
Purpose We characterized anti-seizure medication (ASM) prescribing during hospitalization and at discharge in critically ill children undergoing continuous electroencephalography (CEEG) and identified clinical and electrographic factors associated with inpatient ASM administration and discharge prescribing. Methods This prospective cohort included consecutive children with acute encephalopathy undergoing CEEG in a pediatric intensive care unit, excluding those with pre-existing epilepsy. ASM use during hospitalization and prescribing at discharge were evaluated. Results Among 705 patients, 54% received ASM. Levetiracetam was the predominant first-administered non-benzodiazepine ASM (88%), and 67% of treated patients received ≥2 ASMs. Inpatient ASM use was independently associated with electrographic seizures/status epilepticus (ES/ESE) during CEEG (OR 19.73), clinical seizures/status epilepticus (SE) before CEEG (OR 3.77), acute structural etiology (OR 2.20), and abnormal EEG background (discontinuous OR 3.17; slow-disorganized OR 1.80). Among patients without clinical seizures/SE documented before CEEG or ES/ESE identified during CEEG, 34% received ASM. Among 590 survivors, 37% were discharged on ASM. Discharge ASM prescribing was associated with ES/ESE (OR 4.79), clinical seizures/SE before CEEG (OR 2.18), acute structural etiology (OR 2.43), epileptiform discharges (OR 1.76), and abnormal EEG background (discontinuous OR 3.13; slow-disorganized OR 1.57). Among patients without clinical seizures/SE documented before CEEG or ES/ESE identified during CEEG, 23% were discharged on ASM. Conclusion ASM use in critically ill children was common and associated with clinical and electrographic seizures/SE but frequently extended beyond documented seizures. Levetiracetam predominated as the first-administered non-benzodiazepine ASM, and many treated patients received multiple ASMs. ASM prescribing at discharge remained common and was associated with seizures and structural etiology. These findings demonstrate substantial variability in subsequent ASM selection and support evaluation of risk-guided approaches to ASM initiation and continuation.
BACKGROUND:Focal cortical dysplasia type II (FCD II) and tuberous sclerosis complex (TSC) are two major structural causes of drug-resistant epilepsy. While the effectiveness of surgical resection in controlling seizures is well-established, the comparative efficacy and safety profile of magnetic resonance-guided laser interstitial thermal therapy (MRgLITT) has not been thoroughly investigated. OBJECTIVE:To compare the efficacy and safety of MRgLITT with resective surgery in patients with MRI-positive FCD II and TSC. DESIGN:A systematic review and meta-analysis was conducted with all available randomized clinical trials and observational studies. MATERIALS AND METHODS:A systematic literature search was conducted across four databases: PubMed, Web of Science, the Cochrane Library, and Embase. Study quality was assessed with the ROBINS-I V2 tool. The primary outcome was the postoperative seizure freedom rate, and the secondary outcome was the complication rate. RESULTS:Overall, 36 studies were included, comprising 25 on resective surgery (1220 patients) and 13 on MRgLITT (87 patients), with two studies reporting data on both surgical modalities. No significant difference was observed in seizure freedom rates between MRgLITT and resective surgery for the combined FCD II/TSC group (0.72 vs. 0.71, P = 0.838), the FCD II-only (0.74 vs. 0.85, P = 0.065), and the TSC-only (0.50 vs. 0.59, P = 0.510), respectively. Surgery age-based subgroup (<18 vs. ≥18 years) analysis also revealed no significant difference in seizure freedom. Similarly, in the combined FCD II/TSC group, complication rates did not differ significantly between MRgLITT and resective surgery (P = 0.469). CONCLUSION:Both resective surgery and MRgLITT are effective treatment options for epilepsy caused by MRI-positive FCD II and TSC, demonstrating comparable efficacy in seizure control and safety. Postoperative seizure outcomes were not influenced by patient age group.
