What Is Progressive Supranuclear Palsy?
Progressive supranuclear palsy, or PSP, is a rare neurodegenerative disease that is often misdiagnosed as Parkinson's disease because its symptoms are similar. The disease affects an estimated 10 to 12 people out of every 100,000. Because it's so rare, PSP is mostly unknown by the general public.
PSP vs. Parkinson’s
PSP is often mistaken for Parkinson's disease. The two disorders have similar symptoms, especially early in PSP. The biggest difference between the two is that PSP gets worse quicker than Parkinson's disease does.
Other differences between PSP and Parkinson's include:
- If you have PSP, you'll have something called "axial rigidity." This causes you to lean backward and extend your neck. This can sometimes cause people with the condition to fall backward. If you have Parkinson's, you'll tend to lean forward instead.
- With PSP, you'll develop an eye movement problem that affects looking up and down.
- With PSP, speech and swallowing issues are a lot more common. They're also worse with PSP, compared to Parkinson's, and tend to show up earlier in the disease.
- It's rare to have tremor (body shakes) with PSP, but it's very common in Parkinson's.
- People with Parkinson's disease usually get help from levodopa therapy, but if you have PSP, this treatment will only help a little or not at all.
- With PSP, there's a buildup of protein (called tau) in affected brain cells. With Parkinson's, a different protein (alpha-synuclein) builds up in your brain cells instead.
Progressive Supranuclear Palsy Causes
PSP develops because of the deterioration of brain cells in a few small but very important areas at the base of the brain. The most important affected area is the substantia nigra. When this area of the brain is affected by the disease, a lot of the palsy's symptoms become more visible. Researchers are still looking into why the brain cells degenerate.
Progressive Supranuclear Palsy Risk Factors
Age is the only definite risk factor for PSP. Symptoms usually start when a person is in their 60s or 70s, but rarely, they may begin to show as early as when they're in their 40s.
Men have a slightly greater chance of having PSP, but race doesn't appear to play a role.
Progressive Supranuclear Palsy Symptoms
The beginning stages of PSP include not being able to walk, falling spells, and stiffness. PSP patients tend to get dizzy before they fall. This dizziness is sometimes misdiagnosed as an inner ear problem or a hardening of the arteries that are blocking blood flow to the brain.
Other common symptoms of PSP include:
- Change in personality
- Loss of interest in usual socializing with family and friends
The word "progressive" was included in the palsy's name because symptoms typically progressively worsen for someone with the condition. After 7 to 9 years, PSP gets harder to deal with. The disease usually causes physical imbalance and stiffness of the body to grow worse, making walking very difficult or sometimes impossible.
Problems with eyesight also occur in the later stages of PSP. Usually, visual problems can become as much of an issue as impaired walking. Eyesight is most affected by the difficulty to aim the eye properly, making it hard to do things like read. Another eyesight problem that you may have is not being able to keep eye contact with another person when you talk to them. PSP can also cause "tunnel vision," which sometimes causes problems when you try to drive.
Other symptoms that get worse over time include:
- Slurred or slower speech
- Issues swallowing (which can cause choking or gagging)
- Sleep issues
- Sensitivity to bright lights
- Laughing or crying for no reason, or other impulsive behaviors
- Issues with problem- solving, decision-making, or reasoning
- Anxiety or depression
- Frightened or surprised facial expressions due to rigid muscles in your face
Progressive Supranuclear Palsy Stages
There are four stages of PSP. They are:
Early stage. This usually lasts for about a year. The symptoms include:
- Being able to walk, but falling every so often
- Poor balance
- Possible eyesight issues that affect your ability to read
- Changes in your voice, such as being unable to speak as loudly as usual
- Less desire to be social
- Mood shifts and behavior change, including anxiety or a lack of interest in things
Mid stage. A lot of people are already at this stage before they're diagnosed with PSP. It usually lasts from year 2 to year 3. You may want to start thinking about palliative care or advance care planning and decisions on when you'll start to stop treatment. You may have these symptoms at this stage:
- Being able to walk around, but with aids
- Issues with your sight that affect your ability to care for yourself (For example, this might affect your ability to walk or eat.)
