Progressive Supranuclear Palsy
What is progressive supranuclear palsy?
Progressive supranuclear palsy (PSP) is a complex condition that affects the brain.
- Progressive means that the condition’s symptoms will keep worsening over time.
- Supranuclear refers to the region of the brain affected by the disorder — the section above 2 small areas called nuclei.
- 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, making it difficult to focus and see things clearly.
Progressive supranuclear palsy is rare. It may be easily mistaken for Parkinson disease, which is much more common and has similar symptoms. But with PSP, speech and difficulty swallowing are usually affected more significantly than with Parkinson disease. Problems moving the eyes, especially problems 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.
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.
What causes progressive supranuclear palsy?
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.
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
- 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 evaluation 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 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.
How is progressive supranuclear palsy treated?
While there is no medicine or procedure available to cure PSP or completely control its symptoms, there are strategies and methods that can help manage many of the symptoms.
To improve balance and improve flexibility of the muscles, medicines used to treat Parkinson disease may be effective. These include the medicine 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 relieve symptoms.
If you have PSP, you may be able to use certain aids to make life easier. For example:
- 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 hard, you may need a feeding tube. This tube goes from an opening made in the skin of your abdomen into the stomach and provides you with needed nutrition.
What are the 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 increases the risk of suffering a serious injury to the head or breaking a bone.
Living with supranuclear palsy
Although there is no known cure, 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. PSP is not fatal but it is important that you do not breathe in food particles (aspirate) while you are eating because it could be life threatening.
When should I call my healthcare provider?
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.
Always seek advice from your healthcare provider if you or your caregiver notice sudden or significant changes in your symptoms.
Key points about supranuclear palsy
- 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 healthcare provider if you notice any problems with your eyes, vision, or balance.
Tips to help you get the most from a visit to your healthcare provider:
- Know the reason for your visit and what you want to happen.
- Before your visit, write down questions you want answered.
- Bring someone with you to help you ask questions and remember what your provider tells you.
- At the visit, write down the name of a new diagnosis, and any new medicines, treatments, or tests. Also write down any new instructions your provider gives you.
- Know why a new medicine or treatment is prescribed, and how it will help you. Also know what the side effects are.
- Ask if your condition can be treated in other ways.
- Know why a test or procedure is recommended and what the results could mean.
- Know what to expect if you do not take the medicine or have the test or procedure.
- If you have a follow-up appointment, write down the date, time, and purpose for that visit.
- Know how you can contact your provider if you have questions.
April 08, 2017
Progressive supranuclear palsy (PSP), Up To Date
Dozier, Tennille, RN, BSN, RDMS,Shelat, Amit, MD