new-variant Creutzfeldt-Jakob disease


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new-variant Creutzfeldt-Jakob disease; nvCJD spongiform encephalopathy affecting predominantly young people; characterized by a shorter period of incubation than CJD; associated with ingestion of food containing bovine spongiform encephalopathy (BSE) prion-contaminated meat/animal products (i.e. beef cattle reared on feed containing protein from animals with BSE); presents as neuropsychiatric illness, progressing to dementia, and ataxia with monoclonus or chorea; death occurs within 6 months of onset; BSE prion is highly resistant to heat destruction, and survives autoclaving