Internal Medicine
Online ISSN : 1349-7235
Print ISSN : 0918-2918
ISSN-L : 0918-2918
Early Recovery from Cryptogenic NORSE Following Repeated C-NORSE Score Assessment and Cyclophosphamide Treatment: A Case Report
Ryotaro OkochiJun SakaiSho ShimohamaKei IshizuchiJin NakaharaTakahiro IizukaMunenori Oyama
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JOURNAL OPEN ACCESS Advance online publication

Article ID: 6055-25

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Abstract

Cryptogenic new-onset refractory status epilepticus (C-NORSE) is a rare, severe condition characterized by refractory status epilepticus without identifiable causes. It is often unresponsive to first-line immunotherapy. The C-NORSE score is a useful diagnostic tool, but in the early phase, when the score remains low and antibody test results are unavailable, it is difficult to initiate second-line immunotherapy, including cyclophosphamide. We report a 27-year-old man with C-NORSE who achieved early recovery and favorable outcome following administration of intravenous cyclophosphamide, despite a low C-NORSE score on admission, suggesting the importance of repeated assessment using the C-NORSE score and early administration of cyclophosphamide.

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© 2025 by The Japanese Society of Internal Medicine

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