Epileptic Seizures In Children Research Articles

Element Changes Occurring in Brain Point at the White Matter Abnormalities in Rats Exposed to the Ketogenic Diet During Prenatal Life.

A large number of clinical studies demonstrate that the ketogenic diet (KD) may be an effective approach to the reduction of epileptic seizures in children and adults. Such dietary therapy could also help pregnant women with epilepsy, especially since most antiseizure drugs have teratogenic action. However, there is a lack of medical data, considering the safety of using KD during gestation for the progeny. Therefore, we examined the influence of KD used prenatally in rats on the elemental composition of the selected brain regions in their offspring. For this purpose, synchrotron radiation-induced X-ray fluorescence (SR-XRF) microscopy was utilized, and elements such as P, S, K, Ca, Fe, and Zn were determined. Moreover, to verify whether the possible effects of KD are temporary or long-term, different stages of animal postnatal development were taken into account in our experiment. The obtained results confirmed the great applicability of SR-XRF microscopy to track the element changes occurring in the brain during postnatal development as well as those induced by prenatal exposure to the high-fat diet. The topographic analysis of the brains taken from offspring of mothers fed with KD during pregnancy and appropriate control individuals showed a potential influence of such dietary treatment on the brain levels of elements such as P and S. In the oldest progeny, a significant reduction of the surface of brain areas characterized by an increased P and S content, which histologically/morphologically correspond to white matter structures, was noticed. In turn, quantitative elemental analysis showed significantly decreased levels of Fe in the striatum and white matter of 30-day-old rats exposed prenatally to KD. This effect was temporary and was not noticed in adult animals. The observed abnormalities may be related to the changes in the accumulation of sphingomyelin and sulfatides and may testify about disturbances in the structure and integrity of the myelin, present in the white matter.

Automatic diagnosis of epileptic seizures using entropy-based features and multimodel deep learning approaches

Epilepsy is one of the most common brain diseases, characterised by repeated seizures that occur on a regular basis. During a seizure, a patient's muscles flex uncontrollably, causing a loss of mobility and balance, which can be harmful or even fatal. Developing an automatic approach for warning patients of oncoming seizures necessitates substantial research. Analyzing the electroencephalogram (EEG) output from the human brain's scalp region can help predict seizures. EEG data were analyzed to extract time domain features such as Hurst exponent (Hur), Tsallis entropy (TsEn), enhanced permutation entropy (impe), and amplitude-aware permutation entropy (AAPE). In order to automatically diagnose epileptic seizure in children from normal children, this study conducted two sessions. In the first session, the extracted features from the EEG dataset were classified using three machine learning (ML)-based models, including support vector machine (SVM), K nearest neighbor (KNN), or decision tree (DT), and in the second session, the dataset was classified using three deep learning (DL)-based recurrent neural network (RNN) classifiers in The EEG dataset was obtained from the Neurology Clinic of the Ibn Rushd Training Hospital. In this regard, extensive explanations and research from the time domain and entropy characteristics demonstrate that employing GRU, LSTM, and BiLSTM RNN deep learning classifiers on the All−time−entropy fusion feature improves the final classification results.

Relationship between Serum IgA concentration and epileptic seizures in children: A prospective study

Purpose. This study investigated the relationship between serum immunoglobulin A (IgA) concentration and epileptic seizures in children. Methods. Pediatric patients with epilepsy were included in this cross-sectional investigation. Serum IgA levels were analyzed using a biochemical analyzer during admission for initial diagnosis before any medical treatment was administered. Results. Among the 94 patients, 19.1% had decreased serum IgA levels, with a median value of 0.57 g/L. No significant relationship existed between serum IgA concentration and gender, age, epilepsy type, seizure duration or epilepsy waves. Children with low IgA levels experienced more spastic episodes and had a higher seizure frequency compared to those without decreased IgA levels. Changes in serum IgA levels were significantly associated with seizure frequency after three months of treatment. Initially, the occurrence of seizures in the low IgA group was 10.5 times lower than in the non-low IgA group, but, after three months, the low IgA group had a significantly higher seizure rate (72.2%) compared to the non-low IgA group (19.7%). After six months, the low IgA group had a higher proportion of children without seizures (100% versus 84.8%), although the difference was not statistically significant. Conclusion. Serum IgA concentration plays a crucial role in diagnosing and predicting outcomes for pediatric epilepsy.

