Chapter 54 - Prion Diseases

Abstract

This chapter presents a discussion on prion diseases. The chapter discusses Creutzfeldt-Jakob disease (CJD), which is one of the transmissible spongiform encephalopathies. . Like all prion diseases, CJD is invariably progressive and fatal. Although rare, it is of intense current scientific and popular interest, largely because of the appearance of a clinicopathological variant, which is considered to be because of the transmission of bovine spongiform encephalopathy (BSE) from cattle to man. There are concerns that this form of CJD may affect considerable numbers of people with the additional possibility of secondary human-to-human spread of disease. CJD exists in the following four clinic pathological forms: sporadic CJD (spCJD); genetic CJD (gCJD); Iatrogenic CJD (iCJD); and variant CJD (vCJD). spCJDis the most common form, with a worldwide distribution and an annual incidence of around one per million of the population per year. It is typically a disease of middle to late life, and its cause is unknown. gCJDis an autosomal dominantly inherited disease reflecting an underlying mutation of the PRNP gene. iCJD is the result of accidental transmission of CJD (usually spCJD) during medical or surgical treatment. vCJD, formerly new variant CJD, was first reported in 1996 and is thought to be because of the transmission of BSE from cattle to man by means of food. The chapter discusses also discusses specific possibilities of therapy, including reducing PrPc production, prevention of the posttranslational alteration and accumulation of PrPc, preventing the neurotoxic effects of PrPSc and amyloid deposits, and others.