To describe a case of Angiomatosis Cerebri discovered in a female patient with history of recurrent strokes. 

Angiomatosis Cerebri aka Cerebral Angiomatosis is a rare angiographic finding used to describe neovascularization characterized by thin and irregular vessels providing collateral circulation. It is a finding not well-described in literature and generally only associated with extremely rare clinical entities like Divry van Bogaert Syndrome (DBS) and Sneddon Syndrome (SS). Patients with these disease entities typically present under age 45 with recurrent strokes and have family history of recurrent strokes at a young age.

Case report and literature review.

33 year-old female with history of prior right Posterior Cerebral Artery (PCA) ischemic stroke, hypertension, and active tobacco use presented to the ED with 8-hour history of right hemi-paresis, confusion, and decreased responsiveness. CT head was normal. CTA showed focal cut-off of left peri-callosal artery and no opacification within distal left Anterior Cerebral Artery (ACA) branches. MRI was motion degraded but showed a left ACA territory ischemic infarct. Her motor weakness persisted and she remained abulic with akinetic mutism. Retinal examination by ophthalmologist was normal. Cerebral angiography revealed diffuse angiomatosis changes and neovascularization involving medium and small vessels in bilateral Middle Cerebral Artery and ACA distributions and distal left PCA distribution. Additionally, there was delayed cortical flow in all vascular distributions, as well as left A3 subocclusive thrombus with peri-callosal branch occlusion. Initial workup for infectious and inflammatory etiologies was non-revealing.

We present a young female patient with Angiomatosis Cerebri, which is a common feature of DBS and SS, but with no family history and not fitting the classic diagnostic criteria of DBS or SS, this may represent a phenotypic variant or a completely new entity.