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Progressive Supranuclear Palsy

Progressive supranuclear palsy (PSP) is a complex condition that affects the brain. Palsy is a disorder that results in weakness of certain muscles. Supranuclear refers to the region of the brain affected by the disorder—the section above two small areas called nuclei. Progressive means that the condition’s symptoms will keep worsening over time.

PSP affects your ability to walk normally by impairing your balance. PSP also affects the muscles controlling your eyes, making it difficult to focus and see things clearly.

Facts about progressive supranuclear palsy

Progressive supranuclear palsy is rare. Only about one in 100,000 Americans has the disorder. PSP may be easily mistaken for Parkinson's disease, which is much more common, because the conditions share many of the same symptoms. But with PSP, speech and difficulty swallowing are usually affected more significantly than with Parkinson's disease. Problems moving the eyes, especially problems looking downward, are also more common in PSP. And unlike people with Parkinson's disease, people with PSP are more likely to lean backward (and fall backward) rather than forward.

PSP is more common in men than women. Most of the time, it affects people in late middle age or older. And although experts basically understand how PSP happens, they don't understand why it happens. PSP occurs when brain cells in an area of the brain stem become damaged, but how and why these cells are damaged isn't clear.

Although PSP isn't fatal, symptoms do continue to worsen and it can't be cured. Complications that result from worsening symptoms, such as pneumonia (from breathing in food particles while choking during eating), can be life-threatening.

Symptoms

Symptoms of PSP tend to start out subtly. Then over time they become more noticeable and severe. Often, the first sign is a problem with balance while walking. You may fall a lot or find that you feel a bit rigid or uncomfortable when you walk.

These are also early signs of PSP:

  • Becoming more forgetful and cranky

  • Having unusual emotional outbursts, like crying or laughing at unexpected times

  • Becoming angry for no real reason

  • Tremors in the hands

  • Difficulty controlling eye movements

  • Blurred vision

  • Slurred speech

  • Difficulty swallowing

  • Dementia

  • Depression

  • Difficulty directing your eyes where you want them to go

  • Inability to control the eyelids, such as unwanted blinking or being unable to open your eyes

  • Difficulty holding someone's gaze

Diagnosis

A careful evaluation of symptoms can diagnose PSP. But PSP is difficult to diagnose in its early stages. Often, it may mimic either Parkinson's disease or an inner ear infection because balance is so affected—diagnosis usually includes ruling out other conditions.

Balance problems and changes in gait are the clearest symptoms that can identify PSP, particularly when combined with an inability to control or move the eyes.

Treatment

No medication or procedure is available to cure PSP or completely control its symptoms. But strategies and methods can help manage many of the symptoms.

To improve balance and improve flexibility of the muscles, medications used to treat Parkinson's disease may be effective. These include the drug levodopa, which may sometimes be used in conjunction with other drugs. Some of the older types of antidepressants, such as amitriptyline, fluoxetine, and imipramine, can also provide some symptom relief.

If you have PSP, you may be able to use certain aids to make life easier. Special glasses with prisms may improve your vision. A weighted tool that helps you walk more easily can prevent you from falling backward. Physical therapy and exercise may slightly improve flexibility in some people.

When symptoms are advanced and swallowing becomes too difficult, you may need a feeding tube—a tube that goes from an opening made in the skin of your abdomen into the stomach—to provide you with needed nutrition.

Calling the doctor

Although it's easy to try to brush off initial symptoms as being a little clumsy or maybe having an ear infection, it's a good idea to see a doctor at the earliest sign of symptoms, especially if you have problems with your eyes or vision.

Complications

PSP can cause serious complications when symptoms affect your ability to swallow. You could easily choke on food or breathe food into your lungs. And being more likely to fall increases the risk of suffering a serious injury to the head or breaking a bone.

Key points to remember

Experts are still working to understand more about progressive supranuclear palsy and find more effective ways to treat it. Although the disease itself isn't life-threatening, its complications can be. Be aware of suspicious symptoms and talk with your doctor if you notice any problems with your eyes, vision, or balance.

Online Medical Reviewer: Fraser, Marianne, MSN, RN
Online Medical Reviewer: Sohrabi, Farrokh, MD
Date Last Reviewed: 6/16/2012
© 2000-2014 Krames StayWell, 780 Township Line Road, Yardley, PA 19067. All rights reserved. This information is not intended as a substitute for professional medical care. Always follow your healthcare professional's instructions.
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