Rolandic Epilepsy Research Articles

Faecal Incontinence: An Uncommon Manifestation of Panayiotopoulos Syndrome

Panayiotopoulos Syndrome (PS) is a benign, common, age-dependent syndrome, with susceptibility to idiopathic vegetative seizures and autonomic status epilepticus. It is formally recognized by the International League against Epilepsy (ILAE). It is one of the three focal epileptic child benign syndromes next to rolandic epilepsy and idiopathic occipital epilepsy in children. It is characterized by its relative mildness and confusing manifestations. It is frequently misdiagnosed as non-epileptic disorders.

TMT-Based Proteomic Analysis of Plasma from Children with Rolandic Epilepsy.

Rolandic epilepsy is one of the most common epileptic syndromes in childhood. We used TMT-based proteomics and bioinformatics analysis to identify the differentially expressed proteins in plasma of children with Rolandic epilepsy. Our aim was to provide a molecular basis for exploring possible mechanisms underlying the pathogenesis of epilepsy. Subjects were divided into two groups (five in each): patients with Rolandic epilepsy as cases and patients with migraine as controls. Total proteins were extracted and quantitatively labeled with TMT, then analyzed using liquid chromatography mass spectrometry. Bioinformatics analysis was used to identify the hub genes. A total of 752 proteins were identified, of which 670 contained quantitative proteins. 217 differentially expressed proteins were identified, 46 of which were only upregulated in more than two groups and 111 of which were only downregulated in more than two groups. Bioinformatics analysis revealed top 10 hub genes in the up- and downregulated groups, respectively. Our study demonstrates that some differentially expressed proteins are associated with epilepsy. Activation of acute-phase or innate immune response and complement and fibrinogen systems and repression of glycolysis, lipoprotein metabolism, and antioxidant activity may play a role in the development of epilepsy.

The relationship between epilepsy and cognitive function in benign childhood epilepsy with centrotemporal spikes

IntroductionThis study was aimed to explore the relationship between neural network changes in newly diagnosed children with Benign Childhood Epilepsy with Centrotemporal Spikes (BECTS) and cognitive impairment.MethodsChildren's cognition was evaluated using the Wechsler Intelligence Scale for Children‐Fourth Edition (WISC‐IV). Magnetoencephalographic (MEG) data of 18 healthy children and 22 BECTS patients were recorded in order to construct a functional connectivity (FC) network, which was quantified by graph theory (GT).ResultsThe mean age of the control group was 7.94 ± 1.89 years, and the mean age of BECTS patients was 8.14 ± 1.73 years. Our results show that the WISC‐IV index scores in the BECTS group were significantly lower than those in the control group. Besides, the FC network pattern of BECTS patients changed significantly in the 12–30, 30–80, and 250–500 Hz frequency band. The local functional connections between posterior cingulate cortex (PCC) and frontal lobe varied significantly in 12–30, 80–250, and 250–500 Hz. Our GT analysis shows that the connection strength of BECTS patients increases significantly in the 12–30 Hz frequency band, the path length decreases significantly in the 12–30 Hz and 30–80 Hz frequency bands, with the clustering coefficient decreasing significantly in the 12–30 Hz, 30–80 Hz, and 250–500 Hz frequency bands. Correlation analysis showed that the full‐scale IQ (FSIQ) was positively correlated with the 12–30 Hz clustering coefficient, verbal comprehension index (VCI) was positively correlated with the 250–500 Hz clustering coefficient, perceptual reasoning index (PRI) was positively correlated with the 12–30 Hz clustering coefficient, and perceptual reasoning index (PSI) was negatively correlated with the 12–30 Hz path length.ConclusionThere is a trend of cognitive impairment in patients with early BECTS. This trend of cognitive impairment in early BECTS children may be related to the changes in the FC network pattern.

