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Creutzfeldt-Jakob disease (CJD)



General Information


Image of a human brain scan from a CJD patient.

Creutzfeldt-Jakob disease (CJD) is a rare, fatal brain disorder believed to be caused by a protein called a prion. A prion is a nonliving, self-replicating infectious agent made of protein. It is thought to arise from a change in shape of a prion protein. This abnormally-folded protein is then thought to cause other prion proteins to change shape. This process leads to the destruction of brain cells.

CJD is one of a family of related diseases found in many animal species, called Transmissible Spongiform Encephalopathies (TSEs). These include scrapie in sheep and Bovine Spongiform Encephalopathy (BSE) in cattle. BSE is also known as ‘Mad Cow disease.’

CJD is not related to Mad Cow disease.


CJD, the disease in humans, can take one of four forms including sporadic (sCJD), familial or inherited, acquired or iatrogenic and variant (vCJD).

  • Sporadic CJD (sCJD) is the most common of the human prion diseases and accounts for approximately 85% of all cases. The cause of sCJD is unknown. It occurs worldwide at a rate of approximately one case per 1 million population per year, although rates of up to two cases per million are not unusual. Most sporadic cases occur in people over 50 years of age and death usually occurs within months.
  • Familial or Inherited CJD is the second most common form of CJD and accounts for approximately 10-15% of cases worldwide. People usually develop familial CJD at a younger age than sCJD and the course of illness is longer.
  • Acquired or Iatrogenic CJD accounts for less than 1% of all cases. This form of CJD is caused by contamination with tissue from an infected person. While rare, human-to-human transmission of CJD can occur as the result of tissue implant, use of contaminated neurosurgical instruments, or administration of human hormones extracted from the organs of human cadavers. Today, no organs are procured from people known to be infected with CJD. Additionally, surgical instruments used on people with CJD undergo stringent chemical and autoclave sterilization methods or are never used again.
  • Variant (vCJD) is another form of acquired CJD. This form was first reported in 1996 in the United Kingdom. It is thought to be transmitted through the consumption of beef from cattle infected with BSE or Mad Cow disease. There have been no known cases of vCJD that originated in the United States.

Basic
CJD Information


CJD FAQ Sheet


[ CJD FAQ Sheet -
Text Version
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Centers for Disease Control
and Prevention (CDC)
About CJD
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CDC - Prion Diseases
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CJD is confirmed only through brain biopsy or brain autopsy.

Family Support

CJD Foundation

Support is a key ingredient in coping with CJD. Support from those who have had similar experiences can be more helpful than any written information. The primary goal of the CJD Foundation is to provide support for CJD patients and their families.

  • CJD Foundation Toll Free Helpline: 1-800-659-1991

CJD Insight, Information & Support

Provide support to families afflicted with familial or inherited CJD.

CJD Infection Control Guidelines - Information for the Healthcare Industry, Surgical Considerations & Funeral Home / Crematory Practitioners

World Health Organization (WHO) Guidelines for Transmissible Spongiform Encephalopathies (TSE), March 1999

National Prion Disease Pathology Surveillance Center (NPDPSC)

The NPDPSC was established in 1997 at the Division of Neuropathology of Case Western Reserve University and is sponsored by the American Association of Neuropathologists (AANP). The NPDPSC monitors the occurrence of prion disease through characterization of brain tissue pathology from clinically diagnosed cases of CJD. A confirmatory diagnosis of CJD can only be made through brain biopsy or brain autopsy. Autopsy provides the only method to fully classify the type of CJD (sporadic, familial, iatrogenic, or variant). The NPDPSC can help establish the diagnosis of prion disease by analyzing cerebrospinal fluid (CSF), blood, and brain tissue obtained either at biopsy or autopsy.

For questions regarding services offered by the NPDPSC or assistance in arranging an autopsy, please call the Center at (216-368-0587).

Blood Bank of Delmarva (BBD)

Although there have been no documented cases of a patient developing CJD from a transfusion of blood or plasma, all potential donors are screened to determine if they fall into a CJD risk group that has been determined by the Food and Drug Administration (FDA).

BBD defers all potential donors who may be at risk for CJD according to the following criteria:

  1. People who have received human pituitary-derived growth hormone.
  2. People who have received a dura mater transplant.
  3. People told their families are at risk for CJD.
  4. People with one or more blood relatives with CJD.

NOTE: A person who has only one family member with CJD may be at increased risk for developing the disease. However, he or she is much less likely to develop CJD than a person who has two or more family members diagnosed with CJD.

For more information, contact Blood Bank of Delmarva Donor Advocate at (302)737-8405 (or 1-800-533-6957).

Reporting and Surveillance

CJD became a reportable condition in Delaware in 2006: Regulations for the Control of Communicable and Other Disease Conditions

Purpose of Reporting and Surveillance:

  • To monitor trends in the epidemiology of human prion diseases.
  • To maximize laboratory confirmation of suspected cases in collaboration with the National Prion Disease Pathology Surveillance Center.
  • To detect the emergence of vCJD or novel prion diseases in the United States.
  • To prevent potential iatrogenic (acquired) transmission.

Healthcare providers should contact the Delaware Division of Public Health (DPH), Bureau of Epidemiology to report any suspect case of CJD.

Additional Resources:


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