进行性核上性麻痹：我们了解多少？

Title:Progressive Supranuclear Palsy: What Do We Know About it?

Volume: 22 Issue: 10

Author(s): Ling Long, Xiao-Dong Cai, Xiao-Bo Wei, Jin-Chi Liao, Yun-Qi Xu, Hui-Min Gao, Xiao-Hong Chen and Qing Wang

Affiliation:

关键词: 诊断标准，微管结合tau蛋白，神经成像，进行性核上性麻痹，发病原

摘要: Progressive supranuclear palsy (PSP) is a progressive tauopathy characterized by supranuclear ophthalmoplegia, pseudobulbar palsy, dysarthria, axial rigidity, frontal lobe dysfunction, and dementia. The typical pathology includes neuronal loss, gliosis and microtubule-associated protein tau (MAPT)-positive inclusions in neurons and glial cells, primarily in basal ganglia, brainstem and cerebellum. The pathogenesis of PSP is not yet completely understood; however, there are several hypotheses. This article reviews the present knowledge about PSP, and the concepts underlying mitochondrial dysfunction, lipoperoxidation, and gene mutations. The clinical features of PSP are also discussed; these include vertical gaze palsy, pseudobulbar palsy, aphasia, dysarthria, axial rigidity, and neuropsychiatric symptoms, such as amnesia, irritability, loss of interest, and dementia. In terms of diagnosis, there is considerable interest in neuroimaging for detecting PSP; therefore, neuroimaging techniques such as magnetic resonance imaging (MRI) and [18F]- fluorodeoxyglucose positron-emission tomography (FDG-PET) are reviewed. A definitive diagnosis of PSP depends on pathology, and the introduction of new clinical subtypes challenges presents the widely adopted diagnosis criteria. PSP treatments such as serotonin antagonists, α2 receptor antagonists, and coenzyme Q10 are also discussed. There is no curative therapy for PSP; all of the available treatments are palliative.

Cite this article as:

Ling Long, Xiao-Dong Cai, Xiao-Bo Wei, Jin-Chi Liao, Yun-Qi Xu, Hui-Min Gao, Xiao-Hong Chen and Qing Wang , Progressive Supranuclear Palsy: What Do We Know About it?, Current Medicinal Chemistry 2015; 22 (10) . https://dx.doi.org/10.2174/0929867322666150302170552