Creutzfeldt-Jakob disease in England and Wales, 1980-1984: a case-control study of potential risk factors.

Abstract

SUMMARY An attempt was made to ascertain all cases of Creutzfeldt-Jakob disease occurring in England and Wales during the 5 year period 1980-1984. The mean annual mortality rate was 0-49/million; women were more frequently affected than men. The age-specific mortality rate reached a peak in the seventh decade. A case-control study involving 92 of the 122 definite and probable cases ascertained failed to confirm the reality of previously suspected aetiological agents in the environment. Although there was no confirmed instance of familial Creutzfeldt-Jakob disease in the case-control study, dementia in close relatives was significantly more common than in controls.

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).…”

Risikofaktoren der sporadischen Creutzfeldt-Jakob-Krankheit

“…Einen Überblick über die bisherigen Studien geben die Tabellen 15 und 16. Die Studienergebnisse von Bobowick et al (1973), Kondo und Kuroiwa (1982), Davanipour et al (1985), Harries-Jones et al (1988) (Poser et al 1999, Heinemann et al 2007a (Davanipour et al 1985). …”

“…Es sind 4 vCJK-Fälle beschrieben, die wahrscheinlich durch eine Bluttransfusion übertragen wurden (Llewelyn et al 2004, Peden et al 2004, Wroe et al 2006 In bisherigen Studien wurden bisher entweder nicht signifikante Ergebnisse oder verminderte Risiken für den Zusammenhang einer Bluttransfusion mit der sCJK publiziert (Kondo und Kuroiwa 1982, Davanipour et al 1985, Harries-Jones et al 1988, Duijn et al 1998, Zerr et al 2000b, Ward et al 2002und 2008. Zudem wurden bei Patienten mit Hämophilie, die vor der Einführung von rekombinanten Gerinnungsfaktoren Blutprodukten von sehr vielen Spendern ausgesetzt waren, keine CJK-Fälle festgestellt (Evatt et al 1998, Lee et al 1998).…”