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Curr Rheumatol Rep. 2006 Dec;8(6):442-9.

Central nervous system vasculitis in children.

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  • 1Division of Pediatric Emergency Medicine and Rheumatology, Population Health Sciences Program, Research Institute, The Hospital for Sick Children, 555 University Avenue, Toronto, Ontario M5G 1X8, Canada.


Central nervous system (CNS) vasculitis is an increasingly recognized, often devastating inflammatory brain disease of children and adults. In primary or isolated CNS vasculitis/angiitis of childhood (cPACNS), the vascular inflammation is limited to the brain and spinal cord. Secondary CNS vasculitis occurs in a variety of conditions including infections, collagen vascular diseases, systemic vasculidities, and malignancies. Mimics of CNS vasculitis in children include dissections and noninflammatory vasculopathies. Diagnosis of primary CNS vasculitis in both adults and children is based on the Calabrese criteria. This review summarizes recent data on CNS vasculitis in children; reviews the clinical spectrum at presentation and the role of laboratory tests, neuroimaging, and brain biopsy; and discusses treatment strategies, outcome data, and overlapping conditions of cPACNS.

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