Search Results (211)

Hypoxic–ischaemic encephalopathy (HIE), a leading cause of perinatal mortality and neurological impairment, affects 1–8/1000 live births in developed countries. Therapeutic hypothermia (TH), the standard treatment for moderate to severe HIE, reduces brain injury by lowering metabolic demand and inhibiting apoptosis. This case study presents a full-term female newborn delivered via caesarean section due to intrauterine asphyxia, with meconium aspiration syndrome and severe HIE (Apgar 0/0/0/2). Notwithstanding the presence of multiorgan failure and grade II intraventricular haemorrhage, TH was initiated within six hours. The patient received circulatory and respiratory support, sedation, and nitric oxide. Early rehabilitation was initiated immediately. Neurofunctional assessment using the TIMP test revealed initial delays (16–25th percentile) at 11 weeks of age; however, the subsequent two evaluations, conducted approximately every two weeks, indicated that the patient was within normal developmental ranges. A similar outcome was observed in the AIMS assessment conducted at seven months of age, which also yielded normal results. Despite MRI findings post-TH showing hypoxic and haemorrhagic lesions, the patient achieved normal development. This case demonstrates the effectiveness of combining TH with early physiotherapy in mitigating severe consequences of HIE, such as cerebral palsy and epilepsy. Long-term follow-up remains crucial for detecting later deficits, particularly during school age. The outcome of this case underscores the significance of timely intervention and multidisciplinary care. While TH and rehabilitation have been shown to improve prognosis, ongoing monitoring is crucial to ensure optimal neurological development trajectories. Full article

Background: Refractory epilepsy refers to a type of epilepsy in which anti-epileptic medications do not yield results, necessitating alternative medical interventions. The ketogenic diet (KD) is utilized as a non-pharmacologic treatment for refractory epilepsy. This study aims to assess details regarding caregivers’ awareness and practices, challenges faced, and other details regarding the KD as a treatment option for children with refractory epilepsy. Methods: A cross-sectional study included 15 caregivers of children with refractory epilepsy who adhere to the KD. Data were collected using an online, self-administered questionnaire including socio-demographic characteristics and descriptive aspects of KD on 15 caregivers. Results: Caregivers’ awareness scores varied, over half of the caregivers (53.3%) demonstrated low awareness levels, scoring below the midpoint on the awareness scale, while both caregivers’ adherence (t value = 31.5; p < 0.001) and patients’ adherence levels (t value = 26.1; p < 0.001) significantly exceeded the minimum expected thresholds, indicating effective implementation of the diet protocols. Challenges faced by caregivers were categorized as follow: logistical challenges (e.g., issues related to KD availability, cost, and weighing (15.6%)), behavioral challenges (e.g., avoiding prohibited foods (18.8%), feelings of hunger, the social environment, and never eating without caregivers), and medical challenges (e.g., frequent blood glucose monitoring (25%)). The most common side effects of the KD were gastrointestinal symptoms, including constipation (33.3%) and gastroesophageal reflux disease (GERD). Physical symptoms such as dyslipidemia were also reported by 13.3% of participants. In terms of neurological and behavioral improvements, the three most reduced symptoms following KD adoption were hyperactive attention deficit (25%), tremors (20%), and insomnia (20%). Conclusions: This study reveals a significant gap between high adherence and low awareness of the KD among caregivers of children with refractory epilepsy. While adherence is crucial for the success of KD, this discrepancy highlights the need for comprehensive caregiver education that not only focuses on practical implementation but also on the underlying rationale of the diet. This study underscores the importance of multidisciplinary support, by including dietitians, to bridge the knowledge gap. These findings suggest that dietary education programs should aim to improve caregivers’ understanding, ultimately enhancing adherence and long-term outcomes. Future research should explore the psychosocial factors influencing adherence and the role of trust in healthcare professionals in shaping caregiver behaviors. The findings also call for further exploration into the impact of caregiver education on child health outcomes in the long term. The varied knowledge among caregivers indicates a need for further educational interventions or increased visits from dietitians. Strategies need to be framed to overcome the challenges faced and improve adherence. Full article

