Bovine spongiform encephalopahy and Creutzfeldt-Jakob disease

The identification of 10 cases of a highly stereotyped clinicopathological variant of Creutzfeldt-Jakob disease (CJD) in adolescents and young adults occurring in Britain within the past 24 months (R G Will, J Ironside, et al, personal communication) is cause for serious rethinking about the possibility of human infection from bovine spongiform encephalopathy (BSE). Typically, “sporadic” (idiopathic) cases of Creutzfeldt-Jakob disease begin in the sixth or seventh decade of life with a loss of memory or, less commonly, with behavioural changes or higher cortical function deficits such as dysphasia or dyslexia. Over several weeks the mental deterioration progresses to frank dementia in association with abnormalities of vision or coordination, rigidity, and involuntary movements (especially myoclonic jerks), which often occur in synchrony with periodic spike waves on electroencephalography.2 Death usually occurs within six months, and at necropsy the brain shows a pathognomonic spongiosis with neuronal loss and gliosis in …