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Gerstmann-Straussler-Scheinker syndrome

What is Gerstmann-Straussler-Scheinker syndrome?

Gerstmann-Straussler-Scheinker syndrome (GSSS) is a particular and rare form of human transmissible spongiform encephalopathy (TSE) due to a defective gene encoding the prion protein (PRNP gene) and marked by particular multicentric amyloid plaques in the brain.

Other condition names

  • Subacute spongiform encephalopathy, Gerstmann-Straussler type

Inheritance type

Autosomal dominant, Not applicable

Age of Onset

  • Adult
Orphadata: Free access data from Orphanet. © INSERM 1999. Available on "https://www.orphadata.com". Data version 1.3.16 / 4.1.7.