Clinic of Child Neuropsychiatry, Second University of Naples, Via Pansini, 5 80131 Naples, Italy.
Eur J Paediatr Neurol. 2010 May;14(3):229-34. doi: 10.1016/j.ejpn.2009.06.006. Epub 2009 Jul 25.
The ketogenic diet for the treatment of refractory epileptic encephalopathies has been suggested as an early treatment option in very young children. The aim of the present study was to assess the efficacy and tolerability of the ketogenic diet in children younger than 5 years, all affected by different types of catastrophic childhood encephalopathies. The study group is composed of 38 children (22 males and 16 females), aged between 3 months and 5 years, affected by symptomatic partial epilepsy (6) and cryptogenic-symptomatic epileptic encephalopathies (32). Psychomotor delay-mental retardation was present in all of the patients: mild to moderate (9), severe (7), and profound (22). Cerebral palsy was present in 74% of the cases. Children were started on a 4:1 ketogenic diet as ketocal formula alone or supporting about the 80% of the daily caloric amount. Children poorly complying with ketocal milk were shifted to a classic 4:1 ketogenic diet. The average time (months +/- S.D.) on the diet was 10.3 +/- 7.4. All the children initiating the diet remained on it at 1 month and 35 of them (92%) at 3 months, 28 (73.7%) remained on it at 6 months, and 20 (52.7%) at 1 year. At 12-month follow-up, 11 children (28.9%) had a greater than 50% reduction of seizures and the other 9 (23.7%) were seizure-free. Adverse side effects were recorded in 25 of 38 patients (65.8%), including drowsiness, constipation, weight loss, vomiting, gastroesophageal reflux, fever, and hyperlipidemia. This report confirms that severe epileptic encephalopathies are much suitable for the ketogenic diet.
生酮饮食治疗难治性癫痫性脑病，已被建议作为非常年幼的儿童的早期治疗选择。本研究的目的是评估生酮饮食在 5 岁以下患有不同类型灾难性儿童脑病的儿童中的疗效和耐受性。研究组由 38 名儿童（22 名男性和 16 名女性）组成，年龄在 3 个月至 5 岁之间，患有症状性部分性癫痫（6 例）和隐源性症状性癫痫性脑病（32 例）。所有患者均存在精神运动发育迟缓-智力低下：轻度至中度（9 例）、重度（7 例）和重度（22 例）。脑瘫见于 74%的病例。儿童开始使用 4:1 的生酮饮食，即单独使用生酮配方或提供大约 80%的每日热量。对生酮奶接受不良的儿童改为经典的 4:1 生酮饮食。开始饮食的平均时间（月+/-S.D.）为 10.3+/-7.4。所有开始饮食的儿童在 1 个月时仍在饮食中，其中 35 名（92%）在 3 个月时，28 名（73.7%）在 6 个月时，20 名（52.7%）在 1 年时。在 12 个月的随访中，11 名儿童（28.9%）的癫痫发作减少了 50%以上，另外 9 名儿童（23.7%）无癫痫发作。在 38 名患者中，有 25 名（65.8%）出现了不良反应，包括嗜睡、便秘、体重减轻、呕吐、胃食管反流、发热和高血脂。本报告证实，严重的癫痫性脑病更适合生酮饮食。