Epilepsy associated with periventricular nodular heterotopia (PVNH) frequently begins in childhood and often proves refractory to medication. Surgical management is challenging, as lesions are frequently deep-seated or bilateral, and seizure onset often involves networks extending beyond the heterotopic tissue itself, with the precise contribution of the nodules varying between patients. This systematic review synthesises all available evidence on neurosurgical and invasive interventions for childhood-onset PVNH-associated epilepsy, focusing on outcomes and prognostic factors. We conducted a PRISMA 2020-compliant systematic review searching PubMed/MEDLINE, Scopus, and Web of Science from inception to 13 December 2025. Eligible studies reported neurosurgical interventions in patients with MRI- or histopathology-confirmed PVNH whose epilepsy began before age 18, with outcomes reported using Engel or ILAE classification. Two reviewers independently screened and extracted data. Methodological quality was appraised using GRADE. Findings were synthesised narratively due to the inherent heterogeneity of the included studies. Thirty studies comprising 134 patients met our inclusion criteria. Excellent outcomes, defined as seizure freedom or auras only, were achieved in 50.7% (n = 68), with 76.9% (n = 103) experiencing meaningful benefit, defined as at least a 50% reduction in seizure frequency. Outcomes were better in unilateral than bilateral PVNH (75.9%vs 33.8%, p < 0.001) and with complete rather than incomplete nodule removal (72.9%vs 25.0%, p < 0.001). Permanent morbidity was 10.4% (n = 14), predominantly visual field deficits. Invasive EEG-guided approaches can achieve seizure freedom in around half of children with PVNH-related epilepsy, with outcomes strongly predicted by laterality. As with all subgroup comparisons in this review, reported p-values reflect effect size across pooled retrospective cohorts rather than formal hypothesis testing. Minimally invasive ablation appears effective and safe, while neuromodulation offers palliation where curative treatment is not feasible. Evidence remains limited by retrospective study designs and small sample sizes, and prospective multicentre data are needed.
Objective Idiopathic generalized epilepsies (IGEs) and long-term antiseizure treatment may differentially affect retinal neuronal structures. This study aimed to evaluate optical coherence tomography (OCT)–derived retinal and optic nerve head (ONH) parameters in adolescents with IGEs, in order to explore retinal alterations associated with epilepsy and valproic acid (VPA) exposure. Methods This cross-sectional study included three groups: patients with IGEs receiving VPA monotherapy for at least one year (PwE+VPA, n = 35), newly diagnosed patients with IGEs who had not yet initiated antiseizure medication (PwE−VPA, n = 22), and healthy controls (HC, n = 30). Retinal and ONH structures were evaluated using OCT. Segmental and global measurements of the Bruch’s membrane opening (BMO), peripapillary retinal nerve fiber layer (RNFL), ganglion cell layer (GCL), inner plexiform layer (IPL), and inner nuclear layer (INL) were obtained. Results A total of 87 individuals aged 10–18 years (47 males and 40 females) were included in the study. In PwE+VPA, the mean duration of epilepsy/VPA exposure was 3.9 ± 2.3 years, and the mean serum VPA concentration was 69.34 ± 18.03 mg/L. There were no statistically significant differences among the groups in age or sex distribution. Compared with HC, PwE+VPA showed significant thinning of the global RNFL, RNFL temporal-inferior, nasal IPL, and global INL. In the comparison between PwE+VPA and PwE−VPA, BMO temporal-inferior was significantly lower in PwE+VPA, whereas the difference in global RNFL thickness did not reach statistical significance. In PwE−VPA, RNFL temporal-inferior, nasal IPL, and global INL were significantly thinner than in HC. In multivariable analyses, nasal IPL and global INL were independently associated with the presence of epilepsy, while RNFL temporal-inferior showed a borderline association. BMO temporal-inferior was independently associated with VPA exposure status, whereas global RNFL was not. Within PwE+VPA, longer epilepsy/VPA treatment duration was independently associated with lower global RNFL thickness but was not associated with BMO temporal-inferior. Conclusion Our findings suggest that retinal involvement may be detectable from the early stages of epilepsy and may predominantly affect the peripapillary RNFL and inner retinal layers. Within the PwE+VPA group, longer epilepsy/VPA exposure duration was associated with lower global RNFL thickness. Further longitudinal studies are needed to clarify the temporal relationship and underlying mechanisms.
