慢性炎症性脱髓鞘性多发性神经根神经病最新进展PPT课件.pptx

ChronicInflammatoryDemyelinatinPolyradiculoneuropathy updateonclinicalfeatures phenotypesandtreatmentoptions DepartmentofNeurology FujianProvincialHospitalXingyongChen 1 Introduction Traditionally chronicinflammatorydemyelinatingpolyradiculoneuropathy CIDP hasbeenconsideredaheterogeneousdisorderincludingabroadspectrumofclinicalphenotypes TheEuropeanFederationofNeurologicalSocieties PeripheralNerveSociety EFNS PNS CIDPtreatmentguidelinehasdefinedseveralclinicalpicturesasatypicalCIDPphenotypes additionaltotheclassical typical pictureofCIDP 2 prevalenceofaround6casesper100 000predominantlyaffectmales andtypicallyoccurinmiddletooldageCIDPcanalsooccurinchildren TheincidenceofCIDPincreaseswithage risingto1 5timestheoverallaverageinpeopleover65yearsofage 3 Clinicalpresentation ClassicCIDPischaracterizedbytheoccurrenceofsymmetricalweaknessinbothproximalanddistalmuscles impairedsensationandparasthesiaandabsentordiminishedtendonreflexesThediseaseevolvesovermorethan8weeks thusdistinguishingtheconditionfromGBSwhichhasanacuteonset Thetimecoursemayberelapsing chronicprogressive monophasicorGBSlikeonset 4 NewlyrecognisedclinicalfeaturesinCIDP AcuteonsetofCIDPmayoccurinupto18 ofCIDPpatients resemblingtheGBSdiagnosiswaschangedtoCIDPin5 ofGBSpatientsFatiguecanbethemaincomplaintinCIDPpatients 75 Activity inducedweaknessSeverepainispresentinonlyaminorityofCIDPpatients TremorcanbeadisablingsymptominCIDP 50 ahigherincidenceofalmost40 ofrestlesslegssyndromeinpatientswithCIDPAutonomicsymptoms 23 ofwhichgastrointestinalandgenitourinarysymptomsweremostfrequent mild severeautonomicdysfunctionshouldberegardedasaredflagwhenconsideringthediagnosisofCIDP 5 CIDPPHENOTYPES TheclinicalpresentationofCIDPisvariabledeterminedbythenumberanddistributionofthedemyelinatingperipheralnervelesions 6 SM sensoryandmotor PE plasmaexchange RR relapsing remitting CS corticosteroids 7 AtypicalCIDP 8 Distalparesthesiaandhypesthesiaarethemostfrequentsymptoms followedbyproprioceptiveataxia 9 DADSP distalacquireddemyelinatingsymmetricpolyneuropathy distalorpredominantlysensorylargefibreneuropathylengthdependentaxonalneuropathy DML distalmotorlatency 10 TheLewis Sumnersyndrome LSS ormultifocalacquireddemyelinatingsensoryandmotorneuropathy MADSAM hasbeenreportedinupto15 ofpatientsfulfillingtheEFNS PNScriteriaforCIDP 11 Thecombinationofsymmetricpatternofweaknesswithoutbulbarinvolvementclinicallydistinguishesthisphenotypefromlowermotorneurondisease butdistinctioncanbedifficult especiallyifelectrophysiologicalcriteriaofdemyelinationarenotmetcompletely 12 FocalCIDPisdefinedasinvolvementofthebrachialorlumbosacralplexusorofoneormoreperipheralnervesinoneupperorlowerlimb EFNS 13 Associatedconditions CIDPmaybeassociatedwithvariousdiseases suchasinfectionwiththehumanimmunodeficiencyvirusorhepatitisC Sj gren ssyndrome inflammatoryboweldisease melanoma lymphoma diabetesmellitus andIgM IgG orIgAmonoclonalgammopathyofunknownsignificance Thepathogeneticrelevanceofsuchconcurrentdiseasesisunclear 14 update Anti neurofascin 神经束蛋白 IgG4antibodieswereassociatedwithasubgroupofpatientswithCIDPshowingayoungerageatonset ataxia tremor CNSdemyelination andapoorresponsetoIVimmunoglobulin 15 Diffusion weightedimagesinpatient10showedsignalabnormalitiesinthespleniumofthecorpuscallosum 胼胝体压部 Fluid attenuatedinversionrecoveryimagesinpatients10and31showedmultiplesclerosis likelesionsinthejuxtaventricular 脑室旁 regions 16 TherapeuticoptionsforCIDP 17 Theacquiredchronicdemyelinatingneuropathiesinclude chronicinflammatorydemyelinatingpolyneuropathy CIDP neuropathyassociatedwithmonoclonalIgMantibodiestomyelin associatedglycoprotein MAG anti MAGneuropathy multifocalmotorneuropathy MMN POEMSsyndrome Theyhavecharacteristic thoughoverlapping clinicalpresentations aremediatedbydistinctimmunemechanisms andrespondtodifferenttherapies 18 Figure1 Asuggesteddiagnosticpathwayforchronicacquireddemyelinatingpolyneuropathies Differentialdiagnosisisbasedonelectrodiagnosticstudies biopsies seru