Objective To investigate the clinicalmanifestations, electroencephalogram (EEG) characteristics, surgical treatment and prognosis of epilepsy secondary to Sturge-Weber syndrome (SWS) in children.Methods The data of 7 children with epilepsysecondaryto Sturge-Weber syndrome who were treated by surgery from May 2015 to May 2020 in our Children's Epilepsy Center were retrospectively reviewed. Their demographic characteristics, seizure forms, results of EEG and cranial imaging investigations, surgical methods, postoperative pathological reports and prognosis during follow-upwere summarized and analyzed. The prognosis were evaluated byEngel classificationat the last time point during follow-up. Results Totally 7 pediatric patients were enrolled, including 1 male (16/25, 64.0%) and 6 females.All the 7 cases presented with focal seizures at the onset among whom 2 cases developed status epilepticus during the course of the disease(epilepsiapartialiscontinuain 1 case),one case had epileptic spasmsand 1 case was characterized by cluster seizures. The interictal EEG manifestations of the cases gradually deteriorated as the course of the disease prolonged, including the slow wave on the affected side gradually increases (7/7), the amplitude gradually decreases (7/7), and the physiological wave disappears (4/7). Besides, no epileptiform discharges/incidental or a few epileptiform dischargeswere found in their interictal EEGs. Four cases underwent hemispherotomy, 1 case underwent temporo-parieto-occipital disconnection and 2 cases underwent lesion resection. The cases were followed up for 6 months to 5 years, and the average follow-up duration was 79.29 months. Six cases were rated as Engel Ⅰa during the regularfollow-up. Only 1 case was rated asEngel Ⅱ and Engel Ⅲ at 3 month and 1 year after the operation. ConclusionChildren with epilepsy secondary to SWS usually present with focal seizures and have diverse seizure forms. The EEG show characteristic changes. For the caseswith drug refractory epilepsy, detailed preoperative evaluation and reasonable surgical methods can result in a better therapeutic effect.
ObjectiveTo summarize the clinical phenotype, electrophysiological characteristics, imaging characteristics, surgical treatment and prognosis of Rasmussen encephalitis (RE), so as to deepen the understanding of the disease. MethodsThe clinical data of patients with RE who underwent surgical therapy from October 2014 to October 2019 at Children's Epilepsy Center in Peking University First Hospital were retrospectively reviewed. Demographic characteristics, seizure forms, electroencephalogram (EEG), cranial nuclear magnetic resonance (MRI), operative methods as well as surgical outcomes evaluated by Engel classification during follow-up of the subjects were collected and analyzed. ResultsTotally 21 pediatric patients were enrolled, including 8 males and 13 females. The age at onset was (5.0±2.0) years old, the age at the time of surgery was (6.9±2.7) years old, and the disease duration at the time of surgery was (1.7±1.3) years. Twenty (20/21, 95.2%) patients had focal motor seizures, and 10 (10/21, 47.6%) patients had 2 or 3 forms of focal motor seizures. Fifteen patients (15/21, 71.4%) had epilepsia partialis continua (EPC), which occurred (0.7±0.6) years after the onset. All patients had hemiplegia, which appeared at (0.9±0.6) years after the onset. All patients showed a slow rhythm at their affected hemispheres during the EEG monitoring and 4 of them also showed slow rhythm at the contralateral hemispheres as the disease progressed. All patients had epileptiform discharges at the involved hemisphere, and 6 patients also had independent epileptiform discharges on the contralateral side. All 21 patients underwent hemispheric disconnection. The duration of follow-up was 2 to 7 years, and all patients achieved Engel class I after the surgery. The neurological dysfunction recovered to varying degrees during the postoperative period. ConclusionRE mostly occurs around the school age. Focal motor seizures are the main manifestations and the most common onset symptoms. With the progress of the disease, the condition of patients worsened gradually. The EEG of patients was mainly characterized by broad slow wave and spike wave in the affected hemisphere. Some patients can also have bilateral involvement, which was obviously asymmetrical. Through surgical treatment, the patients all achieved good results in terms of seizures and development.