Children with epilepsy often experience poor social and educational outcomes. This study aimed to determine the timing of services with respect to the onset of seizures. It also aimed to identify the aspects of childhood epilepsy (type of epilepsy, etiology, seizure control, and treatment) that are associated with the use of special education services. As part of a prospective community-based study, 613 children were recruited when first diagnosed with epilepsy. Mean age at first seizure was 5 years 11 months (SD 4, range 1mo to 15y 8mo). Parents were interviewed 5 years after children were first diagnosed with epilepsy (n=542; 276 [51%] males). Children's mean age at time of interview was 11 years 10 months (SD 4y 1mo, range 5y 8mo to 21y 8mo). Etiology was classified as idiopathic (n=181, 33.4%), cryptogenic (n=261, 48.2%), and remote symptomatic (n=100, 18.5%). Service use was reported in 315 (58%) children. Compared with neurologically intact children (i.e. cryptogenic and idiopathic etiology; n=415, 77%), children with a remote symptomatic etiology and/or an epileptic encephalopathy (n=127, 23%) received services more frequently (88% vs 49%, p<0.001). In the former group, services were initiated for 66 (15%) children before their first seizure; according to age at onset, services were initiated before the first seizures in 12/164 (7.3%) if <5 years, 34/171 (19.9%) if 5-9 years, and 20/80 (25%) if >10 years. A large proportion of children with epilepsy, even if neurologically otherwise normal, receive special education services. Initiation of services often precedes onset of seizures even in neurologically intact children. This suggests that behavioral and cognitive abnormalities may predate the onset of epilepsy and are not necessarily the direct consequences of epilepsy.

OBJECTIVES To evaluate mortality in children with newly diagnosed epilepsy, to determine the risk of death, and to identify predictors of death from the point of diagnosis. DESIGN Prospective community-based cohort of 613 children with newly diagnosed epilepsy. The outcome measure was death. Chi2 Tests were used for bivariate analyses and the Cox proportional hazards model for multivariable analyses. Standardized mortality ratios were used to quantify the excess mortality in the cohort relative to the population. RESULTS Thirteen deaths occurred during 4733 person-years of follow-up, for a crude death rate of 2.7 per 1000 person-years (0.52 per 1000 person-years in those with nonsymptomatic epilepsy and 12.6 per 1000 person-years in those with symptomatic epilepsy). Ten deaths were associated with the underlying cause of the seizures, 2 were associated with the occurrence or probable occurrence of seizures, and 1 was unrelated to seizures or the underlying disorder. On multivariable analysis, symptomatic etiology (rate ratio, 10.2; 95% confidence interval [CI], 2.1-49.6) and epileptic encephalopathy (rate ratio, 13.3; 95% CI, 3.4-51.7) were independently associated with mortality. The overall standardized mortality ratio for the cohort was 7.54 (95% CI, 4.38-12.99). In children with symptomatic epilepsy, the standardized mortality ratio was 33.46 (95% CI, 18.53-60.43), and in those with nonsymptomatic epilepsy, it was 1.43 (95% CI, 0.36-5.73). CONCLUSIONS Children with epilepsy have an increased risk of death. Most deaths occur in children with severe underlying conditions and are not directly related to the occurrence of seizures.

Objectives: To determine the risk and predictors of status epilepticus in children after they have been diagnosed with epilepsy. Methods: In a prospective community-based cohort study of 613 children, the occurrence of status epilepticus after the initial diagnosis of epilepsy was ascertained. Parents were called every 3 months, and interval medical records were reviewed every 6 months. Predictors of primary interest included a history of status before the diagnosis of epilepsy, age at onset, underlying etiology, and epilepsy syndrome. Data were analyzed with chi(2) tests, Kaplan-Meier analyses, and Cox proportional hazards models. Results: Of 613 children followed a median of 8.0 years, 58 (9.5%) had greater than or equal to1 episode of status epilepticus during follow-up evaluation. The first episode occurred a median of 2.5 years after initial diagnosis (range, <1 month to 8.8 years). A history of previous status epilepticus was strongly associated with subsequent status epilepticus (18/56 [32.1%] vs 40/557 [7.2%]; p<0.0001). Younger age at onset and symptomatic etiology contributed independently to the risk of status epilepticus. Mortality was higher in children with status epilepticus before diagnosis, largely secondary to underlying cause. Conclusions: Status epilepticus occurs in similar to10% of children after initial diagnosis of epilepsy. Status epilepticus before initial diagnosis, young age at onset, and symptomatic etiology independently influence the risk of status epilepticus. In those without status epilepticus before diagnosis, the risk is modest and is realized over a prolonged period. For children at highest risk, maintaining abortive therapy in the home may be a reasonable precaution.

