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