Impact of benign childhood epilepsy with centrotemporal spikes (BECTS) on school performance

BECTS represents the vast majority of childhood focal epilepsy. Owing to the age peculiarity of children who suffer from this disease, i.e., school-going age of between 6 and 9 years, the condition is often referred to as a school disorder by parents and teachers.ObjectiveThe aim of this study was to evaluate the academic performance of children with BECTS, according to the clinical and electroencephalographic ILAE criteria, and compare the results of neuropsychological tests of language and attention to the frequency of epileptic discharges.MethodsThe performances of 40 school children with BECTS were evaluated by applying a school performance test (SBT), neuropsychological tests (WISC and Trail-Making), and language tests (Illinois Test Psycholinguistic Abilities – ITPA – and Staggered Spondaic Word – SSW). The same tests were applied in the control group.ResultsChildren with BECTS, when compared to those in the control group, showed lower scores in academic performance (SPT), digits and similarities subtests of WISC, auditory processing subtest of SSW, and ITPA – representational and automatic level. The study showed that epileptic discharges did not influence the results.ConclusionChildren with BECTS scored significantly lower scores in tests on academic performance, when compared with those in the control group probably due to executive dysfunction.     

Cognition and behavior in children with benign epilepsy with centrotemporal spikes (BECTS)

Characteristics of cognitive deficits in benign childhood epilepsy with centrotemporal spikes (BECTS) remain unclear. The authors screened 200 BECTS children presenting for a clinical trial, finding relative weaknesses in fine motor control, visual learning, and attention in the presence of overall normal intellect, with simple partial seizures associated with more difficulty. Parental concerns for psychosomatic and learning problems were noted. Monitoring select cognitive and behavioral features in BECTS appears appropriate.     

The influence of sulthiame on EEG in children with benign childhood epilepsy with centrotemporal spikes (BECTS)

PURPOSE: To evaluate the effects of sulthiame (Ospolot; STM) monotherapy compared with placebo on the EEG in children with benign childhood epilepsy with centrotemporal spikes (BECTS). METHODS: Sixty-six patients (aged 3-11 years) entered a 6-month double-blind trial and were randomized to either STM (n = 31) or placebo (n = 35). Clinical data and general results have been reported elsewhere (1). One-hundred seventy-nine sleep EEGs were recorded at screening and after 4 weeks, 3 months, and 6 months. EEGs were analyzed by a blind reviewer using a standard protocol for each EEG. This standard protocol collected data on general changes, specific epileptiform, and nonspecific focal and generalized changes. A classification system was defined depending on rating of pathologic EEG changes. Because of the higher number of treatment-failure events (i.e., seizures) in the placebo group, there was an increasing imbalance between the two groups regarding the number of recorded sleep EEGs over time (STM, 104; placebo, 74). A Wilcoxon-Mann-Whitney U test was used to describe differences in the grade of pathology during individual follow-up between the two groups. RESULTS: The sleep-EEG was found to be normalized in 21 patients treated with STM (12/21 transient) and in five patients treated with placebo (4/5 transient). In the STM group, the EEG showed a marked improvement during intraindividual course when comparing the classification of follow-up EEGs at each time point with the screening EEG. Comparable improvements were not observed in the placebo group (exact two-tailed p value at 4 weeks, p < 0.0001; at 3 months, p = 0.0010; and at 6 months, p < 0.0001). CONCLUSIONS: STM had marked effects on the EEG in BECTS, which led to normalization in the majority of the patients. Most of those whose EEGs were not normalized showed improvement in the grade of EEG pathology. Normalization persisted in >50% of patients during the investigation. Spontaneous normalization in the placebo group reflects the wide spectrum of individual courses, which must be considered when analyzing drug effects on EEG in BECTS.     

