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Background. The problem of antiepileptic therapy in children remains highly relevant due to the insufficient efficacy of standard drugs, adverse events, and limitations of clinical guidelines.Aim. To assess the efficacy, tolerability, and continuation of zonisamide (Zonegran) therapy in children with epilepsy.Materials and methods. This retrospective multicenter study was conducted in epileptology centers in various regions of the Russian Federation, including Moscow, Novosibirsk, Krasnodar, Tyumen, Krasnoyarsk, Voronezh, Ryazan). We included 340 patients under the age of 18 (202 boys and 138 girls) with various epileptic syndromes receiving zonisamide (mean age was 10.63 years). Zonisamide was administered to patients with the following epileptic syndromes: age-dependent self-limited focal epilepsy of childhood (n = 49; 14 %) structural focal epilepsy (n = 102; 30 %), focal epilepsy of childhood with structural brain changes and benign epileptiform discharges of childhood visualized at electroencephalography (n = 38; 11.2 %), idiopathic generalized epilepsy (n = 23; 6.7 %), developmental and epileptic encephalopathies (n = 44; 12.9 %) (including developmental and epileptic encephalopathies with spike-and-wave activation during sleep (n = 23; 6.7 %)), Lennox–Gastaut syndrome and infantile epileptic spasms syndrome (n = 32; 9.4 %), genetic epilepsy with generalized and focal seizures (n = 81; 23.8 %). The type of the seizures was distributed as follows: focal motor (n = 205), bilateral tonic-clonic (n = 192), generalized (n = 197), including generalized convulsive (n = 44), tonic (n = 70), atonic (n = 2), myoclonic (n = 26), epileptic spasms (n = 36), absence seizures (n = 19). One patient could have more than one type of seizures. Magnetic resonance imaging demonstrated some changes in 185 patients, whereas 117 patients had no magnetic resonance imaging changes. Thirty-eight patients did not undergo magnetic resonance imaging as they had no indications to it. The most common structural epileptogenic changes were the consequences of perinatal lesions manifesting as cystic-gliotic transformation, atrophy (125 cases in total); less common changes included focal cortical dysplasia, developmental abnormalities (such as polymicrogyria, lissencephaly, holoprosencephaly), mesial temporal sclerosis, and tumors. Zonegran (zonisamide) as an additional therapy was administered at a dose of 3–8 mg/kg/day (mean dose 5 mg/kg/day) to 321 patients.Results. Zonegran (zonisamide) therapy was effective in 257 (75.6 %) of patients; remission was achieved in 174 patients (67.7 %), while reduced seizure frequency (50 % and greater) was registered in 83 patients (32.3 %). A total of 72 patients (21.2 %) reported no adverse events. Twenty participants (5.8 %) had some adverse events; in 4 cases, they were resolved after dosage decrease; in 16 cases, treatment with zonisamide was discontinued.Conclusion. Zonegran (zonisamide) is effective for comprehensive treatment of both focal and generalized epilepsy in children*.

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. [ABSTRACT FROM AUTHOR]

