Spike-wave Index Research Articles

Diagnostic and prognostic significance of serum interleukins in epileptic encephalopathy with spike wave activation in sleep (EE-SWAS) syndrome

ObjectiveTo study serum interleukin-6(IL-6), interleukin-8(IL-8) and interleukin-10(IL-10) levels in Epilpetic encephalopathy with spike-wave activation in sleep(EE-SWAS), drug refractory epilepsy(DRE) and well controlled epilepsy(WCE). MethodsChildren(2–12 years) with immunotherapy naïve EE-SWAS, DRE and WCE were enrolled. Valid psychometric tools were used to assess cognition and behavior. Children with EE-SWAS were longitudinally followed. They received a three-month steroid course alongwith the ongoing antiseizure drugs. Electroclinical responders were defined as change in social quotient by 5-points with improvement in atleast one behavioral domain by 5-points and 50 % reduction in mean seizure frequency if active seizures were present alongwith a 25 % reduction in Spike-wave-index(SWI) at three months. Change in serum Interleukin levels at one month follow up was compared between participants who eventually became responders or non-responders at three months. ResultsTwenty children with EE-SWAS, 18 with DRE and WCE each were enrolled. Serum IL-6(pg/ml){(EE-SWAS: 3.775(IQR 2.205, 11.28); DRE: 3.01(IQR 2.04, 4.56); WCE: 1.655(IQR 1.27, 2.29), p = 0.0065} and IL-8(pg/ml){(EE-SWAS: 103.2(IQR 34.01, 200.82); DRE: 19.595(IQR 16.54, 39.7); WCE: 18.97(IQR 16.54, 21.91) p = 0.0002} was significantly different between the three groups. In EE-SWAS group 12/20(60 %) showed electroclinical response to steroids. Responders had significant reduction in IL6 levels (pg/ml){4.045(IQR 2.605, 18.96) to 1.13(IQR 054, 1.74)} at one month follow up compared to non responders {3.12(IQR 1.655, 5.27) to 4.37(IQR 2.83, 9.855)} (p = 0.0069). ConclusionsProinflammatory cytokines (IL-6 and IL-8) are significantly elevated in EE-SWAS compared to DRE and WCE. Reduction in IL-6 levels at one month post-therapy predicted electroclinical responders at 3months follow up.

The First 100 Seconds of Sleep of rEEGs Can Be a Reliable Scoring Method for D/EE-SWAS.

Developmental/epileptic encephalopathy with spike wave activation with sleep, formerly known as electrical status epilepticus in sleep, is an electrographic pattern in which the interictal epileptiform activity is augmented by transition to sleep. Recent studies demonstrate the utility of the first 100 seconds of sleep of long-term monitoring (LTM) as a scoring method for electrical status epilepticus in sleep. Our aim was to measure the reliability of the spike-wave index (SWI) of the first 100 seconds of sleep of routine EEG (rEEG) as a tool for diagnosis of developmental/epileptic encephalopathy with spike wave activation with sleep. Approximately three hundred forty LTMs were reviewed, and 25 studies from 25 unique patients had comparable rEEGs. Two neurophysiologists calculated the SWI of the first 100 seconds of spontaneous stage II non-random eye movement sleep, the first 5-minute bin of sleep, and three separate 5-minute bins throughout sleep in LTM. This was compared to the SWI of the first 100 seconds of sleep in rEEG. Agreement was analyzed using Lin's concordance correlation coefficient (CCC). Using 50% as a diagnostic cut-off, we observed moderate agreement between the SWI of the first 100 seconds of sleep of rEEG and three bin LTM (CCC = 0.94, 95% CI: 0.88-0.97). Agreement was slightly higher for the comparison to first bin LTM SWI (CCC = 0.96, 95% CI: 0.92-0.98) and first 100 seconds LTM SWI (CCC = 0.96, 95% CI: 0.92-0.98). This study demonstrates the first 100 seconds of sleep of rEEG technique as a time efficient diagnostic tool for patients with concern for developmental/epileptic encephalopathy with spike wave activation with sleep.

