title: Ictal ontogeny in Dravet syndrome.
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 (0-5years old); group 2 (6-10years 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%).
  
  
  CONCLUSIONS
  The phenotype of seizures and ictal EEG patterns in DS vary with age.
  
  
  CONCLUSIONS
  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.
authors.affiliation: Epilepsy Center, Department of Pediatrics, Ann & Robert H. Lurie Children's Hospital of Chicago, Northwestern University Feinberg School of Medicine, Chicago, Illinois, USA.
authors.forename: Se Hee
authors.initials: SH
authors.lastname: Kim
authors.affiliation: Epilepsy Center, Department of Pediatrics, Ann & Robert H. Lurie Children's Hospital of Chicago, Northwestern University Feinberg School of Medicine, Chicago, Illinois, USA.
authors.forename: Douglas R
authors.initials: DR
authors.lastname: Nordli
authors.affiliation: Epilepsy Center, Department of Pediatrics, Ann & Robert H. Lurie Children's Hospital of Chicago, Northwestern University Feinberg School of Medicine, Chicago, Illinois, USA.
authors.forename: Anne T
authors.initials: AT
authors.lastname: Berg
authors.affiliation: Epilepsy Center, Department of Pediatrics, Ann & Robert H. Lurie Children's Hospital of Chicago, Northwestern University Feinberg School of Medicine, Chicago, Illinois, USA.
authors.forename: Sookyong
authors.initials: S
authors.lastname: Koh
authors.affiliation: Epilepsy Center, Department of Pediatrics, Ann & Robert H. Lurie Children's Hospital of Chicago, Northwestern University Feinberg School of Medicine, Chicago, Illinois, USA. Electronic address: LLaux@luriechildrens.org.
authors.forename: Linda
authors.initials: L
authors.lastname: Laux
mesh_terms: D000293:Adolescent; D002540:Cerebral Cortex / Q000503:physiopathology*; D002648:Child; D002675:Child, Preschool; D003645:Death, Sudden; D004569:Electroencephalography; D004831:Epilepsies, Myoclonic / Q000150:complications / Q000235:genetics / Q000503:physiopathology*; D005260:Female; D006801:Humans; D007223:Infant; D008297:Male; D062550:NAV1.1 Voltage-Gated Sodium Channel / Q000235:genetics; D012640:Seizures / Q000150:complications / Q000235:genetics / Q000503:physiopathology*; D055815:Young Adult
chemical_list: D062550:NAV1.1 Voltage-Gated Sodium Channel; C568242:SCN1A protein, human
keywords: Channelopathy; Epilepsy; Postictal generalized EEG suppression (PGES); SCN1A; Sudden unexpected death in epilepsy (SUDEP)