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Treatment with vigabatrin may mimic alpha-aminoadipic aciduria
1Laboratoire de Biochimie B, Hôpital Saint-Eloi, CHU de Montpellier, Montpellier, France.
Insights
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.
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.