Epileptic Disorders最新文献

Objective: To evaluate the efficacy of vagus nerve stimulation (VNS) as an adjunctive treatment in pediatric patients with treatment-resistant epilepsy during the transition to adolescence.

Methods: We performed a retrospective cohort study of 30 children (ages 2-18 years) with medication-resistant epilepsy who underwent VNS implantation between January 2019 and January 2023 at a tertiary epilepsy center. Clinical data included demographics, epilepsy etiology and syndrome, age at implantation, seizure frequency and severity (McHugh classification), number of antiseizure medications (ASM), magnet use, and EEG findings. Outcomes were assessed at the last available follow-up, with a minimum duration of 12 months post-implantation. Statistical analyses comprised chi-squared or Fisher's exact tests, Spearman rank correlation, and logistic regression; significance was set at p < 0.05.

Results: At the last follow-up, 20 of 30 patients (66.7%) achieved ≥50% reduction in seizure frequency, and 73% of patients experienced a significant decrease in seizure severity. Status epilepticus (SE) incidence fell from 67% pre-VNS to 17% post-VNS (p = 0.024). The number of ASMs decreased in 57% of patients. Successful magnet activation strongly predicted responder status (OR 10.8, 95% CI 1.2-95.4; p = 0.024). Among females, 12 of 16 (75%) experienced a transient seizure worsening around menarche. Earlier VNS implantation correlated with better seizure reduction (ρ = -0.43; p = 0.015). EEG improvement-defined as reduced interictal epileptiform activity or background normalization-was observed in 23% of patients, predominantly in males and pre-menarche females.

Significance: VNS is an effective adjunctive therapy in pediatric treatment-resistant epilepsy, yielding substantial reductions in seizure frequency and severity, lowering status epilepticus risk, and simplifying medication regimens. Pubertal status and magnet responsiveness modulate outcomes. Early VNS intervention and proactive management during adolescence may optimize therapeutic benefit.

Objective: Focal brain lesions may underlie generalized tonic seizures, as seen in Lennox-Gastaut syndrome, by engaging bilateral neural networks. However, this seizure type is often not considered surgically remediable. Here, we describe the resolution of apparent electroclinically classic generalized tonic seizures in children originating from a unifocal brain lesion following resective or ablative surgery. This study aims to contribute to emerging evidence that prompt removal of a lesion may resolve generalized seizures by ameliorating aberrant network activity.

Methods: Boston Children's Hospital's (BCH) epilepsy surgical database was reviewed to identify children with tonic seizures due to a focal brain lesion who remained seizure-free for longer than 1 year following resective or ablative surgery.

Results: Five children were identified, of whom three underwent resective surgery and two laser interstitial thermal therapy (LITT). Age at epilepsy onset varied from 1 month to 7.25 years, and age at first epilepsy surgery ranged from 5.6 to 9.5 years. Lesions were predominantly located in the frontal lobe (n = 3), and focal cortical dysplasia (FCD) was the most common underlying etiology (n = 3), followed by vascular lesions (n = 2). At last follow-up, seizure freedom (Engel Class 1A) ranged between 1.7 and 4.4 years.

Significance: This study presents evidence that resection or ablation of a focal cortical lesion can resolve generalized tonic seizures. The findings also lend credence to the hypothesis that heterogeneous brain lesions can underlie shared electroclinical features through engaging similar brain networks. Children with tonic seizures in whom a lesional etiology is presumed should undergo timely surgical evaluation, as removal of a focal lesion may arrest the evolution of a secondary epileptic network and allow for the restoration of normal brain network development.

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."