Prion diseases have been the subject of much attention in the scientific and lay media for several years, especially recently. No doubt, in large part this has been due to the appearance and recognition of variant Creutzfeldt-Jakob disease (vCJD) in the population. First striking Britain and Europe in the 1990s, this past year has seen the entire beef industry in Canada and the United States brought to its knees by this disease. Feeding practices have been forever changed. Major trading partners have come to restrict or place outright bans on the transborder movement of cattle and beef products to protect their populations from the ingestion of prions. Hospitals, surgeons, and pharmaceutical companies underwent a similar process several years ago concerning the use of cadaveric material (eg, dura, growth hormone) and the reuse and sterilization of instruments used in neurosurgery to avoid iatrogenic CJD (iCJD). Despite the recognition and attention that these newer subtypes of CJD have attracted (vCJD and iCJD), the most common form is still the sporadic type (sCJD), seen in about 85% of the cases (1 case per 1,000,000 population per year), whereas familial cases account for most of the balance. Characteristically it affects people older than 60 years. Rapidly progressive dementia, ataxia, and myoclonus are the usual symptoms. All cases are fatal, usually within 6–8 months.
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