Progressive Supranuclear Palsy (PSP) or Steele-Richardson-Olszewski syndrome

  • a rare degenerative disease.
  • involves degernatation and death of areas of the  brain.
  • causes serious and permanent problems with control of gait and balance.
  • The most obvious sign of the disease is compromised eye movement, occuring due to lesions in the area of the brain that coordinates eye movements.

Manifestations:

  1. loss of balance
  2. lunging forward when mobilsing
  3. speed-walking
  4. knocking into objects and people
  5. falls.
  6. personality changes
  7. general slowing of movement

 

Later manifestations:

  1. dementia – loss of inhibition and ability to organize information
  2.  slurring of speech
  3. dysphagia
  4. difficulty moving the eyes, particularly in the vertical direction (accounts for some of the falls experienced by these patients as they are unable to look up or down)
  5. poor eyelid function
  6. contracture of the facial muscles
  7. a backward tilt of the head with stiffening of the neck muscles
  8. sleep disruption

Treatment and Management:

  • symptom management
  • The average age at symptoms onset is 63 and survival from onset averages 7 years with a wide variance.
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