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Creutzfeldt-Jakob Disease

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  • First Online:
Encyclopedia of Clinical Neuropsychology

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Synonyms

CJD; Prion disease; Transmissible spongiform encephalopathy (TSE

Short Description or Definition

Creutzfeldt-Jakob Disease (CJD) is a rare, fatal neurodegenerative disease, which is one of the transmissible spongiform encephalopathies or prion diseases. These conditions are characterized pathologically by neuronal loss, spongiform change, and astrocytic gliosis. Cell loss can be seen microscopically as multiple perforations to the brain tissue creating the characteristic “spongelike” appearance. Prion diseases are caused by infectious agents, which are abnormal self-replicating forms of a normal brain protein, prion protein.

Categorization and Epidemiology

Creutzfeldt-Jakob disease (CJD) may be sporadic (that is develop spontaneously without apparent cause), familial (inherited), or acquired (transmitted by infection).

CJD occurs worldwide with a mean annual incidence of approximately one to two cases per million population (Ladogana et al. 2005). Except for variant and...

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References and Readings

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Author information

Authors and Affiliations

  1. Neuropsychology Department, Addenbrooke’s Hospital, Cambridge, UK

    Kari Hawkins

  2. University of Edinburgh, Edinburgh, UK

    Robert G. Will

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

    Narinder Kapur

Authors
  1. Kari Hawkins
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  2. Robert G. Will
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  3. Narinder Kapur
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Corresponding author

Correspondence to Narinder Kapur .

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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Hawkins, K., Will, R.G., Kapur, N. (2018). Creutzfeldt-Jakob 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_547

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