Creutzfeldt-Jakob Disease (CJD) Mad Cows
Creutzfeldt-Jakob disease (CJD) is a rare, degenerative, fatal brain disorder that affects about one in every million people per year worldwide. Creutzfeldt-Jakob disease belongs to a family of human and animal diseases known as the transmissible spongiform encephalopathies (TSEs). This includes bovine spongiform encephalopathy (BSE), which is found in cows and often referred to as “mad cow” disease. Symptoms usually begin after age 60 and most patients die within 1 year. Many researchers believe Creutzfeldt-Jakob disease results from an abnormal form of a protein called a prion. Most cases of Creutzfeldt-Jakob disease occur sporadically – that is, in people who have no known risk factors for the disease. However, about 5% to 10% of cases of Creutzfeldt-Jakob disease in the United States are hereditary, caused by a mutation in the gene for the prion protein. Patients with Creutzfeldt-Jakob disease may initially experience problems with muscular coordination; personality changes, including impaired memory, judgment, and thinking; and impaired vision. Other symptoms may include insomnia and depression. As the illness progresses, mental impairment becomes severe. Patients often develop myoclonus and they may go blind. They eventually lose the ability to move and speak, and go into a coma. Pneumonia and other infections often occur in these patients and can lead to death.
