胸腺瘤相關(guān)性副腫瘤邊緣性腦炎課件

胸腺瘤相關(guān)性副腫瘤邊緣性腦炎課件

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1、Thymoma-associatedparaneoplasticencephalitis(TAPE)胸腺瘤相關(guān)的副腫瘤性腦炎Upto50%ofpatientswiththymomahaveparaneoplasticneurologicsyndromes,themostcommonbeingmyastheniagravis.Therearerarecasereportsofthymoma-associatedparaneoplasticlimbicorextralimbicencephalitis

2、thatcanleadtoprogressiveneurologicdeclineanddeathwithouttreatment.胸腺瘤相關(guān)的副腫瘤邊緣性及邊緣外腦炎報(bào)道的比較少,但這種情況可導(dǎo)致進(jìn)行性的神經(jīng)功能障礙甚至死亡。50%胸腺瘤患者會(huì)出現(xiàn)副腫瘤性神經(jīng)系統(tǒng)癥狀,最常見的是重癥肌無力。JThoracCardiovascSurg2011;141:e17-20病例Apreviouslyasymptomatic61-year-oldwomanhadgeneralizedintractablese

3、izures.Brainmagneticresonanceimaging(MRI)showedbilateralfociofcorticalandsubcorticalnonenhancingsignalabnormalitiesonT2-weightedimages(Figure1,A).Chestcomputedtomography(CT)revealedalargeanteriormediastinalmass(Figure1,C)that,onbiopsy,showedmalignantt

4、hymoma.AnalysisofcerebrospinalfluiddetectedapredominantlylymphocyticpleocytosisandpositiveLGI1antibodiesThepatientreceivedanticonvulsants,humanintravenousimmunoglobulinG(IgG)(400g/kgdaily),andcorticosteroids(methylprednisolone1gmdaily)for5daysplusapre

5、dnisonetaperwithresolutionofneurologicsymptoms.Fourweekslater,thepatientunderwentcomplete,enblocresectionofthemediastinalmassthatwasinvadingthroughthepericardium(typeB3,WorldHealthOrganizationclassification,MasaokastageIII).Shereceivedadjuvantradiatio

6、ntherapy(5040cGy)owingtosuspectedinvasionintothepericardialspace.Fourweekslater,brainMRIshoweddecreaseinsizeofthebrainlesions(Figure1,B).Twoyearsaftertheoperation,thepatienthasnoevidenceoftumorrecurrence(Figure1,D)orneurologicimpairment.Since1988,when

7、thefirstcaseofparaneoplasticencephalitisinthesettingofthymiccancerwasreported,28casesofthymoma-associatedparaneoplasticencephalitis(TAPE)havebeendescribed(Table1).從1988-2010共報(bào)道了28例胸腺瘤相關(guān)的副腫瘤腦炎。AntineuronalAuthorAge/sexSymptomsStageMRI,T2-weightedPleocy

8、tosisantibodiesTreatmentOutcomeMcArdle41/MConfusion,memoryIIINoANA(serum)Partialthymectomy,Death(1mo)-1988deficit,oligoclonalradiation,hallucinationsIgGbands(CSF)corticosteroidsIngenito59/MConfusion,memoryINormal13WBCCorticosteroidsDeath(51d)-

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