International League Against Epilepsy Research Articles

Solving the Etiology of Developmental and Epileptic Encephalopathy with Spike-Wave Activation in Sleep (D/EE-SWAS).

To understand the etiological landscape and phenotypic differences between 2 developmental and epileptic encephalopathy (DEE) syndromes: DEE with spike-wave activation in sleep (DEE-SWAS) and epileptic encephalopathy with spike-wave activation in sleep (EE-SWAS). All patients fulfilled International League Against Epilepsy (ILAE) DEE-SWAS or EE-SWAS criteria with a Core cohort (n = 91) drawn from our Epilepsy Genetics research program, together with 10 etiologically solved patients referred by collaborators in the Expanded cohort (n = 101). Detailed phenotyping and analysis of molecular genetic results were performed. We compared the phenotypic features of individuals with DEE-SWAS and EE-SWAS. Brain-specific gene co-expression analysis was performed for D/EE-SWAS genes. We identified the etiology in 42/91 (46%) patients in our Core cohort, including 29/44 (66%) with DEE-SWAS and 13/47 (28%) with EE-SWAS. A genetic etiology was identified in 31/91 (34%). D/EE-SWAS genes were highly co-expressed in brain, highlighting the importance of channelopathies and transcriptional regulators. Structural etiologies were found in 12/91 (13%) individuals. We identified 10 novel D/EE-SWAS genes with a range of functions: ATP1A2, CACNA1A, FOXP1, GRIN1, KCNMA1, KCNQ3, PPFIA3, PUF60, SETD1B, and ZBTB18, and 2 novel copy number variants, 17p11.2 duplication and 5q22 deletion. Although developmental regression patterns were similar in both syndromes, DEE-SWAS was associated with a longer duration of epilepsy and poorer intellectual outcome than EE-SWAS. DEE-SWAS and EE-SWAS have highly heterogeneous genetic and structural etiologies. Phenotypic analysis highlights valuable clinical differences between DEE-SWAS and EE-SWAS which inform clinical care and prognostic counseling. Our etiological findings pave the way for the development of precision therapies. ANN NEUROL 2024;96:932-943.

2023 IFCN & ILAE minimum recording standards for routine and sleep EEG. Applicability assessment in Russia

In 2023, the International Federation of Clinical Neurophysiology (IFCN) and the International League Against Epilepsy (ILAE) published the most up-to-date recommendations for conducting EEG examinations. The document sets out minimum standards necessary to improve the accuracy, efficiency, and reliability of routine and sleep EEG recordings. At the same time, it is indicated that based on availability specific rules and methods for conducting examinations should be proposed by medical organizations or departments independently. We present a selective translation of such recommendations and an assessment of their compliance with contemporary real-world opportunities in the Russian Federation. The possible “growth points” for the EEG technique in Russia are highlighted as well.

Terminology of medicines used to control seizure disorders: 2024 ILAE Recommendations and profile of real-world term use

Background. The International League Against Epilepsy (ILAE) has provided recommendations on the terminology of medicines for control of seizure disorders, which should be adapted into different languages.Objective: to evaluate the usage profile of terms describing the effects of medicines used in patients with epilepsy.Material and methods. The search query analysis systems Google Trends and Yandex Wordstat as well as PubMed/MEDLINE, Google Scholar and eLibrary databases were used to evaluate the general public and professional usage profile, respectively.The following terms in Russian and English were studied: “antiepileptic”, “antiepileptic drug”, “antiseizure”, “antiseizure drug”. In order to ascertain a prevailing opinion within professional and patient communities, a questionnaire survey was conducted on the public online resources of the non-profit partnership “The Association of Epileptologists and Patients” carried out via the cloud-based cross-platform social media as well as instant messaging service Telegram and the social media platform VK.Results. In the English-speaking internet segment, the term “antiepileptic” is used more than 5 times frequently than “antiseizure” by the general public. Conversely, in the Russian-language segment, the term “antiseizure” is almost 2.5 times more common. The term “antiepileptic” is more prevalent in scientific community in both segments. This finding aligns with the profile of terminology usage observed among epileptologists and epilepsy patients. The questionnaire survey results revealed that 56.42% of 179 respondents were in favor of the terms “antiepileptic drug”, “AED” and “antiepileptic therapy”, whereas as few as 17.32% of those endorsed the ILAE-proposed terminology.Conclusion. In light of the established practice of terminology use in both professional scientific community and among practicing epileptologists and epileptic patients, as well as the differences in the semantics of English- and Russian-language terms and the identified lack of precision in the ILAE definition of epilepsy, it can be concluded that for the Russian-speaking population, the terms “antiepileptic drug”, “AED” are more preferable rather than the terms “antiseizure medication”, “ASM”. Additionally, it is preferable to use the terms “convulsive ictus” and “epileptic ictus” due to negative connotation with term “seizure” in Russian. The terms “antiseizure” and “antiepileptic” should not be regarded as mutually exclusive in the context of treatment that has a direct impact on epilepsy course. Finally, it is necessary to further refine epilepsy definition and classification.

