CJD FACT SHEET

What is CJD?
Creutzfeldt-Jakob Disease (CJD) is a rare, fatal brain disorder. The statistical incidence of CJD cases is one case, per million, per population. In the United States, this translates to approximately 250-300 new cases per year.  Eighty-five percent of the cases are sporadic, meaning there is no known cause at present.
 
"In the early stages of the disease, CJD patients may exhibit failing memory, behavior changes, impaired coordination and/or visual disturbances. As the illness progresses, mental deterioration becomes more pronounced, and involuntary movements, blindness, weakness of extremities, and, ultimately, coma may occur. sCJD usually occurs later in life, and typically leads to death within a few weeks or months to one year following the onset of symptoms - in the United States, the mean age of death from sCJD is 67 years." [1]
 
There are three types of CJD:
  • Sporadic (sCJD)
  • Familial (fCJD) - Genetic
  • Acquired:
    •  Iatrogenic Contaminated Surgical Instruments 
    •  Contaminated Dura mater transplant 
    •  Contaminated Corneal transplant 
    •  Contaminated Human Growth Hormone 
    •  Contaminated beef (vCJD)
The sporadic form (meaning from unknown cause) is the prevalent form in the US-85% of cases. More information can be found in the CJD pamphlet. As of August 2005 there are no known cases of endemic vCJD (ingestion of contaminated beef) in the U.S.
 
Diagnosis of CJD is very difficult and often happens through a process of elimination of other diseases. The diagnosis of CJD can only be confirmed through a brain biopsy or autopsy. Cerebral spinal fluid testing positive for the 14-3-3 protein is often used to confirm a possible diagnosis.  This test, however, can be inconclusive.
 
[1] (Richard T. Johnson, M.D., Special Advisor to the National Institute of Neurological Disorders and Stroke, excerpt from testimony to the Senate Commerce Subcommittee on Consumer Affairs, Foreign Commerce and Tourism, April 4, 2001.)