RNF213 and familial isolated congenital asplenia: A different study of 352 patients showed that the variant was observed in half of ICAS patients and further demonstrated that the frequency of RNF213 protein-altering variants was higher with an increasing number of angiographic criteria observed—out of all the patients that met all three of the MMD diagnostic criteria, 75.6% had the variant, 57.7% of patients met two criteria, and 28.6% of patients met one criterion [84].