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1、周围神经病与脊髓病中国协和医科大学ChronicInflammatoryDemyelinatingPolyradiculoneuropathy(CIDP)Prevalence:0.5/100,000inchildren,2/100,000inadult.CIDPandGBS--Similarity:Botharewidespreadpolyradiculoneuropathies,usuallywithcytoalbuminologicdissociationoftheCSF(raisedproteinconcent
2、rationwithfewerornocells);bothexhibitnerveconductionabnormalitiescharacteristicofademyelinatingneuropathy(reducedconductionvelocityandpartialconductionblockinmotornerves),pathologically,bothshowsimilarmultifocalperivenousinflammatoryinfiltratesDifference1.modeo
3、fevolutionandprognosisCIDPbeginsinsidiouslyandevolvesslowly,eitherinasteadilyprogressiveorstepwisemanner,attainingitsmaximumseverityafterseveralmonthsorlongerFromthebeginningitmaybeasymmetricalorinvolvethearmspredominantly2.CIDPmaybedistinctimmunologicallyfromG
4、BS3.DifferenceresponsetotheadministrationofprednisoneClinicalfeaturesWeaknessofthelimbs,particularlyoftheproximallegmuscles,ornumbness,paresthesias,anddysesthesiasofthehandsandfeetweretheinitialsymptomsamixedsensorimotorpolyneuropathywithweaknessoftheshoulder,u
5、pperarm,andthighmuscles,inadditiontomotorandsensorylossinthedistalpartsofthelimbsCranialnerveabnormalitiesweredistinctlyunusualTherewasasystemicconditionsuchasparaproteinemia,lymphoma,anundifferentiatedreactiveadenopathy,orlupusinassociationwithaninflammatoryde
6、myelinatingpolyneuropathyTheclinicalcoursewasmonophasicandslowlyprogressiveinaboutone-third,stepwiseandprogressiveinanotherthird,andrelapsingintheremainingthird.DiagnosisThechronicsymmetricsensorimotorlosscoupledwithEMGfindingsofdemyelinationlargelydefinetheill
7、ness.ThetypicalEMGfindingsareofmultifocalconductionblock,prolongeddistallatencies(distalblock),nerveconductionslowingtolessthan80percentofnormalinseveralnerves,lossoflateresponses,anddispersionofthecompoundmuscleactionpotentials,furtherreflectingdemyelinationin
8、motornerves.LaboratoryFeaturesTheCSFproteiniselevatedin80percentofpatientswithCIDP,typicallyintherangeof75to200mg/dL.Inbiopsymaterial(suralnerve),halfofcasesarefoundtohavein
