Gerstmann–Sträussler–Scheinker disease(English)

• a rare human neurodegenerative disease characterized clinically by early cerebellar ataxia and later progressive dementia, and neuropathologically by abundant multicentric amyloid plaques composed of the prion protein (PrP). It belongs to the genetic prion diseases such as genetic or familial Creutzfeldt–Jakob disease (gCJD/fCJD) and fatal familial insomnia (FFI), and is associated with specific missense mutations in the PrP gene
• PRNP, CJD, FFI, PrP
• Ataxia, Dementia, Neuropathy
• https://doi.org…0038-004-0150-7
• https://doi.org…74984-0.00665-3