Department of Neurology, Hugo Moser Kennedy Krieger Research Institute, Baltimore, Maryland.
Division of Hematology, Cincinnati Children's Hospital Medical Center, Cincinnati, Ohio; Department of Pediatrics, University of Cincinnati College of Medicine, Cincinnati, Ohio.
Pediatr Neurol. 2021 Jun;119:3-10. doi: 10.1016/j.pediatrneurol.2021.02.006. Epub 2021 Mar 5.
Epilepsy in typical Sturge-Weber syndrome (SWS) is common, and many questions remain regarding the treatment outcomes. We analyzed a large multicenter database with focus on neurological drug treatment in different demographic and SWS characteristic groups.
A total of 268 patients with brain involvement and a history of seizures were selected from a research data registry generated from a multicenter cross-sectional questionnaire. We examined associations between medication use and binary variables such as sex, ethnicity, and brain, skin, and eye involvement laterality. We analyzed group differences in mean number of antiseizure medications and age at diagnosis, enrollment, and seizure onset and examined differences in median SWS neurological scores in groups of interest.
The most frequently used medications were levetiracetam (48.1%), low-dose aspirin (44.8%), oxcarbazepine (39.9%), and phenobarbital (14.9%). Lamotrigine was more frequently used in adults than in children (P = 0.001). History of neurosurgery was associated with no current antiseizure medication use (P = 0.001), whereas bilateral brain involvement and family history of seizures were associated with using a higher number of antiseizure medications (P = 0.002, P = 0.027, respectively). Subjects with bilateral brain involvement and early seizure onset were associated with using a higher number of antiseizure medications (P = 0.002) and phenobarbital use (0.003).
Levetiracetam, low-dose aspirin, and oxcarbazepine were the most frequently used medications. More severely affected patients were frequently on a greater number of antiseizure medications. Surgery for epilepsy was associated with the ability to discontinue antiseizure medication. Longitudinal studies are needed to further investigate medication use in patients with SWS.
典型斯特奇-韦伯综合征(SWS)中癫痫很常见,关于治疗结果仍存在许多问题。我们分析了一个大型多中心数据库,重点关注不同人口统计学和 SWS 特征组的神经药物治疗。
从一项多中心横断面问卷调查生成的研究数据登记处中选择了 268 名有脑受累和癫痫发作史的患者。我们检查了药物使用与性别、种族以及脑、皮肤和眼受累侧性等二项变量之间的关联。我们分析了不同组间抗癫痫药物的平均数量和诊断、入组、癫痫发作年龄以及感兴趣组间 SWS 神经学评分中位数的差异。
最常使用的药物是左乙拉西坦(48.1%)、低剂量阿司匹林(44.8%)、奥卡西平(39.9%)和苯巴比妥(14.9%)。拉莫三嗪在成人中比在儿童中更常使用(P=0.001)。有神经外科手术史与未使用抗癫痫药物相关(P=0.001),而双侧脑受累和癫痫家族史与使用更多种抗癫痫药物相关(P=0.002,P=0.027)。双侧脑受累和早期癫痫发作的患者与使用更多种抗癫痫药物(P=0.002)和苯巴比妥(P=0.003)相关。
左乙拉西坦、低剂量阿司匹林和奥卡西平是最常使用的药物。病情更严重的患者通常使用更多种抗癫痫药物。癫痫手术与停止使用抗癫痫药物的能力相关。需要进行纵向研究以进一步调查 SWS 患者的药物使用情况。