What is CJD?
Creutzfeldt-Jakob Disease (CJD) is a serious progressive degenerative disorder of the brain. It is caused by an abnormal form of a protein in the brain - known as the "prion protein" - that leads to loss of brain function or encephalopathy. The disease is extremely rare and occurs at a rate of one case per million in populations worldwide. CJD is categorized as a transmissible spongiform encephalopathy (TSE) along with other prion diseases that affect both humans and animals because direct inoculation of tissues with the abnormal prion protein can cause the disease.

Is CJD the same as "Mad Cow Disease?"
Although CJD is caused by protein (prion) transmission and is classified as a spongiform encephalopathy, CJD is a distinctly different disease. It is not associated with consuming meat products contaminated by feeding beef cattle and sheep bone meal from infected animals.

How do you get CJD?
In most cases, CJD occurs as a sporadic disease with no pattern of transmission or recognized way that the disease is spread. A smaller number of CJD cases have developed because of inherited changes or mutations in brain cell proteins that are passed in families. An even smaller number of CJD cases (less than one percent) have been due to iatrogenic (medical) interventions linked to the use of contaminated human growth hormone, corneal or dura mater (covering of the brain) grafts, and neurosurgical equipment.

What are its symptoms?
In most cases of CJD the symptoms occur long after the appearance of the abnormal protein - sometimes as long as 20-30 years - but occasionally sooner. Symptoms can include a variety of neurological abnormalities such as ataxia (unsteady gait or walking), confusion, dementia, and psychiatric and sensory perception problems. Once symptoms of the disease appear and a CJD diagnosis is considered medically likely, the symptoms worsen and the patient's death often occurs within one to two years.

Can patients be exposed to CJD through medical procedure or surgery in the hospital?
The unsuspected transmission of CJD through medical equipment or procedures has been reported in approximately 260 patients worldwide. The majority of cases (97%) were linked to contaminated human growth hormone, dura mater and corneal grafts from human cadavers. A small number of cases (five) were related to contaminated neurosurgery instruments and two were related to the implantation of contaminated electroencephalograph (EEG) electrodes. All equipment-related cases occurred outside of the United States before 1976 and before the introduction of more powerful, effective, high quality cleaning and sterilization procedures in hospitals. The likelihood of acquiring the disease in the hospital is now extremely low.

How are surgical instruments used on suspected and confirmed CJD patients sterilized?
Recommendations for the cleaning and sterilization of instruments used on suspected or confirmed CJD patients have been provided by the World Health Organization (WHO). The current recommendations on sterilization and disinfection came from studies that intentionally contaminated equipment with brain tissue or tissue homongenates from CJD patients. CJD prions are very resistant to routine sterilization procedures. The sterilization procedures include soaking in sodium hydroxide or sodium hypochlorite (bleach) and autoclaving. Recently an extended autoclaving cycle has been recommended as an alternative to soaking.

What are the chances of acquiring CJD from a surgical instrument that may have been exposed to a patient with the disease?
No one can answer this question with certainty. All surgical instruments are routinely thoroughly cleaned and autoclaved before being used on another patient. There is no way to accurately tell if any prions could be left on a surgical instrument after repeated cleaning and processing. The best answer is to say that exposure is possible but the likelihood of developing disease is extremely low. How is CJD diagnosed? Physicians may suspect CJD in patients with very abnormal neurological symptoms. A specific protein can be detected in the CSF fluid of advanced cases and an EEG (electroencephalogram) may portray a typical CJD pattern. A definite diagnosis can only be made through brain biopsy or autopsy. There is no way to determine if a person has been exposed to CJD or if they will get the disease even if they have been exposed.

What should be done to evaluate a patient who may have been exposed to CJD?
Currently there is no test available to determine if a patient may have been exposed to CJD in the hospital. The best advice is to inform your Primary Care Provider (PCP), neurologist and/or neurosurgeon that you may have been exposed to the disease and that the likelihood of infection is extremely low. Your PCP can note that in your medical history. No additional medical follow-up is required unless a neurological problem develops in the future.

Where can you find more information on CJD?
The Centers for Disease Control (CDC) has a website for CJD information at http://www.cdc.gov.

Who can I contact at UPMC Presbyterian or the Allegheny County Health Department to get more information on CJD?
You can contact the Department of Neurological Surgery at (412) 647-3685. UPMC Presbyterian representatives are also available to help with any patient questions at (412) 647 UPMC(8762) as are Allegheny County Health Department representatives at (412) 687-2243.