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基于人群的婴儿癫痫综合征研究尚不足。该研究的目的在于,从人群中一组出生后1年内起病的癫痫患儿中,提炼出有关综合征发病率及预后的数据。纳入1997年-2006年间住在赫尔辛基大学医院服务区域的所有12月龄内起病的癫痫患儿。通过查看所有患儿病历资料,根据医院的统计数据确认可否纳入研究。再对从患儿病历资料中总结出癫痫综合征、起病年龄、病因,以及在24个月龄时的结局进行了再评估。共158例婴儿达到了纳入标准,其中92%随访满24个月或死亡。出生后第1年的癫痫发病率为124/10万。根据国际抗癫痫联盟(ILAE)癫痫分类标准修正版,其中58%可被归类为某种癫痫综合征。最常见的癫痫综合征为West综合征(41/10万)和良性家族性或非家族性婴儿癫痫(22/10万)。35%的病因为结构-代谢性,17%为基因性,还有48%病因不明。起病早与结构-代谢性病因相关。有7例(4.4%)患儿在2岁以前死亡。1例有SCN2A基因突变的患儿死于癫痫猝死。研究显示良性家族性及非家族性婴儿癫痫比以往的估计更为高发,仅次于West综合征。起病早并不是预后差的独立危险因素。
Population-based infant epilepsy syndrome is not enough. The aim of the study was to extract data on the incidence and prognosis of the syndrome from a group of children with epilepsy who developed disease within one year of birth. Including all children with epilepsy within 12 months of age who were hospitalized in Helsinki University Hospital between 1997 and 2006. Through the view of all children’s medical records, according to the hospital’s statistics to confirm whether the study can be included. A review of epilepsy syndromes, age of onset, etiology, and outcome at 24 months of age was summarized from the pediatric history data. A total of 158 infants met the inclusion criteria, 92% of them were followed up for 24 months or died. The first year after birth, the incidence of epilepsy 124 / 100,000. According to the revised edition of the International Epilepsy League (ILAE) Epilepsy Classification Standard, 58% of them can be classified as some kind of epilepsy syndrome. The most common epilepsy syndromes were West syndrome (41/10 million) and benign familial or non-familial infantile epilepsy (22/10 million). 35% of the cause of the structure - metabolic, 17% of the genetic, and 48% of the etiology. Early onset is associated with structural-metabolic etiology. Seven (4.4%) children died before age two. One patient with SCN2A mutation died of sudden death from epilepsy. Studies have shown that benign and non-familial infantile epilepsy is more frequent than previously estimated, second only to West syndrome. Early onset is not an independent risk factor for poor prognosis.