Palaces For The People
Saturday, January 03, 2004
 
NINDS Transmissible Spongiform Encephalopathies Information Page

NINDS Transmissible Spongiform Encephalopathies Information Page
Synonym(s): Prion Diseases
Reviewed 12-10-01

Get Web page suited for printing
Email this to a friend or colleague

Table of Contents (click to jump to sections)
What are Transmissible Spongiform Encephalopathies?
Is there any treatment?
What is the prognosis?
What research is being done?

Organizations
Related NINDS Publications and Information

What are Transmissible Spongiform Encephalopathies?
Transmissible spongiform encephalopathies (TSEs), also known as prion diseases, are a group of rare degenerative brain disorders characterized by tiny holes that give the brain a "spongy" appearance. These holes can be seen when brain tissue is viewed under a microscope.

Creutzfeldt-Jakob disease (CJD) is the most well-known of the human TSEs. It is a rare type of dementia that affects about one in every one million people each year. Other human TSEs include kuru, fatal familial insomnia (FFI), and Gerstmann-Straussler-Scheinker disease (GSS). Kuru was identified in people of an isolated tribe in Papua New Guinea and has now almost disappeared. FFI and GSS are extremely rare hereditary diseases, found in just a few families around the world. A new type of CJD, called variant CJD (vCJD), was first described in 1996 and has been found in Great Britain and several other European countries. The initial symptoms of vCJD are different from those of classic CJD and the disorder typically occurs in younger patients. Research suggests that vCJD may have resulted from human consumption of beef from cattle with a TSE disease called bovine spongiform encephalopathy (BSE), also known as "mad cow disease." Other TSEs found in animals include scrapie, which affects sheep and goats; chronic wasting disease, which affects elk and deer; and transmissible mink encephalopathy. In a few rare cases, TSEs have occurred in other mammals such as zoo animals. These cases are probably caused by contaminated feed. CJD and other TSEs also can be transmitted experimentally to mice and other animals in the laboratory. ...

Powered by Blogger