Interictal Electroencephalogram Research Articles

The concomitant anxiety and depression in patients with epilepsy caused by low-grade brain tumors

The aim of the study was to evaluate the concomitant psychiatric disorders of anxiety and depression in patients with epilepsy caused by low-grade brain tumors (LBTs). We retrospectively reviewed the clinical data of patients who underwent preoperative neuropsychological evaluations of anxiety and depression and subsequent epilepsy surgery for LBTs. The univariate and multivariate analyses were conducted to analyze the risk factors of the occurrence of anxiety and depression. Of the 107 patients included in the study, 42 patients (39.3%) were female, and 28 patients (26.2%) were children. The median age at surgery was 22 years (interquartile range [IQR]: 17–27 years old), the median age of seizure onset was 12 years (IQR: 6–18 years old), and the median duration of epilepsy was 84 months (IQR: 42–180 months). In total, 21 patients (19.6%) had psychiatric disorders of anxiety, and 26 patients (24.3%) had psychiatric disorders of depression. Through univariate and multivariate analysis, discordant (vs. concordant) interictal electroencephalogram (EEG) findings were found to be related to the presence of anxiety (P = 0.035, odds ratio [OR] = 3.35). Discordant (vs. concordant) ictal EEG findings (P = 0.015, OR = 4.44) and temporal location of tumor (P = 0.015, OR = 13.52) were found to be associated with the presence of depression. Psychiatric disorders of anxiety and/or depression could frequently occur in patients with epilepsy caused by LBTs. Thus, early screening and mental intervention are necessary, especially for those with discordant or wider epileptic discharges and temporal invasion of tumors.

The significance of interictal electroencephalogram analysis based on the Grand Total Electroencephalogram score in early assessment of cognitive impairment in epilepsy patients

The significance of interictal electroencephalogram analysis based on the Grand Total Electroencephalogram score in early assessment of cognitive impairment in epilepsy patients

Autosomal dominant intellectual developmental disorder 60 with seizures: a case report

The patient is a 10-month and 21-day-old girl who began to show developmental delays at 3 months of age, with severe language developmental disorders, stereotyped movements, and easily provoked laughter. Physical examination revealed fair skin and a flattened occiput. At 10 months of age, a video electroencephalogram suggested atypical absence seizures, with migrating slow-wave activity observed during the interictal period. Whole exome sequencing of three family members indicated a novel mutation in the AP2M1 gene, c.508C>T (p.R170W), in the patient. A total of six cases of autosomal dominant intellectual developmental disorder 60 with seizures associated with mutations in the AP2M1 gene have been reported both domestically and internationally (including this study). The main clinical features included developmental delays (6 cases), language developmental disorders (5 cases), stereotyped movements (3 cases), a tendency to smile (1 case), and atypical absence seizures (4 cases). Interictal electroencephalograms showed widespread spike waves and spike-slow wave discharges (5 cases), and migrating slow-wave activity (1 case). The c.508C>T (p.R170W) mutation may be a hotspot for mutations in the AP2M1 gene, and its clinical features are similar to those of Angelman syndrome.

Clinical Findings and Outcome of Moyamoya Disease/Syndrome

Objective: Moyamoya disease is a rare progressive cerebrovascular disorder. It is characterized by progressive stenosis in the terminal branches of the internal carotid arteries, leading to the formation of an abnormal vascular network. The aim of this study was to present the clinical findings and outcomes of pediatric patients diagnosed with Moyamoya disease by analyzing our cohort to identify the common clinical presentations, diagnostic challenges, and treatment outcomes associated with this rare cerebrovascular disorder. Material and Methods: Nine pediatric cases of Moyamoya disease admitted over a 10-year period were retrospectively reviewed. Clinical presentations, associated diseases, radiological findings, treatments, and outcomes were analyzed. Results: The median age at diagnosis was 48 months (3-87). Presenting symptoms included hemiparesis in five patients, seizures in six patients, headache in one patient, and choreathetosis with headache in one patient. Three patients experienced symptoms triggered by fever, and one patient by exposure to hot water. Seven patients presented with ischemic symptoms, while two presented with non-ischemic symptoms. Neurofibromatosis type-1 (NF-1) was associated with the disease in four patients. Recurrent attacks occurred in two patients. Interictal electroencephalograms (EEGs) showed hemispheric/focal slowing in five cases. Cranial magnetic resonance imaging (MRI) revealed infarctions in seven patients, and MR angiography showed bilateral findings in six patients. Two patients experienced no long-term sequelae. Conclusion: Moyamoya disease manifests with both ischemic and non-ischemic symptoms. Recognition of non-ischemic presentations requires a high index of suspicion for accurate diagnosis.

