"Frontal variant Alzheimer's disease": a reappraisal

Abstract

Two cases of clinically diagnosed sporadic Alzheimer's disease with early and prominent behavioural features (social disinhibition, emotional blunting, stereotyped verbal utterances) sufficient to prompt an initial diagnosis of frontotemporal dementia are presented. It is suggested that the term "frontal variant AD" be used for this clinically defined phenotype, which has also been described in cases of inherited AD associated with certain presenilin-1 gene mutations. This differs from previous usage of the term "frontal variant AD" to describe AD with predominant frontal lobe neuropathological change (although the clinical phenotype may reflect regional distribution of pathology), but parallels the clinical definition of visual agnosic, aphasic and apraxic presentations of AD. The proposed usage would also emphasise differential diagnosis.