Progressive Supranuclear Palsy

Progressive supranuclear palsy is a brain condition that affects the areas above a collection of cells in the brain stem. This condition gets worse over time and causes difficulty balancing, slowing of movement and abnormal eye movement.

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Understanding Progressive Supranuclear Palsy

What is progressive supranuclear palsy?

Progressive supranuclear palsy (PSP) is a complex condition that affects the brain.

  • Progressive means that symptoms will keep getting worse over time.

  • Supranuclear refers to the part of the brain affected by the disorder. It's the section above small areas called nuclei in the brain stem.

  • Palsy is a disorder that results in weakness of certain muscles.

PSP affects your ability to walk normally by impairing your balance. It also affects the muscles controlling your eyes. It makes it hard to focus and see things clearly.

PSP is rare. It may be easily mistaken for Parkinson disease, which is much more common and has similar symptoms. But with PSP, speech and trouble swallowing are usually affected much more than with Parkinson disease. Problems moving the eyes, especially looking downward, are also more common in PSP. And unlike people with Parkinson 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.

PSP symptoms continue to get worse, and the condition can't be cured. Problems that result from worsening symptoms can be life threatening. An example is pneumonia from breathing in food particles while choking during eating.

What causes progressive supranuclear palsy?

Experts basically understand how PSP happens. But 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.

What are the symptoms of progressive supranuclear palsy?

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.

  • Trouble controlling eye movements.

  • Blurred vision.

  • Slurred speech.

  • Trouble swallowing.

  • Dementia.

  • Depression.

  • Trouble 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.

  • Trouble holding someone's gaze.

How is progressive supranuclear palsy diagnosed?

A careful assessment of symptoms can diagnose PSP. But it is often hard to diagnose in its early stages as it may mimic Parkinson disease or an inner ear infection. This is because balance is so affected by PSP. Diagnosis often includes ruling out other health problems.

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

How is progressive supranuclear palsy treated?

No medicine or procedure can cure PSP or completely control its symptoms. But there are strategies and methods that can help manage many of the symptoms. These include:

  • Certain medicines. Medicines used to treat Parkinson’s disease may improve balance and flexibility of the muscles. These include levodopa, which may be used along with other medicines. Some of the older types of antidepressants, such as amitriptyline, fluoxetine, and imipramine, can also help ease symptoms.

  • Special glasses with prisms. These may improve your vision.

  • A weighted tool that helps you walk more easily. It can prevent you from falling backward.

  • Physical therapy and exercise. These may slightly improve flexibility in some people.

Feeding tube. You may need a feeding tube if your symptoms are advanced and swallowing becomes too hard. This tube goes from an opening in the skin of your abdomen into the stomach. It provides you with needed nutrition.

What are the possible complications of progressive supranuclear palsy?

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 raises the risk of suffering a serious injury to the head or breaking a bone.

Living with progressive supranuclear palsy

There is no known cure for PSP. But medicines and devices can help you live with the symptoms. Work with your healthcare provider to find ways to make walking safer and improve your vision. Also, it is important that you do not breathe in food particles (aspirate) while you are eating. It could be life threatening.

When should I call my healthcare provider?

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

Always seek advice from your healthcare provider if you or your caregiver notice sudden or major changes in your symptoms.

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