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Treatment with vigabatrin may mimic alpha-aminoadipic aciduria
1Laboratoire de Biochimie B, Hôpital Saint-Eloi, CHU de Montpellier, Montpellier, France.
Epilepsia
|August 1, 1996
Summary
Vigabatrin (VGB) treatment in children can increase alpha-aminoadipic acid (AAA) levels, mimicking a rare metabolic disorder. This finding necessitates amino acid testing before VGB initiation to prevent misdiagnosis.
Area of Science:
- Biochemistry
- Pediatric Neurology
- Clinical Chemistry
Background:
- Vigabatrin (VGB) is an antiepileptic drug used for specific seizure types.
- Secondary metabolic effects of VGB are not fully understood.
- Alpha-aminoadipic aciduria (AAAuria) is a rare inherited metabolic disorder.
Purpose of the Study:
- To report a secondary effect of vigabatrin (VGB) treatment in children.
- To highlight the significant increase in alpha-aminoadipic acid (AAA) observed in VGB-treated children.
- To note the potential for VGB-induced AAA to mimic alpha-aminoadipic aciduria (AAAuria).
Main Methods:
- Studied eight children (3 months to 5 years) with drug-resistant partial epilepsies receiving VGB.
- Assayed plasma and urine amino acid levels using automated Beckman 6300 analyzer with ninhydrin detection.
Main Results:
- All eight children showed a significant increase in plasma and urine AAA.
- Elevated plasma AAA levels ranged from 7 to 8 microM (controls < 5 microM).
- Elevated urinary AAA levels ranged from 67 to 274 mmol/mol creatinine (controls < 25 mmol/mol creatinine).
Conclusions:
- VGB-induced AAA concentrations are comparable to those in inherited AAAuria.
- This biochemical change can lead to diagnostic errors and incorrect genetic counseling.
- Recommend amino acid chromatography testing before VGB initiation when metabolic disease is suspected.