title: Wegener's granulomatosis confined to nervous system.
abstract: Wegener's granulomatosis (WG) is a multisystemic necrotising granulomatous vasculitis of small and medium sized vessels, that primarily involves the upper and lower respiratory tracts, lung tissues and kidneys. Serum antineutrophil cytoplasmic antibodies (ANCA) are a sensitive and specific marker of WG. Whereas the peripheral nervous system is often involved in WG, central nervous system manifestations are reported only in 2-8%, and are rarely present at onset. We report on a patient with atypical neurological presentation of ANCA negative WG in whom the diagnosis was made only after a meningeal biopsy.
authors.affiliation: University of Padova, Neurosciences, Via Giustiniani 5, Padova, Italy, 35128.
authors.forename: M
authors.initials: M
authors.lastname: Lucchetta
authors.forename: M
authors.initials: M
authors.lastname: Favero
authors.forename: F
authors.initials: F
authors.lastname: Schiavon
authors.forename: A
authors.initials: A
authors.lastname: Cagnin
authors.forename: R
authors.initials: R
authors.lastname: Manara
authors.forename: A
authors.initials: A
authors.lastname: Rotilio
authors.forename: E
authors.initials: E
authors.lastname: Orvieto
authors.forename: C
authors.initials: C
authors.lastname: Giannini
authors.forename: C
authors.initials: C
authors.lastname: Briani
mesh_terms: D000368:Aged; D019268:Antibodies, Antineutrophil Cytoplasmic / Q000097:blood; D001706:Biopsy; D001921:Brain / Q000473:pathology*; D002493:Central Nervous System Diseases / Q000150:complications / Q000175:diagnosis* / Q000209:etiology*; D003680:Deglutition Disorders / Q000209:etiology; D055154:Dysphonia / Q000209:etiology; D005260:Female; D014890:Granulomatosis with Polyangiitis / Q000150:complications / Q000175:diagnosis* / Q000188:drug therapy; D006801:Humans
chemical_list: D019268:Antibodies, Antineutrophil Cytoplasmic