CJD is one of a group of diseases that affects humans and animals, known as the transmissible spongiform encephalopathies (TSE) or prion diseases. There is currently no cure or control for CJD but research continues on many drugs. CJD is still confirmed by autopsy although a test performed on cerebrospinal fluid to detect a protein marker can help diagnose CJD in people who already show clinical symptoms of the disease.

Scientific evidence has linked variant CJD to eating contaminated beef products from animals infected with bovine spongiform encephalopathy (BSE) or "mad-cow disease." Processed meat products made from cows infected with BSE carry a high risk for transmitting the disease.

Variant CJD symptoms include early psychiatric symptoms such as anxiety, depression, withdrawal and behavioural changes. Persistent pain or odd sensations in the face or limbs often develop. The disease then progresses to motor difficulties, involuntary movements and mental deterioration, often ending in a persistent vegetative state. The patient may live on average for about one year after the onset of symptoms.

Once a suspected case of variant CJD is reported to the Public Health Agency of Canada's CJD Surveillance System, the Department coordinates testing and works with case physicians and international experts to confirm the diagnosis. The diagnosis of variant CJD is very difficult, but brain scans, using magnetic resonance imaging (MRI), and tonsil biopsy are two types of tests used to establish a probable diagnosis.

The first signs of a CJD are rather unspecific: decline in efficiency, social retreat, loss of weight, sleep disturbances and signs of depression. Within a few weeks the general condition deteriorates. Deficits in memory, speech impediments, disturbed coordination as well motoric defects are signs of a progressive CJD. A few weeks later the patient develops: involuntary muscular twitching, muscle stiffness and difficulties in walking leading to total confinement to bed.

Creutzfeldt-Jakob Disease (CJD) is one of a small group of fatal diseases caused by infectious agents called prions. These attack the brain, killing cells and creating gaps in tissue. The disease is always fatal.

The agent that causes variant CJD is believed to be an abnormal form of a protein, known as a prion. Prions are normal cellular proteins that are present in many organs and tissues, including the brain, spinal cord and eyes of healthy humans and animals. The abnormal form of prion causes surrounding proteins to change their shape. The abnormal protein collects in central nervous tissue and by an unknown mechanism causes the nerve cells to die.

No screening test is available for persons who may have been exposed to "mad-cow disease," hence there is no known method of detecting variant CJD before symptoms appear.

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

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 possibly via blood and blood products.

Scientists haven't yet determined if variant CJD can be transmitted by blood. To date, there has never been a documented case of variant CJD transmitted through blood transfusion. However, evidence from experimental work in animals suggests that transmission of variant CJD by blood is theoretically possible. There have been two reported cases of transfusion-associated vCJD infection in UK.

In August, 2002 the first confirmed case of variant CJD was diagnosed in Canada. However, all the evidence indicates that, in this case, the individual was infected in the UK. There have been no subsequent cases of variant CJD in Canada. The Public Health Agency of Canada works closely with other federal agencies and experts on CJD around the world to protect the health of Canadians.

BSE, sporadic CJD, and variant CJD are all Transmissible Spongiform Encephalopathies (TSEs), which is a class of rare brain diseases, some of which affect humans while others affect animals. All TSEs are associated with the accumulation of abnormal prion proteins in the brain. While BSE is found only in cattle, sporadic CJD and variant CJD are found in humans. Sporadic CJD and variant CJD are distinctly separate brain diseases, each with its own unique clinical and histopathological features.

CJD (Creutzfeldt-Jakob Disease) occurs worldwide with a frequency of one case per million inhabitants/year. More than 90% of the CJD cases emerge sporadically and are thus termed sporadic CJD (sCJD). The source of infection of those cases is unclear, although proponents of the prion hypothesis assume that the disease might develop spontaneously. More than 90% of the sCJD cases affect humans older than 60 years.

Since 1994 CJD is a notifiable disease in the EU. Surveillance of CJD became mandatory in Germany as of January 1st, 2001, in the framework of new legislation (Infectious Disease Protection Act- "Infektionsschutzgesetz IfSG) passed to supplement and expand the Federal Disease Protection Act (Bundesseuchengesetz ? BseuchG). CJD surveillance is a passive monitoring system in Germany, i.e.

The frequency of 1 to 2 cases of CJD per one million inhabitants and year in Germany matches the average of comparable European countries. In a case control study of the "Surveillance centre for CJD" 1666 patients have been admitted so far (April 2004).

Variant CJD is a relatively new disease, and knowledge about it is limited. However, to date there is no evidence that transmission of variant CJD from a mother to her unborn child or through breast milk has occurred in people with variant CJD.

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

Ring or email us if you are a recipient of human pituitary hormones or at risk of CJD and would like to become of member of our Network.

The blood bank 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.