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Purpose To describe the seizure pattern, treatment strategies and outcome in a series of children with Rolandic seizures or childhood epilepsy with centrotemporal spikes. Materials and methods Patients were defined as Rolandic epilepsy if on electroencephalographic studies high voltage spike and waves were seen in centrotemporal areas, could be followed by slow waves, often activated on sleep and could shift from one side to other or be secondarily generalized. Typical (TRS) or benign were those with normal intellect. Atypical rolandic seizures (ARS) were those associated with neuroregression of language and cognitive milestones. Patients were treated with antiepileptic drugs if more than one episode occurred or the first episode was generalized status epilepticus. Results Thirty-three patients were included over the period of eight years (2012-2020). There was male preponderance (21 males versus 12 females). Four patients (12.12%) later evolved into Landau Kleffner syndrome (ARS group). The mean age of onset of epilepsy in the TRS group (29 patients) was 7.2 (+/-2.2) with the youngest patient being 4 years and the eldest being 12 years. In the ARS group the mean age of onset was 5 yrs. (+/-1.41). In the TRS group, 23 (79.31%) patients were managed on monotherapy AED. Seventeen patients (58.62%) responded (remission) to carbamazepine monotherapy alone. Six patients (20.68%) could afford oxcarbazepine monotherapy and went in remission with this therapy. In the ARS group all patients required three drugs (valproate, clobazam and levetiracetam). By the end of the study period, 23/33 (75.75%) patients remained seizure free. Conclusions Most patients with rolandic seizures have excellent prognosis being seizure free around puberty. The neurological outcome in most patients was normal.

The objective of this narrative review was to investigate how the clinical aspects, such as age-at-onset, epilepsy duration, centrotemporal spikes, spike location, and seizure frequency, affect various domains of language, cognition, and behavior in children with benign childhood epilepsy. Data were collected using various research databases, including Wiley Online Library, PubMed Central, Elsevier ClinicalKey, and Springer Complete Journals. Keywords such as “Benign Childhood Epilepsy” or “BECTS and language impairment” were used among other terms. Case reports, meta-analyses, and reviews were excluded. Children with benign childhood epilepsy are mainly impaired in semantic processing (receptive language), working memory, attention/inhibitory control, complex visuospatial skills, and social skills. Functional magnetic resonance imaging reveals not only structural abnormalities, but also alterations in language, sensorimotor, attentional, and social networks, suggesting long-term consequences. It so seems that the occurrence of centrotemporal spikes (with or without seizures), especially at a young age (below 6 years) and for an extended period of time, is the most meaningful contributor to the language, cognitive, and behavioral deficits in benign childhood epilepsy with centrotemporal spikes (BECTS), while the distribution of centrotemporal spikes (left, right, bilateral) seems of only little significance.

Epilepsy is a neurological disorder characterized by abnormal electrical discharges in a group of brain cells. Benign childhood epilepsy, which affect children under the age of 12 years, has been reported to contribute to the cognitive impairment of these children, even in the absence of structural abnormalities. Functional connectivity models have been applied to provide a deeper understanding of the processes that control and regulate interictal activity of benign childhood epilepsy. These studies have shown regions of increased connectivity and activity, particularly at the epileptic zone, which is usually the central region around the sensorimotor cortex, and in the immediate regions surrounding the zone and reduced activity in distant regions, such as the frontal lobe and temporal regions. The present study was designed to identify the neural drivers involved in the initiation and propagation of epileptic activity and the causal relationships between brain regions with increased and decreased connectivity and functional activity. We used three different models to identify neural drivers and casual connectivity with dynamic causal modelling (DCM) of EEG data. All models showed that the central region, the source of the epileptic activity, is the major driver of the brain network during interictal discharges. Other regions include the temporoparietal junction and temporal pole. The central region also had influence on the frontal and contralateral hemisphere, which might explain the cognitive deficits observed in these patients., Highlights • The epileptic source is the major driver of the brain network • Other drivers include the temporoparietal junction and temporal pole • Epileptic source had influence on the frontal region which might explain the cognitive deficits • The right epileptic region drives the left hemisphere during interictal epileptic discharges

