Epilepsia. Official Journal of the International League Against Epilepsy

Abstract

Objective

Seizures that are refractory to medical and surgical therapy increase the risk of morbidity and mortality in children with epilepsy. Novel therapeutic trials for this population remain sparse and suboptimal. This Phase 1 study evaluates the (1) safety, tolerability, and (2) preliminary efficacy of ABI-009 (nab-rapamycin) in children with medically and surgically refractory epilepsy.

Methods

Children with medically intractable epilepsy and a history of resective surgery who experienced >8 seizures during the 30-day baseline were included. Participants maintained their existing antiseizure medication ...

Abstract

Objective

This study was undertaken to determine the thalamic nuclei that are different between juvenile myoclonic epilepsy (JME) and healthy controls from the Juvenile Myoclonic Epilepsy Connectome Project and then to determine their relationship with other subcortical gray matter volumes, disease covariates, and motor performance.

Methods

Sixty-two patients with JME and 41 age-matched controls (mean age = 20 years) were examined using T1-weighted images. Thalamic nuclei volumes were compared after normalization to total intracranial volume. The relationship between thalamic nuclei volumes and age, duration of epilepsy, ...

Abstract

Objective

This study aimed to assess the complementary role of high-density electroencephalography (HD-EEG)–based electrical source imaging (ESI) in localizing the seizure-onset zone (SOZ) during ictal events and the irritative zone (IZ) during the interictal period, using simultaneous acquired stereo-EEG (SEEG) recordings as a reference. In addition, we investigated the relationship of ictal cortical source activity with their scalp and intracranial EEG correlates, focusing on factors influencing ESI accuracy.

Methods

We enrolled 29 patients with drug-resistant epilepsy who underwent simultaneous HD-EEG and SEEG recordings. ...

Abstract

Objective

L-arginine:glycine amidinotransferase (AGAT) deficiency is a rare autosomal recessive disorder affecting creatine biosynthesis, leading to developmental delay, intellectual disabilities, and myopathy. Unlike other creatine deficiency disorders, its link to epilepsy remains uncertain. This study presents the first reported epilepsy cases in AGAT deficiency, analyzing seizure patterns and response to creatine monohydrate supplementation.

Methods

We retrospectively analyzed two AGAT-deficient probands identified through a national collaboration. Biochemical assessments of creatine and guanidinoacetate (GAA) levels in plasma and urine were performed using electrospray ionization tandem ...

Open-label CBD treatment was associated with sustained seizure reduction through 144 weeks, with a consistent safety profile in patients with treatment-resistant focal epilepsies, regardless of epilepsy type.

Abstract

Objective

Cannabidiol (CBD) treatment was associated with long-term seizure reduction in patients with various treatment-resistant epilepsies who participated in the CBD Expanded Access Program. A phase 3 trial showed CBD effectively treats tuberous sclerosis complex (TSC)-associated seizures, ...

Abstract

Objective

The in vivo effects of fenfluramine result from the combined actions of four active molecular entities (l-fenfluramine, d-fenfluramine, and the corresponding enantiomers of the primary metabolite norfenfluramine). Each of these compounds has different properties, with cardiovascular toxicity risk being ascribed primarily to the d-enantiomers. To determine whether l-fenfluramine or l-norfenfluramine is a better candidate for development as an enantiomerically pure antiseizure medication, we investigated dose–response and brain/plasma concentration–response relationships for each of the four individual enantiomers in mice.

Methods

Antiseizure activity was evaluated ...

Abstract

Objective

Mesial temporal lobe epilepsy (MTLE) is generally associated with cognitive and psychiatric comorbidities. Here we examined whether similar comorbidities are observed in mice injected with kainate in the dorsal hippocampus, a model known to recapitulate many features of human MTLE, and if these comorbidities are sex dependent.

Methods

Male and female C57BL/6 mice were unilaterally injected with kainate in the dorsal hippocampus (KA-MTLE), and hippocampal paroxysmal discharges were recorded over 2 months. Behavioral tests assessed food intake (Novelty Palatable Food Intake test), well-being ...

Abstract

Objective

Traumatic brain injury (TBI) is a leading cause of long-term disability, and infections such as pneumonia represent a common and serious complication for patients with TBI in the acute and subacute post-injury period. Although the acute effects of infections have been documented, their long-term consequences on neurological and behavioral recovery as well as the potential precipitation of seizures after TBI remain unclear. This study aimed to investigate the chronic effects of Klebsiella pneumoniae infection following TBI, focusing on post-traumatic seizure ...

Abstract

Objective

The pulvinar is increasingly recognized as a promising target for neuromodulation in drug-resistant epilepsy (DRE). Despite growing interest, empirical evidence substantiating the efficacy and mechanism of its deep brain stimulation (DBS) in patients with epilepsy remains scarce. This study endeavors to address this knowledge gap by investigating the electrophysiological properties of pulvinar.

Methods

We enrolled 35 patients with DRE who underwent stereoelectroencephalography with electrodes extended to the pulvinar and analyzed the pulvinar’s involvement in seizures originating from different brain lobes. Repeated single ...

Abstract

Objective

Seizure unpredictability is a significant burden in the lives of people with epilepsy. Previously published approaches to seizure forecasting analyzed intracranial electroencephalography (iEEG) recordings and showed that seizures can be forecast above chance levels. Although passive observation of the brain might provide some insights, repeated active perturbation of the cortex and measuring the cortical response may provide more direct information about time-varying cortical excitability. The aim of this study is to investigate whether seizures can be forecast by stimulating the ...