Progressive supranuclear palsy, also called Steele-Richardson-Olszewski syndrome, is an uncommon brain disorder that causes serious problems with walking, balance and eye movements. The disorder results from deterioration of cells in areas of your brain that control body movement and thinking.
Progressive supranuclear palsy worsens over time and can lead to life-threatening complications, such as pneumonia and swallowing problems. There's no cure for progressive supranuclear palsy, so treatment focuses on managing the signs and symptoms.
The characteristic signs and symptoms of progressive supranuclear palsy include:
- A loss of balance while walking. A tendency to fall backward can occur very early in the disease.
- An inability to aim your eyes properly. You may have particular difficulty looking downward, or experience blurring and doubled vision. This difficulty with focusing the eyes can make some people spill food or appear disinterested in conversation because of poor eye contact.
Additional signs and symptoms of progressive supranuclear palsy vary and may mimic those of Parkinson's disease and dementia. These signs and symptoms worsen as the disease advances, and may include:
- Stiffness and awkward movements
- Problems with speech and swallowing
- Sensitivity to light
- Sleep disturbances
- Loss of interest in pleasurable activities
- Impulsive behavior, possibly including laughing or crying for no reason
- Difficulties with memory, reasoning, problem-solving and decision-making
- Depression and anxiety
- A surprised or frightened facial expression, resulting from rigid facial muscles
When to see a doctor
Make an appointment with your doctor if you experience signs and symptoms of progressive supranuclear palsy.
The cause of progressive supranuclear palsy isn't known. The signs and symptoms of the disorder result from deterioration of cells in areas of your brain, especially those that help you control body movements (midbrain) and thinking (frontal lobe).
Researchers have found that the deteriorating brain cells of people with progressive supranuclear palsy have abnormal amounts of a protein called tau. Clumps of tau are characteristic of other neurodegenerative disorders, such as Alzheimer's disease.
Rarely, progressive supranuclear palsy occurs within a family. But a genetic link isn't clear, and most people with progressive supranuclear palsy haven't inherited the disorder.
The only proven risk factor for progressive supranuclear palsy is age. The condition typically affects people around the age of 60, and is virtually unknown in people under the age of 40.
Complications of progressive supranuclear palsy result primarily from hindered muscle movements. These complications may include:
- Falling, which could lead to head injuries, fractures and other injuries.
- Difficulty focusing your eyes, which also can lead to injuries.
- Difficulty sleeping.
- Difficulty looking at bright lights.
- Problems swallowing, which can lead to choking or inhaling food or liquid into your airway (aspiration). Aspiration can develop into pneumonia, the most common cause of death in people with progressive supranuclear palsy.
- Impulsive behaviors — for example, standing up without waiting for assistance — which can lead to falls.
To avoid the hazards of choking, your doctor may recommend a feeding tube. To avoid injuries due to falling, a walker or a wheelchair may be used.
You may be referred to a doctor who specializes in disorders of the brain and nervous system (neurologist).
What you can do
- Write down your symptoms, including any that may seem unrelated to the reason why you scheduled the appointment.
- Make a list of all your medications, vitamins and supplements.
- Write down your key medical information, including other conditions.
- Write down key personal information, including any recent changes or stressors in your life.
- Write down questions to ask your doctor.
- Ask a relative or friend to accompany you, to help you remember what the doctor says.
Questions to ask your doctor
- What's the most likely cause of my symptoms?
- What kinds of tests do I need?
- How does progressive supranuclear palsy usually progress?
- What treatments are available, and what types of side effects can I expect?
- I have other health conditions. How can I best manage these conditions together?
- Should I restrict my activities?
In addition to the questions that you've prepared to ask your doctor, don't hesitate to ask other questions during your appointment.
What to expect from your doctor
Your doctor is likely to ask you a number of questions. Being ready to answer them may make time to go over points you want to spend more time on. You may be asked:
- Have you experienced problems with balance or walking?
- Do you find it difficult to see items below you, such as the plate when you are eating?
- Do you have trouble speaking or swallowing?
- Have your movements felt stiff or shaky?
- Have you experienced any troubling mood changes?
- When did you begin experiencing these symptoms? Have they been continuous or occasional?
- Does anything seem to improve or worsen these symptoms?
Progressive supranuclear palsy can be difficult to diagnose because signs and symptoms are similar to those of Parkinson's disease. Indications that you have progressive supranuclear palsy rather than Parkinson's disease include:
- A lack of shaking (tremors)
- A poor response to Parkinson's medications
- Difficulty moving your eyes, particularly downward
Your doctor may recommend MRI to determine if you have shrinkage in specific regions of the brain associated with progressive supranuclear palsy. MRI can also help to exclude disorders that may mimic progressive supranuclear palsy, such as stroke.
