{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,9,9]],"date-time":"2026-09-09T08:06:37Z","timestamp":1788941197511,"version":"build-2803163510"},"reference-count":56,"publisher":"Wiley","issue":"9","license":[{"start":{"date-parts":[[2026,8,4]],"date-time":"2026-08-04T00:00:00Z","timestamp":1785801600000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/creativecommons.org\/licenses\/by\/4.0\/"},{"start":{"date-parts":[[2026,8,4]],"date-time":"2026-08-04T00:00:00Z","timestamp":1785801600000},"content-version":"tdm","delay-in-days":0,"URL":"http:\/\/doi.wiley.com\/10.1002\/tdm_license_1.1"}],"content-domain":{"domain":["onlinelibrary.wiley.com"],"crossmark-restriction":true},"short-container-title":["Ann Clin Transl Neurol"],"published-print":{"date-parts":[[2026,9]]},"abstract":"<jats:title>ABSTRACT<\/jats:title>\n                  <jats:p>\n                    The claustrum sign is a distinctive neuroimaging finding characterized by bilateral T2\/FLAIR hyperintensity of the claustrum, one of the most interconnected regions of the human brain. It was first described in new\u2010onset refractory status epilepticus (NORSE) and febrile infection\u2013related epilepsy syndrome (FIRES). A systematic search was conducted according to PRISMA guidelines from 2018 (formal definition of NORSE) to February 2026 (PROSPERO 2026 CRD420261307306). We included peer\u2010reviewed original studies reporting MRI findings and patient\u2010level data, and compared clinical characteristics, treatments, and outcomes of patients with claustrum alteration versus NORSE\/FIRES patients without this finding. Of 785 records identified, 65 studies met inclusion criteria; 41 provided individual patient data, yielding 206 NORSE cases (72 with the claustrum sign, 134 without). All but one patient with the claustrum sign met criteria for cryptogenic NORSE. Patients were predominantly young (mean age 24.9\u2009years) and had a febrile prodrome in 93% of cases. Convulsive status epilepticus (SE) occurred in 98%, progressing to super\u2010refractory SE (SRSE) in more than half reported cases. Bilateral claustrum hyperintensity was detected in 68\/71 patients during the acute phase, typically emerging 7\u2009days after SE onset. Immunotherapy was administered in 92% of patients. In\u2010hospital mortality was 9%, while 74% of patients developed chronic epilepsy. Compared with patients without claustrum involvement, those with the claustrum sign more often had febrile onset (93% vs. 51%,\n                    <jats:italic>p<\/jats:italic>\n                    \u2009&lt;\u20090.001) and convulsive SE (98% vs. 81.5%,\n                    <jats:italic>p<\/jats:italic>\n                    \u2009=\u20090.008), lower rates of SRSE (51% vs. 96.5%,\n                    <jats:italic>p<\/jats:italic>\n                    \u2009&lt;\u20090.001), and ICU admission (71% vs. 99%,\n                    <jats:italic>p<\/jats:italic>\n                    \u2009&lt;\u20090.001), and a trend toward lower mortality (9% vs. 16%,\n                    <jats:italic>p<\/jats:italic>\n                    \u2009=\u20090.240). Recognition of the claustrum sign may support early diagnosis and targeted treatment.\n                  <\/jats:p>\n                  <jats:p>\n                    <jats:bold>Trial Registration:<\/jats:bold>\n                    PROSPERO 2026 [CRD420261307306]\n                  <\/jats:p>","DOI":"10.1002\/acn3.70490","type":"journal-article","created":{"date-parts":[[2026,8,5]],"date-time":"2026-08-05T04:57:01Z","timestamp":1785905821000},"page":"1750-1758","update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":0,"title":["Claustrum Involvement in New Onset Refractory Status Epilepticus: A Systematic Review"],"prefix":"10.1002","volume":"13","author":[{"ORCID":"https:\/\/orcid.org\/0009-0004-0981-4788","authenticated-orcid":false,"given":"Margherita","family":"Burani","sequence":"first","affiliation":[{"name":"Department of Biomedical, Metabolic and Neural Sciences University of Modena and Reggio Emilia  Modena Italy"},{"name":"Neurophysiology Unit and Epilepsy Centre, Neuroscience Department Modena AOU  Modena Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-6827-0099","authenticated-orcid":false,"given":"Lorenzo","family":"Muccioli","sequence":"additional","affiliation":[{"name":"IRCCS Istituto delle Scienze Neurologiche di Bologna (Full Member of the ERN EpiCARE)  Bologna Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-3585-5872","authenticated-orcid":false,"given":"Giada","family":"Giovannini","sequence":"additional","affiliation":[{"name":"Neurophysiology Unit and Epilepsy Centre, Neuroscience Department Modena AOU  Modena Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-5717-7363","authenticated-orcid":false,"given":"Niccol\u00f2","family":"Orlandi","sequence":"additional","affiliation":[{"name":"Neurophysiology Unit and Epilepsy Centre, Neuroscience Department Modena AOU  Modena Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-1109-7296","authenticated-orcid":false,"given":"Francesca","family":"Bisulli","sequence":"additional","affiliation":[{"name":"IRCCS Istituto delle Scienze Neurologiche di Bologna (Full Member of the ERN EpiCARE)  Bologna Italy"},{"name":"Department of Biomedical and Neuromotor Sciences University of Bologna  Bologna Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-0334-539X","authenticated-orcid":false,"given":"Stefano","family":"Meletti","sequence":"additional","affiliation":[{"name":"Department of Biomedical, Metabolic and Neural Sciences University of Modena and Reggio Emilia  Modena Italy"},{"name":"Neurophysiology Unit and Epilepsy Centre, Neuroscience Department Modena AOU  Modena Italy"},{"name":"Department of Medicine and Surgery University of Parma  Parma 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