What Is Progressive Supranuclear Palsy?

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Progressive supranuclear palsy is a rare neurological disorder which often first manifests in the form of difficulty balancing. Also known as Steele-Richardson-Olszewski syndrome, this condition is sometimes misdiagnosed as Parkinson's or Alzheimer's, due to the similarity in symptoms. A proper diagnosis requires evaluation by a neurologist who can perform a complete neurological exam and discuss the patient's history. This condition has no known cure, although the medical community has developed some tactics for managing it.

The cause of progressive supranuclear palsy is not known. The condition starts with degeneration of the area of the brain directly above the region responsible for eye movements. The patient usually has trouble balancing and walking, and may develop slurred speech, difficulty swallowing, and a weakening of the facial muscles. Classically, the patient also has trouble moving his or her eyes, and the condition is also associated with personality changes.

Some researchers have theorized that progressive supranuclear palsy may be caused by a virus-like entity which infects the brain, perhaps lingering for years before symptoms begin to manifest. Others believe that it may be related to free radical damage, or that it may be the result of random genetic mutations. Progressive supranuclear palsy tends to onset in middle age, and is more common in men than women, but beyond this, there are no clear risk factors for the disease.

As the name implies, progressive nuclear palsy is progressive, with the damage becoming worse over time. While patients cannot die directly from this condition, they usually die from a complication related to it. The most common complication which leads to death is pneumonia, caused by difficulty swallowing. Patients can also experience fractures as a result of falls caused by their loss of balance, and head injuries can occur for the same reason.

Many neurodegenerative conditions look similar, especially in the early stages. Damage to the brain may not start to show up on medical imaging studies until the advanced stages of the disease. For this reason, it is important to be carefully evaluated when neurological symptoms occur. The symptoms associated with progressive supranuclear palsy can be caused by other conditions which may be treatable, and it would be a great shame to miss out on that treatment as a result of misdiagnosis. It can help to see several neurologists for evaluation, and to consider attending a specialized neurology clinic to get a chance to work with doctors who see patients with such conditions on a regular basis, and are therefore highly familiar with them.

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1
In the year 2000 my husband died from the results of this sickness. It was actually years before it was said he has PSP. In fact one doctor said he was in the last stages of Alzheimer's. He was very flippant about it. Taking him to another specialist, he said at once what he had. Less than 10 months later he died. He was falling and soon he was unable to use any of his functions and it was almost more than I can talk about. It took a health toll on me. He had no pain but wanted to live. It was so sad. I had little help. And at the last I did not sleep for 5 days and 5 nights. I did not want him to slip away without his hand in mine. I was so tired. So often a doctor is so sure and too fast to call what they think, is not what they see. They are often wrong.
- anon47038

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Written by S.E. Smith
Last Modified: 30 September 2009

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