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Progressive supranuclear palsy

Last reviewed: 21 Oct 2024
Last updated: 16 May 2024

Summary

Definition

History and exam

Key diagnostic factors

  • postural instability with unexplained (often backward) falls
  • abnormal vertical eye movements
  • parkinsonism
  • cognitive dysfunction
  • rapidly progressive symptoms
Full details

Other diagnostic factors

  • speech problems
  • dysphagia
  • behavior problems
  • facial and/or limb dystonia
  • apraxia
  • sleep disturbance
  • constipation
  • urinary symptoms
  • myoclonus
  • pyramidal signs
Full details

Risk factors

  • increasing age (>40 years)
  • exposure to chromate and phosphate
Full details

Diagnostic tests

1st tests to order

  • brain MRI
  • levodopa trial
Full details

Tests to consider

  • polysomnography
  • tilt table test/active stand test
  • biopsy of bone marrow or skin
  • PCR for Tropheryma whippelii
  • 24-hour urinary copper
  • paraneoplastic antibody panel
  • CSF biomarkers for prion disease
  • genetic testing
  • PET imaging
  • CSF biomarkers for Alzheimer disease
Full details

Treatment algorithm

ONGOING

all patients

Contributors

Authors

Nahid Olfati, MD

Postdoctoral Researcher

UC San Diego Department of Neurosciences

La Jolla

CA

Disclosures

NO declares that she has no competing interests.

Hamidreza Ghodsi, MD

Postdoctoral Researcher

UC San Diego Department of Neurosciences

La Jolla

CA

Disclosures

HG declares that he has no competing interests.

Irene Litvan, MD, FAAN, FANA, MPhil

Director of the Parkinson and Other Movement Disorders Center

UC San Diego Department of Neurosciences

La Jolla

CA

Disclosures

IR's research is supported by: National Institutes of Health grants: the Michael J Fox Foundation; the Parkinson Foundation; the Lewy Body Association; CurePSP; Roche; Abbvie; Biogen; Centogene; EIP-Pharma; Biohaven Pharmaceuticals; Novartis; United Biopharma; and UCB. She is a member of the scientific advisory board for Amydis, but does not receive funds. She receives her salary from the University of California San Diego and as Chief Editor of Frontiers in Neurology.

Peer reviewers

James H. Bower, MD, MSc

Professor of Neurology

Chair of Division of Movement Disorders

Mayo Clinic

Rochester

MN

Disclosures

JHB receives research support from Novartis, Inc.

Peter Nigel Leigh, BSc, MB, BS, PhD, FRCP, FMedSci

Professor of Neurology

Brighton and Sussex Medical School

Honorary Consultant Neurologist

King’s College Hospital NHS Foundation Trust and University Hospitals Sussex NHS Foundation Trust

Chair

PSP Association Research Committee

East Sussex

UK

Disclosures

PNL declares that he has no competing interests.

  • Progressive supranuclear palsy images
  • Differentials

    • Idiopathic Parkinson disease (PD)
    • Corticobasal degeneration (CBD)
    • Multiple system atrophy (MSA)
    More Differentials
  • Guidelines

    • ​Best practices in the clinical management of progressive supranuclear palsy and corticobasal syndrome
    • Clinical diagnosis of progressive supranuclear palsy
    More Guidelines
  • Videos

    Tendency to fall on pull test

    Slow vertical saccades

    More videos
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