Hsieh Tzu-Yun, Su Ting-Yu, Hung Kai-Yin, Hsu Mei-Shin, Lin Ying-Jui, Kuo Hsuan-Chang, Hung Pi-Lien
Division of Pediatric Neurology, Department of Pediatrics at Kaohsiung Chang Gung Memorial Hospital and Chang Gung University College of Medicine, Kaohsiung, Taiwan; Pediatric Neurologic Rare Disease Center, Kaohsiung Chang Gung Memorial Hospital, Kaohsiung, Taiwan.
Department of Nutritional Therapy at Kaohsiung Chang Gung Memorial Hospital and Chang Gung University College of Medicine, Kaohsiung, Taiwan.
Epilepsy Behav. 2023 Sep;146:109315. doi: 10.1016/j.yebeh.2023.109315. Epub 2023 Aug 5.
Ketogenic diet Therapy (KDT) has been reported as a possible beneficial management strategy for controlling seizures in infants aged <2 years, but the safety and efficacy of this therapy remain to be investigated. We investigated the achievability, tolerability, efficacy, and safety of KDT for patients under 2 years old.
Infants younger than 2 years old with pharmacoresistant epilepsy were enrolled in this prospective study. We divided cases into three age groups: I) neonates; II) infants aged 1-12 months; III) infants aged 12-24 months. KDT initiation protocol were administration through parenteral route, enteral route or oral feeding. Seizure reduction rate, physical growth, and adverse effects were assessed at monthly visit.
Thirteen patients who completed 6 months of KDT were recruited. There was one neonate in group I, 9 infants in group II, and 3 infants in group III. Eleven of them (11/13, 84.6%) were responders to KDT. All infants with underlying genetic etiology were seizure free after treating with KDT. The starting keto ratio was 1.1 mmol/L in group I, 2.3 mmol/L in group II, and 2.8 mmol/L in group III, which gradually approached 3:1-4:1 over 5-7 days. There were no symptomatic adverse effects or growth retardation in any of the study subjects.
KDT is a promising alternative therapy with high feasibility, safety, and efficacy for pharmacoresistant epilepsy in infants under 2 years old, especially for those with genetic etiology. The starting keto ratio should be lower, and the keto ratio titration period should be longer than for children older than 2 years.
生酮饮食疗法(KDT)已被报道为控制2岁以下婴儿癫痫发作的一种可能有益的管理策略,但该疗法的安全性和有效性仍有待研究。我们调查了KDT对2岁以下患者的可行性、耐受性、疗效和安全性。
本前瞻性研究纳入了2岁以下药物难治性癫痫婴儿。我们将病例分为三个年龄组:I)新生儿;II)1至12个月的婴儿;III)12至24个月的婴儿。KDT起始方案通过肠外途径、肠内途径或口服给药。每月随访时评估癫痫发作减少率、身体生长情况和不良反应。
招募了13名完成6个月KDT的患者。I组有1名新生儿,II组有9名婴儿,III组有3名婴儿。其中11名(11/13,84.6%)对KDT有反应。所有具有潜在遗传病因的婴儿在接受KDT治疗后均无癫痫发作。I组起始酮比为1.1 mmol/L,II组为2.3 mmol/L,III组为2.8 mmol/L,在5至7天内逐渐接近3:1至4:1。所有研究对象均未出现有症状的不良反应或生长发育迟缓。
KDT是一种有前景的替代疗法,对2岁以下药物难治性癫痫婴儿具有高可行性、安全性和有效性,尤其是对那些具有遗传病因的婴儿。起始酮比应更低,且酮比滴定期应比2岁以上儿童更长。