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酮饮食在继发于皮质发育障碍的难治性癫痫儿童中的应用:两中心经验。

Ketogenic diet use in children with intractable epilepsy secondary to malformations of cortical development: A two- centre experience.

机构信息

Department of Child Neurology and Psychiatry, IRCCS "C. Mondino" National Neurological Institute, Pavia, Italy.

Department of Neurology, Juan P Garrahan Pediatric Hospital, Buenos Aires, Argentina.

出版信息

Seizure. 2018 Apr;57:34-37. doi: 10.1016/j.seizure.2018.03.005. Epub 2018 Mar 8.

Abstract

PURPOSE

To evaluate the efficacy and tolerability of the ketogenic diet (KD) as a treatment for drug-resistant epilepsy secondary to malformations of cortical development.

METHODS

A two-centre retrospective analysis of 45 paediatric patients with refractory epilepsy due to malformation of cortical development was carried out. Patients were divided into three groups based on malformation type: abnormal neural proliferation (Group 1); abnormal neural migration (Group 2) and abnormal post-migrational development (Group 3). The efficacy of the KD was assessed in terms of seizure frequency reduction. We identified the proportion of patients achieving > 50% seizure frequency reduction overall and in the three subgroups.

RESULTS

The adherence to KD was variable. KD was pursued from a minimum of 4 months to a maximum of 96 months. 20 patients (44%) obtained a seizure reduction of > 50% and 2 patients became seizure free. >50% seizure reduction was most commonly achieved by patients in group 3 (64.7%) than in groups 2 (31.8%) and 1 (33.3%).

CONCLUSIONS

The best response was observed in patients with malformations of post migrational development. Considering its tolerability, the use of KD should be considered in patients with drug-resistant epilepsy secondary to malformations of cortical development when surgery is not a viable option.

摘要

目的

评估生酮饮食(KD)治疗皮质发育障碍相关耐药性癫痫的疗效和耐受性。

方法

对 45 例皮质发育障碍相关难治性癫痫患儿进行了为期 2 年的回顾性分析。根据畸形类型将患者分为三组:异常神经增殖(第 1 组);异常神经迁移(第 2 组)和异常迁移后发育(第 3 组)。根据发作频率减少的情况评估 KD 的疗效。我们确定了总体和三个亚组中达到>50%发作频率减少的患者比例。

结果

KD 的依从性各不相同。KD 的治疗时间从最短 4 个月到最长 96 个月不等。20 名患者(44%)发作减少>50%,2 名患者无发作。3 组患者(64.7%)较 2 组(31.8%)和 1 组(33.3%)更常达到>50%的发作减少。

结论

在迁移后发育畸形患者中观察到最佳反应。鉴于其耐受性,当手术不是可行选择时,对于皮质发育障碍相关耐药性癫痫患者,应考虑使用 KD。

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