Neuropathological Diagnostic Criteria for Creutzfeldt-Jakob Disease (CJD) and Other Human Spongiform Encephalopathies (Prion Diseases) |
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| Authors: | Herbert Budka Adriano Aguzzi Paul Brown Jean-Marie Brucher Orso Bugiani Filippo Gullotta Matti Haltia Jean-Jacques Hauw James W. Ironside Kurt Jellinger Hans A. Kretzschmar Peter L. Lantos Carlo Masullo Wolfgang Schlote Jun Tateishi Roy O. Weller |
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| Affiliation: | Institute of Neurology, University of Vienna, Vienna, Austria;Institute of Neuropathology, University of Zurich, Zurich, Switzerland;Laboratory of Central Nervous System Studies, NINDS, NIH, Bethesda, MD, USA;Neuropathology Unit, Catholic University Louvain, Brussels, Belgium;National Institute of Neurology C. Besta, Milan, Italy;Institute of Neuropathology, University of Müster, Münster, FRG;Institute of Pathology, University of Helsinki, Helsinki, Finland;Neuropathological Laboratory R. Escourolle, H![]("/giflibrary/bpg4/006f030c.gif"/) pital de la Salpětriere, Paris, France;Neuropathology Laboratory, Department of Pathology, Western General Hospital, Edinburgh, UK;Neuropathological Laboratory, Department of Neurology, Lainz Hospital, Vienna, Austria;Institute of Neuropathology, University of Göttingen, Göttingen, FRG;Department of Neuropathology, Institute of Psychiatry, University of London, London, UK;Institute of Neurology, Catholic University of the Sacred Heart, Rome, Italy;Neurological Institute, University of Frankfurt, Frankfurt, FRG;Department of Neuropathology, Institute of Neurology, Kyushu University, Fukuoka, Japan;Neuropathology, Southampton University Hospital, Southampton, UK |
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| Abstract: | Neuropathological diagnostic criteria for Creutzfeldt-Jakob disease (CJD) and other human transmissible spongiform encephalopathies (prion diseases) are proposed for the following disease entities: CJD - sporadic, iatrogenic (recognised risk) or familial (same disease in 1st degree relative): spongiform encephalopathy in cerebral and/or cerebellar cortex and/or subcortical grey matter; or encephalopathy with prion protein (PrP) immuno-reactivity (plaque and/or diffuse synaptic and/or patchy/perivacuolar types). Gerstmann-Sträussler-Scheinker disease (GSS) (in family with dominantly inherited progressive ataxia and/or dementia): encephalo(myelo)pathy with multicentric PrP plaques. Familial fatal insomnia (FFI) (in member of a family with PRNP178 mutation): thalamic degeneration, variable spongiform change in cerebrum. Kuru (in the Fore population). Without PrP data, the crucial feature is the spongiform change accompanied by neuronal loss and gliosis. This spongiform change is characterised by diffuse or focally clustered small round or oval vacuoles in the neuropil of the deep cortical layers, cerebellar cortex or subcortical grey matter, which might become confluent. Spongiform change should not be confused with non-specific spon-giosis. This includes status spongiosus (“spongiform state”), comprising irregular cavities in gliotic neuropil following extensive neuronal loss (including also lesions of “burnt-out” CJD), “spongy” changes in brain oedema and metabolic encephalopathies, and artefacts such as superficial cortical, perineuronal, or perivascular vacuolation; focal changes indistinguishable from spongiform change may occur in some cases of Alzheimer's and diffuse Lewy body diseases. Very rare cases might not be diagnosed by these criteria. Then confirmation must be sought by additional techniques such as PrP immunoblotting, preparations for electron microscopic examination of scrapie associated fibrils (SAF), molecular biologic studies, or experimental transmission. |
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