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Which ante mortem clinical features predict progressive supranuclear palsy pathology?

Gesine Respondek1,2, Carolin Kurz3, Thomas Arzberger2,3,4

  • 1Department of Neurology, Technische Universität München, Munich, Germany.

Movement Disorders : Official Journal of the Movement Disorder Society
|May 14, 2017
PubMed
Summary

Clinical features can help diagnose progressive supranuclear palsy (PSP) and its varied presentations. Identifying specific signs aids in optimizing diagnostic criteria for this challenging neurological disease.

Keywords:
Progressive supranuclear palsyclinical featuresclinico-pathological seriesdiagnosissystematic review

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Area of Science:

  • Neurology
  • Neuroscience
  • Pathology

Background:

  • Progressive supranuclear palsy (PSP) is a neuropathologically defined condition with diverse clinical manifestations.
  • Accurate in-life diagnosis of PSP remains challenging due to its varied presentation.

Purpose of the Study:

  • To identify clinical features and investigations that predict or exclude PSP pathology during life.
  • To optimize clinical diagnostic criteria for PSP.

Main Methods:

  • Systematic literature review of studies published since 1996.
  • Extraction of standardized data from pathologically diagnosed PSP cases and disease controls.
  • Calculation of sensitivity, specificity, and positive predictive value for key clinical features.

Main Results:

  • Literature review identified predictive clinical features in ocular motor, postural, akinesia, and cognitive domains.
  • No validated biomarker or genetic feature reliably predicts definite PSP.
  • Analysis of 206 PSP cases and 231 controls revealed clinical features predicting PSP pathology, including non-Richardson's syndrome phenotypes.

Conclusions:

  • PSP exhibits significant clinical variability, with frequent presentations beyond Richardson's syndrome.
  • Specific features of variant PSP phenotypes can enhance diagnostic accuracy.
  • Optimizing clinical diagnosis requires recognizing these specific, sensitive, and specific features.