Clinical, genetic and neuropathological features of frontotemporal dementia: an update and guide

Frontotemporal Lobar Degeneration encompasses a group of heterogeneous disorders with shared behavioural and cognitive symptoms, as well as gross pathological features. The genetic underpinnings and histopathological aspects are quite diverse and form the basis of molecular classification, which is not easy to correlate with clinical findings and syndromes. Scientific research has brought to light an array of knowledge, often not easy to keep up with, especially in the last few years with regard to genetics and histopathology.

The authors have searched the published literature on this topic, chose relevant references, and extracted and systematized the data.

this manuscript presents an updated review of clinical, genetic and histopathological findings in Frontotemporal Lobar Degeneration, with special focus on behavioural variant Frontotemporal Dementia, the most common disorder. Current management is also reviewed, and genetic testing strategies are proposed by the authors for use in clinical practice. Public awareness on this group of disorders should be raised.