Sevoflurane as bridge therapy for plasma exchange and Anakinra in febrile infection–related epilepsy syndrome

Abstract Febrile infection–related epilepsy syndrome (FIRES) is a devastating immune inflammatory–mediated epileptic encephalopathy. Herein, we discuss a previously healthy 8‐year‐old boy with FIRES in whom high dosages of conventional and nonconventional anesthetics were ineffective in treating SE,...

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Main Authors: Manuela L’Erario, Rosa Maria Roperto, Anna Rosati
Format: Article
Language:English
Published: Wiley 2021-12-01
Series:Epilepsia Open
Subjects:
Online Access:https://doi.org/10.1002/epi4.12545
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author Manuela L’Erario
Rosa Maria Roperto
Anna Rosati
author_facet Manuela L’Erario
Rosa Maria Roperto
Anna Rosati
author_sort Manuela L’Erario
collection DOAJ
description Abstract Febrile infection–related epilepsy syndrome (FIRES) is a devastating immune inflammatory–mediated epileptic encephalopathy. Herein, we discuss a previously healthy 8‐year‐old boy with FIRES in whom high dosages of conventional and nonconventional anesthetics were ineffective in treating SE, as were ketogenic diet, intravenous corticosteroids, and immunoglobulins. After 29 days of prolonged SRSE, the patient was successfully treated with sevoflurane paired with plasma exchange, for a total of five days, thus obtaining a stable EEG suppression burst pattern with no adverse events. Anakinra at the dosage of 100 mg b.i.d. was started seven days after sevoflurane and plasma exchange had been discontinued and was effective in ensuring non‐recurrence of SE. Sevoflurane as bridge therapy for immunosuppressive treatment could be considered an early, safe, and effective option in treating convulsive SE in which an autoimmune‐inflammatory etiology can reasonably be hypothesized.
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spelling doaj.art-6637943c79914a3d8d5eb456ffa10d0c2022-12-21T19:16:42ZengWileyEpilepsia Open2470-92392021-12-016478879210.1002/epi4.12545Sevoflurane as bridge therapy for plasma exchange and Anakinra in febrile infection–related epilepsy syndromeManuela L’Erario0Rosa Maria Roperto1Anna Rosati2Intensive Care Unit Meyer Children’s Hospital Florence ItalyNephrology Department Meyer Children’s Hospital University of Florence Florence ItalyPaediatric Neurology Unit Meyer Children’s Hospital University of Florence Florence ItalyAbstract Febrile infection–related epilepsy syndrome (FIRES) is a devastating immune inflammatory–mediated epileptic encephalopathy. Herein, we discuss a previously healthy 8‐year‐old boy with FIRES in whom high dosages of conventional and nonconventional anesthetics were ineffective in treating SE, as were ketogenic diet, intravenous corticosteroids, and immunoglobulins. After 29 days of prolonged SRSE, the patient was successfully treated with sevoflurane paired with plasma exchange, for a total of five days, thus obtaining a stable EEG suppression burst pattern with no adverse events. Anakinra at the dosage of 100 mg b.i.d. was started seven days after sevoflurane and plasma exchange had been discontinued and was effective in ensuring non‐recurrence of SE. Sevoflurane as bridge therapy for immunosuppressive treatment could be considered an early, safe, and effective option in treating convulsive SE in which an autoimmune‐inflammatory etiology can reasonably be hypothesized.https://doi.org/10.1002/epi4.12545childrenFIRESNORSEplasma exchangerefractory status epilepticussevoflurane
spellingShingle Manuela L’Erario
Rosa Maria Roperto
Anna Rosati
Sevoflurane as bridge therapy for plasma exchange and Anakinra in febrile infection–related epilepsy syndrome
Epilepsia Open
children
FIRES
NORSE
plasma exchange
refractory status epilepticus
sevoflurane
title Sevoflurane as bridge therapy for plasma exchange and Anakinra in febrile infection–related epilepsy syndrome
title_full Sevoflurane as bridge therapy for plasma exchange and Anakinra in febrile infection–related epilepsy syndrome
title_fullStr Sevoflurane as bridge therapy for plasma exchange and Anakinra in febrile infection–related epilepsy syndrome
title_full_unstemmed Sevoflurane as bridge therapy for plasma exchange and Anakinra in febrile infection–related epilepsy syndrome
title_short Sevoflurane as bridge therapy for plasma exchange and Anakinra in febrile infection–related epilepsy syndrome
title_sort sevoflurane as bridge therapy for plasma exchange and anakinra in febrile infection related epilepsy syndrome
topic children
FIRES
NORSE
plasma exchange
refractory status epilepticus
sevoflurane
url https://doi.org/10.1002/epi4.12545
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AT rosamariaroperto sevofluraneasbridgetherapyforplasmaexchangeandanakinrainfebrileinfectionrelatedepilepsysyndrome
AT annarosati sevofluraneasbridgetherapyforplasmaexchangeandanakinrainfebrileinfectionrelatedepilepsysyndrome