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Diseases

Genetic and Rare Diseases Information Center (GARD)

Gerstmann-Straussler-Scheinker disease


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

Newline Maker

How is Gerstmann-Straussler-Scheinker disease inherited?

Gerstmann-Straussler-Scheinker disease (GSS) is inherited in an autosomal dominant manner.[1] This means that to be affected, a person only needs a change (mutation) in 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 history of the disorder in their family. A person with GSS has a 50% chance with each pregnancy of passing along the altered gene to his or her child.
Last updated: 8/19/2015

References
  1. Henry G Brown, MD, PhD; John M Lee, MD, PhD. Diseases of the central nervous system caused by prions. UpToDate. October 2014; Accessed 8/19/2015.


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