Rare brain disease concerns in US after death

by Holly Ramer

Public health officials believe one person in the U.S. has died of a rare, degenerative brain disease, and they 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 New Hampshire, said officials are 95 percent certain that a patient who had 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 .

Nearly 90 percent of cases of Creutzfeldt-Jakob disease happen spontaneously, when an agent causes to fold incorrectly. 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.

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, state public health director Dr. Jose Montero said. He would not identify the other states but said no more than five additional patients were potentially exposed.

Pepe said the potentially exposed patients 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 fewer than 1 percent of the cases, the disease is transmitted by exposure to brain or nervous system tissue, Montero said. Only four cases of transmission via surgical instruments have been recorded, none in the United States, he said.

not rated yet
add to favorites email to friend print save as pdf

Related Stories

Surgical gear quarantined over rare brain disease

Sep 04, 2013

New Hampshire public health officials believe one person died of a rare, degenerative brain disease, and there's a remote chance up to 13 others in multiple states were exposed to it through surgical equipment.

Recommended for you

WHO revises Ebola toll

1 hour ago

The World Health Organization Friday revised its figures showing more people killed by the deadly Ebola virus, but the number of cases of the disease was slightly lower.

Routines most vital in avoiding Ebola infection: WHO

18 hours ago

Meticulously following stringent routines when putting on and removing protective equipment is more important than the kind of gear health care workers use to ward off Ebola infection, the World Health Organization said Friday.

A look at latest Ebola developments

19 hours ago

No African countries are on the United Nations list of contributors to fight Ebola. With few exceptions, African governments and institutions are offering only marginal support as the continent faces its ...

Liberia opens one of largest Ebola treatment centers

20 hours ago

Remembering those who have died in the world's deadliest Ebola outbreak, Liberia's president opened one of the country's largest Ebola treatment centers in Monrovia on Friday amid hopes that the disease is ...

User comments

Please sign in to add a comment. Registration is free, and takes less than a minute. Read more

Click here to reset your password.
Sign in to get notified via email when new comments are made.