This study investigates the feasibility of using a two-channel subcutaneous EEG device (SubQ) to detect and monitor PGES. The SubQ device, developed by UNEEG Medical A/S, offers a minimally invasive alternative to scalp EEG, enabling ultra-long-term monitoring and remote data analysis. We used annotated scalp EEG data and data from the SubQ device. The pre-processing pipeline included channel reduction, resampling, filtering, and feature extraction. A Variational Auto-Encoder (VAE) was employed for anomaly detection, trained to identify PGES instances, and post-processing was applied to predict their duration. The VAE achieved a 100% detection rate for PGES in both scalp and SubQ datasets. However, the predicted durations had an average offset of 35.67 s for scalp EEG and 26.42 s for SubQ data. The model’s false positive rate (FPR) was 59% for scalp EEG and 56% for SubQ data, indicating a need for further refinement to reduce false alarms. This study demonstrates the potential of subcutaneous EEG as a valuable tool in the study of epilepsy and the monitoring of PGES, ultimately contributing to a better understanding and management of SUDEP risk. Full article

Epilepsy remains a major therapeutic challenge, with approximately one-third of patients experiencing drug-resistant epilepsy (DRE) despite the availability of multiple antiseizure medications (ASMs). This review aims to evaluate emerging ASMs—cenobamate, fenfluramine, ganaxolone, ezogabine (retigabine), and perampanel—with a focus on their mechanisms of action, pharmacological profiles, and potential role in precision medicine. A comprehensive literature search was conducted using PubMed, Scopus, and Web of Science to identify preclinical and clinical studies evaluating the pharmacodynamics, pharmacokinetics, efficacy, and safety of the selected ASMs. Relevant trials, reviews, and mechanistic studies were reviewed to synthesize the current understanding of their application in DRE and specific epilepsy syndromes. Each ASM demonstrated unique mechanisms targeting hyperexcitability, including the modulation of γ-aminobutyric acid receptor A (GABA-A) receptors, sodium and potassium channels, α-amino-3-hydroxy-5-methyl-4-isoxazolepropionic acid receptor (AMPA receptors), and serotonin systems. These mechanisms correspond with specific pathophysiological features in syndromes such as Dravet and Lennox–Gastaut. Evidence from clinical trials supports their use as adjunctive therapies with generally favorable tolerability, though adverse events and variable efficacy profiles were noted. The mechanistic diversity of these emerging ASMs supports their value in personalized epilepsy management, particularly in treatment-resistant cases. While the promise of precision medicine is evident, further studies are required to address challenges related to long-term safety, cost, and equitable access. Full article

Hemolytic uremic syndrome (HUS), a thrombotic microangiopathy primarily affecting the kidneys, can also involve the central nervous system (CNS), often leading to significant morbidity and mortality. Neurologic manifestations are among the most severe extra-renal complications, particularly in children and during outbreaks of Shiga toxin-producing Escherichia coli (STEC)-associated HUS (typical (tHUS)). This review explores the clinical spectrum, pathophysiology, diagnostic workup, and age-specific outcomes of neurologic involvement in both typical (tHUS) and atypical (aHUS). Neurologic complications occur in up to 11% of pediatric and over 40% of adult STEC-HUS cases in outbreak settings. Presentations include seizures, encephalopathy, focal deficits, movement disorders, and posterior reversible encephalopathy syndrome (PRES). Magnetic resonance imaging (MRI) commonly reveals basal ganglia or parieto-occipital lesions, though subtle or delayed findings may occur. Laboratory workup typically confirms microangiopathic hemolytic anemia (MAHA), thrombocytopenia, and kidney damage, with additional markers of inflammation or metabolic dysregulation. Eculizumab is the first-line treatment for aHUS with CNS involvement, while its utility in STEC-HUS remains uncertain. Although many children recover fully, those with early CNS involvement are at greater risk of developing epilepsy, cognitive delays, or fine motor deficits. Adults may experience lingering neurocognitive symptoms despite apparent clinical recovery. Differences in presentation and imaging findings between age groups emphasize the need for tailored diagnostic and therapeutic strategies. Comprehensive neurorehabilitation and long-term follow-up are crucial for identifying residual deficits. Continued research into predictive biomarkers, neuroprotective interventions, and standardized treatment protocols is needed for improving outcomes in HUS patients with neurological complications. Full article