Supplemental Digital Content is available in the text, There are 2 intrinsic networks in the human brain: the task positive network (TPN) and task negative network (alternately termed the default mode network, DMN) in which inverse correlations have been observed during resting state and event-related functional magnetic resonance imaging (fMRI). The antagonism between the 2 networks might indicate a dynamic interaction in the brain that is associated with development. To evaluate the alterations in the relations of the 2 networks in children with benign childhood epilepsy with centrotemporal spikes (BECTS), resting state fMRI was performed in 17 patients with BECTS and 17 healthy controls. The functional and effective connectivities of 29 nodes in the TPN and DMN were analyzed. Positive functional connectivity (FC) within the networks and negative FC between the 2 networks were observed in both groups. The patients exhibited increased FC within both networks, particularly in the frontoparietal nodes such as the left superior frontal cortex, and enhanced antagonism between the 2 networks, suggesting abnormal functional integration of the nodes of the 2 networks in the patients. Granger causality analysis revealed a significant difference in the degree of outflow to inflow in the left superior frontal cortex and the left ventral occipital lobe. The alterations observed in the combined functional and effective connectivity analyses might indicate an association of an abnormal ability to integrate information between the DMN and TPN and the epileptic neuropathology of BECTS and provide preliminary evidence supporting the occurrence of abnormal development in children with BECTS.

Objective: The GRIN2A gene has been associated with both benign childhood epilepsies and severe epileptic encephalopathies. This study evaluates the genotype-phenotype correlations in patients with GRIN2A variants. Methods: A systematic literature search was performed, identifying all patients reported before May 2014 with GRIN2A variants. Patients were classified in two groups based on their GRIN2A genotype. Group I genotypes had an expected detrimental effect on protein expression: nonsense, frameshift, splice site, or initiation codon sequence variants, translocations with breakpoints within GRIN2A, or deletions comprising GRIN2A. Group II genotypes were expected to result in an altered protein structure or function: missense or in-frame sequence variants. Results: Of 136 patients, 74 were included in group I and 62 in group II. Epilepsy was diagnosed in 86% of patients in both groups, with focal seizures being most common (82%). Benign epilepsy with centrotemporal spikes (BECTS) was significantly more often diagnosed in group II than in group I (51% versus 27%, p=0.03). Continuous spikes and waves during slow-wave sleep (CSWS) and Landau-Kleffner syndrome (LKS) occurred more often in group I than in group II (50% versus 37%), although not statistically significant (p=0.29). Mild to severe developmental problems related to speech and language (80%), cognition (66%), and motor skills (48%), and behavioural problems (61%) were present in both groups. Speech and language problems were significantly more often reported in group I than in group II (90% versus 69%, p=0.02), also in patients with no LKS/CSWS (94% versus 50%, p=0.01). Conclusion: GRIN2A variants are associated with a spectrum of epilepsies, mainly accompanied by language developmental problems. This epilepsy-aphasia syndrome spectrum ranges from relatively mild BECTS to more severe LKS/CSWS. This study shows that GRIN2A genotypes with an expected milder effect on protein function are associated with a relatively more benign phenotype including BECTS without language problems.

Objective: The GRIN2A gene has been associated with both benign childhood epilepsies and severe epileptic encephalopathies. This study evaluates the genotype-phenotype correlations in patients with GRIN2A variants. Methods: A systematic literature search was performed, identifying all patients reported before May 2014 with GRIN2A variants. Patients were classified in two groups based on their GRIN2A genotype. Group I genotypes had an expected detrimental effect on protein expression: nonsense, frameshift, splice site, or initiation codon sequence variants, translocations with breakpoints within GRIN2A, or deletions comprising GRIN2A. Group II genotypes were expected to result in an altered protein structure or function: missense or in-frame sequence variants. Results: Of 136 patients, 74 were included in group I and 62 in group II. Epilepsy was diagnosed in 86% of patients in both groups, with focal seizures being most common (82%). Benign epilepsy with centrotemporal spikes (BECTS) was significantly more often diagnosed in group II than in group I (51% versus 27%, p=0.03). Continuous spikes and waves during slow-wave sleep (CSWS) and Landau-Kleffner syndrome (LKS) occurred more often in group I than in group II (50% versus 37%), although not statistically significant (p=0.29). Mild to severe developmental problems related to speech and language (80%), cognition (66%), and motor skills (48%), and behavioural problems (61%) were present in both groups. Speech and language problems were significantly more often reported in group I than in group II (90% versus 69%, p=0.02), also in patients with no LKS/CSWS (94% versus 50%, p=0.01). Conclusion: GRIN2A variants are associated with a spectrum of epilepsies, mainly accompanied by language developmental problems. This epilepsy-aphasia syndrome spectrum ranges from relatively mild BECTS to more severe LKS/CSWS. This study shows that GRIN2A genotypes with an expected milder effect on protein function are associated with a relatively more benign phenotype including BECTS without language problems.

