Epilepsia. Official Journal of the International League Against Epilepsy

Abstract

Objective

Super-refractory status epilepticus (SRSE) is an enduring or reoccurring SE after 24 hours or more of general anesthesia. This study aimed to evaluate the efficacy and safety of phenobarbital (PB) for the treatment of SRSE.

Methods

This retrospective, multicenter study included neuro-intensive care unit (NICU) patients with SRSE treated with PB between September 2015 and September 2020 from 6 participating centers of the Initiative of German Neuro-intensive Trial Engagement (IGNITE) to evaluate the efficacy and safety of PB-treatment for SRSE. The primary ...

Abstract

Objective

Succinic semialdehyde dehydrogenase deficiency (SSADHD) is a rare inherited metabolic disorder caused by a defect of γ-aminobutyrate (GABA) catabolism. Despite the resultant hyper-GABAergic environment facilitated by the metabolic defect, individuals with this disorder have a paradoxically high prevalence of epilepsy. We aimed to study the characteristics of epilepsy in SSADHD and its concordance with GABA-related metabolites and neurophysiologic markers of cortical excitation.

Methods

Subjects in an international natural history study of SSADHD underwent clinical assessments, electroencephalography, transcranial magnetic stimulation (TMS), magnetic resonance ...

Abstract

New onset refractory status epilepticus (NORSE) is a rare and devastating condition occurring in a previously healthy patient. It is called febrile infection-related epilepsy syndrome (FIRES) when preceded by a febrile infection. It often leads to intensive care treatment, including antiseizure drugs in combination with anesthetic agents, and sometimes ketogenic diet. The mortality rate is high, and severe epileptic and neuropsychiatric sequelae are usually observed. Based on the possible role of neuroinflammation, intravenous immunoglobulin, corticosteroids, and immunomodulatory treatment (anti-IL1, IL6) ...

Abstract

The Critical Success Index (CSI) and Gilbert Skill score (GS) are verification measures that are commonly used to check the accuracy of weather forecasting. In this article, we propose that they can also be used to simplify the joint interpretation of positive predictive value (PPV) and sensitivity estimates across diagnostic accuracy studies of epilepsy data. This is because CSI and GS each provide a single measure that takes the weather forecasting equivalent of PPV and sensitivity into account. We have ...

Abstract

Objective

Previous studies using advanced MRI techniques have documented abnormal transmantle bands connecting ectopic nodules to overlying cortex in patients with periventricular nodular heterotopia (PNH). We describe a similar finding using conventional MRI techniques.

Methods

Patients were identified by means of a full-text search of radiological reports. All scanning was performed using conventional sequences at 3T. Scans were reviewed by three neuroradiologists, and we characterized imaging features based on type of PNH and cortical irregularities associated with the transmantle band.

Results

A total 57 PNH ...

Abstract

The etiology of New-Onset Refractory Status Epilepticus (NORSE), including its subtype with prior fever known as FIRES (Febrile Infection-Related Epilepsy Syndrome), remains uncertain. Several arguments suggest NORSE is a disorder of immunity, likely post-infectious. Consequently, seasonal occurrence might be anticipated. Here, we investigated if seasonality is a notable factor regarding NORSE presentation.

We combined four different datasets with a total of 342 cases, all from the northern hemisphere, and 62% adults.

The incidence of NORSE cases differed between seasons (p=0.0068) and was ...

Abstract

This retrospective study assessed long-term effectiveness of add-on perampanel (PER) in patients with Lennox-Gastaut syndrome (LGS). Outcomes included time to PER-failure and time to seizure-relapse in responders. Perampanel failure was defined as either discontinuation of PER or initiation of another treatment. Seizure relapse in responders was defined as occurrence of a seizure in seizure-free patients and increase of at least 50% in average monthly seizure frequency for those who were responders.

Eighty-seven patients were included. Treatment failure occurred in 52 (59.8%) ...

Abstract

Objectives

To investigate the comparative antiseizure activity of the individual enantiomers of fenfluramine and its major active primary metabolite norfenfluramine in rodent seizure models, and its relationship with the pharmacokinetics of these compounds in plasma and brain.

Methods

The antiseizure potency of d,l-fenfluramine (racemic fenfluramine) was compared with the respective potencies of its individual enantiomers and the individual enantiomers of norfenfluramine using the maximal electroshock (MES) test in rats and mice, and the 6-Hz 44 mA test in mice. Minimal motor impairment was ...

Abstract

Objective

To characterize the relationship between neighborhood disadvantage on cognitive function as well as clinical, sociodemographic, and family factors in children with new onset idiopathic epilepsy and healthy controls.

Methods

Research participants were 288 children aged 8-18 years with recent onset epilepsy (CWE, n = 182; mean age = 12.2 ±3.2 years), healthy first-degree cousin controls (HC, n = 106; mean age = 12.5 ±3.0), and one biological or adopted parent per child (n=279). All participants were administered a comprehensive neuropsychological battery (reasoning, language, memory, executive function, motor function, and academic achievement). Family ...