PMC3436935_01
A) Coronal gross brain specimen displaying enlarged ventricles, generalized atrophy, and temporal atrophy more prominent on the left (right side of figure), B) Low power photomicrograph of left frontal centrum semiovale with white matter pallor (H&E stain, original magnification 20x), C, D) High power photomicrographs of white matter with diffuse polyglucosan bodies and reactive astrocytosis (original magnification 200x, C - H&E stain, D - ubiquitin immunohistochemistry).
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F1
Severe frontal atrophy and moderate temporal atrophy were noted, worse on the right, as well as caudate atrophy, and were consistent with a clinical diagnosis of behavioral variant FTLD (Fig. 1A). Microscopic findings included marked pallor and rarefaction in the white matter (Fig. 1B) including brainstem tracts, and gliosis (Fig. 1C). Frequent polyglucosan bodies were noted diffusely throughout the cortex and white matter, including the brainstem, subiculum, middle frontal gyrus, superior and middle temporal gyrus, occipital lobe, cingulate gyrus, insula, amygdala, and cerebellum (Fig. 1C,D).
A) TDP-43 neuronal inclusions in frontal cortex (TDP-43 immunohistochemistry), B) Lens shaped or cat's eye inclusion in the caudate nucleus (ubiquitin immunohistochemistry). (magnification bar for both A and B - 10 um).
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F2
Ubiquitin and TDP-43 immunostaining revealed cytoplasmic and intranuclear inclusions with a lens-shaped or cat's eye appearance as described in (Fig. 2).