title: A case of primary systemic necrotizing vasculitis presenting primarily with neurologic involvement.
abstract: Systemic necrotizing vasculitis (SNV) is a type of vasculitis that presents with necrosis, predominantly involving large, medium-sized and small arteries. Peripheral neuropathy is a major clinical feature of the primary and secondary systemic vasculitides, and is often observed during the early phases of the disease, causing axonal neuropathy. The prevalence of central nervous system (CNS) involvement ranges from 4% to 45%. Encephalopathy, focal neurological deficits, and seizures are the most common manifestations and usually occur late during the course of SNV. In this report, we describe a 61-year-old woman with SNV who had both CNS and peripheral nervous system vasculitic involvement. We also discuss the pathophysiology of nervous system involvement in patients with SNV.
authors.affiliation: Department of Neurology, Jilin University First Hospital, Changchun 130031, China.
authors.forename: Jing-Yao
authors.initials: JY
authors.lastname: Liu
authors.forename: Ren-Sheng
authors.initials: RS
authors.lastname: Zhang
authors.forename: Chun-Kui
authors.initials: CK
authors.lastname: Zhou
mesh_terms: D001921:Brain / Q000473:pathology; D002493:Central Nervous System Diseases / Q000150:complications / Q000175:diagnosis* / Q000503:physiopathology; D003937:Diagnosis, Differential; D005260:Female; D006801:Humans; D008279:Magnetic Resonance Imaging; D008875:Middle Aged; D010523:Peripheral Nervous System Diseases / Q000150:complications / Q000175:diagnosis* / Q000503:physiopathology; D056647:Systemic Vasculitis / Q000150:complications / Q000175:diagnosis* / Q000503:physiopathology