Creutzfeldt-Jakob disease (CJD) is a human prion disease caused by a normal prion protein that becomes misshapen into an infectious prion, builds up in the brain and disrupts normal brain function. It is a rare disorder that is fatal, usually within 6 months of diagnosis and a year of the first symptom. In the United States, there are about 300 new cases per year.
Creutzfeldt-Jakob Disease (CJD)
-
-
There are three main forms of prion disease, categorized by how the disease occurs. Each form differs with regards to typical signs, symptoms and duration of illness. The three forms are sporadic, genetic and acquired.
-
At UCSF we call CJD the “Great Mimicker” because its symptoms can resemble those seen in other neurological diseases. The good news is that some people who think they have prion disease may not actually have it. Many other diseases, some treatable, appear similar to CJD. Your doctor’s first mission is to rule out these other treatable illnesses.
-
The signs and symptoms of CJD vary between patients because the prions build up in different areas of the brain in each person, which results in varied symptoms. Despite this variability, the first symptoms typically include memory loss, confusion, personality changes (such as depression and anxiety) and difficulty with coordination and balance.
-
CJD is not known to be spread through normal social contact, the air, sharing food or utensils, or intimate contact. When caring for someone with CJD, you do not need to follow special precautions in your daily life. Common sense healthcare practices, such as washing your hands and using gloves if you come into contact with bodily fluids, are appropriate.
-
There is no known treatment or cure for CJD yet. Management of CJD involves making patients as comfortable as possible. Certain caretaking techniques, lifestyle changes and various medications may improve the quality of life for people with CJD.
-
In 1982, Dr. Stanley B. Prusiner at UCSF identified an infectious agent uniquely made of protein and named it “prion”, a combination of “protein” and “infectious.” Dr. Prusiner received the Nobel Prize in Physiology or Medicine in 1997 for his radical discovery of a new agent that enables prion disease to be spontaneous, genetic and infectious.
-
The UCSF Memory and Aging Center has consulted on the evaluation of people diagnosed with rapidly progressive dementia from all over the world. If your doctor thinks you may have CJD or another rapidly progressive dementia, our specialists can review your medical records and see if a clinic visit is appropriate.
-
Ben ArevaloAutopsy Program Coordinator
-
Kendra BechtelStaff Research Associate
-
Jamie Fong, MS, CGCGenetic Counselor
-
Rosalie Gearhart, MSN, RNAdministrative Nurse
-
Michael Geschwind, MD, PhDAssociate Professor of Neurology
-
Kelly HitchnerAdministrative Manager
-
Iryna V. Lobach, PhDAssistant Professor
-
Bruce L. Miller, MDCenter Director
