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[与局灶性阵挛性发作相关的癫痫综合征]

[Epileptic syndromes associated with focal clonic seizures].

作者信息

Mironov M B, Chebanenko N V, Zykov V P, Bychenko V G, Mednaya D M, Krasilshchikova T M, Milovanova O A

机构信息

Russian Medical Academy of Continuous Professional Education, Moscow, Russia.

Kulakov Research Center for Obstetrics, Gynecology and Perinatology, Moscow, Russia.

出版信息

Zh Nevrol Psikhiatr Im S S Korsakova. 2023;123(3):41-45. doi: 10.17116/jnevro202312303141.

Abstract

OBJECTIVE

To study clinical, electroencephalographic and neuroimaging features in children with epileptic syndromes associated with focal clonic seizures (FCS).

MATERIAL AND METHODS

We examined 1258 patients with various forms of epilepsy with the onset of seizures from the first day of life to 18 years.

RESULTS

FCS was identified in 263 patients (20.9%). FCS were included in the structure of 13 different epileptic syndromes: Rolandic epilepsy (28.1%), structural focal epilepsy (27.5%), structural focal epilepsy associated with benign epileptiform discharges of childhood (SFE-BEDC) (20.6%), focal epilepsy of unknown etiology (7.5%), epilepsia partialis continua (4.6%), pseudo-Lennox syndrome (3.4%), ESES syndrome (2.7%), Landau-Kleffner syndrome (1.5%), Dravet syndrome (1.1%), benign occipital epilepsy (1.1%), benign focal epilepsy in infancy (0.8%), MISF syndrome (0.8%), cognitive epileptiform disintegration (0.8%). In 50% of cases, epilepsy associated with FCS debuts before the age of 5 years (from 1 month to 18 years, average age 4.26±3.9).

CONCLUSION

The groups of syndromes associated with FCS have different prognosis for remission of seizures. Prognostic predictors of seizure remission are: epileptic syndromes associated with BEDC, the presence of periventricular leukomalacia. A severe prognosis for the course of epilepsy is associated with local structural changes in the neocortex. Despite a favorable prognosis for seizures, continued diffuse interictal epileptiform activity with BEDC on the electroencephalogram is a predictor of the onset of cognitive impairment in children.

摘要

目的

研究伴有局灶性阵挛发作(FCS)的癫痫综合征患儿的临床、脑电图及神经影像学特征。

材料与方法

我们检查了1258例各种形式癫痫的患者,其癫痫发作始于出生第一天至18岁。

结果

263例患者(20.9%)被诊断为FCS。FCS包含于13种不同癫痫综合征的结构中:罗兰多癫痫(28.1%)、结构性局灶性癫痫(27.5%)、与儿童期良性癫痫样放电相关的结构性局灶性癫痫(SFE - BEDC)(20.6%)、病因不明的局灶性癫痫(7.5%)、持续性部分性癫痫(4.6%)、假性 Lennox 综合征(3.4%)、ESES 综合征(2.7%)、Landau - Kleffner 综合征(1.5%)、Dravet 综合征(1.1%)、良性枕叶癫痫(1.1%)、婴儿期良性局灶性癫痫(0.8%)、MISF 综合征(0.8%)、认知性癫痫样解体(0.8%)。50%的病例中,与FCS相关的癫痫在5岁前起病(年龄范围从1个月至18岁,平均年龄4.26±3.9岁)。

结论

与FCS相关的综合征组癫痫发作缓解的预后不同。癫痫发作缓解的预后预测因素为:与BEDC相关的癫痫综合征、脑室周围白质软化的存在。癫痫病程预后不良与新皮质的局部结构改变有关。尽管癫痫发作预后良好,但脑电图上持续存在伴有BEDC的弥漫性发作间期癫痫样活动是儿童认知障碍发作的一个预测因素。

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