Creutzfeldt-Jakob disease (CJD) is a rare brain disease that affects about one in one million people each year. It develops when certain proteins in the brain do not form correctly, and then are not eradiacted as they are supposed to be. This build-up causes a range of non-specific symptoms that can make diagnosing this condition very difficult.
Creutzfeldt-Jakob disease falls into the family of prion diseases. Prions are harmless proteins present in most body tissues, and their highest concentration is in the brain. They go through a process of folding, and if there is a mistake in they process, the body naturally destroys them.
In CJD, misfolded prions are not destroyed, instead accumulating in high levels and damaging the brain cells. This damage results in tiny cavities in the brain tissue that make it sponge-like in appearance. These alterations are called spongiform changes.
Prions are infectious, but they are not like bacteria or viruses and the usual sterilization processes cannot destroy them, nor do antibiotics and antiviral drugs affect them. The prions can be passed on either by direct contact with contaminated material such as infected meat or human tissues, or genetically by inheriting an abnormal gene. In most cases, doctors cannot determine the cause of CJD.
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