Xiaokun Wu,1,&ast; Shu Cai,1,&ast; Ye Zhou,2 Yutao Lan,1 Yan Lin3 1School of Nursing, Guangdong Pharmaceutical University, Guangzhou, People’s Republic of China; 2Department of Nursing, Luzhou Traditional Chinese Medicine Hospital, Luzhou, People’s Republic of China; 3Department of Nursing, Guangzhou Women and Children’s Medical Center, Guangzhou, People’s Republic of China&ast;These authors contributed equally to this workCorrespondence: Yutao Lan, Guangdong Pharmaceutical University, 283 Jianghai Avenue, Haizhu Distric, Guangzhou, People’s Republic of China, Tel +86 13710667351, Email lanyutao@gdpu.edu.cnPurpose: This study aimed to develop a medication literacy scale for parents of children with epilepsy (MLSPCE) and to test the reliability and validity of the scale.Patients and Methods: The pilot scale was formulated based on the concept of medication literacy, the knowledge-attitude-practice model, and relevant literature reviews. It was formed through two rounds of expert consultations using the Delphi method. A survey of 657 parents of children with epilepsy, who were admitted to the neurology department or examined in the neuro-electrophysiological outpatient department of Guangzhou Women and Children Medical Center, using the pilot scale was conducted from October 2021 to January 2022 to test the reliability and validity of the scale questionnaire. The content validity of the scale questionnaire was assessed by consulting 20 neurology nursing, neurology clinician, and nursing education experts. Numbers, percentages, t-test, correlation analysis, Cronbach’s alpha reliability coefficient and factor analysis were used for data analysis.Results: The MLSPCE included 34 items in four dimensions. Ten factors were drawn from the explorative factor analysis, with a cumulative variance contribution rate of 62.32%. The content validity index of each item on the 34-item scale was between 0.81 and 1.0, and the scale-content validity index/ average was 0.97. The correlation coefficient between each item and its dimension was between 0.399 and 0.760, the correlation coefficients between dimensions were between 0.150 and 0.382, and the correlation coefficients between each dimension and the total scale were between 0.390 and 0.845. Differences for all comparisons were statistically significant (P < 0.05). Cronbach’s alpha coefficient for the total scale was 0.864, and the split-half reliability of the total scale was 0.923.Conclusion: All the statistical procedures performed in the validity and reliability stages of the study showed that MLSPCE is a valid and reliable tool for measuring medication literacy among Chinese parents of children with epilepsy.Keywords: epilepsy in children, medication literacy, scale development, reliability test, validity test

Williams syndrome is a multisystem disorder characterized by developmental delay, characteristic facial features, growth abnormalities, and cardiovascular abnormalities. The disorder is an autosomal dominant genetic syndrome that occurs due to microdeletion at chromosomal locus 7q11.23. Seizures occur uncommonly in association with Williams syndrome. Mutations in the  TNK2  gene have been found in rare cases of autosomal recessive infantile-onset epilepsy. We describe a rare co-occurrence of Williams syndrome and  TNK2  gene-related epilepsy in a child born of consanguineous parents. This case report emphasizes the role of genetic testing in the diagnosis of rare diseases. This is the fourth case report of epilepsy with biallelic mutations in the  TNK2  gene, to the best of the authors' knowledge., Competing Interests: Human subjects: Consent was obtained or waived by all participants in this study. Aster CMI Hospital Institutional Ethics Committee issued approval IEC Letter no Aster/IEC/Other/001/2023-24. This case report was considered and approved under “exempt” category by institutional ethics committee. Conflicts of interest: In compliance with the ICMJE uniform disclosure form, all authors declare the following: Payment/services info: All authors have declared that no financial support was received from any organization for the submitted work. Financial relationships: All authors have declared that they have no financial relationships at present or within the previous three years with any organizations that might have an interest in the submitted work. Other relationships: All authors have declared that there are no other relationships or activities that could appear to have influenced the submitted work., (Copyright © 2024, Angel et al.)

Advances in autoimmune encephalitis studies in the past 10 years have led to the identification of new syndromes and biomarkers that have transformed the diagnostic approach to the disorder. The disorder or syndrome has been linked to a wide variety of pathologic processes associated with the neuron-specific autoantibodies targeting intracellular and plasma membrane antigens. However, current criteria for autoimmune encephalitis are quite dependent on antibody testing and responses to immunotherapy, which might delay the diagnosis. This form of encephalitis can involve the multifaceted presentation of seizures and unexpected behavioral changes. The spectrum of neuropsychiatric symptoms in children is less definitive than that in adults, and the incorporation of clinical, immunological, electrophysiological, and neuroradiological results is critical to the diagnostic approach. In this review, we document the clinical and immunologic characteristics of autoimmune encephalitis known to date, with the goal of helping clinicians in differential diagnosis and to provide prompt and effective treatment.

