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首页> 外文期刊>European journal of paediatric neurology: EJPN : official journal of the European Paediatric Neurology Society >Typical absence epilepsy presenting prior to age of 3 years: An uncommon form of idiopathic generalized epilepsy.
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Typical absence epilepsy presenting prior to age of 3 years: An uncommon form of idiopathic generalized epilepsy.

机译：3岁之前出现的典型失神癫痫：特发性全身性癫痫的罕见形式。

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PURPOSE: An attempt to allocate patients with the clinical features and electroencephalography (EEG) abnormalities of typical absence epilepsy presenting before the age of 3 years, similar to childhood and juvenile absence epilepsy (JAE) and delineate the clinical manifestations, EEG abnormalities, therapy and outcome of such an epileptic disorder by conducting a nationwide survey. RESULTS: Overall, eight infants, six males and two females, abided by the inclusion criteria of typical absence epilepsy: They were born after an unremarkable pregnancy and labor presenting at the age of 12-34 months (mean: 19.6 months) with frequent absences time-linked with an EEG demonstrating generalized occasionally irregular epileptiform discharges of 3-4Hz spike/wave and normal background activity along with an electrographic photosensitive response in one patient. Neurological examination was intact in all infants. All eight infants were initially treated with valproic acid, of whom seven immediately responded and one had increase in frequency and duration of absences completely aborted with treatment of lamotrigine. Three relapsed after termination of therapy of whom two again presented with recurrent absences and another one with generalized tonic-clonic seizures and as such these children had virtually transformed into a later form of idiopathic generalized epilepsy (IGE) during childhood. All eight patients are seizure-free, seven still on therapy; seven children within a follow-up period of 2-7 years and the most recently diagnosed infant for 6 months. Cognitive skills were found normal in all children within the low normal range in three children with short attention and concentration spans. CONCLUSION: The data presented here delineate a very rare form of idiopathic benign generalized epilepsy presenting with typical absences before age of 3 years and a favorable outcome, similar to childhood and JAE, recognized as distinct IGE syndromes by the International League Against Epilepsy (ILAE) classification.
机译：目的：尝试分配具有3岁之前出现的典型失神癫痫的临床特征和脑电图（EEG）异常的患者，类似于儿童和青少年失神癫痫（JAE），并描述临床表现，EEG异常，治疗方法和通过进行全国性调查得出这种癫痫症的后果。结果：总体上，有8例婴儿，6例男性和2例女性，遵循典型的失神癫痫的纳入标准：他们出生于平常的妊娠后，分娩的年龄为12-34个月（平均：19.6个月），且经常缺勤。与脑电图的时间相关性，表明一名患者中偶尔出现不规则的癫痫样放电，频率为3-4Hz尖峰/波，背景活动正常，以及电子照相感光反应。所有婴儿的神经系统检查均完好无损。最初，所有8名婴儿均接受丙戊酸治疗，其中7名立即反应，其中1名因拉莫三嗪治疗而完全中止的频率和持续时间增加。终止治疗后有3例复发，其中2例再次出现反复失神，另外1例出现全身性强直-阵挛性癫痫发作，因此，这些儿童实际上已在儿童时期转变为后来的特发性全身性癫痫（IGE）。所有八名患者均无癫痫发作，其中七名仍在接受治疗; 7名儿童在2-7岁的随访期内，最近被诊断为6个月的婴儿。在三名注意力和注意力集中时间较短的儿童中，所有儿童的认知技能均处于正常范围的低位。结论：此处提供的数据描述了一种非常罕见的特发性良性全身性癫痫，表现为3岁之前的典型缺席，并且有良好的结局，类似于儿童期和JAE，被国际抗癫痫联盟（ILAE）识别为独特的IGE综合征分类。

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