Learn about this brain disorder that interferes with the ability to walk, move your eyes, talk and eat.
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.
Deterioration of cells in the brainstem, cerebral cortex, cerebellum and basal ganglia — a cluster of cells deep within your brain — is what causes the coordination and movement issues of progressive supranuclear palsy.
The characteristic signs and symptoms of progressive supranuclear palsy include:
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:
Make an appointment with your doctor if you experience any of the signs and symptoms listed above.
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.
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 of progressive supranuclear palsy result primarily from slow and difficult muscle movements. These complications may include:
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.
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:
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.
Although there is no cure for progressive supranuclear palsy, treatments are available to help ease symptoms of the disorder. The options include:
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.
To minimize the effects of progressive supranuclear palsy, you can take certain steps at home:
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:
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.
You may be referred to a doctor who specializes in disorders of the brain and nervous system (neurologist).
In addition to the questions that you've prepared to ask your doctor, don't hesitate to ask other questions during your appointment.
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: