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In this section, you or a loved one can find out more about medical treatments and practical information about progressive supranuclear palsy. Read on to find answers to some of your questions as well as links to other information. Being informed is an important first step toward becoming an active decision-maker in your care plan.
Progressive supranuclear palsy (PSP), sometimes called Steele-Richardson-Olszewski syndrome, is a relatively rare degenerative brain disease. The supranuclear and palsy parts of the name refer to the eye muscle weakness that is characteristic of the disease. PSP affects many ways a person functions, including movement, balance, speech, mood and thinking. It is typically diagnosed in people who are older than age 60.
Although researchers are studying possible genetic and environmental links, the exact cause of PSP is unknown. The disease causes brain cells to deteriorate, especially brain cells in the areas that control movement and thinking. Researchers have found that the deteriorating brain cells contain abnormal amounts of a protein called tau, which is also characteristic of other diseases, including Alzheimer's disease.
PSP causes problems with walking, vision, speech, swallowing, thinking and mood regulation. This contributes to more serious problems such as pneumonia, head injuries, choking and fractures. PSP is often misdiagnosed because of its initial similarity to Parkinson's disease; however, there are differences. Patients with PSP typically stand exceptionally straight (or tilt their heads and sometimes fall backward), but patients with Parkinson's disease typically bend forward. Tremor is also less common in PSP than in Parkinson's disease. There is a type of PSP called PSP-parkinsonism that more closely resembles Parkinson's disease in its early stages, with fewer balance and behavior issues and more tremor. PSP typically progresses more quickly than Parkinson's disease.
PSP affects the brain and many parts of the body controlled by the brain and may mimic both Parkinson's disease and dementia.
The most common symptoms are:
Effects on individuals can vary, but PSP will typically lead to severe disability within 3 to 5 years.
Bladder Complications: PSP affects the nerve path linking the bladder to the spinal cord, which can cause sudden feelings of urgency and incontinence. If the problem isn't caused by a bladder infection, the symptoms often respond to drugs that stimulate the muscle at the neck of the bladder.
Pressure sores: People with PSP who sit or recline in one position too long may suffer pressure sores, also known as bedsores. Pressure sores can start in less than 2 hours, and they easily become infected. If left untreated, they can quickly reach to the bone.
Because there is currently no cure for PSP, treatment focuses on managing its signs and symptoms. Good medical and nutritional care can help patients with PSP to live a decade or more after disease onset. Patients may want to find a movement disorder specialist. Other care team members may include an occupational therapist, a physical therapist, a speech pathologist and a social worker.
Doctors may treat people with PSP with the following types of medications:
Please be sure to consult with your physician.
For most people, regardless of having PSP or not, exercise, healthy eating, and good sleep habits are recommended. A healthy lifestyle can lead to enhanced quality of life for most people. Talk to your doctor before making any lifestyle changes.
Nutrition and fluid intake: People with PSP often have trouble swallowing, which can lead to choking, aspiration and pneumonia. To avoid these complications, patients with PSP may eventually need a feeding tube. People with PSP should also be sure to take in enough fluids to lessen the risk of bladder infections and to include fiber in their diets to prevent constipation.
Avoiding falls: Because PSP can cause loss of balance and falls, people with PSP may want to use a weighted walker or wheelchair. Shoes with built-up heels may be another option to avoid the backward falls characteristic of PSP. Smooth soles may be better than athletic soles because some patients with PSP experience difficulty lifting the foot to initiate a step. In the home, it may be a good idea to install grab bars and remove scatter rugs and other objects that are hard to see without looking downward. People with PSP should avoid stairs and other tripping hazards as well.
Eye care: People with PSP may want to use eyedrops to help ease problems with dry eyes as well as persistent tearing. Use of glasses with special prisms may also help people with PSP more easily see downward. Special eyelid crutches that attach to eyeglasses may help some people who are having trouble keeping their eyes open.
Oral hygiene: People with PSP may have trouble swallowing, which puts them at increased risk for aspiration and pneumonia. Maintaining good oral hygiene, with regular or increased dentist visits, can cut down on the amount of harmful bacteria in the mouth that could enter the lungs during coughing or choking. Good oral hygiene remains important even if food is being given through a feeding tube.
Mental health: Living with PSP can be frustrating for patients and for their caregivers, and depression and anxiety are common. Ways to manage the stress include talking honestly with your doctor, seeking help from mental health professionals, and joining support groups.
Exercise: Though formal physical therapy has no proven benefit, certain exercises, performed while seated or reclining for safety, may keep the joints limber and provide psychological benefits.