Transient oromotor deficits in children with benign childhood epilepsy with central temporal spikes

PURPOSE: To describe transient oromotor deficits in benign childhood epilepsy with centrotemporal spikes (BCECTS), an idiopathic age-specific epileptic syndrome with a benign course. METHODS: Five children with BCECTS and intermittent dysarthria and drooling not in the context of typical clinical seizures are presented. RESULTS: The periods of oromotor deficits correlated with increased seizure frequency in all children. Concomitant EEGs that were recorded during periods of dysarthria in four of the children revealed focal electrographic seizures. The reported children did not differ from other patients with BCECTS in any other respect. CONCLUSIONS: Transient oromotor dysfunction is a rare ictal phenomenon that occurs in children with BCECTS with no other unique clinical features.     

Carbamazepine versus sulthiame in treating benign childhood epilepsy with centrotemporal spikes

We compared the therapeutic efficacy of carbamazepine versus sulthiame in patients with benign childhood epilepsy with centrotemporal spikes. Drug efficacy was evaluated only in those patients who initiated treatment with any drug after at least three seizures. Thirty-eight patients who received carbamazepine and 18 patients who received sulthiame were included in the analysis. Cessation of seizures was observed in 73.6% of the former and in 66.7% of the latter (P = not significant). Five of eight patients who were switched to sulthiame after failing carbamazepine became seizure free, whereas none of the three patients who failed sulthiame became seizure free after being switched to carbamazepine. The rate of drug discontinuation owing to adverse reaction was 15% in carbamazepine and 14.3% in sulthiame. Normalization of interictal epileptiform activity on electroencephalography was seen more often following treatment with sulthiame (71%) than with carbamazepine (42%) (P = not significant). No significant differences between these two medications were found in the treatment of benign childhood epilepsy with centrotemporal spikes in this small patient sample.     

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.     

Comparison of auditory event-related potentials between children with benign childhood epilepsy with centrotemporal spikes and children with temporal lobe epilepsy

The abnormal brain discharges observed in benign childhood epilepsy with centrotemporal spikes (BECTS) and temporal lobe epilepsy (TLE) are located close to areas responsible for auditory and language processing. This study aimed to analyze the results of auditory event-related potentials (P300) in children with BECTS and TLE in order to assess whether the epileptic activity in centrotemporal and temporal regions may compromise the integrity and physiology of auditory system structures. This was a prospective, comparative, and cross-sectional study. Group I (GI) consisted of 13 children diagnosed with BECTS, group II (GII), 7 children diagnosed with TLE, and control group (GIII), 16 healthy children, with no hearing or academic complaints. After neurological and basic audiological assessments, P300 was applied. The P300 latency and amplitude were compared between groups. Regarding latency, GI showed 324.1 (+ 31.5) ms, GII 336.3 (+ 23.5) ms, and GIII 318 (+ 27.7) ms. Amplitudes were 4.80 (+ 3.2) μV in GI, 4.7 (+ 2.5) μV in GII, and 5.8 (+ 2.4) μV in GIII. Although children with BECTS showed prolonged latencies and reduced amplitudes, these differences were not considered statistically significant. Children with TLE showed statistically significant prolonged P300 latency compared with the control group (P = 0.037). We speculate that abnormal electrical discharges in centrotemporal and temporal regions led to the slowing of auditory processing in our sample.     

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.     

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.     

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.

     

儿童良性中央-颞棘波型癫痫随访研究

目的：探讨儿童良性中央－颞棘波型癫痫的预后。方法;对１３８例儿童良笥中央－颞棘波型癫痫进行了２～１２年的随防研究。结果：抗癫痫药物均能控制临床发作,智商和社会行为不受影响,其中１１３例（８２％）终止抗癫痫药物２～１２年临床无发作,脑电图均恢复正常。结论：脑电图睡眠诱发试验可以提高本病诊断阳性率。经抗癫痫药物治疗,临床无发作,脑电图正常我年后可以停药。     

伴有中央-颞区局灶棘波儿童良性癫痫的动态脑电图研究

目的 探讨伴有中央-颞区局灶棘波儿童良性癫痫(BECT)的脑电图特征.方法 对115例BECT患儿进行动态脑电图检查,并对检查结果及临床表现进行分析.结果 BECT主要表现为面部抽搐、流涎、喉咙发声、发作性肢体阵挛或强直,发作与睡眠密切相关.脑电图背景活动正常,发作间期脑电图一侧或双侧中央-颞区出现棘波或尖波,可向其它部位扩散,还可从一侧半球转移至对侧半球.入睡后局灶放电频率明显增多.结论 BECT临床发作和睡眠密切相关.脑电图是诊断本病的主要依据,动态脑电图可提高脑电图诊断的阳性率.     

