The insidious degeneration of white matter and cognitive decline in Fabry disease

Fabry disease is a rare X-linked deficiency of lysosomal alpha-galactosidase that causes glycolipid accumulation in tissues, including the brain. The most common neurologic sequelae of Fabry are cognitive decline and white matter lesions (WMLs) on brain magnetic resonance imaging (MRI). In the at-large population, however, WMLs are non-specific, highly prevalent, and most are clinically silent. Thus, we compared Fabry to typical brain aging to identify factors unique to Fabry-related cognitive decline. Twenty adult Fabry patients (75% female; median age 36.4 yrs, range: 19.8-63.2 yrs; 95% on enzyme replacement therapy) without a history of stroke or other neurologic diseases and 20 age/sex-matched healthy controls were enrolled in a case-control study. All participants underwent a neurocognitive assessment and a 3.0 T MRI study of the brain that used structural MRI (e.g., fluid-attenuated inversion recovery, FLAIR), semi-quantitative MRI (e.g., normalized FLAIR signal intensity), and qu..., , # The insidious degeneration of white matter and cognitive decline in Fabry disease

Dataset DOI: [10.5061/dryad.gb5mkkx3g](https://doi.org/10.5061/dryad.gb5mkkx3g)

## Description of the data and file structure

Magnetic resonance imaging files for each study participant are located in the participant's study ID folder. 

          F = Fabry participant, C = healthy control participant

Data in DICOM format is available in the subfolder that contains the following strings:

          DTI = diffusion tensor imaging

          FLAIR = fluid attenuated inversion recovery

          T2W = T2-weighted 

          MP_Rage = magnetization prepared rapid acquisition gradient echo

          MT = magnetization transfer:

                    4000 = off-resonance of 4 kHz

                    8000 = off-resonance of 8 kHz

                    96000 = off-resonance of 96 kHz

          VFA = variable flip angle:

                    3 = 3 degree flip angle

                    10 = 10 degree flip ..., The magnetic resonance images were anonymized by using the participant's randomly-generated study identifier instead of the participant's name at the time of image acquisition. The images do not contain any personally identifiable information (PII; e.g., name, date of birth, etc.). All participants consented to publish the de-identified data in the public domain.