Convulsive seizures and some behavioral comorbidities are uncoupled in the Scn1aA1783V Dravet syndrome mouse model

Abstract

Objective

Dravet syndrome (Dravet) is a severe childhood epileptic encephalopathy. The disease begins with a febrile stage, characterized by febrile seizures with otherwise normal development. Progression to the worsening stage features recurrent intractable seizures and the presentation of additional nonepileptic comorbidities, including global developmental delay, hyperactivity, and motor deficits. Later in life, at the stabilization stage, seizure burden decreases, whereas Dravet‐associated comorbidities persist. To date, it remains debated whether the nonepileptic comorbidities result from severe epilepsy or represent an independent phenotypic ...

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Biomarkers of seizure response to vagus nerve stimulation: A scoping review

Abstract

Although vagus nerve stimulation (VNS) is a common procedure, seizure outcomes are heterogeneous, with few available means to preoperatively identify the ideal surgical candidate. Here, we perform a scoping review of the literature to identify biomarkers of VNS response in patients with drug‐resistant epilepsy. Several databases (Ovid MEDLINE, Ovid Embase, BIOSIS Previews, and Web of Science) were searched for all relevant articles that reported at least one biomarker of VNS response following implantation for intractable epilepsy. Patient demographics, seizure data, ...

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Trends in lobectomy/amygdalohippocampectomy over time and the impact of hospital surgical volume on hospitalization outcomes: A population‐based study

Abstract

Objective

Despite national guidelines supporting surgical referral in drug‐resistant epilepsy, it is hypothesized that surgery is underutilized. We investigated the volumes of lobectomy/amygdalohippocampectomy surgeries over time and examined differences in outcomes between (1) high‐volume (HV), middle‐volume (MV), and low‐volume (LV) hospitals and (2) Level 4 Centers versus non–Level 4 Centers.

Methods

The 2003‐2014 National Inpatient Sample (the largest all‐payer hospitalization database, representative of the US population) was utilized. Epilepsy was identified using a previously validated International Classification of Diseases, Ninth Revision, Clinical Modification ...

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Blood biomarkers predictive of epilepsy after an acute stroke event

Abstract

Objective

Blood biomarkers have not been widely investigated in poststroke epilepsy. In this study, we aimed to describe clinical factors and biomarkers present during acute stroke and analyze their association with the development of epilepsy at long term.

Methods

A panel of 14 blood biomarkers was evaluated in patients with ischemic and hemorrhagic stroke. Biomarkers were normalized and standardized using Z‐scores. Stroke and epilepsy‐related variables were also assessed: stroke severity, determined by National Institutes of Health Stroke Scale (NIHSS) score, stroke type and ...

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Integrating quality improvement into the ECHO model to improve care for children and youth with epilepsy

Abstract

Objective

Project ECHO (Extension for Community Healthcare Outcomes), a telementoring program, utilizes lectures, case‐based learning, and an “all teach–all learn” approach to increase primary care provider (PCP) knowledge/confidence in managing chronic health conditions. The American Academy of Pediatrics (AAP) Epilepsy and Comorbidities ECHO incorporated quality improvement (QI) methodology to create meaningful practice change, while increasing PCP knowledge/self‐efficacy in epilepsy management using the ECHO model.

Methods

Monthly ECHO sessions (May 2018 to December 2018) included lectures, case presentations/discussion, and QI review. Pediatric practices were ...

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A new era in electroencephalographic monitoring? Subscalp devices for ultra–long‐term recordings

Abstract

Inaccurate subjective seizure counting poses treatment and diagnostic challenges and thus suboptimal quality in epilepsy management. The limitations of existing hospital‐ and home‐based monitoring solutions are motivating the development of minimally invasive, subscalp, implantable electroencephalography (EEG) systems with accompanying cloud‐based software. This new generation of ultra–long‐term brain monitoring systems is setting expectations for a sea change in the field of clinical epilepsy. From definitive diagnoses and reliable seizure logs to treatment optimization and presurgical seizure foci localization, the clinical need ...

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Collaborative Cross mice reveal extreme epilepsy phenotypes and genetic loci for seizure susceptibility

Abstract

Objective

Animal studies remain essential for understanding mechanisms of epilepsy and identifying new therapeutic targets. However, existing animal models of epilepsy do not reflect the high level of genetic diversity found in the human population. The Collaborative Cross (CC) population is a genetically diverse recombinant inbred panel of mice. The CC offers large genotypic and phenotypic diversity, inbred strains with stable genomes that allow for repeated phenotypic measurements, and genomic tools including whole genome sequence to identify candidate genes and candidate ...

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Continuous epileptiform discharges during sleep as an evolutionary pattern in patients with congenital Zika virus syndrome

Abstract

Congenital Zika virus syndrome (CZVS) is associated with severe neurological deficits. Clinical characteristics of epilepsy and the electroencephalographic (EEG) pattern in CZVS were documented in infancy. In this study, we aimed to describe the EEG findings observed during the follow‐up of children with CZVS. Seventy‐six EEGs of 55 children (60% female; mean age = 50 months) with confirmed CZVS were analyzed, considering the background, interictal, and ictal epileptiform discharges. Continuous (or almost continuous) epileptiform discharges during non–rapid eye movement sleep were ...

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Cardiovascular safety of fenfluramine in the treatment of Dravet syndrome: Analysis of an ongoing long‐term open‐label safety extension study

Abstract

Objective

Fenfluramine, which was previously approved as a weight loss drug, was withdrawn in 1997 when reports of cardiac valvulopathy emerged. The present study was conducted in part to characterize the cardiovascular safety profile of low‐dose fenfluramine when used in a pediatric population to reduce seizure frequency in patients with Dravet syndrome.

Methods

Patients 2‐ to 18‐years‐old with Dravet syndrome who had completed any of three randomized, placebo‐controlled clinical trials of fenfluramine were offered enrollment in this open‐label extension (OLE) study. All patients ...

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Nonelective hospital admissions, discharge disposition, and health services utilization in epilepsy patients: A population‐based study

Abstract

Objective

Identifying adverse outcomes and examining trends and causes of nonelective admissions among persons with epilepsy would be beneficial to optimize patient care and reduce health services utilization. We examined the association of epilepsy with discharge status, in‐hospital mortality, length‐of‐stay, and charges. We also examined 10‐year trends and causes of hospital admissions among those with and without epilepsy.

Methods

Nonelective hospital admission in persons with epilepsy was identified in the 2005‐2014 National Inpatient Sample (NIS) using a validated International Classification of Diseases, 9th ...

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