Background: Abnormal levels of glutamate constitute a key pathophysiologic mechanism in epilepsy. The use of glutamate chemical exchange saturation transfer (GluCEST) imaging to measure glutamate levels in pediatric epilepsy is rarely reported in research., Purpose: To investigate hippocampal glutamate level variations in pediatric epilepsy and the correlation between glutamate and hippocampal subregional volumes., Study Type: Cross-sectional, prospective., Subjects: A total of 38 school-aged pediatric epilepsy patients with structurally normal MRI as determined by at least two independent radiologists (60% males; 8.7 ± 2.5 years; including 20 cases of focal pediatric epilepsy [FE] and 18 cases of generalized pediatric epilepsy [GE]) and 17 healthy controls (HC) (41% males; 9.0 ± 2.5 years)., Field Strength/sequence: 3.0 T; 3D magnetization prepared rapid gradient echo (MPRAGE) and 2D turbo spin echo GluCEST sequences., Assessment: The relative concentration of glutamate was calculated through pixel-wise magnetization transfer ratio asymmetry (MTR asym ) analysis of the GluCEST data. Hippocampal subfield volumes were computed from MPRAGE data using FreeSurfer., Statistical Tests: This study used t tests, one-way analysis of variance, Kruskal-Wallis tests, and Pearson correlation analysis. P < 0.05 was considered statistically significant., Results: The MTR asym values of both the left and right hippocampi were significantly elevated in GE (left: 2.51 ± 0.23 [GE] vs. 2.31 ± 0.12 [HCs], right: 2.50 ± 0.22 [GE] vs. 2.27 ± 0.22 [HCs]). The MTR asym values of the ipsilateral hippocampus were significantly elevated in FE (2.49 ± 0.28 [ipsilateral] vs. 2.29 ± 0.16 [HCs]). The MTR asym values of the ipsilateral hippocampus were significantly increased compared to the contralateral hippocampus in FE (2.49 ± 0.28 [ipsilateral] vs. 2.35 ± 0.34 [contralateral]). No significant differences in hippocampal volume were found between different groups (left hippocampus, P = 0.87; right hippocampus, P = 0.87)., Data Conclusion: GluCEST imaging have potential for the noninvasive measurement of glutamate levels in the brains of children with epilepsy., Level of Evidence: 2 TECHNICAL EFFICACY: Stage 1., (© 2024 International Society for Magnetic Resonance in Medicine.)

Study Question: Is maternal use of hormonal contraception associated with the development of epilepsy in the offspring?Summary Answer: We found that maternal use of hormonal contraception was associated with a slightly increased risk of epilepsy in the offspring.What Is Known Already: Foetal exposure to exogenous hormones has been associated with changes in brain development. However, little is known about maternal hormonal contraception use and development of epilepsy in the offspring.Study Design, Size, Duration: A nationwide cohort of all live born children born in Denmark between 1 January 1998 and 31 December 2014, was followed from day 29 after birth for epilepsy (first diagnosis of epilepsy or first redeemed prescription for anti-epileptic medication) to censoring (emigration, death) or 31 December 2015, whichever occurred first.Participants/materials, Setting, Methods: Diagnoses of epilepsy were obtained from the National Patient Registry. The Danish National Prescription Registry supplied information on redeemed prescriptions for hormonal contraception and anti-epileptic medication. Maternal hormonal contraception use was categorized as never use (reference group), previous use (prescriptions redeemed >3 months before pregnancy start) and recent use (prescriptions redeemed ≤3 months before or during pregnancy).Main Results and the Role Of Chance: The data show that 17 585 children developed epilepsy during a median follow-up of 9.2 years (9 732 635 person-years). The hazard ratio (HR) for epilepsy was 1.07 (95% CI 1.02-1.13) in children of mothers who had used any type of hormonal contraception recently, compared with children of mothers who had not used hormonal contraception. The HR was similar for recent use of oral combined products, while the HRs for recent or previous use of non-oral combined products were 1.32 (95% CI 0.98-1.77) and 1.16 (95% CI 1.02-1.32), respectively. For non-oral progestin-only products, the HRs were 1.19 (95% CI 1.04-1.38) and 1.53 (95% CI 1.31-1.80), respectively, for recent and previous use.Limitations, Reasons For Caution: There may be some misclassification of maternal hormonal contraception use, as some women may not have used the redeemed prescriptions or used them at a different point in time; potentially leading to an attenuation of the estimates. In addition, although we were able to account for known risk factors for epilepsy, unknown or residual confounding cannot be ruled out.Wider Implications Of the Findings: Our findings are based on nationwide population-based data and can therefore be applied to other similar populations. However, as this is the first study in this field, further studies are needed to confirm our findings.Study Funding/competing Interest(s): No specific funding was obtained for this study, which was supported by internal funding at the Unit of Virus, Lifestyle and Genes. All authors report no conflicts of interest.Trial Registration Number: N/A. [ABSTRACT FROM AUTHOR]

