Orphanet: Gerstmann Straussler Scheinker syndrome

Search for a rare disease

* (*) mandatory field

Other search option(s)

Suggest an update

(*) Required fields.


Only comments seeking to improve the quality and accuracy of information on the Orphanet website are accepted. For all other comments, please send your remarks via contact us. Only comments written in English can be processed.

Captcha image

Gerstmann-Straussler-Scheinker syndrome

Disease definition

Gerstmann-Straussler-Scheinker syndrome (GSSS) is a particular and rare form of human transmissible spongiform encephalopathy (TSE) due to a defective gene encoding the prion protein (PRNP gene) and marked by particular multicentric amyloid plaques in the brain.


Classification level: Disorder
  • Synonym(s):
    • Subacute spongiform encephalopathy, Gerstmann-Straussler type
  • Prevalence: Unknown
  • Inheritance: Autosomal dominant or Not applicable 
  • Age of onset: Adult
  • ICD-10: A81.8
  • OMIM: 137440
  • UMLS: C0017495
  • MeSH: D016098
  • GARD: 7690
  • MedDRA: 10072075

Detailed information


The documents contained in this web site are presented for information purposes only. The material is in no way intended to replace professional medical care by a qualified specialist and should not be used as a basis for diagnosis or treatment.