• Ryan M Hess, Jeff F Zhang, Justin M Cappuzzo, Amade Bregy, and Elad I Levy.
• Department of Neurosurgery, Jacobs School of Medicine and Biomedical Sciences, University at Buffalo, Buffalo, New York, USA; Department of Neurosurgery, Gates Vascular Institute at Kaleida Health, Buffalo, New York, USA.
• World Neurosurg. 2022 Aug 1; 164: 281-289.

AbstractCerebral proliferative angiopathy (CPA) is a rare cerebrovascular pathology that presents with unique clinical features due to distinct histologic, angiographic, and pathophysiologic characteristics that separate it from classical arteriovenous malformation. The disorder is characterized by uncontrolled angiogenesis in which functional brain parenchyma is interspersed with abnormal vascular channels without a distinct nidus. Common presenting symptoms include headache, seizures, and stroke-like symptoms. Hemorrhagic presentations are rare in contrast to the typical presentations of classical arteriovenous malformation. Here, we report a young woman with a history of a suspected connective tissue disorder who presented to the emergency department with worsening headaches in a delayed fashion after experiencing minor head trauma and was found to have a left-sided subdural hematoma. Angiography confirmed a diagnosis of CPA after abnormal cortical vasculature was noted during the patient's craniotomy. A systematic review of CPA cases described in the literature was performed using the Preferred Reporting Items for Systematic Reviews and Meta-Analyses guidelines, with the findings discussed.Copyright © 2022 Elsevier Inc. All rights reserved.

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