Ketogenic diet treatment for pediatric super-refractory status epilepticus
Ketogenic diet treatment for pediatric super-refractory status epilepticus
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DOI：
10.1016/j.seizure.2016.07.006
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发表时间：
2016-10-01
影响因子：
3
通讯作者：
Jarrar, Randa
中科院分区：
文献类型：
--
作者：
Appavu, Brian;Vanatta, Lisa;Jarrar, Randa
目的：我们的目的是研究生酮饮食（KID）治疗是否能在不造成显著伤害的情况下解决儿科患者的超难治性癫痫持续状态。方法：对2011年至2015年在凤凰城儿童医院接受生酮饮食治疗的超难治性癫痫持续状态患者进行回顾性分析。结果：确定了10名2-16岁的超难治性癫痫持续状态儿童。4/10例患者有免疫介导的脑炎，包括Rasmussen脑炎、抗N-甲基-N-天冬氨酸受体脑炎和感染后支原体脑炎。其他病因包括伦诺克斯加斯托综合征、非酮症高甘氨酸血症、PCDH 19和GABRG 2遗传性癫痫、新发难治性癫痫持续状态和发热性惊厥相关癫痫综合征。4/10例EEG表现为局灶性伴癫痫持续状态，6/10例表现为全身性伴癫痫持续状态。平均住院时间为61天，平均ICU时间为27天。饮食开始前抗癫痫药物的中位数为3.0药物，生酮饮食治疗后的中位数为3.5药物。RD前癫痫持续状态的中位持续时间为18天。9/10例患者在饮食开始后中位7天内超难治性癫痫持续状态消退。8/9例患者在饮食开始后15天内和达到酮尿后1天内脱离麻醉。1/10的患者因需要补充饮食而出现副作用。结论：大多数患者通过RD治疗获得了癫痫持续状态的缓解，这表明它可能是一种有效的治疗方法，可以早期用于治疗患有超难治性癫痫持续状态的儿童。(C)2016由Elsevier Ltd代表英国癫痫协会出版。
Purpose: We aimed to study whether ketogenic diet (KID) therapy leads to resolution of super-refractory status epilepticus in pediatric patients without significant harm.Method: A retrospective review was performed at Phoenix Children's Hospital on patients with super refractory status epilepticus undergoing ketogenic diet therapy from 2011 to 2015.Results: Ten children with super-refractory status epilepticus, ages 2-16 years, were identified. 4/10 patients had immune mediated encephalitis, including Rasmussen encephalitis, anti-N-methyl-n-aspartate receptor encephalitis, and post-infectious mycoplasma encephalitis. Other etiologies included Lennox Gastaut Syndrome, non-ketotic hyperglycinemia, PCDH19 and GABRG2 genetic epilepsy, New Onset Refractory Status Epilepticus, and Febrile Infection-Related Epilepsy Syndrome. 4/10 patients' EEG features suggested focal with status epilepticus, and 6/10 suggested generalized with status epilepticus. Median hospital length was 61 days and median ICU length was 27 days. The median number of antiepileptic medications prior to diet initiation was 3.0 drugs, and the median after ketogenic diet treatment was 3.5 drugs. Median duration of status epilepticus prior to RD was 18 days. 9/10 patients had resolution of super-refractory status epilepticus in a median of 7 days after diet initiation. 8/9 patients were weaned off anesthesia within 15 days of diet initiation, and within 1 day of achieving ketonuria. 1/10 patients experienced side effects on the diet requiring supplementation.Conclusion: Most patients achieved resolution of status epilepticus on RD therapy, suggesting it could be an effective therapy that can be utilized early in the treatment of children with super refractory status epilepticus. (C) 2016 Published by Elsevier Ltd on behalf of British Epilepsy Association.