Definition

What is Creutzfeldt-Jakob disease?

Creutzfeldt-Jakob disease is a degenerative brain disorder that leads to dementia and, ultimately, death. Symptoms of Creutzfeldt-Jakob disease (CJD) can resemble those of other dementia-like brain disorders, such as Alzheimer’s. But Creutzfeldt-Jakob disease usually progresses much more rapidly.

How common is Creutzfeldt-Jakob disease?

It affects about one person in every one million people per year worldwide. CJD usually appears in later life and runs a rapid course. CJD usually appears in later life and runs a rapid course. Typically, onset of symptoms occurs about age 60, and about 90 percent of individuals die within 1 year. Please discuss with your doctor for further information.

Symptoms

What are the symptoms of Creutzfeldt-Jakob disease?

The common symptoms of Creutzfeldt-Jakob disease are:

  • Personality changes
  • Anxiety
  • Depression
  • Memory loss
  • Impaired thinking
  • Blurred vision or blindness
  • Insomnia
  • Difficulty speaking
  • Difficulty swallowing
  • Sudden, jerky movements

As the disease progresses, mental symptoms worsen. Most people eventually lapse into a coma. Heart failure, respiratory failure, pneumonia or other infections are generally the cause of death. Death usually occurs within a year.

In people with the rarer vCJD, psychiatric symptoms may be more prominent in the beginning, with dementia — the loss of the ability to think, reason and remember — developing later in the illness. In addition, this variant affects people at a younger age than classic CJD does and appears to have a slightly longer duration — 12 to 14 months.

There may be some symptoms not listed above. If you have any concerns about a symptom, please consult your doctor.

When should I see my doctor?

If you have any signs or symptoms listed above or have any questions, please consult with your doctor. Everyone’s body acts differently. It is always best to discuss with your doctor what is best for your situation.

Causes

What causes Creutzfeldt-Jakob disease?

CJD appears to be caused by an abnormal infectious protein called a prion. These prions accumulate at high levels in the brain and cause irreversible damage to nerve cells, resulting in the symptoms described above.

While the abnormal prions are technically infectious, they’re very different to viruses and bacteria. For example, prions aren’t destroyed by the extremes of heat and radiation used to kill bacteria and viruses, and antibiotics or antiviral medicines have no effect on them.

Risk factors

What increases my risk for Creutzfeldt-Jakob disease?

There are many risk factors for Creutzfeldt-Jakob disease, such as:

  • Sporadic CJD tends to develop later in life, usually around age 60. Onset of familial CJD occurs slightly earlier and vCJD has affected people at a much younger age, usually in their late 20s.
  • People with familial CJD have a genetic mutation that causes the disease. The disease is inherited in an autosomal dominant fashion, which means you need to inherit only one copy of the mutated gene, from either parent, to develop 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 inheriting identical copies of certain variants of the prion gene may increase your risk of developing CJD if you’re exposed to contaminated tissue.
  • Exposure to contaminated tissue. People who’ve received 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 contaminated beef is difficult to determine. In general, if countries are effectively implementing public health measures, the risk is virtually nonexistent.

Diagnosis & treatment

The information provided is not a substitute for any medical advice. ALWAYS consult with your doctor for more information.

How is Creutzfeldt-Jakob disease diagnosed?

Only a brain biopsy or an examination of brain tissue after death (autopsy) can confirm the presence of Creutzfeldt-Jakob disease. But doctors often can make an accurate diagnosis based on your medical and personal history, a neurological exam, and certain diagnostic tests.

The exam is likely to reveal characteristic symptoms such as muscle twitching and spasms, abnormal reflexes, and coordination problems. People with CJD also may have areas of blindness and changes in visual-spatial perception.

In addition, doctors commonly use these tests to help detect 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 abnormal pattern.
  • This imaging technique uses radio waves and a magnetic field to create cross-sectional images of your head and body. It’s especially useful in diagnosing brain disorders because of its high-resolution images of the brain’s white and gray matter.
  • Spinal fluid tests. Cerebral spinal fluid surrounds and cushions your brain and spinal cord. In a test called a lumbar puncture — popularly known as a spinal tap — doctors use a needle to withdraw a small amount of this fluid for testing. The presence of a particular protein in spinal fluid is often an indication of CJD or vCJD.

How is Creutzfeldt-Jakob disease treated?

No effective treatment exists for Creutzfeldt-Jakob disease or any of its variants. A number of drugs have been tested — including steroids, antibiotics and antiviral agents — and have not shown benefits. For that reason, doctors focus on alleviating pain and other symptoms and on making people with these diseases as comfortable as possible.

Lifestyle changes & home remedies

What are some lifestyle changes or home remedies that can help me manage Creutzfeldt-Jakob disease?

The following lifestyles and home remedies might help you cope with Creutzfeldt-Jakob disease:

There is no known way to prevent sporadic CJD. If you have a family history of neurological disease, you may benefit from talking with a genetics counselor, who can help you sort through the risks associated with your situation.

If you have any questions, please consult with your doctor to better understand the best solution for you.

Hello Health Group does not provide medical advice, diagnosis or treatment.

Review Date: August 14, 2017 | Last Modified: August 14, 2017

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