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.
Citation：
WANG Yuqing, DAI Yu, TANG Jihong, XIAO Xiao, SHI Xiaoyan, ZHANG Bingbing. Long-term follow-up study of symptomatic epilepsy in patients with encephalofacial angiomatosis. Journal of Epilepsy, 2026, 12(1): 21-27. doi: 10.7507/2096-0247.202512001
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Copyright ? the editorial department of Journal of Epilepsy of West China Medical Publisher. All rights reserved
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Shah AD, Alexieff P, Tatachar P. Sturge-Weber syndrome: a narrative review of clinical presentation and updates on management. Journal of Clinical Medicine, 2025, 22(14)(7): 2182.
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- 5. Shah AD, Alexieff P, Tatachar P. Sturge-Weber syndrome: a narrative review of clinical presentation and updates on management. Journal of Clinical Medicine, 2025, 22(14)(7): 2182.
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