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Updated Creutzfeldt–Jakob disease infection control guidelines: sifting facts from fiction

New guidelines aimed at reducing iatrogenic disease and discrimination against patients

Creutzfeldt–Jakob disease (CJD) is a rare neurodegenerative disorder which causes the death of about 25–30 Australians each year, giving an average mortality rate of 1.2 cases/million/year.1 It is untreatable. CJD is the commonest human form of prion disease2 and is a notifiable disease in all Australian states and territories, with notification to the relevant jurisdictional health department required for all cases in which a strong clinical suspicion for CJD exists.

CJD may be acquired through medical intervention (iatrogenic CJD), for example, from the use of cadaver-derived pituitary hormones; or it may be inherited in an autosomal dominant pattern with high penetrance (familial CJD, occurring in about 10%–15% of cases); but in at least 85% of cases it occurs sporadically (sporadic CJD),2 with the person having no recognised cause for the disease. Variant CJD (vCJD) is the form zoonotically linked to bovine spongiform encephalopathy (mad cow disease), with a median age at death of around 28 years.2