PMC4628904_01
MRI and FDG-PET imaging. Magnetization-prepared rapid acquisition with gradient-echo (MP-RAGE) images show left mesial temporal atrophy on coronal images (A1) and medial pre-central atrophy on sagittal images (A2). FDG-PET measuring glucose metabolism (green = mild hypometabolism; yellow=moderate hypometabolism; red=severe hypometabolism) shows mostly mild hypometabolism in the left anterior temporal lobe and left inferior frontal region (B1) which is also evident on coronal (B2) and axial images (B3). Abbreviations: A, anterior; L, left; P, posterior; R, right.
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Head MRI revealed left amygdala, hippocampal, and anterior temporal lobe atrophy (Figure 1A) and 18-F-Flouro-deoxy-glucose-positron-emission-tomography (FDG-PET) scan showed left anterior medial temporal and posterior inferior frontal hypometabolism (Figure 1B).
MRI atrophy patterns using differential diagnosis based on structural abnormality in neurodegeneration (differential-STAND).. There is atrophy in bilateral mid and superior frontal regions, bilateral precentral regions (worse on the left), bilateral precuneus, and postcentral regions. The left hippocampus, fusiform and mid temporal regions also showed significant atrophy.. Abbreviations (z score refers to the number of standard deviations): L, left; R, right (note for this analysis the normal MRI left-right orientation is reversed; the left brain is on the left of the figure and the right brain is on the right of the figure for each panel).
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The differential diagnosis based on structural abnormality in neurodegeneration (differential-STAND) maps (figure 2) showed atrophy in the following regions: bilateral mid and superior frontal regions, bilateral precentral regions with left hemisphere being more severely involved, bilateral precuneus, and postcentral regions.
Figure 3 demonstrates two separate pathological processes in our patient with unclassified PPA.. On the left are tau immunoreactive coiled bodies and grains consistent with argyrophilic grains disease. On the right are TDP-43 immunoreactive neuronal cytoplasmic inclusions consistent with frontotemporal lobar degeneration with TDP; Type B pathology.
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Type B proteinopathy (Figure 3). The whole brain weight was 1040 grams. The argyrophilic grain disease (with many grains, pre-tangles and coiled bodies) was largely limited to hippocampus, amygdala and nucleus accumbens (Figure 3). TDP-43 immunoreactive neuronal cytoplasmic inclusions were moderate in amygdala and entorhinal, occipitotemporal and inferior temporal cortices, and sparse in peri-Rolandic cortices, dentate fascia, CA1 and subiculum of the hippocampus and basal nucleus of Meynert (Figure 3).