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结节性硬化症合并双侧多发性病灶儿童侵入性术前评估后切除性癫痫手术的长期疗效

Long-term outcomes of resective epilepsy surgery after invasive presurgical evaluation in children with tuberous sclerosis complex and bilateral multiple lesions.

作者信息

Arya Ravindra, Tenney Jeffrey R, Horn Paul S, Greiner Hansel M, Holland Katherine D, Leach James L, Gelfand Michael J, Rozhkov Leonid, Fujiwara Hisako, Rose Douglas F, Franz David N, Mangano Francesco T

机构信息

Divisions of Neurology.

出版信息

J Neurosurg Pediatr. 2015 Jan;15(1):26-33. doi: 10.3171/2014.10.PEDS14107.

Abstract

OBJECT

Tuberous sclerosis complex (TSC) with medically refractory epilepsy is characterized by multifocal brain abnormalities, traditionally indicating poor surgical candidacy. This single-center, retrospective study appraised seizurerelated, neuropsychological, and other outcomes of resective surgery in TSC patients with medically refractory epilepsy, and analyzed predictors for these outcomes.

METHODS

Patients with multilesional TSC who underwent epilepsy surgery between 2007 and 2012 were identified from an electronic database. All patients underwent multimodality noninvasive and subsequent invasive evaluation. Seizure outcomes were classified using the International League Against Epilepsy (ILAE) scale. The primary outcome measure was complete seizure remission (ILAE Class 1). Secondary outcome measures included 50% responder rate, change in full-scale IQ, electroencephalography improvement, and reduction in antiepileptic drug (AED) burden.

RESULTS

A total of 37 patients with TSC underwent resective surgery during the study period. After a mean follow-up of 5.68 ± 3.67 years, 56.8% achieved complete seizure freedom (ILAE Class 1) and 86.5% had ILAE Class 4 outcomes or better. The full-scale IQ on follow-up was significantly higher in patients with ILAE Class 1 outcome (66.70 ± 12.36) compared with those with ILAE Class 2 or worse outcomes (56.00 ± 1.41, p = 0.025). In 62.5% of the patients with ILAE Class 2 or worse outcomes, the number of AEDs were found to be significantly reduced (p = 0.004).

CONCLUSIONS

This study substantiates the evidence for efficacy of resective epilepsy surgery in patients with bilateral multilesional TSC. More than half of the patients were completely seizure free. Additionally, a high proportion achieved clinically meaningful reduction in seizure burden and the number of AEDs.

摘要

目的

结节性硬化症(TSC)伴药物难治性癫痫的特征是多灶性脑异常,传统上提示手术候选性差。这项单中心回顾性研究评估了TSC伴药物难治性癫痫患者接受切除性手术后的癫痫相关、神经心理学及其他结局,并分析了这些结局的预测因素。

方法

从电子数据库中识别出2007年至2012年间接受癫痫手术的多灶性TSC患者。所有患者均接受了多模态非侵入性及后续侵入性评估。癫痫结局采用国际抗癫痫联盟(ILAE)量表进行分类。主要结局指标为癫痫完全缓解(ILAE 1级)。次要结局指标包括50%反应率、全量表智商变化、脑电图改善情况以及抗癫痫药物(AED)负担减轻情况。

结果

研究期间共有37例TSC患者接受了切除性手术。平均随访5.68±3.67年后,56.8%的患者实现了癫痫完全缓解(ILAE 1级),86.5%的患者达到了ILAE 4级或更好的结局。与ILAE 2级或更差结局的患者相比,ILAE 1级结局的患者随访时的全量表智商显著更高(66.70±12.36)(56.

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