Journal ArticleDOI
New-onset refractory status epilepticus and febrile infection-related epilepsy syndrome.
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TLDR
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.Abstract:
New-onset refractory status epilepticus (NORSE) and febrile infection-related epilepsy syndrome (FIRES) are relatively rare clinical presentations. They are characterized by de novo onset of refractory status epilepticus (RSE) without clearly identifiable acute or active cause (structural, toxic, or metabolic). We reviewed the literature using PubMed reports published between 2003 and 2019 and summarized the clinical, neurophysiological, imaging, and treatment findings. Focal motor seizures, which tend to evolve into status epilepticus, characterize the typical presentation. Disease course is biphasic: acute phase followed by chronic phase with refractory epilepsy and neurological impairment. Aetiology is unknown, but immune-inflammatory-mediated epileptic encephalopathy is suspected. Electroencephalograms show variety in discharges (sporadic or periodic, focal, generalized, or more frequently bilateral), sometimes with a multifocal pattern. About 70% of adult NORSE have abnormal magnetic resonance imaging (MRI); in paediatric series of FIRES, 61.2% of patients have a normal brain MRI at the beginning and only 18.5% during the chronic phase. No specific therapy for FIRES and NORSE currently exists; high doses of barbiturates and ketogenic diet can be used with some effectiveness. Recently, anakinra and tocilizumab, targeting interleukin pathways, have emerged as potential specific therapies. Mortality rate is around 12% in children and even higher in adults (16-27%).read more
Citations
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Journal ArticleDOI
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.
Journal ArticleDOI
Proposal to Optimize Evaluation and Treatment of Febrile Infection‐Related Epilepsy Syndrome (FIRES): A Report from FIRES Workshop
Sookyong Koh,Elaine C. Wirrell,Annamaria Vezzani,Rima Nabbout,Eyal Muscal,Marios Kaliakatsos,Ronny Wickström,James J. Riviello,Andreas Brunklaus,Eric T. Payne,Antonio Valentin,Elizabeth Wells,Jessica L. Carpenter,Kihyeong Lee,Yi-Chen Lai,Krista Eschbach,Craig A. Press,Mark Gorman,Coral M. Stredny,William P Roche,Tara K. Mangum +20 more
TL;DR: An international group of pediatric epileptologists, pediatric neurointensivists, rheumatologists and basic scientists with interest and expertise in FIRES convened to propose an algorithm for a standardized approach to the diagnosis and treatment of FIRES.
Journal ArticleDOI
International consensus recommendations for management of new onset refractory status epilepticus including febrile infection‐related epilepsy syndrome: Statements and supporting evidence
R. Wickstrom,Olga D. Taraschenko,Robertino Dilena,Eric T. Payne,Nicola Specchio,Rima Nabbout,Sookyong Koh,Nicolas Gaspard,Lawrence J. Hirsch,Stéphane Auvin,Andreas van Baalen,Ettore Beghi,Susanne M. Benseler,P. S. Bergin,Tom Bleck,Andreas Brunklaus,R. Caraballo,Mackenzie C. Cervenka,Daniel J. Costello,Frank W. Drislane,Raquel Farias-Moeller,William Brian Gallantine,Emily J. Gilmore,Teneille Gofton,M. A. P. Jiménez,Sara E. Hocker,Marios Kaliakatsos,Marissa Kellogg,Jong Woo Lee,Tobias Loddenkemper,Stefano Meletti,Masashi Mizugushi,Eyal Muscal,James J. Riviello,Eric Rosenthal,Andrea O. Rossetti,Stefan Ruegg,Rana R. Said,Claudine Sculier,Sarah E. Schmitt,Stephan U. Schuele,Coral M. Stredny,Eugen Trinka,Mark S. Wainwright,Stephen VanHaerents,Elisabeth Wells,Elaine C. Wirrell,Sameer M. Zuberi +47 more
TL;DR: The recommendations generated by this consensus can be used as a guide for the diagnosis, evaluation, and management of patients with NORSE/FIRES, and for planning of future research.
