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THE FIGHT FOR MIKE
Creutzfeldt-Jakob Disease (CJD): Frequently Asked Questions

What is Creutzfeldt-Jakob Disease?
Creutzfeldt-Jakob Disease (CJD or Jakob-Creutzfeldt Disease) is a rapidly progressive, fatal brain disorder. It is part of a family of diseases, called transmissible spongiform encephalopathies, which are caused by an agent known as a “prion” (pree-on), a proteinaceous infectious particle. Prions in animals cause diseases such as bovine spongiform encephalopathy (BSE or mad cow disease) in cattle, scrapie in sheep and goats, and chronic wasting disease (CWD) in deer and elk. Human prion diseases include Creutzfeldt-Jakob disease (CJD), Fatal Familial Insomnia (FFI), Gerstmann-Sträussler-Scheinker (GSS) syndrome, Kuru, and New Variant CJD (nvCJD or vCJD).

Are there different forms of CJD?
Like all prion diseases of humans, CJD comes in sporadic (spontaneous), genetic (familial), and infectious forms. Eighty-five percent of prion disease cases are sporadic (sCJD), which is the type of case that we believe Mike has and occurs for reasons that are not yet known. Up to fifteen percent of cases are familial (fCJD, GSS, FFI), and less than one percent are acquired by infection.

How common is CJD?
Surveillance and epidemiologic studies report the annual incidence of human prion disease to be one per one million per year. In the United States this translates to 280-300 new cases per year. It is well known that CJD is very difficult to diagnose, leading to speculation that the one case per million report may be incorrect. CJD occurs worldwide, although cases have not been reported in countries in central Africa. There is no compelling evidence that correlates place of residence, occupation, diet, medical history, blood transfusion, or drug therapy as risk factors for CJD.

What are the symptoms of CJD?
At UCSF, we refer to CJD as the “great mimicker,” because its effect on many different areas of the brain causes symptoms that can occur in many neurological diseases. First symptoms vary widely from patient to patient and can include, but are not limited to, memory loss or difficulty with balance and walking, dizziness, behavioral change, visual disturbance, and involuntary movements. While sporadic CJD varies clinically, the rapid progression of multiple deficits that fail to improve is an indication that the underlying disease could be CJD. In the advanced stages of the disease, patients commonly develop significant difficulties in movement and become unable to talk and swallow.

Who is affected by CJD?
Peak incidence for sporadic CJD occurs from ages 65-69, although age can range significantly. CJD is especially rare in patients less than 30 years old, and there is a sharp drop in incidence over the age of 75 years. Males and females are affected equally, although some studies have indicated that there is a slightly higher incidence of CJD in females (52.9 percent).

What is the prognosis for those diagnosed with CJD?
The median survival time for sporadic CJD is 4.5 months, and 90 percent of patients die within one year of the first symptom. However, some cases are very rapid, with patients surviving for only a few weeks, and others have a prolonged disease duration lasting for a few (two to three) years.

Can you “catch” CJD from someone?
Prion diseases are not infectious in the usual way. For example, they are not spread by airborne droplets like colds and flu, or by body fluids or sexual contact like HIV. The overall evidence suggests that there is no increased risk of developing CJD from contact with a person suffering from the condition. No special precautions are required by anyone coming into contact with someone with CJD. However, it is sensible for anyone who might be exposed to the blood of a CJD patient (usually medical staff) to wear gloves.

What is being done to protect us from CJD?
At present there is no way of protecting people from sporadic or familial CJD. The form of CJD acquired by infection is guarded against by destroying surgical instruments that have been used on people with CJD, and by not using their organs for transplant.

Is there a treatment for CJD?
There is no established treatment or cure currently approved for this disease. Researchers at UCSF have identified compounds that may be potential treatments for prion diseases. Please see http://prusinerlab.ucsf.edu for more information about UCSF’s research efforts in this area.

Sources: UCSF Memory and Aging Center, Creutzfeldt-Jakob Disease Foundation, Inc.