Benign childhood epilepsy with centrotemporal spikes: a study of 50 Chinese children

doi:10.1016/S0387-7604(03)00003-2

Brain and Development

Volume 25, Issue 6, September 2003, Pages 390-395

https://doi.org/10.1016/S0387-7604(03)00003-2 Get rights and content

Abstract

In order to collect local data for children with Benign Childhood Epilepsy with Centrotemporal Spikes (BECTS), we conducted a retrospective study of 50 Chinese children (32 males and 18 females) with BECTS diagnosed in two regional hospitals in Hong Kong from 1995 to 1998. Their peak age of onset was 7 years (range 3–13 years) and a male predominance was observed. Seven patients (14%) had a past history of febrile convulsions and five cases (10%) had a family history of epilepsy. The presentation was protean, but most of them had infrequent, short, nocturnal generalised seizures. The EEG spike foci were most frequently found in mid-temporal regions, followed by centrotemporal regions. Fourteen percent of children did not require anti-epileptic drug treatment. For those who were treated, they were easily controlled on a low dose of carbamazepine (median dosage of 12.75 mg/kg per day) or sodium valproate (median dosage of 20 mg/kg per day). Our study suggested a generally good prognosis for BECTS. No risk factors of frequent seizure recurrence could be identified.

Introduction

Benign childhood epilepsy with centrotemporal spike (BECTS) is the most common focal epilepsy in childhood [1]. Although Martinius Rulandus described the first case of typical benign hemifacial seizures in 1597 [2], the specific clinical and electrographic features were only recognised in the last 40 years. The reasons for the late recognition of this common syndrome despite the characteristic clinical feature and the striking electroencephalographic (EEG) findings are twofold. Firstly, the key symptoms of somatosensory stimulation and motor interference with the tongue and the nearby structures can propagate rapidly and submerges all into the maelstrom of a generalised convulsion [3], [4]. More importantly is that neurologists have been taught over the years to identify partial seizures and focal epilepsy with cortical lesions. Sutherland et al. emphasised in 1974 that ‘It is vital to recognise that partial seizures have an underlying pathology’ [5]. Thus, the concept that focal EEG discharges can be associated with a benign form of epilepsy takes time to evolve.

Since then this benign form of epilepsy had been given a variety of names. Finally, in its revised proposal for classification of epilepsies and epileptic syndromes, the International League Against Epilepsy classified it as an idiopathic localisation-related epilepsy; and renamed it “Benign Childhood Epilepsy with Centrotemporal Spikes” (BECTS) [6]. By definition, it is age-related, without demonstrable anatomic lesions, and is subject to spontaneous remissions. It is an important epileptic syndrome to recognise, as diagnosis of this syndrome allows the clinician to offer the patient and parents a rational plan for treatment and prognosis. However, reports on BECTS are scarce in Chinese children. As there are no local data on this common epileptic syndrome, we undertook a retrospective study to review 50 cases of BECTS in order to describe its characteristics in our locality.

Section snippets

Patients

This was an observational study to examine local Chinese children with BECTS in a retrospective manner. The characteristics of these children were examined by reviewing the EEGs and the case records. The study period was from 01/07/1995 to 31/06/1998. We retrieved all the EEG reports in United Christian Hospital and Princess Margaret Hospital during the study period. The EEG recordings were obtained from 19 surface electrodes according to the international 10–20 electrode placement system. We

Results

Fifty cases of BECTS were retrieved.

Discussion

There was a male predominance (64%) for children with BECTS in our study, which is similar to series reported elsewhere [1], [3], [7], [9], [10], [11], [12]. The age of onset ranged from 3 to 13 years, with a peak age of 7. This is younger than the reported peak age of 8–9 years in most Western series [9], [10], [11], [13], but is similar to that reported by a group of Taiwanese investigators on 94 Chinese children [14]. Approximately 80% of all cases occurred between 5 and 10 years old.

Degen

Conclusion

BECTS is an important childhood epileptic syndrome. It is easy to treat and the majority of patients have good outcome. Early counseling should be given to children with BECTS. Further follow-up of this group of children is warranted to ascertain their long-term prognosis.

Acknowledgements

We would like to acknowledge C.W. Chin (Statistical Officer, Department of Statistics, Prince of Wales Hospital) for his assistance in the statistics and data analysis. We would also like to express our gratitude to the Electro-diagnostic Unit and the Medical Records Office of United Christian Hospital and Princess Margaret Hospital for their help in collecting the EEGs and the case notes. Special thanks are given to Dr P.Ip (COS, Department of Paediatrics, UCH) for her advice.