Blink induced centrotemporal spikes in benign childhood epilepsy with centrotemporal spikes

A 10 year old girl with benign childhood epilepsy withcentrotemporal spikes showed centrotemporal spikes induced by blinking even in a dark room. Spikes could not be induced by photic stimulation, eye closure, eye movement, eye deviation, or passive blinks. There havebeen no previous reports of spikes induced by blinks in benign childhood epilepsy with centrotemporal spikes.

     

Cognitive and behavioral outcomes in benign childhood epilepsy with centrotemporal spikes

We review the evidence that BECTS may be associated with cognitive dysfunction and behavioral problems, the extent to which these problems may be associated with patterns of EEG abnormalities in BECTS, and the impact of antiepileptic medication on cognition and behavior in BECTS.A growing literature examining cognitive and behavioral outcomes suggests that children with BECTS perform below the level of their peers. Consistent with this, neuroimaging studies reveal that BECTS has an impact on structural and functional brain development, but the potential influence of frequency and lateralization of centrotemporal spikes (CTS) on cognition and behavior is not well understood. Treatment with AEDs is an option in BECTS, but existing studies have not clearly shown a clear relationship between elimination of CTS and improved cognitive or behavioral outcomes.     

Neuropsychological findings in children with benign childhood epilepsy with centrotemporal spikes

Benign childhood epilepsy with centrotemporal spikes (BCECTS) is a well-known idiopathic age- and localization-related epileptic syndrome with characteristic clinical and EEG manifestations. Due to the reported benign evolution of this epilepsy syndrome, neuropsychological assessment has been considered unnecessary. However, the benign nature of BCECTS has recently been challenged: verbal dysfunction as well as impaired visuomotor coordination, specific learning disabilities, and attention deficit have been noticed. These findings prompted this research study in which all children with BCECTS attending our epilepsy clinic underwent neuropsychological assessment. Seventeen children (10 boys and seven girls) aged 7 to 14 years were investigated with a neuropsychological test battery focusing on immediate and delayed recall of auditory-verbal and visual material, verbal fluency, problem-solving ability, and visuospatial constructional ability. Raven's coloured matrices and questionnaires regarding school functioning and behaviour were also administered. The children were matched with control subjects for age, sex, and school. Children with BCECTS had significantly lower scores than their control subject partners on the neuropsychological items. Intellectual abilities did not differ and neither did school functioning or behaviour according to teachers. Parents, however, recognized greater difficulties with concentration, temperament, and impulsiveness in children with BCECTS.     

Neuropsychological profile in new-onset benign epilepsy with centrotemporal spikes (BECTS): Focusing on executive functions

PurposeIncreased evidence of subnormal neuropsychological functioning in new-onset childhood epilepsy has been obtained, although results are still rare and controversial. With a prospective study, we aimed to define the very early neuropsychological profile of children with benign epilepsy with centrotemporal spikes (BECTS), including executive functions (EF) because of their key role in learning. Additionally, we enrolled drug-naïve children, with a NREM sleep frequency of discharges < 85% and with a Performance Intelligence Quotient equal or superior to 85, in order to exclude additional effects on the neuropsychological functioning.MethodsFifteen school-aged children with BECTS (mean age: 8.8 years, standard deviation [SD]: 2.4 years) and fifteen healthy children (mean age: 9.2 years, [SD]: 2.5 years) were enrolled and assessed with a comprehensive neuropsychological battery. The assessment included domain-specific standardized tests of language, EF, academic skills, visuomotor and visuospatial skills, and short-term memory. A p-value < 0.05 was considered significant.ResultsSignificant differences between patients and controls emerged with respect to 3 domains. Language was affected in color naming (p = .026), spoonerism (p = .003), and phonemic synthesis (p = .009). Executive functions appeared inadequate in the five point test with respect to the number of correct figures (p = .003) and errors (p = .008). In the domain of academic skills, significant differences between groups emerged regarding the number of mistakes in nonword writing (p = .001), nonword reading speed (p = .027), nonword reading number of mistakes (p = .019), and word reading errors (p = .023).DiscussionResults showed that children with new-onset BECTS may demonstrate a range of neuropsychological dysfunctions, particularly affecting executive attention, despite a normal IQ, a low frequency of NREM sleep discharges, and the absence of drugs. These difficulties indicate a frontal dysfunction with cascading effects on language and academic skills. The inclusion of EF in the assessment battery and in the intervention since the very onset is warranted in order to avoid further and persistent academic difficulties.     

