Background

Sturge-Weber syndrome (SWS) patients have risks of intractable epilepsy and cognitive decline. We hypothesized that leptomeningeal capillary malformation (LCM) extent related to severity of neurological affection in SWS. This study tested the hypothesis in a cross-sectional study of seizure severity and electroencephalography (EEG) findings and a retrospective cohort study for surgical indications related to extent of LCM.

Methods

We enrolled 112 patients and classified them according to LCM distribution: (1) Bilateral, (2) Hemispheric, (3) Multilobar, and (5) Single lobe. Seizure onset age, semiology and frequency, and EEG findings were compared. Surgical indications were antiepileptic drug resistance, progressive cerebral atrophy, and cognitive decline, and evaluated in each group by Fisher’s exact test, while predictors for surgery by univariate and multivariate analyses. Therapeutic efficacy was evaluated by the SWS-neurological score (SWS-NS).

Results

Bilateral and Hemispheric groups had early-onset age (4.0-months and 3.0-months), frequent seizures (88.9% and 80.6% had more than once-a-month), focal-to-bilateral tonic-clonic seizures (88.9% and 74.2%), and status epilepticus (100% and 87.1%). Groups did not differ significantly in EEG findings. Surgical indications were present in 77.8% of Bilateral, 88.1% of Hemispheric, and 46.8% of Multilobar groups. Seizure more than once monthly were a surgical treatment predictor. Seizure sub-score improved significantly postoperatively in Hemispheric and Multilobar groups. Even after the surgical treatment, Bilateral and Hemispheric groups SWS-NSs were higher than those of other groups.

Conclusion

Our study demonstrated a strong association between extensive LCM and epileptic severity. Surgical intervention improved seizure outcome in SWS patients with large LCMs. (248)

中文翻译：

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软脑膜毛细血管畸形的程度与 Sturge-Weber 综合征癫痫的严重程度相关

背景

Sturge-Weber 综合征 (SWS) 患者有顽固性癫痫和认知能力下降的风险。我们假设软脑膜毛细血管畸形 (LCM) 的程度与 SWS 中神经系统影响的严重程度有关。本研究在癫痫发作严重程度和脑电图 (EEG) 发现的横断面研究以及与 LCM 范围相关的手术适应症的回顾性队列研究中检验了这一假设。

方法

我们招募了 112 名患者并根据 LCM 分布对他们进行分类：（1）双侧，（2）半球，（3）多叶，和（5）单叶。比较癫痫发作年龄、症状学和频率以及脑电图结果。手术适应症是抗癫痫药物耐药性、进行性脑萎缩和认知能力下降，并通过Fisher精确检验对每组进行评估，同时通过单变量和多变量分析预测手术。通过SWS-神经学评分（SWS-NS）评估治疗效果。

结果

双侧和半球组有早发年龄（4.0 个月和 3.0 个月），频繁癫痫发作（88.9% 和 80.6% 每月超过一次），局灶性至双侧强直阵挛性癫痫发作（88.9% 和74.2%）和癫痫持续状态（100% 和 87.1%）。各组的脑电图结果没有显着差异。77.8% 的双侧组、88.1% 的半球组和 46.8% 的多叶组存在手术指征。每月一次以上的癫痫发作是手术治疗的预测指标。半球组和多叶组术后癫痫发作子评分显着改善。即使在手术治疗后，双侧和半球组的 SWS-NSs 也高于其他组。

结论

我们的研究表明，广泛的 LCM 与癫痫严重程度之间存在很强的关联。手术干预改善了具有大 LCM 的 SWS 患者的癫痫发作结果。(248)