Fatal propofol infusion syndrome in association with ketogenic diet

Neuropediatrics. 2004 Aug;35(4):250-2. doi: 10.1055/s-2004-820992.


Propofol is used for the treatment of refractory status epilepticus. When given as a 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. Propofol infusion syndrome is caused by impaired fatty acid oxidation. Beside anticonvulsants the ketogenic diet, a high-fat, low-carbohydrate, adequate-protein diet, is an effective treatment for difficult-to-control seizures. We report a 10-year-old boy with catastrophic epilepsy, who developed fatal propofol infusion syndrome when a ketogenic diet was initiated. Substances like propofol which impair fatty acid oxidation may pose an increased risk if combined with ketogenic diet.

Publication types

  • Case Reports
  • Research Support, Non-U.S. Gov't

MeSH terms

  • Acidosis / etiology
  • Anticonvulsants / administration & dosage
  • Anticonvulsants / adverse effects*
  • Child
  • Diet Therapy / adverse effects
  • Fatal Outcome
  • Humans
  • Infusions, Parenteral
  • Male
  • Propofol / administration & dosage
  • Propofol / adverse effects*
  • Rhabdomyolysis / etiology
  • Status Epilepticus / diet therapy*
  • Status Epilepticus / drug therapy*
  • Syndrome
  • Tachycardia, Ventricular / etiology*


  • Anticonvulsants
  • Propofol