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Objective Neonatal seizures are often the first symptom of perinatal brain injury. High-frequency oscillations (HFOs) are promising new biomarkers for epileptogenic tissue and can be found in intracranial and surface EEG. To date, we cannot reliably predict which neonates with seizures will develop childhood epilepsy. We questioned whether epileptic HFOs can be generated by the neonatal brain and potentially predict epilepsy. Methods We selected 24 surface EEGs sampled at 2048 Hz with 175 seizures from 16 neonates and visually reviewed them for HFOs. Interictal epochs were also reviewed. Results We found HFOs in thirteen seizures (7%) from four neonates (25%). 5025 ictal ripples (rate 10 to 1311/min; mean frequency 135 Hz; mean duration 66 ms) and 1427 fast ripples (rate 8 to 356/min; mean frequency 298 Hz; mean duration 25 ms) were marked. Two neonates (13%) showed interictal HFOs (285 ripples and 25 fast ripples). Almost all HFOs co-occurred with sharp transients. We could not find a relationship between neonatal HFOs and outcome yet. Conclusions Neonatal HFOs co-occur with ictal and interictal sharp transients. Significance The neonatal brain can generate epileptic ripples and fast ripples, particularly during seizures, though their occurrence is not common and potential clinical value not evident yet.

Several studies have been conducted to determine the risk of recurrence after withdrawal of antiepileptic drugs (AEDs) in recent years. There is no consensus concerning the circumstances affecting discontinuation of AEDs. This study was designed to determine the recurrence rate of epilepsy after withdrawal of AEDs and the risk factors related with recurrence.Children with epilepsy onset between 1 month and 16 years of age who were followed up at least 3 years after AED treatment withdrawal were enrolled. Patients were classified into groups according to defined risk factors for recurrence.A total of 284 patients, 137 (48.2%) girls and 147 (51.8%) boys were included, and seizures recurred after withdrawal in 51 patients (18%). Thirty-three (64.7%) patients had recurrence in the first year after withdrawal. The recurrence risk was calculated based on the electro-clinical syndromes classification; the recurrence risk was the highest in the juvenile myoclonic/absence group and lowest in the benign infantile seizure group. No recurrence was observed in the infantile spasm group. Data evaluated by multivariable analysis showed that having the structural-metabolic and unknown epilepsy and3 years seizure free period before withdrawal of AEDs were the main risk factors for recurrence after AED withdrawal in our study.We suggest a seizure-free period of at least 3 years under AED medication and we must be cautious in patients with structural-metabolic and unknown epilepsy before AED withdrawal.

Child neurologists should provide initial care for the mental health problems of children and adolescents with epilepsy. Attention deficit hyperactivity disorder (ADHD), autism spectrum disorders are common comorbidities of childhood epilepsy. The psychotropic drugs used to treat mental health disorders can be safely employed in children with seizures. Child neurologists can diagnose common behavioral problems, should be comfortable with first-line agents to treat common psychiatric illnesses, and should recognize when support from psychologists or child and adolescent psychiatrists is needed. This article is part of the Special Issue "Obstacles of Treatment of Psychiatric Comorbidities in Epilepsy".

Objective: Caring for a child with a chronic illness adds stress to the typical parenting stress in healthy developing children. This stress can place a heavy burden on parents and may increase when a child displays problem behavior. In general, parenting and child's behavior problems are associated. Furthermore, externalizing (more outgoing) behavior is reported frequently in children with frontal lobe epilepsy (FLE). Therefore, in this study, we first investigated the burden of parents of children with FLE, and second, we investigated the relation between the experienced burden and reported behavioral problems. The validity of parents' reports on proxy measures as well as duration of epilepsy is taken into account.Methods: Thirty-one parents of children with FLE completed validated questionnaires about behavioral problems and burden of parenting. To examine if parents tend to be inconsistent or unusually negative, we used the two validity scales of the Behavioral Rating Inventory of Executive Function (BRIEF) (Negativity and Inconsistency).Results: Only parents of children with FLE who have had epilepsy for 5 years or longer report more problems on the Nijmeegse Vragenlijst voor de Opvoedingssituatie (NVOS) subscales 'Able to manage', 'Child is a burden', and 'Good Interaction' compared with the healthy controls. The subscale 'Child is a burden' significantly predicts scores in about 20% to 49% on the main scales of the Child Behavior Checklist (CBCL), the Global Executive Composite (GEC), and Behavioral Regulation Index (BRI) of the BRIEF. Only 6% of parents scored in the dinical range of the negativity scale of the BRIEF. For the inconsistency scale, this was 45%.Conclusion: Parents of children with FLE do not report excessive parental burden. Longer duration of epilepsy might be a risk factor in experiencing burden. The findings suggest a link between parental burden and behavioral problems in children with FLE. Externalizing behavioral problems are the most marked behavioral problems, which relate to the parental burden. Parents tend to be inconsistent in their ratings. (C) 2019 Elsevier Inc. All rights reserved.

Children with Benign Epilepsy with Centrotemporal Spikes (BECTS), despite high likelihood for seizure remission, are reported to have subtle difficulties in language and other cognitive skills. We used functional MRI and a story listening task to examine the effect of BECTS on patterns of activation and connectivity. Language and cognitive skills were assessed using standardized measures. Twenty-four children with recently diagnosed BECTS and 40 typically-developing children participated. In a functionally-defined region of interest in right inferior frontal gyrus, BECTS patients showed a lower level of activation. Across both groups combined, increased activation in superior/middle temporal regions of interest was associated with better language scores. Connectivity in the story processing network was similar between groups, but connectivity within left inferior frontal gyrus was decreased in children with BECTS. These results suggest that language networks are largely maintained in new-onset BECTS, but some subtle changes in activation and connectivity can be observed.

