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结节性硬化症幼儿难治性癫痫:早期手术治疗及预后。

Refractory epilepsy in preschool children with tuberous sclerosis complex: Early surgical treatment and outcome.

机构信息

Pediatric Neurosurgery Unit, Rothschild Foundation Hospital, Paris, France.

Pediatric Neurosurgery Unit, Rothschild Foundation Hospital, Paris, France.

出版信息

Seizure. 2018 Aug;60:71-79. doi: 10.1016/j.seizure.2018.06.005. Epub 2018 Jun 18.

DOI:10.1016/j.seizure.2018.06.005
PMID:29929109
Abstract

PURPOSE

Epilepsy surgery has been shown to be effective in treating focal epilepsy related to tuberous sclerosis complex (TSC). We analyzed the advantage of early surgical management in terms of seizure frequency and development.

METHOD

We retrospectively studied the 15 patients younger than 6 years who underwent resective surgery between 2006 and 2016. Fourteen of them had invasive monitoring while the 15th was operated on under corticography.

RESULTS

Epilepsy began before 5 months of age in all patients. Overall 13 patients (86%) had a dramatic improvement of epilepsy after surgery (Engel 1 and 2) including 9 patients (60%) seizure free (Engel 1 A). In the group of 9 patients younger than 20 months at the time of surgery who presented with catastrophic epilepsies, 77% are Engel 1 A and the other 23% Engel 2. In this subpopulation, no one developed autism and four (44%) regained normal development.

CONCLUSIONS

In early onset epilepsies associated with TSC, surgical treatment is highly effective, in particular when performed early. Invasive monitoring contributes to the successful outcome. Those data have to be confirmed by multicentric studies including quantitative analyses of the recordings.

摘要

目的

癫痫手术已被证明可有效治疗与结节性硬化症(TSC)相关的局灶性癫痫。我们分析了早期手术治疗在发作频率和发育方面的优势。

方法

我们回顾性研究了 2006 年至 2016 年间接受切除术的 15 名年龄小于 6 岁的患者。其中 14 名患者进行了有创监测,而第 15 名患者则在皮质图下进行了手术。

结果

所有患者的癫痫均在 5 个月之前发作。总体而言,13 名患者(86%)术后癫痫明显改善(Engel 1 和 2),包括 9 名患者(60%)无发作(Engel 1A)。在手术时年龄小于 20 个月且患有灾难性癫痫的 9 名患者中,77%为 Engel 1A,23%为 Engel 2。在这一亚组中,没有患者出现自闭症,44%的患者恢复了正常发育。

结论

在与 TSC 相关的早发性癫痫中,手术治疗非常有效,尤其是早期进行时。有创监测有助于获得良好的结果。这些数据需要通过包括记录的定量分析在内的多中心研究来证实。

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