Adrenoleukodystrophy: CT and MRI findings (original) (raw)

A case of adrenoleukodystrophy (ALD) with CT and MRI findings is described. The CT scan showed low densities in the white matter of the parietal and occipital lobes. No calcifications were seen. Post-contrast CT showed an abnormal enhancement within the involved white matter. MRI showed changes of demyelination around the atria of the lateral ventricles bilaterally involving the posterior aspect of the cerebrum symmetrically. The posterior part of the posterior corpus callosum, splenium and pyramidal tracts also showed increased signal intensity. From a review of the literature, these findings are typical of the radiological changes seen in ALD. ALD can be diagnosed from typical history and biochemical changes as well as from CT and MRI findings. Adrenoleukodystrophy (ALD) is a sex-linked metabolic encephalopathy of childhood in which the basic defect is an impaired capacity to degrade very long chain fatty acids, caused by a peroxisomal defect in beta-oxidation. The fatty acids thus accumulate, decreasing the stability of the myelin membrane and leading to the demyelination of the central nervous system. The adrenal cortex, testes, dermal and conjunctival nerve fascicles are also involved [1].