Predictors of drug-resistant epilepsy in childhood epilepsy syndromes: A subgroup analysis from a prospective cohort study.

Previous studies assessing factors associated with drug-resistant epilepsy (DRE) were constrained by their amalgamation of all epilepsy syndromes in their analyses and the absence of uniform criteria for defining DRE. Our objective was to identify predictors of DRE among the four primary childhood epilepsy syndrome groups within a cohort of children with new onset seizures, using the International League Against Epilepsy (ILAE) definition of DRE and the recent classification of epilepsies. This is a prospective study of 676 children with new onset seizures initiated on antiseizure medication. Patients were monitored for the occurrence of DRE according to the ILAE criteria and were categorized into one of four epilepsy groups: self-limited focal epilepsies (SeLFEs), genetic generalized epilepsies (GGEs), developmental epileptic encephalopathies (DEEs), and focal epilepsies. Cox regression analysis was performed to identify predictors of DRE within each epilepsy group. Overall, 29.3% of children were classified as having DRE, with the highest incidence observed among children diagnosed with DEEs (77.7%), followed by focal epilepsies (31.5%). Across the entire cohort, predictors of DRE included the presence of an epileptogenic lesion, a higher pretreatment number of seizures, experiencing multiple seizure types, presence and severity of intellectual and developmental delay, myoclonus, and younger age at epilepsy onset. Within the GGEs, only a younger age at seizure onset and experiencing multiple seizure types predicted DRE. Among focal epilepsies, predictors of DRE included the presence of an epileptogenic lesion, experiencing multiple seizure types, and having a greater number of pretreatment seizures. Within the DEEs, predictors of DRE were the occurrence of tonic seizures. Predictors of DRE within SeLFEs could not be identified. This study indicates that different epilepsy syndromes are associated with distinct predictors of drug resistance. Anticipation of drug resistance within various groups is feasible using accessible clinical variables throughout the disease course.

Consensus-based recommendations for the diagnosis and treatment of anxiety and depression in children and adolescents with epilepsy: A report from the Psychiatric Pediatric Issues Task Force of the International League Against Epilepsy.

The Psychiatric Pediatric Issues Task Force of the International League Against Epilepsy (ILAE) aimed to develop recommendations for the diagnosis and treatment of anxiety and depression in children and adolescents with epilepsy. The Task Force conducted a systematic review and identified two studies that assessed the accuracy of four screening measures for depression and anxiety symptoms compared with a psychiatric interview. Nine studies met the eligibility criteria for treatment of anxiety and depressive disorders or symptoms. The risk of bias and certainty of evidence were assessed. The evidence generated by this review followed by consensus where evidence was missing generated 47 recommendations. Those with a high level of agreement (≥80%) are summarized. Diagnosis: (1) Universal screening for anxiety and depression is recommended. Closer surveillance is recommended for children after 12 years, at higher risk (e.g., suicide-related behavior), with subthreshold symptoms, and experiencing seizure worsening or therapeutic modifications. (2) Multiple sources of ascertainment and a formal screening are recommended. Clinical interviews are recommended whenever possible. The healthcare provider must always explain that symptom recognition is essential to optimize treatment outcomes and reduce morbidity. (3) Questioning about the relationship between symptoms of anxiety or depression with seizure worsening/control and behavioral adverse effects of antiseizure medications is recommended. Treatment: (1) An individualized treatment plan is recommended. (2) For mild depression, active monitoring must be considered. (3) Referral to a mental health care provider must be considered for moderate to severe depression and anxiety. (4) Clinical care pathways must be developed. (5) Psychosocial interventions must be tailored and age-appropriate. (6) Healthcare providers must monitor children with epilepsy who are prescribed antidepressants, considering symptoms and functioning that may not improve simultaneously. (7) Caregiver education is essential to ensure treatment adherence. (8) A shared-care model involving all healthcare providers is recommended for children and adolescents with epilepsy and mental health disorders. We identified clinical decisions in the management of depression and anxiety that lack solid evidence and provide consensus-based guidance to address the care of children and adolescents with epilepsy.

