慢性炎癥性脫髓鞘性多發(fā)性神經(jīng)根神經(jīng)病進(jìn)展課件

ChronicInflammatoryDemyelinatin

Polyradiculoneuropathy:

updateonclinicalfeatures,

phenotypesandtreatmentoptionsDepartmentofNeurology,FujianProvincialHospitalXingyongChenChronicInflammatoryDemyelina1IntroductionTraditionally,chronicinflammatorydemyelinatingpolyradiculoneuropathy(CIDP)hasbeenconsideredaheterogeneousdisorderincludingabroadspectrumofclinicalphenotypes.TheEuropeanFederationofNeurologicalSocieties/PeripheralNerveSociety(EFNS/PNS)CIDPtreatmentguidelinehasdefinedseveralclinicalpicturesasatypicalCIDPphenotypes,additionaltotheclassical(typical)pictureofCIDPIntroductionTraditionally,chr2prevalenceofaround6casesper100,000predominantlyaffectmales,andtypicallyoccurinmiddletooldageCIDPcanalsooccur

inchildren.TheincidenceofCIDPincreaseswithage,risingto1.5timestheoverallaverageinpeopleover65

yearsofageprevalenceofaround6casesp3ClinicalpresentationClassicCIDPischaracterizedbytheoccurrenceofsymmetricalweaknessinbothproximalanddistalmuscles,impairedsensationandparasthesiaandabsentordiminishedtendonreflexesThediseaseevolvesovermorethan8weeks,thusdistinguishingtheconditionfromGBSwhichhasanacuteonset.Thetimecoursemayberelapsing,chronicprogressive,monophasicorGBSlikeonset.ClinicalpresentationClassicC4NewlyrecognisedclinicalfeaturesinCIDPAcuteonsetofCIDPmayoccurinupto18%ofCIDPpatients,resemblingtheGBSdiagnosiswaschangedtoCIDPin5%ofGBSpatientsFatiguecanbethemaincomplaintinCIDPpatients(75%)Activity-inducedweaknessSeverepainispresentinonlyaminorityofCIDPpatients;TremorcanbeadisablingsymptominCIDP(50%)ahigherincidenceofalmost40%ofrestlesslegssyndromeinpatientswithCIDPAutonomicsymptoms:23%,ofwhichgastrointestinalandgenitourinarysymptomsweremostfrequent(mild)severeautonomicdysfunctionshouldberegardedasaredflagwhenconsideringthediagnosisofCIDP.Newlyrecognisedclinicalfeat5CIDPPHENOTYPESTheclinicalpresentationofCIDPisvariabledeterminedbythenumberanddistributionofthedemyelinatingperipheralnervelesionsCIDPPHENOTYPESTheclinicalpr6SM:sensoryandmotor.PE:plasmaexchange;RR:relapsing–remitting;CS:corticosteroids;SM:sensoryandmotor.PE:pla7AtypicalCIDPAtypicalCIDP8Distalparesthesiaandhypesthesiaarethemostfrequentsymptoms,followedbyproprioceptiveataxiaDistalparesthesiaandhypesth9DADSP(distalacquireddemyelinatingsymmetricpolyneuropathy):distalorpredominantlysensorylargefibreneuropathylengthdependentaxonalneuropathy.DML:distalmotorlatencyDADSP(distalacquireddemyelin10TheLewis–Sumnersyndrome(LSS),ormultifocalacquireddemyelinatingsensoryandmotorneuropathy(MADSAM),hasbeenreportedinupto15%ofpatientsfulfillingtheEFNS/PNScriteriaforCIDPTheLewis–Sumnersyndrome(LSS11Thecombinationofsymmetricpatternofweaknesswithoutbulbarinvolvementclinicallydistinguishesthisphenotypefromlowermotorneurondisease,butdistinctioncanbedifficult,especiallyifelectrophysiologicalcriteriaofdemyelinationarenotmetcompletely.Thecombinationofsymmetricp12FocalCIDPisdefinedasinvolvementofthebrachialorlumbosacralplexusorofoneormoreperipheralnervesinoneupperorlowerlimb(EFNS).FocalCIDPisdefinedasinvol13AssociatedconditionsCIDPmaybeassociatedwithvariousdiseases,suchasinfectionwiththehumanimmunodeficiencyvirusorhepatitisC,Sj?gren'ssyndrome,inflammatoryboweldisease,melanoma,lymphoma,diabetesmellitus,andIgM,IgG,orIgAmonoclonalgammopathyofunknownsignificance.ThepathogeneticrelevanceofsuchconcurrentdiseasesisunclearAssociatedconditionsCIDPmay14updateAnti-neurofascin(神經(jīng)束蛋白)IgG4antibodieswereassociatedwithasubgroupofpatientswithCIDPshowingayoungerageatonset,ataxia,tremor,CNSdemyelination,andapoorresponsetoIVimmunoglobulinupdateAnti-neurofascin(神經(jīng)束蛋白)15Diffusion-weightedimagesinpatient10showedsignalabnormalitiesinthespleniumofthecorpuscallosum(胼胝體壓部).Fluid-attenuatedinversionrecoveryimagesinpatients10and31showedmultiplesclerosis–likelesionsinthejuxtaventricular（腦室旁）

regions.Diffusion-weightedimagesinp16TherapeuticoptionsforCIDPTherapeuticoptionsforCIDP17Theacquiredchronicdemyelinatingneuropathiesinclude：chronicinflammatorydemyelinatingpolyneuropathy(CIDP)neuropathyassociatedwithmonoclonalIgMantibodiestomyelin-associatedglycoprotein(MAG;anti-MAGneuropathy)multifocalmotorneuropathy(MMN)

