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Treatment with Vigabatrin May Mimic α‐Aminoadipic Aciduria
Author(s) -
Vallat C.,
Rivier F.,
Bellet H.,
Bornier B. Magnan,
Mion H.,
Echenne B.
Publication year - 1996
Publication title -
epilepsia
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 2.687
H-Index - 191
eISSN - 1528-1167
pISSN - 0013-9580
DOI - 10.1111/j.1528-1157.1996.tb00655.x
Subject(s) - vigabatrin , urine , ninhydrin , creatinine , medicine , urinary system , endocrinology , amino acid , anticonvulsant , chemistry , epilepsy , biochemistry , psychiatry
Summary:Purpose : We describe a secondary effect of treatment with vigabatrin (VGB). A significant increase in α‐aminoadipic acid (AAA) occurred in plasma and urine of VGB‐treated children, thus mimicking a known rare metabolic disease, α‐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 18 μ M (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.