{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,4,29]],"date-time":"2026-04-29T04:21:17Z","timestamp":1777436477042,"version":"3.51.4"},"reference-count":28,"publisher":"Frontiers Media SA","license":[{"start":{"date-parts":[[2024,4,8]],"date-time":"2024-04-08T00:00:00Z","timestamp":1712534400000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0\/"}],"content-domain":{"domain":["frontiersin.org"],"crossmark-restriction":true},"short-container-title":["Front. Neurol."],"abstract":"<jats:sec><jats:title>Background and objectives<\/jats:title><jats:p>Primary CNS vasculitis (PCNSV) is a rare inflammatory disorder that affects the blood vessels of the central nervous system (CNS). We aimed to analyze the neurological presentations, clinical follow-up, and long-term outcomes of patients with primary central nervous system vasculitis.<\/jats:p><\/jats:sec><jats:sec><jats:title>Methods<\/jats:title><jats:p>We conducted a retrospective analysis of medical records to assess the neurological presentation, rate of remission, and functional status at the last follow-up in patients with primary central nervous system vasculitis seen in our center in the last 13\u2009years (2010\u20132023).<\/jats:p><\/jats:sec><jats:sec><jats:title>Results<\/jats:title><jats:p>We identified five patients, whose median age at symptom onset was 31\u2009years (range: 15\u201341\u2009years), including four male individuals (80%) from Muslim Arab (<jats:italic>n<\/jats:italic>\u2009=\u20094) and Ashkenazi Jewish (<jats:italic>n<\/jats:italic>\u2009=\u20091) backgrounds. Symptoms persisted for a median of 36\u2009weeks (range: 3\u2009weeks to 4\u2009years) before diagnosis, with one case exceeding 3\u2009years. Follow-up lasted a median of 56\u2009months (range: 20\u2013161\u2009months). Clinical symptoms varied, presenting unilateral weaknesses (<jats:italic>n<\/jats:italic>\u2009=\u20092), cognitive and gait abnormality (<jats:italic>n<\/jats:italic>\u2009=\u20091), headaches (<jats:italic>n<\/jats:italic>\u2009=\u20091), and epileptic seizures (<jats:italic>n<\/jats:italic>\u2009=\u20091). MRI scans revealed abnormalities in the basal ganglia, corona radiata, parietal, and frontal lobes, showing hemorrhage, vasogenic edema, restricted diffusion, and enhancement post-gadolinium. All patients reported progressive holocephalic headaches and cognitive changes with overall progressive symptoms. Initial neurological examinations revealed abnormalities in all patients and included one or more of the following: cognitive or visual impairment (<jats:italic>n<\/jats:italic>\u2009=\u20092), seizures (<jats:italic>n<\/jats:italic>\u2009=\u20091), and unilateral UMN signs (<jats:italic>n<\/jats:italic>\u2009=\u20092) at the initial neurological examination, all but one patient required walking aids including (cane 2, wheelchair, bedridden 1). Patients were stable (<jats:italic>n<\/jats:italic>\u2009=\u20092), deteriorated (<jats:italic>n<\/jats:italic>\u2009=\u20091), or improved (<jats:italic>n<\/jats:italic>\u2009=\u20092). Following treatment, two patients still required ambulatory aids, with one using a cane and the other using a wheelchair, while the remaining three did not require any ambulatory aids.<\/jats:p><\/jats:sec><jats:sec><jats:title>Discussion<\/jats:title><jats:p>The study on PCNSV highlights varied symptoms and diagnostic challenges, including delayed diagnosis and a spectrum of neurological issues from cognitive impairments to seizures. Brain biopsies showed lymphocytic infiltration, thrombi, and necrosis. Immunotherapy significantly improved clinical and radiological outcomes. Over 56\u2009months of follow-up, outcomes varied from stability and deterioration to improvement.