Fronto temporal dementias

Abstract

Frontotemporal dementias (FTD) are a heterogenous group of neurocognitive disorders which varies with regard to their clinical presentation, radiological characteristics, pathology and genetics. The core FTD clinical syndromes are behavioural variant FTD (bvFTD), semantic variant primary progressive aphasia (svPPA) and non-fluent/ agrammatic variant of primary progressive aphasia (nfvPPA). FTD related syndromes include frontotemporal dementia with motor neuron disease (FTD-MND), progressive supranuclear palsy syndrome (PSP-S) and corticobasal syndrome (CBS). This article discusses the clinical presentation, diagnosis, neuropathology, neurogenetics and therapeutics of FTDs.