中华实用儿科临床杂志
中華實用兒科臨床雜誌
중화실용인과림상잡지
Journal of Applied Clinical Pediatrics
2015年
11期
857-859
,共3页
获得性癫痫性失语%预后%临床特征%治疗研究%生酮饮食
穫得性癲癇性失語%預後%臨床特徵%治療研究%生酮飲食
획득성전간성실어%예후%림상특정%치료연구%생동음식
Landau-Kleffner syndrome%Prognosis%Clinical feature%Curative effect analysis%Ketogenic diet
目的 分析预后不良Landau-Kleffner综合征(LKS)的临床特征与治疗研究.方法 以2008年9月至2012年12月在复旦大学附属儿科医院神经科收治的预后不良的5例LKS患儿为研究对象,分析5例LKS临床资料与视频脑电图资料,分析LKS病例抗癫(痫)药物疗效、皮质激素治疗失语与睡眠中癫(痫)性电持续状态(ESES)疗效,以及采用生酮饮食治疗疗效.Engel分级评估用于癫痫疗效的评估,棘波指数减少程度作为ESES疗效的评估.结果 5例患儿中男2例,女3例;均有听觉性失认/失语及癫(痫)发作;脑电图背景活动正常,均有(痫)样放电,放电部位以双侧颞区为主;伴ESES 3例.5例患儿接受抗癫痫药物治疗,4例为药物难治性癫(痫),1例癫痫发作控制;4例患儿接受6个月皮质激素中程疗法,1例失语消失,1例失语改善,2例无效;ESES3例均无效;3例患儿接受生酮饮食治疗,1例患儿癫痫发作控制,持续性ESES现象消失,1例持续性ESES现象好转,1例癫痫发作较前减少.家长描述所有患儿语言表达及理解能力较皮质激素或生酮饮食治疗前有所改善,但均仍存在不同程度的认知障碍.结论 预后不良的LKS患儿癫(痫)发作类型多,多为药物难治性癫痫和/或持续ESES;皮质激素治疗失语,可不同程度改善认知;生酮饮食可作为LKS难治或难治性ESES的治疗选择.
目的 分析預後不良Landau-Kleffner綜閤徵(LKS)的臨床特徵與治療研究.方法 以2008年9月至2012年12月在複旦大學附屬兒科醫院神經科收治的預後不良的5例LKS患兒為研究對象,分析5例LKS臨床資料與視頻腦電圖資料,分析LKS病例抗癲(癇)藥物療效、皮質激素治療失語與睡眠中癲(癇)性電持續狀態(ESES)療效,以及採用生酮飲食治療療效.Engel分級評估用于癲癇療效的評估,棘波指數減少程度作為ESES療效的評估.結果 5例患兒中男2例,女3例;均有聽覺性失認/失語及癲(癇)髮作;腦電圖揹景活動正常,均有(癇)樣放電,放電部位以雙側顳區為主;伴ESES 3例.5例患兒接受抗癲癇藥物治療,4例為藥物難治性癲(癇),1例癲癇髮作控製;4例患兒接受6箇月皮質激素中程療法,1例失語消失,1例失語改善,2例無效;ESES3例均無效;3例患兒接受生酮飲食治療,1例患兒癲癇髮作控製,持續性ESES現象消失,1例持續性ESES現象好轉,1例癲癇髮作較前減少.傢長描述所有患兒語言錶達及理解能力較皮質激素或生酮飲食治療前有所改善,但均仍存在不同程度的認知障礙.結論 預後不良的LKS患兒癲(癇)髮作類型多,多為藥物難治性癲癇和/或持續ESES;皮質激素治療失語,可不同程度改善認知;生酮飲食可作為LKS難治或難治性ESES的治療選擇.
목적 분석예후불량Landau-Kleffner종합정(LKS)적림상특정여치료연구.방법 이2008년9월지2012년12월재복단대학부속인과의원신경과수치적예후불량적5례LKS환인위연구대상,분석5례LKS림상자료여시빈뇌전도자료,분석LKS병례항전(간)약물료효、피질격소치료실어여수면중전(간)성전지속상태(ESES)료효,이급채용생동음식치료료효.Engel분급평고용우전간료효적평고,극파지수감소정도작위ESES료효적평고.결과 5례환인중남2례,녀3례;균유은각성실인/실어급전(간)발작;뇌전도배경활동정상,균유(간)양방전,방전부위이쌍측섭구위주;반ESES 3례.5례환인접수항전간약물치료,4례위약물난치성전(간),1례전간발작공제;4례환인접수6개월피질격소중정요법,1례실어소실,1례실어개선,2례무효;ESES3례균무효;3례환인접수생동음식치료,1례환인전간발작공제,지속성ESES현상소실,1례지속성ESES현상호전,1례전간발작교전감소.가장묘술소유환인어언표체급리해능력교피질격소혹생동음식치료전유소개선,단균잉존재불동정도적인지장애.결론 예후불량적LKS환인전(간)발작류형다,다위약물난치성전간화/혹지속ESES;피질격소치료실어,가불동정도개선인지;생동음식가작위LKS난치혹난치성ESES적치료선택.
Objective To analyze the clinical features and efficacy of different treatments on 5 cases with Landau-Kleffner syndrome (LKS) with poor prognosis.Methods Five patients with LKS who had poor prognosis were enrolled in the Children's Hospital of Fudan University from September of 2008 to December of 2012.The clinical features and findings of video electroencephalograph (VEEG) of these patients were analyzed.In addition,the outcomes of treatments (antiepileptic drug,corticosteroids and ketogenic diet) on LKS who had poor prognosis were studied.The outcome of epilepsy was classified by using the Engel classification scheme and the outcome of electrical status epilepticus during sleep (ESES) was classified by the decrease in spike wave index (SWI).Results All of 5 patients (2 male and 3 female) had auditory agnosia and epileptic seizure.All patients had normal background activity and epileptiform discharges,and the location of epileptiform discharge was mainly from bilateral temporal;3 patients had ESES.Four out of the 5 patients had refractory epilepsy and the other one got seizure free with antiepileptic drug.Four patients received corticosteroids for 6 months.The aphasia disappeared in 1 patient,improved in 1 patient and sustained in the other 2 patients.Three patients who had ESES did not response to corticosteroids.Among 3 patients receiving ketogenic diet,1 patient was seizure free and the ESES disappeared;in another SWI was reduced;the rest one had a significant reduction in seizure.All the patients had improvement in their aphasia and cognition,however they still had cognitive disorder of various degrees.Conclusions The patients who had multiple seizure type or special seizure type may have poor outcome.Most of these patients have refractory epilepsy or persistent ESES.Corticosteroids can improve aphasia and cognition,but the persistence of ESES can make aphasia and cognition worse.Ketogenic diet can be used as an alternative treatment for refractory epilepsy or persistent ESES in LKS.
