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.