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The natural history of seizures and neuropsychiatric symptoms in childhood epilepsy with centrotemporal spikes (CECTS)
2020, Epilepsy and Behavior
Childhood epilepsy with centrotemporal spikes (CECTS) (formally benign epilepsy with centrotemporal spikes, BECTS) is a common childhood epilepsy syndrome characterized by psychiatric, behavioral, and cognitive abnormalities and self-limited seizures. Although CECTS is one of the most well-characterized electroclinical epilepsy syndromes, the natural history of neuropsychiatric outcomes is poorly understood. We report the psychiatric, behavioral, and cognitive profiles over the course of disease from a large, prospectively-enrolled, longitudinal cohort of children with CECTS. We further characterize the detailed seizure course and test the relationship between several proposed risk factors and neuropsychiatric and seizure outcomes in these children.
Patients diagnosed with CECTS were enrolled as part of a community-based study and followed from diagnosis through disease resolution (16.0 ± 3.1 years, N = 60). Twenty sibling controls were also recruited. We report the natural history of premorbid neuropsychiatric concerns, postmorbid neuropsychiatric diagnoses, long-term neuropsychological performance, seizure course, antiseizure medication (ASM) treatment response, and the relationship between duration seizure-free and remission. Age at onset and premorbid neuropsychiatric concerns were tested as predictors of seizure count, epilepsy duration, postmorbid neuropsychiatric diagnoses, and long-term neuropsychological performance. Antiseizure medication treatment duration, seizure count, and epilepsy duration were tested as predictors of postmorbid neuropsychiatric diagnoses and long-term neuropsychological performance.
Children with CECTS had a high incidence of ADD/ADHD symptoms (18.3%) or learning difficulties (21.7%) before diagnosis. New or persistent ADHD (20%), mood disorders (23.6%), learning difficulties (14.5%), and behavioral disorders (7.3%) were common after CECTS diagnosis. At 9-year follow-up, performance on formal neuropsychological testing was comparable to population statistics and sibling controls. More than two-thirds of treated children experienced at least one seizure during treatment. Most children (61.7%) had entered terminal resolution after 12 months seizure-free. Among all children, for each month seizure-free, there was a 6–7% increase in the probability of achieving terminal remission (p < 1e-10). The presence of a premorbid neurodevelopmental concern predicted a longer epilepsy duration (p = 0.02), higher seizure count (p = 0.02), and a postmorbid psychiatric or neurodevelopmental diagnosis (p = 0.002). None of the tested features predicted long-term neuropsychological performance.
Children are at high risk of neuropsychiatric symptoms along the course of the disease in CECTS, however, long-term cognitive performance is favorable. The majority of children had a seizure while being treated with ASMs, suggesting that CECTS is not as pharmacoresponsive as assumed or that treatment approaches are not optimized. Among treated and untreated children, future seizure-risk can be estimated from duration seizure-free. The presence of a premorbid neuropsychiatric concern predicted a more severe disease course in CECTS.
Prognostic significance of failure of the initial antiepileptic drug in children with benign childhood epilepsy with centrotemporal spikes
2015, Brain and Development
Citation Excerpt :
Zhao et al. [14] found that 80% of patients experienced seizures during sleep, 5% had seizures during the waking states, and 15% had seizures in both in the sleeping and waking states in their study. In other study, Ma et al. [13] reported seizures only during sleep in 86% patients, during both sleep and wakefulness in 10% patients, and during wakefulness alone in 4% patients. In our study, seizures occurred only during sleep in 62 patients (73.8%), during both sleep and wakefulness in 19 patients (22.6%), during wakefulness alone in three patients (3.6%).
Background: Benign epilepsy with centrotemporal spikes is the most common partial epilepsy syndrome in children. The long-term prognosis for children with BECTS is believed to be generally excellent with seizures usually responding well to AEDs. The goal of the present study was to determine the risk factors associated with a poor prognosis. Methods: Eighty-four children with BECTS were retrospectively analyzed. Fifty-four (64.3%) were boys and 30 (35.7%) were girls with the mean age at seizure onset 7.1 ± 2.01 years (range: 3–12 years). Results: Of the 84 patients, 72 (85.7%) were treated successfully with the first AED (Group A), and 12 (14.3%) failed to responded to the initial AED treatment (Group B [poor prognosis]). Univariate analyses suggested that younger age of seizure onset, presence of generalized seizures, and frequent seizures (>3 prior to the initial treatment) were associated with failure to control seizures with the initial AED. Multivariate analysis suggested that younger age of seizure onset was the independent risk factor predicting a poor response to initial AED treatment. Conclusion: About 14% of our cohort of children with BECTS continued to have seizures following the initial AED treatment. Further prospective studies are warranted to determine how well prognosis can be predicted by age of seizure onset, type of seizures, and frequency of pre-existing seizures in children with BECTS.
Electrical status epilepticus in sleep: Clinical presentation and pathophysiology
2012, Pediatric Neurology
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“Benign” epilepsy with centrotemporal spikes constitutes the most frequent pediatric focal epileptic syndrome, accounting for 10-24% of new-onset pediatric epilepsies [119-122]. Boys are more frequently affected than girls, with a ratio of 3:2 [123]. The onset of seizures peaks at around 7-10 years of age (Fig 1).
