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- Title
Monitoring of 4-hydroxybutyric acid levels in body fluids during vigabatrin treatment in succinic semialdehyde dehydrogenase deficiency.
- Authors
Katrin Ergezinger; Reinhard Jeschke; Georg Frauendienst-Egger; Herbert Korall; K. Michael Gibson; Volker H. Schuster
- Abstract
We report the successful treatment using low-dose vigabatrin (21.534mg/kg/day) of a 10-year-old girl with succinic semialdehyde dehydrogenase (SSADH) deficiency We verified that 4-hydroxybutyric acid (GHB) concentrations in serum, cerebrospinal fluid, and urine continuously decreased in parallel with significant clinical improvement. Our results suggest that GHB quantification in physiological fluids may be a useful laboratory parameter for monitoring efficacy of vigabatrin treatment in SSADH deficiency. Ann Neurol 2003;54:686689
- Publication
Annals of Neurology, 2003, Vol 54, Issue 5, p686
- ISSN
0364-5134
- Publication type
Article