Cerebral Proliferative Angiopathy—Description of a Rare Clinical Entity

Abstract

The cerebral proliferative angiopathy (CPA) is an uncommon vascular malformation of the brain, representing approximately 3.4% of arteriovenous malformation (AVM) cases, with complex morphology and poorly understood origin. Thus, we present the case of a woman patient aged 27 years, who performed magnetic resonance imaging (MRI) of the brain because of a headache. Extensive malformation was identified vascular right frontoparietal, with the presence of the brain tissue mixed with the vessel. CPA identified multiple feeder arteries, with the absence of nidus and slowing the flow into the venous system, confirming that it was CPA. During evaluation at another hospital she underwent partial embolization of the lesion, without success. Currently, the patient is progressing with left hemiparesis. CPA has distinct characteristics, such as large dimensions, presence of functional brain tissue intermingled with the vessels, no nidus, multiple nourishing vessels, and small veins. It does not present well-defined nidus or flow-related aneurysms. These characteristics classify it as a disease itself within the group of AVMs. This case clinically presents with seizures, refractory headaches, focal neurologic deficits, and low risk of bleeding. Alternatives for curative treatment imply significant neurologic deficits and the most accepted options aimed at easing the symptoms and the partial embolization alternative that is best associated with risks and benefits.