Electrical status epilepticus in sleep involves an electroencephalographic pattern where interictal epileptiform activity is potentiated in the transition from wakefulness to sleep. Near-continuous spikes and waves that occupy a significant proportion of nonrapid eye movement sleep appear as a result of sleep-potentiated epileptiform activity. This electroencephalographic pattern appears in different electroclinical syndromes that present three common characteristics with different degrees of severity: seizures, sleep-potentiated epileptiform activity, and neuropsychologic regression. Continuous spikes and waves during sleep comprise the severest epileptic encephalopathy in the electroclinical spectrum. Landau-Kleffner syndrome presents with intermediate severity. Some “benign” pediatric focal epileptic syndromes represent the mildest end of this continuum. Based on published data, we provide a framework for clinical and electrical events. The underlying mechanisms leading to sleep potentiation of epileptiform activity in electrical status epilepticus in sleep are incompletely understood. A genetic basis or acquired early developmental insult may disrupt the normal maturation of neuronal networks. These factors may dynamically alter normal processes of brain development, leading to an age-related pattern of electroclinical expression of electrical status epilepticus in sleep.
Epileptic syndromes with focal seizures of childhood and adolescence
2012, Handbook of Clinical Neurology
Citation Excerpt :
Incidence among children under 16 years has been estimated to be 21 per 100 000 (Heijbel et al., 1975a). There is a mild male predominance with a male to female ratio of approximately 3 : 2 (Beaussart, 1972; Ma and Chan, 2003). Patients have a predisposition to febrile seizures (16%) and present more frequently with seizures in infancy (1–8%) (Chahine and Mikati, 2006).
The first description of benign focal epilepsy of childhood likely dates back to the 16th century. Further delineation over the past 60 years led to the identification “benign” rolandic epilepsy and subsequently of occipital subtypes and other variants, including epilepsy with continuous spike-and-wave activity during slow-wave sleep and Landau–Kleffner syndrome. The pattern of spontaneous occurrence and resolution with age, frequently in association with cognitive deficits, has led to the term benign seizure susceptibility syndrome. In this review we outline epidemiological features, clinical and EEG presentations in relationship to diurnal patterns and to sleep, neuropsychological findings, genetics, pathophysiology, management, and prognosis of childhood seizure susceptibility syndromes. Interactions between brain development and maturation processes as well as genetic influences may play a role in the development of various childhood epileptic syndromes associated with language and cognitive deficits.
Benign epilepsy of childhood with centrotemporal spikes (BECTS): To treat or not to treat, that is the question
2010, Epilepsy and Behavior
The goal of this review is to assess the value of treating versus not treating benign epilepsy (of childhood) with centrotemporal spikes (BECTS). Among 110 recommendations from 96 articles, two-thirds generally favored and one-third generally did not favor treatment with antiepileptic drugs (AEDs). Two studies concluded that all patients should be treated, but most investigators added qualifications, for example, treating those with early onset, multiple seizures at onset, and large numbers of seizures, especially generalized tonic–clonic seizures, and limiting treatment to 1 year. Other studies made treatment dependent on epileptiform discharges or amelioration of symptoms. Specific AEDs were reviewed, and in the largest number of positive studies, valproic acid or carbamazepine was favored. Among the studies generally opposing treatment, none opposed treatment for all patients in all circumstances. Usually, qualifications to treat were added, for example, if generalized tonic–clonic seizures occurred or if there was a change in quality of life. One AED associated with negative effects was carbamazepine, treatment with which can result in the development of epileptic negative myoclonus, absence seizures, and generalized spike–wave complexes on the EEG. Thus, if treatment is planned, valproic acid may be considered the drug of choice in BECTS. Although many neurologists oppose treatment; twice as many studies concluded in favor of treatment. The typical benign aspect of this disorder may allow for nontreatment to be without serious consequences.
Long term outcome of benign childhood epilepsy with centrotemporal spikes: Dutch Study of Epilepsy in Childhood
2010, Seizure
To determine long-term outcome in a cohort of children with newly diagnosed benign childhood epilepsy with centrotemporal spikes (BECTS).
29 children with BECTS were included in the Dutch Study of Epilepsy in Childhood. Each child was followed for 5 years, and subsequently contacted 12–17 years after enrolment to complete a structured questionnaire. Twenty children had typical BECTS, nine had atypical BECTS (age at onset <4 years, developmental delay or learning difficulties at inclusion, other seizure types, atypical EEG abnormalities).
Mean age at onset of epilepsy was 8.0 years with slight male preponderance. Most common seizure-types before enrolment were generalized tonic–clonic seizures (GTCS) and simple partial seizures; in 86% of the children seizures occurred during sleep. After 12–17 years, 96% had a terminal remission (TR_F) of more than 5 years and 89% of more than 10 years. Mean duration of epilepsy was 2.7 years; mean age at reaching TR_F was 10.6 years. Many children (63%) had experienced one or more (secondary) GTCS. Antiepileptic drugs were used by 79% of the children with a mean duration of 3.0 years. None of the children seemed to have developed learning problems or an arrest of cognitive development during follow-up. No significant differences were observed in patient characteristics or outcome between children with typical BECTS and children with atypical BECTS.
All children in our cohort, both those with typical and atypical BECTS, had a very good prognosis with high remission rates after 12–17 years. None of the predictive factors for disease course and outcome observed in earlier studies (other seizure types, age at onset, multiple seizures at onset) were prognostic in our cohort.

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Original articleBenign childhood epilepsy with centrotemporal spikes: a study of 50 Chinese children