BACKGROUND:Ataxia-telangiectasia (AT) is a rare disorder leading to multisystem involvement due to impaired DNA repair. Recent advances in targeted therapies highlight the importance of early diagnosis. This study aimed to evaluate clinical, radiological, and genetic variability in patients with AT and examine genotype-phenotype associations and diagnostic timing. METHODS:A retrospective study was conducted on genetically confirmed AT patients followed in four pediatric neurology centers in Türkiye over a 12-year period. RESULTS:A total of 21 patients with AT were included; the median age at symptom onset was 26 months, and the median age at diagnosis was 60 months, with a diagnostic delay of 21 months. Ocular telangiectasia was present in all patients. Ataxia, oculomotor apraxia, and dysarthria were other common features. Involuntary movements and strabismus were each observed in 52.4%. Cerebellar atrophy was noted in 57.1%, and decreased immunoglobulin levels in 95.2%. No significant differences in the frequency of clinical findings were observed between early and late diagnosis groups, except that recurrent respiratory tract infections were significantly more common in the early-diagnosis group (p = 0.035). CONCLUSION:In this study, clinical variability and key clinical features of AT were identified. AT should be suspected and immunoglobulin levels assessed in patients with neurological symptoms and recurrent infections. Improving recognition of AT may contribute to earlier diagnosis and better outcomes.
The aim of this study was to establish ultrasonography-based reference values for subarachnoid space width and to examine associations between anthropometric Z-scores and these measurements in healthy infants aged 0–3 months. This retrospective cross-sectional study included 125 healthy infants aged 0–3 months who underwent cranial ultrasound examinations. Subarachnoid space dimensions were measured bilaterally at the coronal level of the foramen of Monro, including craniocortical width (CCW), sinocortical width (SCW), and interhemispheric width (IHW). Age, body weight (BW), body height (BH), body mass index (BMI), body surface area (BSA), and head circumference (HC) Z-scores were analyzed for associations with subarachnoid space dimensions. Intraobserver reliability was evaluated using the intraclass correlation coefficient (ICC, two-way mixed-effects model). Mean values for CCW, SCW, and IHW increased progressively with age. Age was the only variable independently associated with CCW and SCW, while no significant anthropometric associations were found for IHW. Based on the 95th percentile, the proposed upper limits were 3.1 mm for CCW, 3.8 mm for SCW, and 4.2 mm for IHW. ICC values ranged from 0.93 to 0.95, indicating excellent intraobserver agreement. This study establishes ultrasonography-based normative data and describes growth-related associations for subarachnoid space width during the first 3 months of life. These findings provide a practical reference for interpreting subarachnoid space dimensions during early infancy.
OBJECTIVE:To evaluate the efficacy and tolerability of lacosamide (LCM) adjunctive therapy in a nationwide pediatric epilepsy cohort, including drug-refractory epilepsies (DREs). METHODS:A retrospective nationwide Turkish cohort study included 334 pediatric epilepsy patients (aged 1-18 years) treated with LCM adjunctive therapy. The population was divided into the two cohorts: (I) DREs with developmental and epileptic encephalopaties (DEEs); early-onset DEEs (group A) and late-onset DEEs (group B), and (II) DRE without DEEs: focal/generalized electroclinical syndromes (group C) and well-defined epilepsy syndromes (group D). The effectiveness of LCM adjunctive therapy was assessed by seizure outcome (seizure freedom, ≥50% reduction, <50% reduction, no change, or worsening) and EEG outcome (no change, <50% improvement, >50% improvement, complete improvement, or worsening). Tolerability was evaluated via drug retention rate and treatment-emergent adverse events (TEAEs). RESULTS:The mean duration of LCM adjunctive therapy was 24.63 ± 20.09 months in the cohort (mean age:13.28 ± 4.71 years; 53.8% male). The mean daily effective dose was 6.95 ± 2.25 mg/kg/day, and the time to effectiveness was 2.09 months. Etiological distribution differed across cohorts (p < 0.001), with structural etiology most frequent in groups C (47.1%) and A (34.4%), genetic in group A (22.9%), metabolic in groups A and B (6.3%), and immune/infectious in group D (13.6%). LCM adjunctive therapy provided sustained long-term seizure control in pediatric patients with DRE, with overall seizure control rates of 56.9% at 12 months and 55.6% at 24 months and a consistent responder rate (>50% seizure reduction) of 20.7% (n = 69/334). Cumulative EEG improvement was observed in 33.8% of patients receiving LCM adjunctive therapy, while complete EEG recovery rate was achieved in 6.3%. Similar seizure control rates was identified for DRE with DEEs (28.9%) and DRE without DEEs (35%). Drug retention was 73.9% at 12 months and 67.7% at 24 months, while TEAEs occurred in 20.7%, most commonly somnolence (10.4). CONCLUSIONS:LCM adjunctive therapy was associated with favorable electro-clinical outcomes and good tolerability across a broad spectrum of DREs with DEEs or without DEEs in childhood.
