Progressive Supranuclear Palsy

Overview

What is progressive supranuclear palsy?

Progressive supranuclear palsy (PSP) – or Steele-Richardson-Olszewski syndrome – is a rare neurodegenerative disorder that damages your brain (particularly the basal ganglia, substantia nigra, subthalamic nucleus, pars reticulata and other midbrain structures) and affects how you walk, think, swallow and move your eyes. It may also cause a number of other symptoms. PSP is often confused with Parkinson’s disease. There is no proven cure or disease-modifying treatment.

“Neurodegenerative” refers to the collapse of your nervous system, especially the death of neurons and gia (certain types of cells) in your brain. “Progressive” means that the symptoms get worse over time and “palsy” means that the disorder impairs your eye movements. “Supranuclear” refers to the damage localized above the eye-moving centers (nuclei) in your brain.

Is progressive supranuclear palsy (PSP) fatal?

PSP itself does not directly kill. Unfortunately, PSP symptoms increase the risk of complications such as pneumonia, which can be deadly. Aspiration pneumonia is the most common cause of death in people with PSP. It happens because the muscles in your throat become weak and uncoordinated, and food and drink accidentally trickle down your windpipe and into your lungs.

Another common cause of death in PSP falling. You may suffer fractures and head trauma.

What parts of the brain does progressive supranuclear palsy (PSP) affect?

Eventually PSP will spread to virtually every part of your brain. PSP affects various parts of your brain to a varying extent and at various stages of the disease. Your basal ganglia and brainstem tend to be affected early and more severely. The brainstem is responsible for a large number of vital functions, including your ability to swallow, and posture control. The basal ganglia also help with posture, as well as eye movements, thinking and emotions. Additionally, the basal ganglia are essential to the control of automated movements.

Is there a cure for progressive supranuclear palsy (PSP)?

There is no cure for PSP, but there are ways to manage your symptoms to improve your quality of life. (See the “Treatment” section.) Also, there is extensive ongoing research to find treatments that will halt, slow, or prevent disease progression.

Who is at risk of getting progressive supranuclear palsy (PSP)?

You are at a greater risk of developing PSP if you are age 60 or older. Men are slightly more likely to get PSP than women.

How common is progressive supranuclear palsy (PSP)?

Five to six out of every 100,000 people have PSP. About one per 100,000 gets diagnosed each year.

What are the types of progressive supranuclear palsy (PSP)?

There are many types of PSP but the two most common types are Richardson syndrome and PD-like variant (PSP-P) – which means a Parkinson’s disease-variant. Together, they make up 75% of PSP cases. The symptoms are similar, but there are some unique differences.

Richardson syndrome has many symptoms including problems with walking and balance, abnormal speech, memory and thinking problems, trouble controlling eye movement (especially looking down) and an unusual wide-eyed, staring facial expression. People with Richardson syndrome also have personality changes, forgetfulness, apathy (loss of interest), depression, irritability and problems with attention and concentration. These are the most common symptoms.

PD-like variant (PSP-P) consists of the above symptoms, but also more closely resembles Parkinson’s disease. A tremor (an involuntary muscle contraction) is the main symptom over balance problems and behavioral changes. PSP-parkinsonism responds better to antiparkinson drugs.

What is the difference between progressive supranuclear palsy and Parkinson’s disease (PD)?

PSP frequently resembles Parkinson’s disease, often making it difficult to distinguish these conditions from one another. Both cause difficulty with stiffness and clumsiness. Both cause slow movement and start later in life. And both show damage to the same areas of the brain that affect movement. In particular, PSP-P can be nearly indistinguishable from Parkinson’s disease by clinical examination.

But, PSP and Parkinson’s disease are different in several ways. On average, PSP gets worse quicker than Parkinson’s and doesn’t respond as well to medications. People with Parkinson’s usually bend forward, while people with PSP stand very straight, or even slightly backwards. Problems with swallowing and with speaking appear early with PSP and they are far more severe. A tremor is rare with people who have PSP and common in people who have Parkinson’s. Motor symptoms, such as slowing and loss of dexterity, typically start very asymmetrically in Parkinson’s disease (one side of the body is significantly worse than the other, whereas a more symmetric presentation is typical in PSP even during early stages of the disease).

