Computed tomography in late-onset metachromatic leucodystrophy

Summary In late-onset metachromatic leucodystrophy (MLD), early clinical diagnosis is difficult since initial symptoms frequently consist of misleading nonspecific psychopathological alterations. On cranial computed tomography (CT), however, symmetrical attenuation decrease of the white matter and mild cerebral atrophy can already be found in an early stage of the disease, and may even precede clinical symptoms. On the basis of observations in four patients with late-onset MLD who were followed between 1 and 5 years, characteristic CT appearances in different stages of the disease are outlined and compared with the literature.