BACKGROUND:EEG abnormalities are frequently reported in children with developmental language disorders, particularly in language-associated regions, though existing data remain inconsistent. AIM:This study investigated the frequency, density, and localization of epileptiform discharges in children with language delays, and the incidence of epilepsy within a 12-month follow-up. METHOD:A total of 132 children with language delay were evaluated. Ten later diagnosed with autism spectrum disorder were excluded, leaving 122 for analysis. Epileptiform discharges were assessed for frequency, localization, and spike-wave index (SWI). Clinical, demographic, neuroimaging, and developmental data were collected. Comparative analyses were performed between children with and without epileptiform discharges, and between those who did and did not develop epilepsy. RESULTS:The mean age at admission was 39.6 months, with boys comprising 73.8% of the cohort. Epileptiform discharges were present in 14 children (11.5%), predominantly in the temporoparietal region (83.3%), mostly right-sided or bilateral. Notably, no patient exhibited unilateral discharges on the left. Epilepsy developed in six children during follow-up, all of whom had epileptiform discharges on initial EEG. None with a normal baseline EEG developed epilepsy. Age, sex, parental consanguinity, or family history of language delay, epilepsy, or febrile seizures showed no significant differences between groups. Although SWI values increased significantly over one year, neither baseline SWI nor its temporal change was associated with the development of epilepsy. CONCLUSION:EEG abnormalities and epilepsy are not rare in children with language delay. Those with epileptiform discharges on initial EEG require careful monitoring, as they carry a higher risk of developing epilepsy.
Objective This study aims to evaluate the efficacy, safety and impact on growth of ketogenic diet therapy (KDT) in infants under two years of age with drug-resistant epilepsy. Methods A retrospective analysis was conducted on 36 infants who received KDT. Demographic data, seizure frequency, adverse effects, dietary adherence, and growth metrics were assessed at baseline and during follow-up intervals. Results In the intent-to-treat analysis, all patients who initiated the ketogenic diet were included, and the responder rates at 1, 3, 6, 12, 18, and 24 months were 52.8%, 58.3%, 58.3%, 52.8%, 38.9%, and 36.1%, respectively. Among those continuing KDT, one-third remained seizure-free at Month 24. Metabolic etiologies showed higher early responsiveness. Dyslipidemia was the most frequent adverse event but improved significantly over time. Adverse effects such as nephrolithiasis and gastrointestinal symptoms were common but manageable. Growth demonstrated a mild but statistically significant decline in Z-scores. Medication burden decreased over time, and 23.3% of patients who maintained long-term adherence ultimately became medication-free. Conclusion KDT is an effective and generally well-tolerated treatment for drug-resistant epilepsy in infants, providing meaningful seizure control, reduced medication use, and an acceptable safety profile when carefully monitored.
