Diagnosed independently by Drs.

Creutzfeldt and Jakob in the early 1920s, the disease that bears their names is a rare, degenerative brain disorder that affects approximately one person per million per year worldwide. This translates into 250-300 new U.S. cases annually. However, because Creutzfeldt-Jakob Disease (CJD) is very difficult to diagnose, this number may actually be higher. CJD affects men and women of all cultural backgrounds, with symptoms appearing most often in those aged 50 to 75. Creutzfeldt-Jakob Disease is known as a prion disease, which means that healthy brain tissue deteriorates into an abnormal protein that the body cannot break down. CJD is a type of transmissible spongiform encephalopathy (TSE), a classification that includes both human and animal diseases. "Spongiform" refers to the characteristic appearance of an infected brain, which becomes filled with holes until it looks like a sponge under a microscope. Prior to the "mad cow disease" epidemic of the 1990s, few people had heard of Creutzfeldt-Jakob Disease. However, once a growing number of young people in the United Kingdom started developing symptoms that appeared congruent with CJD, the world became alarmed. This new form of the illness, named variant Creutzfeldt-Jakob Disease or vCJD, results only from exposure to beef that is contaminated with bovine spongiform encephalopathy (BSE), the scientific term for mad cow disease. What are the types of Creutzfeldt-Jakob Disease? There are four types of Creutzfeldt-Jakob Disease: sporadic (or classical), inherited (or familial), iatrogenic (or acquired) and variant.