Electrical Status Epilepticus Research Articles

IVIg Treatment Ameliorates Inflammation in Patients with Sleep-related Electrical Status Epilepticus (ESES) (P5-14.003)

IVIg Treatment Ameliorates Inflammation in Patients with Sleep-related Electrical Status Epilepticus (ESES) (P5-14.003)

Pharmacoresistant epilepsy with electrical status epilepticus during slow-wave sleep: a clinical case

The article presents a clinical case of epilepsy with electrical status epilepticus during slow-wave sleep, the example of which shows the relevance of the issue of timely diagnosis and prescription of adequate anti-epileptic therapy in this syndrome. The role of regular electroencephalographic sleep monitoring in order to monitor the effectiveness of therapeutic measures is emphasized, and the main difficulties in making this diagnosis and choosing the correct anti-epileptic treatment are shown.

Using scalp EEG to predict seizure recurrence and electrical status epilepticus in children with idiopathic focal epilepsy

PurposeSome individuals with idiopathic focal epilepsy (IFE) experience recurring seizures accompanied by the evolution of electrical status epilepticus during sleep (ESES). Here, we aimed to develop a predictor for the early detection of seizure recurrence with ESES in children with IFE using resting state electroencephalogram (EEG) data. MethodsThe study group included 15 IFE patients who developed seizure recurrence with ESES. There were 17 children in the control group who did not experience seizure recurrence with ESES during at least 2-year follow-up. We used the degree value of the partial directed coherence (PDC) from the EEG data to predict seizure recurrence with ESES via 6 machine learning (ML) algorithms. ResultsAmong the models, the Xgboost Classifier (XGBC) model achieved the highest specificity of 0.90, and a remarkable sensitivity and accuracy of 0.80 and 0.85, respectively. The CATC showed balanced performance with a specificity of 0.85, sensitivity of 0.73, and an accuracy of 0.80, with an AUC equal to 0.78. For both of these models, F4, Fz and T4 were the overlaps of the top 4 features. ConclusionsConsidering its high classification accuracy, the XGBC model is an effective and quantitative tool for predicting seizure recurrence with ESES evolution in IFE patients. We developed an ML-based tool for predicting the development of IFE using resting state EEG data. This could facilitate the diagnosis and treatment of patients with IFE.

Electrical status epilepticus during slow sleep (ESES) and clinical features of cognitive and behavioral disorders in children with idiopathic forms of epileptic syndromes

Electrical status epilepticus during slow sleep (ESES) and clinical features of cognitive and behavioral disorders in children with idiopathic forms of epileptic syndromes

Electro-clinical features and long-term outcomes in guanidinoacetate methyltransferase (GAMT) deficiency

ObjectiveTo evaluate clinical characteristics and long-term outcomes in patients with guanidinoacetate methyltransferase (GAMT) deficiency with a special emphasis on seizures and electroencephalography (EEG) findings. MethodsWe retrospectively analyzed the clinical and molecular characteristics, seizure types, EEG findings, neuroimaging features, clinical severity scores, and treatment outcomes in six patients diagnosed with GAMT deficiency. ResultsMedian age at presentation and diagnosis were 11.5 months (8–12 months) and 63 months (18 months −11 years), respectively. Median duration of follow-up was 14 years. Global developmental delay (6/6) and seizures (5/6) were the most common symptoms. Four patients presented with febrile seizures. The age at seizure-onset ranged between 8 months and 4 years. Most common seizure types were generalized tonic seizures (n = 4) and motor seizures resulting in drop attacks (n = 3). Slow background activity (n = 5) and generalized irregular sharp and slow waves (n = 3) were the most common EEG findings. Burst-suppression and electrical status epilepticus during slow-wave sleep (ESES) pattern was present in one patient. Three of six patients had drug-resistant epilepsy. Post-treatment clinical severity scores showed improvement regarding movement disorders and epilepsy. All patients were seizure-free in the follow-up. ConclusionsEpilepsy is one of the main symptoms in GAMT deficiency with various seizure types and non-specific EEG findings. Early diagnosis and initiation of treatment are crucial for better seizure and cognitive outcomes. This long-term follow up study highlights to include cerebral creatine deficiency syndromes in the differential diagnosis of patients with global developmental delay and epilepsy and describes the course under treatment.

