Fatal propofol infusion syndrome in association with ketogenic diet in a child with catastrophic epilepsy

Introduction: Beside anticonvulsants the ketogenic diet (KD), a high-fat, low-carbohydrate, adequate-protein diet, is an effective treatment for difficult-to-control seizures. For KD a fully competent fatty-acid oxidation is an essential prerequisite.

Propofol is used for the treatment of refractory status epilepticus. When given as long-term infusion propofol may cause a rare but frequently fatal complication, the propofol infusion syndrome. The hallmarks are metabolic acidosis, lipemia, rhabdomyolysis and myocardial failure. Recently it has been demonstrated, that propofol infusion syndrome is caused by impaired fatty-acid oxidation which is induced by propofol itself.

Case Report: A 10-year old boy with catastrophic epilepsy is reported. The course was malign with repeated difficult to control status epilepticus. He developed fatal propofol infusion syndrome while treated with ketogenic diet.

Conclusion: Severe complications in association with KD are rare. For KD appropriate candidates or additional medications must be selected carefully. Propofol can impair fatty-acid oxidation and should therefore not be used in combination with KD.

Keywords: propofol infusion syndrome, ketogenic diet