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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

The hallmark of CNS vasculitis is inflammation of the cerebral blood vessel wall, which has devastating consequences. The mononuclear infiltrate thickens the layers of the vessel walls and subsequently narrows the vascular lumen, causing arterial ischemic strokes in the vascular territory.7 Activation of the vascular endothelium in the acute stage of inflammation increases the risk of platelet clot formation and further propagates the risk of ischemia in the vascular territory. The inflammation also expands into the perivascular brain tissue. Longstanding, small-vessel vasculitis is commonly found to involve substantial perivascular neuronal death, demyelination, and reactive gliosis, which can all cause considerable neurological deficits.8

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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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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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