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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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Inheritance

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Is progressive supranuclear palsy inherited?

It is very rare for a person with progressive supranuclear palsy (PSP) to have a family history of the condition. This means that most cases of PSP are sporadic, and are not inherited.[1] Some people with PSP do have a family history of related conditions, such as parkinsonism and/or dementia.[2]

In the few families with multiple affected family members, autosomal dominant inheritance has been suggested.[3][2] In autosomal dominant inheritance, having only one mutated copy of a disease-causing gene is enough to cause signs and symptoms of the condition. A few cases of PSP have been associated with having certain variations or mutations in the MAPT gene.[1][2] However, in most cases, the responsible gene has yet to be identified. There may also be genes in which variants increase a person's risk to develop PSP. This means that the condition itself is not inherited, but a predisposition to developing PSP or a related condition may be inherited.[1]
Last updated: 9/2/2014

References
  1. Progressive supranuclear palsy. NORD. January 27, 2014; http://www.rarediseases.org/rare-disease-information/rare-diseases/byID/287/viewAbstract. Accessed 9/2/2014.
  2. Progressive supranuclear palsy. Genetics Home Reference. March 2011; http://ghr.nlm.nih.gov/condition/progressive-supranuclear-palsy. Accessed 9/7/2012.
  3. Cassandra L. Kniffin. Progressive supranuclear palsy. OMIM. May 31, 2012; http://omim.org/entry/601104. Accessed 9/2/2014.


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.