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Creutzfeldt-Jacob disease

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CJD (Creutzfeldt-Jakob Disease) has been and still is in the news. What is this disease? It is fortunately rare - or it is at the present time and hopefully it will remain so.

However, as the incubation period (that is, the time which may elapse before the disease becomes manifest and can be diagnosed), is anything from 5-15 years, the possibility arises that there will be more cases, just how many is not known. What we do know is that the age group involved is usually middle age and the mean age at death is 63 years.

The main symptoms are progressive dementia (a severe loss of normal mental ability and functioning). Unfortunately, a new variant of this disease was identified in March 1996. By November, 47 cases of the 'NV CJD' had been diagnosed in the UK and 1 in France. This type has affected a much younger population (mean age death of 29 years). The early symptoms have been noted to be numerous. The most distinctive appear to have been neurological involving difficulty in walking and lack of co-ordination. The later symptoms are hallicinatory and delusional leading to Dementia.

The causative agent is thought to be abnormal prior proteins (PrP's) which are normal constituents of mammalion cells. They are also thought to be responsible for Transmissable Bovine Encephalopahy in animals (BSE).

The cause of nv CJD is almost certainly due to exposure of the population to BSE infected beef products and was first encountered in the 1980's. Unfortunately, there is no way of making a definite diagnosis in life. It can be suspected, but can only be verified by identification of changes in the brain at post mortem. It cannot be emphasised more strongly that CJD is a rare disease. A CJD surveillance unit has been established and it is because of their vigilance that the new version of CJD was discovered.

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