嬰幼兒額葉FCD難治性癲癇的外科治療,浙江大學醫學院附屬第二醫院功能神經外科 癲癇中心朱君明 鄭喆 傅偉明 張建民王爽 湯業磊 郭誼 丁美萍,兒童難治性癲癇大多數皮質發育異常引起。頻繁發作影響兒童的發育。嬰幼兒難治性癲癇外科治療時機？,2,CASE1,HistoryYJS 14 months old, Male, Seizure started at 1 month old. Mother found bilateral 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 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 China. 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 significant 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,Development 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 decreased.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 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 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 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,術中,20,21,術中棘波發放及切除范圍示意圖,22,術中初步切除范圍,23,暴露深部FCD,24,切除深部FCD后,25,發現中央前回持續棘波發放,26,27,繼續切除中央前回上部,28,術中再次腦電檢測,29,30,術后MRI,31,術后MRI,32,常規病理：皮層分層紊亂，異形神經元,33,美國UCLA,34,隨訪近四個月無癲癇發作藥物：Trileptal: 150mg, 180mg Bid.Keppra: 250mg, 300mg Bid,35,病史（病例2）,DXY ，女，1歲4個月，反復發作肢體抽搐，意識喪失2月余。患兒于2月余前活動中突發四肢僵硬，牙關緊閉，雙眼上翻，口唇發紺，口吐白沫，意識喪失，持續約半小時。后相同癥狀反復發作，多次發作后伴體溫升高，期間以“病毒性腦炎”多次住院治療，發作嚴重時發作1分鐘,緩解1-2分鐘，持續1-2小時。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，仍反復發作，多時一天27次。,36,術前頭皮腦電（病例2）發作間期：尖波，區域性，左側額葉（FP1、F3、F7）,術前頭皮腦電（病例2）強直發作：Sz1-4P，左側額葉（FP1、F3、F7）,影像（病例2）,40,術中SEEP確定中央溝,術中皮層腦電（病例1，病灶切除前）,1,術中皮層腦電（病例1，病灶切除前）,2,術中皮層腦電（病例1，病灶切除前）,3,術中皮層腦電（病例1，病灶切除前）,4,術中皮層腦電（病例1，病灶切除前）,5,術中皮層腦電（病例1，病灶切除前）,6,術中皮層腦電（病例2，病灶切除前）,7,術中皮層腦電（病例2，病灶切除前）,8,術中Broca區,術中（病例2）,術中皮層腦電（病例2，病灶切除后）,病例2術后MRI，病理（HE）：ILAE FCD IIb型,病例2術后MRI,病例2術后MRI,病理（HE）：ILAE FCD IIb型,FCD皮層（HE 100）：灰白質相對模糊，皮層結構紊亂，神經元擁擠，形態異常，排列混亂。,57,FCD氣球細胞（HE 200）：灰白質交界處多見（箭頭示），該細胞本質為變性神經元，體積較大，缺乏尼氏體，胞質紅染，細胞核核偏位。,58,病理診斷：指定處腦組織 浙二醫院2014-04536 A3:-局灶性皮質發育不良，ILAE FCD IIb型說明：說明：評價巨腦回受顯微切片限制，但我們看到的嚴重皮質發育不良符合病變的存在。顯微鏡下檢查：標本切片由皮質和皮質下白質組成，皮質顯示正常皮質結構畸形和神經元擁擠、混亂，皮質層較正常增寬，但復雜的旋轉定向限制了確定性，巨細胞性的和形態異常的神經元常見，皮質和皮質下白質中見較多,術后3月時無發作，目前隨訪5月偶有發作。,60,病史（病例3）,TCR 患兒，男，1歲7月6天，反復發作性肢體抽搐11月余。患兒8個月大時無明顯誘因下出現肢體抽搐，雙眼上翻，意識喪失后患者抽搐逐漸頻繁加重，4-5次/天。陸續予“曲萊、妥泰”治療，治療效果不佳，仍有發作10余次。,61,癥狀學：簡單運動發作過度運動發作發作頻率：最多時1天20多次，最少時1次/天。既往用藥：德巴金、妥泰目前用藥：德巴金500mg BID 妥泰100mg bid,62,63,64,65,（1歲,頻繁抽搐發作-）,術前頭皮腦電（病例3）發作間期：,1、后頭部背景左側明顯；2、持續性慢波，右側半球；3、間歇性慢波，左側額葉；4、尖波，區域性，右側額葉；5、尖波，區域性，右側顳頂區（T6、T4、P4明顯）；6、尖波，區域性，左側額葉（FP1明顯）,術前頭皮腦電（病例3）發作間期：,術前頭皮腦電（病例3）發作期：右側額葉（雙上肢強直發作）,病例3術中，病理（HE）：ILAE FCD IIb型,病例3術中,術中皮層腦電（病例3，病灶切除前）,1,術中皮層腦電（病例3，病灶切除前）,2,術中皮層腦電（病例3，病灶切除前）,3,術中皮層腦電（病例3，病灶切除前）,4,術中皮層腦電（病例2，病灶切除后）,術后CT,77,術后MRI,78,病理（HE）：ILAE FCD IIb型,病理診斷：右側額葉OSR#2013-38208-局灶腦皮質發育不良， ILAE FCD類型IIb顯微鏡下檢查：切片可見腦皮質及白質。腦皮質結構異常、神經元擁擠，可見增大的形態不良的神經元。偶爾可于腦皮質及白質中見到氣球樣細胞。可見到Chaslins神經膠質增生。未見明顯的腫瘤性、炎癥性、傳染性病變。,隨訪11月無發作。藥物同術前。,81,Case（4）,ZQS 男，2歲發作性四肢抽搐神志不清2年。每天