University of Pennsylvania
Primary central nervous system vasculitis (CNSV) is a potentially fatal, single-organ vasculitis that often involves a spectrum of neurologic complications, including strokes, cognitive and speech impairment, visual loss, dementia, and encephalopathy. The purpose of this study is to establish a research cohort to investigate the disease process, treatments, and patient outcomes in CNSV.
Primary central nervous system vasculitis (CNSV) is a potentially fatal, single-organ vasculitis that often involves a spectrum of neurologic complications, including strokes, cognitive and speech impairment, visual loss, dementia, and encephalopathy. Originally described in the early 1950s, research in the disease has been slow to progress, largely limited by the rarity of the disease and the lack of biologic materials for basic and translational investigations. To date, the bulk of research in the field has been clinical, largely consisting of retrospective case series, predominately from si…
Inclusion Criteria: i. Diagnostic criteria for CNSV 1. The presence of an acquired and otherwise unexplained neurologic deficit 2. The presence of either classic angiographic or histopathologic features of angiitis in the CNS 3. No evidence of systemic vasculitis or any condition that could cause the angiographic or pathologic features ii. ≥18 years of age Exclusion Criteria: i. Unwilling to sign informed consent.
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