{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,5,14]],"date-time":"2026-05-14T08:39:50Z","timestamp":1778747990034,"version":"3.51.4"},"reference-count":20,"publisher":"Wiley","issue":"5","license":[{"start":{"date-parts":[[2025,5,3]],"date-time":"2025-05-03T00:00:00Z","timestamp":1746230400000},"content-version":"vor","delay-in-days":2,"URL":"http:\/\/creativecommons.org\/licenses\/by-nc-nd\/4.0\/"}],"content-domain":{"domain":["onlinelibrary.wiley.com"],"crossmark-restriction":true},"short-container-title":["Euro J of Neurology"],"published-print":{"date-parts":[[2025,5]]},"abstract":"<jats:title>ABSTRACT<\/jats:title><jats:sec><jats:title>Background<\/jats:title><jats:p>To determine whether hierarchical unsupervised cluster analysis identifies a phenotypic distinction in adult patients with primary CNS vasculitis (PCNSV).<\/jats:p><\/jats:sec><jats:sec><jats:title>Methods<\/jats:title><jats:p>An agglomerative hierarchical cluster analysis based on the Ward method was conducted, including 153 patients with complete baseline phenotypic characterization in the COVAC' registry.<\/jats:p><\/jats:sec><jats:sec><jats:title>Results<\/jats:title><jats:p>The hierarchical analysis identified two main clusters. In Cluster 1 (<jats:italic>n<\/jats:italic>\u2009=\u2009109 patients, 71%), patients more frequently had a motor deficit (<jats:italic>p<\/jats:italic>\u2009=\u20090.039), \u2265\u20091 acute brain infarct (<jats:italic>p<\/jats:italic>\u2009&lt;\u20090.001), and \u2265\u20091 intracranial stenosis on CT or MR angiogram (<jats:italic>p<\/jats:italic>\u2009&lt;\u20090.001) than patients in Cluster 2 (<jats:italic>n<\/jats:italic>\u2009=\u200944 patients, 29%). Conversely, patients in Cluster 2 more frequently had seizures (<jats:italic>p<\/jats:italic>\u2009&lt;\u20090.001), cognitive impairment (<jats:italic>p<\/jats:italic>\u2009=\u20090.002), gadolinium\u2010enhanced parenchymal lesions (<jats:italic>p<\/jats:italic>\u2009&lt;\u20090.001), leptomeningeal enhancement (<jats:italic>p<\/jats:italic>\u2009&lt;\u20090.001), \u2265\u20091 cerebral microbleed (<jats:italic>p<\/jats:italic>\u2009&lt;\u20090.001), and intracranial hemorrhage(s) (<jats:italic>p<\/jats:italic>\u2009&lt;\u20090.001). In multivariable logistic regression, gadolinium\u2010enhanced parenchymal lesions were significantly associated with Cluster 2 lesions (OR\u2009=\u200935.53 [95% CI: 3.91\u2013322.81], <jats:italic>p<\/jats:italic>\u2009=\u20090.002). Conversely, \u2265\u20091 acute brain infarct was significantly associated with Cluster 1 (OR\u2009=\u20090.003 [95% CI: 0.01\u20130.03], <jats:italic>p<\/jats:italic>\u2009&lt;\u20090.001). A CNS biopsy was positive in 11\/40 (28%) patients from Cluster 1 and 35\/37 (95%) patients from Cluster 2 (<jats:italic>p<\/jats:italic>\u2009&lt;\u20090.001). At 12\u2009months, functional independence (modified Rankin scale score \u2264\u20092) did not differ between the two groups (<jats:italic>p<\/jats:italic>\u2009=\u20090.17). Relapse and mortality rates did not differ between the clusters (<jats:italic>p<\/jats:italic>\u2009=\u20090.17 and <jats:italic>p<\/jats:italic>\u2009=\u20090.23, respectively).<\/jats:p><\/jats:sec><jats:sec><jats:title>Conclusion<\/jats:title><jats:p>This unsupervised analysis of a large PCNSV cohort identified two different clinical and radiological phenotypes with different diagnostic work\u2010ups, which confirms the relevance of distinguishing PCNSV phenotypes according to the sizes of affected vessels.<\/jats:p><\/jats:sec>","DOI":"10.1111\/ene.70174","type":"journal-article","created":{"date-parts":[[2025,5,3]],"date-time":"2025-05-03T09:56:00Z","timestamp":1746266160000},"update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":1,"title":["Data\u2010Driven Clustering Approach to Identify Different Phenotypes of Primary Central Nervous System Vasculitis"],"prefix":"10.1111","volume":"32","author":[{"ORCID":"https:\/\/orcid.org\/0000-0001-9083-8365","authenticated-orcid":false,"given":"Hubert","family":"de Boysson","sequence":"first","affiliation":[{"name":"Service de M\u00e9decine Interne, CHU de Caen  Caen France"},{"name":"Universit\u00e9 Caen\u2010Normandie  Caen France"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Ahmad","family":"Nehme","sequence":"additional","affiliation":[{"name":"Universit\u00e9 Caen\u2010Normandie  Caen France"},{"name":"Service de Neurologie, CHU de Caen  Caen France"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Anais R.","family":"Briant","sequence":"additional","affiliation":[{"name":"Unit\u00e9 de Biostatistiques et de Recherche Clinique, CHU de Caen\u2010Normandie  Caen France"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Sonia","family":"Alamowitch","sequence":"additional","affiliation":[{"name":"Urgences C\u00e9r\u00e9bro\u2010Vasculaires, H\u00f4pital Piti\u00e9\u2010Salp\u00eatri\u00e8re, Sorbonne Universit\u00e9, STARE Team\u2010 iCRIN, Institut du Cerveau et de la Moelle \u00e9pini\u00e8re  Paris France"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Achille","family":"Aouba","sequence":"additional","affiliation":[{"name":"Service de M\u00e9decine Interne, CHU de Caen  Caen France"},{"name":"Universit\u00e9 Caen\u2010Normandie  Caen France"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Caroline","family":"Arquizan","sequence":"additional","affiliation":[{"name":"Service de Neurologie, CHU de Montpellier  Montpellier France"},{"name":"INSERM U1266  Paris France"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Gr\u00e9goire","family":"Boulouis","sequence":"additional","affiliation":[{"name":"Service de Radiologie, CHU de Tours  Tours France"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-1788-8897","authenticated-orcid":false,"given":"Jean","family":"Capron","sequence":"additional","affiliation":[{"name":"Service de Neurologie, H\u00f4pital Piti\u00e9\u2010Salp\u00eatri\u00e8re, AP\u2010HP  Paris France"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-4199-995X","authenticated-orcid":false,"given":"Barbara","family":"Casolla","sequence":"additional","affiliation":[{"name":"Service de Neurologie, CHU de Nice  Nice France"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-2087-5563","authenticated-orcid":false,"given":"Christian","family":"Denier","sequence":"additional","affiliation":[{"name":"Service de Neurologie, H\u00f4pital Bic\u00eatre, AP\u2010HP  Paris France"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Nelly","family":"Dequatre","sequence":"additional","affiliation":[{"name":"Service de Neurologie, CHU de Lille  Lille France"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Olivier","family":"Detante","sequence":"additional","affiliation":[{"name":"Service de Neurologie, CHU de Grenoble  Grenoble France"},{"name":"Univ. 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