title: Topiramate as add-on drug in severe myoclonic epilepsy in infancy: an Italian multicenter open trial.
abstract: OBJECTIVE
  This study was to evaluate the efficacy and safety of topiramate (TPM) in patients with severe myoclonic epilepsy in infancy (SMEI) and refractory seizures.
  
  
  METHODS
  We performed a prospective multicentric open label add-on study in 18 patients (age 2-21 years, mean 9 years) with SMEI and refractory seizures of different types. TPM was added to one or two other baseline drugs and the efficacy was rated according to seizure type and frequency.
  
  
  RESULTS
  TPM was initiated at a daily dose of 0.5-1 mg/kg, followed by a 2-week titration at increments of 1-3 mg/kg/24 h up to a maximum daily dose of 12 mg/kg. After a mean period of 11.9 months (range 2-24 months), three patients (16.7%) had 100% fewer seizures and ten patients (55.5%) had a more than 50% seizure decrease. In no patient there was a seizure worsening. Mild to moderate adverse events were present in four patients (22.2%), represented by weight loss, hypermenorrhoea, renal microlithiasis, nervousness and dysarthric speech.
  
  
  CONCLUSIONS
  TPM may be a useful drug in patients with SMEI, being particularly effective against generalized tonic-clonic seizures. Further studies are needed to evaluate the early use of this drug in such a severe syndrome.
authors.affiliation: Clinic of Child Neuropsychiatry, Department of Pediatrics, Second University of Naples, Via Pansini, 5, 80131, Napoli, Italy.
authors.forename: Giangennaro
authors.initials: G
authors.lastname: Coppola
authors.forename: Giuseppe
authors.initials: G
authors.lastname: Capovilla
authors.forename: Alessandra
authors.initials: A
authors.lastname: Montagnini
authors.forename: Antonino
authors.initials: A
authors.lastname: Romeo
authors.forename: Maria
authors.initials: M
authors.lastname: Spanò
authors.forename: Gaetano
authors.initials: G
authors.lastname: Tortorella
authors.forename: Pierangelo
authors.initials: P
authors.lastname: Veggiotti
authors.forename: Maurizio
authors.initials: M
authors.lastname: Viri
authors.forename: Antonio
authors.initials: A
authors.lastname: Pascotto
mesh_terms: D000293:Adolescent; D000328:Adult; D000927:Anticonvulsants / Q000009:adverse effects / Q000627:therapeutic use*; D002648:Child; D002675:Child, Preschool; D004359:Drug Therapy, Combination; D004831:Epilepsies, Myoclonic / Q000188:drug therapy*; D005260:Female; D005632:Fructose / Q000009:adverse effects / Q000031:analogs & derivatives / Q000627:therapeutic use*; D006801:Humans; D007558:Italy; D008297:Male; D011446:Prospective Studies; D000077236:Topiramate
chemical_list: D000927:Anticonvulsants; D000077236:Topiramate; D005632:Fructose