CNS vasculopathies: Challenging mimickers of primary angiitis of the central nervous system

Abstract

Primary angiitis of the central nervous system (CNS) is an inflammatory vasculopathy affecting the brain and spinal cord. It is a difficult diagnosis to make because of its insidious nonspecific course and its multiple mimics. This review identifies and discusses some noninfectious mimickers of primary CNS angiitis, including: reversible cerebral vasoconstriction syndrome, Sneddon's Syndrome, amyloid-beta-related angiopathy, Susac Syndrome, and neurosarcoidosis. Each condition will be reviewed in terms of epidemiology, pathology, clinical presentation, diagnostic approach, and treatment. Distinguishing these mimics from the primary angiitis of the CNS is important for proper treatment and prognosis.

Keywords: Amyloid-beta-related angiopathy; Cerebral vasculitis; Cerebral vasculopathy; Neurosarcoidosis; Primary angiitis of the central nervous system; Reversible cerebral vasoconstriction syndrome; Sneddon's syndrome; Susac syndrome.