Surface-based morphometry study of the brain in benign childhood epilepsy with centrotemporal spikes

BackgroundThe study aimed to explore cortical morphology in benign childhood epilepsy with centrotemporal spikes (BECTS) and the relationship between cortical characteristics and age of onset and intelligence quotient (IQ).MethodsCortical morphometry with surface-based morphometry (SBM) was used to compare changes in cortical thickness, gyrification, sulcal depth, and fractal dimension of the cerebral cortex between 25 BECTS patients and 20 healthy controls (HCs) with two-sample t-tests [P<0.05, family-wise error (FWE) corrected]. Relationships between abnormal cortical morphological changes and age of onset and IQ, which included verbal intelligence quotient (VIQ), performance intelligence quotient (PIQ), and full-scale intelligence quotient (FIQ) were investigated with Spearman correlation analysis (P<0.05, uncorrected).ResultsThe BECTS patients showed extensive cortical thinning predominantly in bilateral frontal, temporal regions, and limbic system. Cortical gyrification increased in the left hemisphere and partial right hemisphere, and the decreased cortical gyrification was only in the left hemisphere. The increased sulcal depth was the left fusiform gyrus. There are no statistically significant differences in the fractal dimension. Correlation analysis revealed the negative correlation between age of onset and cortical thickness in the right precentral gyrus. It also revealed the negative correlation between the age of onset and cortical gyrification in the left inferior parietal gyrus. Also, there was negative correlation between VIQ and cortical gyrification in the left supramarginal gyrus of BECTS patients.ConclusionsThis study reveals aberrant cortical thickness, cortical gyrification, and sulcal depth of BECTS in areas related to cognitive functions including language, attention and memory, and the correlation between some brain regions and VIQ and age of onset, providing a potential marker of early neurodevelopmental disturbance and cognitive dysfunction in BECTS.

Altered Static and Dynamic Spontaneous Neural Activity in Drug-Naïve and Drug-Receiving Benign Childhood Epilepsy With Centrotemporal Spikes.

The present study aims to investigate intrinsic abnormalities of brain and the effect of antiepileptic treatment on brain activity in Benign childhood epilepsy with centrotemporal spikes (BECTS). Twenty-six drug-naïve patients (DNP) and 22 drug-receiving patients (DRP) with BECTS were collected in this study. Static amplitude of low frequency fluctuation (sALFF) and dynamic ALFF (dALFF) were applied to resting-state fMRI data. Functional connectivity (FC) analysis was further performed for affected regions identified by static and dynamic analysis. One-way analysis of variance and post hoc statistical analyses were performed for between-group differences. Abnormal sALFF and dALFF values were correlated with clinical features of patients. Compared with healthy controls (HC), DNP group demonstrated alterations of sALFF and/or dALFF in medial prefrontal cortex (MPFC), supplementary motor areas (SMA), cerebellum, hippocampus, pallidum and cingulate cortex, in which the values were close to normal in DRP. Notably, sALFF and dALFF showed specific sensitivity in detecting abnormalities in basal ganglia and cerebellum. Additionally, DRP showed additional changes in precuneus, inferior temporal gyrus, superior frontal gyrus and occipital visual cortex. Compared with HC, the DNP showed increased FC in default network and motion-related networks, and the DRP showed decreased FC in default network. The MPFC, hippocampus, SMA, basal ganglia and cerebellum are indicated to be intrinsically affected regions and effective therapeutic targets. And the FC profiles of default and motion-related networks might be potential core indicators for clinical treatment. This study revealed potential neuromodulatory targets and helped understand pathomechanism of BECTS. Static and dynamic analyses should be combined to investigate neuropsychiatric disorders.

