ACTA2 and spondylometaphyseal dysplasia, Sedaghatian type: However, the reported familial case demonstrates that genetic screening for ACTA2 variants should also be considered in Moyamoya-like arteriopathy with a straight course of intracranial arteries, even in the absence of a family history of TAAD or features of SMDS, and even if a basal collateral network is found.