New onset refractory status epilepticus: Long-term outcomes beyond seizures.

We propose and prioritize important outcome domains that should be considered for future research investigating long-term outcomes (LTO) after new onset refractory status epilepticus (NORSE). The study was led by the international NORSE Institute LTO Working Group. First, literature describing the LTO of NORSE survivors was identified using a PubMed search and summarized to identify knowledge gaps.

Age-associated differences in FIRES: Characterizing prodromal presentation and long-term outcomes via the web-based NORSE/FIRES Family Registry.

Febrile infection-related epilepsy syndrome (FIRES) is a rare clinical presentation of refractory status epilepticus following a febrile infection. This study analyzes data from the NORSE/FIRES Family Registry, an international web-based registry available in six languages with data entered by patients, families, and clinicians to explore clinical presentations, survivorship, and long-term outcomes in adult and pediatric FIRES patients. We characterize and examine differences in demographics, prodromal symptoms, seizure frequency, anti-seizure medications (ASMs), quality of life, cognition, mood, and anxiety in adults vs pediatric populations with FIRES.

Targeting N-Myc in neuroblastoma with selective Aurora kinase A degraders.

The N-Myc transcription factor, encoded by MYCN, is a mechanistically validated, yet challenging, target for neuroblastoma (NB) therapy development. In normal neuronal progenitors, N-Myc undergoes rapid degradation, while, in MYCN-amplified NB cells, Aurora kinase A (Aurora-A) binds to and stabilizes N-Myc, resulting in elevated protein levels. Here, we demonstrate that targeted protein degradation of Aurora-A decreases N-Myc levels.

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.