title: Primary angiitis of the central nervous system: 2 atypical cases.
abstract: 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.
authors.affiliation: Department of Pathology, Università Milano Bicocca, San Gerardo Hospital, Monza, Italy. fabio.pagni@unimib.it
authors.forename: Fabio
authors.initials: F
authors.lastname: Pagni
authors.forename: Giuseppe
authors.initials: G
authors.lastname: Isimbaldi
authors.forename: Francesco
authors.initials: F
authors.lastname: Vergani
authors.forename: Paolo
authors.initials: P
authors.lastname: Casiraghi
authors.forename: Laura
authors.initials: L
authors.lastname: Marzorati
authors.forename: Guglielmo
authors.initials: G
authors.lastname: Migliorino
authors.forename: Giorgio
authors.initials: G
authors.lastname: Cattoretti
mesh_terms: D000328:Adult; D017809:Fatal Outcome; D005260:Female; D006801:Humans; D020293:Vasculitis, Central Nervous System / Q000175:diagnosis* / Q000601:surgery*; D055815:Young Adult