Objective: This study aimed to investigate two key aspects of scalp high-frequency oscillations (HFOs) in pediatric focal lesional epilepsy: (1) the stability of scalp HFO spatial distribution across consecutive nights, and (2) the variation in scalp HFO rates in response to changes in antiseizure medication (ASM).
Methods: We analyzed 81 whole-night scalp electroencephalography (EEG) recordings from 20 children with focal lesional epilepsy. We used a previously validated automated HFO detector to assess scalp HFO rates (80-250 Hz) during non-rapid eye movement (NREM) sleep.
Objective: This study was undertaken to characterize the clinical and genetic features of patients with 22q11.2 deletion syndrome (22q11.2DS) and generalized epilepsy compared with 22q11.
View Article and Find Full Text PDFGlucose transporter type 1 deficiency syndrome (GLUT1DS) commonly presents with early-onset epilepsy that often resists conventional pharmacological treatment. Ketogenic diet therapy (KDT) is the preferred approach to address the underlying metabolic anomaly. However, a subset of GLUT1DS patients presents resistance to KDT, with the causes remaining elusive.
View Article and Find Full Text PDFPurpose: Arterial spin labeling (ASL), a noninvasive magnetic resonance (MRI) perfusion sequence, holds promise in the presurgical evaluation of pediatric lesional epilepsy patients, including those with low-grade epilepsy-associated tumors (LEATs). The interpretation of ASL-derived perfusion patterns, however, presents challenges. Our study aims to elucidate these perfusion changes in children with LEATs, exploring their correlations with clinical, electroencephalography (EEG), and anatomical MRI findings.
View Article and Find Full Text PDFPurpose: To assess the trends in administered 2-[F]fluoro-2-deoxy-D-glucose ([F]FDG) doses, computed tomography (CT) radiation doses, and image quality over the last 15 years in children with drug-resistant epilepsy (DRE) undergoing hybrid positron emission tomography (PET) brain scans.
Methods: We retrospectively analyzed data from children with DRE who had [F]FDG-PET/CT or magnetic resonance scans for presurgical evaluation between 2005 and 2021. We evaluated changes in injected [F]FDG doses, administered activity per body weight, CT dose index volume (CTDIvol), and dose length product (DLP).
Developmental and epileptic encephalopathies (DEEs) present significant treatment challenges due to frequent, drug-resistant seizures and comorbidities that impact quality of life. DEEs include both developmental encephalopathy from underlying pathology and epileptic encephalopathy where seizures exacerbate cognitive and behavioral impairments. Classification by syndrome and etiology is essential for therapy and prognosis, with common syndromes like infantile epileptic spasms syndrome and Dravet syndrome having specific first-line treatments.
View Article and Find Full Text PDFInterictal Epileptiform Discharges (IED) and High Frequency Oscillations (HFO) in intraoperative electrocorticography (ECoG) may guide the surgeon by delineating the epileptogenic zone. We designed a modular spiking neural network (SNN) in a mixed-signal neuromorphic device to process the ECoG in real-time. We exploit the variability of the inhomogeneous silicon neurons to achieve efficient sparse and decorrelated temporal signal encoding.
View Article and Find Full Text PDFArterial spin labelling (ASL), an MRI sequence non-invasively imaging brain perfusion, has yielded promising results in the presurgical workup of children with focal cortical dysplasia (FCD)-related epilepsy. However, the interpretation of ASL-derived perfusion patterns remains unclear. Hence, we compared ASL qualitative and quantitative findings to their clinical, EEG, and MRI counterparts.
View Article and Find Full Text PDFBackground And Objectives: Heterozygous variants in RAR-related orphan receptor B () have recently been associated with susceptibility to idiopathic generalized epilepsy. However, few reports have been published so far describing pathogenic variants of this gene in patients with epilepsy and intellectual disability (ID). In this study, we aimed to delineate the epilepsy phenotype associated with pathogenic variants and to provide arguments in favor of the pathogenicity of variants.
View Article and Find Full Text PDFRecently, precision medicine has attracted much attention in the management of epilepsies, but it remains unclear if the increasingly utilized ketogenic diet approaches can truly be considered precision medicine in all epilepsy treatment. Currently, it is the standard treatment for patients with GLUT1 deficiency and the latest NICE guidelines highlight ketogenic diet as a therapeutic option for multi-drug resistant epilepsy patients. Ketogenic diet is presumed to be a precision medicine tool when applied to the treatment of seizures secondary to GLUT1 transporter deficiency.
View Article and Find Full Text PDFObjective: We aimed to evaluate determinants of functional outcome after pediatric hemispherotomy in a large and recent multicenter cohort.
Methods: We retrospectively investigated the functional outcomes of 455 children who underwent hemispherotomy at 5 epilepsy centers in 2000-2016. We identified determinants of unaided walking, voluntary grasping with the hemiplegic hand, and speaking through Bayesian multivariable regression modeling using missing data imputation.
Objective: This systematic review aimed to assess the intellectual outcome of children who underwent surgery for epilepsy.
Methods: A systematic review of electronic databases was conducted on December 3, 2021, for PubMed and January 11, 2022, for Web of Science. The review was conducted according to the PRISMA guidelines.
Central nervous system (CNS) disorders are among the most frequent presentations in critically ill children. Status epilepticus (SE) is a frequent scenario in the resuscitation bay. In patients with altered mental status, non-convulsive SE (NCSE) is often underrecognized and critically impacts the neurological outcome and duration of hospitalization.
View Article and Find Full Text PDFObjective: We aimed to investigate (1) whether an automated detector can capture scalp high-frequency oscillations (HFO) in neonates and (2) whether scalp HFO rates can differentiate neonates with seizures from healthy neonates.
Methods: We considered 20 neonates with EEG-confirmed seizures and four healthy neonates. We applied a previously validated automated HFO detector to determine scalp HFO rates in quiet sleep.
Cerebellar lesional epilepsy is rare, commonly manifesting in early life and posing diagnostic and treatment challenges. Seizure semiology may be subtle, with repetitive eye blinking, face twitching, and irregular breathing, while EEG commonly remains unremarkable. Pharmacoresistance is the rule, and surgical intervention is the only treatment with the potential for cure.
View Article and Find Full Text PDFSlow waves are an electrophysiological characteristic of non-rapid eye movement sleep and a marker of the restorative function of sleep. In certain pathological conditions, such as different types of epilepsy, slow-wave sleep is affected by epileptiform discharges forming so-called "spike-waves". Previous evidence shows that the overnight change in slope of slow waves during sleep is impaired under these conditions.
View Article and Find Full Text PDFObjectives: Although hemispheric surgeries are among the most effective procedures for drug-resistant epilepsy (DRE) in the pediatric population, there is a large variability in seizure outcomes at the group level. A recently developed HOPS score provides individualized estimation of likelihood of seizure freedom to complement clinical judgement. The objective of this study was to develop a freely accessible online calculator that accurately predicts the probability of seizure freedom for any patient at 1-, 2-, and 5-years post-hemispherectomy.
View Article and Find Full Text PDFBackground: Cognitive development in children and adolescents with focal lesional epilepsy is determined by the underlying epileptogenic lesion, in addition to epilepsy itself. However, the impact of lesion-related variables on intelligence quotient (IQ) and developmental quotient (DQ) remains largely unexplored. Here, we aimed to determine the effect of lesion-related predictors and their relation with epilepsy-related predictors of intellectual functioning.
View Article and Find Full Text PDF