Sulthiame use in children with pharmacoresistant epilepsies: A retrospective study.

This retrospective study aimed to assess the efficacy and tolerability of sulthiame as an add-on treatment in children with pharmacoresistant epilepsies. All patients with epilepsy who received sulthiame at Montreal Children's Hospital over an 11-year period were included. Medical charts were reviewed, and extracted data included patient age and sex, seizure types, epilepsy syndrome, electroencephalography (EEG) reports, brain imaging reports, antiseizure treatments trialed, starting and final dose of sulthiame, duration of sulthiame treatment, adverse events attributed to sulthiame, and seizure frequency before and after sulthiame treatment. EEG studies were also analyzed and spike-wave index (SWI) in the first 10 min of sleep was calculated. Sixteen patients were included, all of whom had pharmacoresistant epilepsies (mean of 9.9 trials of other antiseizure treatments). Six had genetic diagnoses, four had in utero/perinatal acquired brain injury, one had a suspected focal cortical dysplasia, and five were idiopathic. Ten patients had developmental and epileptic encephalopathy with spike-wave activation in sleep, three had Lennox-Gastaut syndrome, and one each had sleep-related hyperkinetic epilepsy, self-limited epilepsy with centrotemporal spikes, and mixed generalized and multifocal epilepsy. Of the 12 patients with uncontrolled seizures at the time of sulthiame initiation, 4 had improvement in seizure frequency, including 2 who became seizure free. Eight patients had EEG data available that allowed calculation of sleep SWI; from this group, SWI decreased from 81.1% +/- 17.6% to 45.1% +/- 36.5% (p = .007). The most common adverse events reported were somnolence/drowsiness, aggression, and increased seizure frequency. Of the patients with genetic etiologies, significant positive responses were seen in patients with pathogenic variants in NDUFS1 and SATB1. These data demonstrate the therapeutic potential of sulthiame, even in patients with highly pharmacoresistant epilepsy. Improvements may be seen in both seizure frequency and sleep SWI.

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.

A Multicenter Study of Self-Limited Epilepsy With Centrotemporal Spikes: Effectiveness of Antiseizure Medication With Respect to Spike-Wave Index

BackgroundThere is no certain validated electroencephalographic (EEG) parameters for outcome prediction in children with self-limited epilepsy with centrotemporal spikes. To assess the effectiveness of antiseizure medication (ASM) for seizure outcome with respect to the spike-wave index (SWI) on serial EEG recordings. MethodsIn this multicenter study, the study cohort consisted of 604 children with self-limited epilepsy with centrotemporal spikes. A data set of epilepsy centers follow-up between 2010 and 2022. The cohort was divided into 4 groups as those receiving 3 different monotherapy (carbamazepine [CBZ]/valproic acid [VPA]/levetiracetam [LEV]) and dual therapy. SWI analysis was performed with the percent of spikes in the 2-minute epoch in the 5th 6th minutes of the nonrapid eye movement sleep EEG record. The study group were also categorized according to seizure burden with seizure frequency (I) >2 seizures and (II) >5 seizures. Seizure outcome was evaluated based on the reduction in seizure frequency over 6-month periods: (1) 50% reduction and (2) seizure-free (complete response). ResultsASM monotherapy was achieved in 74.5% children with VPA, CBZ, and LEV with similar rates of 85.8%, 85.7%, and 77.9%. Dual therapy was need in the 25.5% of children with SeLECT. More dual therapy was administered in children aged below 5 years with a rate of 46.2%. Earlier seizure-free achievement time was seen in children with LEV monotherapy with more complete-response rate (86.7%) compared the VPA and CBZ. ConclusionsWe also determined that the children on dual therapy had more SWI clearance in the subsequent EEG recordings. The ROC curve analyses were performed to predict initial drug selection with using the SWI% might be used for the prediction of ASM type and drug selection in children.

Clinical Analysis of Lacosamide Monotherapy in the Treatment of Self-Limited Epilepsy with Centrotemporal Spikes.

