New Hampshire patient likely died of rare brain disease

published Thursday, September 5th, 2013

by Associated Press
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CONCORD, N.H. — Public health officials believe one person in New Hampshire has died of a rare, degenerative brain disease, and say there's a remote chance up to 13 others in multiple states were exposed to the fatal illness through surgical equipment.
Dr. Joseph Pepe, president of Catholic Medical Center in Manchester, said officials are 95 percent certain that a patient who had brain surgery in May and died in August had sporadic Creutzfeldt-Jakob Disease.
The disease progresses rapidly once symptoms appear and is always fatal, usually within a few months. But the symptoms can take decades to show up. They include behavior changes, memory loss, impaired coordination and other neurological problems.
Nearly 90 percent of cases of Creutzfeldt-Jakob disease happen spontaneously, when an agent causes proteins in the brain to fold incorrectly. And because those abnormal proteins can survive standard sterilization practices, there is a small risk of exposure for those who had surgery after the patient who died, Pepe said.
"The risk of exposure is extremely low, but it's not zero," he said.
In fewer than 1 percent of the cases, the disease is transmitted by exposure to brain or nervous system tissue, state public health director Dr. Jose Montero said. Only four cases of transmission via surgical instruments have been recorded, none in the United States, he said. Another 10 to 15 percent of the cases of Creutzfeldt-Jakob involve a genetic mutation that is passed down among families.
The disease can be verified only through a brain biopsy or autopsy. New Hampshire officials are still awaiting those results.
Meanwhile, the hospital has notified eight of its patients who may have been exposed, and hospitals in other states are working to do the same because some of the surgical equipment was rented and used elsewhere after being used in Manchester, Montero said. He would not identify the other states but said no more than five additional patients were potentially exposed.
Catholic Medical Center also has assigned a staffer to work with the potentially exposed patients, whom Pepe said range in age from mid-30s to mid-80s.
"They took it very well. I don't believe that people were angry or extremely emotionally upset," he said. "We did the best job we could in trying to alleviate their fear."
Worldwide, Creutzfeldt-Jakob affects about one person in every one million each year; in the United States, about 200 cases are recorded annually, according to the National Institutes of Health.
In South Carolina, Greenville Hospital System last year began increasing the temperature used to sterilize its surgical instruments after a patient was discovered to have Creutzfeldt-Jakob. Eleven patients there were notified that they might have been exposed.
But Pepe said the measures required to eliminate all traces of the proteins would effectively render the equipment unusa