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Progressive supranuclear palsy
Other Resources UpToDate PubMed

Progressive supranuclear palsy

Contributors: Jamie Adams MD, Richard L. Barbano MD, PhD
Other Resources UpToDate PubMed

Synopsis

Progressive supranuclear palsy is a neurodegenerative disorder caused by destruction of brain cells, primarily in the brain stem, and accumulation of tau protein. Average age of onset is 60-65 years, and it is more common in men. Symptoms include parkinsonism (bradykinesia, rigidity, with or without tremor), axial rigidity, gait / postural instability and early falls, eye movement abnormalities, blurry vision, blepharospasm, eyelid opening apraxia, dysarthria, dysphagia, retrocollis, cognitive decline, depression, anxiety, apathy, behavioral changes, and pseudobulbar palsy.

Lack of response to levodopa is common. Prognosis is poor; most patients are significantly disabled within 5 years of diagnosis and die within 10 years of diagnosis.

Codes

ICD10CM:
G23.1 – Progressive supranuclear ophthalmoplegia [Steele-Richardson-Olszewski]

SNOMEDCT:
192976002 – Progressive supranuclear palsy

Look For

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Diagnostic Pearls

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Differential Diagnosis & Pitfalls

  • Parkinson disease
  • Corticobasal syndrome
  • Dementia with Lewy bodies
  • Frontotemporal lobar degeneration
  • Alzheimer disease
  • Dementia and/or Parkinsonism
  • Multiple system atrophy
  • Normal pressure hydrocephalus
  • Creutzfeldt-Jakob disease
  • Paraneoplastic encephalomyelitis
  • Huntington disease
  • Parkinsonism
  • Neurodegeneration with brain iron accumulation
  • Whipple disease
  • Wilson disease
  • Neuroacanthocytosis
  • Tertiary syphilis
  • Vitamin B12 deficiency
  • Human immunodeficiency virus disease / Acquired immune deficiency syndrome dementia
  • Wernicke-Korsakoff syndrome

Best Tests

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Management Pearls

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Therapy

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References

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Last Reviewed:10/18/2018
Last Updated:01/20/2022
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Progressive supranuclear palsy
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A medical illustration showing key findings of Progressive supranuclear palsy : Dysarthria, Gait disturbance, Dysphagia, Axial rigidity
Copyright © 2024 VisualDx®. All rights reserved.