A positron emission tomography (PET) scan also may be recommended to check for early signs of brain abnormalities that may not appear on MRI. Researchers are working to develop medical tests that can diagnose progressive supranuclear palsy.
Mayo Clinic researchers discovered that some people with progressive supranuclear palsy develop speech problems (apraxia of speech) years before experiencing other symptoms. This speech problem may be the first sign of progressive supranuclear palsy.
Although there is no cure for progressive supranuclear palsy, treatments are available to help ease symptoms of the disorder. The options include:
- Parkinson's disease medications, which increase levels of a brain chemical involved in smooth, controlled muscle movements (dopamine). The effectiveness of these medications is limited and usually temporary, lasting about two to three years.
- Botulinum toxin type A (Botox), which may be injected in small doses into the muscles around your eyes. Botox blocks the chemical signals that cause muscles to contract, which can improve eyelid spasms.
- Eyeglasses with bifocal or prism lenses, which may help ease problems with looking downward.
- Speech and swallowing evaluations, to help you learn safer swallowing techniques.
- Physical therapy and occupational therapy, to improve balance.
Researchers are working to develop medication to treat progressive supranuclear palsy, including therapies that may block the formation of tau.
To minimize the effects of progressive supranuclear palsy, you can take certain steps at home:
- Use eyedrops to help ease dry eyes that can occur as a result of problems with blinking or persistent tearing.
- Install grab bars in hallways and bathrooms, to help you avoid falls.
- Use a walker that is weighted, to help you avoid falls.
- Remove scatter rugs or other items that are hard to see without looking downward.
- Avoid climbing stairs.
Living with any chronic illness can be difficult, and it's normal to feel angry, depressed or discouraged at times. Progressive supranuclear palsy presents special problems because it can cause changes in your brain that make you feel anxious or laugh or cry for no reason. Progressive supranuclear palsy can also become extremely frustrating as walking, talking and eating become more difficult.
To manage the stress of living with progressive supranuclear palsy, consider these suggestions:
- Maintain a strong support system of friends and family.
- Contact a support group, for yourself or for family members.
- Discuss your feelings and concerns about living with progressive supranuclear palsy with your doctor or a counselor
Caring for someone with progressive supranuclear palsy can be difficult physically and emotionally. It's not easy to juggle tasks as you try to adapt to the constantly fluctuating moods and physical needs that accompany this condition. Remember that these moods and physical capabilities may change from hour to hour and are not under the person's conscious control.
- Experience. Mayo Clinic doctors have treated more than 400 people with this uncommon condition.
- Expertise. Mayo Clinic has neurologists who specialize in speech pathology and movement disorders. These specialists have experience identifying speech problems that are a very early feature of progressive supranuclear palsy, and distinguishing the condition from other movement disorders such as Parkinson's disease.
- Latest diagnostic tools. Mayo Clinic doctors use specialized MRI and positron emission tomography (PET) to diagnose progressive supranuclear palsy.
- New ideas. Mayo Clinic researchers are working to improve diagnosis and treatment of progressive supranuclear palsy. You have access to the expertise of Mayo's clinician-researchers.
Mayo Clinic in Rochester, Minn., ranks #1 for neurology and neurosurgery in the U.S. News & World Report Best Hospitals rankings. Mayo Clinic in Scottsdale, Ariz., is ranked among the Best Hospitals for neurology and neurosurgery, and Mayo Clinic in Jacksonville, Fla., is ranked high performing for neurology and neurosurgery by U.S. News & World Report. Mayo Clinic also ranks among the Best Children's Hospitals for neurology and neurosurgery.
At Mayo Clinic, we assemble a team of specialists who take the time to listen and thoroughly understand your health issues and concerns. We tailor the care you receive to your personal health care needs. You can trust our specialists to collaborate and offer you the best possible outcomes, safety and service.
Mayo Clinic is a not-for-profit medical institution that reinvests all earnings into improving medical practice, research and education. We're constantly involved in innovation and medical research, finding solutions to improve your care and quality of life. Your doctor or someone on your medical team is likely involved in research related to your condition.
Our patients tell us that the quality of their interactions, our attention to detail and the efficiency of their visits mean health care — and trusted answers — like they've never experienced.
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Mayo Clinic works with hundreds of insurance companies and is an in-network provider for millions of people. In most cases, Mayo Clinic doesn't require a physician referral. Some insurers require referrals or may have additional requirements for certain medical care. All appointments are prioritized on the basis of medical need.
Specialists in neurology at Mayo Clinic in Arizona diagnose and treat adults with progressive supranuclear palsy.
For appointments or more information, call the Central Appointment Office at 800-446-2279 (toll-free) 8 a.m. to 5 p.m. Mountain Standard Time, Monday through Friday or complete an online appointment request form.
