Barker Megan S, Manoochehri Masood, Huey Edward D
Warren Alpert Medical School of Brown University, Department of Psychiatry and Human Behavior; Memory and Aging Program, Butler Hospital, Providence, RI.
R I Med J (2013). 2025 May 1;108(5):17-22.
The behavioral variant of frontotemporal dementia (bvFTD) is a progressive, neurodegenerative disorder, characterized by profound changes in personality, behavior, and social comportment. Diagnosis of bvFTD is challenging, and it is frequently misdiagnosed as an idiopathic psychiatric disorder (e.g., major depressive disorder, bipolar disorder) or another neurodegenerative disease (e.g., Alzheimer's disease dementia). The diagnostic challenge is exacerbated by a lack of reliable in vivo biological markers of disease pathology, which means that, at present, diagnosis relies largely on detailed behavioral and cognitive assessments. In this article, we discuss how clinical diagnostic criteria for bvFTD have evolved over the past three decades, and emphasize the diagnostic uncertainty that can arise when trying to distinguish between bvFTD and primary psychiatric disorders or other neurodegenerative diseases. In highlighting the strengths and limitations of the revised diagnostic criteria, and taking into account current diagnostic predicaments, we provide evidence-based recommendations for clinicians facing this diagnostic question. Finally, we touch on the importance of early (i.e., prodromal) diagnosis, and explain the utility of biomarkers for bvFTD diagnosis, with a nod to exciting research developments in this area.
额颞叶痴呆的行为变异型（bvFTD）是一种进行性神经退行性疾病，其特征是人格、行为和社交行为发生深刻变化。bvFTD的诊断具有挑战性，它经常被误诊为特发性精神障碍（如重度抑郁症、双相情感障碍）或另一种神经退行性疾病（如阿尔茨海默病性痴呆）。由于缺乏可靠的疾病病理学体内生物学标志物，诊断挑战更加严峻，这意味着目前诊断很大程度上依赖于详细的行为和认知评估。在本文中，我们讨论了bvFTD的临床诊断标准在过去三十年中是如何演变的，并强调了在试图区分bvFTD与原发性精神障碍或其他神经退行性疾病时可能出现的诊断不确定性。在突出修订后的诊断标准的优势和局限性，并考虑到当前的诊断困境时，我们为面临这一诊断问题的临床医生提供基于证据的建议。最后，我们提及早期（即前驱期）诊断的重要性，并解释生物标志物在bvFTD诊断中的作用，同时关注该领域令人兴奋的研究进展。
