Migraine with prolonged aphasic aura associated with a CACNA1A mutation: A case report and narrative review.

Objective: To demonstrate that a known CACNA1A variant is associated with a phenotype of prolonged aphasic aura without hemiparesis.

Background: The usual differential diagnosis of prolonged aphasia without hemiparesis includes vascular disease, seizure, metabolic derangements, and migraine. Genetic mutations in the CACNA1A gene can lead to a myriad of phenotypes, including familial hemiplegic migraine (FHM) type 1, an autosomal dominant disorder characterized by an aura of unilateral, sometimes prolonged weakness.

Recognizing seizure clusters in the community: The path to uniformity and individualization in nomenclature and definition.

Seizure emergencies experienced by patients with epilepsy include status epilepticus and seizure clusters. Although an accepted definition of status epilepticus exists, no clear consensus definition of seizure clusters has emerged; this is further complicated by the appearance in the literature of various empirically based definitions that have been developed for clinical trial study designs. In general, patients with intractable epilepsy have been shown to have a significant risk for acute episodes of increased seizure activity called seizure clusters (also referred to as acute repetitive seizures, among other terms) that differ from their usual seizure pattern.

Epilepsy-related stigma and attitudes: Systematic review of screening instruments and interventions - Report by the International League Against Epilepsy Task Force on Stigma in Epilepsy.

Objective: This is a systematic review aimed at summarizing the evidence related to instruments that have been developed to measure stigma or attitudes toward epilepsy and on stigma-reducing interventions.

Methods: This review followed the Preferred Reporting Items for Systematic Reviews and Meta-Analyses (PRISMA) standards. A broad literature search (1985-2019) was performed in 13 databases.

Psychogenic nonepileptic seizures during the COVID-19 pandemic in New York City - A distinct response from the epilepsy experience.

Although psychogenic nonepileptic seizures (PNES) are a common neurologic condition, there remains a paucity of literature on the COVID-19 pandemic's effect on these patients. Using a cross-sectional questionnaire study, our group examined the experience of patients with PNES at a single Comprehensive Epilepsy Center in New York City, the epicenter of the initial COVID-19 outbreak in the United States. Among our cohort of 18 subjects with PNES, 22.

Factors associated with patients not proceeding with proposed resective epilepsy surgery.

Background: This study evaluated the association between eligible patients not proceeding with resective epilepsy surgery and various demographic, disease-specific, and epilepsy-evaluation variables.

Methods: This retrospective case-control study included patients identified as candidates for resective epilepsy surgery at the Montefiore Medical Center between January 1, 2009 and June 30, 2017. Chi-squared, two-tailed, independent sample t-test, Mann-Whitney U test and logistic regression were utilized to identify variables associated with patients not proceeding with surgery.

Folding a neuroscience center into streamlined COVID-19 response teams: Lessons in origami.

In response to the COVID-19 pandemic epicenter in Bronx, NY, the Montefiore Neuroscience Center required rapid and drastic changes when considering the delivery of neurologic care, health and safety of staff, and continued education and safety for house staff. Health care leaders rely on principles that can be in conflict during a disaster response such as this pandemic, with equal commitments to ensure the best care for those stricken with COVID-19, provide high-quality care and advocacy for patients and families coping with neurologic disease, and advocate for the health and safety of health care teams, particularly house staff and colleagues who are most vulnerable. In our attempt to balance these principles, over 3 weeks, we reformatted our inpatient neuroscience services by reducing from 4 wards to just 1, in the following weeks delivering care to over 600 hospitalized patients with neuro-COVID and over 1,742 total neuroscience hospital bed days.

EEG findings in acutely ill patients investigated for SARS-CoV-2/COVID-19: A small case series preliminary report.

Objective: Acute encephalopathy may occur in COVID-19-infected patients. We investigated whether medically indicated EEGs performed in acutely ill patients under investigation (PUIs) for COVID-19 report epileptiform abnormalities and whether these are more prevalent in COVID-19 positive than negative patients.

Methods: In this retrospective case series, adult COVID-19 inpatient PUIs underwent EEGs for acute encephalopathy and/or seizure-like events.

Individualizing the definition of seizure clusters based on temporal clustering analysis.

Objective: Seizure clusters are often encountered in people with poorly controlled epilepsy. Detection of seizure clusters is currently based on simple clinical rules, such as two seizures separated by four or fewer hours or multiple seizures in 24 h. Current definitions fail to distinguish between statistically significant clusters and those that may result from natural variation in the person's seizures.

Arfgef1 haploinsufficiency in mice alters neuronal endosome composition and decreases membrane surface postsynaptic GABA receptors.

ARFGEF1 encodes a guanine exchange factor involved in intracellular vesicle trafficking, and is a candidate gene for childhood genetic epilepsies. To model ARFGEF1 haploinsufficiency observed in a recent Lennox Gastaut Syndrome patient, we studied a frameshift mutation (Arfgef1) in mice. Arfgef1 pups exhibit signs of developmental delay, and Arfgef1 adults have a significantly decreased threshold to induced seizures but do not experience spontaneous seizures.

Seizure self-prediction in a randomized controlled trial of stress management.

Objective: Using electronic diaries as part of a randomized controlled trial of stress reduction for epilepsy, we evaluated factors associated with successful seizure self-prediction.

