Febrile Infection-Related Epilepsy Syndrome (FIRES) in a Young Adult: A Case Report Highlighting Advanced Neuroimaging

MCML Authors

Fabian Bongratz

→ Group Christian Wachinger
 Artificial Intelligence in Medical Imaging

Christian Wachinger

Prof. Dr.

Principal Investigator

Artificial Intelligence in Medical Imaging

Abstract

Febrile infection-related epilepsy syndrome (FIRES) is a rare and severe subtype of new-onset refractory status epilepticus (NORSE), typically affecting previously healthy children and young adults. It usually follows a nonspecific febrile illness and progresses rapidly to refractory status epilepticus, often without an identifiable infectious agent. The pathogenesis remains unclear but likely involves an infection-triggered inflammatory response [1]. Diagnosis is often delayed due to overlap with other causes of new-onset seizures, including autoimmune encephalitis, infectious encephalitis, other infection-induced encephalopathies, and toxic-metabolic disturbances. MRI may aid in diagnosis, with bilateral T2-hyperintensities in the claustrum (“claustrum sign”) being a characteristic, though not pathognomonic, finding. While anti-seizure medications alone are often insufficient, immunomodulatory therapies including corticosteroids, IVIG, and cytokine-targeted therapies—as well as other supportive approaches—have shown promise and have contributed to the development of a consensus-based treatment framework [2]. The outcome is often poor and frequently associated with long-term cognitive impairment and chronic epilepsy [3]. This case adds to the growing body of literature on young adult-onset FIRES, and the potential benefit of early immunotherapy. Furthermore, advanced neuroimaging was useful in monitoring disease evolution and resolution in this patient.

article

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Clinical Neuroradiology

Oct. 2025.

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Authors

E. O. Riedel • F. Bongratz • P. T. Zebhauser • M. Mühlau • C. Wachinger • D. M. Hedderich

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Links

DOI

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Research Area

 C1 | Medicine