Objective: The GRIN2A gene has been associated with both benign childhood epilepsies and severe epileptic encephalopathies. This study evaluates the genotype-phenotype correlations in patients with GRIN2A variants. Methods: A systematic literature search was performed, identifying all patients reported before May 2014 with GRIN2A variants. Patients were classified in two groups based on their GRIN2A genotype. Group I genotypes had an expected detrimental effect on protein expression: nonsense, frameshift, splice site, or initiation codon sequence variants, translocations with breakpoints within GRIN2A, or deletions comprising GRIN2A. Group II genotypes were expected to result in an altered protein structure or function: missense or in-frame sequence variants. Results: Of 136 patients, 74 were included in group I and 62 in group II. Epilepsy was diagnosed in 86% of patients in both groups, with focal seizures being most common (82%). Benign epilepsy with centrotemporal spikes (BECTS) was significantly more often diagnosed in group II than in group I (51% versus 27%, p=0.03). Continuous spikes and waves during slow-wave sleep (CSWS) and Landau-Kleffner syndrome (LKS) occurred more often in group I than in group II (50% versus 37%), although not statistically significant (p=0.29). Mild to severe developmental problems related to speech and language (80%), cognition (66%), and motor skills (48%), and behavioural problems (61%) were present in both groups. Speech and language problems were significantly more often reported in group I than in group II (90% versus 69%, p=0.02), also in patients with no LKS/CSWS (94% versus 50%, p=0.01). Conclusion: GRIN2A variants are associated with a spectrum of epilepsies, mainly accompanied by language developmental problems. This epilepsy-aphasia syndrome spectrum ranges from relatively mild BECTS to more severe LKS/CSWS. This study shows that GRIN2A genotypes with an expected milder effect on protein function are associated with a relatively more benign phenotype including BECTS without language problems.

Benign familial infantile convulsions (BFIC) is a recently identified partial epilepsy syndrome with onset between 3 and 12 months of age. We describe the clinical characteristics and outcome of 43 patients with BFIC from six Dutch families and one Dutch-Canadian family and the encountered difficulties in classifying the syndrome. Four families had a pure BFIC phenotype; in two families BFIC was accompanied by paroxysmal kinesigenic dyskinesias; in one family BFIC was associated with later onset focal epilepsy in older generations. Onset of seizures was between 6 weeks and 10 months, and seizures remitted before the age of 3 years in all patients with BFIC. In all, 29 (67%) of the 43 patients had been treated with anti-epileptic drugs for a certain period of time. BFIC is often not recognized as (hereditary) epilepsy by the treating physician. Seizures often remit shortly after the start of anti-epileptic drugs but, because of the benign course of the syndrome and the spontaneous remission of seizures, patients with low seizure frequency do not necessarily have to be treated. If prescribed, anti-epileptic drugs can probably be withdrawn after 1 or 2 years of seizure freedom.

Purpose: Determine long-term outcome in a cohort of children with newly diagnosed benign childhood epilepsy with centrotemporal spikes (BECTS). Method: Thirty children with BECTS were included in the Dutch Study of Epilepsy in Childhood. All children were followed for 12-17 years. Twenty children had typical BECTS, ten had atypical BECTS (age at onset 5 years and 89% of >10 years. Mean duration of epilepsy from onset to TRFwas 2.7 years; mean age at reaching TRFwas 10.6 years. Many children (63%) had experienced one or more (secondary) GTCS. Eighty percent had used antiepileptic drugs (mean duration 3.0 years). As far as we know, none of the children had developed learning problems or had shown any 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. Conclusion: All children in our cohort, both 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 as described in earlier studies was prognostic in our cohort.