Purpose Benign childhood epilepsy with centrotemporal spikes (BECTS) is strongly related to age, both to age at the time of seizure onset and to age at remission. However, the age of remission varies. The present study analyzed factors associated with remission of BECTS. Methods Sixty-nine children with BECTS were retrospectively analyzed. Thirty-eight (55.1%) were boys and 31 (44.9%) were girls. Mean age at seizure onset was 86.36 ± 24.55 months (range: 41–151 months). Results The mean age at the time of EEG improvement or remission was 138.31 ± 19.71 months. Of the 69 patients, 36 (52.2%) exhibited electroencephalography (EEG) improvement or remission before age 11.5 years, whereas 33 (47.8%) showed later improvement or remission. The two groups differed significantly in age at seizure onset. When patients were divided into groups using an age cutoff for last seizure of 9 years, the two groups differed significantly in age at seizure onset. Conclusions The most important predictor of early BECTS remission is age at seizure onset.

Rationale It is still not clear if there are EEG parameters that may be related to the epilepsy etiology in epilepsies presenting with rolandic spikes. Rolandic spikes are not pathognomonic for rolandic epilepsy and could be related to the area of discharges itself. The initial hypothesis was that even visually identical spikes have some difference, because of the different etiology. Objective The aim of the study was to find the differences in rolandic spike morphology in two epilepsy groups, different by etiology, but presenting with visually identical spikes. Methods A novel algorithm for automatic classification of interictal electroencephalogram (EEG) rolandic spikes according to the epilepsy type (Group I – patients with benign focal childhood epilepsy, self-limiting, with no causal lesion in the brain, Group II – patients with structural focal epilepsy) is proposed. The algorithm consists of three stages: 1) EEG spike detection, 2) determination of EEG spike parameters, 3) classification of EEG by epilepsy type based on estimated spike parameters. Automatic classification method is defined by artificial neural network. The algorithm has been trained and tested on a large data sample provided by Children's Hospital, Affiliate of Vilnius University Hospital Santaros Klinikos. Only those EEGs that were visually identical and inaccessible for manual clustering to the groups according the visual spike morphology and contained 50 or more spikes have been analyzed. Training and testing pools have been selected as non overlapping (containing different patients) data sets. Results The proposed methodology let us to achieve up to 75% of accuracy of classification of EEG.

Objective The aim of this study was to assess measurement equivalence in the 16-item short-form Quality of Life in Childhood Epilepsy Questionnaire (QOLCE-16) across age, sex, and time in a sample of children with newly diagnosed epilepsy. Methods Data came from 373 children participating in the Health-related Quality of Life in Children with Epilepsy Study (HERQULES), a multicenter prospective cohort study. Measurement equivalence was assessed using multigroup confirmatory factor analysis methods. Comparison groups were stratified by age (4–7 years vs. 8–12 years), sex (male vs. female), and time (at diagnosis vs. 24 months postdiagnosis). Results The QOLCE-16 demonstrated measurement equivalence at the level of strict invariance for each comparison group tested — age: χ2 (3, 274) = 429.6, p Significance The findings provide support for the robust psychometric profile of the QOLCE-16 as a reliable and valid measure of health-related quality of life for children with epilepsy. Demonstrating good properties and a multidimensional structure, the QOLCE-16 is an appropriate short measure for both clinicians and researchers wanting to obtain health-related quality of life information on children with epilepsy.

Introduction Epilepsy is a common childhood neurological condition and a major public health concern worldwide. A higher incidence of epilepsy is reported in low- and middle-income countries, particularly in rural areas. However, no Japanese reports on the incidence of childhood epilepsy have been published in the past 25 years. We estimated the annual incidence of epilepsy in children aged 1–14 years in Uwajima, a city in a rural, relatively isolated area of Japan. Methods Candidates were extracted from Japan’s public insurance database following the International Classification of Diseases code for epilepsy. Epilepsy was defined as two or more unprovoked seizures more than 24 hours apart, as per the International League Against Epilepsy definition. The study sample was divided into three cohorts based on age at diagnosis: 1–4, 5–9, and 10–14 years. The incidence of epilepsy was calculated as the number of children with epilepsy divided by the person-years in each cohort. Results The annual incidence rate of epilepsy in children aged 1–14 years was 70.4/100,000 children (95% confidence interval, 44.8–96.0). There was no significant difference in incidence between boys and girls. This rate was similar to those reported in other countries, although the incidence in children aged 1–4 years was slightly higher in our study than in other countries. Conclusion The annual incidence of childhood epilepsy in rural areas of Japan is generally comparable with rates of childhood epilepsy reported in other countries.

Purpose Epilepsy is an important neurological condition that frequently associated with neurobehavioral disorders in childhood. Our aim was to identify the risk of developing epilepsy subsequent to febrile seizure and the association between epilepsy risk factors and neurobehavioral disorders. Subjects and methods This longitudinal population-based cohort data included 952 patients with a febrile seizure diagnosis and 3808 age- and sex-matched controls. Participants were recruited for the study from 1996 to 2011, and all patients were followed up for maximum 12.34 years. Results The association of epilepsy was significantly higher (18.76-fold) in individuals that experienced febrile seizure compared to controls. Further, of those individuals who experienced febrile seizure, the frequency of subsequent development of epilepsy was 2.15-fold greater in females, 4.846-fold greater in patients with recurrent febrile seizure, and 11.26-fold greater patients with comorbid autism. Conclusions Our study showed that being female, comorbid autism with febrile seizure and recurrent febrile seizure had an increased association with development of epilepsy. Increased recognition the association for epilepsy might be warranted in those febrile seizure children with certain characteristics.

