As the eponym suggests, CARASIL (cerebral autosomal recessive arteriopathy with subcortical infarcts and leukoencephalopathy) is similar to CADASIL from a clinical and neuroradiological point of view, although it is inherited in an autosomal recessive manner and caused by the mutations in the high-temperature requirement A serine peptidase 1 (HTRA1) gene. Here, HTRA1 is linked to CARASIL.