title: Long-term developmental outcome in patients with West syndrome after epilepsy surgery.
abstract: It has been hypothesized that early seizure control may prevent children with intractable epileptic spasms (ES) from developmental regression and may contribute to better developmental outcome. The effectiveness of surgery for ES has been reported. We investigated long-term post-operative outcomes of seizure control and development in patients with symptomatic West syndrome (S-WS) who underwent epilepsy surgery. Six children who underwent surgical intervention for intractable ES were retrospectively investigated. Cortical malformations were observed on pre-operative MRI in all patients, with hemispheric or multilobar involvement in four children and focal lesions in two. Following surgery, we measured motor function, developmental age (DA), language skills, and sociopsychological function for up to 7years (mean, 4.9years). Post-operative seizure outcome was Engel Class I (n=4) or III (n=2). Motor function and DA was increased following surgery in six and five patients, respectively. Two patients started to speak in sentences following focal resection. Autistic features were noted in four of the five examined patients post-operatively. None of the patients showed developmental regression following surgery. Epilepsy surgery for S-WS with ES may result in good seizure control and improvement in motor development. Improvement in cognitive function was modest in this small cohort of children and autistic features were noted post-operatively in a substantial proportion of the children. While seizure control can be obtained by epilepsy surgery, early intervention for sociopsychological comorbidities may be warranted in children with S-WS.
authors.affiliation: Department of Pediatrics, Osaka University Graduate School of Medicine, Osaka, Japan.
authors.forename: Yoshiko
authors.initials: Y
authors.lastname: Iwatani
authors.forename: Kuriko
authors.initials: K
authors.lastname: Kagitani-Shimono
authors.forename: Koji
authors.initials: K
authors.lastname: Tominaga
authors.forename: Takeshi
authors.initials: T
authors.lastname: Okinaga
authors.forename: Ikuko
authors.initials: I
authors.lastname: Mohri
authors.forename: Haruhiko
authors.initials: H
authors.lastname: Kishima
authors.forename: Amami
authors.initials: A
authors.lastname: Kato
authors.forename: Wakako
authors.initials: W
authors.lastname: Sanefuji
authors.forename: Tomoka
authors.initials: T
authors.lastname: Yamamoto
authors.forename: Aika
authors.initials: A
authors.lastname: Tatsumi
authors.forename: Emi
authors.initials: E
authors.lastname: Murata
authors.forename: Masako
authors.initials: M
authors.lastname: Taniike
authors.forename: Toshisaburo
authors.initials: T
authors.lastname: Nagai
authors.forename: Keiichi
authors.initials: K
authors.lastname: Ozono
mesh_terms: D001921:Brain / Q000000981:diagnostic imaging / Q000473:pathology; D004569:Electroencephalography; D004827:Epilepsy / Q000601:surgery; D019788:Fluorodeoxyglucose F18; D006801:Humans; D007223:Infant; D007231:Infant, Newborn; D008137:Longitudinal Studies; D008279:Magnetic Resonance Imaging; D019635:Neurosurgical Procedures / Q000009:adverse effects*; D049268:Positron-Emission Tomography; D011183:Postoperative Complications / Q000209:etiology*; D012189:Retrospective Studies; D013036:Spasms, Infantile / Q000175:diagnosis / Q000209:etiology*; D015899:Tomography, Emission-Computed, Single-Photon
chemical_list: D019788:Fluorodeoxyglucose F18