慢性炎症性脱髓鞘性多发性神经根神经病

ChronicInflammatoryDemyelinatin

Polyradiculoneuropathy:

updateonclinicalfeatures,

phenotypesandtreatmentoptionsDepartmentofNeurology,FujianProvincialHospitalXingyongChen第一页，共二十一页。整理课件IntroductionTraditionally,chronicinflammatorydemyelinatingpolyradiculoneuropathy(CIDP)hasbeenconsideredaheterogeneousdisorderincludingabroadspectrumofclinicalphenotypes.TheEuropeanFederationofNeurologicalSocieties/PeripheralNerveSociety(EFNS/PNS)CIDPtreatmentguidelinehasdefinedseveralclinicalpicturesasatypicalCIDPphenotypes,additionaltotheclassical(typical)pictureofCIDP第二页，共二十一页。整理课件prevalenceofaround6casesper100,000predominantlyaffectmales,andtypicallyoccurinmiddletooldageCIDPcanalsooccur

inchildren.TheincidenceofCIDPincreaseswithage,risingto1.5timestheoverallaverageinpeopleover65

yearsofage第三页，共二十一页。整理课件ClinicalpresentationClassicCIDPischaracterizedbytheoccurrenceofsymmetricalweaknessinbothproximalanddistalmuscles,impairedsensationandparasthesiaandabsentordiminishedtendonreflexesThediseaseevolvesovermorethan8weeks,thusdistinguishingtheconditionfromGBSwhichhasanacuteonset.Thetimecoursemayberelapsing,chronicprogressive,monophasicorGBSlikeonset.第四页，共二十一页。整理课件NewlyrecognisedclinicalfeaturesinCIDPAcuteonsetofCIDPmayoccurinupto18%ofCIDPpatients,resemblingtheGBSdiagnosiswaschangedtoCIDPin5%ofGBSpatientsFatiguecanbethemaincomplaintinCIDPpatients(75%)Activity-inducedweaknessSeverepainispresentinonlyaminorityofCIDPpatients;TremorcanbeadisablingsymptominCIDP(50%)ahigherincidenceofalmost40%ofrestlesslegssyndromeinpatientswithCIDPAutonomicsymptoms:23%,ofwhichgastrointestinalandgenitourinarysymptomsweremostfrequent(mild)severeautonomicdysfunctionshouldberegardedasaredflagwhenconsideringthediagnosisofCIDP.第五页，共二十一页。整理课件CIDPPHENOTYPESTheclinicalpresentationofCIDPisvariabledeterminedbythenumberanddistributionofthedemyelinatingperipheralnervelesions第六页，共二十一页。整理课件SM:sensoryandmotor.PE:plasmaexchange;RR:relapsing–remitting;CS:corticosteroids;第七页，共二十一页。整理课件AtypicalCIDP第八页，共二十一页。整理课件Distalparesthesiaandhypesthesiaarethemostfrequentsymptoms,followedbyproprioceptiveataxia第九页，共二十一页。整理课件DADSP(distalacquireddemyelinatingsymmetricpolyneuropathy):distalorpredominantlysensorylargefibreneuropathylengthdependentaxonalneuropathy.DML:distalmotorlatency第十页，共二十一页。整理课件TheLewis–Sumnersyndrome(LSS),ormultifocalacquireddemyelinatingsensoryandmotorneuropathy(MADSAM),hasbeenreportedinupto15%ofpatientsfulfillingtheEFNS/PNScriteriaforCIDP第十一页，共二十一页。整理课件Thecombinationofsymmetricpatternofweaknesswithoutbulbarinvolvementclinicallydistinguishesthisphenotypefromlowermotorneurondisease,butdistinctioncanbedifficult,especiallyifelectrophysiologicalcriteriaofdemyelinationarenotmetcompletely.第十二页，共二十一页。整理课件FocalCIDPisdefinedasinvolvementofthebrachialorlumbosacralplexusorofoneormoreperipheralnervesinoneupperorlowerlimb(EFNS).第十三页，共二十一页。整理课件AssociatedconditionsCIDPmaybeassociatedwithvariousdiseases,suchasinfectionwiththehumanimmunodeficiencyvirusorhepatitisC,Sjögren'ssyndrome,inflammatoryboweldisease,melanoma,lymphoma,diabetesmellitus,andIgM,IgG,orIgAmonoclonalgammopathyofunknownsignificance.Thepathogeneticrelevanceofsuchconcurrentdiseasesisunclear第十四页，共二十一页。整理课件updateAnti-neurofascin(神经(shénjīng)束蛋白)IgG4antibodieswereassociatedwithasubgroupofpatientswithCIDPshowingayoungerageatonset,ataxia,tremor,CNSdemyelination,andapoorresponsetoIVimmunoglobulin第十五页，共二十一页。整理课件Diffusion-weightedimagesinpatient10showedsignalabnormalitiesinthespleniumofthecorpuscallosum(胼胝(piánzhī)体压部).Fluid-attenuatedinversionrecoveryimagesinpatients10and31showedmultiplesclerosis–likelesionsinthejuxtaventricular（脑室旁）

regions.第十六页，共二十一页。整理课件TherapeuticoptionsforCIDP第十七页，共二十一页。整理课件Theacquiredchronicdemyelinatingneuropathiesinclude：chronicinflammatorydemyelinatingpolyneuropathy(CIDP)neuropathyassociatedwithmonoclonalIgMantibodiestomyelin-associatedglycoprotein(MAG;anti-MAGneuropathy)multifocalmotorneuropathy(MMN)

POEMSsyndrome.Theyhavecharacteristic-thoughoverlapping-clinicalpresentations,aremediatedbydistinctimmunemechanisms,andrespondtodifferenttherapies.第十八页，共二十一页。整理课件Figure1|Asuggesteddiagnosticpathwayforchronicacquireddemyelinatingpolyneuropathies.Differentialdiagnosisisbasedonelectrodiagnosticstudies,biopsies,serumbiomarkersandantibodies.Abbreviations:CIDP,chronicinflammatorydemyelinatingpolyneuropathy;IFE,immunofixationelectrophoresis;MAG,myelin-associatedglycoprotein;VEGF,vascular