{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2025,10,10]],"date-time":"2025-10-10T01:13:20Z","timestamp":1760058800609,"version":"build-2065373602"},"reference-count":18,"publisher":"MDPI AG","issue":"5","license":[{"start":{"date-parts":[[2025,4,29]],"date-time":"2025-04-29T00:00:00Z","timestamp":1745884800000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0\/"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["Brain Sciences"],"abstract":"<jats:p>Background: Cerebral amyloid angiopathy-related inflammation (CAA-RI) is a rare subtype of cerebral amyloid angiopathy (CAA), which presents mostly as a subacute and reversible encephalopathy. Primary symptoms include behavioral changes and cognitive decline in the form of rapidly progressive dementia, headache, seizures, and focal neurological deficits. It can also manifest as a varied range of typical and atypical presentations. Misdiagnosis is common because it shares symptoms with other infectious, ischemic and autoimmune pathologies and there is also a significant overlap of MRI findings. Methods: Gold standard diagnosis requires brain biopsy in appropriate clinical setting, but diagnostic criteria is established for probable and possible CAA-RI using clinical symptoms and MRI findings in the absence of other inflammatory, infectious or autoimmune processes. Immunomodulatory therapy is the mainstay of treatment, with variable response. Results: We present a case series of three patients with CAA-RI highlighting disease course, neuroradiological manifestation, treatment response, and clinical outcomes. We also provide a literature review to increase insight into this rare pathology. Conclusions: Early diagnosis and prompt initiation of immunosuppressive therapy is beneficial in most cases.<\/jats:p>","DOI":"10.3390\/brainsci15050472","type":"journal-article","created":{"date-parts":[[2025,4,29]],"date-time":"2025-04-29T09:00:39Z","timestamp":1745917239000},"page":"472","update-policy":"https:\/\/doi.org\/10.3390\/mdpi_crossmark_policy","source":"Crossref","is-referenced-by-count":0,"title":["Cerebral Amyloid Angiopathy Related Inflammation: A Single-Center Case Series Analysis"],"prefix":"10.3390","volume":"15","author":[{"ORCID":"https:\/\/orcid.org\/0000-0001-7007-269X","authenticated-orcid":false,"given":"Syed Zahid","family":"Ali","sequence":"first","affiliation":[{"name":"Department of Neurology, School of Medicine, University of Louisville, 500 S Preston St., Louisville, KY 40202, USA"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-7518-9210","authenticated-orcid":false,"given":"Hanah","family":"Alley","sequence":"additional","affiliation":[{"name":"Department of Neurology, School of Medicine, University of Louisville, 500 S Preston St., Louisville, KY 40202, USA"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"James","family":"Johnson","sequence":"additional","affiliation":[{"name":"Department of Neurology, School of Medicine, University of Louisville, 500 S Preston St., Louisville, KY 40202, USA"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Harshini","family":"Sirvisetty","sequence":"additional","affiliation":[{"name":"School of Medicine, University of Louisville, 323 E Chestnut St., Louisville, KY 40202, USA"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Michael","family":"Sowell","sequence":"additional","affiliation":[{"name":"Department of Neurology, School of Medicine, University of Louisville, 500 S Preston St., Louisville, KY 40202, USA"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-3027-7276","authenticated-orcid":false,"given":"Alex","family":"Glynn","sequence":"additional","affiliation":[{"name":"Kornhauser Health Sciences Library, University of Louisville, 540 S Preston St., Louisville, KY 40202, USA"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-2699-4085","authenticated-orcid":false,"given":"Peter","family":"Hedera","sequence":"additional","affiliation":[{"name":"Department of Neurology, School of Medicine, University of Louisville, 500 S Preston St., Louisville, KY 40202, USA"}],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"1968","published-online":{"date-parts":[[2025,4,29]]},"reference":[{"key":"ref_1","doi-asserted-by":"crossref","first-page":"2039","DOI":"10.1016\/j.jstrokecerebrovasdis.2015.04.015","article-title":"Cerebral Amyloid Angiopathy Associated with Inflammation: Report of 3 Cases and Systematic Review","volume":"24","author":"Silva","year":"2015","journal-title":"J. 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