학술논문
Time to FIRE NORSE: A single acronym for a heterogeneous presentation. Further information from a case series and discussion of the literature.
Document Type
Academic Journal
Author
Alaskar AM; King Salman Ibn Abdulaziz Medical City, Madinah, Saudi Arabia.; Aljohani MA; King Faisal Specialist Hospital & Research Center, Riyadh, Saudi Arabia.; Dionisio S; King Faisal Specialist Hospital & Research Center, Riyadh, Saudi Arabia; Alfaisal University, Riyadh, Saudi Arabia; University of Queensland, Brisbane, Australia.; Asiry MA; King Faisal Specialist Hospital & Research Center, Riyadh, Saudi Arabia.; Alqadi K; King Salman Ibn Abdulaziz Medical City, Madinah, Saudi Arabia. Electronic address: dr.k.alqadi@gmail.com.
Source
Publisher: Elsevier/North-Holland Country of Publication: Netherlands NLM ID: 8109498 Publication Model: Print-Electronic Cited Medium: Internet ISSN: 1872-8421 (Electronic) Linking ISSN: 01655728 NLM ISO Abbreviation: J Neuroimmunol Subsets: MEDLINE
Subject
Language
English
Abstract
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.
Competing Interests: Declaration of competing interest The authors have no competing interests, have not used AI and AI-assisted technologies in the writing process and reports no disclosures relevant to the manuscript.
(Copyright © 2023. Published by Elsevier B.V.)
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.
Competing Interests: Declaration of competing interest The authors have no competing interests, have not used AI and AI-assisted technologies in the writing process and reports no disclosures relevant to the manuscript.
(Copyright © 2023. Published by Elsevier B.V.)