Fatal infantile polyarteritis nodosa with predominant central nervous system involvement.

Abstract:

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.

journal_name

Stroke

journal_title

Stroke

authors

Engel DG,Gospe SM Jr,Tracy KA,Ellis WG,Lie JT

doi

10.1161/01.str.26.4.699

subject

Has Abstract

pub_date

1995-04-01 00:00:00

pages

699-701

issue

4

eissn

0039-2499

issn

1524-4628

journal_volume

26

pub_type

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