Define Creutzfeldt-Jakob disease (CJD)
The most commonly known disease in prion diseases group among humans is Creutzfeldt-Jakob Disease (CJD). This is a rare and fatal form of dementia and mainly occurs in individuals between the ages of 40 and 80. In 1996, scientists discovered a new strain of CJD that occurs predominantly in younger people.
More recent evidence has shown that the protein that accumulates in the brains of individuals with this new form of CJD is similar to the protein found in cattle infected with BSE, rather than that found in classical CJD. Because of this newly discovered difference, the new illness in humans is known as variant CJD or vCJD. The occurrence of a new form of CJD in the UK, where there is a high incidence of BSE, suggested that there might be a direct link between the two diseases. Some
cases that have developed vCJD are known to have eaten BSE-infected meat. Like BSE in cattle, vCJD is always fatal in people. How does ati individual get infected by vCJD? As discussed earlier, a victim of vCJD becomes infected through consumption of cattle products contaminated with the BSE agent. What are the symptoms and consequences? Generally, vCJD patients' show atypical clinical features with prominent psychiatric or sensory symptoms, with delayed onset of neurological abnormalities, including ataxia within weeks or months, dementia and myoclonus late in the illness.
Next, who are the most at risk of this disease? The risk to travelers (who visit the countries where outbreaks of BSE have taken place) and importers (who import beef and beef products from countries where vCJD cases have been reported) is the most. As far as travelers are concerned, the risk can be controlled by avoiding beef and beef products altogether or by selecting solid muscle pieces of beef with less chance of contamination and avoiding calf brains and burgers and sausages. Milk and