湖州會(huì)嬰幼兒fcd外科治療匯報(bào)

1、嬰幼兒額葉FCD難治性癲癇的外科治療,浙江大學(xué)醫(yī)學(xué)院附屬第二醫(yī)院功能神經(jīng)外科 癲癇中心朱君明 鄭喆 傅偉明 張建民王爽 湯業(yè)磊 郭誼 丁美萍,,兒童難治性癲癇大多數(shù)皮質(zhì)發(fā)育異常引起。頻繁發(fā)作影響兒童的發(fā)育。嬰幼兒難治性癲癇外科治療時(shí)機(jī)？,2,CASE1,HistoryYJS 14 months old, Male, Seizure started at 1 month old. Mother found bilat

2、eral limbs convulsion for 10-50 sec. During seizures, the right limbs were probably more tonic, with eye and head deviated to the right. The seizure frequency is 3-8 per day, it is more often on awakening. The baby is

3、now on Keppra, Trileptal and not responsive. He was on Valproate but got hepatic dysfunction.MRI scan showed a lesion on the left frontal (cortical malformation). The family took the kid to several famous hospitals in C

4、hina. Surgical resection of the lesion was recommend.The development was obviously delayed. The baby cannot walk, can say “mama” or “papa” unintentionally. The family report no history of complicated delivery. No signi

5、ficant family history.,Current Anti-epileptic Medication:Trileptal: 150mg, 180mg Bid.Keppra: 250mg, 300mg BidPhysical examination: Normal Body weight: 10 kg, Head circumference: 45.4 cmBody height: 80 cm,Developme

6、nt function Appraisal,A. Bay ley: cognition function (equivalent to a 8 months old level); movement function (equivalent to a 7-8 months old level)B. Normal muscle tone. Muscle strength of right hand and leg is slight d

7、ecreased.C. Able to sit and stand (but cannot take the position by himself), not able to walk and crawl.,VEEG (2014,5,19-2014,5, 21),Background, 6 HzSleep structure: well-differentiated, symmetrical Interitcal sharp

8、wave: Left anterior head region(F7,F3), right frontal (rare, right frontal, F4)Ictal recordings:Generalized tonic-clonic seizure (10 recorded)Description: the tonic movement were more severe on the right limbs, and

9、 post-ictal limb weakness were more sever on the right side. EEG seizure: Left hemisphere,Background,,Sharp wave F7,F3,,Sharp wave (F7,F3,C3),,Sharp wave (F4, rarely found),,Sz 2P EEG onset,,Sz 2P EEG onset + 10 sec,,Sz

10、 2P EEG onset + 20 sec,,Sz 6P EEG onset,,Sz 6P EEG onset +10 sec,,Sz 6p EEG onset + 20 sec,,MRI(local hospital, March 29, 2013) : Left frontal cortical malformation,,MRI,18,PET-MRI,19,術(shù)中,20,,21,術(shù)中棘波發(fā)放及切除范圍示意圖,22,術(shù)中初步切除范圍

11、,23,暴露深部FCD,24,切除深部FCD后,25,發(fā)現(xiàn)中央前回持續(xù)棘波發(fā)放,26,,27,繼續(xù)切除中央前回上部,28,術(shù)中再次腦電檢測(cè),29,,30,術(shù)后MRI,31,術(shù)后MRI,32,常規(guī)病理：皮層分層紊亂，異形神經(jīng)元,33,美國(guó)UCLA,34,,隨訪近四個(gè)月無癲癇發(fā)作藥物：Trileptal: 150mg, 180mg Bid.Keppra: 250mg, 300mg Bid,35,病史（病例2）,DXY ，女，1歲4個(gè)

12、月，反復(fù)發(fā)作肢體抽搐，意識(shí)喪失2月余?；純河?月余前活動(dòng)中突發(fā)四肢僵硬，牙關(guān)緊閉，雙眼上翻，口唇發(fā)紺，口吐白沫，意識(shí)喪失，持續(xù)約半小時(shí)。后相同癥狀反復(fù)發(fā)作，多次發(fā)作后伴體溫升高，期間以“病毒性腦炎”多次住院治療，發(fā)作嚴(yán)重時(shí)發(fā)作1分鐘,緩解1-2分鐘，持續(xù)1-2小時(shí)。服用奧卡西平，卡馬西平，丙戊酸鈉，硝西泮，注射力月西等藥物，癥狀控制不佳，仍反復(fù)發(fā)作，多時(shí)一天27次。,36,術(shù)前頭皮腦電（病例2）發(fā)作間期：尖波，區(qū)域性，左側(cè)額

13、葉（FP1、F3、F7）,術(shù)前頭皮腦電（病例2）強(qiáng)直發(fā)作：Sz1-4P，左側(cè)額葉（FP1、F3、F7）,影像（病例2）,,,,,,40,術(shù)中SEEP確定中央溝……,術(shù)中皮層腦電（病例1，病灶切除前）,1,術(shù)中皮層腦電（病例1，病灶切除前）,2,術(shù)中皮層腦電（病例1，病灶切除前）,3,術(shù)中皮層腦電（病例1，病灶切除前）,4,術(shù)中皮層腦電（病例1，病灶切除前）,5,術(shù)中皮層腦電（病例1，病灶切除前）,6,術(shù)中皮層腦電（病例2，病灶切除前）,

