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...

Full description

Bibliographic Details
Main Authors: Gaku Yamanaka, Yu Ishida, Kanako Kanou, Shinji Suzuki, Yusuke Watanabe, Tomoko Takamatsu, Shinichiro Morichi, Soken Go, Shingo Oana, Takashi Yamazaki, Hisashi Kawashima
Format: Article
Language:English
Published: MDPI AG 2021-06-01
Series:International Journal of Molecular Sciences
Subjects:
Online Access:https://www.mdpi.com/1422-0067/22/12/6282
_version_ 1797530557606789120
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.
first_indexed 2024-03-10T10:30:43Z
format Article
id doaj.art-d96b4e02c3f24e4fbb9beb5e3de6c500
institution Directory Open Access Journal
issn 1661-6596
1422-0067
language English
last_indexed 2024-03-10T10:30:43Z
publishDate 2021-06-01
publisher MDPI AG
record_format Article
series International Journal of Molecular Sciences
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
work_keys_str_mv AT gakuyamanaka towardsatreatmentforneuroinflammationinepilepsyinterleukin1receptorantagonistanakinraasapotentialtreatmentinintractableepilepsy
AT yuishida towardsatreatmentforneuroinflammationinepilepsyinterleukin1receptorantagonistanakinraasapotentialtreatmentinintractableepilepsy
AT kanakokanou towardsatreatmentforneuroinflammationinepilepsyinterleukin1receptorantagonistanakinraasapotentialtreatmentinintractableepilepsy
AT shinjisuzuki towardsatreatmentforneuroinflammationinepilepsyinterleukin1receptorantagonistanakinraasapotentialtreatmentinintractableepilepsy
AT yusukewatanabe towardsatreatmentforneuroinflammationinepilepsyinterleukin1receptorantagonistanakinraasapotentialtreatmentinintractableepilepsy
AT tomokotakamatsu towardsatreatmentforneuroinflammationinepilepsyinterleukin1receptorantagonistanakinraasapotentialtreatmentinintractableepilepsy
AT shinichiromorichi towardsatreatmentforneuroinflammationinepilepsyinterleukin1receptorantagonistanakinraasapotentialtreatmentinintractableepilepsy
AT sokengo towardsatreatmentforneuroinflammationinepilepsyinterleukin1receptorantagonistanakinraasapotentialtreatmentinintractableepilepsy
AT shingooana towardsatreatmentforneuroinflammationinepilepsyinterleukin1receptorantagonistanakinraasapotentialtreatmentinintractableepilepsy
AT takashiyamazaki towardsatreatmentforneuroinflammationinepilepsyinterleukin1receptorantagonistanakinraasapotentialtreatmentinintractableepilepsy
AT hisashikawashima towardsatreatmentforneuroinflammationinepilepsyinterleukin1receptorantagonistanakinraasapotentialtreatmentinintractableepilepsy