Prion diseases are disorders affecting the central nervous system caused by alterations in the conformation of the cellular prion protein. They can be sporadic, hereditary, or acquired and usually present with myoclonus and rapidly progressive dementia in human patients. This article discusses the epidemiology, pathogenesis, diagnosis, and laboratory testing of prion diseases with a primary focus on Creutzfeldt-Jakob disease.
2010 Elsevier Inc. All rights reserved.