title: Central nervous system vasculitis in adults: An update.
abstract: Primary central nervous system vasculitis (PCNSV) is a challenging diagnosis due to broad clinical manifestations and variable specificity and sensitivity of laboratory and imaging diagnostic tools. Differential diagnosis includes reversible cerebral vasoconstriction syndrome (RCVS), secondary vasculitis of the CNS and other noninflammatory vasculopathies. Brain biopsy is essential for definitive diagnosis and to exclude mimickers. Recent data show that data large-vessel PCNSV present worse prognosis when compared to small-vessel PCNSV. Herein we review diagnosis and management of PCNSV, secondary vasculitis of CNS and RCVS.
authors.affiliation: General Neurology Division, Department of Neurology and Neurosurgery, Universidade Federal de São Paulo, São Paulo, Brazil; Hospital Israelita Albert Einstein, São Paulo, Brazil.
authors.forename: Lívia Almeida
authors.initials: LA
authors.lastname: Dutra
authors.affiliation: Rheumatology Division, Universidade Federal de São Paulo, São Paulo, Brazil.
authors.forename: Alexandre Wagner Silva
authors.initials: AW
authors.lastname: de Souza
authors.affiliation: Hospital Israelita Albert Einstein, São Paulo, Brazil.
authors.forename: Gabriela
authors.initials: G
authors.lastname: Grinberg-Dias
authors.affiliation: General Neurology Division, Department of Neurology and Neurosurgery, Universidade Federal de São Paulo, São Paulo, Brazil.
authors.forename: Orlando Graziani Povoas
authors.initials: OG
authors.lastname: Barsottini
authors.affiliation: Rheumatology Division, Universidade Estadual de Campinas, Campinas, São Paulo, Brazil. Electronic address: appenzellersimone@gmail.com.
authors.forename: Simone
authors.initials: S
authors.lastname: Appenzeller
mesh_terms: D000328:Adult; D003937:Diagnosis, Differential; D006801:Humans; D020293:Vasculitis, Central Nervous System / Q000175:diagnosis*