Abstract

Frontotemporal dementia (FTD) is characterized by progressive changes in behavior and personality with an early decline in social and personal interaction and emotional blunting, disinhibition, language disorders, and in later stages, more general cognitive decline ( 1 Viskontas I.V. Possin K.L. Miller B.L. Symptoms of frontotemporal dementia provide insights into orbitofrontal cortex function and social behavior. Ann N Y Acad Sci. 2007; 1121: 528-545 Crossref PubMed Scopus (99) Google Scholar ). Frontotemporal dementia is genetically heterogeneous, and the discovery of the progranulin (PGRN) gene role in the pathogenesis of the disease constitutes a major breakthrough in the genetic of the dementias ( 2 Baker M. Mackenzie I.R. Pickering-Brown S.M. Gass J. Rademakers R. Lindholm C. et al. Mutations in progranulin cause tau-negative frontotemporal dementia linked to chromosome 17. Nature. 2006; 442: 916-919 Crossref PubMed Scopus (1587) Google Scholar ).

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