Cerebral autosomal dominant arteriopathy with subcortical infarcts and leukoencephalopathy (CADASIL) often starts with silent lacunar infarcts or white matter changes in the third decade of life and cognitive or other neurological signs become apparent within the next 10 years. In the following 20 years, dementia and psychiatric disturbances become more and more apparent and death frequently occurs in the sixth decade of life.^1–3

Age is an important predictor of clinical deterioration, with an odds ratio of 1.10⁴ per year starting at the age of 24. Cognitive impairment affects about 60% of CADASIL …