To evaluate the efficacy and safety of lacosamide (LCM) monotherapy in the treatment of self-limited epilepsy with centrotemporal spikes (SeLECTS). In this study, 89 children with SeLECTS who were treated with LCM monotherapy in the Children's Hospital Affiliated to Soochow University from June 2019 to June 2021 were included. Clinical seizures and spike wave index (SWI) on video EEG during slow-wave sleep were evaluated before and after treatment. The role of LCM monotherapy in improving SWI, controlling clinical seizures and improving cognition was analyzed, and corresponding adverse reactions were documented. There were 52 males and 37 females in this group, with an average age of 7.6 ± 2.1 years. The total effective rate was 93.83% and at 18 months of treatment, the cumulative control rate was 85.19%, the retention rate was 91.01% and the effective rate in terms of EEG spike index was 72.92%, all of which showed a high rate; there was no statistically significant difference in intelligence quotient before and after treatment (P > 0.05). In addition, it was found in the study that the earlier the age of onset, the less obvious the improvement in SWI after LCM treatment; the lower the baseline seizure frequency, the more significant the improvement in SWI after LCM treatment. LCM monotherapy had curative effect and adverse reactions for SeLECTS, with no negative impact on cognition. These significant findings indicate that LCM is likely to become a widely prescribed ASM for the treatment of SeLECTS. Meanwhile, the onset age and baseline seizure frequency had certain value in judging prognosis and predicting curative effect.

How the Spreading and Intensity of Interictal Epileptic Activity Are Associated with Visuo-Spatial Skills in Children with Self-Limited Focal Epilepsy with Centro-Temporal Spikes.

This paper investigates brain-behaviour associations between interictal epileptic discharges and cognitive performance in a population of children with self-limited focal epilepsy with centro-temporal spikes (SeLECTS). Sixteen patients with SeLECTS underwent an extensive neuropsychological assessment, including verbal short-term and episodic memory, non-verbal short-term memory, attentional abilities and executive function. Two quantitative EEG indices were analysed, i.e., the Spike Wave Index (SWI) and the Spike Wave Frequency (SWF), and one qualitative EEG index, i.e., the EEG score, was used to evaluate the spreading of focal SW to other parts of the brain. We investigated associations between EEG indices and neuropsychological performance with non-parametric Spearman correlation analyses, including correction for multiple comparisons. The results showed a significant negative correlation between (i) the awake EEG score and the Block Tapping Test, a visuo-spatial short-term memory task, and (ii) the sleep SWI and the Tower of London, a visuo-spatial planning task (pcorr < 0.05). These findings suggest that, in addition to the usual quantitative EEG indices, the EEG analysis should include the qualitative EEG score evaluating the spreading of focal SW to other parts of the brain and that neuropsychological assessment should include visuo-spatial skills.

Automated analysis of a large-scale paediatric dataset illustrates the interdependent relationship between epilepsy and sleep

Slow waves are an electrophysiological characteristic of non-rapid eye movement sleep and a marker of the restorative function of sleep. In certain pathological conditions, such as different types of epilepsy, slow-wave sleep is affected by epileptiform discharges forming so-called “spike-waves”. Previous evidence shows that the overnight change in slope of slow waves during sleep is impaired under these conditions. However, these past studies were performed in a small number of patients, considering only short segments of the recording night. Here, we screened a clinical data set of 39′179 pediatric EEG recordings acquired in the past 25 years (1994–2019) at the University Children’s Hospital Zurich and identified 413 recordings of interest. We applied an automated approach based on machine learning to investigate the relationship between sleep and epileptic spikes in this large-scale data set. Our findings show that the overnight change in the slope of slow waves was correlated with the spike-wave index, indicating that the impairment of the net reduction in synaptic strength during sleep is spike dependent.

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.

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.

Tolerability and Effectiveness of Cathodal Transcranial Direct Current Stimulation in Children with Refractory Epilepsy: A Case Series.

