Cerebral proliferative angiopathy: Clinical, angiographic features and literature review

Here we present our experience with five cerebral proliferative angiopathy (CPA) patients to better delineate the clinical and angiographic features as well as the treatment selection of this disease.

Between October 2008 and October 2012, five consecutive patients diagnosed with CPA were admitted to our department in our hospital. All the five patients received magnetic resonance imaging, digital subtraction angiography, and positron emission computed tomography (PET) to definitively confirm this disease. We also collected 15 previously published instances of CPA to analyze the characteristics of this rare entity.

As to the five patients, three were female and two were male, between the ages of 4 and 52 years with a mean age of 24.8±20.6 years. The PET results showed that perfusion was decreased over the affected hemispheres in all five patients. As to the treatment, only one patient received encephalo-duro-arterio-synangiosis (EDAS) revascularization surgery. The other four patients were conservatively observed. During the follow-up period (range 3-6 years, mean 4±1.9 years), the patient who underwent EDAS surgery manifested relief of clinical symptoms. In the conservative series, the symptoms in two patients aggravated and suffered permanent neurologic deficits.

CPA is a rare entity. Natural history has showed this disease is not stable and may progress at a certain time point. The EDAS procedure may be a treatment for CPA-related oligemia since there is currently little data and follow-up available.