Purpose Surgical procedures are increasingly used as a treatment option for paediatric patients with refractory epilepsy as seizure relief is often assumed to be associated with positive psychosocial and quality of life outcomes. This study aimed to explore children’s and parents’ experiences of epilepsy surgery and associated psychosocial outcomes. Methods Semi-structured interviews were conducted with parents and their children aged between 7 and 17 years old, who had undergone epilepsy surgery within the previous six months to three years. Analysis of interview data was interpretive and iterative, using the constant comparison approach, to develop a rich understanding of family experiences throughout the surgery journey. Results Interviews were conducted with 16 participants including seven children (aged 8–15 years) and their nine parents. Epilepsy had significant impact on quality of life for children and parents. Concerns about long term wellbeing, risks to safety and hope for a ‘normal’ life contributed to the decision to pursue surgery. All participants described improvements in seizure control, alongside improved psychological wellbeing, quality of life, social relationships and family functioning. However, children and their parents experienced difficulties adjusting to a new post-surgical identity and ‘missing’ aspects of their pre-surgical life with epilepsy. Conclusions Epilepsy surgery had a positive impact on participants’ lives, but families described difficulties in adjusting to postsurgical changes and leading a ‘normal’ life. Our findings suggest that families would benefit from additional information and clinical support leading up to epilepsy surgery to help them prepare for and adjust to a life without epilepsy.

According to the operational epilepsy definition adopted by the International League Against Epilepsy (ILAE) in 2014, in patients with one unprovoked seizure, clinicians must stratify the recurrence risk to determine if the criteria for diagnosis of epilepsy have been met and if antiseizure medications (ASM) are required. A remote symptomatic etiology was considered to be one of the best predictors for seizure recurrence, also according to the available prediction tools, but in children with a previously negative history and a normal neurological examination, estimating the probability of seizure relapse remains less obvious. This meta-analysis aimed to fill this gap of knowledge.The PubMed, Embase, and Scopus databases were searched from January 2000 to December 2020. We selected studies reporting children (1 month-18 years old) presenting a first unprovoked seizure. The absence of a known remote neurological pathology had to be clearly stated in the paper or the idiopathic/cryptogenic group data were used; the finding of epileptogenic structural brain MRI abnormalities during the diagnostic workup at the moment of the first unprovoked seizure was not an exclusion criterion. Factors analyzed, as possible predictors of recurrence, included: age at onset, sex, family history of epilepsy, type of seizure (focal or generalized), epileptiform abnormalities on EEG, and epileptogenic structural brain MRI abnormalities.Four studies met the inclusion criteria and the sample size consisted of 741 children. The estimated recurrence rate within 3 years was 50% (95%CI:33.9%-66.0%). Among the predictors of recurrence, focal seizure (OR = 1.52; 95%CI = 1.05-2.19), epileptiform abnormalities on EEG (OR = 1.97; 95%CI = 1.31-2.96), and positive family history for epilepsy (OR = 2.37; 95%CI = 1.56-3.59) were associated with a statistically significant increased risk.The analysis of data available until now cannot adequately assess the risk of recurrence after a first unprovoked seizure in neurotypical children. Prospective and multicenter cohort studies are expected.

Epileptic encephalopathy with continuous spikes-and-waves during sleep (EE-CSWS) is a rare childhood epilepsy syndrome characterized by a regression in cognitive, behavioral and psychiatric functioning, seizures and a specific electroencephalographic pattern. An early recognition and an appropriate treatment might play a key role in the outcome of this epileptic encephalopathy. We conducted a case-control study to evaluate if there is any clinical or electroencephalographic sign suggestive of EE-CSWS after the first seizure. We retrospectively identified 10 EE-CSWS patients with available EEG recordings at time of the first seizure. We matched them with 10 controls from our first seizure clinics. All EEG recording were analyzed for the study. We did not find any clinical or EEG features that would suggest later development of EE-CSWS. As reported by others, the occurrence of multiple seizures types and a seizure worsening during the follow-up is more frequent in the cases than in the controls. These clinical criteria might be used as a red flag in clinical practice to identify the very few patients with EE-CSWS among the frequent patients with BECTS.

Callosotomy is a palliative surgery method for selected individuals with severe, drug-resistant epilepsy. The aim of this retrospective study was to explore parental experiences of the family's life situation before and long after their child had undergone callosotomy. Semistructured interviews of the parents of 12 children were analyzed using a combination of inductive and deductive qualitative content analysis. Before surgery, parents felt that they lived in a chaotic bubble with an unbearable situation; their child had severe and frequent seizures and had to be looked after constantly. Most parents were both satisfied and dissatisfied with the given support and information. However, if the child did not improve after surgery, parents often felt that the information before surgery had not been adequate. After surgery, they found a glimpse of hope. They felt that the family got a new life; the reduced seizure severity led to a better life situation for the family. The support was described as both good and poor. The family life situation was complex, and even if they were partly satisfied with the support, it was still not enough. However, the life situation was also very stressful because of remaining seizures, behavioral problems, and sometimes, adverse effects of surgery. The families lived in disappointment and difficulty and had to fight for their rights. This indicates that these families need more information and social service coordination both before and long after surgery. They need not only tools to manage the child's disabilities but also substantial help to care for the child and to receive the social support they need.

The mainstay of treatment of childhood epilepsy is to administer antiepileptic drugs (AEDs). This article provides an overview of the clinical approach to drug treatment of childhood epilepsy, focusing on general principles of therapy and properties of recently introduced medications. Initiation and cessation of therapy, adverse medication effects, drug interactions, indications for the various AEDs, and off-label use of AEDs are reviewed. The distinct challenges in treatment of epileptic spasms and neonatal seizures are addressed. Finally, ideas for the future of drug treatment of childhood epilepsy are presented, with particular attention to precision medicine.

