Pathology of variant Creutzfeldt-Jakob disease.

摘要: Variant Creutzfeldt-Jakob disease (vCJD) is a novel prion in man which was first described 1996 the UK. There substantial evidence to indicate that vCJD represents effects of bovine spongiform encephalopathy (BSE) agent man. The neuropathology characterised by florid plaque, composed central amyloid core with fibrillary periphery, surrounded rim change an intact neuropil. Unique patterns PrP accumulation are revealed immunocytochemistry cerebral and cerebellar cortices, basal ganglia, thalamus brainstem. midbrain also severe neuronal loss gliosis. distinct from other human diseases disease-associated accumulates within follicular dendritic cells lymphoid tissue, consistently peripheral sensory ganglia. All patients so far have been methionine homozygotes at codon 129 gene. no cases BSE infection occurred individuals UK who MV or VV It conceivable incubation periods these groups may be longer than homozygotes, hence precise numbers future difficult estimate present.