Benign childhood epilepsy with centro-temporal spikes (BECTS) is a form of epilepsy with no demonstrable anatomical lesion showing spontaneous seizure remission. During the active phase of the disease the children may show cognitive deficits. The objective of this study was to assess, in children with BECTS, the relationship between clinical-EEG aspects and performance in the school performance test (SPT), Raven's progressive matrixes test and the Wechsler Intelligence Scale for Children (WISC-III). Forty-two 7 to 11 year old children were included and the following tests carried out: anamnesis, neurological examination, electroencephalogram (EEG), SPT, Raven's test and WISC-III. The children with BECTS had normal IQ values but showed inferior performance in the SPT more frequently than "healthy" children, paired with respect to age and maternal scholastic level. There was moderate positive correlation between WISC-III results and the age when the seizures started and the educational level of the parents. On the other hand, aspects linked to the epileptic nature of BECTS, such as the number of seizures, time since last seizure and the number and lateralization of the centro-temporal spikes on the EEG, showed no correlation with the neuropsychological tests. A epilepsia benigna da infância com pontas centrotemporais (EBICT) é uma forma de epilepsia na qual não existem lesões anatômicas demonstráveis e há remissão espontânea das crises. Na fase ativa da epilepsia as crianças podem apresentar déficits cognitivos. O objetivo deste estudo foi avaliar, em crianças com EBICT, a relação entre aspectos clínico-eletrencefalográficos e o desempenho no teste de desempenho escolar (TDE), no teste das matrizes progressivas de Raven e na Escala Wechsler de Inteligência para Crianças (WISC-III). Foram incluídas 42 crianças de 7 a 11 anos de idade. Foram realizados: anamnese, exame neurológico, eletrencefalograma (EEG), TDE, teste de Raven e WISC-III. As crianças com EBICT tiveram valores normais de QI e apresentaram desempenho inferior no TDE mais freqüentemente que crianças "sadias" pareadas quanto à idade e à escolaridade materna. Houve correlação positiva moderada entre idade de início das crises e escolaridade dos pais com resultados do WISC-III. Por outro lado, aspectos ligados à natureza epiléptica da EBICT como número de crises, tempo decorrido da última crise, número e lateralidade das pontas centrotemporais ao EEG não mostraram correlação com os resultados dos testes neuropsicológicos.

To study clinical and EEG features of children with ictal vomiting and no underlying brain lesions (Panayiotopoulos syndrome). The subjects were 36 children aged 2-13 years. The onset of seizures occurred between 1 and 5 years of age. Fourteen children (38.8%) had a single seizure. Fourteen children (38.8%) had autonomic status epilepticus. Impairment of consciousness was reported in 30 (83.3%) children, eye deviation in 10 (27.7%) other autonomic symptoms and head deviation in 9, generalization in 8, visual symptoms in one child, and, speech arrest or hemifacial motor symptoms in 8 cases. The EEG showed occipital spikes or spike-wave complexes in 27 (75.0%) children, blocked by opening of the eyes in 8 (22.2%) cases. Nine patients (25%) also had rolandic spikes and 3 had extraoccipital spikes. Six (16.6%) patients had normal EEG. No clinical differences were observed between patients having occipital or extraoccipital spikes. In children only with autonomic seizures, the spikes are predominantly occipital but blockage by opening of the eyes is a less frequent feature. In some children there is an overlapping of different focal childhood idiopathic syndromes. Estudar aspectos clínico-eletrencefalográficos de crianças com vômito ictal e sem sinais de lesão cerebral (síndrome de Panayiotopoulos). Foram estudadas 36 crianças na faixa etária de 2-13 anos. O início das crises ocorreu entre 1 e 5 anos de idade. Quatorze crianças tiveram crise única. Status epilepticus foi observado em 14 (38,8%) casos. Distúrbio da consciência foi relatado em 83,3% das crianças, desvio ocular em 27,7%, outros sintomas autonômicos e desvio da cabeça em 26,4%, generalização em 23,5%, bloqueio da fala ou sintomas motores da hemiface em 23,5% das crianças e sintomas visuais em um caso. O EEG mostrou pontas ou complexos de ponta-onda em 27 (75,0%) casos, bloqueados pela abertura dos olhos em 8 (22,2%) pacientes. Nove pacientes tiveram também pontas rolândicas e 3, pontas extraoccipitais outras. O EEG foi normal em 6 crianças. Não houve diferença clínica entre as crianças com pontas occipitais e extraoccipitais. Em crianças com crises autonômicas as pontas foram de predomínio occipital, mas bloqueio pela abertura dos olhos foi pouco freqüente. Em alguns casos houve sobreposição de diferentes síndromes idiopáticas focais da infância.

The aim of this paper is to promote the correct classification of, and provide guidelines on, the diagnosis and management of panayiotopoulos syndrome (ps). an international consortium of established researchers in the field collaborated to produce a consensus document. the resulting document defines ps, characterizes its electro-clinical features, considers its likely pathogenesis, and provides guidance on appropriate management. we conclude that ps is a common idiopathic, benign seizure disorder of childhood, which should be classified as an autonomic epilepsy, rather than an occipital epilepsy.

