Encephalopathic Diseases One of the great biomedical quests of the last 20 years surrounds a handful of rare and mysterious brain diseases that affect humans and a small group of other mammals. An ongoing international research effort is aimed at understanding the mechanism of these neurodegenerative diseases. Recently, this area has been of particular relevance because one of the disorders-mad cow disease-has become a major, well-publicized health problem in Europe.

Four encephalopathic diseases occur in people: Creutzfeldt-Jakob disease (CJD), Gerstmann- Straussler- Scheinker disease (GSS), fatal familial insomnia, and kuru, an ailment once associated with ritualistic cannibalism among Fore highlanders of Papua New Guinea. Related animal diseases include sheep scrapie and bovine spongiform encephalopathy (BSE, or mad cow disease). Other encephalopathic disorders are also found in domestic cats, mink, antelope, and mule deer.

The fatal diseases cause the brain to become pitted, taking on a distinctive spongiform, or Swiss-cheese-like, appearance. Over...

Interested in reading more?

Become a Member of

Receive full access to digital editions of The Scientist, as well as TS Digest, feature stories, more than 35 years of archives, and much more!
Already a member?