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Sporadic Creutzfeldt-Jakob disease

Disease definition

Sporadic Creutzfeldt-Jakob disease (sCJD) is a subacute fatal neurodegenerative disease belonging to the group of prion diseases, characterized by a clinical triad of dementia, myoclonus, and EEG anomalies, along with neuropathological evidence of neuronal loss, spongiform changes, and astrocytosis. There are three types of CJD: sporadicCJD (sCJD), inherited CJD (see this term), and iatrogenic and variant CJD (vCJD).

ORPHA:204

  • Synonym(s):
    • Sporadic CJD
  • Prevalence: 1-9 / 1 000 000
  • Inheritance: Not applicable 
  • Age of onset: Adult, Elderly
  • ICD-10: A81.0
  • OMIM: 123400
  • UMLS: C0022336
  • MeSH: D007562
  • GARD: 6956
  • MedDRA: 10011384

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