To investigate the morphological changes of cerebral cortex correlating with anti-seizure medication in Childhood Epilepsy with Centrotemporal Spikes (CECTS), and their relationships with seizure control. This study included a total of 188 children, including 62 patients with CECTS taking anti-seizure drugs, 56 patients with drug-naive, and 70 healthy controls. A portion of cases were also followed-up for longitudinal analysis. Cortical morphological parameters were quantitatively measured by applying surface-based morphometry analysis to high-resolution three-dimension T1 weighted images. Among the three groups, the morphological indices were compared to quantify any cortical changes affected by seizures and medication. The relationships among anti-seizure medication, seizure controls and cortical morphometry were investigated using causal mediator analysis. The Rolandic cortex of the drug-naive patients showed abnormal cortical thickness by comparing with that of healthy controls, and thinning by comparing with that of patients with medication. The cortical thickness in the Rolandic regions was negatively correlated with duration of medication and duration of seizure-free. Longitudinal analysis further demonstrated that the thickness of Rolandic cortex thinned in post-medication state relative to the pre-medication state. Mediation analysis revealed that morphological alteration of the Rolandic cortex might act as a mediator in the path of anti-seizure medication on seizure control. Our findings highlighted that anti-seizure medication was associated with regression of abnormal increment of cortical thickness in the Rolandic regions in CECTS. The neuroanatomical alteration might be a mediating factor in the process of seizure control by anti-seizure medication.

Background:- \ud \ud Benign 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.\ud \ud Method:- \ud \ud Twenty-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.\ud \ud Results:- \ud \ud When 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.\ud \ud Discussion:- \ud \ud Children 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.

By exploring the meanings children and their parents attached to two household treatments for childhood epilepsy (antiepileptic drugs and emergency medications), this paper broadens our understanding of the ways in which children view their medications and how these views can impact on their adherence to treatment. The paper draws on data collected during 2013 and 2014 from 24 families across the UK that had a child with epilepsy aged 3–13 years. In-depth semi-structured interviews were conducted with the parents and 10 children participated in autodriven photo-elicitation interviews. Parents' and children's perceptions of medications were compared and contrasted and the findings show that although both parents and children viewed medications as an unpleasant necessity, parents’ concerns centred on the perceived side effects of medications, whereas the children commented on the process of ingesting medications. Additionally, some of the children had to learn that their medications were a preventative measure, as they originally viewed them as a cure. Furthermore, among the children, it was found that treatment could be seen as either a positive or negative symbol of difference. Lastly, emergency medications were often viewed as a saviour, particularly by parents. Through this analysis, the study shows that the meanings children attached to medications were often linked to the form the medication took and that the way in which children perceived their medications had implications for their adherence; indeed, those who viewed their medications as an unpleasant necessity and those who initially saw their treatment as a cure were most likely to stop taking their medications. Consequently, this paper contributes to the current literature on the meanings individuals attach to treatments by providing the first detailed insight into children's views on epilepsy medications and by illustrating the similarities and differences in children's and parents' perspectives.

Purpose Benign Childhood Epilepsy with Centrotemporal Spikes (BCECTS) is the most common childhood idiopathic focal epilepsy with a good outcome. However, an increasing number of reports suggest a ‘not so benign’ outcome showing atypical evolution. Methods We analyzed the electroencephalographic (EEG) spike source dipoles of BCECTS patients to compare patients with confirmed typical and atypical outcome showing intractable epilepsy and cognitive decline. Thirty-seven patients that were diagnosed for BCECTS and whose clinical information was sufficient enough to confirm long-term outcome were included in the study. Eight patients (22%, 7 patients for poor seizure control and 1 patient for cognitive decline) were classified as showing atypical outcome. Forty-seven averaged spike dipole sources were analyzed using the single equivalent current dipole (ECD) method. The inverse problem was solved using the 4 shells ellipsoidal model. Results The coordinate value of yori differed significantly between the two groups (mean ± standard deviation, 0.32 ± 0.33 for the typical group and −0.33 ± 0.77 for the atypical group, P Conclusion We can conclude that there are significant differences in spike source dipole in patients with BCECTS that shows atypical outcome, and we can use this finding as a marker for predicting an outcome at the time of diagnosis.

Background: Benign childhood epilepsy with centrotemporal spikes (BECTS), also known as Rolandic Epilepsy, is one of the most common forms of focal childhood epilepsy. Despite its prevalence, BECTS is challenging to diagnose because of the nocturnal and brief nature of the seizures. While most epilepsy research has focused on early warning signs of seizure onset, a separate challenge is to detect functional characteristics - biomarkers - that indicate the brain has entered a dynamical state in which seizures are more likely to occur. This has the potential to more reliably evaluate the underlying neurophysiology associated with BECTS and potentially predict clinical outcomes, and improve diagnostic screening for BECTS. Methods: Electronic medical records were retrospectively reviewed to identify patients with BECTs diagnosis codes admitted to the long-term monitoring unit at Boston Children's Hospital (BCH). Thirty one patients with BECTS (13 female, median age 9.5 years) and thirty-one control patients (median age 10 years, 10 female) were selected for inclusion in this study. EEGs were reviewed to confirm the BECTS diagnosis. EEG segments that lacked epileptiform activity were selected for analysis from awake BECTS patients and controls. Multiscale nonlinear methods were used to computed dynamical values from the EEG segments. Findings: Two related findings are reported in this study. First, several nonlinear measures derived from EEGs are clearly different in the centrotemporal region of BECTS patients when compared to the same region in controls. Because all results are from awake patients, this suggests a possible biomarker for BECTS in an awake patient. Secondly, these measures clearly distinguish the centrotemporal and extra-centrotemporal regions in the same patient, suggesting that these nonlinear measures may tentatively be useful for detecting the epileptogenic zone. Interpretation: The results presented in this study suggest that nonlinear features computed from EEG signals may be useful for identifying BECTS in awake patients, introducing a digital biomarker with the potential to improve diagnostic screening for BECTS and predict clinical outcomes. The finding that the centrotemporal region appears to be dynamically different from the rest of the brain in BECTS patients suggests the possibility that the nonlinear methods used in this study may be useful for identifying the irritative or epileptic zone. Funding Statement: Dr. Sathyanarayana was supported by T32HD040128 from the NICHD/NIH. Dr. Loddenkemper, Dr. El Atrache and Ms. Jackson were supported by the Epilepsy Research Fund. Declaration of Interests: WJB and TL are named on a patent submitted and held by the Boston Children’s Hospital Technology Development Office that includes the signal analysis methods discussed in this article. TL is part of patent applications to detect and predict clinical outcomes, and to manage, diagnose, and treat neurological conditions, epilepsy, and seizures. The authors declare that they have no other competing financial or nonfinancial interests. Ethics Approval Statement: Approval from the Boston Children’s Hospital Institutional Review Board was obtained prior to data acquisition.

