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Objective: Hyperorality is a core feature of behavioral variant frontotemporal dementia (bvFTD); however, the cognitive, psychiatric, and neuroanatomical correlates of hyperorality across the bvFTD stages remain unclear. The authors explored these associations in early- and advanced-stage bvFTD.
Methods: Participants with sporadic or genetic bvFTD were enrolled in the ARTFL LEFFTDS Longitudinal Frontotemporal Lobar Degeneration (ALLFTD) consortium study. Baseline cognitive and psychiatric symptoms of participants with or without hyperorality were compared after stratification by disease severity. Linear multivariable regressions adjusted for age and total intracranial volume were used to examine associations between regional gray matter volume (GMV) and hyperorality status. Five anatomical regions of interest were preselected for analysis on the basis of previously identified neuroanatomical correlates of hyperorality in bvFTD.
Results: Hyperorality was present in 50% of early-stage bvFTD participants (N=136) and was associated with higher rates of ritualistic-compulsive behavior and difficulty detecting social-emotional expressions. Hyperorality was present in 63% of advanced-stage participants (N=208) and was associated with higher rates of apathy, ritualistic-compulsive behavior, and socially aberrant behavior. Regional GMV was similar for those with or without hyperorality among early-stage participants. Among advanced-stage participants, hyperorality was associated with lower GMV in the right dorsal and ventral striatum.
Conclusions: Hyperorality emerged early in bvFTD and was accompanied by deficits in social cognition and complex-ritualistic behavior before clinically significant GMV loss. These findings suggest that early identification and management of hyperorality could improve neuropsychiatric trajectories in bvFTD.
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http://dx.doi.org/10.1176/appi.neuropsych.20240134 | DOI Listing |
Background: Pick's disease (PiD) is a subtype of frontotemporal lobar degeneration. However, the pathogenesis and symptomatic lesions remain unclear. We report a case of PiD with a short disease duration and compare it to a case series to reveal the association between degenerative patterns and clinical manifestations.
View Article and Find Full Text PDFJ Neuropsychiatry Clin Neurosci
March 2025
Department of Psychiatry and Behavioral Sciences (Morrow, Onyike, Smith, Leoutsakos, Balaji, Kamath), Department of Neurology (Pantelyat, Tsapkini), and Russell Morgan Department of Radiology (Smith, Faria), Johns Hopkins University School of Medicine, Baltimore; Department of Neurology, Mayo Clinic
Objective: Hyperorality is a core feature of behavioral variant frontotemporal dementia (bvFTD); however, the cognitive, psychiatric, and neuroanatomical correlates of hyperorality across the bvFTD stages remain unclear. The authors explored these associations in early- and advanced-stage bvFTD.
Methods: Participants with sporadic or genetic bvFTD were enrolled in the ARTFL LEFFTDS Longitudinal Frontotemporal Lobar Degeneration (ALLFTD) consortium study.
Psychiatry Clin Neurosci
June 2025
Department of Psychiatry and Behavioral Sciences, Johns Hopkins University School of Medicine, Baltimore, Maryland, USA.
Aim: Cognitive and behavioral phenomena define behavioral variant frontotemporal dementia (bvFTD), but neuropsychiatric symptoms (NPS) outside the core criteria are common throughout the illness. Identifying how NPS cluster in bvFTD may guide development of future therapies.
Methods: Participants (n = 354) with sporadic and genetic bvFTD were enrolled in the ARTFL LEFFTDS Longitudinal Frontotemporal Lobar Degeneration Consortium.
Neurocase
August 2025
Alzheimer's Disease Research Center, University of Pittsburgh, Pittsburgh,PA, USA.
Atypical frontotemporal lobar degeneration with ubiquitin-positive inclusions (aFTLD-U) is a rare subtype of Frontotemporal Lobar Degeneration. aFTLD-U is reported with a typically young onset behavioral variant of frontotemporal dementia syndrome, more specifically with younger age, predominant hyperorality, obsessive-compulsive features, and severe caudate atrophy. Very few cases have been reported with prominent language impairment with no major behavioral features.
View Article and Find Full Text PDFEur J Neurol
January 2025
Department of Clinical Sciences, Clinical Memory Research Unit, Faculty of Medicine, Lund University, Lund, Sweden.