International consensus on diagnosis and management of Dravet syndrome

Abstract

Objective

This study was undertaken to gain consensus from experienced physicians and caregivers regarding optimal diagnosis and management of Dravet syndrome (DS), in the context of recently approved, DS-specific therapies and emerging disease-modifying treatments.

Methods

A core working group was convened consisting of six physicians with recognized expertise in DS and two representatives of the Dravet Syndrome Foundation. This core group summarized the current literature (focused on clinical presentation, comorbidities, maintenance and rescue therapies, and evolving disease-modifying therapies) and nominated the 31-member expert ...

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Automated seizure detection with non‐invasive wearable devices: A systematic review and meta‐analysis

SUMMARY

Objective

To review the reported performance of non-invasive wearable devices in detecting epileptic seizures and psychogenic non-epileptic seizures (PNES).

Methods

We conducted a systematic review and meta-analysis of studies reported up to November 15, 2021. We included studies that used video-EEG monitoring as the gold standard to determine the sensitivity and false alarm rate (FAR) of non-invasive wearables for automated seizure detection.

Results

Twenty-eight studies met the criteria for the systematic review, of which 23 were eligible for meta-analysis. These studies (1269 patients in total; ...

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Mesenchymal stem cell therapy for focal epilepsy: A systematic review of preclinical models and clinical studies

Abstract

Drug-resistant epilepsy (DRE) is characterized by recurrent seizures despite appropriate treatment with antiseizure medication (ASM). Due to their regenerative and immunomodulatory potential, therapies with biologics such as mesenchymal stem cells (MSCs) offer a potential therapeutic benefit for structural causes of epilepsy, such as hippocampal sclerosis. In this article, we report a systematic review of the literature evaluating the preclinical and clinical studies of MSCs for DRE. Medline, Ovid EMBASE, Scopus, and the Cochrane Databases were searched electronically from their dates ...

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Value of genetic testing for pediatric epilepsy: Driving earlier diagnosis of ceroid lipofuscinosis type 2 Batten disease

Abstract

This study assessed the effectiveness of genetic testing in shortening the time to diagnosis of late infantile neuronal ceroid lipofuscinosis type 2 (CLN2) disease. Individuals who received epilepsy gene panel testing through Behind the Seizure®, a sponsored genetic testing program (Cohort A), were compared to children outside of the sponsored testing program during the same period (Cohort B). Two cohorts were analyzed: children aged ≥24 to ≤60 months with unprovoked seizure onset at ≥24 months between December 2016 and January 2020 (Cohort ...

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Bumetanide for neonatal seizures: no light in the pharmacokinetic/dynamic tunnel

Abstract

In his editorial, Kevin Staley criticizes our recent work demonstrating the lack of effect of bumetanide in a novel model of neonatal seizures. The main points in our response are that (i) our work is on an asphyxia model, not one on ‘hypercarbia-only’; (ii) clinically relevant parenteral doses of bumetanide applied in vivo lead to concentrations in the brain parenchyma which are at least an order of magnitude lower than what would be sufficient to exert any direct effect – ...

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Efficacy, safety, and economic impact of diazepam suppositories with as‐needed acetaminophen for prevention of seizure recurrence during the same fever episode in children with suspected simple febrile seizures

Abstract

Objective

To evaluate the efficacy, safety, and economic impact of diazepam suppositories with as-needed acetaminophen in comparison with as-needed acetaminophen alone for prevention of seizure recurrence during the same fever episode in suspected pediatric simple febrile seizures (SFS).

Methods

This single-center, prospective, observational study was conducted from July 29, 2019 to February 15, 2021 at a children’s hospital. Children aged 6 months to 60 months presenting to the emergency department with suspected SFS were included. Participants receiving both diazepam suppositories and as-needed acetaminophen ...

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