Objectives. The outcomes of childhood-onset epilepsy are highly varied and have several potential determinants. We examined the independent effects of syndrome type, seizure control, and etiology over time on adaptive behavior as measured by the Vineland Scales of Adaptive Behavior. Methods. As part of a prospective community-based study of newly diagnosed epilepsy, parents of children who were younger than 3 years at the time of initial onset of epilepsy completed the Vineland Adaptive Behavior Scales screener version at entry into the study and once a year thereafter for up to 3 years. Longitudinal analyses were performed on the composite score as the primary outcome and on the 4 domain scores (communication, socialization, motor, and daily living) as confirmatory/secondary outcomes to determine the effects of syndrome (epileptic encephalopathy or other), seizure control (intractable or not), and etiology (symptomatic or not) on adaptive behavior at the time of initial diagnosis and over time. Results. A total of 613 children were enrolled in the study, and 191 (31%) of these children met the age criterion for this analysis. Of these, 172 (90%) had adequate follow-up and had completed baseline and at least 1 subsequent Vineland assessment. Overall, Vineland scores (composite and individual domains) were somewhat below average at baseline (initial diagnosis). All declined significantly over time. All of the effects at baseline, however, were limited to children with epileptic encephalopathies and symptomatic etiology. Substantial declines over time occurred in these children, and there was an independent effect of intractable seizures as well. In children with none of these factors (∼75% of the study group), baseline scores were consistent with average performance for the test norms and there was no evidence of any decline over time. Conclusions. Children with an underlying symptomatic etiology or a syndrome that can be characterized as 1 of the epileptic encephalopathies demonstrate impaired adaptive behavior at the time of initial diagnosis and experience additional declines in assessments of age-adjusted performance over time. Our results suggest that future seizure outcome is not strongly reflected in adaptive behavior at initial diagnosis but that it takes its toll on the child over time. Understanding how each of these factors affects development and how they interact with each other is the next step in designing effective interventions for lessening the impact of these disorders on the child. In the majority of children with onset of epilepsy during infancy or early childhood, adaptive behavior is within the normal range and does not show any evidence of declining over time. Although this is encouraging, it does not contradict other studies that have demonstrated behavioral and relatively subtle cognitive difficulties in school-aged children with epilepsy. Long-term follow-up in this cohort will permit us to examine the predictive value of the Vineland for later behavioral and cognitive difficulties in this group that, so far, seems to be doing well.

Due to a printing error, in the above article, Table 1 and Table 4 were incorrectly represented. Those tables should have appeared as shown here. We apologize to the authors for this error.

PURPOSE:To examine different approaches to classifying seizure outcomes. METHODS:In a prospective cohort study of children (N=613) with newly diagnosed epilepsy, seizure outcomes at 2 years were classified as 'good' (> or =1 year remission), 'bad' or 'intractable' (> or = AED failures, > or =1 seizure/month over > or =18 months), and 'indeterminate' (neither 'good' nor 'bad'). Outcomes at 2 years were compared to outcomes in those followed 4 or more years. The associations of three commonly studied prognostic factors, etiology, age at onset, and syndromic grouping with the three-level outcome were assessed. RESULTS:595 (97.1%) children were followed > or =2 years. A 'good', indeterminate, and 'bad' outcome was present in 314 (52.8%), 235 (38.3%), and 46 (7.7%) children. Problems with treatment were recorded in 64.7% of the indeterminate group. In 390 children followed > or =4 years, early 'good' and 'bad' outcomes persisted in approximately 80%. About half of those with indeterminate 2-year outcomes later achieved remission, 8% met criteria for intractability, and 37% remained indeterminate. Most of the associations with etiology, age, and syndrome were due to variation in the proportion that met criteria for intractability and not remission. CONCLUSIONS:Many children have indeterminate outcomes, often in association with treatment issues. Clearly 'good' and 'bad' early outcomes can be identified and persist > or =2 years later. In the absence of pharmaco-resistance, lack of early remission (indeterminate outcome) is usually not associated with a bad outcome, at least over the next few years.

