Teaching NeuroImage: Claustrum Sign in Febrile Infection-Related Epilepsy Syndrome

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.

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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

Epilepsy Currents ◽

10.1177/15357597211002869 ◽

2021 ◽

pp. 153575972110028

Author(s):

Karnig Kazazian ◽

Marissa Kellogg ◽

Nora Wong ◽

Krista Eschbach ◽

Raquel Farias Moeller ◽

...

Keyword(s):

Status Epilepticus ◽

Refractory Status Epilepticus ◽

Epilepsy Syndrome ◽

Refractory Status ◽

New Onset

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Hypothesis: Febrile infection‐related epilepsy syndrome is a microglial NLRP3 inflammasome/IL‐1 axis‐driven autoinflammatory syndrome

Clinical & Translational Immunology ◽

10.1002/cti2.1299 ◽

2021 ◽

Vol 10 (6) ◽

Author(s):

Wei‐Sheng Lin ◽

Ting‐Rong Hsu

Keyword(s):

Nlrp3 Inflammasome ◽

Epilepsy Syndrome ◽

Autoinflammatory Syndrome

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Possible Role of High-Dose Barbiturates and Early Administration of Parenteral Ketogenic Diet for Reducing Development of Chronic Epilepsy in Febrile Infection-Related Epilepsy Syndrome: A Case Report

Neuropediatrics ◽

10.1055/s-0040-1716903 ◽

2020 ◽

Author(s):

Shimpei Baba ◽

Tohru Okanishi ◽

Koichi Ohsugi ◽

Rika Suzumura ◽

Keiko Niimi ◽

...

Keyword(s):

Continuous Infusion ◽

Ketogenic Diet ◽

Epilepsy Syndrome ◽

High Dose ◽

Early Administration ◽

Chronic Epilepsy ◽

Time Period ◽

Irreversible Brain Damage ◽

Electroencephalogram Eeg

AbstractWe describe the efficacy of high-dose barbiturates and early administration of a parenteral ketogenic diet (KD) as initial treatments for acute status epilepticus (SE) in an 8-year-old girl with febrile infection-related epilepsy syndrome (FIRES). The patient was admitted to our hospital with refractory focal SE. Abundant epileptic discharges over the left frontal region were observed on electroencephalogram (EEG). Treatment with continuous infusion of thiamylal for 4 hours, increased incrementally to 40 mg/kg/h, successfully ended the clinical SE, and induced a burst-suppression coma. The infusion rate was then gradually decreased to 4 mg/kg/h over the next 12 hours. Parenteral KD was administered from days 6 to 21 of illness. Continuous infusion of thiamylal was switched to midazolam on day 10 without causing seizures or EEG exacerbations. The patient has remained seizure free in the 15 months since hospital discharge. The effectiveness of KD for the treatment of FIRES has attracted attention amongst clinicians, but KD treatment may need to last for 2 to 4 days before it can stop SE, a time period that could cause irreversible brain damage. Considering the severity of SE in our patient and the dose of barbiturates needed to treat it, we consider this case to have had a good clinical outcome. The results suggest that rapid termination of seizure using high-dose barbiturates in conjunction with early administration of parenteral KD could reduce the development of chronic epilepsy in patients with FIRES.

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

International Journal of Molecular Sciences ◽

10.3390/ijms22126282 ◽

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.

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