There are considerable variations in the reported incidence of early and late epilepsy in children with lymphoproliferative diseases. The etiology of seizures in pediatric patients with hemoblastosis during polychemotherapy can be diverse: dysmetabolic, infectious, toxic, ischemic, posthemorrhagic. The treatment strategy selected by the neurologist largely depends on the causes of the paroxysmal conditions. When choosing the anti-seizure therapy one should consider the course of hemoblastoses and the possible effects of polychemotherapy. The article presents pediatric cases and the analysis of acute lymphoblastic leukemia with developing epileptic seizures.

Hemiconvulsion-hemiplegia-epilepsy (HHE) syndrome is a rare consequence of focal status epilepticus (SE) in childhood. The major discerning feature of the syndrome is the unilateral brain lesion during SE with subsequent development of hemiparesis and drug-resistant epilepsy. The unilateral brain damage in SE is not fully understood. We hypothesized that comorbidity could be the cause of this the hemisphere damage; to that end, we searched for the evidence of comorbidity in patients with HHE.Objective: to analyze the concomitant pathology in patients with HHE, which was the possible cause of unilateral brain damage.Materials and methods. Four patients with HHE syndrome (aged from 1 year 2 months to 6 years; three boys and a girl) were examined.Results. The course of the disease in all patients met the criteria of HHE; various manifestations of comorbidity were found in all four patients. Thus, patient A.A. (6 years old) had hypothyroidism, the history of consciousness loss, acetone smell from the mouth, and spontaneous generalized seizure attacks starting from the age of 11 months. Patient V.S. (6 years old) had adrenogenital syndrome and inborn glaucoma. Patient D.Sh. (1 year 10 months old) had a structural heart defect, a high degree of stigmatization, and suspected Wolf-Hirschhorn syndrome. Patient V.Kh. (3 years old) had cerebral palsy and post-hemorrhagic occlusion hydrocephaly.Conclusion. We propose that comorbidity can be the cause of hidden or obvious lesions in one hemisphere, which can explain the unilateral brain damage in HEE syndrome.

Byline: Ashfak. Mandli, Neelu. Desai, Rahul. Badheka, Vrajesh. Udani Aims: We aimed to study the frequency, age, and gender distribution of paroxysmal nonepileptic events (PNEs) in children referred to epilepsy [...]

While short-term memory (STM) and working memory (WM) are understood as being crucial for learning, and children with epilepsy often experience learning difficulties, little is known about the age-related development of memory span tasks in children with epilepsy. Short-term memory and WM, operationalized as digit span forwards (DSF) or digit span backwards (DSB), respectively, were studied. Participants were 314 children with epilepsy and 327 typically developing children in ages between 5 and 15 years and full scale intelligence quotient (FS-IQ) ≥ 75. Cross-sectional analyses of the data were done with analyses of variance and analyses of covariance ((M)ANCOVAs) and generalized linear analyses. The analyses revealed that STM problems in epilepsy were mediated by age-related gains in WM as well as by differences in IQ. Working memory developed at a quick pace in the younger children, the pace slowed down to some extent in the later primary school years and resumed again later on. Working memory problems prevailed in epilepsy, independent of IQ and development of STM. Timing of the epilepsy in terms of age at onset and duration determined memory development. The youngest children with epilepsy showed age-appropriate development in STM but were the most vulnerable in terms of WM development. Later in the course of the epilepsy, the WM problems of the young children attenuated. In later onset epilepsy, WM problems were smaller but persisted over time. [ABSTRACT FROM AUTHOR]