistherefore:24cm/4.4ms=54.5m/s.Right:Patientwithademyelinatingneuropathy.Notehowthefirstresponse(wriststimulation)hasalonglatency(7.6ms)andhowthesecondresponse(elbowstimulation)hasasmalleramplitude,indicatingpartialconductionblock.Left:TwocompoundmuscleactiSerumantibodiestospecificgangliosidesandglycolipidsinmyelinofperipheralnerveGM1AMANGD1bMFSandBBESerumantibodiestospecificgDiagnosisDiagnosisofGBScanbemadeclinicallyinthemajorityofpatients,thefeaturesstronglysupportdiagnosisAcuteweakness,whichmayaffectthelimbsand/ortheterritoriesservedbymotorcranialnerves,tendstobesymmetric.Theclinicalcourseshouldbemonophasic.

AntecedentinfectioussymptomsDistallimbnumbness,paraesthesiaorpain,anyofwhichbecomes

initialsymptom.AlbuminocytologicdissociationTypicalelectrodiagnosticfeaturesDiagnosisDiagnosisofGBScanAlternativediagnosisshouldbeexcluded.However,othercausesforrapidlydevelopingflaccidweaknessshouldbehighlyunlikelybaseduponhistoryandifnecessaryadditionaltests.AlternativediagnosisshouldbDifferentialDiagnosisSpinalcordlesions(poliomyelitis,transversemyelitis)Neuromuscularjunctiondisorders(botulism,myastheniagravis)PeripheralNeuropathies(toxic,infections,criticalillnesspolyneuropathy)Myopathies(periodicparalysis,dermatomyositis,criticalillnessmyopathy)FunctionalparalysisDifferentialDiagnosisSpinalcTreatmentTreatmentofGBShastwocomponents:supportivecareandspecifictherapy.Generalsupportivecareremainsthecornerstoneoftherapy.AllGBS

patientsshouldbeadmittedtoahospitalforcloseobservationforrespiratorycompromise,cranialnervedysfunction,andautonomicinstability.Asrespiratorymusclesweaken,electiveendotrachealintubationandmechanicalventilationshouldbeconsidered.TreatmentTreatmentofGBShasPatientswithseveredysphagiamayrequirenasogastricor

feedingtubes.Painandpsychologicstressshouldbetreated.Physiotherapy(passivelimbmovementsandrehabilitationprogram),preventingdeepveinthrombosis.

