Eight N.H. Patients are Being Monitored for Rare Fatal Brain Disease

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Creutzfeldt-Jakob Disease
New Hampshire officials released a statement last Wednesday saying they are closely monitoring eight patients who may have been exposed to a deadly brain disease. Surgical equipment used on these eight patients was found to have been contaminated by proteins that cause Creutzfeldt-Jakob disease (CJD) from an earlier procedure.

The instruments had been sterilized using traditional methods for each subsequent procedure, but this method is not effective against the prion proteins that cause CJD. These infectious prions are formed when normal prion proteins become misshapen. These misshapen prions then begin catalyzing the refolding of other prion proteins, eventually causing a lethal encephalopathy known for its sponge-like appearance.

The initial patient suspected to be infected with CJD underwent surgery at Catholic Medical Center in Manchester, N.H., in May and died in August. CJD can only be confirmed in autopsy, which MedPage Today reports is currently underway. Some reports estimate up to 13 could be at risk for infection.

“Our concern is with the health and well-being of the eight patients who may have been exposed to CJD,” Dr. Joseph Pepe, the hospital’s CEO, said in a statement. “We will work closely with these families to help them in any way possible, even though the risk of infection is extremely low.”

The doctors believe the chance of infection to these eight patients is low, as they all underwent spinal cord surgery. The chance of transmission would be higher had they undergone brain surgery.

CJD is well known after several people developed a form of the disease called variant Creutzfeldt-Jakob disease (vCJD), more known by it’s common name “mad cow” disease which is transmitted by ingesting meat from cattle with these misshapen prions. However, the “classic” form of CJD which is suspected in New Hampshire is not transmitted this way.

Most cases of CJD are developed sporadically, or with no apparent reason. It can then be transmitted genetically, as 5 to 10 percent of people with CJD have a family history of the disease. It can also be transmitted, such as in this case, by contamination. Patients can develop CJD after being exposed to infected tissue or contaminated equipment during a medical procedure.

The common symptoms of CJD include:

  • Personality changes
  • Anxiety
  • Depression
  • Memory Loss
  • Impaired Thinking
  • Blurred Vision
  • Insomnia
  • Difficulty Speaking
  • Difficulty Swallowing
  • Sudden Jerky Movements

As the disease progresses, symptoms continuously worsen until most patients fall into a coma. Following coma, heart failure, respiratory failure, or infections such as pneumonia usually cause death. In most cases, the disease runs its course over a period of seven months.

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