title: Successful treatment of migrating partial seizures in Wolf-Hirschhorn syndrome with bromide.
abstract: A girl with mild psychomotor developmental delay developed right or left hemiclonic convulsion at 10months of age. One month later, clusters of hemiclonic or bilateral tonic seizures with eyelid twitching emerged, resulting in status epilepticus. Treatment with phenobarbital and potassium bromide completely terminated the seizures within 10days. Ictal electroencephalography revealed a migrating focus of rhythmic 3-4Hz waves from the right temporal to right frontal regions and then to the left frontal regions. Genetic analysis was conducted based on the characteristic facial appearance of the patient, which identified a 2.1-Mb terminal deletion on chromosome 4p. This is the first case of Wolf-Hirschhorn syndrome complicated by epilepsy with migrating partial seizures.
authors.affiliation: Division of Child Neurology, Department of Brain and Neurosciences, Faculty of Medicine, Tottori University, Yonago, Japan.
authors.forename: Ayako
authors.initials: A
authors.lastname: Itakura
authors.affiliation: Division of Child Neurology, Department of Brain and Neurosciences, Faculty of Medicine, Tottori University, Yonago, Japan. Electronic address: saitoyo@med.tottori-u.ac.jp.
authors.forename: Yoshiaki
authors.initials: Y
authors.lastname: Saito
authors.affiliation: Division of Child Neurology, Department of Brain and Neurosciences, Faculty of Medicine, Tottori University, Yonago, Japan.
authors.forename: Yoko
authors.initials: Y
authors.lastname: Nishimura
authors.affiliation: Division of Child Neurology, Department of Brain and Neurosciences, Faculty of Medicine, Tottori University, Yonago, Japan.
authors.forename: Tetsuya
authors.initials: T
authors.lastname: Okazaki
authors.affiliation: Division of Child Neurology, Department of Brain and Neurosciences, Faculty of Medicine, Tottori University, Yonago, Japan.
authors.forename: Koyo
authors.initials: K
authors.lastname: Ohno
authors.affiliation: Department of Pediatrics, Matsue Red-Cross Hospital, Matsue, Japan.
authors.forename: Hitoshi
authors.initials: H
authors.lastname: Sejima
authors.affiliation: Tokyo Women's Medical University Institute for Integrated Medical Sciences, Tokyo, Japan.
authors.forename: Toshiyuki
authors.initials: T
authors.lastname: Yamamoto
authors.affiliation: Division of Child Neurology, Department of Brain and Neurosciences, Faculty of Medicine, Tottori University, Yonago, Japan.
authors.forename: Yoshihiro
authors.initials: Y
authors.lastname: Maegaki
mesh_terms: D000927:Anticonvulsants / Q000627:therapeutic use*; D001921:Brain / Q000187:drug effects / Q000503:physiopathology; D001965:Bromides / Q000627:therapeutic use*; D002872:Chromosome Deletion; D002894:Chromosomes, Human, Pair 4; D055028:Comparative Genomic Hybridization; D004569:Electroencephalography; D004827:Epilepsy / Q000150:complications / Q000188:drug therapy / Q000235:genetics / Q000503:physiopathology; D005260:Female; D006801:Humans; D007223:Infant; D010634:Phenobarbital / Q000627:therapeutic use; D017680:Potassium Compounds / Q000627:therapeutic use*; D012640:Seizures / Q000150:complications* / Q000188:drug therapy* / Q000235:genetics / Q000503:physiopathology; D054877:Wolf-Hirschhorn Syndrome / Q000150:complications* / Q000188:drug therapy* / Q000235:genetics / Q000503:physiopathology
chemical_list: D000927:Anticonvulsants; D001965:Bromides; D017680:Potassium Compounds; C039004:potassium bromide; D010634:Phenobarbital
keywords: 4p-Syndrome; Bromide; Migrating partial seizures in infancy; Status epilepticus