PatientswithseveredysphagiaSpecifictreatmentshouldbeinitiatedsoonafterdiagnosis.High-doseintravenousimmunoglobulin(IVIgadministeredat0.4g/kg/dayfor5days)Plasmapheresis(40mg/kg,4-6plasmexchangesover5-8days)Specifictreatmenthasbeenshowntohastenrecovery.SpecifictreatmentshouldbeiPrognosisandRecoveryApproximately80%patientswithGBSachieveafullandfunctionalrecoverywithin6-12months.Approximately7-15%patientshavepermanentneurologicsequelaeincludingbilateralfootdrop,intrinsichandmusclewasting,andsensoryataxia.Themortalityrateis<5%intertiarycarecenterswithateamofmedicalprofessionalswhoarefamiliarwithGBSmanagement.PrognosisandRecoveryApproximThanks!Thanks!Guillain-BarreSyndrome吉兰-巴雷综合征Guillain-BarreSyndrome吉兰-巴雷综合DefinitionGuillain-Barresyndrome(GBS)isaspectrumofimmune-mediatedneuropathies,characterizedbyacuteonset,rapidlyevolving,monophasicpolyradiculoneuropathy,consistingofdemyelinatingandacuteaxonaldegeneratingforms.GBSisacommoncauseofacuteflaccidparalysisworldwide,withanaverageincidenceofabout0.6-1.9per100,000.DefinitionGuillain-BarresyndrPathophysiologyGBSisapostinfectious,immune-mediateddisease.MostpatientsreportaninfectiousillnessintheweekspriortotheonsetofGBS.MolecularmimicryManyoftheidentifiedinfectiousagentsarethoughttoinduceproductionofantibodiesthatcross-reactwithspecificgangliosidesandglycolipids,suchasGM1andGD1b,thataredistributedthroughoutthemyelinintheperipheralnervoussystem,resultinginimmunologicdamagetotheperipheralnervoussystem.PathophysiologyGBSisapostin神经病学英文课件：04GuillainBarreSyndromePathologicfindingsinGBSlymphocyticinfiltrationofspinalrootsandperipheralnerves(cranialnervesmaybeinvolvedaswell),followedbymacrophage-mediated,multifocalstrippingofmyelin,resultsindefectsinthepropagationofelectricalnerveimpulses,witheventualabsenceorprofounddelayinconduction,causingflaccidparalysis.PathologicfindingsinGBSSubtypesofGuillain-BarrésyndromeClassicGBSPharyngeal–cervical–brachialweakness(3%,5%,1%)ParapareticGBS(2%,6%)Bifacialweaknesswithparaesthesias(1%,5%,)MillerFishersyndrome(MFS)(5%,7%)Bickerstaffbrainstemencephalitis(BBE)(7%)SubtypesofGuillain-BarrésynClinicalManifestationClassicGBSWeaknessandareflexia/hyporeflexiainallfourlimbs,symmetricorasymmetric.WeaknessusuallystartsinthelegsandascendsbutmaystartinthearmsWeaknessmaybemild,moderateorcompleteparalysisCranial-nerve-innervatedmusclesorrespiratorymusclesmaybeinvolvedMusclestretchreflexesmaybenormalorexaggeratedin10%ofcasesElectrophysiologicalevidenceofneuropathy.ClinicalManifestationClassicPharyngeal–cervical–brachialweaknessOropharyngeal,neckandarmweaknessandarmareflexia/hyporeflexiaAbsenceoflegweaknessParapareticGBSLegweaknessandlegareflexia/hyporeflexiaAbsenceofarmweaknessBifacialweaknesswithparaesthesiasFacialweaknessandlimbareflexia/hyporeflexiaAbsenceofophthalmoplegia,ataxiaandlimbweaknessPharyngeal–cervical–brachialwMillerFishersyndrome(MFS)Ophthalmoplegia,ataxiaandareflexia/hyporeflexiaAbsenceoflimbweaknessandhypersomnolencePresenceofanti-GQ1bIgGantibodies(83%)MillerFishersyndrome(MFS)Bickerstaffbrainstemencephalitis(BBE)HypersomnolenceandophthalmoplegiaandataxiaAbsenceoflimbweaknessPresenceofanti-GQ1bIgGantibodies(68%)BickerstaffbrainstemencephalCorefeaturesforGBSspectrumdisordersMostlysymmetricpatternoflimband/ormotorcranial-nerveweaknessMonophasicdiseasecoursewithintervalbetweenonsetandnadirofweaknessof12hto28days,followedbyclinicalplateauAbout73%ofpatientsreachanadirofclinicalfunctionatoneweek,80%within2weeksand98%atfourweeks.Approximately25-30%ofpatientswillrequireventilatoryassistanceatsometimeduringtheillness,withamortalityrateashighas20%occurringprimarilyinpatientswhorequiremechanicalventilation.CorefeaturesforGBSspectrumOtherfeaturesofGBSPresenceofdistalparaesthesiaatorbeforetheonsetofweakness.Two-thirdsofcasesofGBSareassociatedwithanantecedentinfection.GBScanoccuratanyage,butthereappearstobeabimodaldistribution,withpeaksinyoungadulthood(15-35ys)andintheelderly(50-75ys).OtherfeaturesofGBSPresenceAxonalvariantofGBSAcutemotoraxonneuropathy(AMAN)AsummerepidemicsinNorthernChinaoftheaxonalvariantwithCampylobacterJejuniinfectionwerereported,thisvariantismuchmorecommoninNorthernChina,JapanandtherestofAmerica.FeaturesofAMANacuteonset,symptomsdevelopin24-48hrs,flaccidlimbsweakness,rarelyinvolvedwithsensation,severeanddisable.20-30%GM1andGD1bantibodypositiveElectrophysioligydemostratesaxonaldegnerationwithdecreasedCMAPAxonalvariantofGBSAcutemotLaboratoryfeaturesAnalysisoftheCSFAlbuminocytologicdissociation----normalnumberofcellswithanelevatedproteinconcentration(greaterthan45mg/dL,with<3cells/mm3).Thisfindingmaybedelayedtotheendofthefirstweek,50%ofthepatientspuncturedinthefirstweekand80%puncturedinthesecondweek(priortotreatment)hadincreasedtotalprotein.Maximumproteinvaluesmaybeseeninthirdorfourthweek.LaboratoryfeaturesAnalysisofElectrophysiologicstudiesBeessentialtoconfirmthediagnosisandexcludeitsmimics.Theearliestfinding

isprolongationorabsenceoftheF-waveandabsentHreflex,whichindicatesdemyelinationoftheproximalnerveroots.Mostpatientsdemonstratetypicaldemyelinationfeatures2-3weeksaftertheonsetofsymptoms,whichincludeslowedmotornerveconductionvelocities,prolongeddistallatenciesandF-wavelatency,andabnormaltemporaldispersion.ElectrophysiologicstudiesMedianmotorstimulationMedianmotorstimulationLeft:Twocompoundmuscleactionpotentialswererecordedfromstimulationatthewristandthenattheelbow.Thelatenciesoftheresponseswererespectively3.6msand8ms.Thedistancebetweenelbowandwristwasmeasuredat24cm.Theconductionvelocityistherefore:24cm/4.4ms=54.5m/s.Right:Patientwithademyelinatingneuropathy.Notehowthefirstresponse(wriststimulation)hasalonglatency(7.6ms)andhowthesecondresponse(elbowstimulation)hasasmalleramplitude,indicatingpartialconductionblock.Left:TwocompoundmuscleactiSerumantibodiestospecificgangliosidesandglycolipidsinmyelinofperipheralnerveGM1AMANGD1bMFSandBBESerumantibodiestospecificgDiagnosisDiagnosisofGBScanbemadeclinicallyinthemajorityofpatients,thefeaturesstronglysupportdiagnosisAcuteweakness,whichmayaffectthelimbsand/ortheterritoriesservedbymotorcranialnerves,tendstobesymmetric.Theclinicalcourseshouldbemonophasic.

AntecedentinfectioussymptomsDistallimbnumbness,paraesthesiaorpain,anyofwhichbecomes

initialsymptom.AlbuminocytologicdissociationTypicalelectrodiagnosticfeaturesDiagnosisDiagnosisofGBScanAlternativediagnosisshouldbeexcluded.However,othercausesforrapidlydevelopingflaccidweaknessshouldbehighlyunlikelybaseduponhistoryandifnecessaryadditionaltests.AlternativediagnosisshouldbDifferentialDiagnosisSpinalcordlesions(poliomyelitis,tra