Clinical evaluation of attentional processes in children with benign childhood epilepsy with centrotemporal spikes (BCECTS).

The aim of this study was to identify the attentional processes specifically affected in children with benign childhood epilepsy with centrotemporal spikes (BCECTS). The impact of several factors - laterality of discharges, age-at-onset and duration of epilepsy, and medication - on these cognitive functions was also studied. A group of 29 children with BCECTS was evaluated using standardized tests performed in routine clinical practice and specifically designed to assess executive and attentional functions. This group obtained mean scores significantly lower than normative means specifically for tasks involving attention control processes, i.e. cognitive flexibility and inhibitory processes. Neither the epilepsy-related factors that we studied nor the medication appeared to influence performance of these tasks. These data suggest an impact of BCECTS on attentional processes, the most affected being attention control processes that develop late.     

Benign childhood epilepsy with centrotemporal spikes (BECTS) and developmental co-ordination disorder

BackgroundBenign epilepsy with centro-temporal spikes (BECTS) is a common childhood epilepsy syndrome also known as Rolandic Epilepsy (RE). Neurocognitive phenotypes have been described with greater focus on attention, reading and language domains but there have been far fewer studies focusing on motor functioning. This study included measures of motor, language and cognition in order to investigate the range, degree and pattern of difficulties associated with BECTS in a case series of children, but with a particular emphasis on motor skills.MethodTwenty-one children aged between 8 and 16 years with a diagnosis of BECTS were asked to complete standardized assessments for language, cognition, motor functioning and handwriting.ResultsWhen measuring across language, cognitive and motor domains, 19 (90.48%) of the twenty-one children with a diagnosis of BECTS showed some difficulties on at least one area of functioning using standardized assessment tests. Of particular note nearly half (47.62%) of the children had some difficulties in one or more areas of motor functioning.DiscussionChildren with BECTS have a heterogeneous pattern of neurocognitive impairments. The presence of motor difficulties (DCD) should be considered in all children routinely seen in clinical settings with BECTS and included in any screening processes.     

Written language skills in children with benign childhood epilepsy with centrotemporal spikes

PURPOSE: The goal of this work was to study written language skills in children with benign childhood epilepsy with centrotemporal spikes (BCECTS) in the absence of atypical clinical or electroencephalographic (EEG) features (n = 32), as compared with controls (n = 36). METHODS: BCECTS patients (7-16 years), attending regular school, without cognitive or behavioral regression, or atypical EEG patterns, completed four tests assessing written language skills and one nonverbal cognitive test. School performance information was recorded. Seizure types, duration, and frequency; awake and sleep interictal EEG findings; and medication status were documented. Epilepsy and educational outcome was recorded for a period of 1-5 years after diagnosis. RESULTS: As a group, BCECTS patients performed significantly worse than controls in spelling, reading aloud, and reading comprehension; presented dyslexic-type errors; and frequently had below-average school performance. Among 11 with poor written language performance, 4 had developmental dysfunctions before school and seizure onset and, as a group, demonstrated low performance on a nonverbal cognitive test. Even though 65.6% were on anticonvulsants due to frequent seizures and/or seizures while awake, none belonged to the atypical BCECTS spectrum with respect to clinical or EEG findings. Increased epilepsy duration and seizure frequency were less represented in patients with no or mild written language problems than in those with poor performance. Otherwise, clinical course and EEG findings in this group were no different than those for the 11 children with severe written language problems. Clinical follow-up indicated that learning problems appear persistent and several children require remedial classes and/or tutoring after the epilepsy has resolved. CONCLUSIONS: Children with severe but not atypical BCECTS performed, as a group, significantly worse than controls in written language skills, specifically in spelling, reading, aloud and reading comprehension; they also presented many difficulties also found in children with dyslexia. However, poor written language performance was not considered specific to BCECTS because it occurred in children with generally low cognitive capacity and/or preexisting developmental dysfunctions. Moreover, a dissociation between epilepsy outcome and learning problem outcome, in the subgroup with poor performance, casts doubt on the existence of a possible causal link between BCECTS and educational performance.     

