{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,7,7]],"date-time":"2026-07-07T14:15:45Z","timestamp":1783433745801,"version":"3.54.6"},"reference-count":7,"publisher":"Wiley","issue":"6","license":[{"start":{"date-parts":[[2026,6,23]],"date-time":"2026-06-23T00:00:00Z","timestamp":1782172800000},"content-version":"vor","delay-in-days":22,"URL":"http:\/\/creativecommons.org\/licenses\/by-nc\/4.0\/"},{"start":{"date-parts":[[2026,6,1]],"date-time":"2026-06-01T00:00:00Z","timestamp":1780272000000},"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":["Euro J of Neurology"],"published-print":{"date-parts":[[2026,6]]},"abstract":"<jats:title>ABSTRACT<\/jats:title>\n                  <jats:sec>\n                    <jats:title>Background and Purpose<\/jats:title>\n                    <jats:p>Familial cerebral cavernous malformations (fCCM) may cause focal neurological deficits (FND) due to hemorrhagic or non\u2010hemorrhagic events and epileptic seizures (ES). With the widespread use of magnetic resonance imaging (MRI) and genetic testing, diagnosis is now frequently established at a presymptomatic stage, before any clinical manifestation. However, the subsequent risk of a first clinical event in these individuals remains poorly defined.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Methods<\/jats:title>\n                    <jats:p>We conducted a cohort\u2010based analysis of retrospectively collected longitudinal data from 79 patients with fCCM who were asymptomatic at diagnosis (median age, 41.7\u2009years) and managed in a national referral center.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Results<\/jats:title>\n                    <jats:p>\n                      Over 649.6 patient\u2010years (median follow\u2010up, 4.0\u2009years), 23 patients (29%) experienced a first clinical event. Post\u2010diagnostic event\u2010free survival was approximately 97% at 1\u2009year, 79% at 5\u2009years, and 69% at 10\u2009years. Incidence rates for a first event were 35.4 per 1000 person\u2010years for any first event (FND or ES), 23.1 per 1000 person\u2010years for a first FND, and 15.4 per 1000 person\u2010years for a first ES. Male sex was independently associated with higher risk (HR 4.20; 95% CI, 1.60\u201311.04;\n                      <jats:italic>p<\/jats:italic>\n                      \u2009=\u20090.0036).\n                    <\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Conclusions<\/jats:title>\n                    <jats:p>These data provide pragmatic post\u2010diagnostic risk estimates to guide counseling and surveillance in asymptomatic fCCM.<\/jats:p>\n                  <\/jats:sec>","DOI":"10.1111\/ene.70669","type":"journal-article","created":{"date-parts":[[2026,6,23]],"date-time":"2026-06-23T12:55:01Z","timestamp":1782219301000},"update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":0,"title":["Risk of Clinical Events in Presymptomatic Familial Cerebral Cavernous Malformations"],"prefix":"10.1111","volume":"33","author":[{"ORCID":"https:\/\/orcid.org\/0009-0005-7185-6396","authenticated-orcid":false,"given":"Constantina Rodica","family":"Popa","sequence":"first","affiliation":[{"name":"Lariboisi\u00e8re Hospital, CNVT\u2010CERVCO, AP\u2010HP, FHU NeuroVasc2030, DMU Neurosciences  Paris France"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Dominique","family":"Herv\u00e9","sequence":"additional","affiliation":[{"name":"Lariboisi\u00e8re Hospital, CNVT\u2010CERVCO, AP\u2010HP, FHU NeuroVasc2030, DMU Neurosciences  Paris France"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Nassira","family":"Alili","sequence":"additional","affiliation":[{"name":"Lariboisi\u00e8re Hospital, CNVT\u2010CERVCO, AP\u2010HP, FHU NeuroVasc2030, DMU Neurosciences  Paris France"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Estelle","family":"Dubus","sequence":"additional","affiliation":[{"name":"Lariboisi\u00e8re Hospital, CNVT\u2010CERVCO, AP\u2010HP, FHU NeuroVasc2030, DMU Neurosciences  Paris France"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Abbas","family":"Taleb","sequence":"additional","affiliation":[{"name":"Lariboisi\u00e8re Hospital, CNVT\u2010CERVCO, AP\u2010HP, FHU