Recently, early-onset cerebrovascular diseases ascribable to small vessel disease (SVD) and Cerebral Arteriopathy autosomal Dominant with Subcortical Infarcts and Leukoencephalopathy type 1 (CADASIL)-like neuroimaging pattern have been attributed to heterozygous missense or frameshift TREX1 mutations [32,33]. This evidence concerns the gene TREX1 and CADASIL.