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Extreme cryptogenic new onset refractory status epilepticus/febrile infection‐related epilepsy syndrome: Evidence of profound neuroinflammation and neuronal injury.

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Title: Extreme cryptogenic new onset refractory status epilepticus/febrile infection‐related epilepsy syndrome: Evidence of profound neuroinflammation and neuronal injury.
Authors: Muccioli, Lorenzo (AUTHOR), Ferri, Lorenzo (AUTHOR), Di Vito, Lidia (AUTHOR), Pasini, Elena (AUTHOR), Mostacci, Barbara (AUTHOR), Castioni, Carlo Alberto (AUTHOR), Bisulli, Francesca (AUTHOR)
Source: Epilepsia (Series 4). Apr2026, Vol. 67 Issue 4, p2041-2044. 4p.
Subjects: Neuroinflammation, Status epilepticus, Seizures (Medicine), Brain damage, Epilepsy, Brain injuries, Cerebrospinal fluid examination
Abstract: The article focuses on an extreme subtype of cryptogenic new onset refractory status epilepticus (c-NORSE), overlapping with febrile infection-related epilepsy syndrome (FIRES), characterized by stimulus-induced seizures during isoelectric EEG states and associated with poor prognosis. It presents a detailed case of a 32-year-old woman who developed superrefractory status epilepticus following a febrile illness, exhibiting electroclinical features consistent with this subtype, including seizures triggered by tactile stimulation despite multiple anesthetics and immunotherapies. Neuroimaging revealed extensive bilateral brain lesions, and cerebrospinal fluid analysis showed marked neuroinflammation and elevated biomarkers indicative of severe neuronal injury. These findings underscore the severity of this c-NORSE/FIRES phenotype, highlight the limitations of current treatments, and emphasize the need for improved understanding and targeted therapies. [Extracted from the article]
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Database: Psychology and Behavioral Sciences Collection
Description
Abstract:The article focuses on an extreme subtype of cryptogenic new onset refractory status epilepticus (c-NORSE), overlapping with febrile infection-related epilepsy syndrome (FIRES), characterized by stimulus-induced seizures during isoelectric EEG states and associated with poor prognosis. It presents a detailed case of a 32-year-old woman who developed superrefractory status epilepticus following a febrile illness, exhibiting electroclinical features consistent with this subtype, including seizures triggered by tactile stimulation despite multiple anesthetics and immunotherapies. Neuroimaging revealed extensive bilateral brain lesions, and cerebrospinal fluid analysis showed marked neuroinflammation and elevated biomarkers indicative of severe neuronal injury. These findings underscore the severity of this c-NORSE/FIRES phenotype, highlight the limitations of current treatments, and emphasize the need for improved understanding and targeted therapies. [Extracted from the article]
ISSN:00139580
DOI:10.1002/epi.70157