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Transmissible spongiform encephalopathy

ORPHA56970
Synonym(s) Prion disease
Prevalence 1-9 / 1 000 000
Inheritance Autosomal dominant
or Not applicable
Age of onset Adult
ICD-10
  • A81.0
  • A81.1
  • A81.8
  • A81.9
OMIM -
UMLS -
MeSH -
MedDRA -

Summary

Human prion diseases or transmissible spongiform encephalopathies (TSE) are rare transmissible diseases affecting the central nervous system consisting of lesions limited to the central nervous system without inflammatory or immunologic reaction but with accumulation of an abnormal form of prion protein (PrPsc). Sporadic Creutzfeldt-Jakob disease (CJD) is the most frequent form of the disease (85% of all forms of TSE). Other forms of TSE is known: genetic TSE (genetic CJD, Gerstmann-Straussler-Scheinker and Fatal Familial Insomnia), and acquired TSE (Kuru, iatrogenic CJD, variant CJD (vCDJ)).


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