eISSN: 1509-572x
ISSN: 1641-4640
Folia Neuropathologica
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vol. 50

Case report
Primary angiitis of the central nervous system: 2 atypical cases

Fabio Pagni
Giuseppe Isimbaldi
Francesco Vergani
Paolo Casiraghi
Laura Marzorati
Guglielmo Migliorino
Giorgio Cattoretti

Folia Neuropathol 2012; 50 (3): 293-299
Online publish date: 2012/09/27
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The presence of an angiitis process in the central nervous system (CNS) characterizes different groups of conditions: from idiopathic pachymeningitis to lymphoproliferative disorders. In absence of specific infections, inflammatory and neoplastic diseases, the term “PACNS” (Primary Angiitis of the CNS) was proposed to indicate a peculiar vascular inflammation of unknown origin of meningeal vessels extending to the brain or spinal cord parenchyma. We report two cases of PACNS with peculiar and atypical features: the first one with a possible Epstein Barr Virus (EBV) relationship, the second one with spinal cord involvement only, treated surgically. We also hypothesize a correlation between EBV

chronic infection and possible subtypes of PACNS stressing the importance of EBER (EBV-encoded RNA) test in the routine examination of brain biopsies suspicious for PACNS.

PACNS, granulomatous angiitis, EBV-related disorders

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