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.