SummaryIntroduction:Benign Epilepsy of Childhood with Centrotemporal Spikes (BECTS) is one of the most common childhood epilepsies with a good prognosis regarding the seizure and neuropsychological outcomes. However, recent reports indicate the presence of neuropsychological problems in a significant percentage of children with BECTS. Our study was aimed to examine the educational performance and neuropsychological functions along with clinical and electrographic characteristics in a cohort of children with BECTS.Methods:We identified a cohort of children with BECTS by screening medical and EEG recordings of patients attending our institute. Data were collected with a standard protocol. Their educational performance was evaluated by an interview with the parents. Neuropsychological and language tests were administered to children who had educational problems. Statistical analysis was done using the χ2-test.Results:Fifty children (29 boys and 21 girls; mean age of onset of epilepsy 7.84±2.87 years) who met the criteria for BECTS were included in this study. Atypical seizure characteristics for BECTS were observed in 26 (52%) children. EEG showed typical centrotemporal spike and wave discharges in all children, 42% of them had a tangential dipole in the frontocentral region. An additional extra-rolandic focus in the EEG was found in seven children (14%). Educational problems were identified in 27 children (54%); 19 of them had neuropsychological or language impairment (p=0.003). We found a statistically significant correlation between the occurrence of educational problems and the absence of a tangential dipole in the EEG (p

Rationale: Young people with epilepsy experience high death rates compared to the general population. Understanding the magnitude of risk and the causes of death (CoD) is essential for counseling and for potential prevention. Methods: We combined the mortality experiences of four cohort studies of newly diagnosed childhood epilepsy (onset 1m-16 or 17y). Deaths were ascertained by parent and physician report, review of national death indices and health records, and a surveillance system in place at one site. Death certificates, hospital records, and autopsy reports were obtained when available. CoD was categorized as SUDEP (Nasheff, 2012), other seizure-related, other natural, nonnatural, and unknown. Epilepsy was designated as complicated (neuro-deficit, intellectual impairment, brain lesion) or uncomplicated (normal exam and intellect, no identified underlying cause). Results: 2260 subjects were followed for 30,427 person-years (PY) for an average of 13.5 years (range 9.8 to 16.4y across studies). 79 deaths occurred; mean age at onset = 3.9y (range: 1m - 15.7y), age at death= 11y (range: 2m-30y). 42 (53%) were male; 71 (80%) had complicated epilepsy, and 60 (76%) had substantial cognitive impairment. Drug resistance was noted in 47 (59%), convulsions in 52 (66%), status epilepticus in 37 (47%), and 61 (77%) had seizures in the prior year. The crude death rate (CDR) was 260/100,000PY (95% CI=202, 317). The CDR for complicated epilepsy was 851 (653, 1049) and for uncomplicated epilepsy, 36 (95% CI=11, 61) per 100,000PY. Most deaths were due to natural causes and unrelated to seizures (N=58) with respiratory CoDs being the most common (N=43). These included 36 pneumonia (5 primary infectious, 19 aspiration, 12 unspecified), 5 respiratory insufficiency-NOS, and 2 other. Other natural CoDs were sepsis (N=3), shunt failure (N=3), and unspecified secondary to neurometabolic disease (N=3). 50/52 of these patients had moderate to severe cognitive impairment. Two children died of strokes occurring after the onset of seizures, and four died of medical conditions unrelated to their epilepsy (e.g. cancer). SUDEP accounted for 10 deaths (6 definite, 2 probable, 2 possible). Two of these deaths occurred in people with uncomplicated epilepsy. The CDR for SUDEP was 32.9 (CI=12.5, 53.2) overall and 9 (CI=0, 21.6) for uncomplicated and 95.1 (CI=29.5, 162.4) for complicated epilepsy. The remaining CoDs were other seizure-related (N=3), non-natural (2 suicide, 1 homicide, 2 accidental), and unknown (N=3). In all, 13 (16%) of deaths were seizure-related (including SUDEP). Conclusions: Deaths in young people with epilepsy are largely due to infections and complications of severe neurologic impairment. While seizure-related deaths account for

Purpose: Determine long-term outcome in a cohort of children with newly diagnosed benign childhood epilepsy with centrotemporal spikes (BECTS). Method: Thirty children with BECTS were included in the Dutch Study of Epilepsy in Childhood. All children were followed for 12-17 years. Twenty children had typical BECTS, ten had atypical BECTS (age at onset 5 years and 89% of >10 years. Mean duration of epilepsy from onset to TRFwas 2.7 years; mean age at reaching TRFwas 10.6 years. Many children (63%) had experienced one or more (secondary) GTCS. Eighty percent had used antiepileptic drugs (mean duration 3.0 years). As far as we know, none of the children had developed learning problems or had shown any 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. Conclusion: All children in our cohort, both 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 as described in earlier studies was prognostic in our cohort.