A rare form of dementia that is sometimes familial. Clinical features include aphasia; APRAXIA; confusion; anomia; memory loss; and personality deterioration. This pattern is consistent with the pathologic findings of circumscribed atrophy of the poles of the frontal lobe and temporal lobe. Neuronal loss is maximal in the hippocampus, entorhinal cortex, and amygdala. Some ballooned cortical neurons contain argentophylic (Pick) bodies. (From brain Pathol 1998 Apr;8(2):339-54; Adams et al., Principles of neurology, 6th ed, pp1057-9)


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