胸腺瘤相关性副肿瘤边缘性脑炎课件

胸腺瘤相关性副肿瘤边缘性脑炎课件

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时间:2018-10-11

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1、Thymoma-associatedparaneoplasticencephalitis(TAPE)胸腺瘤相关的副肿瘤性脑炎Upto50%ofpatientswiththymomahaveparaneoplasticneurologicsyndromes,themostcommonbeingmyastheniagravis.Therearerarecasereportsofthymoma-associatedparaneoplasticlimbicorextralimbicencephalitis

2、thatcanleadtoprogressiveneurologicdeclineanddeathwithouttreatment.胸腺瘤相关的副肿瘤边缘性及边缘外脑炎报道的比较少,但这种情况可导致进行性的神经功能障碍甚至死亡。50%胸腺瘤患者会出现副肿瘤性神经系统症状,最常见的是重症肌无力。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).从1988-2010共报道了28例胸腺瘤相关的副肿瘤脑炎。AntineuronalAuthorAge/sexSymptomsStageMRI,T2-weightedPleocy

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

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