Determinants of Cerebral Microbleed Presence and Burden in CADASIL

Background (2) MRI markers; and (3) clinical variables. Results Beyond the consistent effect of the MRI sequence, in Model 1, CMB presence was associated with age, male sex and hypertension, while counts were associated only with age. In Model 2, CMB presence was associated with age, hypertension, and lacune count; counts were higher with WMH and antiplatelet use, and lower with EGFr 1–6 mutations and BPF. In Model 3, CMB presence was associated with age, hypertension, stroke history and gait disturbances; counts were associated with education, lacune count, MMSE ≤24, and hypercholesterolemia measured at the study visit. Conclusions Hurdle modeling distinguishes determinants of CMB occurrence from accumulation. Hypertension appears to lower the threshold for lesion emergence along aging. CMB burden is associated with the burden of cerebrovascular lesions, brain atrophy and clinical severity, supporting worsening of the microangiopathic process, with specific impact related to mutation type.