Creutzfeldt-Jakob Disease MRI Image

Creutzfeldt-Jakob Disease

Creutzfeldt-Jakob (KROITS-felt YAH-kobe) disease is a degenerative brain disorder that results in dementia and, eventually, death. Symptoms of Creutzfeldt-Jakob disease (CJD) can look like those of other dementia-like brain conditions, such as Alzheimer’s. However Creutzfeldt-Jakob disease normally progresses a lot more quickly.

CJD captured spotlight in the 1990s when some people in the United Kingdom developed a kind of the disease — alternative CJD (vCJD) — after eating meat from diseased cattle. Nevertheless, “traditional” Creutzfeldt-Jakob disease hasn’t been connected to polluted beef.

Although major, CJD is uncommon, and vCJD is the least common kind. Worldwide, there is an estimated one case of CJD detected per million people each year, usually in older adults.

Creutzfeldt-Jakob Disease Symptoms

Creutzfeldt-Jakob disease is marked by quick mental deterioration, normally within a few months. Initial signs and symptoms usually consist of:

  • Stress and anxiety
  • Blurred vision or blindness
  • Sleeping disorders
  • Depression
  • Amnesia
  • Impaired thinking
  • Trouble swallowing
  • Character changes
  • Problem speaking
  • Abrupt, jerky movements

As the disease advances, mental symptoms get worse. Most people ultimately lapse into a coma. Heart failure, breathing failure, pneumonia or other infections are usually the cause of death. Death typically occurs within a year.

In people with the rarer vCJD, psychiatric symptoms might be more popular in the start, with dementia– the loss of the ability to believe, factor and remember — developing later in the disease. In addition, this alternative impacts people at a younger age than traditional CJD does and appears to have a somewhat longer period — 12 to 14 months.


Creutzfeldt-Jakob disease and its variations come from a broad group of human and animal diseases known as transmissible spongiform encephalopathies (TSEs). The name derives from the spongy holes, visible under a microscope, that develop in affected brain tissue.

The cause of Creutzfeldt-Jakob disease and other TSEs appears to be abnormal variations of a kind of protein called a prion. Normally these proteins are safe. But when they’re misshapen, they end up being contagious and can damage normal biological procedures.

How CJD is Transmitted

The risk of CJD is low. The disease cannot be transmitted through coughing or sneezing, touching or sexual contact. The 3 methods it develops are:

  • Sporadically. Many people with traditional CJD establish the disease for no apparent reason. Called spontaneous CJD or sporadic CJD, this type accounts for most of cases.
  • By inheritance. In the United States, about 5 to 10 percent of people with CJD have a family history of the disease or test favorable for a hereditary mutation related to CJD. This type is referred to as familial CJD.
  • By contamination. A small number of people have developed CJD after being exposed to infected human tissue during a medical procedure, such as a cornea or skin transplant. Also, since basic sterilization methods do not ruin abnormal prions, a few people have actually established CJD after undergoing brain surgery with polluted instruments.

Cases of CJD associated to medical treatments are described as iatrogenic CJD. Variant CJD is linked mostly to consuming beef infected with mad cow disease (bovine spongiform encephalopathy or BSE).

Risk Factors

A lot of cases of Creutzfeldt-Jakob disease occur for unidentified reasons, and no risk factors can be recognized. Nevertheless, a couple of aspects appear to be associated with different sort of CJD.

  • Age. Sporadic CJD tends to establish later on in life, normally around age 60. Beginning of familial CJD occurs a little earlier and vCJD has affected people at a much more youthful age, normally in their late 20s.
  • Genes. People with familial CJD have a genetic mutation that causes the disease. The disease is acquired in an autosomal dominant style, which means you have to inherit only one copy of the mutated gene, from either parent, to establish the disease. If you have the mutation, the chance of passing it on to your children is 50 percent.
    Genetic analysis in people with iatrogenic and vCJD suggest that acquiring identical copies of certain variations of the prion gene might increase your risk of establishing CJD if you’re exposed to polluted tissue.
  • Direct exposure to polluted tissue. People who’ve gotten human growth hormone derived from human pituitary glands or who’ve had grafts of tissue that covers the brain (dura mater) may be at risk of iatrogenic CJD.
    The risk of contracting vCJD from eating infected beef is tough to determine. In general, if countries are effectively implementing public health measures, the risk is virtually nonexistent.

