期刊名称：Aktuelnosti iz Neurologije, Psihijatrije i Graničnih Područja
印刷版ISSN：0354-2726
出版年度：2011
卷号：XIX
期号：1
页码：13-19
出版社：Clinical Center of Vojvodina
摘要：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 apha-sia (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. Histopathological-ly, there are: FTD with tau-positive inclusions (FTD-tau), FTD with tau-negative, ubiquitin positive inclu-sions (FTD-U) and FTD without inclusions. Almost all FTD are TAR-DNA-binding protein 43 (TDP-43) proteinopathies or tauopathies. Currently, there is no cure for this disease and the therapy is only symptom-atic. 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.
