Progressive supranuclear palsy

  • Definition
    • Progressive supranuclear palsy (PSP) is a movement disorder that occurs from damage to certain nerve cells in the brain.

  • Alternative Names
    • Dementia - nuchal dystonia; Richardson-Steele-Olszewski syndrome; Palsy - progressive supranuclear

  • Causes
    • PSP is a condition that causes symptoms similar to those of Parkinson disease.

      It involves damage to many cells of the brain. Many areas are affected, including the part of the brainstem where cells that control eye movement are located. The area of the brain that controls steadiness when you walk is also affected. The frontal lobes of the brain are also affected, leading to personality changes.

      The cause of the damage to the brain cells is unknown. PSP gets worse over time.

      People with PSP have deposits in brain tissues that look like those found in people with Alzheimer disease. There is a loss of tissue in most areas of the brain and in some parts of the spinal cord.

      The disorder is most often seen in people over 60 years old, and is somewhat more common in men.

  • Symptoms
    • Symptoms may include any of the following:

      • Loss of balance, repeated falls
      • Lunging forward when moving, or fast walking
      • Bumping into objects or people
      • Changes in expressions of the face
      • Deeply lined face
      • Eye and vision problems such as different sized pupils, difficulty moving the eyes (supranuclear opthalmoplegia), lack of control over the eyes, problems keeping the eyes open
      • Difficulty swallowing
      • Tremors, jaw or face jerks or spasms
      • Mild-to-moderate dementia
      • Personality changes
      • Slow or stiff movements
      • Speech difficulties, such as low voice volume, not able to say words clearly, slow speech
      • Stiffness and rigid movement in the neck, middle of the body, arms, and legs
  • Exams and Tests
    • An exam of the nervous system (neurologic examination) may show:

      • Dementia that is getting worse
      • Difficulty walking
      • Limited eye movements, especially up and down movements
      • Normal vision, hearing, feeling, and control of movement
      • Stiff and uncoordinated movements like those of Parkinson disease

      The health care provider may do the following tests to rule out other diseases:

  • Treatment
    • The goal of treatment is to control symptoms. There is no known cure for PSP.

      Medicines such as levodopa may be tried. These drugs raise the level of a brain chemical called dopamine. Dopamine is involved in the control of movement. The medicines may reduce some symptoms, such as rigid limbs or slow movements for a time. But they are usually not as effective as they are for Parkinson disease.

      Many people with PSP will eventually need around-the-clock care and monitoring as they lose brain functions.

  • Outlook (Prognosis)
    • Treatment sometimes can reduce symptoms for a while, but the condition will get worse. Brain function will decline over time. Death commonly occurs in 5 to 7 years.

      Newer drugs are being studied to treat this condition.

  • Possible Complications
    • Complications of PSP include:

      • Blood clot in veins (deep vein thrombosis) due to limited movement
      • Injury from falling
      • Lack of control over vision
      • Loss of brain functions over time
      • Pneumonia due to trouble swallowing
      • Poor nutrition (malnutrition)
      • Side effects from medicines
  • When to Contact a Medical Professional
    • Call your provider if you often fall, and if you have a stiff neck/body, and vision problems.

      Also, call if a loved one has been diagnosed with PSP and the condition has declined so much that you can no longer care for the person at home.

  • References
    • Jankovic J. Parkinson disease and other movement disorders. In: Daroff RB, Jankovic J, Mazziotta JC, Pomeroy SL, eds. Bradley's Neurology in Clinical Practice. 7th ed. Philadelphia, PA: Elsevier; 2016:chap 96.

      Ling H. Clinical approach to progressive supranuclear palsy. J Mov Disord. 2016;9(1):3-13. PMID: 26828211