BACKGROUND:Duchenne muscular dystrophy (DMD) is an inherited neuromuscular disorder causing progressive degeneration of skeletal and cardiac muscles due to dystrophin deficiency. Cardiac involvement ranges from mild to severe, including heart failure, arrhythmias, conduction defects, and sudden cardiac death. OBJECTIVE:This study aimed to evaluate early markers of cardiac repolarization abnormalities in DMD patients by analyzing standard 12‑lead ECG parameters-QT interval, corrected QT (QTc), T peak to T end (Tp-e), Tp-e/QT, and Tp-e/QTc ratios-and microvolt T-wave alternans (MTWA) from 24-h Holter monitoring. METHODS:Seventy-four individuals participated: 39 DMD patients and 35 age- and sex-matched healthy controls. Data on demographics, ambulation status, ECG, and Holter recordings were collected and compared. All participants underwent standard 12‑lead electrocardiography and 24-h Holter electrocardiogram monitoring. ECG measurements were performed manually by a blinded operator using standardized techniques. Holter recordings were collected and compared (analyzable data obtained from 33 patients and 34 controls). The QTc interval was calculated using Bazett's formula. Correlation analyses and statistical comparisons between groups were performed using appropriate parametric and non-parametric tests. RESULTS:DMD patients exhibited significantly higher resting heart rates. The Tp-e interval was similar between groups, while the QT interval was significantly shorter in the patient group, the QTc interval showed no significant difference between groups. Tp-e/QT ratio was higher in the patient group; Tp-e/QTc ratios showed no difference. The QTc interval was significantly prolonged in the non-ambulatory DMD group. MTWA values did not differ significantly between groups. Due to the limited sample size and absence of established pediatric reference values, MTWA findings must be considered inconclusive. CONCLUSION:In conclusion, our study reveals subtle repolarization alterations in DMD patients, including elevated resting heart rate and a trend toward increased Tp-e/QT ratio. While these findings do not yet establish a definitive arrhythmic phenotype, they suggest the presence of early electrophysiological changes that may warrant longitudinal cardiac evaluation. Prospective follow-up studies are essential to determine the prognostic significance of these parameters and their relationship to clinical arrhythmic outcomes in the pediatric DMD population.
Spinal muscular atrophy (SMA) Type 1 in infants with two SMN2 copies is characterised by rapid motor neuron loss and a historically fatal course if untreated; however, nationwide real-world comparative data evaluating screening efficacy remain scarce. This nationwide retrospective, multicentre study evaluated the real-world impact of Turkey's national newborn screening (NBS) program on clinical outcomes in a high-risk population-of infants with genetically confirmed SMA and two SMN2 copies-treated with nusinersen. Patients were classified into a historical symptomatic cohort diagnosed before NBS implementation (pre-NBS, n = 162) and an NBS cohort identified through screening (n = 96); all received nusinersen. Outcomes included survival, respiratory and nutritional independence, and acquisition of WHO-defined motor milestones. Motor function was evaluated using the Children's Hospital of Philadelphia-Infant Test of Neuromuscular Disorders (CHOP-INTEND) scale. The NBS program markedly reduced the mean age at diagnosis and treatment initiation. Mortality was 6.3% in the NBS cohort compared with 30.9% in the pre-NBS group (p < 0.001). CHOP-INTEND scores at baseline were higher in the NBS cohort. Sustained motor gains were confirmed in both cohorts, though the NBS cohort maintained superior function throughout follow-up; 48.3% of NBS-identified infants achieved independent walking, compared with only 8.6% in the pre-NBS group. Preservation of bulbar and respiratory function was superior in the NBS cohort, whereas the pre-NBS group demonstrated progressive decline. NBS program fundamentally altered the clinical trajectory of SMA Type 1 in infants with two SMN2 copies in the present cohort. Pre-symptomatic treatment in the NBS cohort supports sustained motor development and preserves bulbar and respiratory functions, underscoring the importance of minimising delays between birth, diagnosis, and treatment initiation.
Background: TRACK syndrome, first described by Canpolat et al., is a rare disorder caused by splice-site mutations in TSEN2, characterized by craniofacial malformations, central nervous system (CNS) anomalies, progressive kidney disease. Case Presentation: We present a 2.5-year-old boy, third child of consanguineous parents, admitted with fever and cough, diagnosed with atypical hemolytic uremic syndrome (aHUS). Whole exome sequencing revealed a homozygous intronic TSEN2 variant (c.1100-5T>A), consistent with TRACK syndrome. Two deceased siblings had similar dysmorphic features and early-onset renal failure. The proband exhibited microcephaly, craniofacial dysmorphism, left atrial dilation, and laboratory findings consistent with thrombotic microangiopathy (TMA). Despite pathogenic TSEN2 variant, he’d normal intellectual and motor development. He’s currently 6 years old, undergoing peritoneal dialysis, receiving biweekly eculizumab, and antiepileptic therapy. Conclusion: This report highlights a rare cause of aHUS and end-stage kidney disease (ESKD) due to a TSEN2 splice-site mutation, further expanding the phenotypic spectrum of TRACK syndrome.