A-149 Boy with Rolandic Epilepsy Benefited from Visuospatial Training

Abstract Objective Children with epilepsy can have delay in the development of neurocognitive abilities. It is important to receive the evidence for efficiency of different treatments that are aimed to help children with epilepsy. The goal of this study was to assess the impact of 16 weeks of visuospatial training on the visuospatial abilities in 7 years of age child with Rolandic epilepsy. Methods The child is 7 year old boy with Rolandic epilepsy. The neuropsychological assessment of child has revealed the deficit in visuospatial abilities. Child participated in 16 weeks of visuospatial training. A total of 42 therapy sessions lasting 50 minutes were performed. This training trains the child to do different visuospatial exercises both on motor and cognitive level. This training is built on the conceptual framework derived from the work of Luria’s theory of restoration of neurocognitive functions (Luria, 1963, 1974). Results Neuropsychological assessment of child after the intervention period has revealed apparent progress in performance of 3 subtests from Luria’s neuropsychological assessment battery for children which designed to asses visuospatial functions (Head subtest, mental rotation, design copying). Conclusion According to result of this case report it can be assumed that visuospatial training can be used as a prospective approach for development of visuospatial functions in children with Rolandic epilepsy. However, we need to prove this result using visuospatial training for children with Rolandic epilepsy who suffer from deficit in visuospatial abilities.

A-150 Motor Sequencing Training Improved Executive Abilities in a Boy with Rolandic Epilepsy

Abstract Objective It is known that children with epilepsy can have deficit in neurocognitive abilities. It is of great significance to receive the evidence for efficiency of different treatments that are aimed to help children with epilepsy. The goal of this study was to reveal the impact of 12 weeks of motor sequencing training on the executive abilities in a child with Rolandic epilepsy. Methods The child is 7 year old boy. The neuropsychological assessment of child has revealed the mild deficit in executive abilities. Child participated in 12 weeks of motor sequencing training. A total of 36 therapy sessions lasting 50 minutes were performed.This therapy trains the child to plan, sequence and process information more effectively through repetition of goal-directed movements. This programme is built on the conceptual framework derived from the work of Luria’s theory of restoration of neurocognitive functions (Luria, 1963, 1974). Results Neuropsychological assessment (NEPSY) of child after the intervention period has revealed apparent progress in performance of 4 subtests which are designed to assess executive abilities and attention (Tower, Auditory Attention and Response Set, Visual Attention, Statue). Conclusion According to result of this case report it can be assumed that motor sequencing training can be used as a prospective treatment approach for development of executive abilities in children with Rolandic epilepsy.

The relationship between neuromagnetic activity and cognitive function in benign childhood epilepsy with centrotemporal spikes

PurposeOur aim was to explore the pathophysiological mechanism of cognitive function changes in early untreated children with benign childhood epilepsy with centrotemporal spikes (BECTS). MethodsMagnetoencephalography (MEG) was performed in 33 children with BECTS and 18 healthy children. Wechsler Intelligence Scale for Children, fourth edition (WISC-IV) was used to divide children with BECTS into two groups. Normal cognitive function was defined as a full-scale intelligence quotient (FSIQ) of >80, while decreased cognitive function was defined as a FSIQ of <80. Accumulated source imaging was used to evaluate the neuromagnetic source activity in multifrequency bands. ResultsOf the 33 patients with early untreated BECTS, a total of 17 had a FSIQ of <80 and 16 had FSIQ of >80. The course of epilepsy and number of seizures in the FSIQ <80 group were higher than that in the FSIQ >80 group. Our MEG results showed that in the 4–8 Hz frequency band, both patient groups had inactivation of the posterior cingulate cortex (PCC) region compared with the healthy control group. In the 30–80 Hz frequency band, the FSIQ <80 group showed inactivation of the PCC region compared with both the healthy control group and the FSIQ >80 group. In the 80–250 Hz frequency band, the FSIQ <80 group had inactivated of the medial frontal cortex (MFC) region compared with the healthy control group. In the 30–80 Hz frequency band, the strength of neuromagnetic source in patients with BECTS with FSIQ <80 was higher than that in the FSIQ >80 group and the healthy control group. ConclusionsThe magnetic source inactivation of the MFC and PCC regions during the interictal time may be the reason for cognitive decline in early untreated children with BECTS. Children with BECTS with cognitive decline had a longer course of epilepsy and more seizures. The magnetic source localization in the 4–8 Hz frequency band may be a new imaging marker for the diagnosis of new BECTS.

