LivaNova Deutschland GmbH, LivaNova PLC-owned Subsidiary, Lindberghstraße 25, 80939, Munich, Germany; Department of Neurosurgery, Medical Faculty, Heinrich-Heine-University, Moorenstraße 5, D-40225, Düsseldorf, Germany.
Department of Neuroscience, Biomedicine and Movement Sciences, University of Verona, Verona, Italy; Department of Neurology, Franz Tappeiner Hospital, Merano, Italy.
Brain Stimul. 2019 Sep-Oct;12(5):1101-1110. doi: 10.1016/j.brs.2019.05.011. Epub 2019 May 14.
Refractory status epilepticus (RSE) is the persistence of status epilepticus despite second-line treatment. Super-refractory SE (SRSE) is characterized by ongoing status despite 48 h of anaesthetic treatment. Due to the high case fatality in RSE of 16-39%, off label treatments without strong evidence of efficacy in RSE are often administered. In single case-reports and small case series totalling 28 patients, acute implantation of VNS in RSE was associated with 76% and 26% success rate in generalized and focal RSE respectively. We performed an updated systematic review of the literature on efficacy of VNS in RSE/SRSE by including all reported patients.
We systematically searched EMBASE, CENTRAL, Opengre.eu, and ClinicalTrials.gov, and PubMed databases to identify studies reporting the use of VNS for RSE and/or SRSE. We also searched conference abstracts from AES and ILAE meetings.
45 patients were identified in total of which 38 were acute implantations of VNS in RSE/SRSE. Five cases had VNS implantation for epilepsia partialis continua, one for refractory electrical status epilepticus in sleep and one for acute encephalitis with refractory repetitive focal seizures. Acute VNS implantation was associated with cessation of RSE/SRSE in 74% (28/38) of acute cases. Cessation did not occur in 18% (7/38) of cases and four deaths were reported (11%); all of them due to the underlying disease and unlikely related to VNS implantation. Median duration of the RSE/SRSE episode pre and post VNS implantation was 18 days (range: 3-1680 days) and 8 days (range: 3-84 days) respectively. Positive outcomes occurred in 82% (31/38) of cases.
VNS can interrupt RSE and SRSE in 74% of patients; data originate from reported studies classified as level IV and the risk for reporting bias is high. Further prospective studies are warranted to investigate acute VNS in RSE and SRSE.
难治性癫痫持续状态（RSE）是指二线治疗后持续癫痫状态。超难治性癫痫持续状态（SRSE）的特征是在麻醉治疗 48 小时后仍持续癫痫状态。由于 RSE 的病死率高达 16-39%，因此经常使用未经证实对 RSE 有效的治疗方法。在总共 28 例患者的个案报告和小病例系列中，RSE 中急性植入 VNS 的成功率分别为全身性和局灶性 RSE 的 76%和 26%。我们通过纳入所有报告的患者，对 VNS 在 RSE/SRSE 中的疗效进行了文献的系统回顾。
我们系统地检索了 EMBASE、CENTRAL、Opengre.eu 和 ClinicalTrials.gov 以及 PubMed 数据库，以确定报告 VNS 用于 RSE 和/或 SRSE 的研究。我们还检索了 AES 和 ILAE 会议的会议摘要。
总共确定了 45 例患者，其中 38 例为 RSE/SRSE 的急性 VNS 植入。5 例为局灶性癫痫持续状态的 VNS 植入，1 例为睡眠中电癫痫持续状态的 VNS 植入，1 例为急性脑炎伴难治性局灶性重复癫痫发作的 VNS 植入。急性 VNS 植入与 74%（28/38）的急性病例中 RSE/SRSE 的停止有关。18%（7/38）的病例没有停止，有 4 例死亡（11%）；所有死亡均因基础疾病所致，不太可能与 VNS 植入有关。VNS 植入前后 RSE/SRSE 发作的中位数持续时间分别为 18 天（范围：3-1680 天）和 8 天（范围：3-84 天）。38 例中有 82%（31/38）的病例有阳性结果。
VNS 可中断 74%的 RSE 和 SRSE 患者的癫痫发作；数据来源于被分类为 IV 级的报告研究，存在报告偏倚的风险较高。需要进一步的前瞻性研究来调查 RSE 和 SRSE 中的急性 VNS。
