Objective: To investigate CSF biomarkers in presymptomatic and symptomatic mutation carriers with hereditary cerebral hemorrhage with amyloidosis-Dutch type (HCHWA-D), a model for sporadic cerebral amyloid angiopathy, and to determine the earliest deposited form of b-amyloid (Ab).Methods: HCHWA-D mutation carriers and controls were enrolled in the cross-sectional EDAN (Early Diagnosis of Amyloid Angiopathy Network) study. The HCHWA-D group was divided into symptomatic carriers with a previous intracerebral hemorrhage and presymptomatic carriers. CSF concentrations of Ab 40 , Ab 42 , total tau, and phosphorylated tau 181 proteins were compared to those of controls of a similar age. Correlations between CSF biomarkers, MRI markers, and age were investigated with multivariate linear regression analyses.Results: We included 10 symptomatic patients with HCHWA-D (mean age 55 6 6 years), 5 presymptomatic HCHWA-D carriers (mean age 36 6 13 years), 31 controls ,50 years old (mean age 31 6 7 years), and 50 controls $50 years old (mean age 61 6 8 years).