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Childhood adrenoleukodystrophy: assessment with proton MR spectroscopy
A A Tzika1, W S Ball, D B Vigneron
1Department of Radiology, Children's Hospital Medical Center, Cincinnati, Ohio.
Radiology
|November 1, 1993
Summary
Proton magnetic resonance (MR) spectroscopy reveals distinct white matter changes in adrenoleukodystrophy (ALD). This noninvasive technique can aid in diagnosing and predicting ALD progression.
Area of Science:
- Biomedical Imaging
- Neuroscience
- Biochemistry
Background:
- Adrenoleukodystrophy (ALD) is a rare genetic disorder affecting the white matter of the brain and adrenal glands.
- Early diagnosis and monitoring of ALD are crucial for managing disease progression and patient outcomes.
Observation:
- This study utilized image-guided, single-voxel, localized proton magnetic resonance (MR) spectroscopy to examine white matter in childhood ALD.
- Eleven patients with X-linked ALD, including symptomatic and asymptomatic individuals, were compared to nine age-matched healthy volunteers.
Findings:
- MR spectra from white matter lesions in ALD patients showed a significant reduction in N-acetyl aspartate (NAA) and an increase in choline-containing compounds (Cho) and glutamate/glutamine/inositol relative to total creatine (tCr).
- Lactate accumulation was observed in four patients with ALD.
- Asymptomatic patients without visible brain lesions still exhibited elevated Cho-to-tCr ratios, suggesting early metabolic alterations.
Implications:
- Proton MR spectroscopy offers a noninvasive method for assessing white matter integrity in ALD.
- These spectroscopic findings may serve as valuable biomarkers for the diagnosis and prognosis of adrenoleukodystrophy.