Effectiveness of vigabatrin for infantile epileptic spasm syndrome categorized by etiologies

PurposeWe aimed to assess the effectiveness of vigabatrin (VGB) in patients diagnosed with infantile epileptic spasm syndrome (IESS) and categorize these patients based on their etiologies. MethodsThis retrospective study included patients diagnosed with IESS who exhibited epileptic spasms before the age of 2 years between January 1, 2015, and October 31, 2023 at Saitama Children's Medical Center. Patients with tuberous sclerosis as the identified etiology were excluded. The effectiveness of VGB was assessed based on the resolution of ES for three months with the absence of hypsarrhythmia on interictal electroencephalogram. ResultsThis study analyzed 41 patients (26 boys). The etiologies included genetic, congenital structural, acquired structural, and unknown in 12, 11, 10, and 8 patients, respectively. Patient characteristics did not significantly differ among the four groups. The overall effectiveness of VGB for IESS was 39.0 % (16/41). Categorized based on etiology, VGB was effective in 41.7 % (5/12), 9.1 % (1/11), 50 % (5/10), and 75 % (6/8) in the genetic, congenital structural, acquired structural, and unknown groups, respectively. Statistical analysis revealed a significant difference in effectiveness among the four groups (p = 0.03). Categorized based on diseases, VGB was effective in 28.6 % (2/7) and 50 % (4/8) in trisomy 21 and perinatal brain injury, respectively. ConclusionThe effectiveness of VGB in patients with IESS varied with etiology. Further investigations into the effectiveness of VGB in etiological subtypes of IESS could facilitate the development of tailored treatment algorithms for each etiology, representing valuable guidelines for future medical practice.

Evaluating cortical excitatory and inhibitory activity through interictal intracranial electroencephalography in mesial temporal lobe epilepsy.

Gamma oscillation regularity (GOR) indicates the synchronization of inhibitory interneurons, while the reactivity of cortico-cortical evoked potentials (CCEPs) is supposed to reflect local cortical excitability. Under the assumption that the early response of CCEP near the stimulation site also indicates excitatory activity primarily mediated by pyramidal cells, we aimed to visualize the cortical inhibitory and excitatory activities using GOR and CCEP in combination and to use them to predict the epileptogenic zone (EZ) in mesial temporal lobe epilepsy (MTLE). In five patients who underwent intracranial electrode implantation, GOR and CCEP reactivity in the vicinity of the stimulation site was quantified. The interictal GOR was calculated using multiscale entropy (MSE), the decrease of which was related to the enhanced GOR. These parameters were compared on an electrode-and-electrode basis, and spatially visualized on the brain surface. As a result, elevated GOR and CCEP reactivities, indicative of enhanced inhibitory and excitatory activities, were observed in the epileptogenic regions. Elevated CCEP reactivity was found to be localized to a restricted area centered on the seizure onset region, whereas GOR elevation was observed in a broader region surrounding it. Although these parameters independently predicted the EZ with high specificity, we combined the two to introduce a novel parameter, the excitatory and inhibitory (EI) index. The EI index predicted EZ with increased specificity compared with GOR or CCEP reactivity alone. Our results demonstrate that GOR and CCEP reactivity provided a quantitative visualization of the distribution of cortical inhibitory and excitatory activities and highlighted the relationship between the two parameters. The combination of GOR and CCEP reactivities are expected to serve as biomarkers for localizing the epileptogenic zone in MTLE from interictal intracranial electroencephalograms.

Total posterior quadrant disconnection for drug-resistant epilepsy in children.