- U.S. Patients
- International Patients
Specialists in neurology at Mayo Clinic in Florida diagnose and treat adults with progressive supranuclear palsy.
For appointments or more information, call the Central Appointment Office at 904-953-0853 8 a.m. to 5 p.m. Eastern time, Monday through Friday or complete an online appointment request form.
- U.S. Patients
- International Patients
Specialists in neurology at Mayo Clinic in Minnesota diagnose and treat adults with progressive supranuclear palsy.
For appointments or more information, call the Central Appointment Office at 507-538-3270 7 a.m. to 6 p.m. Central time, Monday through Friday or complete an online appointment request form.
- U.S. Patients
- International Patients
See information on patient services at the three Mayo Clinic locations, including transportation options and lodging.
Mayo Clinic researchers are working to improve diagnosis and treatment of progressive supranuclear palsy. Specific efforts involve mapping changes in the brains of people with the condition, comparing familial and sporadic cases, using imaging to predict disease progression and to pinpoint diagnosis, and looking for environmental and genetic factors that may be associated with the condition.
Mayo Clinic also has one of the largest progressive supranuclear palsy brain banks in the world.
Read more about research in Mayo Clinic's Parkinson's Disease and Clinical Genetics Laboratory and in the Movement Disorders Laboratory.
See a list of publications by Mayo Clinic doctors on progressive supranuclear palsy on PubMed, a service of the National Library of Medicine.
- J. Eric Ahlskog, M.D., Ph.D.
- Charles H. Adler, M.D., Ph.D.
- Bradley F. Boeve, M.D.
- James H. Bower, M.D.
- John N. Caviness, M.D.
- Keith A. Josephs, M.D.
- Ryan J. Uitti, M.D.
- Jay A. Van Gerpen, M.D.
- Zbigniew K. Wszolek, M.D.
Apr. 10, 2014
- Factor SA. Progressive supranuclear palsy (PSP).http://www.uptodate.com/home. Accessed Dec. 3, 2013.
- Ferri FF. Ferri's Clinical Advisor 2014: 5 Books in 1. Philadelphia, Pa.: Mosby Elsevier; 2014. https://www.clinicalkey.com. Accessed Dec. 3, 2013.
- Ropper AH, et al. Adams & Victor's Principles of Neurology. 9th ed. New York, N.Y.: The McGraw-Hill Companies; 2009. http://www.accessmedicine.com/resourceTOC.aspx?resourceID=54. Accessed Dec. 3, 2013.
- Boeve BF. Progressive supranuclear palsy. Parkinsonism and Related Disorders. 2012;18S1:S192.
- Josephs KA, et al. Modeling trajectories of regional volume loss in progressive supranuclear palsy. Movement Disorders. 2013;28:1117.
- Fujioka S, et al. Similarities between familial and sporadic autopsy-proven progressive supranuclear palsy. Neurology. 2013;80:2076.
- Progressive supranuclear palsy fact sheet. National Institute of Neurological Disorders and Stroke. http://www.ninds.7.gov/disorders/psp/detail_psp.htm. Accessed Dec. 3, 2013.
- Liscic RM, et al. Differentiation of progressive supranuclear palsy: Clinical, imaging and laboratory tools. Acta Neurologica Scandinavica. 2013;127:362.
- Josephs KA, et al. A clinicopathological study of vascular progressive supranuclear palsy. Archives of Neurology. 2002;59:1597.
- Josephs KA, et al. Apraxia of speech and nonfluent aphasia: A new clinical marker for corticobasal degeneration and progressive supranuclear palsy. Current Opinion in Neurology. 2008;21:688.
- Whitwell JL, et al. Neuroimaging comparison of primary progressive apraxia of speech and progressive supranuclear palsy. European Journal of Neurology. 2013;20:629.
- Whitwell JL, et al. Imaging measures predict progression in progressive supranuclear palsy. Movement Disorders. 2012;87:1801.
- Botha H, et al. The pimple sign of progressive supranuclear palsy. Parkinsonism and Related Disorders. In press. Accessed Dec. 3, 2013.
- Coon EA, et al. Primary lateral sclerosis as progressive supranuclear palsy: Diagnosis by diffusion tensor imaging. Movement Disorders. 2012;27:903.
- Josephs KA (expert opinion). Mayo Clinic, Rochester, Minn. Dec. 13, 2013.
- AskMayoExpert. Can rehabilitation help patients with Parkinson's disease and other movement disorders? Rochester, Minn.: Mayo Foundation for Medical Education and Research; 2012.
- A guide for people living with PSP, CBD, and other atypical Parkinsonian disorders. CurePSP. http://www.psp.org/education/. Accessed Dec. 4, 2013.