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 (<i>PRNP</i> 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
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