EEG features of Glut-1 deficiency syndrome
2002 | journal article. A publication with affiliation to the University of Göttingen.
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- Authors
- von Moers, A.; Brockmann, Knut; Wang, D.; Korenke, C. G.; Huppke, Peter; De Vivo, D. C.; Hanefeld, Folker
- 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.
- Issue Date
- 2002
- Status
- published
- Publisher
- Blackwell Publishing Inc
- Journal
- Epilepsia
- ISSN
- 0013-9580