CADASIL (cerebral autosomal dominant arteriopathy with subcortical infarcts and leukoencephalopathy) is an inherited systemic arterial vessel disease caused by mutations in the NOTCH3 gene, located on the 19p13 chromosome [1,2], which encodes a transmembrane receptor that is mainly expressed in the smooth muscle cells of blood vessels and pericytes [3]. Here, NOTCH3 is linked to cerebral arteriopathy with subcortical infarcts and leukoencephalopathy.