Human prion diseases affect the brain. They are rare and invariably fatal. The first symptoms are usually personality changes, depression and unsteadiness; this eventually leads to severe mental impairment and loss of movement and speech.
Human prion diseases include Creutzfeldt-Jakob disease (CJD), the human form of mad cow disease, Gerstmann-Straussler-Scheinker disease, which is a very rare inherited disease that is only found in a few families worldwide, and Kuru, which is found in cannibalistic tribes in New Guinea.
These diseases only affect about 1 person per million worldwide each year. However, an increased rate of variant CJD occurred in Britain following the UK mad cow disease epidemic in the 1990s.
In the PRION-1 trial, the MRC Clinical Trials Unit collaborated with the MRC Prion Unit to evaluate the role of Quinacrine in the treatment of human prion diseases.
