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September 04. 2013 8:35PM

UPDATED: CMC surgery patient with rare brain disease may have exposed 8 others

MANCHESTER — Eight people who underwent brain surgery between May and August at Catholic Medical Center are waiting to learn if they could be under a death sentence.

At a news conference Wednesday at CMC, health officials said testing is underway to determine if a man who had brain surgery in May and died in August had an extremely rare and fatal ailment called Creutzfeldt-Jakob Disease.

Dr. Joseph Pepe, CEO of CMC, said the eight patients on whom the same neurosurgery instruments were used have been identified and notified of the possibility of transmission.

"The risk is close to zero, but it's not zero," he said. "Patients have the right to know."

New Hampshire Director of Public Health Jose Montero said the neuroprobe used in the CMC surgery was rented from the medical technology firm Medtronic, and was also used in other states. He would not identify the other states, but said five additional patients have been identified as being at risk if Creutzfeldt-Jakob Disease is confirmed in the original patient at CMC.

Pepe said the hospital has stopped brain surgeries; it usually does about a dozen a year.

Only about one percent of cases result from transmission through material called prions on instruments used in certain brain surgeries.

Pepe and Montero said it is possible to sterilize the instruments to eliminate prions, but that would render the equipment unusable. They said all the instruments used in the brain surgery have been taken out of service and, if the testing shows the original patient had CJD, they will be destroyed.

"You need a brain biopsy to confirm the diagnosis," Montero said, noting results are not expected for four to six weeks.

There is no treatment for the illness; death follows quickly once symptoms appear, which can be a year or much longer after infection. The disease results in rapid mental deterioration. Health officials stressed that although symptoms are similar, Creutzfeldt-Jakob Disease is not the so-called mad cow disease, which can be contracted through consumption of contaminated meat.

Pepe said there is no danger to anyone else at the hospital or to the friends or relatives of the patients.

"It is not a virus," he said. "It is a prion."

The word prion is derived from protein and infection. The infectious protein causes brain tissue to develop holes and take on a sponge-like texture.

Creutzfeldt-Jakob Disease occurs about once in a million people and there are approximately 200 cases a year in the United States. Eighty-five percent of cases are hereditary; most of the rest are spontaneous.

Montero said the patient had no family history of CJD and had not had any other brain surgery during which the disease could have been transmitted, so it would be a spontaneous case — one that could only be spread by contact with the diseased prions during surgery with contaminated instruments.

Pepe said the original patient had signs of brain cysts when he underwent surgery in May and there was no sign of CJD at the time. A few months later, based on the man's rapid deterioration, CJD "was strongly suspected," he said.

The patient was readmitted in August, said Pepe. He and Montero said the swift deterioration of the patient's mental abilities, as well as spinal fluid, MRIs and other preliminary tests, pointed to CJD, but they had never seen the disease before.

On the same day initial test results were received, the patient died, said Pepe.

Manchester Health Director Tim Soucy praised both CMC and the state for their handling of the situation.

"This has been a complex and sensitive case," he said.

dvincent@unionleader.com
Previous story follows:
MANCHESTER – Health officials are holding a news conference this afternoon to discuss a case of sporadic Creutzfeldt-Jakob Disease.
A spokesman for CMC, Michelle Marshall, director of marketing and communications, said, "It is not the same as mad cow disease. It is a variant. It is complicated. We will go over the details at 3 o'clock."
In animals, mad cow disease is known as bovine spongiform encephalopathy (BSE). If a person eats BSE-infected beef, the person is at a higher risk for getting the human form of the disease, called Creutzfeldt-Jakob disease, or CJD.

The N.H. Department of Health and Human Services (DHHS), Division of Public Health Services (DPHS) in collaboration with Catholic Medical Center (CMC) and the Manchester Health Department are investigating the case.

The "scope and breadth of the investigation" will be discussed at the news conference with Dr. Jose Montero, DHHS' director of public health, along with Dr. Joseph Pepe, CMC's CEO, and Tim Soucy, Manchester public health director, according to the news release.

Health officials did not say in the release the location of the case they are investigating or any of the specific circumstances. At this afternoon's conference, they are expected to provide more information about the investigation and answer questions.

According to the Mayo Clinic, Creutzfeldt-Jakob Disease is a degenerative brain disorder that leads to dementia and, ultimately, death.

Symptoms of Creutzfeldt-Jakob disease (CJD) sometimes resemble those of other dementia-like brain disorders, such as Alzheimer's, but Creutzfeldt-Jakob disease usually progresses much more rapidly.

Creutzfeldt-Jakob disease captured public attention in the 1990s when some people in the United Kingdom developed a form of the disease — variant CJD (vCJD) — after eating meat from diseased cattle. However, "classic" Creutzfeldt-Jakob disease has not been linked to contaminated beef.

Although serious, CJD is rare, and vCJD is the least common form. Worldwide, there is an estimated one case of Creutzfeldt-Jakob Disease diagnosed per million people each year, most commonly in older adults.


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