To assess seizure outcomes, prognostic factors, and developmental changes in children undergoing total posterior quadrant disconnection (PQD) for drug-resistant epilepsy (DRE). We conducted a retrospective analysis of the clinical data of children with DRE who underwent total PQD surgery. The study focused on Engel's classification for seizure outcomes, exploring correlation of preoperative data and surgical effectiveness, and predictors of seizure prognosis. It involved a comparative analysis of developmental levels pre- and 3 months postoperatively using Griffiths Mental Development Scales-China (GMDS-C), and the correlation between clinical characteristics and GMDS-C results. Out of 61 pediatric patients, 70.5% showed no seizure recurrence postoperatively. In the univariate analysis, interictal electroencephalogram (EEG), magnetic resonance imaging (MRI), fluorodeoxyglucose positron emission tomography (FDG-PET), and acute postoperative seizure (APOS) were significantly related to surgical prognosis. In multivariate analysis, interictal EEG and APOS were identified as predictors of seizure prognosis. Survival analysis indicated significant associations between MRI, interictal EEG, FDG-PET, APOS and postoperative seizure occurrence. Preoperative GMDS-C levels were significantly correlated with epilepsy duration, seizure frequency, interictal EEG, and FDG-PET. GMDS-C scores improved postoperatively, while developmental quotients remained stable. For patients with structural abnormalities in the entire posterior quadrant, thorough preoperative assessment and timely total PQD surgery can effectively control seizures without causing neurological development deterioration. APOS and interictal EEG abnormalities beyond the posterior quadrant are predictors for seizure prognosis but should not be deemed contraindications for surgery. Due to lack of analysis on pediatric total PQD cases, 61 pediatric patients who underwent total PQD surgery were retrospectively enrolled. Seizure and development results were collected and analyzed as dependent variables. The study found that 70.5% of patients were seizure-free and showed development improvement, with no deaths or severe complications reported. Prognosis predictors included APOS and interictal EEG abnormalities beyond the posterior quadrant.

Acquired motor speech disorders in childhood epilepsy.

To evaluate a group of children with epilepsy and motor speech regression, with the aim of characterizing their speech disorders, electrographic features, and outcomes. Children referred to a tertiary developmental epilepsy clinic with epilepsy and motor speech regression were identified retrospectively. A clinical history was taken, and longitudinal speech and cognitive data were recorded. Speech samples were scored for severity and speech features. Seizure frequency and epileptiform discharges in the interictal electroencephalogram were analysed. Eighteen children (10 female) were evaluated, including seven with Landau-Kleffner syndrome and six with Rasmussen syndrome. Speech regression occurred at a mean age of 5 years (SD = 2 years 6 months), which was concurrent with seizure onset or peak seizure burden in eight children. Speech features included dysarthria (n = 13), phonological errors (n = 7), and dyspraxia (n = 6). Electrographic abnormalities occurred most frequently in the left centrotemporal and right frontal regions. Among children who were followed up, intelligibility of speech was affected in 13 at baseline and seven at follow-up (p = 0.03). Expressive language standardized scores increased from a mean (SD) of 50.0 (11.3) to 91.4 (27.8) in children with Landau-Kleffner syndrome (mean change = 41.4, 95% confidence interval [CI] 0.04-82.8, p = 0.0498) and decreased from 75.2 (15.3) to 59.0 (9.8) in children with Rasmussen syndrome (mean change -16.2, 95% CI -9.0 to -23.4, p = 0.002) over the follow-up. Motor speech disorders in epilepsy were severe, multifarious, and often fluctuated with seizure burden. Symptoms typically improved, especially in children with Landau-Kleffner syndrome, but rarely fully resolved.

Attention deficit hyperactivity disorder in children with epilepsy: a multicenter cross-sectional analysis in China.

