Nodaway County, MO Health Officials Explain "Mad Cow"
The Nodaway County Health Center, in northwest Missouri, has provided some excellent information on the nature and perceived sources of "mad cow" disease in humans, as reported in Nodaway News Leader. Apparently, there is public concern over an unconfirmed report of a local death attributable to Creutzfeldt-Jakob disease, CJD.
I found these paragraphs very helpful. First, the basic nature of the nearly always fatal CJD is explained.
CJD is believed to be caused by an abnormal form of a naturally-occurring protein that results in destructive changes to the brain. This abnormal protein is called a prion. Prion diseases have been recognized in various species of animals for many years.
Next the different types of CJD, and their respective causes are delineated.
• CJD occurs in several forms. Sporadic CJD, sCJD, is the most common, accounting for about 80 percent of the cases. It occurs mainly in persons 55-75 years of age, but can occur in younger and older individuals as well. This disease is rapidly progressive, with death occurring an average of seven months after symptoms first begin. It
• Genetic CJD is another form, accounting for 10-15 percent of the cases. This disease may be caused when persons inherit genes that make them more susceptible to development of prions in the brain.
• A third form is hospital-acquired CJD. A small number of people have apparently contracted this disease when they received materials such as pituitary hormone, brain tissues or corneal grafts from an infected person, or when surgical instruments used on an infected person were then used on an uninfected person.
• A final and more recently recognized form of CJD is variant CJD, vCJD. Variant CJD was first recognized in 1996, and has been linked to bovine spongiform encephalopathy, BSE. Human cases are thought to have been caused by consumption of BSE-contaminated meat or other tissues, primarily in Great Britain during the 1980s. This disease tends to affect younger persons than sCJD; the average age at death for vCJD is 29 years. The course of vCJD is longer than that of sCJD, with an average survival time of 14 months.
Finally, the health center notes that to date, the apparent threat of CJD in humans in the U.S. is very small. Thanks to Nodaway County health officials for some very informative writing.
The family of the United Kingdom's Andrew 'Rew' Hawker are confident that when test results are in, they will show he died from variant CJD---linked to eating BSE infected meat.