There are limited treatment options for drug-resistant epilepsy (DRE) in children. We performed a pilot study to investigate the tolerability and effectiveness of cathodal transcranial direct current stimulation (tDCS) in DRE. Twelve children with DRE of varied etiology underwent three to four daily sessions of cathodal tDCS. The seizure frequency at 2 weeks before and after tDCS was obtained from seizure diaries; clinic reviews at 3 and 6 months assessed any longer-term benefits or adverse effects. The spike wave index (SWI) was analyzed in the EEGs done immediately before and after tDCS on the first and last day of tDCS. One child remained seizure free for a year after tDCS. One child had reduced frequency of ICU admissions for status epilepticus for 2 weeks, likely due to reduced severity of seizures. In four children, an improvement in alertness and mood was reported for 2-4 weeks after tDCS. There was no benefit following tDCS in the other children. There were no unexpected or serious adverse effects in any child. Benefit was seen in two children, and the reasons for the lack of benefit in the other children need further study. It is likely that tDCS stimulus parameters will need to be tailored for different epilepsy syndromes and etiologies.

Developmental and/or epileptic encephalopathy with spike-and-wave activation in sleep in Saudi Arabia: Electroclinical, etiologic, genetic, and outcome multicenter study

ObjectivesTo investigate the clinical features of developmental and/or epileptic encephalopathy with spike-and-wave activation in sleep (D/EE-SWAS), its electrographic characteristics, and etiology and to compare the effects of different treatment strategies on the outcomes using a Saudi Arabian database. MethodsThis multicenter study included children with D/EE-SWAS who were evaluated between 2010 and 2020 at 11 tertiary centers. Data were collected on their baseline clinical features, etiologies, and treatment modalities. Seizure reduction, spike-wave index, and cognitive state were examined as potential therapeutic outcomes. ResultsNinety-one children were diagnosed with D/EE-SWAS, with a median age of 7 years (IQR: 3–5) and an almost equal sex distribution. The average age at which epilepsy was diagnosed was 3 years (IQR: 5–2). A genetic/metabolic etiology was found in 35.1% of the patients, and a structural etiology was found in 27.4%. Children with underlying genetic/metabolic diseases exhibited an earlier seizure onset (P = 0.001) than children with other etiologies. Benzodiazepines (76.6%) were the most common treatment, followed by steroids (51.9%). Sodium valproate (75%) was the most frequently used antiseizure medication, followed by levetiracetam (64.9%). Children with a later seizure onset were more likely to have better clinical responses (P = 0.046), EEG responses (P = 0.012), and cognitive outcomes (P = 0.006) than children with an earlier onset. Moreover, better seizure response and electrographic response were seen in patients with bilateral interictal discharges on the EEG than otherwise. Children had a higher likelihood of both clinical and electrographic improvement with combination therapy of benzodiazepines (P = 0.001) and steroids (P = 0.001) than with other therapies. SignificanceThis study shows a higher prevalence of genetic/metabolic causes and suggests the superior efficacy of combination therapy with steroids and benzodiazepines in D/EE-SWAS. Prospective studies that strictly assess the treatment protocols and outcomes are needed.

Evaluation of long-term neurocognitive functions in patients with epileptic encephalopathy with continuous spike-and-wave during sleep (CSWS)/epileptic encephalopathy with spike-and-wave activation in sleep (EE-SWAS)

