Progressive supranuclear palsy

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Progressive Supranuclear Palsy (PSP) is also known as Steele-Richardson-Olszewski syndrome after the authors of the 1964 description of the condition.[1] Patients fitting its characterstic features are not as rare (1/100000) as originally described. This is no doubt because of misdiagnoses, typically as Parkinson's Disease, although few experienced at making that diagnosis will be caught out, as they will tend to reserve the diagnosis.

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  • The characteristic gaze palsy is NOT of upward gaze, even though this may be the first to develop and may be a feature of ageing.
  • Voluntary downwards or lateral gaze palsies are characteristic!
  • Postural instability, often more prominent than in others with Parkinsonism. Tremor is rare.
  • Features of a subcortical dementia
    • Personality changes
      • Apathy
      • Cantankerous
      • Irritability
  • Emotional lability
    • Suddenly laughing or crying for no reason
    • Spontaneous angry outbursts
  • Voluntary gaze palsy
    • Care:Paresis of voluntary upwards gaze is common, and more characteristic of generalised white matter damage than PSP.
    • Loss of eye contact in conversation
  • Dysphagia
  • Speech slurring

Aetiology

Unknown

Subtypes

Subtypes may exist:[2]

  • Richardson's syndrome (RS)
    • 50% of cases
      • Early onset of postural instability and falls
      • Supranuclear vertical gaze palsy
      • Cognitive dysfunction
  • PSP-parkinsonism (PSP-P)
    • 33% have Parkinsonism with:
      • Asymmetric onset
      • Tremor
      • Moderate initial therapeutic response to levodopa

References

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