Encyclopedia of Clinical Neuropsychology

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

Creutzfeldt-Jakob Disease

  • Kari Hawkins
  • Robert G. Will
  • Narinder KapurEmail author
Reference work entry
DOI: https://doi.org/10.1007/978-3-319-57111-9_547


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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Copyright information

© Springer International Publishing AG, part of Springer Nature 2018

Authors and Affiliations

  1. 1.Neuropsychology DepartmentAddenbrooke’s HospitalCambridgeUK
  2. 2.University of EdinburghEdinburghUK
  3. 3.Research Department of Clinical, Educational and Health PsychologyUniversity College LondonLondonUK