Abstract WP388: Basilar Artery Diameter is Associated With Cerebral Small Vessel Disease and the Duration of Enzyme-replacement Therapy in Fabry Disease

Introduction: Fabry disease (FD), an X-linked lysosomal storage disorder, causes intracellular accumulation of glycosphingolipids leading to progressive renal, cardiac, and cerebrovascular disease. Enzyme replacement therapy (ERT) is effective in reducing cardiac hypertrophy but has a limited effect on renal function, whereas there is still no clear evidence of the benefit of ERT for incident stroke. An enlarged basilar artery (BA) diameter has been of particular interest as an early and potential risk factor for stroke. However, the long-term effect of ERT for BA diameter in patients with FD are unknown. Hypothesis: We hypothesized that effect of ERT exerts a certain influence on for BA diameter in patients with FD. The aim of our study was to investigate the clinical characteristics of BA diameter in patients with FD. Methods: We retrospectively enrolled consecutive patients with FD who were treated ERT and underwent brain MRI. The short axis of BA diameter was measured at the midpons level on the axial...