Treatment with vigabatrin may mimic alpha-aminoadipic aciduria

Epilepsia. 1996 Aug;37(8):803-5. doi: 10.1111/j.1528-1157.1996.tb00655.x.


Purpose: We describe a secondary effect of treatment with vigabatrin (VGB). A significant increase in alpha-aminoadipic acid (AAA) occurred in plasma and urine of VGB-treated children, thus mimicking a known rare metabolic disease, alpha-aminoadipic aciduria (AAAuria).

Methods: We studied eight children, aged from 3 months to 5 years, who were receiving VGB for drug-resistant partial epilepsies. Plasma and urine amino acids were assayed with ninhydrin detection on an automated Beckman 6300 analyzer.

Results: In eight out of eight children, there was a significant increase of AAA in plasma and in urine. Plasma values ranged from 7 to 8 microM (control values, < 5) and urinary values from 67 to 274 mmol/mol creatinine (control values, < 25).

Conclusions: The concentrations of AAA in these VGB-treated children were as high as the concentrations found in the inherited metabolic disease, AAAuria. This could lead to incorrect diagnosis and to inappropriate genetic counseling. Thus whenever a genetic metabolic disease is suspected, amino acid chromatography testing should be performed before initiation of treatment with VGB.

MeSH terms

  • 2-Aminoadipic Acid / urine*
  • 4-Aminobutyrate Transaminase / adverse effects
  • 4-Aminobutyrate Transaminase / antagonists & inhibitors
  • 4-Aminobutyrate Transaminase / pharmacology
  • 4-Aminobutyrate Transaminase / therapeutic use*
  • Amino Acid Metabolism, Inborn Errors / urine
  • Anticonvulsants / therapeutic use*
  • Child
  • Child, Preschool
  • Enzyme Inhibitors / adverse effects
  • Enzyme Inhibitors / pharmacology
  • Enzyme Inhibitors / therapeutic use
  • Epilepsies, Partial / drug therapy*
  • Epilepsies, Partial / urine
  • Female
  • Humans
  • Infant
  • Male
  • Vigabatrin
  • gamma-Aminobutyric Acid / adverse effects
  • gamma-Aminobutyric Acid / analogs & derivatives*
  • gamma-Aminobutyric Acid / pharmacology
  • gamma-Aminobutyric Acid / therapeutic use


  • Anticonvulsants
  • Enzyme Inhibitors
  • 2-Aminoadipic Acid
  • gamma-Aminobutyric Acid
  • 4-Aminobutyrate Transaminase
  • Vigabatrin