Risk factors of electrical status epilepticus during sleep in children with benign childhood epilepsy with centro-temporal spikes.

To explore risk factors of electrical status epilepticus during sleep in children with benign childhood epilepsy with centro-temporal spikes (BECT). This is a clinical comparative study. The subjects of study were 67 children with BECT from the Outpatient Department of Pediatric Neurology in Xingtai People's Hospital from January 2019 to January 2022. According to the occurrence of ESES, the enrolled children were divided into control group which included BECT children without ESES and the observation group which included BECT children with ESES. Compared differences of the two groups in the age of first seizure, the frequency of seizures before treatment, the classification of treatment drugs, cranial MRI, and discharge side of electroencephalogram (EEG). There was no statistical difference between the two groups in the frequency of seizures before treatment, the classification of treatment drugs, cranial MRI, and the distribution of EEG discharges in the left and right cerebral areas(P>0.05). Statistical differences were observed in the age of the first seizure, whether the seizures occurred after treatment, and EEG discharges in unilateral/bilateral cerebral areas (P<0.05). Furthermore, the collinearity test and Logistic regression analysis showed that the age of the first seizure, the frequency of seizures before treatment, and whether the seizures occurred after treatment were independent risk factors for the occurrence of ESES in BECT (P<0.05). Clinically, the occurrence of ESES in children with BECT may be related to the younger age of the first seizure, higher frequency of seizures before treatment, and the occurrence of seizures after treatment.

An attention-based deep learning method for the detection of electrical status epilepticus during sleep from electroencephalogram waveform analysis in children

Electrical status epilepticus during sleep (ESES) is a special interictal electroencephalographic phenomenon observed in children with epilepsy. ESES refers to continuous or nearly continuous epileptiform discharges of bilateral (or unilateral) spikes and waves induced during non-rapid eye movement (NREM) sleep. It mainly manifests in a series of childhood epileptic syndromes characterized by seizures, ESES, and cognitive impairment, which seriously affect the health of children. Typically, the spike-wave index (SWI), which is the percentage of spikes and waves duration in the total NREM time, is an important criterion for diagnosing ESES. The SWI measures the proportion of the total duration of spikes and waves to the NREM sleep time within several hours. Currently, SWI is obtained primarily by manual measurement and calculation, which has a certain subjective bias, low accuracy, time and labor consumption, and is a waste of medical resources. Therefore, we designed a novel data segmentation model based on a multiresolution convolutional neural network and a self-attention mechanism to calculate the SWI quickly and accurately. This can assist clinicians in making more accurate judgments and providing a better guide for the treatment of children with epilepsy. A large amount of clinical patient data was used to test the proposed model. The results confirmed that this model has an acceptable generalization ability for data from different patients and the same patient at different times. Each performance index of the model was superior to that of current common models, thus fulfilling the requirements for clinical testing.

The prospective study of 54 children with ESES: how to simplify the EEG diagnosis and guide the treatment.

To simplify the electroencephalogram (EEG) diagnosis and guide the treatment of electrical status epilepticus during sleep (ESES). We recruited 54 children with ESES from December 2019 to December 2020 and compared various spike-wave index (SWI) calculation methods. Time-frequency analysis assessed the correlation between high-frequency oscillations (HFOs) energy and the SWI. We divided 42 children into responder and non-responder treatment groups based on the observations made during a 12-month follow-up period and evaluate different treatment and the independent risk factors of refractory ESES. The SWI of five minutes before the second sleep cycle of non-rapid eye movement (NREM; long method II) and that of all NREM sleep (total method) were not significantly different (P = 0.06). The average energy of γ (r = 0.288, P = 0.002) and ripple (r = 0.203, P = 0.04) oscillations were correlated with the SWI. Encephalomalacia was an independent risk factor for refractory ESES (OR: 10.48, 95% CI: 1.62-67.63). The clinical seizure improvement rates of ASMs, ASMs with benzodiazepines, and ASMs with benzodiazepines and steroids after 12 months were 9.3%, 42.8%, and 53.8%, EEG improvement rate were 5.5%, 30.9% and 37%, respectively. The intelligence of the children in the responder treatment group has improved during the 1-year follow-up. These findings demonstrate EEG and clinical features of ESES and may provide basis for simplifying diagnosis and guiding the treatment of children with ESES.

