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Progressive supranuclear palsy, also called Steele-Richardson-Olszewski syndrome, is a brain disorder that causes serious problems with walking, balance and eye movements. Although the cause is unknown, progressive supranuclear palsy results from deterioration of cells in areas of your brain that control movement.

Progressive supranuclear palsy is a rare disorder that slowly worsens over time. It usually affects adults over the age of 60, though some people are diagnosed as young as age 40. While progressive supranuclear palsy is not life-threatening itself, it can lead to life-threatening complications such as pneumonia and swallowing problems.

Because there's no cure for progressive supranuclear palsy, treatment focuses on managing and improving the disorder's signs and symptoms.

The characteristic signs and symptoms of progressive supranuclear palsy include:

• A loss of balance while walking. You'll likely have a greater tendency to fall backward, not forward. This can occur very early on in the disease.
• An inability to aim your eyes properly. This is particularly true when looking downward, or may occur as a blurring or doubling of vision. This difficulty focusing the eyes can make some people appear disinterested in conversation because of poor eye contact.

Additional signs and symptoms of progressive supranuclear palsy vary from person to person, and may mimic those of Parkinson's disease and dementia. These can include:

• Stiffness
• Awkward movements
• Falling
• Problems with speech and swallowing
• Irritability
• Loss of interest in pleasurable activities (apathy)
• Anxiety
• Laughing or crying for no reason
• Forgetfulness

True to its name, the signs and symptoms of progressive supranuclear palsy tend to become progressively worse as the disease advances.

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. However, the signs and symptoms of the disease result from deterioration of brain cells in your brainstem, cortex and basal ganglia. These areas of your brain help you control body movements. This explains why their deterioration leads to the coordination and movement problems of the disease.

Although no known cause exists, scientists do have several theories about what might be behind supranuclear palsy. These include:

• A virus. Some speculate that a unique virus enters your body, taking many years before it becomes active and starts causing signs and symptoms.
• Genetic mutations. It's possible that random changes (mutations), which may develop in everyone's genes, occur in specific genes to damage the cells involved in progressive supranuclear palsy.
• Environmental exposure. Some scientists think an unknown chemical in the environment could be what causes this disease. This chemical could be in something you eat, drink or breathe. For example, certain tropical fruits have been linked to progressive supranuclear palsy-like symptoms in people on some Caribbean islands.
• Damage from free radicals. As you process food for energy, your body produces substances called free radicals. Free radicals are believed to contribute to aging and certain diseases. They may damage the brain cells involved with the signs and symptoms of progressive supranuclear palsy.

Age is the most significant known risk factor for progressive supranuclear palsy. The disease typically affects people over the age of 60. Also, men are affected somewhat more often than women.

Complications of progressive supranuclear palsy result primarily from hindered muscle movements. These complications may include:

• Frequent falling, which can lead to head injuries, fractures and other injuries
• Difficulty focusing your eyes, which also can lead to injuries
• Problems reading, or with other tasks requiring hand-eye coordination
• The need for a wheelchair
• Dependence on others for care
• Difficulty sleeping
• Difficulty looking at bright lights
• Problems swallowing

Swallowing difficulties 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.

Because progressive supranuclear palsy is diagnosed by your signs and symptoms, bring a list of any symptoms you're experiencing to your appointment. Write down any unusual symptoms you've noticed — even if you don't think they're related to your condition. To help your appointment run smoothly, it's also a good idea to write down any questions you have for your doctor ahead of time.

What to expect from your doctor
Your doctor is likely to ask you a number of questions, as well. He or she may ask:

• Have you experienced problems with balance or walking?
• Do you find it difficult to see below you, for example seeing your plate when 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 first begin experiencing these symptoms?
• Have these symptoms been continuous, or occasional?
• Does anything seem to improve or worsen these symptoms?
• What, if any, treatments have you tried?

Your doctor diagnoses progressive supranuclear palsy by identifying the disorder's key signs — problems with balance and walking and difficulty moving your eyes, particularly downward — and ruling out other similar disorders.

