ArticlesCase-control study of risk factors of Creutzfeldt-Jakob disease in Europe during 1993-95*
Introduction
Creutzfeldt-Jakob disease (CJD) is the most clinically significant spongiform encephalopathy in man.1, 2 Although the disease is rare,3 the rapid development of bovine spongiform encephalopathy (BSE) from a sporadic to an endemic disease in cattle in the UK4 underscores the potential transmissibility of these diseases. The discovery of a new variant (nv) of CJD in the UK following the BSE5 epidemic has re-opened discussion on whether spongiform encephalopathies may be transmitted from animals to man. Also, the potential of iatrogenic transmission of CJD remains a matter of concern. CJD has been transmitted from person to person through human pituitary derived growth and gonadotropin hormones,6, 7, 8, 9 neurosurgery and electroencephalography electrode implantation,10, 11, 12, 13 and corneal transplantation.14 Up until now there has been no epidemiological evidence of transmission through blood transfusion,15 but research in animal models indicates that this possibility cannot be excluded.16, 17 Here, we present the findings of a collaborative study of risk factors for CJD in Europe, in which genetic factors, medical history, occupational history, animal exposure, and diet were studied.
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Patients and methods
The study was embedded within the European Union (EU) collaborative studies monitoring the incidence of CJD in Belgium (Flanders only), France, Germany, Italy, the Netherlands, and the UK.3 The registers aimed to ascertain all patients diagnosed with definite or probable CJD living in a defined geographical area, mainly at the national level. A standardised diagnostic protocol was used according to the criteria of Masters and colleagues.18 The pooled data set of 613 cases of CJD described
Results
54 cases and 22 controls had a positive family history of dementia other than CJD. In the cases of CJD the frequency of dementia in first degree relatives proved to be 2·26 (95% CI 1·31-3·90) that of the controls. Table 1 shows the medical history in patients and controls. Surgery of the vertebral column was found less often among cases, whereas there was a non-significant increase in the risk of CJD associated with surgery of the brain. A history of blood transfusion, electromyography, and
Discussion
In our collaborative study of risk factors of CJD, we found evidence for familial aggregation of CJD with dementia due to causes other than CJD. We did not find significant evidence for iatrogenic transmission of CJD. With regard to the exposures to animals and animal products studied, a significant increase in risk of CJD was found for cases exposed to leather products and fertiliser containing hoofs and horns. Of the dietary factors studied, the consumption of brain and raw meat were
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Cited by (184)
Prion diseases or transmissible spongiform encephalopathies
2022, Revue de Medecine InternePrions: detection of bovine spongiform encephalopathy and links to variant Creutzfeldt–Jakob disease
2022, Present Knowledge in Food Safety: A Risk-Based Approach through the Food ChainThe zoonotic potential of animal prion diseases
2018, Handbook of Clinical NeurologySafety of blood, blood derivatives, and plasma-derived products
2018, Handbook of Clinical NeurologyCitation Excerpt :Although there are a small number of reports of patients who have developed sporadic CJD following exposure to blood components (Klein and Dumble, 1993; Bennett and Daraktchiev, 2013), plasma derivatives (Creange et al., 1995, 1996; de Silva, 1996; Patry et al., 1998), or organ transplantation (Creange et al., 1995), these reports have not established a convincing link with sporadic CJD in the donors. To the contrary, a large number of epidemiologic case–control (Kondo and Kuroiwa, 1982; Davanipour et al., 1985; Harries-Jones et al., 1988; Esmonde et al., 1993; Wientjens et al., 1996; van Duijn et al., 1998; Will et al., 1998; Colins et al., 1999; Wilson et al., 2000; Zerr et al., 2000), look-back (Heye et al., 1994; Riggs et al., 2001), and surveillance (Brown, 1995; Operskalski and Mosley, 1995; Evatt, 1998; Evatt et al., 1998; Lee et al., 1998) studies over the past 35 years have demonstrated no clear evidence of transmission of sporadic CJD by blood transfusion or plasma derivatives. It should be borne in mind, however, that CJD is a rare disease and some cases of transmission by blood components or plasma products could have been missed if a detailed medical history and lookback to original donors were not undertaken.
Infectious and Sporadic Prion Diseases
2017, Progress in Molecular Biology and Translational ScienceCitation Excerpt :de Pedro Cuesta et al. reviewed the methodological difficulties in studying possible risk factors such as surgery in sCJD.13 A European case–control study of sCJD found no definite medical, surgical, or other environmental risk factors but provided evidence that could support the role of genetic factors other than PRNP mutations.14 Several studies have been undertaken to assess proposed genetic risk factors with negative results in relation to PRNP-117, PRNP-24b deletions, tau gene haplotypes, and a Cathepsin D polymorphism but positive results for the PLCXD3 gene, GRM8, and a polymorphism in the of a PRNP regulatory region.15–19
Plasma-derived products and Creutzfeldt-Jakob disease: Comparison of legislation around the world
2016, Pharmacien Hospitalier et ClinicienCitation Excerpt :Two limits are associated with this selection: CJD incubation time (from a few years to a few decades) and the absence of a reliable diagnosis test (currently, diagnosis is performed on clinical, radiological and electroencephalographic criteria when patient is still alive, but definitive diagnosis is performed post-mortem) [1,29]. For these reasons, different measures were implemented to select blood donors and to reduce potential risk of transmission of acquired or familial CJD, but cannot prevent this transmission during incubation of the disease [6,30,31]. In most of the countries, the 1980–1986 period has been selected for blood donors’ exclusion because first cases of bovine spongiform encephalopathy were identified in 1986.
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Members of the EU Collaborative Study Group of CJD are listed at end of paper