UNTREATABLE CAUSES OF DEMENTIA: CREUTZFELDT-JAKOB DISEASE
Creutzfeldt-Jakob disease (CJD) is probably a collection of different clinical syndromes, or patterns of disease presentation, which have in common the same transmissible causal agent. That is, they may be caused by infection from one individual to another. The infection can even be transmitted by using on one patient neurosurgical instruments that have been earlier used in an operation on another patient suffering from this condition, despite the instruments having been sterilized in the normal manner between operations. For this reason great care is now taken of the instruments when a person who may have CJD is being operated on.
It should be stressed, however, that this condition is very rare, which means that it must be a very difficult disease to transmit from one person to another; there is no evidence of anyone developing CJD as a result of caring for someone with the disease. Nevertheless those who are working with or looking after a sufferer from CJD, should take seriously the precautions advised by the doctors in charge.
The disease is accompanied by a pathological process that causes lots of small ‘holes’ to appear within the substance of the brain. It is marked in most cases by a particularly rapid progression of dementia associated with abnormalities of movement. Unlike Huntington’s chorea, the movement problem is more a case of difficulty in maintaining balance and coordination, than the development of extra abnormal movements, with the exception of ‘twitches’, which occur in most sufferers from both diseases. These are called myoclonic jerks. The physical manifestations of the disease are very variable, depending upon which structures in the brain are particularly affected.
CJD usually runs its course in three to twelve months, but in some cases it is much more prolonged. As with the other chronic conditions that cause dementia there is no diagnostic test although often one is helped, albeit late in the disease, by an electroencephalograph (EEG) picture, showing up an abnormality.
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