Department of Neurology, Zvolen Hospital, Kuzmányho nábrežie 28, 960 01, Zvolen, Slovakia.
Neurol Sci. 2021 Dec;42(12):4927-4936. doi: 10.1007/s10072-021-05601-8. Epub 2021 Sep 17.
Progressive supranuclear palsy (PSP) is a progressive atypical parkinsonian syndrome characterised by postural instability, supranuclear ophthalmoplegia, dysarthria, dysphagia, executive dysfunction and other features. This clinical presentation represents the classic PSP-Richardson syndrome (PSP-RS). However, several other clinical subtypes have been recognised, including PSP-parkinsonism (PSP-P), probably the second most common PSP variant. Unlike PSP-RS, PSP-P often presents with an asymmetric onset, tremor and a moderate initial response to levodopa, especially during the first years of the disease, thus resembling Parkinson's disease (PD). It runs a more favourable course, but over time, PSP-P may evolve clinically into PSP-RS. Therefore, it may seem that PSP-P stands clinically between PD and PSP. There are several peculiarities that can distinguish PSP-P from these entities. As there is lack of systematic reviews on PSP-P in the literature, we decided to summarise all the necessary data about the epidemiology, clinical picture, neuroimaging, genetics and other aspects of this PSP variant in order to provide complete information for the reader.
进行性核上性麻痹(PSP)是一种进行性非典型帕金森综合征,其特征为姿势不稳、核上性眼肌麻痹、构音障碍、吞咽困难、执行功能障碍和其他特征。这种临床表现代表了经典的 PSP-Richardson 综合征(PSP-RS)。然而,已经认识到其他几种临床亚型,包括 PSP-帕金森病(PSP-P),可能是第二常见的 PSP 变体。与 PSP-RS 不同,PSP-P 通常以不对称起病、震颤和左旋多巴的中度初始反应为特征,尤其是在疾病的最初几年,因此类似于帕金森病(PD)。它的病程更有利,但随着时间的推移,PSP-P 可能会在临床上演变为 PSP-RS。因此,PSP-P 似乎在临床上介于 PD 和 PSP 之间。有几个特点可以将 PSP-P 与这些实体区分开来。由于文献中缺乏关于 PSP-P 的系统评价,我们决定总结有关该 PSP 变体的流行病学、临床表现、神经影像学、遗传学和其他方面的所有必要数据,以便为读者提供完整的信息。