title: Tailored disconnection based on presurgical evidence in catastrophic epilepsy: report of 2 cases.
abstract: Catastrophic epilepsy in infants, often due to extensive cortical dysplasia, has devastating consequences with respect to brain development. Conventional lobar, multilobar, or hemispheric resection in these infants is challenging, carrying an increased operative risk compared with that in older children. Removing a larger tissue volume versus removing or disconnecting the epileptogenic region does not always guarantee better seizure outcome. The authors describe 2 infants with catastrophic epilepsy who benefited from individually tailored disconnections based on a hypothesized epileptogenic zone following intensive presurgical evaluation. Two infants with catastrophic epilepsy and epileptic spasms underwent leukotomies between 3 and 12 months of age. They were followed up postoperatively for 19-36 months. Both patients had 90%-100% seizure reduction and a significantly improved neurodevelopmental outcome without postoperative complication. Cortical malformation was seen in both patients. Modifications of established surgical disconnection techniques, tailored to each patient's specific epileptogenic zone, optimized seizure and neurodevelopmental outcomes while minimizing the risks associated with more extensive resections.
authors: Section of Pediatric Epilepsy and.|Epilepsy Center and.
authors: Jun T
authors: JT
authors: Park
authors: Department of Neurosurgery, Neurological Institute; and.
authors: Sunil V
authors: SV
authors: Manjila
authors: Division of Developmental and Behavioral Pediatrics and Psychology, Department of Pediatrics, Rainbow Babies & Children's Hospital;
authors: Rachel B
authors: RB
authors: Tangen
authors: Department of Pathology, University Hospital, Case Western University School of Medicine, Cleveland, Ohio.
authors: Mark L
authors: ML
authors: Cohen
authors: Section of Pediatric Epilepsy and.|Epilepsy Center and.
authors: Asim M
authors: AM
authors: Shahid
authors: Department of Neurosurgery, Neurological Institute; and.
authors: Jennifer A
authors: JA
authors: Sweet
authors: Section of Pediatric Epilepsy and.
authors: Ingrid E
authors: IE
authors: Tuxhorn
authors: Department of Neurosurgery, Neurological Institute; and.
authors: Jonathan P
authors: JP
authors: Miller
mesh_terms: D002388:Catastrophic Illness / Q000523:psychology*; D004569:Electroencephalography; D004827:Epilepsy / Q000000981:diagnostic imaging / Q000503:physiopathology* / Q000523:psychology* / Q000601:surgery; D005260:Female; D019788:Fluorodeoxyglucose F18; D038421:Hemispherectomy / Q000379:methods*; D006801:Humans; D007223:Infant; D008279:Magnetic Resonance Imaging; D008297:Male; D049268:Positron-Emission Tomography; D015899:Tomography, Emission-Computed, Single-Photon
chemical_list: D019788:Fluorodeoxyglucose F18
keywords: AED = antiepilepsy drug; CD = cortical dysplasia; EEG = electroencephalography; ILAE = International League Against Epilepsy; MSEL = Mullen Scales of Early Learning; PLED = periodic lateralized epileptiform discharge; TPO = temporal, parietal, occipital; catastrophic epilepsy; cortical dysplasia; disconnection; epilepsy surgery; epileptic spasms; epileptogenic zone; leucotomy; seizure semiology