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.

     

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.     

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.     

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.     

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.     

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.     

Atypical presentations of benign childhood epilepsy with centrotemporal spikes: a review

Benign childhood epilepsy with centrotemporal spikes is the most common epileptic syndrome in childhood. Atypical forms of benign childhood epilepsy with centrotemporal spikes are common. The different atypical forms of the condition are believed to represent a continuum of the same underlying genetic mechanism. The atypical forms of benign childhood epilepsy with centrotemporal spikes include electrical status epilepticus in slow waves sleep, Landau-Kleffner syndrome, status epilepticus of benign childhood epilepsy with centrotemporal spikes, "classic" atypical form, and others. This review delineates the different forms with emphasis on the cognitive hazards of the more malignant types.     

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.     

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.     

伴有中央颞区棘波的儿童良性癫痫同步脑电图-功能磁共振研究

目的 研究伴有中央颞区棘波的儿童良性癫痫(BCECTS)患者发作间期棘波相关的血氧依赖水平(BOLD)变化情况. 方法 选择海南医学院附属医院神经内科自2010年1月至2011年12月收治的频繁出现发作间期放电的BCECTS患者进行同步脑电图(EEG)联合功能磁共振(EEG-fMRI)扫描,将离线EEG滤波后确定棘波的时间点,采用统计参数图软件(SPM)提供的经典血氧动力学函数(HRF)模型计算棘波相关的BOLD变化. 结果 共纳入6例BCECTS患者,常规EEG显示频繁出现发作间期中央区和中颞区棘波.EEG-fRI扫描时1例未见放电,获得5例患者5个序列的fMRI数据,5个序列均出现BOLD激活信号,两侧中央颞区同时出现激活信号2例,单侧中央颞区出现激活信号3例,4例最大激活区位于中央-中颞区,1例位于枕叶.5例在中央颞区外的其他脑区具有激活信号. 结论 中央颢区可能是BCECTS的致痫灶,致痫灶与其他部位共同构成的神经网络可能与BCECTS的发生相关.     

Phenobarbital-induced atypical absence seizure in benign childhood epilepsy with centrotemporal spikes.

This report describes atypical absence seizures induced by phenobarbital (PB) in a patient with benign childhood epilepsy with centrotemporal spikes. An 11-year-old girl had nocturnal motor seizures, beginning at the age of 7 years. Interictal EEG displayed centrotemporal sharp waves in the left and right sides alternatively, which increased significantly in frequency during sleep. She was given a diagnosis of benign childhood epilepsy with centrotemporal spike and was treated with valproic acid (VPA), which controlled her seizures for 3 years. She experienced nocturnal Rolandic seizures again, at the age of 10 years 2 months, 10 years 10 months in spite of the regular medication, and PB 60 mg daily was added to the VPA. Her parents noted de novo a few seconds of unresponsive fits 7 days after PB administration. Ictal EEG of the fits revealed diffuse irregular spike and wave bursts (1-4 seconds). VPA and PB in her blood was within the therapeutic ranges. After discontinuing PB, the atypical absence seizures immediately disappeared. Phenobarbital should be added to the list of drugs that potentially provoke de novo seizures.     

Nightly oral administration of topiramate for benign childhood epilepsy with centrotemporal spikes

Objective

The objective of this study was to explore the feasibility of nightly oral administration of topiramate for treating benign childhood epilepsy with centrotemporal spikes (BECTS).

Methods

Eighty-five children with BECTS receiving topiramate treatment were randomly divided into A group (44 patients) and B group (41 patients). In A group, topiramate was orally administrated once a night, with a final dose of 2 mg/kg/day. In B group, topiramate was orally administrated twice a day, with a final dose of 4 mg/kg/day. At the end of the 12-month follow-up period, clinical efficacy, changes in electroencephalographic (EEG) activity, and adverse reactions were analyzed.

Results

There was no significant difference in overall efficacy rate, percentages of patients achieving seizure free, or changes in EEG activity between the two groups (P > 0.05). The rate of adverse reactions for A group was 9.1 %, which was significantly lower than the 29.3 % for B group (χ ² = 4.262, P < 0.05).

Conclusion

Nightly oral administration of topiramate is a feasible strategy for the treatment of BECTS, with the advantages of comparable efficacy, convenience, and fewer adverse reactions.

     

Topographic mapping and clinical analysis of benign childhood epilepsy with centrotemporal spikes

We studied the topographic mapping of the electroencephalography (EEG) of 47 children whose clinical history and course were compatible with typical benign childhood epilepsy with centrotemporal spikes (BCECT). Twenty-nine (62%) patients showed typical dipole fields, with a negative potential field in the centrotemporal region and a positive field in the frontal region. Eighteen children did not demonstrate the typical dipole field. Their non-dipole rolandic discharges were localized in small fields of centrotemporal region. The patients with dipole fields in BCECT had significantly less frequent seizures than patients without dipole fields. Twelve of the 47 patients with BCECT (26%) had more than one EEG focus. The clinical courses of patients with multiple foci were not worse than those of patients with a single focus. We conclude that EEG topographic mapping is helpful in identifying typical or atypical EEG topographic patterns in patients with clinically diagnosed BCECT. We also conclude that the presence of dipole field usually indicates a better clinical course of epilepsy and multiple foci do not mean a poor clinical course.     

Two epileptic syndromes, one brain: childhood absence epilepsy and benign childhood epilepsy with centrotemporal spikes

Childhood absence epilepsy (CAE) and benign childhood epilepsy with centrotemporal spikes (BCECTS), or benign rolandic epilepsy (BRE), are the most common forms of childhood epilepsy. CAE and BCECTS are well-known and clearly defined syndromes; although they are strongly dissimilar in terms of their pathophysiology, these functional epileptic disturbances share many features such as similar age at onset, overall good prognosis, and inheritance factors. Few reports are available on the concomitance of CAE and BCECTS in the same patients or the later occurrence of generalized epilepsy in patients with a history of partial epilepsy. In most cases described in the literature, absence seizures always started after the onset of benign focal epilepsy but the contrary has never occurred yet. We describe two patients affected by idiopathic generalized epileptic syndrome with typical absences, who experienced BCECTS after remission of seizures and normalization of EEG recordings. While the coexistence of different seizure types within an epileptic syndrome is not uncommon, the occurrence of childhood absence and BCECTS in the same child appears to be extremely rare, and this extraordinary event supports the hypothesis that CAE and BCECTS are two distinct epileptic conditions. However, recent interesting observations in animal models suggest that BCECTS and CAE could be pathophysiologically related and that genetic links could play a large role.     

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.