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September 1996

Diagnostic Criteria for Sporadic Creutzfeldt-Jakob Disease

Author Affiliations

From the Department of Neuropathology, University of Göttingen, Göttingen, Germany (Dr Kretzschmar); the Department of Pathology, Neuropathology Laboratory, University of Edinburgh, Edinburgh, Scotland (Dr Ironside); the Department of Pathology, Neuropathology Unit, University of California, San Francisco (Dr DeArmond); and the Department of Neuropathology, Neurological Institute, Kyushu University, Fukuoka, Japan (Dr Tateishi).

Arch Neurol. 1996;53(9):913-920. 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.