Jennifer Fisher Wilson
Fisher Wilson J.; Why Prion Diseases Are a Mystery, and Why They Matter. Ann Intern Med. 2005;143:773-776. doi: 10.7326/0003-4819-143-10-200511150-00037
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Published: Ann Intern Med. 2005;143(10):773-776.
Prion diseases are rare in humans. Only about 300 Americans per year develop these fatal brain-wasting diseases, such as sporadic Creutzfeldt-Jakob Disease (CJD), Gerstmann-Sträussler-Scheinker syndrome, or fatal insomnia. New variant CJD, from consumption of beef infected with bovine spongiform encephalopathy, is even more rare—only about 150 people worldwide have been infected so far, and the number of new cases is waning. Prion diseases that infect animals other than cows—scrapie in goats and sheep and chronic wasting disease in elk and deer—do not appear to infect humans.
However, just because these diseases are rare in humans does not make them irrelevant. Indeed, the prion-only hypothesis of infection, based on the idea that misfolded proteins themselves cause infection, has already forced a fundamental change in the basic understanding of infectious diseases. Now, prion researchers hope that their findings will clarify the mechanisms behind protein-based infections. The work opens the possibility of effective prevention and treatment strategies to combat not only prion diseases but also the many other neurodegenerative diseases caused by protein misfolding, like Alzheimer disease and Parkinson disease. Learning how to treat neurodegenerative diseases would be a remarkable advance as these diseases become more prevalent in our aging society.
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Infectious Disease, Neurology, CNS Infections, Encephalopathy.
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Print ISSN: 0003-4819 | Online ISSN: 1539-3704
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