An algorithm for drug-resistant epilepsy in Danish national registers.

Patients with drug-resistant epilepsy (DRE) have increased risks of premature death, injuries, psychosocial dysfunction, and a reduced quality of life. Identification of persons with DRE in administrative data can allow for effective large-scale research, and we therefore aimed to construct an algorithm for identification of DRE in Danish nation-wide health registers. We used a previously generated sample of 525 persons with medical record-validated incident epilepsy between 2010-2019, of which 80 (15%) fulfilled International League Against Epilepsy (ILAE) criteria of DRE at the time of the latest contact - this cohort was considered the gold standard. We linked information in the validated cohort to Danish national health registers and constructed register-based algorithms for identification of DRE-cases. The accuracy of each algorithm was validated against the medical record-validated gold standard. We applied the best performing algorithm according to test accuracy (F1 score) to a large cohort with incident epilepsy identified in the Danish National Patient Registry between 1995 and 2013 and performed descriptive and logistic regression analyses to characterize the cohort with DRE as identified by the algorithm. The best performing algorithm in terms of F1 score was defined as 'fillings of prescriptions for ≥ 3 distinct antiseizure medications (ASMs) within 3 years or acute hospital visit with epilepsy/convulsions following fillings of prescriptions for two distinct ASMs' (sensitivity 0.59, specificity 0.93, positive predictive value 0.59, negative predictive value 0.92, area under the receiver operating characteristic curve 0.77, and F1 score 0.595). Applying the algorithm to a register-based cohort of 83,682 individuals with incident epilepsy yielded 8,650 cases (10.3 %) with DRE. In multivariable logistic regression analysis, early onset of epilepsy, focal or generalized epilepsy, somatic co-morbidity, and substance abuse, were independently associated with risk of being classified with DRE. We developed an algorithm for the identification of DRE in Danish national registers, which can be applied for a variety of research questions. We identified early onset of epilepsy, focal or generalized epilepsy, somatic co-morbidity, and substance abuse as risk factors for DRE.

The Spectrum of Self-Limited Infantile Epilepsy Syndromes

AbstractInfantile epilepsy syndromes' nomenclature has changed over time. The International League Against Epilepsy (ILAE) revised its 2021 classification and definition of epilepsy syndromes in neonates and infants, replacing the term “benign” with “self-limited,” and now identifies them as “self-limited infantile epilepsy” (SeLIE). SeLIE is characterized by seizures that begin during infancy and resolve spontaneously with normal developmental progress. The recognition of infantile seizures with favorable outcomes dates back more than 60 years, as noted by Fukuyama in Japan. Thirty years later, Watanabe et al reported benign focal seizures in infancy, with the majority of cases being nonfamilial. These seizures' self-limited nature during infancy has since been acknowledged in various countries, spanning diverse ethnic populations beyond Japan. Infants who undergo such seizures are now recognized as having self-limited nonfamilial infantile epilepsy (SeLNFIE). Initially, Vigevano et al detailed the familial variant in five infants, coining the term “benign familial infantile seizures” to characterize this condition, now known as self-limited familial infantile epilepsy (SeLFIE). SeLNFIE and SeLFIE may present similarly with the exception of a positive family history. After the initial description and classification of these syndromes (familial and nonfamilial) in the ILAE's 1989 Classification of Epilepsies and Epileptic Syndromes, several less frequently encountered related syndromes have been recognized. These conditions comprise a spectrum including SeLFIE with choreoathetosis and paroxysmal dyskinesia, now termed infantile convulsions with paroxysmal choreoathetosis syndrome (ICCA); self-limited focal epilepsy in infancy with midline spikes and waves during sleep (SeLIMSE); self-limited infantile seizures with mild gastroenteritis (SeLISwG); SeLFIE associated with familial hemiplegic migraine (FHM); and self-limited familial neonatal-infantile epilepsy (SeLFNIE). This review aims to document the prevalence of these SeLIEs, elucidate their unique characteristics, and underscore their self-limited nature.

