title: Treatment of epilepsy in severely disabled children with bilateral brain malformations.
abstract: To determine a management strategy for the epilepsy in children with bilateral cortical malformations, clinical data of 23 patients (age, 3-23 years, M:F=7:16) were retrospectively reviewed. Among these patients, 15 were bedridden and 16 were profoundly retarded and could not even smile. The patients were categorized into the following five groups based on the findings of neuroimaging, seizure types, and electroencephalographic patterns. Group 1: Diffuse cortical malformation with epileptic spasms and secondarily generalized tonic seizures, group 2: diffuse cortical malformation with erratic twitches, group 3: bilaterally extended but not diffuse cortical malformations, group 4: bilateral polymicrogyria with persistent epileptic spasms (Aicardi syndrome), and group 5: bilateral cortical malformation with drop attacks (subcortical band heterotopia and congenital bilateral perisylvian syndrome). Eleven patients suffered from infantile spasms; adrenocorticotropic hormone was effective in group 1 but ineffective in group 4. Treatment of tonic seizures in groups 1-3 and erratic twitching in group 3 with phenobarbital, zonisamide and potassium bromide was beneficial. Epileptic spasms and tonic seizures were prominent in group 4 and were refractory to medical treatment, except that zonisamide, clobazam, and a ketogenic diet were partially or transiently effective. Complex partial and astatic/atonic seizures in group 5 were refractory to medications other than that carbamazepine and clobazam provided limited benefits. Total callosotomy resulted in better seizure control for three patients in group 5, and functional hemispherectomy was effective for one patient in group 4. These results provide the basis for the appropriate choice of medical and surgical treatment for managing bilateral, widespread cortical malformations.
authors.affiliation: Department of Child Neurology, National Center Hospital for Mental, Nervous and Muscular Disorders, National Center of Neurology and Psychiatry (NCNP), Japan. saitoyo@ncnp.go.jp
authors.forename: Yoshiaki
authors.initials: Y
authors.lastname: Saito
authors.forename: Kenji
authors.initials: K
authors.lastname: Sugai
authors.forename: Eiji
authors.initials: E
authors.lastname: Nakagawa
authors.forename: Hiroshi
authors.initials: H
authors.lastname: Sakuma
authors.forename: Hirofumi
authors.initials: H
authors.lastname: Komaki
authors.forename: Masayuki
authors.initials: M
authors.lastname: Sasaki
authors.forename: Yoshihiro
authors.initials: Y
authors.lastname: Maegaki
authors.forename: Kousaku
authors.initials: K
authors.lastname: Ohno
authors.forename: Noriko
authors.initials: N
authors.lastname: Sato
authors.forename: Yuu
authors.initials: Y
authors.lastname: Kaneko
authors.forename: Taisuke
authors.initials: T
authors.lastname: Otsuki
mesh_terms: D000293:Adolescent; D000927:Anticonvulsants / Q000008:administration & dosage* / Q000009:adverse effects; D002540:Cerebral Cortex / Q000002:abnormalities* / Q000503:physiopathology; D002648:Child; D002675:Child, Preschool; D004569:Electroencephalography; D004827:Epilepsy / Q000188:drug therapy* / Q000473:pathology* / Q000503:physiopathology; D005260:Female; D007839:Functional Laterality; D006801:Humans; D008607:Intellectual Disability / Q000473:pathology* / Q000503:physiopathology; D008279:Magnetic Resonance Imaging; D015225:Magnetoencephalography; D008297:Male; D012189:Retrospective Studies; D012720:Severity of Illness Index; D055815:Young Adult
chemical_list: D000927:Anticonvulsants