van der Louw Elles J T M, Williams Tanya J, Henry-Barron Bobbie J, Olieman Joanne F, Duvekot Johannes J, Vermeulen Marijn J, Bannink Natalja, Williams Monique, Neuteboom Rinze F, Kossoff Eric H, Catsman-Berrevoets Coriene E, Cervenka Mackenzie C
Department of Dietetics, Erasmus MC, Rotterdam, The Netherlands.
Department of Neurology, Johns Hopkins University School of Medicine, 600 North Wolfe Street, Meyer 2-147, Baltimore, MD, USA.
Seizure. 2017 Feb;45:198-201. doi: 10.1016/j.seizure.2016.12.019. Epub 2016 Dec 26.
Evaluation of ketogenic diet (KD) therapies for seizure control during pregnancy when safety and appropriate management become considerations. Until now, no information has been available on seizure reduction and human pregnancy related outcomes in women treated with KD therapies.
We describe two cases of pregnant women with epilepsy treated with KD therapy either as monotherapy (Case 1) or as adjunctive therapy (Case 2).
Case 1: A 27 year old woman, gravida1, started the classic KD with medium chain triglyceride (MCT) emulsion and 75g carbohydrate-restriction, later reduced to 47g. Glucose levels were 4-6mmol/L and blood ketone levels ranged from 0.2 to 1.4mmol/L. Seizure frequency decreased and seizure-free days increased. Mild side effects included intolerance to MCT, reduced serum carnitine and vitamin levels, and mild hyperlipidemia. Fetal and neonatal growth was normal as was growth and development at 12 months. Case 2: A 36 year-old nulliparous woman was treated with a 20 gram carbohydrate-restricted Modified Atkins Diet (MAD) and lamotrigine, resulting in reduction of seizure frequency to once per month prior to pregnancy. Once pregnant, carbohydrates were increased to 30g. When seizures increased, lamotrigine dose was doubled. Urine ketones trended down during second trimester. A male was born with bilateral ear deformities of unknown significance. The child had a normal neurodevelopment at eight months.
Non-pharmacological epilepsy therapies like KD and MAD may be effective during human pregnancy. However, safety still has to be established. Further monitoring to identify potential long term side effects is warranted.
评估生酮饮食（KD）疗法在孕期控制癫痫发作的效果，此时安全性和恰当管理成为需要考虑的因素。到目前为止，尚无关于接受KD疗法治疗的女性癫痫发作减少情况及与人类妊娠相关结局的信息。
我们描述了两例接受KD疗法治疗的癫痫孕妇病例，其中一例为单药治疗（病例1），另一例为辅助治疗（病例2）。
病例1：一名27岁初产妇，开始采用含中链甘油三酯（MCT）乳剂的经典KD疗法，碳水化合物限制量为75克，后减至47克。血糖水平为4 - 6毫摩尔/升，血酮水平在0.2至1.4毫摩尔/升之间。癫痫发作频率降低，无癫痫发作天数增加。轻微副作用包括对MCT不耐受、血清肉碱和维生素水平降低以及轻度高脂血症。胎儿和新生儿生长正常，12个月时的生长发育也正常。病例2：一名36岁未生育女性采用碳水化合物限制量为20克的改良阿特金斯饮食（MAD）并联合拉莫三嗪治疗，在怀孕前癫痫发作频率降至每月一次。怀孕后，碳水化合物增加至30克。癫痫发作增加时，拉莫三嗪剂量加倍。孕中期尿酮水平呈下降趋势。一名男性出生时双耳畸形，意义不明。该儿童8个月时神经发育正常。
KD和MAD等非药物性癫痫疗法在人类孕期可能有效。然而，安全性仍有待确定。有必要进行进一步监测以识别潜在的长期副作用。
