title: Combined treatment with vigabatrin and topiramate in West syndrome.
abstract: The clinical and electroencephalographic (EEG) response to combined therapy with vigabatrin and topiramate was evaluated in five patients ages 7 to 15 months affected by West syndrome in an open-label trial. Four patients had cryptogenic and one patient had symptomatic (tuberous sclerosis) West syndrome. In cryptogenic patients who failed to respond to pyridoxine, vigabatrin was titrated to 80 to 100 mg/kg. Because control of infantile spasms or an EEG improvement was not obtained with vigabatrin treatment, topiramate was added (3-3.8 mg/kg/day). In all patients, the combined therapy with topiramate and vigabatrin achieved a rapid and complete normalization of infantile spasms, and in three patients with cryptogenic West syndrome, the EEG also became normal. In only one patient, transient anorexia was observed. This drug combination led to rapid neurodevelopmental normalization in cryptogenic patients. The results are promising and justify more trials in larger numbers of children with West syndrome.
authors.affiliation: Department of Pediatrics, Obstetric and Reproductive Medicine, Policlinico Le Scotte, University of Siena, Italy. ftnp@libero.it
authors.forename: Sabrina
authors.initials: S
authors.lastname: Buoni
authors.forename: Raffaella
authors.initials: R
authors.lastname: Zannolli
authors.forename: Mirella
authors.initials: M
authors.lastname: Strambi
authors.forename: Alberto
authors.initials: A
authors.lastname: Fois
mesh_terms: D000927:Anticonvulsants / Q000008:administration & dosage*; D001921:Brain / Q000503:physiopathology; D002657:Child Development; D004359:Drug Therapy, Combination; D005260:Female; D005500:Follow-Up Studies; D005632:Fructose / Q000008:administration & dosage* / Q000031:analogs & derivatives*; D006801:Humans; D007223:Infant; D008297:Male; D011597:Psychomotor Performance; D013036:Spasms, Infantile / Q000188:drug therapy* / Q000503:physiopathology; D000077236:Topiramate; D020888:Vigabatrin / Q000008:administration & dosage*
chemical_list: D000927:Anticonvulsants; D000077236:Topiramate; D005632:Fructose; D020888:Vigabatrin