Progressive supranuclear palsy can be difficult to diagnose because some signs and symptoms, such as stiffness, movement difficulties, and changes in mood or personality, are similar to those of Parkinson's disease and dementia. In fact, because of its similarity to Parkinson's disease, it's considered a "parkinsonism-plus syndrome."

Indications that you have progressive supranuclear palsy rather than Parkinson's disease include a lack of shaking (tremors) and a poor response to Parkinson's medications.

Though a diagnosis can be made through this evaluation process, currently the only definitive way to determine whether a person has progressive supranuclear palsy is through a brain autopsy. Studies are ongoing to develop medical tests that can help diagnose progressive supranuclear palsy.

No cure exists for progressive supranuclear palsy. However, certain medications and other measures can help improve some symptoms of this disorder.

Medications

• Parkinson's disease medications. These include carbidopa-levodopa, dopamine agonists, and amantadine, which increase levels of dopamine — a chemical that transmits signals between areas of your brain to allow smooth, controlled muscle movements. These medications may help improve slowness, stiffness and balance problems in about 30 percent of people. However, the effectiveness of the drugs is limited and usually temporary, lasting about two years.
• Certain antidepressants. Antidepressant drugs including fluoxetine (Prozac), imipramine (Tofranil) and amitriptyline, may improve symptoms, as well. How they do this is not known; benefits don't seem related to their ability to treat depression.

Therapies

• Botulinum toxin (Botox). This purified form of botulinum toxin may be injected into the muscles or tissue around your eyes. When injected in small doses into specific muscles, Botox blocks the chemical signals that cause muscles to contract, which can improve eyelid spasms, although not the movement of the eyes.
• Eyeglasses with bifocal or prism lenses. These aids may help alleviate problems with looking downward.
• Speech and swallowing evaluations. Conducted by a speech therapist, these evaluations can help the therapist advise you on safer swallowing techniques.
• Physical therapy and occupational therapy. This can help improve balance to avoid falls and to help with daily living activities.

There are some things you can do at home to minimize the effects of progressive supranuclear palsy. They may include:

• Eyedrops. Eyedrops may help ease dry eyes that can occur as a result of problems blinking.
• Fall-avoidance aids. Installing grab bars in hallways and bathrooms or using a walker that's weighted to help prevent backward falls can help you avoid falls. Making home modifications, such as removing scatter rugs or other items that are hard to see without looking downward, also can help with balance and vision problems. When possible, 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 even eating become more difficult.

The good news is that there are steps you can take to manage the stress of living with progressive supranuclear palsy. Consider these suggestions:

• Learn all you can about your illness. Find out how the disease progresses, your prognosis, and your treatment options and their side effects. The more you know, the more active you can be in your own care. In addition to talking to your health care team, look for books and information on the Internet, including the Web sites of various progressive supranuclear palsy organizations.
• Be proactive. Although you may often feel anxious or discouraged, don't let others — including your family and your doctors — make important decisions for you. Take an active role in your treatment.
• Maintain a strong support system. Strong relationships are crucial in dealing with chronic illnesses. Although friends and family can be your best allies, the understanding of people who know what you're going through can be especially helpful. Support groups aren't for everyone, but for many people, they can be a good resource for practical information. You may also find that you develop lasting bonds with people who are going through the same experiences as you. Support groups may also exist for the families of people with progressive supranuclear palsy. To learn about support groups in your community, talk to your doctor, a social worker or a local public health nurse.
• Maintain good communication with your partner and support system. It's extremely important to be open about your feelings, especially when it comes to living with progressive supranuclear palsy. The disease may affect your life and the lives of your loved ones in a number of ways. Mood and personality changes or inappropriate behavior, such as sudden bursts of laughing or crying, may make communication challenging. It's best if you can talk honestly about these changes. The amount of care a person with progressive supranuclear palsy needs also is often an issue. Because it may take you longer to do ordinary tasks, your partner or family might want to help. But you may want to remain as independent as possible. You'll need to let them know when you need help and when you don't.

Most important, try to talk frankly about your feelings and concerns. Repressed feelings can be harmful to your immediate well-being and long-term health. If necessary, discuss your problems with your doctor or counselor.