Diagnostic role of serum prolactin level in different kinds of seizure and seizure-like episode in children: A hospital-based study

Background: Serum prolactin level has been previously used in distinguishing epileptic seizure from non-epileptic seizure, as prolactin level usually rises following an epileptic seizure in children. Aims and Objectives: We conducted this study to determine the role of serum prolactin as a surrogate marker of seizure disorder and whether serum prolactin level varies between different types of seizure, febrile seizure, and seizure mimics. Materials and Methods: This cross-sectional comparative study was conducted for 1 year among 113 children between 6 months and 16 years of age of either sex. They were divided into 3 groups (1) epileptic seizure, (2) febrile seizure, and (3) seizure mimics. The blood sample was collected within 1 h of the occurrence of seizure for estimation of serum prolactin level. Each child was investigated and treated according to the protocol. Results: Mean post-ictal prolactin level was found significantly higher in epileptic seizure (29.94±7.97 ng/mL) compared to febrile seizure (10.21±0.94 ng/mL), and seizure mimics (8.73±0.67 ng/mL). Among group 1, serum prolactin levels significantly elevated in children with generalized tonic-clonic seizure (GTCS) (34.18±2.76 ng/mL) and complex partial seizure (CPS) (31.38±1.59 ng/mL) compared to simple partial seizure (SPS) (14.36±2.33 ng/mL). Post-ictal serum prolactin levels remain elevated for a longer duration in GTCS (54.26±5.19 min) and CPS (45.19±1.73 min) compared to children with SPS (26.76±2.33 min). Conclusion: Estimation of serum prolactin level within 1 h after a seizure can be used for screening purposes to distinguish between epileptic and non-epileptic seizures. A high prolactin level is suggestive of GTCS or CPS whereas a low level is indicative of SPS, febrile convulsion, or seizure mimics with a prolactin cutoff taken 24 ng/mL in our study.

Genetic epilepsy and role of mutation variants in 27 epileptic children: results from a "single tertiary centre" and literature review

<sec><title>Aim and scope</title><p>The wide use of next-generation sequencing has allowed professionals to reach relevant progress in the medical field including diagnoses, prognoses, and genetic counselling and to help in recognizing the pathogenic mechanisms which underlie several epileptic disorders. Genetic epilepsy refers to a disorder in which genetic mutations are recognized as the primary cause of epileptic seizures in the patients. Various types of epilepsy have been highlighted alongside a clear etiologic relationship with genetic mutations; however, for a select number of patients, some doubts remain on the role played by the variant mutations, as well as the adverse pathologic events which may interfere with the clinical manifestations. In this study, twenty-seven children affected by epileptic seizures are reported, with the aim to describe the clinical and neurological involvement presented by the children according to the genes and variant mutations, and to compare the neurological signs observed in these children with those reported in children with similar genetic mutations. Additionally, the roles played by single or associated variants on the clinical expression of the affected children are discussed.</p></sec><sec><title>Material and methods</title><p>A retrospective observational study was conducted on the gene mutations observed in 27 children affected by a clinical history and diagnosis of epilepsy, alongside an electroencephalographic analysis performed with the Pediatric Department of the University-Hospital S. Marco during the years ranging from January 2020 to January 2022. A genetic analysis was performed using an array comparative genomic hybridization (array CGH), a gene panel for epilepsy, Whole Exome Sequencing (WES), and a single nucleotide polymorphism (SNP) array.</p></sec><sec><title>Results</title><p>Nineteen children showed a single nucleotide variant, eight children carried two or more gene mutations in different genes, and one child was not reported. In this study, we identified a total of thirty-six gene mutations, where seventeen were de novo mutations, and twenty-two had either a maternal or paternal inheritance. The genes in which one or more allelic variants were found included <italic>GRIN2A</italic>, <italic>GABRA1</italic>, <italic>SCN2A1,</italic> <italic>KCNT1</italic>, <italic>PCDH19</italic>, <italic>SCN8A</italic>, <italic>KCNK4</italic>, <italic>SLC2A1</italic>, <italic>DNM1</italic>, <italic>ARID1B</italic>, <italic>WWOX</italic>, <italic>GABRG2,</italic> and others.</p></sec><sec><title>Conclusions</title><p>Genetic analyses are recognized to have a central role in the diagnostic process of children with epileptic seizures, alongside their family history, and clinical, neuroradiologic, and neurophysiologic examinations. The gene mutation anomalies reported in this study may be useful to be included in the wide field of factors related to epileptic seizures in children. The roles played by single or associated variant mutations in the clinical expression of the affected epileptic children remain difficult to be defined. Moreover, other factors such as pre-, peri-, and post-natal adverse events may negatively interfere with the severity of the clinical manifestations.</p></sec>

