Friday, February 14, 2014

Exposure to Creutzfeldt-Jakob Disease; A Deadly Neurodegenerative Disorder in North Carolina

Novant Health Forsyth Medical Center has released a statement, stating they are reaching out to 18 neurosurgery patients exposed to Creutzfeldt-Jakob disease (CJD), which has occurred over the last three weeks.

CJD is a rare, neurodegenerative, fatal disease that affects roughly one person per million, per year, worldwide. 

Credit - Centers for Disease Control
The patient from which the CJD originated had sporadic CJD, which is different that mad cow disease (variant CJD). The CDC has a helpful table that distinguishes between the two here. Briefly, the differences are;
  • Median age of death (CJD=68, vCJD=28)
  • Median duration of illness (CJD=4-5 months, vCJD=14-15 months)
  • Presence of agent in lymphoid tissue (CJD=not detectable, vCJD=easily detectable)
On 18 Jan 2014, an operation was performed on a patient with symptoms that could have been attributed to CJD. Extra precautions should have been taken, but apparently they were not. Forsyth Medical has said the exposure occurred through surgical instruments that were not properly sterilized. 

For instruments that may have come into contact with prion material, standard sterilization is not enough, and enhanced methods are necessary. It is often suggested that the instruments simply be destroyed after use.
It is unlikely that the patients exposed will develop the disease, however if it does appear it likely won't happen for 20 to 30 years.
According to the CDC, no cases of iatrogenic (hospital-acquired) transmission of CJD have been reported since 1976, when current sterilization procedures were adopted. 

CJD onset is usually later in life and runs a rapid course. As mentioned above, median age of onset is mid-60s, and about 90 percent of individuals die within one year. There is no therapy for CJD.

Lance D. Presser has a PhD in Microbiology and Immunology and currently is a Public Health Laboratorian.

Hire Lance as a consultant using Zintro.

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