Fatal Hemorrhage in Cerebral Proliferative Angiopathy

Abstract

Cerebral proliferative angiopathy (CPA) is a rare vascular abnormality with several angiomorphological features that are distinct from brain arteriovenous malformations (AVMs). The natural history of CPAs indicates a lower risk for hemorrhage compared to brain AVMs. A 62-year-old woman presented with gait instability and dysarthria. MRI and angiography revealed a diffuse vascular network involving the tectum and cerebellar vermis with intermingled brain parenchyma. This lesion had no dominant feeder, high-flow arteriovenous shunt, flow-related aneurysm or highly dilated veins on angiogram. These findings were consistent with a diagnosis of CPA. During follow-up, she developed progressive gait instability and eye movement abnormalities, but no remarkable change was detected on the repeated MRI and angiography. Nine years later, she died of mesencephalic hemorrhage originating from the CPA. To the best of our knowledge, this is the first description of a patient with CPA who died as a result of the initial hemorrhage. It is important to recognize that a part of CPAs is aggressive and can be more vulnerable to critical hemorrhage.