ObjectivesEpileptic encephalopathy with continuous spike-and-wave during sleep (CSWS) or the newly named Epileptic encephalopathy with spike-and-wave activation in sleep (EE-SWAS) is a syndrome in which epileptiform abnormalities are associated with the progressive impairment of cognitive functions. This study aimed to evaluate the neurocognitive executive functions of patients at later ages and determine the long-term prognosis of the condition, as well as the factors affecting this. MethodsThis is a hospital-based cross-sectional study of 17 patients with a diagnosis of CSWS, and a minimum age of 7.5 years. The Wechsler Intelligence Scale for Children-Fourth Edition (WISC-IV) was used for neurocognitive assessment. The use of immunotherapy (intravenous immunoglobulin and/or steroid for at least 6 months) at the time of initial diagnosis, baseline activity and spike wave index (SWI) of the last wake and sleep EEG, cranial MRI findings, active epileptic seizures since the last examination, and WISC-IV parameters were statistically compared. The results of patients with genetic etiology determined by the whole exome sequencing (WES) method are also reported. ResultsA total of 17 patients were included in the study, with a mean age of 10.30 ± 3.15 years (range from 7.9 to 15.8 years). The mean full scale IQ score of the subjects was 61.41 ± 17.81 (range 39–91), classified as follows: 5.9% (n = 1), average; 23.5% (n = 4), low average; 5.9% (n = 1), very low; 35.3% (n = 6), extremely low (upper range); 29.4% (n = 5), extremely low (lower range) intelligence. Among the four domains of WISC-IV, the most affected index was the Working Memory Index (WMI). EEG parameters, cranial MRI findings and treatment with immunotherapy did not have a significant effect on neurocognitive outcomes. Thirteen patients (76%) were evaluated with WES for a genetic etiology. Pathogenic variants in 5 different genes (GRIN2A, SLC12A5, SCN1A, SCN8A, ADGRV1) associated with epilepsy were detected in 5/13 patients (38%). ConclusionThese results indicated that neurocognition is highly affected in the long term in CSWS.

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.

Classification of electrical status epilepticus in sleep based on EEG patterns and spatiotemporal mapping of spikes

We firstly aimed to describe and classify EEG patterns in electrical status epilepticus in sleep (ESES), and secondly subclassify EEG patterns based on analysis of spikes using spatio-temporal mapping and electrical source analysis. Overnight EEGs (minimum: eight hours) of 30 children, aged 2-12 years, with ESES (spike-wave index: at least 50%) were selected. Average reference montage was used for dipole analysis and mapping. The location and orientation of the dipoles were determined by mapping positive and negative poles and applying the rules of mapping. The onset and propagation of the spikes and the latency between the two hemispheres (for bisynchronous spikes) were determined (based on source analysis using BESA research 7.1). (1) ESES was classified as “generalised” (80%) and focal (20%) patterns; (2) the bisynchronous subtype in the “generalised” pattern was due to apparently synchronous bilateral activation of spikes (with lead-in of 20-60 ms from one hemisphere) with a tangential/oblique dipole (source analysis localised these spikes to around the peri-rolandic cortex); (3) the classic description of ESES spikes as “diffuse” spikes with bifrontal maxima is a misinterpretation using the 10-20 EEG system .Using voltage mapping and source analysis, cortical activation in the rolandic cortex was identified which imparts diffuse frontal negativity and parieto-occipital positivity; (4) ESES spikes showed intraspike and interspike dipole instability and the orientation of dipoles changed due to local spike propagation around the source and into the depth of the sulcus (which we refer to herein as “dancing dipoles”); and (5) focal ESES were classified as parietal, occipital and temporo-occipital patterns;a frontal ESES pattern was not seen. Based on detailed mapping and source analysis of ESES, we have successfully reinterpreted various misconceptions in the literature. We have simplified the interpretation of complicated EEG patterns by extracting the primary and propagated sources which aid the classification of ESES. As the dipole is always stable in self-limited childhood epilepsy with centrotemporal spikes, we believe that the phenomenon of an intrinsically unstable dipole is a reliable qualitative EEG marker of ESES.

Relationship between brain activity, cognitive function, and sleep spiking activation in new-onset self-limited epilepsy with centrotemporal spikes.

