Creutzfeldt-Jakob Disease

Creutzfeldt-Jakob Disease is a reportable disease in Oklahoma. Creutzfeldt-Jakob disease (CJD) is a rare, degenerative, fatal brain disorder. CJD is believed to be caused by a rare type of self-replicating protein called a prion, either through gene mutation or external ingestion of the protein. It affects about one person in every one million people worldwide and about 200 people in the United States annually. CJD usually appears later in life and runs a rapid course. Typically, onset of symptoms occurs about age 60, and about 90% of patients die within one year. There are three major categories of CJD:

• In sporadic CJD, the disease appears even though the person has no known risk factors for the disease. This is by far the most common type of CJD and accounts for at least 85% of cases.
• In hereditary CJD, the person has a family history of the disease and/or tests positive for a genetic mutation associated with CJD. About five to ten percent of cases of CJD in the U.S. are hereditary.
• In acquired CJD, the disease is transmitted by exposure to brain or nervous system tissue, usually through certain medical procedures. There is no evidence that CJD is contagious through casual contact with a CJD patient.

The first symptoms of Creutzfeldt-Jakob disease typically include dementia, personality changes together with impaired memory, judgment, and problems with muscular coordination, behavior changes, and visual disturbances. As the illness progresses, persons quickly deteriorate mentally along with developing involuntary movements, blindness, and weakness of extremities. Individuals with CJD eventually lose the ability to move and speak and enter a coma. Pneumonia and other infections often occur in these patients and can lead to death. People with the disease also may experience insomnia, depression, or unusual sensations.

CJD does not cause a fever or other flu-like symptoms. Some symptoms of CJD can be similar to symptoms of other progressive neurological disorders, such as Alzheimer’s or Huntington’s disease. It also tends to cause more rapid deterioration of a person’s abilities than Alzheimer’s disease or most other types of dementia. The incubation period of CJD is usually very long ranging from 15 months to several decades.

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.

Creutzfeldt-Jakob Disease Fact Sheets and Information:

Creutzfeldt-Jakob Disease Fact Sheet (50k.pdf)

External Creutzfeldt-Jakob Disease Resources:

Creutzfeldt-Jakob Disease (CDC)