胸腺瘤相关性副肿瘤边缘性脑炎PPT课件.pptx

胸腺瘤相关的副肿瘤性脑炎 1 Upto50 ofpatientswiththymomahaveparaneoplasticneurologicsyndromes themostcommonbeingmyastheniagravis Therearerarecasereportsofthymoma associatedparaneoplasticlimbicorextralimbicencephalitisthatcanleadtoprogressiveneurologicdeclineanddeathwithouttreatment 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 2 病例 Apreviouslyasymptomatic61 year oldwomanhadgeneralizedintractableseizures Brainmagneticresonanceimaging MRI showedbilateralfociofcorticalandsubcorticalnonenhancingsignalabnormalitiesonT2 weightedimages Figure1 A Chestcomputedtomography CT revealedalargeanteriormediastinalmass Figure1 C that onbiopsy showedmalignantthymoma AnalysisofcerebrospinalfluiddetectedapredominantlylymphocyticpleocytosisandpositiveLGI1antibodies 3 Thepatientreceivedanticonvulsants humanintravenousimmunoglobulinG IgG 400g kgdaily andcorticosteroids methylprednisolone1gmdaily for5daysplusaprednisonetaperwithresolutionofneurologicsymptoms Fourweekslater thepatientunderwentcomplete enblocresectionofthemediastinalmassthatwasinvadingthroughthepericardium typeB3 WorldHealthOrganizationclassification MasaokastageIII Shereceivedadjuvantradiationtherapy 5040cGy owingtosuspectedinvasionintothepericardialspace Fourweekslater brainMRIshoweddecreaseinsizeofthebrainlesions Figure1 B Twoyearsaftertheoperation thepatienthasnoevidenceoftumorrecurrence Figure1 D orneurologicimpairment 4 Since1988 whenthefirstcaseofparaneoplasticencephalitisinthesettingofthymiccancerwasreported 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 5 TABLE1 Clinicalfeaturesofthymoma associatedparaneoplasticencephalitis 6 7 8 Theencephalitiscanbeconfinedtothelimbicsystemormultifocalandextralimbic Inmostpatients 82 neurologicmanifestationsincludememoryloss confusion andseizures SeventypercentexhibitedbilateralfociofnonenhancinghyperintensityonbrainMRI someappearingweeksafteronsetofsymptoms Thedifferentialdiagnosisincludesmetastases primarybraintumors andinfection 这种脑炎可累及边缘系统 边缘系统外结构或是呈多灶损伤 82 的病人出现的神经症状有记忆丢失 精神错乱和癫痫 70 的患者在MRI上表现为双侧非强化的高信号 并可以持续数周 需与转移性 原发性脑肿瘤及感染鉴别 9 Althoughroutineanalysisofcerebrospinalfluidisnonspecific samplingofserumandcerebrospinalfluidforantineuronalantibodies foundin75 ofpatientswithTAPE willaidinestablishingaparaneoplasticpathogenesis ThediagnosisofTAPEdependsonthecombinationoftheencephalitissyndrome MRIfindings anddetectionofantineuronalautoantibodiesinthesettingofathymicneoplasm 此类患者脑脊液常规生化没有很特异的表现 脑脊液淋巴细胞增多 蛋白水平增高 并发现寡克隆带 均支持边缘叶脑炎的诊断可能 但是 相似的脑脊液异常亦可见于其它炎症性或感染性疾病 而且 边缘叶脑炎患者的脑脊液 亦可正常 75 的患者可以在血及脑脊液中检测到抗神经元抗体 对于TAPE的诊断主要依靠在胸腺瘤患者中出现脑炎症状 头颅MRI表现及检测到抗神经元抗体 10 OncethediagnosisofTAPEhasbeenmade immediateinterventionisnecessarytopreventprogressiveneurologicdeclineanddeath ImmunotherapywithIgGandcorticosteroidswillimproveneurologicsymptomsresultingfrominflammatoryresponse butcompleteresectionofthethymomaconfersthebestchanceatdisease freesurvivalfromoncologicaswellasparaneoplasticstandpoints Antibodytitersdecreaseafterthymectomy thusabatingongoinginflammationandcelldeath Ifcorticosteroids IgG andthymectomyfailtoimproveneurologicsymptoms diminishingantineuronalantibodieswithplasmapheresis 一旦诊断TAPE应给以积极治疗干预 以避免出现进行性神经功能损害甚至死亡 IVIgG 激素可以改善神经症状 但胸腺瘤切除是让患者存活的做好选择 若IVIgG 激素 胸腺瘤切除都没改善神经症状 可选择血浆置换 11 Thirty threepercentofpatientswithTAPEdied allasaconsequenceofthenervoussystemcompromise ThemajorityofthesepatientswhodiedhadstageIthymomas EarlyMasaokastagethymomasmayproducemorefunctionalandneurotoxicautoantibodiesthantheirmalignantcounterparts Therefore outcomeisdrivenmorebyongoingneurologicinsultthantheunderlyingthymicdisease Only2