This study aimed to investigate the relationship between cognitive function sleep spiking activation and brain activity in self-limited epilepsy with centrotemporal spikes (SeLECTS). We used spike-wave index (SWI), which means the percentage of the spike and slow wave duration to the total non-REM (NREM) sleep time, as the grouping standard. A total of 14 children with SeLECTS (SWI ≥ 50%), 21 children with SeLECTS (SWI < 50%), and 20 healthy control children were recruited for this study. Cognitive function was evaluated using the Wechsler Intelligence Scale for Children, Fourth Edition (Chinese version) (WISC-IV). Magnetic source activity was assessed using magnetoencephalography calculated for each frequency band using the accumulated source imaging (ASI) technique. Children with SeLECTS (SWI ≥ 50%) had the lowest cognitive function scores, followed by those with SeLECTS (SWI < 50%) and then healthy controls. There were significant differences in the localization of magnetic source activity between the three groups: in the alpha (8-12 Hz) frequency band, children with SeLECTS (SWI ≥ 50%) showed deactivation of the medial frontal cortex (MFC) region; in the beta (12-30 Hz) frequency band, children with SeLECTS (SWI ≥ 50%) showed deactivation of the posterior cingulate cortex (PCC) segment; and in the gamma (30-80 Hz) frequency band, children in the healthy group showed activation of the PCC region. This study revealed significant decreases in cognitive function in children with SeLECTS (SWI ≥ 50%) compared to children with SeLECTS (SWI < 50%) and healthy children, as well as significant differences in magnetic source activity between the three groups. The findings suggest that deactivation of magnetic source activity in the PCC and MFC regions is the main cause of cognitive function decline in SeLECTS patients with some frequency dependence.

Are Interictal Discharges Associated with Neuronal Cell Loss in Electrical Status Epilepticus in Sleep?

Neuron-specific enolase is an established biomarker of neuronal damage. This study aimed to reveal the relationship between serum neuron-specific enolase level and continuous interictal discharges in a group of encephalopathy with electrical status epilepticus in sleep patients for the first time and determine whether there is a neuronal cell loss or damage. We analyzed serum neuron-specific enolase levels in patients with an electrical status epilepticus in sleep pattern on their electroencephalographs with age- and sex-matched control subjects. Patients with a spike-wave index of at least 50% and acquired neuropsychological regression were included in the study. Magnetic resonance imaging of all electrical status epilepticus in sleep patients and control subjects included in the study was within normal limits. Neuron-specific enolase is measured by the enzyme-linked immunosorbent assay kit based on the sandwich technique. In this study, 14 patients diagnosed with electrical status epilepticus in sleep and 21 healthy controls were included. The median age of electrical status epilepticus in sleep patients was 7.1 years (min-max: 4.5-10.7 years) and 7.7 years (min-max: 3.2-14 years) in the control subjects. According to the results of serum neuron-specific enolase measurements, the mean ± standard deviation level of neuron-specific enolase was 7.61 ± 3.19 ng/dL for the electrical status epilepticus in sleep group and 6.93 ± 2.55 ng/dL for the control group. Serum neuron-specific enolase levels between electrical status epilepticus in sleep patients and the control group were not statistically significant (P = .749). No significant difference was observed in serum neuron-specific enolase levels between electrical status epilepticus in sleep patients and control subjects. Our results may indicate that frequent interictal discharges do not result in neuronal cell loss or damage in electrical status epilepticus in sleep patients.

Effectiveness of perampanel in the treatment of pediatric patients with focal epilepsy and ESES: A single-center retrospective study.

Objective: To investigate the therapeutic effect and influencing factors of perampanel (PER) on electrical status epilepticus during sleep (ESES). Methods: We retrospectively analyzed the clinical data of pediatric patients with focal epilepsy and ESES who were treated at the Epilepsy Center of Shengjing Hospital of China Medical University between January 2016 and March 2022. Changes in the spike wave index (SWI) after 24 weeks of PER add-on treatment were compared. Kaplan‒Meier survival analysis, the log-rank test and multivariate Cox regression analysis were performed. Results: A total of 54 pediatric patients met the inclusion criteria, including 33 males and 21 females. The mean age at the diagnosis of epilepsy was 6.41 ± 2.14 years and at ESES diagnosis was 7.58 ± 2.40 years. The mean ESES duration before add-on PER was 25.31 ± 15.12 months. The mean age of the patients at add-on PER initiation was 9.69 ± 2.12 years. The ESES resolved in 29 children after 6 months of PER add-on treatment, and the response rate was 53.7%. Univariate analysis with the log-rank test showed that the therapeutic effect of PER differed according to the age at ESES diagnosis and ESES duration before add-on PER treatment. Multivariate Cox regression analysis showed that only ESES duration before PER administration was a risk factor for PER treatment failure, and the other factors had no effect on the therapeutic effect. Conclusion: PER add-on treatment has a good therapeutic effect on ESES and can be used as an alternative to corticosteroid and benzodiazepines. The therapeutic effect of PER add-on treatment was not related to the dose. A longer ESES duration results in a worse therapeutic effect. Therefore, more aggressive treatment measures should be implemented for ESES.

