Creutzfeldt-Jakob Disease Defined

Creutzfeldt-Jakob Disease (CJD) is a rare, fatal brain disorder caused by a transmissible infectious organism, probably a virus. Early symptoms of CJD include failing memory, changes in behavior, and a lack of coordination. As the disease progresses, usually very rapidly, mental deterioration becomes pronounced, involuntary movements (especially muscle jerks) appear, and the patient may become blind, develop weakness in the arms or legs, and ultimately lapse into a coma. The death of CJD patients is usually caused by infections in the bedridden, unconscious patient. Like Alzheimer's disease, a definitive diagnosis of CJD can be obtained only through an examination of brain tissue, usually at autopsy.

"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" Richard T. Johnson, M.D., Special Advisor to the National Institute of Neurological Disorders and Stroke.

There are three types of CJD:
By Creutzfeldt-Jakob Disease Foundation

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 found on the website. 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.