Creutzfeldt-Jakob disease
(CJD · Classical CJD · Variant CJD)
In this condition factsheet:
Diagnosing Creutzfeldt-Jakob disease
CJD is a very rare disease, and it is difficult to diagnose. Although some symptoms might look like they are due to CJD, they are more likely to be caused by another condition. Only close observation over time might give the doctor clues that point to a CJD diagnosis. For instance, dementia appears much more quickly in CJD than in conditions like Alzheimer's.
A detailed medical history will be taken. Clues towards a diagnosis of CJD may be gained from tests that monitor brain wave activity (EEG), special genetic tests looking for susceptibility to vCJD, magnetic resonance imaging (MRI) to obtain images of the brain, and spinal fluid tests ("spinal taps") to test for a specific protein.
The only test to confirm a diagnosis of CJD is a brain biopsy, in which a sample of brain tissue is removed, or an autopsy, where the brain is examined after death.
Treating and Preventing Creutzfeldt-Jakob disease
There is no known cure or treatment for CJD.
Classical CJD cannot be completely prevented or avoided, but it occurs so rarely that the odds are greatly against being infected with it. Health care providers can minimize risk by handling tissues and bodily fluids with extreme caution, and using effective sterilization methods consistently to disinfect equipment. People with classical CJD can have genetic testing done to see if the disease runs in their family. Genetic counselling may be recommended before this testing is done.
The risk of variant CJD can be decreased by not eating the brains and nervous system tissue of prion-infected animals. Obviously, you can't guarantee that a particular piece of meat is prion-free. However, when reports of infected cows occur, countries and officials are typically quick to isolate and trace the affected animals, making human infection very unlikely.
In North America, there is a prion disease affecting deer and elk, called chronic wasting disease. If you hunt, you might want to think twice before shooting or eating an elk or deer that appears confused, deranged, emaciated, or unconcerned about its own survival. By law, animals with signs of chronic wasting disease must be reported to provincial authorities.
Prions tend to accumulate in nerve cells, and cuts of meat that come from near the spinal cord and brain are the most dangerous. You are probably less likely to eat prions if you eat cuts of meat from other parts of the animal (e.g., steaks, roasts). Keep in mind that prions are not destroyed by cooking meat.
Canada is taking the following steps to prevent the spread of BSE in cattle, which will decrease the risk of variant CJD in humans:
- Health Canada is working closely with the Canadian Food Inspection Agency (CFIA) as part of the agency's National Response Team.
- Health Canada is assessing the implications of BSE and any emerging information from trace-back studies.
- The Canadian Food Inspection Agency (CFIA) has taken various precautions to prevent the introduction and spread of BSE, including creating a surveillance program in which the brains of cattle are tested for the disease.
- Since 1997, Canada has banned the feeding of specific tissues that are known to accumulate BSE-causing prions (specified risk material; SRM) from ruminant animals (cattle, sheep, goats, bison, elk, or deer) to other ruminants.
- In 2009, Canada banned SRM from all animal feeds, pet foods and fertilizers. By 2017, it is expected that BSE should be completely eliminated from Canadian cattle.
- In 2021, the World Organisation for Animal Health (OIE) World Assembly of Delegates recognized Canada as a country with negligible risk for BSE.
- The CFIA has mandated that all suspected BSE cases be reported to a federal veterinarian. The CFIA has also created a Canadian Cattle Identification Program for cattle and bison, making it possible to trace individual animal movements from the herd of origin to the time of slaughter.
All material copyright MediResource Inc. 1996 – 2024. Terms and conditions of use. The contents herein are for informational purposes only. Always seek the advice of your physician or other qualified health provider with any questions you may have regarding a medical condition. Source: www.medbroadcast.com/condition/getcondition/Creutzfeldt-Jakob-disease
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