Commentary on: psychotic symptoms in frontotemporal dementia: a diagnostic dilemma?

Extract

Frontotemporal dementia is clearly a very important condition, not only because alongside Alzheimer's disease, vascular dementia, and Lewy body dementia it is one of the four most common causes of dementia, but because its broad symptom profile and younger age of onset makes it a particularly challenging condition to diagnose and manage. In recent years, great progress has been made in understanding genetic and pathological underpinnings of frontotemporal dementias, and in characterizing specific pathological causes. In particular, three major subtypes can be delineated: those associated pathologically with tau; transactive responsive DNA binding protein (TDP-43); or fused in sarcoma (FUS). Of these, tau and TDP-43 are by far the two most common subtypes of frontotemporal dementia. In addition, there have been important genetic advances and several autosomal dominant mutations have been described, for example associated with MAPT, progranulin, and C9ORF72. However, despite these important advances regarding pathophysiological heterogeneity, relatively little is known about the frequency, nature, and correlates of psychotic symptoms in the disorder. It is therefore very timely that in this month's Paper of the Month, Waldo and colleagues (Waldo et al., 2015) describe a very detailed description of a cohort of 97 consecutive subjects seen in Lund who received a neuropathological diagnosis of frontotemporal lobar degeneration.