Pathology, diagnosis and discussion

At autopsy extensive atrophy of the anterior temporal lobes, orbitofrontal and medial frontal cortex was evident. Extensive spongiosus, gliosis and neuronal loss were evident in these frontotemporal regions. Ubiquitin-positive inclusions were evident in the frontal and anterior temporal lobes.

This patient developed classic symptoms and findings of SD. Like many patients with asymmetric left-sided temporal lobe degeneration, his first symptoms related to naming people and objects. Also typical of other SD patients was his compulsive preoccupation with shells, jewelry, solitaire and squash. As his disease moved from the left to the right anterior temporal lobe, deficits in the recognition of faces became evident. His abnormalities in object naming and verbal memory in association with sparing of frontal and parietal lobe functions also typify this FTLD subtype.