Non-epileptic paroxysmal events at pediatric video-electroencephalography monitoring unit over a 15-year period

ObjectiveNon-epileptic paroxysmal events (NEPEs) are common in pediatric patients and may be misdiagnosed as epileptic seizures. We aimed to study the distribution of NEPEs across age groups and with different comorbidities, and to correlate the patients' presenting symptoms with their final diagnosis after video-EEG. MethodsWe retrospectively analyzed video-EEG recordings of children aged one month to 18 years who were admitted between March 2005 and March 2020. Patients who experienced any NEPE while under video-EEG monitorization were evaluated in this study. Subjects with concomitant epilepsy were also included. The patients were first divided into 14 groups according to the basic characteristics of symptoms they reported at admission. The events captured on video-EEG were then classified into six NEPE categories based on the nature of the events. These groups were compared according to video-EEG results. ResultsWe retrospectively evaluated 1338 records of 1173 patients. The final diagnosis was non-epileptic paroxysmal event in 226 (19.3%) of 1173 patients. The mean age of the patients was 105.4 ± 64.4 months at the time of the monitoring. The presenting symptoms were motor in 149/226 (65.9%) patients, with jerking being the most common (n = 40, 17.7%). Based on video-EEG, the most common NEPE was psychogenic non-epileptic seizures (PNES) (n = 66, 29.2%), and the most common PNES subtype was major motor movements (n = 19/66, 28.8%). Movement disorders (n = 46, 20.4%) were the second most common NEPE and the most common NEPE (n = 21/60, 35%) in children with developmental delay (n = 60). Other common NEPEs were physiological motor movements during sleep (n = 33, 14.6%), normal behavioral events (n = 31, 13.7%), and sleep disorders (n = 15, 6.6%). Almost half of the patients had a prior diagnosis of epilepsy (n = 105, 46.5%). Following the diagnosis of NEPE, antiseizure medication (ASM) was discontinued in 56 (24.8%) patients. ConclusionNon-epileptiform paroxysmal events can be difficult to distinguish from epileptic seizures in children, especially in patients with developmental delay, epilepsy, abnormal interictal EEG, or abnormal MRI findings. Correct diagnosis of NEPEs by video-EEG prevents unnecessary ASM exposure in children and guides appropriate management of NEPEs.

Applications of Capillary Electrophoresis for the Determination of Cannabinoids in Different Matrices.

Cannabinoids, terpenophenolic chemicals found only in cannabis, are primarily responsible for cannabis pharmacologic effects; nearly 150 distinct cannabinoids have been identified thus far. Among these, the main psychoactive molecule, tetrahydrocannabinol (THC), and the non-psychoactive counterpart, cannabidiol (CBD) are distinguishable. In the past decade, a CBD-containing pharmaceutical preparation was approved by Food and Drug Administration (FDA) for the treatment of drug-resistant epileptic seizures in children, and research trials for a variety of additional medical conditions for which CBD has been suggested as a therapy are being conducted. Additionally, the number of "CBD-containing" dietary supplements, largely available online, is increasing rapidly. Consequently, the necessity for the development of qualitative and quantitative methodologies for the analysis of the bioactive components of Cannabis is rising because of the increase in the production of therapeutic cannabis products. One of the analytical methods with good potential in cannabinoids analysis is capillary electrophoresis (CE). It has advantages related to high separation efficiency, relatively short analysis time, and the small consumption of analytes and reagents which generates relatively lower operational costs than other methods. This review focuses on the use of CE techniques to examine biological matrices and plant materials for the presence of cannabinoids and other bioactive compounds found in cannabis. The advantages, drawbacks, and applicability of the various electromigration approaches are also assessed. The article provides an overview of the "state of the art" and the latest trends in CE-based methods for the determination of cannabinoids.

