Long-term outcomes of adult cryptogenic febrile infection–related epilepsy syndrome (FIRES)
BackgroundCryptogenic febrile infection-related epilepsy syndrome (FIRES) is a rare but catastrophic encephalopathic condition. We aimed to investigate the long-term outcome in adult cryptogenic FIRES.MethodsThis was a retrospective study based on the prospective database in the neuro-intensive care...
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Frontiers Media S.A.
2023-01-01
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| Series: | Frontiers in Neurology |
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| Online Access: | https://www.frontiersin.org/articles/10.3389/fneur.2022.1081388/full |
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| author | Xiaojing Shi Yuanyuan Wang Xuan Wang Xiaogang Kang Fang Yang Fang Yuan Wen Jiang |
| author_facet | Xiaojing Shi Yuanyuan Wang Xuan Wang Xiaogang Kang Fang Yang Fang Yuan Wen Jiang |
| author_sort | Xiaojing Shi |
| collection | DOAJ |
| description | BackgroundCryptogenic febrile infection-related epilepsy syndrome (FIRES) is a rare but catastrophic encephalopathic condition. We aimed to investigate the long-term outcome in adult cryptogenic FIRES.MethodsThis was a retrospective study based on the prospective database in the neuro-intensive care unit of a tertiary hospital in China. Consecutive adult patients with cryptogenic FIRES between July 2007 to December 2021 were included. Long-term outcomes included function independence, the development of drug-resistant epilepsy (DRE), remote recurrent status epilepticus (SE), anti-seizure medications (ASMs), and changes in the brain Magnetic Resonance Imaging (MRI).ResultsA total of 11 adult patients with cryptogenic FIRES were identified from 270 patients with SE. Four (36%) patients died in the hospital, with three of them withdrawing treatments, and one patient died 12 months after discharge. After the follow-up ranging from 12 to 112 months, 6 (55%) patients were still alive, and all of them achieved functional independence [modified Rankin Scale (mRS) 0-3]. 45% (5/11) patients developed DRE, 18% (2/11) had remote recurrent SE, and 55% (6/11) were on polytherapy with ASMs at the last follow-up. Most of the patients with initial normal or abnormal MRI had abnormalities in the hippocampus at follow-up, and most of the other MRI abnormalities found in the acute stage disappeared over time.ConclusionThe outcome of adult cryptogenic FIRES is daunting. More than one-third of patients die in the hospital. Survivors of cryptogenic FIRES may regain functional independence, but they usually develop DRE and receive polytherapy of ASMs for a long time. |
| first_indexed | 2024-04-11T01:02:20Z |
| format | Article |
| id | doaj.art-b4cb350de8c24acca570d333c5465c5d |
| institution | Directory Open Access Journal |
| issn | 1664-2295 |
| language | English |
| last_indexed | 2024-04-11T01:02:20Z |
| publishDate | 2023-01-01 |
| publisher | Frontiers Media S.A. |
| record_format | Article |
| series | Frontiers in Neurology |
| spelling | doaj.art-b4cb350de8c24acca570d333c5465c5d2023-01-04T15:46:07ZengFrontiers Media S.A.Frontiers in Neurology1664-22952023-01-011310.3389/fneur.2022.10813881081388Long-term outcomes of adult cryptogenic febrile infection–related epilepsy syndrome (FIRES)Xiaojing Shi0Yuanyuan Wang1Xuan Wang2Xiaogang Kang3Fang Yang4Fang Yuan5Wen Jiang6Department of Neurology, Xijing Hospital, Fourth Military Medical University, Xi'an, ChinaDepartment of Neurology, Xijing Hospital, Fourth Military Medical University, Xi'an, ChinaDepartment of Neurology, Xijing Hospital, Fourth Military Medical University, Xi'an, ChinaDepartment of Neurology, Xijing Hospital, Fourth Military Medical University, Xi'an, ChinaDepartment of Neurology, Xijing Hospital, Fourth Military Medical University, Xi'an, ChinaDepartment of Neurology, The Second Affiliated Hospital of Guangzhou University of Chinese Medicine, Guangzhou, ChinaDepartment of Neurology, Xijing Hospital, Fourth Military Medical University, Xi'an, ChinaBackgroundCryptogenic febrile infection-related epilepsy syndrome (FIRES) is a rare but catastrophic encephalopathic condition. We aimed to investigate the long-term outcome in adult cryptogenic FIRES.MethodsThis was a retrospective study based on the prospective database in the neuro-intensive care unit of a tertiary hospital in China. Consecutive adult patients with cryptogenic FIRES between July 2007 to December 2021 were included. Long-term outcomes included function independence, the development of drug-resistant epilepsy (DRE), remote recurrent status epilepticus (SE), anti-seizure medications (ASMs), and changes in the brain Magnetic Resonance Imaging (MRI).ResultsA total of 11 adult patients with cryptogenic FIRES were identified from 270 patients with SE. Four (36%) patients died in the hospital, with three of them withdrawing treatments, and one patient died 12 months after discharge. After the follow-up ranging from 12 to 112 months, 6 (55%) patients were still alive, and all of them achieved functional independence [modified Rankin Scale (mRS) 0-3]. 45% (5/11) patients developed DRE, 18% (2/11) had remote recurrent SE, and 55% (6/11) were on polytherapy with ASMs at the last follow-up. Most of the patients with initial normal or abnormal MRI had abnormalities in the hippocampus at follow-up, and most of the other MRI abnormalities found in the acute stage disappeared over time.ConclusionThe outcome of adult cryptogenic FIRES is daunting. More than one-third of patients die in the hospital. Survivors of cryptogenic FIRES may regain functional independence, but they usually develop DRE and receive polytherapy of ASMs for a long time.https://www.frontiersin.org/articles/10.3389/fneur.2022.1081388/fullfebrile infection-related epilepsy syndromeadultnew-onset refractory status epilepticusrefractory status epilepticuslong-term outcomecase series |
| spellingShingle | Xiaojing Shi Yuanyuan Wang Xuan Wang Xiaogang Kang Fang Yang Fang Yuan Wen Jiang Long-term outcomes of adult cryptogenic febrile infection–related epilepsy syndrome (FIRES) Frontiers in Neurology febrile infection-related epilepsy syndrome adult new-onset refractory status epilepticus refractory status epilepticus long-term outcome case series |
| title | Long-term outcomes of adult cryptogenic febrile infection–related epilepsy syndrome (FIRES) |
| title_full | Long-term outcomes of adult cryptogenic febrile infection–related epilepsy syndrome (FIRES) |
| title_fullStr | Long-term outcomes of adult cryptogenic febrile infection–related epilepsy syndrome (FIRES) |
| title_full_unstemmed | Long-term outcomes of adult cryptogenic febrile infection–related epilepsy syndrome (FIRES) |
| title_short | Long-term outcomes of adult cryptogenic febrile infection–related epilepsy syndrome (FIRES) |
| title_sort | long term outcomes of adult cryptogenic febrile infection related epilepsy syndrome fires |
| topic | febrile infection-related epilepsy syndrome adult new-onset refractory status epilepticus refractory status epilepticus long-term outcome case series |
| url | https://www.frontiersin.org/articles/10.3389/fneur.2022.1081388/full |
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