Rolandic Epilepsy Research Articles

Complete cessation of epileptiform events caused by benign epilepsy with centrotemporal spikes (BECTS) in 9-year-old male

Event Abstract Back to Event Complete cessation of epileptiform events caused by benign epilepsy with centrotemporal spikes (BECTS) in 9-year-old male Shannon Leon1 and Jason Langhough1* 1 Dr. Shannon Leon Functional Neurology, United States Presentation Two prior EEG reports confirm presence of benign rolandic epilepsy of childhood with higher amplitude in bilateral temporal regions, right more than left, and with no activation of epileptiform activity induced by hyperventilation. Pertinent highlights of neurological exam were hypermetric saccades rightward, dyspraxic alternating hand movements on the right, impaired neural integrators resulting in decrease time constant of eccentric gaze rightward and up-and-rightward, thenar myoclonus in the left hand, decreased posture stability on foam platform with eyes closed in bilateral otolithic organs and bilateral posterior semicircular canals. Videonystagmography confirmed a decrease in amplitude of the right vestibulo-ocular reflex, and a right eye accommodation spasm. Musculoskeletal exam showed decrease rib expansion in the right side and overall poor posture. Intervention The nidus of treatment consisted of vestibular recalibration maneuvers to elicit, via canals and otoliths, the neural activation of putamen streams for inhibition of globus pallidus interna and thalamus, among other nuclei; sinusoidal micropursuits directed up-and-right to down-and-left performed in the patient’s field of views in lower left and in the upper right in order to elicit parietal pathways and thereby bypass, to degrees, deep central nuclei activation; and joint articulation of the right rib cage was restored to mobility to prevent hypoxic circumstance of nervous tissue. Patient scheduled for a frequency of one visit per week. Outcome After one visit, patient remarks on feeling “happier,” “calmer,” and “clear-minded.” Objective measurements saw improvement of balance on foam platform with eyes closed in all balance organ orientations to within normal limits, and vestibulo-ocular reflexes restored to accurate amplitude and velocity. Since initial treatment over one year ago, patient reports zero epileptic events, and patient’s parents observe noticeable increases in sports performances as well as an overall calmer demeanor. Perspective The wellspring of energy in childhood is meant for play and learning. When neuronal activation exceeds physiologic capacity and manifests epileptiform activity, even if benign, then rectifying therapy should be sought. BECTS is considered one of the most common childhood epileptic syndromes, and as such, it highlights the widespread significance of our outcome using a non-invasive, drug-free, phenomenological approach. Keywords: BECTS, benign epilepsy with centrotemporal spikes, Epilepsy, seizure, carrick eye exercises, Vestibulal Evoked Myogenic Potentials, Vestibular rehabilitation therapy Conference: International Symposium on Clinical Neuroscience, Orlando, United States, 24 May - 26 May, 2019. Presentation Type: Poster Presentation Topic: Clinical Neuroscience Citation: Leon S and Langhough J (2019). Complete cessation of epileptiform events caused by benign epilepsy with centrotemporal spikes (BECTS) in 9-year-old male. Front. Neurol. Conference Abstract: International Symposium on Clinical Neuroscience. doi: 10.3389/conf.fneur.2019.62.00026 Copyright: The abstracts in this collection have not been subject to any Frontiers peer review or checks, and are not endorsed by Frontiers. They are made available through the Frontiers publishing platform as a service to conference organizers and presenters. The copyright in the individual abstracts is owned by the author of each abstract or his/her employer unless otherwise stated. Each abstract, as well as the collection of abstracts, are published under a Creative Commons CC-BY 4.0 (attribution) licence (https://creativecommons.org/licenses/by/4.0/) and may thus be reproduced, translated, adapted and be the subject of derivative works provided the authors and Frontiers are attributed. For Frontiers’ terms and conditions please see https://www.frontiersin.org/legal/terms-and-conditions. Received: 30 Mar 2019; Published Online: 27 Sep 2019. * Correspondence: Dr. Jason Langhough, Dr. Shannon Leon Functional Neurology, Woodbury, Minnesota, 11797, United States, j.langhough@gmail.com Login Required This action requires you to be registered with Frontiers and logged in. To register or login click here. Abstract Info Abstract The Authors in Frontiers Shannon Leon Jason Langhough Google Shannon Leon Jason Langhough Google Scholar Shannon Leon Jason Langhough PubMed Shannon Leon Jason Langhough Related Article in Frontiers Google Scholar PubMed Abstract Close Back to top Javascript is disabled. Please enable Javascript in your browser settings in order to see all the content on this page.

