FIGURE 1.

Neurocognitive and brain structural and metabolic assessment of the reported patient. A, Changes in the total score of Mattis dementia rating scale (MDRS), indicating progressive cognitive decline (109/144, 69/144, and 58/144, at the age of 58, 61, and 62, respectively). Shadowed range indicated on the panel represents a 25 to 75 percentile interval, previously established for the population younger than 70 years old. B, Specific MDRS subscores collected at the age of 62, and expressed as a percentage of the maximal possible value for each subscore. C-D, The coronal FLAIR imaging, demonstrating a clear progression of the hippocampal atrophy (white arrows) within 5 years of follow-up, from the age of 57 (C) to 62 (D). Note the right hemispheric dominance of the hippocampal atrophy and consecutive dilation of the temporal horns of the lateral ventricles. E-F, The axial T2 shows a mild parietal atrophy (white arrows), again with right hemispheric predominance, which only slowly progressed between the age to 57 (E) and 62 (F). Note the absence of microvascular white matter lesions. G, Metabolic neuroimaging. DaTScan imaging shows normal uptake of the tracer in the caudate and putamen. FDG-PET indicates global decrease in cerebral metabolism, particularly severe in the right temporo-parietal region (white arrow). SPECT reveals generalized hypoperfusion, slightly predominant on the right side (white arrow). Positron-emission tomography with Pittsburgh compound-B (PET-PiB) shows decreased tracer activity after 1 to 2 minutes, especially in the right temporo-parietal region (white arrow), and massive and widespread retention of PiB after 50 to 70 minutes, again with right predominance.