Neurological prognostic factors for human herpes virus 6/7-associated acute encephalopathy in children: A single-center study

AimTo identify prognostic factors for severe neurological sequelae and epileptic seizures in children with human herpes virus (HHV) 6/7-associated acute encephalopathy (AE). MethodsWe retrospectively studied pediatric cases of HHV6/7-associated AE between April 2011 and March 2021. Neurological sequelae were assessed using the Pediatric Cerebral Performance Category scale (PCPC) and the presence of epileptic seizures 1 year after onset. We investigated the prognostic factors between the non-severe sequelae group (PCPC scores ≤ 2) and severe sequelae group (PCPC scores ≥ 3) in patients without severe neurological complications before onset. ResultsForty patients, ranging from 4 to 95 months old, were included. AE with biphasic seizures and late reduced diffusion were the most common types of encephalopathy (n = 28). Among the 36 patients evaluated neurological sequelae, 17, nine, eight, and two were categorized as PCPC 1, 2, 3 and 4, respectively. Epileptic seizures were observed in nine patients. In the severe sequelae group, significantly more cases with coma in the acute phase and thalamic lesions on MRI and higher serum aspartate aminotransferase, alanine aminotransferase (ALT), and lactate dehydrogenase levels were observed. Multivariate analysis showed a significant between-group difference in the rate of coma (p = 0.0405). Patients with epileptic seizures had a higher rate of coma and thalamic lesions and higher serum ALT and urinary beta 2-microglobulin levels, but there was no significant difference in the multivariate analysis. ConclusionsIn HHV6/7-associated AE, coma was a significant prognostic factor for severe neurological sequelae.

Пароксизмальні розлади сну в дітей з епілептичними енцефалопатіями та розладами аутистичного спектра

Purpose - to determine the prevalence of sleep disorders in children with epileptic encephalopathies and autism spectrum disorder (ASD); to evaluate the effectiveness of neuroadaptogenic «Silenta» in the treatment of sleep disorders in children in this group. Materials and methods. We examined 50 children with clinical manifestations of ASD aged 2 to 8 years (mean age was 4.3 years±1.5), including 31 boys and 19 girls. The examination included anamnesis to clarify the features of the pre- and perinatal period, clinical and neurological examination, video EEG monitoring during night sleep for 8 hours, survey of parents on the quality of sleep in children using the Children’s Sleep Habits Questionnaire (CSHQ). All children were divided into 2 groups depending on clinical features: Group 1 - 28 children with ASD who had epileptic seizures at the time of examination or in the anamnesis (children with epileptic encephalopathies); Group 2 - 22 children with ASD without clinical epileptic seizures. Children from the 1st group were prescribed neuroadaptogen «Silenta» for 30 days. Results. Children with ASD and epileptic seizures had higher sub-scales «Difficulty falling asleep», «Parasomnia» and the overall score on the CSHQ-A scale. The results indicate a negative impact of epileptic seizures in children with ASD on sleep quality and the frequency of paroxysmal sleep disorders. The overall prevalence of sleep disorders in children with ASD was determined using the CSHQ-A questionnaire before and after treatment. The incidence of sleep disorders in children with ASD and epileptic seizures after treatment decreased from 85.7% to 53.6%, and in children with ASD without epileptic seizures - from 63.6% to 22.7%. In both groups statistically significant reduction in the proportion of children with sleep disorders after a course of treatment with neuroadaptogen «Silenta». Conclusions. It is shown that the frequency of sleep disorders in children with ASD and epileptic seizures is 85.7%, and in children with ASD without epileptic seizures - 63.6%. In children with ASD and epileptic seizures the incidence of sleep disorders during 30 days of the neuroadaptogen «Silenty» decreased to 53.6%, and in children with ASD and without epileptic seizures the incidence of sleep disorders decreased to 22.7%. The research was carried out in accordance with the principles of the Helsinki Declaration. The study protocol was approved by the Local Ethics Committee of the participating institution. The informed consent of the patient was obtained for conducting the studies. No conflict of interests was declared by the authors.

Postoperative Epileptic Seizures in Children.

Background: Postoperative seizures (PS) occur in 10–15% of patients. This study aims to provide an update on the role of surgery in PS. Methods: All children undergoing a craniotomy for supratentorial lesions in the last 10 years were considered except those with preoperative seizures, perioperative antiepileptic drugs prophylaxis, head-injury and infections, repeated surgery, or preoperative hyponatremia. Children undergoing surgery for intra-axial lesions (Group 1, 74 cases) were compared with those harboring extra-axial lesions (Group 2, 91 cases). Results: PS occurred in 9% of 165 cases and epilepsy in 3% of 165 cases (mean follow-up: 5.7 years). There was no difference between the two study groups with regard to demographic data or tumor size. Group 1 showed a higher rate of gross total tumor resection (p = 0.002), while Group 2 had a higher rate of postoperative hyponatremia (p < 0.0001). There were no differences between the two groups in the occurrence of seizures (6.7% vs. 11%) or epilepsy (2.7% vs. 3.2%). No correlations were found between seizures and age, tumor location, histotype, tumor size, or the extent of tumor resection. Hyponatremia affected the risk of PS in Group 2 (p = 0.02). Conclusions: This study shows a lower rate of PS and epilepsy than series including children with preoperative seizures. Hyponatremia has a significant role. Neurosurgery is safe but surgical complications may cause late epilepsy.

