ONE of the questions we get asked on the myriad of health questionnaires that we have to fill in at the doctors, chiropractors or for x-rays is - do you are any of your family have Creutzfeldt-Jakob disease? Most of us will fortunately answer ‘No,’ but what is it?

Creutzfeldt-Jakob disease (CJD) is a rare brain disease that is caused by abnormal brain proteins called prions. Classical CJD usually occurs by chance.

Variant CJD, which is linked to consumption of contaminated meat products, does not occur in Australia.

In CJD, the structure of a normal brain protein changes slightly forming prions.

The build-up of prions damages brain cells and causes the neurological symptoms of CJD. Unlike bacteria or viruses, prions resist normal methods of heat and chemical sterilization.

There are two different types of Creutzfeldt-Jakob disease:

Classical CJD occurs in Australia and about one in one million people per year develops the disease.

There are three types of classical CJD. About 90 per cent of classical CJD cases occur by chance (sporadic CJD) and 10 per cent of cases are hereditary (familial or inherited CJD).

The disease has very rarely been transferred between patients following medical procedures such as brain surgery or the use of dura mater grafts or contaminated human pituitary hormones (iatrogenic CJD).

Variant CJD, is a disease that emerged in the UK in the 1990s.

Variant CJD is linked to the consumption of meat products from cattle infected with bovine spongiform encephalopathy (BSE, or "mad cow disease").

Variant CJD is a separate disease to classical CJD, although some of the symptoms are similar.

No cases of variant CJD have been identified in Australia to date. Australian cattle remain free of BSE.

What are the symptoms?

Classical CJD have advancing neurological symptoms that may include changes to behaviour, blindness, weakness and loss of balance.

Coordination difficulty is common - presenting as difficulty walking, speaking or muscle spasms.

Confusion will usually lead to dementia.

The disease is fatal - usually weeks or months from the onset of symptoms.

The great majority of cases are caused by mutations within the persons brain - each year about one in every million Australian people will be diagnosed with CJD.

The average age of onset is around 65 years of age.

There is no treatment and diagnosis is made by brain tissue sample and it most often confirmed after death.

With all this information onboard; spare a thought for Wannon MP, Dan Tehan.

Mr Tehan took part in the annual Run4CJD charity run from Adelaide Oval to Terang covering more than 900km to raise awareness and funds. 

Mr Tehan said, “Earlier this month, I took part in the Run4CJD charity run”.

“I lost both my mum and my aunty to Creutzfeldt–Jakob disease (CJD), a rare disease that causes the brain to degenerate, leading to dementia and death,” he said.

“At the time, we didn’t know much about it, and we felt very alone.

“The goal of the run, now in its second year, is to raise funds for critical research for CJD.

“Our group of 12 ran 900kms from Adelaide to Terang via Mildura, Horsham and Hamilton.

“Thank you to everyone who supported us with donations and moral support when the rain set in!

“My mum Marie passed away from CJD in 2004 and my aunt Helen Arthur in 1984.

“Our hope is this charity run has helped raise much needed awareness and funding for CJD.

 “I want people to know they are not alone, and I want to help raise money for research that leads to a cure.

“If anyone wants to support our effort, they can donate via our donation page at the CJD Support Group Network webpage.

“An elected Coalition Government will fund $265,000 to progress the next two stages of critical research into Creutzfeldt–Jakob disease (CJD), which takes the lives of approximately 80 Australians per year.”