Appavu Brian, Vanatta Lisa, Condie John, Kerrigan John F, Jarrar Randa
Barrow Neurological Institute at Phoenix Children's Hospital, 1919 E. Thomas Road, Ambulatory Building, 3rd Floor, Phoenix, AZ 85016, United States.
Seizure. 2016 Oct;41:62-5. doi: 10.1016/j.seizure.2016.07.006. Epub 2016 Jul 21.
We aimed to study whether ketogenic diet (KD) therapy leads to resolution of super-refractory status epilepticus in pediatric patients without significant harm.
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.
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-d-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 61days and median ICU length was 27days. 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 KD was 18days. 9/10 patients had resolution of super-refractory status epilepticus in a median of 7days after diet initiation. 8/9 patients were weaned off anesthesia within 15days of diet initiation, and within 1day of achieving ketonuria. 1/10 patients experienced side effects on the diet requiring supplementation.
Most patients achieved resolution of status epilepticus on KD therapy, suggesting it could be an effective therapy that can be utilized early in the treatment of children with super refractory status epilepticus.
我们旨在研究生酮饮食（KD）疗法是否能使小儿患者的超难治性癫痫持续状态得到缓解且无明显危害。
对2011年至2015年在凤凰城儿童医院接受生酮饮食疗法的超难治性癫痫持续状态患者进行回顾性研究。
确定了10例年龄在2至16岁的超难治性癫痫持续状态患儿。10例患者中有4例患有免疫介导性脑炎，包括拉斯穆森脑炎、抗N-甲基-D-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综合征、非酮症高甘氨酸血症、PCDH19和GABRG2基因癫痫、新发难治性癫痫持续状态以及发热感染相关癫痫综合征。10例患者中有4例的脑电图特征提示局灶性癫痫持续状态，6例提示全身性癫痫持续状态。中位住院时间为61天，中位重症监护病房时间为27天。开始饮食前抗癫痫药物的中位数量为3.0种，生酮饮食治疗后的中位数量为3.5种。KD治疗前癫痫持续状态的中位持续时间为18天。10例患者中有9例在开始饮食后的中位7天内超难治性癫痫持续状态得到缓解。9例患者中有8例在开始饮食后的15天内以及出现酮尿症后的1天内停用了麻醉药。10例患者中有1例在饮食上出现需要补充的副作用。
大多数患者通过KD疗法使癫痫持续状态得到缓解，这表明它可能是一种有效的疗法，可在超难治性癫痫持续状态患儿的治疗早期使用。
