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1、钾通道抗体相关脑病：一种潜在的对免疫治疗敏感的边缘叶脑炎PotassiumChannelAntibody-AssociatedEncephalopathy:APotentiallyImmunotherapy-ResponsiveFormofLimbicEncephalitisVincentA,BuckleyC,SchottJM 2009/5/2710:50:00 Brain,2004,AbstractIntroduction:Patientspresentingwithsubacuteamnesiaarefrequentlys
2、eeninacuteneurologicalpractice.Amongstthedifferentialdiagnoses,herpessimplexencephalitis,Korsakoff'ssyndromeandlimbicencephalitisshouldbeconsidered.Limbicencephalitisistypicallyaparaneoplasticsyndromewithapoorprognosis;thus,identifyingthosepatientswithpotentiallyrev
3、ersiblesymptomsisimportant.Voltage-gatedpotassiumchannelantibodies(VGKC-Ab)haverecentlybeenreportedinthreecasesofreversiblelimbicencephalitis.Herewereviewtheclinical,immunologicalandneuropsychologicalfeaturesof10patients(ninemale,onefemale;agerange44–79years),eighto
4、fwhomwereidentifiedintwocentresoveraperiodof15months.Thepatientspresentedwith1–52weekhistoriesofmemoryloss,confusionandseizures.Lowplasmasodiumconcentrations,initiallyresistanttotreatment,werepresentineightoutof10.BrainMRIatonsetshowedsignalchangeinthemedialtemporal
5、lobesineightoutof10cases.Paraneoplasticantibodieswerenegative,butVGKC-Abrangedfrom450to5128pM(neurologicalandhealthycontrols<100pM)(Fig1).CSFoligoclonalbandswerefoundinonlyone,butbandsmatchedwiththoseintheserumwerefoundinsixotherpatients.VGKC-AbsintheCSF,testedinfiv
6、eindividuals,variedbetween<1and10%ofserumvalues.Onlyonepatienthadneuromyotonia,whichwasexcludedbyelectromyographyinsevenoftheothers.Formalneuropsychologytestingshowedsevereandglobalimpairmentofmemory,withsparingofgeneralintellectinallbuttwopatients,andofnominalfunct
7、ionsinallbutone.Variableregimesofsteroids,plasmaexchangeandintravenousimmunoglobulinwereassociatedwithvariablefallsinserumVGKC-Abs,tovaluesbetween2and88%oftheinitialvalues,togetherwithmarkedimprovementofneuropsychologicalfunctioninginsixpatients,slightimprovementint
8、hreeandnoneinone.Theimprovementinneuropsychologicalfunctioninginsevenpatientscorrelatedbroadlywiththefallinantibodies.However,varyingdegre