Impaired social behavior in children with benign childhood epilepsy with centrotemporal spikes

The aim of the present study was to assess the emotional and cognitive aspects of social cognition among patients with rolandic epilepsy. A computerized neuropsychological battery was used for cognitive evaluation. Affective and cognitive social cognition were evaluated using two computerized Theory of Mind tasks. Cognitive abilities and social behavior of 15 children, diagnosed with rolandic epilepsy, ages 7 to 13 years were assessed and compared with 15 age- and education-matched healthy controls. Compared with controls, the epileptic patients had lower scores on verbal and visual learning rate parameters and on verbal processing and were significantly impaired on "affective Theory of Mind" tasks but not on "cognitive Theory of Mind" conditions. Our findings raise the possibility that rolandic epilepsy may affect neural networks affecting cognition and mediating social cognition essential for social behavior, thus challenging the benign nature of the condition.     

Altered attention networks in benign childhood epilepsy with centrotemporal spikes (BECTS): A resting-state fMRI study

It is noteworthy that some children with benign childhood epilepsy with centrotemporal spikes (BECTS) show attention problems despite their favorable seizure outcome. Resting-state functional magnetic resonance imaging (fMRI) is a method widely used to detect brain network alterations in neuropsychiatric diseases. We used resting-state functional magnetic resonance imaging (fMRI) to investigate specific brain networks related to attention deficit in children with BECTS. Resting-state fMRI was performed in patients with BECTS with ADHD (n = 15) and those with BECTS without ADHD (n = 15) and in healthy controls (n = 15). Unbiased seed-based whole-brain functional connectivity analysis was used to study the connectivity pattern of three resting-state networks, including the ventral attention network (VAN) and the dorsal attention network (DAN) and the default mode network (DMN). Patients with BECTS with ADHD displayed decreased functional connectivity in the DAN compared with other two groups, while patients with BECTS without ADHD showed increased functional connectivity in the DAN. Moreover, we found increased functional connectivity in the VAN and in the DMN in patients with BECTS with or without ADHD when comparing with controls. These results showed that the newly-diagnosed children with BECTS displayed brain activity alterations in the ventral and dorsal attention networks. The difference in the extent of impairment in the dorsal attention network of patients with BECTS with ADHD and patients with BECTS without ADHD may lead to improved understanding of the underlying neuropathophysiology and treatment of BECTS with ADHD and BECTS without ADHD.     

