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An Automated Analysis Tool for Diffusion Tensor Imaging-Based Quantitative MRI in X-Linked Adrenoleukodystrophy.

Eda G Kabak1, Fenna van Dodewaard2, Stefan D Roosendaal3

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Summary

Quantitative MRI (qMRI) using BrainQuant effectively tracked myelopathy progression in X-linked adrenoleukodystrophy (X-ALD) patients. While not differentiating cerebral ALD (CALD), radial diffusivity (RD)-global emerged as a promising biomarker for monitoring disease advancement.

Keywords:
X‐linked adrenoleukodystrophybrainQuantcerebral ALDdiffusion tensor imagingmyelopathyquantitative MRIradial diffusivity

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Area of Science:

  • Neuroimaging
  • Neurology
  • Biomarkers

Background:

  • X-linked adrenoleukodystrophy (X-ALD) is a genetic disorder caused by ABCD1 variants, leading to very long-chain fatty acid accumulation.
  • Phenotypes range from cerebral ALD (CALD) to adrenomyeloneuropathy (AMN), impacting neurological function.
  • Quantitative MRI (qMRI) offers potential for objective disease assessment.

Purpose of the Study:

  • To evaluate if automated qMRI parameters from BrainQuant can differentiate CALD from non-CALD phenotypes.
  • To determine if qMRI metrics reflect myelopathy severity in X-ALD patients.
  • To assess the longitudinal changes in qMRI parameters and their correlation with clinical outcomes.

Main Methods:

  • Adult males with X-ALD underwent annual neurological exams and brain MRI (2015-2024).
  • BrainQuant processed diffusion tensor imaging (DTI) to extract global metrics: fractional anisotropy (FA), mean diffusivity (MD), axial diffusivity (AD), and radial diffusivity (RD).
  • Patients were stratified by Loes score and expanded disability status scale (EDSS); correlations with clinical scores (SSPROM, 6-MWT) were analyzed.

Main Results:

  • Global DTI metrics did not significantly differentiate CALD from non-CALD.
  • Patients with higher EDSS (>2) exhibited significantly worse global DTI values, correlating with clinical severity.
  • Longitudinally, global radial diffusivity (RD) significantly increased over time, irrespective of EDSS group.

Conclusions:

  • BrainQuant qMRI effectively tracks myelopathy progression in X-ALD but does not distinguish CALD from non-CALD.
  • Global radial diffusivity (RD) is a sensitive biomarker for monitoring X-ALD disease advancement.
  • Automated qMRI holds promise for objective assessment and management of X-ALD patients.