PMID: 2092086Jan 1, 1990Paper

Adrenoleukodystrophy. Value of contrast-enhanced MR imaging

Journal of Neuroradiology. Journal De Neuroradiologie
C RomeroD Christmann

Abstract

In its early stage adrenoleukodystrophy (ALD) is characterized by hypodensity at CT and signal abnormalities at RMI (low-intensity signal on T1-weighted sequences, high-intensity signal on T2-weighted sequences) in the white matter of the parieto-occipital region and the splenium of the corpus callosum. These CT and RMI abnormalities are suggestive of ALD in children with progressive alteration of the superior brain functions, but they are not specific of the disease. The authors present two cases of ALD and underline the almost pathognomonic value of contrast-enhanced ribbons found at the periphery of low-intensity signal plaques after gadolinium injection. These areas of blood-barrier disruption on a background of inflammation and active demyelination appear, on T2-weighted sequences, as ribbons of low-intensity signal within plaques of high-intensity signal. MRI is also superior to CT in detecting abnormalities located in the posterior fossa, notably lesions of the auditory fibres.

Related Concepts

Related Feeds

Adrenoleukodystrophy

Adrenoleukodystrophy (ALD), the most frequent peroxisomal disorder, is an X-linked disorder caused by a defect in the metabolism of long chain fatty acids leading to demyelination, neurodegeneration, and death. Here is the latest research.