Department of Radiology, Mayo Clinic, Rochester, Minnesota, USA.
Curr Opin Neurol. 2023 Aug 1;36(4):283-290. doi: 10.1097/WCO.0000000000001175. Epub 2023 Jun 12.
The aim of this study was to discuss how recent work has increased our understanding of progressive supranuclear palsy (PSP) and corticobasal degeneration (CBD). The investigation of large and autopsy-confirmed cohorts, imaging modalities to assess different aspects of pathophysiology, clinical phenotypes and the application of advanced machine learning techniques, have led to recent advances in the field that will be discussed.
Literature over the past 18 months will be discussed under the following themes: studies assessing how different neuroimaging modalities can improve the diagnosis of PSP and CBD from other neurodegenerative and parkinsonian disorders, including the investigation of pathological targets such as tau, iron, neuromelanin and dopamine and cholinergic systems; work improving our understanding of clinical, neuroanatomical and pathological heterogeneity in PSP and CBD; and work using advanced neuroimaging tools to investigate patterns of disease spread, as well as biological mechanisms potentially driving spread through the brain in PSP and CBD.
The findings help improve the imaging-based diagnosis of PSP and CBD, allow more targeted prognostic estimates for patients accounting for phenotype or disease, and will aid in the development of appropriate and better-targeted disease biomarkers for clinical treatment trials.
综述目的：本研究旨在讨论最近的研究工作如何提高我们对进行性核上性麻痹（PSP）和皮质基底节变性（CBD）的认识。通过对大型尸检证实队列的研究、评估不同病理生理方面的成像方式、临床表型以及先进机器学习技术的应用，推动了该领域的最新进展，本文将对此进行讨论。
最新发现：将在以下主题下讨论过去 18 个月的文献：评估不同神经影像学方式如何改善 PSP 和 CBD 与其他神经退行性和帕金森病的鉴别诊断，包括对 tau、铁、神经黑色素和多巴胺及胆碱能系统等病理靶标的研究；旨在提高对 PSP 和 CBD 临床、神经解剖和病理异质性的认识的研究；以及利用先进的神经影像学工具来研究疾病传播模式以及可能在 PSP 和 CBD 中通过大脑传播的生物学机制的研究。
总结：这些发现有助于提高基于影像学的 PSP 和 CBD 诊断，为患者提供更有针对性的表型或疾病预后估计，并将有助于开发适当和更有针对性的疾病生物标志物，用于临床治疗试验。
