Progressive Supranuclear Palsy

Overview

Progressive supranuclear palsy is an uncommon brain disorder that causes serious problems with walking, balance and eye movements, and later with swallowing. The disorder results from deterioration of cells in areas of your brain that control body movement, coordination, thinking and other important functions. Progressive supranuclear palsy is also called Steele-Richardson-Olszewski syndrome.

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.

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 not be able to look downward, or experience blurring and double 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. They generally get worse over time and may include:

• Stiffness (especially of the neck) and awkward movements
• Falling, especially falling backward
• Slow or slurred speech
• Problems with swallowing, which may cause gagging or choking
• Sensitivity to bright light
• Sleep disturbances
• Loss of interest in pleasurable activities
• Impulsive behavior, or laughing or crying for no reason
• Difficulties with reasoning, problem-solving and decision-making
• Depression and anxiety
• A surprised or frightened facial expression, resulting from rigid facial muscles
• Dizziness

When to see a doctor

Make an appointment with your doctor if you experience any of the signs and symptoms listed above.

Causes

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 and thinking.

Researchers have found that the deteriorating brain cells of people with progressive supranuclear palsy have excess amounts of a protein called tau. Clumps of tau are also found in 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.

Risk factors

The only proven risk factor for progressive supranuclear palsy is age. The condition typically affects people in their late 60s and 70s. It's virtually unknown in people under the age of 40.

Complications

Complications of progressive supranuclear palsy result primarily from slow and difficult 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, which can lead to a feeling of tiredness and excessive daytime sleeping
• Difficulty looking at bright lights
• Problems swallowing, which can lead to choking or inhaling food or liquid into your airway (aspiration)
• Pneumonia, which can be caused by aspiration and is 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.

Diagnosis

Progressive supranuclear palsy can be difficult to diagnose because signs and symptoms are similar to those of Parkinson's disease. Your doctor may suspect that you have progressive supranuclear palsy rather than Parkinson's disease if you:

• Don't have tremors
• Are having a lot of unexplained falls
• Have little or no response to Parkinson's medications
• Have difficulty moving your eyes, particularly downward

Your doctor may recommend an MRI to determine if you have shrinkage in specific regions of the brain associated with progressive supranuclear palsy. An MRI can also help to exclude disorders that may mimic progressive supranuclear palsy, such as a stroke.

A positron emission tomography (PET) scan also may be recommended to check for early signs of changes in the brain that may not appear on an MRI.

Treatment

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. The effectiveness of these medications is limited and usually temporary, lasting about 2 to 3 years in most patients.
• OnabotulinumtoxinA (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. Prism lenses allow people with progressive supranuclear palsy to see downward without moving their eyes down.
• Speech and swallowing evaluations, to help you learn about alternative means to communicate and safer swallowing techniques.
• Physical therapy and occupational therapy, to improve balance. Facial exercises, talking keyboards, gait and balance training also can help with many of the symptoms of progressive supranuclear palsy.

Researchers are working to develop medication to treat progressive supranuclear palsy, including therapies that may block the formation of tau or help to destroy tau.

Clinical trials

Explore Mayo Clinic studies testing new treatments, interventions and tests as a means to prevent, detect, treat or manage this condition.

Lifestyle and home remedies

To minimize the effects of progressive supranuclear palsy, you can take certain steps at home:

• Use eye drops multiple times a day 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 prevent falling backward.
• Remove small area rugs or other items that are hard to see without looking downward.
• Don't climb stairs.

Coping and support

Living with any chronic illness can be challenging, and it's common 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

For caregivers

Caring for someone with progressive supranuclear palsy can be challenging physically and emotionally. It's not easy to juggle tasks as you try to adapt to the constantly changing 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 control.

Preparing for your appointment

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 allow more time to go over points you'd like to address. 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?