What is Creutzfeldt-Jakob Disease (CJD)?

What is Creutzfeldt-Jakob Disease (CJD)?
Creutzfeldt-Jakob disease (CJD) is an extremely rare, fatal, degenerative disease. It is one of a group of diseases called prion diseases, which can occur in both humans and animals. Despite its extreme rarity, Creutzfeldt-Jakob disease has become fairly well known over the past decade because of its association with bovine spongiform encephalopathy (BSE), or "mad cow disease." This association is somewhat misleading, however. While Creutzfeldt-Jakob is the human-affected prion disease that appears to be the result of infection by ingestion of BSE contaminated meat, or by exposure to the blood products contaminated by the disease, the cases of CJD caused by BSE exposure make up only a very small fraction of Creutzfeldt-Jakob disease cases.

Symptomology of CJD begins with rapid onset of dementia, which leads to memory loss, personality changes, and hallucinations. Other symptoms include speech impairments, sudden, jerky movements, problems with balance and coordination, changes in gait, rigid posture, and the onset of seizures. (Wikipedia)

Typically, the onset of symptoms of CJD occurs at about 60 years of age. There are three forms of Creutzfeldt-Jakob disease, sporadic CJD, familial CJD, and acquired CJD. Creutzfeld-Jakob disease affects approximately 1 in 1 million people, per year. This translates to less than 300 cases per year in the United States. (The Creutzfeldt-Jakob Disease Foundation)

It is believed that the CJD can incubate in the body for a period of several years or more before the onset of symptoms, with no evidence of its existence. There is no cure for Creutzfeldt-Jakob disease, and death usually occurs within six months to one year of the onset of symptoms.


What is the Connection Between Creutzfeldt-Jakob Disease (CJD) and Mad Cow Disease?