A recent report from the US Geisinger database suggested NOTCH3 variants are associated with an increased risk of stroke and MRI features of SVD, although no association was found with dementia.8 Our findings are broadly confirmatory of those in the Geisinger cohort although we reported a slightly higher prevalence of NOTCH3 variants (0.22% vs 0.14%) and our larger sample size also allowed an association with vascular dementia to be detected. Here, NOTCH3 is linked to snowflake vitreoretinal degeneration.