慢性炎症性脱髓鞘性多发性神经根神经病进展-豆柴文库

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ChronicInflammatoryDemyelinatinPolyradiculoneuropathy:updateonclinicalfeatures,phenotypesandtreatmentoptionsIntroductionprevalenceofaround6casesper100,000
predominantlyaffectmales,andtypicallyoccurinmiddletooldage
CIDPcanalsooccurinchildren.
TheincidenceofCIDPincreaseswithage,risingto1.5timestheoverallaverageinpeopleover65yearsofage

ClinicalpresentationNewlyrecognisedclinicalfeaturesinCIDPCIDPPHENOTYPESSM:sensoryandmotor.PE:plasmaexchange;RR:relapsing–remitting;CS:corticosteroids;


AtypicalCIDPDistalparesthesiaandhypesthesiaarethemostfrequentsymptoms,followedbyproprioceptiveataxia
DADSP(distalacquireddemyelinatingsymmetricpolyneuropathy):distalorpredominantlysensorylargefibreneuropathy
lengthdependentaxonalneuropathy.

TheLewis–Sumnersyndrome(LSS),ormultifocalacquireddemyelinatingsensoryandmotorneuropathy(MADSAM),hasbeenreportedinupto15%ofpatientsfulfillingtheEFNS/PNScriteriaforCIDP
Thecombinationofsymmetricpatternofweaknesswithoutbulbarinvolvementclinicallydistinguishesthisphenotypefromlowermotorneurondisease,butdistinctioncanbedifficult,especiallyifelectrophysiologicalcriteriaofdemyelinationarenotmetcompletely.
FocalCIDPisdefinedasinvolvementofthebrachial
orlumbosacralplexusorofoneormoreperipheral
nervesinoneupperorlowerlimb(EFNS).
AssociatedconditionsupdateDiffusion-weightedimagesinpatient10showedsignalabnormalitiesinthespleniumofthecorpuscallosum(胼胝体压部).
Fluid-attenuatedinversionrecoveryimagesinpatients10and31showedmultiplesclerosis–likelesionsinthejuxtaventricular（脑室旁）regions.
TherapeuticoptionsforCIDPTheacquiredchronicdemyelinatingneuropathiesinclude：Figure1|Asuggesteddiagnosticpathwayforchronicacquireddemyelinatingpolyneuropathies.Differentialdiagnosisisbasedonelectrodiagnosticstudies,biopsies,serumbiomarkersandantibodies.Abbreviations:CIDP,chronicinflammatorydemyelinatingpolyneuropathy;IFE,immunofixationelectrophoresis;MAG,myelin-associatedglycoprotein;VEGF,vascularendothelialgrowthfactor.
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