scholarly journals Interleukin-6 Blockade With Tocilizumab in Anakinra-Refractory Febrile Infection-Related Epilepsy Syndrome (FIRES)

2020 ◽  
Vol 7 ◽  
pp. 2329048X2097925
Author(s):  
Coral M. Stredny ◽  
Siobhan Case ◽  
Arnold J. Sansevere ◽  
MaryBeth Son ◽  
Lauren Henderson ◽  
...  
Keyword(s):  
Receptor Antagonist ◽  
Interleukin 6 ◽  
Interleukin 1 ◽  
Refractory Status Epilepticus ◽  
Cognitive Outcomes ◽  
Epilepsy Syndrome ◽  
Immune System Dysfunction ◽  
Interleukin 6 Receptor ◽  
Pro Inflammatory Cytokines ◽  
New Onset

Febrile infection-related epilepsy syndrome (FIRES) is characterized by new onset refractory status epilepticus in a previously healthy child that is associated with poor cognitive outcomes and chronic epilepsy. Innate immune system dysfunction is hypothesized to be a key etiologic contributor, with a potential role for immunotherapy blocking pro-inflammatory cytokines, such as interleukin-1β and interleukin-6. We present a case of FIRES refractory to anakinra, an interleukin-1 receptor antagonist, subsequently treated with the ketogenic diet and tocilizumab, an interleukin-6 receptor antagonist, temporally associated with seizure cessation and a favorable 1-year outcome.

The effect of tocilizumab - an interleukin-6 receptor antagonist - on lipid levels in rheumatoid arthritis

2017 ◽  
Author(s):  
Ifigenia Kostoglou-Athanassiou ◽  
Lambros Athanassiou ◽  
Aikaterini Tzanavari ◽  
Charoula Katsavouni ◽  
Markos Kostopoulos ◽  
...  
Keyword(s):  
Rheumatoid Arthritis ◽  
Receptor Antagonist ◽  
Interleukin 6 ◽  
Lipid Levels ◽  
Interleukin 6 Receptor

scholarly journals How to Help Your Patients Enroll in the New-Onset Refractory Status Epilepticus (NORSE) and Febrile Infection-Related Epilepsy Syndrome (FIRES) Family Registry, and Other Rare Epilepsy Registries

2021 ◽  
pp. 153575972199832
Author(s):  
Karnig Kazazian ◽  
Marissa Kellogg ◽  
Nora Wong ◽  
Krista Eschbach ◽  
Raquel Farias Moeller ◽  
...  
Keyword(s):  
Status Epilepticus ◽  
Refractory Status Epilepticus ◽  
Epilepsy Syndrome ◽  
Neurologic Disorder ◽  
Mortality And Morbidity ◽  
Refractory Status ◽  
Rigorous Research ◽  
Active Epilepsy ◽  
New Onset

New-onset refractory status epilepticus (NORSE) is a rare clinical presentation of refractory status epilepticus (RSE) that occurs in people without active epilepsy or preexisting neurologic disorder. Febrile infection-related epilepsy syndrome (FIRES) is a subcategory of NORSE. New-onset refractory status epilepticus/FIRES are becoming increasingly recognized; however, information pertaining to disease course, clinical outcomes, and survivorship remains limited, and mortality and morbidity are variable, but often high. The objective of the NORSE/FIRES Family Registry is to (1) provide an easily accessible and internationally available multilingual registry into which survivors or NORSE/FIRES surrogates or family members of people affected by NORSE/FIRES or their physicians can enter data in a systematic and rigorous research study from anywhere in the world where internet is available; and (2) to examine past medical history, outcomes, and quality of life for people affected by NORSE/FIRES.

scholarly journals Towards a Treatment for Neuroinflammation in Epilepsy: Interleukin-1 Receptor Antagonist, Anakinra, as a Potential Treatment in Intractable Epilepsy

