Caraballo R, Trípoli J, Escobal L, Cersósimo R, Tenembaum S, Palacios C, Fejerman N
Servicio de Neurología, Hospital de Pediatría Juan P. Garraham, Buenos Aires, Argentina.
Rev Neurol. 1998 Jan;26(149):61-4.
Prospective study to evaluate efficacy of ketogenic diet (KD) in the treatment of children with intractable epilepsies (IE). Tolerability of the KD was also considered.
Criteria for inclusion were: 1. Epilepsy refractary to treatments with antiepileptic drugs (AED) in monotherapy and combining two or three AED. 2. Acknowledgment of blood levels of these drugs in therapeutic range. 3. Absence of liver or kidney disease, metabolic abnormalities, inborn errors of metabolism or other progressive encephalopathies. 4. Family supposedly in economic and psychologic conditions to accept the difficulties of strictly maintaining KD. We used a classic KD following the criteria suggested by the John Hopkins Pediatrics Epilepsy Center. Baseline neurological and physical examination, EEG, blood chemistry including lipid profile were obtained prior to initiative and during the KD. KD efficacy was measured as percent reduction of baseline seizures frequency, considering positive results as reductions of 50% or over. Acceptance of the diet and quality of life were specially considered. Eighteen patients with ages from 2 to 11 years were admitted. Ten of them were males. Diagnosis followed the last Classification of Epileptic Syndromes of the ILAE, and distribution was: Symptomatic partial epilepsies, 8 cases (one had West syndrome at age 5 months); cryptogenic partial epilepsies, 1 case; Lennox-Gastaut syndrome, 2 cases; severe myoclonic epilepsy of infancy, 6 cases and epilepsy with myoclonic astatic seizures 1 case.
Four patients were not able to achieve persistent ketosis either due to patient's rejection of KD or to parents non compliance. KD was kept for at least two months before considering failures. Five patients did not show significant improvement and KD was stopped. At present nine patients have been on KD from 6 to 24 months (average 16 months). Four of them showed a 75-100% reduction in seizures frequency and in three the reduction was of 50-75%.
KD was fairly well tolerated by 14 of 18 children and their families. Fifty percent of the 14 patients complying KD showed significant improvement in seizure frequency and in quality of life. Due to the small number of patients and short follow-up, we can not speculate about results in relation to each epileptic syndrome, neither the risk of late complications.
一项前瞻性研究,旨在评估生酮饮食(KD)治疗儿童难治性癫痫(IE)的疗效。同时也考虑了KD的耐受性。
纳入标准为:1. 对抗癫痫药物(AED)单药治疗及两药或三药联合治疗无效的癫痫。2. 确认这些药物的血药浓度在治疗范围内。3. 无肝脏或肾脏疾病、代谢异常、先天性代谢缺陷或其他进行性脑病。4. 家庭在经济和心理状况上应能接受严格维持KD带来的困难。我们采用了约翰霍普金斯儿科癫痫中心建议的经典KD方案。在开始KD之前及期间,进行了基线神经和体格检查、脑电图检查以及包括血脂谱在内的血液化学检查。KD的疗效通过基线癫痫发作频率降低的百分比来衡量,将降低50%或以上视为阳性结果。特别考虑了对饮食的接受程度和生活质量。18名年龄在2至11岁之间的患者入院。其中10名是男性。诊断遵循国际抗癫痫联盟(ILAE)最新的癫痫综合征分类,分布情况为:症状性部分性癫痫8例(其中1例在5个月大时患有韦斯特综合征);隐源性部分性癫痫1例;伦诺克斯 - 加斯东综合征2例;婴儿严重肌阵挛癫痫6例;伴有肌阵挛 - 失张力发作的癫痫1例。
4例患者由于患者拒绝KD或家长不依从,未能实现持续酮症。在考虑失败之前,KD至少维持了两个月。5例患者未显示出明显改善,KD被停用。目前有9例患者接受KD治疗6至24个月(平均16个月)。其中4例癫痫发作频率降低了75% - 100%,3例降低了50% - 75%。
18名儿童及其家庭中有14名对KD耐受性良好。在坚持KD治疗的14名患者中,50%的患者癫痫发作频率和生活质量有显著改善。由于患者数量少且随访时间短,我们无法推测与每种癫痫综合征相关的结果,也无法推测晚期并发症的风险。