Navigating life after New-onset refractory status epilepticus (NORSE) and Febrile infection-related epilepsy syndrome (FIRES): Insights from caregiver and patient interviews.

Objective: To describe the lived experience of patients with NORSE and explore quality of life (QOL) for patients and their caregivers.

Background: NORSE is a rare condition characterized by refractory status epilepticus, often of unknown cause, in a previously neurologically healthy individual. Febrile infection-related epilepsy syndrome (FIRES) is a subset of NORSE.

Accuracy of a Rapid-Response EEG's Automated Seizure-Burden Estimator: AccuRASE Study.

Background And Objectives: The use of rapid response EEG (rr-EEG) has recently expanded in limited-resource settings and as a supplement to conventional EEG to rapidly detect and treat nonconvulsive status epilepticus. The study objective was to test the accuracy of an rr-EEG's automated seizure burden estimator (ASBE).

Methods: This is a retrospective observational study using multiple blinded reviewers.

Corrigendum: A practical approach to in-hospital management of new-onset refractory status epilepticus/febrile infection related epilepsy syndrome.

[This corrects the article DOI: 10.3389/fneur.2023.

Brain on Fire: How Brain Infection and Neuroinflammation Drive Worldwide Epilepsy Burden.

Roughly 80% of the global burden of epilepsy resides in low- and middle-income countries (LMICs; WHO, 2022). Despite numerous new therapies for the treatment of epilepsy, the number of patients who remain resistant to available medications is unchanged. Additionally, no therapy has yet been clinically proven to prevent or attenuate the development of epilepsy in at-risk individuals.

Polygenic Risk of Epilepsy and Poststroke Epilepsy.

Background: Epilepsy is highly heritable, with numerous known genetic risk loci. However, the genetic predisposition's role in poststroke epilepsy (PSE) remains understudied. This study assesses whether a higher genetic predisposition to epilepsy raises poststroke survivor's risk of PSE.

The human claustrum tracks slow waves during sleep.

Slow waves are a distinguishing feature of non-rapid-eye-movement (NREM) sleep, an evolutionarily conserved process critical for brain function. Non-human studies suggest that the claustrum, a small subcortical nucleus, coordinates slow waves. We show that, in contrast to neurons from other brain regions, claustrum neurons in the human brain increase their spiking activity and track slow waves during NREM sleep, suggesting that the claustrum plays a role in coordinating human sleep architecture.

Stability of infraslow correlation structure in time-shifted intracranial EEG signals.

It is increasingly understood that the epilepsies are characterized by network pathology that can span multiple spatial and temporal scales. Recent work indicates that infraslow (<0.2 Hz) envelope correlations may form a basis for distant spatial coupling in the brain.

Cyclic Alternating EEG Patterns: From Sleep to Encephalopathy.

In the 2021 version of the Standardized Critical Care EEG Terminology, the American Clinical Neurophysiology Society introduced new definitions, including for the cyclic alternating pattern of encephalopathy (CAPE). CAPE refers to changes in background EEG activity, with two patterns alternating spontaneously in a regular manner. CAPE shares remarkable similarities with the cyclic alternating pattern, a natural EEG phenomenon occurring in normal non-rapid eye movement sleep, considered the main electrophysiological biomarker of sleep instability.

Antiseizure Medication Withdrawal, Risk of Epilepsy, and Longterm EEG Trends in Acute Symptomatic Seizures or Epileptic EEG Patterns.

Background And Objectives: Patients with acute symptomatic seizures (ASyS) and acute epileptiform findings on EEG are common. They are often prescribed long-term antiseizure medications (ASMs); it is unknown whether or when this is necessary. Primary outcome was late unprovoked seizure occurrence and association with ASM taper.

Evaluating the Impact of Point-of-Care Electroencephalography on Length of Stay in the Intensive Care Unit: Subanalysis of the SAFER-EEG Trial.

Background: Electroencephalography (EEG) is needed to diagnose nonconvulsive seizures. Prolonged nonconvulsive seizures are associated with neuronal injuries and deleterious clinical outcomes. However, it is uncertain whether the rapid identification of these seizures using point-of-care EEG (POC-EEG) can have a positive impact on clinical outcomes.

Seizure Assessment and Forecasting With Efficient Rapid-EEG: A Retrospective Multicenter Comparative Effectiveness Study.