2021 ◽  
Vol 22 (12) ◽  
pp. 6282
Author(s):  
Gaku Yamanaka ◽  
Yu Ishida ◽  
Kanako Kanou ◽  
Shinji Suzuki ◽  
Yusuke Watanabe ◽  
...  
Keyword(s):  
Receptor Antagonist ◽  
Small Sample Size ◽  
Interleukin 1 ◽  
Intractable Epilepsy ◽  
Evidence Base ◽  
Small Sample ◽  
Epilepsy Syndrome ◽  
Interleukin 1 Receptor Antagonist ◽  
Epileptic Syndromes ◽  
Systemic Symptoms

Febrile Infection-Related Epilepsy Syndrome (FIRES) is a unique catastrophic epilepsy syndrome, and the development of drug-resistant epilepsy (DRE) is inevitable. Recently, anakinra, an interleukin-1 receptor antagonist (IL-1RA), has been increasingly used to treat DRE due to its potent anticonvulsant activity. We here summarized its effects in 38 patients (32 patients with FIRES and six with DRE). Of the 22 patients with FIRES, 16 (73%) had at least short-term seizure control 1 week after starting anakinra, while the remaining six suspected anakinra-refractory cases were male and had poor prognoses. Due to the small sample size, an explanation for anakinra refractoriness was not evident. In all DRE patients, seizures disappeared or improved, and cognitive function improved in five of the six patients following treatment. Patients showed no serious side effects, although drug reactions with eosinophilia and systemic symptoms, cytopenia, and infections were observed. Thus, anakinra has led to a marked improvement in some cases, and functional deficiency of IL-1RA was indicated, supporting a direct mechanism for its therapeutic effect. This review first discusses the effectiveness of anakinra for intractable epileptic syndromes. Anakinra could become a new tool for intractable epilepsy treatment. However, it does not currently have a solid evidence base.

New onset refractory status epilepticus research

Neurology ◽  
2019 ◽  
Vol 92 (17) ◽  
pp. 802-810 ◽  
Author(s):  
Teneille E. Gofton ◽  
Nicolas Gaspard ◽  
Sara E. Hocker ◽  
Tobias Loddenkemper ◽  
Lawrence J. Hirsch
Keyword(s):  
Status Epilepticus ◽  
Professional Education ◽  
Age Groups ◽  
Refractory Status Epilepticus ◽  
Epilepsy Syndrome ◽  
Current State ◽  
Refractory Status ◽  
Management Approaches ◽  
Active Epilepsy ◽  
New Onset

New-onset refractory status epilepticus (NORSE) is a clinical presentation, not a specific diagnosis, in a patient without active epilepsy or other preexisting relevant neurologic disorder, with new onset of refractory status epilepticus (RSE) that does not resolve after 2 or more rescue medications, without a clear acute or active structural, toxic, or metabolic cause. Febrile infection-related epilepsy syndrome is a subset of NORSE in which fever began at least 24 hours prior to the RSE. Both terms apply to all age groups. Until recently, NORSE was a poorly recognized entity without a consistent definition or approach to care. We review the current state of knowledge in NORSE and propose a roadmap for future collaborative research. Research investigating NORSE should prioritize the following 4 domains: (1) clinical features, etiology, and pathophysiology; (2) treatment; (3) adult and pediatric evaluation and management approaches; and (4) public advocacy, professional education, and family support. We consider international collaboration and multicenter research crucial in achieving these goals.

Comparative study of anti-VEGF Ranibizumab and Interleukin-6 receptor antagonist Tocilizumab in Adjuvant-induced Arthritis

2018 ◽  
Vol 356 ◽  
pp. 65-75 ◽  
Author(s):  
Amany El-Shahawy Abdel-Maged ◽  
Amany M. Gad ◽  
Amal Kamal Abdel-Aziz ◽  
Mohammad M. Aboulwafa ◽  
Samar S. Azab
Keyword(s):  
Comparative Study ◽  
Receptor Antagonist ◽  
Interleukin 6 ◽  
Adjuvant Induced Arthritis ◽  
Anti Vegf ◽  
Interleukin 6 Receptor
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