18 may have been exposed to incurable disease

Reblogged from CNN.com

(CNN) — Doctors and hospital officials from Novant Health Forsyth Medical Center in Winston-Salem, North Carolina, are notifying 18 neurosurgery patients that they might have been exposed to Creutzfeldt-Jakob disease, a serious and incurable neurological disorder.

“Today we are reaching out to 18 neurosurgery patients who were exposed to Creutzfeldt-Jakob disease over the last three weeks at Forsyth Medical Center,” said Jeff Lindsay, president of the center, according to CNN affiliate WGHP.

The hospital is in the process of contacting the 18 people, spokeswoman Jeanne Mayer said Tuesday. She was not sure how many had been reached.

According to the National Institute of Neurological Disorders and Stroke, CJD affects about one person in every 1 million people per year worldwide.
Gupta: Disease takes years to develop

“It is important to note that there are multiple variations of CJD and this case is not related to mad cow disease,” Novant Health said in a statement.

The hospital confirmed that on January 18, an operation was performed on a patient with CJD symptoms who later tested positive for the illness.

Even though the surgical instruments were sterilized by standard hospital procedures, they should have gone through enhanced sterilization procedures used when there are confirmed or suspected cases of CJD.

The original patient “had neurological symptoms that could have been attributed to CJD or another brain disease,” Novant Health said. “There were reasons to suspect that this patient might have had CJD. As such, the extra precautions should have been taken, but were not.”

The Centers for Disease Control and Prevention, as well as the World Health Organization, recommends that surgical equipment used on CJD patients be destroyed or decontaminated through an intense disinfecting process.

Although CJD can be transferred through surgical equipment, hospital officials say the likelihood of these patients contracting the disease is very low.

The CDC corroborates that assessment.

It says that no cases of the disease have been linked to the use of contaminated medical equipment since 1976.

But Lindsay made no excuses.

“On behalf of the entire team at Novant Health, I apologize to the patients and their families, for having caused this anxiety.”

CJD is a rare, degenerative and fatal brain disorder, according to the National Institutes of Health. It’s characterized by rapid, progressive dementia. Initial symptoms can include problems with muscular coordination, personality changes including impaired memory and thinking; and impaired vision.

CJD is believed to be caused by a type of protein called a prion. It can be sporadic, hereditary or acquired; the acquired type is the rarest form, according to the NIH, and seen in fewer than 1% of cases. It is not contagious through casual contact.

Asked whether the 18 people would be tested, Mayer said there is no quick test for CJD. The original patient underwent brain surgery and then the disease was found through a number of tests afterward, she said. In some cases, CJD can take years to show up, Mayer said.

The hospital has instituted the enhanced sterilization process on all surgical instruments used in brain surgery, Novant Health said.

In September, 13 patients received similar warnings from two hospitals in New Hampshire and Massachusetts, when a patient who had undergone neurosurgery was later suspected to have CJD.

The hospitals shared the specialized surgical equipment that was used to operate on the patient and continued to use it until the suspicion of exposure to the disease surfaced.

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Kuru (You get it from eating brains!)

Eating-human-brains-300x150

Posted by Dr Abhimanyu Kaura, PGYIII (Medicine), Monmouth Medical Center

Kuru disease is one of the five human spongioform encephalopathies caused by prions.  This group also includes Creutzfeldt-Jakob disease (CJD), variant CJD, Gerstmann-Straussler Scheinker syndrome and fatal familial insomnia.

Bovine spongiform encephalopathy also known as “mad cow disease”, is another prion disease that affects cattle, and was responsible for bringing more attention to all of these disorders in the 1990s.

Kuru is confined the Fore tribes of Papua, New Guinea.

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In the 1950s there were 2100 cases of Kuru in these tribes leading to about 1000 deaths per year.

As per the culture of the Fore tribe, if a person in a family dies his meat is eaten by his family members, especially the wife and children,  as a mark of respect to him.  90% of people affected by the disease were women and children.

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Between 1996 to 2004 only 11 new cases of Kuru were identified in the region. Currently with the ban of cannibalism in the Farah tribes the disease had become virtually non existent.

Kuru first presents with tremors, then unsteady gait and progresses to leg weakness, ataxia, incoherent speech, sporadic laughter finally.  In later stages, affected patients become demented, bed bound and unable to swallow.

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Death occurs most commonly from respiratory distress and pneumonia or infection of pressure sores. The disease is fatal within 1-2  years of the onset of symptoms and had no known cure.

Here’s a video introduction

Watch the full story here:

The causative organism is a prion, a mutated protein which replicates itself like a virus, and is spread by eating the neuronal tissue of infected people.

prions

The precise incubation period of the disease is unknown, but can be up to fifty years.

The pathological hallmark is the presence spongioform encephalopathy with of Prion reactive plaques mostly in the cerebellum:
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The detailed studies of theses cases of Kuru has helped us understand other prion diseases such as variant CJD, sporadic CJD whose clinical features and course is similar to this condition.

And maybe another link between Zombies and neurology?

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