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Death usually occurs within a year.

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It has been reported to be about one case per million population. It is a very rare disease.

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Prion diseases occur when normal prion protein, found on the surface of many cells, becomes abnormal and clump in the brain, causing brain damage.

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Creutzfeldt-Jakob disease is marked by changes in mental abilities. Symptoms get worse quickly, usually within several weeks to a few months. Early symptoms include:

Personality changes

Anxiety

Depression

Memory loss

Impaired thinking

Blurred vision or blindness

Insomnia

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The word "prions" refers to abnormal, pathogenic organisms that can spread and are capable of causing specific, normal cellular proteins known as prion proteins

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There is no effective treatment that is available for the disease, therefore the focus is laid on easing the pain and other symptoms.

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A cellular glycoprotein identified as the prion protein is thought to be the cause of the neurodegenerative condition Creutzfeldt-Jakob disease, which progresses quickly and is always fatal.