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Its rarity and kaleidoscopic presentation make it difficult to diagnose and even to suspect.<\/jats:p><\/jats:sec><jats:sec><jats:title>Objective<\/jats:title><jats:p>(1) To provide an up-to-date review on PACNS and (2) to create a preliminary screening algorithm based on clinical and radiological first-level data, useful to suspect PACNS and guide further investigations.<\/jats:p><\/jats:sec><jats:sec><jats:title>Methods<\/jats:title><jats:p>Review of PUBMED case series on PACNS, published from 2002 to 2017, collection of frequencies of clinical and neuroimaging features and calculation of median values. Classification of features as \u201cmajor\u201d or \u201cminor\u201d if frequency was higher or lower than median value. Combination of features in sets of criteria represented by all possible combinations of major and minor clinical and neuroradiological features. Application of criteria to published PACNS case reports and selection of the ones best identifying patients with definite PACNS.<\/jats:p><\/jats:sec><jats:sec><jats:title>Results<\/jats:title><jats:p>We reviewed 24 case series. \u201cMajor\u201d clinical features were headache, stroke, cognitive impairment, focal neurological deficits; \u201cminor\u201d were seizures, altered consciousness, psychiatric disorders. \u201cMajor\u201d neuroradiological features were multiple parenchymal lesions, parenchymal\/meningeal contrast enhancement, magnetic resonance angiography vessel abnormalities, vessel wall enhancement; \u201cminor\u201d were parenchymal\/subarachnoid hemorrhage, single parenchymal lesion. The selected sets of criteria able to identify all PACNS patients were (1) one clinical (major\/minor) + one major neuroradiological feature; and (2) Two clinical (\u2265 1 major) + one minor neuroradiological feature.<\/jats:p><\/jats:sec><jats:sec><jats:title>Conclusion<\/jats:title><jats:p>Our review provides a detailed clinical\/neuroradiological picture of PACNS. The proposed algorithm should be regarded as a preliminary screening tool to move the first steps towards PACNS diagnosis that needs validation.<\/jats:p><\/jats:sec>","DOI":"10.1007\/s10072-020-04583-3","type":"journal-article","created":{"date-parts":[[2020,8,10]],"date-time":"2020-08-10T10:02:56Z","timestamp":1597053776000},"page":"3135-3148","update-policy":"https:\/\/doi.org\/10.1007\/springer_crossmark_policy","source":"Crossref","is-referenced-by-count":24,"title":["\u201cWhen should primary angiitis of the central nervous system (PACNS) be suspected?\u201d: literature review and proposal of a preliminary screening algorithm"],"prefix":"10.1007","volume":"41","author":[{"ORCID":"https:\/\/orcid.org\/0000-0002-8409-4782","authenticated-orcid":false,"given":"Cristina","family":"Sarti","sequence":"first","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Antonella","family":"Picchioni","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Roberta","family":"Telese","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Marco","family":"Pasi","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Ylenia","family":"Failli","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Giovanni","family":"Pracucci","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Daniele","family":"Cammelli","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Domenico","family":"Inzitari","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"297","published-online":{"date-parts":[[2020,8,10]]},"reference":[{"key":"4583_CR1","doi-asserted-by":"crossref","first-page":"662","DOI":"10.1002\/art.10797","volume":"47","author":"R Hajj-Ali","year":"2002","unstructured":"Hajj-Ali R, Furlan A, Abou-Chebel A, Calabrese L (2002) Benign angiopathy of the central nervous system: cohort of 16 patients with clinical course and long-term followup. 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