Role of various antiepileptic drugs in electrical status epilepticus during sleep and its complications

Electrical Status Epilepticus during Sleep (ESES) is an uncommon epileptic encephalopathy typically seen in children. It features pronounced epileptiform activity during non-REM sleep stages and is linked with behavioral and cognitive impairments. This article introduces a novel method for managing patients with ESES.

Neurocognitive Profile and 18 F-Fluorodeoxyglucose Positron Emission Tomography Brain Imaging Correlation in Children with Electrical Status Epilepticus during Sleep.

Objective  Electrical status epilepticus in sleep (ESES) is defined by near-continuous epileptiform discharges during sleep along with cognitive, behavioral, and/or imaging abnormalities. We studied the neurocognitive profile and their correlation with 18 F fluorodeoxyglucose positron emission tomography (FDG PET) brain abnormalities in children with ESES. Methods  Fourteen children with ESES with normal magnetic resonance imaging (MRI) from March to December 2019 were included. The intelligence quotient (IQ) and child behavior checklist (CBCL) scores were estimated using validated scales, and FDG PET brain was done at the same point of time to look for cerebral metabolic defects which was compared with a control group. Results  Fourteen patients with a mean age of 8.2 ± 2.7 years were analyzed. The average duration of epilepsy was 6 ± 2.8 years. The mean IQ was 72.4 ± 18.2 and mean CBCL score was 37.3 ± 11.8. There was negative correlation between IQ and CBCL ( r  = -0.55, p  < 0.001). The duration of epilepsy also showed negative correlation with IQ ( r  = -4.75, p  < 0.001). FDG PET scan showed predominant thalamic hypometabolism in 12 of 14 patients (85.7%) on visual analysis with multiple other hypometabolic cortical and subcortical regions in the brain. The quantitative analysis showed significant difference in metabolism of basal ganglion when compared with control group. The total number of hypometabolic regions seen in the brain showed moderate positive correlation with CBCL score but no significant correlation with the IQ of cases. Conclusion  This study demonstrates functional impairment of cerebral cortical, basal ganglia, and thalamic hypometabolism in a cohort of ESES patients with normal structural MRI brain study. There was a moderate correlation of extent and pattern of cerebral hypometabolism with the neuropsychological status of the child and duration of epilepsy.

The Prevalence and Risk Factors of Electrical Status Epilepticus During Slow-Wave Sleep in Self-Limited Epilepsy With Centrotemporal Spikes.

Objective. To investigate the prevalence and risk factors for electrical status epilepticus during slow-wave sleep (ESES) in patients with self-limited epilepsy with centrotemporal spikes (SeLECTS). Methods. The clinical and follow-up data of children with SeLECTS were collected between 2017 and 2021. Patients were divided into typical ESES, atypical ESES, and non-ESES groups according to spike-wave indices (SWI). Clinical and electroencephalography characteristics were retrospectively analyzed. Logistic regression was used to identify risk factors for ESES. Results. A total of 95 patients with SeLECTS were enrolled. Seven patients (7.4%) developed typical ESES, 30 (31.6%) developed atypical ESES, 25 (26.3%) developed ESES at the first visit, and 12 (12.6%) developed ESES during treatment and follow-up. Multivariate logistic regression analysis showed that the risk factors for SeLECTS combined with ESES were Rolandic double or multiple spikes (OR = 8.626, 95% CI: 2.644-28.147, P < .001) and Rolandic slow waves (OR = 53.550, 95% CI: 6.339-452.368, P < .001). There were no significant differences in seizure characteristics, electroencephalogram (EEG) findings, or cognitive impairment between the atypical and typical ESES groups. Conclusion. More than one-third of the SeLECTS patients combined with ESES. Both atypical and typical ESES scores can affect cognitive function. On electroencephalography, interictal Rolandic double/multiple spikes and slow-wave abnormalities may indicate SeLECTS with ESES.

Self-limited childhood epilepsies are disorders of the perisylvian communication system, carrying the risk of progress to epileptic encephalopathies-Critical review.

