Frontotemporal dementia, Pick's disease.
A significant expansion of knowledge in the last few years, especially in the molecular biology of frontotemporal dementia (FTD) is summarized. This condition, formerly known as Pick's disease and considered rare, is estimated to be 12-15% of all dementias and 30-50% early onset ones. The clinical picture is protean, mainly a behavioural and language impairment, but the extrapyramidal syndromes of CBD and PSP also belong. These seemingly different presentations converge, as one or other areas in the brain are affected. Less than half of the cases are tauopathies, the majority has been discovered to have a TDP-43 and most recently a FUS proteinopathy, shared with ALS, opening potential opportunities for pharmacological approaches to treatment. Tau and progranulin mutations on Ch-17 and some others, point to molecular mechanisms. A glossary is provided to navigate the complex terminology.
Kertesz
Andrew
A
University of Western Ontario, St. Joseph's Hospital, 268 Grosvenor St. London, Ontario, Canada N6A4V2. Andrew.Kertesz@sjhc.london.on.ca
D001037:Aphasia / Q000209:etiology; D057180:Frontotemporal Dementia / Q000150:complications / Q000175:diagnosis / Q000453:epidemiology / Q000235:genetics / Q000628:therapy; D006801:Humans; D036341:Intercellular Signaling Peptides and Proteins / Q000235:genetics; D016472:Motor Neuron Disease / Q000150:complications; D009154:Mutation; D020774:Pick Disease of the Brain / Q000150:complications / Q000175:diagnosis / Q000453:epidemiology / Q000235:genetics / Q000628:therapy; D011379:Prognosis; D000077153:Progranulins; D012660:Semantics; D016875:tau Proteins / Q000235:genetics
C492314:GRN protein, human; D036341:Intercellular Signaling Peptides and Proteins; C054369:MAPT protein, human; D000077153:Progranulins; D016875:tau Proteins