Risks and benefits of epilepsy surgery in a pediatric population: Consequences for memory and academic skills.

We examined benefits and risks for memory and academic functioning associated with epilepsy surgery in a pediatric population. A total of 46 patients with intractable seizures and a single seizure focus were divided into four groups according to focus localization: right temporal, left temporal, frontal, and parietal/occipital region. Pre- and postsurgery performance measures were compared across groups and with a fifth group of patients that had intractable seizures but did not undergo surgery. Both groups with temporal lobe epilepsy showed significant declines in memory test scores, while performance of the group with frontal lobe epilepsy improved. These changes were mirrored in parental reports of everyday memory. Consistent with other pediatric studies, no lateralized material-specific declines in the groups with temporal lobe epilepsy were found. When memory improved, the improvement was associated with decreases in seizure frequency and the number of anticonvulsant medications. Presurgical performance was the best predictor of declines in memory test performance. Deterioration of academic test scores in the group that did not have surgery exemplified a potential risk of living with seizures and antiepilepsy medication.

Social functioning in pediatric epilepsy reported by parents and teachers: Contributions of medically related variables, verbal skills, and parental anxiety.

Children with epilepsy are at increased risk for deficits in social functioning, though the underlying causes are not well-understood. We examined multiple seizure-related, demographic, and cognitive variables in a group of 93 pediatric patients with intractable seizures who were at risk for social skills deficits and social problems at home and in the classroom. Verbal intelligence and parental anxiety about epilepsy were found to be the two primary predictors of social functioning in children with epilepsy as reported by parents and teachers. Though other social variables and secondarily generalized seizures were significantly correlated with certain aspects of parent-reported social functioning, the impact of these variables appeared to be mediated through verbal intelligence and/or parental anxiety about epilepsy. These findings emphasize the importance of family characteristics on social functioning in children with epilepsy and also suggest that parental anxiety about their child's epilepsy may be a specific risk factor for this population. The findings from this study suggest that the factors associated with social functioning in children with epilepsy are similar regardless of whether social functioning is assessed by the parent or the classroom teacher.

Adaptive functioning in pediatric epilepsy: contributions of seizure-related variables and parental anxiety.

Young people with epilepsy are less likely to achieve the level of independence attained by their peers. We examined the seizure-related variables that placed a group of 97 pediatric patients with intractable seizures at risk for poor adaptive functioning. Analyses evaluated both the direct effects of the medical variables and indirect effects that were mediated through increased parental anxiety about their child's epilepsy. Higher numbers of anticonvulsants, presence of seizures that secondarily generalize, longer duration of seizure disorder, and younger age at onset were all identified as risk factors for poor adaptive functioning. Depending on the specific behavioral domain of adaptive functioning, the effects were sometimes direct and sometimes indirect. Lower levels of parental education and positive family history of seizures were associated with higher levels of parental anxiety. Interventions that target parental anxiety about seizures may mitigate the deleterious effects of epilepsy on social development.

Processing speed, attention, and working memory after treatment for medulloblastoma: an international, prospective, and longitudinal study.

PURPOSE: The current study prospectively examined processing speed (PS), broad attention (BA), and working memory (WM) ability of patients diagnosed with medulloblastoma over a 5-year period. PATIENTS AND METHODS: The study included 126 patients, ages 3 to 21 years at diagnosis, enrolled onto a collaborative protocol for medulloblastoma. Patients were treated with postsurgical risk-adapted craniospinal irradiation (n = 36 high risk [HR]; n = 90 average risk) followed by four cycles of high-dose chemotherapy with stem-cell support. Patients completed 509 neuropsychological evaluations using the Woodcock-Johnson Tests of Cognitive Abilities Third Edition (median of three observations per patient). RESULTS: Linear mixed effects models revealed that younger age at diagnosis, HR classification, and higher baseline scores were significantly associated with poorer outcomes in PS. Patients treated as HR and those with higher baseline scores are estimated to have less favorable outcomes in WM and BA over time. Parent education and marital status were significantly associated with BA and WM baseline scores but not change over time. CONCLUSION: Of the three key domains, PS was estimated to have the lowest scores at 5 years after diagnosis. Identifying cognitive domains most vulnerable to decline should guide researchers who are aiming to develop efficacious cognitive intervention and rehabilitation programs, thereby improving the quality of survivorship for the pediatric medulloblastoma population.

Behavioral and academic problems in children with Sturge-Weber syndrome: differences between children with and without seizures.

Although Sturge-Weber (SWS) syndrome is associated with behavioral and academic problems in childhood, it is unknown whether those problems are concomitants of the disorder itself or of the seizure disorder that is common in SWS. We compared two groups of children with SWS-- one with seizures (n=20) and one without seizures (n=14)--on parent-report and teacher-report measures of behavioral and academic functioning. The two subgroups were compared with each other as well as with children with epilepsy alone (n=29) and a group of healthy controls (n=21). The SWS group with seizures was more impaired than the seizure-free group on 9 of 15 measures and the children with seizures were 10 times as likely to have received special education services. Overall, children with SWS and seizures were similar to the epilepsy group, whereas children with SWS and no seizures were similar to the controls.

Everyday verbal memory and pediatric epilepsy.