Background: Electroencephalography (EEG) signals play a crucial role in diagnosing epilepsy by reflecting distinct patterns associated with normal brain activity, ictal (seizure) states, and interictal (between-seizure) periods. However, the manual classification of these patterns is labor-intensive, time-consuming, and depends heavily on specialized expertise. While deep learning methods have shown promise, many current models suffer from limitations such as excessive complexity, high computational demands, and insufficient generalizability. Developing lightweight and accurate models for real-time epilepsy detection remains a key challenge. Methods: This study proposes a novel dual-channel deep learning model to classify epileptic EEG signals into three categories: normal, ictal, and interictal states. Channel 1 integrates a bidirectional long short-term memory (BiLSTM) network with a Squeeze-and-Excitation (SE) ResNet attention module to dynamically emphasize critical feature channels. Channel 2 employs a dual-branch convolutional neural network (CNN) to extract deeper and distinct features. The model’s performance was evaluated on the publicly available Bonn EEG dataset. Results: The proposed model achieved an outstanding accuracy of 98.57%. The dual-channel structure improved specificity to 99.43%, while the dual-branch CNN boosted sensitivity by 5.12%. Components such as SE-ResNet attention modules contributed 4.29% to the accuracy improvement, and BiLSTM further enhanced specificity by 1.62%. Ablation studies validated the significance of each module. Conclusions: By leveraging a lightweight design and attention-based mechanisms, the dual-channel model offers high diagnostic precision while maintaining computational efficiency. Its applicability to real-time automated diagnosis positions it as a promising tool for clinical deployment across diverse patient populations. Full article

Epilepsy affects over 12 million children worldwide, with approximately 30% classified as having drug-resistant epilepsy (DRE), often accompanied by neuropsychiatric comorbidities that severely impact quality of life. The endocannabinoid system (ECS) functions as a multifaceted neuromodulatory network regulating neuronal excitability, synaptic plasticity, and immune homeostasis from early life through adolescence and into aging. In pediatric epilepsies, alterations in ECS components, particularly CB1 receptor expression and endocannabinoid levels, reveal disorder-specific vulnerabilities and therapeutic opportunities. Cannabidiol (CBD), a non-psychoactive compound from Cannabis sativa, has shown strong preclinical and clinical efficacy in treating DRE and is approved for Dravet syndrome, Lennox–Gastaut syndrome, and Tuberous Sclerosis Complex. Other ECS-based strategies, such as the use of CB1 receptor-positive allosteric modulators, can selectively enhance endogenous cannabinoid signaling where and when it is active, potentially reducing seizures in conditions like Dravet and absence epilepsy. Similarly, FAAH and MAGL inhibitors may help restore ECS tone without directly activating CB1 receptors. Precision targeting of ECS components based on regional expression and syndrome-specific pathophysiology may optimize seizure control and associated comorbidities. Nonetheless, long-term pediatric use must be approached with caution, given the critical role of the ECS in brain development. Full article

Background/Objectives: Sudden unexpected death in epilepsy (SUDEP) is a major cause of mortality in pediatric epilepsy. Cardiac arrhythmias, possibly reflected by electrocardiographic (ECG) abnormalities, are thought to contribute significantly to SUDEP risk. This study aimed to evaluate ECG indices associated with an increased risk of both atrial and ventricular arrhythmias and sudden cardiac death in pediatric patients with generalized and focal seizures, excluding those with underlying channelopathies. Materials and Methods: Pediatric patients aged 0–18 years with generalized or focal epilepsy followed at our center between October 2024 and April 2025 were enrolled. Comprehensive cardiac evaluations, including echocardiography and 12-lead ECG, were conducted. Patients with channelopathies, structural heart defects, or significant congenital heart disease were excluded. ECG parameters—QT dispersion (QT Disp), corrected QT interval (QTc), QTc dispersion (QTc Disp), P-wave dispersion (P Disp), and T peak-T end interval (Tp-e)—were analyzed across epilepsy subgroups and compared to healthy controls. Effects of antiepileptic drug (AED) use and gender were also assessed. Results: A total of 151 participants were included (generalized: n = 51; focal: n = 50; controls: n = 50). QTc and Tp-e intervals were prolonged in both epilepsy groups compared to controls (p = 0.001 and p = 0.036, respectively), however, they fell within the conventional parameters. AED use was associated with further prolongation of QTc (p = 0.035) and Tp-e (p = 0.037), these metrics were similarly found to be within the established normative boundaries. Phenobarbital and lamotrigine users showed the longest QTc, albeit not statistically significant. Males with generalized seizures had longer maximum P-wave duration (P Max) than females (p = 0.009). A moderate correlation was found between Tp-e and QTc (r = 0.557, p = 0.001). Conclusions: Although there are findings in our study that may suggest a relationship between SUDEP and arrhythmia according to electrocardiographic markers associated with arrhythmia risk, larger and prospective studies with long-term follow-up are needed in the future. Full article

