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Diseases

Genetic and Rare Diseases Information Center (GARD)

Gerstmann-Straussler-Scheinker disease


Other Names for this Disease
  • GSSD
  • Gerstmann Straussler Scheinker syndrome
  • Cerebellar ataxia, progressive dementia, and amyloid deposits in the central nervous system
  • Encephalopathy subacute spongiform Gerstmann-Straussler type
  • Amyloidosis cerebral with spongiform encephalopathy
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Inheritance

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How is Gerstmann-Straussler-Scheinker disease inherited?

Gerstmann-Straussler-Scheinker disease (GSS) is inherited in an autosomal dominant manner.[1] Every cell of the body has two copies of a gene. In autosomal dominant disorders, to be affected, a person needs a change (mutation) in only one copy of the responsible gene in each cell. In some cases, an affected person inherits the mutation from an affected parent. Other cases may result from new (de novo) mutations in the gene. These cases occur in people with no family history of the disorder. A person with GSS has a 50% chance with each pregnancy of passing along the mutated gene to his or her child.
Last updated: 7/11/2016

References
  1. Brown HG & Lee JM. Diseases of the central nervous system caused by prions. UpToDate. October 2014; http://www.uptodate.com/contents/diseases-of-the-central-nervous-system-caused-by-prions.


Other Names for this Disease
  • GSSD
  • Gerstmann Straussler Scheinker syndrome
  • Cerebellar ataxia, progressive dementia, and amyloid deposits in the central nervous system
  • Encephalopathy subacute spongiform Gerstmann-Straussler type
  • Amyloidosis cerebral with spongiform encephalopathy
See Disclaimer regarding information on this site. Some links on this page may take you to organizations outside of the National Institutes of Health.