儿童良性癫痫伴中央颞区棘波患者的认知损害研究

目的探讨儿童良性癫痫伴中央颞区棘波(Benign epilepsy of childhood with centrotemporal spikes,BECTS)患者的认知损害特点。方法纳入2010年9月-2019年12月于首都医科大学宣武医院神经内科就诊的BECTS患者61例(BECTS组)。选取60名年龄、性别和教育程度相匹配的健康儿童和青少年作为对照组。对BECTS组及对照组进行神经心理学任务测试以评估其注意力、记忆力、计算力、语言功能、执行能力、视空间功能、视知觉及反应能力等多项认知功能。应用SPSS 20.0统计软件,对两组的认知任务测试得分进行独立样本t检验和秩和检验,对影响认知功能损害的各项临床因素进行多元线性回归分析。结果与对照组相比,BECTS组患者的配对联结学习平均(19.56±2.91)分,词语辨析测验的平均(23.67±9.50)分,物体数量认知的平均(61.45±13.14)分,言语工作记忆的平均(6.54±1.47)分,声音知觉测验的平均(5.79±5.90)分,Taylor复杂图形测验的平均(35.10±2.33)分,选择反应时的平均(700.34±493.05)分,均显著低于对照组(P<0.05)。在BECTS组中有36例起病<8岁,与起病≥8岁组相比,前者在视觉追踪任务、空间记忆任务、简单减法任务、数字数量大小比较、汉字押韵测试、词语辨析测试和视觉感知任务等大部分神经心理测试中得分均较低(P<0.05);34例接受单药治疗,27例接受两种及以上抗癫痫药物治疗,多药治疗组在注意力、记忆力、视知觉、反应力等方面均低于单药治疗组(P<0.05)。结论 BECTS患者存在注意力、记忆力、视知觉、声音知觉及反应速度的损害。起病年龄越小其认知损害的程度越重;多药治疗患者的认知损害程度较单药治疗重。     

Evaluation of levetiracetam and valproic acid as low-dose monotherapies for children with typical benign childhood epilepsy with centrotemporal spikes (BECTS)

PurposeThis study aimed to compare the monotherapeutic efficacies of levetiracetam (LEV) and valproic acid (VPA) in a cohort of newly diagnosed children with typical benign childhood epilepsy with centrotemporal spikes (BECTS).MethodsA total of 56 children with typical BECTS were retrospectively reviewed in the analyses. Thirty-three children received LEV and 23 received VPA as initial monotherapy, and the treatments lasted for at least 18 months.ResultsThe average dosage of LEV was 22.7 ± 4.7 mg/kg/day, and that of VPA was 18.7 ± 5.7 mg/kg/day. The seizure-freedom rates were not significantly different between the two groups at 6 (57.5% vs. 60.9%), 12 (81.8% vs. 73.9%) or 18 months (100% vs. 100%). However, a greater number of the children taking VPA achieved Electroencephalography (EEG) normalization compared to those taking LEV both at 12 (78.3% vs. 45.5%) and 18 months (95.7% vs. 72.7%; p < 0.05). No children discontinued therapy due to adverse effects during the follow-up. Only one child (4.7%) in the VPA group exhibited mild weight gain (BMI increase of 2 at the end of follow-up) but did not withdraw from treatment.ConclusionLow-dosage VPA and LEV monotherapies are equally effective in controlling seizures, but VPA exhibited better efficacy than LEV in improving the electrophysiological abnormalities of children with BECTS. None of the patients discontinued therapy, which was likely due to the administration of low dosages.     

Deficit of endogenous spatial orienting of attention in children with benign epilepsy with centrotemporal spikes (BECTS)

Attention difficulties have been reported in children with benign epilepsy with centrotemporal spikes (BECTS) but have not yet been fully specified. The aim of this study was to evaluate the functions of exogenous and endogenous spatial orienting of attention and alerting in these children. Two versions of the spatial cueing paradigm and an alerting task, including trials with and without warning signal, were performed by 25 children with BECTS aged 6–12 years and 25 controls matched for age, gender and IQ. In these three tasks, patients were slower and made more omissions than controls. The alert effect amplitude was comparable in both groups at the longer SOAs (450 ms and 800 ms) while, at the shortest SOA (100 ms), it was greater in controls than in the BECTS group. In the first version of the spatial cueing task (peripheral cues and no probability information), the validity effect amplitude, measured by longer response times (RTs) in invalid trials compared to valid trials, was comparable in both groups. In the second version (central cues and a 75% probability that the target would appear at the cued location), the validity effect was larger in the BECTS group compared to controls because of a higher cost of invalid trials compared to neutral trials. These results suggest the existence of impairments in the endogenous orienting of attention in children with BECTS, in particular an attention disengagement deficit, while exogenous orienting of attention appears to be preserved.     

