Memory and phonological awareness in children with Benign Rolandic Epilepsy compared to a matched control group.

PURPOSE: In a previous study we demonstrated children with Benign Rolandic Epilepsy have normal intelligence and language ability. However, difficulties in verbal and visual memory and aspects of phonological awareness were found compared to normative data. To address the methodological limitations related to the use of normative data, we compared the same cohort of children with Benign Rolandic Epilepsy to a matched control group. METHOD: Controls (n=40) matched on age and gender to the Benign Rolandic Epilepsy cohort underwent neuropsychological assessment. The life functioning of the control group was assessed using a modified version of the Quality of Life in Childhood Epilepsy Questionnaire (QOLCE). RESULTS: The study confirmed the previous findings of memory and phonological awareness difficulties. In addition, the children with Benign Rolandic Epilepsy had significantly lower IQ scores than the matched control group. Paired sample t-tests showed that on 8 of 11 QOLCE scales, children with Benign Rolandic Epilepsy were rated by parents as having poorer life functioning compared to matched controls, including lower parental ratings on the subscales of memory and language. DISCUSSION: Benign Rolandic Epilepsy has an excellent seizure prognosis, but this study further emphasizes potential cognitive difficulties. Using an age and gender matched control group, the previous findings of memory and phonological awareness difficulties were validated. These problems in cognition were also identified by parents of children with Benign Rolandic Epilepsy as problematic and impacting upon the child's quality of life.

Longitudinal assessment of neuropsychologic and language function in children with benign rolandic epilepsy.

Previous studies of benign rolandic epilepsy have reported improvement in cognitive functioning over time. Their focus was the impact of paroxysmal electroencephalographic (EEG) activity on neuropsychologic function. Comprehensive longitudinal language assessment has not previously been undertaken. In a cross-sectional study, we demonstrated that some children with benign rolandic epilepsy have difficulties in verbal and visual memory and phonologic awareness. The current study evaluated a subgroup longitudinally to determine if difficulties improved. Twenty-eight patients underwent comprehensive longitudinal neuropsychologic and language assessments. The clinical features evaluated included seizure frequency, absolute age, medications, and a follow-up EEG. Differences in performance were analyzed using t-tests. Improvement in cognitive functioning, particularly in the areas of verbal memory, receptive language ability, and phonemic manipulation, was demonstrated. Visual memory and aspects of phonologic awareness showed no change. The improvements were not related to the clinical variables. It is important to recognize cognitive difficulties in children with benign rolandic epilepsy. Some difficulties can resolve; however, continued monitoring, particularly in areas of visual memory and phonologic awareness, is required.

Quality of life of children with benign rolandic epilepsy.

The first objective of this study was to determine the quality of life of children with benign rolandic epilepsy. Secondly, this investigation aimed to predict the influence of cognition on quality of life, controlling for the emotional impact of the epilepsy on the parent. Initial recruitment was through the major electroencephalography laboratories of metropolitan Sydney. The syndrome was defined using the International League Against Epilepsy classification. Patients underwent a comprehensive cognitive assessment, and parents completed the Child Health Questionnaire, Child Behavior Checklist, and Quality of Life in Childhood Epilepsy Questionnaire. Parental emotional impact was assessed using a subscale from the Child Health Questionnaire. The cohort included 30 patients (22 males, 8 females), mean age 9.67 years. There was a higher incidence of competence problems compared with normative data. The average psychosocial score was significantly lower than normative data. Controlling for parental emotional impact, general intellectual ability predicted quality of life in the areas of self-esteem and language. Clinical variables had minimal impact and were not included in the regression models. Parental emotional impact, however, was a major independent predictor of quality of life. Quality of life may be compromised in children with benign rolandic epilepsy and is related to cognitive variables and emotional impact of the epilepsy on the parent.

The neuropsychological and language profile of children with benign rolandic epilepsy.

