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:
Keywords:
Diagnosis criteria, microtubule-associated protein tau, neuroimaging, progressive supranuclear palsy, pathogenesis.
Abstract: 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.
