{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,11,1]],"date-time":"2026-11-01T22:37:11Z","timestamp":1793572631945,"version":"4.5.0"},"reference-count":17,"publisher":"Walter de Gruyter GmbH","license":[{"start":{"date-parts":[[2026,8,6]],"date-time":"2026-08-06T00:00:00Z","timestamp":1785974400000},"content-version":"unspecified","delay-in-days":0,"URL":"http:\/\/creativecommons.org\/licenses\/by-nc-nd\/3.0"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":[],"abstract":"<jats:title>Abstract<\/jats:title>\n                  <jats:p>Primary angiitis of the central nervous system is an extremely rare diagnosis, with an incidence of 2.4 cases per 1,000,000 person-years. The exact cause of PACNS remains unknown, and typically cases present with a long prodrome. It is a diagnosis of exclusion, and various infectious, autoimmune, and malignant processes must be excluded prior to final diagnosis. Given the rarity of this condition, there is often a delay in diagnosis that can range from 6 to 23 months. It is imperative that all clinical providers be aware of this diagnosis when managing patients who present with insidious and non-specific neurological symptoms. We present a case of PACNS that responded well to a therapeutic regimen of high-dose steroids and cyclophosphamide.<\/jats:p>","DOI":"10.2478\/rjim-2026-0017","type":"journal-article","created":{"date-parts":[[2026,8,6]],"date-time":"2026-08-06T05:58:09Z","timestamp":1785995889000},"source":"Crossref","is-referenced-by-count":1,"title":["Primary Angiitis of the Central Nervous System: A Medical Mystery"],"prefix":"10.2478","author":[{"given":"Seby","family":"Robert","sequence":"first","affiliation":[{"name":"Department of Internal Medicine , Mayo Clinic Florida , Jacksonville , Florida , United States of America ."}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Reddy","family":"Deepica","sequence":"additional","affiliation":[{"name":"Department of General Internal Medicine Executive Health Program , Mayo Clinic Florida , United States of America"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Ketty","family":"Arpitha","sequence":"additional","affiliation":[{"name":"Department of Rheumatology , Mayo Clinic Florida , United States of America"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Claire","family":"Wilson","sequence":"additional","affiliation":[{"name":"Department of Rheumatology , Mayo Clinic Florida , United States of America"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"374","published-online":{"date-parts":[[2026,8,6]]},"reference":[{"key":"2026081913080223293_j_rjim-2026-0017_ref_001","doi-asserted-by":"crossref","unstructured":"SALVARANI C. et el. Primary Central Nervous System Vasculitis: Analysis of 101 Patients. Annals of Neurology. 2007;62 (5):442\u2013451.","DOI":"10.1002\/ana.21226"},{"key":"2026081913080223293_j_rjim-2026-0017_ref_002","doi-asserted-by":"crossref","unstructured":"KRAEMER M., BERLIT P. Primary central nervous system vasculitis - An update on diagnosis, differential diagnosis and treatment. J Neurol Sci. 2021;424:117422.","DOI":"10.1016\/j.jns.2021.117422"},{"key":"2026081913080223293_j_rjim-2026-0017_ref_003","doi-asserted-by":"crossref","unstructured":"CHANG H., GAO M., ZHANG J., CAO W., GUO S., WANG P., CHENG G., ZHAO H. Retrospective Analysis of 28 Cases Confirmed for Primary Angiitis of the Central Nervous System by Biopsy. J Stroke Cerebrovasc Dis. 2020;29(12):105400.","DOI":"10.1016\/j.jstrokecerebrovasdis.2020.105400"},{"key":"2026081913080223293_j_rjim-2026-0017_ref_004","doi-asserted-by":"crossref","unstructured":"AGARWAL A., SHARMA J., SRIVASTAVA M., SHARMA M., BHATIA R., DASH D., GOYAL V. et al. Primary CNS vasculitis (PCNSV): a cohort study. Sci Rep. 2022;12(1):13494.","DOI":"10.1038\/s41598-022-17869-7"},{"key":"2026081913080223293_j_rjim-2026-0017_ref_005","unstructured":"POMPER M. et al. CNS vasculitis in Autoimmune Disease: MR imaging Findings and Correlation with Angiography. Am J Neuroradiol. 1999;20 (1):75\u201385."},{"key":"2026081913080223293_j_rjim-2026-0017_ref_006","doi-asserted-by":"crossref","unstructured":"GIANNINI C. et al. Primary Central Nervous System Vasculitis: Pathology and Mechanisms. 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Primary Angiitis of the Central Nervous System. Autoimmun Rev. 2013;12:463\u2013466.","DOI":"10.1016\/j.autrev.2012.08.004"},{"key":"2026081913080223293_j_rjim-2026-0017_ref_011","doi-asserted-by":"crossref","unstructured":"De Boysson H., ZUBER M., NAGGARA O., NEAU J., GRAY F., BOUSSER M., CRASSARD I., TOUZE E., COURAUD P. et al. Primary Angiitis of the Central Nervous System: Description of the First Fifty-Two Adults Enrolled in the French Cohort of Patients With Primary Vasculitis of the Central Nervous System. Arthritis Rheumatol. 2014;66:1315\u20131326.","DOI":"10.1002\/art.38340"},{"key":"2026081913080223293_j_rjim-2026-0017_ref_012","doi-asserted-by":"crossref","unstructured":"SALVARANI C. et al. Adult Primary Central Nervous System Vasculitis Treatment and Course: Analysis of One Hundred Sixty-Three Patients. 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