Journal
NEUROLOGICAL SCIENCES
Volume 34, Issue 8, Pages 1267-1274Publisher
SPRINGER-VERLAG ITALIA SRL
DOI: 10.1007/s10072-013-1317-9
Keywords
Behavioral variant of frontotemporal dementia; Frontotemporal lobar degeneration; Social cognition disorders; Voxel-based morphometry; [F-18]FDG PET imaging
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The behavioral variant of frontotemporal dementia (bvFTD) is one of the most frequent neurodegenerative disorders with a presenile onset. It is characterized by a long phase of subclinical behavioral changes and social conduct disorders, associated with a progressive modification of personality. Recently, an international consortium of experts developed revised guidelines for its clinical diagnosis, which highlight the supportive role of biomarkers in the diagnostic process. According to new criteria, bvFTD can be classified in possible (requiring three of six specific clinical features), probable (in the presence of functional disability and typical neuroimaging features), and with definite frontotemporal lobar degeneration (requiring the presence of a known causal mutation or a histopathological confirmation). Familial aggregation is frequently reported in bvFTD and frontotemporal lobar degeneration in general, with an autosomal dominant transmission in about 10 % cases. The aim of this paper is to review and discuss recent advances in the knowledge of clinical, neuropsychological, and imaging features of bvFTD. We also briefly summarize the available genetic information about the frontotemporal lobar degeneration spectrum.
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