Progressive Supranuclear Palsy
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What is progressive supranuclear palsy?
Progressive supranuclear palsy (PSP) is a rare and chronic neurodegenerative disorder that damages certain areas of your brain. It affects how you walk, think, swallow and move your eyes. It may also cause other symptoms. PSP is also known as Steele-Richardson-Olszewski syndrome.
“Progressive” means that the symptoms get worse over time from the underlying neurodegenerative condition. “Supranuclear” and “palsy” together mean the inability to move your eyes, as “supranuclear” means that the damage is located above the eye-moving centers (nuclei) in your brain. “Palsy” means weakness or difficulty using your muscles.
PSP may closely resembles Parkinson’s disease, especially early on in its course. Experts consider it an atypical parkinsonian syndrome (or Parkinson-plus disorder). Healthcare providers often misdiagnose PSP as Parkinson’s disease, especially in the early stages of the condition. But PSP progresses faster than Parkinson’s disease.
The condition most commonly affects people over the age of 60. It very rarely develops before the age of 40.
What are the types of progressive supranuclear palsy (PSP)?
There are four different types, or phenotypes, of PSP. They all share similar symptoms, but there are some unique differences. The two most common types are Richardson syndrome and PD-like variant (PSP-P), which means a Parkinson’s disease-like variant. Together, they make up 75% of PSP cases.
Richardson syndrome has many symptoms, some of which include:
- Issues with walking and balance.
- Abnormal speech.
- Memory and thinking problems.
- Difficulty controlling eye movement (especially looking down).
- A wide-eyed, staring facial expression.
PD-like variant (PSP-P) has the same symptoms as Richardson syndrome, but more closely resembles Parkinson’s disease. A tremor (an involuntary muscle contraction that leads to shaking) is the main symptom (instead of balance problems and behavioral changes). PSP-parkinsonism may respond better to antiparkinsonian medications than other types of PSP, at least for some time.
The other two phenotypes include:
- Corticobasal syndrome.
- Pure akinesia and gait freezing.
How common is PSP?
PSP is rare. Around 5 people out of every 100,000 have it. About 1 per 100,000 people get diagnosed each year.
Symptoms and Causes
What are the symptoms of progressive supranuclear palsy (PSP)?
The symptoms of progressive supranuclear palsy vary from person to person. They tend to begin gradually and typically become more severe over years.
The most common first signs of PSP include:
- A loss of balance when walking or climbing stairs. This often involves falling, especially falling backward.
- Difficulty looking downward with your eyes.
- A wide-eyed staring expression.
Other symptoms of PSP include:
- Difficulty swallowing.
- Stiff muscles that affect mobility (your ability to move).
- Difficulty speaking. Your speech might be quieter and slurred, making it hard to pronounce words.
- Mood changes, such as depression, apathy (loss of interest) and irritability.
- Personality changes.
- Changes in behavior, such as impulsivity and poor judgment.
- REM sleep behavior disorder (RBD).
- Sensitivity to bright light (photophobia).
What causes progressive supranuclear palsy?
Scientists don’t know the exact cause of progressive supranuclear palsy. However, experts know that a protein called tau is involved. Tau is an important protein for brain health. It helps preserve the normal structure of neurons (brain cells).
When you have PSP, the tau in your brain clumps together (aggregates). The clumps then damage neurons. Researchers have several theories for why this might happen, some of which include:
- Random genetic mutations.
- Unknown infectious agents.
- An unknown chemical in the air, water or your food that slowly damages certain vulnerable areas of your brain.
PSP affects everyone differently. It impacts various parts of your brain to varying degrees at different stages. Eventually, PSP will spread to most of your brain. The condition tends to affect your basal ganglia and brainstem early and more severely.
Your brainstem is responsible for a large number of vital functions, including your ability to swallow and hold your body steady (your posture). Your basal ganglia also help with posture, as well as eye movements, thinking and emotions.
Is PSP hereditary?
No, PSP rarely runs in families. If you have PSP, there’s very little additional risk of your other family members having it. That includes your biological siblings and children.
What are the risk factors for progressive supranuclear palsy?
The main risk factor for PSP is age. Your risk for developing PSP increases as you age if you’re 60 or older. Men and people assigned male at birth are slightly more likely to get PSP than women and people assigned female at birth.
What are the complications of PSP?
As PSP progresses to an advanced stage, people with the condition typically have increasing trouble controlling the muscles in their mouths, throats and tongues.
The loss of control of your throat muscles can lead to severe swallowing problems, and you may need a feeding tube to prevent choking and chest infections.
Many people with PSP also develop problems with bowel and bladder functions, including:
- Difficulty passing urine.
- Frequent urination during the night.
- Bowel incontinence.
- Urinary incontinence.
Diagnosis and Tests
How is progressive supranuclear palsy diagnosed?
It can be difficult for healthcare providers to efficiently diagnose PSP. They often mistake it for Parkinson’s disease, especially in the early stages. There’s no single test that can diagnose PSP. Providers usually diagnose PSP based on your symptoms and with imaging tests to take pictures of your brain.
