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Childhood CNS vasculitis: a treatable cause of new neurological deficit in children

Nature Clinical Practice Rheumatology volume 4, pages 460–461 (2008)Cite this article

Central nervous system (CNS) vasculitis is a devastating inflammatory brain disease that causes stroke, intractable seizures, severe cognitive deficits and death. In 1988, Calabrese, who was the first to review CNS vasculitis in adults,1 coined the term 'primary angiitis of the CNS' (PACNS) and proposed diagnostic criteria that are now widely accepted for use in adults and children.2 The diagnosis of primary CNS vasculitis is made on the basis of clinical evidence of a newly acquired focal or diffuse neurological deficit, plus angiographic or brain biopsy evidence of CNS vasculitis, in the absence of a systemic condition known to cause or mimic CNS vasculitis.2 With his groundbreaking work, Calabrese set the stage for the mandatory collaboration of adult neurologists and rheumatologists in the care of patients with suspected inflammatory brain diseases. The first small case series of childhood primary CNS vasculitis was published in 2001,3,4 and, in 2005, the first larger pediatric cohort of primary CNS vasculitis of childhood (cPACNS) was reported.5 Subsequently, pediatric subspecialties in leading centers have started to collaborate in order to recognize and treat children with primary and secondary CNS vasculitides.6

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Authors and Affiliations

  1. S Cantez is a General Pediatrician and Consultant in Rheumatology at the International Hospital in Istanbul, Turkey.,

    Serdar Cantez

  2. SM Benseler is Clinical Director of the Division of Rheumatology, a Staff Physician in the Division of Rheumatology and Pediatric Emergency Medicine, and an Associate Scientist in the Research Institute at The Hospital for Sick Children, and an Assistant Professor at the University of Toronto, Toronto, ON, Canada.,

    Susanne M Benseler

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  1. Serdar Cantez
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Correspondence to Susanne M Benseler.

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Cantez, S., Benseler, S. Childhood CNS vasculitis: a treatable cause of new neurological deficit in children. Nat Rev Rheumatol 4, 460–461 (2008). https://doi.org/10.1038/ncprheum0860

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