"Sleep plasticity is a double-edged sword: a powerful machinery of neural build-up, with a risk to epileptic derailment." We aimed to review the types of self-limited focal epilepsies..."i.e. keep as two separate paragraphs" We aimed to review the types of self-limited focal epilepsies: (1) self-limited focal childhood epilepsy with centrotemporal spikes, (2) atypical Rolandic epilepsy, and (3) electrical status epilepticus in sleep with mental consequences, including Landau-Kleffner-type acquired aphasia, showing their spectral relationship and discussing the debated topics. Our endeavor is to support the system epilepsy concept in this group of epilepsies, using them as models for epileptogenesis in general. The spectral continuity of the involved conditions is evidenced by several features: language impairment, the overarching presence of centrotemporal spikes and ripples (with changing electromorphology across the spectrum), the essential timely and spatial independence of interictal epileptic discharges from seizures, NREM sleep relatedness, and the existence of the intermediate-severity "atypical" forms. These epilepsies might be the consequences of a genetically determined transitory developmental failure, reflected by widespread neuropsychological symptoms originating from the perisylvian network that have distinct time and space relations from secondary epilepsy itself. The involved epilepsies carry the risk of progression to severe, potentially irreversible encephalopathic forms.

Effects of altered excitation-inhibition imbalance by repetitive transcranial magnetic stimulation for self-limited epilepsy with centrotemporal spikes.

Patients with self-limited epilepsy with centrotemporal spikes (SeLECTS) with electrical status epilepticus in sleep (ESES) have generalized cognitive impairment, yet treatment options are limited. Our study aimed to examine the therapeutic effects of repetitive transcranial magnetic stimulation (rTMS) on SeLECTS with ESES. In addition, we applied electroencephalography (EEG) aperiodic components (offset and slope) to investigate the improvement of rTMS on the excitation-inhibition imbalance (E-I imbalance) in the brain of this group of children. Eight SeLECTS patients with ESES were included in this study. Low-frequency rTMS (≤1 Hz) was applied for 10 weekdays in each patient. To assess the clinical efficacy and changes in E-I imbalance, EEG recordings were performed both before and after rTMS. Seizure-reduction rate and spike-wave index (SWI) were measured to investigate the clinical effects of rTMS. The aperiodic offset and slope were calculated to explore the effect of rTMS on E-I imbalance. Five of the eight patients (62.5%) were seizure-free within 3 months after stimulation, with treatment effects decreasing with longer follow-ups. The SWI decreased significantly at 3 and 6 months after rTMS compared with the baseline (P = 0.0157 and P = 0.0060, respectively). The offset and slope were compared before rTMS and within 3 months after stimulation. The results showed a significant reduction in the offset after stimulation (P < 0.0001). There was a remarkable increase in slope after the stimulation (P < 0.0001). Patients achieved favorable outcomes in the first 3 months after rTMS. The ameliorative effect of rTMS on SWI may last up to 6 months. Low-frequency rTMS could reduce firing rates in neuronal populations throughout the brain, which was most pronounced at the site of stimulation. A significant reduction in the slope after rTMS treatment suggested an improvement in the E-I imbalance in the SeLECTS.

Ocular emotion discrimination disorders in self-limited epilepsy patients with centrotemporal spikes complicated with electrical status epilepticus during sleep: A pediatric neuropsychological study.

