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The Lingering Shadow of Mad Cow Disease

Optimists are proclaiming that variant Creutzfeldt-Jakob disease (CJD), the human form – always fatal – of bovine spongiform encephalopathy (BSE), or “Mad Cow Disease,” is on the wane. Obviously, given the degree of suffering and public anxiety that variant CJD has caused, the possibility that it is receding is welcome news. But is it true?

CJD belongs to the family of what are called prion diseases, a unique group of neurodegenerative diseases that can be transmitted. Although the precise nature of the disease’s transmission remains uncertain, a key event in these disorders is the conversion of the prion protein’s normal cells to an abnormal form that appears to be the major (if not the sole) component of the infection.

Variant CJD was first described in 1996, following intensive surveillance activities undertaken by the UK National CJD Surveillance Unit (NCJDSU) in Edinburgh. This new form of prion disease had distinctive clinical and pathological features, and occurred in young patients within a single genetic subgroup.

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