Trevathan E, Murphy C C, Yeargin-Allsopp M
Pediatric Epilepsy Center, Department of Neurology, Washington University School of Medicine, St. Louis Children's Hospital, Missouri 63110, USA.
Epilepsia. 1999 Jun;40(6):748-51. doi: 10.1111/j.1528-1157.1999.tb00773.x.
To determine the population-based epidemiology of infantile spasms (IS) among Atlanta children.
By using data from a cross-sectional, population-based surveillance system that included 21 EEG laboratories, we identified children born in 1975-1977 in metropolitan Atlanta with IS. Cumulative incidence up to age 2 years was estimated from the number of children with IS born in the study area in 1975-1977, and age-specific prevalence was calculated from the number of children previously diagnosed with IS who lived in the study area at age 10 years. Data regarding coexisting disabilities were available from the surveillance system for developmental disabilities.
The cumulative incidence of IS was 2.9/10,000 live births; half of the children with IS had cryptogenic IS. The age-specific prevalence of IS was 2.0/10,000 among 10-year-old children. Eighty-three percent of 10-year-old children with a history of IS had mental retardation (MR, IQ < or =70); 56% of children with a history of IS had profound MR (IQ <20). Developmental outcome did not differ between the children with cryptogenic IS and those with symptomatic IS. Among the 10-year-old children with profound MR who were living in Atlanta at age 10 years, 12% had a history of IS. Fifty percent of children with IS developed Lennox-Gastaut syndrome (LGS) before age 11 years.
The syndrome of IS is rare in the general population, yet a significant percentage of all children with profound MR and severe childhood epilepsy syndromes in the general population have a history of IS.
确定亚特兰大儿童中婴儿痉挛症(IS)基于人群的流行病学特征。
利用一个横断面、基于人群的监测系统的数据,该系统包括21个脑电图实验室,我们确定了1975 - 1977年在大亚特兰大地区出生且患有IS的儿童。根据1975 - 1977年在研究区域出生的患有IS的儿童数量估算至2岁的累积发病率,并根据10岁时居住在研究区域且先前被诊断为IS的儿童数量计算特定年龄患病率。关于并存残疾的数据可从发育障碍监测系统获得。
IS的累积发病率为2.9/10000活产;患有IS的儿童中有一半为隐源性IS。10岁儿童中IS的特定年龄患病率为2.0/10000。有IS病史的10岁儿童中83%有智力障碍(MR,智商≤70);有IS病史的儿童中56%有重度智力障碍(智商<20)。隐源性IS儿童和症状性IS儿童的发育结局无差异。在10岁时居住在亚特兰大且有重度智力障碍的10岁儿童中,12%有IS病史。50%的IS儿童在11岁前发展为Lennox - Gastaut综合征(LGS)。
IS综合征在普通人群中罕见,但普通人群中所有患有重度智力障碍和严重儿童癫痫综合征的儿童中有相当比例有IS病史。