A Systematic Review of Neurovascular Complications in Patients with Pseudoxanthoma Elasticum

Background: Pseudoxanthoma elasticum (PXE) is a rare, hereditary connective tissue disorder characterized by ectopic calcification of multiple organ systems. Cardiovascular complications secondary to accelerated atherosclerosis are well-documented in published literature; however, there is a paucity of studies that reviews serious neurovascular complications including CVA(cerebral vascular accident), TIA(Transient ischemic attack), and intracranial aneurysm in PXE patients. Objective: To review current literature reporting cases of serious ischemic and hemorrhagic neurovascular complications in PXE patients as of September 2020 and reexamine current guidelines on antiplatelet therapy in PXE patients Methods: A search of the PubMed database limited to English language case reports using the key words “pseudoxanthoma elasticum”, “neuro*”, “cerebr*”, “CVA”, “TIA”, and “aneurysm” was performed. Results: A total of 67 cases of cerebral disease were reported; ranging from ages 2-71 years from 12 countries. The most common neurovascular complication seen was ischemic stroke (68.7%) followed by TIA (20.8%), intracranial aneurysm (6.0%) and lastly hemorrhagic stroke (4.5%). Conclusions: PXE patients have a greater incidence of ischemic strokes compared to the general population. Clinicians should thus monitor this patient population for signs of neurovascular complications and carefully weigh the benefits of antiplatelet therapy in patients with known neurovascular disease against the risk of bleeding.