Impact of Rolandic Epilepsy on Language, Cognitive, and Behavioral Functioning in Children: A Review

AbstractThe 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.

Antiepileptic Drug of Levetiracetam Decreases Centrotemporal Spike-Associated Activation in Rolandic Epilepsy.

The objective was to study the modulation effects of levetiracetam on the fMRI activation/deactivation patterns associated with centrotemporal spikes (CTS) in Rolandic epilepsy. Forty patients with Rolandic epilepsy, including levetiracetam-medicated patients (n = 20) and drug-naive patients (n = 20), were studied. Single and sequential hemodynamic response functions-based EEG-fMRI analysis was performed to detect dynamic activation/deactivation associated with CTS. Comparisons of spatiotemporal features of activation/deactivation were performed between the two groups. Both the groups (CTS were detected in 12 cases of levetiracetam-medicated group, and 11 cases of drug-naive group) showed CTS-associated activation in the Rolandic cortex, whereas activation strength, time-to-peak delay, and overall activation were diminished in the levetiracetam-medicated group. Moreover, the drug-naive group showed deactivation in the regions engaged in higher cognition networks compared with the levetiracetam-medicated group. Levetiracetam inhibits CTS-associated activation intensity and alters the temporal pattern of this activation in the epileptogenic regions, and it also affects the brain deactivation related to higher cognition networks. The findings sheds a light on the pharmocological mechanism of levetiracetam therapy on Rolandic epilepsy.

Benign rolandic epilepsy of childhood and central auditory processing disorder: A noncasual neurophysiological association

PurposeThe purpose of this study was to demonstrate the association between benign rolandic epilepsy of childhood (BREC) and central auditory processing disorders (CAPDs) and to test the hypothesis that an early onset of BREC could be associated with more cases of CAPD. MethodThis study has a retrospective cross-sectional design conducted from January 2006 to January 2016 including 93 patients with BREC and without intellectual disability, dyslexia, and attention-deficit hyperactivity disorders. All patients were evaluated for central auditory processing (CAP), and its presence or absence was compared with age of seizure onset. ResultsIn all patients, audiometric test results were normal, and in 43 cases (46.2%), CAPD was detected. There was no significant statistical difference in the mean age of seizure onset. ConclusionA significant proportion of children with BREC will have a diagnosis of CAPD at school age.

Algorithm for automatic EEG classification according to the epilepsy type: Benign focal childhood epilepsy and structural focal epilepsy

Abstract 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.

Efficacy of levetiracetam for reducing rolandic discharges in comparison with carbamazepine and valproate sodium in rolandic epilepsy

PurposeThe main purpose of this study was to compare the efficacy of levetiracetam (LEV) with the older antiepileptic drugs (AEDs) for preventing atypical evolution in children with Rolandic epilepsy (RE). Accordingly, the present study compared the efficacy of older AEDs (carbamazepine (CBZ) and valproate sodium (VPA)) with LEV in reducing rolandic discharges (RDs) on interictal electroencephalogram (EEG) in children with RE. MethodsPatients in this heterogenous study were subdivided into CBZ, VPA and LEV groups in accordance with the initial monotherapy. The CBZ and VPA groups were studied retrospectively, but the LEV group was studied prospectively. Appearances of discharges were counted and these rates were computed. In comparison with the baseline RD frequency, EEG response to AED treatment was classified such as complete disappearance and response (≥50% reduction in RD frequency). The time taken to attain complete disappearance or response in EEG responders was assessed for each AED treatment group. ResultsResponders comprised 10 (11.2%) of the 89 patients treated with CBZ, 41 (56.2%) of the 73 patients with VPA, and 25 (71.4%) of the 35 patients with LEV. Mean interval to achievement of EEG response in the CBZ, VPA, and LEV groups were 36.3, 23.1, and 14.7 months, respectively. EEG response was achieved significantly more rapidly with LEV than with CBZ (p < 0.001) or VPA (p < 0.005). Seizure control was not significantly different in all 3 investigated drugs. ConclusionsLEV seems to be superior to CBZ and VPA in its ability to suppress RDs in children with RE.

Sleep Related Epilepsy and Pharmacotherapy: An Insight.