Clinical Spectrum and Treatment Outcomes of Patients with Developmental and/or Epileptic Encephalopathy with Spike-and-Wave Activation in Sleep

Purpose: Developmental and/or epileptic encephalopathy with spike-and-wave activation in sleep (D/EE-SWAS) is a spectrum of conditions characterized by various phenotypes of cognitive, linguistic, and behavioral regression associated with spike-and-wave activation in sleep. We aimed to investigate the phenotypic spectrum and treatment outcomes of pediatric patients with D/EE-SWAS.Methods: We retrospectively analyzed the medical records of pediatric patients diagnosed with D/EE-SWAS and treated at Severance Children’s Hospital from 2006 to 2022. We extracted information from their medical records on electroencephalography before and after treatment, types of treatment, seizure frequency, and developmental profiles. The primary outcome was reduction of the spike-wave index on electroencephalography after treatment.Results: Twenty-one patients with a median age of 5.3 years (interquartile range, 4.1 to 6.6) at diagnosis were included. Ten patients had delayed development. The patients received various anti-seizure medications. Fourteen received long-term, high-dose steroid therapy, 10 were placed on a ketogenic diet, four received intravenous steroid pulse therapy, and one each was treated with intravenous immunoglobulin and cannabidiol. The most effective treatments were steroid therapy and a ketogenic diet, which were also effective in reducing seizures and improving cognition. Side effects during treatment were transient and treatable.Conclusion: We described the clinical spectrum of pediatric patients with D/EE-SWAS. Steroid therapy and a ketogenic diet can be considered effective therapeutic options for patients with D/EE SWAS.

Are Interictal Discharges Associated with Neuronal Cell Loss in Electrical Status Epilepticus in Sleep?

Neuron-specific enolase is an established biomarker of neuronal damage. This study aimed to reveal the relationship between serum neuron-specific enolase level and continuous interictal discharges in a group of encephalopathy with electrical status epilepticus in sleep patients for the first time and determine whether there is a neuronal cell loss or damage. We analyzed serum neuron-specific enolase levels in patients with an electrical status epilepticus in sleep pattern on their electroencephalographs with age- and sex-matched control subjects. Patients with a spike-wave index of at least 50% and acquired neuropsychological regression were included in the study. Magnetic resonance imaging of all electrical status epilepticus in sleep patients and control subjects included in the study was within normal limits. Neuron-specific enolase is measured by the enzyme-linked immunosorbent assay kit based on the sandwich technique. In this study, 14 patients diagnosed with electrical status epilepticus in sleep and 21 healthy controls were included. The median age of electrical status epilepticus in sleep patients was 7.1 years (min-max: 4.5-10.7 years) and 7.7 years (min-max: 3.2-14 years) in the control subjects. According to the results of serum neuron-specific enolase measurements, the mean ± standard deviation level of neuron-specific enolase was 7.61 ± 3.19 ng/dL for the electrical status epilepticus in sleep group and 6.93 ± 2.55 ng/dL for the control group. Serum neuron-specific enolase levels between electrical status epilepticus in sleep patients and the control group were not statistically significant (P = .749). No significant difference was observed in serum neuron-specific enolase levels between electrical status epilepticus in sleep patients and control subjects. Our results may indicate that frequent interictal discharges do not result in neuronal cell loss or damage in electrical status epilepticus in sleep patients.