Background: Oxidative stress is associated with the pathogenesis of epilepsy. Long-term treatment with anti-seizure medications (ASMs) may reduce antioxidant levels, which consequently impairs the brain’s ability to counteract oxidative damage. This study aimed to assess the concentrations of selected antioxidants (i.e., glutathione, retinol, and α- and γ-tocopherols) in children with epilepsy treated with polytherapy. Methods: The study included 21 children with epilepsy treated with ≥2 ASMs for at least 6 months (mean age 7.1 ± 4.4 years) and 23 control children without epilepsy (mean age 7.4 ± 3.9 years). Both groups were recruited at the Department of Pediatric Neurology, the Medical University of Silesia in Katowice (Poland). The concentrations of glutathione, retinol, and α- and γ-tocopherols were determined in blood serum by HPLC. The antioxidant levels were compared between sex and age subgroups of individuals with epilepsy. Results: In the group of individuals with epilepsy, the percentage of females was 38% and in the control group it was 30%. There were no differences in antioxidant levels between female and male individuals with epilepsy, nor between younger epileptic children (0–6 years) and older children (>6 years). Individuals with epilepsy had significantly lower glutathione levels than the control group (1.5 ± 0.3 µmol/L vs. 2.4 ± 1.2 µmol/L, respectively, p < 0.001). In turn, the ratios of both α-tocopherol/glutathione and γ-tocopherol/glutathione were higher in individuals with epilepsy than in the control group (p = 0.042 and p = 0.004, respectively). Individuals with epilepsy taking ASM combinations other than valproic acid (VPA) and levetiracetam (LEV) had a lower level of both retinol and glutathione than individuals on VPA and LEV treatment (for retinol 0.44 ± 0.13 µmol/L vs. 0.6 ± 0.1 µmol/L, respectively, p = 0.047, and for glutathione 1.3 ± 0.3 µmol/L vs. 1.8 ± 0.3 µmol/L, respectively, p = 0.003). In the individuals with epilepsy, the level of α-tocopherol decreased with age (r = −0.505, p = 0.019). In turn, in the control group, the levels of retinol and γ-tocopherol increased with age (r = 0.573, p = 0.004 and r = 0.461, p = 0.027, respectively). Conclusions: Glutathione levels significantly differed between children with and without epilepsy. The concentration of α-tocopherol decreased with age in pediatric individuals with epilepsy. The levels of both retinol and glutathione were higher in individuals with epilepsy taking VPA and LEV treatment compared to individuals on ASMs combination other than VPA and LEV. Full article

Despite advances in technology, the overall management of type 1 diabetes mellitus (T1DM) remains suboptimal. The idea of restricting carbohydrate intake to decrease glycemic spikes and insulin requirements has been revisited in recent years. After impressive results in the fields of type 2 diabetes (T2DM) and epilepsy, low-carbohydrate (LCD) and ketogenic (KD) diets have gained renewed interest as a possible treatment option for T1DM. In this narrative review, we discuss the available data regarding LCDs and KDs in both the adult and pediatric populations. Research data is still scarce, as most studies are short-term and show considerable heterogeneity in dietary composition and patient outcomes. In general, carbohydrate restriction enhances glycemic control by reducing postprandial glucose excursions, improving time-in-range, and lowering HbA1c, with conflicting effects on other parameters such as lipid profile and body weight. Adverse effects such as hypoglycemia and diabetic ketoacidosis are rarely reported, although some concerns have been raised regarding growth in children. The correct implementation of these diets requires a multidisciplinary approach by highly specialized healthcare professionals, who will address the medical, social, and psychological concerns that a restrictive diet entails. Large-scale and long-term studies are needed to provide more robust data before carbohydrate restriction can be widely applied to patients with T1DM. Full article

