Figure 2

Magnetic resonance imaging (MRI) of index patient. (a) T1 axial sequences, showing prominent atrophy, right predominant, affecting frontal, temporal, insular and parietal lobes, present in the first evaluation (4 years after clinical onset). (b) Similar but more pronounced findings one year later. Positron emission tomography (PET) images of the proband. 18 F-FDG PET (c) showed pronounced hypometabolism in the right frontal, temporal and parietal cortex, while Aβ-PET (11C-PIB, d) showed elevated cortical retention bilaterally. MRI images are shown in radiological orientation and PET images in neurological orientation SUVR – standardized uptake value ratio (t- = 30-60 min, normalized to mean activity in the pons); DVR – Distribution Volume Ratio (0–90 min, Logan graphical analysis, reference region = cerebellum gray matter).