Encyclopedia of Clinical Neuropsychology

2018 Edition
| Editors: Jeffrey S. Kreutzer, John DeLuca, Bruce Caplan

Prion Disease

  • Robert G. WillEmail author
  • Narinder Kapur
Reference work entry
DOI: https://doi.org/10.1007/978-3-319-57111-9_570


These diseases are caused by unconventional, infectious agents, which are composed of a posttranslationally modified form of a normal host-encoded protein, prion protein. This protein is expressed in many tissues, but mainly in the central nervous system, and these diseases are characterized by progressive and fatal neurodegenerative disorders. Prion diseases affect both animal species and humans (Table 1).
Prion Disease, Table 1

Prion diseases



Sporadic Creutzfeldt-Jakob disease (CJD)

Scrapie (sheep and goats)

Genetic CJD, fatal familial insomnia (FFI)

Transmissible mink encephalopathy

Gerstmann-Sträussler syndrome (GSS)

Chronic wasting disease (mule deer and elk)

Iatrogenic CJD


Variant CJD

Bovine spongiform encephalopathy (BSE)

Feline spongiform encephalopathy

Spongiform encephalopathy of captive ungulates


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References and Readings

  1. Cohen, O., Prohovnik, I., Korczyn, A., et al. (2011). The Creutzfeldt-Jakob (CJD) neurological status scale: A new tool for evaluation of disease severity and progression. Acta Neurological Scandinavica, 124, 368–374.CrossRefGoogle Scholar
  2. Hornlimann, B., Riesner, D., & Kretzschmar, H. (Eds.). (2007). Prions in humans and animals. Berlin: De Gruyter.Google Scholar
  3. Maddox, R., Blasé, J., Mercaldo, N., et al. (2015). Clinically unsuspected prion disease among patients with dementia diagnoses in an Alzheimer’s Disease Database. American Journal of Alzheimer’s Disease and Other Dementias, 30, 752–755.PubMedCentralCrossRefPubMedGoogle Scholar
  4. Prusiner, S. B. (2003). Prion biology and diseases. New York: Cold Spring Harbour Laboratory Press.Google Scholar

Copyright information

© Springer International Publishing AG, part of Springer Nature 2018

Authors and Affiliations

  1. 1.University of EdinburghEdinburghUK
  2. 2.Research Department of Clinical, Educational and Health PsychologyUniversity College LondonLondonUK