Progressive supranuclear palsy

Overview

Progressive supranuclear palsy is a rare brain disease that affects walking, balance, eye movements and swallowing. The disease results from the damage of cells in areas of the brain that control body movement, coordination, thinking and other important functions. Progressive supranuclear palsy also is called Steele-Richardson-Olszewski syndrome.

Progressive supranuclear palsy worsens over time and can lead to dangerous complications, such as pneumonia and trouble swallowing. There's no cure for progressive supranuclear palsy, so treatment focuses on managing the symptoms.

Symptoms

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. People with progressive supranuclear palsy may not be able to look downward. Or they may experience blurring and double vision. Not being able to focus the eyes can make some people spill food. They also may appear disinterested in conversation because of lack of eye contact.

Additional symptoms of progressive supranuclear palsy vary and may mimic those of Parkinson's disease and dementia. Symptoms get worse over time and may include:

• Stiffness, especially of the neck, and awkward movements.
• Falling, especially falling backward.
• Slow or slurred speech.
• Trouble swallowing, which may cause gagging or choking.
• Being sensitive to bright light.
• Trouble with sleep.
• Loss of interest in pleasurable activities.
• Impulsive behavior, or laughing or crying for no reason.
• Trouble 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 healthcare professional if you experience any of the symptoms listed above.

Causes

The cause of progressive supranuclear palsy isn't known. Its symptoms result from the damage of cells in areas of the brain, especially areas that help you control body movements and thinking.

Researchers have found that the damaged brain cells of people with progressive supranuclear palsy have excess amounts of a protein called tau. Clumps of tau also are found in other brain diseases, such as Alzheimer's disease.

Rarely, progressive supranuclear palsy occurs within a family. But a genetic link isn't clear. 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.
• Trouble focusing your eyes, which also can lead to injuries.
• Trouble sleeping, which can lead to feeling tired and excessive daytime sleeping.
• Not being able to look at bright lights.
• Trouble swallowing, which can lead to choking or inhaling food or liquid into the airway, known as aspiration.
• Pneumonia, which can be caused by aspiration. Pneumonia 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 healthcare professional 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 hard to diagnose because symptoms are similar to those of Parkinson's disease. Your healthcare professional 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, temporary or no response to Parkinson's medicines.
• Have trouble moving your eyes, particularly downward.

You may need an MRI to learn if you have shrinkage in specific regions of the brain associated with progressive supranuclear palsy. An MRI also can help 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 medicines, which increase levels of a brain chemical involved in smooth, controlled muscle movements. The effectiveness of these medicines 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.
• Antidepressants. Some antidepressant medicines may have a modest effect on symptoms such as impulsive behavior.
• 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 other ways to communicate and safer swallowing techniques.
• Physical therapy and occupational therapy, to improve balance. Facial exercises, talking keyboards and gait and balance training also can help with many of the symptoms of progressive supranuclear palsy.

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

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. Some people may feel angry, depressed or discouraged at times. Progressive supranuclear palsy can cause changes in your brain that make you feel anxious or laugh or cry for no reason. Progressive supranuclear palsy also can become frustrating as walking, talking and eating become harder.

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 healthcare professional 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 an 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 medicines, 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 healthcare professional.
• Ask a relative or friend to come with you to help you remember what the healthcare professional 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, don't hesitate to ask other questions during your appointment.

What to expect from your doctor

Your healthcare professional 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 hard 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?