CJD Support Network Group | Australian Creutzfeldt-Jakob Disease Support Group

Frequently Asked Questions

What is "MAD COW " Disease?

Variant CJD (vCJD) the human form of bovine spongiform encephalopathy (BSE) commonly known as “mad cow" disease has not occurred in Australia, although to 2006 more than 190 cases had occurred worldwide, most in the UK.

vCJD was first recognised in 1996 in the UK after the first death occurred in England in 1995.

Variant CJD is probably related to the consumption of bovine spongiform encephalopathy (BSE) contaminated meat products following the epidemic of BSE (a prion disease that occurs in cattle) during the 1980 and 1990s in the UK.

Is Variant CJD different to other forms of CJD?

Yes, vCJD sounds the same as CJD but they are different diseases. vCJD has

A predominantly psychiatric presentation
Younger age at onset – approx 28 years
Longer duration of illness – average 14 months
Different investigation results and distinctive pathological changes to the brain

See articles of interest – What is variant CJD and how does it differ from other forms of CJD?

What is Kuru?

Kuru is a human prion disease that was identified only in the central highlands of Papua New Guinea. People of of the Fore and closely linked tribes practiced endocannibalism, the eating of close relatives, as a mourning rite. These rituals were discouraged and outlawed by the late 1950s and correspondingly the numbers of recorded cases declined. Kuru has now almost disappeared.

Is CJD Contagious?

There is no evidence that CJD can be transmitted through social contact, transmitted through the air or through any type of touching, drinking from the same cup, kissing or sexual intercourse.

How is CJD Transmitted?

cCJD has been spread to other people by injections of contaminated human pituitary hormones, from grafts of dura mater, transplanted corneas and exposure to contaminated neurosurgical equipment.

vCJD may be transmitted to humans via the consumption of contaminated beef products and via blood transfusions in the UK.

Why am I deferred from donating blood if I have lived in UK for a total of six months during 1980 to 1996?

There is emerging evidence that vCJD, believed to be a human form of bovine spongiform encephalopathy (BSE or “Mad Cow" Disease) can be transmitted via blood transfusion. People who have spent six months or more in the UK during the BSE risk period of 1980–1986 have been deferred from donating blood in Australia. This has now been extended to include people who have received a transfusion or injection of blood products in the UK since 1980.

Why am I deferred from donating blood if a first degree family member has died of CJD?

The Red Cross Blood Service has no way of knowing if your relative died of sporadic CJD or a familial or inherited form of CJD.

Although there is no evidence that CJD is transmitted via blood in classical CJD cases there is no way to prove it is not. The blood banks of Australia currently defer anyone from donating blood who is at risk of CJD. This includes people who have a family history of inherited CJD.

Current infection control guidelines recommend deferral when there is a family history of two or more first degree relatives but the Red Cross Blood Service questionnaire refers to one first degree relative.

Technically, people who have had PRNP genotyping with no mutation confirmed, should be able to give blood.

Why am I deferred from donating blood if I have received human pituitary hormones prior to 1985 or dura mater grafts prior to 1990?

As a recipient of human pituitary hormones or dura mater grafts you are considered to be at “low risk” of CJD. Although transmission of classical forms of CJD by blood products is highly unlikely, there is still a theoretical risk that cannot be disproved.

Is CJD a notifiable disease in Australia?

In June 2004 the Communicable Diseases Network of Australia agreed to designate any TSE as a notifiable disease in Australia.