Diagnostic criteria for sporadic Creutzfeldt-Jakob disease

Arch Neurol. 1996 Sep;53(9):913-20. doi: 10.1001/archneur.1996.00550090125018.


Background: Making a clinical diagnosis of sporadic Creutzfeldt-Jakob disease relies on the evaluation of rapidly progressive dementia, ataxia, myoclonus, changes on the electroencephalogram, and other neurological signs. A definite diagnosis, however, is confined to cases that have been evaluated neuropathologically or by equivalent diagnostic techniques. This places a high priority on the establishment of reliable neuropathologic methods for the investigation and diagnosis of Creutzfeldt-Jakob disease.

Objective: To evaluate existing morphological and laboratory diagnostic techniques to reach a consensus on the definition of "definite Creutzfeldt-Jakob disease."

Methods: The existing morphological techniques, particularly immunohistochemistry, used in 4 laboratories--Germany, Great Britain, Japan, and the United States--are evaluated, and various laboratory diagnostic techniques are discussed.

Results: Immunohistochemistry with antibodies against the prion protein combined with special tissue pretreatment regimens gives reliable diagnostic results and, for its applicability to formalin-fixed and paraffin-embedded tissue, is superior to other techniques that may be more sensitive but require fresh, unfixed brain tissue.

Conclusions: Our experience suggests the following regimen for the diagnosis of suspected Creutzfeldt-Jakob disease: light microscopy of various brain regions, which in typical cases may lead to definite diagnosis. Immunohistochemistry with antibodies against the prion protein is preferable in all suspected cases of Creutzfeldt-Jakob disease and is mandatory whenever a routine histological workup does not yield definite results. Additional special techniques can be applied if required.

Publication types

  • Research Support, Non-U.S. Gov't
  • Research Support, U.S. Gov't, P.H.S.

MeSH terms

  • Animals
  • Astrocytes / pathology
  • Brain / pathology*
  • Brain / ultrastructure
  • Cerebellar Cortex / pathology
  • Cerebral Cortex / pathology
  • Clinical Laboratory Techniques / methods
  • Creutzfeldt-Jakob Syndrome / diagnosis*
  • Creutzfeldt-Jakob Syndrome / pathology*
  • Creutzfeldt-Jakob Syndrome / physiopathology
  • Diagnosis, Differential
  • Electroencephalography
  • Germany
  • Humans
  • Immunohistochemistry
  • Japan
  • Microscopy, Electron
  • Neurons / pathology
  • Prions / analysis*
  • Prions / genetics
  • Synapses / pathology
  • United Kingdom
  • United States


  • Prions