Geographical distribution of cases of Creutzfeldt-Jakob disease in England and Wales 1970-84.

Abstract

Two hundred and sixty seven patients with a definite or probable diagnosis of Creutzfeldt-Jakob disease (CJD) were identified in an attempt to ascertain all cases of the disease in England and Wales in the period 1970-84. No evidence of space-time clustering of dates and places of onset was found for these cases. Two age-and sex-matched controls were selected for 72 of the 122 cases diagnosed in the period 1980-84 and life histories of places of residence were obtained for these cases and the controls. No evidence was found that cases had lived closer to each other than had controls at any time in their lives, except that there was an excess of cases born in London. The study does not provide supportive evidence for case-to-case transmission of CJD being an important component in the aetiology of the disease.

Transmission of Creutzfeldt-Jakob disease from humans to transgenic mice expressing chimeric human-mouse prion protein.

“…The BSE epidemic has led to considerable concern about the safety for humans consuming beef and dairy products. Although epidemiologic studies over the past two decades argue that humans do not contract CJD from scrapie-infected sheep products (43)(44)(45), it is unknown whether any of the seven amino acid substitutions that distinguish bovine from sheep PrP render bovine prions permissive in humans (46). Whether Tg(MHu2M) mice are susceptible to bovine or sheep prions is unknown.…”

Molecular characterization of proteolytic cleavage sites of the Pseudomonas syringae effector AvrRpt2

“…Why have older individuals not developed vCJD-based neuropathologic criteria? It is noteworthy that epidemiological studies over the past three decades have failed to find evidence for transmission of sheep prions to humans (9)(10)(11)(12). Attempts to predict the future number of cases of vCJD, assuming exposure to bovine prions prior to the offal ban, have been uninformative because so few cases of vCJD have occurred (312)(313)(314).…”

“…Epidemiologic studies designed to identify the source of the CJD infection were unable to identify any predisposing risk factors, although some geographic clusters were found (9)(10)(11)(12). Libyan Jews living in Israel developed CJD about 30 times more frequently than other Israelis (13).…”

“…How prions causing disease arise in patients with sporadic forms is unknown; hypotheses include horizontal transmission of prions from humans or animals (242), somatic mutation of the PrP gene, and spontaneous conversion of PrP C into PrP Sc (5,15). Because numerous attempts to establish an infectious link between sporadic CJD and a preexisting prion disease in animals or humans have been unrewarding, it seems unlikely that transmission features in the pathogenesis of sporadic prion disease (9)(10)(11)(12)243).…”

Variation on a theme of Creutzfeldt-Jakob disease: implications of new cases with a young age at onset

“…No rural clusters were identified and there was little evidence for space-time clustering. In similar studies of CJD in England and Wales between 1970-1984, Cousens et al (1990 found no evidence of space-time clustering of dates and places at onset although they did find an excess of cases born in London. However, these authors warned that a number of statistically significant relationships in the earlier reports based on data for 1970--1979 were no longer significant when the data were extended to cover [1970][1971][1972][1973][1974][1975][1976][1977][1978][1979][1980][1981][1982][1983][1984].…”