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Variant Creutzfeldt-Jakob disease

Variant Creutzfeldt-Jakob disease (vCJD) is a relatively new and rare neurological disease, classified as a Transmissible Spongiform Encephalopathy (TSE). It was first identified in March 1996 in the UK, when 10 cases of a new disease with neurological symptoms were reported and soon associated with the Bovine Spongiform Encephalopathy (BSE), “mad cow”-disease.

Causative agents of vCJD are prions, composed of misfolded prion proteins (PrPSc), which form aggregates in neurological tissue leading to progressive brain damage and characteristic signs and symptoms of the disease. Prions are stable and relatively resistant to proteases, high temperatures, UV radiation, and commonly used disinfectants.

Patients with vCJD have prominent psychiatric (frequently depression, anxiety and withdrawal) or sensory symptoms and delayed onset of neurologic abnormalities, including ataxia within weeks or months, and dementia and myoclonus late in the illness. The disease always progresses to death. Disease duration is 14 months on average. vCJD tends to affect younger individuals, with an average age of onset of around 28 years, compared to sporadic CJD, which tends to affect middle-aged and elderly individuals.

The definite diagnosis of vCJD requires post-mortem examination of brain tissue.

The incubation period for vCJD after food borne exposure is thought to be around 10 years. No vaccine or treatment is available.

Most reported vCJD cases appear to have been infected through the consumption of bovine meat products contaminated with the agent of BSE. In three cases, reported by the UK, the mode of transmission is thought to be through receipt of blood from an asymptomatic, infected donor.

 

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