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The magnitude and causes of death among a cohort of children with epilepsy were determined. A follow-up study with a population-based cohort of 10-year-old children in the metropolitan Atlanta area with epilepsy was conducted. The National Death Index and linkage to State of Georgia death certificates were used to identify deaths. The authors estimated the expected numbers of deaths by applying mortality rates adjusted by age, race, and sex for the entire state of Georgia to the population for the follow-up period. Among the 688 children who were in the final epilepsy cohort, 64 deaths occurred; 20.6 deaths were expected (mortality ratio adjusted for age, race, and sex = 3.11). The mortality ratios for children with Lennox-Gastaut syndrome and infantile spasms were 13.92 and 11.91, respectively. Children and adolescents with epilepsy, especially those with Lennox-Gastaut syndrome or infantile spasms, have an increased risk of death.

Purpose: To determine the population-based epidemiology of infantile spasms (IS) among Atlanta children., Methods: 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., Results: 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., Conclusions: 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.

Purpose: To determine the prevalence and descriptive epidemiology of Lennox-Gastaut Syndrome (LGS) among metropolitan Atlanta children., Methods: We conducted a population-based study of LGS as part of the Metropolitan Atlanta Developmental Disabilities Study (MADDS) using a multiple-source surveillance system for epilepsy and developmental disabilities. Children were defined as having LGS if they had onset of multiple seizure types before age 11 years, with at least one seizure type resulting in falls, and an EEG demonstrating slow spike-wave complexes (<2.5 Hz). Mental retardation (MR) was not used as a diagnostic criterion., Results: The lifetime prevalence of LGS at age 10 years was 0.26/1,000. Ninety-one percent of those with LGS had MR (IQ < or = 70), and 39% had a history of infantile spasms (IS). A comparison of children with LGS and those with multiple seizure types without slow spike-wave complexes demonstrated that those with LGS were more likely to have MR, history of IS, and multiple disabilities (MR, cerebral palsy, blindness, hearing impairment). Seventeen percent of all children in Atlanta with profound MR (IQ < 20) had LGS., Conclusions: LGS accounts for only 4% of all childhood epilepsy, yet is a significant contributor to childhood morbidity.