Department of Neurosurgery, Juntendo University, Bunkyo-ku, Tokyo, Japan.
Department of Neurosurgery, Juntendo University, Bunkyo-ku, Tokyo, Japan.
Pediatr Neurol. 2021 Apr;117:64-71. doi: 10.1016/j.pediatrneurol.2020.12.012. Epub 2020 Dec 31.
Individuals with Sturge-Weber syndrome (SWS) often expereince intractable epilepsy and cognitive decline. We hypothesized that the extent of the leptomeningeal capillary malformation (LCM) may correlate with the severity of neurological impairment due to SWS. We tested the hypothesis in a cross-sectional study of seizure severity and electroencephalographic (EEG) findings and a retrospective cohort study for surgical indications related to the extent of the LCM.
We enrolled 112 patients and classified them according to LCM distribution: (1) bilateral, (2) hemispheric, (3) multilobar, and (4) single lobe. Age at seizure onset, seizure semiology and frequency, and EEG findings were compared. Surgical indications were evaluated for each group by Fisher exact test, and predictors for surgery were evaluated by univariate and multivariate analyses. Therapeutic efficacy was evaluated by the SWS-Neurological Score (SWS-NS).
The bilateral and hemispheric groups had early seizure onset (4.0 months old and 3.0 months old), frequent seizures (88.9% and 80.6% had more than one per month), focal-to-bilateral tonic-clonic seizures (88.9% and 74.2%), and status epilepticus (100% and 87.1%). The groups' EEG findings did not differ substantially. Surgical indications were present in 77.8% of the bilateral, 88.1% of the hemispheric, and 46.8% of the multilobar groups. Seizure more than once per month was a predictor of surgical treatment. Seizure subscore improved postoperatively in the hemispheric and multilobar groups. Even after surgical treatment, the bilateral and hemispheric groups exhibited higher SWS-NSs than members of the other groups.
Our study demonstrated a strong association between extensive LCM and epilepsy severity. Surgical intervention improved seizure outcome in patients with SWS with large LCMs.
斯特奇-韦伯综合征（SWS）患者常伴有难治性癫痫和认知能力下降。我们假设软脑膜毛细血管畸形（LCM）的范围可能与 SWS 引起的神经损伤严重程度相关。我们在一项关于癫痫严重程度和脑电图（EEG）发现的病例对照研究和一项与 LCM 范围相关的手术适应证的回顾性队列研究中检验了这一假说。
我们纳入了 112 名患者，并根据 LCM 分布将其分类：（1）双侧，（2）半球性，（3）多叶性，（4）单叶性。比较了发病年龄、癫痫发作类型和频率以及 EEG 发现。对每组患者的手术适应证进行 Fisher 确切检验评估，采用单因素和多因素分析评估手术的预测因素。采用 SWS 神经评分（SWS-NS）评估治疗效果。
双侧和半球性组的癫痫发作较早（4.0 个月和 3.0 个月），发作频繁（88.9%和 80.6%每月发作超过一次），局灶性至双侧强直阵挛发作（88.9%和 74.2%），以及癫痫持续状态（100%和 87.1%）。这些组的 EEG 发现没有显著差异。双侧组有 77.8%、半球性组有 88.1%和多叶性组有 46.8%有手术适应证。每月发作超过一次是手术治疗的预测因素。半球性和多叶性组术后癫痫发作评分有所改善。即使在手术后，双侧和半球性组的 SWS-NS 仍高于其他组。
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。对于 LCM 较大的 SWS 患者，手术干预可改善癫痫发作结局。
