Efficacy and safety of ketogenic dietary theraphies in infancy. A single-center experience in 42 infants less than two years of age

• Published in: Seizure (London, England) England), 2021-11, Vol.92, p.106-111
• Main Authors: Ruiz-Herrero, Jana, Cañedo-Villarroya, Elvira, Pérez-Sebastián, Isabel, Bernardino-Cuesta, Beatriz, Pedrón-Giner, Consuelo
• Format: Article
• Language: English
• Subjects: Classic ketogenic diet; Drug resistant epilepsy; Epilepsy; Infancy; Ketogenic dietary therapies
• ISSN: 1059-1311; 1532-2688
• DOI: 10.1016/j.seizure.2021.08.018

•The onset of epilepsy in infancy is associated with intractability and mortality.•Ketogenic dietary therapies are effective and safe treatments for infants.•Classic ketogenic diet is the most accepted diet in infants.•Modified Atkins Diet is useful in older infants with compliance difficulties.•Most frequent side effects are mild and easy to treat. Ketogenic dietary therapies (KDT) are high-fat and low-carbohydrate diets that may achieve seizure control and improve cognitive state. We describe our KDT experience in infants (children less than two years of age). We conducted a retrospective, descriptive and observational study of 42 infants treated with KDT between 2000-2018. The types of KDT started were: classic ketogenic diet ratio 3:1 (40), ratio 4:1 (1) and modified ketogenic diet with medium-chain triglycerides (1). Four patients switched to a modified Atkins diet. During follow-up, 79%, 57%, 38% and 17% of infants remained on KDT at 3, 6, 12 and 24 months, respectively. Seizure reduction ≥50% compared to baseline was achieved in 50%, 45%, 38% and 17% at 3, 6, 12 and 24 months, respectively. Seizure control was excellent (reduction >90%) in 33%, 31%, 26% and 12%, and seizure-free infants were 9, 9, 10 and 4, at different follow-up intervals, respectively. Sixty-three percent of infants with West syndrome were responders to KDT. Mean length of KDT was 390 days (16 days-4.9 years). Ineffectiveness was the reason for withdrawal in 50% of patients. Early adverse effects (during first month) occurred in 40% of infants. The most frequent early side effects were asymptomatic hypoglycemia and gastrointestinal disturbances. Late-onset side effects occurred in 55-14% of infants during therapy, and most frequent were hypercalciuria and dyslipidaemia. KDT are useful and effective treatments in infancy. Side effects are frequent but mild and easy to manage.