{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,11,7]],"date-time":"2026-11-07T10:03:12Z","timestamp":1794045792639,"version":"4.8.0"},"reference-count":33,"publisher":"Ovid Technologies (Wolters Kluwer Health)","issue":"5","content-domain":{"domain":["www.ahajournals.org"],"crossmark-restriction":true},"short-container-title":["Stroke"],"published-print":{"date-parts":[[2021,5]]},"abstract":"<jats:sec>\n                    <jats:title>Background and Purpose:<\/jats:title>\n                    <jats:p>\n                      Vascular Ehlers-Danlos syndrome is a rare inherited connective tissue disorder because of pathogenic variants in the\n                      <jats:italic toggle=\"yes\">COL3A1<\/jats:italic>\n                      gene. Arterial complications can affect all anatomic areas and about 25% involve supra-aortic trunks (SATs) but no systematic assessment of cervical artery lesions has been made. The primary objective was to determine an accurate prevalence of spontaneous SAT lesions in a large series of patients with vascular Ehlers-Danlos syndrome at diagnosis and during follow-up. Secondary objectives were to study their neurological consequences (transient ischemic attack or stroke) and the possible relationships with sex, genotype, ascertainment status.\n                    <\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Methods:<\/jats:title>\n                    <jats:p>A retrospective review of a monocentric cohort of patients with molecularly proven vascular Ehlers-Danlos syndrome followed in a tertiary referral center from 2000 to 2017.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Results:<\/jats:title>\n                    <jats:p>\n                      One hundred forty-four patients were analyzed, 56.9% (n=82) had SAT lesions: 64.6% females, 74.4% index-case patients. Most lesions were identified in early arterial assessment (48% at first work-up, mean age of 35.7\u00b113.0 years). Cumulative incidence of a first identification of a SAT lesion was 41.7% at 40 years old. On the complete period of survey, 183 SAT lesions (with 132 dissections and 33 aneurysms) were identified, mainly in internal carotid arteries (56.3%) and vertebral arteries (28.9%), more rarely in patients with\n                      <jats:italic toggle=\"yes\">COL3A1<\/jats:italic>\n                      null mutations (\n                      <jats:italic toggle=\"yes\">P<\/jats:italic>\n                      =0.008). Transient ischemic attack or stroke were reported in n=16 (19.5%) of the 82 patients with SAT lesions without relation with age, sex, treatment, or hypertension.\n                    <\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Conclusions:<\/jats:title>\n                    <jats:p>Cervical artery lesions are frequent and mostly asymptomatic in patients with vascular Ehlers-Danlos syndrome. Local dissections and aneurysms are the most frequent type of lesions, but transient ischemic attack or stroke seem rare.<\/jats:p>\n                  <\/jats:sec>","DOI":"10.1161\/strokeaha.120.032106","type":"journal-article","created":{"date-parts":[[2021,3,1]],"date-time":"2021-03-01T05:00:31Z","timestamp":1614574831000},"page":"1628-1635","update-policy":"https:\/\/doi.org\/10.1161\/crossmarkpolicy","source":"Crossref","is-referenced-by-count":35,"title":["Spontaneous Cervical Artery Dissection in Vascular Ehlers-Danlos Syndrome"],"prefix":"10.1161","volume":"52","author":[{"ORCID":"https:\/\/orcid.org\/0000-0003-2955-0922","authenticated-orcid":true,"given":"Salma","family":"Adham","sequence":"first","affiliation":[{"name":"AP-HP, H\u00f4pital Europ\u00e9en Georges Pompidou, D\u00e9partement de G\u00e9n\u00e9tique, Centre de R\u00e9f\u00e9rence des Maladies Vasculaires Rares, Paris, France (S.A., C.B., A.L., N.D., X.J., M.F.)."},{"name":"Universit\u00e9 de Paris, INSERM, U970 PARCC, Paris, France (S.A., C.B., A.L., X.J., M.F.)."}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Clarisse","family":"Billon","sequence":"additional","affiliation":[{"name":"AP-HP, H\u00f4pital Europ\u00e9en Georges Pompidou, D\u00e9partement de G\u00e9n\u00e9tique, Centre de R\u00e9f\u00e9rence des Maladies Vasculaires Rares, Paris, France (S.A., C.B., A.L., N.D., X.J., M.F.)."},{"name":"Universit\u00e9 de Paris, INSERM, U970 PARCC, Paris, France (S.A., C.B., A.L., X.J., M.F.)."}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-5703-3493","authenticated-orcid":true,"given":"Anne","family":"Legrand","sequence":"additional","affiliation":[{"name":"AP-HP, H\u00f4pital Europ\u00e9en Georges Pompidou, D\u00e9partement de G\u00e9n\u00e9tique, Centre de R\u00e9f\u00e9rence des Maladies Vasculaires Rares, Paris, France (S.A., C.B., A.L., N.D., X.J., M.F.)."},{"name":"Universit\u00e9 de Paris, INSERM, U970 PARCC, Paris, France (S.A., C.B., A.L., X.J., M.F.)."}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Val\u00e9rie","family":"Domigo","sequence":"additional","affiliation":[{"name":"GH Sainte-Anne, Service d\u2019imagerie morphologique et fonctionnelle, Paris, France (V.D.)."},{"name":"INSERM, U894, IMA-BRAIN, DHU NeuroVasc, Paris, France (V.D.)."}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Nicolas","family":"Denari\u00e9","sequence":"additional","affiliation":[{"name":"AP-HP, H\u00f4pital Europ\u00e9en Georges Pompidou, D\u00e9partement de G\u00e9n\u00e9tique, Centre de R\u00e9f\u00e9rence des Maladies Vasculaires Rares, Paris, France (S.A., C.B., A.L., N.D., X.J., M.F.)."}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Etienne","family":"Charpentier","sequence":"additional","affiliation":[{"name":"AP-HP, H\u00f4pital Europ\u00e9en Georges Pompidou, Service de Radiologie, Paris, France 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