BACKGROUND:Ketogenic diet (KD) is an established treatment option for pediatric drug-resistant epilepsy; however, early indicators of treatment response are limited. This study aimed to explore whether early changes in routine electroencephalographic (EEG) findings, particularly the interictal epileptiform discharge index (IED index), are associated with subsequent clinical response to KD therapy. METHODS:We conducted a single-center retrospective cohort study including children with drug-resistant epilepsy who initiated a classical ketogenic diet. Standardized natural sleep EEG recordings were obtained at baseline and approximately one month after diet initiation. IED ındex was calculated during the first five minutes of non-rapid eye movement sleep, and background activity was visually assessed using a standardized grading scale. Clinical response was defined as a ≥50% reduction in seizure frequency or seizure freedom at six months. EEG changes were compared between responders and non-responders, and receiver operating characteristic analysis was performed to evaluate the association between early EEG changes and clinical outcome. RESULTS:Thirty-four patients were included, of whom 23 (67.6%) were classified as responders at six months. Baseline EEG characteristics did not differ between responders and non-responders. At one month, responders showed a significant reduction in IED index, whereas no significant change was observed in non-responders. A reduction in IED index of approximately 40% was associated with clinical response, demonstrating good discriminative performance. Changes in background EEG activity were observed in both groups and were not associated with seizure outcome. CONCLUSION:Early reduction in IED index on routine sleep EEG is associated with favorable clinical response to the ketogenic diet. Routine EEG assessment may offer supportive electrophysiological information during early follow-up of KD therapy in pediatric drug-resistant epilepsy.
Background: X-linked intellectual disability (XLID) is a clinically and genetically heterogeneous disorder. In this study, we aimed to describe the clinical and molecular spectrum of patients with XLID. We also evaluated the clinical efficacy of a targeted gene panel in patients with suspected XLID. Methods: Eighty-four patients with suspected XLID were enrolled in the study. Array comparative genomic hybridization, fragile X fragman analysis, and targeted XLID gene panel were performed. Results: Genetic diagnosis was established in a total of 24 patients (22 male and two female) with XLID. Different copy number variations of the X chromosome were detected in four patients, including two duplications and two deletions. Fifteen patients had fragile X syndrome. Point mutations were detected in five unrelated patients. Variants detected in RPS6KA3 gene were previously reported by our team. A novel two-nucleotide deletion was shown in the MID1 gene. Additionally, novel missense variations were revealed in IL1RAPL1 and ATRX genes. The IL1RAPL1 variant was detected in additional five affected male patients in the same family. The patient, who had ATRX variation, had pachygyria in the cerebral cortex and hypoplasia of cerebellar vermis. Conclusions: Our findings have broadened the spectrum of mutations and clinical manifestations of patients with XLID. Additionally, this represents the second reported missense variation in the IL1RAPL1 gene identified in patients with XLID. We also emphasized the importance of a stepwise diagnostic algorithm that incorporates chromosomal microarray analysis, FMR1 gene repeat analysis, and next- generation sequencing analysis for patients with XLID. (c) 2025 Elsevier Inc. All rights are reserved, including those for text and data mining, AI training, and similar technologies.
Introduction: Children with chronic neurological diseases such as spinal muscular atrophy (SMA) are at high risk of incomplete or delayed vaccination. This study aimed to assess the age-appropriate vaccination status of SMA patients and evaluate related factors in case of a mismatch. Methods This multicenter, cross-sectional survey was conducted in 13 pediatric neurology clinics across Türkiye. Clinical characteristics, concomitant diseases, and vaccination status according to the National Immunization Program (NIP) were analyzed. Additionally, the rates of non-NIP vaccines (rotavirus, influenza, meningococcal, and HPV) were investigated, and influenza vaccine recommendations were correlated with vaccination rates. Results A total of 315 SMA patients (54.6% male) with a mean age of 7 ± 5.2 years were included. SMA type distribution was 42.5% type 1, 32.7% type 2, and 24.8% type 3. Nusinersen was administered to 95.8% of patients. Age-appropriate vaccination rates according to the NIP ranged from 81.6% (hepatitis A) to 98.7% (BCG). However, the rates for non-NIP vaccines were significantly lower: rotavirus 8.9%, meningococcal 4.2%, and HPV 0.3%. Although 78.7% of patients were recommended the influenza vaccine, the administration rate was only 7.6%, with a significant decline during the COVID-19 pandemic (p = 0.008). The most common reason for incomplete vaccination was prolonged hospitalization due to intensive treatment, while parental vaccine refusal was 1.8%. Conclusion Vaccination rates were low in this nationwide SMA cohort. Well-structured vaccine programs should be implemented to ensure adherence to the NIP schedule and improve non-NIP vaccine uptake.
