We included 20 publications from 298 identified for screening and extracted data on 268 NOTCH3 cysteine-sparing mutations individuals with clinical suspicion of CADASIL (Supplementary Table S3) [4,12,13,14,15,16,17,18,19,20,21,22,23,24,25,26,27,28,29,30]. The gene discussed is NOTCH3; the disease is CADASIL.