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Neurol Clin. 2015 May;33(2):515-26. doi: 10.1016/j.ncl.2014.12.004. Epub 2015 Feb 2.

Primary angiitis of the central nervous system: diagnostic criteria.

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Department of Neurology, University of North Carolina at Chapel Hill, Room 2131, CB #7025, 170 Manning Drive, Chapel Hill, NC 27514, USA. Electronic address:


Primary angiitis of the central nervous system (PACNS) is a vasculitis of small arteries and veins of unknown cause restricted to the central nervous system (CNS), and controversy and disagreement exist over the means to establish the diagnosis. Cerebral arteriography, cerebrospinal fluid examination, and MRI singly or in combination do not have sufficiently demonstrated positive predictive value to establish the diagnosis. An alternative diagnosis is established at biopsy in 35% of cases. Histologic confirmation is required for the diagnosis of PACNS. Patients without histologic confirmation should not be included in case reports, case series, or reviews.


CNS angiitis; CNS vasculitis; Primary angiitis of the central nervous system

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