Molecular Imaging of Extrapyramidal Movement Disorders With Dementia: The 4R Tauopathies.

Molecular Imaging of Extrapyramidal Movement Disorders With Dementia: The 4R Tauopathies.
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DOI:
10.1053/j.semnuclmed.2020.12.003
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发表时间:
2021-05
影响因子:
4.9
通讯作者:
Frey KA
Frey KA
中科院分区:
医学2区
文献类型:
--
作者:
Frey KA

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两种病理学上不同的神经退行性疾病,进行性核上性麻痹和皮质基底节变性,共有tau蛋白的共同沉积物,其在分子和超微结构上均不同于阿尔茨海默病的常见tau沉积物。这些疾病中的蛋白质病的特征在于4R tau蛋白质的聚集。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如帕金森病或额颞叶变性亚型相混淆。这两种4R tau疾病都没有有效的治疗方法,虽然有新兴的分子成像方法可以在疾病过程的早期识别患者,但目前还没有可靠的敏感和特异性方法来诊断生活中的疾病。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记物成像研究的各个方面。提出了更精确的分子成像方法的未来发展。
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.
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