Rare Brain Disease May Have Killed Patient in New Hampshire; 13 Others at Risk

One patient at Catholic Medical Center in Manchester, N.H. may have died of a rare brain disease, says the Washington Post.

The patient, who had undergone a brain surgery in May, died in August. According to the hospital's president, Dr. Joseph Pepe, officials of the medical facility are almost certain that the patient died of sporadic Creutzfeldt-Jakob Disease. However, officials are still waiting for results from the brain biopsy or autopsy of the patient for confirmation.

According to the Centers for Disease Prevention and Control, Creutzfeldt-Jakob Disease is a human prion disease. Prions are defined by News Medical as infectious agents primarily composed of protein. Creutzfeldt-Jakob Disease occurs sporadically or without pattern and is caused by the spontaneous transformation of normal prion proteins into abnormal prions.

One of the problems with these abnormal prions is their ability to withstand standard sterilization practices in hospitals. This means that medical instruments and equipment used for brain surgery in one patient who has developed the disease have the possibility of being contaminated.

Although the rate of transmission of the abnormal prions through surgical instruments is typically low, there are still recorded cases of such. In fact, State Public Health Director at the New Hampshire Department of Health and Human Service. Jose Montero told the Washington Post that there have been 4 cases of transmission via surgical instruments, though not in the U.S.

Aside from transmission via surgical instruments and equipment, Creutzfeldt-Jakob Disease may also be passed on from generation to generation in families through genetic mutation. The disease can also be transmitted via exposure to brain or nervous system tissue.

The surgical instruments used for the deceased were reportedly rented. They were also used for brain surgeries elsewhere after the deceased's brain surgery in May.

Medical officials of the Catholic Medical Center have already informed 8 other patients in their facility of the perceived risk. Five additional patients in other states are also potentially exposed and have been notified.

"The risk to these individuals is considered extremely low, 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," says Montero in a report by USA Today.

The surgical instruments have been quarantined.