Cerebral proliferative angiopathy in a three-year-old

Abstract

Background: Cerebral proliferative angiopathy (CPA) is a rare vascular malformation with intervening normal brain tissue interspersed among abnormal vascular channels. There are 77 reported cases, the youngest being 9 years old, with persistent symptoms and recurring symptoms being rare. Methods: A three-year-old girl with CPA is described and compared to the literature. Results: A previously healthy girl with early left-handedness and a left forehead nevus flammeus presented with sudden onset of right arm and leg weakness, along with abrupt speech arrest and right homonymous hemianopia. Head CT Angiogram and MRI revealed an abnormal vascular network with densely packed, moderately enlarged vessels arising within the white matter with no dominant feeding vessel and both old and acute infarcts in the left hemisphere. Eye exam was unremarkable. The clinical and radiologic features were most consistent with a diagnosis of CPA. Her visual deficits and motor symptoms persisted, and she had a recurrent event shortly after. Conclusions: This is the youngest reported case of CPA, with novel features including radiologic evidence of previous infarcts, clinical recurrence of symptoms, and permanent deficits. This case demonstrates the need for further research into the surveillance and management of this rare entity, possibly unique in young children