Creutzfeldt-Jakob disease

About 50-60 cases occur each year in the UK. The cause is unknown, but it is not BSE in cattle (so-called 'mad cow disease').

Variant CJD

Originally called 'new variant CJD', this is related to BSE. It is also known as 'human BSE'. It was first identified in 1996 and the first case first developed symptoms in 1994. The numbers of people dying from vCJD in the UK are given in the table below:

Iatrogenic CJD

'Iatrogenic' basically means the disease is acquired through a medical accident. CJD has been inadvertently transmitted by several medical and surgical treatments. But these cases are rare with only about 250 reported worldwide.

What causes CJD?

In normal brain cells, a normal gene (the PRNP gene, on chromosome 20) produces a normal prion protein, PrPC. But in CJD, this somehow changes into an abnormal protein, PrPSc. Either the loss of the normal protein or the accumulation of the abnormal protein, or both, leads to disease in the brain resulting in progressive and, eventually, fatal neurological disease.

Sporadic CJD The trigger for the protein change is unknown. But there are two popular theories. Firstly, PrPC might, rarely, spontaneously change to PrPSc. Secondly, a copy of the PRNP gene in a brain cell might spontaneously mutate and produce abnormal PrPC, which then turns into PrPSc. While no environmental source has yet been identified, spCJD might result from infection.

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