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Progressive
supranuclear palsy is characterized by muscle stiffness (rigidity),
inability to move the eyes, weakness of the throat muscles, and
a tendency to fall backward.
Progressive supranuclear palsy, which is much rarer than Parkinson's disease, affects many parts of the brain, particularly 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.) Brain cells in these areas degenerate, but why they do is unknown.
Symptoms usually begin in late middle age. The first symptom may be difficulty looking up without bending the neck or difficulty climbing up and down stairs. People with the disorder cannot roll their eyes downward, 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. Muscles become rigid, and movements are slow. Walking is unsteady, with a tendency to fall backward. Speaking and swallowing are difficult. 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 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. The drugs used to treat Parkinson's disease provide some relief.
Last full review/revision August 2007 by David Eidelberg, MD; Michael Pourfar, MD
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