{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,8,26]],"date-time":"2026-08-26T15:24:41Z","timestamp":1787757881948,"version":"build-2784847793"},"reference-count":25,"publisher":"Ovid Technologies (Wolters Kluwer Health)","issue":"9","license":[{"start":{"date-parts":[[2026,9,1]],"date-time":"2026-09-01T00:00:00Z","timestamp":1788220800000},"content-version":"tdm","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0\/"}],"content-domain":{"domain":["www.ahajournals.org"],"crossmark-restriction":true},"short-container-title":["Stroke"],"published-print":{"date-parts":[[2026,9]]},"abstract":"<jats:sec>\n                    <jats:title>BACKGROUND:<\/jats:title>\n                    <jats:p>Children with Down syndrome (DS) are at high risk for moyamoya syndrome (MMS) and ischemic stroke, yet early detection strategies remain poorly defined despite the condition being surgically treatable.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>METHODS:<\/jats:title>\n                    <jats:p>We conducted a multicenter retrospective cohort study comparing children with Down syndrome\u2013associated moyamoya syndrome (DS-MMS) and MMS without DS (MMS). The primary outcome was stroke as the primary presenting symptom. Secondary outcomes included diagnostic delays, angiographic features, prediagnostic systolic blood pressure percentiles, and 1-year neurological outcomes. Multivariable models adjusted for demographic and access-related covariates.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>RESULTS:<\/jats:title>\n                    <jats:p>\n                      In total, 271 patients were identified; 198 (73.1%) met inclusion criteria and comprised the analytic cohort. Among 198 patients (77 DS-MMS; 121 MMS), DS-MMS mean age was 9.5\u00b15.2 years (50.6% female patients), and MMS mean age was 7.4\u00b13.0 years (54.5% female patients). Stroke at presentation was more common in DS-MMS (71.4% versus 20.7%; absolute difference, 50.8%), corresponding to an adjusted odds ratio of 14.50 ([95% CI, 6.65\u201331.60];\n                      <jats:italic toggle=\"yes\">P<\/jats:italic>\n                      &lt;0.001). Children with DS-MMS experienced longer delays from symptom onset to presentation and from presentation to diagnostic confirmation (both\n                      <jats:italic toggle=\"yes\">P<\/jats:italic>\n                      &lt;0.001). Posterior circulation involvement was more frequent in DS-MMS (adjusted odds ratio, 2.18 [95% CI, 1.09\u20134.37];\n                      <jats:italic toggle=\"yes\">P<\/jats:italic>\n                      =0.03), whereas angiographic severity was similar between groups. In the prediagnostic period, DS-MMS demonstrated a progressive rise in systolic blood pressure percentiles, exceeding the MMS cohort by 6 months (\n                      <jats:italic toggle=\"yes\">P<\/jats:italic>\n                      &lt;0.001). At 1 year, DS-MMS was associated with greater disability (adjusted odds ratio, 2.58 [95% CI, 1.45\u20134.57];\n                      <jats:italic toggle=\"yes\">P<\/jats:italic>\n                      &lt;0.001) and spasticity (adjusted odds ratio, 6.33 [95% CI, 3.12\u201312.83];\n                      <jats:italic toggle=\"yes\">P<\/jats:italic>\n                      &lt;0.001).\n                    <\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>CONCLUSIONS:<\/jats:title>\n                    <jats:p>DS-MMS represents a high-risk cerebrovascular phenotype characterized by delayed recognition and a markedly increased likelihood of stroke at presentation. Rising blood pressure percentiles preceding diagnosis may serve as an early physiological signal. These findings support targeted early detection strategies and a lower threshold for vascular imaging in symptomatic patients.