The diagnosis and treatment of attention deficit hyperactivity disorder (ADHD) comorbid with epilepsy have been insufficiently addressed in China. We conducted a study in China to investigate the current status, diagnosis, and treatment of ADHD in children to further our understanding of ADHD comorbid with epilepsy, strengthen its management, and improve patients' quality of life. We carried out a multicenter cross-sectional survey of children with epilepsy across China between March2022 and August2022. We screened all patients for ADHD and compared various demographic and clinical factors between children with and without ADHD, including gender, age, age at epilepsy onset, duration of epilepsy, seizure types, seizure frequency, presence of epileptiform discharges, and treatment status. Our objective was to explore any possible associations between these characteristics and the prevalence of ADHD. Overall, 395 epilepsy patients aged 6-18years were enrolled. The age at seizure onset and duration of epilepsy ranged from 0.1-18 to 0.5-15years, respectively. Focal onset seizures were observed in 212 (53.6%) patients, while 293 (76.3%) patients had epileptiform interictal electroencephalogram (EEG) abnormalities. Among the 370 patients treated with anti-seizure medications, 200 (54.1%) had monotherapy. Although 189 (47.8%) patients had ADHD, only 31 received treatment for it, with the inattentive subtype being the most common. ADHD was more common in children undergoing polytherapy compared to those on monotherapy. Additionally, poor seizure control and the presence of epileptiform interictal EEG abnormalities may be associated with a higher prevalence of ADHD. While the prevalence of ADHD was higher in children with epilepsy than in normal children, the treatment rate was notably low. This highlights the need to give more importance to the diagnosis and treatment of ADHD in children with epilepsy.

Phenotypes in children with GNAO1 encephalopathy in China.

The GNAO1 gene encodes the α-subunit (Gαo) of the heterotrimeric guanine nucleotide-binding protein (G protein). The aim of this study was to explore the clinical characteristics of patients with GNAO1 pathogenic variations. Ten patients with pathogenic variations in GNAO1 were enrolled from the Shenzhen Children's Hospital. Clinical data from several cases previously reported from China were also included and analyzed. Twenty-seven patients with variations in GNAO1 were analyzed (10 patients from Shenzhen Children's Hospital, 17 patients from previously published studies) including 12 boys and 15 girls. The median age of onset was 3 months with moderate to severe global developmental delay. Nineteen different GNAO1 heterozygous variants were identified. Epilepsy was observed in 18 patients (67%, 18/27), movement disorder (MD) was observed in 22 patients (81%, 22/27), and both were seen in 13 patients (48%, 13/27). Seizures typically presented as focal seizures in all patients with epilepsy. MD typically presented as dystonia and chorea. Loss-of-function (LOF) or partial loss-of-function (PLOF) mutations were more frequent in patients with developmental and epileptic encephalopathy (p = 0.029). Interictal electroencephalograms showed multifocal or diffuse epileptiform discharges. The most common magnetic resonance imaging finding was widened extracerebral space. In contrast to MD, in which improvements were not common, seizures were easily controlled by anti-seizure medications. Severe dystonia in three patients was effectively treated by deep brain stimulation. Seven (26%, 7/27) patients died of respiratory complications, status dystonicus, choreoathetosis, or sudden unexpected death in epilepsy. We analyzed clinical data of 27 cases of GNAO1-related encephalopathy in China. MD seemed to be the central feature and was most difficult to control. LOF or PLOF variants were significantly associated with developmental and epileptic encephalopathy. The active intervention of severe dystonia may prevent death due to status dystonicus. However, future studies with larger samples are needed to confirm these results.

Outcomes of resective surgery in pediatric patients with drug-resistant epilepsy: A single-center study from the Eastern Mediterranean Region.

Epilepsy surgery is widely accepted as an effective therapeutic option for carefully selected patients with drug-resistant epilepsy (DRE). There is limited data on the outcome of epilepsy surgery, especially in pediatric patients from the Eastern Mediterranean region. Hence, we performed a retrospective study examining the outcomes of resective surgery in 53 pediatric patients with focal DRE. Patients with focal DRE who had undergone epilepsy surgery were included in the present study. All patients underwent a comprehensive presurgical evaluation. Postoperative seizure outcomes were classified using the Engel Epilepsy Surgery Outcome Scale. After surgery, 33 patients (62.2%) were Class I according to the Engel classification of surgical outcomes; eight patients (15.0%) were Class II, 11 (20.7%) were Class III, and one (1.8%) was Class IV. The relationships of presurgical, surgical, and postsurgical clinical variables to seizure outcomes were compared. Older age at seizure onset, older age at the time of surgery, the presence of focal to bilateral tonic-clonic seizures, seizure duration over 2 minutes, unsuccessful treatment with three or fewer antiseizure medications, lesions confined to one lobe (as demonstrated via magnetic resonance imaging [MRI]), surgical site in the temporal lobe, and histopathology including developmental tumors were significantly linked to an Engel Class I outcome. A univariate analysis of excellent surgical outcomes showed that lateralized semiology, localized interictal and ictal electroencephalogram (EEG) discharges, lateralized single-photon emission computed tomography and positron emission tomography findings, and temporal lobe resections were significantly related to excellent seizure outcomes. The results of our study are encouraging and similar to those found in other centers around the world. Epilepsy surgery remains an underutilized treatment for children with DRE and should be offered early.