<\/jats:p><\/jats:sec>","DOI":"10.3389\/fneur.2024.1363985","type":"journal-article","created":{"date-parts":[[2024,4,8]],"date-time":"2024-04-08T14:53:30Z","timestamp":1712588010000},"update-policy":"https:\/\/doi.org\/10.3389\/crossmark-policy","source":"Crossref","is-referenced-by-count":5,"title":["Primary CNS vasculitis: insights into clinical, neuropathological, and neuroradiological characteristics"],"prefix":"10.3389","volume":"15","author":[{"given":"Tahani Saker","family":"Sheikh","sequence":"first","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Ayal","family":"Rozenberg","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Goni","family":"Merhav","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Alla","family":"Shifrin","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Polina","family":"Stein","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Shahar","family":"Shelly","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"1965","published-online":{"date-parts":[[2024,4,8]]},"reference":[{"key":"ref1","doi-asserted-by":"publisher","first-page":"29","DOI":"10.1177\/1756285609347123","article-title":"Diagnosis and treatment of cerebral vasculitis","volume":"3","author":"Berlit","year":"2010","journal-title":"Ther Adv Neurol Disord"},{"key":"ref2","doi-asserted-by":"publisher","first-page":"442","DOI":"10.1002\/ana.21226","article-title":"Primary central nervous system vasculitis: analysis of 101 patients","volume":"62","author":"Salvarani","year":"2007","journal-title":"Ann Neurol"},{"key":"ref3","doi-asserted-by":"publisher","first-page":"704","DOI":"10.1001\/archneurol.2009.76","article-title":"Primary angiitis of the central nervous system","volume":"66","author":"Birnbaum","year":"2009","journal-title":"Arch Neurol"},{"key":"ref4","doi-asserted-by":"publisher","first-page":"1189","DOI":"10.1002\/1529-0131(199707)40:7<1189::AID-ART2>3.0.CO;2-4","article-title":"Vasculitis in the central nervous system","volume":"40","author":"Calabrese","year":"1997","journal-title":"Arthritis Rheum"},{"key":"ref5","doi-asserted-by":"publisher","first-page":"561","DOI":"10.1016\/S1474-4422(11)70081-3","article-title":"Primary angiitis of the CNS","volume":"10","author":"Hajj-Ali","year":"2011","journal-title":"Lancet Neurol"},{"key":"ref6","doi-asserted-by":"publisher","first-page":"767","DOI":"10.1016\/S0140-6736(12)60069-5","article-title":"Adult primary central nervous system vasculitis","volume":"380","author":"Salvarani","year":"2012","journal-title":"Lancet"},{"key":"ref7","first-page":"1779","article-title":"Current viewpoints on the risks of various types of fat distribution","volume":"134","author":"Seidell","year":"1990","journal-title":"Ned Tijdschr Geneeskd"},{"key":"ref8","doi-asserted-by":"publisher","first-page":"e738","DOI":"10.1097\/MD.0000000000000738","article-title":"An update of the Mayo Clinic cohort of patients with adult primary central nervous system vasculitis: description of 163 patients","volume":"94","author":"Salvarani","year":"2015","journal-title":"Medicine (Baltimore)"},{"key":"ref9","first-page":"75","article-title":"CNS vasculitis in autoimmune disease: MR imaging findings and correlation with angiography","volume":"20","author":"Pomper","year":"1999","journal-title":"AJNR Am J Neuroradiol"},{"key":"ref10","doi-asserted-by":"publisher","first-page":"1221","DOI":"10.1212\/WNL.44.7.1221","article-title":"Serial angiography in isolated angiitis of the central nervous system","volume":"44","author":"Alhalabi","year":"1994","journal-title":"Neurology"},{"key":"ref11","doi-asserted-by":"publisher","first-page":"827","DOI":"10.3892\/mco.2016.784","article-title":"A rare case of tumor-mimicking primary angiitis of the central nervous system","volume":"4","author":"Sun","year":"2016","journal-title":"Mol Clin Oncol"},{"key":"ref12","doi-asserted-by":"publisher","first-page":"20","DOI":"10.1097\/00005792-198801000-00002","article-title":"Primary angiitis of the central nervous system. 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