Clinical features and surgical prognosis of epilepsy caused by Sturge-Weber syndrome
Objective To analyze and summarize the clinical characteristics and prognosis of epilepsy secondary to Sturge-Weber syndrome(SWS).Methods A retrospective analysis was conducted on 9 SWS patients who underwent preoperative evaluation for epilepsy in the Department of Neurosurgery at Tsinghua University Yuquan Hospital(Tsinghua University Integrated Traditional Chinese and Western Medicine Hospital)from January 2013 to March 2024.Six patients underwent surgical treatment after preoperative evaluation,which included 1 case of right hemispherectomy,1 case of right hemispherotomy,and 4 cases of resections of less than a hemisphere(1 case of unilateral temporo-parieto-occipital resection,2 cases of unilateral temporo-parieto-occipital disconnections,and 1 case of focal tailored resection).Three patients received medical treatment.The clinical characteristics and outcomes were documented.Results This group had 5 SWS Ⅰ and 4 SWS Ⅲ cases.The median age of seizure onset was 8 months(range:1 month to 7.5 years).All 9 patients had focal seizures,including 2 with focal epileptic spasms.MRI T1-enhanced scans of 8 patients showed localized leptomeningeal enhancement(one hemisphere involved in 4,part of one hemisphere involved in 3,and both hemispheres involved in 1).All patients showed signs of focal cortical dysplasia(FCD)on MRI,such as unclear gray-white matter junctions.The median follow-up time for the 6 surgical patients was 2.9 years(range:0.6-10.5 years),with 4 patients achieving Engel class Ⅰ and 2 patients achieving Engel class Ⅱ(relapsed at 10 months and 5.0 years postoperatively,with＞90％reduction in seizure frequency).Two patients with preoperative video EEG abnormalities exceeding the surgical resection area relapsed after surgery,while among the two patients with imaging abnormalities exceeding the resection area,only one relapsed.None of the three patients receiving medical treatment had recurrence during follow-up.Conclusions Epilepsy secondary to SWS has an early onset,with focal seizures being the most common type.In addition to leptomeningeal vascular malformations,MRI often shows FCD features.Resective surgery could lead to good postoperative seizure control.Preoperative video EEG abnormalities exceeding the resection area may be associated with postoperative seizure relapse.
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