Neuropathological Diagnostic Criteria for Creutzfeldt‐Jakob Disease (CJD) and Other Human Spongiform Encephalopathies (Prion Diseases)

• DOI: 10.1111/j.1750-3639.1995.tb00625.x
• Publication Date: 2008-01-29T06:26:49Z
• 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
• Keywords: Fatal familial insomnia; Neuropil; Grey matter; Kuru; Gliosis; Creutzfeldt-Jakob Syndrome; Bovine spongiform encephalopathy; Slow virus; Neuropathology

Neuropathological diagnostic criteria for Creutzfeldt‐Jakob disease (CJD) and other human transmissible spongiform encephalopathies (prion diseases) are proposed the following entities: CJD ‐ sporadic, iatrogenic (recognised risk) or familial (same in 1st degree relative): encephalopathy cerebral and/or cerebellar cortex subcortical grey matter; with prion protein (PrP) immuno‐reactivity (plaque diffuse synaptic patchy/perivacuolar types). Gerstmann‐Sträussler‐Scheinker (GSS) (in family dominantly inherited progressive ataxia dementia): encephalo(myelo)pathy multicentric PrP plaques. Familial fatal insomnia (FFI) member of a PRNP178 mutation): thalamic degeneration, variable change cerebrum. Kuru Fore population). Without data, crucial feature is accompanied by neuronal loss gliosis. This characterised focally clustered small round oval vacuoles neuropil deep cortical layers, matter, which might become confluent. Spongiform should not be confused non‐specific spon‐giosis. includes status spongiosus (“spongiform state”), comprising irregular cavities gliotic extensive (including also lesions “burnt‐out” CJD), “spongy” changes brain oedema metabolic encephalopathies, artefacts such as superficial cortical, perineuronal, perivascular vacuolation; focal indistinguishable from may occur some cases Alzheimer's Lewy body diseases. Very rare diagnosed these criteria. Then confirmation must sought additional techniques immunoblotting, preparations electron microscopic examination scrapie associated fibrils (SAF), molecular biologic studies, experimental transmission.