In the last several decades, sleep-related epilepsy has drawn considerable attention among epileptologists and neuroscientists in the interest of new paradigms of the disease etiology, pathogenesis and management. Sleep-related epilepsy is nocturnal seizures that manifest solely during the sleep state. Sleep comprises two distinct stages i.e., non-rapid eye movement (NREM) and rapid eye movement (REM) that alternate every 90 min with NREM preceding REM. Current findings indicate that the sleep-related epilepsy manifests predominantly during the synchronized stages of sleep; NREM over REM stage. Sleep related hypermotor epilepsy (SHE), benign partial epilepsy with centrotemporal spikes or benign rolandic epilepsy (BECTS), and Panayiotopoulos Syndrome (PS) are three of the most frequently implicated epilepsies occurring during the sleep state. Although some familial types are described, others are seemingly sporadic occurrences. In the present review, we aim to discuss the predominance of sleep-related epilepsy during NREM, established familial links to the pathogenesis of SHE, BECTS and PS, and highlight the present available pharmacotherapy options.

Genetic association analysis with pedigrees: Direct inference using the composite likelihood ratio.

The likelihood function represents statistical evidence given data and a model. The evidential paradigm (EP), an alternative to Bayesian and Frequentist paradigms, provides considerable theory demonstrating evidence strength for different parameter values via the ratio of likelihoods at different parameter values; thus, enabling inference directly from the likelihood function. The likelihood function, however, can be difficult to compute; for example, in genetic association studies with a binary outcome in large pedigrees. Composite likelihood (CL) is an alternative when the real likelihood is intractable. We show CLs have the two large sample properties of the EP for reliable evidence interpretation: (1) CL supports the true value over a false value by an arbitrarily large factor; and (2) the probability of favouring a false value over the true value is small and bounded. Using simulation, and in a genetic association analysis of reading disability (RD) in large rolandic epilepsy pedigrees, we show that the CL approach yields valid statistical inference and identifies RD associated variants. When compared to analyses using generalized estimating equations, results show a similar prioritization of SNPs, although the CL approach provides additional complementary information, and more intuitive solutions to the multiple hypothesis testing problem.

Rare gene deletions in genetic generalized and Rolandic epilepsies.

Genetic Generalized Epilepsy (GGE) and benign epilepsy with centro-temporal spikes or Rolandic Epilepsy (RE) are common forms of genetic epilepsies. Rare copy number variants have been recognized as important risk factors in brain disorders. We performed a systematic survey of rare deletions affecting protein-coding genes derived from exome data of patients with common forms of genetic epilepsies. We analysed exomes from 390 European patients (196 GGE and 194 RE) and 572 population controls to identify low-frequency genic deletions. We found that 75 (32 GGE and 43 RE) patients out of 390, i.e. ~19%, carried rare genic deletions. In particular, large deletions (>400 kb) represent a higher burden in both GGE and RE syndromes as compared to controls. The detected low-frequency deletions (1) share genes with brain-expressed exons that are under negative selection, (2) overlap with known autism and epilepsy-associated candidate genes, (3) are enriched for CNV intolerant genes recorded by the Exome Aggregation Consortium (ExAC) and (4) coincide with likely disruptive de novo mutations from the NPdenovo database. Employing several knowledge databases, we discuss the most prominent epilepsy candidate genes and their protein-protein networks for GGE and RE.

Abnormal cortical activation during an auditory word comprehension task in benign childhood epilepsy with centrotemporal spikes: A magnetoencephalographic study

