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Description


Creutzfeldt-Jakob disease (CJD) is a rare brain disorder. There are three major types of CJD:

sporadic CJD, thought to be caused by a spontaneous mutation in the brain cells. It has been estimated that approximately one person in a million is affected.

hereditary CJD, which occurs in approximately 5?15 per cent of cases; and

acquired CJD, which, while rare, has led to its being classified as an infectious disease.


The risk of transmission from person-to-person is very small. Acquired CJD is more usually transmitted accidentally, either during surgery, through use of contaminated instruments, or through use of diseased human tissue. In Australia, five people are known to have died after treatment with pituitary hormone products. In Britain, CJD has been associated with eating beef infected by bovine spongiform encephalopathy ("mad cow disease”).

Onset of symptoms is usually slow, typically occurring at about the age of 60. Changes in behaviour include impaired judgment and memory loss, irritability, involuntary muscle spasms and, frequently, seizures. As the disease progresses, patients develop severe dementia, losing both mental and physical function, and frequently lapsing into a coma before death.


Treatment

Currently, there is no cure for CJD, and treatment is aimed at alleviating symptoms. Anti-convulsant drugs (such as clonazepam and sodium valproate) may be used to relieve muscle spasms, while opiate drugs may be prescribed for pain.


Prognosis

Approximately 90?95 per cent of patients die within a year of diagnosis; a small number of patients have survived for more than two years.

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