PURPOSE:Recurrent brief vestibular attacks are often evaluated as peripheral disorders, but some reflect epileptic vestibular seizures requiring electroencephalographic (EEG) assessment. We sought to identify clinical clues distinguishing isolated epileptic vertigo/dizziness (iEVD) from vestibular paroxysmia (VP). METHODS:We retrospectively compared 50 patients with iEVD and 50 with VP at a tertiary centre. Vestibular symptom descriptions were summarised according to the predominant motion percept documented in the initial clinical account. Clinical features, associated symptoms, EEG findings, and short-term treatment outcomes were reviewed. Independent discriminators were assessed using Firth's penalised logistic regression. RESULTS:Attack frequency independently distinguished iEVD from VP (odds ratio 0.024, 95% confidence interval 0.005-0.120, p < 0.001); daily attacks were uncommon in iEVD but typical in VP (10.0% vs. 88.0%). Heave-like vertical linear motion (floating, rising, sinking, or falling) occurred in 13/50 patients with iEVD (26.0%) and in no patient with VP (specificity 100.0%, 95% CI 92.9-100.0; sensitivity 26.0%). Cortical sensory auras (auditory or visual hallucinations, paraesthesia) affected 20.0% of iEVD patients; auditory hallucinations occurred only in iEVD and unilateral tinnitus only in VP. EEG abnormalities most often involved the temporal region (56.0%). CONCLUSION:Lower attack frequency and heave-like vertical linear motion help distinguish iEVD from VP. Heave-like motion is specific but insensitive, so its absence does not argue against iEVD. Targeted history-taking on motion quality and associated symptoms may help physicians identify patients warranting epilepsy or EEG evaluation before epileptic vestibular events are misclassified as VP.
Seizures and epilepsy are increasingly recognized in neurodegenerative diseases, yet their burden in frontotemporal dementia (FTD) remains poorly defined. We conducted a systematic review and meta-analysis to estimate the prevalence of epilepsy or seizures in FTD, assess comparative risk versus controls, and explore variation across clinical subtypes. MEDLINE/PubMed, Embase, Cochrane Library, LILACS, and ClinicalTrials.gov were searched from inception to August 15, 2025, without language restrictions. Observational studies reporting epilepsy or clinical seizures in adults with FTD were included. Two reviewers independently screened and extracted data following PRISMA 2020 guidelines, with risk of bias assessed using Joanna Briggs Institute checklists. Random-effects meta-analyses were used to pool prevalence estimates. Eleven studies with 3,182 individuals were included; 5 were at low and 6 at high risk of bias. The pooled prevalence of epilepsy or seizures was 7% (95% CI, 4%-11%), but heterogeneity was substantial (I² = 82.3%). Prevalence varied across clinical variants (P = .04), including 3% (95% CI, 1%-11%) in behavioral-variant FTD and 9% (95% CI, 0%-69%) in primary progressive aphasia. Comparative data were sparse: the 2 studies with control counts showed increased odds in FTD but were too heterogeneous to pool, and an exploratory synthesis of all 3 gave a ratio measure of 3.72 (95% CI, 1.38-10.06). Epilepsy and seizures occur in a nontrivial proportion of individuals with FTD. However, substantial heterogeneity, variable methodological quality, and inconsistent definitions compromise the reliability of prevalence estimates. Prospective studies with standardized seizure phenotyping are needed.
PURPOSE:To characterize age-stratified distributions of interictal epileptiform discharges (IEDs) in SCN1A-related epilepsy, compare findings between genotype classes, and explore associations with early exposure to sodium channel-blocking antiseizure medications (SCBs). METHODS:We conducted a retrospective observational study of 57 individuals with pathogenic or likely pathogenic SCN1A variants and at least one interictal electroencephalogram (EEG). EEGs were assigned to predefined developmental age bands (0-1, 1-3, 3-6, 6-13, and 13-20 years). Within each interval, one EEG per participant was selected according to the highest degree of IED involvement. EEGs were classified as no IEDs, focal IEDs, or bilateral/multifocal IEDs. Cumulative exposure to SCBs during the first five years of life was categorized as <11 months or ≥11 months. Associations within the 6-13-year age band were examined using Firth's bias-reduced logistic regression. RESULTS:IEDs were not observed during infancy and became more frequent in later developmental stages, peaking between 6 and 13 years. No significant overall difference in the age-specific distribution of IEDs was observed between truncating and non-truncating variants. In the 6-13-year age band (n = 39), IEDs were present in 9/30 (30.0%) patients with <11 months of SCB exposure and 8/9 (88.9%) patients with ≥11 months of exposure (OR 18.67; 95% CI 2.01-173.2; p = 0.005). The association persisted after adjustment for genotype class, sex, and age at seizure onset (adjusted OR 28.74; 95% CI 3.71-497.64; p = 0.001) and was materially unchanged after additional adjustment for age at genetic diagnosis (adjusted OR 28.33; 95% CI 3.26-605.00; p = 0.001). CONCLUSION:Prolonged early exposure to SCBs was associated with the presence of IEDs in the 6-13-year age band in individuals with SCN1A-related epilepsy. These exploratory findings should be considered hypothesis-generating and require confirmation in prospective multicenter cohorts.
