{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2025,10,30]],"date-time":"2025-10-30T11:28:15Z","timestamp":1761823695487},"reference-count":15,"publisher":"Cambridge University Press (CUP)","issue":"4","license":[{"start":{"date-parts":[[2014,12,2]],"date-time":"2014-12-02T00:00:00Z","timestamp":1417478400000},"content-version":"unspecified","delay-in-days":1250,"URL":"https:\/\/www.cambridge.org\/core\/terms"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["Can. J. Neurol. Sci."],"published-print":{"date-parts":[[2011,7]]},"abstract":"<jats:sec id=\"S0317167100012178_abs1\"><jats:title>Objective:<\/jats:title><jats:p>Amyloid-\u03b2 (A\u03b2) related angiitis (ABRA) is a recently described clinicopathological entity characterized by cerebrovascular A\u03b2 deposition and arteritis. Cerebral A\u03b2 deposition is commonly present in cerebal amyloid angiopathy (CAA) and Alzheimer's disease (AD) but is rarely associated with inflammatory infiltration of vessel walls. Our objective is to help clarify the clinical spectrum, radiographic findings, response to treatment, and outcomes of ABRA. The neuropathological relationship between ABRA, cerebral amyloid angiopathy, and Alzheimer's disease is discussed.<\/jats:p><\/jats:sec><jats:sec id=\"S0317167100012178_abs2\"><jats:title>Methods:<\/jats:title><jats:p>We present three cases of ABRA managed at a tertiary care centre.<\/jats:p><\/jats:sec><jats:sec id=\"S0317167100012178_abs3\"><jats:title>Results:<\/jats:title><jats:p>All three patients presented with seizures and cognitive dysfunction; one had multifocal neurologic findings. Brain biopsies revealed inflammatory arteritis with A\u03b2 deposits in the vessel walls. All were treated with steroids and cyclophosphamide. Two had favorable outcomes and one stabilized but with severe residual neurologic disability.<\/jats:p><\/jats:sec><jats:sec id=\"S0317167100012178_abs4\"><jats:title>Conclusions:<\/jats:title><jats:p>ABRA is an unusual but likely under-recognized and potentially treatable disorder. As in other reported cases, our findings suggest that many patients respond favorably to immunosuppressive therapy. We believe that all biopsy specimens consistent with primary angiitis of the central nervous system (CNS) should be further examined for vascular A\u03b2 deposition.<\/jats:p><\/jats:sec>","DOI":"10.1017\/s0317167100012178","type":"journal-article","created":{"date-parts":[[2014,12,12]],"date-time":"2014-12-12T15:09:53Z","timestamp":1418396993000},"page":"626-630","source":"Crossref","is-referenced-by-count":18,"title":["Amyloid \u03b2-related Angiitis of the Central Nervous System: Report of 3 Cases"],"prefix":"10.1017","volume":"38","author":[{"given":"Heather","family":"Rigby","sequence":"first","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Alexander","family":"Easton","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Virender","family":"Bhan","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"56","published-online":{"date-parts":[[2014,12,2]]},"reference":[{"key":"S0317167100012178_ref001","doi-asserted-by":"publisher","DOI":"10.1093\/brain\/awh379"},{"key":"S0317167100012178_ref009","doi-asserted-by":"publisher","DOI":"10.1212\/01.wnl.0000260066.98681.2e"},{"key":"S0317167100012178_ref005","doi-asserted-by":"publisher","DOI":"10.1093\/rheumatology\/ken328"},{"key":"S0317167100012178_ref014","first-page":"2935","article-title":"Primary angiitis of the central nervous system and Alzheimer\u2019s disease: clinically and pathologically evident in a single patient","volume":"27","author":"Brotman","year":"2000","journal-title":"J Rheumatol"},{"key":"S0317167100012178_ref002","doi-asserted-by":"publisher","DOI":"10.1007\/BF00687163"},{"key":"S0317167100012178_ref015","doi-asserted-by":"publisher","DOI":"10.1111\/j.1365-2990.1996.tb00897.x"},{"key":"S0317167100012178_ref004","doi-asserted-by":"publisher","DOI":"10.1212\/WNL.53.4.858"},{"key":"S0317167100012178_ref010","doi-asserted-by":"publisher","DOI":"10.1007\/BF00383267"},{"key":"S0317167100012178_ref006","doi-asserted-by":"publisher","DOI":"10.1002\/ana.10810"},{"key":"S0317167100012178_ref007","doi-asserted-by":"publisher","DOI":"10.1136\/jnnp.2009.204180"},{"key":"S0317167100012178_ref003","first-page":"20","article-title":"Primary angiitis of the central nervous system","volume":"67","author":"Calabrese","year":"1988","journal-title":"Report of 8 new cases, review of the literature, and proposal for diagnostic criteria"},{"key":"S0317167100012178_ref011","doi-asserted-by":"publisher","DOI":"10.1001\/jama.1987.03400060054012"},{"key":"S0317167100012178_ref012","doi-asserted-by":"publisher","DOI":"10.1097\/00005072-199911000-00004"},{"key":"S0317167100012178_ref013","doi-asserted-by":"publisher","DOI":"10.1212\/WNL.0b013e3181ae7cd0"},{"key":"S0317167100012178_ref008","doi-asserted-by":"crossref","first-page":"1619","DOI":"10.1523\/JNEUROSCI.21-05-01619.2001","article-title":"Spontaneous hemorrhagic stroke in a mouse model of cerebral amyloid angiopathy","volume":"21","author":"Winkler","year":"2001","journal-title":"J Neurosci"}],"container-title":["Canadian Journal of Neurological Sciences \/ Journal Canadien des Sciences Neurologiques"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/www.cambridge.org\/core\/services\/aop-cambridge-core\/content\/view\/S0317167100012178","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2019,4,27]],"date-time":"2019-04-27T08:48:36Z","timestamp":1556354916000},"score":1,"resource":{"primary":{"URL":"https:\/\/www.cambridge.org\/core\/product\/identifier\/S0317167100012178\/type\/journal_article"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2011,7]]},"references-count":15,"journal-issue":{"issue":"4","published-print":{"date-parts":[[2011,7]]}},"alternative-id":["S0317167100012178"],"URL":"https:\/\/doi.org\/10.1017\/s0317167100012178","relation":{},"ISSN":["0317-1671","2057-0155"],"issn-type":[{"value":"0317-1671","type":"print"},{"value":"2057-0155","type":"electronic"}],"subject":[],"published":{"date-parts":[[2011,7]]}}}