This study addressed the reliability and validity of reports of everyday verbal memory with a sample of 132 pediatric patients with epilepsy. Each patient and one parent completed a questionnaire on everyday verbal memory comprising two scales assessing learning/retrieval and prospective memory. Each patient was also administered tests of memory, attention, and academic skills. Information about attention, mood, and academic performance was obtained from parent and teacher report, as well as self-report. Memory test scores were correlated with children's reports of learning and retrieval in everyday activities, but were not significantly associated with reports of prospective memory. Reports of everyday memory were found to be reliable and predictive of academic performance. Performance on tests of memory, conversely, was unrelated to reports of academic performance. Reports of everyday memory may, therefore, provide more useful information than tests when evaluating the effects of epilepsy and its treatments.

How parents cope with their child's diagnosis and treatment of an embryonal tumor: results of a prospective and longitudinal study.

The current study reports longitudinal coping responses among parents of children diagnosed with an embryonal brain tumor. Patients (n = 219) were enrolled on a treatment protocol for a pediatric embryonal brain tumor. Their parents (n = 251) completed the Coping Response Inventory at time of their child's diagnosis and yearly thereafter, resulting in 502 observations. Outcomes were examined with patient and parent age at diagnosis, patient risk, parent gender and education as covariates. At the time of diagnosis, the highest observed coping method was seeking guidance with well above average scores (T = 61.6). Over time, younger parents were found to seek guidance at a significantly higher rate than older parents (P = .016) and the use of acceptance resignation and seeking alternative results by all parents significantly increased (P = .011 and P < .0001 respectively). The use of emotional discharge was also observed above average at time of diagnosis (T = 55.4) with younger fathers being more likely to exhibit emotional discharge than older fathers (P = .002). Differences in coping according to age of the patient and parent education level are also discussed. Results show a high need for guidance, and above average emotional discharge, especially among younger parents. It is imperative for the healthcare team to lead with accurate information so that these parents may make informed decisions about the care of their child. This need remains high years after diagnosis. Therefore it is critical to continue a consistent level of effective communication and support, even following treatment.

Arithmetic performance in children with Tourette syndrome: relative contribution of cognitive and attentional factors.

The study addressed the issue of arithmetic deficiencies in children with Tourette syndrome (TS) as well as explanations for such deficiencies. A total of 47 children with TS were assigned to three subgroups based on a composite attention score from the Test of Variables of Attention (TOVA). These children, along with 17 normal controls between 8 and 16 years of age, were tested on standardized measures of IQ, attention, visuospatial ability, and arithmetic achievement. The children also were administered an experimental calculation task with two levels of structure. Children with TS scored below controls on tests of IQ, attention, and arithmetic achievement but not visuospatial ability. The TS subgroup with the greatest impairment of attention accounted for most of the differences in arithmetic achievement. Regression analysis, based on the 47 children with TS, indicated that IQ and TOVA scores were the best predictors of arithmetic achievement. Likewise, the experimental calculation task indicated that the poor performance of some children with TS could be attributed to deficient attention. Irrespective of structure, children in the TS subgroup with the greatest attentional impairment made more attention (but not visuospatial) errors than did controls on the experimental task. Thus, on both the standardized and the experimental tasks, poor arithmetic skill was found only in children with TS who had significant attentional deficits.

Spinocerebellar ataxia type 2 presenting with cognitive regression in childhood.

Spinocerebellar ataxia type 2 typically presents in adulthood with progressive ataxia, dysarthria, tremor, and slow saccadic eye movements. Childhood-onset spinocerebellar ataxia type 2 is rare, and only the infantile-onset form has been well characterized clinically. This article describes a girl who met all developmental milestones until age 3(1/2) years, when she experienced cognitive regression that preceded motor regression by 6 months. A diagnosis of spinocerebellar ataxia type 2 was delayed until she presented to the emergency department at age 7 years. This report documents the results of her neuropsychologic evaluation at both time points. This case broadens the spectrum of spinocerebellar ataxia type 2 presentation in childhood, highlights the importance of considering a spinocerebellar ataxia in a child who presents with cognitive regression only, and extends currently available clinical information to help clinicians discuss the prognosis in childhood spinocerebellar ataxia type 2.

Adaptive functioning in children with seizures: impact of maternal anxiety about epilepsy.

This study evaluated the impact of maternal anxiety about a child's epilepsy on parental overprotection and the child's adaptive functioning. Specific maternal and family characteristics that contribute to elevated maternal anxiety about epilepsy were also studied over a year's time in a group of 56 mothers with children recently diagnosed with epilepsy. Overall, the primary predictor of maternal anxiety about epilepsy was the mother's level of coping resources, although family stress aggravated anxiety at the initial time point. Maternal anxiety about epilepsy was associated with overprotective and overly directive parenting styles, but it was the anxiety level itself that was most strongly related to the child's adaptive functioning. Maternal anxiety about epilepsy decreased over time, as did the relationship of maternal anxiety to the child's adaptive functioning. Nonetheless, after a year had elapsed, maternal anxiety was still associated with poorer adaptive skills.

Executive functions in children with frontal and temporal lobe epilepsy.

Even though frontal lobe epilepsy (FLE) is a relatively common seizure type, no formal psychometric studies of children with FLE have been reported. We compared 12 children with FLE and 15 children with temporal lobe epilepsy (TLE) on neuropsychological tests of attention, memory, executive functioning, and adaptive functioning. The results of these tests indicated that the children with FLE had deficits in planning and executive functions, whereas their verbal and nonverbal memory was intact. The opposite pattern was observed in children with TLE. Measures of executive functioning and impulse control were the best predictors of adaptive functioning. The findings suggest that children with FLE have a pattern of cognitive deficits that differs markedly from the pattern seen in children with TLE. Children with FLE have prominent deficits in executive functioning that appear to be related to poor behavioral adaptation.