If your healthcare provider suspects you have PSP, they’ll likely recommend a brain MRI (magnetic resonance imaging). This will help rule out other possible causes of your symptoms, such as Parkinson’s disease or stroke. It may also show shrinking of your midbrain, which raises the likelihood of PSP.
You’ll likely need to see a neurologist specializing in Parkinson’s disease and movement disorders to receive a diagnosis.
Although there are many symptoms of PSP, the one that often confirms the diagnosis is difficulty moving your eyes up and down. Other common symptoms like falling and difficulty swallowing also point to PSP.
Management and Treatment
What is the treatment for progressive supranuclear palsy?
There’s currently no cure for PSP and no way to slow it down. But several treatments can help manage your symptoms and improve your quality of life.
- Oral medications.
- Movement therapies.
- Eye treatments.
- Percutaneous endoscopic gastrostomy (PEG).
- Palliative care.
There may also be clinical trials for PSP that you can participate in. Ask your healthcare team if this is an option.
Antiparkinsonian medications are oral (taken by mouth) medicines typically used to help people with Parkinson’s disease. They can sometimes help manage progressive supranuclear palsy symptoms, too. They can help — temporarily — with balance, stiff muscles and slow movements, and tremors. They don’t work for every single person. Providers most often prescribe the following medications for PSP:
- Levodopa (Atamet®, Rytary®, Sinemet®).
- Levodopa with anticholinergic agents.
- Amantadine (Symmetrel®).
Antidepressant medications help with clinical depression. Your provider may prescribe them to help you cope with any mental health symptoms of PSP. The most commonly prescribed antidepressants for PSP include:
- Fluoxetine (Prozac®).
- Amitriptyline (Elavil®).
- Imipramine (Tofranil®).
Psychotherapy (talk therapy) can also help with mental health symptoms and coping with the diagnosis.
The following therapies can help with some symptoms of PSP:
- Physical therapy: Physical therapy can help you manage symptoms like pain, stiffness and discomfort that make it hard to move.
- Occupational therapy: An occupational therapist is a healthcare provider who helps you improve your ability to perform daily tasks. They’ll help you practice how to stand, sit, move or use different tools to participate in your activities safely.
- Speech therapy: Speech therapists can help you improve speaking and swallowing issues.
Certain treatments can help various eye issues, including:
- Botox® (botulinum toxin) injections: If you're having issues controlling your eyelids, Botox injections may temporarily help relax the muscles of your eyelids.
- Eye drops and artificial tears: These can help lubricate your eyes if you’re having dry eyes due to reduced blinking.
- Special glasses: Special eyeglasses with prism lenses can help you to see what’s below your line of vision.
- Wraparound glasses: Wearing dark wraparound glasses can help if you’re sensitive to bright light.
Percutaneous endoscopic gastrostomy (PEG)
As PSP worsens, you might reach the point where you can’t swallow at all. This will prevent you from eating and drinking. Before that point, your healthcare provider may recommend that you get a percutaneous endoscopic gastrostomy (PEG).
A PEG is a procedure in which a surgeon inserts a tube through your abdomen into your stomach. Your food, medications and liquids will enter your body through the tube.
Palliative care is a specialized form of care that provides symptom relief, comfort and support to people living with serious conditions, including PSP. It also provides support to caregivers and those impacted by a loved one’s condition.
Palliative care complements the care you receive from the providers who help you manage PSP. Palliative care helps you live more comfortably — with the medical, social and emotional support needed to cope with a serious illness.
Can I prevent progressive supranuclear palsy?
Because researchers don’t know what causes PSP, there’s no way to prevent it.
Outlook / Prognosis
What is the prognosis for progressive supranuclear palsy?
The prognosis (outlook) for progressive supranuclear palsy (PSP) is generally poor. The symptoms worsen with time, and there currently isn’t treatment that can reverse or stop PSP. However, the sooner you receive a diagnosis and start a treatment plan, the better your quality of life will be.
Most people with PSP eventually need a wheelchair. You may need part- or full-time care as early as three to four years into living with PSP. But this varies from person to person.
Over time, PSP complications can be fatal.
What is the life expectancy of someone with progressive supranuclear palsy?
People with progressive supranuclear palsy typically die six to nine years after their diagnosis. But this can vary.
PSP symptoms increase your risk of developing pneumonia, which can be fatal. Aspiration pneumonia is the most common cause of death in people with PSP. It happens when the muscles in your throat become weak and uncoordinated and food and liquids accidentally trickle down your windpipe and into your lungs.
People with PSP also have a higher risk of falls, which can result in bone fractures and head traumas. Falls that cause serious injuries are a common cause of death among people with PSP.
When should I see my healthcare provider?
You’ll need to see your healthcare team regularly to monitor the progression of your symptoms and to make sure your treatment plan is working well.
If you develop any concerning symptoms, call your provider.
A note from Cleveland Clinic
Understanding your progressive supranuclear palsy (PSP) diagnosis can be overwhelming. Your healthcare team will help you find a symptom management plan that’s unique to your needs. It’s important to make sure you’re getting the support you need and to stay attentive to your health. Know that your healthcare team will be there to support you and your family.
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