Temporal stability of intracranial electroencephalographic abnormality maps for localizing epileptogenic tissue.

Identifying abnormalities on interictal intracranial electroencephalogram (iEEG), by comparing patient data to a normative map, has shown promise for the localization of epileptogenic tissue and prediction of outcome. The approach typically uses short interictal segments of approximately 1 min. However, the temporal stability of findings has not been established. Here, we generated a normative map of iEEG in nonpathological brain tissue from 249 patients. We computed regional band power abnormalities in a separate cohort of 39 patients for the duration of their monitoring period (.92-8.62 days of iEEG data, mean = 4.58 days per patient, >4800 hours recording). To assess the localizing value of band power abnormality, we computed -a measure of how different the surgically resected and spared tissue was in terms of band power abnormalities-over time. In each patient, the value was relatively consistent over time. The median of the entire recording period separated seizure-free (International League Against Epilepsy [ILAE] = 1) and not-seizure-free (ILAE 1) patients well (area under the curve [AUC] = .69). This effect was similar interictally (AUC = .69) and peri-ictally (AUC = .71). Our results suggest that band power abnormality D_RS, as a predictor of outcomes from epilepsy surgery, is a relatively robust metric over time. These findings add further support for abnormality mapping of neurophysiology data during presurgical evaluation.

Recognition and epileptology of protracted CLN3 disease.

This study was undertaken to analyze phenotypic features of a cohort of patients with protracted CLN3 disease to improve recognition of the disorder. We analyzed phenotypic data of 10 patients from six families with protracted CLN3 disease. Haplotype analysis was performed in three reportedly unrelated families. Visual impairment was the initial symptom, with onset at 5-9 years, similar to classic CLN3 disease. Mean time from onset of visual impairment to seizures was 12 years (range = 6-41 years). Various seizure types were reported, most commonly generalized tonic-clonic seizures; focal seizures were present in four patients. Progressive myoclonus epilepsy was not seen. Interictal electroencephalogram revealed mild background slowing and 2.5-3.5-Hz spontaneous generalized spike-wave discharges. Additional interictal focal epileptiform discharges were noted in some patients. Age at death for the three deceased patients was 31, 31, and 52 years. Molecular testing revealed five individuals were homozygous for c.461-280_677 + 382del966, the "common 1-kb" CLN3 deletion. The remaining individuals were compound heterozygous for various combinations of recurrent pathogenic CLN3 variants. Haplotype analysis demonstrated evidence of a common founder for the common 1-kb deletion. Dating analysis suggested the deletion arose approximately 1500 years ago and thus did not represent cryptic familial relationship in this Australian cohort. We highlight the protracted phenotype of a disease generally associated with death in adolescence, which is a combined focal and generalized epilepsy syndrome with progressive neurological deterioration. The disorder should be suspected in an adolescent or adult patient presenting with generalized or focal seizures preceded by progressive visual loss. The common 1-kb deletion has been typically associated with classic CLN3 disease, and the protracted phenotype has not previously been reported with this genotype. This suggests that modifying genetic factors may be important in determining this somewhat milder phenotype and identification of these factors should be the subject of future research.

West Syndrome – Infantile Spasms: A Rare Paediatric Case Report

West syndrome (WS) is a rare, severe form of epilepsy with onset in infancy and early childhood. It involves clustered epileptic spasm episodes, the aberrant interictal electroencephalogram pattern known as hypsarrhythmia, and neuropsychomotor delay. In this view, we present a case of a 6-year-old female child a known case of west syndrome, global developmental delay, hypothyroidism, and post-operative laparoscopic gastrotomy performed under general anaesthesia on March 31/2022, in view of recurrent aspiration and came with the chief complaints of seizure 6-7 episodes per day, decreased urine output, constipation for two days, straining to pass urine, and three episodes of vomiting. The history included that the baby cried immediately after birth, seizure on day 5 of life, and was shifted to the Neonatal intensive care unit (NICU), admitted there for 5 days because of respiratory distress syndrome and hyperalbuminemia. During their stay in the hospital, the child has been prescribed Lamotrigine 50mg, Levocarnitine 500mg, Levetiracetam 250mg, Clobazam 5mg, Phenobarbital 60mg, Sodium Valproate 100mg, Thyronorm 50mcg. Now the patient is symptomatically better. This case report intends to sensitize physicians in this region toward this rare neurological syndrome.
 Keywords: Hypsarrhythmia, Infantile, Seizure, West syndrome.

