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Variant Creutzfeldt-Jakob disease

Henry G Brown, MD, PhD
John M Lee, MD, PhD
Section Editor
Steven T DeKosky, MD, FAAN, FACP, FANA
Deputy Editor
Janet L Wilterdink, MD


Prion diseases are neurodegenerative diseases that have long incubation periods and progress inexorably once clinical symptoms appear. Five human prion diseases are currently recognized: kuru, Creutzfeldt-Jakob disease (CJD), variant Creutzfeldt-Jakob disease (vCJD), Gerstmann-Sträussler-Scheinker syndrome (GSS), and fatal familial insomnia (FFI).

These human prion diseases share certain common neuropathologic features including neuronal loss, proliferation of glial cells, absence of an inflammatory response, and the presence of small vacuoles within the neuropil, which produces a spongiform appearance.

Variant CJD (vCJD) may be distinguished from cases of typical sporadic Creutzfeldt-Jakob disease (sCJD) by the following features:

  • A considerably younger age at the onset of symptoms
  • Less rapid progression of illness
  • Differences in clinical presentation and course
  • Differences in neuropathology

The clinical manifestations and diagnosis of variant CJD will be reviewed here. Classic CJD (sporadic, iatrogenic and familial forms of CJD) is discussed separately. The prion diseases Kuru, GSS, and FFI are also discussed separately, as are the biology and genetics of prion diseases. (See "Creutzfeldt-Jakob disease" and "Diseases of the central nervous system caused by prions" and "Biology and genetics of prions".)


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Literature review current through: Sep 2016. | This topic last updated: Aug 1, 2013.
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