Cognitive functions in children with benign childhood epilepsy with centrotemporal spikes (BECTS).

Benign childhood epilepsy with centrotemporal spikes (BECTS) is regarded as a benign form of epilepsy because of its usually favorable outcome, in terms of seizures. Eighteen children with BECTS were studied in terms of neuropsychological and learning abilities: intellectual quotient, oral language (phonological production, naming skills, verbal fluency and syntactic comprehension), drawing and visuo-spatial skills, visual and selective attention, verbal and visuo-spatial memory, reading, numeracy and spelling. The mean IQ of the population was within the normal range, but individual results were heterogeneous. Verbal functions and memory were normal. In contrast, drawing and visuo-spatial skills, attention and visuo-spatial memory were significantly weak compared to the normal range for age. Reading, numeracy and/or spelling ability were significantly delayed by one academic year or more in ten of the children. In conclusion, despite its benign outcome in terms of epilepsy, BECTS can be accompanied by specific cognitive disorders and low academic achievement.     

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.     

Oxcarbazepine monotherapy in benign childhood epilepsy with centrotemporal spikes: a clinical and cognitive evaluation

We report on 70 patients (aged 5.2-11.6 years) newly diagnosed with benign childhood epilepsy with centrotemporal spikes (BECTS) who were assigned to oxcarbazepine (OXC) monotherapy. All of them underwent clinical and electroencephalographic examination at baseline and at 3- to 6-month intervals during the study. Psychometric assessment was performed at baseline and after 18 months of treatment with the WISC-III, Illinois Test of Psychomotor Abilities, DSM-IV, and Bender-Santucci test. The Mann-Whitney U test was used to describe differences in the frequency of abnormal findings: (1) at initial evaluation, comparing patients with a matched group of 45 healthy controls, and (2) after 18 months of OXC monotherapy, as an individual follow-up in the patient group. Cognitive assessment at baseline revealed mild learning disabilities in 9% of patients and 7% of controls; all participants had a normal intelligence quotient. During the follow-up, sustained cessation of seizures under medication was observed in 53% of patients; an additional 21% had some relapse but were subsequently rendered seizure free, 21% experienced a >50% improvement, and 5% showed no improvement. Normalization of interictal epileptiform activity was observed in 58% of patients, 35% showed an improvement in the grade of electroencephalographic pathology, and 7% manifested no change at all. The initial mildly weak scores in isolated cognitive domains did not deteriorate, and even improved in some cases, during the course of the study, with concomitant electroencephalographic improvement or normalization and effective seizure control. The results of this study suggest that OXC is effective in preventing seizures and normalizing electroencephalograms and seems to preserve cognitive functions and behavioral abilities as long-term monotherapy in children with typical BECTS.     

Intellectual functioning in children with epilepsy: Frontal lobe epilepsy,childhood absence epilepsy and benign epilepsy with centro-temporal spikes

PurposeThe purpose of our study is to describe intellectual functioning in three common childhood epilepsy syndromes – frontal lobe epilepsy (FLE), childhood absence epilepsy (CAE) and benign epilepsy with centro-temporal spikes (BECTS). And also to determine the influence of epilepsy related variables, type of epilepsy, age at epilepsy onset, duration and frequency of epilepsy, and treatment on the scores.MethodsIntellectual functioning was examined in a group of 90 children with epilepsy (30 FLE, 30 CAE, 30 BECTS), aged 6–15 years, and compared with a control group (30). All subjects obtained a Full Scale IQ ≥ 70 and they were receiving no more than two antiepileptic medications. Participants completed the Wechsler Intelligence Scale for Children – Third Edition. The impact of epilepsy related variables (type of epilepsy, age at epilepsy onset, duration of epilepsy, seizure frequency and anti-epileptic drugs) on intellectual functioning was examined.ResultsChildren with FLE scored significantly worse than controls on WISC-III Verbal IQ, Full Scale IQ and Processing Speed Index. There was a trend for children with FLE to have lower intelligence scores than CAE and BECTS groups. Linear regression analysis showed no effect for age at onset, frequency of seizures and treatment. Type of epilepsy and duration of epilepsy were the best indicators of intellectual functioning.ConclusionIt is crucial that children with FLE and those with a longer active duration of epilepsy are closely monitored to allow the early identification and evaluation of cognitive problems, in order to establish adequate and timely school intervention plans.     

