title: Add-on treatment with pyridoxine and sulthiame in 12 infants with West syndrome: an open clinical study.
abstract: To investigate the effect of sulthiame (STM) in West syndrome (WS) an open, uncontrolled add-on study was undertaken during initial pyridoxine (PDX) therapy in 12 infants, two with idiopathic and ten with symptomatic WS. All patients were initially treated with PDX (150-300 mg x kg (-1)body weight day(-1) ). In seven patients (58%) seizures and hypsarrhythmia stopped during the week after introduction of STM (10 mg x kg (-1)body weight day (-1)). In one the positive effect was temporary. Five of the responders (42%) remained seizure-free and without hypsarrhythmia under STM monotherapy, while one developed complex partial seizures after 25 months. STM was most effective in idiopathic WS (2 /2). During treatment with STM medication no patient suffered side effects attributable to the substance. Further controlled studies are necessary to evaluate the benefit of this potentially effective treatment.
authors.affiliation: University Children's Hospital, Department of Neuropediatrics, Westfälische-Wilhelms-Universität Münster, Albert-Schweitzer-Str. 33, D - 48149 Münster, Germany. debuso@uni-muenster.de
authors.forename: Otfried Martin
authors.initials: OM
authors.lastname: Debus
authors.forename: Jessika
authors.initials: J
authors.lastname: Köhring
authors.forename: Barbara
authors.initials: B
authors.lastname: Fiedler
authors.forename: Maike
authors.initials: M
authors.lastname: Franssen
authors.forename: Gerhard
authors.initials: G
authors.lastname: Kurlemann
mesh_terms: D000927:Anticonvulsants / Q000627:therapeutic use*; D002220:Carbamazepine / Q000627:therapeutic use*; D004359:Drug Therapy, Combination; D006801:Humans; D007223:Infant; D008297:Male; D011736:Pyridoxine / Q000627:therapeutic use*; D013036:Spasms, Infantile / Q000188:drug therapy*; D013843:Thiazines / Q000627:therapeutic use*; D000096926:Benzenesulfonamides
chemical_list: D000927:Anticonvulsants; D002220:Carbamazepine; D011736:Pyridoxine; D013843:Thiazines; C084593:sulthiame; D000096926:Benzenesulfonamides