We assessed the impact of age at onset of epilepsy and duration and frequency of seizures on cognitive development in children less than 3 years old. Retrospective analysis was conducted on clinical data and neuropsychological testing of 33 infants with epilepsy. Developmental quotients were calculated and were correlated with age at epilepsy onset, duration of epilepsy, seizure frequency, brain pathology, and types of seizures (with/without spasms) as potential predictors. Infants with longer duration and earlier onset of epilepsy performed worse on developmental neuropsychological testing. Regression analyses showed that age at epilepsy onset and percentage of life with epilepsy were both strongly associated (regression model P < 0.0001) with developmental quotient. There was no correlation with seizure frequency. Infants with spasms had worse developmental quotients than infants without spasms (P < 0.001). These results suggest that duration of epilepsy and age at onset may be the best developmental predictors during the first years of life in patients with epilepsy. Early aggressive intervention should be considered.

Background-ADHD coexisting with epilepsy is poorly understood, thus we compared the clinical correlates and psychiatric comorbidities in 36 children with epilepsy and ADHD, aged 6 to 17 years enrolled in an ADHD treatment trial, to those reported in the literature on children with ADHD without epilepsy. Methods-Measures included the Kiddie Schedule for Affective Disorders and Schizophrenia forSchool-Age Children (KSADS), the Wechsler Abbreviated Scale of Intelligence (WASI), and the Scales for Independent Behavior-Revised (SIB-R).Results-Mean IQ was 86±19, and SIB-R Standard Score was 72±26. The ADHD-Combined subtype, composed of both inattentive and hyperactive symptoms, was most frequent (58%). Sixtyone percent exhibited a comorbid disorder, including anxiety disorders (36%), and Oppositional Defiant Disorder (31%).Conclusions-Comorbidity in ADHD with epilepsy is similar to that of ADHD without epilepsy reported in the literature. These preliminary data argue that the pathophysiology of ADHD has common components in both populations.

Satisfaction with pediatric neuropsychological evaluations was surveyed by asking parents or guardians of children who completed pediatric neuropsychological evaluations at a large children's hospital over a 2-year period to complete a 30-item rating scale. The scale included items drawn from published measures of consumer satisfaction, and incorporated a well-validated measure of general satisfaction. A total of 338 surveys were distributed, with 117 completed, for a return rate of 35%. Respondents were generally similar to non-respondents, except that respondents had a higher average level of maternal education and were more likely to have been referred for neuropsychological evaluations by sources outside the hospital. Parents were generally satisfied with pediatric neuropsychological evaluations, although some parents indicated that the evaluations did not provide as much help as expected. A factor analysis of the survey instrument revealed four dimensions of satisfaction: General Satisfaction, Clinician Acceptance/Empathy, Provision of Help, and Facilities/Administrative Assistance. Maternal education was negatively correlated with all four factors, but no other demographic, patient, or clinician variables were significantly related to satisfaction. Future studies could survey physicians and educators to provide a more complete understanding of satisfaction with pediatric neuropsychological evaluations.

Children with benign rolandic epilepsy (BRE) experience elevated rates of cognitive, behavioral, and affective problems. Frequent epileptiform spike discharges may impair behavioral functioning. To elucidate this relationship, we evaluated associations between the EEG spike frequency index (SI) and parental ratings of psychosocial adjustment and executive functioning in school-aged children with EEGs typical of BRE. Twenty-one children (6–12 years) participated. Parents completed validated questionnaires at a median of 5 months (range: 1–8) after a routine outpatient EEG. The EEG SI was calculated for wakefulness and sleep. The strength of association between the SI and behavioral variables was evaluated by simple and multivariate correlation. Higher awake and sleep SIs were associated with more symptoms of depression (P<0.001), aggression and conduct problems (P<0.01). Higher sleep SI was associated with executive dysfunction and anxiety (P<0.05). Symptoms of hyperactivity and inattention had no correlation. Increased epileptiform activity in children with BRE may predict higher rates of mood and behavioral problems.

