Abstract

MTI is a quantitative MR imaging technique that has recently demonstrated structural integrity differences between controls and patients with HD. Potentially, MTI can be used as a biomarker for monitoring disease progression. To establish the value of MTI as a biomarker, we aimed to examine the change in these measures during the course of HD. From the Leiden TRACK-HD study, 25 controls, 21 premanifest gene carriers, and 21 patients with manifest HD participated at baseline and during a 2-year follow-up visit. Brain segmentation of the cortical gray matter, white matter, caudate nucleus, putamen, pallidum, thalamus, amygdala, and hippocampus was performed by using the automated tools FAST and FIRST in FSL. Individual MTR values were calculated from these regions, and MTR histograms were constructed. In the premanifest HD group stage "far from disease onset," a significant increase in MTR peak height of the putamen was observed with time. During the manifest HD stage, neither the mean MTR nor the MTR peak height showed a significant change during a 2-year follow-up. MTI-derived measures are not suitable for monitoring in Huntington disease during a 2-year period because there was no decrease in structural integrity detected in any of the manifest HD groups longitudinally. The finding of increased putaminal MTR peak height in the premanifest far from disease onset group could relate to a predegenerative process, compensatory mechanisms, or aberrant development but should be interpreted with caution until future studies confirm this finding.

Highlights

  • ObjectivesTo establish the value of MTI as a biomarker, we aimed to examine the change in these measures during the course of HD

  • BACKGROUND AND PURPOSEMTI is a quantitative MR imaging technique that has recently demonstrated structural integrity differences between controls and patients with HD

  • In the premanifest HD group stage “far from disease onset,” a significant increase in MTR peak height of the putamen was observed with time

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Summary

Objectives

To establish the value of MTI as a biomarker, we aimed to examine the change in these measures during the course of HD. We aimed to examine whether MTI measures change during a 2-year period during the progressive course of HD. The goal of this study was to examine the potential of MTI to detect loss of structural integrity in HD to serve as an outcome measure in future therapeutic trails, as was the overall main goal of the TRACK-HD study.

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