Causal association between epilepsy and its DNA methylation profile and atrial fibrillation

BackgroundThe "epileptic heart" concept is emerging, but the causal relationship between epilepsy and atrial fibrillation (AF) remains unclarified. ObjectiveThis study explores the genetic correlations and bidirectional causality between various epilepsy phenotypes and AF. MethodsGenome-wide association study (GWAS) statistics for 10 epilepsy subtypes (29,944 cases, 52,538 controls) and AF (60,620 cases, 970,216 controls) were sourced from the International League Against Epilepsy (ILAE) and HGRI-EBI Catalog-GWAS, respectively. Linkage disequilibrium score regression (LDSC) and genome-wide Mendelian Randomization (MR) evaluated genetic correlations and bidirectional causal relationships. Epilepsy-related DNA methylation data (N= ∼800) from EWAS catalog were analyzed to identify causal CpG sites influencing AF risk through epigenetic MR. ResultsLDSC revealed significant genetic correlations between four epilepsy subtypes and AF (rg from 0.116 to 0.241). Forward MR suggested a significant causal effect of focal epilepsy with hippocampal sclerosis (FE with HS) on AF risk (IVW and MR-PRESSO: OR = 1.046, P ≤ 0.004), with results robust against heterogeneity, horizontal pleiotropy, and outliers. Epigenetic MR indicated that lower methylation at cg06222062 (OR = 0.994, P = 3.16E-04) mapped to PLA2G5 and cg08461451 mapped to SPPL2B gene (OR = 0.954, P = 1.19E-03), and higher cg10541930 in the C10orf143 promoter (OR = 1.043, P = 4.18E-22) increases AF risk. Sensitivity analyses affirmed no pleiotropic bias. ConclusionFE with HS significantly increases AF risk, highlighting the natural neural-cardiac connection and the need for cardiac monitoring in epilepsy patients. Specific methylated CpG sites may serve as biomarkers and preventive targets for AF susceptibility.

Seizures in small brains

Neonatal seizures are the most common neurological emergency in newborns; often signalling significant neurological dysfunction. Their pathophysiology is complex and relates to the imbalance between excitatory and inhibitory neurotransmitters in the immature brain. During the neonatal period, excitatory circuits mediated via neuro-transmitters like glutamate predominate while inhibitory circuits mediated via gamma-aminobutyric acid (GABA) signalling are underdeveloped. Additionally, immature ion channels, such as sodium, chloride, potassium, and calcium, increase neuronal excitability. The most common aetiology of seizures is attributed to neonatal hypoxic cerebral injury. However, metabolic vulnerabilities, such as hypoglycaemia and electrolyte imbalances, can also precipitate seizures. Identifying the underlying cause, which in a large majority is acute symptomatic, is critical for prompt treatment. Early intervention is essential to prevent long-term neurodevelopmental consequences.Diagnosing neonatal seizures is challenging due to their subtle presentations. Distinguishing them from non-seizure events is crucial. Accurate diagnosis is essential for appropriate treatment and management. Continuous electroencephalography (cEEG) is the gold standard for diagnosis, but an amplitude-integrated EEG (aEEG) is a useful alternative tool in neonatal intensive care units (NICUs), for high-risk infants. This facilitates diagnosis of electroclinical as well as electrical seizures; commonly encountered in the very sick newborns and those born prematurely. Neuroimaging, like magnetic resonance imaging (MRI) and computed tomography (CT) is required to identify structural causes, while laboratory and genetic tests check for infectious, metabolic and genetic aetiologies. The new classification proposed by the International League Against Epilepsy (ILAE), underscores the importance of recognition of electrical seizures and highlights the value of recognising the seizure semiology for the diagnosis of the underlying aetiology.

