[Magnetic resonance tomography of adrenoleukodystrophy]

Adrenoleucodystrophy (ALD) is an uncommon, sex-linked disease, characterised by the accumulation of very long-chain fatty acids in various tissues. There is a great predilection for young boys between the age of three and ten years. The disease ends fatally after a clinical course of several years. The clinical and MR findings of a child with typical ALD are presented. T2-weighted MR demonstrates confluent, symmetrical, hyperintense lesions of the occipital white matter bilaterally. After the application of Gd-DTPA, contrast enhancement is seen in the margins of the lesions indicating active demyelination. MR is recommended as imaging modality of choice in patients with suspected or confirmed diagnosis of adrenoleucodystrophy.