Cognitive and behavioral effects of nocturnal epileptiform discharges in children with benign childhood epilepsy with centrotemporal spikes

This review addresses the effects of subclinical localized epileptiform discharges during sleep in children with benign childhood epilepsy with centrotemporal spikes (BCECTS) on cognition and behavior. A diversity of educational deficits, behavioral impairments, language delay, and neuropsychological test results have been reported in children with BCECTS. The occurrence of atypical seizure characteristics seems to be related to language delay, and several atypical EEG characteristics are related to cognitive or behavioral problems. It remains to be shown whether treatment of nocturnal discharges in children with BCECTS is indicated to improve cognitive and behavioral problems.     

Default mode network alterations during language task performance in children with benign epilepsy with centrotemporal spikes (BECTS)

Benign epilepsy with centrotemporal spikes (BECTS) is the most common idiopathic epileptic disorder in children. Besides reported cognitive deficits, functional alterations mostly in the reorganization of language areas have also been described. In several publications, it has been reported that activation of the default mode network (DMN) can be reduced or altered in different neuropsychiatric and neurological disorders in adults. Whether this also holds true for children with epilepsy has so far not been clarified.To determine the functional activation of the DMN in children with BECTS, 20 patients and 16 healthy controls were examined using functional magnetic resonance imaging (fMRI), while a sentence generation task and a reading task were applied in a block design manner. To study the default mode network and the functional alterations between groups, an independent component analysis (ICA) was computed and further analyzed using SPM5.Compared with controls, children with BECTS showed not only significantly less activation of the DMN during the rest condition but also less deactivation during cognitive effort. This was most apparent in the precuneus, a key region of the DMN, while subjects were generating sentences.From these findings, we hypothesize that children with BECTS show a functional deficit that is reflected by alterations in the DMN.     

Neuropsychological aspects of benign childhood epilepsy with centrotemporal spikes

PurposeTo establish whether the disability in benign epilepsy with centrotemporal spikes (BECTS) is the result of the number of seizures, the anti-epileptic therapy or is an inherent characteristic of the syndrome itself.MethodsThirty-six children with BECTS were tested for cognitive functions prior to commencing treatment with anti-epileptic drugs, and the findings were compared with those in 15 children with normal electroencephalograms, performed for unrelated reasons. The data in the study group were further correlated with the laterality of the epileptic focus and the number of seizures.ResultsScores for verbal functioning on neuropsychological tests were significantly lower in the study group than the control group. There was no relationship between the neuropsychological scores in the patients and either lateralization of the epileptic focus or number of seizures.DiscussionChildren with BECTS have an impaired ability to process verbal information. The deficiency is apparently a result of the pathological electrical discharges that are part of the syndrome and are not dependent on the epileptic focus laterality, the number of seizures, or the anti-epileptic treatment.     

EEG-related functional MRI in benign childhood epilepsy with centrotemporal spikes

The localization of epileptic foci is an important issue in children with extratemporal epilepsies. However, the value of noninvasive methods such as the EEG-assisted functional magnetic resonance imaging (fMRI) has not been sufficiently investigated in children. As a model of extratemporal epilepsies, we studied 7 patients aged 5 to 12 (median 10) years with benign childhood epilepsy and centrotemporal (rolandic) spikes. Interictal spikes were recorded during the fMRI acquisition on a MR-compatible battery-powered digital EEG system with 16 channels. The fMRI sequences were correlated off-line with the EEG spikes and analyzed with the software Statistical Parametrical Mapping SPM99. The fMRI results demonstrated the spike-related activation in the perisylvian central region in three patients; we could not demonstrate fMRI activation despite active spiking in 2 patients, and 2 patients did not produce sufficient spikes for fMRI analysis. We currently consider the spike-related fMRI as a research tool that localizes epileptic activity in selected patients. Further improvements of the technique are necessary to allow a clinical application of this method.