BACKGROUND:Little is known about early prediction of intractable epilepsy (IE) in children. Such information could help guide the early use of new therapies in selected patients. METHODS:Children with newly diagnosed epilepsy (n = 613) were prospectively identified from child neurology practices in Connecticut (1993--1997) and followed-up for the occurrence of IE (failure of > or = 2 drugs, > or = 1 seizure/month, over 18 months) [corrected]. Etiology and epilepsy syndromes were classified per International League Against Epilepsy guidelines. RESULTS:The median follow-up is 4.8 years, and 599 (97.7%) have been followed for more than 18 months. Sixty children (10.0%) have met the criteria for IE, including 34.6% with cryptogenic/symptomatic generalized, 2.7% with idiopathic, 10.7% with other localization-related, and 8.2% with unclassified epilepsy (p < 0.0001). After multivariable adjustment for epilepsy syndrome, initial seizure frequency (p < 0.0001), focal EEG slowing (p = 0.02), and acute symptomatic or neonatal status epilepticus (p = 0.001) were associated with an increased risk of IE, and age at onset between 5 and 9 years was associated with a lowered risk (p = 0.03). The absolute number of seizures and unprovoked or febrile status epilepticus were not associated substantially with IE. CONCLUSIONS:Approximately 10% of children meet criteria for IE early in the course of their epilepsy. Cryptogenic/symptomatic generalized syndromes carry the highest risk and idiopathic syndromes the lowest. Half of IE occurs in children with nonidiopathic localization-related syndromes. Initial seizure frequency is highly predictive of IE. By contrast, absolute number of seizures and unprovoked or febrile status epilepticus are not.

Purpose: Epilepsy syndromes can be identified very early in the course of a seizure disorder. It is unclear how accurate and resilient such early classifications are. We compared the classification of epilepsy syndromes made previously on the basis of information available at diagnosis with those made 2 years later in a cohort of children with newly diagnosed epilepsy.Methods: Children (n = 613) were prospectively identified at the time of initial diagnosis by participating physicians in Connecticut between 1993 and 1997. Classification of epilepsy syndrome according to International League Against Epilepsy guidelines was made previously based on all relevant information available at diagnosis. All cases were reclassified again after 2 years of additional evidence had accumulated. The distributions of syndromes at diagnosis and at 2 years are compared and reasons for changes examined.Results: After 2 years, syndromes remained the same in 86.3% of the cohort and changes occurred in 13.7% (n = 84). Evolution of the syndrome occurred in 24 children (3.9%), and rectification to the initial diagnosis occurred in 60 children (9.8%). The most common scenario for evolution of a syndrome was from West syndrome (n = 5), undetermined (n = 4), or symptomatic localization-related epilepsy (n = 3) to the Lennox-Gastaut syndrome. The most common rectification of initial classifications involved incompletely classified syndromes (cryptogenic localization-related and undetermined syndromes; n = 36). In a few instances, a fully specified syndrome was reclassified to another apparently unrelated syndrome. In these cases, initial information at diagnosis had been difficult to interpret.Conclusions: Epilepsy syndromes can, for the most part, be identified at the time of initial diagnosis. Two years later, rectifications were made in only 9.8% of cases, and most of these involved syndromes that represented incomplete classifications in the first place. Significant changes were rare. The International League Against Epilepsy classification of the epilepsies can be meaningfully applied in epidemiological studies of newly diagnosed pediatric epilepsy.