Prion diseases are a group of neurodegenerative diseases caused by prions, which are “proteinaceous infectious particles”. Prion diseases are caused by misfolded forms of the prion protein, also known as PrP. These diseases affect mainly mammals and are rare in humans.
The human forms of prion disease are Creutzfeldt-Jakob disease (CJD), Fatal Familial Insomnia (FFI), Gertsmann-Straussler-Scheinker syndrome (GSS), Kuru and Variably Protease-Sensitive Prionopathy (VPSPr). All of these diseases are caused by slightly different versions of the same protein, so we refer to them all as prion diseases.
Prions slowly spread across the brain and neurons become damaged and die, this is when symptoms appear.
Symptoms include; a loss of memory, change of personality, slurred speech and loss of co-ordination and balance.
Visit the National Prion Clinic website for more information: