Search

Objective: The aim of this study is to describe the pre‐ and post‐operative developmental and intellectual functions in a cohort of patients who underwent surgery for drug‐resistant epilepsy (DRE) before the age of 5 years. Method: We retrospectively reviewed the medical records and neurodevelopmental assessments of a cohort of 80 surgically treated pediatric patients with DRE. We included patients if they had at least one pre‐ and one post‐surgical neuropsychological assessments; 27 met the inclusion criteria. We evaluated Developmental Quotient (DQ) and Intelligence Quotient (IQ) before and after surgery. We identified two groups based on psychological evaluation outcome: Group 1, with stable or improved developmental and intellectual functions, and Group 2, experiencing developmental and intellectual loss. Results: The mean age at seizure onset was 1.2 ± 1.0 years, and the mean age at surgery was 2.9 ± 1.2 years. At the last follow‐up (mean 4 years, SD ± 2), 19/27 (70%) patients were seizure‐ and drug‐free; 18/27 patients (67%) fit in Group 1, and 9/27 (33%) fit in Group 2. The mean age at surgery was 2.6 years (SD ± 1.1; range 1.2–5.1) in Group 1 and 3.4 years in Group 2 (SD ± 1.1; range 1.6–5.0). Group 1 had a lower pre‐operative DQ/IQ total score than Group 2 (median DQ/IQ respectively 82 vs 108, p = 0.05). Between pre‐ and post‐assessments, we found that in Group 1, Performance scores improved (82.7 vs 102, p = 0.001), while in Group 2, the Total and Verbal scores worsened (respectively 108 vs 75, p = 0.008, and 100 vs 76, p = 0.021). Significance: Our study's results emphasize the positive impact of surgery before the age of 5 years on developmental and intellectual outcomes. Despite limitations such as a small sample size, lack of a control group, and diverse etiologies, our findings support the crucial role of early intervention in preserving or enhancing developmental and intellectual functions in young patients with DRE. Plain Language Summary: This retrospective study, conducted at the Bambino Gesù Children Hospital in Italy, reports neuropsychological and developmental and/or cognitive data for children undergoing early epilepsy surgery (before the age of 5). It found that children with lower developmental or cognitive profiles gained the highest scores on post‐operative neuropsychological evaluations. This study provides information on the potential benefits of early surgery in shortening the duration of epilepsy, preventing or arresting deterioration, and enhancing plasticity and recovery. [ABSTRACT FROM AUTHOR]

Objectives: Hemispherotomy (HT) is a surgical option for treatment of drug-resistant seizures due to hemispheric structural lesions. Factors affecting seizure outcome have not been fully clarified. In our study, we used a brain Machine Learning (ML) approach to evaluate the possible role of Inter-hemispheric EEG Connectivity (IC) in predicting post-surgical seizure outcome. Methods: We collected 21 pediatric patients with drug-resistant epilepsy; who underwent HT in our center from 2009 to 2020; with a follow-up of at least two years. We selected 5-s windows of wakefulness and sleep pre-surgical EEG and we trained Artificial Neuronal Network (ANN) to estimate epilepsy outcome. We extracted EEG features as input data and selected the ANN with best accuracy. Results: Among 21 patients, 15 (71%) were seizure and drug-free at last follow-up. ANN showed 73.3% of accuracy, with 85% of seizure free and 40% of non-seizure free patients appropriately classified. Conclusions: The accuracy level that we reached supports the hypothesis that pre-surgical EEG features may have the potential to predict epilepsy outcome after HT. Significance: The role of pre-surgical EEG data in influencing seizure outcome after HT is still debated. We proposed a computational predictive model, with an ML approach, with a high accuracy level. [ABSTRACT FROM AUTHOR]

Objective Methods Results Significance Developmental and epileptic encephalopathies (DEEs) are neurological disorders characterized by developmental impairment and epilepsy. Our study aims to assess disease progression by comparing clinical findings, electroencephalography (EEG), and neuropsychological data from seizure onset to the last follow‐up evaluation.We retrospectively reviewed patients with genetic DEEs who were followed‐up at the epilepsy unit of Bambino Gesù Children's Hospital, Rome. We collected information regarding gender, family history, genetic variant, age at onset and at last follow‐up, neurological examination, type of seizure, drug resistance, occurrence of status epilepticus, and movement and cognitive and behavioral disorders. We compared EEG background activity, epileptiform abnormalities, and cognitive functions between seizure onset and the last follow‐up evaluation using the McNemar‐Bowker test (α = 5%).A total of 160 patients (94 female) were included. Genetic analysis revealed a spectrum of pathogenic variants, with SCN1A being the most prevalent (25%). The median age at seizure onset and at the last follow‐up was 0.37 (interquartile range [IQR]: 0.09–0.75) and 8.54 years (IQR: 4.32–14.55), respectively. We documented a statistically significant difference in EEG background activity (p = .017) and cognitive impairment (p = .01) from seizure onset to the last follow‐up evaluation. No significant differences were detected for epileptiform abnormalities (p = .2). In addition, high prevalence rates were observed for drug resistance (81.9%), movement disorders (60.6%), behavioral and autism spectrum disorders (45%), neurological deficits (31.3%), and occurrence of status epilepticus (23.1%).Our study provides evidence that a clinical progression may appear in genetic DEEs, manifesting as development or worsening of cognitive impairment and disruption of EEG background activity. These results highlight the challenging clinical course and the importance of early intervention and personalized care in the management of patients with DEEs. [ABSTRACT FROM AUTHOR]

Background: The aim of this study was to investigate the features of cyclic vomiting syndrome (CVS) in a pediatric cohort and assess the safety and efficacy of cyproheptadine regardless of age., Methods: This retrospective, monocentric study enrolled children diagnosed with CVS. After collecting data from the medical records, a telephone-based questionnaire interview with the caregivers was conducted to obtain missing or unclear data. Prodromal symptoms, triggers, and comorbidities were analyzed. The safety and efficacy of cyproheptadine were evaluated in the entire patient cohort and compared between children under 5 years old and those 5 years of age or older., Results: A cohort of 24 subjects with CVS were included. Prodromal symptoms were reported in 45.8%, triggers in 33.3%, and comorbidities in 58.3% of patients. Migraine was present in 54% of the patients. Cyproheptadine was prescribed to 15 of the 24 patients (62.5%), with 40% and 80% achieving a reduction in the frequency of vomiting clusters of over 75% after a median duration of 6.03 and 12.13 months respectively. Prophylactic therapy did not provide any benefit to 20% of the patients. No statistically significant differences were found in vomiting features or cyproheptadine response between children above and below 5 years, except for a negative correlation between age and the presence of diarrhea during CVS episodes. No serious adverse effects were reported., Conclusions: Assessment of prodromes, triggers, and comorbidities aids in CVS diagnosis in children. Cyproheptadine is effective and safe for the management of CVS regardless of age.