The impact of low-risk genetic variants in self-limited epilepsy with centrotemporal spikes aka Rolandic epilepsy.

Epilepsy is a common neurological disorder with a lifetime prevalence of 3%–4%. The disorder is defined by recurrent, unprovoked seizures resulting from abnormal, synchronized neuronal firing in the brain and encompasses a heterogeneous group of disease entities with diverse etiologies and outcomes. Genetic factors are known to play a significant role in epilepsy, either as monogenic causes in rare Mendelian epilepsies, or as multiple genetic risk factors in common epilepsies such as genetic generalized and focal epilepsies.

Seizures in self-limited epilepsy with centrotemporal spikes: video-EEG documentation

Self-limited epilepsy with centrotemporal spikes, formerly called benign epilepsy with centrotemporal spikes, or rolandic epilepsy, is an age-related and well-defined epileptic syndrome. Since seizures associated with rolandic spikes are infrequent and usually occur during sleep, and repetitive or prolonged EEG recording for diagnostic purposes is not necessary for diagnosis, reports of ictal video-electroencephalographic seizures in this syndrome are rare. We aimed to show ictal video-EEG of typical rolandic seizures. We report the ictal video-EEG recordings of two children with rolandic epilepsy who presented typical rolandic seizures during routine recording. Case 1: A 9-year-old boy, with normal development, had his first seizure at 8years old, characterized by paresthesia in his left face, blocking of speech, and drooling. Carbamazepine was started with seizure control. Case 2: A 10-year-old boy, with normal development, started with focal seizures during sleep, characterized by eye and perioral deviation, and speech arrest at age of 7. He started using oxcarbazepine. Both patients underwent routine electroencephalography for electroclinical diagnosis and presented a seizure. Although self-limited epilepsy with centrotemporal spikes is a very common epileptic syndrome, seizure visualization is very difficult, and these videos may bring didactical information for recognition of this usual presentation of benign childhood focal epilepsy.

Electroencephalogram spike detection and classification by diagnosis with convolutional neural network

This work presents convolutional neural network (CNN) based methodology for electroencephalogram (EEG) classification by diagnosis: benign childhood epilepsy with centrotemporal spikes (rolandic epilepsy) (Group I) and structural focal epilepsy (Group II). Manual classification of these groups is sometimes difficult, especially, when no clinical record is available, thus presenting a need for an algorithm for automatic classification. The presented algorithm has the following steps: (i) EEG spike detection by morphological filter based algorithm; (ii) classification of EEG spikes using preprocessed EEG signal data from all channels in the vicinity of the spike detected; (iii) majority rule classifier application to all EEG spikes from a single patient. Classification based on majority rule allows us to achieve 80% average accuracy (despite the fact that from a single spike one would obtain only 58% accuracy).

Identification of susceptibility variants to benign childhood epilepsy with centro-temporal spikes (BECTS) in Chinese Han population.

BackgroundBenign Childhood Epilepsy with Centro-temporal Spikes (BECTS) is the most common form of idiopathic epilepsy in children, accounting for up to 23% of pediatric epilepsy. The pathogenesis of BECTS is unknown, but it is thought that genetic factors play a role in susceptibility to the disease.MethodsTo investigate the role of common genetic variants in BECTS pathogenesis, a 2-stage genome-wide association study (GWAS) was performed in 1,800 Chinese Han BECTS patients, and 7,090 healthy controls. Genetic findings were used in a Mendelian Randomization study in the UK Biobank dataset to investigate the potential role of smoking in BECTS.FindingsDefinitive evidence of a role for common-variant heritability was demonstrated, with heritability of BECTS of >10% observed even with conservative disease prevalence assumptions. Although no individual locus achieved genome-wide significance, twelve loci achieved suggestive evidence of association (5 × 10-8<P<10−5). Using combined genetic and brain tissue gene expression data analyzed by Summary-data-based Mendelian Randomization (SMR), causative association of BECTS was demonstrated with SNP rs1948 and the CHRNA5 t3603436 transcript (Peqtl = 2·10 × 10−12, Psmr = 7·9 × 10−5). This finding indicates rs1948 is significantly associated with BECTS through effects on expression of CHRNA5 in brain tissue. The identification of novel loci suggests involvements of KALRN and the CHRNA5-A3-B4 cluster in BECTS. Using a generalized SMR approach we demonstrate that maternal smoking around birth is significantly associated with increased risk of BECTS (odds ratio = 3·90, P = 0·0099).InterpretationThis study shows that BECTS risk is at least partially heritable and due to common genetic variants. Additionally, we demonstrate that BECTS risk is substantially increased by maternal smoking around birth.

