Progressive supranuclear palsy (PSP) is a rare neurological disorder that damages the nerve cells in the brain that control body movements, swallowing, eye movements, and thinking.
UPMC neurology experts provide advanced care for people with PSP, including medications and supportive care to help manage symptoms and prevent disease complications.
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What Is Progressive Supranuclear Palsy (PSP)?
Progressive supranuclear palsy (PSP) is a rare neurological disorder that damages the nerve cells in the brain that control body movements, swallowing, eye movements, and thinking.
What are the types of PSP?
There are multiple clinical types of PSP, including:
- PSP-Richardson Syndrome (PSP-RS) — The most common clinical subtype, this form causes falls, problems with eye movements and swallowing, and changes in thinking and behavior.
- PSP-Parkinsonism (PSP-P) — This variant causes symptoms that resemble Parkinson’s disease, sometimes including tremor.
- PSP-progressive gait freezing (PSP-PGF) — This variant causes difficulty starting to walk and difficulty moving legs when turning around, particularly in confined spaces.
There are multiple other variants of PSP, and an experienced specialist is usually able to distinguish them.
What is the difference between Parkinson’s disease and PSP?
Parkinson’s disease and PSP are both neurodegenerative disorders that have several symptoms in common. However, they are caused by a buildup of different types of proteins in overlapping but different parts of brain, which cause distinct symptoms.
PSP is associated with a buildup of 4R-tau protein in the brain, whereas Parkinson’s disease is caused by a buildup of alpha-synuclein protein. The diseases can look similar clinically, but the characteristic eye movement problems of PSP are usually not present in Parkinson’s disease, and PSP symptoms may not respond to common medications used to treat Parkinson’s disease.
How common is PSP?
PSP is uncommon. It affects approximately six out of every 100,000 people worldwide.
What causes PSP?
Researchers aren’t sure what causes PSP, but they believe that it may occur when a protein called 4R-tau builds up in the brain and causes nerve cell damage.
PSP risk factors
You may be at higher risk of PSP if you are over age 60. It is slightly more common in males compared to females.
Complications of PSP
Complications of PSP may include:
- Bowel and bladder problems.
- Injuries from falls.
- Loss of independence.
- Loss of mobility.
- Pneumonia.
- Swallowing problems.
What Are the Signs and Symptoms of PSP?
Symptoms of PSP typically begin gradually and worsen over time.
Although symptoms may vary, early symptoms often include:
- Loss of balance and falls.
- Difficulty initiating walking movements.
- Difficulty looking downward.
- Changes in thinking or behavior.
As symptoms progress, they may include:
Physical symptoms
- Difficulty swallowing and coughing or choking after eating or drinking.
- Muscle stiffness, particularly around the neck.
- Problems with speech.
- Involuntary eyelid closure.
- Urinary incontinence.
Cognitive and sleep symptoms
- Behavior, mood, and personality changes.
- Dementia.
When should I see a doctor about my PSP symptoms?
If you have symptoms of PSP, you should schedule an appointment with your doctor right away. Your doctor may refer you to a neurologist who specializes in diagnosing and managing PSP.
How Do You Diagnose PSP?
It's crucial to have an expert diagnosis of PSP. Diagnosing PSP is complex because symptoms may be similar to other conditions, such as Parkinson’s disease.
To diagnose PSP, your doctor will review your symptoms and medical history, and perform a neurological examination. Your doctor may also order tests to rule out other problems.
What to expect during your visit
During your visit, your doctor will:
- Ask you about your symptoms.
- Perform a physical and neurological exam.
- Review your medical history.
- Possibly order tests.
Tests to diagnose PSP
There's no test to diagnose PSP. However, your doctor may order tests to provide information or rule out other problems, depending on the examination findings, including:
- Blood tests — Check for abnormalities and rules out other conditions.
- Magnetic resonance imaging (MRI) — Creates images of your brain using contrast dye to look for damaged or abnormal areas.
- Neuropsychological examination — Checks your ability to think, reason, and remember.
PSP prognosis
There is no cure for PSP, and symptoms get worse over time. Eventually, most people with PSP lose the ability to walk and require assistance with everyday tasks. Although PSP itself is not fatal, related complications, such as pneumonia or injuries from falls, may cause death.
What is the life expectancy of someone with PSP?
PSP typically causes death within six to nine years of diagnosis. However, your life expectancy may vary depending on how quickly your condition progresses, your age at the time of diagnosis, your overall health, and other factors.
How Do You Treat PSP?
There's no cure for PSP, but you can manage some symptoms of the disease and improve your quality of life.
Doctors may recommend PSP treatments including:
Medications
Your doctor may recommend medications including:
- Parkinson’s disease medications — May treat gait freezing.
- Botox® (botulinum toxin) — Injections can help prevent involuntary eyelid closure.
- Antidepressants — Treat depression and anxiety.
Supportive care
Supportive treatments for PSP may include:
- Neuro-ophthalmology evaluation— Customized glasses can sometimes help with double vision.
- Physical and occupational therapy — Physical therapy exercises can help with movement and balance, and occupational therapy can help maintain independence in performing everyday tasks.
- Speech and language therapy — A speech therapist may be able to help with communication skills and swallowing.
- Feeding support — If you are no longer able to swallow, your doctor may recommend a percutaneous endoscopic gastrostomy (PEG) procedure to help deliver food, water, and medication using a tube inserted through your abdomen and into your stomach.
- Palliative care — Provides symptom relief and supportive care to treat the physical, emotional, and social effects of PSP.
Clinical trials for PSP
You may be eligible to participate in clinical research to help doctors better understand PSP and develop new therapies to treat the condition. Your doctor will let you know if you may be eligible to participate in a clinical trial.
Why Choose UPMC for PSP Care?
When you choose UPMC for PSP care, you will receive:
- Multidisciplinary care — Our team of neurology experts will work with you and your loved ones to develop a customized treatment plan that optimizes your quality of life and reduces your risk of complications.
- Access to advanced clinical trials — Our team participates in national, multicenter clinical trials evaluating new treatment options, giving you the opportunity to contribute to research advancingneurological care.
- Seamless, ongoing treatment — If you are diagnosed with PSP, our team will develop a plan for your follow-up care, including ongoing symptom management and long-term care planning, as needed.
