Friday, March 17, 2006

"The cow's not mad. She's got a brain issue."

Mad Cow Disease Only Sounds Scary. Yeah, it's just a trifle:
Does BSE or a similar disease occur in humans? BSE belongs to a group of progressive degenerative neurological diseases known as transmissible spongiform encephalopathies (TSEs). TSE diseases are always fatal. The TSE diseases include scrapie, which affects sheep and goats; transmissible mink encephalopathy; feline (cat) spongiform encephalopathy; and chronic wasting disease of deer and elk. There are six TSE diseases that affect people: kuru, classical Creutzfeldt-Jakob disease (CJD) and variant Creutzfeldt-Jakob disease (vCJD), Gerstmann-Sträussler-Scheinker syndrome, fatal familial insomnia, and sporadic fatal insomnia. The human diseases are very rare; for example, classical CJD has been well studied and occurs sporadically worldwide at a rate of about one case per one million people each year. What is the new variant form of CJD that the experts in the United Kingdom believe might be related to the BSE outbreak in cattle? In contrast to the classic form of CJD, the new variant or variant form (vCJD) in the United Kingdom and France affects younger persons (average age at onset: 26 years), and has different clinical features from CJD. People with vCJD begin with serious psychiatric problems or problems with their senses (ears, eyes or smell). This first set of symptoms is followed weeks or months later by poor muscle coordination, muscle spasms, and mental confusion. The illness lasts for at least 6 months, and on average people with vCJD die approximately 13 months after their symptoms begin. When patients' brains are examined by autopsy, there are clear changes in brain tissue structure, including many "spongiform," or open spongy-looking areas, abnormal clumps of prion protein called plaques, and other areas with less prominent accumulations of abnormal prion protein.


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