Infantile Spasms without Hypsarrhythmia and Paroxysmal Eye-Head Movements in an Infant with a Pyridoxine-Dependent Epilepsy due to PLPBP/PLPHP Deficiency.

To describe a new phenotype and the diagnostic workup of a vitamin-B6-dependent epilepsy due to pyridoxal 5'-phosphate-binding protein (PLPBP) deficiency in an infant with early-onset epilepsy at the age of 5 years 6 months. Following immediate and impressive clinical response to treatment with pyridoxine, metabolic screening for vitamin-B6-dependent epilepsies and targeted next-generation sequencing (NGS)-based gene panel analysis were performed. Potentially pathogenic variants were confirmed by Sanger sequencing in the patient, and variants were analyzed in both parents to confirm biallelic inheritance. The clinical phenotype and course of disease were compared to the 44 cases reported in the literature, harboring variants in pyridoxal phosphate homeostasis protein (PLPHP) and with cases of vitamin-B6-dependent epilepsy due to other known causative genes. Levels of alpha-aminoadipic semialdehyde in urine and amino acids were normal. Two inherited pathogenic variations in PLPHP were found in compound heterozygosity, including one novel deletion. We here describe a previously unreported individual harboring biallelic pathogenic PLPHP variants presenting with paroxysmal eye-head movements followed by epileptic spasms and an almost normal interictal electroencephalogram, thus expanding the clinical spectrum of PLPBP deficiency. This warrants consideration of vitamin-B6-dependent epilepsies in patients with early-onset epilepsy, including epileptic spasms, and eye movement disorders also beyond the neonatal period even when metabolic screening for vitamin-B6-dependent epilepsies is negative. PLPHP should be included systematically in NGS epilepsy gene panels.

Evaluation of the effectiveness of anticonvulsant and hormonal therapy for epileptic spasms in the group of Russian patients

Introduction. Epileptic spasms (ES) are a special type of epileptic seizures, accompanied by hypsarrhythmia on the interictal electroencephalogram (EEG) and regression of psychomotor development. ES are the most common cause of epileptic encephalopathy in infancy. Materials and methods. We examined two hundred three children with the age of onset of epileptic spasms up to 2 years. The patient were selected in retrospective group including 180 children (boys - 95/180 (52.7%), girls - 85/180 (47.3%)) (χ2=0.9, p=0.3428) and prospective group consisted of 23 children (boys - 13/23 (56.5%), girls - 10/23 (43.5%) (χ2 = 0.3478, p = 0.5553. Results. The present study demonstrates the need for sleep electroencephalogram in children with developmental delay or regression. Our work confirms in general, antiepileptic drugs, with the exception of vigabatrin in children with tuberous sclerosis-associated epilepsy, to show low efficacy in the group of children without tuberous sclerosis as seizures were stopped in 14.3% of cases. The use of methylprednisolone (the regimen includes pulse therapy followed by prolonged oral administration of the drug) makes it possible to achieve the cessation of seizures and the disappearance of hypsarrhythmia in 69.5% of patients. Methylprednisolone is a fairly safe and well tolerated hormone. The study did not record any life-threatening side effects among the prospective and retrospective groups.The treatment regimen using methylprednisolone shows equal efficacy with the regimen using tetracosactide. Conclusion. The results of the study demonstrate the need for early corticosteroid therapy in ES children.