We investigated the characteristics and factors influencing eye emotion recognition in self-limited epilepsy patients with centrotemporal spikes (SeLECTS) complicated with electrical status epilepticus during sleep (ESES). We selected SeLECTS (n=160) patients treated in the outpatient and inpatient departments of Anhui Children's Hospital from September 2020 to January 2022. According to the video electroencephalogram monitoring slow-wave index (SWI), SeLECTS patients with SWI<50% were assigned into the typical SeLECTS group (n=79), and patients with SWI ≥ 50% were assigned into the ESES group (n=81). Patients in the two groups were assessed by The Eye Basic Emotion Discrimination Task (EBEDT) and The Eye Complex Emotion Discrimination Task (ECEDT), respectively. Comparisons were made with age-, sex- and education level-matched healthy control participants. The correlation between the characteristics of emotional discrimination disorder in the eye area and the clinical influencing factors was analyzed in ESES group, and p ≤ .050 was the threshold for significance. Relative to the healthy control group, scores of sadness and fear in the typical SeLECTS group were markedly lower (p=.018, p=.023), while differences in scores of disgust, happiness, surprise, and anger were not significantly different between the groups (p=.072, p=.162, p=.395, p=.380, respectively). Compared with the healthy control group, the ESES group had significantly low scores in recognition of sadness, fear, disgust, and surprise (p=.006, p=.016, p=.043 and p=.038, respectively). However, differences in recognition of happiness and anger between the groups were not significant (p=.665 and p=.272). Univariate logistic analysis showed that the score of eye recognition for sadness in the ESES group was affected by age of onset, SWI, ESES duration and number of seizures. The score of eye recognition for fear was mainly affected by SWI, while the score of eye recognition for disgust was affected by SWI and number of seizures. The surprised eye emotion recognition score was mainly affected by the number of seizures. Variables with p<.1 were considered to be independent variables of multivariable ordered logistic regression. Multivariate logistic analysis showed that sadness emotion recognition was mainly affected by SWI and ESES duration, while disgust was mainly affected by SWI. The typical SeLECTS group showed impaired emotion (sadness and fear) recognition function in the eye area. The ESES group was associated with more intense emotional (sadness, fear, disgust, and surprise) recognition impairment in the eye region. The higher the SWI, the younger the onset age and the longer the duration of ESES, while the more the number of seizures, the more serious the impairment of emotional recognition function in the affected eye area.

N°12 – Efficacy of steroids in seizure control and dipole stabilisation in drug resistant Electrical Status Epilepticus in Sleep due to structural cause

N°12 – Efficacy of steroids in seizure control and dipole stabilisation in drug resistant Electrical Status Epilepticus in Sleep due to structural cause

N°11 – Classification of Electrical Status Epilepticus in Sleep based on EEG patterns and spatiotemporal mapping of spikes

N°11 – Classification of Electrical Status Epilepticus in Sleep based on EEG patterns and spatiotemporal mapping of spikes

Evaluation of Thiol-Disulfide Homeostasis with Electrical Status Epilepticus in Slow Sleep (ESES).

Electrical status epilepticus in sleep (ESES) is an epileptic syndrome specific to childhood and has a broad clinical spectrum that included seizures, behavioral/cognitive impairments, and motor neurological symptoms. Antioxidants are seen as promising neuroprotective strategies for the epileptic state by combating the harmful effects of excessive oxidant formation in mitochondria. This study aims to evaluate the thiol-disulfide balance and to determine whether it can be used in the clinical and electrophysiological follow-up of patients with ESES, especially in addition to the electroencephalography (EEG) examination. The study included 30 patients, aged 2-18 years and diagnosed with ESES in the Pediatric Neurology Clinic of the Training and Research Hospital and a control group of 30 healthy children. Total thiol, native thiol, disulfide, and ischemia-modified albumin (IMA) levels were measured, and disulfide-thiol ratios were calculated for both groups. Native thiol and total thiol levels were significantly lower and IMA level and disulfide-native thiol percentage ratio were significantly higher in the ESES patient group than in the control group. Serum thiol-disulfide homeostasis is an accurate marker of oxidative stress in ESES, and standard and automated measures of thiol-disulfide balance as an indicator of oxidative stress showed a shift toward oxidation in ESES patients in this study. The negative correlation between spike-wave index (SWI) and thiol levels, and serum thiol-disulfide levels suggest that they can be used as biomarkers for follow-up of patients with ESES in addition to EEG. IMA can also be used for long-term response to monitoring purposes at ESES.