Paradoxical vocal fold motion (PVFM) is characterized by vocal fold adduction during respiration. Benign Rolandic epilepsy (BRE) is the most common childhood epilepsy and can cause oropharyngolaryngeal or facial manifestations. A 9-year-old male presented with intermittent apnea lasting 30-60 seconds and presumed PVFM. The patient's physical and fiberoptic exam were normal. He was admitted and found to have episodes of oxygen desaturation, neck twitching, and tongue burning. An EEG revealed focal epilepsy. After starting anti-epileptic medications, he had resolution of symptoms. Our patient was eventually diagnosed with BRE, a focal onset epilepsy that can mimic primary otolaryngologic disease.

Highlights • Occipital Intermittent Rhythmic Delta Activity (OIRDA) is seen in pediatric focal epilepsies. • Spatial and temporal association is noted between OIRDA and other epileptiform discharges. • Atypical or focal features of OIRDA may suggest a link to a focal epileptogenesis in these patients., In this case series, we have identified an atypical pattern of OIRDA (Occipital intermittent rhythmic delta activity) on the electroencephalograms (EEGs) of three pediatric patients with self-limited focal epilepsies, including Childhood Epilepsy with Centrotemporal Spikes (CECTS), and Panayiotopoulos syndrome (PS). Previously, OIRDA was described as a symmetric sinusoidal occipital-maximal activity, often associated with childhood idiopathic generalized epilepsies, although it was also reported among other physiologic or pathological entities including focal epilepsy. We have observed in our case series that OIRDA, without prominent field effect, is lateralized or maximal on the hemispheric side ipsilateral to the more defining epileptiform discharges in these focal epilepsies. They also exhibit a notched morphology due to the intermixed sharp wave activities, although the sharp waves are not occurring repetitively. This report provides additional evidence that OIRDA can be associated with a spectrum of idiopathic focal epilepsies and may suggest a cortical origin of OIRDA in these patients as opposed to a hypothesized subcortical generator in patients with primary generalized absence epilepsy, even though further investigation is warranted for either hypothesis.

Background Cannabidiol (CBD) has gained popularity among parents of children with epilepsy, even before evidence of efficacy and safety was available. The aim of our survey was to gain information about parental attitude to CBD, as well as expectations and knowledge of CBD for treatment of their child's epilepsy. Methods A survey using an open-access online questionnaire for parents or caregivers of children with epilepsy within German-speaking countries from March to June 2019 was used. Results Of 378 complete questionnaires (mean age of children: 11.1 (standard deviation [SD] 7.4) years), 28% (n = 106) reported previous or current CBD treatment over a mean time of 17.31 months (SD: 19.74), whereas 72% had no personal experience with CBD. Treatment was proposed by parents and not by physicians in 83% of cases and was mainly carried out with prescription-only products (71%, n = 67). Nevertheless, 29% used unregulated, artisanal products. Of all parents with previous experience, n = 77 (73%) reported that they expected CBD to be more efficient than the common antiseizure drugs (ASDs) at the beginning. Forty-five percent reported that their expectations were not met during therapy. Consistently, lack of seizure reduction was the most common reason to discontinue CBD (12/26). Of those responders without CBD experience, 93% would consider CBD for their child. However, the self-reported level of information was considered to be poor or very poor regarding efficacy (76%, n = 177), tolerance (83%, n = 191), interaction with other medication (91%, n = 211), and potential long-term effects (87%, n = 212). Conclusions There is a huge interest in CBD but includes potentially unrealistic expectations of its efficacy and tolerance combined with a low level of information. Neuropediatricians should address parents of children with epilepsy regarding potential motivation and expectations of CBD. In addition, parental education, especially on interactions and potential side effects, is strongly recommended.

Objectives Disease burden has always been based on associated mortality. An accurate measurement of the burden of epilepsy should rely on both morbidity and mortality. This will close any existing gap in knowledge and provide useful information to aid evidence-based decision-making. In this study, burden of epilepsy was estimated, using disability-adjusted-life-years (DALYs), using disability weights for epilepsy that were part of the Global Burden of Disease 2010 work. Methods The study was conducted at the University of Nigeria Teaching Hospital, Enugu. Interviewer-administered questionnaire was used to collect information from patients with epilepsy who presented to neurology clinic. The prevalence of epilepsy, and case-fatality were obtained from previous publications. The DALYs were estimated by adding together the years lost to disability (YLDs) and years lost to life (YLLs) to epilepsy (DALYs = YLD + YLL). DALYs were dis-aggregated by age group and by whether or not epilepsy was treated. Results A total of 134 children with epilepsy-interviews were conducted. Some 56% and 44% of the subjects had primary and secondary epilepsy, respectively. The childhood epilepsy caused 1.63 YLLs per 1000 population, 0.45 YLDs per 1000 population and 2.08 DALY per 1000 population. The highest burden was in children within the age group of 5–14 years at 2.18 DALY per 1000 people. The YLDs was higher (0.63/1000 population) among the untreated group, compared with the YLDs (0.27/1000 population) among the treated group. The YLLs lost for children with secondary epilepsy (2.23/1000 population) was higher than primary epilepsy YLLs of 1.07/1000 population. Significance The DALYs due to childhood epilepsy was high. The YLDs was high among children with epilepsy who were not on treatment. The YLLs were found to be the same in all children with epilepsy, irrespective of their treatment status. This imperatively necessitates the de-emphasis on just mortality as an indicator of the burden of childhood epilepsy but rather a holistic approach should be adopted in considering both the mortality and disability in monitoring the outcome of health interventions.

