Adrenoleukodystrophy

Demyelination in adrenoleukodystrophy (ALD) leads to rapid and severe brain damage. The aim of this study is to detect white matter lesions in neurologically asymptomatic patients before irreversible damage occurs. Eight patients with biochemically proved ALD have been studied with computed tomography (CT) and magnetic resonance (MR) imaging. CT findings were normal, but MR imaging disclosed abnormalities in four patients (high signal of pyramidal tracts and scattered areas of abnormal signal in the white matter). Rapid extension of the images preceded the onset of clinical symptoms in one patient. MR imaging provides highly contributive information in such patients for whom a specific treatment has been proposed