Electrical Status Epilepticus during Sleep in a Male Filipino with Rare Nonsense Mutation Variant of Sotos Syndrome on Carbamazepine Monotherapy

Electrical status epilepticus during sleep (ESES) is an electrographic pattern associated with specific genetic disorders, brain malformations, and use of some antiseizure medications. This case report aims to present the management of ESES in Sotos syndrome (SoS) on carbamazepine.A nine-year-old Filipino male with clinical features suggestive of overgrowth syndrome presented with febrile seizure at one year old. Cranial imaging showed cavum septum pellucidum, corpus callosal dysgenesis, and ventriculomegaly. He was on carbamazepine monotherapy starting at three years old. A near continuous diffuse spike–wave discharges in slow wave sleep was recorded at nine years old hence shifted to valproic acid. Follow-up study showed focal epileptiform discharges during sleep with disappearance of ESES. Next generation sequencing tested positive for rare nonsense mutation of nuclear receptor binding set-domain protein 1 confirming the diagnosis of SoS.Advanced molecular genetics contributed to determination of ESES etiologies. To date, this is the first documented case of SoS developing ESES. Whether an inherent genetic predisposition or drug-induced, we recommend the avoidance of carbamazepine and use of valproic acid as first-line therapy. &nbsp; &nbsp

Early electrophysiological predictors of progression of hydrocephalus in children with epilepsy

Hydrocephalus is a severe disease in children of all age groups. Failure to treat hydrocephalus in a timely manner can lead to progressive neurological deficits and death. Instead, early diagnosis and treatment of progressive hydrocephalus can completely recover the child. The term «early diagnosis» of hydrocephalus is the most difficult task for a pediatric neurologist. There are no pathognomonic clinical signs of this disease. Detection of electrophysiological signs of hydrocephalus progression in the early stages of the disease could significantly affect on treatment outcomes. The aim of our work is to establish early electrophysiological predictors of the progression of hydrocephalus in children with epilepsy. Materials and methods. 28 children with epilepsy were examined at the Transcarpathian Regional Children’s Hospital and the Medical Center «Diamed» in Uzhhorod from 2018 to 2022. All children were examined prospectively using video-EEG monitoring with sleep fixation, magnetic resonance imaging (MRI), multispiral computer tomography (MSCT). Results. The electrical status epilepticus during slow-wave sleep (ESES) was registered in 35 % of examined patients (8 children). The average duration from detection of the electrical status of sleep to the establishment of shunt system in the brain is 37 days (from 15 to 60 days). The ESES disappeared in 6 children during the first month after the implantation of the shunt system in the brain, in 2 children during the next 3 and 6 months of observation. We have developed our own algorithm for examining children with hydrocephalus and epilepsy up to 1 year and older than 1 year. Conclusion. An intense increase of epileptiform activity and appearance of electrical status of slow-wave sleep may be one of the predictors of the progression and decompensation of hydrocephalus and requires dynamic control. Further randomized trials are needed to assess the significance of these changes. The developed algorithm will increase the effectiveness of the use of additional examination methods for early detection of the progression of hydrocephalus in children with epilepsy.

Electrical Status Epilepticus during Sleep: Risk Factors, Clinical Course, and Treatment Approaches

Abstract Background The efforts of clinicians are focused on determining the predictors for electrical status epilepticus in sleep (ESES) manifestation, due to the negative effect of ESES on cognition. Treatment approaches remain a leading problem because of therapeutic resistance. Objective We looked for potential risk factors for ESES manifestation and summarize the clinical course and therapeutic approaches in patients with idiopathic and symptomatic ESES. Patients and Methods We retrospectively reviewed the medical data of 51 children with idiopathic ESES and 20 children with symptomatic ESES. Results We observed an earlier age of seizure onset (p = 0.0002) and a higher percentage of cases with multiple seizures (p &lt; 0.00001) and with postictal paralysis (p &lt; 0.00001) in idiopathic ESES compared with childhood epilepsy with centrotemporal spikes. In the idiopathic ESES, the treatment consisted of corticosteroids in patients with permanent ESES remission and transient remission, levetiracetam (LEV) children with permanent ESES remission and transient, clonazepam (CZP) children with permanent ESES remission and transient, ethosuximide (ESM), and sulthiame. The patients with symptomatic ESES had more unfavorable evolution, as 19 children had persistent or relapsing ESES course. Conclusion We consider the earlier age of seizure onset (below 5 years) and the presence of multiple seizures and postictal paresis as risk factors for ESES manifestation. ESES is characterized by a significant therapeutic resistance, especially in the group of symptomatic cases. Good results are observed with LEV, ESM, CZP, and steroids.