门诊行生酮饮食疗法治疗儿童难治性癫痫的病例研究

Clinic management of the ketogenic diet on children with intractable epilepsy

目的探讨生酮饮食(ketogenic diet,KD)疗法在门诊实施的安全性和有效性,同时分析影响KD治疗12个月疗效的因素。方法对2013年6月至2014年12月于大连医科大学附属第二医院辽宁省癫痫疾病诊疗中心门诊采用KD疗法的10例难治性癫痫患儿进行为期至少1年的观察随访。记录患儿行KD疗法前后的发作频率、不良反应、身高及体重;评估性别、起病年龄、开展KD时年龄、开展KD前病程、癫痫诊断类型、KD开始时用抗癫痫药种类、癫痫相关家族史等对KD 12个月疗效的影响;分析KD 3个月疗效与12个月疗效间的关系。观察KD治疗3个月及12个月前后身高、体重的变化。结果 KD启动后1、3、6、12个月分别有100%、100%、80%、70%患儿继续坚持治疗,其中有50%、60%、60%、50%患儿发作减少50%以上,2例患儿分别在2个月、3个月时达到完全缓解。KD治疗3个月的有效率与12个月的有效率呈高度正相关(r=0.816,P=0.048)。此外,本研究未发现与KD治疗12个月疗效有关的影响因素。KD不良反应主要为轻微的胃肠道不适,对症处理可缓解;KD治疗3个月及12个月身高z分数(Height z-score,HAz)均较治疗前减小(P<0.05),体重z分数(Weight z-score,WAz)无变化。结论在门诊行KD疗法治疗儿童难治性癫痫具有一定的疗效,且较为安全。KD治疗3个月时有效的患儿可能大部分在12个月时仍有效。

Objective To evaluate the safety and efficacy of ketogenic diet （KD）in the treatment of children with intractable epilepsy in outpatient clinic and to determine which factors are more likely related to the efficacy at 12month. Methods This was a prospective study of intractable epilepsy children with the KD in the Second Affiliated Hospital of Dalian Medical University from June 2013 to December 2014. A total of 10 patients were enrolled and followed up for at least a year. Outcome was measured by seizure frequencies before and after the diet, adverse effects, heightand weight. Variables（ gender,age at epilepsy onset, age at diet initiation, time elapsed between epilepsy onset and diet initiation starting age, seizure type,number of anti -epileptic drugs taken prior to KD and family history） and success of the KD at 3 month follow - up were evaluated in their relation to a successful treatment at 12 month after diet initiation. Results There were 100% ,100% ,80% ,70% patients remained on the diet respectively,and 50% ,60% ,60% ,50% had a ≥ 50% reduction in their seizure frequency at 1,3,6 and 12 months after initiation, including two patients became seizure free after initiation. No significant relationships between variables and efficacy at 12 month were revealed. We found that success of the KD at three month was related to a successful outcome at 12 month after diet initiation （r =0. 816, P = 0. 048 ）. Most complications were mild and reversible after conservative treatment, and gastro -intestinal disturbances were the main adverse reactions. HAz declined while WAz was unchanged. Conclusion The Clinic management of KD is probably a safe and effective therapy on children with intractable epilepsy. Children with a successful response at 3 month are more likely to achieve success at 12 month of KD treatment.