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.

PurposeTo explore intrahemispheric, cortico-cortical EEG functional connectivity (EEGfC) in benign childhood epilepsy with rolandic spikes (BECTS).Methods21-channel EEG was recorded in 17 non-medicated BECTS children and 19 healthy controls. 180s of spike- and artifact-free activity was selected for EEGfC analysis. Correlation of Low Resolution Electromagnetic Tomography- (LORETA-) defined current source density time series were computed between two cortical areas (region of interest, ROI). Analyses were based on broad-band EEGfC results. Groups were compared by statistical parametric network (SPN) method. Statistically significant differences between group EEGfC values were emphasized at p

To explore the information sensing of 1 of 18F-FDG PET / CT brain metabolic imaging in measuring preoperative localization of childhood epilepsy focus and surgical decision-making. The clinical data of 69 childhood patients who underwent 18F-FDG PET / CT brain metabolism imaging measurement in our hospital from June 2013 to September 2019 were retrospectively analysed to evaluate 18F-FDG PET / CT brain metabolism. The application value of imaging in the localization of epilepsy-induced epilepsy, and compared with MRI, explore the application value of 18F-FDG PET / CT brain metabolic imaging in epilepsy focus location and surgical treatment options before epilepsy surgery. Of the 69 patients, 53 patients had positive 18F-FDG PET / CT brain metabolic imaging, 16 patients showed negative, and the epilepsy foci were accurately located in 48 patients, and some of them were partially detected. For epilepsy foci, 18F-FDG PET / CT brain metabolic imaging has a consistent localization rate of 73.9% (51/69). 43 cases were positive by magnetic resonance examination, accurate positioning of the epileptogenic focus in 38 children, and partial positioning of 3 children. The consistent rate of localization of the epileptogenic focus was 59.4% (41/69). 18F-FDG PET / CT brain the coincidence rate of metabolic and MRI examinations was 76.8% (53/69). After Kappa consistency test, Kappa = 0.466. 18F-FDG PET / CT brain metabolism imaging has higher consistency in the location of epilepsy focus and electrophysiological examination before refractory epilepsy, which is better than MRI examination. The combined use of the two examinations can improve preoperative epilepsy. The positioning of the foci helps clinicians to choose suitable children for surgery and exclude children who are not suitable for surgery.

6 páginas, 3 figuras, Lafora disease (LD) is both a fatal childhood epilepsy and a glycogen storage disease caused by recessive mutations in either the Epilepsy progressive myoclonus 2A (EPM2A) or EPM2B genes. Hallmarks of LD are aberrant, cytoplasmic carbohydrate aggregates called Lafora bodies (LBs) that are a disease driver. The 5th International Lafora Epilepsy Workshop was recently held in Alcala de Henares, Spain. The workshop brought together nearly 100 clinicians, academic and industry scientists, trainees, National Institutes of Health (NIH) representation, and friends and family members of patients with LD. The workshop covered aspects of LD ranging from defining basic scientific mechanisms to elucidating a LD therapy or cure and a recently launched LD natural history study., Research reported in this publication was supported by the National Institute of Neurological Disorders and Stroke of the National Institutes of Health under Award Numbers R01NS070899 (MSG) and P01NS097197 (MSG), the Spanish Ministry of Education and Science Award Number SAF2017-83151-R (PS), a grant from the Fundacion Ramon Areces CIVP18A3935 (PS), Spanish MINECO BFU2017-84345-P (JJG), Valerion Therapeutics (MSG), Ionis Pharmaceuticals.

Purpose To assess the role of resective brain surgery in childhood epilepsy. Methodology We retrospectively analysed the seizure outcomes in 55 children with epilepsy who had resective brain surgery between 1997 and 2012, at our centre. The children were 1.5–18 years at the time of surgery; their seizure onset was between 0.2 andto 15 years of age. 48 had refractory epilepsy. One child died of tumour progression. Follow-up duration in the survivors ranged from 2 to −16 years (mean: 9).Presurgical evaluation included clinical profiles, non-invasive V-EEG monitoring, neuroimaging with MRIs in all; SPECT and PET in selected patients. 54 had intraoperative ECoG. Results An Engel Class 1 outcome was seen in 78% of the cohort, with 67% being off all AEDs at the most recent follow-up. Children with tumours constituted the majority (56%), with 87% of this group showing a Class 1 outcome and 84% being off AEDs. Children with cortical dysplasia had a Class 1 outcome in 56%. Conclusion Resective brain surgery is an efficacious option in some children with epilepsy. We found ECoG useful to tailor the cortical resection and in our opinion ECoG contributed to the good seizure outcomes.

Dravet syndrome is a rare form of epilepsy largely refractory to current antiepileptic medications. The only precedents of randomized placebo-controlled trials in Dravet syndrome are the two small trials that led to the approval of stiripentol. With the arrival of new clinical trials for Dravet syndrome, we sought to determine the characteristics of the patient population with Dravet syndrome in Europe today, which has possibly evolved subsequent to the approval of stiripentol and the ability to diagnose milder clinical cases via genetic testing. From May to June 2014, we conducted an online parent-reported survey to collect information about the demographics, disease-specific clinical characteristics, as well as current and past use of antiepileptic medications by European patients with Dravet syndrome. We present data from 274 patients with Dravet syndrome from 15 European countries. Most patients were between 4 and 8years of age, and 90% had known mutations in SCN1A. Their epilepsy was characterized by multiple seizure types, although only 45% had more than 4 tonic–clonic seizures per month on average. The most common drug combination was valproate, clobazam, and stiripentol, with 42% of the total population currently taking stiripentol. Over a third of patients with Dravet syndrome had taken sodium channel blockers in the past, and most had motor and behavioral comorbidities. Our study helps define the current typical European patient with Dravet syndrome. The results from this survey may have important implications for the design of future clinical trials that investigate new treatments for Dravet syndrome.

