Objective To investigate the clinical features and long-term prognosis of children with symptomatic epilepsy complicated by encephalofacial angiomatosis. Methods A total of 38 children with Sturge-Weber syndrome (SWS) complicated by symptomatic epilepsy who were diagnosed and treated in the Children's Hospital of Soochow University from January 2011 to June 2020 were selected as the research objects. Their clinical data were collected, and long-term follow-up studies were carried out. Results The clinical manifestations of SWS complicated by symptomatic epilepsy were diverse, with focal seizures being the most common (86.84%). Abnormalities were found in the cranial CT and MRI of all 38 children, mainly involving the parietal lobe and occipital lobe. Among the 38 children undergoing electroencephalogram (EEG) examinations, 31 had abnormal results. Among the abnormal EEGs, the background waves were asymmetrical on the left and right sides in 28 cases (the amplitude on the affected side was lower), 8 cases showed unilateral discharges to varying degrees, and 1 case showed bilateral high-amplitude discharges. Among the 38 cases, 2 did not use antiepileptic drugs, and 36 were treated with antiepileptic drugs, mainly with oxcarbazepine, topiramate, and levetiracetam. Eventually, 2 children were given surgical treatment due to drug-resistant epilepsy. Among the 38 cases, 19 (50.00%) were diagnosed with refractory epilepsy. Epilepsy occurring before the age of 2 are prone to develop into refractory epilepsy (P<0.05). All 38 children had sequelae to varying degrees, including 25 cases (65.79%) with severe sequelae and 13 cases (34.21%) with milder sequelae. Moreover, the earlier the age of epilepsy onset in children, the more likely it was to leave severe sequelae in the later stage (P=0.001). Conclusion Children with Sturge-Weber syndrome complicated by symptomatic epilepsy mainly have focal seizures. About half of them suffer from refractory epilepsy. The earlier the age of epileptic seizures, the more likely it is to develop into refractory epilepsy. The long-term prognosis of such children is rather poor. Among the 38 cases followed up, all had sequelae of varying degrees.
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.
