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ChronicInflammatoryDemyelinatinPolyradiculoneuropathy:updateonclinicalfeatures,phenotypesandtreatmentoptionsDepartmentofNeurology,FujianProvincialHospitalXingyongChenIntroductionTraditionally,chronicinflammatorydemyelinatingpolyradiculoneuropathy(CIDP)hasbeenconsideredaheterogeneousdisorderincludingabroadspectrumofclinicalphenotypes.TheEuropeanFederationofNeurologicalSocieties/PeripheralNerveSociety(EFNS/PNS)CIDPtreatmentguidelinehasdefinedseveralclinicalpicturesasatypicalCIDPphenotypes,additionaltotheclassical(typical)pictureofCIDPprevalenceofaround6casesper100,000predominantlyaffectmales,andtypicallyoccurinmiddletooldageCIDPcanalsooccurinchildren.TheincidenceofCIDPincreaseswithage,risingto1.5timestheoverallaverageinpeopleover65yearsofageClinicalpresentationClassicCIDPischaracterizedbytheoccurrenceofsymmetricalweaknessinbothproximalanddistalmuscles,impairedsensationandparasthesiaandabsentordiminishedtendonreflexesThediseaseevolvesovermorethan8weeks,thusdistinguishingtheconditionfromGBSwhichhasanacuteonset.Thetimecoursemayberelapsing,chronicprogressive,monophasicorGBSlikeonset.NewlyrecognisedclinicalfeaturesinCIDPAcuteonsetofCIDPmayoccurinupto18%ofCIDPpatients,resemblingtheGBSdiagnosiswaschangedtoCIDPin5%ofGBSpatientsFatiguecanbethemaincomplaintinCIDPpatients(75%)Activity-inducedweaknessSeverepainispresentinonlyaminorityofCIDPpatients;TremorcanbeadisablingsymptominCIDP(50%)ahigherincidenceofalmost40%ofrestlesslegssyndromeinpatientswithCIDPAutonomicsymptoms:23%,ofwhichgastrointestinalandgenitourinarysymptomsweremostfrequent(mild)severeautonomicdysfunctionshouldberegardedasaredflagwhenconsideringthediagnosisofCIDP.CIDPPHENOTYPESTheclinicalpresentationofCIDPisvariabledeterminedbythenumberanddistributionofthedemyelinatingperipheralnervelesionsSM:sensoryandmotor.PE:plasmaexchange;RR:relapsing–remitting;CS:corticosteroids;AtypicalCIDPDistalparesthesiaandhypesthesiaarethemostfrequentsymptoms,followedbyproprioceptiveataxiaDADSP(distalacquireddemyelinatingsymmetricpolyneuropathy):distalorpredominantlysensorylargefibreneuropathylengthdependentaxonalneuropathy.DML:distalmotorlatencyTheLewis–Sumnersyndrome(LSS),ormultifocalacquireddemyelinatingsensoryandmotorneuropathy(MADSAM),hasbeenreportedinupto15%ofpatientsfulfillingtheEFNS/PNScriteriaforCIDPThecombinationofsymmetricpatternofweaknesswithoutbulbarinvolvementclinicallydistinguishesthisphenotypefromlowermotorneurondisease,butdistinctioncanbedifficult,especiallyifelectrophysiologicalcriteriaofdemyelinationarenotmetcompletely.FocalCIDPisdefinedasinvolvementofthebrachialorlumbosacralplexusorofoneormoreperipheralnervesinoneupperorlowerlimb(EFNS).AssociatedconditionsCIDPmaybeassociatedwithvariousdiseases,suchasinfectionwiththehumanimmunodeficiencyvirusorhepatitisC,Sjögren'ssyndrome,inflammatoryboweldisease,melanoma,lymphoma,diabetesmellitus,andIgM,IgG,orIgAmonoclonalgammopathyofunknownsignificance.ThepathogeneticrelevanceofsuchconcurrentdiseasesisunclearupdateAnti-neurofascin(神经束蛋白)IgG4antibodieswereassociatedwithasubgroupofpatientswithCIDPshowingayoungerageatonset,ataxia,tremor,CNSdemyelination,andapoorresponsetoIVimmunoglobulinDiffusion-weightedimagesinpatient10showedsignalabnormalitiesinthespleniumofthecorpuscallosum(胼胝体压部).Fluid-attenuatedinversionrecoveryimagesinpatients10and31showedmultiplesclerosis–likelesionsinthejuxtaventricular（脑室旁）regions.TherapeuticoptionsforCIDPTheacquiredchronicdemyelinatingneuropathiesinclude：chronicinflammatorydemyelinatingpolyneuropathy(CIDP)neuropathyassociatedwithmonoclonalIgMantibodiestomyelin-associatedglycoprotein(MAG;anti-MAGneuropathy)multifocalmotorneuropathy(MMN)POEMSsyndrome.Theyhavecharacteristic-thoughoverlapping-clinicalpresentations,aremediatedbydistinctimmunemechanisms,andrespondtodifferenttherapies.Figure1|Asuggesteddiagnosticpathwayforchronicacquireddemyelinatingpolyneuropathies.Differentialdiagnosisisbasedonelectrodiagnosticstudies,biopsies,serumbiomarkersandantibodies.Abbreviations:CIDP,chronicinflammatorydemyelinatingpolyneuropathy;IFE,immunofixationelectrophoresis;MAG,myelin-associatedglycoprotein;VEGF,vascularendothelialgrowthfactor.THANKS