Ictal ontogeny in Dravet syndrome

Se Hee Kim, Douglas R. Nordli, Anne T. Berg, Sookyong Koh, Linda Laux*

*Corresponding author for this work

Research output: Contribution to journalArticlepeer-review

11 Scopus citations

Abstract

Objective: To define seizure characteristics of Dravet syndrome (DS) with video-electroencephalographic (EEG) recording in different age groups. Methods: We reviewed 23 patients with 63 seizures in different age groups: group 1 (0-5. years old); group 2 (6-10. years old); and group 3 (11 or above). Results: We included 7, 11 and 5 patients in groups 1, 2, and 3 respectively. Younger children had seizures while awake = p = 0.005), provoked seizures = p = 0.05), focal seizure semiology = p = 0.02) and long seizure duration = p = 0.0004). Older children had seizures from sleep = p = 0.004), generalized seizure semiology = p = 0.01) and short seizure duration = p = 0.0007). A generalized ictal discharge was the most commonly observed EEG pattern (15/23, 65%), more frequently found in older children = p = 0.01). Ten patients (43%) had unclassified seizures or seizures with discordant EEG results. Postictal EEG suppression was found in 9 (39%). Conclusion: The phenotype of seizures and ictal EEG patterns in DS vary with age. Significance: These findings will enhance the recognition of DS in the adolescent population. The incidence of postictal EEG suppression seen in DS is significant because it is a possible biomarker for sudden unexpected death in epilepsy.

Original languageEnglish (US)
Pages (from-to)446-455
Number of pages10
JournalClinical Neurophysiology
Volume126
Issue number3
DOIs
StatePublished - Mar 1 2015

Keywords

  • Channelopathy
  • Epilepsy
  • Postictal generalized EEG suppression (PGES)
  • SCN1A
  • Sudden unexpected death in epilepsy (SUDEP)

ASJC Scopus subject areas

  • Sensory Systems
  • Neurology
  • Clinical Neurology
  • Physiology (medical)

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