What Are the Causes of CJD?

Creutzfeldt--Jakob disease is the most common among the types of transmissible nerve disorders. It causes the brain to develop holes like a sponge. According to the National Institute of Neurological Disorders and Stroke, CJD affects about one person in every million people per year worldwide. There are two main categories of CJD: classic and new variant. Classic CJD is subdivided into sporadic and familial forms. Although many equate CJD with mad cow disease, only the more rare new variant CJD is related. Classic CJD and new variant CJD are two distinct forms of the nervous disorder with different causes.

Signs and Symptoms

• The rapid death of the brain nerve cells causes the signs and symptoms of the disease. Usually, the first symptom to occur is dementia, which rapidly progresses to include memory loss, personality changes and hallucinations. Many patients also experience muscle twitching, loss of coordination, speech changes, balance dysfunction, changes in walking gait and seizures. CJD differs from other nervous disorders because of the rapid progression of symptoms; the disease can cause death within weeks.

Causes

• The leading theory is that all forms of CJD are caused by a protein called a prion. Prion proteins have two forms: normal and infectious. The infectious form of prion proteins has a different final shape that causes them to clump together, leading to nerve cell death. The classic sporadic type of CJD is thought to be caused by a spontaneous mutation of normal prion proteins. The classic familial type of CJD results from a mutation of the gene that controls the formation of a normal prion protein.

Transmission

• The infectious protein can be transmitted a number of ways. CJD cannot be transmitted via casual contact or through the air like a cold or flu. Familial CJD is transmitted via genetics. Exposure through organ tissue, blood, cellular tissue and spinal cord fluid may lead to the disease. There have been a small number of people with the new variant form of CJD that appear to have contracted the disease from beef contaminated with mad cow disease. Even though scientists consider transmission from one person to another rare, transplant tissue, blood and food should be thoroughly screened to reduce the risk.

Treatment

• Currently, there are no treatments for CJD. Scientists have had no luck controlling or curing the disease with any steroids, antiviral agents, antibiotics or other treatments. Physicians and other caregivers focus on reducing symptoms and making the patient as comfortable as possible.