{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,6,5]],"date-time":"2026-06-05T02:28:54Z","timestamp":1780626534042,"version":"3.54.1"},"reference-count":13,"publisher":"Wiley","issue":"12","license":[{"start":{"date-parts":[[2023,10,5]],"date-time":"2023-10-05T00:00:00Z","timestamp":1696464000000},"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"],"published-print":{"date-parts":[[2023,12]]},"abstract":"<jats:title>Abstract<\/jats:title><jats:p>Most cases with new onset refractory status epilepticus (NORSE) remain cryptogenic despite extensive diagnostic workup. The aim of this study was to analyze the etiology and clinical features of NORSE and investigate known or potentially novel autoantibodies in cryptogenic NORSE (cNORSE). We retrospectively assessed the medical records of adults with status epilepticus at a Swiss tertiary referral center between 2010 and 2021. Demographic, diagnostic, therapeutic, and outcome parameters were characterized. We performed post hoc screening for known or potentially novel autoantibodies including immunohistochemistry (IHC) on rat brain with cerebrospinal fluid (CSF) and serum samples of cNORSE. Twenty patients with NORSE were identified. Etiologies included infections (<jats:italic>n<\/jats:italic>\u2009=\u20094), Creutzfeldt\u2013Jakob disease (<jats:italic>n<\/jats:italic>\u2009=\u20091), CASPR2 autoimmune encephalitis (<jats:italic>n<\/jats:italic>\u2009=\u20091), and carotid artery stenosis with recurrent perfusion deficit (<jats:italic>n<\/jats:italic>\u2009=\u20091). Thirteen cases (65%) were cryptogenic despite detailed evaluation. A posteriori IHC for neuronal autoantibodies yielded negative results in all available serum (<jats:italic>n<\/jats:italic>\u2009=\u200911) and CSF (<jats:italic>n<\/jats:italic>\u2009=\u20099) samples of cNORSE. Our results suggest that neuronal antibodies are unlikely to play a major role in the pathogenesis of cNORSE. Future studies should rather focus on other\u2014especially T\u2010cell\u2010 and cytokine\u2010mediated\u2014mechanisms of autoinflammation in this devastating disease, which is far too poorly understood so far.<\/jats:p>","DOI":"10.1111\/epi.17755","type":"journal-article","created":{"date-parts":[[2023,8,22]],"date-time":"2023-08-22T19:39:59Z","timestamp":1692733199000},"update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":10,"title":["The role of neuronal antibodies in cryptogenic new onset refractory status epilepticus"],"prefix":"10.1111","volume":"64","author":[{"ORCID":"https:\/\/orcid.org\/0000-0002-9791-169X","authenticated-orcid":false,"given":"Amanda","family":"Eisele","sequence":"first","affiliation":[{"name":"Department of Neurology and Clinical Neuroscience Center University Hospital and University of Zurich  Zurich Switzerland"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Matthias","family":"Schwager","sequence":"additional","affiliation":[{"name":"Institute for Intensive Care Medicine, University Hospital Zurich  Zurich Switzerland"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-5386-0811","authenticated-orcid":false,"given":"Stefan Yu","family":"B\u00f6gli","sequence":"additional","affiliation":[{"name":"Department of Neurology and Clinical Neuroscience Center University Hospital and University of Zurich  Zurich Switzerland"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Ina","family":"Reichen","sequence":"additional","affiliation":[{"name":"Department of Neurology and Clinical Neuroscience Center University Hospital and University of Zurich  Zurich Switzerland"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Justina","family":"Dargvainiene","sequence":"additional","affiliation":[{"name":"Institute of Clinical Chemistry, University Hospital Schleswig\u2010Holstein  Kiel Germany"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Klaus\u2010Peter","family":"Wandinger","sequence":"additional","affiliation":[{"name":"Institute of Clinical Chemistry, University Hospital Schleswig\u2010Holstein  Kiel Germany"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-6135-8642","authenticated-orcid":false,"given":"Lukas","family":"Imbach","sequence":"additional","affiliation":[{"name":"Swiss Epilepsy Center, Klinik Lengg  Zurich Switzerland"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Marcellina","family":"Haeberlin","sequence":"additional","affiliation":[{"name":"Department of Neurology and Clinical Neuroscience Center University Hospital and University of Zurich  Zurich Switzerland"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Emanuela","family":"Keller","sequence":"additional","affiliation":[{"name":"Institute for Intensive Care Medicine, University Hospital Zurich  Zurich Switzerland"},{"name":"Department of Neurosurgery and Clinical Neuroscience Center University Hospital and University of Zurich  Zurich Switzerland"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Ilijas","family":"Jelcic","sequence":"additional","affiliation":[{"name":"Department of Neurology and Clinical Neuroscience Center University Hospital and University of Zurich  Zurich Switzerland"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Marian","family":"Galovic","sequence":"additional","affiliation":[{"name":"Department of Neurology and Clinical Neuroscience Center University Hospital and University of Zurich  Zurich Switzerland"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Giovanna","family":"Brandi","sequence":"additional","affiliation":[{"name":"Institute for Intensive Care Medicine, University Hospital Zurich  Zurich Switzerland"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"311","published-online":{"date-parts":[[2023,10,5]]},"reference":[{"key":"e_1_2_10_2_1","doi-asserted-by":"publisher","DOI":"10.1111\/epi.17391"},{"key":"e_1_2_10_3_1","doi-asserted-by":"publisher","DOI":"10.1212\/WNL.0000000000001940"},{"key":"e_1_2_10_4_1","doi-asserted-by":"publisher","DOI":"10.1111\/ene.15149"},{"key":"e_1_2_10_5_1","doi-asserted-by":"publisher","DOI":"10.1212\/WNL.0000000000003414"},{"key":"e_1_2_10_6_1","doi-asserted-by":"publisher","DOI":"10.1002\/ana.10713"},{"key":"e_1_2_10_7_1","doi-asserted-by":"publisher","DOI":"10.1136\/jnnp-2016-314758"},{"key":"e_1_2_10_8_1","doi-asserted-by":"publisher","DOI":"10.1212\/NXI.0000000000000396"},{"key":"e_1_2_10_9_1","doi-asserted-by":"publisher","DOI":"10.1136\/jnnp-2014-309388"},{"key":"e_1_2_10_10_1","doi-asserted-by":"publisher","DOI":"10.1111\/epi.16275"},{"key":"e_1_2_10_11_1","doi-asserted-by":"publisher","DOI":"10.1002\/ana.25439"},{"key":"e_1_2_10_12_1","first-page":"293","article-title":"Diminished toll\u2010like receptor response in febrile infection\u2010related epilepsy syndrome (FIRES)","volume":"43","author":"Hsieh MY","year":"2020","journal-title":"Biom 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