Memory and Aging Center, Department of Neurology, Weill Institute for Neurosciences, University of California, San Francisco, CA, USA.
Neurotherapeutics. 2021 Apr;18(2):728-752. doi: 10.1007/s13311-021-01101-x. Epub 2021 Aug 13.
Frontotemporal dementia encompasses a group of clinical syndromes defined pathologically by degeneration of the frontal and temporal lobes. Historically, these syndromes have been challenging to diagnose, with an average of about three years between the time of symptom onset and the initial evaluation and diagnosis. Research in the field of neuroimaging has revealed numerous biomarkers of the various frontotemporal dementia syndromes, which has provided clinicians with a method of narrowing the differential diagnosis and improving diagnostic accuracy. As such, neuroimaging is considered a core investigative tool in the evaluation of neurodegenerative disorders. Furthermore, patterns of neurodegeneration correlate with the underlying neuropathological substrates of the frontotemporal dementia syndromes, which can aid clinicians in determining the underlying etiology and improve prognostication. This review explores the advancements in neuroimaging and discusses the phenotypic and pathologic features of behavioral variant frontotemporal dementia, semantic variant primary progressive aphasia, and nonfluent variant primary progressive aphasia, as seen on structural magnetic resonance imaging and positron emission tomography.
额颞叶痴呆包括一组临床综合征，这些综合征在病理学上由额叶和颞叶的退化定义。从历史上看，这些综合征的诊断一直具有挑战性，从症状出现到首次评估和诊断的平均时间约为三年。神经影像学领域的研究揭示了各种额颞叶痴呆综合征的许多生物标志物，这为临床医生提供了一种缩小鉴别诊断范围和提高诊断准确性的方法。因此，神经影像学被认为是评估神经退行性疾病的核心研究工具。此外，神经退行性变的模式与额颞叶痴呆综合征的潜在神经病理学基础相关，这可以帮助临床医生确定潜在病因并改善预后。这篇综述探讨了神经影像学的进展，并讨论了在结构磁共振成像和正电子发射断层扫描上观察到的行为变异型额颞叶痴呆、语义变异型原发性进行性失语症和非流利变异型原发性进行性失语症的表型和病理特征。
