Background: The aim of this study was to identify early clinical features of patients with new-onset refractory status epilepticus (NORSE) that could direct the treatment in the first days of hospitalisation.
Methods: A retrospective cohort study of adult NORSE patients treated in the intensive care units of Helsinki University Hospital 2007-2018.
Results: We found 19 adult NORSE patients who divided into three subgroups on the basis of their clinical features: viral encephalitis (n = 5, 26%), febrile infection-related epilepsy syndrome (FIRES) (n = 6, 32%) and afebrile NORSE (n = 8, 42%). FIRES and afebrile NORSE patients remained without confirmed etiology, but retrospectively two paraneoplastic and two neurodegenerative causes were suspected in the afebrile NORSE group. Viral encephalitis patients were median 64 years old (IQR 55-64), and four (80%) had prodromal fever and abnormal findings in the first brain imaging. FIRES patients were median 21 years old (IQR 19-24), all febrile and had normal brain imaging at onset. In the afebrile NORSE group, median age was 67 (IQR 59-71) and 50% had prodromal cognitive or psychiatric symptoms. FIRES patients differed from other NORSE patients by younger age (p = 0.001), respiratory prodromal symptoms (p = 0.004), normal brain MRI (p = 0.044) and lack of comorbidities (p = 0.011). They needed more antiseizure medications (p = 0.001) and anesthetics (p = 0.002), had a longer hospital stay (p = 0.017) and more complications (p < 0.001).
Conclusions: Among febrile NORSE patients, FIRES group was distinctive due to patients' young age, prodromal respiratory symptoms and normal first brain imaging. These features should be confirmed by subsequent studies as basis for selecting patients for early intensive immunotherapy.
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http://dx.doi.org/10.1186/s12883-022-03028-y | DOI Listing |
Croat Med J
December 2024
Athanasios, Nafpliou 1 C, Gerakas 15344, Athens, Greece,
Although severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) primarily affects the respiratory system, neurological symptoms were reported both during acute and post-acute COVID-19. Notably, patients with no history of epilepsy or other neurological conditions developed new-onset refractory status epilepticus (NORSE) weeks, months, or even up to a year following the viral infection. While NORSE is uncommon, it carries a high mortality rate and can result in permanent epilepsy.
View Article and Find Full Text PDFEpilepsia
January 2025
Schulich School of Medicine and Dentistry, Western University, London, Ontario, Canada.
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.
View Article and Find Full Text PDFEpilepsia Open
January 2025
Neurology Department, Wellstar MCG Health at the Medical College of Georgia, Augusta, Georgia.
New Onset Refractory Status Epilepticus (NORSE) is a rare and severe condition characterized by refractory seizures in individuals without a prior history of epilepsy. This case report describes a 37-year-old woman diagnosed with anti-glutamic acid decarboxylase 65 (anti-GAD65) antibody-positive encephalitis-related NORSE. Her seizures were refractory to multiple interventions, including anti-seizure medications, anesthetics, immunotherapies, a ketogenic diet, and electroconvulsive therapy.
View Article and Find Full Text PDFJ Neuroimmunol
January 2025
Department of Neurology, All India Institute of Medical Sciences, New Delhi, India.
Myelin oligodendrocyte glycoprotein antibody-associated disease (MOGAD) is a rare cause of NORSE. We describe the case of a young girl who presented with NORSE associated with MOGAD along with a systematic review of all cases of NORSE associated with MOGAD till date. Seizures associated with MOGAD are usually associated with good outcome but can occasionally be catastrophic and non-responsive to conventional therapies.
View Article and Find Full Text PDFBMC Emerg Med
January 2025
Department of Emergency Medicine, University of Florida College of Medicine-Jacksonville, 655 West 8th Street, Jacksonville, FL, 32209, USA.
Background: The ongoing opioid epidemic in the United States has reinforced the need to provide multimodal and non-opioid pain management interventions. The PAMI-ED ALT program employed a multifaceted approach in the Emergency Department (ED) developing electronic health record (EHR) pain management order panels and discharge panels, as well as educating patients, clinicians, and ED staff on opioid alternatives, including non-pharmacologic interventions. The primary objective of this analysis was to compare changes in opioid and non-opioid analgesic administrations and prescribing in ED patients with select pain conditions (renal colic, headache, low back, and non-low back musculoskeletal pain) before and after implementation of PAMI ED-ALT.
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