The value of immunotherapy in children with initial short-term frequent seizures.

This study aimed to discuss clinical characteristics, therapy, and antibody prevalence in epilepsy (APE) score for short-term, frequent epileptic seizures in children who are autoimmune-antibody negative and respond well to immunotherapy. The clinical characteristics, imaging manifestations, electrophysiology, and effective treatment plan of 9 children who met the above criteria were retrospectively analyzed in the Pediatric Neurology Department of Qilu Hospital at Shandong University from June 2019 to December 2021. All 9 patients (6 boys, 3 girls; aged 13 months−11 years and 5 months, median 3.5 years) had acute-onset seizures within 3 months. All had previous normal growth/development with no family history of disease. Seizure types were focal motor seizures (6), generalized tonic-clonic seizures (2), and generalized secondary-to-focal (1); occurred >10 times/day; and lasted <1 min/episode. Formal treatment with ≥2 types of antiseizure medicine (ASM) achieved an unsatisfactory effect. Cranial magnetic resonance imaging showed an abnormal result in 1 case. The APE score was ≥4 in 3 cases and <4 in 6 cases. All patients experienced symptomatic relief with immunotherapy; subsequently, 8 patients were free of recurrence and 1 had significantly reduced seizure frequency. Autoimmune antibody screening is recommended for children who were previously well and have acute-onset epilepsy; high frequency, short-duration seizures; no good response to 2 types of ASM; and other etiologic factors excluded, even with APE score <4. Even with negative autoimmune antibody results, the possibility of autoimmune epilepsy should be considered for urgent initiation of immunotherapy, which can achieve good results.

Predictive value of video alone in diagnosis of epileptic vs paroxysmal nonepileptic events in children

ObjectivePrevious studies examined the use of video-based diagnosis and the predictive value of videos for differentiation of epileptic seizures (ES) from paroxysmal nonepileptic events (PNEE) in the adult population. However, there are no such published studies strictly on the pediatric population. Using video-EEG diagnosis as a gold standard, we aimed to determine the diagnostic predictive value of videos of habitual events with or without additional clinical data in differentiating the PNEE from ES in children. MethodsConsecutive admissions to our epilepsy monitoring unit between June 2020 and December 2020 were analyzed for events of interest. Four child neurologists blinded to the patient’s diagnosis formulated a diagnostic impression based upon the review of the video alone and again after having access to basic clinical information, in addition to the video. Features of the video which helped to make a diagnosis were identified by the reviewers as a part of a survey. ResultsA total of 54 patients were included (ES n = 24, PNEE n = 30). Diagnostic accuracy was calculated for each reviewer and combined across all the ratings. Diagnostic accuracy by video alone was 74.5% (sensitivity 80.8%, specificity 66.7%). Providing reviewers with basic clinical information in addition to the videos significantly improved diagnostic accuracy compared to viewing the videos alone. Inter-rater reliability between four reviewers based on the video alone showed moderate agreement (κ = 0.51) and unchanged when additional clinical data were presented (κ = 0.51). The ES group was significantly more likely to demonstrate changes in facial expression, generalized stiffening, repetitive eye blinks, and eye deviation when compared with the PNEE group, which was more likely to display bilateral myoclonic jerking. ConclusionsVideo review of habitual events by Child Neurologists may be helpful in reliably distinguishing ES from PNEE in children, even without included clinical information.

Clinical Analysis of Childhood Acute Lymphoblastic Leukemia With Epilepsy Seizures.

