Creutzfeldt-Jakob disease

Creutzfeldt-Jakob Disease (CJD) is a very rare and incurable brain disease that is ultimately fatal. It is the most common of the transmissible spongiform encephalopathies (TSEs). TSEs (also known as prion diseases) are caused by a unique type of infectious agent called prions, an abnormally-structured form of a protein found in the brain. Other prion diseases include Gerstmann-Sträussler-Scheinker syndrome (GSS), fatal familial insomnia (FFI) and kuru in humans, as well as BSE and scrapie in animals.

References

• Peden AH, et al (2004). Preclinical vCJD after blood transfusion in a PRNP codon 129 heterozygous patient. Lancet 364, 527-29
• Johnson RT, Gibbs CJ (1998). Creutzfeldt-Jakob disease and related transmissible spongiform encephalopathies. N. Engl. J. Med. 339 (27), 1994-2004