Efficacy of vagus nerve stimulation for drug-resistant epilepsy in a survivor of poor-grade subarachnoid hemorrhage: A case report

A man in his 60s had undergone clipping for a subarachnoid hemorrhage (SAH) due to a ruptured anterior communicating artery aneurysm in his 40s. He survived cerebral vasospasms following surgery for poor-grade SAH and required anti-seizure medications (ASMs) for post-stroke epilepsy. More than 20 years after the clipping, he developed focal impaired awareness seizure, which decreased his quality of life (QOL). Computed tomography (CT) showed an infarct in the left frontal lobe. 123I-iomazenil single-photon emission CT showed decreased benzodiazepine accumulation in the left frontal and medial temporal lobes. An interictal electroencephalogram showed epileptic discharges in the left temporal region. We diagnosed focal epilepsy and adjusted ASMs based on seizure control. However, the seizures were not controlled, and the adverse effects worsened with dosage increase. The condition was diagnosed as drug-resistant epilepsy, and the patient underwent vagus nerve stimulation. Postoperatively, we confirmed control of seizures and improvement of QOL.

The Potential of Bemegride as an Activation Agent in Electroencephalography in Dogs.

The present study investigated the potential of bemegride as a pharmacological activation agent that elicits epileptiform discharges (EDs) in interictal electroencephalogram (EEG) recordings in dogs. Four laboratory dogs with idiopathic epilepsy and four without epilepsy were included. The dogs were anesthetized using sevoflurane during EEG recordings. Bemegride was administered intravenously and repeatedly until EDs were enhanced or induced, or until the maximum dose (20 mg/kg) had been administered. Bemegride activated EDs in all dogs with epilepsy. These EDs predominantly occurred in each dog's spontaneous irritative zones, which were identified without the administration of bemegride. EDs occurred after the administration of bemegride in 50% of dogs without epilepsy. The dose required for activation was significantly lower in dogs with epilepsy (median; 7.3 mg/kg) than in those without (median; 19.7 mg/kg) (p = 0.0294). The only suspected adverse effect associated with the administration of bemegride was vomiting in two dogs after awakening from anesthesia. There were no other adverse effects, including seizures. The present results demonstrated the potential of bemegride as a safe and effective pharmacological activation agent of EDs in anesthetized dogs with epilepsy and provided more options for the diagnosis and therapeutic planning of epilepsy, including presurgical evaluations, in dogs.

Clinical phenotype and genetic features of 16p11.2 microdeletion-related epilepsy in children

To study the clinical phenotype and genetic features of 16p11.2 microdeletion-related epilepsy in children. The medical data of 200 children with epilepsy who underwent a genetic analysis of epilepsy by the whole exon sequencing technology were collected retrospectively, of whom 9 children with epilepsy had 16p11.2 microdeletion. The clinical phenotype and genetic features of the 9 children with 16p11.2 microdeletion were analyzed. The detection rate of 16p11.2 microdeletion was 4.5% (9/200). The 9 children with 16p11.2 microdeletion were 3-10 months old. They experienced focal motor seizures with consciousness disturbance, and some of the seizures developed into generalized tonic-clonic seizures. The interictal electroencephalogram showed focal or multifocal epileptiform discharge, and all 9 children responded well to antiepileptic drugs. The 9 children had a 16p11.2 deletion fragment size of 398-906 kb, and the number of deleted genes was 23-33 which were all pathogenic mutations. The mutation was of maternal origin in 2 children, of paternal origin in 1 child, and de novo in the other children. 16p11.2 microdeletion can be detected in some children with epilepsy. Most of the 16p11.2 microdeletion is de novo mutation and large gene fragment deletion. The onset of 16p11.2 microdeletion-related epilepsy in children is mostly within 1 year of life, and the epilepsy is drug-responsive.

3D residual-attention-deep-network-based childhood epilepsy syndrome classification

Interictal electroencephalograms (EEGs) usually contain important information for epilepsy analysis and diagnosis. However, the focus of existing research has mainly been on epilepsy seizure onset detection, and only a few studies have been conducted on childhood epilepsy syndrome classification, which is usually more complicated than seizure detection. In this study, a novel 3D residual-attention-module-based deep network (AR3D) is developed to explore the spatial and time–frequency features of multichannel EEGs. The interictal EEGs of 37 patients with five typical childhood epilepsy syndromes, namely, benign childhood epilepsy with centrotemporal spikes, childhood absence epilepsy, febrile seizures plus, infantile spasms, unknown epilepsy syndrome, and one control group, are studied. The proposed AR3D algorithm, with a 97.03% F1 score, outperforms several state-of-the-art 2D and 3D convolution deep networks.