ObjectiveIn order to analyze the clinical characteristics of epileptic seizures in children with acute lymphoblastic leukemia (ALL) during treatment.MethodsThe clinical and imaging data of children diagnosed as ALL with epilepsy seizures from January 2013 to December 2020 were retrospectively analyzed.ResultsA total of 2217 children with ALL were admitted during the study, of whom 229 (10.33%) had epileptic seizures after ALL treatment. Among them, 45 (19.65%) were in the high-risk group and 184 (80.35%) were in the low-risk group. Epileptic seizures mainly occurred in the induction remission period (24.02%), maintenance treatment period (25.33%) and after bone marrow transplantation (21.40%). The common causes were MTX-related demyelinating encephalopathy (34.06%) and reversible posterior encephalopathy syndrome (PRES) (25.3%). The first symptom was mainly convulsion (34.50%). The first attack had a comprehensive attack and partial attack. Most patients stop themselves. 30 cases (13.10%) had acute recurrence of epilepsy (recurrence within 3 months after the first attack), and 49 cases (25.76%) had neurological dysfunction after follow-up. 36 cases developed symptomatic epilepsy. Among the 130 children who completed the follow-up, 78 (60.00%) had no obvious neurological sequelae, and 52 (40.0%) had neurological sequelae. Among the 52 cases, there were 34 cases of mild sequelae and 18 cases of severe sequelae, including 8 cases of epilepsy combined with cognitive impairment.ConclusionEpileptic seizure is a common neurological complication during ALL treatment. The etiology and associated manifestations of the first epileptic seizure are diverse. Early neuroimaging and EEG examination are helpful for early diagnosis and treatment.

An analysis of breakthrough seizures and related factors in paediatric epilepsy patients.

To determine the frequency of breakthrough seizures among paediatric patients suffering from epilepsy, and factors related to the precipitation of these seizures. The cross-sectional study was conducted from July 1, 2018, to July 1, 2020, at the Combined Military Hospital Lahore and the Military Hospital, Rawalpindi, Pakistan, and comprised children of either gender aged 2-12 years diagnosed with any type of epilepsy presenting at the children outpatient department. Diagnosis of epilepsy was established by either a consultant adult neurophysician or a consultant paediatrician. The presence of breakthrough seizures was assessed by obtaining detailed history from the patient and the primary caregiver. Socio-demographic profile, duration of epilepsy and poly-pharmacy were noted. Data was analysed using SPSS 23. Of the 450 subjects, 259(57.6%) were boys and 191(42.4%) were girls. The overall mean age was 6.353±4.732 years. The presence of breakthrough seizures was noted in 227(50.4%) subjects. Children with young age, with low family income, and those in need of poly-pharmacy showed significantly higher odds for breakthrough seizures (p<0.05). The incidence of breakthrough seizures in epileptic children was high despite the anticonvulsant agents that were previously effective in controlling seizures.

Children's Neurological Status Epilepticus and Poor Prognostic Factors through Electroencephalogram Image under Composite Domain Analysis Algorithm.

This study aimed to analyze the application of composite domain analysis algorithm for electroencephalogram (EEG) images of children with epilepsy and to investigate the risk factors related to poor prognosis. 70 children with neurological epilepsy admitted to the hospital were selected as the research objects. Besides, the EEG of the children during the intermittent and seizure phases of epilepsy were collected, so as to establish a composite domain analysis algorithm model. Then, the model was applied in EEG analysis. The clinical disease type and prognosis of children were statistically analyzed, and the risk factors that affected the prognosis of children were investigated. The results showed that the EEG signal values of the detail coefficients (d51 and d52) and the approximate coefficient (c5) during the epileptic seizure period were higher markedly than the signal values of the epileptic intermittent period; the EEG signal of the epileptic intermittent period was a transient waveform, which appeared as sharp waves or spikes. The EEG signal of epileptic seizures was continuous, with a composite waveform of sharp waves and spikes, and the change amplitude of the wavelet envelope spectrum during epileptic seizures was also higher hugely than that of intermittent epilepsy. The accurate identification rate, specificity, and sensitivity of EEG analysis with the composite domain algorithm were higher than those without the algorithm. Among the five types of epileptic seizures in children, the proportion of systemic tonic-clonic status was the largest, and the proportion of myoclonic status was equal to that of complex partial epileptic status, both of which were relatively small. The proportion of children with a better prognosis was 75.71% (53/70), which was higher than those with a poor prognosis 24.29% (17/70). Abnormal imaging examination (odds ratio (OR) = 3.823 and 95% confidence interval (CI) = 1.643–8.897); seizure duration greater than 1 hour (OR = 1.855 and 95% CI = 1.076–3.199); C-reactive protein (CRP) (OR = 5.089 and 95% CI = 1.507–17.187); and abnormal blood glucose (OR = 3.077, 95%CI = 1.640–5.773) were all independent risk factors for poor prognosis (all P < 0.05). The composite domain analysis algorithm was helpful for clinicians to find the difference in the EEG signals between the epileptic seizure period and the epileptic intermittent period in a short time, thereby improving the doctor's analysis of the results, which could reflect its marked superiority. In addition, abnormal imaging examinations, convulsion duration greater than 1 hour, CRP, and abnormal blood glucose were independent risk factors for poor prognosis in children. Therefore, the invasion of related risk factors could be reduced clinically by prognostic review with medical advice, attention to food safety and hygiene, and improvement of children's immunity.

