Medical College of Wisconsin, Milwaukee, WI, USA.
Clement J. Zablocki VA Medical Center, Milwaukee, WI, USA.
Clin Neuropsychol. 2024 Jul;38(5):1256-1271. doi: 10.1080/13854046.2024.2315726. Epub 2024 Feb 15.
Behavioral variant frontotemporal dementia (bvFTD) is a neurodegenerative condition characterized by progressive changes in behavior, cognition, and day-to-day functioning. Progression of the disease usually leads to death 3-5 years after diagnosis. However, there are reports of individuals who are initially diagnosed with bvFTD but fail to progress. These individuals are thought to have what is becoming known as phenocopy bvFTD (phFTD). : This manuscript reviews a single case study of a 68-year-old male Veteran who was diagnosed with bvFTD in 2010, which has not progressed over time. : Review of serial neuropsychological evaluations was broadly normal with mild evidence of executive dysfunction with minimal reliable change in his performances from 2015, 2017, and 2022 evaluations. He also has not developed neuroimaging evidence of FTD. This case illustrates the importance of monitoring individuals over time and incorporating neuroimaging data into the diagnosis. We believe this Veteran's presentation is most consistent with what has been described as phFTD.
行为变异额颞叶痴呆（bvFTD）是一种神经退行性疾病，其特征是行为、认知和日常功能逐渐发生变化。疾病的进展通常导致诊断后 3-5 年内死亡。然而，有报道称一些最初被诊断为 bvFTD 的患者并未进展。这些患者被认为患有表现型变异额颞叶痴呆（phFTD）。本文回顾了一例 68 岁男性退伍军人的单病例研究，该患者于 2010 年被诊断为 bvFTD，但并未随时间进展。对系列神经心理学评估进行了广泛的正常检查，表现出轻微的执行功能障碍证据，其在 2015、2017 和 2022 年评估中的表现变化极小，且无可靠变化。他也没有发展出额颞叶痴呆的神经影像学证据。该病例说明了随着时间的推移对个体进行监测并将神经影像学数据纳入诊断的重要性。我们认为这位退伍军人的表现最符合已描述的 phFTD。
