Progressive supranuclear palsy is a movement disorder that occurs from damage to certain nerve cells in the brain.
Dementia-nuchal dystonia; Richardson-Steele-Olszewski syndrome; Palsy - progressive supranuclear
Progressive supranuclear palsy is a condition that causes symptoms similar to those of Parkinson's disease.
It involves damage to many cells of the brain. Many areas are affected, including the part of the brainstem where cells that control eye movement are located. Also affected is an area of the brain that controls steadiness when you walk. The frontal lobes of the brain are also affected, leading to personality changes.
The cause of the damage to the brain cells is unknown. The disease gets worse over time.
People with this condition have deposits in brain tissues that look like those found in patients with Alzheimer's disease. There is a loss of tissue in most areas of the brain and in some parts of the spinal cord.
The disorder is most often seen in people over 60 years old, and is somewhat more common in men.
An exam of the nervous system (neurological examination) may show:
The health care provider may do the following tests to rule out other diseases:
The goal of treatment is to control symptoms. There is no known cure for progressive supranuclear palsy.
Levodopa or other drugs (such as benztropine or trihexyphenidyl) block the action of a nervous system chemical called acetylcholine (anticholinergic medications). They may temporarily reduce some symptoms, such as rigid limbs or slow movements. However, these medications are usually not as effective as they are for Parkinson's disease.
Many people with this condition will need around-the-clock care and monitoring as they lose brain functions.
Treatment sometimes can reduce symptoms for a period of time, but the condition will get worse. Brain function will decline over time. Death commonly occurs in 5 to 7 years.
Newer drugs are being studied to treat this condition.
Call your health care provider if you often fall, and if you have a stiff neck/body and vision problems.
Also, call if a loved one has been diagnosed with progressive supranuclear palsy and the condition has declined so much that you can no longer care for the person at home.
Boeve BF. Progressive supranuclear palsy. 2012. 18 Suppl 1:S192-194.