Assessing efficacy and safety of rectally vs. intravenously administered Sibazon in treatment of generalized epileptic seizures in children

Objective: to prove the therapeutic equivalence and similar safety profile of “Sibazon, rectal solution” (international nonproprietary name: diazepam) and “Sibazon, solution for intravenous and intramuscular administration” in children with primary generalized and bilateral tonic, clonic and tonic-clonic seizures.Material and methods. An open-label, randomized clinical trial on efficacy and safety was conducted in 20 patients suffering from epilepsy with generalized seizures aged 1 to 17 years. Clinical blood and urine tests, biochemical blood analysis were used for diagnostics (glucose, total protein, albumin, total bilirubin, cholesterol, aspartate aminotransferase, alanine aminotransferase, creatine phosphokinase, alkaline phosphatase, creatinine, urea, creatinine clearance), as well as data on electrocardiographic (ECG) and electroencephalographic (EEG) studies. The patients were divided into two groups: in Group 1 (n=8), a rectal solution was used, in Group 2 (n=12) – a solution for intravenous and intramuscular administration.Results. The number of cases in which seizures were completed within 10 minutes after using the drug without resuming within subsequent 60 minutes, in Group 1 was 7 (87.5%), and in Group 2 – 9 (75.0%) (Fisher exact test (FET): p=0.617). Repeated primary generalized or bilateral tonic/clonic/tonic-clonic seizures within 24 hours after drug administration, in Group 1 were absent in 5 (62.5%) patients, in Group 2 – in 6 (50%) (FET: p=0.670); within 48 hours after drug administration – in 5 (62.5%) and 7 (58.3%) children, respectively (FET: p=1.00). Physical examination revealed no pathology in all patients at the final visit. While comparing ECG and EEG data at the final visit, no inter-group differences were found by the number of children with deviations from the norm. The results of laboratory studies confirmed that using the studied drugs had no negative effect on the main indicators of clinical and biochemical blood tests as well as clinical urine analysis.Conclusion. The effectiveness of the rectal form of Sibazon in relieving pediatric generalized epileptic seizures is comparable to that of Sibazon for intramuscular administration. The drug rectal form, due to easy-to-use administration, is preferable for outpatient practice. “Sibazon, rectal solution” is safe and has good tolerability.

Deficiency of the Lysosomal Protein CLN5 Alters Lysosomal Function and Movement.

Batten disease is a devastating, childhood, rare neurodegenerative disease characterised by the rapid deterioration of cognition and movement, leading to death within ten to thirty years of age. One of the thirteen Batten disease forms, CLN5 Batten disease, is caused by mutations in the CLN5 gene, leading to motor deficits, mental deterioration, cognitive impairment, visual impairment, and epileptic seizures in children. A characteristic pathology in CLN5 Batten disease is the defects in lysosomes, leading to neuronal dysfunction. In this study, we aimed to investigate the lysosomal changes in CLN5-deficient human neurons. We used an induced pluripotent stem cell system, which generates pure human cortical-like glutamatergic neurons. Using CRISPRi, we inhibited the expression of CLN5 in human neurons. The CLN5-deficient human neurons showed reduced acidic organelles and reduced lysosomal enzyme activity measured by microscopy and flow cytometry. Furthermore, the CLN5-deficient human neurons also showed impaired lysosomal movement—a phenotype that has never been reported in CLN5 Batten disease. Lysosomal trafficking is key to maintain local degradation of cellular wastes, especially in long neuronal projections, and our results from the human neuronal model present a key finding to understand the underlying lysosomal pathology in neurodegenerative diseases.

