Epilepsia. Official Journal of the International League Against Epilepsy

Abstract

Objective

Postictal generalized EEG suppression (PGES) is a surrogate marker of sudden unexpected death in epilepsy (SUDEP). It is still unclear which ictal phenomena lead to prolonged PGES and increased risk of SUDEP. Semiology features of generalized convulsive seizures (GCS type 1) have been reported as a predictor of prolonged PGES. Progressive slowing of clonic phase (PSCP) has been observed in GCSs, with gradually increasing inhibitory periods interrupting the tonic contractions. We hypothesized that PSCP is associated with prolonged PGES.

Methods

We analyzed ...

Abstract

Objective

To evaluate the long-term safety and effectiveness of fenfluramine in patients with Lennox-Gastaut syndrome (LGS).

Methods

Eligible patients with LGS who completed a 14-week phase 3 randomized clinical trial enrolled in an open-label extension (OLE; NCT03355209). All patients were initially started on 0.2 mg/kg/day fenfluramine and, after 1 month, were titrated to effectiveness and tolerability, which were assessed at 3-month intervals. The protocol-specified treatment duration was 12 months, but COVID-19-related delays resulted in 142 patients completing their final visit after 12 months.

Results

As ...

Abstract

Individuals use the observation of a conspecific to learn new behaviors and skills in many species. Whether observational learning is affected in epilepsy is not known. Using the pilocarpine rat model of epilepsy, we assessed learning by observation in a spatial task.

The task involves a naïve animal observing a demonstrator animal seeking a reward at a specific spatial location. After five observational sessions, the observer is allowed to explore the rewarded space and look for the reward.

Although control observer rats ...

Abstract

Objective

Epilepsy treatment trials typically rely on seizure diaries to determine seizure frequency, but these are time-consuming and difficult to maintain accurately. Fast, reliable, and objective biomarkers of treatment response are needed, particularly in Lennox–Gastaut syndrome (LGS), where high seizure frequency and comorbid cognitive and behavioral issues are additional obstacles to accurate diary-keeping. Here, we measured generalized paroxysmal fast activity (GPFA), a key interictal electrographic feature of LGS, and correlated GPFA burden with seizure diaries during a thalamic deep brain stimulation ...

Abstract

Objective

Laser Interstitial Thermal Therapy (LiTT) is a minimally invasive surgical procedure for intractable mesial temporal epilepsy (mTLE). LiTT is safe and effective, but seizure outcomes are highly variable due to patient variability, suboptimal targeting, and incomplete ablation of the epileptogenic zone. Apparent Diffusion Coefficient (ADC) is an MRI sequence that can identify potential epileptogenic foci in the mesial temporal lobe to improve ablation and seizure outcomes. The objective of this study was to investigate whether ablation of tissue clusters with ...

Abstract

Objective

Cortical tremor/myoclonus is the hallmark feature of benign adult familial myoclonic epilepsy (BAFME), which mechanism remains elusive. A hypothesis is that a defective control in the pre-existing cerebellar-motor loop drives cortical tremor. Meanwhile, the basal ganglia system might also participate in BAFME. This study aimed to discover the structural basis of cortical tremor/myoclonus in BAFME.

Methods

Nineteen patients with BAFME type 1 (BAFME1) and thirty matched healthy controls underwent T1-weighted and DTI scans. FreeSurfer and SUIT toolboxes were utilized to assess the ...

Abstract

It is well established that epilepsy is associated with numerous neurobehavioral comorbidities, with a bidirectional relationship: People with epilepsy have an increased incidence of depression, anxiety, learning and memory difficulties, and numerous other psychosocial challenges, and the occurrence of epilepsy is higher in individuals with those comorbidities. Although the cause-and-effect relationship is uncertain, a fuller understanding of the mechanisms of comorbidities within the epilepsies could lead to improved therapeutics. Here, we review recent data on epilepsy and its neurobehavioral comorbidities, ...

Abstract

Objective

Monogenic epilepsies are rare but often severe. Because of their rarity, they are neglected by traditional drug developers. Hence, many lack effective treatments. Treatments for a disease can be discovered more quickly and economically by computationally predicting drugs that can be repurposed for it. We aimed to create a computational method to predict the efficacy of drugs for monogenic epilepsies, and to use the method to predict drugs for Dravet syndrome, as (1) it is the archetypal monogenic catastrophic epilepsy, ...

Abstract

Pharmacogenomics hold the potential to identify variants associated with adverse drug reactions and treatment efficacy of anti-seizure medications. A model-based cost-utility analysis by Gordon and colleagues showed that genetically-guided therapy costs more, yielded higher quality-adjusted life years outcomes, and was considered to be cost-effective compared to usual care. The study provided preliminary evidence on the value of pharmacogenetic testing in patients with drug-resistant epilepsy. However, data input for the model was based on assumptions that need to be empirically tested. ...

Abstract

Objective

Our goal was to determine if animals with a history of epileptic spasms have learning and memory deficits. We also used continuous (24/7) long-term EEG recordings to evaluate the evolution of epileptiform activity in the same animals over time.

Methods

Objective Recognition Memory and Object Location Memory tests were undertaken, as well as a Matching to Place Water Maze test that evaluated working memory. A retrospective analysis was undertaken of long-term video/EEG recordings from rats with epileptic spasms. The frequency and duration ...