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Prion Disease

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Encyclopedia of Clinical Neuropsychology

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Definition

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
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References and Readings

  • 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.

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  • Hornlimann, B., Riesner, D., & Kretzschmar, H. (Eds.). (2007). Prions in humans and animals. Berlin: De Gruyter.

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  • 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.

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  • Prusiner, S. B. (2003). Prion biology and diseases. New York: Cold Spring Harbour Laboratory Press.

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Author information

Authors and Affiliations

  1. University of Edinburgh, Edinburgh, UK

    Robert G. Will

  2. Research Department of Clinical, Educational and Health Psychology, University College London, London, UK

    Narinder Kapur

Authors
  1. Robert G. Will
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  2. Narinder Kapur
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Corresponding author

Correspondence to Robert G. Will .

Editor information

Editors and Affiliations

  1. Department of Physical Medicine and Rehabilitation, Virginia Commonwealth University, Richmond, VA, USA

    Jeffrey S. Kreutzer

  2. Kessler Foundation, Pleasant Valley Way, West Orange, NJ, USA

    John DeLuca

  3. Independent Practice, Wynnewood, PA, USA

    Bruce Caplan

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Will, R.G., Kapur, N. (2018). Prion Disease. In: Kreutzer, J.S., DeLuca, J., Caplan, B. (eds) Encyclopedia of Clinical Neuropsychology. Springer, Cham. https://doi.org/10.1007/978-3-319-57111-9_570

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