RT Journal Article SR Electronic T1 Cerebral arteriopathy associated with Arg179His ACTA2 mutation JF Journal of NeuroInterventional Surgery JO J NeuroIntervent Surg FD BMJ Publishing Group Ltd. SP e46 OP e46 DO 10.1136/neurintsurg-2013-010997.rep VO 6 IS 9 A1 Amans, Matthew R A1 Stout, Charles A1 Fox, Christine A1 Narvid, Jared A1 Hetts, Steven W A1 Cooke, Daniel L A1 Higashida, Randall T A1 Dowd, Christopher F A1 McSwain, Hugh A1 Halbach, Van V YR 2014 UL http://jnis.bmj.com/content/6/9/e46.abstract AB ACTA2 mutations have recently been shown to cause a multisystem smooth muscle dysfunction syndrome that may result in pediatric stroke. We report a case of ACTA2 mutation in a 3-year-old girl presenting with acute ischemic stroke and provide high resolution imaging of the cerebral arteries demonstrating novel findings of multiple tiny aneurysms (particularly in the posterior circulation), as well as the more characteristic imaging phenotype of straightened and narrowed proximal intracranial vessels, dilated cervical vessels and occlusion of the M1 MCA segment without lenticulostriate collateral formation. This newly identified disease should be added to the differential diagnosis of pediatric stroke and cerebral vasculopathy. Neuroradiologists, interventionalists, surgeons and neurologists should become familiar with this rare disease and its clinical sequelae.