Early initiation of intravenous cyclophosphamide and one‐year outcome in super‐refractory cryptogenic‐new onset refractory status epilepticus
Abstract To explore the potential efficacy of early initiation of intravenous cyclophosphamide (IVCPA), we reviewed consecutive four cases of super‐refractory cryptogenic‐new onset refractory status epilepticus (C‐NORSE) between 2015 and 2023. We compared functional outcomes at 3 months and 1 year a...
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Wiley
2025-02-01
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| Series: | Epilepsia Open |
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| Online Access: | https://doi.org/10.1002/epi4.13055 |
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| author | Yasufumi Yorichika Shuichiro Neshige Hideaki Sakahara Narumi Ono Megumi Nonaka Yuichiro Tagane Tomoaki Watanabe Keisuke Tachiyama Haruka Ishibashi Masahiro Nakamori Takeo Shishido Shiro Aoki Hiroki Ueno Yu Yamazaki Takahiro Iizuka Hirofumi Maruyama |
| author_facet | Yasufumi Yorichika Shuichiro Neshige Hideaki Sakahara Narumi Ono Megumi Nonaka Yuichiro Tagane Tomoaki Watanabe Keisuke Tachiyama Haruka Ishibashi Masahiro Nakamori Takeo Shishido Shiro Aoki Hiroki Ueno Yu Yamazaki Takahiro Iizuka Hirofumi Maruyama |
| author_sort | Yasufumi Yorichika |
| collection | DOAJ |
| description | Abstract To explore the potential efficacy of early initiation of intravenous cyclophosphamide (IVCPA), we reviewed consecutive four cases of super‐refractory cryptogenic‐new onset refractory status epilepticus (C‐NORSE) between 2015 and 2023. We compared functional outcomes at 3 months and 1 year after the onset between patients who received IVCPA within 20 days (early‐treated) and those who received it later (late‐treated). All patients (median age: 43 years) had a prodromal fever. Brain MRI revealed symmetrically increased FLAIR signals in the medial temporal lobes of all patients. Despite initiating antiseizure medications (ASMs) and first‐line immunotherapy (intravenous‐methylprednisolone and immunoglobulins) within a median of 3 days from onset, SE persisted >5 days. The diagnosis of C‐NORSE was suggested based on a high C‐NORSE score (6/6). Thus, all patients received IVCPA a median of 15.5 days after seizure onset (three within 20 days and one at 31 days). One of the three early‐treated patients also received tocilizumab. Early‐treated patients exhibited shorter sedation periods (median 29 vs. 75 days) and better 1 year functional status (mRS 1–2 vs. mRS 4) compared to the late‐treated patient. Early initiation of IVCPA and/or tocilizumab, along with ASMs, may contribute to a better one‐year functional status in super‐refractory C‐NORSE patients. Plain Language Summary This study demonstrates the potential efficacy of early administration of intravenous cyclophosphamide on one‐year functional status in patients with super‐refractory cryptogenic‐new onset refractory status epilepticus. “Early‐treated patients” who received it within 20 days of seizure onset achieved a good one‐year functional status. The “late‐treated patient” (Case 4) who received it later did not achieve a good functional status. Early initiation of cyclophosphamide, along with antiseizure medications, may contribute to a better one‐year functional status in this population. |
| format | Article |
| id | doaj-art-9717669deb944ccca7c4843fb4f2e643 |
| institution | Kabale University |
| issn | 2470-9239 |
| language | English |
| publishDate | 2025-02-01 |
| publisher | Wiley |
| record_format | Article |
| series | Epilepsia Open |
| spelling | doaj-art-9717669deb944ccca7c4843fb4f2e6432025-02-07T09:12:45ZengWileyEpilepsia Open2470-92392025-02-0110130731310.1002/epi4.13055Early initiation of intravenous cyclophosphamide and one‐year outcome in super‐refractory cryptogenic‐new onset refractory status epilepticusYasufumi Yorichika0Shuichiro Neshige1Hideaki Sakahara2Narumi Ono3Megumi Nonaka4Yuichiro Tagane5Tomoaki Watanabe6Keisuke Tachiyama7Haruka Ishibashi8Masahiro Nakamori9Takeo Shishido10Shiro Aoki11Hiroki Ueno12Yu Yamazaki13Takahiro Iizuka14Hirofumi Maruyama15Department of Clinical Neuroscience and Therapeutics Hiroshima University Graduate School of Biomedical and Health Sciences Hiroshima JapanDepartment of Clinical Neuroscience and Therapeutics Hiroshima University Graduate School of Biomedical and Health Sciences Hiroshima JapanDepartment of Clinical Neuroscience and Therapeutics Hiroshima University Graduate School of Biomedical and Health Sciences Hiroshima JapanDepartment of Clinical Neuroscience and Therapeutics Hiroshima University Graduate School of Biomedical and Health Sciences Hiroshima JapanDepartment of Clinical Neuroscience and Therapeutics Hiroshima University Graduate School of Biomedical and Health Sciences Hiroshima JapanDepartment of Clinical Neuroscience and Therapeutics Hiroshima University Graduate School