Background: Fabry disease (FD) is a lysosomal storage disorder that is associated with marked cerebrovascular disease. Conventional MRI shows an extensive load of white matter lesions (WMLs) already at an early stage in FD.
Objective: Investigator independent and sensitive quantification of brain structural changes in clinically affected men and women with FD.
Methods: The authors performed a voxel-based analysis of diffusion tensor images (DTI) in 25 FD patients and 20 age-matched normal controls.
Results: DTI revealed significant increases of cerebral white matter diffusivity (MD) in FD patients that were pronounced in the periventricular white matter. Even the subgroup of patients without significant WMLs-load (n = 18) showed increased diffusivity in the cerebral white matter. In gray matter areas MD elevation could only be detected in the posterior part of the thalamus, independently from visible pulvinar alterations on the T1-weighted images. Voxel-based anisotropy (FA) measurements did not differ significantly between patients and controls.
Conclusions: The present study demonstrates the clinical feasibility of voxel-based analysis of DTI as a sensitive tool to quantify brain tissue alterations in FD. The pattern of increased brain tissue diffusivity is probably due to microangiopathic alterations mainly affecting the long perforating arteries.
- diffusion-tensor Imaging
- fabry disease
- voxel-based DTI
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