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[与继发性全面强直-阵挛发作相关的儿童癫痫综合征]

[Epileptic syndromes in childhood associated with secondary generalized tonic-clonic seizures].

作者信息

Mironov M B, Burd S G, Klimov Yu A, Krasilshikova T M, Bobylova M Yu, Rubleva Yu V, Gunchenko M M, Sarzhina M N, Batysheva T T

机构信息

Institute for Advanced Studies, Moscow, Russia; Research Center of Child Psychoneurology, Moscow, Russia.

Pirogov Russian National Research Medical University, Moscow, Russia; Research Center of Child Psychoneurology, Moscow, Russia.

出版信息

Zh Nevrol Psikhiatr Im S S Korsakova. 2017;117(11. Vyp. 2):23-32. doi: 10.17116/jnevro201711711223-32.

Abstract

AIM

To study a group of patients with secondary generalized tonic-clonic seizures (SGTCS) in view of nosology, medical history, clinical, electroencephalographic and neuroimaging features.

MATERIAL AND METHODS

The study included 471 patients, 244 (51.8%) men and 227 (48.2%) women.

RESULTS

SGTCS were observed in many epileptic syndromes. The most frequent were symptomatic focal epilepsy (33.8%), cryptogenic focal epilepsy (23.8%), rolandic epilepsy (12.6%), FEBL-BEDC syndrome (12.3%). Other forms of epilepsy were less frequent. The onset of epilepsy ranged over a wide age range from the first month of life to 18 years. The average age of onset was 5.7±4.96 years. SGTCS as the only type of paroxysms were observed in 28.3% of cases. Two or more types of seizures were observed in 71.7% of patients, three or more types in 39.3%. Epileptiform activity on EEG during long VEM was detected in 91.3% of patients with SGTCS. In 37.2% of patients, benign epileptiform discharges of childhood were recorded. Treatment with antiepileptic drugs (AEP) led to complete remission in 57.1% of cases of epilepsy associated with SGTCS. A reduction of the frequency of seizures by 50% or more was found in 33.6% of patients treated with AEP. No effect was observed in 9.3% of patients.

CONCLUSION

Significant differences in the prognosis and therapeutic approaches to specific epileptic syndromes associated with SGTCS necessitate the use of the entire spectrum of diagnostic measures, which should include careful history taking, clinical examination, video-EEG monitoring with the inclusion of sleep dynamics, MRI / CT brain, genetic testing.

摘要

目的

从疾病分类学、病史、临床、脑电图及神经影像学特征方面研究一组继发性全面性强直阵挛发作(SGTCS)患者。

材料与方法

该研究纳入471例患者,其中男性244例(51.8%),女性227例(48.2%)。

结果

SGTCS可见于多种癫痫综合征。最常见的是症状性局灶性癫痫(33.8%)、隐源性局灶性癫痫(23.8%)、罗兰多癫痫(12.6%)、FEBL - BEDC综合征(12.3%)。其他癫痫形式较少见。癫痫发作起始年龄范围广泛,从出生后第一个月至18岁。平均发病年龄为5.7±4.96岁。SGTCS作为唯一发作类型见于28.3%的病例。71.7%的患者观察到两种或更多类型的发作,39.3%的患者观察到三种或更多类型的发作。在91.3%的SGTCS患者中,长程视频脑电图监测(VEM)期间检测到癫痫样活动。37.2%的患者记录到儿童良性癫痫样放电。抗癫痫药物(AEP)治疗使57.1%与SGTCS相关的癫痫病例完全缓解。接受AEP治疗的患者中,33.6%发作频率降低50%或更多。9.3%的患者未观察到效果。

结论

与SGTCS相关的特定癫痫综合征在预后和治疗方法上存在显著差异,因此需要采用全面的诊断措施,包括仔细的病史采集、临床检查、纳入睡眠动态的视频脑电图监测、头颅MRI/CT、基因检测。

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