Methods: Adults with medication-resistant focal epilepsy were recruited from 3 centers and randomized to treatment with progressive muscle relaxation or control focused attention. An 8-week baseline was followed by 12 weeks of double-blind treatment.

Behavioral interventions in epilepsy.

Purpose Of Review: Behavioral intervention describes multiple modalities of treatments which are of increasing interest in epilepsy. This review addresses recent behavioral clinical trials in epilepsy including cognitive behavioral therapy (CBT), mindfulness, progressive muscle relaxation (PMR), and self-management. Results and conclusions from updated Cochrane reviews and the recent International League Against Epilepsy Psychology task force are presented.

Characteristics of large patient-reported outcomes: Where can one million seizures get us?

Objective: To analyze data from Seizure Tracker, a large electronic seizure diary, including comparison of seizure characteristics among different etiologies, temporal patterns in seizure fluctuations, and specific triggers.

Methods: Zero-inflated negative binomial mixed-effects models were used to evaluate temporal patterns of seizure events (during the day or week), as well as group differences in monthly seizure frequency between children and adults and between etiologies. The association of long seizures with seizure triggers was evaluated using a mixed-effects logistic model with subject as the random effect.

Mentored peer review of standardized manuscripts as a teaching tool for residents: a pilot randomized controlled multi-center study.

Background: There is increasing need for peer reviewers as the scientific literature grows. Formal education in biostatistics and research methodology during residency training is lacking. In this pilot study, we addressed these issues by evaluating a novel method of teaching residents about biostatistics and research methodology using peer review of standardized manuscripts.

Behavioral interventions as a treatment for epilepsy: A multicenter randomized controlled trial.

Objective: To evaluate the effect of a stress-reduction intervention in participants with medication-resistant epilepsy.

Methods: Adults with medication-resistant focal epilepsy (n = 66) were recruited from 3 centers and randomized to 1 of 2 interventions: (1) progressive muscle relaxation (PMR) with diaphragmatic breathing, or (2) control focused-attention activity with extremity movements. Following an 8-week baseline period, participants began 12 weeks of double-blind treatment.

Clinical characterization of the pre-ictal state in the pediatric population: A caretaker's perspective.

The unpredictability of seizures causes distress to patients with epilepsy and their caretakers. To date, no studies have explored seizure prediction specifically in the pediatric population. If the period of time preceding a seizure can be reliably identified, either by child or caretaker, there may be a role for pre-emptive interventions.

Instruction manual for the ILAE 2017 operational classification of seizure types.

This companion paper to the introduction of the International League Against Epilepsy (ILAE) 2017 classification of seizure types provides guidance on how to employ the classification. Illustration of the classification is enacted by tables, a glossary of relevant terms, mapping of old to new terms, suggested abbreviations, and examples. Basic and extended versions of the classification are available, depending on the desired degree of detail.

A dedicated scholarly research program in an adult and pediatric neurology residency program.

Objective: To describe and assess the effectiveness of a formal scholarly activity program for a highly integrated adult and pediatric neurology residency program.

Methods: Starting in 2011, all graduating residents were required to complete at least one form of scholarly activity broadly defined to include peer-reviewed publications or presentations at scientific meetings of formally mentored projects. The scholarly activity program was administered by the associate residency training director and included an expanded journal club, guided mentorship, a required grand rounds platform presentation, and annual awards for the most scholarly and seminal research findings.

Benzodiazepine use in seizure emergencies: A systematic review.

Purpose: The aim of this review was to systematically examine safety and efficacy outcomes, as well as patient/caregiver satisfaction, from clinical studies in pediatric and adult patients treated with benzodiazepines (BZDs) through various administration routes in response to seizure emergencies.

Methods: A literature search was conducted to identify articles describing the use of various routes of administration (RoAs) of BZDs for the treatment of seizure emergencies through April 21, 2015, using Embase™ and PubMed®. Eligible studies included (a) randomized controlled trials or (b) controlled nonrandomized clinical trials, either retrospective or prospective.

Termination of seizure clusters is related to the duration of focal seizures.

Objective: Clustered seizures are characterized by shorter than usual interseizure intervals and pose increased morbidity risk. This study examines the characteristics of seizures that cluster, with special attention to the final seizure in a cluster.

Methods: This is a retrospective analysis of long-term inpatient monitoring data from the EPILEPSIAE project.

Childhood trauma in patients with self-reported stress-precipitated seizures.

Objective: Stress is the most commonly reported precipitant of epileptic seizures, but the mechanism by which stress precipitates seizures and the risk factors for stress as a seizure precipitant are poorly understood. Previously, we observed higher levels of anxiety symptoms in patients with epilepsy who reported stress as a seizure precipitant. Given that childhood trauma increases the risk of general psychiatric symptom burden, including anxiety symptoms, we sought to examine the relationship between childhood adversity and stress as a seizure precipitant.

Seizure clusters: characteristics and treatment.

Purpose Of Review: Many patients with epilepsy experience 'clusters' or flurries of seizures, also termed acute repetitive seizures (ARS). Seizure clustering has a significant impact on health and quality of life. This review summarizes recent advances in the definition and neurophysiologic understanding of clustering, the epidemiology and risk factors for clustering and both inpatient and outpatient clinical implications.