预后不良Landau-Kleffner综合征5例的临床特征与治疗

Clinical features and efficacy of different treatments on 5 cases with Landau - Kleffner syndrome with poor prognosis

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ESES3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。

Objective To analyze the clinical features and efficacy of different treatments on 5 cases with Lan- dau - 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 fea- tures 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 epilepti- cus 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 epilepti- form 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 antiepileptie 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 cortieosteroids. 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 re- duction 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. Cortieosteroids can improve aphasia and cognition, but the persistence of ESES can make aphasia and cognition worse. Ketogenic diet can be used as an al- ternative treatment for refractory epilepsy or persistent ESES in LKS.