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Papers of the Week

2022 May 27

World Neurosurg

Cerebral Proliferative Angiopathy Presenting as Subdural Hematoma: A Case Report and Systematic Literature Review.


Hess RM, Zhang JF, Cappuzzo JM, Bregy A, Levy EI
World Neurosurg. 2022 May 27.
PMID: 35636668.


Cerebral proliferative angiopathy (CPA) is a rare cerebrovascular pathology that presents with unique clinical features due to distinct histological, angiographic, and pathophysiologic characteristics that separate it from classical arteriovenous malformation (AVM). 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 AVM. 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.