生酮饮食治疗皮质发育畸形导致儿童药物难治性癫痫的有效性和安全性  

The efficacy and safety of ketogenic diet in the treatment of drug-resistant epilepsy in children with malformations of cortical development

作　　者：张洪伟[1] 赵芬[1] 胡万冬 张欢 刘勇[1] 金瑞峰[1] ZHANG Hongwei;ZHAO Fen;HU Wandong;ZHANG Huan;LIU Yong;JIN Ruifeng(Epilepsy Center,Children's Hospital Affiliated to Shandong University(Jinan Children's Hospital),Jinan 250022,China)

机构地区：[1]山东大学附属儿童医院(济南市儿童医院)癫痫中心,济南250022

出　　处：《癫痫杂志》2023年第6期461-465,共5页Journal of Epilepsy

基　　金：中国抗癫痫协会CAAE癫痫科研基金-奇酮基金(CJ-2022-008);济南市卫生健康委员会科技计划项目(2018-2-34)。

摘　　要：目的探究生酮饮食(Ketogetic diet,KD)疗法在皮层发育畸形(Malformations of cortical development,MCD)致儿童药物难治性癫痫的有效性和安全性。方法回顾性分析2021年5月—2023年2月山东大学附属儿童医院癫痫中心收治的10例MCD致药物难治性癫痫患儿的临床资料,均为首次接受经典KD治疗。分别于KD治疗后1、3、6、12个月对患儿进行随访,采用Engel分级评估临床疗效,同时记录不良反应情况。结果本研究10例患者,男女各5例,起病年龄为0.2~36.0(10.3±11.1)月龄,病程2.0~31.0(9.7±8.5)个月,开始KD的年龄为3.0~50.0(20.0±15.7)个月。头颅影像学提示:2例为半侧巨脑症、1例为无脑回、1例为巨脑回合并多微小脑回畸形、6例FCD、3例有基因异常(2例DEPDC5基因、1例ARX基因),所有患儿KD治疗前均有不同程度的发育迟缓。KD治疗前服用的抗癫痫发作药物为2.0~5.0(3.2±0.9)种,KD治疗3个月的无发作率为20%(2/10),有效率为50%(5/10)。发育改善率:3个月为50.0%(5/10)。5例有轻微的不良反应(50%),其中1例为低钾、4例为胃肠道反应,均在下调KD饮食比例后缓解,没有患儿因不良反应停用KD。结论KD疗法是一种治疗MCD致儿童药物难治性癫痫的安全有效的方法,部分患儿可改善发育水平。Objective To explore the efficacy and safety of ketogenic diet(KD)in the treatment of drug-resistant epilepsy in children with malformations of cortical development(MCD).Methods The clinical data of 10 children with drug-resistant epilepsy caused by MCD treated in the Epilepsy Center of Children's Hospital affiliated to Shandong University from May 2021 to February 2023 were analyzed retrospectively.All of them received classical KD treatment for the first time.The patients were followed up at 1,3,6 and 12 months after KD treatment.The clinical efficacy was evaluated by Engel grade and the adverse reactions were recorded at the same time.Results There were 10 patients in this study,including 5 males and 5 females.The age of onset was 0.2~36.0(10.3±11.1)months,2.0~31.0(9.7±8.5)months,and the age of starting KD was 3.0~50.0(20.0±15.7)months.Cranial imaging showed that there were 2 cases of hemimegalencephaly,1case of lissencephaly,1 case of pachygyria combine polymicrogyria,and 6 cases of FCD,3 had gene abnormality(2 cases of DEPDC5 gene,1 case of ARX gene).All children had different degrees of developmental retardation before KD treatment.The antiseizure medications taken before KD treatment were 2.0~5.0(3.2±0.9).5/10(50%)children had a>50%reduction in seizure frequency at 3 months on the diet,2/10(20%)children had a seizure free response.The rate of development improvement was 50.0%(5/10)at 3 months.5 cases had mild adverse reactions(50%),including 1 case of hypokalemia and 4 cases of gastrointestinal reactions,all of which were relieved after the reduction of the proportion of KD diet.None of the children stopped using KD.Conclusion KD therapy is a safe and effective method for the treatment of drug-resistant epilepsy in children caused by MCD,and some children can improve their developmental level.

关 键 词：生酮饮食 皮层发育畸形 癫痫 疗效 

分 类 号：R742.1[医药卫生—神经病学与精神病学]