title: Successful Treatment of Refractory Seizures With Rufinamide in Children With Schizencephaly: Report of 3 Cases.
abstract: Schizencephaly is an uncommon malformation of cortical development. Patients with schizencephaly present with a broad range of severe neurologic symptoms including pharmacoresistant epilepsy. Rufinamide is a new antiepileptic drug approved for use as adjunctive therapy of seizures associated with Lennox-Gastaut syndrome and it is also effective for refractory partial seizures. We report 3 cases of pediatric patients aged 7.2, 8.1, and 10.1 years, respectively, with intractable epilepsy associated with bilateral open-lip schizencephaly and septo-optic dysplasia. The follow-up ranged from 3.8 to 4.1 years. In our patients, the introduction of rufinamide as adjunctive drug led to a dramatic decline in the number of seizures and an improvement in EEG epileptic activity without side effects. Rufinamide seems to be efficacious and safe in patients with epileptic encephalopathies associated with pharmacoresistant epilepsy; further and larger clinical reports and controlled studies could confirm the usefulness of this anticonvulsant drug.
authors: Department of Pediatrics, University of Perugia, Perugia, Italy averrott@unich.it.
authors: Alberto
authors: A
authors: Verrotti
authors: Department of Pediatrics, University of Chieti, Chieti, Italy.
authors: Giulia
authors: G
authors: Loiacono
authors: Department of Pediatrics, University of Chieti, Chieti, Italy.
authors: Alessandra
authors: A
authors: Rossi
authors: Department of Neuroscience and Imaging, Institute of Advanced Technology, G. d'Annunzio University, Chieti, Italy.
authors: Armando
authors: A
authors: Tartaro
authors: Department of Neuroscience and Imaging, Institute of Advanced Technology, G. d'Annunzio University, Chieti, Italy.
authors: Andrea
authors: A
authors: Delli Pizzi
authors: Child Neuropsychiatry Clinic, Medical School, University of Salerno, Salerno, Italy.
authors: Giangennaro
authors: G
authors: Coppola
mesh_terms: D000927:Anticonvulsants / Q000627:therapeutic use*; D002648:Child; D004569:Electroencephalography; D006801:Humans; D008279:Magnetic Resonance Imaging; D008297:Male; D065707:Schizencephaly / Q000150:complications*; D012640:Seizures / Q000175:diagnosis / Q000188:drug therapy* / Q000209:etiology*; D014230:Triazoles / Q000627:therapeutic use*
chemical_list: D000927:Anticonvulsants; D014230:Triazoles; C079703:rufinamide
keywords: antiepileptic drugs; epilepsy; rufinamide; schizencephaly; septo-optic dysplasia