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Clinical features

Authoring team

Presenting symptoms of the Steele Richardson Syndrome include:

  • unexplained falls
  • mental disturbances
  • slurring of speech

On examination of the eyes:

  • both eyes are affected
  • pupils are often unequal but fixed
  • generally there is no diplopia
  • reflex eye movements e.g. on neck flexion or extension are intact

Elements of the clinical syndrome include:

  • mild dementia
  • mask-like facies
  • pseudobulbar palsy
  • mild extrapyramidal rigidity may be present in the limbs
  • tendon reflexes are often increased
  • there is often a positive Babinkski sign

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