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长期生酮饮食治疗耐药性癫痫:216 例儿科患者的阿根廷多中心经验。

Long-term follow-up of the ketogenic diet for refractory epilepsy: multicenter Argentinean experience in 216 pediatric patients.

机构信息

Hospital Nacional de Pediatría Prof Dr Juan P. Garrahan, Buenos Aires, Argentina.

出版信息

Seizure. 2011 Oct;20(8):640-5. doi: 10.1016/j.seizure.2011.06.009. Epub 2011 Jul 16.

Abstract

PURPOSE

In this Argentinean retrospective, collaborative, multicenter study, we examine the efficacy and tolerability of the ketogenic diet (KD) for different epilepsy syndromes.

MATERIALS AND METHODS

we evaluated the clinical records of 216 patients started on the KD between March 1, 1990 and December 31, 2010.

RESULTS

One hundred forty of the initial patients (65%) remained on the diet at the end of the study period. Twenty-nine patients (20.5%) became seizure free and 50 children (36%) had a 75-99% decrease in seizures. Thus, 56.5% of the patients had a seizure control of more than 75%. The best results were found in patients with epilepsy with myoclonic-astatic seizures, Lennox-Gastaut syndrome, and West syndrome. Good results were also found in patients with Dravet syndrome, in those with symptomatic focal epilepsy secondary to malformations of cortical development, and in patients with tuberous sclerosis. Seizures were significantly reduced in four patients with fever-induced refractory epileptic encephalopathy in school-age children and in two patients with epileptic encephalopathy with continuous spikes and waves during slow sleep. The median period of follow-up after discontinuation of the diet was 6 years. Twenty patients who had become seizure free discontinued the diet, but seizures recurred in five (25%). Of 40 patients with a seizure reduction of more than 50% who discontinued the diet, 10 presented with recurrent seizures.

CONCLUSION

The ketogenic diet is a good option in the treatment of refractory epilepsy. After discontinuing the diet, seizures recurrence occurred in few patients.

摘要

目的

在这项阿根廷回顾性、合作性多中心研究中,我们研究了生酮饮食(KD)对不同癫痫综合征的疗效和耐受性。

材料和方法

我们评估了 1990 年 3 月 1 日至 2010 年 12 月 31 日期间开始接受 KD 的 216 名患者的临床记录。

结果

在研究结束时,最初的 140 名患者(65%)仍在接受饮食治疗。29 名患者(20.5%)无癫痫发作,50 名儿童(36%)癫痫发作减少了 75%-99%。因此,56.5%的患者癫痫发作得到了超过 75%的控制。最好的结果是在有肌阵挛-强直发作、 Lennox-Gastaut 综合征和 West 综合征的癫痫患者中发现的。在 Dravet 综合征患者、皮质发育畸形引起的症状性局灶性癫痫患者和结节性硬化症患者中也取得了良好的效果。在四名学龄期儿童发热性难治性癫痫性脑病和两名睡眠中持续棘慢波癫痫性脑病患者中,癫痫发作明显减少。停止饮食后的中位随访期为 6 年。20 名无癫痫发作的患者停止了饮食,但其中 5 名(25%)癫痫发作复发。在停止饮食后癫痫发作减少超过 50%的 40 名患者中,有 10 名出现了复发的癫痫发作。

结论

生酮饮食是治疗难治性癫痫的一种较好的选择。停止饮食后,少数患者出现癫痫发作复发。

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