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Neuropediatrics 1992; 23(5): 257-259
DOI: 10.1055/s-2008-1071353
Original article

© Georg Thieme Verlag KG Stuttgart · New York

Disturbance of GABA Metabolism in Pyridoxine-Dependent Seizures

G.  Kurlemann , R.  Ziegler , Marianne  Grüneberg , T.  Bömelburg , K.  Ullrich , D. G. Palm
  • University of Münster, Department of Pediatrics, Neuropediatrics, Albert-Schweitzer-Str. 33, D-4400 Münster, Germany
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Publikationsdatum:
19. März 2008 (online)

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Abstract

In an infant with typical pyridoxine-dependent seizures, CSF GABA level, was determined before treatment with pyridoxine. Before onset of treatment, level of GABA in CSF was highly lowered (16 pmol/ml), pyridoxine level in serum was within normal range. Immediately after application of 80 mg pyridoxine fits stopped and the EEG was without seizure activity. The data substantiate previous findings in brain tissue from a patient with pyridoxine dependent seizures. They are proof of a disturbed GABA metabolism in pyridoxine dependent seizures.

Key words

Pyridoxine-dependent seizures - GABA - CSF