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Adrenoleukodystrophy. Value of contrast-enhanced MR imaging
C Romero1, J L Dietemann, D Kurtz
1Service de Radiologie B (Unité de Neuroradiologie et de Scanographie), Hospices Civils, Universitaire de Strasbourg, France.
Journal of Neuroradiology = Journal De Neuroradiologie
|January 1, 1990
Summary
Early adrenoleukodystrophy (ALD) shows brain abnormalities on CT and MRI. Contrast-enhanced MRI ribbons are highly indicative of ALD, aiding in early diagnosis.
Area of Science:
- Neurology
- Radiology
- Medical Imaging
Background:
- Adrenoleukodystrophy (ALD) is a rare genetic disorder affecting the adrenal glands and white matter of the brain.
- Early-stage ALD presents with non-specific white matter changes on CT and MRI, complicating diagnosis.
Observation:
- Standard CT and MRI reveal hypodensity and signal abnormalities in the parieto-occipital white matter and corpus callosum.
- These initial findings, while suggestive, lack specificity for ALD in pediatric patients with cognitive decline.
Findings:
- Contrast-enhanced MRI, particularly gadolinium injection, reveals pathognomonic "ribbons" at the edge of demyelination plaques.
- These "ribbons" represent blood-brain barrier disruption, inflammation, and active demyelination, appearing as low-intensity signals on T2-weighted sequences.
- MRI demonstrates superior sensitivity for detecting posterior fossa lesions, including auditory pathway involvement, compared to CT.
Implications:
- The identification of contrast-enhanced ribbons significantly improves the diagnostic accuracy for early-stage ALD.
- Enhanced MRI protocols can aid in earlier detection and intervention for ALD, potentially improving patient outcomes.
- This imaging finding is crucial for differentiating ALD from other white matter diseases.