Resting state signal latency assesses the propagation of intrinsic activations and estimates anti-epileptic effect of levetiracetam in Rolandic epilepsy

PurposeWe assessed the potential of resting-state fMRI lag analysis (RSLA) in detecting epileptic activation and in estimating anti-epileptic effects of Levetiracetam (LEV) in Rolandic epilepsy. MethodsForty-three children with Rolandic epilepsy underwent simultaneous EEG-fMRI. They were grouped into LEV vs drug-naïve groups according to their medication, and into patients who showed or did not show central-temporal spike (CTS) discharges during scans. We calculated the lag structure of rs-fMRI for all patients and assessed interactions with drug (LEV vs. drug-naïve) and CTS status (CTS vs. no-CTS). We furthermore assessed correlations between lag values and number of CTS and medication conditions. ResultsRSLA analysis indicated earlier intrinsic activations in bilateral Rolandic areas when CTS occurred. More frequent epileptic discharges were correlated with earlier intrinsic activations (r=-0.46, p = 0.03 left Rolandic). Patients with LEV therapy, on the other hand, displayed delayed intrinsic activity in Rolandic areas compared to drug-naïve patients ConclusionOur RSLA analysis indicated an association between centro-temporal spikes and earlier hemodynamic activations in epileptogenic regions in Rolandic epilepsy, which were counteracted by LEV treatment. As it allows for the mapping of propagation features of intrinsic activity and drug-effects, our findings suggest potential of lag based analyses in detecting focus localization and estimating treatment effects.

Exome sequencing in 57 patients with self-limited focal epilepsies of childhood with typical or atypical presentations suggests novel candidate genes.

Self-limited focal epilepsies of childhood (SFEC) are amongst the best defined and most frequent epilepsy syndromes affecting children with usually normal developmental milestones. They include core syndromes such as Rolandic epilepsy or "Benign" epilepsy with Centro-Temporal Spikes and the benign occipital epilepsies, the early onset Panayiotopoulos syndrome and the late-onset Gastaut type. Atypical forms exist for all of them. Atypical Rolandic epilepsies are conceptualized as belonging to a continuum reaching from the "benign" RE to the severe end of the Landau-Kleffner (LKS) and Continuous Spike-Waves during Sleep syndromes (CSWS). GRIN2A has been shown to cause the epilepsy-aphasia continuum that includes some patients with atypical Rolandic epilepsy with frequent speech disorders, LKS and CSWS. In the present study, we searched novel genes causing SFEC with typical or atypical presentations. Exome sequencing was performed in 57 trios. Patients presented with typical or atypical SFEC, negative for GRIN2A pathogenic variant. We found rare candidate variants in 20 patients. Thirteen had occurred de novo and were mostly associated to atypical Rolandic Epilepsy. Two of them could be considered as disease related: a null variant in GRIN2B and a missense variant in CAMK2A. Others were considered good candidates, including a substitution affecting a splice site in CACNG2 and missense variants in genes encoding enzymes involved in chromatin remodeling. Our results further illustrate the fact that atypical SFEC are more likely to have Mendelian inheritance than typical SFEC.

