Cerebral Proliferative Angiopathy: Imaging Spectrum and Diagnostic Insights

Cerebral proliferative angiopathy (CPA) is a rare cerebrovascular malformation that differs fundamentally from classical 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 trans-dural supply. We conducted a comprehensive review of 75 published studies comprising 153 patients to summarize the full imaging spectrum. It 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. Digital subtraction angiography shows absence of dominant feeders, proximal arterial stenoses, frequent trans-dural collaterals, rarely flow-related aneurysms and modest venous enlargement. It has a progressive nature, with risks of delayed infarction, hemorrhage, and neurological deficits.Multimodal imaging is essential for distinguishing this underrecognized yet clinically important entity from AVMs and other mimics, as management differs substantially. Standardized reporting frameworks may help refine diagnostic criteria and improve clinical outcomes.ABBREVIATIONS: CPA＝ Cerebral Proliferative Angiopathy; AVM＝ Arterio-venous malformation; VEGF＝ Vascular Endothelial Growth Factor.