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What is Your Risk of Getting Creutzfeldt-Jakob Disease?


An elderly New Hampshire person died last month after having brain surgery in May and a spinal-fluid test led health authorities to suspect he may have been affected with Creutzfeldt-Jakob Disease (CJD), which is a rare and fatal brain disorder. The disease can only be confirmed through autopsy, and those results could take about four weeks to obtain.

The concern is that between three hospitals, the instruments were used in 15 other cases. The instruments were sterilized between use, but typical sterilization techniques aren’t guaranteed to wipe out CJD. The instruments used included a guided imaging navigation system manufactured by Medtronic, a metal reference frame and brace used during the procedure, and two other metal tools. The equipment in question is currently under quarantine.

Dr. Jose Montero, director of public health at the New Hampshire Department of Health and Human Services, says that the risk of infection is very remote. CJD cannot be transmitted through the air or through touching or most other forms of casual contact. Transmission of the disease from a transfusion of blood and related products has never been shown to occur in humans.

Exposure to brain tissue and spinal cord fluid from infected individuals would be the only way to become affected. Examples would include receiving an infected graft of dura mater (brain tissue), transplanted corneas, or implantation of inadequately sterilized electrodes in the brain.

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There are no known US cases where CJD has spread through neurosurgery instruments, says the Centers for Disease Control and Prevention. It has happened four times overseas, but not since 1976.

Creutzfeldt-Jakob disease afflicts only about one of every million people each year – only about 200 cases annually in the US. It is similar to “mad cow” disease, but not linked to beef consumption. It occurs when normal cellular proteins known as prion proteins turn into abnormal, misfolded versions.

The patient in question is thought to have a sporadic form of CJD which occurs spontaneously without known cause, as is the case with about 85% of patients. There is no known treatment or cure. Symptoms include failing memory, personality changes, blindness and sudden jerky movements.

Despite the risk to the other surgical patients is considered “extremely low”, the hospitals plus public health officials are taking the utmost of precautions. If any patient did in fact contract the disease, though, it could take years before they know. The incubation period for CJD can be as long as 50 years.

References: Wall Street Journal, Reuters, NBC News, National Institutes of Neurological Disorders and Stroke.