ObjectiveBenign childhood epilepsy with centrotemporal spikes (BECTS), also known as rolandic epilepsy, has recently been reported to be associated with variable degrees of cognitive dysfunction. Many studies reported poor language ability in children with BECTS compared with healthy control children. To elucidate the harmful effects of BECTS on language cognition, we studied the magnetoencephalographic activity elicited by an auditory language comprehension task. MethodsThe participants (N = 20) included 10 children diagnosed with BECTS (aged 10.8 ± 2.8 years) and 10 age-matched healthy children (control) (aged 10.6 ± 1.6 years). Cognitive function was assessed using general intellectual function and language ability. In patients with BECTS, we reviewed the clinical course and electroencephalogram (EEG) findings. We recorded the cortical responses elicited by an auditory language comprehension task using magnetoencephalography (MEG). We compared those results between groups and analyzed the correlation with cognitive scores and frequency of spikes. ResultsThe full-scale intelligence quotient (FSIQ) by the Wechsler Intelligence Scale for Children—4th edition was significantly reduced in the group with BECTS (96.4 ± 12.3) compared with the control group (110.0 ± 7.4). In half of the group with BECTS, the auditory comprehension score fell below the age-standard level. In the group with BECTS, the cortical activation during the task showed reduced intensity in language-associated areas such as the bilateral primary auditory cortex, left superior and mid-temporal areas, and inferior frontal area compared with those in the control group. In addition, the cortical activation in the left superior temporal area was negatively correlated with spike frequency and positively correlated with FSIQ in the group with BECTS. Conversely, the right inferior frontal and mid-temporal areas had increased the activations in the group with BECTS. From the time frequency analysis, low gamma band event-related desynchronization was reduced in the group with BECTS. ConclusionEpileptic spikes negatively influenced responsiveness to the auditory language comprehension task in the language-associated cortices. These findings suggest that epileptic spikes could have a negative impact on the functional activity in rolandic areas and become a reason to change the functional development of the language network.

Could Rolandic spikes be a prognostic factor of the neurocognitive outcome of children with BECTS?

IntroductionRolandic epilepsy, also known as benign childhood epilepsy with centrotemporal spikes (BECTS), is one of the most common epileptic syndromes in previously healthy children. Despite what was known about the benignity of this syndrome, there is always more evidence about the involvement of the cognitive functions with different deficits in several domains to be investigated. Aim of the studyThe aim of our study was to describe prognostic electroencephalogram (EEG) pattern of an adverse cognitive development to recognize patients at higher risk of lasting cognitive deficits that could need antiepileptic drugs (AEDs) or an improved neurocognitive therapy. In addition, we wanted to investigate the existence of a possible linkage between the number of interictal epileptiform discharges (IEDs) in the EEG and the more pronounced cognitive deficits. Material and methodsWe performed a case–control study on a cohort of 16 patients (10 male and 6 female) aged 4–14, diagnosed with BECTS who underwent EEG, magnetic resonance imaging (MRI), and neurocognitive assessment at the Pediatric Neurology Unit at the Umberto I Hospital, Sapienza University of Rome. Patients were divided into two groups according to the percentage of IEDs evaluated based on their sleep EEG: group A with less than 50% of the entire EEG invaded by discharges in more than 70% of the total number of EEG performed, so-called with low or intermediate activation. On the contrary, group B had a high activation, with more than 50% of the entire EEG invaded by discharges in the same percentage of the EEG performed. All children were assessed based on a protocol designed to study neuropsychological functions with specific tests chosen depending on age (Wechsler Intelligence Scale for Children IV: WISC IV; Wechsler Preschool and Primary Scale of Intelligence III: WPPSI III). Groups were compared for cognitive outcomes achieved by each patient through Student's t-test with a significance level of p<0.05 (two-tailed). ResultsThere is no statistically significant difference in the cognitive outcomes of these patients: Student's t-test showed a statistical significance (p) for each cognitive index always higher than 0.05, demonstrating that the intellectual quotient (IQ) and all other indexes analyzed (verbal comprehension index (VCI), perceptual reasoning index (PRI), working memory index (WMI), and processing speed index (PSI)) are not affected by the difference in EEG anomalies presented by our patients. Interestingly, all patients had an IQ equal to or greater than the Italian average (12 out of 16 patients showed an IQ>100), with selective drops, particularly significant in the WMI and also in the PSI. ConclusionsOur results clearly demonstrate the importance of a proper evaluation of patients with this kind of epilepsy, without paying attention only to those with the greatest number of IEDs or seizures because all of them had a neurocognitive impairment, especially in memory. These data may be reinforced by a larger sample for an even more significant statistical value. These results also highlight the importance of a neurocognitive therapy for these children to treat for their specific needs.

