Frontotemporal Dementia

Frontotemporal dementias (FTD), or frontotemporal lobar degeneration (FTLD), are the fourth most frequent dementias with the prevalence of 3-10%. Dominant clinical expression in FTD are changes in behavior and personality and disorders of language. Also, in some cases there is parkinsonism and/or signs of motor neuron involvement. Clinically, FTLD includes frontal variant FTD, primary progressive aphasia (PPA), semantic dementia, FTD with motor neuron disease, and hereditary FTD with parkinsonism linked to chromosome 17q21.1 (FTDP-17) and corticobasal degeneration (CBD). Heredity is positive in 50% of cases. Th e age of onset in FTD is from twenties do nineties with mean of 59 years. Disease duration may range from several months to more than 17 years, most frequently 8 to 10 years. Histopathologically, there are: FTD with tau-positive inclusions (FTD-tau), FTD with tau-negative, ubiquitin positive inclusions (FTD-U) and FTD without inclusions. Almost all FTD are TAR-DNA- inding protein 43 (TDP-43)proteinopathies or tauopathies. Currently, there is no cure for this disease and the therapy is only symptomatic. As there are serotonergic and dopaminergic defi cits in FTD, drugs that enhance the neurotransmission of these substances are of a potential, but yet not proven benefi t. Acethylcholinesterase inhibitors show some promise. Th ere are several potential targets for therapy of FTD but no studies have been published yet.