The current clinical picture of cerebral proliferative angiopathy: systematic review

Cerebral proliferative angiopathy (CPA) is a rare subset of arteriovenous malformations (AVM). It has unique clinical presentation, angiographic characteristics, and pathophysiology which often brings challenges for the treatment. We aimed to define its epidemiology, pathophysiology are unknown, and best management strategies.

A systematic review was conducted according to the PRISMA guidelines. MEDLINE was searched for articles regarding CPA. Extracted data included epidemiological, clinical, and angiographical characteristics, treatment, and outcomes. Treatment was classified as conservative, radiosurgery, endovascular, decompression, and indirect vascularization. A meta-analytical approach was employed for description of the data as study-size adjusted percentages or weighted means, as appropriate.

Thirty-three studies were analyzed, rendering a total 95 cases-half of which came from a single study. Patients were predominantly young (mean 23 years old) and female (60.0%) presenting with headaches (44.9%), seizures (37.1%), or transient ischemic attacks (33.7%). Hemorrhage was present in 18.0%, but rebleeding rates were as high as 67%. The majority of nidus were > 6 cm (52.5%) with hemispheric extension (73.0%). Capillary angioectatic appearance (85.7%), transdural supply (62.5%), and deep venous drainage (73.0%) were also frequent features. Most patients were treated conservatively (54.4%), followed by endovascular (34.2%). Indirect vascularization and radiosurgery were attempted in five and two patients, respectively. Mean follow-up was 110.8 patient-years. Neurological status improved in 50.7%, remained stable in 40.2%, and worsened in 9.0%.

Conservative and endovascular treatments seem adequate interventions, despite limited evidence. Complementary techniques can be used in patients throughout disease history, according to symptom-based, individualized approach. More studies are required for choosing interventions based on reliable long-term results.