Open AccessNeuropathological Diagnostic Criteria for Creutzfeldt‐Jakob Disease (CJD) and Other Human Spongiform Encephalopathies (Prion Diseases)
Author(s) -
Budka Herbert,
Aguzzi Adriano,
Brown Paul,
Brucher JeanMarie,
Bugiani Orso,
Gullotta Filippo,
Haltia Matti,
Hauw JeanJacques,
Ironside James W.,
Jellinger Kurt,
Kretzschmar Hans A.,
Lantos Peter L.,
Masullo Carlo,
Schlote Wolfgang,
Tateishi Jun,
Weller Roy O.
Publication year - 1995
Publication title -
brain pathology
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 1.986
H-Index - 132
eISSN - 1750-3639
pISSN - 1015-6305
DOI - 10.1111/j.1750-3639.1995.tb00625.x
Subject(s) - pathology , fatal familial insomnia , neuropil , grey matter , kuru , gliosis , creutzfeldt jakob syndrome , bovine spongiform encephalopathy , thalamus , medicine , cerebellar cortex , scrapie , slow virus , neuropathology , white matter , neuroscience , disease , biology , cerebellum , virology , central nervous system , magnetic resonance imaging , progressive multifocal leukoencephalopathy , prion protein , virus , radiology
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.