title: Antiepileptic treatment before the onset of seizures reduces epilepsy severity and risk of mental retardation in infants with tuberous sclerosis complex.
abstract: BACKGROUND
  Epilepsy appears in 70-80% of patients with tuberous sclerosis complex, most commonly in the first year of age. Early manifestation of epilepsy is associated with drug-resistant epilepsy and mental retardation in more than 80% of patients. Clinical epileptic seizures are preceded by deterioration of EEG recording thus infants with high risk of epilepsy can be identified.
  
  
  OBJECTIVE
  We hypothesized that preventative antiepileptic treatment of infants with multifocal activity on EEG might lower the incidence of drug-resistant epilepsy and mental retardation.
  
  
  METHODS
  Forty-five infants with early diagnosis of tuberous sclerosis complex were included in the open-label study. They were divided in two groups: standard (n=31) and preventative one (n=14). In standard group the antiepileptic treatment was launched early, but after the onset of seizures. In preventative group medication was commenced when active epileptic discharges were seen on EEG, but before the onset of clinical seizures. Children were followed till the end of 2 years of age.
  
  
  RESULTS
  At 24 months of age mental retardation was significantly more frequent and severe in "standard" vs "preventative" group (48% vs 14%; p=0.031; mean IQ score 68.7 vs 92.3; p<0.05). The "preventative" group was characterized by higher ratio of seizure-free patients (93% vs 35%; p=0.004), lower incidence of drug-resistant epilepsy (7% vs 42%; p=0.021) and lower number of patients requiring polytherapy (21% vs 55%; 0.039) than the "standard group.
  
  
  CONCLUSIONS
  Preventative antiepileptic treatment of infants with tuberous sclerosis complex and high risk of epilepsy markedly improves their neurodevelopmental outcome and reduces the incidence of drug-resistant seizures.
authors.affiliation: Department of Neurology and Epileptology, The Children's Memorial Health Institute, Warsaw, Poland. sergiusz.jozwiak@gmail.com
authors.forename: Sergiusz
authors.initials: S
authors.lastname: Jóźwiak
authors.forename: Katarzyna
authors.initials: K
authors.lastname: Kotulska
authors.forename: Dorota
authors.initials: D
authors.lastname: Domańska-Pakieła
authors.forename: Barbara
authors.initials: B
authors.lastname: Lojszczyk
authors.forename: Małgorzata
authors.initials: M
authors.lastname: Syczewska
authors.forename: Dariusz
authors.initials: D
authors.lastname: Chmielewski
authors.forename: Dorota
authors.initials: D
authors.lastname: Dunin-Wąsowicz
authors.forename: Tomasz
authors.initials: T
authors.lastname: Kmieć
authors.forename: Joanna
authors.initials: J
authors.lastname: Szymkiewicz-Dangel
authors.forename: Maria
authors.initials: M
authors.lastname: Kornacka
authors.forename: Wanda
authors.initials: W
authors.lastname: Kawalec
authors.forename: Dariusz
authors.initials: D
authors.lastname: Kuczyński
authors.forename: Julita
authors.initials: J
authors.lastname: Borkowska
authors.forename: Katarzyna
authors.initials: K
authors.lastname: Tomaszek
authors.forename: Elżbieta
authors.initials: E
authors.lastname: Jurkiewicz
authors.forename: Maria
authors.initials: M
authors.lastname: Respondek-Liberska
mesh_terms: D000927:Anticonvulsants / Q000008:administration & dosage*; D002675:Child, Preschool; D004827:Epilepsy / Q000188:drug therapy* / Q000453:epidemiology / Q000517:prevention & control; D005260:Female; D006801:Humans; D007223:Infant; D007231:Infant, Newborn; D008607:Intellectual Disability / Q000175:diagnosis / Q000453:epidemiology / Q000517:prevention & control*; D008297:Male; D011247:Pregnancy; D011446:Prospective Studies; D012307:Risk Factors; D012720:Severity of Illness Index; D016896:Treatment Outcome; D014402:Tuberous Sclerosis / Q000175:diagnosis / Q000453:epidemiology / Q000503:physiopathology*
chemical_list: D000927:Anticonvulsants