Epileptic Disorders最新文献

Executive functions are a set of high-level cognitive processes necessary for planning, organization, decision-making, self-control, and attention, and are carried out in the anterior frontal lobes. An impairment in executive functioning might present as difficulties in planning and organizing activities, in attention and concentration, in cognitive flexibility, impulsiveness, and working memory fragility. These might result in greater emotional and psychopathological difficulties and poorer academic performance. Self-limited epilepsy with centro-temporal spikes (SeLECTS), the most common epileptic syndrome occurring in the pediatric population, is characterized by seizure remission around puberty in most cases. However, despite the favorable seizure outcome, previous studies have suggested that executive function deficits might be present and persist after epilepsy remission. We conducted a scoping review to investigate the current knowledge on executive functioning in children with SeLECTS. Furthermore, we explored psychopathological and emotional dimensions and daily functioning in this population. Starting from two reviews published in 2021, we conducted a complementary search and included 41 articles, from which we analyzed clinical data, neuropsychological findings, and their respective correlations. Our results confirmed the possible presence of executive dysfunction in patients with SeLECTS in the domains of inhibition and cognitive flexibility. We also strengthen possible impairments in working memory and higher order executive functions. We confirmed the correlation between executive dysfunction and both early age at onset and high frequency of electroencephalogram abnormalities and observed a possible role for high seizure frequency, secondary bilateralization, and the use of anti-seizure medications. We also found a higher prevalence of psychopathological dimensions, most commonly attention deficit-hyperactivity disorder, compared with controls. Overall, our findings support the need for neuropsychological assessment in clinical practice for children with SeLECTS to characterize executive functioning and its impact on psychopathological and emotional dimensions, as well as academic performance.

Objective: Refractory status epilepticus (RSE) is a medical emergency defined as "status epilepticus persisting despite administration of at least 2 appropriately selected and dosed parenteral medications including a benzodiazepine." Control of RSE is critical to avoid irreversible neuronal damage, with midazolam and propofol as the most commonly used agents. This study evaluates the effectiveness of midazolam versus propofol in preventing mortality and complications of RSE.

Methods: Patients from the TriNetX Research Network who received either midazolam or propofol monotherapy on the day of RSE onset were included. Outcomes were assessed at 30 days and maximal follow-up (≤20 years) using Cox proportional hazard models. Propensity score matching (1:1) controlled for demographics and 93 comorbidities from the Charlson Comorbidity Index.

Results: Among 117 736 patients with RSE, 5310 received midazolam and 2136 received propofol. Midazolam was associated with significantly decreased hazards of mortality at 30 days (HR = 0.509 [95% CI: 0.397, 0.653]) but not at maximal follow-up (HR = 0.922 [0.797, 1.067]). Midazolam was also associated with significantly reduced hazards of lactic acidosis (HR = 0.537 [0.427, 0.674]), rhabdomyolysis (HR = 0.295 [0.150, 0.578]), hypertriglyceridemia (HR = 0.316 [0.135, 0.740]), tracheostomy (HR = 0.633 [0.438, 0.916]), PEG placement (HR = 0.519 [0.371, 0.725]), and mechanical ventilation (HR = 0.313 [0.265, 0.370]). Among patients with a traumatic brain injury in the week prior to RSE, midazolam was associated with a significantly lower hazard of 30-day mortality (HR = 0.381 [0.136, 0.993]), while the hazards were not significantly changed in patients with CNS infections (HR = 1.150 [0.351, 3.768]) or cerebrovascular disease (HR = 0.656 [0.421, 1.025]) in the week prior to RSE onset.

Significance: Midazolam monotherapy for RSE was associated with decreased mortality and adverse effects compared to propofol monotherapy in the short term, but relatively equivalent in the long term. Prospective comparative trials are needed to ascertain superiority of either intervention in reducing morbidity and mortality in patients with RSE.

Objective: This study aimed to evaluate the usefulness of intraoperative electrocorticography (iECoG) in providing a more accurate surgical strategy, thereby yielding seizure freedom following resective surgery in children with temporal lobe epilepsy (TLE).

Methods: The authors conducted a retrospective review of pediatric patients with drug-resistant TLE due to various etiologies, within a relatively long follow-up (range 8.5-11.5 years). Patients were divided into two groups based on whether they were operated on using iECoG or not, which was employed in cases of uncertain delineation of the EZ or anticipated extended resection. The efficacy of surgical treatment was assessed using Engel's classification. Seizure-freedom rate for each etiology was compared between groups using Fisher's exact test with a 95% confidence interval.

Results: A total of 81 patients were included in the study (mean age 11.8 years, range 1-18 years), of whom 63 (77.8%) achieved Engel I status after 10 years. The main etiology was hippocampal sclerosis (34/81, 41.9%), followed by tumors (25/81, 30.8%) and focal cortical dysplasia (22/81, 27.1%). iECoG was performed in 29 (35.8%) patients. Overall, there were no significant differences in the proportion of Engel I (p = .78) among those who performed iECoG (22/29, 75.9%) and did not perform iECoG (n = 41/52, 78.8%). Among tumor-associated cases, Engel I was achieved in 100% of patients with iECoG, compared with 76.5% without iECoG (p = .27). No significant differences were observed in focal cortical dysplasia (p = .61) or hippocampal sclerosis (p = .35).