NeuroVasc2030, DMU Neurosciences  Paris France"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Fanny","family":"Fernandes","sequence":"additional","affiliation":[{"name":"Lariboisi\u00e8re Hospital, CNVT\u2010CERVCO, AP\u2010HP, FHU NeuroVasc2030, DMU Neurosciences  Paris France"},{"name":"Universit\u00e9 Paris Cit\u00e9, INSERM U1127, Paris Brain Institute, GenoVasc Team  Paris France"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Florence","family":"Riant","sequence":"additional","affiliation":[{"name":"Department of Medical Genetics, Molecular and Cellular Neurogenetics Unit Piti\u00e9\u2010Salp\u00eatri\u00e8re Hospital, AP\u2010HP, Sorbonne University  Paris France"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Elisabeth Tournier","family":"Lasserve","sequence":"additional","affiliation":[{"name":"Paris Brain Institute, INSERM UMR1127, GenoVasc Team, Sorbonne University  Paris France"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Louis","family":"Lambert","sequence":"additional","affiliation":[{"name":"Paris Brain Institute, INSERM UMR1127, GenoVasc Team, Sorbonne University  Paris France"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-8436-6074","authenticated-orcid":false,"given":"Hugues","family":"Chabriat","sequence":"additional","affiliation":[{"name":"Lariboisi\u00e8re Hospital, CNVT\u2010CERVCO, AP\u2010HP, FHU NeuroVasc2030, DMU Neurosciences  Paris France"},{"name":"Universit\u00e9 Paris Cit\u00e9, INSERM U1127, Paris Brain Institute, GenoVasc Team  Paris France"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-6096-3595","authenticated-orcid":false,"given":"St\u00e9phanie","family":"Guey","sequence":"additional","affiliation":[{"name":"Lariboisi\u00e8re Hospital, CNVT\u2010CERVCO, AP\u2010HP, FHU NeuroVasc2030, DMU Neurosciences  Paris France"},{"name":"Universit\u00e9 Paris Cit\u00e9, INSERM U1127, Paris Brain Institute, GenoVasc Team  Paris France"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"311","published-online":{"date-parts":[[2026,6,23]]},"reference":[{"key":"e_1_2_11_2_1","doi-asserted-by":"publisher","DOI":"10.1161\/STROKEAHA.118.022314"},{"key":"e_1_2_11_3_1","doi-asserted-by":"publisher","DOI":"10.1016\/S1474-4422(07)70053-4"},{"key":"e_1_2_11_4_1","doi-asserted-by":"publisher","DOI":"10.1111\/ene.15253"},{"key":"e_1_2_11_5_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.jstrokecerebrovasdis.2024.108041"},{"key":"e_1_2_11_6_1","doi-asserted-by":"publisher","DOI":"10.3171\/2023.1.JNS222434"},{"key":"e_1_2_11_7_1","doi-asserted-by":"publisher","DOI":"10.1212\/WNL.0000000000213798"},{"key":"e_1_2_11_8_1","doi-asserted-by":"publisher","DOI":"10.1212\/WNL.57.10.1825"}],"container-title":["European Journal of Neurology"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/pdf\/10.1111\/ene.70669","content-type":"application\/pdf","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/full-xml\/10.1111\/ene.70669","content-type":"application\/xml","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/pdf\/10.1111\/ene.70669","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2026,7,7]],"date-time":"2026-07-07T13:37:07Z","timestamp":1783431427000},"score":1,"resource":{"primary":{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/10.1111\/ene.70669"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2026,6]]},"references-count":7,"journal-issue":{"issue":"6","published-print":{"date-parts":[[2026,6]]}},"alternative-id":["10.1111\/ene.70669"],"URL":"https:\/\/doi.org\/10.1111\/ene.70669","archive":["Portico"],"relation":{},"ISSN":["1351-5101","1468-1331"],"issn-type":[{"value":"1351-5101","type":"print"},{"value":"1468-1331","type":"electronic"}],"subject":[],"published":{"date-parts":[[2026,6]]},"assertion":[{"value":"2026-03-04","order":0,"name":"received","label":"Received","group":{"name":"publication_history","label":"Publication History"}},{"value":"2026-06-02","order":2,"name":"accepted","label":"Accepted","group":{"name":"publication_history","label":"Publication History"}},{"value":"2026-06-23","order":3,"name":"published","label":"Published","group":{"name":"publication_history","label":"Publication History"}}],"article-number":"e70669"}}