14、7,術(shù)中皮層腦電（病例2，病灶切除前）,8,術(shù)中Broca區(qū)……,術(shù)中（病例2）,術(shù)中皮層腦電（病例2，病灶切除后）,病例2術(shù)后MRI，病理（HE）：ILAE FCD IIb型,病例2術(shù)后MRI,病例2術(shù)后MRI,病理（HE）：ILAE FCD IIb型,FCD皮層（HE ×100）：灰白質(zhì)相對(duì)模糊，皮層結(jié)構(gòu)紊亂，神經(jīng)元擁擠，形態(tài)異常，排列混亂。,57,FCD氣球細(xì)胞（HE ×200）：灰白質(zhì)交界處多見（箭頭示），該

15、細(xì)胞本質(zhì)為變性神經(jīng)元，體積較大，缺乏尼氏體，胞質(zhì)紅染，細(xì)胞核核偏位。,58,病理診斷：指定處腦組織 [浙二醫(yī)院2014-04536 A3]:-局灶性皮質(zhì)發(fā)育不良，ILAE FCD IIb型說明：說明：評(píng)價(jià)巨腦回受顯微切片限制，但我們看到的嚴(yán)重皮質(zhì)發(fā)育不良符合病變的存在。顯微鏡下檢查：標(biāo)本切片由皮質(zhì)和皮質(zhì)下白質(zhì)組成，皮質(zhì)顯示正常皮質(zhì)結(jié)構(gòu)畸形和神經(jīng)元擁擠、混亂，皮質(zhì)層較正常增寬，但復(fù)雜的旋轉(zhuǎn)定向限制了確定性，巨細(xì)胞性的和形態(tài)

16、異常的神經(jīng)元常見，皮質(zhì)和皮質(zhì)下白質(zhì)中見較多,,術(shù)后3月時(shí)無發(fā)作，目前隨訪5月偶有發(fā)作。,60,病史（病例3）,TCR 患兒，男，1歲7月6天，反復(fù)發(fā)作性肢體抽搐11月余?；純?個(gè)月大時(shí)無明顯誘因下出現(xiàn)肢體抽搐，雙眼上翻，意識(shí)喪失后患者抽搐逐漸頻繁加重，4-5次/天。陸續(xù)予“曲萊、妥泰”治療，治療效果不佳，仍有發(fā)作10余次。,61,,癥狀學(xué)：簡(jiǎn)單運(yùn)動(dòng)發(fā)作過度運(yùn)動(dòng)發(fā)作發(fā)作頻率：最多時(shí)1天20多次，最少時(shí)1次/天。既往用藥：德巴金

17、、妥泰目前用藥：德巴金500mg BID 妥泰100mg bid,62,,63,,64,,65,（1歲,頻繁抽搐發(fā)作-）,術(shù)前頭皮腦電（病例3）發(fā)作間期：,1、后頭部背景左側(cè)明顯；2、持續(xù)性慢波，右側(cè)半球；3、間歇性慢波，左側(cè)額葉；4、尖波，區(qū)域性，右側(cè)額葉；5、尖波，區(qū)域性，右側(cè)顳頂區(qū)（T6、T4、P4明顯）；6、尖波，區(qū)域性，左側(cè)額葉（FP1明顯）,術(shù)前頭皮腦電（病例3）發(fā)作間期：,術(shù)前頭皮腦電（病例3）發(fā)作期：右側(cè)額葉

18、（雙上肢強(qiáng)直發(fā)作）,病例3術(shù)中，病理（HE）：ILAE FCD IIb型,病例3術(shù)中,術(shù)中皮層腦電（病例3，病灶切除前）,1,術(shù)中皮層腦電（病例3，病灶切除前）,2,術(shù)中皮層腦電（病例3，病灶切除前）,3,術(shù)中皮層腦電（病例3，病灶切除前）,4,術(shù)中皮層腦電（病例2，病灶切除后）,術(shù)后CT,77,術(shù)后MRI,78,病理（HE）：ILAE FCD IIb型,病理診斷：右側(cè)額葉[OSR#2013-38208]-局灶腦皮質(zhì)發(fā)育不良， IL

19、AE FCD類型IIb顯微鏡下檢查：切片可見腦皮質(zhì)及白質(zhì)。腦皮質(zhì)結(jié)構(gòu)異常、神經(jīng)元擁擠，可見增大的形態(tài)不良的神經(jīng)元。偶爾可于腦皮質(zhì)及白質(zhì)中見到氣球樣細(xì)胞?？梢姷紺haslin‘s神經(jīng)膠質(zhì)增生。未見明顯的腫瘤性、炎癥性、傳染性病變。,,隨訪11月無發(fā)作。藥物同術(shù)前。,81,Case（4）,ZQS 男，2歲發(fā)作性四肢抽搐神志不清2年。每天發(fā)作5-40次奧卡西平；妥泰；德巴金；魯米那,82,術(shù)前MRI,83,,84,術(shù)中,85,標(biāo)