Seizures in myelin oligodendrocyte glycoprotein antibody-associated disorders and related immune factors

PurposeEpileptic seizures in myelin oligodendrocyte glycoprotein (MOG) antibody-associated disorders (MOGAD) have been increasingly reported in the past two decades. However, the characteristics and possible mechanism largely remain unknown. This study aimed to investigate the clinical characteristics and immune mechanism of epileptic seizures in children with MOGAD. MethodsWe conducted a retrospective single-center case-control study from February 2019 to February 2021. Clinical characteristics and immune parameters of enrolled patients with seizures (n = 12) in MOGAD (n = 32), as compared with the antibody-negative (n = 13) and control (n = 23) groups were then analyzed. ResultsSeizures occurred in 24.5% (12/49, including cases that were not enrolled) of MOGAD. In this group, magnetic resonance imaging (MRI) abnormalities, especially unilateral cortical lesion and leptomeningeal enhancement, were more frequent in patients with seizures than in individuals without seizures (p < 0.05). Levels of thyroid peroxidase antibodies (TPOAb) in the seizure+/MOG+ group were significantly (p < 0.05) higher than those in the seizure-/MOG+ and control groups, whereas no significant differences as compared with the seizure+/MOG- group were observed. TPOAb level and brain MRI performance may be associated with disease course, since two cases with increased TPOAb and abnormal brain MRI were reported, after a second seizure attack occurred. ConclusionUnilateral cerebral cortical encephalitis and isolated seizures, especially in clusters and focal seizures, may be special phenotypes of seizures in MOG-AD. TPOAb may be associated with seizures in immune-related neurological diseases, but not a specific marker of MOGAD. Therefore, TPOAb may be considered but not strongly recommended to be monitored in MOGAD.

Сучасний підхід до діагностики та лікування судом у новонароджених та дітей раннього віку

The article is devoted to the topical problem of neonatology and pediatric neurology — the diagnosis and treatment of seizures in newborns and young children. The work presents an algorithm for the diagnosis and treatment of epileptic seizures in children. It is indicated that the therapeutic effect of most antiepileptic drugs consists of modulation of voltage-gated and ligand-gated channels of membranes of neurons of the cerebral cortex, enhancement of inhibitory synaptic transmission or inhibition of activating synaptic transmission. The issues of the pharmacokinetics and pharmacodynamics of antiepileptic drugs are considered, taking into account the age characteristics of the child's body, in particular, newborns and early age children. The problems of drug interaction were discussed. The properties of individual antiepileptic drugs, which are used in newborns and young children, are considered. Emphasized are «polar» differences in the work of phenobarbital, which depend on the stage of epileptogenesis, namely: suppression of epileptiform activity at the early stage of epileptogenesis and its enhancement in the already formed epileptic focus (epileptic system). The literature data on the differentiated treatment of certain epileptic syndromes are presented. The issues on the prospects for the treatment of genetically determined diseases, which are accompanied by seizures associated with metabolic disorders, are considered. The modern high-tech methods of treatment of these diseases are noted. The role of diet therapy, co-factor therapy in the treatment of hereditary metabolic disorders, in particular, the ketogenic diet as a method of alternative treatment for drug-resistant epilepsy in children, is shown. No conflict of interest was declared by the authors. Key words: newborn, epilepsy, epileptic encephalopathy, treatment, antiepileptic drugs, review.

До питання вивчення судом у новонароджених та дітей раннього віку (особливості діагностики та клініко-генетичні характеристики епілептичних енцефалопатій)

The article is devoted to the urgent problem of neonatology and pediatric neurology — seizures in newborns and young children. In the work, a short review of the clinical and genetic characteristics of monogenic epilepsy is presented, in particular, the main attention is paid to the variants that begin in neonatal and early childhood. It has been shown that a significant number of epileptic encephalopathies are caused by mutations in genes whose protein products form voltage-dependent (sodium and potassium), ligan(dependent (γ-aminobutyric acid — GABA) channels, the functioning of which ensures the passage of a nerve impulse in neurons of the cerebral cortex. The necessity of including the molecular genetic methods into the algorithm for examining a child with epilepsy, in particular with epileptic encephalopathy, is emphasized. It is noted that congenital metabolic disorders are one of the etiological reasons for the development of epileptic seizures in children, in particular in newborns and young children. It was shown that congenital metabolic disorders have phenotypic manifestations of epileptic encephalopathy. Some curable metabolic defects that are accompanied by seizures, their diagnosis and timely treatment are described. No conflict of interest was declared by the authors. Key words: newborn, epilepsy, epileptic encephalopathy, diagnosis, genetic examination, metabolic defects, review.