Clinical manifestations of severe NOTCH3-SVD, or CADASIL, are mid-adult onset of ischemic stroke and transient ischemic attacks, migraine with aura, mood disorders, and cognitive decline, ultimately leading to vascular dementia.25 Neuroimaging features are progressive symmetrical white matter hyperintensities (WMHs), lacunes, cerebral microbleeds, perivascular spaces, and atrophy. This evidence concerns the gene NOTCH3 and snowflake vitreoretinal degeneration.