Learn About Gerstmann-Straussler-Scheinker Syndrome

What is the definition of Gerstmann-Straussler-Scheinker Syndrome?
Gerstmann-Straussler-Scheinker disease (GSS) is a type of prion disease. Prion diseases are a group of conditions that affect the nervous system. The main feature of GSS is a progressive degeneration of the cerebellum (a part of the brain that controls coordination, balance, equilibrium and muscle tone), as well as different degrees of dementia. It is caused by changes in the PRNP gene and inheritance is autosomal dominant.
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What are the alternative names for Gerstmann-Straussler-Scheinker Syndrome?
  • Gerstmann-Straussler-Scheinker disease
  • Amyloidosis cerebral with spongiform encephalopathy
  • Cerebellar ataxia, progressive dementia, and amyloid deposits in the central nervous system
  • Encephalopathy subacute spongiform Gerstmann-Straussler type
  • GSSD
  • Gerstmann Straussler Scheinker syndrome
  • Gerstmann-Straussler-Scheinker syndrome
  • Subacute spongiform encephalopathy, Gerstmann-Straussler type
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Published Date: May 02, 2022
Published By: Genetic and Rare Diseases Informnation Center

What are the Latest Advances for Gerstmann-Straussler-Scheinker Syndrome?

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