Towards a Treatment for Neuroinflammation in Epilepsy: Interleukin-1 Receptor Antagonist, Anakinra, as a Potential Treatment in Intractable Epilepsy
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 anticonvuls...
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2021-06-01
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author | Gaku Yamanaka Yu Ishida Kanako Kanou Shinji Suzuki Yusuke Watanabe Tomoko Takamatsu Shinichiro Morichi Soken Go Shingo Oana Takashi Yamazaki Hisashi Kawashima |
author_facet | Gaku Yamanaka Yu Ishida Kanako Kanou Shinji Suzuki Yusuke Watanabe Tomoko Takamatsu Shinichiro Morichi Soken Go Shingo Oana Takashi Yamazaki Hisashi Kawashima |
author_sort | Gaku Yamanaka |
collection | DOAJ |
description | 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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issn | 1661-6596 1422-0067 |
language | English |
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spelling | doaj.art-d96b4e02c3f24e4fbb9beb5e3de6c5002023-11-21T23:42:46ZengMDPI AGInternational Journal of Molecular Sciences1661-65961422-00672021-06-012212628210.3390/ijms22126282Towards a Treatment for Neuroinflammation in Epilepsy: Interleukin-1 Receptor Antagonist, Anakinra, as a Potential Treatment in Intractable EpilepsyGaku Yamanaka0Yu Ishida1Kanako Kanou2Shinji Suzuki3Yusuke Watanabe4Tomoko Takamatsu5Shinichiro Morichi6Soken Go7Shingo Oana8Takashi Yamazaki9Hisashi Kawashima10Department of Pediatrics and Adolescent Medicine, Tokyo Medical University, Tokyo 160-0023, JapanDepartment of Pediatrics and Adolescent Medicine, Tokyo Medical University, Tokyo 160-0023, JapanDepartment of Pediatrics and Adolescent Medicine, Tokyo Medical University, Tokyo 160-0023, JapanDepartment of Pediatrics and Adolescent Medicine, Tokyo Medical University, Tokyo 160-0023, JapanDepartment of Pediatrics and Adolescent Medicine, Tokyo Medical University, Tokyo 160-0023, JapanDepartment of Pediatrics and Adolescent Medicine, Tokyo Medical University, Tokyo 160-0023, JapanDepartment of Pediatrics and Adolescent Medicine, Tokyo Medical University, Tokyo 160-0023, JapanDepartment of Pediatrics and Adolescent Medicine, Tokyo Medical University, Tokyo 160-0023, JapanDepartment of Pediatrics and Adolescent Medicine, Tokyo Medical University, Tokyo 160-0023, JapanDepartment of Pediatrics and Adolescent Medicine, Tokyo Medical University, Tokyo 160-0023, JapanDepartment of Pediatrics and Adolescent Medicine, Tokyo Medical University, Tokyo 160-0023, JapanFebrile 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.https://www.mdpi.com/1422-0067/22/12/6282anakinracytokinefebrile infection-related epilepsy syndromeIL-1βIL-1 receptor antagonist |
spellingShingle | Gaku Yamanaka Yu Ishida Kanako Kanou Shinji Suzuki Yusuke Watanabe Tomoko Takamatsu Shinichiro Morichi Soken Go Shingo Oana Takashi Yamazaki Hisashi Kawashima Towards a Treatment for Neuroinflammation in Epilepsy: Interleukin-1 Receptor Antagonist, Anakinra, as a Potential Treatment in Intractable Epilepsy International Journal of Molecular Sciences anakinra cytokine febrile infection-related epilepsy syndrome IL-1β IL-1 receptor antagonist |
title | Towards a Treatment for Neuroinflammation in Epilepsy: Interleukin-1 Receptor Antagonist, Anakinra, as a Potential Treatment in Intractable Epilepsy |
title_full | Towards a Treatment for Neuroinflammation in Epilepsy: Interleukin-1 Receptor Antagonist, Anakinra, as a Potential Treatment in Intractable Epilepsy |
title_fullStr | Towards a Treatment for Neuroinflammation in Epilepsy: Interleukin-1 Receptor Antagonist, Anakinra, as a Potential Treatment in Intractable Epilepsy |
title_full_unstemmed | Towards a Treatment for Neuroinflammation in Epilepsy: Interleukin-1 Receptor Antagonist, Anakinra, as a Potential Treatment in Intractable Epilepsy |
title_short | Towards a Treatment for Neuroinflammation in Epilepsy: Interleukin-1 Receptor Antagonist, Anakinra, as a Potential Treatment in Intractable Epilepsy |
title_sort | towards a treatment for neuroinflammation in epilepsy interleukin 1 receptor antagonist anakinra as a potential treatment in intractable epilepsy |
topic | anakinra cytokine febrile infection-related epilepsy syndrome IL-1β IL-1 receptor antagonist |
url | https://www.mdpi.com/1422-0067/22/12/6282 |
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