NR AJZN
AU Ridley,R.M.; Baker,H.F.
TI Occupational risk of Creutzfeldt-Jakob disease
QU Lancet 1993 Mar 6; 341(8845): 641-2
KZ Lancet. 1993 Jan 9; 341(8837): 123-4. PMID: 8093398
PT comment; letter
VT
Sir,- Readers should be cautious about Dr Weber and colleagues' (Jan 9, p 123) suggestion that occupational transmission of Creutzfeldt-Jakob disease (CJD) may have taken place in a neurosurgeon, a pathologist, 2 histology technicians, and an orthopaedic surgeon. Large epidemiological surveys (1,2) have failed to find a link between occupation and CJD. This disease has been reported in several people working in occupations in which exposure to neural tissue could have happened (eg, butchers, farmers, and various health professionals (3)) but the number of these cases is not in excess of that which would be expected by random association. In the absence of a clear excess of cases, as has occurred in the iatrogenic transmission of spongiform encephalopathy by exposure to human derived growth hormone (4), the occurrence of CJD in people from the medical and paramedical professions is no more remarkable than its occurence in people of any other profession. Brown et al (1) reported six cases among clerics, but this does not necessarily implicate their occupation in their ultimate demise.
The notion that CJD is always acquired (as opposed to idiopathic) and that the existence of any hypothetical risk factor must therefore be the cause of the disease led to the much cited claim that the high incidence of CJD among Libyan Jews was due to their consumption of sheep's eyeballs (5), despite a lack of evidence that their dietary habits differed from their ethnic neighbours in whom no increased incidence of this disease was recorded. The high frequency of CJD in the Libyan Jews is now known to be due to a codon 200 mutation in the PrP gene in affected families in that ethnic group (6).
CJD is a peculiar disease that does not fit into any single pattern of distribution. The great majority of cases cannot be attributed to environmental exposure. Very particular precautions are required to prevent transmission from cases of human and animal spongiform encephalopathy since, when this does occur, a major outbreak of disease can arise. Under these circumstances it is especially important that the occurrence of CJD is viewed from an epidemiological rather than an anecdotal perspective.
REFERENCES
1. Brown P, Cathala F, Raubertas RF, et al. The epidemiology of Creutzfeldt-Jakob: conclusion of a 15-year investigation in France and a review of the world literature. Neurology 1987; 37: 895-904.
2. Harries-Jones R, Knight R, Will RG, et al. Creutzfeldt-Jakob disease in England and Wales, 1980-1984; a case control study of potential risk factors. J Neurol Neurosurg Psychiatry 1988; 51: 1113-19.
3. Masters CL, Harris JO, Gajdusek C, et al. Creutzfeldt-Jakob disease: patterns of worldwide occurrence and the significance of familial and sporadic clustering. Ann Neurol 1978; 5: 177-88.
4. Brown P, Gajdusek C, Gibbs CJ, Asher DM. Potential epidemic of Creutzfeldt-Jakob disease from human growth hormone therapy. N Engl J Med 1985; 313: 728-31.
5. Kahana E, Alter M, Braham J, Sofer D. Creutzfeldt-Jakob disease: focus among Libyan Jews in Israel. Science 1974; 183: 90-91.
6. Hsiao K, Meiner Z, Kahana E, et al. Mutation of the prion protein in Libyan Jews with Creutzfeldt-Jakob disease. N Engl J Med 1991; 324: 1091-97.
MH Creutzfeldt-Jakob Syndrome/*epidemiology/transmission; Food Habits; Health Occupations/statistics & numerical data; Human; Occupational Diseases/*epidemiology
SP englisch
PO England