Epilepsy is a neurological disorder characterized by recurrent, unprovoked seizures, affecting millions worldwide. While anti-seizure medications serve as first-line treatment, approximately one-third of patients develop drug-resistant epilepsy (DRE), necessitating alternative interventions. Deep brain stimulation (DBS) has emerged as a promising therapy for DRE, particularly for patients who are ineligible for resective surgery. DBS involves stereotactic implantation of electrodes into target brain regions, such as the anterior nucleus of the thalamus (ANT), centromedian nucleus (CMT), and hippocampus (HC), to modulate aberrant neural activity and to reduce seizure frequency. Anesthesia plays a critical role in DBS implantation, influencing both patient safety and procedural success. The choice of anesthetic technique must balance patient comfort with the preservation of neurophysiological signals used for intraoperative electrode localization. A well-chosen anesthetic strategy can enhance the efficacy of electrode placement by minimizing patient movement and preserving critical neurophysiological signals for real-time monitoring. This precise targeting enhances safety via a reduction in perioperative risks and an improvement in long-term seizure control. Anesthetic considerations in epilepsy patients differ from those in movement disorders due to variations in their nuclei targets during DBS. Despite the increasing use of DBS for epilepsy following its FDA approval in 2018, research on anesthetic effects specific to this population remains limited. This narrative review, therefore, examines anesthetic approaches, pharmacological implications, potential complications, and evolving methods for DBS implantation in epilepsy patients, highlighting new insights and unique considerations in this population. Understanding these factors is essential for optimizing surgical outcomes and improving the safety and efficacy of DBS in epilepsy treatment. Full article

Background: Developmental epileptic encephalopathies (DEEs) are often associated with variably severe cognitive and motor impairment and frequent refractory epilepsy, with many children not achieving adequate seizure control via standard antiepileptic medications. The classic ketogenic diet (KD) has proven effective in reducing seizure frequency and/or severity in a category of DEEs and in certain refractory epilepsies of infancy. However, its multifaceted mechanisms, e.g., epigenetic modulation, anti-inflammatory and antioxidative effects, and direct neuronal excitability changes, are balanced by a high burden and low long-term adherence. Medium-chain triglycerides (MCTs), particularly decanoic acid (C10:0), have gained attention in recent years for their potential direct inhibitory action on AMPA receptors, contributing to seizure reduction. Methods: A systematic review was conducted, including articles from January 2000 to January 2025, to explore the potential role of medium-chain triglyceride (MCT) add-on to classic KD and as MCT supplementation in free diets in the management of pediatric drug-resistant epilepsy (DRE). Results: Selected studies show how the action of MCTs, and decanoic acid in particular, is via negative modulation of AMPA receptors, with a positive impact on epileptic seizures. Conclusions: This review discusses the complexities of implementing and sustaining KD in children and presents recent pre-clinical and clinical evidence, including trials where MCTs (often enriched in decanoic acid) serve as an add-on therapy in both ketogenic and free/unrestricted diets. The summarized findings reinforce the therapeutic potential of MCTs, highlighting both the beneficial seizure outcomes and the hurdles that remain to be addressed through future research. Full article

