Slight forgetfulness and ...

A 72-year-old man was admitted to outpatient as he had developed slight forgetfulness and a progressive change in his personality. His mood was aggravated, irritable, negativist, authoritarian, and uncooperative. He was forgetful, denied previous events, and made dishonest claims for non-existent happenings. Within a few years, he had evolved severe verbal inertia, and stereotyped expression appeared, reducing communication to a minimum. Motor activity was generally slow. In addition, the patient had observed eccentric behavior in other family members.

The neurological examination findings revealed muscle weakness in the four limbs, especially in the legs, severe dysarthria, and dementia. In addition, all the deep tendon reflexes were increased, and the Babinski's sign was absent bilaterally.

Routine biochemical and hematological were normal, and routine immunological investigations were negative. APOE was e3/e3 alleles. EEG was normal. However, the CT showed bilateral frontal hypodensity. MRI revealed severe bilateral atrophy of the frontotemporal lobes, especially the left temporal lobe. AS SHOWN IN THE FIGURE, brain SPECT showed moderate bilateral frontal and temporal hypoperfusion. On microscopic examination, Neuronal swelling (ballooned cell) and argyrophilic inclusions were seen on the left and right hippocampi, as shown in the figure.