Cerebral proliferative angiopathy (CPA) is a rare cerebrovascular malformation that differs fundamentally from classic arteriovenous malformations (AVMs). Unlike AVMs, CPA is characterized by a diffuse vascular network intermingled with normal brain parenchyma, absence of a compact nidus or dominant feeders, and transdural supply. We conducted a comprehensive review of 75 published studies comprising 153 patients to summarize the full imaging spectrum. CPA most commonly presents in adolescence or young adulthood, with a slight female predominance, and generally involves the supratentorial brain, spanning multiple lobes. On CT and MRI, CPA typically demonstrates dispersed cortical flow voids and serpiginous enhancing vascular channels, with secondary findings like ischemia, cortical atrophy, calcifications, hemorrhage, and variable venous drainage patterns. Perfusion and metabolic imaging consistently reveal widespread hypoperfusion and impaired cerebrovascular reserve, supporting its ischemia-driven pathogenesis. DSA shows an absence of dominant feeders, proximal arterial stenoses, frequent transdural collaterals, rarely flow-related aneurysms, and modest venous enlargement. It can occasionally have a progressive nature, with risks of delayed infarction, hemorrhage, and neurologic deficits. Multimodal imaging is essential for distinguishing this under-recognized yet clinically important entity from AVMs and other mimics, because management differs substantially. A standardized reporting framework may help refine diagnostic criteria and improve clinical outcomes.
