CJD or ‘Mad Cow Disease’?

Both Creutzfeldt-Jakob Disease (CJD) and its close relative Variant Creutzfeldt-Jakob Disease (vCJD) are caused by Prions, incorrectly folded proteins, which destroy brain tissue. Neither are infectious but types of CJD can be spread by exposure to infected blood products, meat or medical instruments.

BSE (Bovine Spongiform Encephalopathy) or ‘Mad Cow Disease’ is a form of Transmissible Spongiform Encephalopathy (TSEs). The ‘spongiform’ in the name refers to the fact that the infected brain is filled with holes, resembling a sponge.

CJD is a degenerative brain disorder, estimated to affect one in a million people annually worldwide. Appearing in later life usually, the onset is rapid and fatal. Early symptoms include problems with vision, coordination problems, changes in behaviour, and memory problems, which lead to blindness, involuntary movements and coma.

vCJD was acquired by humans by exposure to meat infected with BSE. It is estimated that only 1% of CJD is ‘variant’. It was discovered in 1996 in the UK and, according to the government, 260 cases have been reported since then.

 

The Different Types of CJD

When it comes Creutzfeldt-Jakob Disease, there are 4 types:

  1. Sporadic CJD
  • Sufferers have no known risk factors.
  • Most common type of CJD
  • 85% of all CJD cases

 

  1. Hereditary CJD
  • Family history of the disease
  • 10 to 15% of CJD cases

 

  1. Acquired or Iatrogenic CJD
  • Transmitted by exposure to bodily tissue during medical procedures
  • Improperly sterilised medical instruments are also thought to spread this
  • Before 1985, was spread by a treatment using glands from deceased humans

 

  1. Variant CJD (vCJD)
  • Caused by consuming meat from a cow infected with BSE AKA ‘Mad Cow Disease’
  • 1% of all CJD cases
  • Possible 10 year+ incubation period

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