生酮饮食对25例癫痫性脑病患儿疗效的临床回顾性研究  

Clinical retrospective study on the effect of ketogenic diet on 25 children with epileptic encephalopathy

作　　者：樊海珍[1] 王燕梅[1] 韩虹[1] Fan Haizhen;Wang Yanmei;Han Hong(Department of Neurology,Children′s Hospital of Shanxi,Taiyuan030013,China)

机构地区：[1]山西省儿童医院神经内科,太原030013

出　　处：《山西医药杂志》2020年第23期3204-3209,共6页Shanxi Medical Journal

摘　　要：目的评估生酮饮食(KD)对癫痫性脑病患儿的疗效及影响因素。方法选择2010年3月至2019年10月在我院确诊的25例癫痫性脑病患儿,其中west综合征14例,Lennox-Gastaut综合征2例,Dravet综合征1例,其他8例,采用改良的Johns-Hopkins方案启动KD,采用Eagle量表评价其疗效,观察记录KD治疗的依从性、不良反应以及KD对脑电图(EEG)和认知功能的影响。结果KD治疗12周时,Ⅰ、Ⅱ、Ⅲ、Ⅳ级疗效分别为8%(2/25例)、12%(3/25例)、32%(8/25例)和36%(9/25例),退出12%(3例)。KD对不同癫痫证型疗效不同,Dravet综合征1例治疗后无发作;治疗West综合征的有效率为32%,疗效较好。治疗12周时总有效率为12%。但在KD治疗过程,随持续时间延长,疗效有波动,亦有无法坚持而退出治疗者。患儿退出治疗的主要因素为不耐受、疗效不佳及配餐不便。至48周时认知功能明显改善3例。共有18例患者出现不良反应,不良反应随治疗的持续多可逐渐改善。1例因肝功异常退出,6例不耐受退出,9例因疗效不佳退出治疗。结论KD对癫痫性脑病患儿有一定疗效,EEG背景节律改善,发作间期癫痫放电频率下降,有利于减少癫痫发作。KD对认知功能可能有改善作用。其不良反应是影响患者坚持KD治疗的主要因素之一,后续应关注于提高KD治疗的依从性以及配餐口感与便利性。Objective To evaluate the effect of ketogenic diet(KD)on children with intractable epilepsy and the factors influencing the effect.Methods Twenty five children with intractable epilepsy diagnosed in our hospital from March 2010 to October 2019 were selected.Among them,14 cases were West syndrome,2 cases were Lennox Gastaut syndrome,1 case was Dravet syndrome and 8 cases were others.KD was initiated by the modified Johns Hopkins program.The therapeutic effect was evaluated by Eagle scale.The compliance,adverse reactions,EEG and cognition changes of KD were observed and recorded.Results After 12 weeks KD treatment,the gradeⅠ,Ⅱ,ⅢandⅣcurative effects were 8%(2/25),12%(3/25),32%(8/25)and 36%(9/25),respectively,and 12%(3 cases)were withdrawn.KD had different curative effect on different epilepsy syndromes,one case of Dravet syndrome had no seizures after treatment,the effective rate of West syndrome was 32%,of which curative effect was good.The total effective rate was 12%at 12 weeks.However,during KD treatment,the curative effect fluctuated with the prolongation of the duration,and there were some cases who could not continue the treatment and quit.The main factors of withdrawal from treatment were intolerance,poor efficacy and inconvenient meal arrangement.At 48 weeks,the cognitive function was improved in 3 cases.A total of 18 patients had adverse reactions,which could be improved gradually with the continuous treatment.One case quit because of abnormal liver function,6 cases quit because of intolerance,9 cases quit because of poor curative effect.Conclusion KD is effective in the treatment of epileptic encephalopathy.The background rhythm of EEG is improved,and the frequency of epileptogenic discharge is decreased between seizures.KD can improve cognitive function.The adverse reactions of KD are mainly refusal to eat and constipation,which is also two of the main factors that affect the patients'adherence to KD treatment.

关 键 词：儿童 癫痫性脑病 生酮饮食 

分 类 号：R742.1[医药卫生—神经病学与精神病学]