A case series of the efficacy and tolerability of ketogenic diet in children with drug-resistant epilepsy due to neonatal hypoglycemia

Objective: The objective of this study was to investigate the effectiveness and tolerance of the ketogenic diet (KD) in children who have drug-resistant epilepsy (DRE) caused by neonatal hypoglycemia.

Methods: We conducted a retrospective analysis of the data from pediatric patients who were diagnosed with neonatal hypoglycemia-related DRE and initiated a KD at Xi'an Children's Hospital between May 2014 and March 2023.

Results: Nine patients were enrolled in this study. The mean age of seizure onset was 6.16 ± 3.97 months, with a mean age of dietary therapy initiation at 21.56 ± 10.94 months and a median diet duration of nine months (range: 3-39.6 months). All of the nine patients (seven males) had daily seizures, and the most common seizure type was epileptic spasms. Magnetic resonance imaging (MRI) of the brain in all nine patients revealed bilateral atrophy of the occipital-parietal cortex, with or without gliosis. Of the patients studied, 22.2 % were classified as definite KD responders. These individuals achieved seizure freedom after one month of starting KD treatment and maintained a seizure-free state for over 22 months. One patient (11.1 %) was a partial KD responder who had a > 50 % seizure reduction within three months from KD initiation and continued the diet therapy for more than one year. None of the patients stopped taking KD because they could not tolerate the majority of the adverse effects, which were mild. At the last follow-up, six patients (66.7 %) had stopped their diet therapy with lack of efficacy being the leading cause.

Conclusions: KD is a safe and tolerable alternative treatment in pediatric patients with DRE secondary to neonatal hypoglycaemia. Most of the time, KD has minimal efficacy; however, diet therapy may be quite helpful for some patients. Thus, for DRE associated with neonatal hypoglycemia, KD treatment is appropriate to consider.