Evolution of Concepts of Differential Diagnostics in Frontotemporal Dementia

Y. Fedorova
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Abstract

Background:  frontotemporal dementia (FTD) is a group of neurodegenerative diseases, with onset usually in presenile age, the clinical picture is manifested by behavioral disorders and relatively intact cognitive features in the initial disease. In  the early stages of FTD, it is difficult to differentiate this type from other dementias or other mental diseases. The  aim was to analyse recent scientific publications on the problem of differential diagnostics of frontotemporal dementia.  Material and methods:  using the keywords “frontotemporal dementia”, “frontotemporal lobar degeneration”, “differential diagnosis of frontotemporal dementia”, “behavioral variant of frontotemporal dementia”, selected and analyze publications for the last two decades. Results: the behavioral variant of FTD (bv-FTD) is the most common form of FTD, accounting for 50% of all cases of FTD, and especially in cases with early onset. Predominantly, this variant of FTD presents diagnostic difficulties, due to the limited accuracy of neuroimaging examinations and the lack of specific biomarkers. The clinical symptoms of bv-FTD are characterized by considerable overlap with symptoms of neurodegenerative diseases and mental diseases, such as schizophrenia, bipolar affective disorder, obsessive-compulsive disorder, and personality disorders. Conclusion: the diagnosis of FTD at the initial stage of the disease is problematic and difficult, the sensitivity and specificity of almost all diagnostic methods increase as the disease progresses. This literature review highlights some of the diagnostic methods that can be used in suspected cases of FTD and informs about the differential diagnostics recommendations that have been developed to improve the accuracy of FTD diagnosis.
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额颞叶痴呆症鉴别诊断概念的演变
背景:额颞叶痴呆(frontotemporal dementia,FTD)是一组神经退行性疾病,发病年龄多在青春期前,临床表现为行为障碍,发病初期认知功能相对完整。在 FTD 的早期阶段,很难将其与其他痴呆症或其他精神疾病区分开来。本研究旨在分析近期有关额颞叶痴呆症鉴别诊断问题的科学出版物。 材料与方法:以 "额颞叶痴呆"、"额颞叶变性"、"额颞叶痴呆的鉴别诊断"、"额颞叶痴呆的行为变异 "为关键词,选取并分析了近二十年来的出版物。结果:额颞叶痴呆的行为变异型(bv-FTD)是额颞叶痴呆最常见的形式,占所有额颞叶痴呆病例的50%,尤其是在早发病例中。由于神经影像学检查的准确性有限且缺乏特异性生物标志物,这种变异型 FTD 主要给诊断带来困难。bv-FTD 的临床症状与神经退行性疾病和精神疾病(如精神分裂症、双相情感障碍、强迫症和人格障碍)的症状有很大的重叠。结论:FTD 在发病初期的诊断是有问题和困难的,随着病情的发展,几乎所有诊断方法的敏感性和特异性都会增加。本文献综述重点介绍了可用于 FTD 疑似病例的一些诊断方法,并介绍了为提高 FTD 诊断的准确性而提出的鉴别诊断建议。
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