Determining Clinical Disease Progression in Symptomatic Patients With CADASIL

Our results suggest a gradual and heterogeneous decline in different clinical and cognitive performances over the lifetime of patients with CADASIL. Two progression profiles-one rapid and early and the other, more delayed and slower-are possible after the onset of symptoms. A major limitation of our study is that the clusters were assessed post hoc, which may induce some bias. Overall, male sex, low level of education, pathogenic variant location in EGFr 1 to 6 domains, smoking, and/or arterial hypertension may affect the clinical progression of the disease.