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结节性硬化症的耐药性癫痫:迷走神经刺激术联合或不联合后续切除术。

Refractory epilepsy in tuberous sclerosis: vagus nerve stimulation with or without subsequent resective surgery.

机构信息

Department of Neurosurgery, New York University Langone Medical Center, New York, NY 10016, USA.

出版信息

Epilepsy Behav. 2009 Nov;16(3):454-60. doi: 10.1016/j.yebeh.2009.08.018. Epub 2009 Sep 19.

DOI:10.1016/j.yebeh.2009.08.018
PMID:19767244
Abstract

OBJECTIVE

The goal of the work described here was to assess the efficacy and safety of vagus nerve stimulation in a cohort of patients with tuberous sclerosis complex with refractory epilepsy. Furthermore, we examined the impact of vagus nerve stimulation failure on the ultimate outcome following subsequent intracranial epilepsy surgery.

METHODS

A retrospective review was performed on 19 patients with refractory epilepsy and TSC who underwent vagus nerve stimulator (VNS) implantation. There were 11 (58%) females and 8 (42%) males aged 2 to 44 years when the VNS was implanted (mean: 14.7+/-12 years). Twelve patients underwent primary VNS implantation after having failed a mean of 7.1 antiepileptic drugs. Two patients (17%) had generalized epilepsy, one had a single seizure focus, three (25%) had multifocal epilepsy, and six (50%) had multifocal and generalized epilepsy. Seven patients were referred for device removal and evaluation for intracranial procedures. One patient in the primary implantation group was lost to follow-up and excluded from outcome analysis.

RESULTS

All implantations and removals were performed without permanent complications. The duration of treatment for primary VNS implants varied from 8.5 months to 9.6 years (mean: 4.9 years). Mean seizure frequency significantly improved following VNS implantation (mean reduction: 72%, P<0.002). Two patients had Engel Class I (18%), one had Class II (9%), seven had Class III (64%), and one had Class IV (9%) outcome. Three patients with poor response to vagus nerve stimulation therapy at our center underwent resection of one or more seizure foci (Engel Class I, two patients; Engel Class III, one patient). Seven patients referred to our center for VNS removal and craniotomy underwent seizure focus resection (6) or corpus callosotomy (1) (Engel Class II: 2, Engel III: 2; Engel IV: 3). In total, 8 of 10 (80%) patients experienced improved seizure control following intracranial surgery (mean reduction: 65%, range: 0-100%, P<0.05).

CONCLUSIONS

VNS is a safe and effective treatment option for medically refractory epilepsy in patients with tuberous sclerosis complex. Nine of 11 patients (82%) experienced at least a 67% reduction in seizure burden. Lack of response to vagus nerve stimulation does not preclude subsequent improvement in seizure burden with intracranial epilepsy surgery.

摘要

目的

描述性研究的目标是评估迷走神经刺激术在伴有难治性癫痫的结节性硬化症患者中的疗效和安全性。此外,我们还研究了迷走神经刺激术失败对随后颅内癫痫手术最终结果的影响。

方法

对 19 例接受迷走神经刺激器(VNS)植入术的难治性癫痫和结节性硬化症患者进行回顾性分析。植入 VNS 时,女性 11 例(58%),男性 8 例(42%),年龄 2 至 44 岁(平均:14.7+/-12 岁)。12 例患者在经历了平均 7.1 种抗癫痫药物治疗失败后接受了原发性 VNS 植入。2 例(17%)患者患有全身性癫痫,1 例患者仅有 1 个癫痫灶,3 例(25%)患者为多灶性癫痫,6 例(50%)患者为多灶性和全身性癫痫。7 例患者因设备移除和颅内手术评估而转介。原发性植入组的 1 例患者失访,未纳入结果分析。

结果

所有植入和移除手术均无永久性并发症。原发性 VNS 植入的治疗时间从 8.5 个月到 9.6 年不等(平均:4.9 年)。VNS 植入后癫痫发作频率显著降低(平均减少:72%,P<0.002)。2 例患者为 Engel Ⅰ级(18%),1 例为 Engel Ⅱ级(9%),7 例为 Engel Ⅲ级(64%),1 例为 Engel Ⅳ级(9%)。我们中心 3 例对迷走神经刺激治疗反应不佳的患者接受了一个或多个癫痫灶切除术(Engel Ⅰ级,2 例;Engel Ⅲ级,1 例)。7 例因 VNS 移除和开颅手术而转至我们中心的患者接受了癫痫灶切除术(6 例)或胼胝体切开术(1 例)(Engel Ⅱ级:2 例;Engel Ⅲ级:2 例;Engel Ⅳ级:3 例)。总的来说,颅内手术后 10 例患者中有 8 例(80%)癫痫发作控制得到改善(平均减少:65%,范围:0-100%,P<0.05)。

结论

VNS 是结节性硬化症患者药物难治性癫痫的一种安全有效的治疗选择。11 例患者中有 9 例(82%)癫痫发作负担至少减少了 67%。对迷走神经刺激术无反应并不排除随后颅内癫痫手术可改善癫痫发作负担。

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