PURPOSE:Status epilepticus (SE) is a major contributor to morbidity in Dravet syndrome (DS). The impact of recently introduced anti-seizure medications (ASMs)-stiripentol (STP), fenfluramine (FFA), and cannabidiol (CBD)-on SE burden remains incompletely defined. We aimed to characterize acute SE management and evaluate the association between these therapies and SE outcomes. METHODS:In this multicenter retrospective cohort study, patients with genetically confirmed DS were included. Clinical data, SE characteristics, treatments, and outcomes were collected. Associations between exposure to STP, FFA, and CBD and SE incidence, duration, refractoriness, and pediatric intensive care unit (PICU) admission were assessed using regression models adjusted for age and follow-up time. RESULTS:Sixty-one patients (median age 13 years) experienced 1033 SE episodes, predominantly convulsive (89.9%). Benzodiazepines were highly effective as first-line therapy, achieving seizure termination in 95.8% of treated episodes. In regression analyses, FFA was associated with reduced SE incidence (IRR 0.19, p < 0.001), while STP showed a non-significant reduction (IRR 0.75, p = 0.286). A cumulative effect was observed, with each additional ASM further lowering SE incidence (IRR 0.63 per drug, p = 0.030). Stiripentol was associated with shorter SE duration (β -5.0 min, p = 0.003), whereas CBD showed a non-significant effect (β -2.7 min, p = 0.506). SE duration decreased with each additional ASM (β -3.7 min per drug, p = 0.038). CONCLUSION:In this large real-world cohort, early benzodiazepine administration was highly effective for SE management. Exposure to novel ASMs was associated with a reduced SE burden, supporting optimized long-term therapy to reduce SE frequency and severity in DS.
PURPOSE:Following reports of declining epilepsy surgery numbers and changing patient populations, this retrospective single-center study examined whether the patients referred for neuropsychological assessment at a level-4 epilepsy center changed over four decades, and whether neuropsychological practice mirrored these changes. METHODS:We analyzed 9,877 first-time neuropsychological assessments performed at the University Epilepsy Center in Bonn between 1986 and 2024, grouped into four time-based quartiles, and tested trends in demographics, etiology, education, cognitive impairment, referral context, and assessment format (ANOVA, χ²). RESULTS:Over time, referred patients became older, had later epilepsy onsets, and were more often assessed early in the disease course. The proportion aged >50 years rose from 12.0% to 33.6%, late-onset epilepsy (>50 years) from 3.2% to 20.7%, and new-onset cases from 5.3% to 25.7%. Presurgical referrals declined sharply from 82.0% to 17.9%. Localization remained predominantly temporal, whereas etiology shifted: among cases with pathology/etiology data (n = 3,362), hippocampal sclerosis declined from 51.4% to 28.3% and tumors decreased, while suspected limbic encephalitis rose from 1.8% to 23.0%. Estimated verbal IQ rose from 101.4 to 106.7, and ICCoDE/Bonn Code classifications showed a shift toward less widespread and less severe impairment profiles. Comprehensive batteries remained common, especially in surgical cases, while short, repeatable assessments of executive function and memory became the most frequent format (69.0% in 2024). CONCLUSION:Over four decades, the referred population changed markedly: fewer surgical referrals, older patients, more new- and late-onset epilepsies, and more focused, less severe impairment. Neuropsychological practice shifted accordingly, from predominantly presurgical risk assessment toward targeted baseline testing and longitudinal monitoring of disease and treatment effects. The main future challenge is assessment that is both efficient and sensitive to dynamic cognitive change, including early neurodegeneration in late-onset epilepsy.