Progressive Supranuclear Palsy

In progressive supranuclear palsy, muscles become stiff (rigid), the eyes become unable to move, and the throat muscles become weak. Usually, this incurable disorder progresses rapidly.

Progressive supranuclear palsy is similar to Parkinson's disease, but it is much rarer. Progressive supranuclear palsy usually begins after age 50. The cause is unknown.

Progressive supranuclear palsy destroys parts of the basal ganglia and the brain stem. The basal ganglia help coordinate and smooth out movements. The brain stem controls vital body functions (such as breathing, heart rate, and swallowing). Clusters of nerve cells in the brain stem control eye movements.

Symptoms

Usually, the most obvious symptoms are vision problems. People with progressive supranuclear palsy cannot roll their eyes downward. They have difficulty focusing on a stationary object or following a moving object. Because they cannot move their eyes normally, reading is difficult. The upper eyelids may pull back, making people with the disorder look astonished. People may have difficulty opening and closing their eyes. They may blink less often, resulting in dry eyes. The neck tends to arch backward. Muscles become very rigid, and movements are slow. Steps become wider, and walking becomes unsteady. Falls, particularly backward, are more likely.

Speaking becomes slurred and guttural. Swallowing becomes increasingly difficult. As a result, aspiration pneumonia is likely to develop.

As the disorder progresses, people may have insomnia. They become irritable and agitated easily. Emotions may change rapidly. They may laugh one moment and cry the next. They may become apathetic. Late in the disorder, depression and confusion due to dementia are common.

Diagnosis and Treatment

The diagnosis is based on symptoms, particularly vision problems and the tendency to fall backward. Progressive supranuclear palsy is sometimes mistaken for Parkinson's disease because the symptoms of the two disorders are similar. However, noticeable tremors are less common than in Parkinson's disease. No tests can directly confirm the diagnosis.

No effective treatment exists. But the drugs used to treat Parkinson's disease, such as levodopa and pramipexole, may provide some relief for rigid muscles. Antidepressants may help relieve symptoms of depression and reduce the rapid changes in emotion.

People may use weighted walking aids or even a wheelchair to help prevent falls. Bifocals and special glasses with prisms or tinted lenses may help with vision problems.

People may be evaluated periodically to determine how well they swallow. If swallowing becomes difficult, health care practitioners may recommend eating certain foods that are more easily swallowed. Swallowing may become so difficult late in the disorder that practitioners recommend placing a tube directly into the stomach (a procedure called gastrostomy).

Outlook

Progressive supranuclear palsy results in disability, usually within 3 to 5 years. Usually, death, often due to infection, occurs within 10 years after symptoms begin.

People with progressive supranuclear palsy should prepare advance directives, indicating what kind of medical care they want at the end of life.