Additionally, their underlying pathological processes differ at the molecular level. Abnormalities relating to the protein tau and its effect on brain cells is central to PSP pathology. And, when looked at through a microscope, the damaged brain cells in someone with PSP look different than those of people who have Parkinson’s.

Symptoms and Causes

What causes progressive supranuclear palsy (PSP)?

The exact cause of PSP is unknown. However, experts have determined that a protein called tau is involved. Tau is an important protein that helps with brain health. It holds together and stabilizes microtubules. (Microtubules help with transporting cells, among other purposes.) Tau also helps preserve the normal structural integrity of neurons, and maintain genomic DNA.

When you have PSP, the tau in your brain aggregates, which means that it clumps together. The clumps damage the neurons (brain cells).

What are the signs and symptoms of progressive supranuclear palsy (PSP)?

If you have PSP, you’ll likely experience the following symptoms that worsen with time. You’ll likely:

  • Lose your balance frequently. You may fall, especially backwards.
  • Lose the ability to aim your eyes. You might not be able to look up or down, follow something that’s moving, or keep your eyes fixed on something still. Your eyelids might retract (pull back), giving your face a surprised look.
  • Have trouble swallowing.
  • Feel stiff muscles and move slowly.
  • Have trouble speaking. Your speech might be quieter and slurred, impairing your ability to pronounce words.
  • Feel mood changes and observe behavioral changes.
  • Experience depression and apathy (loss of interest).
  • Get dementia. Your thoughts will be slow, you’ll have difficulty resisting impulses and you may find problem-solving difficult.
  • Have insomnia (inability to sleep).
  • Feel irritable and agitated.

Are there signs and symptoms of progressive supranuclear palsy (PSP) that are more common, or appear first?

The most common first sign is a loss of balance when walking or climbing stairs. You might feel stiff, awkward when walking and have falls for reasons you can’t explain. The second most common symptom is difficulty looking downward. Then, you might have a hard time finding words, freezing in the middle of walking and have forgetfulness, personality changes, increased irritability and loss of interest. Another very common initial symptom is a “stare” look on your face. Other symptoms that occur at first, but are less common, include slurred speech, difficulty driving and shaky hands.

Is progressive supranuclear palsy (PSP) permanent?

Unfortunately, because there is no cure, you will have PSP for the rest of your life.

Is progressive supranuclear palsy (PSP) inherited?

No, PSP rarely runs in families. If you have PSP, there is very little risk of your other family members having it, and that includes your siblings and your children. Family history of PSP has an almost zero effect on an individual’s lifetime PSP risk, meaning people have about the same risk of developing PSP in their lifetime regardless if they do or do not have someone in their family who had or has PSP.

Diagnosis and Tests

How is progressive supranuclear palsy (PSP) diagnosed?

PSP is diagnosed on the basis of clinical signs and exam findings, but it often isn’t easy to diagnose. There’s no single test that points right at it with certainty. It is often mistaken for Parkinson’s disease (PD) and it can sometimes take years to distinguish PSP from PD.

If your healthcare provider suspects PSP based on the symptoms you report, he or she will likely send you to get a MRI (magnetic resonance imaging). This will help to rule out other diseases such as Parkinson’s and occasionally it can show shrinking of your midbrain that raises the possibility of PSP in the appropriate clinical setting. You’ll also be sent to a neurologist specializing in Parkinson’s disease and movement disorders.

Although there are many symptoms of PSP, the one that confirms the diagnosis is trouble moving your eyes up and down. Other common symptoms like falling and difficulty swallowing also point at PSP.

Do I need to see a specialist?

Yes. A movement disorder neurologist can confirm the diagnosis of PSP with the highest level of certainty.

What questions might my healthcare provider ask to diagnose progressive supranuclear palsy?

  • Are you having falls you can’t explain?
  • Are you falling backward?
  • Are your muscles stiff?
  • Are you talking normally or have you noticed changes in your speech?
  • Are you feeling depressed?
  • Are you still enjoying the same activities you always have?
  • Are you sleeping normally?
  • Are you overly irritated or aggravated?

