Morrow Christopher B, Onyike Chiadi, Pantelyat Alexander, Smith Gwenn S, Leoutsakos Jeannie, Faria Andreia V, Graff-Radford Neill R, Darby R Ryan, Ghoshal Nupur, Staffaroni Adam M, Rascovsky Katya, Miyagawa Toji, Balaji Akshata, Tsapkini Kyrana, Lapid Maria I, Mendez Mario F, Litvan Irene, Pascual Belen, Rojas Julio C, Wszolek Zbigniew K, Domoto-Reilly Kimiko, Kornak John, Kamath Vidyulata
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, Jacksonville, Fla. (Graff-Radford, Wszolek); Department of Neurology, Vanderbilt University Medical Center, Nashville (Darby); Department of Neurology, Washington University School of Medicine, St. Louis (Ghoshal); Department of Neurology, Memory and Aging Center, Weill Institute for Neurosciences (Staffaroni, Rojas), and Department of Epidemiology and Biostatistics (Kornak), University of California San Francisco, San Francisco; Department of Neurology and Penn Frontotemporal Degeneration Center, University of Pennsylvania Perelman School of Medicine, Philadelphia (Rascovsky); Department of Psychiatry and Psychology, Mayo Clinic, Rochester, Minn. (Miyagawa, Lapid); Department of Neurology, David Geffen School of Medicine, University of California Los Angeles, Los Angeles (Mendez); Department of Neurosciences, University of California San Diego, La Jolla (Litvan); Department of Neurology, Houston Methodist Research Institute, Houston (Pascual); Department of Neurology, University of Washington School of Medicine, Seattle (Domoto-Reilly).
J Neuropsychiatry Clin Neurosci. 2025 Mar 26:appineuropsych20240134. doi: 10.1176/appi.neuropsych.20240134.
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.
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.
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.
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.
口欲亢进是行为变异型额颞叶痴呆（bvFTD）的核心特征；然而，bvFTD各阶段口欲亢进的认知、精神和神经解剖学相关性仍不清楚。作者在bvFTD的早期和晚期阶段探讨了这些关联。
散发性或遗传性bvFTD患者参加了ARTFL LEFFTDS额颞叶变性纵向研究（ALLFTD）联盟研究。根据疾病严重程度分层后，比较有无口欲亢进的参与者的基线认知和精神症状。使用调整年龄和总颅内体积的线性多变量回归来检查区域灰质体积（GMV）与口欲亢进状态之间的关联。基于先前确定的bvFTD口欲亢进的神经解剖学相关性，预先选择五个感兴趣的解剖区域进行分析。
50%的bvFTD早期参与者（N = 136）存在口欲亢进，且与仪式性强迫行为发生率较高以及难以察觉社会情感表达有关。63%的晚期参与者（N = 208）存在口欲亢进，且与冷漠、仪式性强迫行为和社会异常行为发生率较高有关。早期参与者中，有无口欲亢进者的区域GMV相似。在晚期参与者中，口欲亢进与右侧背侧和腹侧纹状体的GMV较低有关。
口欲亢进在bvFTD早期出现，且在临床上显著的GMV损失之前伴有社会认知和复杂仪式行为缺陷。这些发现表明，早期识别和处理口欲亢进可能改善bvFTD的神经精神病程。
