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Creutzfeldt-Jakob Disease

Creutzfeldt-Jakob disease (CJD) is a rapidly progressive, invariably fatal neurodegenerative disorder believed to be caused by an abnormal isoform of a cellular glycoprotein known as the prion protein. CJD occurs worldwide and the estimated annual incidence in many countries, including the United States, has been reported to be about one case per million population.

CJD occurs worldwide and the estimated annual incidence in many countries, including the United States, has been reported to be about one case per million population.

Creutzfeldt-Jakob Disease is a reportable disease in Oklahoma.

While CJD can be transmitted to other people, it cannot be transmitted through the air, through touching, or most other forms of casual contact. However, direct or indirect contact with brain tissue or spinal cord fluid from infected patients should be avoided to prevent transmission of the disease through these materials.

Normal sterilization procedures such as cooking, washing, and boiling do not destroy prions. The tissues and fluids considered infectious for CJD are corneas, brain and spinal cord tissue, and cerebrospinal fluid. For the routine daily care of a CJD patient, universal precautions are considered adequate. Caregivers, healthcare workers, and undertakers should take the following precautions when they are working with a person with CJD:

  • Wash hands and exposed skin before eating, drinking, or smoking.
  • Cover cuts and abrasions with waterproof dressings.
  • Wear surgical gloves when handling a patient’s tissues and fluids or dressing the patient’s wounds.
  • Avoid cutting or sticking themselves with instruments contaminated by the patient’s blood or other tissues.
  • Use face protection if there is a risk of splashing contaminated material such as blood or cerebrospinal fluid.
  • Soak instruments that have come in contact with the patient in undiluted chlorine bleach for an hour or more, then use an autoclave (pressure cooker) to sterilize them in distilled water for at least one hour at 132° - 134° Centigrade.

There is no treatment that can cure or control CJD. Current treatment for CJD is aimed at helping symptoms and making the patient as comfortable as possible.

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