AANS Neurosurgeon | Volume 27, Number 1, 2018

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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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Calendar/Courses

Second International Brain Mapping Course
April 26-27, 2018; New Orleans

Surgical Approaches to Skull Base
April 26-28, 2018; St. Louis, MO

2018 AANS Annual Scientific Meeting
April 28-May 2, 2018; New Orleans

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