Caraballo Roberto Horacio, Cersósimo Ricardo Oscar, Sakr Diego, Cresta Araceli, Escobal Nidia, Fejerman Natalio
Servicio de Neurología, Hospital de Pediatría Prof Dr Juan P Garrahan, Buenos Aires, Argentina.
Epilepsia. 2005 Sep;46(9):1539-44. doi: 10.1111/j.1528-1167.2005.05705.x.
The ketogenic diet (KD) has been used as a therapeutic alternative to antiepileptic drugs (AEDs) for refractory epilepsy. Severe myoclonic epilepsy in infants or Dravet syndrome (DS) is one of the most malignant epileptic syndromes. In this retrospective study, we evaluated the efficacy and tolerability of the KD in patients with diagnostic criteria of DS.
Between March 1, 1990, and August 31, 2004, 52 patients who met diagnostic criteria for DS were enrolled in a study at our department. Twenty of them were placed on the KD with the Hopkins protocol and followed up for a minimum of 1 year.
Three of the 20 original children stayed on the diet for 12 months, four children for 2 years, four children for 3 years, and two children for 4 years. One year after initiating the diet, 13 (65%) of the initial patients remained on the diet. Two (15%) patients were seizure free, eight (61.7%) children had a 75-99% decrease in seizures, and the remaining three (23%) children had a 50-74% decrease in seizures. Thus 1 year after starting the diet, 10 (77%) children had achieved a >75% decrease in their seizures. Four patients have been off the diet for >2 years; one of them is seizure free, two have sporadic seizures, and one, who abandoned the diet after 2 years of adhering to it, relapsed. No differences in seizure control when compared with age, sex, or seizure type were found.
Considering the severity and intractability of seizures in patients with DS, the fact that 10 of the 13 children who remained on the diet had a significant reduction in number of seizures shows that the KD is at present an interesting therapeutic alternative. Even in patients in whom seizure reduction was not dramatic, quality of life improved, and in all of them, the number of AEDs was reduced to one or two. We consider that children with DS should be offered the KD immediately after three adequate trials of AEDs have failed.
生酮饮食（KD）已被用作难治性癫痫抗癫痫药物（AEDs）的一种治疗替代方法。婴儿严重肌阵挛癫痫或德雷维特综合征（DS）是最严重的癫痫综合征之一。在这项回顾性研究中，我们评估了KD对符合DS诊断标准患者的疗效和耐受性。
在1990年3月1日至2004年8月31日期间，52例符合DS诊断标准的患者在我们科室入组研究。其中20例采用霍普金斯方案进行生酮饮食，并随访至少1年。
最初的20名儿童中，3名坚持饮食12个月，4名儿童坚持2年，4名儿童坚持3年，2名儿童坚持4年。开始饮食1年后，13名（65%）初始患者仍在坚持饮食。2名（15%）患者无癫痫发作，8名（61.7%）儿童癫痫发作减少75% - 99%，其余3名（23%）儿童癫痫发作减少50% - 74%。因此，开始饮食1年后，10名（77%）儿童癫痫发作减少超过75%。4例患者已停止饮食超过2年；其中1例无癫痫发作，2例有散发性癫痫发作，1例在坚持饮食2年后放弃饮食，癫痫复发。在癫痫控制方面，未发现与年龄、性别或癫痫发作类型存在差异。
考虑到DS患者癫痫发作的严重性和难治性，13名坚持饮食的儿童中有10名癫痫发作次数显著减少，这一事实表明目前KD是一种有吸引力的治疗选择。即使在癫痫发作减少不显著的患者中，生活质量也有所改善，并且所有患者的AEDs数量都减少到了一两种。我们认为，在对AEDs进行三次充分试验失败后，应立即为DS儿童提供KD。