POEMSsyndrome.Theyhavecharacteristic-thoughoverlapping-clinicalpresentations,aremediatedbydistinctimmunemechanisms,andrespondtodifferenttherapies.Theacquiredchronicdemyelina18Figure1|Asuggesteddiagnosticpathwayforchronicacquireddemyelinatingpolyneuropathies.Differentialdiagnosisisbasedonelectrodiagnosticstudies,biopsies,serumbiomarkersandantibodies.Abbreviations:CIDP,chronicinflammatorydemyelinatingpolyneuropathy;IFE,immunofixationelectrophoresis;MAG,myelin-associatedglycoprotein;VEGF,vascularendothelialgrowthfactor.Figure1|Asuggesteddiagnos19THANKSTHANKS20ChronicInflammatoryDemyelinatin

Polyradiculoneuropathy:

updateonclinicalfeatures,

phenotypesandtreatmentoptionsDepartmentofNeurology,FujianProvincialHospitalXingyongChenChronicInflammatoryDemyelina21IntroductionTraditionally,chronicinflammatorydemyelinatingpolyradiculoneuropathy(CIDP)hasbeenconsideredaheterogeneousdisorderincludingabroadspectrumofclinicalphenotypes.TheEuropeanFederationofNeurologicalSocieties/PeripheralNerveSociety(EFNS/PNS)CIDPtreatmentguidelinehasdefinedseveralclinicalpicturesasatypicalCIDPphenotypes,additionaltotheclassical(typical)pictureofCIDPIntroductionTraditionally,chr22prevalenceofaround6casesper100,000predominantlyaffectmales,andtypicallyoccurinmiddletooldageCIDPcanalsooccur

inchildren.TheincidenceofCIDPincreaseswithage,risingto1.5timestheoverallaverageinpeopleover65

yearsofageprevalenceofaround6casesp23ClinicalpresentationClassicCIDPischaracterizedbytheoccurrenceofsymmetricalweaknessinbothproximalanddistalmuscles,impairedsensationandparasthesiaandabsentordiminishedtendonreflexesThediseaseevolvesovermorethan8weeks,thusdistinguishingtheconditionfromGBSwhichhasanacuteonset.Thetimecoursemayberelapsing,chronicprogressive,monophasicorGBSlikeonset.ClinicalpresentationClassicC24NewlyrecognisedclinicalfeaturesinCIDPAcuteonsetofCIDPmayoccurinupto18%ofCIDPpatients,resemblingtheGBSdiagnosiswaschangedtoCIDPin5%ofGBSpatientsFatiguecanbethemaincomplaintinCIDPpatients(75%)Activity-inducedweaknessSeverepainispresentinonlyaminorityofCIDPpatients;TremorcanbeadisablingsymptominCIDP(50%)ahigherincidenceofalmost40%ofrestlesslegssyndromeinpatientswithCIDPAutonomicsymptoms:23%,ofwhichgastrointestinalandgenitourinarysymptomsweremostfrequent(mild)severeautonomicdysfunctionshouldberegardedasaredflagwhenconsideringthediagnosisofCIDP.Newlyrecognisedclinicalfeat25CIDPPHENOTYPESTheclinicalpresentationofCIDPisvariabledeterminedbythenumberanddistributionofthedemyelinatingperipheralnervelesionsCIDPPHENOTYPESTheclinicalpr26SM:sensoryandmotor.PE:plasmaexchange;RR:relapsing–remitting;CS:corticosteroids;SM:sensoryandmotor.PE:pla27AtypicalCIDPAtypicalCIDP28Distalparesthesiaandhypesthesiaarethemostfrequentsymptoms,followedbyproprioceptiveataxiaDistalparesthesiaandhypesth29DADSP(distalacquireddemyelinatingsymmetricpolyneuropathy):distalorpredominantlysensorylargefibreneuropathylengthdependentaxonalneuropathy.DML:distalmotorlatencyDADSP(distalacquireddemyelin30TheLewis–Sumnersyndrome(LSS),ormultifocalacquireddemyelinatingsensoryandmotorneuropathy(MADSAM),hasbeenreportedinupto15%ofpatientsfulfillingtheEFNS/PNScriteriaforCIDPTheLewis–Sumnersyndrome(LSS31Thecombinationofsymmetricpatternofweaknesswithoutbulbarinvolvementclinicallydistinguishesthisphenotypefromlowermotorneurondisease,butdistinctioncanbedifficult,especiallyifelectrophysiologicalcriteriaofdemyelinationarenotmetcompletely.Thecombinationofsymmetricp32FocalCIDPisdefinedasinvolvementofthebrachialorlumbosacralplexusorofoneormoreperipheralnervesinoneupperorlowerlimb(EFNS).FocalCIDPisdefinedasinvol33AssociatedconditionsCIDPmaybeassociatedwithvariousdiseases,suchasinfectionwiththehumanimmunodeficiencyvirusorhepatitisC,Sj?gren'ssyndrome,inflammatoryboweldisease,melanoma,lymphoma,diabetesmellitus,andIgM,IgG,orIgAmonoclonalgammopathyofunknownsignificance.ThepathogeneticrelevanceofsuchconcurrentdiseasesisunclearAssociatedconditionsCIDPmay34updateAnti-neurofascin(神經(jīng)束蛋白)IgG4antibodieswereassociatedwithasubgroupofpatientswithCIDPshowingayoungerageatonset,ataxia,tremor,CNSdemyelination,andapoorresponsetoIVimmunoglobulinupdateAnti-neurofascin(神經(jīng)束蛋白)35Diffusion-weightedimagesinpatient10showedsignalabnormalitiesinthespleniumofthecorpuscallosum(胼胝體壓部).Fluid-attenuatedinversionrecoveryimagesinpatients10and31s