Management and Treatment

Although there is no cure for progressive supranuclear palsy (or a way to slow it down or reverse it), experts have found some promising treatments to help manage some of your symptoms and improve your quality of life. These treatments include:

  • Antiparkinsonian medications.
  • Antidepressant medications.
  • Therapy.
  • Surgery (a gastrostomy).

Antiparkinsonian medications: These oral (by mouth) medicines are typically used to help patients with Parkinson’s disease, but they can help with progressive supranuclear palsy as well. They can help – temporarily – with balance, stiff muscles and slow movements, and tremor if tremor is present. They don’t work for every single patient. The most commonly prescribed in PSP medications include:

  • Levodopa (Rytary®, Sinemet®).
  • Levodopa with anticholinergic agents.
  • Amantadine (Symmetrel®).

Antidepressant medications: These oral medicines help with clinical depression. Even if there is no formal diagnosis of depression, they are still sometimes used because they can help with your general resilience and ability to cope. The most commonly prescribed antidepressants include:

  • Fluoxetine (Prozac®).
  • Amitriptyline (Elavil®).
  • Imipramine (Tofranil®).

Unfortunately vision, speech and difficulty swallowing do not respond to any drug treatment.

Therapy: Four types of therapy – physical, occupational, mental health and speech-language – can help with some effects of progressive supranuclear palsy.

  • Physical: Physical therapy will help keep your joints flexible.
  • Occupational: Occupational therapy helps to prevent falls by teaching you to use weighted walking aids.
  • Mental health: Mental health therapy is counseling with a therapist who can help you improve your coping techniques. A social worker can also help you with overall needs including appropriate care settings.
  • Speech-language: Speech and language therapists can help you improve your speaking and swallowing problems.

Surgery: As your progressive supranuclear palsy worsens, you might reach the point where you can’t swallow at all, which prevents you from eating and drinking. Before that point, your healthcare provider may recommend that you get a gastrostomy. A gastrostomy is a procedure in which the surgeon inserts a tube through your abdomen into your stomach. Your food, medications and liquids will enter your body through the tube.

Your healthcare provider might also recommend special eye glasses with prism lenses. Prism lenses will help you look down, which PSP patients are unable to do on their own.

How can I treat my depression and irritability?

See a psychiatrist for medication and a therapist for counseling. Ask your primary healthcare provider for referrals.

What should I avoid eating or drinking with this condition?

There are no foods that improve progressive supranuclear palsy or worsen it. However, you should always drink water, eat healthy and exercise (under your healthcare provider’s supervision).

As your symptoms progress, you may find eating and drinking more difficult because you’ll have difficulty swallowing. Be sure to adjust your diet to avoid choking. The muscles in your throat may not be able to direct your food and water down the right “pipe” – that is, into your stomach and not your lungs. This is dangerous because you could choke, or you could possibly get pneumonia. Talk to your healthcare provider about what you can and can’t eat and drink as your condition progresses.

Prevention

How can I reduce my risk of progressive supranuclear palsy (PSP)?

There are no known ways to reduce your risk of PSP.

Can progressive supranuclear palsy (PSP) be prevented?

PSP can’t be prevented. The symptoms, however, can be managed to improve your quality of life.

Who is at risk of developing progressive supranuclear palsy (PSP)?

PSP is found most frequently in people age 60 and older, but it has also been found in people as young as 40. It’s a little bit more common in men than women.

What medicines can I take to reduce my risk of progressive supranuclear palsy (PSP)?

There are no known medications that will reduce your risk of PSP.

Outlook / Prognosis

What can I expect if I’ve been diagnosed with progressive supranuclear palsy (PSP)?

Your symptoms will slowly worsen with time. PSP can’t be reversed, and it can’t be cured. However, the sooner the diagnosis is made and a treatment plan is put into place, the better your quality of life will be.

Most patients with PSP become wheelchair-bound. They may need part or full time care as early as three to four years into the disease. This depends on the person.

Unfortunately, in the long-term, complications of the disease can be fatal. PSP won’t directly kill you, but it is deadly. Common complications include choking, pneumonia, fractures and head injuries (because of the falls).