BackgroundSMA Type 1 is the most severe form of spinal muscular atrophy with early symptom onset, limited motor development, and poor prognosis. Recent genetic-based therapies, such as nusinersen, have transformed disease outcomes. We aimed to evaluate the long-term effects of nusinersen on motor, bulbar, and respiratory functions in both symptomatic and presymptomatic SMA Type 1 patients over a period of up to 4 years.MethodsThis prospective, non-interventional study included 310 patients with genetically confirmed spinal muscular atrophy at 24 pediatric neurology centers in Turkey. Patients treated with nusinersen were divided into five age-based cohorts at treatment initiation: Cohort A (0–3 months), Cohort B (4–6 months), Cohort C (7–12 months), Cohort D (13–24 months), and Cohort E (>24 months). Efficacy was assessed using the CHOP-INTEND and WHO Motor Milestone Scale. This study also analyzed the respiratory support needs, gastrostomy requirements, and mortality rates across cohorts.ResultsPatients treated before 12 months of age showed the most significant improvements in motor milestones, with 58.7% of Cohort A achieving independent sitting. CHOP-INTEND scores increased notably in all cohorts, with the largest improvement observed in Cohort A (93.5%). Ventilator and gastrostomy requirements decreased in the early treated cohorts. Adverse events were rare, with one discontinuation due to hydrocephalus. The overall mortality rate was 21.3%, with most of the deaths occurring within the first year.InterpretationNusinersen treatment initiated before 12 months of age, especially before 3 months of age, yielded the most favorable motor outcomes in patients with SMA type 1. Early initiation is associated with improved motor milestones and reduced need for ventilatory support. However, no significant improvements were observed in the bulbar function or in patients requiring extensive respiratory support.
ObjectivesTo evaluate the demographic, clinical, laboratory, and prognostic data of children with acute disseminated encephalomyelitis with respect to anti-myelin oligodendrocyte glycoprotein (MOG) antibody status.MethodsAcute disseminated encephalomyelitis patients (n = 245) from 24 centers followed up between 2010 and 2022 were evaluated retrospectively. The short- and long-term outcome characteristics (disease severity and course, clinical relapse, and recovery rates) were assessed. Incomplete clinical recovery was defined as modified Rankin Score ≥1 or the presence of epilepsy. Univariant and multivariant analysis were performed for outcome characteristics.ResultsThe mean age at diagnosis was 6.3 ± 3.8 (0.5-17.7) years and the median follow-up was 22 (3-132) months. The outcome characteristics were evaluated in 180 of 245 patients (73.4%) with at least 12 months' follow-up. Twenty-three patients (12.6%) relapsed. The multivariable logistic regression analysis revealed the following clinical parameters as predictors of relapse: sex, visual impairment, and ataxia at initial presentation. Incomplete clinical recovery (n = 42/180, 23.3%) was associated with the presence of seizures on admission and the need for an intensive care unit. Anti-MOG antibody positivity was not associated with an increased risk of relapse (25% vs 13.1%, P = .164) or incomplete clinical recovery (P = .511).ConclusionThe nationwide cohort presented further supports the typically monophasic nature of acute disseminated encephalomyelitis, and a high rate of complete recovery. The presence of certain symptoms in the acute period may assist the clinician in estimating the outcome.
Febrile infection-related epilepsy syndrome (FIRES) is a subtype of new-onset refractory status epilepticus characterized by refractory status epilepticus following a febrile infection. It is associated with high morbidity and mortality, particularly in children and adults. However, its etiology often remains unclear. The ketogenic diet therapy (KDT), a proven treatment method for drug-resistant epilepsy, continues to be investigated for its mechanisms and efficacy. This case report presents an 11-year-old male diagnosed with FIRES, successfully managed through a multidisciplinary approach and KDT. The patient initially received oral antibiotics for an upper respiratory tract infection but later developed altered consciousness and drowsiness, raising suspicion of encephalitis. He was transferred to the intensive care unit, where refractory status epilepticus occurred 11 days after the onset of fever. Given the clinical presentation, brain magnetic resonance imaging findings, and cerebrospinal fluid analysis consistent with autoimmune encephalitis, the patient was treated with intravenous immunoglobulin, pulse steroid therapy, and plasmapheresis. Despite these interventions, seizure activity persisted, prompting initiation of a classic ketogenic diet at a 3:1 ratio. Seizure activity ceased by the eighth day of KDT. Long-term follow-up revealed that the patient remained seizure-free without antiepileptic drugs, with improved electroencephalography findings. This case highlights the potential role of the ketogenic diet in the early diagnosis and treatment of FIRES. KDT, in combination with immunotherapy, has demonstrated effectiveness in controlling seizures during the acute phase and achieving long-term seizure-free outcomes.
