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What is progressive supranuclear palsy (PSP)?

This rare brain disorder can cause progressive problems with movement and emotions. It can't be cured, but the symptoms can be managed.

Progressive supranuclear palsy (PSP) is a rare disorder that causes serious and permanent damage to cells in the brain stem, according to the National Institutes of Health (NIH). This progressive damage causes increasing difficulty controlling body movement, walking, balance, swallowing, speaking and eye movement.

Symptoms

The symptoms of PSP appear most often in the early 60s, but they can start as early as the 40s, according to CurePSP. They can vary greatly from one person to the next. Because they often resemble the symptoms of Parkinson's or Alzheimer's disease, early PSP is often misdiagnosed.

Some of the more common symptoms of PSP include:

  • Balance difficulties, such as unsteady walking or sudden, unexplained falls.
  • Blurred vision.
  • Trouble controlling eye and eyelid movement, leading to involuntary closing of the eyes, prolonged or infrequent blinking, or difficulty opening the eyes or looking down.
  • Slurred speech.
  • Difficulty swallowing.
  • Mood changes, such as depression, apathy, irritability and emotional outbursts.
  • Mild dementia or forgetfulness.

PSP symptoms increase the risks for choking, pneumonia caused by inhaling food particles, and broken bones and head injuries from falls. Pneumonia is the most common cause of death in people with PSP, according to the NIH. However, the NIH notes that with good attention to medical and nutritional needs, PSP patients can live a decade or more beyond when they first experienced symptoms.

Seeking relief

PSP cannot be slowed or cured, but the symptoms can be managed.

In some people slowness, stiffness and balance problems may respond to drugs used to treat Parkinson's disease. Antidepressant medications have also been mildly successful.

Weighted walking aids may also be helpful, because the balance problems caused by PSP often cause people to fall backward. Bifocals or special glasses called prisms are sometimes prescribed to compensate for difficulty looking down. As symptoms progress, a feeding tube may be used to reduce the risk of choking and pneumonia.

Searching for answers

Research continues on the causes of and treatments for PSP.

Studies have shown that people with the disease have abnormal clumps of proteins in their brains, which lead to the death of brain cells. Research has also shown that almost everyone with PSP has a variation in the same gene, and that environment probably determines which of the people with this gene variant develop PSP. Other gene variants also have been found to be more common in people with PSP than in those without it.

For more information on PSP research, visit the CurePSP website, www.psp.org.

Reviewed 6/25/2021

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