韦尼克脑病16例临床分析_王红星

1003-2754201107-0616-0416[e-mail]’sencephalopathyWE、、。2002～200916WE。WE。、、“”WE“”。MRI、、、T1、T2。Vit-B1。WEMRIWE。Vit-B1。R742AClinicalanalysisofWernicke’sencephalopathyreportof16casesWANGHong-xingZHANGLeYANGXiao-suetal．DepartmentofNeurologyXiangyaHospitalCentralSouthUniversityChangsha410008ChinaAbstractObjectiveToexploretheetiologypathogenesisclinicalmanifestationsandtreatmentofWernicke’sen-cephalopathyWE．MethodsAllclinicaldateof16WEpatientswerereviewedretrospectively．ResultsAlcoholabusewasthemajorcauseofWE．Ocularsignscerebellardysfunctionandconfusionwerethemostcommonandfrequentsymp-tomsofWE．Howevertheclassicaldiagnostictriadwasonlyfoundin6．2%ofpatients．Lacunarinfarctionandbrainatro-phywerethemostcommonfindingsonbrainscan．Thetypicallesionssymmetricallyseeninthethalamushypothalamusmammillarybodiesandperiaqueductalareawereonlyfoundin212．5%patients．WithabundantVit-B1treatmentthegreatmajoritypatientshadafavorablerecovery．ConclusionTheetiologyandclinicalfeaturesofWEvarygreatly．Earlydiagnosisisdifficult．BrainMRIscanshouldbeusedtosupportthediagnosisofWEinalcoholicsandnonalcoholics．EarlysupplementofabundantVit-B1isofprimeimportanceforcompleterecovery．KeywordsWernicke’sencephalopathyEtiologyPathogenesisClinicalmanifestationsTreatmentWernicke’sencephalopathyWEVit-B1。WE、“”。。WEWE。WE2%～3%0．06%～0．13%1WE。WE。。2002～2009WE、、。11．120021～20096WE。18Euro-peanFederationofNeurologicalSocietiesEFNSWE242WE1234。16。·616·JApoplexyandNervousDiseasesJuly2011Vol28No．71．216WE、、、、。22．11627～7552．3±14．0115115∶1。743．8%956．2%。1275．0%。8～5530．9±12．850～1000ml50%584．4±482．3ml212．5%112．5%。2．21616．2%WE“”、。WE“”212．5%。WE743．8%2、425．0%531．3%、、、。2．375．0%。85439B6、、、。1。EEG54。431IgG。13CTMRI310、212．5%、T1、T2。2．4Vit-B1100～200mg/d。2～3w531．3%1062．5%16．2%。1WE∶d1221112∶02∶00∶11∶053．1±12．753．5±19．12765894．6±1303．121．5±4．921102WE7432153211143．8%25．0%18．8%12．5%6．2%31．3%18．8%12．5%6．2%6．2%6．2%3Wernicke’sencephalopathyWEVit-B1Wernicke1881、。WE1．7∶1WE15∶1。WE。3．1WEWE。·716·20117287WE、、、、、、、、、1。、。WE。。WE、34。ATP、。、。pH。、56。7。WE。、、、8。3．2WE、、、。WE“”16%19%“”929%、。、1023%82%9。。31．3%。WE。WEWEWEWE“”WE11～14。。WE。。15。。WEEEG。MRIWE、。53%93%16。MRI、、、、T1、T2。WE17WE18。MRI。DWI、ADC。DWI、ADCMRI212．5%WEMRI。·816·JApoplexyandNervousDiseasesJuly2011Vol28No．73．3WEVit-B1WEVit-B1。2～319、EFNSVit-B1200mg/d+0．9%100ml5%100ml3＞30min2。WEVit-B1WE20。WEVit-B1500mgTid21。WE。WE“”、WE。WE。WE。1SechiGSerraA．Wernicke’sencephalopathynewclinicalsettingsandrecentadvancesindiagnosisandmanagementJ．LancetNeu-rol200765442－455．2GalvinRBrathenGIvashynkaAetal．EFNSguidelinesfordiagno-sistherapyandpreventionofWernickeencephalopathyJ．EurJNeurol201017121408－1418．3SingletonCKMartinPR．Molecularmechanismsofthiamineutiliza-tionJ．CurrMolMed200112197－207．4GuerriniIThomsonADCookCCetal．DirectgenomicPCRse-quencingofthehighaffinitythiaminetransporterSLC19A2genei-dentifiesthreegeneticvariantsinWernickeKorsakoffsyndromeWKSJ．AmJMedGenetBNeuropsychiatrGenet2005137117－19．5DesjardinsPButterworthRF．RoleofmitochondrialdysfunctionandoxidativestressinthepathogenesisofselectiveneuronallossinWer-nicke’sencephalopathyJ．MolNeurobiol200531117－25．6HazellASButterworthRF．Updateofcelldamagemechanismsinthiaminedeficiencyfocusonoxidativestressexcitotoxicityandin-flammationJ．AlcoholAlcohol2009442141－147．7ChanHButterworthRFHazellAS．Primaryculturesofratastro-cytesrespondtothiaminedeficiency-inducedswellingbydownregu-latingaquaporin-4levelsJ．NeurosciLett20043663231－234．8．3J．20037131936－1937．9HarperCGGilesMFinlay-JonesR．ClinicalsignsintheWernicke-Korsakoffcomplexaretrospectiveanalysisof131casesdiagnosedatnecropsyJ．JNeurolNeurosurgPsychiatry1986494341－345．10CookeCAHicksEPageABetal．AnatypicalpresentationofWernicke’sencephalopathyinan11-year-oldchildJ．EyeLond200620121418－1420．11ZuccoliGSantaCruzDBertoliniMetal．MRimagingfindingsin56patientswithWernickeencephalopathynonalcoholicsmaydifferfromalcoholicsJ．AmJNeuroradiol2009301171－176．12FeiGQZhongCJinLetal．ClinicalcharacteristicsandMRima-gingfeaturesofnonalcoholicWernickeencephalopathyJ．AmJNeuroradiol2008291164－169．13KirbasDSutlasNKuscuDYetal．Theimpactofprolongedhun-gerstrikeclinicalandlaboratoryaspectsoftwenty-fivehungerstrik-ersJ．IdeggyogySZ2008619317－324．14PesentiFBrocadelloFManaraRetal．Wernicke’ssyndromeduringparenteralfeedingnotanunusualcomplicationJ．Nutri-tion2009252142－146．15KonoSMiyajimaHYoshidaKetal．Mutationsinathiamine-transportergeneandWernicke’s-likeencephalopathyJ．NEnglJMed2009360171792－1794．16ChungSPKimSWYooISetal．MagneticresonanceimagingasadiagnosticadjuncttoWernickeencephalopathyintheEDJ．AmJEmergMed2003216497－502．17ZuccoliGPipitoneN．NeuroimagingfindingsinacuteWernicke’sencephalopathyreviewoftheliteratureJ．AmJRoentgenol20091922501－508．18ZuccoliGCruzDSBertoliniMetal．MRImagingFindingsin56PatientswithWernickeEncephalopathyNonalcoholicsMayDifferfromAlcoholicsJ．AmJNeuroradiol2009301171－176．19DonninoMWVegaJMillerJetal．MythsandmisconceptionsofWernicke’sencephalopathywhateveryemergencyphysicianshouldknowJ．AnnEmergMed2007506715－721．20ThomsonADMarshallEJ．Thenatura