How long can someone live with progressive supranuclear palsy (PSP)?

People with PSP typically pass away from complications within six to ten years – but it depends on the person. Walking can become difficult, if not impossible, after five or six years.

Living With

How do I take care of myself if I have progressive supranuclear palsy (PSP)?

Take as many precautions as you can to keep yourself safe. Put railings in your hallways and bathtub to help when you lose your balance. Use a weighted walker or a wheelchair. Consider getting rid of objects that are on the floor or near it such as rugs and short coffee tables. It is likely you’ll trip over these objects because, as your symptoms worsen, you’ll be unable to look down without prism glasses.

Also, purchase shoes with smooth soles instead of rubber soles. If you’re unable to lift your foot when you start to walk, you might fall forward. Smooth soles make it easier for the foot to slide forward. You can also wear bifocals or prism glasses to help with looking down. Follow any other instructions your healthcare provider gives you.

How do I cope with the emotional burden of progressive supranuclear palsy (PSP)?

Coping with diseases like PSP is very difficult, and mental health disorders like clinical depression often happen alongside them. Depression is a common symptom of PSP. A psychiatrist can help by prescribing medications and a therapist can help with counseling.

How can my friends and family help if I have progressive supranuclear palsy (PSP)?

Tell your friends and family about your symptoms. Let them know what you can and can’t do. Help them understand that changes in your personality, eye contact and mood are part of the illness. It’s also important to explain to them that the severity of your symptoms can come and go. One hour, you may be walking, eating and writing just fine, and the next hour you might barely be able to do those things at all.

If you’re a caregiver for someone with PSP, be sure to take care of yourself, too!

When should I see my healthcare provider about progressive supranuclear palsy (PSP)?

It’s wise to see a healthcare provider as soon as possible if you have symptoms of PSP. Be alert for the first signs like trouble walking and unexplained falls. Don’t just dismiss the symptoms as signs of aging.

What questions should I ask my healthcare provider about progressive supranuclear palsy?

  • What exercises are safe to do?
  • Are there any vitamins I should take?
  • How can my friends and family support me?
  • Can you refer me to a psychiatrist to help with medications for clinical depression?
  • Can you refer me to a therapist for counseling?
  • Do you think I will need to have a gastrostomy?
  • Is there a speech-language therapist I should see?
  • Should I see an ophthalmologist to help with my eyesight?
  • Where can I get a weighted walker to help me get around?
  • Can I continue to go to work/school?

A note from Cleveland Clinic

Progressive supranuclear palsy is a serious disorder that can negatively affect you in many ways and shorten your lifespan. It’s a rare brain disease that affects your walking, balance, eyesight, thinking, emotions and more. Keep a close eye on your symptoms and report them. Don’t hesitate to stay in contact with your healthcare providers. See your specialists, go to therapy, take prescribed medications and do everything you can to improve your quality of life!

Last reviewed by a Cleveland Clinic medical professional on 10/23/2020.

References

  • NHS. . Accessed 10/23/2020. Progressive supranuclear palsy (https://www.nhs.uk/conditions/progressive-supranuclear-palsy-psp/)
  • National Institute of Neurological Disorders and Stroke. Accessed 10/23/2020. . Progressive Supranuclear Palsy Fact Sheet (https://www.ninds.nih.gov/Disorders/Patient-Caregiver-Education/Fact-Sheets/Progressive-Supranuclear-Palsy-Fact-Sheet)
  • Cure PSP. . Accessed 10/23/2020. Progressive Supranuclear Palsy (PSP) (https://www.psp.org/iwanttolearn/progressive-supranuclear-palsy/)
  • National Organization of Rare Disorders. . Accessed 10/23/2020. Progressive Supranuclear Palsy (https://rarediseases.org/rare-diseases/progressive-supranuclear-palsy/)
  • Merck Manual. . Accessed 10/23/2020.Progressive Supranuclear Palsy (PSP) (https://www.merckmanuals.com/home/brain,-spinal-cord,-and-nerve-disorders/movement-disorders/progressive-supranuclear-palsy-psp?query=Progressive%20Supranuclear%20Palsy%20(PSP%29)

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