- Speech issues or a hard time starting a conversation
- Lack of interest in doing things
- Being more socially withdrawn
- Risky or impulsive behavior
- A hard time swallowing
- Needing a high level of supervision
Advanced stage. At this stage, you should be on a palliative care register and also have specialist palliative care. The advanced stage usually lasts from year 3 to year 6. The symptoms include:
- A high risk of aspiration (when food or liquid enters your airway and lungs) and pneumonia due to swallowing issues
- Serious visual issues
- Severe muscles stiffness
- Needing a wheelchair to get around
- Serious communication issues, even though you still might be able to understand other people
- More periods of sleepiness
- Loss of bladder control
- Being dependent on someone else for most or all things related to your care
- Being very socially withdrawn
End-of-life stage. This stage is hard to identify. But usually your consciousness will be lower and you'll:
- Have acute infection
- Be unable to eat or drink
- Have fallen or had a major fracture
- Have lost a lot of weight quickly
This stage usually lasts about 6 to 8 weeks. The other symptoms include:
- Quick worsening of symptoms
- Many severe disabilities
At this point, if you're a caregiver for someone with PSP, you might need to make decisions about your loved one's treatment based on the wishes they had in the past (advance decisions to refuse treatment).
Does PSP Affect a Person Mentally?
Most PSP patients eventually have a mild to moderate degree of mental problems. The slowing of thoughts and thinking makes it hard to hold a conversation with others or to analyze problems.
Progressive Supranuclear Palsy Diagnosis
Since PSP is similar to Parkinson's disease, it can be hard to figure out which one you have.
Your doctor might look for certain signs that can help them diagnose you with PSP. In PSP you:
- Won't have tremors
- Will have a lot of unexplained falls
- Will have a hard time moving your eyes (especially moving them downward)
- Won't have any or only a small response to medications for Parkinson's disease
Your doctor may want you to have an MRI to see if you have any shrinkage in certain parts if your brain that have to do with PSP. They may also use the MRI to rule out other disorders that look similar to PSP, like a stroke.
A positron emission tomography (PET) scan may also help. This can help check for early signs of brain changes that might not show up on an MRI.
Progressive Supranuclear Palsy Treatment
There's a range of medication that can help curb its symptoms. Experts continue to research more treatment options for people with PSP.
Sinemet is sometimes used, because it contains levodopa, which is a dopamine replacement that helps control shakiness and tremors.
Doctors often suggest antidepressants , along with sleeping pills, to help people with PSP who have sleep problems. Many drugs being developed to treat other neurological disorders are also being used to help treat PSP.
Parkinson's disease medications can help a bit. They heighten levels of a chemical in your brain that can help control muscle movements. These may only help someone for 2 to 3 years, though.
OnabotulinumtoxinA, or Botox, might help as well. Your doctor can inject small amounts into the muscles around your eyes. This can block chemical signals that make your muscle contract. It'll help lessen eyelid spasms.
Glasses with bifocal or prism lenses can help if you have a hard time looking down. They'll allow you to see things that are lower without having to move your eyes.
You may also want to see a physical therapist or occupational therapist to work on your balance. You can make use of talking keyboards and facial exercises to help with other PSP symptoms.
A speech and swallowing evaluation could also help you learn about other ways to communicate and swallow safely.
Is There a Cure for PSP?
There is no cure for PSP. Care for this condition focuses on creating the best quality of life for as long as possible. If you have PSP, you can expect to live for about 5 to 7 years, although many things can affect how the disease progresses.
Progressive Supranuclear Palsy Complications
The complications of PSP usually happen because of slow or troublesome muscle movements. They may include:
- A hard time focusing your eyes, which can cause you to hurt yourself
- Falling, which can cause head injuries, fractures, or similar issues
- Trouble sleeping, causing sleepiness during the day
- Issues with swallowing that can lead to inhaling food (aspiration) or choking
- Pneumonia from inhaling food, which is the most common cause of death in people with PSP
- Trouble looking at bright lights
- Impulsive behaviors that can lead to falls (like standing up without waiting for help)
Talk to your doctor about ways to manage these risks. For example, you can use a feeding tube to avoid aspiration or choking. You can also get a walker or wheelchair to help avoid falls.
Living With Progressive Supranuclear Palsy
There are ways you can make life with PSP a little easier:
Use grab bars or a walker. You can get grab bars installed in your hallways and bathrooms to avoid falls. A weighted walker can help you avoid falling backward. It's also smart to avoid stairs altogether.
Eyedrops. You can use these multiple times a day to avoid dry eyes due to issues with blinking or too much tearing.
Get rid of small area rugs. These are other objects that are hard to see without looking down, and they might put you at risk for tripping or falling. It's best to keep a clear walkway.
Support groups for progressive supranuclear palsy. To find more support and connect with others, you can visit websites such as psp.org or brainsupportnetwork.org.
Clinical Trials for Progressive Supranuclear Palsy
You can ask your doctor about any clinical trials for PSP going on now. They may be able to connect you with one.
You can also browse different clinical trials by going to ClinicalTrials.gov and putting in specific information about what you're looking for.