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(Stroke. 1995;26:699-701.)
© 1995 American Heart Association, Inc.

Articles

Fatal Infantile Polyarteritis NodosaWith Predominant CentralNervous System Involvement

D. G. Engel, MD; S. M. Gospe, Jr, MD, PhD; K. A. Tracy, MD; W. G. Ellis, MD J. T. Lie, MD

From the Department of Neurological Surgery, Northwestern University, Evanston, Ill (D.G.E.), and the Departments of Neurology (S.M.G., W.G.E.), Pediatrics (S.M.G., K.A.T.), and Pathology (W.G.E., J.T.L.), University of California, Davis.

Correspondence to Sidney M. Gospe, Jr, MD, PhD, Child Neurology, University of California, Davis, Medical Center, 2315 Stockton Blvd, Sacramento, CA 95817.

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.

Key Words: central nervous system • infant • vasculitis