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TSC 1基因突变婴儿局灶性癫痫持续状态加癫痫性痉挛的脑白质切断术治疗:病例报告

Treatment of Focal Status Epilepticus plus Epileptic Spasms with Leukotomy in an Infant with TSC 1 Mutation: A Case Study.

作者信息

Park Jun T, Shahid Asim M, Miller Jonathan P, Lüders Hans O

出版信息

Neurodiagn J. 2015 Dec;55(4):251-7. doi: 10.1080/21646821.2015.1089700.

DOI:10.1080/21646821.2015.1089700
PMID:26793902
Abstract

BACKGROUND

Focal status epilepticus and catastrophic epilepsy are not rare in infants. Epilepsy surgery can be safely done in selected infants to cure epilepsy.

PATIENT DESCRIPTION

We report on an infant who began having drug-resistant status epilepticus at 2 weeks of age and developed epileptic spasms. We discuss in detail how the clinical and electroencephalographic data were used to reach a consensus for epilepsy surgery and why a particular surgical approach was preferred over other alternatives.

METHOD

Presurgical evaluation consisted of 32-channel scalp video EEG using the standard 10-20 system of electrodes, 3-Tesla brain magnetic resonance imaging, and 18 F-fluorodeoxyglucose positron emission tomography. The surgery consisted of resection of the extensive epileptogenic lesion, in addition to disconnection of the left frontal lobe anterior to the motor strip.

RESULT

The infant underwent epilepsy surgery at three months of age. At two-year follow up, she remained seizure free, with no focal motor deficit and the epileptic encephalopathy resolved. The disconnected left frontal lobe shows epileptiform discharges restricted to the disconnected tissue.

CONCLUSION

We highlight the importance of epilepsy surgery in selected infants to achieve seizure freedom and to reverse epileptic encephalopathy. In the process, we demonstrate how epileptic spasms, although clinically and electrographically generalized, resolved after disconnecting the epileptogenic zone.

摘要

背景

局灶性癫痫持续状态和灾难性癫痫在婴儿中并不罕见。癫痫手术可以在选定的婴儿中安全地进行以治愈癫痫。

患者描述

我们报告了一名婴儿,其在2周龄时开始出现耐药性癫痫持续状态并发展为癫痫痉挛。我们详细讨论了如何利用临床和脑电图数据就癫痫手术达成共识,以及为什么选择特定的手术方法而非其他替代方法。

方法

术前评估包括使用标准10-20电极系统的32通道头皮视频脑电图、3特斯拉脑磁共振成像和18F-氟脱氧葡萄糖正电子发射断层扫描。手术包括切除广泛的致痫灶,此外还切断运动带前方的左额叶。

结果

该婴儿在3个月大时接受了癫痫手术。在两年的随访中,她无癫痫发作残留,无局灶性运动功能缺损,癫痫性脑病得到缓解。切断的左额叶显示癫痫样放电局限于切断的组织。

结论

我们强调了癫痫手术在选定婴儿中实现无癫痫发作和逆转癫痫性脑病的重要性。在此过程中我们证明,癫痫痉挛尽管在临床和脑电图上表现为全身性,但在切断致痫区后得以缓解。

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Treatment of Focal Status Epilepticus plus Epileptic Spasms with Leukotomy in an Infant with TSC 1 Mutation: A Case Study.TSC 1基因突变婴儿局灶性癫痫持续状态加癫痫性痉挛的脑白质切断术治疗:病例报告
Neurodiagn J. 2015 Dec;55(4):251-7. doi: 10.1080/21646821.2015.1089700.
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