Item does not contain fulltext PURPOSE: The objective of this study was to estimate the expected cost-utility and cost-effectiveness of the ketogenic diet (KD), vague nerve stimulation (VNS) and care as usual (CAU), using a decision analytic model with a 5-year time horizon. METHODS: A Markov decision analytical model was constructed to estimate the incremental costs, quality-adjusted life years (QALYs) and successfully treated patient (i.e. 50% or more seizure reduction) of the treatment strategies KD, VNS and CAU, from a health care perspective. The base case considered children with intractable epilepsy (i.e. two or more antiepileptic drugs had failed) aged between 1 and 18 years. Data were derived from literature and expert meetings. Deterministic and probabilistic sensitivity analyses were performed. RESULTS: Our results suggest that KD is more effective and less costly, and thus cost-effective compared with VNS, after 12 months. However, compared to CAU, neither KD nor VNS are cost-effective options, they are both more effective but also more expensive (euro346,899 and euro641,068 per QALY, respectively). At 5 years, VNS is cost-effective compared with KD and CAU (euro11,378 and euro68,489 per QALY, respectively) and has a 51% probability of being cost-effective at a ceiling ratio of euro80,000 per QALY. CONCLUSIONS: Our results suggest that on average the benefits of KD and VNS fail to outweigh the costs of the therapies. However, these treatment options should not be ignored in the treatment for intractable epilepsy in individual or specific groups of patients. There is a great need for high quality comparative studies with large patient samples which allow for subgroup analyses, long-term follow-up periods and outcome measures that measure effects beyond seizure frequency (e.g. quality of life). When this new evidence becomes available, reassessment of the cost-effectiveness of KD and VNS in children with intractable epilepsy should be carried out.

Epilepsy is a relatively common neurologic disorder in children that has important implications for development, parents, and society. Making the correct diagnosis starts with an accurate and complete history that consequently leads to a directed diagnostic workup. This article outlines a diagnostic and management approach to pediatric seizures and epilepsy syndromes. Making the correct diagnosis of epilepsy or nonepileptic imitators allows the practitioner to prescribe appropriate therapy. Initial management for typical epileptic syndromes and seizures and potential adverse effects are discussed. Alternative treatment options for pharmacologically resistant patients such as ketogenic diet, vagal nerve stimulation, and surgery are also discussed. While most children favorably respond to antiepileptic medications, early identification of medication failure is important to ensure optimal neurodevelopment.

Background This Scandinavian collaborative retrospective study of children treated with ketogenic diet (KD) highlights indications and effectiveness over two years follow-up. Methods Five centres specialised in KD collected data retrospectively on 315 patients started on KD from 1999 to 2009. Twenty-five patients who stopped the diet within four weeks because of compliance-problems and minor side-effects were excluded. Seizure-type(s), seizure-frequency, anti-epileptic drugs and other treatments, mental retardation, autism-spectrum disorder and motor-dysfunction were identified and treatment-response was evaluated. Results An intention-to-treat analysis was used. Responders (>50% seizure-frequency reduction) at 6, 12 and 24 months were 50%, 46% and 28% respectively, seizure-free were 16%, 13% and 10%. Still on the diet were 80%, 64% and 41% after 6, 12 and 24 months. No child had an increased seizure-frequency. The best seizure outcome was seen in the group with not-daily seizures at baseline ( n = 22), where 45%, 41% and 32% became seizure-free at 6, 12 and 24 months A significant improvement in seizure-frequency was seen in atonic seizures at three months and secondary generalised seizures at three and six months. Side-effects were noted in 29 subjects; most could be treated and only two stopped due to hyperlipidaemia and two due to kidney-stones. In 167 patients treated with potassium-citrate, one developed kidney-stones, compared with six of 123 without potassium-citrate treatment (relative risk = 8.1). Conclusions As the first study of implementing KD in children in the Scandinavian countries, our survey of 290 children showed that KD is effective and well tolerated, even in such severe patients with therapy-resistant epilepsy, more than daily seizures and intellectual disability in the majority of patients. Long-term efficacy of KD was comparable or even better than reported in newer AEDs. Addition of potassium citrate reduced risk of kidney-stones. Our data indicate that the response might be predicted by seizure-frequency before initiation of the diet but not by age, seizure-type or aetiology.

This 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).A 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.The 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%; p0.05). 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.Low-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.

We present a case series of three boys with childhood epilepsy with myoclonic–atonic seizures (EMAS) who achieved complete remission during childhood only to develop absence seizures during early adolescence. In all three cases, the recurrent seizures resolved again with antiseizure drugs, and two are currently medication-free for a second time., Highlights • Three boys with epilepsy and myoclonic–atonic seizures were successfully controlled with antiseizure drugs. • All three achieved controlled and were tapered off antiseizure drugs with relapse that achieved electro-clinical control. • Two of 3 boys successfully remain off antiseizure drugs for greater than 1 year following the second relapse.

