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Deaths from nvCJD rise sharply

BMJ 1999; 318 doi: https://doi.org/10.1136/bmj.318.7187.829 (Published 27 March 1999) Cite this as: BMJ 1999;318:829
  1. Judy Jones
  1. MalmesburyWiltshire

    Deaths from new variant Creutzfeldt-Jakob disease (nvCJD) rose to their highest ever reported quarterly total at the end of 1998. Nine deaths from the new variant form of the disease were reported to the National CJD Surveillance Unit in Edinburgh over that period, compared with two recorded for each of the previous three quarters.

    Scientists at the unit say that the “unusual” rise in the number of reported deaths should be interpreted with caution as it may reflect improvements in detecting and confirming cases. They point out that only one death from nvCJD has been notified this year up to 2 March.

    The highest previous total number of deaths reported over three months—five—was notified to the unit in the first quarter of 1996.

    An analysis of the 39 deaths from nvCJD reported so far in the United Kingdom was published in a letter to the Lancet (1999;353:979) from six of the scientific experts monitoring the progress of the disease.

    “The number of variant CJD deaths during the coming years will provide a clearer indication of whether the apparent increase in deaths towards the end of 1998 was a chance observation or marks a change in the underlying mortality rate,” the letter says.

    Bovine spongiform encepha-lopathy was first formally identified in cattle in the United Kingdom in 1986. It resembled scrapie, a spongiform encepha-lopathy affecting sheep. Although cattle are ruminants and have evolved only to digest grass and other plant material efficiently, they were fed on sheep offal during the 1970s and 1980s. The practice was banned only in 1988.

    Ten cases of nvCJD were reported in humans in 1996, which appeared to be distinct both clinically and pathologically, from sporadic and other variants of the disease.

    The first reliable signs of a direct causative link between spongiform disease in cattle and in humans emerged in 1997, with scientific evidence suggesting that the infective prions associated with bovine spongiform encephalopathy were identical with those of nvCJD.

    An editorial in the Lancet (1999353:939) last week lamented the “dangerous folly” that was allowed to prevail in farming in the United Kingdom and in animal feeding practices worldwide that continued to put profit before sound animal husbandry.

    Commenting on the new analysis of the cases, it said: “Clinicians, though, now have at least one good reason to maintain a high index of suspicion concerning unusual presentations of diseases, and to support strict surveillance of these when they occur.”

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