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Diseases

Genetic and Rare Diseases Information Center (GARD)

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Progressive supranuclear palsy


Other Names for this Disease
  • Familial progressive supranuclear palsy (type)
  • PSP
  • Steele-Richardson-Olszewski Syndrome
  • Supranuclear palsy, progressive
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Cause


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What causes progressive supranuclear palsy?

The symptoms of progressive supranuclear palsy are caused by a gradual deterioration of brain cells in a few tiny but important places at the base of the brain, in the region called the brainstem. However, scientists do not know what causes these brain cells to degenerate.[1] In most cases, the genetic cause of this condition is unknown. Rarely, the disease results from mutations in the MAPT gene.[2] 

There are, however, several theories about progressive supranuclear palsy's cause. One possibility is that a virus-like agent infects the body and takes years or decades to start producing visible effects. Another possibility is that random genetic mutations occur in particular cells or certain genes, in just the right combination to injure these cells. A third possibility is that there is exposure to some unknown chemical in the food, air, or water which slowly damages certain vulnerable areas of the brain.[1]

Another possible cause is cellular damage caused by free radicals, reactive molecules produced continuously by all cells during normal metabolism. Although the body has built-in mechanisms for clearing free radicals from the system, scientists suspect that, under certain circumstances, free radicals can react with and damage other molecules. More research is needed and underway to help the medical field better understand this condition and its cause.[1]
Last updated: 8/18/2011

References
  1. Progressive Supranuclear Palsy Fact Sheet. National Institute for Neurological Disorders and Stroke (NINDS). August 16, 2011; http://www.ninds.nih.gov/disorders/psp/detail_psp.htm. Accessed 8/18/2011.
  2. Progressive supranuclear palsy. Genetics Home Reference. March 2011; http://ghr.nlm.nih.gov/condition/progressive-supranuclear-palsy. Accessed 9/7/2012.