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Updated: Oct 31, 2025

Diffusion Tensor Magnetic Resonance Imaging in the Analysis of Neurodegenerative Diseases

Diffusion Tensor Magnetic Resonance Imaging in the Analysis of Neurodegenerative Diseases

Published on: July 28, 2013

Imaging in X-Linked Adrenoleukodystrophy.

Stephanie I W van de Stadt¹, Irene C Huffnagel¹, Bela R Turk²

¹Department of Pediatric Neurology, Amsterdam Leukodystrophy Center, Emma Children's Hospital, Amsterdam University Medical Centers, Amsterdam Neuroscience, Amsterdam, The Netherlands.

Neuropediatrics

|June 30, 2021

Summary

This summary is machine-generated.

Magnetic resonance imaging (MRI) detects cerebral lesions in X-linked adrenoleukodystrophy (ALD). New quantitative MRI techniques may predict disease onset and progression, aiding treatment development.

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Area of Science:

Neurology
Radiology
Biochemistry

Background:

X-linked adrenoleukodystrophy (ALD) is a peroxisomal disorder causing very long chain fatty acid accumulation.
ALD presents with diverse symptoms including adrenal insufficiency, myelopathy, and cerebral demyelination.
Early detection of cerebral demyelination is crucial for effective hematopoietic cell transplantation (HCT).

Purpose of the Study:

To review current and emerging MRI techniques for ALD detection and monitoring.
To highlight the need for imaging markers predicting cerebral ALD onset and myelopathy severity.
To explore the role of quantitative MRI in clinical practice and research.

Main Methods:

Review of structural MRI techniques for detecting cerebral lesions in ALD.
Discussion of quantitative MRI methods: MR spectroscopy, DTI, perfusion imaging, MT imaging, NODDI, and myelin water imaging.
Analysis of MRI's role in ALD surveillance, treatment prediction, and drug development.

Main Results:

Structural MRI detects existing cerebral lesions but cannot predict onset.
Quantitative MRI techniques show promise for predicting cerebral ALD and assessing myelopathy severity.
Advanced imaging markers are needed to guide early intervention and facilitate clinical trials.

Conclusions:

Active MRI surveillance is essential for male ALD patients.
Quantitative MRI offers potential for early diagnosis, prognosis, and therapeutic monitoring in ALD.
Further research into novel imaging techniques is critical for improving ALD patient outcomes.