Prescription patterns and therapeutic gaps among persons with epilepsy in Southwestern Nigeria.

Introduction: Pharmacotherapy with antiseizure medications (ASMs) has been a cornerstone for achieving long-term remissions in persons with epilepsy (PWEs). This study aims to determine the prescription patterns and treatment gaps (TGs) among PWEs. Methods: Accordingly, a descriptive cross-sectional study was conducted with 940 PWEs aged ≥18years having clinically confirmed diagnosis of epilepsy based on the International League Against Epilepsy (ILAE) criteria. At a scheduled interview with each participant, a previously established questionnaire was used to obtain clinical information relating to epilepsy in terms of the age of onset, etiology, duration of epilepsy, frequency, types, and number of ASMs used. Results: There were fewer male participants [445 (47.4%) vs. 495 (53.6%)] than females, with a higher mean age of onset [(35.19 ± 21.10 vs. 31.58 ± 20.82years; p = 0.009]. The medication characteristics showed that 336 (35.7%) of the 940 PWEs recruited were not on any ASMs, whereas the remaining 604 (64.3%) patients were on ASMs, with 504 (83.4%) on monotherapy vs. 100 (16.6%) on polytherapy. The PWEs on ASM monotherapy had a higher mean age [40.92 ± 19.40 vs. 33.61 ± 16.51years; p < 0.001] and higher mean age of onset [34.47 ± 21.80 vs. 25.39 ± 19.78years; p < 0.001] than those on polytherapy. Furthermore, there were more persons on ASM monotherapy among the participants with seizure duration < 2years [251 (87.5%) vs. 36 (12.5%)] and seizure duration > 2years [253 (79.8%) vs 64 (20.2%)]. Conclusion: The majority of the participants receiving ASMs were on monotherapy, with carbamazepine being the most frequently prescribed medication. Furthermore, about a third of the participants had TGs; therefore, healthcare providers should focus on alleviating the TGs among PWEs.

Long-term outcomes and adaptive behavior in adult patients with Lennox-Gastaut syndrome.

Lennox-Gastaut syndrome (LGS) is a severe form of epilepsy characterized by difficult-to-control seizures and cognitive dysfunction. Previous studies mainly focused on pediatric populations, and little is known about the long-term cognitive outcome in adult patients with LGS. The objective of this study was to investigate the long-term functional and adaptive behavior in adult patients with LGS. This cross-sectional study enrolled adult patients diagnosed with LGS according to the recently published International League Against Epilepsy (ILAE) diagnostic criteria. The adaptive behavior of participants was assessed using the Vineland Adaptive Behavior Scales, Survey Interview, Second Edition (VABS-II). Demographic, clinical, electroencephalography (EEG), and antiseizure medication (ASM) data were also collected at different timepoints, to investigate their association with VABS-II scores. The study included 38 adult patients with LGS. A low score on the Adaptive Behavior Composite Scale was found in all patients. When considering single VABS-II domains, particularly low scores were found in daily living skills and socialization, whereas slightly higher performances were observed in communication. An earlier age at LGS diagnosis was identified as the most significant predictor of worse adaptive outcomes in adult life. At the time of study evaluation, high seizure frequency, higher EEG background slowing, and multifocal EEG epileptiform abnormalities were significantly associated with lower VABS-II raw scores. Furthermore, in an exploratory correlation analysis with ASM regimen at the study visit, treatment with cannabidiol was associated with higher adaptive behavior scores, whereas benzodiazepine intake correlated with lower scores. This study provides relevant insights into the long-term challenges faced by adults with Lennox-Gastaut syndrome (LGS), highlighting significant impairments in adaptive behavior as well as the associated clinical and electroencephalography features. Additionally, this study provides a more specific neuropsychological profile in adults with LGS and underscores the importance of comprehensive care approaches that go beyond seizure control in this population. This study examined adults with Lennox-Gastaut syndrome (LGS), a severe type of epilepsy, to understand their long-term abilities to perform daily tasks and adapt socially. We found that these adults have significant difficulties with daily living and social skills, although not all areas were equally affected. They performed somewhat better in communication, particularly in understanding others (receptive communication). Importantly, the younger the age at which LGS was diagnosed, the worse their outcomes were as adults. This study highlights the need for research and treatment approaches that focus not only on controlling seizures but also on improving daily life skills.