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.     

Clinical genetic studies in benign childhood epilepsy with centrotemporal spikes

Purpose: To accurately determine the frequency and nature of the family history of seizures in patients with benign childhood epilepsy with centrotemporal spikes (BECTS). Method: Participants with BECTS were recruited from the electroencephalography (EEG) laboratories of three pediatric centers and by referral. Pedigrees were constructed for up to three degrees of relatedness for each proband. All available affected and unaffected individuals underwent phenotyping using a validated seizure questionnaire. The proportion of affected relatives according to degree of relatedness was calculated and phenotypic patterns were analyzed. Key Findings: Fifty‐three probands with BECTS had a mean age of seizure onset at 7.8 years (range 2–12 years). Thirty‐four (64%) of 53 patients were male. For 51 participants, pedigrees were available for three degrees of relatedness. Fifty‐seven (2.7%) of 2,085 relatives had a history of seizures: Twenty‐one (9.8%) of 214 first‐degree, 15 (3%) of 494 second‐degree, and 21 (1.5%) of 1,377 third‐degree relatives. Febrile seizures were the most frequent phenotype, occurring in 26 of 57 affected relatives. There were 34 relatives with epilepsy: 6.5% (14 of 214) first‐degree, 1.8% (9 of 494) second‐degree, and 0.8% (11 of 1,377) third‐degree relatives. Of 21 affected first‐degree relatives: 8 of 21 had febrile seizures (FS), 4 had BECTS, 2 had epilepsy‐aphasia spectrum disorder, one had temporal lobe epilepsy with hippocampal sclerosis, 2 had focal epilepsy of unknown cause, 2 had genetic generalized epilepsies, and 3 had miscellaneous. Significance: The frequency of epilepsies in relatives and the heterogeneous syndromes observed suggest that BECTS has a genetic component consistent with complex inheritance. Focal epilepsies are the most common seizure disorder observed in relatives, especially BECTS and epilepsy‐aphasia spectrum disorder. Additional acquired or environmental factors are likely to be necessary for expression of the seizure disorder.     

EEG characteristics related to educational impairments in children with benign childhood epilepsy with centrotemporal spikes

PURPOSE: Learning and behavioral difficulties often occur in benign childhood epilepsy with centrotemporal spikes (BCECTS). In recent years, several electroencephalogram (EEG) characteristics have been related to the occurrence of learning and behavioral problems. METHODS: From 28 children medical, school and psychological reports were present and children were rated according to a 4-point scale for educational and behavioral impairment (Part 1). Thirty 24-h EEG recordings were reanalyzed for spike frequency, the presence of atypical EEG criteria, and the presence of a nondipole spike. EEGs were scored during wakefulness, first hour of sleep and whole night sleep (minus the first hour of sleep) separately (Part 2). RESULTS: The presence of I: an intermittent slow-wave focus during wakefulness, II: a high number of spikes in the first hour of sleep (and during whole night sleep), and III: multiple asynchronous bilateral spike-wave foci in the first hour of sleep correlates significantly with a sum score > or = 3 which indicates a complicated course with educational or behavioral impairment. It is sufficient to analyze an EEG during wakefulness and a sleep EEG for only the first hour of sleep instead of a whole night recording to demonstrate those EEG criteria. CONCLUSIONS: On basis of our reanalysis we can possibly conclude that the aforementioned EEG characteristics correlate with educational impairments, and that analysing an EEG recording during wake and the first hour of sleep is sufficient to look adequately for those EEG criteria in children with BCECTS.     

具有中央-颞部棘波的良性儿童期癫痫36例临床分析

目的 探讨具有中央-颞部棘波的良性儿童期癫痫(BECT)的临床特点.方法 分析36例BECT患者的临床及脑电图资料.结果 本组发病年龄为6～14岁,平均10.5岁,全部病例均于睡眠时发作,其中3例睡眠及清醒时均有发作.20例表现为局灶性发作,16例为局灶性发作继发全身性发作.发作间期脑电图均有一侧或双侧中央或颞区尖波和棘波,7例清醒时脑电描记正常,睡眠状态下监测均出现癫痫样放电.有1对孪生患儿先后患病发作.结论 了解BECT特征性的临床表现与典型的脑电图改变,是诊断的主要依据.