Time to FIRE NORSE: A single acronym for a heterogeneous presentation. Further information from a case series and discussion of the literature.

PURPOSE: New-Onset Refractory Status Epilepticus (NORSE) is a rare and severe form of refractory status epilepticus without an apparent underlying cause at presentation or prior history of epilepsy. We aimed to describe the clinical features, etiology, treatment, and outcomes of NORSE in adults in a quaternary-level hospital in Saudi Arabia. METHODOLOGY: In this retrospective cohort study, inclusion criteria involved patients over 14 years old who met the 2018 consensus definition for NORSE. Patients were identified using a combination of medical record admission labels 'status epilepticus' and 'encephalitis', and continuous EEG reports documenting status epilepticus. Demographic, clinical, and radiological data were collected and then analyzed for factors correlated with specific etiologies, better functional outcomes, and future diagnosis of epilepsy. RESULTS: We found 24 patients presenting with NORSE between 2010 and 2021. Fever/infectious symptoms were the most common prodrome. Elevated inflammatory serum and cerebrospinal fluid markers in most patients. Brain MRI revealed T2/FLAIR hyperintensity patterns, predominantly affecting limbic and perisylvian structures. The etiology of NORSE varied, with immune-related causes being the most common. Long-term outcomes were poor, with a high mortality rate and most survivors developing drug-resistant epilepsy. CONCLUSION: This study provides valuable insights into NORSE's clinical characteristics, highlighting the heterogeneity of this condition. The poor outcome is likely related to the progressive nature of the underlying disease, where refractory seizures are a clinical symptom. Thus, we propose to focus future research on the etiology rather than the NORSE acronym.

Vagus nerve stimulation in medically refractory epilepsy: Adverse effects and clinical correlates.

OBJECTIVES: To assess the frequency of adverse effects among pediatric and adult patients and the clinical variables associated with a higher probability of developing side effects. METHODS: This retrospective study enrolled pediatric and adult patients who underwent Vagus nerve stimulation (VNS) implantation at our institution and had documented follow-up during clinic visits for at least 6 months after implantation. Data collected included demographic information, epilepsy diagnosis, and device data. RESULTS: A total of 43 patients with drug-resistant epilepsy who received a VNS device at our institution were enrolled. The median follow-up period was 12 months. Fourteen patients (32.55%) reported no side effects from VNS therapy. Side effects ranged from mild to severe, with significant side effects observed in 8 patients. Data on therapy efficacy were collected, and 10 patients (23.26%) reported no change in seizure frequency following device implantation. CONCLUSION: This study demonstrates that VNS is an important adjunct treatment option for epilepsy patients. Dysphagia and dyspnea can be significant adverse effects leading to treatment discontinuation, aspiration pneumonia, intensive care unit (ICU) admission, and prolonged hospital stay. These effects are more frequent in patients with symptomatic generalized epilepsy, global developmental delay at baseline, previous ICU admissions, abnormal brain magnetic resonance imaging findings, and seizures with multiple semiologies.