| Prevalence | ~1-2 per million per year |
| Mean age of onset | 60-65 years |
| Median survival | 4-6 months (90% die within 1 year) |
| Cause | Unknown; possibly spontaneous misfolding of PrP^C or somatic *PRNP* mutations |
| E200K mutation | Most common worldwide; similar clinical presentation to sCJD but with younger onset |
| D178N with valine at codon 129 | Produces familial CJD phenotype |
| D178N with methionine at codon 129 | Produces fatal familial insomnia (FFI) phenotype |
| V210I, R208H, E196K | Other pathogenic mutations with varying penetrance |
| Pituitary-derived growth hormone | Over 200 cases worldwide before recombinant hormone became available |
| Corneal transplants | Rare cases documented |
| Contaminated neurosurgical instruments | Historical cases; standard autoclaving is insufficient for prion inactivation |
| Blood transfusion | Four confirmed cases of vCJD transmission in the UK |
| Databases | OMIMOrphanetClinicalTrialsPubMed |