Quantification of Total White Matter Perivascular Space Volume: Follow-up in Dutch-type Cerebral Amyloid Angiopathy

Motivation: Most commonly used methods to assess enlarged perivascular spaces (PVS) are visual rating scales that exhibit a strong ceiling effect and are limited to a single slice, limiting the assessment of progression. Goal(s): To develop and apply a method to quantitatively assess whole-brain PVS in Dutch-type Cerebral Amyloid Angiopathy (D-CAA). Approach: We applied a semi-automatic method with an in-house developed tool to assess whole-brain PVS volume fraction (PVSvf) over four-year follow-up. Results: Significant baseline differences were observed. No group differences were seen between symptomatic, pre-symptomatic D-CAA and controls in PVSvf over four years of follow-up. Impact: Sensitive measurement of PVS-enlargement will increase our understanding of disease mechanisms and allow monitoring of disease progression. This is relevant for developing treatment strategies and especially for tracking the effectiveness of treatments.