It’s not 'mad cow,' but the risk of sporadic CJD to patients cannot be dismissed

January 12th, 2015

How would you like to be told by your local hospital after a recent surgery that you have been potentially exposed to a disease of the brain that is rapidly progressive and always fatal?

The exceedingly rare but legitimately real risk of transmission of Creutzfeldt-Jakob Disease (CJD) has changed the lives of eight surgical patients in Concord, NH. They have been informed they were exposed to surgical instruments used on a Catholic Medical Center (CMC) patient who is suspected to have had CJD, public officials report.

The only way to 100% diagnose CJD is through autopsy, which is currently under way at the National Prion Disease Pathology Surveillance Center. The eight patients were potentially exposed through neurosurgical equipment, because the prion that causes CJD may not be eradicated by the standard sterilization processes used at hospitals. In addition to iatrogenic transmission of CJD -- linked to the use of contaminated human growth hormone, dura mater and corneal grafts – there are six documented cases traced to the use of contaminated equipment, according to the Centers for Disease Control and Prevention. Four were associated with neurosurgical instruments, and two with stereotactic EEG depth electrodes.

“All of these equipment-related cases occurred before the routine implementation of sterilization procedures currently used in health care facilities,” the CDC reports. “No such cases have been reported since 1976, and no iatrogenic CJD cases associated with exposure to the CJD agent from surfaces such as floors, walls, or countertops have been identified.”

The New Hampshire index patient is believed to have sporadic CJD, as opposed to variant CJD -- “mad cow” disease. Infection preventionists dealing with CJD often find that the legitimate medical issues can quickly become engulfed by a swirl of misinformation, media hype, and public panic. Indeed, there have already been unconfirmed reports that more patients may have been exposed. However, transmission risks cannot be dismissed outright because of the aforementioned cases where CJD has spread from patient-to-patient on surgical instruments.

“The risk to these individuals is considered extremely low," said José Montero, MD, a state public health official. “But after extensive expert discussion, we could not conclude that there was no risk, so we are taking the step of notifying the patients and providing them with as much information as we can. Our sympathies are with all of the patients and their families, as this may be a confusing and difficult situation."

CJD is classified as a transmissible spongiform encephalopathy, a group that includes other prion diseases in humans and animals. CJD has an incidence of about one in a million people each year worldwide. About 200 people are diagnosed with CJD each year, the CDC reports.