Cerebral proliferative angiopathy: 14 cases in a single-center and a systematic review

Background: Cerebral Proliferative Angiopathy (CPA), previously called diffuse nidus type or holohemispheric giant cerebral AVM, is a recently identified clinical entity with less than a hundred reported cases. Studies estimate a CPA incidence of 3.4–4.5% among all AVMs.

Purposes: The authors described cases of CPA in a single health care center and performed a systematic review of all reported cases.

Methods: In a retrospective analysis 550 cases of brain arteriovenous malformation databank from a reference center since 2009. We found 14 cases (2.54%) with clinical and angioarchitectural findings harmonious to CPA. CPA was determined based on the definitions proposed on the literature recently. The patient’s clinical and hospital charts were reviewed by an experienced interventional neuroradiologist whom classified the cases based on angiomorphological fidings, based on cerebral angiogram and magnetic resonance imaging.

Results: We found a high prevalence of CPA in young adults with median age of 28 years old (range 9–56 years), no gender predominance was notice. Chronic headache was the most common synptom (12/14), also three cases developed a hemorrhagic event and 6 showed a stroke like presentation. None of the patients was managed through endovascular procedure. Only one patient was sent to surgery caused hydrocephalus gradual development.

Conclusions: Neurovascular surgeons and neuroradiologists need to understand this typical neurovascular entity cause its diffuse angiogenetic behaviour is presumably related to a conservative medical management over its natural history. Despite we known its “benign” evolution, CPA can present complications that require an aggressive medical care strategy. At last the systematic review showed up that stroke- like events are the most common presentation and until now no ideal treatment was discovered to cure the patient. Also, we presented offer a score based on this systematic review with our cases to guide specialists to distint CPA from a classic AVM.

No conflict of interest has been declared by the author(s).