title: Clinical dissection of early onset absence epilepsy in children and prognostic implications.
abstract: OBJECTIVE
  To investigate whether patients with typical absence seizures (TAS) starting in the first 3 years of life, conformed to Panayiotopoulos's definition of childhood absence epilepsy (CAE), show different electroclinical course than those not fulfilling CAE criteria.
  
  
  METHODS
  In this multicenter retrospective study, we choose a fixed duration follow-up of 36 months to examine the electroclinical course of epilepsy in all children with TAS starting before 3 years of age. The probands who fulfilled Panayiotopoulos's criteria for CAE were classified as having pure early onset absence epilepsy (P-EOAE), whereas those who did not as nonpure EOAE (NP-EOAE). In addition, these two groups of patients were further stratified according to the number of antiepileptic drugs taken to obtain initial seizure control (mono-, bi-, and tritherapy).
  
  
  RESULTS
  Patients with P-EOAE (n = 111) showed earlier initial seizure control (p = 0.030) and better seizure-free survival curve (p = 0.004) than those with NP-EOAE (n = 77). No mutation in SLC2A1 gene or abnormal neuroimaging was observed in P-EOAE. Among patients with NP-EOAE, those receiving tritherapy showed increased risk of structural brain abnormalities (p = 0.001) or SLC2A1 mutations (p = 0.001) but fewer myoclonic features (p = 0.031) and worse seizure-free survival curve (p = 0.047) than those treated with mono- and bitherapy. Children with NP-EOAE had 2.134 the odds of having relapse during the follow-up compare to those with P-EOAE.
  
  
  CONCLUSIONS
  Children with early onset TAS who did meet Panayiotopoulos's criteria showed a favorable course of epilepsy, whereas patients not fulfilling Panayiotopoulos's criteria showed increased risk of relapse at long-term follow-up.
authors.affiliation: Child Epilepsy Center, Department of Pediatrics, University of Chieti, Chieti, Italy.
authors.forename: Sergio
authors.initials: S
authors.lastname: Agostinelli
authors.forename: Patrizia
authors.initials: P
authors.lastname: Accorsi
authors.forename: Francesca
authors.initials: F
authors.lastname: Beccaria
authors.forename: Vincenzo
authors.initials: V
authors.lastname: Belcastro
authors.forename: Maria Paola
authors.initials: MP
authors.lastname: Canevini
authors.forename: Giuseppe
authors.initials: G
authors.lastname: Capovilla
authors.forename: Silvia
authors.initials: S
authors.lastname: Cappanera
authors.forename: Bernardo
authors.initials: B
authors.lastname: Dalla Bernardina
authors.forename: Francesca
authors.initials: F
authors.lastname: Darra
authors.forename: Luigi
authors.initials: L
authors.lastname: Del Gaudio
authors.forename: Maurizio
authors.initials: M
authors.lastname: Elia
authors.forename: Raffaele
authors.initials: R
authors.lastname: Falsaperla
authors.forename: Lucio
authors.initials: L
authors.lastname: Giordano
authors.forename: Giuseppe
authors.initials: G
authors.lastname: Gobbi
authors.forename: Carlo
authors.initials: C
authors.lastname: Minetti
authors.forename: Francesco
authors.initials: F
authors.lastname: Nicita
authors.forename: Pasquale
authors.initials: P
authors.lastname: Parisi
authors.forename: Piero
authors.initials: P
authors.lastname: Pavone
authors.forename: Marianna
authors.initials: M
authors.lastname: Pezzella
authors.forename: Michela
authors.initials: M
authors.lastname: Sesta
authors.forename: Alberto
authors.initials: A
authors.lastname: Spalice
authors.forename: Salvatore
authors.initials: S
authors.lastname: Striano
authors.forename: Elisabetta
authors.initials: E
authors.lastname: Tozzi
authors.forename: Monica
authors.initials: M
authors.lastname: Traverso
authors.forename: Stella
authors.initials: S
authors.lastname: Vari
authors.forename: Aglaia
authors.initials: A
authors.lastname: Vignoli
authors.forename: Nelia
authors.initials: N
authors.lastname: Zamponi
authors.forename: Federico
authors.initials: F
authors.lastname: Zara
authors.forename: Pasquale
authors.initials: P
authors.lastname: Striano
authors.forename: Alberto
authors.initials: A
authors.lastname: Verrotti
mesh_terms: D017668:Age of Onset; D001921:Brain / Q000503:physiopathology; D002675:Child, Preschool; D004569:Electroencephalography; D004832:Epilepsy, Absence / Q000175:diagnosis* / Q000235:genetics / Q000503:physiopathology; D005260:Female; D051272:Glucose Transporter Type 1 / Q000235:genetics; D006801:Humans; D007223:Infant; D053208:Kaplan-Meier Estimate; D008297:Male; D011379:Prognosis; D012008:Recurrence; D012189:Retrospective Studies; D012307:Risk Factors; D012737:Sex Factors
chemical_list: D051272:Glucose Transporter Type 1; C495677:SLC2A1 protein, human
keywords: Antiepileptic drugs; Childhood absence epilepsy; Early onset absence epilepsy; GLUT1 deficiency; Typical absence seizures