Neurocritical care and target immunotherapy for febrile infection-related epilepsy syndrome
Febrile infection-related epilepsy syndrome (FIRES) is an intractable neurological disease characterized by an unexplained refractory status epilepticus triggered by febrile infection. A Consensus definition of FIRES was proposed in 2018, and its clinical features and prognosis are gradually being c...
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| Format: | Article |
| Language: | English |
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Elsevier
2020-06-01
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| Series: | Biomedical Journal |
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| Online Access: | http://www.sciencedirect.com/science/article/pii/S231941702030038X |
| _version_ | 1818473769999532032 |
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| author | Hiroshi Sakuma Asako Horino Ichiro Kuki |
| author_facet | Hiroshi Sakuma Asako Horino Ichiro Kuki |
| author_sort | Hiroshi Sakuma |
| collection | DOAJ |
| description | Febrile infection-related epilepsy syndrome (FIRES) is an intractable neurological disease characterized by an unexplained refractory status epilepticus triggered by febrile infection. A Consensus definition of FIRES was proposed in 2018, and its clinical features and prognosis are gradually being clarified. However, the development of effective treatments has been hindered as the etiology of this rare disease is as yet unelucidated. The basic approach to the management of FIRES, like other forms of epilepsy, is based on the control of seizures, however seizures are extremely intractable and require intravenous administration of large doses of anticonvulsants, mainly barbiturates. This treatment strategy produces various complications including respiratory depression and drug hypersensitivity syndrome, which make it more difficult to control seizures. Consequently, it is crucial to predict these events and to formulate a planned treatment strategy. As well, it is important to grow out of conventional treatment strategies that rely on only anticonvulsants, and alternative therapies are gradually being developed. One such example is the adoption of a ketogenic diet which may lead to reduced convulsions as well as improve intellectual prognosis. Further, overproduction of inflammatory cytokines in the central nervous system has been shown to be strongly related to the pathology of FIRES which has led to attempts at immunomodulation therapy including anti-cytokine therapy. |
| first_indexed | 2024-04-14T04:26:47Z |
| format | Article |
| id | doaj.art-ea37c893f4564f5f8e00ed9cf7886109 |
| institution | Directory Open Access Journal |
| issn | 2319-4170 |
| language | English |
| last_indexed | 2024-04-14T04:26:47Z |
| publishDate | 2020-06-01 |
| publisher | Elsevier |
| record_format | Article |
| series | Biomedical Journal |
| spelling | doaj.art-ea37c893f4564f5f8e00ed9cf78861092022-12-22T02:12:16ZengElsevierBiomedical Journal2319-41702020-06-01433205210Neurocritical care and target immunotherapy for febrile infection-related epilepsy syndromeHiroshi Sakuma0Asako Horino1Ichiro Kuki2Department of Brain Development and Neural Regeneration, Tokyo Metropolitan Institute of Medical Science, Tokyo, Japan; Corresponding author. Department of Brain Development and Neural Regeneration, Tokyo Metropolitan Institute of Medical Science, 2-1-6 Kamikitazawa, Setagaya-ku, Tokyo, 156-8506, Japan.Department of Brain Development and Neural Regeneration, Tokyo Metropolitan Institute of Medical Science, Tokyo, Japan; Department of Pediatric Neurology, Children's Medical Center, Osaka City General Hospital, Osaka, JapanDepartment of Pediatric Neurology, Children's Medical Center, Osaka City General Hospital, Osaka, JapanFebrile infection-related epilepsy syndrome (FIRES) is an intractable neurological disease characterized by an unexplained refractory status epilepticus triggered by febrile infection. A Consensus definition of FIRES was proposed in 2018, and its clinical features and prognosis are gradually being clarified. However, the development of effective treatments has been hindered as the etiology of this rare disease is as yet unelucidated. The basic approach to the management of FIRES, like other forms of epilepsy, is based on the control of seizures, however seizures are extremely intractable and require intravenous administration of large doses of anticonvulsants, mainly barbiturates. This treatment strategy produces various complications including respiratory depression and drug hypersensitivity syndrome, which make it more difficult to control seizures. Consequently, it is crucial to predict these events and to formulate a planned treatment strategy. As well, it is important to grow out of conventional treatment strategies that rely on only anticonvulsants, and alternative therapies are gradually being developed. One such example is the adoption of a ketogenic diet which may lead to reduced convulsions as well as improve intellectual prognosis. Further, overproduction of inflammatory cytokines in the central nervous system has been shown to be strongly related to the pathology of FIRES which has led to attempts at immunomodulation therapy including anti-cytokine therapy.http://www.sciencedirect.com/science/article/pii/S231941702030038XFebrile infection-related epilepsy syndrome (FIRES)New-onset refractory status epilepticus (NORSE)Burst-suppression comaKetogenic dietAnti-cytokine therapy |
| spellingShingle | Hiroshi Sakuma Asako Horino Ichiro Kuki Neurocritical care and target immunotherapy for febrile infection-related epilepsy syndrome Biomedical Journal Febrile infection-related epilepsy syndrome (FIRES) New-onset refractory status epilepticus (NORSE) Burst-suppression coma Ketogenic diet Anti-cytokine therapy |
| title | Neurocritical care and target immunotherapy for febrile infection-related epilepsy syndrome |
| title_full | Neurocritical care and target immunotherapy for febrile infection-related epilepsy syndrome |
| title_fullStr | Neurocritical care and target immunotherapy for febrile infection-related epilepsy syndrome |
| title_full_unstemmed | Neurocritical care and target immunotherapy for febrile infection-related epilepsy syndrome |
| title_short | Neurocritical care and target immunotherapy for febrile infection-related epilepsy syndrome |
| title_sort | neurocritical care and target immunotherapy for febrile infection related epilepsy syndrome |
| topic | Febrile infection-related epilepsy syndrome (FIRES) New-onset refractory status epilepticus (NORSE) Burst-suppression coma Ketogenic diet Anti-cytokine therapy |
| url | http://www.sciencedirect.com/science/article/pii/S231941702030038X |
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