UC San Diego, Department of Neurosciences, La Jolla, CA, USA.
Department of Neuroscience, Mayo Clinic, Jacksonville, FL, USA.
Adv Exp Med Biol. 2021;1281:151-176. doi: 10.1007/978-3-030-51140-1_11.
Progressive supranuclear palsy (PSP) and corticobasal degeneration (CBD) are neurodegenerative tauopathies with neuronal and glial lesions composed of tau that is composed predominantly of isomers with four repeats in the microtubule-binding domain (4R tau). The brain regions vulnerable to pathology in PSP and CBD overlap, but there are differences, particularly with respect to distribution of neuronal loss, the relative abundance of neuronal and glial lesions, the morphologic features of glial lesions, and the frequency of comorbid pathology. Both PSP and CBD have a wide spectrum of clinical manifestations, including disorders of movement and cognition. Recognition of phenotypic diversity in PSP and CBD may improve antemortem diagnostic accuracy, which tends to be very good for the most common presentation of PSP (Richardson syndrome), but poor for the most characteristic presentation of CBD (corticobasal syndrome: CBS). Development of molecular and imaging biomarkers may improve antemortem diagnostic accuracy. Currently, multidisciplinary symptomatic and supportive treatment with pharmacological and non-pharmacological strategies remains the standard of care. In the future, experimental therapeutic trials will be important to slow disease progression.
进行性核上性麻痹（PSP）和皮质基底节变性（CBD）是神经退行性tau 病，其神经元和神经胶质病变由主要由微管结合域中具有四个重复的异构体（4R tau）组成的 tau 组成。PSP 和 CBD 中易发生病变的脑区重叠，但存在差异，特别是在神经元丢失的分布、神经元和神经胶质病变的相对丰度、神经胶质病变的形态特征以及共病病理的频率方面。PSP 和 CBD 都有广泛的临床表现，包括运动和认知障碍。认识 PSP 和 CBD 的表型多样性可能会提高生前诊断的准确性，这对 PSP 最常见的表现（Richardson 综合征）非常有效，但对 CBD 最典型的表现（皮质基底节综合征：CBS）则效果不佳。分子和成像生物标志物的发展可能会提高生前诊断的准确性。目前，多学科的对症和支持治疗，包括药物和非药物策略，仍然是标准的治疗方法。在未来，实验性治疗试验对于减缓疾病进展将非常重要。
