Division of Nuclear Medicine and Molecular Imaging, Department of Radiology, The University of Michigan Health System, Ann Arbor, MI.
Semin Nucl Med. 2021 May;51(3):275-285. doi: 10.1053/j.semnuclmed.2020.12.003. Epub 2021 Jan 8.
Two pathologically distinct neurodegenerative conditions, progressive supranuclear palsy and corticobasal degeneration, share in common deposits of tau proteins that differ both molecularly and ultrastructurally from the common tau deposits diagnostic of Alzheimer disease. The proteinopathy in these disorders is characterized by fibrillary aggregates of 4R tau proteins. The clinical presentations of progressive supranuclear palsy and of corticobasal degeneration are often confused with more common disorders such as Parkinson disease or subtypes of frontotemporal lobar degeneration. Neither of these 4R tau disorders has effective therapy, and while there are emerging molecular imaging approaches to identify patients earlier in the course of disease, there are as yet no reliably sensitive and specific approaches to diagnoses in life. In this review, aspects of the clinical syndromes, neuropathology, and molecular biomarker imaging studies applicable to progressive supranuclear palsy and to corticobasal degeneration will be presented. Future development of more accurate molecular imaging approaches is proposed.
两种具有明显不同神经退行性特征的疾病，进行性核上性麻痹和皮质基底节变性，其共同点是存在 Tau 蛋白的沉积，这些 Tau 蛋白在分子和超微结构上与阿尔茨海默病常见的 Tau 沉积物不同。这些疾病中的蛋白病变的特征是 4R Tau 蛋白的纤维状聚集。进行性核上性麻痹和皮质基底节变性的临床表现经常与更常见的疾病相混淆，如帕金森病或额颞叶变性的亚型。这两种 4R Tau 疾病都没有有效的治疗方法，虽然有新的分子成像方法可以更早地识别疾病过程中的患者，但目前还没有可靠的、敏感的和特异性的方法来进行生前诊断。在这篇综述中，将介绍适用于进行性核上性麻痹和皮质基底节变性的临床综合征、神经病理学和分子生物标志物成像研究的各个方面。提出了未来发展更准确的分子成像方法的建议。
