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Abstract
In a longitudinal prospective study of dementia, 158 patients were investigated post
mortem. Sixteen patients were classified as frontal lobe dementia (FLD) of non-Alzheimer
type and four cases as Pick's disease. Positive heredity for dementia was reported
in 50% of these cases compared to 30% in a reference group of patients with Alzheimer's
disease (AD). The typical clinical picture in FLD and Pick's disease was that of a
slowly progressive dementia, at an early stage dominated by personality change, lack
of insight, disinhibition, and later on stereotypy and increased apathy. There was
also a progressive dynamic aphasia ending in mutism and amimia. Memory and spatial
functions were comparatively spared. Disinhibition, oral/dietary hyperactivity, and
echolalia were more consistently found in Pick's disease compared to FLD. The differential
diagnosis against AD, cerebrovascular dementia, and other degenerative dementias and
against affective disorders and psychotic reactions are discussed.