Fig. 1

Brain MRI imaging in two patients with Kufs disease type B. Case 1. Axial T2-TSE (a and b), coronal T2-FLAIR (c), and sagittal T1-TSE (d) sections at onset of cognitive impairment showing cortico-subcortical atrophy, predominant in the parieto-occipital regions and the cerebellum, white matter hyperintensities and thinning of the corpus callosum (arrow). Case 2. Images at onset of cognitive impairment: axial T2-TSE (a1), coronal T2-FLAIR (b1) and sagittal T1-TSE (c1) sections displayed brain atrophy, mainly involving the cerebellum, periventricular hyperintensities and thinning of the corpus callosum (arrow). Follow up imaging: axial T2-TSE (a2) and coronal T2-FLAIR (b2) and sagittal T1-TSE (c2) scans taken in the same patient 9 years later