Diffuse proliferative cerebral angiopathy: a case report and literature review on a very rare and misdiagnosed entity

Abstract

Abstract Diffuse proliferative cerebral angiopathy (DPCA) is an uncommon type of cerebral vascular malformation, mostly diagnosed in young females. It is characteristically different from other cerebral arteriovenous malformations and can be differentiated by its peculiar imaging findings. A nidus of normal brain parenchyma is present between the abnormal vascular channels. Therefore, it is crucial to diagnose it as a separate entity because unnecessary treatment of DPCA increases the risk of damage to the normal parenchyma leading to neurological deficits. Here we describe a case of a 60-year-old male who presented with severe neurological deficits and was later diagnosed with DPCA. He was managed conservatively with antiepileptics and almost completely recovered to normal within 2 weeks. A rare case of DPCA confused with other hemorrhagic disorders is discussed here. Rare cases are often overlooked. Correct diagnosis helps to prevent tragic consequences.