Significance: The study did not show that intraoperative iECoG improved Engel class I outcomes. Refinement of iECoG methods and future studies controlling for confounders are warranted.

Introduction: Temporal lobe epilepsy (TLE) is the most common form of focal epilepsy, and about two-thirds of patients with drug-resistant TLE are surgical candidates. While many studies suggest better postoperative outcomes in children subjected to various types of surgery, there is limited data comparing adults and children who have undergone the same procedure over the same time period. This study aimed to compare long-term seizure outcomes and identify prognostic factors in pediatric (defined as children less than or equal to 12 years) and adult patients undergoing TLE surgery at a high-volume epilepsy center in South India.

Methods: The study cohort comprised 684 consecutive patients (127 children, 557 adults) who underwent standard anterior temporal lobectomy. All underwent presurgical evaluation including video EEG, neuroimaging, and surgical decision was made in a multidisciplinary meet. Seizure freedom was defined as "absence of seizures or auras regardless of antiepileptic drug use," which was the primary outcome. The predictors determining outcome in both age groups were also analyzed. Logistic regression identified the predictors, and Kaplan-Meier curves assessed long-term seizure-free survival.

Results: Children had significantly shorter epilepsy duration pre-surgery (8.38 versus 19.2 years, p < .0001) and significantly better seizure outcome (57.4% vs. 45.6%, p = .0165). Kaplan-Meier analysis revealed longer median seizure-free survival in children (120 months) than adults (72 months, p = .027). In adults, predictors of poor outcome included auditory aura, behavioral arrest, spike-wave discharges during ictal onset, and bitemporal IEDs. Febrile seizures predicted a favorable outcome in children and adults. In children, neoplasia as a substrate was protective, while psychiatric co-morbidity and multiple auras predicted seizure recurrence.

Conclusion: Children benefit more from TLE surgery than adults, due to earlier intervention and shorter duration of seizures. The principle of "time is brain" holds true in epilepsy; prolonged duration of uncontrolled seizures fosters network expansion, highlighting the need for "early surgical referral" and "catching them young."

Epilepsy surgery remains the most effective treatment for focal drug-resistant epilepsy, and stereoelectroencephalography (SEEG) is increasingly used to define the epileptogenic-zone network (EZN) and guide curative or palliative interventions. While SEEG is considered a safe invasive procedure, adverse events arising during monitoring itself are rarely described. We report three exceptional cases of postictal self-removal of intracerebral electrodes during SEEG monitoring. Among 591 implanted patients between January 2000 and October 2025 at Timone Hospital, Marseille, three patients (0.5%) met the inclusion criteria. All were young right-handed men with normal neurocognitive development, focal drug-resistant epilepsy and no psychiatric comorbidity. Self-removal occurred during the postictal phase of spontaneous seizures-two following focal-to-bilateral tonic-clonic seizures and one after a focal impaired-awareness seizure-on the second day of monitoring under complete or partial antiseizure medication withdrawal. Postictal behavior was characterized by agitation, wandering, and, in two cases, resistive aggression when nursing staff attempted to intervene. None of the patients sustained neurological sequelae or significant cerebrovascular complications. EZN involved the temporal lobe in all cases. These observations illustrate that postictal confusion, particularly under medication withdrawal, may occasionally manifest as resistive behavior capable of causing self-harm by means of device manipulation. Awareness of this rare, but potentially hazardous phenomenon, identification of at-risk patients, and implementation of tailored preventive measures may help improve the safety of invasive epilepsy monitoring.

Objective: To identify correlations between thalamic electroencephalographic (EEG) signal patterns and clinical seizure semiology in pediatric patients with drug-resistant epilepsy (DRE) treated with responsive neurostimulation (RNS).

Methods: We conducted a retrospective analysis of 14 pediatric patients (≤17 years old) with DRE who received thalamic RNS implants. EEG recordings from the RNS Patient Data Management System were reviewed and correlated with seizure semiology obtained from medical records and structured family interviews. Patterns between seizure types, EEG onset signals, and electrode placement were examined.

Results: Absence seizures were associated with bilateral 3 Hz delta spike-wave complexes in the centromedian (CM) nucleus. Drop seizures corresponded with bilateral hypersynchronous slow-to-fast gamma activity, primarily in patients with CM or anterior nucleus leads. Generalized tonic-clonic seizures exhibited the greatest variability, with gamma-to-delta transitions, synchronous gamma waves, or asynchronous slow-to-gamma patterns depending on whether leads were placed in the CM or pulvinar nuclei. These EEG patterns were consistent within nuclei, suggesting semiology-specific thalamic activity.

Significance: This study demonstrates that seizure onset EEG signals are nucleus- and semiology-specific in pediatric patients receiving thalamic RNS therapy. These findings highlight the CM nucleus's central role in seizure propagation and support individualized RNS programming based on EEG frequency signatures. While limited by sample size, this work provides early evidence that thalamic EEG biomarkers can inform more precise neuromodulation strategies for pediatric DRE.