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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
See Disclaimer regarding information on this site. Some links on this page may take you to organizations outside of the National Institutes of Health.

Your Question

My identical twin sister has been diagnosed with progressive supranuclear palsy (PSP).  As it seems this disease may have a genetic cause, what is the chance that I would also develop PSP?  And within what timeframe?

Our Answer

We have identified the following information that we hope you find helpful. If you still have questions, please contact us.

What is the chance of developing progressive supranuclear palsy if a relative is affected?

Researchers do not yet completely understand the causes of progressive supranuclear palsy (PSP), so it is not possible to predict the chance of a relative developing the condition if one family member has been diagnosed.  Most of the time, only one person in a family develops PSP. It is not very common to have multiple members of a family develop PSP.  One study found that 7% of individuals with PSP had a family history of potentially related conditions (including dementia or parkinsonism), which suggests there may be a shared genetic risk factor.[1][2]

A few genetic risk factors (particularly a gene called MAPT) are known to play a role in the development of PSP.[3][4]  However, these genetic risk factors have only been found in a few families. Researchers suspect that there are other genetic factors that haven’t been found yet that also contribute to progressive supranuclear palsy.
Last updated: 9/12/2012

If a family member has a known genetic risk factor for progressive supranuclear palsy, what is the chance a relative has inherited it?

Close relatives such as parents, siblings, and children of an affected individual have a greater chance of sharing a genetic predisposition than do aunts, uncles, grandparents or cousins. Identical twins share their entire DNA, so they should have the same genetic risk factors.
Last updated: 9/12/2012

If an individual inherited a known risk factor, what is the chance of developing progressive supranuclear palsy?

Having a genetic risk factor increases the chance of developing progressive supranuclear palsy (PSP); it does not guarantee that an individual will develop this disease at some point in their lifetime.  The exact risk of developing PSP in an individual with a genetic predisposition is currently unknown.[5]
Last updated: 9/12/2012

What is the average age at diagnosis for progressive supranuclear palsy?

Individuals with progressive supranuclear palsy usually experience the first symptoms of this disease while in their 60s.[1]
Last updated: 9/7/2012

References
Other Names for this Disease
  • Familial progressive supranuclear palsy (type)
  • PSP
  • Steele-Richardson-Olszewski Syndrome
  • Supranuclear palsy, progressive
See Disclaimer regarding information on this site. Some links on this page may take you to organizations outside of the National Institutes of Health.