生酮饮食治疗儿童难治性癫（痫）性脑病31例短期疗效及其对脑电图的影响  

Influence of ketogenic diet on the clinical effects and electroencephalogram features in 31 children with pharmacoresistant epileptic encephalopathy

作　　者：李保敏[1] 童丽丽[1] 贾桂娟[1] 王纪文[1] 雷革非[1] 尹苹[1] 孙若鹏[1] 

机构地区：[1]山东大学齐鲁医院儿科,济南250012

出　　处：《中华儿科杂志》2013年第5期362-366,共5页Chinese Journal of Pediatrics

基　　金：山东省自然科学基金（ZR2009CM72）;山东省科技发展计划（2010GSF10201）

摘　　要：目的 探讨生酮饮食疗法对难治性癫（痫）性脑病患儿的短期临床疗效及其对脑电图的影响.方法 31例经过2种以上抗癫（痫）药物正规治疗无效的药物难治性癫（痫）性脑病患儿,其中男19例,女12例,年龄7个月～7岁,平均2岁5个月.婴儿痉挛16例,Lennox-Gastaut综合征11例,Doose综合征2例,Dravet综合征2例.生酮饮食方式：3岁以内使用生酮奶,3岁以上按“生酮饮食配餐软件”制定生酮饮食食谱.生酮饮食治疗后原有抗癫（痫）药物不改变.比较生酮饮食治疗前、治疗后1周、1个月及3个月时患儿临床发作,评价生酮饮食的临床疗效.评价生酮饮食对脑电图的影响：分别在治疗前、治疗后1周、1个月、3个月对患儿进行≥8h录像脑电图监测,评价枕区背景节律及发作间期棘（尖）波放电指数的变化.结果 （1）生酮饮食疗效：有效率随时间的延长有增加趋势,1周时16例有效,1个月时21例有效,3个月时22例有效.（2）治疗3个月时对癫（痫）综合征的疗效：不同癫（痫）综合征对生酮饮食的反应不同,其中Doose综合征对生酮饮食反应最好,2例均有效.婴儿痉挛对生酮饮食反应较好,9/16例完全无发作,总有效例数为13例.（3）生酮饮食治疗前后脑电图变化：治疗3个月时,24例患儿背景节律明显改变,19例患儿发作间期棘（尖）波指数明显降低（减少＞30％）.31例中出现不良反应者9例,所有不良反应患儿均能耐受.结论 生酮饮食对于药物难治性癫（痫）具有一定的疗效,可减少发作间期（痫）性放电频率,改善脑电的背景节律.Objective To investigate the effect of ketogenic diet （KD） on the clinical and electroencephalogram features in children with pharmacoresistant epileptic encephalopathy.Method Thirtyone children （19 boys,12 girls） aged 7 months to 7 years （mean 2 years 5 month） with epilepsy refractory to conventional antiepileptic drugs （AEDs） were included in this study.In addition to their original AED treatment,the children were assigned to different ketogenic diets based on their age.The prospective electroclinical assessment was performed prior to the KD and then one week,one month and again 3 months after the initiation of therapy,respectively.Result The reduction of seizure frequency in 52%,68% and 71% of all patients exceeded 50% one week,one month and three months after KD treatment respectively.KD is particularly effective in myoclonic astatic epilepsy （MAE; Doose Syndrome） and West syndrome with 100% and 81.25% of the patients having a greater than 50% seizure reduction,respectively.After 3 months of KD treatment,more than 2/3 patients experienced a reduction in interictal epileptiform discharges （IEDs）and improvement in EEG background.Conclusion The clinical and electroencephalographic improvement confirms that KD is beneficial in children with refractory epilepsy.

关 键 词：生酮饮食 治疗结果 脑电描记术 癫(痫) 

分 类 号：R742.1[医药卫生—神经病学与精神病学]