Mad Cow Disease (BSE) and Creutzfeldt‑Jakob Disease: The Prion Link
Bovine spongiform encephalopathy (BSE) in cattle and Creutzfeldt‑Jakob disease (CJD) in humans are transmissible spongiform encephalopathies caused by misfolded prion protein (PrPSc).
Transmission
- Variant CJD (vCJD): Acquired by consuming BSE‑contaminated beef products (especially CNS tissue). Peak incidence in UK 1990s; now rare due to feed bans and surveillance.
- Sporadic CJD (sCJD): 85% of cases; spontaneous PrP misfolding, no known exposure.
- Iatrogenic CJD: Contaminated neurosurgical instruments, dura mater grafts, pituitary hormones (historical).
- Genetic CJD: PRNP mutations (autosomal dominant).
Clinical Course
Rapidly progressive dementia, myoclonus, ataxia, visual disturbances, akinetic mutism. Median survival 4–6 months. No effective treatment; care is palliative.
Prevention
- Specified risk material (SRM) removal from food chain.
- Prion‑dedicated surgical instrument protocols.
- Deferral of blood donors with UK residence during BSE era (in some countries).
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