"GSSD is caused by autosomal dominant point mutations in the prion protein gene, for example missense or nonsense mutations 10. This results in a misfolded pathogenic isoform of the prion protein gene (PrPSc), which aggregate to form amyloid deposits that damage brain parenchyma10."
"Gerstmann-Straussler-Scheinker disease (GSSD) is a rare type of transmissible spongiform encephalopathy. It manifests with dementia and/or ataxia and is due to a mutation in the prion protein (PRNP) gene, which is inherited in an autosomal dominant pattern."
"Treatment and Prognosis"
Expected headings
"Treatment and Prognosis"