Cerebral Proliferative Angiopathy: Imaging Spectrum and Diagnostic Insights

Area of Science:

• Neurology
• Radiology
• Vascular Medicine

Background:

• Cerebral proliferative angiopathy (CPA) is a rare cerebrovascular malformation.
• CPA differs from typical arteriovenous malformations (AVMs) due to its diffuse vascular network, lack of a compact nidus, and trans-dural supply.

Purpose of the Study:

• To summarize the imaging spectrum of CPA through a comprehensive review of published studies.
• To differentiate CPA from AVMs and other mimics using multimodal imaging.

Main Methods:

• Comprehensive literature review of 75 studies involving 153 CPA patients.
• Analysis of imaging findings across CT, MRI, perfusion/metabolic imaging, and digital subtraction angiography.

Main Results:

• CPA typically presents in adolescents/young adults, predominantly supratentorial, with dispersed cortical flow voids and enhancing channels.
• Imaging reveals hypoperfusion, impaired cerebrovascular reserve, absence of dominant feeders, and trans-dural collaterals.
• CPA is progressive, carrying risks of infarction, hemorrhage, and neurological deficits.

Conclusions:

• Multimodal imaging is crucial for distinguishing CPA from AVMs and other conditions.
• Accurate diagnosis of CPA is essential as management strategies differ significantly.
• Standardized reporting may improve diagnostic criteria and patient outcomes.