CJD Complications

Just like other causes of dementia, Creutzfeldt-Jakob disease exceptionally impacts the brain as well as the body, although CJD and its variants generally progress far more rapidly. People with CJD generally withdraw from friends and family and eventually lose the ability to acknowledge or connect to them. They likewise lose the capability to care for themselves and lots of ultimately slip into a coma. The disease ultimately is fatal.

How Is Creutzfeldt-Jakob Disease Prevented?

There is no known method to prevent sporadic CJD If you have a family history of neurological disease, you might gain from talking with a genetics therapist, who can assist you arrange through the risks associated with your circumstance.

1. Preventing iatrogenic CJD

Healthcare facilities and other medical institutions follow explicit policies to prevent iatrogenic CJD. These measures have included:

  • Unique use of synthetic human development hormone, rather than the kind derived from human pituitary glands
  • Destruction of surgical instruments used on the brain or nervous tissue of somebody with known or suspected CJD
  • Single-use sets for back taps (lumbar leaks).

To assist ensure the safety of the blood supply, people with a risk of direct exposure to CJD or vCJD aren’t eligible to contribute blood. This consists of people who:

  • Spent 5 years or more in France since 1980.
  • Have received a dura mater brain graft.
  • Have injected bovine insulin given that 1980.
  • Have actually gotten human development hormone.
  • Gotten a blood transfusion in the U.K. given that 1980.
  • Have a biological loved one who has actually been detected with CJD.
  • Spent a minimum of 3 months in the United Kingdom from 1980 to 1996.

2. Preventing vCJD

The risk of contracting vCJD in the United States remains incredibly low. Only 3 cases have been reported in the United States. According to the Centers for Disease Control and Prevention, strong proof recommends that these cases were acquired abroad — two in the United Kingdom and one in Saudi Arabia.

In the United Kingdom, where the majority of vCJD cases have actually occurred, fewer than 200 cases have actually been reported. CJD occurrence peaked between 1999 and 2000 and has actually been decreasing since.

3. Managing prospective sources of vCJD

The majority of nations have taken actions to prevent BSE-infected tissue from going into the food supply, consisting of:

  • Tight restrictions on importation of cattle from countries where BSE is common.
  • Constraints on animal feed.
  • Stringent treatments for handling sick animals.
  • Surveillance and testing approaches for tracking livestock health.
  • Limitations on which parts of livestock can be processed for food.

How Is Creutzfeldt-Jakob Disease Identified?

Just a brain biopsy or an examination of brain tissue after death (autopsy) can confirm the presence of Creutzfeldt-Jakob disease. However doctors often can make a precise medical diagnosis based on your medical and individual history, a neurological examination, and specific diagnostic tests.

The examination is most likely to reveal particular symptoms such as muscle twitching and spasms, unusual reflexes, and coordination issues. People with CJD likewise might have areas of blindness and modifications in visual-spatial understanding.

In addition, doctors frequently use these tests to assist identify CJD:

  • Electroencephalogram (EEG). Using electrodes placed on your scalp, this test measures your brain’s electrical activity. People with CJD and vCJD show a characteristically unusual pattern.
  • MRI. This imaging strategy uses radio waves and an electromagnetic field to create cross-sectional images of your head and body. It’s specifically useful in detecting brain conditions since of its high-resolution images of the brain’s white and gray matter.
  • Spinal fluid tests. Cerebral back fluid surrounds and cushions your brain and spinal cord. In a test called a lumbar puncture — commonly referred to as a spinal tap– doctors use a needle to withdraw a small amount of this fluid for testing. The presence of a specific protein in back fluid is often an indicator of CJD or vCJD.

Creutzfeldt-Jakob Disease Treatment

No efficient treatment exists for Creutzfeldt-Jakob disease or any of its variants. A variety of drugs have actually been evaluated– including steroids, antibiotics and antiviral representatives– and have not shown advantages. Because of that, physicians concentrate on minimizing pain and other symptoms and on making people with these diseases as comfortable as possible.

Last modified: February 19, 2018


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