Frequency-specific alterations in the amplitude and synchronization of resting-state spontaneous low-frequency oscillations in benign childhood epilepsy with centrotemporal spikes

ObjectivesSpontaneous low-frequency oscillations in different frequency bands have diverse physiological meanings. The amplitude of low-frequency fluctuation (ALFF) and functional connectivity (FC) in different frequency bands in Benign Childhood Epilepsy with Centrotemporal Spikes (BECTS) are unknown and worth exploring. MethodResting-state functional magnetic resonance imaging data were collected in 51 drug-naïve BECTS patients and 76 healthy controls. The ALFF was calculated for the typical (0.01 – 0.08 Hz), slow-5 (0.01–0.027 Hz), slow-4 (0.027–0.073 Hz), and slow-3 (0.073–0.198 Hz) frequency bands. The bilateral precuneus/posterior cingulate cortex (PCU/PCC) showed a common alteration of ALFF in different frequency bands, and was selected as the seed for calculating FC per voxel. ResultsIn the typical band, BECTS patients showed increased ALFF in the left rolandic operculum and the right pre/postcentral gyrus, and decreased ALFF in the bilateral PCU/PCC, some of which were shared by the slow-5, slow-4, and slow-3 bands. Decreased ALFF in the left angular gyrus was also found in the slow-3 band. Only the bilateral PCU/PCC showed a frequency-dependent correlation with the total seizure frequency and full-scale intelligence quotient. Regions having degenerated FC with the bilateral PCU/PCC in BECTS patients were mainly in the left prefrontal cortex and bilateral anterior cingulate cortex for the typical and slow-5 bands, and in the bilateral temporal limbic system and striatum for the slow-4 and slow-3 bands. ConclusionAlteration of the ALFF and FC differed with distinct frequency bands. Therefore, employing different frequency bands would provide more meaningful findings for BECTS patients.

BRAIN BIOELECTRIC ACTIVITY IN CHILDREN WITH IDIOPATHIC EPILEPSY

The aim was to study the bioelectrical activity of the brain in children with idiopathic epilepsy. Using clinical, neurological and neurophysiological methods, we examined 60 children (32 boys, 53% and 28 girls, 47%) aged 5 to 10 years old. Results . In group 1, 14 children (24%) were found to suffer from absence epilepsy, while the remaining 26 patients (43%) had tonic-clonic seizures. In group 2, 20 cases (33%) of rolandic epilepsy were diagnosed. The results demonstrate that having even few clinical data, it is possible to discern between different types of seizures in children with idiopathic epilepsy by using a routine EEG analysis.

Identification of new risk factors for rolandic epilepsy: CNV at Xp22.31 and alterations at cholinergic synapses

BackgroundRolandic epilepsy (RE) is the most common genetic childhood epilepsy, consisting of focal, nocturnal seizures and frequent neurodevelopmental impairments in speech, language, literacy and attention. A complex genetic aetiology is...

F192. Role of multimodal extra and intraoperative Stereoelectroencephalography (sEEG) monitoring in tailoring staged surgical resection in patients with eloquent cortex (Rolandic) pharmacoresistant epilepsy

Introduction To describe the feasibility, safety and seizure outcome of patients with medically intractable rolandic epilepsy(RE) who underwent staged resections guided by multimodal extra and intraoperative SEEG recordings. Methods We retrospectively studied and analyzed patients, who underwent extra and intraoperative intracranial invasive stereoEEG monitoring, data related to semiology, non-invasive electrophysiology, imaging, type of resection, complications, surgical pathology results and seizure outcome from January 2015 through September 2017, under Cleveland Clinic IRB. Results Five patients were identified as having intractable RE based on analysis of pre-surgical evaluation using 3T magnetic resonance imaging (MRI), magnetoencephalography(MEG) and positron emission tomography (PET). Preoperative magnetic resonance imaging were considered nonlesional in all five patients but after the pre-surgical evaluation, there were abnormal sulcation pattern or increased signal in white matter were noticed which are of unknown significance. All five patients were underwent sEEG implantation with hypothesis of peri-rolandic or paracentral lobule regions being epileptogenic. Extra-operative sEEG data in all patients showed ictal onset zone involving rolandic cortex (hand knob and paracentral lobule), premotor and/or supplementary motor area (SMA). In two patients, hand knob rolandic cortex was the region of interest The other three being paracentral lobule (leg motor). Given all the data (semiology, sEEG data and pre-sugical evaluation), the conclusion of epileptogenic zone (EZ) being close proximity to eloquent cortex. Given the semiology, staged resection approach with continuous sEEG recording was performed in the operating room. Resection of interictally active non-eloquent cortex electrodes was performed first guided by real-time intraoperative sEEG (electrophysiology) data. The resection was also guided by the sEEG electrode in three dimensions. Resolution of rolandic hand motor spike activities was documented after resection of SMA in three patients, cingulate and pre-motor areas in one patient. In the last patient, hand sensory area was resected sparing motor cortex. Three out of five patients did not have any deficit but two had mild foot drop. Pathology showed focal cortical dysplasia type (IB, IIA, and IIB) and a possible vascular malformation in another. All patients were reported to be seizure free at follow up period of 6–28 months. Conclusion Taken into account of hyper-synchronous connectivity in frontal and parietal rolandic regions, we present a case series of patients who underwent staged resections guided by a novel multimodal method of invasive monitoring. The results of this highly selected group of patients testify for the feasibility and safety of a novel multimodal method of invasive monitoring, combining the benefits of both intra and extra-operative SEEG recording, especially operating around the central lobule near hand knob regions.

