Febrile infection-related epilepsy syndrome (FIRES): therapeutic complications, long-term neurological and neuroimaging follow-up.
Hsiu-Fen Lee,Ching-Shiang Chi +1 more
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TLDR
Liver function impairment, skin rash, and arrhythmia are frequently seen during hospitalization for FIRES at the acute stage of disease course, and the higher grade of periventricular white matter lesions suggested poorer neurological outcomes.Citations
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International League Against Epilepsy classification and definition of epilepsy syndromes with onset in childhood: Position paper by the ILAE Task Force on Nosology and Definitions
Nicola Specchio,Elaine C. Wirrell,Ingrid E. Scheffer,Rima Nabbout,Kate Riney,Pauline Samia,Marilisa M. Guerreiro,Sam Gwer,Sameer M. Zuberi,Jo M. Wilmshurst,Elissa G. Yozawitz,Ronit M. Pressler,E. C. Hirsch,Samuel Wiebe,JH Cross,Emilio Perucca,Solomon L. Moshé,Paolo Tinuper,Stéphane Auvin +18 more
TL;DR: The childhood onset epilepsy syndromes are described, most of which have both mandatory seizure type(s) and interictal electroencephalographic (EEG) features, highlighting the mandatory seizure(s), EEG features, phenotypic variations, and findings from key investigations.
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Progress report on new antiepileptic drugs: A summary of the Fifteenth Eilat Conference on New Antiepileptic Drugs and Devices (EILAT XV). I. Drugs in preclinical and early clinical development.
Meir Bialer,Svein I. Johannessen,Matthias J. Koepp,René H. Levy,Emilio Perucca,Piero Perucca,Piero Perucca,Torbjörn Tomson,H. Steve White +8 more
TL;DR: There continues to be a steady flow of potential antiepileptic drugs progressing to clinical development, and many of these compounds show innovative mechanisms of action, and some have already been tested in placebo‐controlled randomized controlled trials, with promising efficacy and safety results.
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Therapeutic effect of Anakinra in the relapsing chronic phase of febrile infection–related epilepsy syndrome
Robertino Dilena,Eleonora Mauri,Eleonora Aronica,Pia Bernasconi,Cristina Bana,Cristina Cappelletti,Giorgio Carrabba,Stefano Ferrero,Roberto Giorda,Sophie Guez,Stefano Scalia Catenacci,Fabio Triulzi,Sergio Barbieri,Edoardo Calderini,Annamaria Vezzani +14 more
TL;DR: The novel evidence that anakinra reduces the relapse of highly recurrent refractory seizures at 1.5 years after FIRES onset is reported, supporting the hypothesis that interleukin 1β and inflammation‐related factors play a crucial role in seizure recurrence in both the acute and chronic stages of the disease.
Journal ArticleDOI
New-onset refractory status epilepticus and febrile infection-related epilepsy syndrome.
TL;DR: No specific therapy for FIRES and NORSE currently exists; high doses of barbiturates and ketogenic diet can be used with some effectiveness, and anakinra and tocilizumab, targeting interleukin pathways, have emerged as potential specific therapies.
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The evolution of the pilocarpine animal model of status epilepticus
TL;DR: The various alterations made to the original pilocarpine model over the years to combat both the high mortality and low induction rates are highlighted.
References
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The treatment of super-refractory status epilepticus: a critical review of available therapies and a clinical treatment protocol
Simon Shorvon,Monica Ferlisi +1 more
TL;DR: There is an urgent need for the establishment of a database of outcomes of individual therapies for super-refractory status epilepticus, which is an uncommon but important clinical problem with high mortality and morbidity rates.
Journal ArticleDOI
Efficacy of ketogenic diet in severe refractory status epilepticus initiating fever induced refractory epileptic encephalopathy in school age children (FIRES)
Rima Nabbout,Michel Mazzuca,Philippe Hubert,Sylviane Peudennier,Catherine Allaire,Vincent Flurin,Marina Aberastury,Walter Silva,Olivier Dulac +8 more
TL;DR: The effect of ketogenic diet (KD) in this condition of Fever induced refractory epileptic encephalopathy in school age children is reported.
Journal ArticleDOI
Febrile infection-related epilepsy syndrome (FIRES): pathogenesis, treatment, and outcome: a multicenter study on 77 children.
Uri Kramer,Ching Shiang Chi,Kuang Lin Lin,Nicola Specchio,Mustafa Sahin,Heather E. Olson,Rima Nabbout,Gerhard Kluger,Jainn Jim Lin,Andreas van Baalen +9 more
TL;DR: The correlations between treatment modalities and selected disease parameters with outcome in febrile infection–related epilepsy syndrome (FIRES), a catastrophic epileptic encephalopathy with a yet undefined etiology, are explored.
Journal ArticleDOI
Febrile infection-related epilepsy syndrome (FIRES): a nonencephalitic encephalopathy in childhood.
Andreas van Baalen,Martin Häusler,Rainer Boor,Axel Rohr,Jürgen Sperner,Gerhard Kurlemann,Axel Panzer,Ulrich Stephani,Gerhard Kluger +8 more
TL;DR: The lack of evidence for encephalitis suggests another infection‐related pathogenesis of this disastrous epileptic encephalopathy, and the term “febrile infection–related epilepsy syndrome” (FIRES) is proposed.
Journal ArticleDOI
Acute encephalopathy with inflammation-mediated status epilepticus
TL;DR: This paper proposed to group these conditions under the concept of acute encephalopathy with inflammation-mediated status epilepticus, and there are theoretical reasons to consider the ketogenic diet as an early means to control both seizures and inflammation.
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Proposed consensus definitions for new-onset refractory status epilepticus (NORSE), febrile infection-related epilepsy syndrome (FIRES), and related conditions.
Lawrence J. Hirsch,Nicolas Gaspard,Andreas van Baalen,Rima Nabbout,Sophie Demeret,Tobias Loddenkemper,Vincent Navarro,Nicola Specchio,Lieven Lagae,Andrea O. Rossetti,Sara E. Hocker,Teneille Gofton,Nicholas S. Abend,Emily J. Gilmore,Cecil D. Hahn,Houman Khosravani,Houman Khosravani,Felix Rosenow,Eugen Trinka +18 more