{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,8,3]],"date-time":"2026-08-03T22:28:50Z","timestamp":1785796130031,"version":"3.56.0"},"reference-count":22,"publisher":"Wiley","issue":"1","license":[{"start":{"date-parts":[[2024,12,19]],"date-time":"2024-12-19T00:00:00Z","timestamp":1734566400000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/creativecommons.org\/licenses\/by-nc-nd\/4.0\/"}],"content-domain":{"domain":["onlinelibrary.wiley.com"],"crossmark-restriction":true},"short-container-title":["Epilepsia Open"],"published-print":{"date-parts":[[2025,2]]},"abstract":"<jats:title>Abstract<\/jats:title><jats:sec><jats:label\/><jats:p>To explore the potential efficacy of early initiation of intravenous cyclophosphamide (IVCPA), we reviewed consecutive four cases of super\u2010refractory cryptogenic\u2010new onset refractory status epilepticus (C\u2010NORSE) between 2015 and 2023. We compared functional outcomes at 3\u2009months and 1\u2009year after the onset between patients who received IVCPA within 20\u2009days (early\u2010treated) and those who received it later (late\u2010treated). All patients (median age: 43\u2009years) 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\u2010line immunotherapy (intravenous\u2010methylprednisolone and immunoglobulins) within a median of 3\u2009days from onset, SE persisted &gt;5\u2009days. The diagnosis of C\u2010NORSE was suggested based on a high C\u2010NORSE score (6\/6). Thus, all patients received IVCPA a median of 15.5\u2009days after seizure onset (three within 20\u2009days and one at 31\u2009days). One of the three early\u2010treated patients also received tocilizumab. Early\u2010treated patients exhibited shorter sedation periods (median 29 vs. 75\u2009days) and better 1\u2009year functional status (mRS 1\u20132 vs. mRS 4) compared to the late\u2010treated patient. Early initiation of IVCPA and\/or tocilizumab, along with ASMs, may contribute to a better one\u2010year functional status in super\u2010refractory C\u2010NORSE patients.<\/jats:p><\/jats:sec><jats:sec><jats:title>Plain Language Summary<\/jats:title><jats:p>This study demonstrates the potential efficacy of early administration of intravenous cyclophosphamide on one\u2010year functional status in patients with super\u2010refractory cryptogenic\u2010new onset refractory status epilepticus. \u201cEarly\u2010treated patients\u201d who received it within 20\u2009days of seizure onset achieved a good one\u2010year functional status. The \u201clate\u2010treated patient\u201d (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\u2010year functional status in this population.<\/jats:p><\/jats:sec>","DOI":"10.1002\/epi4.13055","type":"journal-article","created":{"date-parts":[[2024,12,20]],"date-time":"2024-12-20T00:38:19Z","timestamp":1734655099000},"page":"307-313","update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":5,"title":["Early initiation of intravenous cyclophosphamide and one\u2010year outcome in super\u2010refractory cryptogenic\u2010new onset refractory status epilepticus"],"prefix":"10.1002","volume":"10","author":[{"ORCID":"https:\/\/orcid.org\/0009-0000-5816-8445","authenticated-orcid":false,"given":"Yasufumi","family":"Yorichika","sequence":"first","affiliation":[{"name":"Department of Clinical Neuroscience and Therapeutics Hiroshima University Graduate School of Biomedical and Health Sciences  Hiroshima 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