Gerstmann-Straussler-Scheinker disease

Gerstmann-Straussler-Scheinker disease is a very rare type of human 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.

History and etymology

It is named after the Austrian-American neuropsychiatrist Josef Gerstmann (1887-1969) , also of Gerstmann syndrome fame, Austrian neuropathologist Ernst Sträussler (1872-1959) , and Austrian-American neuropathologist Ilya Mark Scheinker (1902-1954) .

Siehe auch:

• Creutzfeldt-Jakob-Krankheit
• Transmissible spongiforme Enzephalopathie

und weiter:

• Angularis-Syndrom