Bovine Spongiform Encephalopathy, “Mad Cow's Disease” and Variant Creutzfeldt-Jakob Disease in Humans: A Critical Update

Creutzfeldt-Jakob disease (CJD) is a deadly, fast progressing, and contagious neurodegenerative disorder caused by the build-up of misfolded prion protein in the brain. Since the mid-1990s, when variant CJD (vCJD) first emerged as a disease associated with bovine spongiform encephalopathy (BSE) that is a zoonotic disease generally known as "mad cow disease”, there have been active international CJD surveillance efforts. The vast majority of vCJD sufferers acquire the disease after eating BSE-tainted animal products. vCJD has a median incubation time of 13 years. Latent, asymptomatic infection may persist throughout one's entire life. Unlike in traditional CJD, transmission of vCJD has been associated to ingestion of beef products containing neural tissue already infected with the prion protein and blood transfusion. This article highlights the recent reports of BSE / mad cow disease and vCJD, need of vCJD surveillance from the public health perspective and to promote critical research into the pathophysiology of the disease, improved diagnostic tools, desperately needed treatments and salient counteracting measures.