You are viewing AANS Neurosurgeon Volume 25, Number 4, 2016. View our current issue, Volume 26, Number 4, 2017

AANS Neurosurgeon | Volume 25, Number 4, 2016

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Researchers Find Infectious Prions in Creutzfeldt-Jakob Disease Patient Skin

Creutzfeldt-Jakob disease (CJD)—the human equivalent of mad cow disease—is caused by rogue, misfolded protein aggregates termed prions, which are infectious and cause fatal damages in the patient’s brain. CJD patients develop signature microscopic sponge-like holes in their brains.  The initial signs of CJD include memory loss, behavior changes, movement disorder, and vision problems, which usually rapidly progress to death. According to the National Institutes of Health (NIH), 90 percent of CJD patients die within one year of onset, and hundreds of Americans are diagnosed annually. There is no available treatment or cure.

There are numerous types of prion diseases in humans, and CJD is the most common.  About 90 percent of CJD cases have a sporadic origin. Prion infectivity is highly concentrated in CJD patient brain tissue.  Inter-personal CJD transmission has occurred after patients were exposed to surgical tools previously contaminated by CJD brain tissues.

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