title: Treatment with vigabatrin may mimic alpha-aminoadipic aciduria.
abstract: OBJECTIVE
  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.
authors.affiliation: Laboratoire de Biochimie B, Hôpital Saint-Eloi, CHU de Montpellier, Montpellier, France.
authors.forename: C
authors.initials: C
authors.lastname: Vallat
authors.forename: F
authors.initials: F
authors.lastname: Rivier
authors.forename: H
authors.initials: H
authors.lastname: Bellet
authors.forename: B
authors.initials: B
authors.lastname: Magnan de Bornier
authors.forename: H
authors.initials: H
authors.lastname: Mion
authors.forename: B
authors.initials: B
authors.lastname: Echenne
mesh_terms: D015074:2-Aminoadipic Acid / Q000652:urine*; D000612:4-Aminobutyrate Transaminase / Q000009:adverse effects / Q000037:antagonists & inhibitors / Q000494:pharmacology / Q000627:therapeutic use*; D000592:Amino Acid Metabolism, Inborn Errors / Q000652:urine; D000927:Anticonvulsants / Q000627:therapeutic use*; D002648:Child; D002675:Child, Preschool; D004791:Enzyme Inhibitors / Q000009:adverse effects / Q000494:pharmacology / Q000627:therapeutic use; D004828:Epilepsies, Partial / Q000188:drug therapy* / Q000652:urine; D005260:Female; D006801:Humans; D007223:Infant; D008297:Male; D020888:Vigabatrin; D005680:gamma-Aminobutyric Acid / Q000009:adverse effects / Q000031:analogs & derivatives* / Q000494:pharmacology / Q000627:therapeutic use
chemical_list: D000927:Anticonvulsants; D004791:Enzyme Inhibitors; D015074:2-Aminoadipic Acid; D005680:gamma-Aminobutyric Acid; D000612:4-Aminobutyrate Transaminase; D020888:Vigabatrin