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Medical Author:

Melissa Conrad Stöppler, MD, Chief Medical Editor

Melissa Conrad Stöppler, MD, Chief Medical Editor

Melissa Conrad Stöppler, MD, is a U.S. board-certified Anatomic Pathologist with subspecialty training in the fields of Experimental and Molecular Pathology. Dr. Stöppler's educational background includes a BA with Highest Distinction from the University of Virginia and an MD from the University of North Carolina. She completed residency training in Anatomic Pathology at Georgetown University followed by subspecialty fellowship training in molecular diagnostics and experimental pathology.

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Medical Editor:

Charles Patrick Davis, MD, PhD

Charles Patrick Davis, MD, PhD

Dr. Charles "Pat" Davis, MD, PhD, is a board certified Emergency Medicine doctor who currently practices as a consultant and staff member for hospitals. He has a PhD in Microbiology (UT at Austin), and the MD (Univ. Texas Medical Branch, Galveston). He is a Clinical Professor (retired) in the Division of Emergency Medicine, UT Health Science Center at San Antonio, and has been the Chief of Emergency Medicine at UT Medical Branch and at UTHSCSA with over 250 publications.

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Prion diseases are unique and can be transmitted in a variety of ways:

• Some forms can be inherited such as familial CJD, Gerstmann-Sträussler-Scheinker disease (GSS), and fatal familial insomnia (FFI). The disease is caused by a mutation in the prion gene. Other inherited prion diseases are more rare.
• Prion disease may develop sporadically, for no apparent reason and with no pattern, such as sporadic CJD. Cases can occur in men and women of all ages, but the average age is 62 years. The prevalence of sporadic CJD is about one case per million people each year throughout the world, even among vegetarians. Sporadic CJD is the most common type of human prion disease.
• Sporadic prion disease may be introduced into a human through infected surgical instruments or transplant tissues.
• Infectious prion disease such as variant CJD (vCJD or nvCJD) is likely caused by eating BSE-infected meat from cattle. No cases of vCJD in humans have been reported in the U.S. (one Florida case occurred in a woman who had lived in England).
• A few seemingly sporadic cases in young men in Michigan may point to transmission to hunters from deer or elk with chronic wasting disease (CWD), a form of animal prion disease. CWD is becoming common in this animal population in the U.S. Because hunters eat the meat of these animals, there is a potential for the prion disease to cross from hunted to hunter.

One additional form of human prion disease is called kuru. It was found among native peoples of New Guinea who practiced ritualistic cannibalism (eating of humans, and their brains, specifically). Probably the illness began when a person with sporadic CJD was eaten. Death from kuru takes about one year following the onset of symptoms; however, the average incubation period was about 12 years and can be as long as 40 years. With the end of such rituals, this disease has nearly disappeared in New Guinea.

The disease has been experimentally transmitted among cattle and from cattle to monkeys who eat infected tissue (particularly brain tissue) in lab tests.

But the question remains: How do cattle develop BSE? Feed is the major route for transmission among cattle, according to veterinary medicine experts. When ranchers and farmers feed cattle with products made from other cattle or sheep, such as ruminant feed, they are recycling diseased animal protein in feed containing meat and bone meal, thus causing the disease in cattle. This practice has since been outlawed after the association of BSE with vCJD.

Prions may be transmitted among laboratory animals also through broken skin, suggesting the possibility of similar transmission to humans who contact infected tissue or products and who have broken skin.

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