<> "The repository administrator has not yet configured an RDF license."^^ . <> . . . "In vivo and Postmortem Clinicoanatomical Correlations in Frontotemporal Dementia and Parkinsonism Linked to Chromosome 17"^^ . "Background: Frontotemporal dementia and parkinsonism linked to chromosome 17 (FTDP-17) is associated with mutations in the Microtubule-Associated Protein Tau(MAPT) gene or the Progranulin(PGRN) gene. MAPT mutations lead to widespread deposition of hyperphosphorylated tau protein (FTDP-17T). PGRN mutations are associated with ubiquitin- and TDP-43-positive inclusions in the frontotemporal cortex, striatum and hippocampus (FTDP-17U). Despite the differences, FTDP-17T and FTDP-17U share a largely overlapping clinical phenotype. Objective: To determine whether neuroimaging studies may allow an in vivo early differentiation between FTDP-17T and FTDP-17U. Methods: We studied 25 individuals affected with FTDP-17T associated with either the exon 10+3 (24 subjects) or the G335S (1 subject) MAPT mutation, as well as 3 FTDP-17U individuals, who were carriers of the A9D, IVS6-2A>G or R493X PGRN mutation. Neuroimaging studies, obtained along the course of the disease, were compared to the neuropathologic findings. Results: FTDP-17T cases were associated with symmetric frontotemporal atrophy. Behavioral changes constituted the predominant clinical presentation. Conversely, an asymmetric degenerative process was seen in all 3 PGRN cases, who presented with either corticobasal syndrome (A9D) or frontotemporal dementia and language deterioration (IVS6-2A>G and R493X). Conclusion: Neuroimaging data, in the early disease stage of FTDP-17, may offer the possibility of an early differentiation of FTDP-17T and FTDP-17U phenotypes, independent of the genetic analysis."^^ . "2008" . "5" . "3-4" . . "Karger"^^ . . . "Neurodegenerative Diseases"^^ . . . "16602854" . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . "Edward D"^^ . "Huey"^^ . "Edward D Huey"^^ . . "C."^^ . "Keohane"^^ . "C. Keohane"^^ . . "M.R."^^ . "Farlow"^^ . "M.R. Farlow"^^ . . "Jordan"^^ . "Grafman"^^ . "Jordan Grafman"^^ . . "Pietro"^^ . "Pietrini"^^ . "Pietro Pietrini"^^ . . "B."^^ . "Sweeney"^^ . "B. Sweeney"^^ . . "Eric M."^^ . "Wassermann"^^ . "Eric M. Wassermann"^^ . . "Bernardino"^^ . "Ghetti"^^ . "Bernardino Ghetti"^^ . . "Jill R."^^ . "Murrell"^^ . "Jill R. Murrell"^^ . . "Salvatore"^^ . "Spina"^^ . "Salvatore Spina"^^ . . . . . "HTML Summary of #3075 \n\nIn vivo and Postmortem Clinicoanatomical Correlations in Frontotemporal Dementia and Parkinsonism Linked to Chromosome 17\n\n" . "text/html" . . . "RC0321 Neuroscience. Biological psychiatry. Neuropsychiatry"@en . .