Type: Prion disease

Geography: United Kingdom

Cases Per Year: 10 – 20

Fatality Rate: 100%

First Discovered: 1996 in the United Kingdom

VCJD is a variant of Creutzfeldt-Jakob Disease. It is a prion disease, which triggers normal proteins in the brain to fold abnormally. VCJD is a fatal brain disease with an unusually long incubation period. This disease mainly affects young people, with an average age of death of 28 years. The illness usually lasts for 13 to 14 months. Clinical features include prominent psychiatric or sensory symptoms and delayed onset of neurologic abnormalities, including ataxia within weeks or months. Dementia and myoclonus present late in the illness, as does a diffusely abnormal non-diagnostic electroencephalogram. Neuropathologic examination of brain tissue is required to confirm a diagnosis of variant CJD.

There is now strong evidence that the agent responsible for the outbreak of prion disease in cows, bovine spongiform encephalopathy or “mad cow” disease, is the same agent responsible for the outbreak of vCJD in humans. It is believed that people who have developed vCJD became infected through their consumption of cattle products contaminated with the agent of BSE, or in three cases, through receipt of blood from an asymptomatic infected donor. There is no treatment of vCJD and it is invariably fatal.

Photo: VCJD Tonsil biopsy prion immunostain – Micrograph.