Advances in MRI-based diagnosis of temporal lobe epilepsy: Correlating hippocampal subfield volumes with histopathology.

Epilepsy, affecting 0.5%-1% of the global population, presents a significant challenge with 30% of patients resistant to medical treatment. Temporal lobe epilepsy, a common cause of medically refractory epilepsy, is often caused by hippocampal sclerosis (HS). HS can be divided further by subtype, as defined by the International League Against Epilepsy (ILAE). Type 1 HS, the most prevalent form (60%-80% of all cases), is characterized by cell loss and gliosis predominantly in the subfields cornu ammonis (CA1) and CA4. Type 2 HS features cell loss and gliosis primarily in the CA1 sector, and type 3 HS features cell loss and gliosis predominantly in the CA4 subfield. This literature review evaluates studies on hippocampal subfields, exploring whether observable atrophy patterns from in vivo and ex vivo magnetic resonance imaging (MRI) scans correlate with histopathological examinations with manual or automated segmentation techniques. Our findings suggest only ex vivo 1.5Tesla (T) or 3T MRI with manual segmentation or in vivo 7T MRI with manual or automated segmentations can consistently correlate subfield patterns with histopathologically derived ILAE-HS subtypes. In conclusion, manual and automated segmentation methods offer advantages and limitations in diagnosing ILAE-HS subtypes, with ongoing research crucial for refining hippocampal subfield segmentation techniques and enhancing clinical applicability.

Evaluating the Efficacy of Vagus Nerve Stimulation across 'Minor' and 'Major' Seizure Types: A Retrospective Analysis of Clinical Outcomes in Pharmacoresistant Epilepsy.

Background: Evaluating the differential impact of vagus nerve stimulation (VNS) therapy across various seizure types, our study explores its efficacy specifically in patients with categorized minor and major seizures. Methods: We conducted a retrospective cohort study involving 76 patients with pharmacoresistant epilepsy treated at the University Emergency Hospital of Bucharest between 2021 and 2024. Seizures were classified as 'minor' (including focal-aware and non-motor/absence seizures) and 'major' (including focal to bilateral tonic-clonic and generalized motor seizures), based on modified International League Against Epilepsy (ILAE) criteria. This classification allowed us to assess the response to VNS therapy, defined by a 50% or greater reduction in seizure frequency at the 12-month follow-up. Results: Our findings reveal that major seizures respond more favorably to VNS therapy, significantly reducing both frequency and intensity. In contrast, minor seizures showed a less pronounced response in frequency reduction but noted improvements in neurocognitive functions, suggesting a nuanced benefit of VNS in these cases. Conclusion: The study underscores the importance of seizure type in determining the efficacy of VNS therapy, advocating for personalized treatment approaches based on seizure classification. This approach could potentially enhance clinical outcomes by tailoring VNS settings to specific seizure types, improving overall management strategies in pharmacoresistant epilepsy.

The international league against epilepsy primary healthcare educational curriculum: Assessment of educational needs.

To assess the need for an epilepsy educational curriculum for primary healthcare providers formulated by the International League Against Epilepsy (ILAE) and the importance attributed to its competencies by epilepsy specialists and primary care providers and across country-income settings. The ILAE primary care epilepsy curriculum was translated to five languages. A structured questionnaire assessing the importance of its 26 curricular competencies was posted online and publicized widely to an international community. Respondents included epilepsy specialists, primary care providers, and others from three World Bank country-income categories. Responses from different groups were compared with univariate and ordinal logistic regression analyses. Of 785 respondents, 60% noted that a primary care epilepsy curriculum did not exist or they were unaware of one in their country. Median ranks of importance for all competencies were high (very important to extremely important) in the entire sample and across different groups. Fewer primary care providers than specialists rated the following competencies as extremely important: definition of epilepsy (p = .03), recognition of seizure mimics (p = .02), interpretation of test results for epilepsy care (p = .001), identification of drug-resistant epilepsy (0.005) and management of psychiatric comorbidities (0.05). Likewise, fewer respondents from LMICs in comparison to UMICs rated 15 competencies as extremely important. The survey underscores the unmet need for an epilepsy curriculum in primary care and the relevance of its competencies across different vocational and socioeconomic settings. Differences across vocational and country income groups indicate that educational packages should be developed and adapted to needs in different settings.