Journal ArticleDOI
International consensus recommendations for management of new onset refractory status epilepticus (NORSE) including febrile infection‐related epilepsy syndrome (FIRES): Summary and clinical tools
Ronny Wickström,Olga D. Taraschenko,Robertino Dilena,Eric T. Payne,Nicola Specchio,Rima Nabbout,Sookyong Koh,Nicolas Gaspard,Lawrence J. Hirsch,Stéphane Auvin,Andreas van Baalen,Ettore Beghi,Susanne M. Benseler,P. S. Bergin,Thomas P. Bleck,Andreas Brunklaus,R. Caraballo,Mackenzie C. Cervenka,Daniel J. Costello,Frank W. Drislane,Raquel Farias-Moeller,William Brian Gallantine,Emily J. Gilmore,Teneille Gofton,M. A. P. Jiménez,Sara E. Hocker,Marios Kaliakatsos,Marissa Kellogg,Jong Woo Lee,Tobias Loddenkemper,Stefano Meletti,Masashi Mizugushi,Eyal Muscal,James J. Riviello,Eric Rosenthal,Andrea O. Rossetti,Stefan Ruegg,Rana R. Said,Claudine Sculier,Sarah E. Schmitt,Stephan U. Schuele,Coral M. Stredny,Eugen Trinka,Mark S. Wainwright,Stephen VanHaerents,Elisabeth Wells,Elaine C. Wirrell,Sameer M. Zuberi +47 more
TL;DR: Recommendations generated by this consensus can be used as a guide for the diagnosis, evaluation, and management of patients with NORSE/FIRES, and for planning of future research.
Journal ArticleDOI
Parainfectious encephalitis in COVID-19: “The Claustrum Sign”
Frédéric Zuhorn,Hassan Omaimen,Bertram Ruprecht,Christoph Stellbrink,Michael Rauch,Andreas Rogalewski,Randolf Klingebiel,Wolf-Rüdiger Schäbitz +7 more
TL;DR: COVID-19 treatment options currently under study include several antiviral drugs and immune modulators, the antimalarials chloroquine and hydroxychloroquine, corticosteroids, convalescent plasma, pharmaceutical products that target the renin-angiotensin system, hyperbaric oxygen, and nitric oxide.
References
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Journal ArticleDOI
Paraneoplastic anti–N‐methyl‐D‐aspartate receptor encephalitis associated with ovarian teratoma
Josep Dalmau,Erdem Tüzün,Hai-Yan Wu,Jaime Masjuan,Jeffrey E. Rossi,Alfredo Voloschin,Joachim M. Baehring,Haruo Shimazaki,Reiji Koide,Dale King,Warren P. Mason,Lauren H Sansing,Marc A. Dichter,Myrna R. Rosenfeld,David A. Lynch +14 more
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Journal ArticleDOI
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
New-onset refractory status epilepticus: Etiology, clinical features, and outcome
Nicolas Gaspard,Nicolas Gaspard,Brandon Foreman,Vincent Alvarez,Vincent Alvarez,Christian M. Cabrera Kang,John C. Probasco,Amy C. Jongeling,Emma Meyers,Alyssa R. Espinera,Kevin F. Haas,Sarah E. Schmitt,Elizabeth E. Gerard,Teneille Gofton,Peter W. Kaplan,Jong W. Lee,Benjamin Legros,Jerzy P. Szaflarski,Brandon Westover,Suzette M. LaRoche,Lawrence J. Hirsch +20 more
TL;DR: Autoimmune encephalitis is the most commonly identified cause of new-onset refractory status epilepticus, but half remain cryptogenic, and outcome at discharge is poor but improves during follow-up.
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
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
TL;DR: An international group of experts were convened to standardize definitions of New‐Onset Refractory Status Epilepticus (NORSE), Febrile Infection‐Related Epilepsy Syndrome (FIRES), and related conditions to enable improved communication for investigators, physicians, families, patients, and other caregivers.
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Proposed consensus definitions for new-onset refractory status epilepticus (NORSE), febrile infection-related epilepsy syndrome (FIRES), and related conditions.
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