{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,6,16]],"date-time":"2026-06-16T18:54:09Z","timestamp":1781636049240,"version":"3.54.5"},"reference-count":24,"publisher":"Springer Science and Business Media LLC","issue":"1","license":[{"start":{"date-parts":[[2026,2,12]],"date-time":"2026-02-12T00:00:00Z","timestamp":1770854400000},"content-version":"tdm","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0"},{"start":{"date-parts":[[2026,2,12]],"date-time":"2026-02-12T00:00:00Z","timestamp":1770854400000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0"}],"funder":[{"name":"Hospital De Amor"}],"content-domain":{"domain":["link.springer.com"],"crossmark-restriction":false},"short-container-title":["Neurogenetics"],"abstract":"<jats:title>Abstract<\/jats:title>\n                  <jats:p>\n                    Inflammatory cerebral amyloid angiopathy (CAA-RI) is a variant of CAA, rarely seen in young individuals with Down syndrome (DS). We describe a 32-year-old female with DS who developed a systemic inflammatory\n                    <jats:bold>state<\/jats:bold>\n                    followed by acute neurological symptoms, including seizures and hemiparesis. Brain MRI disclosed asymmetric T2\/FLAIR hyperintensities with leptomeningeal enhancement and focal superficial siderosis on susceptibility-weighted imaging, without microbleeds; cerebrospinal fluid showed marked inflammation. After partial improvement with corticosteroids, rituximab was started due to persistent symptoms. This case highlights CAA-RI as a cause of acute decline in DS and supports the use of rituximab in refractory cases.\n                  <\/jats:p>","DOI":"10.1007\/s10048-026-00883-6","type":"journal-article","created":{"date-parts":[[2026,2,12]],"date-time":"2026-02-12T08:59:25Z","timestamp":1770886765000},"update-policy":"https:\/\/doi.org\/10.1007\/springer_crossmark_policy","source":"Crossref","is-referenced-by-count":0,"title":["Probable cerebral amyloid angiopathy - related inflammation in a 32-year-old woman with down syndrome"],"prefix":"10.1007","volume":"27","author":[{"ORCID":"https:\/\/orcid.org\/0000-0002-3302-9913","authenticated-orcid":false,"given":"Thales Pardini","family":"Fagundes","sequence":"first","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Gustavo Maximiano","family":"Alves","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Mateus Gustavo","family":"Favaro","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Joyce Yuri Silvestre","family":"Yamamoto","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Lucas Ravagnani","family":"Silva","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Lucas Giansante","family":"Abud","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Jorge Alberto Martins","family":"Pentiado Junior","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"297","published-online":{"date-parts":[[2026,2,12]]},"reference":[{"issue":"6","key":"883_CR1","doi-asserted-by":"publisher","first-page":"646","DOI":"10.1097\/cm9.0000000000001427","volume":"134","author":"J-J Wu","year":"2021","unstructured":"Wu J-J, Yao M, Ni J (2021) Cerebral amyloid angiopathy-related inflammation: current status and f Uture implications. 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Original work, not under consideration elsewhere.","order":2,"name":"Ethics","group":{"name":"EthicsHeading","label":"Ethics approval"}},{"value":"The authors declare no competing interests.","order":3,"name":"Ethics","group":{"name":"EthicsHeading","label":"Competing interests"}}],"article-number":"14"}}