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Progressive supranuclear palsy.

Nikolaos Giagkou1, Günter U Höglinger2, Maria Stamelou3

  • 1Parkinson's Disease and Movement Disorders Department, HYGEIA Hospital, Athens, Greece.

International Review of Neurobiology
|November 30, 2019
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Summary
This summary is machine-generated.

Progressive supranuclear palsy (PSP) is a rare neurodegenerative disease. While treatments are limited, new diagnostic criteria and ongoing research offer hope for earlier detection and effective therapies for PSP.

Keywords:
4R-tauopathyAtypical parkinsonismProgressive supranuclear palsyRichardson’s syndromeTauopathy

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Area of Science:

  • Neuroscience
  • Neurology
  • Pathology

Background:

  • Progressive supranuclear palsy (PSP) is a tauopathy characterized by 4-repeat tau deposition.
  • Richardson's syndrome (RS) is a primary PSP phenotype, featuring vertical gaze palsy, falls, and frontal dementia.
  • PSP presents with diverse clinical phenotypes beyond RS, including parkinsonism and frontotemporal dementia.

Purpose of the Study:

  • To review the phenotypic variability of PSP.
  • To discuss challenges in early diagnosis due to lack of biomarkers.
  • To summarize current therapeutic limitations and future research directions.

Main Methods:

  • Review of existing literature on PSP and its phenotypes.
  • Analysis of clinical diagnostic criteria evolution.
  • Assessment of current and past therapeutic trial outcomes.

Main Results:

  • PSP exhibits significant clinical heterogeneity, complicating diagnosis.
  • Richardson's syndrome is one of several recognized PSP phenotypes.
  • Current treatments for PSP are symptomatic and inadequate.

Conclusions:

  • Revised diagnostic criteria aim for earlier PSP identification.
  • Despite trial failures, ongoing research pursues neuroprotective strategies for PSP.
  • Development of validated biomarkers and effective treatments remains critical for PSP management.