Dissecting the Many Faces of Frontotemporal Dementia: An Imaging Perspective

Abstract

Frontotemporal dementia (FTD) is a heterogeneous clinical and neuropathological disorder characterized by behavioral abnormalities, executive dysfunctions and language deficits. FTD encompasses a wide range of different pathological entities, associated with the accumulation of proteins, such as tau and TPD-43. A family history of dementia is found in one third of cases, and several genes causing autosomal dominant inherited disease have been identified. The clinical symptoms are preceded by a prodromal phase, which has been mainly studied in cases carrying pathogenetic mutations. New experimental strategies are emerging, in both prodromal and clinical settings, and outcome markers are needed to test their efficacy. In this complex context, in the last few years, advanced neuroimaging techniques have allowed a better characterization of FTD, supporting clinical diagnosis, improving the comprehension of genetic heterogeneity and the earliest stages of the disease, contributing to a more detailed classification of underlying proteinopathies, and developing new outcome markers on clinical grounds. In this review, we briefly discuss the contribution of brain imaging and the most recent techniques in deciphering the different aspects of FTD.