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Creutzfeldt-Jakob Disease

Creutzfeldt-Jakob disease (CJD) is a rare, degenerative and fatal brain disorder caused by an infectious agent known as a "prion." Typically, the disease occurs at about age 60 and 90 percent of patients diagnosed with CJD die within a year.

In the early stages, symptoms may include failing memory, behavioral changes, lack of coordination and vision problems.

As the illness progresses, mental deterioration becomes more severe and involuntary movements, blindness, weakness in the arms and legs and then a coma may occur.

CJD may occur in three ways:

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  • Inherited, when there's a family history or a genetic mutation associated with the disease. About 5 to 10 percent of cases in the United States are hereditary.
  • Sporadically without apparent cause and no known risk factors. This is the common form of Creutzfeldt-Jakob disease and accounts for about 85 percent of cases.
  • Transmitted through infection when exposed to brain or nervous system tissue, usually through medical procedures.

There is no evidence that Creutzfeldt-Jakob disease is contagious or that it can be spread through casual contact with a patient. Since 1920, less than 1 percent of the reported cases were acquired through infection.

The disease is part of a family of diseases known as transmissible spongiform encephalopathies (TSE). Spongiform refers to the appearance of the infected brain, which becomes filled with holes until it resembles a sponge under a microscope. CJD is the most common of these diseases. Other forms occur in animals such as bovine spongiform encephalopathy (BSE), which develops in cows and often is referred to as "mad cow" disease. Similar diseases occur in cats, elk, deer, goats, sheep and exotic zoo animals.

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Creutzfeldt-Jakob disease (CJD) affects many areas of the brain. At UCSF, CJD is sometimes called the "great mimicker" because it causes symptoms that occur in many other neurological diseases. First symptoms vary widely and may include the following:

  • Behavioral and personality changes
  • Confusion and memory problems
  • Depression
  • Insomnia
  • Lack of coordination
  • Strange physical sensations
  • Vision problems

As the disease advances, patients may experience a rapidly progressive dementia and in most cases involuntary and irregular jerking movements called myoclonus.

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Creutzfeldt-Jakob disease (CJD) is typically a rapidly progressive dementia. Early diagnosis is important because the underlying cause of the dementia may be treatable.

If CJD is suspected, you may undergo a series of tests. Your doctor will conduct a neurological examination and other tests such as a spinal tap to rule out more common and treatable forms of dementia and an electroencephalogram (EEG) to record the brain's electrical pattern, which can identify a specific abnormality that sometimes occurs in CJD.

Computerized tomography (CT) of the brain can help rule out the possibility that symptoms are caused by other problems such as a stroke or a brain tumor.

One of the most effective diagnostic tools is a magnetic resonance imaging (MRI) brain scan, which can reveal patterns of abnormal brain signals characteristic of CJD.

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Currently, there is no cure for Creutzfeldt-Jakob disease (CJD). Researchers have tested many drugs, including acyclovir, amantidine, antibiotics, antiviral agents, interferon and steroids. None has shown consistent benefit.

Treatment is aimed at alleviating symptoms and making the patient as comfortable as possible. Drugs can help relieve pain if it occurs. The drugs clonazepam and sodium valproate may help relieve myoclonus or irregular, jerking movements.

Researchers at the UCSF Memory and Aging Center are trying to identify compounds for treatment or a cure for CJD and other diseases caused by the infectious particles called prions.

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Reviewed by health care specialists at UCSF Medical Center.

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UCSF Clinics & Centers

Neurology and Neurosurgery

Memory and Aging Center
1500 Owens St., Suite 320
San Francisco, CA 94158
Phone: (415) 353-2057
Fax: (415) 476-4800
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