Understanding new-onset refractory status epilepticus from an immunological point of view

New-onset refractory status epilepticus (NORSE) is unexpected onset of refractory status epilepticus in individuals with no preexisting relevant neurologic condition. The etiologies remain largely cryptogenic; treatment is challenging after failure to control seizures despite use of multiple antiepi...

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Bibliographic Details
Main Author: Yong-Won Shin
Format: Article
Language:English
Published: Korean Encephalitis and Neuroinflammation Society 2021-07-01
Series:Encephalitis
Subjects:
Online Access:http://www.encephalitisjournal.org/upload/pdf/encephalitis-2021-00045.pdf
Description
Summary:New-onset refractory status epilepticus (NORSE) is unexpected onset of refractory status epilepticus in individuals with no preexisting relevant neurologic condition. The etiologies remain largely cryptogenic; treatment is challenging after failure to control seizures despite use of multiple antiepileptic drugs and anesthetic agents. Frequent fever and other infectious prodromes, elevated proinflammatory cytokine/chemokine levels, and limbic or multifocal brain lesions indicate active inflammation in NORSE. Among identified causes, autoimmune encephalitis is the most common and accounts for more than one-third of all known NORSE cases, followed by infection-related etiologies. Although more evidence is needed, anti-cytokine therapies with tocilizumab and anakinra along with other immunotherapeutic agents used in autoimmune encephalitis can aid in alleviating or hindering the inflammatory cascade and controlling seizures.
ISSN:2765-4559
2734-1461