Fatal infantile polyarteritis nodosa with predominant central nervous system involvement.

Journal Article

BACKGROUND: Infantile polyarteritis nodosa usually presents in children under 2 years of age as a multiorgan system disease with signs of congestive heart failure or renal failure. This disease and Kawasaki disease may share certain clinical and pathological features. CASE DESCRIPTION: We describe a child who first presented at 8 months of age with a febrile illness followed by a delay in motor and language development and a mild right hemiparesis. Five years later he died after developing oculomotor dysfunction, hypertension, and intracranial hemorrhage. Autopsy revealed focal segmental necrotizing vasculitis of cerebral arteries, without involvement of coronary or renal vessels. CONCLUSIONS: Although this child was evaluated on several occasions during this time period, the diagnosis was not made antemortem. The predominant central nervous system features, both clinical and pathological, together with the prolonged course are the two unique features of this child's disease that need to be emphasized.

Full Text

Duke Authors

Cited Authors

  • Engel, DG; Gospe, SM; Tracy, KA; Ellis, WG; Lie, JT

Published Date

  • April 1995

Published In

Volume / Issue

  • 26 / 4

Start / End Page

  • 699 - 701

PubMed ID

  • 7709420

Pubmed Central ID

  • 7709420

International Standard Serial Number (ISSN)

  • 0039-2499

Digital Object Identifier (DOI)

  • 10.1161/01.str.26.4.699


  • eng

Conference Location

  • United States