In this study we validate passive language fMRI protocols designed for clinical application in pediatric epilepsy surgical planning as they do not require overt participation from patients. We introduced a set of quality checks that assess reliability of noninvasive fMRI mappings utilized for clinical purposes. We initially compared two fMRI language mapping paradigms, one active in nature (requiring participation from the patient) and the other passive in nature (requiring no participation from the patient). Group-level analysis in a healthy control cohort demonstrated similar activation of the putative language centers of the brain in the inferior frontal (IFG) and temporoparietal (TPG) regions. Additionally, we showed that passive language fMRI produced more left-lateralized activation in TPG (LI = +0.45) compared to the active task; with similarly robust left-lateralized IFG (LI = +0.24) activations using the passive task. We validated our recommended fMRI mapping protocols in a cohort of 15 pediatric epilepsy patients by direct comparison against the invasive clinical gold-standards. We found that language-specific TPG activation by fMRI agreed to within 9.2 mm to subdural localizations by invasive functional mapping in the same patients, and language dominance by fMRI agreed with Wada test results at 80% congruency in TPG and 73% congruency in IFG. Lastly, we tested the recommended passive language fMRI protocols in a cohort of very young patients and confirmed reliable language-specific activation patterns in that challenging cohort. We concluded that language activation maps can be reliably achieved using the passive language fMRI protocols we proposed even in very young (average 7.5 years old) or sedated pediatric epilepsy patients.

Working memory and information processing speed were examined in children with myelomeningocele and shunted hydrocephalus using the Children's Paced Auditory Serial Addition Test (CHIPASAT). The CHIPASAT was administered to 31 children with myelomeningocele and shunted hydrocephalus and 27 healthy siblings, all between 8 and 15 years of age. They also completed other standardized measures of working memory and processing speed. Children with myelomeningocele made fewer correct responses than siblings, although the magnitude of group differences declined as the rate of stimulus presentation increased. Children with myelomeningocele also made fewer consecutive correct responses and were more likely to provide correct but nonconsecutive responses, suggesting that they responded in a way that circumvents the working memory demands of the task. Standardized measures of processing speed and working memory accounted for significant variance in CHIPASAT performance after controlling for age, group membership, math skill, and general intellectual functioning. The results indicate that children with myelomeningocele and shunted hydrocephalus display deficits in working memory and information processing speed, and suggest that the CHIPASAT may provide a valid measure of these skills.

BackgroundFebrile-infection related epilepsy syndrome (FIRES) is a rare epilepsy syndrome in which a previously healthy individual develops refractory status epilepticus in the setting of a preceding febrile illness. There are limited data regarding detailed long-term outcomes. This study aims to describe the long-term neuropsychological outcomes in a series of pediatric patients with FIRES.MethodsThis is a retrospective multi-center case series of pediatric patients with a diagnosis of FIRES treated acutely with anakinra who had neuropsychological testing at least 12 months after status epilepticus onset. Each patient underwent comprehensive neuropsychological evaluation as part of routine clinical care. Additional data collection included the acute seizure presentation, medication exposures, and outcomes.ResultsThere were six patients identified with a median age of 11.08 years (IQR: 8.19–11.23) at status epilepticus onset. Anakinra initiation was a median of 11 days (IQR: 9.25–13.50) after hospital admission. All patients had ongoing seizures and none of the patients returned to baseline cognitive function with a median follow-up of 40 months (IQR 35–51). Of the five patients with serial full-scale IQ testing, three demonstrated a decline in scores over time. Testing results revealed a diffuse pattern of deficits across domains and all patients required special education and/or accommodations for academic learning.ConclusionsDespite treatment with anakinra, neuropsychological outcomes in this series of pediatric patients with FIRES demonstrated ongoing diffuse neurocognitive impairment. Future research will need to explore the predictors of long-term neurocognitive outcomes in patients with FIRES and to evaluate if acute treatment interventions improve these outcomes.

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