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Progressive supranuclear palsy

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Illustrations

Central nervous system
Central nervous system

Alternative Names    Return to top

Dementia-nuchal dystonia; Richardson-Steele-Olszewski syndrome; Palsy - progressive supranuclear

Definition    Return to top

Progressive supranuclear palsy is a movement disorder caused by damage to certain nerve cells in the brain.

Causes    Return to top

Progressive supranuclear palsy is a condition that causes symptoms similar to Parkinson's disease. Symptoms may include a progressive lack of coordination, stiffness of the neck and body, difficulties with eye movement, slow movements, problems with mental function, and difficulty walking that can result in falls.

Progressive supranuclear palsy involves damage to multiple cells of the brain. The covering of certain nerve cells (myelin sheath) is destroyed. Entire nerves may be damaged in some areas. The cause of the damage to the brain cells in this condition is unknown. The disease is degenerative (gets worse over time).

People with this condition have deposits in brain tissues that resemble those found in patients with Alzheimer's disease. There is a loss of tissue in most areas of the brain.

The disorder is most often seen in people over 60 years old, and is somewhat more common in men.

Symptoms    Return to top

Exams and Tests    Return to top

A neurological examination may show Parkinsonian movements with typical stiffness and lack of coordination. Dementia is mild. Eye movements are limited. However, vision, hearing, sensation, and voluntary control of movement remain normal.

Tests may be done to rule out other diseases. An MRI might show shrinking of the brainstem.

Treatment    Return to top

Treatment is aimed at controlling symptoms. There is no known cure for progressive supranuclear palsy. Levodopa and anticholinergic medications may provide temporary reduction of symptoms.

These are not as effective as in Parkinson's disease, however. Around-the-clock care and monitoring are eventually required because of progressive loss of brain functions.

Outlook (Prognosis)    Return to top

Treatment sometimes provides temporary reduction of symptoms, but the disorder inevitably progresses. There is a steady decline of brain function, with death commonly occurring in 5 to 7 years.

Possible Complications    Return to top

When to Contact a Medical Professional    Return to top

Call your health care provider if frequent falls accompanied by stiff neck/body and vision difficulties occur.

Also, call if progressive supranuclear palsy has been diagnosed and the condition declines to a point when it is not possible to care for the person in the home.

Update Date: 8/26/2006

Updated by: Gail A. Kang, M.D., San Francisco VA Parkinson's Disease Research, Education, & Clinical Center, San Francisco, CA. Review provided by VeriMed Healthcare Network.

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