PURPOSE: Benign rolandic epilepsy (BRE) has an excellent prognosis for seizures, but recent research has raised concerns using cognition as an outcome measure. Methodologic problems related to recruitment bias and assessment processes are evident in previous studies. With well-defined criteria for inclusion and comprehensive assessment, the aim of this study was to define the cognitive profile of children with BRE and to assess the effect of interictal EEG activity. METHODS: Patients (n=42) were recruited from six EEG laboratories. The EEG features analyzed were spike frequency, trains, and laterality. Comprehensive neuropsychological and language assessments were conducted. Group means on cognitive measures were compared with normative means. Tests were correlated with EEG features. RESULTS: The study demonstrated that children with BRE have normal intelligence and language ability. However, a specific pattern of difficulties in memory and phonologic awareness was found. Furthermore, a large proportion of children had disproportionate scores in these areas compared with intellectual and language ability. EEG features were minimally associated with cognitive difficulties, and no correlation was found with memory indices and tests of phonologic awareness. CONCLUSIONS: Some children with BRE have specific difficulties in memory and phonologic processing skills, not explained by interictal activity. We recommend that pediatricians ask about academic performance specifically in areas of prereading, reading, spelling, and memory. If difficulties are suspected, assessment targeting phonologic awareness and memory are recommended, as they may not be reflected in overall intellectual and language ability. Difficulties in phonologic awareness affect literacy, and memory problems affect academic performance.

Spike morphology, location, and frequency in benign epilepsy with centrotemporal spikes.

The literature on benign epilepsy with centrotemporal spikes reports a constellation of neurophysiologic features in selected populations with heterogeneous methodologies. The aim of this study was to determine the specific electroencephalographic (EEG) features (spike morphology, location, and frequency and associated background slowing) in a broad population-based cohort identified through EEG laboratories. The mean spike frequency in the awake state was 9.3 per minute (95% confidence interval 6.5-12.0), in drowsiness, 21.2 per minute (16.7-25.6); and in sleep, 45.6 per minute (38.3-52.8), where 60% of patients had > 40 discharges per minute. In five patients, spike train rates occupied > 80% of the sleep record, and in nine patients, they occupied 61% to 80%. An ambulatory overnight record did not add new information comparing early-onset sleep with a mean spike frequency of 37.1 per minute (27.3-46.9) with slow-wave sleep, 36.0 per minute (27.3-44.7). Patients with benign epilepsy with centrotemporal spikes have a high spike burden, which can impact on cognitive function.

Speech and language deterioration in benign rolandic epilepsy.

A 5-year-old boy presented with typical clinical and electrophysiologic features of benign rolandic epilepsy. His neurodevelopment, language, and behavior prior to the onset of epilepsy were appropriately normal. He demonstrated marked deterioration of language and cognitive function during the course to a mild and then a moderate disability range. Serial sleep electroencephalographic recordings initially showed continuous and bilateral rolandic discharges with evolution to localized left rolandic spikes. Language and cognitive improvements were subsequently seen. Educational support and evolution of the electroencephalogram to a localized focus could have been contributory. It is anticipated, however, that he will have significant long-term problems in complex language.

Tagum study II: follow-up study at two years of age after prenatal exposure to mercury.

OBJECTIVES: To correlate the presence and levels of total mercury (THg) in cord blood and meconium indicating prenatal exposure with developmental milestones at 2 years and to compare these subjects with controls of comparable age using cognitive adaptive test and clinical linguistic auditory milestone scale (CAT/CLAMS). METHODS: In 48 of the original Tagum (T) subjects, cord blood and meconium Hg levels, head circumference (HC) at birth, and duration of breastfeeding were correlated with CAT/CLAMS at 2 years. At 2 years, THg levels using cold atomic vapor absorption spectrometry were determined in the hair of 46 T subjects and 88 Saranggani (S) controls; THg levels in blood were tested in 48 T subjects and 45 S controls. These levels were correlated with CAT/CLAMS. Both groups had standard physical and neurologic examinations, hearing screen using transitory evoked otoacoustic emissions, serum glutamate pyruvate transaminase, and routine urinalysis. A prevalidated Socioeconomic Means Test was given to both groups. RESULTS: The Hg level in cord blood was negatively correlated with CAT/CLAMS at 2 years. The HC at birth was negatively correlated with levels of Hg in hair of T subjects 2 years later. HC at birth and 2 years hence were positively correlated with CAT/CLAMS. The following were significantly higher in S controls than in T subjects: expressive language quotient 82.569 +/- 2.21 versus 71.57 +/- 2.61; CLAMS 87.96 +/- 2.43 versus 77.67 +/- 2.51; CAT 90.57 +/- 2.22 versus 83.15 +/- 1.43; and full-scale developmental quotient 89.31 +/- 2.14 versus 80.56 +/- 1.86. Fifteen percent of T subjects had global delay (full-scale developmental quotient