EEG features of glut-1 deficiency syndrome
- PMID: 12181017
- DOI: 10.1046/j.1528-1157.2002.50401.x
EEG features of glut-1 deficiency syndrome
Abstract
Purpose: Glut-1 deficiency syndrome (Glut-1 DS) is caused by the deficiency of the major glucose transporter in cerebral microvessels.
Methods: We performed pre- and postprandial EEG recordings in two unrelated children with Glut-1 DS with developmental delay and seizures predominantly in the morning before breakfast.
Results: Extensive epileptiform discharges observed in the fasting state were improved markedly by food intake, as documented in EEG recordings 1 and 2 h after a meal. The ratio of cerebrospinal fluid glucose to blood glucose was decreased in both children. Glut-1 deficiency was confirmed by biochemical and molecular genetic investigations.
Conclusions: Pre- and postprandial EEG recordings offer a simple screening test for Glut-1 DS.
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