Background And Objectives: Approximately 30% of critically ill patients have seizures, and more than half of these seizures do not have an overt clinical correlate. EEG is needed to avoid missing seizures and prevent overtreatment with antiseizure medications. Conventional-EEG (cEEG) resources are logistically constrained and unable to meet their growing demand for seizure detection even in highly developed centers.

Combined neuromodulation and resection for functional cortex epilepsy: a case series.

Objective: Medically refractory epilepsy (MRE) often requires resection of the seizure onset zone (SOZ) for effective treatment. However, when the SOZ is in functional cortex (FC), achieving complete and safe resection becomes difficult, due to the seizure network overlap with function. The authors aimed to assess the safety and outcomes of a combined approach involving partial resection combined with focal neuromodulation for FC refractory epilepsy.

Outpatient management of prolonged seizures and seizure clusters to prevent progression to a higher-level emergency: Consensus recommendations of an expert working group.

Objective: The management of prolonged seizures (PS) and seizure clusters (SC) is impeded by the lack of international, evidence-based guidance. We aimed to develop expert recommendations regarding consensus definitions of PS, SC, and treatment goals to prevent progression to higher-level emergencies such as status epilepticus (SE).

Methods: An expert working group, comprising 12 epileptologists, neurologists, and pharmacologists from Europe and North America, used a modified Delphi consensus methodology to develop and anonymously vote on statements.

Comparative analysis of patients with new onset refractory status epilepticus preceded by fever (febrile infection-related epilepsy syndrome) versus without prior fever: An interim analysis.

Febrile infection-related epilepsy syndrome (FIRES) is a subset of new onset refractory status epilepticus (NORSE) that involves a febrile infection prior to the onset of the refractory status epilepticus. It is unclear whether FIRES and non-FIRES NORSE are distinct conditions. Here, we compare 34 patients with FIRES to 30 patients with non-FIRES NORSE for demographics, clinical features, neuroimaging, and outcomes.

The Cost of After-Hour Electroencephalography.

Background And Objectives: High costs associated with after-hour electroencephalography (EEG) constitute a barrier for financially constrained hospitals to provide this neurodiagnostic procedure outside regular working hours. Our study aims to deepen our understanding of the cost elements involved in delivering EEG services during after-hours.

Methods: We accessed publicly available data sets and created a cost model depending on 3 most commonly seen staffing scenarios: (1) technologist on-site, (2) technologist on-call from home, and (3) a hybrid of the two.

Systematic 1 Hz direct electrical stimulation for seizure induction: A reliable method for localizing seizure onset zone and predicting seizure freedom.

Objective: To prospectively investigate the utility of seizure induction using systematic 1 Hz stimulation by exploring its concordance with the spontaneous seizure onset zone (SOZ) and relation to surgical outcome; comparison with seizures induced by non-systematic 50 Hz stimulation was attempted as well.

Methods: Prospective cohort study from 2018 to 2021 with ≥ 1 y post-surgery follow up at Yale New Haven Hospital. With 1 Hz, all or most of the gray matter contacts were stimulated at 1, 5, and 10 mA for 30-60s.

Treatment of Refractory Status Epilepticus With Continuous Intravenous Anesthetic Drugs: A Systematic Review.

Importance: Multiple continuous intravenous anesthetic drugs (CIVADs) are available for the treatment of refractory status epilepticus (RSE). There is a paucity of data comparing the different types of CIVADs used for RSE.

Objective: To systematically review and compare outcome measures associated with the initial CIVAD choice in RSE in accordance with the Preferred Reporting Items for Systematic Reviews and Meta-Analyses guidelines.

Second-line immunotherapy in new onset refractory status epilepticus.

Several pieces of evidence suggest immune dysregulation could trigger the onset and modulate sequelae of new onset refractory status epilepticus (NORSE), including its subtype with prior fever known as febrile infection-related epilepsy syndrome (FIRES). Consensus-driven recommendations have been established to guide the initiation of first- and second-line immunotherapies in these patients. Here, we review the literature to date on second-line immunotherapy for NORSE/FIRES, presenting results from 28 case reports and series describing the use of anakinra, tocilizumab, or intrathecal dexamethasone in 75 patients with NORSE.