of Biomedical and Health Sciences Hiroshima JapanDepartment of Clinical Neuroscience and Therapeutics Hiroshima University Graduate School of Biomedical and Health Sciences Hiroshima JapanDepartment of Clinical Neuroscience and Therapeutics Hiroshima University Graduate School of Biomedical and Health Sciences Hiroshima JapanDepartment of Clinical Neuroscience and Therapeutics Hiroshima University Graduate School of Biomedical and Health Sciences Hiroshima JapanDepartment of Clinical Neuroscience and Therapeutics Hiroshima University Graduate School of Biomedical and Health Sciences Hiroshima JapanDepartment of Neurology Hiroshima North Medical Center Asa Citizens Hospital Hiroshima JapanDepartment of Clinical Neuroscience and Therapeutics Hiroshima University Graduate School of Biomedical and Health Sciences Hiroshima JapanDepartment of Neurology Hiroshima City Hiroshima Citizens Hospital Hiroshima JapanDepartment of Clinical Neuroscience and Therapeutics Hiroshima University Graduate School of Biomedical and Health Sciences Hiroshima JapanDepartment of Neurology Kitasato University School of Medicine Sagamihara JapanDepartment of Clinical Neuroscience and Therapeutics Hiroshima University Graduate School of Biomedical and Health Sciences Hiroshima JapanAbstract To explore the potential efficacy of early initiation of intravenous cyclophosphamide (IVCPA), we reviewed consecutive four cases of super‐refractory cryptogenic‐new onset refractory status epilepticus (C‐NORSE) between 2015 and 2023. We compared functional outcomes at 3 months and 1 year after the onset between patients who received IVCPA within 20 days (early‐treated) and those who received it later (late‐treated). All patients (median age: 43 years) had a prodromal fever. Brain MRI revealed symmetrically increased FLAIR signals in the medial temporal lobes of all patients. Despite initiating antiseizure medications (ASMs) and first‐line immunotherapy (intravenous‐methylprednisolone and immunoglobulins) within a median of 3 days from onset, SE persisted >5 days. The diagnosis of C‐NORSE was suggested based on a high C‐NORSE score (6/6). Thus, all patients received IVCPA a median of 15.5 days after seizure onset (three within 20 days and one at 31 days). One of the three early‐treated patients also received tocilizumab. Early‐treated patients exhibited shorter sedation periods (median 29 vs. 75 days) and better 1 year functional status (mRS 1–2 vs. mRS 4) compared to the late‐treated patient. Early initiation of IVCPA and/or tocilizumab, along with ASMs, may contribute to a better one‐year functional status in super‐refractory C‐NORSE patients. Plain Language Summary This study demonstrates the potential efficacy of early administration of intravenous cyclophosphamide on one‐year functional status in patients with super‐refractory cryptogenic‐new onset refractory status epilepticus. “Early‐treated patients” who received it within 20 days of seizure onset achieved a good one‐year functional status. The “late‐treated patient” (Case 4) who received it later did not achieve a good functional status. Early initiation of cyclophosphamide, along with antiseizure medications, may contribute to a better one‐year functional status in this population.https://doi.org/10.1002/epi4.13055autoantibodiesbrain MRIimmunotherapyNORSE |
| spellingShingle | Yasufumi Yorichika Shuichiro Neshige Hideaki Sakahara Narumi Ono Megumi Nonaka Yuichiro Tagane Tomoaki Watanabe Keisuke Tachiyama Haruka Ishibashi Masahiro Nakamori Takeo Shishido Shiro Aoki Hiroki Ueno Yu Yamazaki Takahiro Iizuka Hirofumi Maruyama Early initiation of intravenous cyclophosphamide and one‐year outcome in super‐refractory cryptogenic‐new onset refractory status epilepticus Epilepsia Open autoantibodies brain MRI immunotherapy NORSE |
| title | Early initiation of intravenous cyclophosphamide and one‐year outcome in super‐refractory cryptogenic‐new onset refractory status epilepticus |
| title_full | Early initiation of intravenous cyclophosphamide and one‐year outcome in super‐refractory cryptogenic‐new onset refractory status epilepticus |
| title_fullStr | Early initiation of intravenous cyclophosphamide and one‐year outcome in super‐refractory cryptogenic‐new onset refractory status epilepticus |
| title_full_unstemmed | Early initiation of intravenous cyclophosphamide and one‐year outcome in super‐refractory cryptogenic‐new onset refractory status epilepticus |
| title_short | Early initiation of intravenous cyclophosphamide and one‐year outcome in super‐refractory cryptogenic‐new onset refractory status epilepticus |
| title_sort | early initiation of intravenous cyclophosphamide and one year outcome in super refractory cryptogenic new onset refractory status epilepticus |
| topic | autoantibodies brain MRI immunotherapy NORSE |
| url | https://doi.org/10.1002/epi4.13055 |
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