Clinical Review

1755 and all that: a historical primer of transmissible spongiform encephalopathy

BMJ 1998; 317 doi: http://dx.doi.org/10.1136/bmj.317.7174.1688 (Published 19 December 1998) Cite this as: BMJ 1998;317:1688
  1. Paul Brown (pwb@codon.nih.gov), senior research scientista,
  2. ymond Bradley, private BSE consultant.b
  1. aLaboratory of CNS Studies, National Institute of Neurological Disorders and Stroke, Building 36, Room 5B20, National Institutes of Health, Bethesda, MD 20892, USA
  2. bCentral Veterinary Laboratory, New Haw, Addlestone, Surrey KT15 3NB
  1. Correspondence and reprint requests to: Dr Brown

    In the political and economic turmoil of 18th century Europe, England gradually established itself as the dominant trading power. By far its most important commercial product was woollens, involving in one way or another nearly a quarter of the British population, which at 10 million people approximated the number of British sheep. Sir Robert Walpole, the embodiment of pragmatic mercantile economics, was prime minister of an increasingly powerful and commercially inclined Whig government, and, with the agricultural revolution in full swing, the invention of the flying shuttle and spinning jenny and the imminent introduction of steam power would soon convert weaving from a handicraft to a mechanical process. Wool, already in short supply, was at a premium. In this setting, it is not surprising that in 1755 a discussion took place in the British parliament about the economic effects of a fatal and spreading disease in sheep, and the need for government to do something about it.1 Thus began the recorded history of scrapie.

    Summary points

    • Scrapie was first described in the 18th century, but it was not proved to be a transmissible disease until 1936

    • Its position as the prototype of a small group of animal and human spongiform encephalopathies, including Creutzfeldt-Jakob disease, wasnot appreciated until a quarter of a century later, in the early 1960s

    • The infectious agent, originally thought to be a “slow virus,” has now come to be considered a conformationally altered, self replicating form of a normal body protein, or prion

    • Numerous mutations in the gene that encodes this protein have been linked to familial forms of disease

    • Recent outbreaks of disease (bovine spongiform encephalopathy and iatrogenic and “new variant” Creutzfeldt-Jakob disease) have highlighted a disconcerting disparity between advances in fundamental science and practical common sense

    Anecdotes and antidotes

    Where or when the disease actually first appeared is unclear, although there …

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