Nonlinear Analysis of Visually Normal EEGs to Differentiate Benign Childhood Epilepsy with Centrotemporal Spikes (BECTS)

Childhood epilepsy with centrotemporal spikes, previously known as Benign Epilepsy with Centro-temporal Spikes (BECTS) or Rolandic Epilepsy, is one of the most common forms of focal childhood epilepsy. Despite its prevalence, BECTS is often misdiagnosed or missed entirely. This is in part due to the nocturnal and brief nature of the seizures, making it difficult to identify during a routine electroencephalogram (EEG). Detecting brain activity that is highly associated with BECTS on a brief, awake EEG has the potential to improve diagnostic screening for BECTS and predict clinical outcomes. For this study, 31 patients with BECTS were retrospectively selected from the BCH Epilepsy Center database along with a contrast group of 31 patients in the database who had no form of epilepsy and a normal EEG based on a clinical chart review. Nonlinear features, including multiscale entropy and recurrence quantitative analysis, were computed from 30-second segments of awake EEG signals. Differences were found between these multiscale nonlinear measures in the two groups at all sensor locations, while visual EEG inspection by a board-certified child neurologist did not reveal any distinguishing features. Moreover, a quantitative difference in the nonlinear measures (sample entropy, trapping time and the Lyapunov exponents) was found in the centrotemporal region of the brain, the area associated with a greater tendency to have unprovoked seizures, versus the rest of the brain in the BECTS patients. This difference was not present in the contrast group. As a result, the epileptic zone in the BECTS patients appears to exhibit lower complexity, and these nonlinear measures may potentially serve as a clinical screening tool for BECTS, if replicated in a larger study population.

Electrical status epilepticus in sleep affects intrinsically connected networks in patients with benign childhood epilepsy with centrotemporal spikes

BackgroundAlthough outcomes of benign childhood epilepsy with centrotemporal spikes (BECTS) are frequently excellent, some atypical forms of BECTS, especially electrical status epilepticus in sleep (ESES), are characterized by worse outcomes and negative impacts on cognitive development. MethodsTo explore specific ESES-related brain networks in patients with BECTS, we used resting-state functional magnetic resonance imaging (fMRI) to scan patients with BECTS with ESES (n = 9), patients with BECTS without ESES (n = 17), and healthy controls (n = 36). Unbiased seed-based whole-brain functional connectivity (FC) was adopted to explore the connectivity mode of three resting-state cerebral networks: the default mode network (DMN), salience network (SN), and central executive network (CEN). ResultsCompared with the other two groups, patients with BECTS with ESES showed FC in the SN or in the CEN decreased, but not in the DMN. Moreover, we found the FC in the CEN in patients with BECTS without ESES decreased when compared with controls. Our currently intrinsically defined anticorrelated networks strength was disrupted in BECTS and connote greater deactivation than the results from FC for a seed region in children with BECTS. ConclusionThese results indicated that children with BECTS with ESES showed brain activity altered in the CEN and the SN. The difference of impairment in the SN and CEN may lead to improve the understanding of the underlying neuropathophysiology, and to assess the activity of patients with BECTS with ESES, which is crucial for measuring disease activity, improving patient care, and assessing the effect of antiepilepsy therapy.

Research progress in genetic studies of Rolandic epilepsy

Rolandic epilepsy (RE), also known as benign epilepsy of childhood with centrotemporal spikes (BECTS), is the most common childhood idiopathic epilepsy syndrome.In the past, RE was often thought to have a good prognosis and usually disappeared spontaneously before puberty.However, Aicardi and Chevrie proposed the concept of RE variant (ARE) in 1982, and claimed that the clinical and electrophysiological manifestations of some RE children patients were in accordance with the characteristics of RE at the early stage of onset, but the clinical and EEG deteriorated during the course of the disease.RE is now considered to be a continuous epileptic-aphasia spectrum (EAS) disorder that causes mild to severe brain damage, often accompanied by varying degrees of cognitive and speech dysfunction.The etiology of RE is complex, and its pathogenesis is still not quite clear.With the development of molecular genetics, a complex interplay between the polygenic inheritance and environment has been found contributed to the etiology of RE.In addition, many striking genes are discovered.In this review, recent gene findings associated with RE were stressed, and the molecular biological characteristics and variants of related genes (GRIN2A, KCNQ2, KCNQ3, DEPDC5, ELP4-PAX6, GABAA-R, RBFOX1/3), clinical-gene correlation and variants-function investigation were described in detail in order to help with interpretation of clinical gene report clinical diagnosis of RE. Key words: Rolandic epilepsy; Epilepsy-aphasia spectrum; Genetics

