Abstract
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.
Highlights
Febrile infection-related epilepsy syndrome (FIRES) is a unique catastrophic, refractory epilepsy syndrome that has attracted attention in recent years
All patients were treated with multiple antiepileptic drugs; ESES, encephalopathy with electrical status epilepticus in sleep; mTOR, mammalian target of rapamycin; VNS, vagal nerve stimulation; IVIG, intravenous immunoglobulin
The first case treated with anakinra showed that the cerebrospinal fluid (CSF) levels of the proinflammatory cytokines, IL-8 and IL-6, decreased after the injection of anakinra, this could be due to steroid pulse treatment and other factors
Summary
Febrile infection-related epilepsy syndrome (FIRES) is a unique catastrophic, refractory epilepsy syndrome that has attracted attention in recent years. Evidence on the effectiveness of these therapies is still poor [16]; altern elevation of inflammatory cytokines in patients with FIRES suggests an immune-mediated terventions, such as tacrolimus, cyclophosphamide, rituximab, hypothermia, k process [12,13,14,15]; immunomodulatory therapies, including high-dose steroids, indiets, and neuromodulation have been attempted [2]. Despite these spec travenous immunoglobulin (IVIG), and plasmapheresis have become the mainstream ments, results regarding intractable epileptic syndromes, including. In this review, we sought to summarize the scientific and clinical literature related to the use of anakinra in patients with FIRES and DRE
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