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局灶性皮质发育不良且癫痫发作始于第一年的儿童中的局灶性发作与癫痫性痉挛

Focal seizures versus epileptic spasms in children with focal cortical dysplasia and epilepsy onset in the first year.

作者信息

Serino Domenico, Freri Elena, Ragona Francesca, D'Incerti Ludovico, Bernardi Bruno, Di Ciommo Vincenzo, Granata Tiziana, Vigevano Federico, Fusco Lucia

机构信息

Ospedale Pediatrico Bambino Gesù, P.zza S. Onofrio 4, 00165 Rome, Italy.

Istituto Neurologico Carlo Besta, Via Luigi Mangiagalli 3, 20133 Milan, Italy.

出版信息

Epilepsy Res. 2015 Jan;109:203-9. doi: 10.1016/j.eplepsyres.2014.11.006. Epub 2014 Nov 26.

Abstract

PURPOSE

Focal cortical dysplasia (FCD) has been recognized as one of the most frequent causes of drug resistant epilepsy, especially in children. In infancy, onset of FCD-related epilepsy is substantially characterized by epileptic spasms (ES) or focal seizures. Which elements pertaining to the FCD are responsible for the onset of one type of seizure over the other is still unclear. Purpose of our study was to compare the characteristics of FCDs in terms of lateralization and site in patients with epileptic spasms versus patients with focal seizures.

METHODS

We retrospectively reviewed data from 41 patients with FCD related epilepsy with onset during the first 14 months of life. Seizure semeiology and drug resistance were analyzed, as were age at onset and FCD site and lateralization.

RESULTS

Twenty-one children had focal seizures, 11 had ES and nine had focal seizures followed by ES. Mean age at onset was respectively 8.2, 5.1 and 1.8 months. Drug resistance was present in respectively 38.5%, 34.6% and 26.9% of children. Among patients with only ES, 90.9% had an exclusively frontal FCD localization, versus 42.9% of patients with focal seizures and 11.1% of patients with focal seizures followed by ES. FCD lateralization was right sided respectively in 47.6%, 81.8% and 66.7% of patients.

CONCLUSIONS

Frontal lobe localization of FCDs was closely associated with ES (p=0.001). Moreover we also found that patients with focal seizures followed by ES had a significantly earlier age at onset compared to patients with focal seizures only (p<0.001). The association between ES and right-sided FCD lateralization, even if numerically suggestive, did not reach statistical significance (p=0.16). There was no significant association between seizure type and drug resistance (p=0.08).

摘要

目的

局灶性皮质发育不良(FCD)已被公认为药物难治性癫痫最常见的病因之一,尤其是在儿童中。在婴儿期,FCD相关癫痫的发作主要表现为癫痫痉挛(ES)或局灶性发作。FCD的哪些因素导致一种类型的发作而非另一种类型的发作仍不清楚。我们研究的目的是比较癫痫痉挛患者与局灶性发作患者FCD在定位和部位方面的特征。

方法

我们回顾性分析了41例在出生后14个月内发病的FCD相关癫痫患者的数据。分析了发作症状学和药物难治性,以及发病年龄、FCD部位和定位。

结果

21例儿童有局灶性发作,11例有ES,9例先有局灶性发作后有ES。平均发病年龄分别为8.2个月、5.1个月和1.8个月。药物难治性分别出现在38.5%、34.6%和26.9%的儿童中。仅患有ES的患者中,90.9%的FCD仅定位于额叶,而局灶性发作患者为42.9%,先有局灶性发作后有ES的患者为11.1%。FCD定位在右侧的患者分别占47.6%、81.8%和66.7%。

结论

FCD的额叶定位与ES密切相关(p = 0.001)。此外,我们还发现,先有局灶性发作后有ES的患者发病年龄明显早于仅有局灶性发作的患者(p < 0.001)。ES与右侧FCD定位之间的关联,即使在数值上有提示意义,但未达到统计学显著性(p = 0.16)。发作类型与药物难治性之间无显著关联(p = 0.08)。

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