NR AGUY
AU Kretzschmar,H.A.; Ironside,J.W.; DeArmond,S.J.; Tateishi,J.
TI Diagnostic criteria for sporadic Creutzfeldt-Jakob disease
QU Archives of Neurology 1996 Sep; 53(9): 913-20
PT journal article
AB 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.
ZR 51
MH Animal; Astrocytes/pathology; Brain/*pathology/ultrastructure; Cerebellar Cortex/pathology; Cerebral Cortex/pathology; Creutzfeldt-Jakob Syndrome/*diagnosis/*pathology/physiopathology; Diagnosis, Differential; Electroencephalography; Germany; Great Britain; Human; Immunohistochemistry; Japan; Laboratory Techniques and Procedures/methods; Microscopy, Electron; Neurons/pathology; Prions/*analysis/genetics; Support, Non-U.S. Gov't; Support, U.S. Gov't, P.H.S.; Synapses/pathology; United States
AD Department of Neuropathology, University of Göttingen, Göttingen, Germany.
SP englisch
PO USA