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What is Creutzfeldt-Jakob Disease?

Creutzfeldt-Jakob Disease) is a rare progressive degenerative brain condition that leads to dementia and is ultimately fatal. It is very similar to the well-known Alzheimer’s disease, however it progresses much more rapidly than it. Creutzfeldt-Jakob disease only usually affects those over 60 years of age. Creutzfeldt-Jakob is classified in a group of human diseases known as the prion diseases which are caused by small, abnormally deformed proteins that cause an infection called prions. In the late nineties in the UK the disease got a lot of new media publicity and public hysteria as ‘mad cow disease’ after some ate meat from diseased cattle, but that is a different variant of the disease to Creutzfeldt-Jakob disease, but the link between the two is firmly stuck in the public’s perception. Most cases do seem to appear spontaneously and they do not appear to have been in contact with another person who has it to catch it.

The initial sign and symptoms are problems with the memory and increasing anxiety with a lot of fatigue. There may also be headaches and some dizziness with changes in the persons behavior. Later on the dementia progresses substantially, also with a weakness and stiffness of legs and arms and they may also go blind if the vision is affected. EEG testing as well as a MRI will show the characteristic changes of the condition.  Up to 90% are fatal within one year of the diagnosis being made, most of those are caused by pneumonia or other infections. There is no specific treatment that is available for Creutzfeldt-Jakob disease and any treatment that is given is usually just directed at helping with the signs and symptoms and to make them as comfortable as is possible as the disease gets worse. Opioids analgesic drugs are often given to help with the pain as well as clonazepam or sodium valproate may be given to help with some of the movements disorders in the limbs that may develop. In many nations Creutzfeldt-Jakob disease is legally required to be reported by the doctors who diagnose a case are required to authorities. The reason for this is so that the community health units that are responsible for the testing and surveillance of disease can take responsibility for monitoring and possible preventative measures.