title: Two siblings with cortical dysplasia: Clinico-electroencephalographic features.
abstract: The older of two siblings began to have spasms and partial seizures at 1 month of age. Head magnetic resonance imaging showed an abnormal area in the left temporo-parieto-occipital region. Interictal electroencephalogram (EEG) showed a suppression-burst pattern. Adrenocorticotropic hormone stopped the spasms, but the seizures continued. Clonazepam, carbamazepine, zonisamide, and clobazam were ineffective. She underwent focal resection at age 8 months. Postoperatively, the seizures disappeared. Histopathologically, the lesion appeared to be focal cortical dysplasia type IIa. The younger sibling had spasms from birth. Head magnetic resonance imaging showed left hemi-megalencephaly. Interictal EEG showed a suppression-burst pattern. Phenobarbital, valproic acid, and zonisamide were ineffective. He underwent hemispherotomy at age 2 months and became seizure free. The histopathological features were consistent with those of hemi-megalencephaly. The siblings' EEG and clinical courses had some similarities. These siblings' conditions may have the same genetic background.
authors.affiliation: Department of Pediatrics, Anjo Kosei Hospital, Aichi, Japan.
authors.forename: Tatsuya
authors.initials: T
authors.lastname: Fukasawa
authors.affiliation: Department of Pediatrics, Anjo Kosei Hospital, Aichi, Japan.
authors.forename: Tetsuo
authors.initials: T
authors.lastname: Kubota
authors.affiliation: Department of Pediatrics, Anjo Kosei Hospital, Aichi, Japan.|Department of Pediatrics, Nagoya University, Aichi, Japan.|Faculty of Child Development, Department of Clinical Psychology, Nihon Fukushi University, Aichi, Japan.
authors.forename: Tamiko
authors.initials: T
authors.lastname: Negoro
authors.affiliation: Department of Child Neurology, National Center Hospital of Neurology and Psychiatry, Tokyo, Japan.
authors.forename: Shinsuke
authors.initials: S
authors.lastname: Maruyama
authors.affiliation: Department of Child Neurology, National Center Hospital of Neurology and Psychiatry, Tokyo, Japan.
authors.forename: Ryoko
authors.initials: R
authors.lastname: Honda
authors.affiliation: Department of Pathology and Laboratory Medicine, National Center Hospital of Neurology and Psychiatry, Tokyo, Japan.
authors.forename: Yuko
authors.initials: Y
authors.lastname: Saito
authors.affiliation: Department of Mental Retardation and Birth Defect Research, National Institute of Neuroscience, National Center of Neurology and Psychiatry, Tokyo, Japan.
authors.forename: Masayuki
authors.initials: M
authors.lastname: Itoh
authors.affiliation: Department of Pathology, Brain Research Institute, University of Niigata, Niigata, Japan.
authors.forename: Akiyoshi
authors.initials: A
authors.lastname: Kakita
authors.affiliation: Department of Child Neurology, National Center Hospital of Neurology and Psychiatry, Tokyo, Japan.
authors.forename: Kenji
authors.initials: K
authors.lastname: Sugai
authors.affiliation: Department of Neurosurgery, National Center Hospital of Neurology and Psychiatry, Tokyo, Japan.
authors.forename: Taisuke
authors.initials: T
authors.lastname: Otsuki
authors.affiliation: Department of Pediatrics, Yamagata University Faculty of Medicine, Yamagata, Japan.
authors.forename: Mitsuhiro
authors.initials: M
authors.lastname: Kato
authors.affiliation: Department of Pediatrics, Nagoya University, Aichi, Japan.
authors.forename: Jun
authors.initials: J
authors.lastname: Natsume
authors.affiliation: Faculty of Health and Medical Sciences, Aichi Shukutoku University, Aichi, Japan.
authors.forename: Kazuyoshi
authors.initials: K
authors.lastname: Watanabe
mesh_terms: D002675:Child, Preschool; D003937:Diagnosis, Differential; D004569:Electroencephalography / Q000379:methods*; D005260:Female; D006801:Humans; D007223:Infant; D008279:Magnetic Resonance Imaging / Q000379:methods*; D008297:Male; D054220:Malformations of Cortical Development / Q000150:complications / Q000175:diagnosis*; D012640:Seizures / Q000175:diagnosis / Q000209:etiology*; D035781:Siblings
keywords: cortical dysplasia; electroencephalogram; epileptic encephalopathy; hemi-megalencephaly; spasm