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.