Recurrent stroke in a child with incontinentia pigmenti.

We report a child with genetically proven incontinentia pigmenti who had bilateral strokes at 5 days of age, and was subsequently found to have recurrent acute strokes on magnetic resonance imaging both at 10 days and 3 months of age. Brain magnetic resonance imaging at 5 days of age showed multiple areas of restricted diffusion throughout both hemispheres. The child was started on antiplatelet therapy after a second stroke was noted on magnetic resonance imaging at 10 days of age. Despite this treatment, she had a third punctate infarct on magnetic resonance imaging 3 months later. A magnetic resonance imaging at 10 months of age showed maturation of the old infarcts but no new strokes. The purpose of this article is to describe a unique presentation of recurrent stroke secondary to incontinentia pigmenti and to highlight the need for a better understanding of the pathophysiology of incontinentia pigmenti to develop appropriate treatment of stroke in incontinentia pigmenti.