Creutzfeldt-Jakob disease (CJD) is a rare, rapidly progressive, and fatal neurodegenerative disorder. It is caused by prions, which are abnormally folded proteins that induce normal proteins in the brain to misfold. CJD primarily affects older adults (sporadic form) worldwide, leading to rapid mental and physical deterioration.
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Creutzfeldt-Jakob disease was first described in the early 1920s by German neurologists Hans Gerhard Creutzfeldt and Alfons Maria Jakob. Its significance grew with the understanding of transmissible spongiform encephalopathies (TSEs), particularly the link between **variant CJD** and bovine spongiform encephalopathy (BSE) in the 1990s, highlighting the unique nature of prion diseases.
CJD is not contagious through casual contact. Most cases are **sporadic**, meaning they occur spontaneously without a clear cause. Transmission can also occur through medical procedures (**iatrogenic CJD**) involving contaminated brain tissue or instruments, or through inheritance (**familial CJD**); **variant CJD (vCJD)** is acquired by consuming contaminated beef products.
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Variant Creutzfeldt-Jakob disease (vCJD) is a fatal prion disorder linked to dietary exposure to bovine spongiform encephalopathy (BSE). The epidemic peaked in the early 2000s, and no new cases have been reported in the UK since 2016. However, uncertainties remain regarding potential future cases, particularly in individuals with non-MM prion protein gene codon 129 genotypes, and possible secondary transmission via blood transfusion. We aimed to update risk estimates with recent data, informed by a probabilistic modelling approach. We developed a cohort-based probabilistic model for variant CJD incidence incorporating genotype-specific attack rates and incubation periods, accounting for competing mortality risks. Model parameters were calibrated using historical case data and life-table an...
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View WHO Resources →Disease information on Virus Watcher is reviewed by our Chief Epidemiologist, a former CDC lead analyst for FluSight forecasting. Outbreak data is aggregated from verified sources including BEACON, ProMED, WHO, CDC, and 50+ national health agencies. This information is for educational purposes and should not replace professional medical advice.
Last reviewed: 2026-07-18
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