ResumenEn la edad pediátrica las epilepsias son frecuentes, con manifestaciones clínicas diversas y edad-dependientes. El reconocimiento preciso de los diferentes síndromes y epilepsias es importante para determinar un tratamiento y pronóstico adecuados. Cerca de un 70% de las epilepsias en niños tiene una buena respuesta a tratamiento médico o remisión espontánea y buen pronóstico en el largo plazo; se agrupan aquí las formas idiopáticas ya sea generalizadas como focales. Un grupo menor, pero no menos importante, es refractario a fármacos antiepilépticos, destacando las Encefalopatías Epilépticas que implican un mal pronóstico neurológico y un grupo de pacientes refractarios que serán candidatos a otras alternativas de tratamiento, como cirugía de la Epilepsia. Se revisan los principales conceptos que subyacen a la actual clasificación de Síndromes Electroclínicos y Epilepsias de ILAE y las características de algunos síndromes electro-clínicos atendiendo a su frecuencia, relevancia de un diagnóstico preciso y aportes recientes de significaciónSummaryEpilepsy is a common condition in the pediatric age, with age-dependent and heterogeneous clinical manifestations. A successful approach to treatment and prognosis requires the precise recognition of the diverse syndromes and epilepsies. Near 70% of pediatric epilepsies, especially de idiopathic focal and generalized syndromes, are responsive to medical treatment or have spontaneous remission and a positive longterm prognosis. A smaller but not less important group is refractory to antiepileptic drugs and will need other treatment options such as epilepsy surgery. Here we discuss the main concepts underlying the ILAE Classification of Electroclinical syndromes and Epilepsies and the characteristics of some syndromes attending to its frequency, relevance of precise diagnosis and recent advances.

Summary This retrospective study evaluates the impact of postoperative antiepileptic drug (AED) withdrawal on psychomotor speed in seizure-free children, operated for medically refractory epilepsy. Post-surgical medication policy and neuropsychological assessments (performed shortly before and 6, 12 and 24 months after surgery), were evaluated in 57 children (32 female, median age at surgery 13 years). Patients were divided into a withdrawal ( n =29) and a no-withdrawal group ( n =28). Scores of four psychomotor tests performed at 12 and 24 months after surgery were compared with those of postoperative baseline measurements, performed 6 months after surgery. At 24 months, the withdrawal group had improved significantly more than the no-withdrawal group on three of four tests; reaction time to light ( p =0.031), reaction time to sound ( p =0.045) and tapping ( p =0.003). At 12 months, a non-significant tendency in the same direction was found for both reaction time tests. Drug withdrawal after surgery improves psychomotor speed and may unleash the potential for cognitive improvement.

The purpose of this investigation was to characterize the neuroanatomical correlates (cortical thickness) of variations in parent-reported Child Behavior Checklist (CBCL) scores. Ninety children with epilepsy (aged 8-18) underwent brain MRI, and their parents completed the CBCL. FreeSurfer-derived measures of cortical thickness were examined in relation to the CBCL broad and narrow band competence and behavioral problem scales, as well as the newer DSM-oriented scales. Parent reports of higher (better) social competence skills were associated with increased cortical thickness, especially in frontal regions. Parent reports of behavioral problems were associated with patterns of decreased cortical thickness that varied as a function of the specific behavioral issue under investigation. Congruence of patterns of cortical thinning between the DSM-oriented scales and conceptually related specific problem scales (e.g., ADHD Problems and Attention Problems) was generally weak. The parent-report version of the CBCL is associated with variations in cortical thickness among children with epilepsy. Anatomic abnormalities specific to selected competence and behavioral problem scales can be identified, with more reliable and robust patterns of thinning across scales assessing externalizing behaviors, with generally less prominent findings on scales assessing internalizing behaviors.

Epilepsy is an important cause of neurological disability in children. Nowadays, an increasing number of parents or caregivers use the Internet as a source of health information concerning symptoms, therapy, and prognosis of epilepsy occurring during childhood. Therefore, high-quality websites are necessary to satisfy this request. Using the DISCERN tool, we evaluated online information on childhood epilepsy provided by the first 50 links displayed on the Google search engine. The same links were evaluated by a team of pediatric neurologists (PNs) and by a lay subject (LS). The evaluation performed by the PNs found out that only 9.6% of the websites showed good reliability, that only 7.2% of the websites had a good quality of information on treatment choices, and that only 21.5% of the websites showed good overall quality of the content. With regard to the evaluation performed by the neutral subject, it was found that 21.4% of the websites showed good reliability, that 59.5% of the websites showed poor quality of information on treatment choices, and that only 2% of the websites showed good overall quality of the content. Our conclusion is that online information about childhood epilepsy still lacks reliability, accuracy, and relevance as well as fails to provide a thorough review of treatment choices.

Purpose: From the overprotection literature, the predictive and interactional (moderation) effects of controlling and indulgent parenting on restrictions in children with epilepsy were examined.Methods: Parents of 73 children with epilepsy completed questionnaires on parenting, restrictions, and functional status. Predictive and moderation effects were tested using multiple regression analysis. Moderation was tested with interactive computational methods.Results: Restrictions were significantly (R2 = .38, FΔ = 6.59***, p < .001) predicted from seizure frequency (β = .24*, p < .05), functional status (β = − .42***, p < .001), and interaction between controlling and indulgent parenting (β = .28**, p < .01). Moderation occurred predominantly for high values of control: controlling parents who were not indulgent imposed fewer restrictions. In contrast, controlling parents who were indulgent imposed more restrictions.Conclusion: Parents who were controlling and more indulgent imposed more restrictions. Clinicians should ask parents about parenting and restrictions. Future research should examine whether the current study's findings can be replicated.

Epilepsy syndromes are defined as distinctive disorders identifiable on the basis of a typical age of onset, specific EEG characteristics, seizure types, and other features. A wide range of epilepsy syndromes present throughout infancy, childhood and adolescence from benign self-limiting syndromes to severe epileptic encephalopathies. Accurate recognition and diagnosis of these syndromes is essential to ensure appropriate investigation and treatment. Recent revisions to the classification of childhood epilepsy and recent scientific, particularly genetic, discoveries have had an impact on our understanding of the childhood epilepsies. In this review we consider the role of syndromic diagnosis and describe the typical features and presentation of the most important epilepsy syndromes of childhood and adolescence.