Frontotemporal dementias: main syndromes and underlying brain changes.

Abstract

Frontotemporal dementia (FTD) is a rare dementia, that accounts for about 15% of all dementia cases. Despite consensus diagnostic criteria, FTD remains difficult to diagnose in life because of its complex and variable clinical phenomenology and heterogeneous disorders. This review provides an update on the current knowledge of the main FTD syndromes -- the behavioural variant, semantic variant, and nonfluent/agrammatic variant-- their brain abnormalities and genetic profiles. The complexity of the clinical features in FTD has become increasingly apparent, particularly in the domain of behaviour. Such behaviour changes are now also being recognized in the language variants of FTD. Initial interest on emotion processing and social cognition is now complemented by studies on other behavioural disturbance, that spans gambling, antisocial behaviours, repetitive behaviours, and apathy. At a biological level, novel pathological subcategories continue to be identified. From a genetic viewpoint, abnormalities in three genes explain nearly three quarters of familial cases of FTD. In the absence of effective drug treatments, novel approaches are needed to target some of the most disabling features of FTD, such as language loss or behaviour disturbance. Recent interventions appear promising but will require confirmation.