<\/jats:p>\n                  <\/jats:sec>","DOI":"10.1161\/strokeaha.126.056969","type":"journal-article","created":{"date-parts":[[2026,7,24]],"date-time":"2026-07-24T09:01:21Z","timestamp":1784883681000},"page":"2731-2743","update-policy":"https:\/\/doi.org\/10.1161\/crossmarkpolicy","source":"Crossref","is-referenced-by-count":0,"title":["Delayed Recognition and Stroke-Predominant Presentation in Down Syndrome\u2013Associated Moyamoya Syndrome"],"prefix":"10.1161","volume":"57","author":[{"ORCID":"https:\/\/orcid.org\/0000-0002-8350-8234","authenticated-orcid":true,"given":"Jonathan D.","family":"Santoro","sequence":"first","affiliation":[{"name":"Division of Neurology, Department of Pediatrics, Children\u2019s Hospital Los Angeles, CA (J.D.S., M.S., D.N., E.H., M.C.L., S.T.O., M.M.Y.)."},{"name":"Department of Neurology, Keck School of Medicine of the University of Southern California, Los Angeles (J.D.S., D.N., E.H., M.S.R.)."}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Mackenzie","family":"Silverman","sequence":"additional","affiliation":[{"name":"Division of Neurology, Department of Pediatrics, Children\u2019s Hospital Los Angeles, CA (J.D.S., M.S., D.N., E.H., M.C.L., S.T.O., M.M.Y.)."}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-9509-6257","authenticated-orcid":true,"given":"Anthony C.","family":"Wang","sequence":"additional","affiliation":[{"name":"Department of Neurosurgery, University of California at Los Angeles (A.C.W.)."}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-4928-0782","authenticated-orcid":true,"given":"Deepti","family":"Nagesh","sequence":"additional","affiliation":[{"name":"Division of Neurology, Department of Pediatrics, Children\u2019s Hospital Los Angeles, CA (J.D.S., M.S., D.N., E.H., M.C.L., S.T.O., M.M.Y.)."},{"name":"Department of Neurology, Keck School of Medicine of the University of Southern California, Los Angeles (J.D.S., D.N., E.H., M.S.R.)."}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Eugenia","family":"Ho","sequence":"additional","affiliation":[{"name":"Division of Neurology, Department of Pediatrics, Children\u2019s Hospital Los Angeles, CA (J.D.S., M.S., D.N., E.H., M.C.L., S.T.O., M.M.Y.)."},{"name":"Department of Neurology, Keck School of Medicine of the University of Southern California, Los Angeles (J.D.S., D.N., E.H., M.S.R.)."}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Maeve C.","family":"Lucas","sequence":"additional","affiliation":[{"name":"Division of Neurology, Department of Pediatrics, Children\u2019s Hospital Los Angeles, CA (J.D.S., M.S., D.N., E.H., M.C.L., S.T.O., M.M.Y.)."}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Samuel T.","family":"Otey","sequence":"additional","affiliation":[{"name":"Division of Neurology, Department of Pediatrics, Children\u2019s Hospital Los Angeles, CA (J.D.S., M.S., D.N., E.H., M.C.L., S.T.O., M.M.Y.)."}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Mariam M.","family":"Yousuf","sequence":"additional","affiliation":[{"name":"Division of Neurology, Department of Pediatrics, Children\u2019s Hospital Los Angeles, CA (J.D.S., M.S., D.N., E.H., M.C.L., S.T.O., M.M.Y.)."}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-8821-6636","authenticated-orcid":true,"given":"Sarah","family":"Lee","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-5959-7134","authenticated-orcid":true,"given":"Elizabeth W.","family":"Mayne","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-5232-9882","authenticated-orcid":true,"given":"Brian G.","family":"Skotko","sequence":"additional","affiliation":[{"name":"Department of Pediatrics, Harvard Medical School, Boston, MA (B.G.S.)."}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-6374-1058","authenticated-orcid":true,"given":"Gary K.","family":"Steinberg","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-2640-2094","authenticated-orcid":true,"given":"Michael S.","family":"Rafii","sequence":"additional","affiliation":[{"name":"Department of Neurology, Keck School of Medicine of the University of Southern California, Los Angeles (J.D.S., D.N., E.H., M.S.R.)."},{"name":"Alzheimer\u2019s Therapeutic Research Institute, Keck School of Medicine of 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