Gerstmann-Straussler-Scheinker disease

Dr Rohit Sharma and Dr Henry Knipe et al.

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) 6, also of Gerstmann syndrome fame, Austrian neuropathologist Ernst Sträussler (1872-1959) 4, and Austrian-American neuropathologist Ilya Mark Scheinker (1902-1954) 5.

Neurodegenerative diseases

Neurodegenerative diseases are legion and their classification just as protean. A useful approach is to divide them according to underlying pathological process, although even using this schema, there is much overlap and thus resulting confusion.

Share article

Article information

rID: 42392
Section: Syndromes
Tag: refs, cases
Synonyms or Alternate Spellings:
  • Gerstmann-Straussler-Scheinker syndrome

Support Radiopaedia and see fewer ads

Updating… Please wait.

 Unable to process the form. Check for errors and try again.

 Thank you for updating your details.