Electroencephalography (EEG) remains an essential method for monitoring brain activity, but the limitations of conventional systems due to the complexity of installation and lack of portability have led to the introduction and development of in-ear EEG technology. In-ear EEG is an emerging method of recording electrical activity in the brain and is an innovative concept that offers multiple advantages both from the point of view of the device itself, which is easily portable, and from the user’s point of view, who is more comfortable with it, even in long-term use. One of the fundamental components of this type of device is the electrodes used to capture the EEG signal. This innovative method allows bioelectrical signals to be captured through electrodes integrated into an earpiece, offering significant advantages in terms of comfort, portability, and accessibility. Recent studies have demonstrated that in-ear EEG can record signals qualitatively comparable to scalp EEG, with an optimized signal-to-noise ratio and improved electrode stability. Furthermore, this review provides a comparative synthesis of performance parameters such as signal-to-noise ratio (SNR), common-mode rejection ratio (CMRR), signal amplitude, and comfort, highlighting the strengths and limitations of in-ear EEG systems relative to conventional scalp EEG. This study also introduces a visual model outlining the stages of technological development for in-ear EEG, from initial research to clinical and commercial deployment. Particular attention is given to current innovations in electrode materials and design strategies aimed at balancing biocompatibility, signal fidelity, and anatomical adaptability. This article analyzes the evolution of EEG in the ear, briefly presents the comparative aspects of EEG—EEG in the ear from the perspective of the electrodes used, highlighting the advantages and challenges of using this new technology. It also discusses aspects related to the electrodes used in EEG in the ear: types of electrodes used in EEG in the ear, improvement of contact impedance, and adaptability to the anatomical variability of the ear canal. A comparative analysis of electrode performance in terms of signal quality, long-term stability, and compatibility with use in daily life was also performed. The integration of intra-auricular EEG in wearable devices opens new perspectives for clinical applications, including sleep monitoring, epilepsy diagnosis, and brain–computer interfaces. This study highlights the challenges and prospects in the development of in-ear EEG electrodes, with a focus on integration into wearable devices and the use of biocompatible materials to improve durability and enhance user comfort. Despite its considerable potential, the widespread deployment of in-ear EEG faces challenges such as anatomical variability of the ear canal, optimization of ergonomics, and reduction in motion artifacts. Future research aims to improve device design for long-term monitoring, integrate advanced signal processing algorithms, and explore applications in neurorehabilitation and early diagnosis of neurodegenerative diseases. Full article

Epilepsy is the most common neurological disorder in cats. However, information on the long-term outcomes and predictive survival factors based on neurological and clinicopathological findings is limited. We aimed to evaluate the clinical manifestations, survival rates, and hazard factors influencing survival in cats with epilepsy by analyzing the medical records of 90 cats diagnosed via brain magnetic resonance imaging (MRI). The cats were divided into the survival and non-survival groups. Univariate and multivariable logistic regression analyses were conducted to identify the significant survival factors. The mortality rates at one and two years were 33.2% and 37.8%, respectively. The median age of cats in the non-survival and survival group were 3 and 1 years, respectively. Age over 7 years (p = 0.002), paresis (p = 0.001), structural brain lesions (p = 0.015), leukocytosis (p = 0.001), neutrophilia (p = 0.001), hyperproteinemia (p = 0.037), hypoalbuminemia (p = 0.001), hyperglobulinemia (p = 0.003), and an elevated neutrophil-to-lymphocyte ratio (p = 0.041), were associated with an increased mortality rate. A multivariable analysis identified several predictors of early mortality in cats with epilepsy, including age over 7 years (p = 0.045), presence with paresis (p = 0.012), structural brain lesions (p = 0.042), leukocytosis (p = 0.005), and hypoalbuminemia (p = 0.030). Older age and structural brain lesions were shown to be key predictors of mortality and were important for prognosis prediction and management. Full article

Sudden unexpected death in epilepsy (SUDEP) is sudden, unexpected, witnessed or unwitnessed, nontraumatic, non-drowning death that occurs in a person with epilepsy. SUDEP is the leading cause of epilepsy-related death in adults with epilepsy, with an incidence of about 1.2 per 1000 person-years in the general epilepsy population. Recent studies have shown similar prevalence in the pediatric population too. Although the precise mechanism remains unclear, well-documented cases of SUDEP suggest that a generalized tonic clonic seizure-induced, centrally mediated change in cardiorespiratory function leads to terminal apnea and cardiac arrest. Risk factors include generalized tonic clonic seizure frequency, duration of epilepsy, nocturnal seizure, and certain genetic syndromes. Orexin, adenosine, and serotonin neurotransmission have been explored as novel drug targets to mitigate SUDEP risk. Neurostimulation and resective epilepsy surgery have been reported to have beneficial effects on long-term SUDEP risk as well. Future studies may aim to clarify the role of sleep and other comorbidities in SUDEP pathophysiology. Full article