Ketogenic Diet in Childhood Pharmacoresistant Epilepsy

Aims: Some childhood epilepsy syndromes (e.g., idiopathic Lennox-Gastaut syndrome) show a distinct pharmacoresistance. In these cases, the ketogenic diet (KD) is a therapeutic option. In children with specific metabolic diseases (e.g., GLUT-1 deficiency) even KD is the first-line treatment and preferred therapy. The study aims to provide the efficacy and safety profile of KD in children with pharmacoresistant epilepsy.

Methods: A total of 31 children aged 1 to 19 years (15 F and 16 M) suffering from pharmacoresistant epilepsy (n = 25) or metabolic disease (n = 6) were included in the retrospective analysis. KD was initiated between 2000 and 2014.

Results: KD was applied after ineffective treatment with at least three antiepileptic drugs. Of 31 patients, 15 patients received eight or more antiepileptic drugs before KD was induced. Ketosis was mostly achieved after 3 days (range, 1–6 days). KD was generally well tolerated; adverse events such as hypoglycemia, acidosis, or gastrointestinal problems occurred only in 8 of 31 patients and no life-threatening metabolic conditions appeared. Of the 31 patients, 23 patients showed improvement in at least one of the categories (development, seizures, and EEG); 13 of 31 children, in 2 and a minority of 6 children improved in all categories. In 8 of 31 children, KD is at least able to stabilize the disease course. After initial improvement, 18 of 31 children deteriorate with increased seizure frequency and susceptibility in EEG, representing the main cause to finish KD after an average period of 159 days (range, 12–683 days). Overall, 13 children are still on KD for a mean period of 2.7 years (range, 0.4–4.5 years) of whom 3 children are diagnosed with Dravet syndrome and 2 children suffered from GLUT-1 deficiency.

Conclusion: On the basis of the results of our retrospective study, the KD is a safe treatment option for children with pharmacoresistant epilepsy. KD might be effective in children with Dravet syndrome. Further studies are warranted to estimate the efficacy of KD especially regarding its long-term effectiveness and toward new antiepileptic drugs.

Keywords: ketogenic diet, childhood pharmacoresistant epilepsy, efficacy, safety.