The disease seems rare but “infection” may be relatively commonVariant Creutzfeldt Jakob Disease (CJD) is the human form of bovine spongiform encephalopathy or “mad cow disease.” It is one of the family of mainly neurodegenerative diseases known as spongiform encephalopathies because of their histological appearance. These diseases afflict animals and humans and are widely accepted as resulting from the toxic build up of an aberrant form of a normal cellular protein, the prion protein. Bovine spongiform encephalopathy was common, with more than 36000 cases in the peak year of the cattle epidemic in the United Kingdom (1992).1 However, variant CJD has remained mercifully rare, with 177 cases in the UK to date (51 in the rest of the world, 27 of which were in France), and only one in the past two years.2
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