F18. EEG and neuroimaging findings in pediatric patients with down syndrome and epilepsy: A single center experience

Down syndrome occurs in 1 in 650–1000 births. Epilepsy has been reported in 1–13% of patients with Down syndrome (Stafstrom et al., 1991) compared to 1.5–5 % in general population (Forsgren et al., 2005). This was a single center retrospective medical record review of patients of less than 18 years of age with Down syndrome and epilepsy from January 2005 to December 2015. Sixteen patients were identified. Patients were grouped by seizure types and data was collected on EEG, MRI and outcome at last follow up. Children with Down syndrome and epilepsy can be grouped in four categories of seizures. Group 1: Infantile spasms – Seven out of sixteen patients in our cohort had infantile spasms with findings of hypsarrhythmia on the EEG. Five patients had MRI’s available for review and were normal. Five patients (70%) were seizure free, three of whom had a normal EEG and were off antiepileptic medications at their last follow up. The two remaining patients had refractory seizures and abnormal EEG findings which included regional sharp waves and hypsarrhythmia with multiregional sharp waves. Group 2: Generalized seizures – Six patients had generalized seizures (GTC, myoclonic, atonic) and regional/multiregional sharp waves on the EEG. Five off these patients had abnormal MRI findings: Two with MCA distribution infarcts and three with more diffuse abnormalities secondary to periventricular leukomalacia, traumatic brain injury and diffuse volume loss. Fifty percent of the patients were seizure free including one following a functional hemispherectomy. All patients continued to have abnormal EEGs at their last follow up. Group 3: Partial seizures – Two patients in our cohort had partial seizures (focal neonatal seizures and benign rolandic epilepsy with centrotemporal spikes) and both were seizure free at their last follow up. Group 4: Partial and Generalized seizures – One patient had partial and generalized seizures with multiregional sharp waves on EEG at diagnosis. This patient continues to have refractory seizures on medications and hypsarrhythmia on the last follow up EEG. Neuroimaging: Thirteen MRI’s were available for review, of which seven were abnormal. 50–55% of patients with normal or abnormal MRI’s became seizure free. All patients except for one (background slowing only) had abnormal EEG’s with epileptiform discharges at diagnosis. Infantile spasms was the most common seizure type in our patient cohort with electro-clinical features resembling those of idiopathic West syndrome. Classic hypsarrhythmia was present in all patients at diagnosis. Patients with infantile spasms had a higher percentage of becoming seizure free with normalization of EEG on long term follow up compared to patients with generalized or partial seizures. Abnormal MRI does not appear to be helpful for predicting seizure outcome.

The relevance of attention deficit hyperactivity disorder in self-limited childhood epilepsy with centrotemporal spikes

In this study, we aimed to evaluate the attentional and executive functions in patients with benign childhood epilepsy with centrotemporal spikes (BCECTS) with and without attention-deficit hyperactivity disorder (ADHD) compared with controls and compared with patients with ADHD without epilepsy. We evaluated 12 patients with BCECTS and ADHD (66.7% boys; mean age of 9.67years); 11 children with non-ADHD BCECTS (63.6% boys; mean age of 11.91years); 20 healthy children (75% boys; mean age of 10.15years); and 20 subjects with ADHD without epilepsy (60% boys; mean age of 10.9years). We used a comprehensive battery of neuropsychological tests to evaluate attentional and executive functions in their broad domains. Patients with BCECTS and ADHD had worse performance in Conners' Continuous Performance Test II (reaction time standard error [p=0.008], variability [p=0.033], perseverations [p=0.044] and in reaction time interstimuli interval [p=0.016]). Patients with ADHD showed worse performance in Trail Making Test B errors [p=0.012]. In conclusion, patients with BCECTS and ADHD had worse executive and attentional performance compared with controls than non-ADHD patients with BCECTS. Regardless of the presence of epilepsy, ADHD also negatively impacted executive and attentional functions but in different executive subdomains compared with patients with epilepsy.

