Child and Adolescent Neurology and Epilepsy, Mayo Clinic, Rochester, Minnesota.
Mayo Clinic, Rochester, Minnesota.
Pediatr Neurol. 2018 May;82:13-18. doi: 10.1016/j.pediatrneurol.2017.10.018. Epub 2018 Jan 31.
This study evaluated tolerability and efficacy of the ketogenic diet in infants less than 12 months of age.
Infants less than 12 months of age, commencing the ketogenic diet between September 2007 and July 2016 were identified. Records were reviewed for epilepsy details, diet initiation details, efficacy and tolerability.
Twenty-seven infants commenced the ketogenic diet (56% male, median age seven months). Median age at seizure onset was 1.9 months and 92% had daily seizures. An epilepsy syndrome was noted in 19 (West-11, epilepsy in infancy with migrating focal seizures-5, early myoclonic encephalopathy-1, Ohtahara-1, Dravet-1). Infants were on a median of two and had failed a median of one medications for lack of efficacy. All initiated a traditional ketogenic diet at full calories without fasting, and all but one started the diet in hospital. Significant hypoglycemia during initiation was seen in two - both had emesis +/- decreased oral intake. Eighty-eight percent developed urinary ketosis by 48 hours and all were successfully discharged on the diet (median ratio 3:1). Of those continuing dietary therapy, responder rates at one, six and 12 months were 68%, 82% and 91%, with 20%, 29% and 27% achieving seizure freedom. By 12 months, two stopped the diet for serious adverse effects, five discontinued for lack of efficacy, six were lost to follow-up and two died of unrelated causes.
The ketogenic diet is an effective and well-tolerated treatment for infants with intractable epilepsy. In-hospital initiation is strongly recommended due to risk of hypoglycemia with emesis or reduced intake.
本研究评估了生酮饮食在 12 个月以下婴儿中的耐受性和疗效。
确定了 2007 年 9 月至 2016 年 7 月期间开始生酮饮食的 12 个月以下婴儿。回顾了记录以获取癫痫详细信息、饮食起始详细信息、疗效和耐受性。
27 名婴儿开始生酮饮食（56%为男性，中位数年龄为 7 个月）。发病中位年龄为 1.9 个月，92%的婴儿每天都有癫痫发作。19 名婴儿患有癫痫综合征（West-11、婴儿期癫痫伴移行性局灶性发作-5、早发性肌阵挛性脑病-1、Ohtahara-1、Dravet-1）。中位数使用了两种药物，因疗效不佳而中位数使用了一种药物。所有婴儿均按全热量起始传统生酮饮食，无需禁食，且除 1 例外均在医院起始饮食。2 例婴儿在起始时出现严重低血糖，均伴有呕吐 +/-减少口服摄入量。88%的婴儿在 48 小时内出现尿酮症，所有婴儿均成功出院（中位数比例为 3:1）。继续进行饮食治疗的婴儿中，1、6、12 个月时的应答率分别为 68%、82%和 91%，20%、29%和 27%的婴儿达到无癫痫发作。12 个月时，2 名婴儿因严重不良反应停止饮食，5 名婴儿因疗效不佳停止饮食，6 名婴儿失访，2 名婴儿因其他原因死亡。
生酮饮食是治疗难治性癫痫婴儿的有效且耐受性良好的治疗方法。由于呕吐或摄入量减少可能导致低血糖，强烈建议在医院起始饮食。