New Pharmacological Therapies in the Treatment of Epilepsy in the Pediatric Population.

Epilepsy is a disorder characterized by abnormal brain neuron activity, predisposing individuals to seizures. The International League Against Epilepsy (ILAE) categorizes epilepsy into the following groups: focal, generalized, generalized and focal, and unknown. Infants are the most vulnerable pediatric group to the condition, with the cause of epilepsy development being attributed to congenital brain developmental defects, white matter damage, intraventricular hemorrhage, perinatal hypoxic-ischemic injury, perinatal stroke, or genetic factors such as mutations in the Sodium Channel Protein Type 1 Subunit Alpha (SCN1A) gene. Due to the risks associated with this condition, we have investigated how the latest pharmacological treatments for epilepsy in children impact the reduction or complete elimination of seizures. We reviewed literature from 2018 to 2024, focusing on the age group from 1 month to 18 years old, with some studies including this age group as well as older individuals. The significance of this review is to present and compile research findings on the latest antiseizure drugs (ASDs), their effectiveness, dosing, and adverse effects in the pediatric population, which can contribute to selecting the best drug for a particular patient. The medications described in this review have shown significant efficacy and safety in the studied patient group, outweighing the observed adverse effects. The main aim of this review is to provide a comprehensive summary of the current state of knowledge regarding the newest pharmacotherapy for childhood epilepsy.

Benign convulsions with mild gastroenteritis in children: An emerging acute symptomatic seizures

AbstractBenign convulsions with mild gastroenteritis (CwG) characterized by afebrile seizures that occurred in the acute period of mild gastroenteritis often occur in infancy and toddlers. Until now, it has not been well acknowledged and thereby classified by the International League against Epilepsy (ILAE) as epilepsy syndrome or acute symptomatic seizures (ASS). Thus far, accumulating data suggest that CwG could fit all the mandatory criteria of ASS from the aspects of pathogenesis, clinical manifestations, and outcomes, rather than epilepsy in spite of the rare cases developing epilepsy over time. This review provides a comprehensive picture of this entity aiming to facilitate the pediatricians, particularly for general practitioners, to better recognize this unique entity and, ultimately, to minimize unnecessary evaluation and treatment.

Updates from the International League Against Epilepsy Classification of Epilepsy (2017) and Focal Cortical Dysplasias (2022): Imaging Phenotype and Genetic Characterization.

The International League Against Epilepsy (ILAE) is an organization of 120 national chapters providing the most widely accepted and updated guidelines on epilepsy. In 2022, the ILAE Task Force revised the prior (2011) classification of focal cortical dysplasias to incorporate and update clinicopathologic and genetic information, with the aim to provide an objective classification scheme. New molecular-genetic information has led to the concept of "integrated diagnosis" on the same lines as brain tumors, with a multilayered diagnostic model providing a phenotype-genotype integration. Major changes in the new update were made to type II focal cortical dysplasias, apart from identification of new entities, such as mild malformations of cortical development and cortical malformation with oligodendroglial hyperplasia. No major changes were made to type I and III focal cortical dysplasias, given the lack of significant new genetic information. This review provides the latest update on changes to the classification of focal cortical dysplasias with discussion about the new entities. The ILAE in 2017 updated the classification of seizure and epilepsy with 3 levels of diagnosis, including seizure type, epilepsy type, and epilepsy syndrome, which are also briefly discussed here.

Assessing the Relationship between Surgical Timing and Postoperative Seizure Outcomes in Cavernoma-Related Epilepsy: A Single-Institution Retrospective Analysis of 63 Patients with a Review of the Literature.

