Clinical Senior Lecturer in the Department of Medicine at the University of Otago
Consultant Neurologist and Clinical Lead at Dunedin.
Director, National CJD Registry, New Zealand.
Prion diseases are fatal neurodegenerative diseases affecting humans and animals. There is no cure for prion diseases. They are caused by an unconventional infectious agent called prion, which is thought to be composed mainly by a misfolded form of a host protein, the prion protein (PrP). In humans, prions cause Creutzfeldt-Jakob disease; in animals, scrapie has been known to ravage sheep flocks for two hundred years, and chronic wasting disease is of concern in the United States. The recent epidemic of bovine spongiform encephalopathy in the United Kingdom has caused major turmoil throughout Europe, and later, in other countries such as Japan, Canada and the United States, because the bovine prion disease is transmissible to humans causing variant Creutzfeldt-Jakob disease. The transmissibility of the latter by blood transfusion created a novel public health issue.