This patient was a 6-year-old female with history of stroke and TIAs. Images demonstrated characteristic cerebral vascular anomalies for this genotype, which was confirmed on genetic testing. ACTA2 mutation results in multisystemic smooth muscle dysfunction syndrome, which can result in cerebral arteriopathy and present as a pediatric stroke. An important distinguishing feature from moyamoya vasculopathy is the lack of lenticulostriate collaterals in this condition. 

The patient underwent an encephaloduroarteriosynangiosis (EDAS) to augment blood flow to the cerebrum and has improved clinically.