Gerstmann-Straussler-Scheinker disease

Last revised by Assoc Prof Frank Gaillard on 23 Sep 2021

Gerstmann-Straussler-Scheinker disease is a very 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. 

History and etymology

It is named after the Austrian-American neuropsychiatrist Josef Gerstmann (1887-1969) 6, also of Gerstmann syndrome fame, Austrian neuropathologist Ernst Sträussler (1872-1959) 4, and Austrian-American neuropathologist Ilya Mark Scheinker (1902-1954) 5.

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