The focal alteration and causal connectivity in children with new-onset benign epilepsy with centrotemporal spikes

The aim of the current study was to find the epileptic focus and examine its causal relationship to other brain regions in children with new-onset benign childhood epilepsy with centrotemporal spikes (BECTS). Resting-state functional magnetic resonance imaging (fMRI) was performed in 66 children with BECTS and 37 matched control children. We compared the amplitude of low frequency fluctuation (ALFF) signals between the two groups to find the potential epileptogenic zone (EZ), then used Granger causality analysis (GCA) to explore the causal effects of EZ on the whole brain. Children with BECTS had significantly increased ALFF in the right Broca’s area, and decreased ALFF in bilateral fusiform gyrus. The patients also showed increased driving effect from the EZ in Broca’s area to the right prefrontal lobe, and decreased effects to the frontal lobe and posterior parts of the language network. The causal effect on left Wernicke’s area negatively correlated with verbal IQ (VIQ) score. Our research on new-onset BECTS patients illustrates a possible compensatory mechanism in the language network at early stages of BECTS, and the negative correlation of GCA and VIQ suggest the disturbance of epileptiform activity on language. These findings shed light on the mechanisms of and language dysfunction in BECTS.

Serial changes in the paroxysmal discharges in rolandic epilepsy may predict seizure recurrence: A retrospective 3-year follow-up study

ObjectiveThe aim of this study was to assess the electrographic criteria related to seizure recurrence and determine age-related seizure recurrence in children with rolandic epilepsy under long-term follow-up. MethodsWe retrospectively analyzed the data belonging to 109 patients with rolandic epilepsy with sufficient information regarding disease course and follow-up duration longer than 3years. Patients were divided into two categories: Group A (n: 75), comprised of “patients having fewer than four seizures”, and Group B (n: 34), the “recurrence group comprised of patients having more than four seizures in the first three months”. The number of spikes per minute during both wakefulness and sleep, the localization of spikes other than centrotemporal region, and the duration of spike–wave activity were evaluated longitudinally, with repeated electroencephalogram (EEG) recordings every 6months. ResultsThe appearance of rolandic spikes in awake EEGs tended to be more prevalent in Group B than Group A. In Group B, spike rates significantly increased in the 12 and 18months after onset whereas spike rates increased significantly only 6months after onset in Group A. Seizure recurrence is mostly seen at 6–8years, and improvement becomes evident by age 12. The mean number of paroxysmal rolandic discharges during sleep was significantly higher in the younger age groups (3–5, 6–8), and the mean number of spikes per minute significantly decreased at ages 9–11 and over 12. ConclusionOur study demonstrates that extended periods of high frequency of paroxysmal discharges, initial frontal EEG focus, and persistence of awake interictal abnormalities are highly effective in predicting seizure recurrence in patients with rolandic epilepsy (RE).

Benign rolandic epilepsy and generalized paroxysms: A study of 13 patients

PurposeTo present a retrospective study of 13 children with benign epilepsy with centrotemporal spikes (BECTS), also known as benign rolandic epilepsy (BRE), associated with generalized spikes and waves as the only EEG manifestation at onset. MethodCharts of children with typical clinical criteria of BRE electroclinically followed-up between February 2000 and February 2015 were reviewed. ResultsAmong 309 patients who met the electroclinical criteria of BRE, we identified 13 children who presented with the typical clinical manifestations but who, on the EEG, only had generalized paroxysms at onset that continued along the course of the syndrome. Generalized spike-and-wave discharges were observed in all patients when awake and during sleep (100%). During the evolution no particular electroclinical pattern was observed. The patients responded well to antiepileptic drugs, such as valproic acid and levetiracetam. Outcome was good in all patients. ConclusionsWe found evidence that patients with BRE may have generalized EEG discharges at onset as the sole manifestation lasting throughout the course of the syndrome. In some, focal paroxysms developed later. The course was benign. In our group of patients, clinical features and evolution were similar to those of typical cases of BRE. Response to valproic acid and levetiracetam was found to be particularly good.