Gerstmann-Straussler-Scheinker disease

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Disease Overview

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. Signs and symptoms generally develop between ages 35 and 50 years and may include weakness in the legs, poor reflexes, abnormal sensations, progressive ataxia, cognitive dysfunction, slurred speech, and spasticity. On average, people affected by GSS survive approximately 60 months (range 2 to 10 years) following diagnosis. It is caused by changes (mutations) in the PRNP gene and inheritance is autosomal dominant. Treatment is based on the signs and symptoms present in each person.[7510][9514]

For information on other prion diseases, please visit GARD’s Creutzfeldt-Jakob disease and fatal familial insomnia pages.


Synonyms

  • 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
  • Gerstmann-Straussler-Scheinker syndrome
  • Subacute spongiform encephalopathy, Gerstmann-Straussler type

For more information, visit GARD.

National Organization for Rare Disorders