Propofol infusion syndrome or adrenoleukodystrophy?

Paediatr Anaesth. 2013 Apr;23(4):368-70. doi: 10.1111/pan.12113. Epub 2013 Jan 10.

Abstract

Following a propofol anesthetic, a 5-year-old girl with lower extremity spasticity seized and developed hypertriglyceridemia, hyperkalemia, and metabolic acidosis. A presumed diagnosis of propofol infusion syndrome (PRIS) was made, but further investigation revealed neonatal adrenoleukodystrophy. PRIS should be considered with this constellation of symptoms, but other neurometabolic disorders must always be ruled out.

Publication types

  • Case Reports

MeSH terms

  • Acidosis / complications
  • Anesthetics, Intravenous / adverse effects*
  • Blood Gas Analysis
  • Cerebral Palsy / complications
  • Child, Preschool
  • Female
  • Humans
  • Liver Function Tests
  • Magnetic Resonance Imaging
  • Mitochondrial Diseases / diagnosis*
  • Mitochondrial Diseases / therapy
  • Peroxisomal Disorders / diagnosis*
  • Peroxisomal Disorders / therapy
  • Propofol / adverse effects*

Substances

  • Anesthetics, Intravenous
  • Propofol