Early and progressive microstructural damage is present in Fabry disease.

<p>In (a), a comparison of FLAIR histograms using the normalized signal intensity (nSI) from the age-based first and fourth quartiles of healthy controls. The histograms for the FLAIR nSI from the younger adult Fabry and control cohorts are shown in (b). Similar comparisons using histograms fr...

Full description

Saved in:

Bibliographic Details
Main Author:	Jacob W. Johnson (20172850) (author)
Other Authors:	Hediyeh Baradaran (9861244) (author), Jubel Morgan (476471) (author), Henrik Odèen (22633876) (author), Emma Friel (22633879) (author), Carrie Bailey (7009100) (author), Brandon A. Zielinski (17051076) (author), Hunter R. Underhill (3151677) (author)
Published:	2025
Subjects:	Medicine Cell Biology Neuroscience Physiology Developmental Biology Mental Health Biological Sciences not elsewhere classified wml severity increased identify factors unique g ., fluid flair ), semi enzyme replacement therapy diffusion tensor imaging corpus callosum atrophied causes glycolipid accumulation became statistically significant attenuated inversion recovery 8 – 63 serial cognitive assessments progressive tissue damage common neurologic sequelae typical brain aging related brain alterations related cognitive decline older fabry patients white matter lesions median age 36 matched healthy controls div >< p using quantitative mri used structural mri younger fabry participants cognitive decline white matter quantitative mri structural mris participants underwent neurologic diseases microstructural damage mri study mri ). fabry drives fabry disease compared fabry rare x phenomena absent p </ normal age neuroradiologist ’ neurocognitive assessment lysosomal alpha linked deficiency large population insidious degeneration impaired cognition highly prevalent fisher ’
Tags:	Add Tag No Tags, Be the first to tag this record!

Description
Summary:	<p>In (a), a comparison of FLAIR histograms using the normalized signal intensity (nSI) from the age-based first and fourth quartiles of healthy controls. The histograms for the FLAIR nSI from the younger adult Fabry and control cohorts are shown in (b). Similar comparisons using histograms from bound-pool fraction maps (<i>f</i>-maps) are shown in (c) and (d), respectively. Distribution of values consistent with gray matter (GM) and white matter (WM) are shown. Associations between age and fractional anisotropy (FA; e) and mean diffusivity (MD, f) demonstrate a decrease and increase, respectively in the Fabry cohort with similar trends in controls. Comparisons of fractional anisotropy (g) and mean diffusivity (h) between cohorts shows differences increased in the older cohort. ns = not significant, <i>P</i> < 0.05, <i>P</i> < 0.01, **<i>P</i> < 0.001.</p>

Early and progressive microstructural damage is present in Fabry disease.

Similar Items