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. |
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| 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] |
| Copyright of Epilepsia (Series 4) is the property of Wiley-Blackwell and its content may not be copied or emailed to multiple sites without the copyright holder's express written permission. Additionally, content may not be used with any artificial intelligence tools or machine learning technologies. However, users may print, download, or email articles for individual use. This abstract may be abridged. No warranty is given about the accuracy of the copy. Users should refer to the original published version of the material for the full abstract. (Copyright applies to all Abstracts.) | |
| Database: | Psychology and Behavioral Sciences Collection |
| FullText | Text: Availability: 0 |
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| Header | DbId: pbh DbLabel: Psychology and Behavioral Sciences Collection An: 192955211 AccessLevel: 6 PubType: Academic Journal PubTypeId: academicJournal PreciseRelevancyScore: 0 |
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| Items | – Name: Title Label: Title Group: Ti Data: Extreme cryptogenic new onset refractory status epilepticus/febrile infection‐related epilepsy syndrome: Evidence of profound neuroinflammation and neuronal injury. – Name: Author Label: Authors Group: Au Data: <searchLink fieldCode="AR" term="%22Muccioli%2C+Lorenzo%22">Muccioli, Lorenzo</searchLink> (AUTHOR)<br /><searchLink fieldCode="AR" term="%22Ferri%2C+Lorenzo%22">Ferri, Lorenzo</searchLink> (AUTHOR)<br /><searchLink fieldCode="AR" term="%22Di+Vito%2C+Lidia%22">Di Vito, Lidia</searchLink> (AUTHOR)<br /><searchLink fieldCode="AR" term="%22Pasini%2C+Elena%22">Pasini, Elena</searchLink> (AUTHOR)<br /><searchLink fieldCode="AR" term="%22Mostacci%2C+Barbara%22">Mostacci, Barbara</searchLink> (AUTHOR)<br /><searchLink fieldCode="AR" term="%22Castioni%2C+Carlo+Alberto%22">Castioni, Carlo Alberto</searchLink> (AUTHOR)<br /><searchLink fieldCode="AR" term="%22Bisulli%2C+Francesca%22">Bisulli, Francesca</searchLink> (AUTHOR) – Name: TitleSource Label: Source Group: Src Data: <searchLink fieldCode="JN" term="%22Epilepsia+%28Series+4%29%22">Epilepsia (Series 4)</searchLink>. Apr2026, Vol. 67 Issue 4, p2041-2044. 4p. – Name: Subject Label: Subjects Group: Su Data: <searchLink fieldCode="DE" term="%22Neuroinflammation%22">Neuroinflammation</searchLink><br /><searchLink fieldCode="DE" term="%22Status+epilepticus%22">Status epilepticus</searchLink><br /><searchLink fieldCode="DE" term="%22Seizures+%28Medicine%29%22">Seizures (Medicine)</searchLink><br /><searchLink fieldCode="DE" term="%22Brain+damage%22">Brain damage</searchLink><br /><searchLink fieldCode="DE" term="%22Epilepsy%22">Epilepsy</searchLink><br /><searchLink fieldCode="DE" term="%22Brain+injuries%22">Brain injuries</searchLink><br /><searchLink fieldCode="DE" term="%22Cerebrospinal+fluid+examination%22">Cerebrospinal fluid examination</searchLink> – Name: Abstract Label: Abstract Group: Ab Data: 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] – Name: AbstractSuppliedCopyright Label: Group: Ab Data: <i>Copyright of Epilepsia (Series 4) is the property of Wiley-Blackwell and its content may not be copied or emailed to multiple sites without the copyright holder's express written permission. Additionally, content may not be used with any artificial intelligence tools or machine learning technologies. However, users may print, download, or email articles for individual use. This abstract may be abridged. No warranty is given about the accuracy of the copy. Users should refer to the original published version of the material for the full abstract.</i> (Copyright applies to all Abstracts.) |
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| RecordInfo | BibRecord: BibEntity: Identifiers: – Type: doi Value: 10.1002/epi.70157 Languages: – Code: eng Text: English PhysicalDescription: Pagination: PageCount: 4 StartPage: 2041 Subjects: – SubjectFull: Neuroinflammation Type: general – SubjectFull: Status epilepticus Type: general – SubjectFull: Seizures (Medicine) Type: general – SubjectFull: Brain damage Type: general – SubjectFull: Epilepsy Type: general – SubjectFull: Brain injuries Type: general – SubjectFull: Cerebrospinal fluid examination Type: general Titles: – TitleFull: Extreme cryptogenic new onset refractory status epilepticus/febrile infection‐related epilepsy syndrome: Evidence of profound neuroinflammation and neuronal injury. Type: main BibRelationships: HasContributorRelationships: – PersonEntity: Name: NameFull: Muccioli, Lorenzo – PersonEntity: Name: NameFull: Ferri, Lorenzo – PersonEntity: Name: NameFull: Di Vito, Lidia – PersonEntity: Name: NameFull: Pasini, Elena – PersonEntity: Name: NameFull: Mostacci, Barbara – PersonEntity: Name: NameFull: Castioni, Carlo Alberto – PersonEntity: Name: NameFull: Bisulli, Francesca IsPartOfRelationships: – BibEntity: Dates: – D: 01 M: 04 Text: Apr2026 Type: published Y: 2026 Identifiers: – Type: issn-print Value: 00139580 Numbering: – Type: volume Value: 67 – Type: issue Value: 4 Titles: – TitleFull: Epilepsia (Series 4) Type: main |
| ResultId | 1 |
