Progressive Supranuclear Palsy

Progressive supranuclear palsy is a disorder characterized by muscle stiffness (rigidity), an inability to move the eyes, and weakness of throat muscles.

Progressive supranuclear palsy, which is much rarer than Parkinson's disease, destroys parts of the basal ganglia and the brain stem. (The basal ganglia help smooth out muscle movements and coordinate changes in posture; the brain stem regulates critical body functions, such as breathing, heart rate, and swallowing, and helps adjust posture.) The cause of the damage to this part of the brain is unknown.

This disorder usually begins in late middle age with abnormalities in posture and an inability to roll the eyes downward. People with the disorder cannot fix their eyes on a stationary object or follow a moving object. They may have blurred or double vision. The upper eyelids may pull back, producing a look of astonishment. Walking is unsteady, with a tendency to fall backward. Speaking and swallowing are difficult, and movements are slow. Other symptoms include insomnia, agitation, irritability, apathy, and rapid changes in emotion.

In the late stages, depression and dementia are common. Like Parkinson's disease, progressive supranuclear palsy results in severe muscle rigidity and disability, usually within 3 to 5 years. Usually, death, often due to infection, occurs within 10 years after symptoms begin.

The diagnosis is based on symptoms. No effective treatment exists, but the drugs used to treat Parkinson's disease may provide some relief.