Background The subarachnoid space is a clinically important anatomical region among various pathologies including subdural effusions, hydrocephalus, and benign enlargement of the subarachnoid space. Therefore, accurate measurement regarding subarachnoid space dimensions is necessary in terms of early diagnosis as well as clinical follow-up. Reference data remain limited and show variability among ethnic and geographical groups although several studies reported normative values for subarachnoid space width in different populations. Up until now, there are no ultrasonography-based normative data that have been established in relation to either age or sex. Head circumference (HC) and Z-scores for body weight (BW), body height (BH), body mass index (BMI), and body surface area (BSA) are applicable during the first three months of life. Objective To establish ultrasonography-based normative reference values for subarachnoid space width in healthy infants during the first three months of life. Materials and methods A total of 125 infants aged 0 to 3 months underwent cranial ultrasound examinations. Subarachnoid space measurements—including craniocortical width (CCW), sinocortical width (SCW), interhemispheric width (IHW), and cisterna magna width (CMW)—were assessed. These measurements were compared with infants’ age, HC, and Z-scores for BW, BH, BMI, and BSA. Age-specific 5th, 50th, and 95th percentiles of the subarachnoid spaces were generated. Independent determinants in subarachnoid space width were identified through linear regression analysis. Results The mean CCW and SCW were 1.72 ± 0.67 mm (range, 0.70–4.40) and 2.45 ± 0.78 mm (range, 0.90–4.90), respectively. The mean IHW and CMW were 2.37 ± 0.94 mm (range, 0.50–5.10) and 3.38 ± 1.23 mm (range, 1.20–6.80), respectively. All subarachnoid space measurements increased significantly with age. CCW and SCW were significantly associated with the Z-scores for BW, BMI, and BSA, while HC was positively correlated with IHW and CMW. Linear regression analysis revealed that age was the only independent predictor of CCW and SCW, whereas both age and HC significantly predicted CMW. Conclusion We report the first national ultrasound-based normative data for subarachnoid space width in healthy infants aged 0–3 months, which may serve as a valuable reference in routine pediatric practice.
Background: Seizure -related self -efficacy is the belief individuals have that they can perform the necessary actions to cope effectively with their seizures. Determining, developing, strengthening, and maintaining the perception of self -efficacy in children with epilepsy facilitates the child 's disease management and their ability to cope with it. This study aimed to assess the impact of epilepsy -related parental fears during the COVID19 period on the seizure self -efficacy of their children. Methods: A total of 321 children with epilepsy and their parents participated in this descriptive, correlational, and cross-sectional study. Data were collected through the Descriptive Information Form, the Seizure SelfEfficacy Scale for Children (SSES-C), and the Epilepsy -Related Fears in Parents Questionnaire (EFPQ). Descriptive statistics, including frequency, percentage, and mean scores, were used to analyze the characteristics of the children and their parents. The Shapiro -Wilk test was utilized to assess the normality of the scale data. Pearson correlation analysis examined the relationship between parents ' epilepsy -related fears and their children 's seizure self -efficacy, while multiple regression analysis determined the effect of parental fears on children 's seizure self -efficacy. Results: The mean age of children included in the study was 12.65 2.37 years. Analysis revealed a strong and significant negative correlation between parents ' epilepsy -related fears during the COVID-19 period and the seizure self -efficacy of their children. In the model created with regression analysis, The mean scores of parents on the short-term fears of parents about epilepsy of the EFPQ explained 85 % of children 's seizure self -efficacy. The mean scores of parents on the long-term fears of parents about epilepsy of the EFPQ explained 85 % of children 's seizure self -efficacy. It was determined that all of these variables together explained 85 % of the seizure self -efficacy of children with epilepsy. Conclusion: The findings of the study underscore the importance of addressing parents ' fears regarding epilepsy, emphasizing the need for healthcare professionals to be aware of and provide support for these concerns. Future studies should focus on interventions to enhance the seizure self -efficacy of children with epilepsy. (c) 2024 French Society of Pediatrics. Published by Elsevier Masson SAS. All rights reserved.