Background: Patients with supratentorial cavernous malformations (SCMs) commonly present with seizures. First-line treatments for cavernoma-related epilepsy (CRE) include conservative management (antiepileptic drugs (AEDs)) and surgery. We compared seizure outcomes of CRE patients after early (≤6 months) vs. delayed (>6 months) surgery. Methods: We compared outcomes of CRE patients with SCMs surgically treated at our large-volume cerebrovascular center (1 January 2010-31 July 2020). Patients with 1 sporadic SCM and ≥1-year follow-up were included. Primary outcomes were International League Against Epilepsy (ILAE) class 1 seizure freedom and AED independence. Results: Of 63 CRE patients (26 women, 37 men; mean ± SD age, 36.1 ± 14.6 years), 48 (76%) vs. 15 (24%) underwent early (mean ± SD, 2.1 ± 1.7 months) vs. delayed (mean ± SD, 6.2 ± 7.1 years) surgery. Most (32 (67%)) with early surgery presented after 1 seizure; all with delayed surgery had ≥2 seizures. Seven (47%) with delayed surgery had drug-resistant epilepsy. At follow-up (mean ± SD, 5.4 ± 3.3 years), CRE patients with early surgery were more likely to have ILAE class 1 seizure freedom and AED independence than those with delayed surgery (92% (44/48) vs. 53% (8/15), p = 0.002; and 65% (31/48) vs. 33% (5/15), p = 0.03, respectively). Conclusions: Early CRE surgery demonstrated better seizure outcomes than delayed surgery. Multicenter prospective studies are needed to validate these findings.

Advances of pathological research and classification in malformations of cortical development associated with refractory epilepsy

With rapid development of genetic testing techniques, neuroimaging and neuroelectrophysiological technologies, our understanding of malformations of cortical development continues to be deepened and updated. In particular, mutations in genes related to the mammalian target of rapamycin (mTOR) signaling pathway have been successively discovered in focal cortical dysplasia (FCD). At the same time, the classification consensus on FCD issued by the International League Against Epilepsy (ILAE) in 2011 has encountered problems and challenges in diagnostic practice. Therefore, in 2022, ILAE proposed an updated version of the FCD classification based on the progress in molecular genetics over the past decade. The main addition to the classification system is "white matter lesions, " and it is also suggested to integrate histopathological, neuroimaging, and molecular testing results for multi-level integrated diagnosis to achieve reliable, clinically relevant, and therapeutic targeted final diagnosis.

The online educational tool "Roadmap to EEGs" significantly improved trainee performance in recognizing EEG patterns.

We created a framework to assess the competency-based EEG curriculum, outlined by the International League Against Epilepsy (ILAE) through a video-based online educational resource ("Roadmap to EEGs") and assessed its effectiveness and feasibility in improving trainees' knowledge. Ten video-based e-learning modules addressed seven key topics in EEG and epileptology (normal EEG, normal variants, EEG artifacts, interictal epileptiform discharges (IED), focal seizures, idiopathic generalized epilepsy (IGE), and developmental and epileptic encephalopathies (DEE)). We posted the educational videos on YouTube for free access. Pre- and post-tests, each comprising 20 multiple-choice questions, were distributed to institution leadership and advertised on social media platforms to reach a global audience. The tests were administered online to assess the participants' knowledge. Pre- and post-test questions showed different EEG samples to avoid memorization and immediate recall. After completing the post-test, participants were asked to respond to 7 additional questions assessing their confidence levels and recommendations for improvement. A total of 52 complete and matched pre- and post-test responses were collected. The probability of a correct response was 73% before teaching (95% CI: 70%-77%) and 81% after teaching (95% CI: 78%-84%). The odds of a correct response increased significantly by 59% (95% CI: 28%-98%, p < .001). For participants having >4 weeks of EEG training, the probability of a correct response was 76% (95% CI: .72-.79) and 81% after teaching (95% CI: .78-.84). The odds of answering correctly increased by 44% (95% CI: 15%-80%, p = .001). Participants felt completely confident in independently interpreting and identifying EEG findings after completing the teaching modules (17.1% before vs. 37.8% after, p-value < .0001). 86.5% of participants expressed a high likelihood of recommending the module to other trainees. The video-based online educational resource allows participants to acquire foundational knowledge in EEG/epilepsy, and participants to review previously learned EEG/epilepsy information.