Cerebral autosomal dominant arteriopathy with subcortical infarcts and leukoencephalopathy is an autosomal dominant cerebral small vessel disease syndrome caused by missense variants in NOTCH3, and it shares some of the clinical and pathophysiological features of HTRA1-related cerebrovascular disease,39 including early-onset stroke and vascular dementia. Here, HTRA1 is linked to Stroke.