Electroencephalographic abnormalities are correlated with cognitive deficits in children with benign childhood epilepsy with centrotemporal spikes: A clinical study of 61 cases

ObjectiveThe objective of this study was to explore the effect of spikes on cognition in patients with benign childhood epilepsy with centrotemporal spikes (BECTS) and to identify electroencephalography (EEG) markers enabling early detection of cognitive impairment. MethodsSixty-one children with BECTS diagnoses and 60 age- and education-matched healthy controls were enrolled. Four-hour EEG recordings were analyzed for each patient to check for interictal spikes, high-frequency oscillations (HFOs), nondipole spikes, and other atypical EEG features and to examine the spike–wave index of nonrapid eye movement (NREM) sleep. All 121 children underwent a series of neuropsychological tests to assess cognitive function. ResultsPatients with a high NREM sleep discharge index (≥55%) in the first sleep cycle exhibited significantly lower scores for arithmetic calculation, executive function, and attention and memory tests than patients with a low discharge index (<55%). Eight patients with HFOs exhibited even poorer performance than HFO-negative patients for arithmetic calculation, executive function, vocabulary comprehension, visual perception, vocal perception, spatial memory ability, and response ability. Children with bilateral discharge exhibited poorer ability in three-dimensional spatial imaging test, poorer memory, and slower response than did those with unilateral discharge (P < .05). Nondipole spikes, multiple asynchronous discharges, and generalized spike–wave discharges respectively had an impact on calculation ability, memory, and reaction ability respectively (P < .05). ConclusionsSpike frequencies in stage 3 and 4 sleep varied from those observed in stage 1 and 2 sleep; the highest spike frequency was in stage 2 sleep. High NREM sleep discharge index (i.e., ≥55%) and HFOs were linked to the highest risk for cognitive deficit, while bilateral discharges, nondipole spikes, multiple asynchronous discharges, and generalized spike–wave discharges were less indicative of cognitive impairment.

Focal cortical hypermetabolism in atypical benign rolandic epilepsy

ObjectiveAtypical benign rolandic epilepsy (BRE) is an underrecognized and poorly understood manifestation of a common epileptic syndrome. Most consider it a focal epileptic encephalopathy in which frequent, interictal, centrotemporal spikes lead to negative motor seizures and interfere with motor and sometimes speech and cognitive abilities. We observed focal cortical hypermetabolism on PET in three children with atypical BRE and investigated the spatial and temporal relationship with their centrotemporal spikes. MethodsEEG, MRI and PET were performed clinically in three children with atypical BRE. The frequency and source localization of centrotemporal spikes was determined and compared with the location of maximal metabolic activity on PET. ResultsCortical hypermetabolism on thresholded PET t-maps and current density reconstructions of centrotemporal spikes overlapped in each child, in the central sulcus region, the distances between the "centers of maxima" being 2 cm or less. Hypermetabolism was not due to recent seizures or frequent centrotemporal spikes at the time of FDG uptake. SignificanceThe findings suggest that localized, increased cortical activity, in the region of the EEG focus, underlies the negative clinical manifestations of atypical BRE. Similar findings are reported in the broader group of epileptic encephalopathies associated with electrical status epilepticus in sleep.

Temporal trends in incidence of Rolandic epilepsy, prevalence of comorbidities and prescribing trends: birth cohort study

ObjectiveTo examine temporal trends in incidence of Rolandic epilepsy (RE), prevalence of comorbidities and antiepileptic drug (AED) prescribing patterns.DesignRetrospective cohort study.SettingThe UK.PatientsChildren aged 0–16 years born 1994–2012 were followed from...